● Catecholamines, released by sympathetic stimulation such as
Management of Patients with
Complications from Heart
Disease
Cardiac Hemodynamics
● The basic function of the heart is to pump blood.
● CO is determined by measuring the heart rate (HR) and
multiplying it by the stroke volume (SV), which is the
amount of blood pumped out of the ventricle with each
contraction. CO usually is calculated using the equation
CO = HR × SV.
● When SV falls, the nervous system is stimulated to increase
HR and thereby maintain adequate CO.
● SV depends on three factors: preload, afterload, and
contractility.
Preload
● Preload is the amount of myocardial stretch just before
systole caused by the pressure created by the volume of blood
within the ventricle.
● The major factor that determines preload is venous return, the
volume of blood that enters the ventricle during diastole.
● Another factor that determines preload is ventricular
compliance, which is the elasticity or amount of “give” when
blood enters the ventricle.
● Elasticity is decreased when the muscle thickens, as in
hypertrophic cardiomyopathy or when there is increased
fibrotic tissue within the ventricle.
● Fibrotic tissue replaces dead cells, such as after a myocardial
infarction.
● Fibrotic tissue has little compliance,making the ventricle stiff.
Given the same volume of blood, a noncompliant ventricle
has a higher intraventricular pressure than a compliant one.
The higher pressure increases the workload of the heart and
can lead to heart failure (HF).
Afterload
● Afterload refers to the amount of resistance to the ejection of
blood from the ventricle. To eject blood, the ventricle must
overcome this resistance.
● Afterload is inversely related to SV. The major factors that
determine afterload are the diameter and distensibility of
the great vessels (aorta and pulmonary artery) and the
opening and competence of the semilunar valves
(pulmonic and aortic valves).
● The more open the valves, the lower the resistance. If the
patient has significant vasoconstriction, hypertension, or a
narrowed opening from a stenotic valve, resistance (afterload)
increases.
● When afterload increases, the workload of the heart must
increase to overcome the resistance and eject blood.
Contractility
● Contractility, which refers to the force of contraction, is
related to the number and status of myocardial cells.
exercise or from administration of positive inotropic
medications, can increase contractility and stroke volume.
● MI causes necrosis of some myocardial cells, shifting the
workload to the remaining cells.
● Significant loss of myocardial cells can decrease contractility
and cause HF.
● Afterload must be reduced by stress reduction techniques or
medications to match the lower contractility.
Heart Failure
● HF, often referred to as congestive heart failure (CHF), is
the inability of the heart to pump sufficient blood to meet the
needs of the tissues for oxygen and nutrients.
● The Agency for Health Care Policy and Research (AHCPR)
HF guidelines panel (1994) defined HF as a clinical
syndrome characterized by signs and symptoms of fluid
overload or of inadequate tissue perfusion.
● These signs and symptoms result when the heart is unable to
generate a CO sufficient to meet the body’s demands.
● The HF guideline panel used the term heart failure because
many patients with HF do not manifest pulmonary or
systemic congestion.
● The term HF is preferred and indicates myocardial heart
disease in which there is a problem with contraction of the
heart (systolic dysfunction) or filling of the heart (diastolic
dysfunction) and which may or may not cause pulmonary or
systemic congestion.
● Some cases of HF are reversible, depending on the cause.
● Most often, HF is a life-long diagnosis that is managed with
lifestyle changes and medications to prevent acute congestive
episodes.
● CHF is usually an acute presentation of HF.
Chronic Heart Failure
● The incidence of HF increases with age.
● The most common reason for hospitalization of people older
than age 65.
● Medical management is based on the type, severity, and cause
of HF.
● There are two types of HF, which are identified by ● Eventually, the myocardial ischemia causes myofibril death,
assessment of left ventricular functioning: an alteration in even in patients without coronary artery disease.
ventricular filling (diastolic heart failure) and an alteration ● The compensatory mechanisms of HF have been called the
in ventricular contraction (systolic heart failure). “vicious cycle of HF” because the heart does not pump
● An assessment of the ejection fraction (EF) is performed to sufficient blood to the body, which causes the body to
assist in determining the type of HF. stimulate the heart to work harder; the heart is unable to
● EF is the percentage of the end-diastolic blood volume in the respond and failure becomes worse.
ventricle minus the end-systolic blood volume in the ventricle ● Diastolic HF develops because of continued increased
divided by the end-diastolic blood volume in the workload on the heart, which responds by increasing the
ventricle—an indication of the amount of blood that was number and size of myocardial cells (ie, ventricular
ejected and the contractile ability of the ventricle. hypertrophy and altered myocellular functioning).
● The EF is normal in diastolic HF, whereas the EF is less than ● These responses cause resistance to ventricular filling, which
40% in systolic HF. The severity of HF is frequently increases ventricular filling pressures despite a normal or
classified according to the patient’s symptoms. reduced blood volume. Less blood in the ventricles causes
decreased CO. The low CO and high ventricular filling
Pathophysiology pressures cause the same neurohormonal responses as
described for systolic HF.
● HF results from a variety of cardiovascular diseases but leads
to some common heart abnormalities that result in decreased
contraction (systole), decreased filling (diastole), or both.
● Significant myocardial dysfunction most often occurs before
the patient experiences signs and symptoms of HF.
● Systolic HF decreases the amount of blood ejected from the
ventricle, which stimulates the sympathetic nervous system to
release epinephrine and norepinephrine.
● The purpose of this initial response is to support the failing
myocardium, but the continued response causes loss of
beta1-adrenergic receptor sites (downregulation) and further
damage to the heart muscle cells.
● The sympathetic stimulation and the decrease in renal
perfusion by the failing heart cause the release of renin by Etiology
the kidney. Renin promotes the formation of angiotensin I, a
● Myocardial dysfunction is most often caused by coronary
benign, inactive substance.
artery disease, cardiomyopathy, hypertension, or valvular
● Angiotensin-converting enzyme (ACE) in the lumen of
disorders.
blood vessels converts angiotensin I to angiotensin II, a
● Atherosclerosis of the coronary arteries is the primary cause
vasoconstrictor that also causes the release of aldosterone.
of HF.
● Aldosterone promotes sodium and fluid retention and
● Coronary artery disease is found in more than 60% of the
stimulates the thirst center. Aldosterone causes additional
patients with HF. Ischemia causes myocardial dysfunction
detrimental effects to the myocardium and exacerbates
because of resulting hypoxia and acidosis from the
myocardial fibrosis.
accumulation of lactic acid.
● Angiotensin, aldosterone, and other neurohormones (eg, atrial
● Myocardial infarction causes focal heart muscle necrosis, the
natriuretic factor, endothelin, and prostacyclin) lead to an
death of heart muscle cells, and a loss of contractility; the
increase in preload and afterload, which increases stress on
extent of the infarction correlates with the severity of HF.
the ventricular wall, causing an increase in the workload of
● Revascularization of the coronary artery by a percutaneous
the heart.
coronary intervention or by coronary artery bypass surgery
● As the heart’s workload increases, contractility of the
may correct the underlying cause so that HF is resolved.
myofibrils decreases. Decreased contractility results in an
● CARDIOMYOPATHY is a disease of the myocardium.
increase in end-diastolic blood volume in the ventricle,
There are three types: dilated, hypertrophic, and restrictive.
stretching the myofibers and increasing the size of the
● Dilated cardiomyopathy, the most common type of
ventricle (ventricular dilation).
cardiomyopathy, causes diffuse cellular necrosis, leading to
● The increased size of the ventricle further increases the stress
decreased contractility (systolic failure).
on the ventricular wall, adding to the workload of the heart.
ー Dilated cardiomyopathy can be idiopathic (unknown
● One way the heart compensates for the increased workload is
cause), or it can result from an inflammatory process, such
to increase the thickness of the heart muscle (ventricular
as myocarditis, from pregnancy, or from a cytotoxic agent,
hypertrophy). However, the hypertrophy is not accompanied
such as alcohol or adriamycin.
by an adequate increase in capillary blood supply, resulting in
● Hypertrophic cardiomyopathy and restrictive
myocardial ischemia.
cardiomyopathy lead to decreased distensibility and
● The sympathetic-induced coronary artery vasoconstriction,
ventricular filling (diastolic failure).
increased ventricular wall stress, and decreased mitochondrial
● Usually, HF due to cardiomyopathy becomes chronic.
energy production also lead to myocardial ischemia.
However, cardiomyopathy and HF may resolve after the end
of pregnancy or with the cessation of alcohol ingestion.
● Systemic or pulmonary hypertension increases afterload
(resistance to ejection), which increases the workload of the
heart and leads to hypertrophy of myocardial muscle fibers;
this can be considered a compensatory mechanism because it
increases contractility.
● However, the hypertrophy may impair the heart’s ability to
fill properly during diastole.
● Valvular heart disease is also a cause of HF. The valves
ensure that blood flows in one direction. With valvular
dysfunction, blood has increasing difficulty moving forward,
increasing pressure within the heart and increasing cardiac
workload, leading to diastolic HF.
● Several systemic conditions contribute to the development
and severity of HF, including increased metabolic rate (eg,
fever, thyrotoxicosis), iron overload (eg, from
hemochromatosis), hypoxia, and anemia (serum hematocrit
less than 25%).
● All of these conditions require an increase in CO to satisfy
the systemic oxygen demand.
● Hypoxia or anemia also may decrease the supply of oxygen
to the myocardium.
● Cardiac dysrhythmias may cause HF, or they may be a result
of HF; either way, the altered electrical stimulation impairs
the myocardial contraction and decreases the overall
efficiency of myocardial function.
● Other factors, such as acidosis (respiratory or metabolic),
electrolyte abnormalities, and antiarrhythmic medications,
can worsen the myocardial dysfunction.
Clinical Manifestations
● The clinical manifestations produced by the different types of
HF (systolic, diastolic, or both) are similar and therefore do
not assist in differentiating the types of HF.
● The signs and symptoms of HF are most often described in
terms of the effect on the ventricles.
● Left-sided heart failure (left ventricular failure) causes
different manifestations than right-sided heart failure (right
ventricular failure).
● Chronic HF produces signs and symptoms of failure of both
ventricles. Although dysrhythmias (especially tachycardias,
ventricular ectopic beats, or atrioventricular [AV] and
ventricular conduction defects) are common in HF, they may
also be a result of treatments used in HF (eg, side effect of
digitalis).
Left-Sided Heart Failure
● Pulmonary congestion occurs when the left ventricle cannot
pump the blood out of the ventricle to the body.
● The increased left ventricular end-diastolic blood volume
increases the left ventricular end-diastolic pressure, which
decreases blood flow from the left atrium into the left
ventricle during diastole.
● The blood volume and pressure in the left atrium increases,
which decreases blood flow from the pulmonary vessels.
● Pulmonary venous blood volume and pressure rise, forcing
fluid from the pulmonary capillaries into the pulmonary
tissues and alveoli, which impairs gas exchange.
● These effects of left ventricular failure have been referred to
as backward failure.
● The clinical manifestations of pulmonary venous congestion
include dyspnea, cough, pulmonary crackles, and
lower-than-normal oxygen saturation levels. An extra heart
sound, S3, may be detected on auscultation.
● Dyspnea, or shortness of breath, may be precipitated by
minimal to moderate activity (dyspnea on exertion [DOE]);
dyspnea also can occur at rest.
● The patient may report orthopnea, difficulty in breathing
when lying flat. Patients with orthopnea usually prefer not to
lie flat. They may need pillows to prop themselves up in bed,
or they may sit in a chair and even sleep sitting up.
● Some patients have sudden attacks of orthopnea at night, a
condition known as paroxysmal nocturnal dyspnea (PND).
● Fluid that accumulated in the dependent extremities during
the day begins to be reabsorbed into the circulating blood
volume when the person lies down.
● Because the impaired left ventricle cannot eject the increased
circulating blood volume, the pressure in the pulmonary
circulation increases, causing further shifting of fluid into the
alveoli.
● The fluid filled alveoli cannot exchange oxygen and carbon
dioxide. Without sufficient oxygen, the patient experiences
dyspnea and has difficulty getting an adequate amount of
sleep.
● The cough associated with left ventricular failure is initially
dry and nonproductive. Most often, patients complain of a
dry hacking cough that may be mislabeled as asthma or
chronic obstructive pulmonary disease (COPD). The cough
may become moist. Large quantities of frothy sputum, which
is sometimes pink (blood tinged), may be produced, usually
indicating severe pulmonary congestion (pulmonary edema).
● Adventitious breath sounds may be heard in various lobes of
the lungs. Usually, bi-basilar crackles that do not clear with
coughing are detected in the early phase of left ventricular
failure. As the failure worsens and pulmonary congestion
increases, crackles may be auscultated throughout all lung
fields. At this point, a decrease in oxygen saturation may
occur.
● In addition to increased pulmonary pressures that cause
decreased oxygenation, the amount of blood ejected from the
left ventricle may decrease, sometimes called forward
failure.
● The dominant feature in HF is inadequate tissue perfusion.
The diminished CO has widespread manifestations because
not enough blood reaches all the tissues and organs (low
perfusion) to provide the necessary oxygen. The decrease in
 SV can also lead to stimulation of the sympathetic nervous
system, which further impedes perfusion to many organs.
● Blood flow to the kidneys decreases, causing decreased
perfusion and reduced urine output (oliguria).
● Renal perfusion pressure falls, which results in the release of
renin from the kidney.
● Release of renin leads to aldosterone secretion.
● Aldosterone secretion causes sodium and fluid retention,
which further increases intravascular volume.
● However, when the patient is sleeping, the cardiac workload
is decreased, improving renal perfusion, which then leads to
frequent urination at night (nocturia).
● Decreased CO causes other symptoms. Decreased
gastrointestinal perfusion causes altered digestion.
● Decreased brain perfusion causes dizziness, lightheadedness,
confusion, restlessness, and anxiety due to decreased
oxygenation and blood flow.
● As anxiety increases, so does dyspnea, enhancing anxiety and
creating a vicious cycle.
● Stimulation of the sympathetic system also causes the
peripheral blood vessels to constrict, so the skin appears pale
or ashen and feels cool and clammy.
● The decrease in the ejected ventricular volume causes the
sympathetic nervous system to increase the heart rate
(tachycardia), often causing the patient to complain of
palpitations.
● The pulses become weak and thready. Without adequate CO,
the body cannot respond to increased energy demands, and
the patient is easily fatigued and has decreased activity
tolerance.
● Fatigue also results from the increased energy expended in
breathing and the insomnia that results from respiratory
distress, coughing, and nocturia.
Right-Sided Heart Failure
● When the right ventricle fails, congestion of the viscera and
the peripheral tissues predominates. This occurs because the
right side of the heart cannot eject blood and cannot
accommodate all the blood that normally returns to it from
the venous circulation.
● The increase in venous pressure leads to jugular vein
distention (JVD).
● The clinical manifestations that ensue include edema of the
lower extremities (dependent edema), hepatomegaly
(enlargement of the liver), distended jugular veins, ascites
(accumulation of fluid in the peritoneal cavity), weakness,
anorexia and nausea, and paradoxically, weight gain due to
retention of fluid
● Edema usually affects the feet and ankles, worsening when
the patient stands or dangles the legs.
● The swelling decreases when the patient elevates the legs.
● The edema can gradually progress up the legs and thighs and
eventually into the external genitalia and lower trunk.
● Edema in the abdomen, as evidenced by increased abdominal
girth, may be the only edema present.
● Sacral edema is not uncommon for patients who are on bed
rest, because the sacral area is dependent. Pitting edema, in
which indentations in the skin remain after even slight
compression with the fingertips, is obvious only after
retention of at least 4.5 kg (10 lb) of fluid (4.5 liters).
● Hepatomegaly and tenderness in the right upper quadrant of
the abdomen result from venous engorgement of the liver.
● The increased pressure may interfere with the liver’s ability
to perform (secondary liver dysfunction).
● As hepatic dysfunction progresses, pressure within the portal
vessels may rise enough to force fluid into the abdominal
cavity, a condition known as ascites. This collection of fluid
in the abdominal cavity may increase pressure on the stomach
and intestines and cause gastrointestinal distress.
● Hepatomegaly may also increase pressure on the diaphragm,
causing respiratory distress.
● Anorexia (loss of appetite) and nausea or abdominal pain
results from the venous engorgement and venous stasis within
the abdominal organs. The weakness that accompanies
right-sided HF results from reduced CO, impaired circulation,
and inadequate removal of catabolic waste products from the
tissues.
Assessment and Diagnostic Findings
● HF may go undetected until the patient presents with signs
and symptoms of pulmonary and peripheral edema
(congestion), which can lead the physician to make a
preliminary diagnosis of CHF.
● However, the physical signs that suggest HF may also occur
with other diseases, such as renal failure, liver failure,
oncologic conditions, and COPD.
● If further assessment and evaluation are not completed, these
patients may be treated for HF inappropriately.
● The term congestive heart failure (CHF) means the patient
has a fluid overload condition (congestion) that may or may
not be caused by HF.
● CHF is caused by HF when ventricular dysfunction (systolic,
diastolic, or both) has been identified.
● Assessment of ventricular function is an essential part of the
initial diagnostic workup
● An echocardiogram is usually performed to confirm the
diagnosis of HF, assist in the identification of the underlying
cause, and determine the patient’s ejection fraction, which
assists in identification of the type and severity of HF.
● A chest x-ray and an electrocardiogram (ECG) are obtained
to assist in the diagnosis and to determine the underlying
cause of HF.
● Laboratory studies usually completed in the initial workup
include serum electrolytes, blood urea nitrogen (BUN),
creatinine, B-type natriuretic peptide (BNP),
thyroid-stimulating hormone (TSH), a complete blood cell
count (CBC), and routine urinalysis.
● The results of these laboratory studies assist in determining
the underlying cause and in establishing a baseline from
which to measure effects of treatment. Exercise testing or
cardiac catheterization may be performed to determine
whether coronary artery disease and cardiac ischemia are
causing the HF.
● Ventricular function should be determined before discharge
from a hospital of patients with acute myocardial infarction
(MI) who are at risk for the development of HF.
● Patients who are at low risk for HF are those who meet all of
the following criteria: no previous myocardial infarction,
inferior myocardial infarction, small (less than two to four
times normal) increase in cardiac enzymes, no Q waves on
the ECG, and an uncomplicated clinical course.
● Evaluation of ventricular function may also be performed for
patients whose initial assessment of HF suggested noncardiac
causes but who failed to respond to treatment.
Medical Management
● A critical step in the management of HF is early identification
and documentation of the type of HF. Medical management,
especially the pharmacologic therapy, varies with the type of
HF. The basic objectives in treating patients with HF are the
following:
ー Eliminate or reduce any etiologic contributory factors,
especially those that may be reversible, such as atrial
fibrillation or excessive alcohol ingestion.
ー Reduce the workload on the heart by reducing afterload
and preload.
● Managing the patient with HF includes providing general
counseling and education about sodium restriction,
monitoring daily weights and other signs of fluid retention,
encouraging regular exercise, and recommending avoidance
of excessive fluid intake, alcohol, and smoking.
● Medications are prescribed based on the patient’s type and
severity of HF.
● Oxygen therapy is based on the degree of pulmonary
congestion and resulting hypoxia.
● Some patients may need supplemental oxygen therapy only
during activity. Others may require hospitalization and
endotracheal intubation.
● If the patient has underlying coronary artery disease,
coronary artery revascularization with percutaneous
transluminal coronary angioplasty (PTCA) or bypass surgery
may be considered.
● If the patient’s condition is unresponsive to advanced
aggressive medical therapy, innovative therapies, including
mechanical assist devices and transplantation, may be
considered.
Pharmacologic Therapy
● Several medications are indicated for systolic HF.
Medications for diastolic failure depend on the underlying
condition, such as hypertension or valvular dysfunction.
● If the patient is in mild systolic failure, an ACE inhibitor
usually is prescribed. If the patient is unable to continue an
ACE inhibitor (eg, because of development of renal
impairment as evidenced by elevated serum creatinine or
persistent serum potassium levels of 5.5 mEq/L or above), an
angiotensin II receptor blocker (ARB) or hydralazine and
isosorbide dinitrate are considered as part of the treatment
plan.
● A diuretic is added if signs of fluid overload develop.
Digitalis is added to ACE inhibitors if the symptoms
continue. Although previously contraindicated in HF, specific
beta-blockers decrease mortality and morbidity if added to
the initial medications. Spironolactone, a weak diuretic may
also be added for persistent symptoms.
★ Angiotensin-Converting Enzyme Inhibitors.
● ACE inhibitors (ACE-Is) have a pivotal role in the
management of HF due to systolic dysfunction.
● They have been found to relieve the signs and symptoms of
HF and significantly decrease mortality and morbidity (when
used to treat a symptomatic patient) by inhibiting
neurohormonal activation.
● Available as oral and intravenous medications, ACE-Is
promote vasodilation and diuresis by decreasing afterload
and preload.
● By doing so, they decrease the workload of the heart.
● Vasodilation reduces resistance to left ven-tricular ejection of
blood, diminishing the heart’s workload and improving
ventricular emptying.
● In promoting diuresis, ACE-Is decrease the secretion of
aldosterone, a hormone that causes the kidneys to retain
sodium.
● ACE-Is stimulate the kidneys to excrete sodium and fluid
(while retaining potassium), thereby reducing left ventricular
filling pressure and decreasing pulmonary congestion.
● ACE-Is may be the first medication prescribed for patients in
mild failure—patients with fatigue or dyspnea on exertion but
without signs of fluid overload and pulmonary congestion.
● Results from studies to identify the specific dose to achieve
this effect are equivocal, although one large study showed
significant reductions in death and hospitalization with higher
doses.
● However, it is recommended to start at a low dose and
increase every 2 weeks until the optimal dose is achieved and
the patient is hemodynamically stable. The final maintenance
dose depends on the patient’s blood pressure, fluid status,
renal status, and degree of cardiac failure.
 ● Patients receiving ACE-I therapy are monitored for
hypotension, hypovolemia, hyponatremia, and alterations in
renal function, especially if they are also receiving diuretics.
● When to observe for these effects and for how long depends
on the onset, peak, and duration of the medication.
● Hypotension is most likely to develop from ACE-I therapy in
patients older than age 75 and in those with a systolic blood
pressure of 100 mm Hg or less, a serum sodium level of less
than 135 mEq/L, or severe cardiac failure.
● Adjusting the dose or type of diuretic in response to the
patient’s blood pressure and renal function may allow for
continued increases in the dosage of ACE-Is.
● Because ACE-Is cause the kidneys to retain potassium, the
patient who is also receiving a diuretic may not need to take
oral potassium supplements. However, patients receiving
potassium sparing diuretics (which do not cause potassium
loss with diuresis) must be carefully monitored for
hyperkalemia, an increased level of potassium in the blood.
● Before the initiation of the ACE-I, hyperkalemic and
hypovolemic states must be corrected. ACE-Is may be
discontinued if the potassium remains above 5.0 mEq/L or if
the serum creatinine is 3.0 mg/dL and continues to increase.
Other side effects of ACE-Is include a dry, persistent cough
that may not respond to cough suppressants. However, the
cough could also indicate a worsening of ventricular function
and failure. Rarely, the cough indicates angioedema. If
angioedema affects the oropharyngeal area and impairs
breathing, the ACE-I must be stopped immediately.
★ Angiotensin II Receptor Blockers (ARBs)
● Although their action is different than that of ACE-Is, ARBs
(eg, losartan [Cozaar]) have a similar hemodynamic effect as
ACE-Is: lowered blood pressure and lowered systemic
vascular resistance.
● Whereas ACE-Is block the conversion of angiotensin I to
angiotensin II, ARBs block the effects of angiotensin II at the
angiotensin II receptor.
● ACE-Is and ARBs also have similar side effects:
hyperkalemia, hypotension, and renal dysfunction.
● ARBs are usually prescribed when patients are not able to
tolerate ACE-Is.
★ Hydralazine and Isosorbide Dinitrate.
● A combination of hydralazine (Apresoline) and isosorbide
dinitrate (Dilatrate-SR, Isordil, Sorbitrate) may be another
alternative for patients who cannot take ACE-Is.
● Nitrates (eg, isosorbide dinitrate) cause venous dilation,
which reduces the amount of blood return to the heart and
lowers preload.
● Hydralazine lowers systemic vascular resistance and left
ventricular afterload.
● It has also been shown to help avoid the development of
nitrate tolerance. As with ARBs, this combination of
medications is usually used when patients are not able to
tolerate ACE-Is.
★ Beta-Blockers.
● When used with ACE-Is, beta-blockers, such as carvedilol
(Coreg), metoprolol (Lopressor, Toprol), or bisoprolol
(Zebeta), have been found to reduce mortality and morbidity
in NYHA class II or III HF patients by reducing the cytotoxic
effects from the constant stimulation of the sympathetic
nervous system.
● These agents have also been recommended for patients with
asymptomatic systolic dysfunction, such as after acute
myocardial infarction or revascularization to prevent the
onset of symptoms of HF. However, beta-blockers may also
produce many side effects, including exacerbation of HF.
● The side effects are most common in the initial few weeks of
treatment.
● The most frequent side effects are dizziness, hypotension, and
bradycardia.
● To minimize these side effects, staggering the administration
of the beta-blocker with the ACE-I is recommended.
● Because of the side effects, betablockers are initiated only
after stabilizing the patient and ensuring a euvolemic (normal
volume) state.
● They are titrated slowly (every 2 weeks), with close
monitoring at each increase in dose. If the patient develops
symptoms during the titration phase, treatment options
include increasing the diuretic, reducing the dose of ACE-I,
or decreasing the dose of the beta-blocker.
● An important nursing role during titration is educating the
patient about the potential worsening of symptoms during the
early phase of treatment, and that improvement may take
several weeks.
● It is very important that nurses provide support to patients
going through this symptom-provoking phase of treatment.
● Because beta-blockade can cause bronchiole constriction, a
beta1-selective beta-blocker (ie, one that primarily blocks the
beta-adrenergic receptor sites in the heart), such as
metoprolol (Lopressor, Toprol), is recommended for patients
with well-controlled, mild to moderate asthma.
● However, these patients need to be monitored closely for
increased asthma symptoms. Any type of beta-blocker is
contraindicated in patients with severe or uncontrolled
asthma.
★ Diuretics
● Diuretics are medications used to increase the rate of urine
production and the removal of excess extracellular fluid from
the body.
● Of the types of diuretics prescribed for patients with edema
from HF, three are most common: thiazide, loop, and
potassium-sparing diuretics.
● These medications are classified according to their site of
action in the kidney and their effects on renal electrolyte
excretion and reabsorption.
● Thiazide diuretics, such as metolazone (Mykrox,
Zaroxolyn), inhibit sodium and chloride reabsorption mainly
in the early distal tubules. They also increase potassium and
bicarbonate excretion.
● Loop diuretics, such as furosemide (Lasix), inhibit sodium
and chloride reabsorption mainly in the ascending loop of
Henle.
● Patients with signs and symptoms of fluid overload should be
started on a diuretic, a thiazide for those with mild symptoms
or a loop diuretic for patients with more severe symptoms or
with renal insufficiency. Both types of diuretics may be used
for those in severe HF and unresponsive to a single diuretic.
These medications may not be necessary if the patient
 responds to activity recommendations, avoidance of
excessive fluid intake.
● Spironolactone (Aldactone) is a potassium-sparing diuretic
that inhibits sodium reabsorption in the late distal tubule and
collecting duct. It has been found to be effective in reducing
mortality and morbidity in NYHA class III and IV HF
patients when added to ACE-Is, loop diuretics, and digoxin.
● Serum creatinine and potassium levels are monitored
frequently (eg, within the first week and then every 4 weeks)
when this medication is first administered.

