• This disease is HOMOGYZOUS

Hematologic Disorders
• Sickle cell trait - one NORMAL hemoglobin
and one ABNORMAL hemoglobin

- rare for people who have sickle cell

Anemia trait to show signs and symptoms of
the disease because they usually
• Anemia is a condition that develops when your blood
have just enough normal
produces a lower-than-normal amount of healthy red
hemoglobin to prevent sickling of
blood cells or low hemoglobin
the red blood cells.
• Types:
• Any condition that increases the need for oxygen
• Sickle Cell Anemia - abnormal hemoglobin S
(Cold temperature, High Altitude, Dehydration)
• Iron-Deficiency Anemia - low IRON levels

The Blood

• Blood is a complex connective tissue composed of living

blood cells and formed elements
• Erythrocytes or red blood cells (RBC)
• Function primarily to ferry oxygen in blood to
all cells of the body.
• Anucleate (lack nucleus)
• Literally a sac of hemoglobin molecules
• Shaped like biconcave discs
• RBC become rigid as they age and begin to
fragment, or fall apart, in 100-120 days
• Hemoglobin - an iron-bearing protein, transport the
bulk of the oxygen that is carried in the blood. Signs and Symptoms
• Hemoglobin A (HbA) is the normal
• Hemoglobin is the most important component • Anemia
of red blood cells.
• It is composed of a protein called heme, which • RBC dying too soon: fussiness, tired,
binds oxygen. tachycardia, jaundice or pale (for dark skinned
patients assess mucous membranes), delayed
growth, shortness of breath
Sickle Cell Anemia
• Dactylitis
• It is where a person has abnormal hemoglobin S (normal
• Early sign seen in babies at 6 months: hand-
hemoglobin is hemoglobin A) on their red blood cell.
foot syndrome - blood flow being blocked to
• This type of hemoglobin is very sensitive to oxygen hands and feet…it will be swollen and fever
changes, which causes the red blood cell to change its present.
shape.
• Infection risk
•
• Spleen recycles old RBCs and helps us fight
infection by filtering out foreign invaders

• Sickled RBCs become trapped in spleen and

this leads it to swell and not work properly

• At risk for infection, especially pneumonia

(needs pneumococcal vaccine and flu,
meningococcal)….some patients need a
Risk Factors
splenectomy
• Genetics
• Gallstones
• The person inherited two hemoglobin S genes
• Sickled RBCs are constantly breaking down
from EACH parent. Therefore, it is an
and releasing bilirubin, which leads to
autosomal recessive disorder.
gallstones because there is too much bilirubin
• Again, this means in order for sickle cell for the gallbladder to manage
disease to occur in a person they must have
• Stroke
received TWO abnormal hemoglobin S genes
for each of their parents (one from each). • If sickled RBCs stick together and block blood
flow to the brain, blood supply is limited to the
 brain leading to a decrease in brain blood
supply….can cause mental and physical
disabilities….NEURO checks are very
important.
• Eye problems
• Vision issues due to the blockage of blood flow
to eye vessels from sickled cells (needs eyes
checked regularly)
• Risk for abnormal clotting due to an increase in blood
coagulation: at risk for DVT or PE
• Leg ulcers (older children and adults): lack of blood
flow…painful and very slow healing
• Acute chest syndrome
• Happens due to infection like pneumonia or
embolism or sickled cells blocking perfusion to
lung tissue
• Chest pain, cough, fever, low oxygen
saturation, new chest x-ray infiltrate….very
deadly…monitor respiratory status
Sickle Cell Crisis
• Periodic episodes of extreme pain, called pain crises, are
a major symptom of sickle cell anemia.
• Types of Sickle Cell Crisis:
• Vaso-occlusive: fever, pain, edema in the hand
and foot (seen in babies)
• Hyperhemolytic: destruction of RBCs at an
accelerated rate….release bilirubin. …the
patient can experience jaundice, gallstones,
anemia etc.
• Aplastic: the halt of red blood cell
production….bone marrow cannot keep up
with producing red blood cells so the patient
will cease in making RBCs….the patient will
have anemia.
• Non-functional spleen: the spleen can
become congested with sickled RBCs, which
leads it to swell and not work properly. The
patient is at risk for infection and many
patients will need their spleen removed.
Factors that can cause Sickle Cell Crisis:
“Sickle”
• Significant blood loss - surgery, trauma etc.
• Illness (at risk for this due to spleen function being
affected)
• Climbing or flying to high altitudes
• Keeping continued stress (physical or mental)
• Low fluid intake (dehydrated)
• Elevated temperature - fever, strenuous exercise
(extreme temperature changes like cold weather or cold
water….like swimming in cold can lead cells to sickle)
How is sickle cell trait diagnosed?
• This disorder tends to be diagnosed in the very young
pediatric populations (usually around 6 to 7 months)
• Because in utero and during the first 6 months of age,
the baby has fetal hemoglobin in their system that is
the main carrier of oxygen.
• At the age of 6 months, this type of hemoglobin
diminishes and the type of hemoglobin their genetic
code gave them takes over, which is unfortunately
hemoglobin SS.
• Newborn screening
• Dithionite (Sickledex): a blood test that
assesses for abnormal hemoglobin S…it
doesn’t differentiate between if the person has
sickle cell trait or sickle cell disease.
• Hemoglobin electrophoresis - assesses for
various types of hemoglobin
Nursing Interventions for Sickle Cell Anemia
• Assessment
• Hydration status
• Respiratory status
• Neurological status
• Pain
• Opioids usually given around the clock
• IV fluids and Oral fluids
• Dilutes blood and helps kidney function
• Oxygenation - helps with alleviating the sickling
• Penicillin – prevents infection
• Blood transfusion to replace RBCs and helps with
anemia
• Bed rest
• Educate about preventing infection (vaccination, hand
hygiene,
• Educate patient to avoid extreme weather and physical
activities, mental/physical stress, staying hydrated,
avoiding smoking, high altitudes)
• Keep extremities elevated and extended to prevent
swelling and helps blood flow
• Remove restrictive clothing because it decreases
perfusion
• Warm compresses (not cold leads to sickling) for painful
areas
• Folic acid administration: helps make RBCs…..NOT
iron….this doesn’t help with this type of anemia but can
actually build up in the body causing toxicity and harm
to organs
Medication for Sickle Cell Anemia

