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Hematologic-Oncology

Hematologic-Oncology. Common Hematologic Disorders in Children. Iron-Deficiency Anemia Sickle Cell Anemia Beta-Thalasemia Major (Cooley’s anemia) Hemophilia A Von Willebrand’s Disease ITP (Immune Thrombocytopenic Pupura). Common Heme-Oncology Diseases in Children.

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Hematologic-Oncology

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  1. Hematologic-Oncology

  2. Common Hematologic Disorders in Children • Iron-Deficiency Anemia • Sickle Cell Anemia • Beta-ThalasemiaMajor (Cooley’s anemia) • Hemophilia A • Von Willebrand’s Disease • ITP (Immune Thrombocytopenic Pupura)

  3. Common Heme-Oncology Diseases in Children • Acute Lymphocytic Leukemia • Hodgkin’s Disease • Non-Hodgkin’s Lymphoma • Retinoblastoma • Neuroblastoma • Nephroblastoma • Osteogenic Sarcoma • Ewing’s Sarcoma

  4. Complete Blood Count • WBC • RBC • Hgb • Hct • Platelet

  5. CBC with Differential • WBC • Neutrophils- phagocytosis • Lymphocytes – T and B cell • Monoocytes – phagocytosis, antigen • Eosophils- allergen • Basophils-inflammatory • RBC • MCV- volume • MCH • MCHC • RCW- width • Hgb • Hct • Platelet • MPV

  6. Other Labs • PT/PTT • Sed Rate (ESR) • Iron • TIBC (Transferrin) • Ferritin • Bilirubin

  7. Pediatric Laboratory Normal Values: Children age 2-12 Years • Platelets: 206,000-403,000 • Fe: 20-105 • Ferritin: 47-110 • TIBC: 240-508 • PT: 10-11 sec • PTT: 42-54 sec • Bilirubin- less than 11.7 • RBC: 3.89-4.96 • HgB: 10.2-13.4 • Hct: 31.7-39.3% • Sed: 1-8 • WBC: 5,400-11,000

  8. ) Anemia’s • Reduction of: • number of red blood cells • the quantity of hemoglobin • the volume of packed red • Iron-Deficiency Anemia • Sickle Cell Anemia • Beta-ThalasemiaMajor (Cooley’s anemia

  9. Iron Deficiency Anemia

  10. Iron-Deficiency Anemia • A nutrient deficiency of inadequate dietary iron • The most common hematologic disorder of infancy and childhood • Peaks at 9 months- 2 years, adolescence • Prevented by use of iron fortified productsn

  11. Children at Risk • low birth weight infants • infants born to mothers with iron deficiency anemia • infants born with GI defects • chronic blood loss in older children

  12. Pathophysiology • Dietary Fe is bloodstream binds to transferrin (TIBC) and is delivered to RBC in bone • Marrow, combines with other cells to make Hgb • Unused dietary Fe is stored in intestinal epithelial cells as ferritin

  13. History • Dietary history usually shows abnormally high milk intake > 32 oz day in toddler • Ask parents specific questions • Begin the dietary history at the time the child awoke yesterday; include all activities and exactly what the child ate

  14. Diagnosis • Low RBC • Low HGB • Mild ( < 10.2), Moderate (8-9), Severe (< 7) • Low HCT • Low Iron • High Transferrin (TIBC) • Low Ferritin

  15. Symptoms • Low Hgb=low O2 tissue perfusion • Hgb of 10.2 or less • May seem asymptomatic, not noticed by caregiver • Pallor/Pale mucous membranes (low hgb, not enough red color to skin) • Poor muscle tone, decreased activity • Fatigue • Increased HR, RR • Hgb < 9 • Above symptoms plus irritability, lack of interest in play

  16. Nursing Considerations • Consume Iron-fortified formula • Limit cow’s milk to 24-32 oz/day for children >12 months • Increase age-appropriate iron-rich foods and Vit C • May be ordered to take Fe supplements- Ferrous Sulfate

  17. Nursing Considerations

  18. Nursing Considerations • Manage side effects of Ferrous Sulfate • Nausea, • Anorexia • Constipation • Abdominal distress • Black stools. • Give on an empty stomach if possible • Monitor bowel movements and suggest increased fluid and fiber.