● Side effects of diuretics include electrolyte imbalances,

symptomatic hypotension (especially with overdiuresis),
hyperuricemia (causing gout), and ototoxicity. Dosages
depend on the indications, patient age, clinical signs and
symptoms, and renal function.
● Careful patient monitoring and dose adjustments are
necessary to balance the effectiveness with the side effects of
therapy. Diuretics greatly improve the patient’s symptoms,
but they do not prolong life.

★ Digitalis
● The most commonly prescribed form of digitalis for pa-tients
with HF is digoxin (Lanoxin).
● The medication increases the force of myocardial contraction
and slows conduction through the AV node.
● It improves contractility, increasing left ventricular output.
● The medication also enhances diuresis, which removes fluid
and relieves edema. The effect of a given dose of medication
depends on the state of the myocardium, electrolyte and fluid
balance, and renal and hepatic function.
● Although digitalis does not decrease the mortality rate, it is
effective in decreasing the symptoms of systolic HF and in
increasing the patient’s ability to perform activities of daily
living.
● A key concern associated with digitalis therapy is digitalis
toxicity.
● The patient is observed for the effectiveness of digitalis
therapy: lessening dyspnea and orthopnea, decrease in
pulmonary crackles on auscultation, relief of peripheral
edema, weight loss, and increase in activity tolerance.
● The serum potassium level is measured at intervals because
diuresis may have caused hypokalemia.
● The effect of digitalis is enhanced in the presence of
hypokalemia, so digitalis toxicity may occur.
● Serum digoxin levels are obtained once each year or more
frequently if there have been changes in the patient’s
medications, renal function, or symptoms.
★ Calcium Channel Blockers.
● First-generation calcium channel blockers, such as verapamil
(Calan, Isoptin, Verelan), nifedipine (Adalat, Procardia), and
diltiazem (Cardizem, Dilacor, Tiazac), are contraindicated in
patients with systolic dysfunction, although they may be used
in patients with diastolic dysfunction.
● Amlodipine (Norvasc) and felodipine (Plendil),
dihydropyridine calcium channel blockers, cause
vasodilation, reducing systemic vascular resistance.
● They may be used to improve symptoms especially in
patients with nonischemic cardiomyopathy, although they
have no effect on mortality.
★ Other Medications
● Anticoagulants may be prescribed, especially if the patient
has a history of an embolic event or atrial fibrillation or mural
thrombus is present.
● Other medications such as antianginal medications may be
given to treat the underlying cause of HF.
● Nonsteroidal anti-inflammatory drugs (NSAIDs), such as
ibuprofen (Aleve, Advil, Motrin) should be avoided. They
can increase systemic vascular resistance and decrease renal
perfusion, especially in the elderly.
● For similar reasons, use of decongestants should be avoided.
Nutritional Therapy
● A low-sodium (≤ 2 to 3 g/day) diet and avoidance of
excessive amounts of fluid are usually recommended.
● Although it has not been shown to affect the mortality rate,
this recommendation reduces fluid retention and the
symptoms of peripheral and pulmonary congestion.
● The purpose of sodium restriction is to decrease the amount
of circulating volume, which would decrease the need for the
heart to pump that volume.
● A balance needs to be achieved between the ability of the
patient to alter the diet and the amount of medications that are
prescribed.
● Any change in diet needs to be done with consideration of
good nutrition as well as the patient’s likes, dislikes, and
cultural food patterns.
Nursing Management
● The nurse is responsible for administering the medications
and for assessing their beneficial and detrimental effects to
the patient.
● It is the balance of these effects that determines the type and
dosage of pharmacologic therapy.
● Nursing actions to evaluate therapeutic effectiveness include
the following:
ー Keeping an intake and output record to identify a negative
balance (more output than input)
ー Weighing the patient daily at the same time and on the
same scale, usually in the morning after urination;
monitoring for a 2- to 3-lb gain in a day or 5-lb gain in
week
ー Auscultating lung sounds at least daily to detect an
increase or decrease in pulmonary crackles
ー Determining the degree of JVD
ー Identifying and evaluating the severity of dependent
edema
ー Monitoring pulse rate and blood pressure, as well as
monitoring for postural hypotension and making sure that
the patient does not become hypotensive from dehydration
ー Examining skin turgor and mucous membranes for signs
of dehydration
ー Assessing symptoms of fluid overload (eg, orthopnea,
paroxysmal nocturnal dyspnea, and dyspnea on exertion)
and evaluating changes
Monitoring and Managing Potential Complications
● Profuse and repeated diuresis can lead to hypokalemia (ie,
potassium depletion). Signs are weak pulse, faint heart
sounds, hypotension, muscle flabbiness, diminished deep
tendon reflexes, and generalized weakness. Hypokalemia
poses new problems for the patient with HF because it
markedly weakens cardiac contractions.
● In patients receiving digoxin, hypokalemia can lead to
digitalis toxicity. Digitalis toxicity and hypokalemia increase
the likelihood of dangerous dysrhythmias.
● Low levels of potassium may also indicate a low level of
magnesium, which can add to the risk for dysrhythmias.
Hyperkalemia may also occur, especially with the use of
ACE-Is or ARBs and spironolactone.
● Prolonged diuretic therapy may also produce hyponatremia
(deficiency of sodium in the blood), which results in
apprehension, weakness, fatigue, malaise, muscle cramps and
twitching, and a rapid, thready pulse.
● Other problems associated with diuretic administration are
hyperuricemia (excessive uric acid in the blood), volume
depletion from excessive urination, and hyperglycemia.
Nursing Process: the Patient with Heart Failure
★ Assessment
● The nursing assessment for the patient with HF focuses on
observing for effectiveness of therapy and for the patient’s
ability to understand and implement self-management
strategies.
● Signs and symptoms of pulmonary and systemic fluid
overload are recorded and reported immediately so that
adjustments can be made in therapy.
● The nurse also explores the patient’s emotional response to
the diagnosis of HF, a chronic illness.
● Health History
ー The nurse explores sleep disturbances, particularly sleep
suddenly interrupted by shortness of breath.
ー The nurse also asks about the number of pillows needed
for sleep (an indication of orthopnea), activities of daily
living, and the activities that cause shortness of breath.
ー The nurse also explores the patient’s understanding of HF,
the self-management strategies, and the desire to adhere to
those strategies.
ー The nurse helps patients to identify things that they have
lost because of the diagnosis, their emotional response to
that loss, and successful coping skills that they have used
previously.
ー Family and significant others are often included in these
discussions.
 ● Physical Examination
ー The lungs are auscultated to detect crackles and wheezes
or their absence. Crackles, which are produced by the
sudden opening of small airways and alveoli that have
adhered together by edema and exudate, may be heard at
the end of inspiration and are not cleared with coughing.
They may also sound like gurgling that may clear with
coughing or suctioning.
ー The rate and depth of respirations are also documented.
The heart is auscultated for an S3 heart sound, a sign that
the heart is beginning to fail and that increased blood
volume remains in the ventricle with each beat.
ー HR and rhythm are also documented. Rapid rates indicate
that SV has decreased and that the ventricle has less time
to fill, producing some blood stagnation in the atria and
eventually in the pulmonary bed.
ー JVD is also assessed; distention greater than 3 cm above
the sternal angle is considered abnormal. This is an
estimate, not a precise measurement, of central venous
pressure.
ー Sensorium and level of consciousness must be evaluated.
As the volume of blood ejected by the heart decreases, so
does the amount of oxygen transported to the brain.
ー The nurse makes sure that dependent parts of the patient’s
body are assessed for perfusion and edema. With
significant decreases in SV, there is a decrease in perfusion
to the periphery, causing the skin to feel cool and appear
pale or cyanotic.
ー If the patient is sitting upright, the feet and lower legs are
examined for edema; if the patient is supine in bed, the
sacrum and back are assessed for edema. Fingers and
hands may also become edematous.
ー In extreme cases of HF, the patient may develop
periorbital edema, in which the eyelids may swell shut.
ー If neck vein distention increases more than 1 cm, the test
finding is positive for increased venous pressure.
ー If the patient is hospitalized, the nurse measures output
carefully to establish a baseline against which to measure
the effectiveness of diuretic therapy.
ー Intake and output records are rigorously maintained. It is
important to know whether the patient has ingested more
fluid than he or she has excreted (positive fluid balance),
which is then correlated with a gain in weight.
ー The patient must be monitored for oliguria (diminished
urine output, <400 mL/24 hours) or anuria (urine output
<50 mL/24 hours).
ー The patient is weighed daily in the hospital or at home, at
the same time of day, with the same type of clothing, and
on the same scale. If there is a significant change in weight
(ie, 2- to 3-lb increase in a day or 5-lb increase in a week),
the patient is instructed to notify the physician or adjust
the medications (eg, increase the diuretic dose).
★ Diagnosis
● Nursing Diagnosis
ー Based on the assessment data, major nursing diagnoses for
the patient with HF may include the following:
○ Activity intolerance (or risk for activity intolerance)
related to imbalance between oxygen supply and
demand because of decreased CO
○ Excess fluid volume related to excess fluid or sodium
intake and retention of fluid because of HF and its
medical therapy
○ Anxiety related to breathlessness and restlessness from
inadequate oxygenation
○ Powerlessness related to inability to perform role
responsibilities because of chronic illness and
hospitalizations
○ Noncompliance related to lack of knowledge
★ Planning and Goals
● Major goals for the patient may include promoting activity
and reducing fatigue, relieving fluid overload symptoms,
decreasing the incidence of anxiety or increasing the patient’s
ability to manage anxiety, teaching the patient about the
self-care program, and encouraging the patient to verbalize
his or her ability to make decisions and influence outcomes.
★ Nursing Interventions
● Promoting Activity Tolerance
ー Although prolonged bed rest and even short periods of
recumbency promote diuresis by improving renal
perfusion, they also promote decreased activity tolerance.
ー Prolonged bed rest, which may be self-imposed, should be
avoided because of the deconditioning effects and hazards,
such as pressure ulcers (especially in edematous
patients), phlebothrombosis, and pulmonary embolism.
ー An acute event that causes severe symptoms or that
requires hospitalization indicates the need for initial bed
rest. Otherwise, a total of 30 minutes of physical activity
three to five times each week should be encouraged.
ー The nurse and patient can collaborate to develop a
schedule that promotes pacing and prioritization of
activities. The schedule should alternate activities with
periods of rest and avoid having two significant
energy-consuming activities occur on the same day or in
immediate succession.
ー Before undertaking physical activity, the patient should be
given the following safety guidelines:
○ Begin with a few minutes of warm-up activities.
○ Avoid performing physical activities outside in
extreme hot, cold, or humid weather.
○ Ensure that you are able to talk during the physical
activity; if you are unable to do so, decrease the
intensity of activity.
○ Wait 2 hours after eating a meal before performing the
physical activity.
○ Stop the activity if severe shortness of breath, pain, or
dizziness develops.
○ End with cool-down activities and a cool-down period.
ー Because some patients may be severely debilitated, they
may need to perform physical activities only 3 to 5
minutes at a time, one to four times per day. The patient
then should be advised to increase the duration of the
activity, then the frequency, before increasing the intensity
of the activity.
ー Barriers to performing an activity are identified, and
methods of adjusting an activity to ensure pacing but still
accomplish the task are discussed. For example, objects
that need to be taken upstairs can be put in a basket at the
bottom of the stairs throughout the day.
ー At the end of the day, the person can carry the objects up
the stairs all at once. Likewise, the person can carry
 cleaning supplies around in a basket or backpack rather
than walk back and forth to obtain the items.
ー Vegetables can be chopped or peeled while sitting at the
kitchen table rather than standing at the kitchen counter.
ー Small, frequent meals decrease the amount of energy
needed for digestion while providing adequate nutrition.
The nurse helps the patient to identify peak and low
periods of energy and plan energy-consuming activities for
peak periods. For example, the person may prepare the
meals for the entire day in the morning.
ー Pacing and prioritizing activities help maintain the
patient’s energy to allow participation in regular physical
activity. The patient’s response to activities needs to be
monitored.
ー If the patient is hospitalized, vital signs and oxygen
saturation level are monitored before, during, and
immediately after an activity to identify whether they are
within the desired range. Heart rate should return to
baseline within 3 minutes.
ー If the patient tolerates the activity, short-term and
long-term goals can be developed to gradually increase the
intensity, duration, and frequency of activity.
ー Referral to a cardiac rehabilitation program may be
needed, especially for HF patients with recent myocardial
infarction, recent open-heart surgery, or increased anxiety.
ー A supervised program may also benefit those who need
the structured environment, significant educational
support, regular encouragement, and interpersonal cont
● Managing Fluid Volume
ー Patients with severe HF may receive intravenous diuretic
therapy, but patients with less severe symptoms may
receive oral diuretic medication.
ー Oral diuretics should be administered early in the morning
so that diuresis does not interfere with the patient’s
nighttime rest.
ー Discussing the timing of medication administration is
especially important for patients, such as elderly people,
who may have urinary urgency or incontinence.
ー A single dose of a diuretic may cause the patient to excrete
a large volume of fluid shortly after administration.
ー The nurse monitors the patient’s fluid status closely—
auscultating the lungs, monitoring daily body weights, and
assisting the patient to adhere to a low-sodium diet by
reading food labels and avoiding high-sodium foods such
as canned, processed, and convenience foods.
ー If the diet includes fluid restriction, the nurse can assist the
patient to plan the fluid intake throughout the day while
respecting the patient’s dietary preferences.
ー If the patient is receiving intravenous fluids, the amount of
fluid needs to be monitored closely, and the physician or
pharmacist can be consulted about the possibility of
maximizing the amount of medication in the same amount
of intravenous fluid (eg, double-concentrating to decrease
the fluid volume administered).
ー The nurse positions the patient or teaches the patient how
to assume a position that shifts fluid away from the heart.
The number of pillows may be increased, the head of the
bed may be elevated (20- to 30-cm [8- to 10-inch] blocks
may be used), or the patient may sit in a comfortable
armchair.
ー In this position, the venous return to the heart (preload) is
reduced, pulmonary congestion is alleviated, and
impingement of the liver on the diaphragm is minimized.
ー The lower arms are supported with pillows to eliminate the
fatigue caused by the constant pull of their weight on the
shoulder muscles.
ー The patient who can breathe only in the upright position
may sit on the side of the bed with the feet supported on a
chair, the head and arms resting on an overbed table, and
the lumbosacral spine supported by a pillow.
ー If pulmonary congestion is present, positioning the patient
in an armchair is advantageous, because this position
favors the shift of fluid away from the lungs.
ー Because decreased circulation in edematous areas
increases the risk of skin injury, the nurse assesses for skin
breakdown and institutes preventive measures.
ー Frequent changes of position, positioning to avoid
pressure, the use of elastic compression stockings, and leg
exercises may help to prevent skin injury.
● Controlling Anxiety
ー Because patients in HF have difficulty maintaining
adequate oxygenation, they are likely to be restless and
anxious and feel overwhelmed by breathlessness. These
symptoms tend to intensify at night.
ー Emotional stress stimulates the sympathetic nervous
system, which causes vasoconstriction, elevated arterial
pressure, and increased heart rate.
ー By decreasing anxiety, the patient’s cardiac work also is
decreased. Oxygen may be administered during an acute
event to diminish the work of breathing and to increase the
patient’s comfort.
ー When the patient exhibits anxiety, the nurse takes steps to
promote physical comfort and psychological support. In
many cases, a family member’s presence provides
reassurance.
ー To help decrease the patient’s anxiety, the nurse should
speak in a slow, calm, and confident manner and maintain
eye contact.
ー When necessary, the nurse should also state specific, brief
directions for an activity. After the patient is comfortable,
the nurse can begin teaching ways to control anxiety and
to avoid anxiety-provoking situations.
ー The nurse explains how to use relaxation techniques and
assists the patient to identify factors that contribute to
anxiety.
ー Lack of sleep may increase anxiety, which may prevent
adequate rest. Other contributing factors may include
misinformation, lack of information, or poor nutritional
status.
ー Promoting physical comfort, providing accurate
information, and teaching the patient to perform relaxation
techniques and to avoid anxiety triggering situations may
relax the patient.
ー In cases of confusion and anxiety reactions that affect the
patient’s safety, the use of restraints should be avoided.
Restraints are likely to be resisted, and resistance
inevitably increases the cardiac workload.
ー The patient who insists on getting out of bed at night can
be seated comfortably in an armchair. As cerebral and
systemic circulation improves, the degree of anxiety
decreases, and the quality of sleep improves.
● Minimizing Powerlessness
ー Patients need to recognize that they are not helpless and
that they can influence the direction of their lives and the
outcomes of treatment.
ー The nurse assesses for factors contributing to a sense of
powerlessness and intervenes accordingly.
ー Contributing factors may include lack of knowledge and
lack of opportunities to make decisions, particularly if
health care providers and family members behave in
maternalistic or paternalistic ways.
ー If the patient is hospitalized, hospital policies may
promote standardization and limit the patient’s ability to
make decisions (eg, what time to have meals, take
medications, prepare for bed).
ー Taking time to listen actively to patients often encourages
them to express their concerns and ask questions.
ー Other strategies include providing the patient with
decision-making opportunities, such as when activities are
to occur or where objects are to be placed, and increasing
the frequency and significance of those opportunities over
time; providing encouragement while identifying the
patient’s progress; and assisting the patient to differentiate
between factors that can be controlled and those that
cannot.
ー In some cases, the nurse may want to review hospital
policies and standards that tend to promote powerlessness
and advocate for their elimination or change (eg, limited
visiting hours, prohibition of food from home, required
wearing of hospital gowns).
● Promoting Home and Community-Based Care
Teaching Patients Self-Care
ー The nurse provides patient education and involves the
patient in implementing the therapeutic regimen to
promote understanding and adherence to the plan.
ー When the patient understands or believes that the
diagnosis of HF can be successfully managed with
lifestyle changes and medications, recurrences of acute HF
lessen, unnecessary hospitalizations decrease, and life
expectancy increases.
ー Patients and their families need to be taught to follow the
medication regimen as prescribed, maintain a low-sodium
diet, perform and record daily weights, engage in routine
physical activity, and recognize symptoms that indicate
worsening HF. Although noncompliance is not well
understood, interventions that may promote adherence
include teaching to ensure accurate understanding.
ー The patient and family members are supported and
encouraged to ask questions so that information can be
clarified and understanding enhanced.
ー The nurse should be aware of cultural factors and adapt the
teaching plan accordingly.
ー Patients and their families need to be informed that the
progression of the disease is influenced in part by choices
made about health care and the decisions about following
the treatment plan.
ー They also need to be informed that health care providers
are there to assist them in reaching their health care goals.
ー Patients and family members need to make the decisions
about the treatment plan, but they also need to understand
the possible outcomes of those decisions.
ーThe treatment plan then will be based on what the patient
wants, not just what the physician or other health care
team members think is needed. Ultimately, the nurse needs
to convey that monitoring symptoms and daily weights,
restricting sodium intake, avoiding excess fluids,
preventing infection with influenza and pneumococcal
immunizations, avoiding noxious agents (eg, alcohol,
tobacco), and participating in regular exercise all aid in
preventing exacerbations of HF.
★ Evaluation
● Expected Patient Outcomes
ー Demonstrates tolerance for increased activity
○ Describes adaptive methods for usual activities
○ Stops any activity that causes symptoms of intolerance
○ Maintains vital signs (pulse, blood pressure,
respiratory rate, and pulse oximetry) within the
targeted range
○ Identifies factors that contribute to activity intolerance
and takes actions to avoid them
○ Establishes priorities for activities
○ Schedules activities to conserve energy and to reduce
fatigue and dyspnea
ー Maintains fluid balance
○ Exhibits decreased peripheral and sacral edema
○ Demonstrates methods for preventing edema
ー Is less anxious
○ Avoids situations that produce stress
○ Sleeps comfortably at night
○ Reports decreased stress and anxiety
ー Makes decisions regarding care and treatment
○ States ability to influence outcomes
ー Adheres to self-care regimen
○ Performs and records daily weights
○ Ensures dietary intake includes no more than 2 to 3 g
of sodium per day
○ Takes medications as prescribed
○ Reports any unusual symptoms or side effects
Acute Heart Failure (Pulmonary Edema)
● Pulmonary edema is the abnormal accumulation of fluid in
the lungs.
● The fluid may accumulate in the interstitial spaces or in the
alveoli.
Pathophysiology
● Pulmonary edema is an acute event that results from HF. It
can occur acutely, such as with myocardial infarction, or it
can occur as an exacerbation of chronic HF.
● Myocardial scarring as a result of ischemia can limit the
ventricular distensibility and render it vulnerable to a sudden
increase in workload.
● With increased resistance to left ventricular filling, the blood
backs up into the pulmonary circulation.
● The patient quickly develops pulmonary edema, sometimes
called flash pulmonary edema, from the blood volume
overload in the lungs.
● Pulmonary edema can also be caused by non-cardiac
disorders, such as renal failure, liver failure, and oncologic
conditions that cause the body to retain fluid.
● The left ventricle cannot handle the resulting hypervolemia,
preventing blood from easily flowing from the left atrium
into the left ventricle.
● This causes the pressure to increase in the left atrium.
● The increase in atrial pressure may result in an increase in
pulmonary venous pressure, which produces an increase in
hydrostatic pressure that forces fluid out of the pulmonary
capillaries into the interstitial spaces and alveoli.
● Impaired lymphatic drainage also contributes to the
accumulation of fluid in the lung tissues.
● The fluid within the alveoli mixes with air, creating
“bubbles” that are expelled from the mouth and nose,
producing the classic symptom of pulmonary edema, frothy
pink (blood-tinged) sputum.
● Because of the fluid within the alveoli, air cannot enter, and
gas exchange is impaired.
● The result is hypoxemia, which is often severe.
● The onset may be preceded by premonitory symptoms of
pulmonary congestion, but it also may develop quickly in the
patient with a ventricle that has little reserve to meet
increased oxygen needs.
● In pulmonary edema, as well as in HF, preload, contractility,
and afterload may be altered, thereby impairing CO.
● Technological advances (eg, impedance cardiography) have
made it easier to implement effective pharmacologic therapy
in treating acute pulmonary edema.
Clinical Manifestations
● As a result of decreased cerebral oxygenation, the patient
becomes increasingly restless and anxious.
● Along with a sudden onset of breathlessness and a sense of
suffocation, the patient’s hands become cold and moist, the
nail beds become cyanotic (bluish), and the skin turns ashen
(gray).
● The pulse is weak and rapid, and the neck veins are
distended.
● Incessant coughing may occur, producing increasing
quantities of mucoid sputum. As pulmonary edema
progresses, the patient’s anxiety and restlessness increase; the
patient becomes confused, then stuporous. Breathing is rapid,
noisy, and moist sounding. The patient’s oxygen levels
(saturation) are significantly decreased. The patient, nearly
suffocated by the blood-tinged, frothy fluid filling the alveoli,
is literally drowning in secretions. The situation demands
immediate action.
Assessment and Diagnostic Findings
● The diagnosis is made by evaluating the clinical
manifestations resulting from pulmonary congestion.
● Most often, a chest x-ray is obtained to confirm that the
pulmonary veins are engorged.
● Abrupt onset of signs and symptoms of left-sided HF(eg,
crackles on auscultation of the lungs, flash pulmonary edema)
without evidence of right-sided HF (eg, no JVD, no
dependent edema) may indicate diastolic failure due to
ischemia.
Prevention
● Like most complications, pulmonary edema is easier to
prevent than to treat. To recognize it in its early stages, the
nurse auscultates the lung fields and heart sounds, measures
JVD, and assesses the degree of peripheral edema and the
severity of breathlessness.
● A dry, hacking cough; fatigue; weight gain; development or
worsening of edema; and decreased activity tolerance may be
early indicators of developing pulmonary edema.
● In an early stage, the condition may be corrected by placing
the patient in an upright position with the feet and legs
dependent, eliminating overexertion, and minimizing
emotional stress to reduce the left ventricular load.
● A re-examination of the patient’s treatment regimen and the
patient’s understanding of and adherence to it are also
needed. The long-range approach to preventing pulmonary
edema must be directed at identifying its precipitating factors.
Medical Management
● Clinical management of a patient with acute pulmonary
edema due to HF is directed toward improving ventricular
function and increasing respiratory exchange. These goals are
accomplished through a combination of oxygen, medication
therapies, and nursing support.
Pharmacologic Therapy
● Various treatments and medications are prescribed for
pulmonary edema, among them oxygen, morphine, diuretics,
and various intravenous medications.
★ Oxygen Therapy
● Oxygen is administered in concentrations adequate to relieve
hypoxemia and dyspnea.
● Usually, a face mask or non-rebreathing mask is initially
used.
● If respiratory failure is severe or persists despite optimal
management, endotracheal intubation and mechanical
ventilation are required.
● Oxygenation is monitored with pulse oximetry and by
measurement of arterial blood gases.
★ Morphine
● Morphine is administered intravenously in small doses (2 to 5
mg) to reduce peripheral resistance and venous return so that
blood can be redistributed from the pulmonary circulation to
other parts of the body.
● This action decreases pressure in the pulmonary capillaries
and decreases seepage of fluid into the lung tissue.
● The effect of morphine in decreasing anxiety is also
beneficial.
★ Diuretics
● Diuretics promote the excretion of sodium and water by the
kidneys.
● Furosemide (Lasix), for example, is administered
intravenously to produce a rapid diuretic effect.
 ● Furosemide also causes vasodilation and pooling of blood in
peripheral blood vessels, which reduces the amount of blood
returned to the heart, even before the diuretic effect.
● Some physicians may prescribe bumetanide (Bumex) and
metolazone (Mykrox, Zaroxolyn) in place of furosemide
★ Dobutamine
● Dobutamine (Dobutrex) is an intravenous medication given
to patients with significant left ventricular dysfunction.
● A catecholamine, dobutamine stimulates the beta1-adrenergic
receptors. Its major action is to increase cardiac contractility.
However, at higher amounts, it also increases the heart rate
and the incidence of ectopic beats and tachydysrhythmias.
● Because it also increases AV conduction, care must be taken
in patients who have underlying atrial fibrillation.
● A medication that protects the AV node, such as digitalis, a
beta-blocker, or a calcium channel blocker, may be indicated
before dobutamine therapy is initiated to prevent increased
ventricular response rate.
★ Milrinone
● Milrinone (Primacor) is a phosphodiesterase inhibitor that
delays the release of calcium from intracellular reservoirs and
prevents the uptake of extracellular calcium by the cells.
● This promotes vasodilation, decreasing preload and afterload,
reducing the workload of the heart.
● Milrinone is administered intravenously, usually to patients
who have not responded to other therapies.
● It is not usually used to treat patients with renal failure.
● The major side effects are hypotension (usually
asymptomatic), gastrointestinal dysfunction, increased
ventricular dysrhythmias, and decreased platelet counts. The
patient’s blood pressure is monitored closely.
★ Nesiritide
● Nesiritide (Natrecor) is an intravenous medication that is
indicated for acutely decompensated HF.
● Natriuretic peptides are produced by the myocardium as a
compensatory response to increased ventricular end-diastolic
pressure and myocardial wall stress and to the increased
release of neurohormones (eg, norepinephrine, renin,
aldosterone) that occur with HF.
● Nesiritide is a human B-type natriuretic peptide (BNP) made
from Escherichia coli using recombinant technology. Human
BNP binds to vascular smooth muscle and endothelial cells,
causing dilation of arteries and veins and suppression of the
neurohormones. The result is improved stroke volume and
reduced preload and afterload.
● This medication causes rapid improvement in the symptoms
of HF and may be used with other HF medications (eg,
beta-blockers, digoxin). The most common side effect is
dose-related hypotension.
Nursing Management
● Positioning the patient to promote circulation
ー Proper positioning can help reduce venous return to the
heart. The patient is positioned upright, preferably with the
legs dangling over the side of the bed.
ーThis has the immediate effect of decreasing venous return,
lowering the output of the right ventricle, and decreasing
lung congestion.
ー If the patient is unable to sit with the lower extremities
dependent, the patient may be placed in an upright position
in bed.
● Providing psychological support
ー As the ability to breathe decreases, the patient’s sense of
fear and anxiety rises proportionately, making the
condition more severe.
ー Reassuring the patient and providing skillful anticipatory
nursing care are integral parts of the therapy. Because this
patient feels a sense of impending doom and has an
unstable condition, the nurse must remain with the patient.
ー The nurse should give the patient simple, concise
information in a reassuring voice about what is being done
to treat the condition and the expected results.
ー The nurse should also identify any anxiety-inducing
factors (eg, a pet left alone at home, presence of an
unwelcome family member at the bedside, a wallet full of
money) and initiate strategies to eliminate the concern or
reduce its effect.
● Monitoring medications
ー The patient receiving morphine is observed for respiratory
depression, hypotension, and vomiting; a morphine
antagonist, such as naloxone hydrochloride (Narcan), is
kept available and given to the patient who exhibits these
side effects.
ー The patient receiving diuretic therapy may excrete a large
volume of urine within minutes after a potent diuretic is
administered.
ー A bedside commode may be used to decrease the energy
required by the patient and to reduce the resultant increase
in cardiac workload induced by getting on and off a
bedpan. If necessary, an indwelling urinary catheter may
be inserted
Other Complications
Cardiogenic Shock
● Cardiogenic shock occurs when the heart cannot pump
enough blood to supply the amount of oxygen needed by the
tissues.
● This may occur because of one significant or multiple smaller
infarctions in which more than 40% of the myocardium
becomes necrotic, because of a ruptured ventricle, significant
valvular dysfunction, trauma to the heart resulting in
myocardial contusion, or as the end stage of HF.
● It also can occur with cardiac tamponade, pulmonary
embolism, cardiomyopathy, and dysrhythmias.
Pathophysiology
● The signs and symptoms of cardiogenic shock reflect the
circular nature of the pathophysiology of HF.
● The degree of shock is proportional to the extent of left
ventricular dysfunction.
● The heart muscle loses its contractile power, resulting in a
marked reduction in SV and CO, which is sometimes called
forward failure.
● The damage to the myocardium results in a decrease in CO,
which reduces arterial blood pressure and tissue perfusion in
the vital organs (heart, brain, lung, kidneys).
● Flow to the coronary arteries is reduced, resulting in
decreased oxygen supply to the myocardium, which increases
ischemia and further reduces the heart’s ability to pump. The
inadequate emptying of the ventricle also leads to increased
pulmonary pressures, pulmonary congestion, and pulmonary
edema, exacerbating the hypoxia, causing ischemia of vital
organs, and setting a vicious cycle in motion.
Clinical Manifestations
● The classic signs of cardiogenic shock are tissue
hypoperfusion manifested as cerebral hypoxia (restlessness,
confusion, agitation), low blood pressure, rapid and weak
pulse, cold and clammy skin, increased respiratory crackles,
hypoactive bowel sounds, and decreased urinary output.
● Initially, arterial blood gas analysis may show respiratory
alkalosis.
● Dysrhythmias are common and result from a decrease in
oxygen to the myocardium.
Assessment and Diagnostic Findings
● Use of a PA catheter to measure left ventricular pressures and
CO is important in assessing the severity of the problem and
planning management.
● The PA wedge pressure is elevated and the CO decreased as
the left ventricle loses its ability to pump.
● The systemic vascular resistance is elevated because of the
sympathetic nervous system stimulation that occurs as a
compensatory response to the decrease in blood pressure.
● The decreased blood flow to the kidneys causes a hormonal
response (ie, increased catecholamines and activation of the
renin-angiotensin-aldosterone system) that causes fluid
retention and further vasoconstriction. Increases in HR,
circulating volume, and vasoconstriction occur to maintain
circulation to the brain, heart, kidneys, and lungs, but at a
cost: an increase in the workload of the heart
● The reduction in blood volume delivered to the tissues results
in an increase in the amount of oxygen that is extracted from
the blood that is delivered to the tissues (to try to meet the
cellular demand for oxygen).
● The increased systemic oxygen extraction results in
decreased venous (mixed and central) oxygen saturation.
● When the cellular oxygen needs cannot be met by the
systemic oxygen delivery and the oxygen extraction,
anaerobic metabolism and the resulting build up of lactic acid
occur.
● Continuous central venous oximetry and measurement of
blood lactic acid levels may assist in assessing the severity of
the shock as well as the effectiveness of treatment.
● Continued cellular hypoperfusion eventually results in organ
failure.
● The patient becomes unresponsive, severe hypotension
ensues, and the patient develops shallow respirations; cold,
cyanotic or mottled skin; and absent bowel sounds.
● Arterial blood gas analysis shows metabolic acidosis, and all
laboratory test results indicate organ dysfunction.
Medical Management
● The major approach to treating cardiogenic shock is to
correct the underlying problems, reduce any further demand
on the heart, improve oxygenation, and restore tissue
perfusion. For example, if the ventricular failure is the result
of an acute myocardial infarction, emergency percutaneous
coronary intervention may be indicated.
● Ventricular assist devices may be implanted to support the
pumping action of the heart.
● Major dysrhythmias are corrected because they may have
caused or contributed to the shock. If the patient has
hypervolemia, diuresis is indicated. Diuretics, vasodilators,
and mechanical devices, such as filtration (continuous renal
replacement therapy [CRRT]) and dialysis, have been used to
reduce the circulating blood volume.
● If hypovolemia or low intravascular volume is suspected or
detected through pressure readings, the patient is given
intravenous volume expanders (eg, normal saline solution,
lactated Ringer’s solution, albumin) to increase the amount of
circulating fluid.
● The patient is placed on strict bedrest to conserve energy.
● If the patient has hypoxemia, as detected by pulse oximetry
or arterial blood gas analysis, oxygen administration is
increased, often under positive pressure when regular flow is
insufficient to meet tissue demands.
● Intubation and sedation may be necessary to maintain
oxygenation. The settings for mechanical ventilation are
adjusted according to the patient’s oxygenation status and the
need for conserving energy.
Pharmacologic Therapy
● Medication therapy is selected and guided according to CO,
other cardiac parameters, and mean arterial blood pressure.
Because of the decreased perfusion to the gastrointestinal
system and the need to adjust the dosage quickly, most
medications are administered intravenously.
● Vasopressors, or pressor agents, are medications used to raise
blood pressure and increase CO. Many pressor medications
are catecholamines, such as norepinephrine (Levophed) and
highdose (>10 µg/kg per minute) dopamine (Intropin).
● Their purpose is to promote perfusion to the heart and brain,
but they compromise circulation to other organs (eg, kidney).
Because they also tend to increase the workload of the heart
by increasing oxygen demand, they are not administered
early in the cardiogenic shock process.
● Diuretics and vasodilators may be administered carefully to
reduce the workload of the heart as long as they do not cause
worsening of the tissue hypoperfusion.
● Agents such as amrinone (Inocor), milrinone (Primacor),
sodium nitroprusside (Nipride), and nitroglycerin (Tridil) are
effective vasoactive medications that lower the volume
returning to the heart, decrease blood pressure, and decrease
cardiac work.
● They cause the arteries and veins to dilate, thereby shunting
much of the intravascular volume to the periphery and
causing a reduction in preload and afterload
● Positive inotropic medications are given to increase
myocardial contractility.
● Dopamine (Intropin, given at more than 2 µg/kg per minute),
dobutamine (Dobutrex), and epinephrine (Adrenalin) are
catecholamines that increase contractility.
● Each of these can cause tachydysrhythmias because they
increase automaticity with increasing dosage.
● Monitoring baseline HR is therefore important. As the
baseline HR increases, so does the risk of developing
tachydysrhythmias.
Nursing Management
● The patient in cardiogenic shock requires constant monitoring
and intensive care. The critical care (intensive care) nurse
must carefully assess the patient, observe the cardiac rhythm,
monitor hemodynamic parameters, and record fluid intake
and urinary output.
● The patient must be closely assessed for responses to the
medical interventions and for the development of
complications, which must be corrected immediately.
● Because of the frequency of nursing interventions and the
technology required for effective medical management, the
patient is always treated in an intensive care environment.
● Critical care nurses are responsible for the nursing
management, which includes frequent assessments and timely
adjustments to medications and therapies based on the
assessment data.
Thromboembolism
● The decreased mobility of the patient with cardiac disease
and the impaired circulation that accompany these disorders
contribute to the development of intracardiac and
intravascular thrombosis.
● Intracardiac thrombus is especially common in patients with
atrial fibrillation, because the atria do not contract forcefully
and blood flow slows through the atrium, increasing
thrombus formation.
● Intracardiac thrombus is detected by an echocardiogram and
treated with anticoagulants, such as heparin and warfarin
(Coumadin). A part of the thrombus may become detached
(embolus) and may be carried to the brain, kidneys,
intestines, or lungs.
● The most common problem is pulmonary embolism. The
symptoms of pulmonary embolism include chest pain,
cyanosis, shortness of breath, rapid respirations, and
hemoptysis (bloody sputum)
● The pulmonary embolus may block the circulation to a part of
the lung, producing an area of pulmonary infarction.
● Usually, there is a significant decrease in oxygenation
measured by arterial blood gas analysis or pulse oximetry.
● Pain experienced is usually pleuritic; it increases with
respiration and may subside when the patient holds the
breath.
● Cardiac pain is usually continuous and does not vary with
respirations. However, it may be difficult to differentiate by
symptoms alone.
● The patient usually undergoes a ventilation perfusion scan or
a pulmonary arteriogram for definitive diagnosis.
● Systemic embolism may manifest as cerebral, mesenteric, or
renal infarction; an embolism can also compromise the blood
supply to an extremity.
● The nurse must be aware of such possible complications and
be prepared to identify and report signs and symptoms.
Pericardial Effusion and Cardiac Tamponade
Pathophysiology
● Pericardial effusion refers to the accumulation of fluid in the
pericardial sac. This occurrence may accompany pericarditis,
advanced HF, metastatic carcinoma, cardiac surgery, trauma,
or nontraumatic hemorrhage.
● Normally, the pericardial sac contains less than 50 mL of
fluid, which is needed to decrease friction for the beating
heart.
● An increase in pericardial fluid raises the pressure within the
pericardial sac and compresses the heart.
● This has the following effects:
ー Increased right and left ventricular end-diastolic pressures
ー Decreased venous return
ー Inability of the ventricles to distend adequately and to fill
● Pericardial fluid may accumulate slowly without causing
noticeable symptoms.
● A rapidly developing effusion, however, can stretch the
pericardium to its maximum size and, because of increased
pericardial pressure, reduce venous return to the heart and
decrease CO.
● The result is cardiac tamponade (compression of the heart).
Clinical Manifestations
● The patient may complain of a feeling of fullness within the
chest or may have substantial or ill-defined pain.
● The feeling of pressure in the chest may result from
stretching of the pericardial sac.
● Because of increased pressure within the pericardium, venous
pressure tends to rise, as evidenced by engorged neck veins.
● Other signs include shortness of breath and a drop and
fluctuation in blood pressure. Systolic blood pressure that is
detected during exhalation but not heard with inhalation is
called pulsus paradoxus.
● The difference in systolic pressure between the point that it is
heard during exhalation and the point that it is heard during
inhalation is measured.
● Pulsus paradoxus exceeding 10 mm Hg is abnormal.
● The cardinal signs of cardiac tamponade are falling systolic
blood pressure, narrowing pulse pressure, rising venous
pressure (increased jugular venous distention), and distant
(muffled) heart sounds.

Assessment and Diagnostic Findings
● Pericardial effusion is detected by percussing the chest and
noticing an extension of flatness across the anterior aspect of
the chest.
● An echocardiogram may be performed to confirm the
diagnosis.
● The clinical signs and symptoms and chest x-ray findings are
usually sufficient to diagnose pericardial effusion.
Medical Management
● Pericardiocentesis
ー If cardiac function becomes seriously impaired,
pericardiocentesis (puncture of the pericardial sac to
aspirate pericardial fluid) is performed to remove fluid
from the pericardial sac.
ー The major goal is to prevent cardiac tamponade, which
restricts normal heart action.
ー During the procedure, the patient is monitored by ECG
and hemodynamic pressure measurements.
ー Emergency resuscitative equipment should be readily
available.
ー The head of the bed is elevated to 45 to 60 degrees,
placing the heart in proximity to the chest wall so that the
needle can be inserted into the pericardial sac more easily.
ー If a peripheral intravenous device is not already in place,
one is inserted, and a slow intravenous infusion is started
in case it becomes necessary to administer emergency
medications or blood products.
ー The pericardial aspiration needle is attached to a 50-mL
syringe by a three-way stopcock.
ー Several possible sites are used for pericardial aspiration.
The needle may be inserted in the angle between the left
costal margin and the xiphoid, near the cardiac apex; at the
fifth or sixth intercostal space at the left sternal margin; or
on the right sternal margin of the fourth intercostal space.
ー The needle is advanced slowly until it has entered the
epicardium and fluid is obtained.
ー The ECG can help determine when the needle has
contacted the epicardium.
ーThe cable of a precordial lead is attached to the aspirating
needle with alligator clamps; contact with the epicardium
is seen by ST segment elevation on the ECG.
ー During the procedure, drainage fluid must be checked for
clotting.
ー Although not entirely accurate, the guideline is that
pericardial blood does not clot readily, whereas blood
obtained from inadvertent puncture of one of the heart
chambers does clot
ー A resulting fall in central venous pressure and an
associated rise in blood pressure after withdrawal of
pericardial fluid indicate that the cardiac tamponade has
been relieved.
ー The patient almost always feels immediate relief. If there
is a substantial amount of pericardial fluid, a small catheter
may be left in place to drain recurrent accumulation of
blood or fluid.
ー Pericardial fluid is sent to the laboratory for examination
for tumor cells, bacterial culture, chemical and serologic
analysis, and differential blood cell count.
ー Complications of pericardiocentesis include ventricular or
coronary artery puncture, dysrhythmias, pleural laceration,
gastric puncture, and myocardial trauma.
ー After pericardiocentesis, the patient’s heart rhythm, blood
pressure, venous pressure, and heart sounds are monitored
to detect any possible recurrence of cardiac tamponade.
ー If it recurs, repeated aspiration is necessary.
ー Cardiac tamponade may require treatment by open
pericardial drainage (pericardiotomy).
ー The patient is ideally in an intensive care unit
● Pericardiotomy
ー Recurrent pericardial effusions, usually associated with
neoplastic diseases, may be treated by a pericardiotomy
(pericardial window).
ー The patient receives a general anesthetic, but
cardiopulmonary bypass is seldom necessary.
ー A portion of the pericardium is excised to permit the
pericardial fluid to drain into the lymphatic system.
ー Uncommonly, catheters are placed between the
pericardium and abdominal cavity to drain the pericardial
fluid. The nursing care is the same as that described for
other cardiac surgery.
Myocardial Rupture
● Myocardial rupture is a rare event. However, it can occur
when a myocardial infarction, infectious process, cardiac
trauma, pericardial disease, or other myocardial dysfunction
weakens the cardiac muscle (eg, ventricular aneurysm)
substantially.
● Persistent elevation of the ST segment is an indication of
ventricular aneurysm.
● In many cases, the result of myocardial rupture is immediate
death, even if the patient undergoes immediate cardiac
surgery.

