MEDSURG (112) LEC

CARE OF PATIENTS WITH PROBLEMS OF

THE HEMATOLOGIC SYSTEM

Functions and Composition of Blood----------------------

• Blood helps maintain homeostasis in several ways:
1. Transport of gases, nutrients, waste products
2. Transport of processed molecules
3. Transport of regulatory molecules
4. Regulation of pH and osmosis
5. Maintenance of body temperature
6. Protects against foreign substances such as
microorganisms and toxins
7. Blood clotting prevents fluid and cell loss and is
part of tissue repair
8. Blood is a connective tissue consisting of
plasma and formed elements
9. Blood is the body’s only fluid tissue
10. It is composed of liquid plasma and formed
elements Formed Elements----------------------------------------------
11. Formed elements include: • Erythrocytes or red blood cells (RBCs)
 Erythrocytes, or red blood cells (RBCs) – About 95% of formed elements
 Leukocytes, or white blood cells (WBCs) – RBCs have no nuclei or organelles
 Platelets • Leukocytes or white blood cells (WBCs)
12. Hematocrit: the percentage of RBCs out of the – Most of the remaining 5% of formed elements
total blood volume (45%) – Only WBCs are complete cells
13. Blood is a sticky, opaque fluid with a metallic – Five types of WBCs
taste • Platelets or thrombocytes
14. Color varies from scarlet to dark red – Just cell fragments
15. The pH of blood is 7.35–7.45 • Most formed elements survive in the bloodstream
16. Temperature is 38C for only a few days
17. Blood accounts for approximately 8% of body
weight
18. Average volume: 5–6 L (1.5 gallons) for
males, and 4–5 L for females

Plasma------------------------------------------------------------
• Pale yellow fluid containing over 100 solutes
• Mostly water (91%)
• Contains proteins (7%)
– Albumin (58% of the plasma proteins)
 Raises osmotic pressure at the capillary
membrane preventing fluid from leaking out
into the tissue spaces
– Globulins (38% of the plasma proteins)
 Gamma globulins: antibodies and
complement
 Alpha and beta globulins: bind to molecules
such as hormones
 Clotting Factors
– Fibrinogen (4% of the plasma proteins)
 Converted to fibrin during clot formation
• Other substances (2%)
– Ions (electrolytes): sodium, potassium, calcium,
chloride, bicarbonate
– Nutrients: glucose, carbohydrates, amino acids
– Waste products: lactic acid, urea, creatinine
– Respiratory gases: oxygen and carbon dioxide