• Hydroxyurea: this helps actually to treat cancer, but it

will help with SCA in that it will help create fetal
hemoglobin (hgb F).

• Side effect: lowers WBC count

• Stem cell transplant: to cure….bone marrow will make

healthy RBCs…needs a matched donor

Nursing Diagnosis

• Impaired Gas Exchange

• Ineffective Tissue Perfusion
• Risk for Deficient Fluid Volume
• Acute Pain
• Risk for Impaired Skin Integrity
• Deficient Knowledge
 • Inflammatory Response:

• Iron regulates immune effector mechanisms –

Iron-Deficiency Anemia
• A type of anemia that is caused by low IRON levels
• Key Points about Iron-Deficiency Anemia:
• Most common type of anemia
• Low levels of iron lead the body to produce
fewer red blood cells
• Signs and symptoms are vague at first and as it
progresses the signs and symptoms become
more noticeable
• CBC (complete blood count) or iron level will
help the doctor detect anemia.
• Women who are childbearing age are at high
risk
cytokine activity, nitric oxide formation, and T-
cell proliferation
• Acquired IDA may be body’s response to a
pathogen – many pathogens require iron to
survive
Pathophysiology
Normal Physiology of Iron in the Body:
1. Iron enters the body through the diet in two forms,
heme and non-heme iron
• Both forms are found in foods of plant and
animal origin

Causes of Iron-Deficiency Anemia 2. If iron is not immediately needed by the body, it is

stored within the cells by ferritin, the major iron-storing
• Poor intake of iron through diet (pregnancy…increased protein
demand of iron for fetus, vegetarians)
3. Newly absorbed or released iron binds to plasma
• Absorption problem: intestinal surgery…gastric bypass, transferrin which distributes the iron around the body
celiac disease, small intestine removal (most iron is
absorbed by the small intestine) 4. The place of most need is erythroid marrow where iron
is utilized for hemoglobin synthesis in the making of new
• Losing iron from blood loss (menstruation, GI bleeding… RBCs
ulcers, hemorrhoids)
5. Iron’s function in the body
• Iron Store Depletion:
• RBC formation, immune function, brain
• Inadequate dietary intake function, muscle function, and energy
production
• Diets low in meat, fish, beans, or iron-fortified
foods – commonly seen with vegetarians or
individuals living in poverty

• Mechanism – low iron stores leads to demand

> supply

• Excessive blood loss

• Hemorrhage, menorrhagia (heavy menstrual

bleeding)