Cardiac Arrest
● Cardiac arrest occurs when the heart ceases to produce an
effective pulse and blood circulation.
● It may be caused by a cardiac electrical event, as when the
HR is too fast (especially ventricular tachycardia or
ventricular fibrillation) or too slow (bradycardia or AV block)
or when there is no heart rate at all (asystole).
● Cardiac arrest may follow respiratory arrest; it may also
occur when electrical activity is present but there is
ineffective cardiac contraction or circulating volume, which
is called pulseless electrical activity (PEA).
● Formerly called electrical-mechanical dissociation(EMD),
PEA can be caused by hypovolemia (eg, with excessive
bleeding), cardiac tamponade, hypothermia, massive
pulmonary embolism, medication overdoses (eg, tricyclic
agents, digitalis, beta-blockers, calcium channel blockers),
significant acidosis, and massive acute myocardial infarction.
Clinical Manifestations
● Consciousness, pulse, and blood pressure are lost
immediately. Ineffective respiratory gasping may occur.
● The pupils of the eyes begin dilating within 45 seconds.
● Seizures may or may not occur.
● The risk of irreversible brain damage and death increases
with every minute from the time that circulation ceases.
● The interval varies with the age and underlying condition of
the patient.
● During this period, the diagnosis of cardiac arrest must be
made, and measures must be taken immediately to restore
circulation.
Emergency Management: Cardiopulmonary Resuscitation
● The ABCDs of basic cardiopulmonary resuscitation (CPR)
are airway, breathing, circulation, and defibrillation.
● Once loss of consciousness has been established, the
resuscitation priority for the adult in most cases is placing a
phone call to activate the code team or the emergency
medical system (EMS).
● Exceptions to this include near drowning, drug or medication
overdose, and respiratory arrest situations, for which 1 minute
of CPR should be performed before activating the EMS.
● Because the underlying cause of arrest in an infant or child is
usually respiratory, the priority is to begin CPR and then
activate the EMS after 1 minute of CPR.
● Because the care of the pediatric patient is individualized, the
following discussion on the care of a cardiac arrest patient
applies only to adults.
● Resuscitation consists of the following steps:
1. Airway: maintaining an open airway
2. Breathing: providing artificial ventilation by rescue
breathing
3. Circulation: promoting artificial circulation by external
cardiac compression
4. Defibrillation: restoring the heartbeat
● If the patient is monitored or is immediately placed on the
monitor using the multifunction pads or the quick-look
paddles (found on most defibrillators) and the ECG shows
ventricular tachycardia or ventricular fibrillation,
defibrillation rather than CPR is the treatment of choice.
● In this scenario, CPR is performed initially only if the
defibrillator is not immediately available.
● The survival rate decreases by 10% for every minute that
defibrillation is delayed.
● If the patient has not been defibrillated within 10 minutes, the
chance of survival is close to zero.
★ Maintaining Airway and Breathing
● The first step in CPR is to obtain an open airway. Any
obvious material in the mouth or throat should be removed.
● The chin is directed up and back, or the jaw (mandible) is
lifted forward.
● The rescuer “looks, listens, and feels” for air movement.
● An oropharyngeal airway is inserted if available. Two rescue
ventilations over 3 to 4 seconds are provided using a
bag-mask or mouth mask device.
● An obstructed airway should be suspected when the rescuer
cannot give the initial ventilations, and the Heimlich
maneuver or abdominal thrusts should be administered to
relieve the obstruction.
● If the first rescue ventilations enter easily, the patient is
ventilated with 12 breaths per minute, and the open airway is
maintained.
● Endotracheal intubation is frequently performed by a
physician, nurse anesthetist, or respiratory therapist during a
resuscitation procedure (also called a code) to ensure an
adequate airway and ventilation.
● The resuscitation bag device is then connected directly to the
endotracheal tube.
★ Restoring Circulation
● After performing ventilation, the carotid pulse is assessed and
external cardiac compressions are provided when no pulse is
detected.
● If a defibrillator is not yet available but a process has been
put into place to obtain one, chest compressions are initiated.
● Compressions are performed with the patient on a firm
surface, such as the floor, a cardiac board, or a meal tray.
● The rescuer (facing the patient’s side) places the heel of one
hand on the lower half of the sternum, two finger widths (3.8
cm [1.5 inches]) from the tip of the xiphoid and positions the
other hand on top of the first hand.
● The fingers should not touch the chest wall.
● Using the body weight while keeping the elbows straight, the
rescuer presses quickly downward from the shoulder area to
deliver a forceful compression to the victim’s lower sternum
about 3.8 to 5 cm (1.5 to 2 inches) toward the spine.
● The chest compression rate is 80 to 100 times per minute.
● If only one rescuer is available, the rate is two ventilations to
every 15 cardiac compressions.
● When two rescuers are available, the first person performs the
cardiac compressions, pausing after the fifth compression,
when the second rescuer gives one ventilation over 1.5 to 2
seconds and at a tidal volume of less than 1 L.
● When the code team or emergency medical personnel arrive,
the patient is quickly assessed to determine cardiac rhythm
and respiratory status, as well as possible causes for the
arrest.
● The specific subsequent advanced life support interventions
depend on the assessment results. For example, after the
patient is placed on a cardiac monitor and ventricular
fibrillation is detected, the patient will be defibrillated up to
three times, and then CPR will be resumed.
● However, if asystole is detected on the monitor, CPR is
resumed immediately while trying to identify the underlying
cause, such as hypovolemia, hypothermia, or hypoxia.
● CPR may be stopped when the patient responds and begins to
breathe, the rescuers are too exhausted or at risk (eg, the
building is at risk of collapsing) to continue CPR, or signs of
death are obvious.
● If the patient does not respond to therapies given during the
arrest, the resuscitation effort may be stopped or “called” by
the physician.
● The decision to terminate resuscitation is based on medical
considerations and takes into account the underlying
condition of the patient and the chances for survival.

Follow-Up Monitoring

● Once successfully resuscitated, the patient is transferred to an

intensive care unit for close monitoring.
● Continuous ECG monitoring and frequent blood pressure
assessments are essential until hemodynamic stability is
reestablished.
● Etiologic factors that precipitated the arrest, such as
metabolic or rhythm abnormalities, must be identified and
treated.
● Possible contributing factors, such as electrolyte or acid-base
imbalances, need to be identified and corrected.
 ● The bronchial walls become thickened, the bronchial lumen
Management of Patients With is narrowed, and mucus may plug the airway.

Chronic Obstructive Pulmonary

Disease
Chronic Obstructive Pulmonary Disease (COPD)

● Is a disease state characterized by airflow limitation that is

not fully reversible.
● COPD may include diseases that cause airflow
obstruction(eg, emphysema, chronic bronchitis) or a
combination of these disorders.
● Other diseases such as cystic fibrosis, bronchiectasis, and
asthma were previously classified as types of chronic
obstructive lung disease. However, asthma is now considered
a separate disorder and is classified as an abnormal airway
condition characterized primarily by reversible inflammation.
● …
Pathophysiology
● Alveoli adjacent to the bronchioles may become damaged
and fibrosed, resulting in altered function of the alveolar
macrophages.
● This is significant because the macrophages play an
important role in destroying foreign particles, including
bacteria. As a result, the patient becomes more susceptible to
respiratory infection.
● A wide range of viral, bacterial, and mycoplasmal infections
can produce acute episodes of bronchitis. Exacerbations of
chronic bronchitis are most likely to occur during the winter.

● In COPD, the airflow limitation is both progressive and

associated with an abnormal inflammatory response of the
lungs to noxious particles or gases.
● The inflammatory response occurs throughout the airways,
parenchyma, and pulmonary vasculature. Because of the
chronic inflammation and the body’s attempts to repair it,
narrowing occurs in the small peripheral airways. Over time,
this injury-and-repair process causes scar tissue formation
and narrowing of the airway lumen. Airflow obstruction may
also be due to parenchymal destruction as seen with
emphysema, a disease of the alveoli or gas exchange units.
● In addition to inflammation, processes relating to imbalances
of proteinases and antiproteinases in the lung may be
responsible for airflow limitation.
● When activated by chronic inflammation, proteinases and
other substances may be released, damaging the parenchyma
of the lung.
● The parenchymal changes may also be consequences of Emphysema
inflammation, environmental, or genetic factors(eg, alpha1
antitrypsin deficiency) ● In emphysema, impaired gas exchange (oxygen, carbon
● Early in the course of COPD, the inflammatory response dioxide) results from destruction of the walls of
causes pulmonary vasculature changes that are characterized overdistended alveoli.
by thickening of the vessel wall. ● “Emphysema” is a pathological term that describes an
● These changes may occur as a result of exposure to cigarette abnormal distention of the air spaces beyond the terminal
smoke or use of tobacco products or as a result of the release bronchioles, with destruction of the walls of the alveoli.
of inflammatory mediators. ● It is the end stage of a process that has progressed slowly for
many years.
Chronic Bronchitis ● As the walls of the alveoli are destroyed (a process
accelerated by recurrent infections), the alveolar surface area
● Chronic bronchitis, a disease of the airways, is defined as the in direct contact with the pulmonary capillaries continually
presence of cough and sputum production for at least 3 decreases, causing an increase in dead space (lung area
months in each of 2 consecutive years. where no gas exchange can occur) and impaired oxygen
● In many cases, smoke or other environmental pollutants diffusion, which leads to hypoxemia.
irritate the airways, resulting in hypersecretion of mucus and ● In the later stages of the disease, carbon dioxide elimination
inflammation. is impaired, resulting in increased carbon dioxide tension in
● This constant irritation causes the mucus-secreting glands and arterial blood (hypercapnia) and causing respiratory acidosis.
goblet cells to increase in number, ciliary function is reduced, ● As the alveolar walls continue to break down, the pulmonary
and more mucus is produced. capillary bed is reduced.
● Consequently, pulmonary blood flow is increased, forcing the
right ventricle to maintain a higher blood pressure in the
pulmonary artery.
● Hypoxemia may further increase pulmonary artery pressure.
Thus, right-sided heart failure (cor pulmonale) is one of the
complications of emphysema.
● Congestion, dependent edema, distended neck veins, or pain
in the region of the liver suggests the development of cardiac
failure.
Clinical Manifestations
● COPD is characterized by three primary symptoms: cough,
sputum production, and dyspnea on exertion. These
symptoms often worsen over time.
● Chronic cough and sputum production often precede the
development of airflow limitation by many years. However,
not all individuals with cough and sputum production will
develop COPD.
● Dyspnea may be severe and often interferes with the patient’s
activities.
● Weight loss is common because dyspnea interferes with
eating, and the work of breathing is energy-depleting. Often
the patient cannot participate in even mild exercise because
of dyspnea; as COPD progresses, dyspnea occurs even at rest.
● As the work of breathing increases over time, the accessory
muscles are recruited in an effort to breathe.
● The patient with COPD is at risk for respiratory insufficiency
and respiratory infections, which in turn increase the risk for
acute and chronic respiratory failure.
● In COPD patients with a primary emphysematous
component, chronic hyperinflation leads to the “barrel
chest” thorax configuration. This results from fixation of the
ribs in the inspiratory position (due to hyperinflation) and
from loss of lung elasticity.
● Retraction of the supraclavicular fossae occurs on inspiration,
causing the shoulders to heave upward.
● In advanced emphysema, the abdominal muscles also
contract on inspiration.
Assessment and Diagnostic Findings
● Obtain a thorough health history
● Spirometry (evaluate airflow obstruction)
● Bronchodilator reversibility testing may be performed to rule
out the diagnosis of asthma.
● Arterial blood gas analysis
● Alpha1 antitrypsin deficiency screening may be performed for
patients under age 45 or for those with a strong family history
of COPD.
● Assessment in COPD
ー Cough
ー Dyspnea
ー Chest pain
ー Sputum production
ー Adventitious breath sounds
ー Pursed lip-breathing
ー Tend to assume upright, leaning forward position (tripod
position)
ー Alteration in LOC (level of consciousness)
ー Alteration in skin color (pallor to cyanosis)
ー Alteration in skin temperature (cold to the touch)
ー Voice changes
ー Decreased metabolism
○ Weakness
○ Fatigue
○ Anorexia
○ Weight loss
ー Alteration in thoracic anatomy (barrel chest)
ー Clubbing of fingers (Schamroth test)
ー Polycythemia
Complications
● Respiratory insufficiency and failure are major
life-threatening complications of COPD.
● The acuity of the onset and the severity of respiratory failure
depend on the patient’s baseline pulmonary function, pulse
oximetry or arterial blood gas values, comorbid conditions,
and the severity of other complications of COPD.
● Respiratory insufficiency and failure may be chronic (with
severe COPD) or acute (with severe bronchospasm or
pneumonia in the patient with severe COPD).
● Acute respiratory insufficiency and failure may necessitate
ventilatory support until other acute complications, such as
infection, can be treated.
 ● Other complications of COPD include pneumonia,
atelectasis, pneumothorax, and cor pulmonale.
Medical Management
★ Risk Reduction
● Smoking cessation is the single most effective intervention to
prevent COPD or slow its progression.
● Patients diagnosed with COPD who continue to smoke must
be encouraged and assisted to quit.
● Factors associated with continued smoking vary among
patients and may include the strength of nicotine addiction,
continued exposure to smoking-associated stimuli (at work or
in social settings), stress, depression, and habit.
● Continued smoking is also more prevalent among those with
low incomes, a low level of education, and psychosocial
problems.
Pharmacologic Therapy
★ Bronchodilators
● Bronchodilators relieve bronchospasm and reduce airway
obstruction by allowing increased oxygen distribution
throughout the lungs and improving alveolar ventilation.
● These medications, which are central in the management of
COPD, are delivered through a metered-dose inhaler, by
nebulization, or via the oral route in pill or liquid form.
● Bronchodilators are often administered regularly throughout
the day as well as on an as-needed basis. They may also be
used prophylactically to prevent breathlessness by having the
patient use them before an activity, such as eating or walking.
★ Corticosteroids
● Inhaled and systemic corticosteroids (oral or intravenous)
may also be used in COPD but are used more frequently in
asthma.
● Although it has been shown that corticosteroids do not slow
the decline in lung function, these medications may improve
symptoms.
● Examples of corticosteroids in the inhaled form are
beclomethasone (Beclovent, Vanceril), budesonide
(Pulmicort), flunisolide (AeroBid), fluticasone (Flovent),
and triamcinolone (Azmacort).
Management of Exacerbation
★ Oxygen Therapy
● Oxygen therapy can be administered as long-term continuous
therapy, during exercise, or to prevent acute dyspnea.
● Long-term oxygen therapy has been shown to improve the
patient’s quality of life and survival.
● For patients with an arterial oxygen pressure (PaO2) of 55
mm Hg or less on room air, maintaining a constant and
adequate oxygen saturation (>90%) is associated with
significantly reduced mortality and improved quality of life.
● Indications for oxygen supplementation include a PaO2 of 55
mm Hg or less or evidence of tissue hypoxia and organ
damage such as cor pulmonale, secondary polycythemia,
edema from right heart failure, or impaired mental status. In
patients with exercise-induced hypoxemia, oxygen
supplementation during exercise can improve performance.
● Patients who are hypoxemic while awake are likely to be so
during sleep. Therefore, nighttime oxygen therapy is
recommended as well, and the prescription for oxygen
therapy is for continuous, 24-hour use. Intermittent oxygen
therapy is indicated for those who desaturate only during
exercise or sleep.
Nursing Management
★ Patient Education
● Depending on the length and setting of the program, topics
may include normal anatomy and physiology of the lung,
pathophysiology and changes with COPD, medications and
home oxygen therapy, nutrition, respiratory therapy
treatments, symptom alleviation, smoking cessation, sexuality
and COPD, coping with chronic disease, communicating with
the health care team, and planning for the future (advance
directives, living wills, informed decision making about
health care alternatives).
★ Breathing Exercises
● The breathing pattern of most people withCOPD is shallow,
rapid, and inefficient; the more severe the disease, the more
inefficient the breathing pattern.
● With practice, this type of upper chest breathing can be
changed to diaphragmatic breathing, which reduces the
respiratory rate, increases alveolar ventilation, and sometimes
helps expel as much air as possible during expiration.
● Pursed-lip breathing helps to slow expiration, prevents
collapse of small airways, and helps the patient to control the
rate and depth of respiration. It also promotes relaxation,
enabling the patient to gain control of dyspnea and reduce
feelings of panic.
★ Activity Pacing
● A patient with COPD has decreased exercise tolerance during
specific periods of the day. This is especially true on arising
in the morning, because bronchial secretions collect in the
lungs during the night while the person is lying down.
● The patient may have difficulty bathing or dressing.
Activities requiring the arms to be supported above the level
of the thorax may produce fatigue or respiratory distress but
may be tolerated better after the patient has been up and
moving around for an hour or more.
 ● Working with the nurse, the patient can reduce these ● Restricted activity (and reversal of family roles due to loss of
limitations by planning self-care activities and determining employment), the frustration of having to work to breathe,
the best time for bathing, dressing, and daily activities. and the realization that the disease is prolonged and
★ Self-Care Activities unrelenting may cause the patient to react with anger,
● As gas exchange, airway clearance, and the breathing pattern depression, and demanding behavior.
improve, the patient is encouraged to assume increasing ● Sexual function may be compromised, which also diminishes
participation in self-care activities. self-esteem. In addition, the nurse needs to provide education
● The patient is taught to coordinate diaphragmatic breathing and support to the spouse/significant other and family
with activities such as walking, bathing, bending, or climbing because the caregiver role in end-stage COPD can be
stairs. difficult.
● The patient should bathe, dress, and take short walks, resting
as needed to avoid fatigue and excessive dyspnea. Nursing Process: The Patient With COPD
● Fluids should always be readily available, and the patient
should begin to drink fluids without having to be reminded. Assessment
● If postural drainage is to be done at home, the nurse instructs
and supervises the patient before discharge or in the
outpatient setting.
★ Physical Conditioning
● Physical conditioning techniques include breathing exercises
and general exercises intended to conserve energy and
increase pulmonary ventilation.
● There is a close relationship between physical fitness and
respiratory fitness.
● Graded exercises and physical conditioning programs using
treadmills, stationary bicycles, and measured level walks can
improve symptoms and increase work capacity and exercise
tolerance.
● Any physical activity that can be done regularly is helpful.
Lightweight portable oxygen systems are available for
ambulatory patients who require oxygen therapy during
physical activity.
★ Oxygen Therapy
● Oxygen supplied to the home comes in compressed gas,
liquid, or concentrator systems.
● Portable oxygen systems allow the patient to exercise, work,
and travel. To help the patient adhere to the oxygen
prescription, the nurse explains the proper flow rate and
required number of hours for oxygen use as well as the
dangers of arbitrary changes in flow rates or duration of
therapy.
● The nurse cautions the patient that smoking with or near
oxygen is extremely dangerous.
● The nurse also reassures the patient that oxygen is not
“addictive” and explains the need for regular evaluations of
blood oxygenation by pulse oximetry or arterial blood gas Diagnosis
analysis.
★ Nutritional Therapy ● Nursing Diagnosis
● Nutritional assessment and counseling are important aspects ー Based on the assessment data, the patient’s major nursing
in the rehabilitation process for the patient with COPD. diagnoses may include the following:
● Approximately 25% of patients with COPD are ○ Impaired gas exchange and airway clearance due to
undernourished. chronic inhalation of toxins
● A thorough assessment of caloric needs and counseling about ○ Impaired gas exchange related to ventilation–perfusion
meal planning and supplementation are part of the inequality
rehabilitation process. ○ Ineffective airway clearance related to
★ Coping Measures bronchoconstriction, increased mucus production,
● Any factor that interferes with normal breathing quite ineffective cough, bronchopulmonary infection, and
naturally induces anxiety, depression, and changes in other complications
behavior. ○ Ineffective breathing pattern related to shortness of
● Many patients find the slightest exertion exhausting. Constant breath, mucus, bronchoconstriction, and airway
shortness of breath and fatigue may make the patient irritable irritants
and apprehensive to the point of panic. ○ Activity intolerance due to fatigue, ineffective
breathing patterns, and hypoxemia
 ○ Deficient knowledge of self-care strategies to be ● Asthma can occur at any age and is the most common chronic
performed at home. disease of childhood.
○ Ineffective coping related to reduced socialization, ● Despite increased knowledge regarding the pathology of
anxiety, depression, lower activity level, and the asthma and the development of better medications and
inability to work management plans, the death rate from asthma continues to
● Collaborative Problems/Potential Complications increase.
ー Based on the assessment data, potential complications that ● For most patients it is a disruptive disease, affecting school
may develop include: and work attendance, occupational choices, physical activity,
○ Respiratory insufficiency or failure and general quality of life.
○ Atelectasis
○ Pulmonary infection
○ Pneumonia
○ Pneumothorax
○ Pulmonary hypertension

Nursing Intervention

● Promoting smoking cessation

● Improving gas exchange
● Achieving airway clearance
● Improving breathing patterns
● Improving activity tolerance
● Enhancing self-care strategies
ー Setting realistic goals
ー Avoiding temperature extremes
ー Modifying lifestyle
● Enhancing individual coping strategies
● Monitoring and managing potential complications