Production of Formed Elements----------------------------
• Most blood cells do not divide but are renewed
by stem cells (hemocytoblasts) in bone marrow
• Hematopoiesis: blood cell production
– Occurs in different locations before and
after birth
 Fetus
 Liver, thymus, spleen, lymph nodes,
and red bone marrow
 After birth
 In the red bone marrow of the
» Axial skeleton and girdles
» Epiphyses of the humerus and
femur
 Some white blood cells are
produced in lymphatic tissues
• Hemocytoblasts give rise to all formed
elements
– Growth factors determine the type of
formed element derived from the stem cell
• Most blood cells do not divide but are renewed
by stem cells (hemocytoblasts) in bone marrow
• Hematopoiesis: blood cell production
– Occurs in different locations before and
after birth
 Fetus
 Liver, thymus, spleen, lymph nodes,
and red bone marrow
 After birth
 In the red bone marrow of the
» Axial skeleton and girdles
» Epiphyses of the humerus and
femur
 Some white blood cells are
produced in lymphatic tissues
• Hemocytoblasts give rise to all formed
elements
– Growth factors determine the type of
formed element derived from the stem ce
Accessory Organs of Hematopoiesis
 These organs help regulate maturation of blood
cells to maintain hematologic homeostasis
• Spleen
– Has 3 layers:
• White pulp - filled with WBCs
(lymphocytes and macrophages). As
blood passes through, unwanted cells
(bacteria, and old blood cells are
removed)
• Red pulp – composed of sinuses that
stores RBCs and Platelets
• Marginal pulp – ends of many arteries
and other blood vessels
 Destroys old and imperfect RBCs
 Breaks down Hb released from
destroyed cells
 Stores platelets
 Filters antigens
• Liver
– Main production site for prothrombin and
most clotting factors
– Proper liver function and bile production is
critical for the production of Vit. K and other
clotting factors
– It stores blood and blood cells
Red Blood Cells
• Biconcave discs, anucleate, essentially no
organelles
• RBCs are an example of how structure fits function
– Biconcave shape has a huge surface area
relative to volume
• Structural characteristics contribute to its
gas transport function
– Biconcave shape and a flexible membrane also
allows RBCs to bend or fold around their thin
center
• Gives erythrocytes their flexibility
• Allow them to change shape as necessary
• RBCs are dedicated to respiratory gas transport
– Filled with hemoglobin (Hb), a protein that
functions in gas transport
• Hemoglobin (Hb)
– Accounts for about a third of the cell’s volume
– Consists of
 The protein globin, made up of two alpha
and two beta chains, each bound to a heme
group
 Each heme group bears an atom of iron,
which can bind to one oxygen molecule
 Heme molecules transport oxygen (Iron is
required)
 Oxygen content determines blood color
» Oxygenated: bright red
» Deoxygenated: darker red
 Globin molecules transport carbon dioxide
• One RBC contains 250 million Hb groups thus it can
carry 1 billion molecules of O2
  In the intestines bilirubin is converted
• Erythropoiesis is the production of RBCs by bacteria into other pigments
– A hemocytoblast is transformed into a  Gives feces its brown color
proerythroblast  Gives urine its yellow color
– Proerythroblasts develop into early
erythroblasts Hemoglobin breakdown
– The developmental pathway consists of
three phases
1. Ribosome synthesis in early erythroblasts
2. Hb accumulation in intermediate erythroblasts and
late erythroblasts
3. Ejection of the nucleus from late erythroblasts and
formation of reticulocytes
– Reticulocytes are released from the red bone
marrow into the circulating blood, which contains
~1-3% reticulocytes
– Reticulocytes then become mature
erythrocytes
• Circulating erythrocytes: The number remains
constant and reflects a balance between RBC
production and destruction
– Too few RBCs leads to tissue hypoxia
– Too many RBCs causes undesirable blood
viscosity
• Erythropoiesis is hormonally controlled and
depends on adequate supplies of iron, amino
acids, and B vitamins (folate and B12)
– Erythropoietin (EPO) released by the
kidneys is triggered by
 Hypoxia due to decreased RBCs
 Decreased oxygen availability
 Increased tissue demand for oxygen
– Enhanced erythropoiesis increases the
 RBC count in circulating blood
 Oxygen carrying ability of the blood

• The life span of an erythrocyte is 100–120 days

• Old RBCs become rigid and fragile, and their
Hb begins to degenerate
• Dying RBCs are engulfed by macrophages
located in the spleen or liver
• Heme and globin are separated and the iron is
salvaged for reuse
– Globin chains are broken down to individual
amino acids and are metabolized or used
to build new proteins
– Iron released from heme is transported to
the red bone marrow and is used to
produce new hemoglobin
– Heme becomes bilirubin that is secreted in
bile