• Mechanism – depleting iron stores faster than

replacing combined while increasing body’s
demand for iron

• Metabolic/Functional:

• Insufficient iron delivery to bone marrow

• Iron stores adequate to meet body’s

need

• Mechanism – delivery to bone

marrow to be utilized in the
production of RBCs is impaired

• Impaired use of iron within bone marrow

• Iron stores adequate to meet body’s

need

• Mechanism – even when delivered,

there is impaired use of iron in the
bone marrow to produce RBCs
 • Iron store levels will be low

• Directly measured by bone marrow biopsy

• Indirectly by serum ferritin level, transferrin

saturation levels, or total iron-binding capacity

Nursing Interventions for Iron-Deficiency Anemia

• Monitoring, education, and administering medications

• Monitor patient for bleeding and hemoglobin levels and

other major signs and symptoms…assessing diet,
menstrual cycles etc.

• Educating how to take IRON supplements:

• Take iron on an empty stomach (increases

absorption…may take with small amounts of
food due to stomach upset)

• Take with Vitamin C…glass of orange juice

(helps increase absorption)

• Don’t take with any milk products, calcium, or

antacids (decreases absorption) and wait 2
hours in between

• Stools will turn black which is normal while

taking iron supplements (tarry stools or having
stools with blood..not normal)

Signs and Symptoms • For liquid preparations: mix in a drink, drink

with straw, and brush teeth afterwards (can
• Remember the Mnemonic: “Low Iron” stain teeth)
• Lethargic • Side effects: constipation (drink plenty of fluids and take
over the counter stool softener if needed)
• Overexerted easily (may be short of breath)
• May give IV iron or blood transfusion if severe per md
• Weird food cravings (ice, dirt, clay), White-faced (pale)
order
• Inflammation of tongue, Increased Heart rate (trying to
compensate for the low oxygen)
• Eat Food High in Iron: Remember the mnemonic “Eat
• Reduced Hemoglobin level
Lots of Iron”
• Observe changes in RBCs with a blood smear test…will
• Egg yolks
appear hypochromic (pale) and microcytic (small)
• Apricots
• Nail changes (Koilonychias….spoon-shaped nails), neuro
change (confused, moody) • Tofu
Diagnosis Criteria • Legumes, Leafy green vegetables
Evaluation of IDA is based on clinical symptoms and blood tests. • Oysters
• Low hgb levels in the blood • Tuna
• Caused by the direct reduction of hemoglobin • Sardines, Seeds
synthesis due to low levels of iron or impaired
RBC production • pOtatoes

• Lower than normal serum iron, ferritin, and transferrin • Fish (halibut, haddock, salmon)
saturation levels
• Iron-fortified cereal and breads
• Ferritin – iron-storing protein
• Raisins, Red meats (beef)
• Transferrin – distributes iron around the body
• pOultry (turkey, chicken)
• If these levels are low, indicated there is less
• Nuts
iron being stored and transferred in the body
 Factors that can Increase Atherosclerosis

• Non - Modifiable

• Age

• Sex

• Family history

• Modifiable

• High cholesterol level (LDL)

• High triglycerides/fats

• Hypertension

• Smoking

• Diabetes

• Obesity

Signs and Symptoms

• Chest pain during activity (stable angina)

• Shortness of breath - easily gets short of breath during

activity because the blood supply is impeded to the
heart muscle.

• Very tired, feeling run down especially with activity

How to Diagnose?

• Blood tests: Lipoprotein profile: total cholesterol, LDL,

HDL, triglycerides

• EKG: assesses if there are any changes in the ST

segments or t-waves (shows if there is a heart attack in
Coronary Artery Disease
• Coronary artery disease (CAD) is a condition that affects
your coronary arteries, which supply blood to your
heart. With CAD, plaque buildup narrows or blocks one
or more of your coronary arteries

• Fatty plaques are caused by a condition called

ATHEROSCLEROSIS which creates fatty plaques in the
artery walls

• Happens overtime

• Limits blood supply to the heart muscle and can rupture
which can lead to thrombosis formation (hence causing
a myocardial infarction)
• Atherosclerosis can also lead to hypertension, chest
pain, and heart failure.
the progress, previous heart attack, or compromised
blood flow)
o A 24 or 48 hour Holter monitor may be
ordered to watch the heart rhythm during the
patient’s regular activities of daily living.