Evaluation
●
● Uses effective coping mechanisms for dealing with
consequences of disease
ー Uses self-care strategies to lessen stress associated with
disease
ー Verbalizes resources available to deal with psychological
burden of disease
ー Participates in pulmonary rehabilitation, if appropriate
● Uses community resources and home-based care
ー Verbalizes knowledge of community resources (eg,
smoking cessation, hospital/community-based support
groups)
ー Participates in pulmonary rehabilitation, if appropriate
● Avoids or reduces complications
Asthma
● Asthma is a chronic inflammatory disease of the airways that
causes airway hyperresponsiveness, mucosal edema, and
mucus production.
● This inflammation ultimately leads to recurrent episodes of
asthma symptoms: cough, chest tightness, wheezing, and
dyspnea.
● Asthma differs from the other obstructive lung diseases in
that it is largely reversible, either spontaneously or with
treatment.
● Patients with asthma may experience symptom-free periods
alternating with acute exacerbations, which last from minutes
to hours or days.
Pathophysiology
● The underlying pathology in asthma is reversible and diffuse
airway inflammation. The inflammation leads to obstruction
from the following:
ー Swelling of the membranes that line the airways (mucosal
edema), reducing the airway diameter;
ー Contraction of the bronchial smooth muscle that encircles
the airways (bronchospasm), causing further narrowing;
ー Increased mucus production, which diminishes airway size
and may entirely plug the bronchi;
ー Bronchial muscles and mucus glands enlarge; thick,
tenacious sputum is produced;
ー The alveoli hyperinflate.
● Some patients may have airway subbasement membrane
fibrosis. This is called airway “remodeling” and occurs in
response to chronic inflammation. The fibrotic changes in the
airway lead to airway narrowing and potentially irreversible
airflow limitation.
● Cells that play a key role in the inflammation of asthma are
mast cells, neutrophils, eosinophils, and lymphocytes.
● Mast cells, when activated, release several chemicals called
mediators.
● These chemicals, which include histamine, bradykinin,
prostaglandins, and leukotrienes, perpetuate the inflammatory
response, causing increased blood flow, vasoconstriction,
fluid leak from the vasculature, attraction of white blood cells
to the area, and bronchoconstriction.
● Regulation of these chemicals is the aim of much of the
current research regarding pharmacologic therapy for asthma.
● When the alpha-adrenergic receptors are stimulated,
bronchoconstriction occurs; when the beta2-adrenergic
receptors are stimulated, bronchodilation results.
● The balance between alpha and beta2 receptors is controlled
primarily by cyclic adenosine monophosphate (cAMP).
Alpha-adrenergic receptor stimulation results in a decrease in
cAMP, which leads to an increase of chemical mediators
released by the mast cells and bronchoconstriction.
● Beta2-receptor stimulation results in increased levels of
cAMP, which inhibits the release of chemical mediators and
causes bronchodilation.
Clinical Manifestations
● The three most common symptoms of asthma are cough,
dyspnea, and wheezing. In some instances, cough may be the
only symptom.
● Asthma attacks often occur at night or early in the morning,
possibly due to circadian variations that influence airway
receptor thresholds.
● There is cough, with or without mucus production. At times
the mucus is so tightly wedged in the narrowed airway that
the patient cannot cough it up.
● There may be generalized wheezing (the sound of airflow
through narrowed airways), first on expiration and then
possibly during inspiration as well. Generalized chest
tightness and dyspnea occur.
● Expiration requires effort and becomes prolonged.
● As the exacerbation progresses, diaphoresis, tachycardia, and
a widened pulse pressure may occur along with hypoxemia
and central cyanosis (a late sign of poor oxygenation).
Assessment and Diagnostic Findings
● A complete family, environmental, and occupational history
is essential. To establish the diagnosis, the clinician must
determine that periodic symptoms of airflow obstruction are
present, airflow is at least partially reversible, and other
etiologies have been excluded.
● A positive family history and environmental factors,
including seasonal changes, high pollen counts, mold, climate
changes (particularly cold air), and air pollution, are primarily
associated with asthma. In addition, asthma is associated with
a variety of occupation-related chemicals and compounds,
including metal salts, wood and vegetable dust, medications
(eg, aspirin, antibiotics, piperazine, cimetidine), industrial
chemicals and plastics, biologic enzymes (eg, laundry
detergents), animal and insect dusts, sera, and secretions.
● Comorbid conditions that may accompany asthma include
gastroesophageal reflux, drug-induced asthma, and allergic
bronchopulmonary aspergillosis. Other possible allergic
reactions that may accompany asthma include eczema,
rashes, and temporary edema.
● During acute episodes, sputum and blood tests may disclose
eosinophilia (elevated levels of eosinophils).
● Serum levels of immunoglobulin E may be elevated if allergy
is present. Arterial blood gas analysis and pulse oximetry
reveal hypoxemia during acute attacks. Initially, hypocapnia
and respiratory alkalosis are present.
● As the condition worsens and the patient becomes more
fatigued, the PaCO2 may rise. A normal PaCO2 value may be
a signal of impending respiratory failure. Because CO2 is 20
times more diffusible than oxygen, it is rare for PaCO2 to be
normal or elevated in a person who is breathing very rapidly.
● During an exacerbation, the FEV1 and FVC are markedly
decreased but improve with bronchodilator administration
(demonstrating reversibility).
● Pulmonary function is usually normal between exacerbations.
The occurrence of a severe, continuous reaction is referred to
as status asthmaticus and is considered life-threatening.
Prevention
● Patients with recurrent asthma should undergo tests to
identify the substances that precipitate the symptoms.
● Possible causes are dust, dust mites, roaches, certain types of
cloth, pets, horses, detergents, soaps, certain foods, molds,
and pollens. If the attacks are seasonal, pollens can be
strongly suspected. The patient is instructed to avoid the
causative agents whenever possible.
● Knowledge is the key to quality asthma care.
Complications
● Complications of asthma may include status asthmaticus,
respiratory failure, pneumonia, and atelectasis.
● Airway obstruction, particularly during acute asthmatic
episodes, often results in hypoxemia, requiring the
administration of oxygen and the monitoring of pulse
oximetry and arterial blood gases.
● Fluids are administered because people with asthma are
frequently dehydrated from diaphoresis and insensible fluid
loss with hyperventilation.
Medical Management
Pharmacologic Therapy
● Two general classes of asthma medications are long-acting
medications to achieve and maintain control of persistent
asthma and quick-relief medications for immediate treatment
of asthma symptoms and exacerbations.
● Because the underlying pathology of asthma is inflammation,
control of persistent asthma is accomplished primarily with
regular use of anti-inflammatory medications.
● These medications have systemic side effects when used long
term. The route of choice for administration of these
medications is the MDI because it allows for topical
administration.
★ Long-Acting Control Medications
● Corticosteroids are the most potent and effective
anti-inflammatory medications currently available. They are
broadly effective in alleviating symptoms, improving airway
function, and decreasing peak flow variability.
● Initially, the inhaled form is used. A spacer should be used
with inhaled corticosteroids and the patient should rinse the
mouth after administration to prevent thrush, a common
complication of inhaled corticosteroid use.
● A systemic preparation may be used to gain rapid control of
the disease; to manage severe, persistent asthma; to treat
moderate to severe exacerbations; to accelerate recovery; and
to prevent recurrence.
 ● Cromolyn sodium (Intal) and nedocromil (Tilade) are mild
to moderate anti-inflammatory agents that are used more
commonly in children.
ー They also are effective on a prophylactic basis to prevent
exercise-induced asthma or in unavoidable exposure to
known triggers.
ー These medications are contraindicated in acute asthma
exacerbations.
● Long-acting beta2-adrenergic agonists are used with
anti-inflammatory medications to control asthma symptoms,
particularly those that occur during the night.
ー These agents are also effective for preventing
exercise-induced asthma.
ー Long-acting beta2-adrenergic agonists are not indicated
for immediate relief of symptoms.
● Methylxanthines (theophylline [Slo-bid, Theo-24,
Theo-Dur]) are mild to moderate bronchodilators usually
used in addition to inhaled corticosteroids, mainly for relief
of nighttime asthma symptoms.
ー There is some evidence that theophylline may have a mild
anti-inflammatory effect.
● Leukotriene modifiers (inhibitors) or antileukotrienes are a
new class of medications.
ー Leukotrienes are potent bronchoconstrictors that also
dilate blood vessels and alter permeability.
ー Leukotriene inhibitors act by either interfering with
leukotriene synthesis or blocking the receptors where
leukotrienes exert their action.
ー At this time, they may provide an alternative to inhaled
corticosteroids for mild persistent asthma or may be added
to a regimen of inhaled corticosteroids in more severe
asthma to attain further control.
★ Quick-Relief Medications
● Short-acting beta-adrenergic agonists are the medications of
choice for relieving acute symptoms and preventing
exercise-induced asthma.
● They have a rapid onset of action. Anticholinergics (eg,
ipratropium bromide [Atrovent]) may bring added benefit in
severe exacerbations, but they are used more frequently in
COPD patients.
Nursing Management
● The immediate nursing care of the patient with asthma
depends on the severity of the symptoms.
● The patient may be treated successfully as an outpatient if
asthma symptoms are relatively mild, or he or she may
require hospitalization and intensive care for acute and severe
asthma.
● The patient and family are often frightened and anxious
because of the patient’s dyspnea. Thus, an important aspect of
care is a calm approach.
● The nurse assesses the patient’s respiratory status by
monitoring the severity of symptoms, breath sounds, peak
flow, pulse oximetry, and vital signs.
● The nurse obtains a history of allergic reactions to
medications before administering medications and identifies
the patient’s current use of medications.
● The nurse administers medications as prescribed and
monitors the patient’s responses to those medications.
● Fluids may be administered if the patient is dehydrated, and
antibiotic agents may be prescribed if the patient has an
underlying respiratory infection.
● If the patient requires intubation because of acute respiratory
failure, the nurse assists with the intubation procedure,
continues close monitoring of the patient, and keeps the
patient and family informed about procedures.
Bronchiectasis
● Bronchiectasis is a chronic, irreversible dilation of the
bronchi and bronchioles.
● Under the new definition of COPD, it is considered a separate
disease process from COPD.
● Bronchiectasis may be caused by a variety of conditions,
including:
ー Airway obstruction
ー Diffuse airway injury
ー Pulmonary infections and obstruction of the bronchus or
complications of long-term pulmonary infections
ー Genetic disorders such as cystic fibrosis
ー Abnormal host defense (eg, ciliary dyskinesia or humoral
immunodeficiency)
ー Idiopathic causes
Pathophysiology
● The inflammatory process associated with pulmonary
infections damages the bronchial wall, causing a loss of its
supporting structure and resulting in thick sputum that
ultimately obstructs the bronchi.
● The walls become permanently distended and distorted,
impairing mucociliary clearance. The inflammation and
infection extend to the peribronchial tissues; in the case of
saccular bronchiectasis, each dilated tube virtually amounts to
a lung abscess, the exudate of which drains freely through the
bronchus.
● Bronchiectasis is usually localized, affecting a segment or
lobe of a lung, most frequently the lower lobes.
● The retention of secretions and subsequent obstruction
ultimately cause the alveoli distal to the obstruction to
collapse (atelectasis).
● Inflammatory scarring or fibrosis replaces functioning lung
tissue. In time the patient develops respiratory insufficiency
with reduced vital capacity, decreased ventilation, and an
increased ratio of residual volume to total lung capacity.
● There is impairment in the matching of ventilation to
perfusion (ventilation–perfusion imbalance) and hypoxemia.
Clinical Manifestations
● Characteristic symptoms of bronchiectasis include chronic
cough and the production of purulent sputum in copious
amounts.
● Many patients with this disease have hemoptysis.
● Clubbing of the fingers also is common because of
respiratory insufficiency.
● The patient usually has repeated episodes of pulmonary
infection.
● Even with modern treatment approaches, the average age at
death is approximately 55 years.

Assessment and Diagnostic Findings
● Bronchiectasis is not readily diagnosed because the
symptoms can be mistaken for those of simple chronic
bronchitis.
● A definite sign is offered by the prolonged history of
productive cough, with sputum consistently negative for
tubercle bacilli.
● The diagnosis is established by a computed tomography (CT)
scan, which demonstrates either the presence or absence of
bronchial dilation.
Medical Management
● Postural drainage is part of all treatment plans because
draining the bronchiectatic areas by gravity reduces the
amount of secretions and the degree of infection.
● Sometimes mucopurulent sputum must be removed by
bronchoscopy.
● Chest physiotherapy, including percussion and postural
drainage, is important in secretion management.
● Smoking cessation
● Infection is controlled with antimicrobial therapy based on
the results of sensitivity studies on organisms cultured from
sputum. A year-round regimen of antibiotic agents may be
prescribed, with different types of antibiotics at intervals.
● Patients should be vaccinated against influenza and
pneumococcal pneumonia.
● Bronchodilators, which may be prescribed for patients who
also have reactive airway disease, may also assist with
secretion management.
● Diseased tissue is removed, provided that the postoperative
lung function will be adequate. It may be necessary to
remove a segment of a lobe (segmental resection), a lobe
(lobectomy), or rarely an entire lung (pneumonectomy).
Segmental resection is the removal of an anatomic
subdivision of a pulmonary lobe. The chief advantage is that
only diseased tissue is removed and healthy lung tissue is
conserved.
Nursing Management
● Nursing management of the patient with bronchiectasis
focuses on alleviating symptoms and assisting the patient to
clear pulmonary secretions.
● Smoking and other factors that increase the production of
mucus and hamper its removal are targeted in patient
teaching.
● The patient and family are taught to perform postural
drainage and to avoid exposure to others with upper
respiratory and other infections.
● If the patient experiences fatigue and dyspnea, strategies to
conserve energy while maintaining as active a lifestyle as
possible are discussed.
● The patient needs to become knowledgeable about early signs
of respiratory infection and the progression of the disorder so
that appropriate treatment can be implemented promptly.
● Because the presence of a large amount of mucus may
decrease the patient’s appetite and result in an inadequate
dietary intake, the patient’s nutritional status is assessed and
strategies are implemented to ensure an adequate diet.
Cystic Fibrosis
● Cystic fibrosis (CF) is the most common fatal autosomal
recessive disease among the Caucasian population.
● An individual must inherit a defective copy of the CF gene
(one from each parent) to have CF.
● Although CF was once considered a fatal childhood disease,
approximately 38% of people living with the disease are 18
years of age or older.
● Cystic fibrosis is usually diagnosed in infancy or early
childhood, but patients may be diagnosed later in life.
● For individuals diagnosed later in life, respiratory symptoms
are frequently the major manifestation of the disease.
Pathophysiology
● This disease is caused by mutations in the CF transmembrane
conductance regulator protein, which is a chloride channel
found in all exocrine tissues.
● Chloride transport problems lead to thick, viscous secretions
in the lungs, pancreas, liver, intestine, and reproductive tract
as well as increased salt content in sweat gland secretions.
● In 1989, major breakthroughs were made in this disease with
the identification of the CF gene. The ability to detect the
common mutations of this gene allows for routine screening
for this disease as well as the detection of carriers.
● Genetic counseling is an important part of healthcare for
couples at risk.
● Airflow obstruction is a key feature in the presentation of CF.
This obstruction is due to bronchial plugging by purulent
secretions, bronchial wall thickening due to inflammation,
and, over time, airway destruction. These chronic retained
secretions in the airways set up an excellent reservoir for
continued bronchial infections.
Clinical Manifestations
● The pulmonary manifestations of this disease include a
productive cough, wheezing, hyperinflation of the lung fields
on chest x-ray, and pulmonary function test results consistent
with obstructive airways disease. Colonization of the airways
with pathogenic bacteria usually occurs early in life.
Staphylococcus aureus and Haemophilus influenzae are
common organisms during early childhood.
● As the disease progresses, Pseudomonas aeruginosa is
ultimately isolated from the sputum of most patients.
● Upper respiratory manifestations of the disease include
sinusitis and nasal polyps.
● Nonpulmonary clinical manifestations include
gastrointestinal problems (eg, pancreatic insufficiency,
recurrent abdominal pain, biliary cirrhosis, vitamin
deficiencies, recurrent pancreatitis, weight loss),
genitourinary problems (male and female infertility), and
clubbing of the extremities.
Assessment and Diagnostic Finding
● Most of the time, the diagnosis of CF is made based on an ● The nurse emphasizes the importance of an adequate fluid
elevated result of a sweat chloride concentration test, along and dietary intake to promote removal of secretions and to
with clinical signs and symptoms consistent with the disease. ensure an adequate nutritional status.
● Repeated sweat chloride values of greater than 60 mEq/L ● Because CF is a life-long disorder, patients often have
distinguish most individuals with CF from those with other learned to modify their daily activities to accommodate their
obstructive diseases. symptoms and treatment modalities.
● A molecular diagnosis may also be used in evaluating ● As the disease progresses, however, assessment of the home
common genetic mutations of the CF gene. environment may be warranted to identify modifications
required to address changes in the patient’s needs, increasing
Medical Management dyspnea and fatigue, and nonpulmonary symptoms.
● Although gene therapy and double lung transplantation are
● Patients with CF have problems with bacteria that are promising therapies for CF, they are limited in availability
resistant to multiple drugs and require multiple courses of and largely experimental.
antibiotic agents over long periods of time. ● As a result, the life expectancy of adults with CF is
● Bronchodilators are frequently administered to decrease shortened. Therefore, end-of-life issues and concerns need to
airway obstruction. be addressed in patients when warranted.
● Inhaled mucolytic agents such as dornase alfa (Pulmozyme) ● For the patient whose disease is progressing and who is
or N-acetylcysteine (Mucomyst) may also be used. These developing increasing hypoxemia, preferences for end-of-life
agents help to decrease the viscosity of the sputum and care should be discussed, documented, and honored.
promote expectoration of secretions. ● Patients and family members need support as they face a
● To decrease the inflammation and ongoing destruction of the shortened life span and an uncertain future.
airways, anti-inflammatory agents may also be used. These
may include inhaled corticosteroids or systemic therapy.
● Other anti-inflammatory medications have also been studied
in CF. Ibuprofen was studied in children with CF and some
benefit was demonstrated, but there is little information on its
use in young or older adults with CF
● Supplemental oxygen is used to treat the progressive
hypoxemia that occurs with CF. It helps to correct the
hypoxemia and may minimize the complications seen with
chronic hypoxemia (pulmonary hypertension).
● Lung transplantation is an option for a small, select
population of CF patients. A double lung transplant technique
is used due to the chronically infected state of the lungs seen
in end-stage CF. Because there is a long waiting list for lung
transplant recipients, many patients die while awaiting a
transplant.
● Gene therapy is a promising approach to management, with
many clinical trials underway. It is hoped that various
methods of administering gene therapy will carry healthy
genes to the damaged cells and correct defective CF cells.
Efforts are underway to develop innovative methods of
delivering therapy to the CF cells of the airways.

Nursing Management

● Nursing care of the adult with CF includes assisting the

patient to manage pulmonary symptoms and to prevent
complications of CF.
● Specific nursing measures include strategies that promote
removal of pulmonary secretions; chest physiotherapy,
including postural drainage, chest percussion, and vibration,
and breathing exercises are implemented and are taught to the
patient and to the family when the patient is very young.
● The patient is reminded of the need to reduce risk factors
associated with respiratory infections (eg, exposure to crowds
and to persons with known infections).
● The patient is taught the early signs and symptoms of
respiratory infection and disease progression that indicate the
need to notify the primary health care provider.

Assessment and Management of
Patients with Hematologic
Disorders
Anatomic and Physiologic Overview
● The hematologic system consists of the blood and the sites
where blood is produced, including the bone marrow and the
reticuloendothelial system (RES).
● Blood is a specialized organ that differs from other organs in
that it exists in a fluid state.
● Blood is composed of plasma and various types of cells.
Plasma is the fluid portion of blood: it contains various
proteins, such as albumin, globulin, fibrinogen, and other
factors necessary for clotting as well as electrolytes, waste
products and nutrients.
● About 55% …
Blood
● The cellular component of blood consists of three primary
cell types: RBCs (red blood cells or erythrocytes), WBCs
(white blood cells or leukocytes), and platelets
(thrombo-cytes).
● These cellular components of blood normally make up 40%
to 45% of the blood volume.
● Because most blood cells have a short life span, the need for
the body to replenish its supply of cells is continuous; this
process is termed hematopoiesis.
● The primary site for hematopoiesis is the bone marrow.
● During embryonic development and in other conditions, the
liver and spleen may also be involved.
● Under normal conditions, the adult bone marrow produces
about 175 billion RBCs, 70 billion neutrophils (mature form
of a WBC), and 175 billion platelets each day.
● When the body needs more blood cells, as in infection (when
WBCs are needed to fight the invading pathogen) or in
bleeding (when more RBCs are required), the marrow
increases its production of the cells required.
● Thus, under normal conditions, the marrow responds to
increased demand and releases adequate numbers of cells into
the circulation.
● The volume of blood in humans is approximately 7% to 10%
of the normal body weight and amounts to 5 to 6 L.
Circulating through the vascular system and serving as a link
between body organs, the blood carries oxygen absorbed
from the lungs and nutrients absorbed from the
gastrointestinal tract to the body cells for cellular metabolism.
● Blood also carries waste products produced by cellular
metabolism to the lungs, skin, liver, and kidneys, where they
are transformed and eliminated from the body.
● Blood also carries hormones, antibodies, and other substances
to their sites of action or use.
● To function, blood must remain in its normally fluid state.
Because blood is fluid, the danger always exists that trauma
can lead to loss of blood from the vascular system.
● To prevent this, an intricate clotting mechanism is activated
when necessary to seal any leak in the blood vessels.
Excessive clotting is equally dangerous, because it can
obstruct blood flow to vital tissues. To prevent this, the body
has a fibrinolytic mechanism that eventually dissolves clots
(thrombi) formed within blood vessels.
Bone Marrow
● The bone marrow is the site of hematopoiesis, or blood cell
formation.
● In a child all skeletal bones are involved, but as the child ages
marrow activity decreases.
● By adulthood, marrow activity is usually limited to the pelvis,
ribs, vertebrae, and sternum. Marrow is one of the largest
organs of the body, making up 4% to 5% of total body
weight.
● It consists of islands of cellular components (red marrow)
separated by fat (yellow marrow).
● As the adult ages, the proportion of active marrow is
gradually replaced by fat; however, in the healthy person, the
fat can again be replaced by active marrow when more blood
cell production is required.
● In adults with disease that causes marrow destruction,
fibrosis, or scarring, the liver and spleen can also resume
production of blood cells by a process known as
extramedullary hematopoiesis.
● The marrow is highly vascular. Within it are primitive cells
called stem cells. The stem cells have the ability to
self-replicate, thereby ensuring a continuous supply of stem
cells throughout the life cycle. When stimulated to do so,
stem cells can begin a process of differentiation into either
myeloid or lymphoid stem cells.
● These stem cells are committed to produce specific types of
blood cells. Lymphoid stem cells produce either T or B
lymphocytes.
● Myeloid stem cells differentiate into three broad cell types:
RBCs, WBCs, and platelets.
 Erythropoiesis