ANEMA
 It is not a disease but rather a term used to
describe a decreased O2-carrying capacity
of the blood caused by an underlying disease
or injury
 It is manifested by an abnormally low RBC
count or hemoglobin or both
 Causes
 Increased destruction of RBCs
 Deficient production of RBCs
 Loss of RBCs
 Can be classified based on the morphology
 Microcytic – small erythrocytes
 Macrocytic – large size cells
 Hypochromic – pale RBCs
 Normochromic- normal cell size and coloe
 Normocytic- fewer RBCs without normal
hemoglobin
 Megaloblastic-unusually large RBC
 Classified based on its etiology
 Hypoproliferative anemia – decreased
production
 Deficiency anemias
 Decreased number of erthrocyte
precursor
 Hemolytic anemia – increased destruction
 May also be classified according to its severity
 Mild - Hb 11 g/dl – asymptomatic, mild
tachycardia with increased activity
 Moderate – 7 g – 10 g/dl – signs and
symptoms become evident
 Severe – 4 g - 6 g/dl – decompensatory
manifestations
***Hb ≤ 3 g/dl – cardiovascular collapse
 Clinical manifestations
 Depends on the severity of tissue hypoxia
and the effectiveness of compensatory
mechanisms employed by the body
 Compensatory mechanisms
 Tachycardia
 Tachypnea
 The hemoglobin releases O2 to the
tissue more readily
 Activation of the RAAS → sodium and
water retention → increased blood
volume and BP
 Increased erythropoiesis
 When compensatory mechanisms decline →
 Pallor
 Fatigue
 CNS: dizziness/fainting, headache, light
headedness, slow thought process,
decreased attention span, restlessness,
apathy
 Heart : palpitations, cardiac murmur, chest
pain, CHF to susceptible individuals
 Lungs: dyspnea progressing from exertion
to rest
 Anorexia
 Poor tolerance to cold
PATHOLOGY, SYMPTOMS, AND SIGNS OF ANEMIA
 Management
 Laboratory investigations
 Treat the underlying cause
 Blood transfusions
 Nutritional supplements
Laboratory Investigations
• Complete Blood Count – a group of tests that
evaluates cells in the blood which includes the
RBCs, WBCs, and Platelets.
– Red blood cell Count – a count of the actual
number of RBCs in 1 mm3 of blood
– White blood cell count – measures all types
of WBCS in 1 mm3 of blood
– Hemoglobin – measures the total amount of
hemoglobin in the blood, which generally
reflects the number of RBCs in the blood
– Platelets – measures the number of platelets in
the blood
– Differential WBC count – determine the
percentage of each type of WBC present in the
blood.
• neutrophils
• Lymphocytes
• Eosinophils
• Basophils
• monocytes
– Reticulocyte count – measurement of the
absolute count or percentage of newly released
young RBCs in the blood sample.
– Hematocrit – measures the percentage of
RBCs to the total volume of blood, it is also
called packed cell volume
• RBC indices
– MCV (mean corpuscular volume) –
measurement of the average size of the RBCs
– MCH (mean corpuscular Hgb) - measurement
of the amount of hemoglobin by weight in a
single cell
 – MCHC ( mean corpuscular Hgb Concentration)
–measurement of the average amount of Hgb
by percentage in a single cell or the
concentration of hemoglobin inside your RBCs.
***Decreased MCH and MCHC means the cell has
a Hgb deficiency and is hypochromic (pale) ---Iron
deficiency anemia
• Peripheral Blood Smear – a drop of blood is
spread on a glass slide -> stained -> examined
under the microscope to evaluate the size and
shape of the blood cells
– Anisocytosis –cells have altered and different
sizes
– Poikilocytosis – alteration in the shape
• Hemoglobin Electrophoresis – used to detect
abnormal types of hemoglobin
– Normal Hemoglobin: A, A2, F. Type A is 96-
98%
– Abnormal types of Hgb: Hgb S, Hgb C
