Pediatric Hematological Disorders
Whaley and Wong
Chapters 35, 36
Components of the Blood
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Blood:
– Plasma
• water, albumin, electrolytes, clotting factors
– Cellular Components
• RBCs, WBCs, Platelets
• All formed in the red bone marrow (after birth)
– In utero- spleen, thymus, liver
• lymphatic system regulates maturation
Erythrocytes
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RBCs
– carry hemoglobin which is attached to
oxygen- provides O2 to the tissues
– life span 120 days
– manufacture regulated by erythropoetin
– Normal Hematocrit- 35-45%
– Normal Hemoglobin- 12-16 grams
Problems of Erythrocyte Production
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Anemia – reduction of RBC volume or
Hgb concentration below normal
Classifications:
1. Etiology/Pathophysiology –
causes of RBC/Hgb depletion
2. Morphology – changes in RBC
size, shape, and color
Causes of Anemia
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Nutritional deficiency – iron, folate, B12
Increased destruction of RBCs – sickle
cell anemia
Impaired or decreased rate of
production – aplastic anemia
Excessive blood loss - hemophilia
Iron Deficiency Anemia
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Causes
- inadequate supply of iron
- impaired absorption
- blood loss
- excessive demands for iron req’d for
growth
- inability for form Hgb
Iron Deficiency Anemia
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Signs and Symptoms: due to tissue
hypoxia > lack of energy, easy
fatigability, pallor
Diagnosis: CBC with diff, red cell
indices (MCV, MCH, MCHC), iron
studies, physical exam
Medical Treatment: supplement with
ferrous sulfate (dosages vary with age),
dietary counseling
Iron Deficiency Anemia
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Nursing Assessment and Interventions:
- educate parents about nutrition
- explain laboratory testing
- teach parents proper administration
of iron preparations, caution about
high toxicity of iron
Sickle Cell Anemia
Causes: genetic transmission, 2 parents
with the trait have 25% chance of
having child with SCD, found primarily
in Blacks, occ Hispanics
 Hgb A is partly or completely replaced
by Hgb S
 With dehydration,acidosis, hypoxia, and
temp elevations, Hgb S “sickles”
Sickle Cell Anemia
Pathophysiology:
- vaso-occlusion from sickled RBCs
- increased RBC destruction
- splenic congestion and enlargement
- hepatomegaly, liver failure
- renal ischemia, hematuria
- osteoporosis, lordosis, kyphosis
- cardiomegaly, heart failure, stroke
Sickle Cell Anemia
Signs/Symptoms:
 Exercise intolerance
 Anorexia
 Jaundiced sclera
 Gallstones
 Chronic leg ulcers
 Growth retardation
Sickle Cell Anemia
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Diagnosis
- Sickledex
- Hgb electrophoresis
- Stained blood smear
Vaso-occlusive crisis
- mild to severe bone pain
- acute abdominal pain
- priapism
- arthralgia
Sickle Cell Anemia
Medical management
 Supportive/symptomatic tx of crises
- bed rest
- hydration
- electrolyte replacement
- analgesics for pain
- blood replacement
- antibiotics
- oxygen therapy
Sickle Cell Anemia
Nursing care:
 Minimize tissue deoxygenation
 Promote hydration
 Minimize crises
 Pain management
 Administering blood transfusions
 Encourage screening and genetic counseling
 Parent education
Thalassemia
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Autosomal recessive disorder – Greeks,
Italians, Syrians
Signs/symptoms – microcytic anemia >
splenomegaly,jaundice,epistaxis, gout
Diagnosis – Hgb electrophoresis
Medical Treatment – transfusions,
chelation
Hemophilia
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Factor 8 or factor 9 deficiency
– prolonged bleeding any where in the body!
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Cause: X-linked recessive disorder,
defects in platelets and clotting factors
Diagnosis: history of bleeding episodes,
evidence of x-linked inheritence, labs
Medical Management: Factor VIII
concentrate, DDAVP (vasopressin)
Hemophilia
Nursing care:
 Prevent bleeding
 Recognize and control bleeding (RICE)
- Rest
- Ice
- Compression
- Elevation
 Prevent crippling effects of bleeding
 Client education
Idiopathic Thrombocytopenic Purpura
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Causes: acquired hemorrhagic disorder of
unknown origin, probably an autoimmune
response to disease-related antigens
Diagnosis: platelet count < 20,000, abnl
bleeding time and clot retraction
Signs and Symptoms: petechiae, bruising,
bleeding from mucous membranes,
prolonged bleeding from abrasions
Medical management: supportive, steroids,
Anti-D antibody, splenectomy
Idiopathic Thrombocytic
Puerpera
Nursing Considerations:
 Client/Parent teaching
 No contact sports
 No aspirin
 Prevent infection
Blood Transfusion
Complications:
 Hemolytic reactions
- chills, shaking, fever
- dyspnea
- flank pain
- progressive signs of shock
 Febrile reactions
 Allergic reactions
- urticaria, flushing
- wheezing
 Circulatory overload
Blood Transfusions
Nursing Care
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Take VS BEFORE administering blood
Check ID of recipient with donor’s blood type
Administer 50 mL or 1/5 volume SLOWLY – STAY
WITH THE CHILD
Administer with NS on piggyback set-up
Use appropriate filter
Use within 30 mins – infuse within 4 hrs
If reaction suspected: Stop the transfusion, maintain
patent IV line with NS, take VS, notify practitioner