● Erythroblasts arise from the primitive myeloid stem cells in

bone marrow.
● The erythroblast is a nucleated cell that, in the process of
maturing within the bone marrow, accumulates hemoglobin
and gradually loses its nucleus.
● At this stage, the cell is known as a reticulocyte.
● Further maturation into an RBC entails the loss of the
dark-staining material and slight shrinkage.
● The mature RBC is then released into the circulation.
● Under conditions of rapid erythropoiesis (RBC production),
● Thus, with the exception of lymphocytes, all blood cells are reticulocytes and other immature cells (eg, nucleated RBCs)
derived from the myeloid stem cell. A defect in the myeloid may be released prematurely into the circulation.
stem cell can cause problems not only with WBC production ● Differentiation of the primitive myeloid stem cell of the
but also with RBC and platelet production. The entire process mar-row into an erythroblast is stimulated by erythropoietin,
of hematopoiesis is highly complex. a hor-mone produced primarily by the kidney.
● If the kidney detects low levels of oxygen (as would occur in
anemia, in which fewer RBCs are available to bind oxygen,
Blood Cells: Red Blood Cells (RBCs)
or in people living at high altitudes), the release of
erythropoietin is increased.
● The normal RBC is a biconcave disk that resembles a soft
● The increased erythropoietin then stimulates the marrow to
ball compressed between two fingers.
increase production of RBCs. The entire process typically
● It has a diameter of about 8 μ m and is so flexible that it can
takes 5 days.
pass easily through capillaries that may be as small as 2.8 μ
● For normal RBC production, the bone marrow also requires
m in diameter.
iron, vitamin B12, folic acid, pyridoxine (vitamin B6),
● The RBC membrane is so thin that gases, such as oxygen and
protein, and other factors. A deficiency of these factors
carbon dioxide, can easily diffuse across it; the disk shape
during erythropoiesis can result in decreased RBC production
provides a large surface area that facilitates the absorption
and anemia.
and release of oxygen molecules.
● Mature RBCs consist primarily of hemoglobin, which
contains iron and makes up 95% of the cell mass. RBCs have Iron Stores and Metabolism
no nuclei, and they have many fewer metabolic enzymes than
● The average daily diet in the UnitedStates contains 10 to 15
do most other cells. The presence of a large amount of
mg of elemental iron; normally 0.5 to 1 mg of ingested iron is
hemoglobin enables the RBC to perform its principal
absorbed from the small intestine.
function, the transport of oxygen between the lungs and
● The rate of iron absorption is regulated by the amount of iron
tissues.
already stored in the body and by the rate of RBC production.
● Occasionally the marrow releases slightly imma-ture forms of
● Additional amounts of iron, up to 2 mg daily, must be
RBCs, called reticulocytes, into the circulation.
absorbed by women to replace blood lost during
● This occurs as a normal response to an increased demand for
menstruation. Total body iron content in the average adult is
RBCs (as in bleeding) or in some disease states.
approximately 3 g, most of which is present in hemoglobin or
● The oxygen-carrying hemoglobin molecule is made up of
in one of its breakdown products.
four subunits, each containing a heme portion attached to a
● Iron is stored in the small intestine as ferritin and in
globin chain.
reticuloendothelial cells. When required, the iron is released
● Iron is present in the heme component of the molecule. An
into the plasma, binds to transferrin, and is transported into
important property of heme is its ability to bind to oxygen
the membranes of the normoblasts (RBC precursor cells)
loosely and reversibly.
within the marrow, where it is incorporated into hemoglobin.
● Oxygen readily binds to hemoglobin in the lungs and is
Iron is lost in the feces, either in bile, blood, or mucosal cells
carried as oxyhemoglobin in arterial blood.
from the intestine.
● Oxyhemoglo-bin is a brighter red than hemoglobin that does
● The concentration of iron in blood is normally about 75 to
not contain oxy-gen (reduced hemoglobin), which is why
175 μ g/dL (13 to 31 μ mol/L) for men and 65 to 165 μ g/dL
arterial blood is a brighter red than venous blood.
(11 to 29 μ mol/L) for women.
● The oxygen readily dissociates (detaches) from hemoglobin
● With iron deficiency, bone marrow iron stores are rapidly
in the tissues, where the oxygen is needed for cellular
depleted; hemoglobin synthesis is depressed, and the RBCs
metabolism.
produced by the marrow are small and low in hemoglobin.
● In venous blood, hemoglobin combines with hydrogen ions
● Iron deficiency in the adult generally indicates that blood has
produced by cellular metabolism and thus buffers excessive
been lost from the body (eg, from bleeding in the
acid.
gastrointestinal tract or heavy menstrual flow).
● Whole blood normally contains about 15 g of hemoglobin per
● In the adult, lack of dietary iron is rarely the sole cause of
100 mL of blood.
iron deficiency anemia.
● The source of iron deficiency should be investigated
promptly, because iron deficiency in an adult may be a sign
of bleeding in the gastrointestinal tract or colon cancer.
Vitamin B12 and Folic Acid Metabolism
● Vitamin B12and folic acid are required for the synthesis of
DNA in many tissues, but deficiencies of either of these
vitamins have the greatest effect on erythropoiesis.
● Both vitamin B12 and folic acid are derived from the diet.
● Folic acid is absorbed in the proximal small intestine, but
only small amounts are stored within the body. If the diet is
deficient in folic acid, stores within the body quickly become
depleted.
● Because vitamin B12 is found only in foods of animal origin,
strict vegetarians may ingest little B12. Vitamin B12
combines with intrinsic factor produced in the stomach.
● The vitamin B–intrinsic factor complex is absorbed in the
distal ileum.
● People who have had a partial or total gastrectomy may have
limited amounts of intrinsic factor, and therefore the
absorption of B12 may be diminished.
● The effects of either decreased absorption or decreased intake
of B12 are not apparent for 2 to 4 years.
● Vitamin B12 and folic acid deficiencies are characterized by
the production of abnormally large RBCs called
megaloblasts.
● Because these cells are abnormal, many are sequestered
(trapped) while still in the bone marrow, and their rate of
release is decreased.
● Some of these cells actually die in the marrow before they
can be released into the circulation. This results in
megaloblastic anemia.
Red Blood Cell Destruction
● The average lifespan of a normal circulating RBC is 120
days.
● Aged RBCs lose their elasticity and become trapped in small
blood vessels, particularly in the spleen.
● They are removed from the blood by the reticuloendothelial
cells, particularly in the liver and the spleen.
● As the RBCs are destroyed, their hemoglobin is largely
recycled.
● Some hemoglobin also breaks down to form bilirubin and is
secreted in the bile.
● Most of the iron is recycled to form new hemoglobin
molecules within the bone marrow; small amounts are lost
daily in the feces and urine and monthly in menstrual flow.
White Blood Cells (WBCs)
● Leukocytes are divided into two general categories:
granulocytes and lymphocytes. In normal blood, the total
leukocyte count is 5000 to 10,000 cells per cubic millimeter.
● Of these, approxi-mately 60% to 70% are granulocytes and
30% to 40% are lym-phocytes. Primarily, WBCs protect the
body against infection and tissue injury.
Granulocytes
● Granulocytes are defined by the presence of granules in the
cytoplasm of the cell. Granulocytes are divided into three
main sub-groups, which are characterized by the staining
properties of these granules.
● Eosinophils have bright-red granules in their cytoplasm,
whereas the granules in basophils stain deep blue.
● The third and by far the most numerous cell in this class is
the neutrophil, with granules that stain a pink to violet hue.
Neutrophils are also called polymorphonuclear neutrophils
(PMNs, or polys) or segmented neutrophils (segs).
● …
Mononuclear White Blood Cells (Agranulocytes)
Monocytes
● Monocytes(also called mononuclear leukocytes) areWBCs
with a single-lobed nucleus and a granule-free cytoplasm—
hence the term agranulocyte. In normal adult blood,
monocytes ac-count for approximately 5% of the total WBCs.
Monocytes are the largest of the WBCs. Produced by the
bone marrow, they remain in the circulation for a short time
before entering the tissues and transforming into
macrophages. Macrophages are particularly active in the
spleen, liver, peritoneum, and the alveoli of the lungs.
Lymphocytes
● Mature lymphocytes are small cells with scant cytoplasm.
Immature lymphocytes are produced in the marrow from the
lymphoid stem cells. A second major source of produc-tion is
the cortex of the thymus. Cells derived from the thymus are
known as T lymphocytes (or T cells); those derived from the
marrow can also be T cells but are more commonly B
lympho-cytes (or B cells). Lymphocytes complete their
differentiation and maturation primarily in the lymph nodes
and in the lymphoid tissue of the intestine and spleen after
exposure to a specific antigen. Mature lymphocytes are
antigen-specific cells.
Function of White Blood Cells
● WBCs protect the body from invasion by bacteria and other
foreign entities. The major function of neutrophils is
phagocytosis.
● Neutrophils arrive at the site within 1 hour after the onset of
an inflammatory reaction and initiate phagocytosis, but they
are short-lived.
● An influx of monocytes follows; these cells continue their
phagocytic activities for long periods as macrophages. This
 process constitutes a second line of defense for the body
against inflammation and infection.
● Although neutrophils can often work adequately against
bacteria without the need for excessive involvement with
macrophages, macrophages are particularly effective against
fungi and viruses. Macrophages also digest senescent (aging
or aged) blood cells, such as RBCs, primarily within the
spleen.
● …
Platelets (Thrombocytes)
● Platelets, or thrombocytes, are not actually cells. Rather, they
are granular fragments of giant cells in the bone marrow
called megakaryocytes.
● Platelet production in the marrow is regulated in part by the
hormone thrombopoietin, which stimulates the production
and differentiation of megakaryocytes from the myeloid stem
cell.
● Platelets play an essential role in the control of bleeding.
They circulate freely in the blood in an inactive state, where
they nurture the endothelium of the blood vessels,
maintaining the integrity of the vessel.
● When vascular injury does occur, platelets collect at the site
and are activated. They adhere to the site of in-jury and to
each other, forming a platelet plug that temporarily stops
bleeding.
● Substances released from platelet granules activate
coagulation factors in the blood plasma and initiate the
formation of a stable clot composed of fibrin, a filamentous
protein. Platelets have a normal life span of 7 to 10 days.
Plasma and Plasma Proteins
● After cellular elements are removed from blood, the
remaining liquid portion is called plasma. More than 90% of
plasma is water.
● The remainder consists primarily of plasma proteins, clot-ting
factors (particularly fibrinogen), and small amounts of other
substances such as nutrients, enzymes, waste products, and
gases. If plasma is allowed to clot, the remaining fluid is
called serum.
● Serum has essentially the same composition as plasma,
except that fibrinogen and several clotting factors have been
removed in the clotting process.
● Plasma proteins consist primarily of albumin and globulins.
The globulins can be separated into three main
fractions—alpha, beta, and gamma—each of which consists
of distinct proteins that have different functions.
● Important proteins in the alpha and beta fractions are the
transport globulins and the clotting factors that are made
in the liver.
● The transport globulins carry various substances in bound
form around the circulation. For example, thyroid-binding
globulin carries thyroxin, and transferrin carries iron.
● The clotting factors, including fibrinogen, remain in an
inac-tive form in the blood plasma until activated by the
clotting cas-cade.
● The gamma globulin fraction refers to the
immunoglobulins, or antibodies. These proteins are
produced by the well-differentiated lymphocytes and plasma
cells.
● The actual fractionation of the globulins can be seen on a
specific laboratory test (serum protein electrophoresis).
● Albumin is particularly important for the maintenance of
fluid balance within the vascular system. Capillary walls are
imperme-able to albumin, so its presence in the plasma
creates an osmotic force that keeps fluid within the vascular
space. Albumin, which is produced by the liver, has the
capacity to bind to several substances that are transported in
plasma (eg, certain medications, bilirubin, some hormones).
People with poor hepatic function may have low
concentrations of albumin, with a resultant decrease in
osmotic pressure and the development of edema.
Reticuloendothelial System (RES)
● The RES is composed of special tissue macrophages, which
are derived from monocytes.
● When released from the marrow, monocytes spend a short
time in the circulation (about 24 hours) and then enter the
body tissues. Within the tissues, the monocytes continue to
differentiate into cells called macrophages, which can survive
for months.
● Macrophages have a variety of important functions. They
defend the body against foreign invaders (ie, bacteria and
other pathogens) via phagocytosis. They remove old or
damaged cells from the circulation.
● They stimulate the inflammatory process and present antigen
to the immune system.
● Macrophages give rise to tissue histiocytes, including Kupffer
cells of the liver, peritoneal macrophages, alveolar
macrophages, and other components of the RES.
● Thus, the RES is a component of many other organs within
the body, particularly the spleen, lymph nodes, lung, and
liver.
● The spleen is the site of activity for most macrophages. Most
of the spleen (75%) is made of red pulp; here the blood enters
the venous sinuses through capillaries that are surrounded by
macrophages.
● Within the red pulp are tiny aggregates of white pulp,
consisting of B and T lymphocytes.
● The spleen is the site of activity for most macrophages. Most
of the spleen (75%) is made of red pulp; here the blood enters
the ve-nous sinuses through capillaries that are surrounded by
macrophages. Within the red pulp are tiny aggregates of
white pulp, consisting of B and T lymphocytes. The spleen
sequesters newly released reticulocytes from the marrow,
removing nuclear fragments and other materials (eg,
denatured hemoglobin, iron) before the now fully mature
RBC returns to the circulation. Although a minority of RBCs
(less than 5%) is pooled in the spleen, a signifi-cant
proportion of platelets (20%–40%) is pooled here. If the
spleen is enlarged, a greater proportion of RBCs and platelets
can be sequestered. The spleen is a major source of
hematopoiesis in fetal life. It can resume hematopoiesis later
in adulthood if neces-sary (eg, in bone marrow fibrosis). The
spleen has important im-munologic functions as well. It
forms a substance that promotes the phagocytosis of
neutrophils; it also forms the antibody IgM after exposure to
antigen.

Hemostasis
● Hemostasis is the process of preventing blood loss from
intact vessels and of stopping bleeding from a severed vessel.
The prevention of blood loss from intact vessels requires
adequate numbers of functional platelets. Platelets nurture the
endothelium and thereby maintain the structural integrity of
the vessel wall. Two processes are involved in arresting
bleeding: primary and secondary hemostasis.
● In primary hemostasis, the severed blood vessel constricts.
Circulating platelets aggregate at the site and adhere to the
vessel and to one another. An unstable hemostatic plug is
formed.
● For the coagulation process to be correctly activated,
circulating inactive coagulation factors must be converted to
active forms. This process occurs on the surface of the
aggregated platelets at the site of vessel injury.
● The end result is the formation of fibrin, which reinforces the
platelet plug and anchors it to the injury site. This process is
termed secondary hemostasis.
● The process of blood coagulation is highly complex. It can be
activated by the intrinsic or the extrinsic pathway. Both
pathways are needed for maintenance of normal hemostasis.
● Many factors are involved in the reaction cascade that forms
fibrin.
● When tissue is injured, the extrinsic pathway is activated by
the release from the tissue of a substance called
thromboplastin. As the result of a series of reactions,
prothrombin is converted to thrombin, which in turn catalyzes
the conversion of fibrinogen to fibrin.
● Clotting by the intrinsic pathway is activated when the
collagen that lines blood vessels is exposed. Clotting factors
are activated sequentially until, as with the extrinsic pathway,
fibrin is ultimately formed.
● Although the intrinsic pathway is slower, this sequence is
probably most often responsible for clotting in vivo.
● As the injured vessel is repaired and again covered with
en-dothelial cells, the fibrin clot is no longer needed.
● The fibrin is digested via two systems: the plasma fibrinolytic
system and the cellular fibrinolytic system.
● The substance plasminogen is required to lyse (break down)
the fibrin. Plasminogen, which is present in all body fluids,
circulates with fibrinogen and is therefore incorporated into
the fibrin clot as it forms.
● When the clot is no longer needed (eg, after an injured blood
vessel has healed), the plasminogen is activated to form
plasmin. Plasmin actually digests the fibrinogen, and the
breakdown particles of the clot (fibrin degradation products)
are released into the circulation. Through this system, clots
are dissolved as tissue is repaired, and the vascular system
returns to its normal baseline state.
Assessment and Diagnostic Findings
● Many hematologic conditions cause few symptoms.
Therefore, the use of extensive laboratory tests is often
required to diagnose a hematologic disorder.
● For most hematologic conditions, contin-ued monitoring via
specific blood tests is required because it is very important to
assess for changes in test results over time.
Hematologic Studies
● The most common tests used are the complete blood count
(CBC) and the peripheral blood smear.
● The CBC identifies the total number of blood cells (WBCs,
RBCs, and platelets) as well as the hemoglobin, hematocrit
(percentage of blood consisting of RBCs), and RBC indices.
● Because cellular morphology (shape and appearance of the
cells) is particularly important in most hematologic disorders,
the physician needs to examine the blood cells involved.
● This process is referred to as the manual examination of the
peripheral smear, which may be part of the CBC.
● In this test, a drop of blood is spread on a glass slide, stained,
and examined under a microscope.
● The shape and size of the RBCs and platelets as well as the
actual appearance of the WBCs provides useful information
in identifying hematologic conditions.
● Blood for the CBC is typically obtained by venipuncture.