(hemoglobinopathy)
• Coomb’s Test –
– Direct test – determines presence of
antibodies against the RBCs
– Indirect Test – determines whether the client
has antibodies against the blood that he/she is
about to receive
• Serum Ferritin
– measures amount of iron present as free iron in
the plasma
– the amount of serum ferritin is related to the
amount of intracellular iron and
represents 1% of the total body iron stores
– Iron stores should not be less than 10gm/dl
• Transferrin – a protein product that transport iron
from the intestines to cell storage sites
• S. Iron/B12/folate
• Total Iron Binding Capacity (TIBC)
– Measures the amount of iron that could still
bind to transferrin
Radiologic Examination
• Radioisotopic Imaging
– The client is given radioisotope IV 3 hrs before
the procedure -> the client is taken to the
nuclear medicine department for the scan
where he/she must lie still for 1 hour.
– Non-invasive imaging technique used to
visualize the functional activity bone marrow
– Bone Marrow Aspiration/Biopsy
• It is an invasive procedure to collect and
examine bone marrow
• Bone marrow examination offers a
detailed information about the condition of
the bone marrow and blood cells.
• Cells and fluids from the bone marrow are
suctioned. Solid tissues and cells are
obtained by coring out an area of the bone
marrow with a large-bore needle
• Purpose:
– To evaluate client’s hematologic status
when other tests show persistent
abnormal findings
– To provide information about bone
marrow function including the
production of blood cells
Nursing Responsibilities in Bone Marrow
Aspiration
• Pre-procedure
– Allay patient’s anxiety, emotional support
– Explain the procedure, what to expect.
– Obtain an informed consent.
– May be done at bedside or in the examination
room.
– Place patient in prone or sidelying position.
Prepare the site, clean with antiseptic solution.
The site (most commonly) is the iliac crest
• Procedure
– Administer analgesic and mild tranquilizer as
ordered.
– Sterile precautions are observed
– Needle is inserted with a twisting motion then
marrow is aspirated
– Apply pressure dressing
• Post Procedure
– Closely observe site for bleeding
– Administer analgesic for comfort
– Ice pack limit bleeding or bruising.
– Other diagnostic studies may be performed to
determine underlying chronic illnesses such as
malignancy, or other source of any blood loss
such as polyps or ulcers within the GI tract.
Assessment
• Physical assessment
– Weakness, fatigue, general malaise
 – Pallor of skin, mucous membranes (conjunctiva
and oral mucosa)
– Jaundice may be present – in hemolytic anemia
– Smooth and red tongue - in Iron deficiency
anemia
– Beefy red and sore tongue – in Megaloblastic
anemia
– Ulcerated corners of the mouth (angular
cheilosis) – in both types
– Craves for ice, starch, dirt (pica) – iron
deficiency anemia
– Nails are brittle ridged and concave -
• Health history
– Medication history – certain medications can
depress bone marrow activity, induce
hemolysis
– History of alcohol intake (amount, duration) –
interferes with folate metabolism
– Family history – certain anemias are inherited
– Athletic endeavors – extreme exercise can
decrease erythropoiesis and erythrocyte
survival
– Nutritional status –
 Iron – liver and other organ meat
 Folic acid – more on green-leafy
vegetables
 Vit. B12 – meat, fish, milk and milk
products, egg
– Cardiac status – increased work load
 Tachycardia, palpitations, dizziness,
orthopnea, exertional dyspnea
 Eventually heart failure – cardiomegaly,
edema, hepatomegaly, etc.
– Menstrual flow and other vaginal bleeding