  19. Nursing Considerations • Monitor development, sleep, and activity/fatigue patterns • Monitor hemoglobin to measure effectiveness of therapy • Instruct families to keep Ferrous Sulfate locked and out of reach of children; poisoning is a serious risk

  20. Sickle Cell Anemia

  21. Sickle Cell Anemia • Autosomal recessive disorder • Seen in African Americans • Characterized by abnormal hemoglobin (HbS) • Clinical manifestations caused by obstructions due to the sickled RBC’s and destruction of sickled and normal RBC’s

  22. Sickle Cell Anemia • Can be diagnosed in-utero • Symptoms may not appear until 6 months of age • Mortality rate children < 3 years old is 35%

  23. Signs and Symptoms • Pallor • Fatigue • SOB • Irritability • Jaundice

  24. Diagnosis • Moderately low Hcb and Hct • Normal Iron, TIBC, Ferritin • Elevated Billirubin

  25. 3 Complications of Sickle Cell Anemia • Vaso-Occlusive Crisis • Acute Chest Syndrome • Splenic Sequestration

  26. Vaso-occlusive crisis • Severe, sudden onset of sickling where many new sickled cells pool in a vessel and cause pain and tissue hypoxia • Caused by: infection, dehydration, anxiety, cold • Most common from hypoxia secondary to rapidly destroyed RBC • Lasts for hours to weeks

  27. Vaso-occlusive Crisis First Crisis in infants • Dactylitis (hand & foot syndrome) • swelling of hands and feet • joints may be warm & swollen • Early Signs • fever • tachycardia • pallor • Late Signs • abdominal pain • back pain • extremity pain

  28. Management • Pain relief • Prevent/Treat occulsions • Adequate oxygenation

  29. Pain • Assess pain every 1-2h or more frequently • Use pain scale appropriate for age • Non-pharmacological pain methods • AROUND THE CLOCK PAIN MEDS • Tylenol for mild pain • Narcotics for mod-severe pain

  30. Hydration • Prevents and treats occlusion • Push PO fluids • IV hydration 1.5 to 2 times normal rate • Risk for fluid overload • Listen for crackles

  31. Treat Sickling-Oxygenation • Administer oxygen • Maintain saturation of 95% or higher • Semi-fowler’s position

  32. Acute Chest Syndrome • Sickle contents break off • Bilateral pulmonary involvement • Causes chest infection, embolism

  33. Nursing Considerations • Symptoms • Chest pain • Fever • Cough • Wheeze • Tachypnea • Analgesics • Oxygen • Hydration • Incentive spirometry • Antibiotics • PRBC

  34. Splenic Sequestration • Sickled cells block the spleen • Blood pools in spleen and/or liver and enlarges • Pooled blood leads to a decrease in circulating volume • Can lead to hypovolemic shock

  35. Nursing Considerations • Symptoms • Irritability • Pale • Tachycardia • Pain to LUQ • Enlarged Spleen • Life Threatening- get child to ED a.s.a.p.! • PRBC • Remove spleen

  36. Post-Splenectomy • Risk for Infection r/t Chronic Immunosuppression • Administer PCN everyday • Up-to-date vaccines • Educate parents • Signs of infection & respiratory distress • possible triggers • treat pain immediately • adequate fluids

  37. Beta-ThalasemiaMajor (Cooley’s Anemia)

  38. Beta-ThalasemiaMajor (Cooley’s anemia) • Hereditary anemia due to abnormal synthesis of hemoglobin • Life long disorder • Mediterranean descent • Life threatening symptoms

  39. Diagnosis • Low RBC’s • Extremely low Hgb < 5 • Increased serum iron

  40. Symptoms • Facial anomalies • Frontal bossing (prominent and protruding forehead) • Maxillary prominence • Wide-set eyes with a flattened nose • Bronze skin color (Greenish yellow skin tone) • Growth and maturation retardation

  41. Management: • RBC transfusions q 2-4 weeks • Iron Chelation therapy • Desferal (deferoxamine) SQ • Splenectomy • Cure isbone marrow stem cell transplant • Estimated 70% do not find a suitable donor

  42. Nursing Considerations • Observe for complications of transfusion- iron overload • Supporting the child and family in dealing with a chronic life-threatening illness • Monitor Growth and Development • Refer the family for genetic counseling

  43. Compare and Contrast

  44. Bleeding Disorders

  45. Clotting • Host of factors • Platelets aggregation at site of injury • Tested by coagulation time (PT/PTT)

  46. Types of Bleeding Disorders • Hemophilia A • Von Willebrand’s Disease • ITP (Immune Thrombocytopenic Pupura)

  47. Hemophilia A

  48. Hemophilia A • Hereditary blood coagulation deficiency (factor 8) • Ability to clot is slower • X-linked recessive (white, males)

  49. If Suspected ask about • Recent traumas and measures used to stop bleeding • Length of time pressure was applied before bleeding subsided • Whether swelling increased after surface bleeding subsided • Whether swelling and stiffness occurred without apparent trauma

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