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Pediatric Hematological Disorders. Whaley and Wong Chapters 35, 36. 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

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pediatric hematological disorders

Pediatric Hematological Disorders

Whaley and Wong

Chapters 35, 36

components of the blood
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
erythrocytes
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
problems of erythrocyte production
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

causes of anemia
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
iron deficiency anemia
Iron Deficiency Anemia
  • Causes

- inadequate supply of iron

- impaired absorption

- blood loss

- excessive demands for iron req’d for

growth

- inability for form Hgb

iron deficiency anemia1
Iron Deficiency Anemia
  • 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 anemia2
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

sickle cell anemia
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 anemia1
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 anemia2
Sickle Cell Anemia

Signs/Symptoms:

  • Exercise intolerance
  • Anorexia
  • Jaundiced sclera
  • Gallstones
  • Chronic leg ulcers
  • Growth retardation
sickle cell anemia3
Sickle Cell Anemia
  • Diagnosis

- Sickledex

- Hgb electrophoresis

- Stained blood smear

  • Vaso-occlusive crisis

- mild to severe bone pain

- acute abdominal pain

- priapism

- arthralgia

sickle cell anemia4
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 anemia5
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
Thalassemia
  • Autosomal recessive disorder – Greeks,

Italians, Syrians

  • Signs/symptoms – microcytic anemia >

splenomegaly,jaundice,epistaxis, gout

  • Diagnosis – Hgb electrophoresis
  • Medical Treatment – transfusions, chelation
hemophilia
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)
hemophilia1
Hemophilia

Nursing care:

  • Prevent bleeding
  • Recognize and control bleeding (RICE)

- Rest

- Ice

- Compression

- Elevation

  • Prevent crippling effects of bleeding
  • Client education
idiopathic thrombocytopenic purpura
Idiopathic Thrombocytopenic Purpura
  • 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
Idiopathic Thrombocytic Puerpera

Nursing Considerations:

  • Client/Parent teaching
  • No contact sports
  • No aspirin
  • Prevent infection
blood transfusion
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
Blood Transfusions

Nursing Care

  • 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