– Neurologic – particularly pernicious anemia
 Presence of peripheral numbness,
paresthesia, poor coordination (ataxia),
confusion
 Delirium particularly in older adults
– GI assessment
 s/s of GI bleeding: “coffee ground” vomitus,
melena (black stools = + occult blood)
 Nausea, diarrhea, anorexia
 Sore tongue (glossitis)
 Monitor relevant laboratory results and note
any changes over time
Nursing Diagnoses
• Fatigue R/t decreased hemoglobin and diminished
carrying capacity of the blood
• Imbalanced nutrition, less than body requirements,
r/t inadequate intake of essential nutrients
• Ineffective tissue perfusion r/t inadequate
hemoglobin and hematocrit.
• Noncompliance with prescribed therapy
• Collaborative Problems/Potential Complications
– Possible complications include
 Heart failure
 Angina
 Injury related to fall
• Activity intolerance
– Promote optimal activity and protect from injury
– let patient participate in self care
– Activities should be spaced at intervals and
allow frequent rest periods
– Provide adequate rest period – re-plan activities
that cause fatigue
– Avoid sudden movement -
• Decreased cardiac output
– Monitor V/S and signs of heart failure: edema,
neck vein distention, decreased urine output,
palpitations, chest pain
Nursing Interventions
• Managing fatigue (most common)
– Assisting patient to prioritize activities
– Establish a balance between activity and rest
– Encourage exercise to prevent deconditioning
that results from inactivity.
– Assess for other conditions that exacerbate
fatigue (pain, depression, sleep disturbance)
• Maintaining adequate nutrition
– Encourage a healthy diet
– Limit alcohol intake
– Dietary education sessions with family
members (consider cultural aspects related to
food preferences and food preparation
– Dietary supplements may be prescribed
 Be careful of indiscriminate use of Iron
Nursing Management
• Increase O2 demand
– Adequate rest
– Quiet activities
– O2 supplement may be necessary
– Fowler’s position
• Support RBC production
– Nutritious diet
– Vitamin and iron supplement
– Small frequent meals
– Prevention of infection
I. BLOOD LOSS ANEMIA
 With blood loss, Hb and Fe will also be lost
A. Acute - associated with trauma, surgery,
platelet dysfunction and coagulation disorders
 Anemia is the direct result of the decrease
of circulating RBCs
 An adult can lose 500 ml of blood without
serious or lasting effects.
Loss of ≥1000 ml -> circulatory collapse
 S/S associated with hypovolemia and
hypoxemia : weakness, stupor, cool moist
skin, tachycardia, hypotension
 RBC indices are normal until several hours
after the blood loss has occurred
 If there is sufficient Fe stores, normal RBC
count returns to normal after 3 to four
weeks
B. Chronic blood loss
 Does not affect the blood volume but it will
lead to Fe deficiency and low hemoglobin
when Fe stores are already depleted
 No symptoms until Hb < 8g /dl
 RBC indices: all are below normal
 Most common cause of Iron deficiency
anemia
  Vinson-Plummer’s Syndrome: angular
Management of blood loss Anemia: stomatitis or cheilosis, dysphagia, atrophic
• Immediate identification of the source of blood loss glossitis
and appropriate treatment  Pica
– Fecal occult blood  Blue sclerae
• Transfusion (PRBC)  Intolerance to cool temperature
• Iron supplement
• Laboratory tests
II. DEFICIENT PRODUCTION OF RBCs – ↓Hb, hematocrit, RBCs
• Iron deficiency anemia – ↓MCV and MCH
• Megaloblastic anemia – peripheral smear – microcytic and hypochromic
– Vitamin B12 deficiency/Pernicious anemia RBCs
– Folic acid deficiency – ↑ serum iron binding capacity
• Aplastic anemia – ↓ serum iron and ferritin levels