Management of Hematologic Disorders
● Commonly encountered blood disorders are anemia,
polycythemia, leukopenia and neutropenia, leukocytosis,
lymphoma, myeloma, leukemia, and various bleeding and
coagulation disorders.
● Nursing management of patients with these disorders requires
skill-ful assessment and monitoring as well as meticulous
care and teach-ing to prevent deterioration and complications.
Anemia
● Anemia, per se, is not a specific disease state but a sign of an
underlying disorder.
● It is by far the most common hematologic condition. Anemia,
a condition in which the hemoglobin concentration is lower
than normal, reflects the presence of fewer than normal RBCs
within the circulation.
● As a result, the amount of oxygen delivered to body tissues is
also diminished.
● There are many different kinds of anemia, but all can be
classified into three broad etiologic categories:
ー Loss of RBCs—occurs with bleeding, potentially from any
major source, such as the gastrointestinal tract, the uterus,
the nose, or a wound
ー Decreased production of RBCs—can be caused by a
defi-ciency in cofactors (including folic acid, vitamin B12,
and iron) required for erythropoiesis; RBC production may
also be reduced if the bone marrow is suppressed (eg, by
tumor, medications, toxins) or is inadequately stimulated
because of a lack of erythropoietin (as occurs in chronic
renal disease).
ー Increased destruction of RBCs—may occur because of an
overactive RES (including hypersplenism) or because the
bone marrow produces abnormal RBCs that are then
destroyed by the RES (eg, sickle cell anemia).
● A conclusion as to whether the anemia is caused by
destruc-tion or by inadequate production of RBCs usually can
be reached on the basis of the following factors:
ー The marrow’s ability to respond to the decreased RBCs (as
evidenced by an increased reticulocyte count in the
circu-lating blood)
ー The degree to which young RBCs proliferate in the bone
marrow and the manner in which they mature (as observed
on bone marrow biopsy)
ー The presence or absence of end products of RBC
destruction within the circulation (eg, increased bilirubin
level, decreased haptoglobin level)
Classification of Anemia
● Anemia may be classified in several ways.
● The physiologic approach is to determine whether the
deficiency in RBCs is caused by a defect in their production
(hypoproliferative anemia), by their destruction (hemolytic
anemia), or by their loss (bleeding).
● In the hypoproliferative anemias, RBCs usually survive
nor-mally, but the marrow cannot produce adequate numbers
of these cells. The decreased production is reflected in a low
reticulocyte count.
● Inadequate production of RBCs may result from marrow
damage due to medications or chemicals (eg,
chloramphenicol, benzene) or from a lack of factors
necessary for RBC formation (eg, iron, vitamin B12, folic
acid, erythropoietin).
● Hemolytic anemias stem from premature destruction of
RBCs, which results in a liberation of hemoglobin from the
RBC into the plasma.
● The increased RBC destruction results in tissue hypoxia,
which in turn stimulates erythropoietin production.
● This increased production is reflected in an increased
reticulocyte count, as the bone marrow responds to the loss of
RBCs.
● The released hemo-globin is converted in large part to
bilirubin; therefore, the biliru-bin concentration rises.
● Hemolysis can result from an abnormality within the RBC
itself (eg, sickle cell anemia, glucose-6-phosphate
dehydrogenase [G-6-PD] deficiency) or within the plasma
(eg, immune hemolytic anemias), or from direct injury to the
RBC within the circulation (eg, hemolysis caused by
mechanical heart valve).
Clinical Manifestations
● Aside from the severity of the anemia itself, several factors
influ-ence the development of anemia-associated symptoms:
ー The speed with which the anemia has developed
ー The duration of the anemia (ie, its chronicity)
ー The metabolic requirements of the individual
ー Other concurrent disorders or disabilities (eg,
cardio-pulmonary disease)
ー Special complications or concomitant features of the
con-dition that produced the anemia
● In general, the more rapidly an anemia develops, the more
severe its symptoms.
● An otherwise healthy person can often tolerate as much as a
50% gradual reduction in hemoglobin without pronounced
symptoms or significant incapacity, whereas the rapid loss of
as little as 30% may precipitate profound vascular collapse in
the same individual.
● A person who has been anemic for a very long time, with
hemoglobin levels between 9 and 11 g/dL, usually has few or
no symptoms other than slight tachycardia on exertion and
fatigue.
● Patients who customarily are very active or who have
significant demands on their lives (eg, a single, working
mother of small children) are more likely to have symptoms,
and those symptoms are more likely to be pronounced than in
a more sedentary person.
● …
Complications
● General complications of severe anemia include heart failure,
paresthesias, and confusion.
● At any given level of anemia, patients with underlying heart
disease are far more likely to have angina or symptoms of
heart failure than those without heart disease.
● Complications associated with specific types of anemia are
included in the description of each type.
Medical Management
● Management of anemia is directed toward correcting or
control-ling the cause of the anemia; if the anemia is severe,
the RBCs that are lost or destroyed may be replaced with a
transfusion of packed RBCs (PRBCs).
Nursing Process: The Patient with Anemia
Assessment
● The health history and physical examination provide
important data about the type of anemia involved, the extent
and type of symptoms it produces, and the impact of those
symptoms on the patient’s life.
● Weakness, fatigue, and general malaise are com-mon, as are
pallor of the skin and mucous membranes (sclera, oral
mucosa).
● Jaundice may be present in patients with megaloblastic
anemia or hemolytic anemia.
● The tongue may be smooth and red (in iron deficiency
anemia) or beefy red and sore (in megaloblastic ane-mia); the
corners of the mouth may be ulcerated (angular cheilo-sis) in
both types of anemia.
● Individuals with iron deficiency anemia may crave ice,
starch, or dirt (known as pica); their nails may be brittle,
ridged, and concave.
● The health history should include a medication history,
because some medications can depress bone marrow activity
or interfere with folate metabolism.
● An accurate history of alcohol intake, including the amount
and duration, should be obtained. Family history is important,
because certain anemias are inherited.
● Athletic endeavors should be assessed, because extreme
exercise can decrease erythropoiesis and RBC survival in
some athletes.
● A nutritional assessment is important, because it may indicate
deficiencies in essential nutrients such as iron, vitamin B12,
and folic acid.
● Children of indigent families may be at higher risk for anemia
because of nutritional deficiencies.
● Strict vegetarians are also at risk for megaloblastic types of
anemia if they do not supplement their diet with vitamin B12.
● Cardiac status should be carefully assessed.
● When the hemoglobin level is low, the heart attempts to
compensate by pumping faster and harder in an effort to
deliver more blood to hypoxic tissue.
● This increased cardiac workload can result in such symptoms
as tachycardia, palpitations, dyspnea, dizziness, orthopnea,
and exertional dyspnea.
● Heart failure may eventually develop, as evidenced by an
enlarged heart (cardiomegaly) and liver (hepato-megaly) and
by peripheral edema.
● Stools should be tested for occult blood.
● Women should be questioned about their menstrual periods
(eg, excessive menstrual flow, other vaginal bleeding) and the
use of iron sup-plements during pregnancy.
● The neurologic examination is also important because of the
effect of pernicious anemia on the central and peripheral
nervous systems.
● Assessment should include the presence and extent of
peripheral numbness and paresthesias, ataxia, poor
coordination, and confusion.
● Finally, it is important to monitor relevant labo-ratory test
results and to note any changes over time.
Diagnosis
● Nursing Diagnoses
ー Based on the assessment data, major nursing diagnoses for
the anemic patient may include:
○ Activity intolerance related to weakness, fatigue, and
general malaise
 ○ Imbalanced nutrition, less than body requirements,
related to inadequate intake of essential nutrients
○ Ineffective tissue perfusion related to inadequate blood
volume or hematocrit
○ Noncompliance with prescribed therapy
● Collaborative Problems / Potential Complications
ー Based on the assessment data, potential complications that
may develop include:
○ Heart failure
○ Paresthesias
○ Confusion
Planning and Goals
● The major goals for the patient may include increased
tolerance of normal activity, attainment or maintenance of
adequate nutri-tion, maintenance of adequate tissue perfusion,
compliance with prescribed therapy, and absence of
complications.
Nursing Interventions
● Managing fatigue
ー The most frequent symptom and complication of anemia is
fatigue. This distressing symptom is too often minimized
by health care providers.
ー Fatigue is often the symptom that has the greater negative
impact on the individual’s level of functioning and
con-sequent quality of life.
ー Patients describe the fatigue from anemia as oppressive.
ー Fatigue can be significant, yet the anemia may not be
severe enough to warrant transfusion. Fatigue can interfere
with an individual’s ability to work, both inside and
outside the home. It can harm relationships with family
and friends.
ー Patients often lose interest in hobbies and activities,
including sexual activity.
ー The distress from fatigue is often related to an individual’s
responsibilities and life demands as well as the amount of
assis-tance and support received from others.
ー Nursing interventions can focus on assisting the patient to
prioritize activities and to establish a balance between
activity and rest that is realistic and feasible from the
patient’s perspective.
ー Patients with chronic anemia need to maintain some
physical activity and exercise to prevent the
deconditioning that results from inactivity.
● Maintaining adequate nutrition
ー Inadequate intake of essential nutrients, such as iron,
vitamin B12, folic acid, and protein can cause some
anemias.
ー The symptoms associated with anemia (eg, fatigue,
anorexia) can in turn interfere with maintaining adequate
nutrition.
ー A healthy diet should be en-couraged. Because alcohol
interferes with the utilization of essen-tial nutrients, the
nurse should advise the patient to avoid alcoholic
beverages or to limit their intake and should provide the
rationale for this recommendation.
ー Dietary teaching sessions should be individualized,
including cultural aspects related to food preferences and
food preparation.
ー The involvement of family members enhances compliance
with dietary recommendations. Dietary supplements (eg,
vitamins, iron, folate, protein) may be prescribed as well.
ー Equally important, the patient and family must understand
the role of nutritional supplements in the proper context,
because many forms of anemia are not the result of a
nutritional deficiency.
ー In such cases, excessive intake of nutritional supplements
will not improve the anemia.
ー A potential problem in individuals with chronic
transfusion requirements occurs with the indiscriminate
use of iron.
ー Unless an aggressive program of chelation therapy is
implemented, these individuals are at risk for iron
overload from their transfusions alone.
ー The addition of an iron supplement only exacerbates the
situation.
● Maintaining adequate perfusion
ー Patients with acute blood loss or severe hemolysis may
have decreased tissue perfusion from decreased blood
volume or reduced circulating RBCs (decreased
hematocrit).
ー Lost volume is replaced with transfusions or intravenous
fluids, based on the symptoms and the laboratory findings.
ー Supplemental oxygen may be necessary, but it is rarely
needed on a long-term basis unless there is underlying
severe cardiac or pulmonary disease as well. The nurse
monitors vital signs closely; other medications, such as
antihypertensive agents, may need to be adjusted or
withheld.
● Promoting compliance with prescribed therapy
ー For patients with anemia, medications or nutritional
supplements are often prescribed to alleviate or correct the
condition.
ー These pa-tients need to understand the purpose of the
medication, how to take the medication and over what
time period, and how to man-age any side effects of
therapy.
ー To enhance compliance, the nurse can assist patients in
developing ways to incorporate the therapeu-tic plan into
their lives, rather than merely giving the patient a list of
instructions.
ー For example, many patients have difficulty taking iron
supplements because of related gastrointestinal effects.
Rather than seeking assistance from a health care provider
in managing the problem, some of these patients simply
stop taking the iron.
ー Abruptly stopping some medications can have serious
conse-quences, as in the case of high-dose corticosteroids
to manage hemolytic anemias.
ー Some medications, such as growth factors, are extremely
expensive.
ー Patients receiving these medications may need assistance
with obtaining needed insurance coverage or with
exploring alternatives for obtaining these medications.
● Monitoring and managing potential complications
 ー A significant complication of anemia is heart failure from
chronic diminished blood volume and the heart’s
compensatory effort to increase cardiac output.
ー Patients with anemia should be assessed for signs and
symptoms of heart failure.
ー A serial record of body weights can be more useful than a
record of dietary intake and output, because the intake and
output measurements may not be accurate.
ー In the case of fluid retention resulting from congestive
heart failure, diuretics may be required.
ー In megaloblastic forms of anemia, the significant potential
complications are neurologic. A neurologic assessment
should be performed for patients with known or suspected
megaloblastic anemia.
ー Patients may initially complain of paresthesias in their
lower extremities. These paresthesias are usually
manifested as numbness and tingling on the bottom of the
foot, and they grad-ually progress.
ー As the anemia progresses and damage to the spinal cord
occurs, other signs become apparent. Position and
vibration sense may be diminished; difficulty maintaining
balance is not uncommon, and some patients have gait
disturbances as well.
ー Initially mild but gradually progressive confusion may
develop.
Evaluation
● Expected patient outcomes
ー Tolerates activity at a safe and acceptable level
○ Follows a progressive plan of rest, activity, and
exercise
○ Prioritizes activities
○ Paces activities according to energy level
ー Attains and maintains adequate nutrition
○ Eats a healthy diet
○ Develops meal plan that promotes optimal nutrition
○ Maintains adequate amounts of iron, vitamins, and
protein from diet or supplements
○ Adheres to nutritional supplement therapy when
prescribed
○ Verbalizes understanding of rationale for using
recom-mended nutritional supplements
○ Verbalizes understanding of rationale for avoiding
non-recommended nutritional supplements
ー Maintains adequate perfusion
○ Has vital signs within baseline for patient
○ Has pulse oximetry (arterial oxygenation) value within
normal limits
ー Absence of complications
○ Avoids or limits activities that cause dyspnea,
palpita-tions, dizziness, or tachycardia
○ Uses rest and comfort measures to alleviate dyspnea
○ Has vital signs within baseline for patient
○ Has no signs of increasing fluid retention (eg,
peripheral edema, decreased urine output, neck vein
distention)
○ Remains oriented to time, place, and situation
○ Ambulates safely, using assistive devices as necessary
○ Remains free of injury
○ Verbalizes understanding of importance of serial CBC
measurements
○ Maintains safe home environment; obtains assistance
as necessary.
Hypoproliferative Anemias
Iron Deficiency Anemia
● Iron deficiency anemia typically results when the intake of
dietary iron is inadequate for hemoglobin synthesis. The body
can store about one fourth to one third of its iron, and it is not
until those stores are depleted that iron deficiency anemia
actually begins to develop. Iron deficiency anemia is the most
common type of ane-mia in all age groups, and it is the most
common anemia in the world. More than 500 million people
are affected, more com-monly in underdeveloped countries,
where inadequate iron stores can result from inadequate
intake of iron (seen with vegetarian diets) or from blood loss
(eg, from intestinal hookworm).
● Iron deficiency is also common in the United States. In
children, ado-lescents, and pregnant women, the cause is
typically inadequate iron in the diet to keep up with increased
growth. However, for most adults with iron deficiency
anemia, the cause is blood loss. In fact, in adults, the cause of
iron deficiency anemia should be considered to be bleeding
until proven otherwise.
● The most common cause of iron deficiency in men and
postmenopausal women is bleeding (from ulcers, gastritis,
inflammatory bowel disease, or gastrointestinal tumors).
● The most common cause of iron deficiency anemia in
premenopausal women is men-orrhagia (excessive menstrual
bleeding) and pregnancy with in-adequate iron
supplementation.
● Patients with chronic alcoholism often have chronic blood
loss from the gastrointestinal tract, which causes iron loss and
eventual anemia.
● Other causes include iron malabsorption, as is seen after
gastrectomy or with celiac disease.
Clinical Manifestations
● Patients with iron deficiency primarily have the symptoms of
anemia.
● If the deficiency is severe or prolonged, they may also have a
smooth, sore tongue, brittle and ridged nails, and angular
cheilosis (an ulceration of the corner of the mouth). These
signs subside after iron-replacement therapy.
● The health history may be significant for multiple
pregnancies, gastrointestinal bleeding, and pica (a craving for
unusual substances, such as ice, clay, or laundry starch).
Assessment and Diagnostic Findings
● The most definitive method of establishing the diagnosis of
iron deficiency anemia is bone marrow aspiration.
● The aspirate is stained to detect iron, which is at a low level
or even absent. However, few patients with suspected iron
deficiency anemia undergo bone marrow aspiration.
● In many patients, the diagnosis can be established with other
tests, particularly in patients with a history of conditions that
predispose them to this type of anemia.
● There is a strong correlation between laboratory values
measuring iron stores and levels of hemoglobin.
Medical Management
● Except in the case of pregnancy, the cause of iron deficiency
should be investigated. Anemia may be a sign of a curable
gastro-intestinal cancer or of uterine fibroid tumors. Stool
specimens should be tested for occult blood.
● People 50 years of age or older should have a colonoscopy,
endoscopy, or other examination of the gastrointestinal tract
to detect ulcerations, gastritis, polyps, or cancer.
● Several oral iron preparations—ferrous sulfate, ferrous
gluconate, and ferrous fumarate—are available for treating
iron deficiency anemia. In some cases, oral iron is poorly
absorbed or poorly tolerated, or iron supplementation is
needed in large amounts. In these situations, intravenous or
intramuscular administration of iron dextran may be needed.
● Before parenteral administration of a full dose, a small test
dose should be administered to avoid the risk of anaphylaxis
with either intravenous or intramuscular injections.
● Emergency medications (eg, epinephrine) should be close at
hand.
● If no signs of allergic reaction have occurred after 30
minutes, the remaining dose of iron may be administered.
● Several doses are required to replenish the patient’s iron
stores.
Nursing Management
● Preventive education is important, because iron deficiency
anemia is common in menstruating and pregnant women.
● Food sources high in iron include organ meats (beef or calf’s
liver, chicken liver), other meats, beans (black, pinto, and
garbanzo), leafy green vegetables, raisins, and molasses.
● Taking iron-rich foods with a source of vitamin C enhances
the absorption of iron.
● Patients with a history of eating fad diets or strict vegetar-ian
diets are counseled that such diets often contain inadequate
amounts of absorbable iron.
● The nurse encourages patients to continue iron therapy as
long as it is prescribed, although they may no longer feel
fatigued.
● Because iron is best absorbed on an empty stomach, patients
should be advised to take the supplement an hour before
meals.
● Most patients can use the less expensive, more standard
forms of ferrous sulfate. Tablets with enteric coating may be
poorly absorbed and should be avoided.
● Other patients have difficulty taking iron supplements
because of gastrointestinal side effects (primarily
constipation, but also cramping, nausea, and vomiting).
● Some iron formulations are designed to limit gastrointestinal
side effects by the addition of a stool softener or use of
sustained-release formulations to limit nausea or gastritis.
● If taking iron on an empty stomach causes gastric distress, the
patient may need to take the iron supplement with meals.
How-ever, doing so diminishes iron absorption by as much as
50%, thus prolonging the time required to replenish iron
stores.
● Antacids or dairy products should not be taken with iron,
because they greatly diminish the absorption of iron.
Polysaccharide iron complex forms that have less
gastrointestinal toxicity are also available, but they are more
expensive.
● Liquid forms of iron that cause less gastrointestinal distress
are available. However, they can stain the teeth; patients
should be instructed to take this medication through a straw,
to rinse the mouth with water, and to practice good oral
hygiene after taking this medication.
● Finally, patients should be informed that iron salts may color
the stool dark green or black. However, iron replacement
therapy does not cause a false-positive result on stool
analyses for occult blood.
● Intramuscular supplementation is used infrequently. The
vol-ume of iron required may be excessive. The
intramuscular injec-tion causes some local pain and can stain
the skin. These side effects are minimized by using the
Z-track technique for administering iron dextran deep into the
gluteus maximus muscle (buttock).
● Avoid vigorously rubbing the injection site after the injection.
Because of the problems with intramuscular administration,
the intravenous route is preferred for administration of iron
dextran.
Anemias in Renal Disease
● The degree of anemia in patients with end-stage renal disease
varies greatly, but in general patients do not become
significantly anemic until the serum creatinine level exceeds
3 mg/100 mL.
● The symptoms of anemia are often the most disturbing of the
patient’s symptoms.
● The hematocrit usually falls to between 20% and 30%,
although in rare cases it may fall to less than 15%.
● The RBCs appear normal on the peripheral smear.
● This anemia is caused by both a mild shortening of RBC life
span and a deficiency of erythropoietin (necessary for
erythropoiesis).
● As renal function decreases, erythropoietin, which is
produced by the kidney, also decreases.
● Because erythropoietin is also produced outside the kidney,
some erythropoiesis does continue, even in patients whose
kidneys have been removed. However, the amount is small
and the degree of erythropoiesis is inadequate.
● Patients undergoing long-term hemodialysis lose blood into
the dialyzer and therefore may become iron deficient. Folic
acid deficiency develops because this vitamin passes into
the dialysate. Therefore, patients who receive hemodialysis
and who are ane-mic should be evaluated for iron and folate
deficiency and treated appropriately.
● The availability of recombinant erythropoietin (epoetin alfa
[Epogen, Procrit]) has dramatically altered the management
of ane-mia in end-stage renal disease by decreasing the need
for RBC transfusion, with its associated risks.
● Erythropoietin, in combination with oral iron supplements,
can raise and maintain hematocrit levels to between 33% and
38%. This treatment has been successful with dialysis
patients.
● Many patients report decreased fatigue, increased energy,
increased feelings of well-being, improved exer-cise
tolerance, better tolerance of dialysis treatments, and
improved quality of life.
● Hypertension is the most serious side effect in this patient
population when the hematocrit rapidly increases to a high
level. Therefore, the hematocrit should be checked frequently
when a patient with renal disease begins erythropoietin
therapy.
● The dose of erythropoietin (epoetin alfa) should be titrated to
the hematocrit. In some patients, the elevated hematocrit and
associated hypertension may necessitate antihypertensive
therapy.
Aplastic Anemia
● Aplastic anemia is a rather rare disease caused by a decrease
in or damage to marrow stem cells, damage to the
microenvironment within the marrow, and replacement of the
marrow with fat.
● It results in bone marrow aplasia (markedly reduced
hematopoiesis).
● Therefore, in addition to severe anemia, significant
neutropenia and thrombocytopenia (a deficiency of platelets)
are also seen.
Pathophysiology
● Aplastic anemia can be congenital or acquired, but most cases
are idiopathic (ie, without apparent cause).
● Infections and pregnancy can trigger it, or it may be caused
by certain medications, chem-icals, or radiation damage.
● Agents that regularly produce marrow aplasia include
benzene and benzene derivatives (eg, airplane glue).
● Certain toxic materials, such as inorganic arsenic and several
pesticides (including DDT, which is no longer used or
available in the United States), have also been implicated as
potential causes.
● Various medications have been associated with aplastic
anemia.
Clinical Manifestations
● The manifestations of aplastic anemia are often insidious.
● Complications resulting from bone marrow failure may occur
before the diagnosis is established.
● Typical complications are infection and symptoms of anemia
(eg, fatigue, pallor, dyspnea).
● Purpura (bruising) may develop later and should trigger a
CBC and hematologic evaluation if these were not performed
initially.
● If the patient has had repeated throat infections, cervical
lymphadenopathy may be seen.
● Other lymphadenopathies and splenomegaly sometimes
occur.
● Retinal hemorrhages are common.
Assessment and Diagnostic Findings
● Findings in many situations, aplastic anemia occurs when a
medication or chemical is ingested in toxic amounts.
● However, in a few people, it develops after a medication has
been taken at the recommended dosage.
● This may be considered an idiosyncratic reaction in those
who are highly susceptible, possibly caused by a genetic
defect in the medication biotransformation or elimination
process.
● A bone marrow aspirate shows an extremely hypoplastic or
even aplastic (very few to no cells) marrow replaced with fat.
Medical Management
● It is presumed that the lymphocytes of patients with aplastic
anemia destroy the stem cells and consequently impair the
production of RBCs, WBCs, and platelets.
● Despite its severity, aplastic anemia can be successfully
treated in most people.
● Potentially, those who are younger than 60 years of age, who
are otherwise healthy, and who have a compatible donor can
be cured of the dis-ease by a bone marrow transplantation
(BMT) or peripheral stem cell transplantation (BSCT).
● In others, the disease can be managed with
immunosuppressive therapy. A combination of antithymocyte
globulin and cyclosporine is used most commonly.
● Immuno-suppressants prevent the patient’s lymphocytes from
destroying the stem cells.
● If relapse occurs (ie, the patient becomes pancytopenic
again), reinstitution of the same immunologic agents may
induce another remission.
● Corticosteroids are not very useful as an immunosuppressive
agent, because patients with aplastic anemia appear
particularly susceptible to the development of bone
complications from corticosteroids (ie, aseptic necrosis of the
head of the femur).
● Supportive therapy plays a major role in the management of
aplastic anemia. Any offending agent is discontinued. The
patient is supported with transfusions of RBCs and platelets
as necessary. Death usually is caused by hemorrhage or
infection.
Nursing Management
● Patients with aplastic anemia are vulnerable to problems
related to RBC, WBC, and platelet deficiencies. They should
be assessed carefully for signs of infection and bleeding.
 Specific interventions are delineated in the sections on
neutropenia and thrombocytopenia.
Megaloblastic Anemia
● In the anemias caused by deficiencies of vitamin B12 or folic
acid, identical bone marrow and peripheral blood changes
occur, because both vitamins are essential for normal DNA
synthesis.
● In either anemia, the RBCs that are produced are abnormally
large and are called megaloblastic RBCs. Other cells derived
from the myeloid stem cell (nonlymphoid WBCs, platelets)
are also abnormal. A bone marrow analysis reveals
hyperplasia (abnormal increase in the number of cells), and
the precursor erythroid and myeloid cells are large and
bizarre in appearance. Many of these abnormal RBCs and
myeloid cells are destroyed within the mar-row, however, so
the mature cells that do leave the marrow are ac-tually fewer
in number.
● Thus, pancytopenia (a decrease in all myeloid-derived cells)
can develop.
● In an advanced situation, the hemoglobin value may be as
low as 4 to 5 g/dL, the WBC count 2,000 to 3,000/mm3, and
the platelet count less than 50,000/mm3. Those cells that are
released into the circulation are often abnormally shaped. The
neutrophils are hypersegmented. The platelets may be
abnormally large. The RBCs are abnormally shaped, and the
shapes may vary widely (poikilocytosis). Because the RBCs
are very large, the MCV is very high, usually exceeding 110
μ m3.
Pathophysiology
★ Folic Acid Deficiency
● Folic acid, a vitamin that is necessary for normal RBC
production, is stored as compounds referred to as folates. The
folate stores in the body are much smaller than those of
vitamin B12, and they are quickly depleted when the dietary
intake of folate is deficient (within 4 months).
● Folate is found in green vegetables and liver. Folate
deficiency occurs in people who rarely eat uncooked
vegetables.
● Alcohol increases folic acid requirements, and, at the same
time, patients with alcoholism usually have a diet that is
deficient in the vitamin.
● Folic acid requirements are also increased in patients with
chronic hemolytic anemias and in women who are pregnant,
because the need for RBC production is increased in these
conditions.
● Some patients with malabsorptive diseases of the small
bowel, such as sprue, may not absorb folic acid normally.
★ Vitamin B12 Deficiency
● A deficiency of vitamin B12 can occur in several ways.
Inadequate dietary intake is rare but can develop in strict
vegetarians who consume no meat or dairy products.
● Faulty absorption from the gastrointestinal tract is more
common. This occurs in conditions such as Crohn’s disease,
or after ileal resection or gastrectomy.
● Another cause is the absence of intrinsic factor, as in
pernicious anemia. Intrinsic factor is normally secreted by
cells within the gastric mucosa; normally it binds with the
dietary vitamin B12 and travels with it to the ileum, where
the vitamin is absorbed. Without intrinsic factor, orally
consumed vitamin B12 cannot be absorbed, and RBC
production is eventually diminished. Even if adequate
vitamin B12 and intrinsic factor are present, a deficiency may
occur if disease involving the ileum or pancreas impairs
absorption.
● Pernicious anemia, which tends to run in families, is