o ST segment depression: demonstrates

ischemia that is reversible

o ST segment elevation: infarction where there

is injury to the heart muscle

• Stress test: monitor the heart rate and rhythm during

exercise and see if there are any EKG changes
 o During activity, if blood flow is reduced ST
segment depression will occur and patient
may have chest pain and then as activity stops
the ST segment will return back to normal. A
patient will probably then be ordered a heart
cath to assess where the potential blockage is
located.
o Also, a nuclear stress test may be ordered.
This is where a tracer is injected and pictures
of the heart are taken to assess blood flow of
the heart muscle during activity
• Heart Cath: a special catheter is inserted into the
femoral or radial artery to assess for blockages in the
artery. Dye is injected into the coronary arteries to
assess if they are blocked (coronary angiography)…
moderate sedation is used and the patient breathes on
their own.
• Cardiac doctor makes the decision if the artery needs:
o PCI (also called angioplasty): Percutaneous
Coronary Intervention
o Balloon angioplasty: inflates a balloon in the
blocked artery to compress the plaque against
the artery wall and a stent is placed to allow
• Nitrates:
blood to flow back through the artery.
o Atherectomy: removal of plaque from the
artery
• Sometimes, arteries cannot be stented and the patient
will have to have surgery known as coronary artery
bypass graft.
• Note: Patients with stable angina will complete a stress
test to assess the need for a heart cath. The most non-
invasive testing is performed first to assess the need of
more invasive procedures
Nursing Interventions
• Goal: Prevent further progression of CAD
• Educating patient about treatment, preventive measure,
medications, and management
o Educate the patient about the significance and
complications of
o Modifying lifestyle:
• Cholesterol lowering medication:
o How to manage with diet (low fat, low calorie)
o Exercise program
o Smoking cessation and why it is important
o Weight loss
o Monitoring heart rate and blood pressure
o Signs and symptoms and when to seek help
o Education about procedures: EKG, stress test,
heart cath, lipid profile blood test
Medications for CAD
• Antiplatelet medications: prevent clots from forming or
growing which decrease the chances of ischemia
• Aspirin: watching for GI bleeding.
• Plavix: for patients who can’t tolerate Aspirin
or just had a stent placed
• Monitor for:
• Thrombotic Thrombocytopenic
Purpura (TTP): clotting disorder where
clots form in blood vessels in the body
which causes decreased blood flow to
vital organs…low platelet count, neuro
changes, bruising, anemia, renal
failure, fever
• Will need to discontinue medication
for 5-7 days before a planned surgical
procedure because of the increase
chance of hemorrhage while taking this
drug. Patients need to let their surgeon
know they are taking Plavix because
they will be switched to another blood
thinner prior to the surgery. Plavix
takes a while to clear in the body’s
system.
• Nitroglycerin: dilates vessels to allow more
blood to get the heart muscle
• Educate about how to take: sublingual
(underneath the tongue)
• Take right when having chest pain
• Place one tab or one spray under the tongue
• Patient may feel dizzy or hot flushing after
taking Nitro. As the nurse, you will need to
monitor their blood pressure because Nitro
causes hypotension
• If not relieved in 5 minutes take another dose
of Nitro a 2nd dose and
• If not relieved in 5 minutes take another one
for a 3rd dose. The patient is NOT to take
more than 3 total doses. If not relieved with
the 3rd dose of Nitro the patient needs to call
911.
• Statins “Lipitor, Crestor, Zocor” (goal: LDL less
than 100 mg/dL) helps lower LDL, total
cholesterol, lower triglycerides, and increase
HDL.
• Educate not tao replace diet and exercise
• Notify doctor if they develop muscle pain or
tenderness
• Monitor CPK (creatine kinase) levels…. which if
elevated it can cause muscle problems
 • Monitor liver function because statins act on
the liver to block it from producing too much
cholesterol.

• Beta blockers:

• End in “lol” Propranolol, Metoprolol

• Lowers heart rate and blood pressure which

reduces work load on the heart. This will help
decrease episodes of chest pain

• Side effects: mask hypoglycemia signs and

symptoms like sweating and tachycardia in
diabetics, bradycardia, breathing problems in
patients with COPD or asthma, don’t take with
grapefruit juice

• ACE inhibitors:

• End in “pril” Lisinopril

• Blocks the conversion of angiotensin I to

angiotensin II which caused
vasodilation….lowers blood pressure…this
decreases the workload on the heart.

• Side effect: nagging dry cough