a) Iron Deficiency Anemia----------------------------- • Collaborative management

• A type of anemia marked by inadequate supply of
iron for optimal formation of hemoglobin → smaller
cells with less color (Microcytic, hypochromic)
• Most common type of anemia
– Determine underlying cause
– Iron supplement
 Oral – ex. ferrous sulfate
 Vit. C enhances iron absorption
 Liquid prepations can stain teeth
 Milk, tea, antacids inhibits absorption
 It changes color of stool to dark green
 Absorbed better in an empty stomach
 IM – ex. Inferron
 Give deep IM, Z-track method; do not
massage

• Management
 O2 inhalation
 BT as needed
 Nutritional supplements
 Transfusion of packed RBCs

• Etiology
– Inadequate dietary intake
 Low socioecomic status
 Vegetarian diet
 Food sources: liver and muscle meat, dark
green, leafy vegetables, dried fruits, whole
grain and enriched cereals, legumes,
potato, molasses
– Blood loss (major cause)
 Bleeding (2ml of whole blood contains 1 mg
of iron)
 Major sources of chronic blood loss: GI and
GU systems, menstrual cycle, parasitism
– Poor absorption of Iron
 Gastric resection or intestinal resection
 Chronic diarrhea
– Increased iron requirement: pregnancy,
lactation, rapid growth, infants
– Dialysis – because of the blood loss in the
dialysis equipment
– Frequent blood sampling
• Common manifestations
– Signs of anemia
– Specific signs
 Dryness of hair/hair falling
 Koilonychia- coarsely ridged, Spoon-
shaped, brittle nails
b) Megaloblastic Anemia-------------------------------
 a condition in which the bone marrow produces
unusually large, structurally abnormal,
immature red blood cells (megaloblasts).
1. Folic Acid Deficiency
2. Vit B12 deficiency / Pernicious anemia
• Folic acid and Vit. B12 are essential in the synthesis
of DNA
• Impaired DNA synthesis → defective maturation of
RBCs → large abnormal RBCs called megaloblast
• Vitamin B12 is also important for the integrity of the
myelin sheath of nerve cells
• Other cells from the myeloid stem cell are also
diminished because of altered DNA synthesis
• Because megaloblast are defective they are
destroyed by the spleen in increased rate
• Causes:
– Decreased dietary intake of Vit. B12 (rare)
 Sources: primarily in foods of animal origin
including cow’s milk and breast milk
– Lack of intrinsic factor → Pernicious anemia
 Intrinsic factor – secreted by the parietal
cells of the stomach; essential for the
absorption of Vit. B12
 Genetic
 Intestinal resection (absorbed in the ileum)
• Clinical Manifestations
– Symptoms of anemia
– Chronic diarrhea
 – Triad of symptoms  Insecticides (DDT)
 Weakness – due to anemia  Benzene products
 Beefy and red tongue  arsenic, glycol ethers
 Neurologic symptoms • Leukopenia – decreased number of WBC → high
 Paresthesia risk for infection
 Neuritis • Thrombocytopenia – decreased number of platelets
 Ataxia (lack of coordination; impaired → bleeding tendency
fine finger movement • Anemia – decreased number of RBC
 Mild jaundice • They die from hemorrhage and infection
 Low serum levels of Vit. B12
 Low Hb, RBCs, Platelets, and WBCs • Collaborative Management
 Schilling’s test – a Vit. B12 absorption test – Removal of the causative agent
 Radioactive Vit. B12 is ingested – Can be cured by a bone marrow transplant
 A non-radioactive Vit. B12 is given IM (BMT) or peripheral blood stem transplant
 24-hr urine collection (PBSCT)
 “+” if no radioactive Vit. B12 is found in  Immunosuppressive therapy – to prevent
the urine lymphocytes from destroying the stem cells
 ↑ unconjugated bilirubin ex. Cyclosporine, androgens,
 Bone marrow – increased number of antithymocytes globulin (ATG)
megaloblasts  Corticosteroids as immunosuppressive
 Tubeless gastric analysis agent – not very useful
 Diagnex/azuressin given PO  Supportive therapy
 Blue color urine → test is negative  PRBC transfusion
• Collaborative management  Platelet transfusion
– Vit. B 12 injections will reverse S/S - Bone Marrow Transplant
– For pernicious anemia:  Its goal is to replace diseased bone marrow
– Monthly Vit. B12 injection for life with healthy bone marrow or to rescue
healthy bone marrow
• Common causes:  The blood stem cells travels to the bone
– Poor nutrition marrow where they produce new blood
– Malabsorption syndromes and bowel disorders cells and promote new growth of new
 Folic acid absorbed in the jejunum marrow
– Drugs that impede the absorption of folic acid  HLA (Human Antigen Leukocyte)
 Methotrexate compatibility
 Oral contraceptives  HLA makes up a person’s tissue type
 Antiseizure agents  3 Types of BM transplants:
– Alcohol abuse and anorexia  Syngeneic – from an identical twin –
– hemodialysis perfect HLA match
• With same clinical manifestations as that of Vit. B12  Allogenic – from a related or unrelated
deficiency but without the neurologic symptoms donor – may or may not be an HLA
match
c) Aplastic Anemia---------------------------------------  Autologous – BM stem cells are
• Any form of anemia caused by aplasia of the bone harvested from the patient’s peripheral
marrow. blood by plasmapheres
 Aplasia – defective development of an organ  peripheral blood -> plasma is
or complete absence due to failure of separated -> stem cells are
development separated by a special machine
• There is depression or cessation of activity of all  Peripheral Blood Stem Cell
blood-producing elements → pancytopenia Transplantation
• Etiology: - BM stem cells can be harvested from the
– Congenital : Fanconi’s anemia posterior iliac crest or from patient’s peripheral
– @ 50% are idiopathic blood
– Identified causes - Transplant process is complex
 Drugs  Conditioning/preparing the patient
 Antineoplastic drugs  series of lab tests and procedures
 Chloramphenicol  chemotherapy
 Sulfonamides  radiation
 phenylbutazone
 Infections • Nursing Management
 Hepatitis (B and C) – Careful assessment and management of
 Epstein-Barr virus infection the complications of pancytopenia
 Cytomegalovirus  Assess carefully for signs of infection
 Miliary TB and bleeding
 Chemicals – Prevention of infection
  Use of protective isolation • Causes:
 Meticulous hygiene  Intrinsic
 Oral care – Usually inherited
 Monitor invasive lines for signs of – Defects of the cell membrane or of the
infection hemoglobin (hemoglobinopathy – sickle
 Avoidance of catheterization cell anemia, G6PD)
 Instruction of handwashing  Abnormal cells don’t function well and are
 Control visitor destroyed by the MPS
– Prevention of bleeding  Extrinsic
 Monitor invasive lines, feces, urine – Drugs
 Minimize venipuncture and IM  Methyldopa (Aldomet) – associated
injections with production of antibodies against
 Use of soft sponges for oral care RBCs
– Care for anemic patient – Bacteria/toxins (sepsis)
– Monitor for side effects of therapy – Antibodies (incompatible blood transfusion,
 Ex. hypersensitive reaction (ATG); autoimmunity)
long-term effects of cyclosporine – Physical trauma ( heart valve prosthesis)
including renal and liver failure • Can occur extravascularly
– Patient and family education about  The spleen removes erythrocytes from the
 Disease circulation at an accelerated rate
 Assisting in developing positive coping • Intravascularly
strategies  The erythrocytes lyse and spill contents into the
plasma
Guidelines of Safe Practice • Common laboratory results
Prevent Prevent Prevent  Elevated unconjugated bilirubin
Infection Hemorrhage Fatigue  Elevated reticulocyte count
-Good -Observe for -Take Examples
handwashing signs such as frequent rest  Sickle cell disease
technique bloody urine between  Thalassemia
-Avoid crowds and stool, ADLs  G6PD deficiency
and persons petichiae, and -Avoid  Spherocytosis
with infection report excessive • A genetic disorder that results in chronic anemia,
-Avoid sharing immediately work load; pain, disability, organ damage and early death
utensils -Use of soft ask for • Abnormal Hb molecule
-Skin care toothbrush; assistance – Normal adult Hb molecule contains 98-99% Hb
-Avoid eating avoid use of -Increase A and a trace of HbF
raw foods dental floss time – In Sickle Cell Disease, 40% of the total Hb
-Report -Keep mouth necessary contains abnormality of beta chains = HbS
immediately clean for routine • HbS
early signs of -Avoid care – Sensitive to changes in the O2 content of the
infection enemas or -Report RBC
rectal signs of – RBCs with large amount of Hbs when exposed
insertions increased to ↓ O2 states -> abnormal beta chains contract
-Avoid picking fatigue and pile together within the cell -> RBC shape
or blowing become distorted (sickle shape), rigid and
nose forcibly clump together -> clump together -> blocks
-Avoid blood flow -> ischemia in affected tissue ->
trauma; avoid more sickling - > more obstruction of blood flow
contact sports -> severe pain. This episode is called Sickle
-Avoid aspirin Cell Crisis
-Use of – Repeated episodes of ischemia -> progressive
electric razor organ infarction (necrosis)
-Prevent  Can happen as often as weekly or as
constipation seldom as 1 year.
-Avoid gastric  Many clients are in good health much of the
irritating foods time and crises occurring only when
-Gentle exposed to causes
sexual
intercourse