primarily a disorder of adults, particularly the elderly. The
abnormality is in the gastric mucosa: the stomach wall
atrophies and fails to secrete intrinsic factor. Therefore, the
absorption of vitamin B12 is significantly impaired.
● The body normally has large stores of vitamin B12, so years
may pass before the deficiency results in anemia. Because the
body compensates so well, the anemia can be severe before
the patient becomes symptomatic. For unknown reasons,
patients with pernicious anemia have a higher incidence of
gastric cancer than the general population; these patients
should have endoscopies at regular intervals (every 1 to 2
years) to screen for early gastric cancer.
Clinical Manifestations
● Symptoms of folic acid and vitamin B12 deficiencies are
similar, and the two anemias may coexist. However, the
neurologic man-ifestations of vitamin B12 deficiency do not
occur with folic acid deficiency, and they persist if B12 is not
replaced. Therefore, care-ful distinction between the two
anemias must be made. Serum lev-els of both vitamins can be
measured. In the case of folic acid deficiency, even small
amounts of folate will increase the serum fo-late level,
sometimes to normal. Measuring the amount of folate within
the RBC itself (red cell folate) is therefore a more sensitive
test in determining true folate deficiency.
● After the body stores of vitamin B12 are depleted, patients
may begin to show signs of the anemia. However, because
the onset and progression of the anemia are so gradual, the
body can compensate very well until the anemia is severe, so
that the typical manifestations of anemia (weakness,
listlessness, fatigue) may not be apparent initially. The
hematologic effects of deficiency are accompanied by effects
on other organ systems, particularly the gastrointestinal tract
and nervous system.
● Patients with pernicious anemia develop a smooth, sore, red
tongue and mild diarrhea. They are extremely pale,
particularly in the mucous membranes.
● They may become confused; more often they have
paresthesias in the extremities (particularly numbness and
tingling in the feet and lower legs).
● They may have difficulty maintaining their balance because
of damage to the spinal cord, and they also lose position
sense (proprioception).
● These symptoms are progressive, although the course of
illness may be marked by spontaneous partial remissions and
exacerbations. Without treatment, patients can die after
several years, usually from heart failure secondary to anemia.
Assessment and Diagnostic Findings
● The classic method of determining the cause of vitamin B12
deficiency is the Schilling test, in which the patient receives a
 small oral dose of radioactive vitamin B12, followed in a few
hours by a large, nonradioactive parenteral dose of vitamin
B12 (this aids in renal excretion of the radioactive dose).
● If the oral vitamin is absorbed, more than 8% will be excreted
in the urine within 24 hours; therefore, if no radioactivity is
present in the urine (ie, the radioactive vitamin B12 stays
within the gastrointestinal tract), the cause is gastrointestinal
malabsorption of the vitamin B12.
● Conversely, if the urine is radioactive, the cause of the
deficiency is not ileal disease or pernicious anemia.
● Later, the same procedure is repeated, but this time intrinsic
factor is added to the oral radioactive vitamin B12.
● If radioactivity is now detected in the urine (ie, the B12 was
absorbed from the gastrointestinal tract in the presence of
intrinsic factor), the diagnosis of pernicious anemia can be
made.
● The Schilling test is useful only if the urine collections are
complete; therefore, the nurse must promote the patient’s
understanding and ability to comply with this collection.
Medical Management
● Folate deficiency is treated by increasing the amount of folic
acid in the diet and administering 1 mg of folic acid daily.
Folic acid is administered intramuscularly only for people
with malabsorption problems.
● With the exception of the vitamins administered during
pregnancy, most proprietary vitamin preparations do not
contain folic acid, so it must be administered as a separate
tablet.
● After the hemoglobin level returns to normal, the folic acid
replacement can be stopped.
● However, patients with alcoholism should continue receiving
folic acid as long as they continue alcohol consumption.
● Vitamin B12 deficiency is treated by vitamin B12
replacement.
● Vegetarians can prevent or treat deficiency with oral
supplements through vitamins or fortified soy milk.
● When, as is more common, the deficiency is due to defective
absorption or absence of intrinsic factor, replacement is by
monthly intramuscular injections of vitamin B12, usually at a
dose of 1000 μ g.
● The reticulocyte count rises within 1 week, and in several
weeks the blood counts are all normal. The tongue improves
in several days.
● However, the neurologic manifestations require more time for
recovery; if there is severe neuropathy, the patient may never
recover fully. To prevent recurrence of pernicious anemia,
vitamin B12 therapy must be continued for life.
Nursing Management
● Assessment of patients who have or are at risk for
megaloblastic anemia includes inspection of the skin and
mucous membranes.
● Mild jaundice may be apparent and is best seen in the sclera
without using fluorescent lights. Vitiligo (patchy loss of skin
pigmentation) and premature graying of the hair are often
seen in patients with pernicious anemia. The tongue is
smooth, red, and sore.
● Because of the neurologic complications associated with
these anemias, a careful neurologic assessment is important,
including tests of position and vibration sense.
Hemolytic Anemia
● In hemolytic anemias, the RBCs have a shortened life span;
thus, the number of RBCs in circulation is reduced.
● Fewer RBCs result in decreased in available oxygen causes
hypoxia, which in turn stimulates an increase in
erythropoietin release from the kidney.
● The erythropoietin stimulates the bone marrow to compensate
by producing new RBCs and releasing some of them into the
circulation somewhat prematurely as reticulocytes.
● If the RBC destruction persists, the hemoglobin is broken
down excessively; about 80% of the heme is converted to
bilirubin, conjugated in the liver, and excreted in the bile.
● The mechanism of RBC destruction varies, but all types of
hemolytic anemia share certain laboratory features:
ー The reticulocyte count is elevated,
ー The fraction of indirect (unconjugated) bilirubin is
increased, and
ー The supply of haptoglobin (a binding protein for free
hemoglobin) is depleted as more hemoglobin is released.
● As a result, the plasma haptoglobin level is low. If the
marrow cannot compensate to replace the RBCs (indicated by
a decreased reticulocyte count), the anemia will progress.
Sickle Cell Anemia
● Sickle cell anemia is a severe hemolytic anemia that results
from inheritance of the sickle hemoglobin gene.
● This gene causes the hemoglobin molecule to be defective.
The sickle hemoglobin (HbS) acquires a crystal-like
formation when exposed to low oxygen tension.
● The oxygen level in venous blood can be low enough to
cause this change; consequently, the RBC containing (HbS)
loses its round, very pliable, biconcave disk shape and
becomes deformed, rigid, and sickle-shaped.
● These long, rigid RBCs can adhere to the endothelium of
small vessels; when they pile up against each other, blood
flow to a region or an organ may be reduced.
● If ischemia or infarction results, the patient may have pain,
swelling, and fever. The sickling process takes time; if the
RBC is again exposed to adequate amounts of oxygen (eg,
when it travels through the pulmonary circulation) before the
membrane becomes too rigid, it can revert to a normal shape.
For this reason, the “sickling crises” are intermittent.
● Cold can aggravate the sickling process, because
vasoconstriction slows the blood flow.
● Oxygen delivery can also be impaired by an increased blood
viscosity, with or without occlusion due to adhesion of
sickled cells; in this situation, the effects are seen in larger
vessels, such as arterioles.
● The HbS gene is inherited in people of African descent and to
a lesser extent in people from the Middle East, the
Mediterranean area, and aboriginal tribes in India.
● Sickle cell anemia is the most severe form of sickle cell
disease. Less severe forms include sickle cell hemoglobin C
(SC) disease, sickle cell hemoglobin D (SD) disease, and
sickle cell beta-thalassemia.
● The clinical manifestations and management are the same as
for sickle cell anemia.
● The term sickle cell trait refers to the carrier state for SC
diseases; it is the most benign type of SC disease, in that less
than 50% of the hemoglobin within an RBC is HbS.
● However, in terms of genetic counseling, it is still an
important condition. If two people with sickle cell trait have
children, the children may inherit two abnormal genes. These
children will produce only HbS and therefore will have sickle
cell anemia.
Clinical Manifestations
● Symptoms of sickle cell anemia vary and are only somewhat
based on the amount of HbS.
● Symptoms and complications result from chronic hemolysis
or thrombosis.
● The sickled RBCs have a shortened life span. Patients are
always anemic, usually with hemoglobin values of 7 to 10
g/dL.
● Jaundice is characteristic and is usually obvious in the
sclerae.
● The bone marrow expands in childhood in a compensatory
effort to offset the anemia, sometimes leading to enlargement
of the bones of the face and skull.
● The chronic anemia is associated with tachycardia, cardiac
murmurs, and often an enlarged heart (cardiomegaly).
Dysrhythmias and heart failure may occur in adults.
● Virtually any organ may be affected by thrombosis, but the
primary sites involve those areas with slowed circulation,
such as the spleen, lungs, and central nervous system.
● All the tissues and organs are constantly vulnerable to
microcirculatory interruptions by the sickling process and
therefore are susceptible to hypoxic damage or true ischemic
necrosis.
● Patients with sickle cell anemia are unusually susceptible to
infection, particularly pneumonia and osteomyelitis.
● Complications of sickle cell anemia include infection, stroke,
renal failure, impotence, heart failure, and pulmonary
hypertension.
Sickle Cell Crisis
● There are three types of sickle cell crisis in the adult
population. The most common is the very painful sickle
crisis, which results from tissue hypoxia and necrosis due to
inadequate blood flow to a specific region of tissue or organ.
● Aplastic crisis results from in-fection with the human
parvovirus. The hemoglobin level falls rapidly and the
marrow cannot compensate, as evidenced by an absence of
reticulocytes.
● Sequestration crisis results when other organs pool the
sickled cells. Although the spleen is the most common organ
responsible for sequestration in children, by 10 years of age
most children with sickle cell anemia have had a splenic
infarction and the spleen is then no longer functional
(autosplenectomy). In adults, the common organs involved in
sequestration are the liver and, more seriously, the lungs.
Assessment and Diagnostic Findings
● The patient with sickle cell trait usually has a normal
hemoglobin level, a normal hematocrit, and a normal blood
smear. In contrast, the patient with sickle cell anemia has a
low hematocrit and sick-led cells on the smear. The diagnosis
is confirmed by hemoglobin electrophoresis.
Prognosis
● Patients with sickle cell anemia are usually diagnosed in
child-hood, because they become anemic in infancy and
begin to have sickle cell crises at 1 or 2 years of age.
● Some children die in the first years of life, typically from
infection, but the use of antibiotics and parent teaching have
greatly improved the outcomes for these children.
● However, with current management strategies, the average
life expectancy is still suboptimal, at 42 years.
● Young adults are often forced to live with multiple, often
severe, complications from their disease.
● In some patients, the symptoms and complications diminish
by 30 years of age; these patients live into the sixth decade or
longer. At this time, there is no way to predict which patients
will fall into this subgroup.
Medical Management
● Treatment for sickle cell anemia is the focus of continued
research.
● Many trials of medications that have antisick-ling properties
are being conducted, as is research using antiadhe-sion
treatment for vasoocclusive crises.
● However, aside from the equally important aggressive
management of symptoms and com-plications, currently there
are only three primary treatment modalities for sickle cell
diseases: BMT, hydroxyurea, and long-term RBC transfusion.
● BMT offers the potential for cure for this disease. However,
this treatment modality is available to only a small subset of
the patient population, because of either the lack of a
compatible donor or the severe organ (eg, renal, liver, lung)
damage already present in the patient.
Pharmacologic Therapy
● Hydroxyurea (Hydrea), a chemotherapy agent, has been
shown to be effective in increasing hemoglobin F levels in
patients with sickle cell anemia, thereby decreasing the
permanent formation of sickled cells.
● Patients who receive hydroxyurea appear to have fewer
painful episodes of sickle cell crisis, a lower incidence of
acute chest syndrome, and less need for transfusions.
● However, whether hydroxyurea can prevent or reverse actual
organ damage remains unknown.
● Side effects of hydroxyurea include chronic suppression of
WBC formation, teratogenesis, and potential for later
development of a malignancy. Patient response to the
medication varies significantly.
● The incidence and severity of side effects are also highly
variable within a dose range.
● Some patients have toxicity when receiving a very small dose
(5 mg/kg per day), whereas others have little toxicity with a
much higher dose (35 mg/kg per day).
● More research is needed to identify specific patient subgroups
that are more likely to respond to this medication.
Transfusion Therapy
● Chronic transfusions with RBCs have been shown to be
highly ef-fective in several situations: in an acute
exacerbation of anemia (eg, aplastic crisis), in the prevention
of severe complications from anesthesia and surgery, and in
improving the response to infection (when it results in
exacerbated anemia).
● Chronic transfusions have also been shown to be effective in
diminishing episodes of sickle cell crisis in pregnant women;
however, these transfusions have not been shown to improve
fetal survival.
● Transfusion therapy may be effective in preventing
complications from sickle cell disease.
● In a recent study (Adams, 2000), chronic transfusion with
RBCs resulted in a 90% reduction of stroke in children at risk
for this complication, as demonstrated by elevated blood
viscosity on transcranial Doppler ultrasonography.
● Transfusions may also be useful in the management of severe
cases of acute chest syndrome.
● The risk of complications from transfusion is important to
consider. These risks include iron overload, which
necessitates chronic chelation therapy (see MDS Nursing
Management); poor venous access, which necessitates a
vascular access device (and its attendant risk for infection or
thrombosis); infections (hepatitis, human immunodeficiency
virus [HIV]); and alloimmunization from repeated
transfusions.
● Another complication from transfusion is the increased
viscosity of blood before the concentration of hemoglobin S
is reduced. Exchange transfusion (in which the patient’s own
blood is removed and replaced via transfusion) may be
performed to diminish the risk of increasing the viscosity
excessively; the objective is to reduce the hematocrit to less
than 30%, with transfusions supplying more than 80% of the
patient’s blood volume.
● Finally, it is important to consider the significant financial
cost of an aggressive transfusion and chelation program.
● Patients with sickle cell anemia require daily folic acid
replacements to maintain the supply required for increased
erythropoiesis from hemolysis. Infections must be treated
promptly with appropriate antibiotics; infection remains a
major cause of death in these patients.
● Acute chest syndrome is managed by prompt initiation of
an-tibiotic therapy. Incentive spirometry has been shown to
decrease the incidence of pulmonary complications
significantly. In severe cases, bronchoscopy may be required
to identify the source of pulmonary disease. Fluid restriction
may be more beneficial than aggressive hydration.
Corticosteroids may also be useful. Trans-fusions reverse the
hypoxia and decrease the level of secretory phospholipase
A2.
● Pulmonary function should be monitored reg-ularly to detect
pulmonary hypertension early, when therapy (hydroxyurea,
transfusions, or transplantation) may have a positive impact.
● Because repeated blood transfusions are necessary, patients
may develop multiple autoantibodies, making cross-matching
difficult.
● In this patient population, a hemolytic transfusion reaction
may mimic the signs and symptoms of a sickle cell crisis.
● The classic distinguishing factor is that, with a hemolytic
trans-fusion reaction, the patient becomes more anemic after
being transfused. These patients need very close observation.
Further transfusion is avoided if possible until the hemolytic
process abates.
● If possible, the patient is supported with corticosteroids
(Prednisone), intravenous immunoglobulin (IVIG;
Gammagard, Sando-globulin, Venoglobulin), and
erythropoietin (Epogen, Procrit).
Supportive Therapy
● Supportive care is equally important. A significant issue is
pain management.
● The incidence of painful sickle cell crises is highly variable;
many patients have pain on a daily basis. The severity of the
pain may not be enough to cause the patient to seek
assistance from health care providers but severe enough to
interfere with the ability to work and function within the
family. Acute pain episodes tend to be self-limited, lasting
hours to days. If the patient cannot manage the pain at home,
intervention is frequently sought in the acute care setting,
usually at an urgent care facility or emergency department.
● Adequate hydration is important during a painful sickling
episode. Oral hydration is acceptable if the patient can
maintain adequate amounts of fluids; intravenous hydration
with dextrose 5% in water (D5W) or dextrose 5% in 0.25
normal saline solution (3 L/m2/24 hours) is usually required
for sickle crisis. Supplemental oxygen may also be needed.
● The use of medication to relieve pain is important. Aspirin is
very useful in diminishing mild to moderate pain; it also
diminishes inflammation and potential thrombosis (due to its
ability to diminish platelet adhesion).
● Nonsteroidal anti-inflammatory drugs (NSAIDs) are useful
for moderate pain or in combination with opioid analgesics.
Although no tolerance develops with NSAIDs, a “ceiling
effect” does develop whereby an increase in dosage does not
increase analgesia. NSAID use must be carefully monitored,
because these medications can precipitate renal dysfunction.
● When opioid analgesics are used, morphine is the medication
of choice for acute pain. Patient-controlled analgesia is
frequently used.
 ● Chronic pain increases in incidence as the patient ages. Here,
the pain is caused by complications from the sickling, such as
avascular necrosis of the hip.
● With chronic pain management, the principal goal is to
maximize functioning; pain may not be completely
eliminated without sacrificing function.
● This concept may be difficult for patients to accept; they may
need repeated explanations and support from nonjudgmental
health care providers.
● Nonpharmacologic approaches to pain management are
crucial in this setting. Examples include physical and
occupational therapy, physiotherapy (including the use of
heat, massage, and exercise), cognitive and behavioral
intervention (including distraction, relaxation, and
motivational therapy), and support groups.
● Working with patients who have multiple episodes of severe
pain can be challenging. It is important for health care
providers to realize that patients with sickle cell disease must
face a lifelong experience with severe and unpredictable pain.
● Such pain is disruptive to the person’s level of functioning,
including social functioning, and may result in a feeling of
helplessness.
● Patients with inadequate social support systems may have
more difficulty coping with chronic pain.
Nursing Process: The Patient With Sickle Cell Crisis
Assessment
● Pain levels should always be monitored; a pain-rating scale,
such as a 0-to-10 scale, best accomplishes this. The quality of
the pain (eg, sharp, dull, burning), the frequency of the pain
(constant versus intermittent), and factors that aggravate or
alleviate the pain are included in this assessment.
● If a sickle cell crisis is suspected, the nurse needs to
determine whether the pain currently experienced is the same
as or different than the pain typically encoun-tered in crisis.
● The respiratory system must be assessed carefully, including
auscultation of breath sounds, measurement of oxygen
saturation levels, and signs of cardiac failure, such as the
presence and extent of dependent edema, an increased point
of maximal impulse, and cardiomegaly (as seen on chest
x-ray).
● The patient should be assessed for signs of dehydration by a
history of fluid intake and care-ful examination of mucous
membranes, skin turgor, urine output, and serum creatinine
and blood urea nitrogen values.
● Because patients with sickle cell anemia are so susceptible to
infections, they are assessed for the presence of any
infectious process. Particular attention is given to
examination of the chest, long bones, and femoral head,
because pneumonia and osteomyelitis are especially
common. Leg ulcers, which may be infected and are slow to
heal, are common.
● The extent of anemia (as measured by the hemoglobin level
and the hematocrit) and the ability of the marrow to replenish
RBCs (as measured by the reticulocyte count) should be
monitored and compared with the patient’s baseline values.
The patient’s current and past history of medical management
should also be assessed, particularly chronic transfusion
therapy, hydroxyurea use, and prior treatment for infection.
Diagnosis
● Nursing Diagnoses
ー Based on the assessment data, major nursing diagnoses for
the patient with sickle cell crisis may include:
○ Acute pain related to tissue hypoxia due to
agglutination of sickled cells within blood vessels
○ Risk for infection
○ Risk for powerlessness related to illness-induced
helplessness
○ Deficient knowledge regarding sickle crisis prevention
● Collaborative Problems / Potential Complications
ー Based on the assessment data, potential complications may
include:
○ Hypoxia, ischemia, infection, and poor wound healing
lead-ing to skin breakdown and ulcers
○ Dehydration
○ Cerebrovascular accident (CVA, brain attack, stroke)
○ Anemia
○ Renal dysfunction
○ Heart failure, pulmonary hypertension, and acute chest
syndrome
○ Impotence
○ Poor compliance
○ Substance abuse related to poorly managed chronic
pain
Planning and Goals
● The major goals for the patient are relief of pain, decreased
inci-dence of crisis, enhanced sense of self-esteem and
power, and ab-sence of complications.
Nursing Interventions
● Managing Pain
ー Acute pain during a sickle cell crisis can be severe and
unpre-dictable. The patient’s subjective description and
rating of pain on a pain scale must guide the use of
analgesics, which are valu-able in controlling the acute
pain of a sickle crisis. Any joint that is acutely swollen
should be supported and elevated until the swelling
diminishes. Relaxation techniques, breathing exercises,
and distraction are helpful for some patients. After the
acute painful episode has diminished, aggressive measures
should be implemented to preserve function. Physical
therapy, whirlpool baths, and transcutaneous nerve
stimulation are examples of such modalities.
● Preventing and Managing Infection
ー Nursing care focuses on monitoring the patient for signs
and symptoms of infection. Prescribed antibiotics should
be initiated promptly, and the patient should be assessed
for signs of dehydration.
ー If the patient is to take prescribed oral antibiotics at home,
he or she must understand the need to complete the entire
course of antibiotic therapy and must be able to identify a
feasible administration schedule.
● Promoting Coping Skills
ー This illness, because of its acute exacerbations that often
result in chronic health problems, frequently leaves the
 patient feeling powerless and with decreased self-esteem.
These feelings can be exacerbated by inadequate pain
management.
ー The patient’s ability to use normal coping resources of
physical strength, psychological stamina, and positive
self-esteem is dramatically diminished.
ー Enhancing pain management can be extremely useful in
establishing a therapeutic relationship based on mutual
trust.
ー Nursing care that focuses on the patient’s strengths rather
than deficits can enhance effective coping skills.
ー Providing the patient with opportunities to make decisions
about daily care may increase the patient’s feelings of
control.
● Minimizing Deficient Knowledge
ー Patients with sickle cell anemia benefit from
understanding what situations can precipitate a sickle cell
crisis and the steps they can take to prevent or diminish
such crises.
ー Keeping warm and main-taining adequate hydration can
be very effective in diminishing the occurrence and
severity of attacks. Avoiding stressful situations is more
challenging.
ー Group education may be more effective if it is carried out
by members of the community who are from the same
ethnic group as those with the disease.
● Monitoring and Managing Potential COmplications
ー Leg ulcers
ー Priapism Leading to Impotence
○ Male patients may develop sudden, painful episodes of
priapism (persistent penile erection).
○ The patient is taught to empty his bladder at the onset
of the attack, exercise, and take a warm bath.
○ If an episode persists longer than 3 hours, medical
attention is recommended. Repeated episodes may
lead to extensive vascular thrombosis, resulting in
impotence.
ー Chronic Pain and Substance Abuse
○ Many patients have considerable difficulty coping
with chronic pain and repeated episodes of sickle
crisis.
○ Those who feel they have little control over their
health and the physical complications that result from
this illness may find it difficult to understand the
importance of complying with a prescribed treatment
plan.
○ Being nonjudgmental and actively seeking
involvement from the patient in establishing a
treatment plan are useful strategies.
ー Some patients with sickle cell anemia develop problems
with substance abuse. For many, this abuse results from
inadequate management of acute pain during episodes of
crisis.
ー Some clinicians suggest that abuse may result from
prescribing inadequate amounts of opioid analgesics for an
inadequate time.
ー The patient’s pain may never be adequately relieved,
promoting mistrust of the health care system and (from the
patient’s perspective) the need to seek care from a variety
of sources when the pain is not severe.
ー This cycle is best managed by prevention.
ー Receiving care from a single provider over time is much
more beneficial than receiving care from rotating
physicians and staff in an emergency department.
ー When crises do arise, the staff in the emergency
department should be in contact with the patient’s primary
health care provider so that optimal management can be
achieved.
ー Once the pattern of substance abuse is established, it is
very difficult to manage, but continuity of care and
establishing written contracts with the patient can be
useful management strategies.
Diagnosis
● Control of pain
ー Acute pain is controlled with analgesics
ー Uses relaxation techniques, breathing exercises,
distrac-tion to help relieve pain
● Is free of infection
ー Has normal temperature
ー Shows WBC count within normal range (5000 to
10,000/mm3)
ー Identifies importance of continuing antibiotics at home (if
applicable)
● Expresses improved sense of control
ー Participates in goal setting and in planning and
imple-menting daily activities
ー Participates in decisions about care
● Increases knowledge about disease process
ー Identifies situations and factors that can precipitate sickle
cell crisis
ー Describes lifestyle changes needed to prevent crisis
ー Describes the importance of warmth, adequate hydra-tion,
and prevention of infection in preventing crisis
● Absence of complications
Polycythemia Vera
● Polycythemia refers to an increased volume of RBCs. It is a
term used when the hematocrit is elevated (to more than 55%
in males, more than 50% in females).
● Dehydration (decreased volume of plasma) can cause an
elevated hematocrit, but not typically to the level to be
considered polycythemia. Polycythemia is classified as either
primary or secondary.
● Polycythemia vera, or primary polycythemia, is a
proliferative disorder in which the myeloid stem cells seem to
have escaped nor-mal control mechanisms.
● The bone marrow is hypercellular, and the RBC, WBC, and
platelet counts in the peripheral blood are elevated.
● However, the RBC elevation is predominant; the hematocrit
can exceed 60%. This phase can last for an extended period
(10 years or longer).
● The spleen resumes its embryonic function of hematopoiesis
and enlarges.
● Over time, the bone marrow may become fibrotic, with a
resultant inability to produce as many cells (“burnt out” or
spent phase).
● The disease evolves into myeloid metaplasia with
myelofibrosis or AML in a significant proportion of patients;
 this form of AML is usually refractory to standard treatments.
The median survival time exceeds 15 years.
Clinical Manifestations
● Patients typically have a ruddy complexion and splenomegaly
(enlarged spleen).
● The symptoms result from the increased blood volume
(headache, dizziness, tinnitus, fatigue, paresthesias, and
blurred vision) or from increased blood viscosity (angina,
claudication, dyspnea, and thrombophlebitis), particularly if
the patient has atherosclerotic blood vessels.
● Another common and bothersome problem is generalized
pruritus, which may be caused by histamine release due to the
increased number of basophils.
● Erythromelalgia, a burning sensation in the fingers and toes,
may be reported and is only partially relieved by cooling.
Assessment and Diagnostic Findings
● Diagnosis is made by finding an elevated RBC mass (a
nuclear medicine procedure), a normal oxygen saturation
level, and an enlarged spleen.
● Other factors useful in establishing the diagno-sis include
elevated WBC and platelet counts.
● The erythropoietin level is not as low as would be expected
with an elevated hematocrit; it is normal or only slightly low.
● Causes of secondary erythrocytosis should not be present (see
later discussion).
● Patients receiving hydroxyurea appear to have a lower
incidence of thrombotic complications; this may result from a
more controlled platelet count.
● The use of aspirin to prevent thrombotic complications is
controversial. Low-dose aspirin is frequently used in patients
with cardiovascular disease, but even this dose is often
avoided in patients with prior bleeding, especially bleeding
from the gastrointestinal tract. Aspirin is also useful in
diminishing pain associated with erythromelalgia.
Nursing Management
● The nurse’s role is primarily that of educator. Risk factors for
thrombotic complications should be assessed, and patients
should be instructed regarding the signs and symptoms of
thrombosis.
● Patients with a history of bleeding are usually advised to
avoid aspirin and aspirin-containing medications, because
these medications alter platelet function.
● Minimizing alcohol intake should also be emphasized to
further diminish any risk for bleeding.
● For pruritus, the nurse may recommend bathing in tepid or
cool water, along with applications of cocoa butter–based
lotions and bath products.

