Pediatric Hematological Disorders

1
Pediatric Hematological Disorders

• Whaley and Wong
• Chapters 35, 36

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Components of the Blood

• 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

3
Erythrocytes

• 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

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Problems of Erythrocyte Production

• 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

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Causes of Anemia

• Nutritional deficiency iron, folate, B12
• Increased destruction of RBCs sickle
• cell anemia
• Impaired or decreased rate of
• production aplastic anemia
• Excessive blood loss - hemophilia

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Iron Deficiency Anemia

• Causes
• - inadequate supply of iron
• - impaired absorption
• - blood loss
• - excessive demands for iron reqd for
• growth
• - inability for form Hgb

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Iron Deficiency Anemia

• Signs and Symptoms due to tissue hypoxia gt 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

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Iron Deficiency Anemia

• Nursing Assessment and Interventions
• - educate parents about nutrition
• - explain laboratory testing
• - teach parents proper administration
• of iron preparations, caution about
• high toxicity of iron

9
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

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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

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Sickle Cell Anemia

• Signs/Symptoms
• Exercise intolerance
• Anorexia
• Jaundiced sclera
• Gallstones
• Chronic leg ulcers
• Growth retardation

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Sickle Cell Anemia

• Diagnosis
• - Sickledex
• - Hgb electrophoresis
• - Stained blood smear
• Vaso-occlusive crisis
• - mild to severe bone pain
• - acute abdominal pain
• - priapism
• - arthralgia

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Sickle Cell Anemia

• Medical management
• Supportive/symptomatic tx of crises
• - bed rest
• - hydration
• - electrolyte replacement
• - analgesics for pain
• - blood replacement
• - antibiotics
• - oxygen therapy

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Sickle Cell Anemia

• Nursing care
• Minimize tissue deoxygenation
• Promote hydration
• Minimize crises
• Pain management
• Administering blood transfusions
• Encourage screening and genetic counseling
• Parent education

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Thalassemia

• Autosomal recessive disorder Greeks,
• Italians, Syrians
• Signs/symptoms microcytic anemia gt
• splenomegaly,jaundice,epistaxis, gout
• Diagnosis Hgb electrophoresis
• Medical Treatment transfusions, chelation

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Hemophilia

• Factor 8 or factor 9 deficiency
• prolonged bleeding any where in the body!
• 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)

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Hemophilia

• Nursing care
• Prevent bleeding
• Recognize and control bleeding (RICE)
• - Rest
• - Ice
• - Compression
• - Elevation
• Prevent crippling effects of bleeding
• Client education

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Idiopathic Thrombocytopenic Purpura

• Causes acquired hemorrhagic disorder of unknown
  origin, probably an autoimmune response to
  disease-related antigens
• Diagnosis platelet count lt 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

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Idiopathic Thrombocytic Puerpera

• Nursing Considerations
• Client/Parent teaching
• No contact sports
• No aspirin
• Prevent infection

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Blood Transfusion

• Complications
• Hemolytic reactions
• - chills, shaking, fever
• - dyspnea
• - flank pain
• - progressive signs of shock
• Febrile reactions
• Allergic reactions
• - urticaria, flushing
• - wheezing
• Circulatory overload

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Blood Transfusions

• Nursing Care
• Take VS BEFORE administering blood
• Check ID of recipient with donors 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