III. HEMOLYTIC ANEMIA

• Premature destruction of RBCs: decreased life
span → fewer circulating erythrocytes → decreased
available O2

a) Sickle Cell Anemia
• Sickled cells go back to normal shape when the
low O2 condition is removed and proper
oxygenation occurs
– Cell membrane become damaged overtime
and remain permanently sickled
– Cell membrane are more fragile with
average life span of 12 – 13 days
• Conditions that cause sickling
– Hypoxia
– Infection
– Venous stasis
– Low environmental/body temperature
– Strenuous exercise
– anesthesia
• Cause of death – organ failure
– Spleen - kidneys
– Liver heart- bones
– Brain- retina
• Collaborative Management
– Diagnostics
 Metabisulfate test (sickle cell solubility
test) –
 peripheral blood smear – shows target
cells or sickle cell forms
 Hb Electrophoresis – the confirmative
test for SCD – a blood test to check the
different types of hemoglobin
– Medications
 Hydroxyurea – decrease the number of
pain episodes, the need for transfusions,
and episodes of acute chest syndrome
and hospitalization
 Erythropoietin
 Supplemental iron, Folic and Vit. B12
 Antibiotics early in the early course of
infection
 NSAIDs, Opiods for pain control
– Treatment
 Hydration and pain management is the
cornerstone of treatment
 O2 therapy
 Frequent blood transfusion (every 3 – 4
weeks)
 Exchange transfusion during
vasoocclusive crisis
 Genetic counseling
 Diet
– High in protein, calcium, vitamins,
and adequate fluids
– Iced liquids may precipitate crisis
 Activity
– Physical stress or overexertion may
precipitate crisis
– Avoid contact sports that may cause
joint injury.
– Non-stressful exercises should be
recommended to maintain muscle
tone and stimulate circulation
 Range-of motion exercises and
regular activities
 Walking
b) Glucose-6-Phosphate Dehydrogenase
Deficiency (G6PD)
– Deficiency of enzymes in the pathways that
metabolize glucose and generate adenosine
triphosphate (ATP) leads to premature RBC
destruction.
– G6PD is the most common enzyme deficiency
anemia
 Inherited as an X-linked recessive disorder
 Patient is usually asymptomatic until
exposure to the triggering agents ->
damage to the hemoglobin on the RBC
membrane -> destruction of the RBCs
(hemolysis) –> Hb is released into the
circulation
 Hemolytic episode lasts for 7 – 10 days
after exposure after exposure
 Only older RBCS with less G6PD are
destroyed
– Collaborative management
– Hydration – to prevent precipitation of bebris of
Hb in the kidney tubules which can lead to
tubular necrosis
– Osmotic diuretic – Mannitol
– Transfusion therapy when needed and kidney
function is normal
c) Spherocytosis
– Most common problem of alteration in
erythrocyte shape
– Hereditary, rare – 1 in 5000 persons the
– There is a defect in the proteins that form the
structure of the RBC -> cells are thick and
spherical -> membrane become increasingly
permeable to sodium → osmotic swelling →
susceptibility to destruction in the spleen
– Its capability to carry O2 is maintained
– Diagnostics
 Red cell survival time
  Peripheral blood smear
 ↑ reticulocyte count,↑ serum bilirubin
– Management
 Sub total splenectomy – to reduce
hemolysis but not to cure

d) Thalassemia
– Inherited disorder of hemoglobin synthesis
– Primarily affects persons of Mediterranean
origin, but it also occurs in southeast Asians,
Chinese, and Africans
– There is a decreased synthesis of one of the
globin chains of hemoglobin (β chain is most
affected) → decreased synthesis of
hemoglobin and an accumulation of the α chain
in the erythrocyte → hemolysis

Collaborative Management
• For chronic anemia - regular Transfusions
– Hgb goal post transfusion of 10g/dl
– needed approximately every 4 weeks
• For Iron overload – Chelation therapy
– Iron overload is a complication of frequent
transfusions
• 2 types – Desferoxamine (Desferal) – Iron chelating
1. Thalassemia Minor agent
 The heterozygous state – SQ over 10-12 hours, 5-6 days/wk
 Mild, usually asymptomatic – avoid if < 3 years old because of toxicity
 Usually presented with mild anemia – Side effects: ototoxicity with high frequency
 No therapy is required hearing loss, retinal changes, bone
2. Thalassemia Major dysplasia/truncal shortening
 The homozygous state – Oral Chelator? So far no safe alternative
 Also called Cooley’s anemia • Folate supplementation
 Characterized by severe anemia • Splenectomy (for hypersplenism) as indicated by:
 RBCs are hypochromic and microcytic – Dramatic increase in transfusion requirements
 Electrophoresis is the definite – massive size that interferes with breathing and
diagnostic test nutrition
 Growth failure usually begins between – severe pain
ages 10 and 12 – avoid before 5 years if at all possible
 Death usually occurs during the young – immunize with pneumovax and menigovax pre-
adult years (17 – 30) splenectomy
– post splenectomy will need penicillin
prophylaxis for life
• Stem cell transplantation - Bone Marrow Transplant
(cure)
• Educate family