Complications

● Patients with polycythemia vera are at increased risk for

thrombosis resulting in a CVA (brain attack, stroke) or heart
attack (MI); thrombotic complications are the most frequent
cause of death.
● Bleeding is also a complication, possibly due to the fact that
the platelets (often very large) are somewhat dysfunctional.
The bleeding can be significant and can occur in the form of
nosebleeds, ulcers, and frank gastrointestinal bleeding.

Medical Management

● Phlebotomy is an important part of therapy and can be

performed repeatedly to keep the hematocrit within normal
range.
● This is achieved by removing enough blood (initially 500 mL
once or twice weekly) to deplete the patient’s iron stores,
thereby rendering the patient iron deficient and consequently
unable to continue to manufacture RBCs excessively.
● Patients need to be instructed to avoid iron supplements,
including those within multivitamin supplements.
● If the patient has an elevated uric acid concentration,
allopurinol (Zyloprim) is used to prevent gouty attacks.
● Antihistamines are not particularly effective in controlling
itching.
● If the patient develops ischemic symptoms, dipyridamole (eg,
Persantine) is sometimes used. Radioactive phosphorus (32P)
or chemotherapeutic agents (eg, hydroxyurea [Hydrea]) can
be used to suppress marrow function, but they may increase
the risk for leukemia.