Hematologic-Oncology Stressors and Adaptation

1. Hematologic-Oncology Stressors and Adaptation

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 • Aplastic Anemia • Acute Lymphocytic Leukemia • Hodgkin’s Disease • Non-Hodgkin’s Lymphoma • Neuroblastoma • Osteogenic Sarcoma • Ewing’s Sarcoma • Retinoblastoma

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

5. CBC with Differential • WBC • Neutrophils • Lymphocytes • Monoocytes • Eosophils • Basophils • RBC • MCV • MCH • MCHC • RCW • Hgb • Hct • Platelet • MPV

6. PT, PTT • The prothrombin time (PT) test measures how long it takes for a clot to form in a sample of blood. • Prothrombin is one of several clotting factors that are produced by the liver. • The PT test evaluates the integrated function of these factors and the body’s ability to produce a clot in a reasonable amount of time. • Because the reagents used to perform the PT test vary from one laboratory to another and even within the same laboratory over time, the normal values also will fluctuate.

7. Other Labs • Sed Rate (ESR) • Iron • TIBC (Transferrin) • Ferritin

8. RBC: 3.89-4.96 HgB: 10.2-13.4 Hct: 31.7-39.3% Sed: 1-8 WBC: 5.4-11 Neutrophils: 34.3-72.9 Esinophils: 2.4-4.8 Basophils: 1 Lymphocytes: 13.5-52.8 Monocytes: 3.5-13.4 Platelets: 206-403 Fe: 20-105 Ferritin: 47-110 TIBC: 240-508 PT: 10-11 sec PTT: 42-54 sec Pediatric Laboratory Values: Children age 2-12 Years

9. Iron-Deficiency Anemia The most common hematologic disorder of infancy and childhood • 9 months- 2 years, adolescence • A nutrient deficiency of inadequate dietary iron Prevention: iron fortified products

10. 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

11. 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

12. Diagnosis Based on levels of • RBC • Hgb • Hct • Iron • Transferrin (TIBC) • Ferritin

13. Diagnosis • RBC’s • hypocytic • hyochromic • HGB < 10-11g • Mild ( < 11), Moderate (7-11), Severe (3-7) • HCT < 33% • Iron 30ug /100ml • Transferrin (TIBC) > 350ug • Ferritin < 10ug

14. Symptoms Low Hgb=low O2 tissue perfusion Hgb of 10 • 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 < 10 • Above plus irritability, lack of interest in play

15. 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

16. Management Dietary Modifications • Iron-fortified formula or supplements 4-6 weeks • Limit cow’s milk to 24-32 oz/day for children >12 months • Increase age-appropriate iron-rich foods and Vit C • Fe supplements • Monitor compliance

17. Sickle Cell Anemia • Autosomal recessive disorder, 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

18. Sickle Cell Anemia Symptoms may not appear until 6 months of age Mortality rate children < 3 yo is 15-35% Diagnosis: Amniocentesis, CVS, Newborn Screen

19. Signs & Symptoms: Initially: fever & anemia at 6 mos • Pallor • Fatigue • Jaundice • SOB • Irritability

20. 3 Sequalea of SCA • Vaso-Occlusive Crisis • Acute Chest Syndrome • Splenic Sequestration

21. Sickle Cell CrisisVaso-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, hypoxia, cold • Lasts for hours to weeks

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

23. Management of Crisis Three primary needs: • pain relief • adequate hydration • adequate oxygenation

24. 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

25. Pain • Narcotics for mod-severe pain • IV hydration: 1.5 to 2 times normal rate • Administer blood transfusions to increase Hgb • Bedrest

26. Altered Tissue Perfusion • Assess vital signs and respiratory status q4h and PRN • Pulse oximetry • Administer oxygen to maintain saturation of 95% or higher • Semi-fowler’s position • Administer PRBC’s

27. Acute Chest Syndrome • Sickle contents break off • bilateral pulmonary involvement • chest pain, fever, cough

28. Splenic Sequestration • Sickled cells block the spleen • pooled blood in spleen and/or liver and enlarges • Pooled blood leads to a decrease in circulating volume= hypovolemic shock • CVA => coma • TX: PRBC, Splenectomy

29. Risk for Infection r/t Chronic Immunosuppression • Administer PCN everyday • Up-to-date vaccines Educate parents: s/s infection & respiratory distress possible triggers treat pain immediately adequate fluids

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

31. Diagnosis • RBC’s • hypochromic • microcytic • Hgb < 5g • Increased serum iron

32. 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 • Hepatosplenomegaly • Diabetes Mellitus • Cardiac dilatation

33. Management: • RBC transfusions q2-4 weeks to get Hgb to 10-12 • Iron Chelation therapy • Desferal (deferoxamine) SQ • Splenectomy • Digitalis, diuretics, low salt diet • Cure: bone marrow stem cell transplant

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

35. Symptoms • Vary according to concentration of factor 8 • Soft tissue bleeding and painful hemorrhage into joints • Severe bleeding may occur in GI tract, peritoneum or CNS

36. Interviewing the Child with Hemophilia–Subjective Data • 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

37. Diagnosis • Above History • Labs: • Platelet level: Normal • PTT: Prolonged (elevated number) > 60

38. Management Acute therapy: • Bleeding must be controlled by IV administration of factor 8 • After trauma, surgery • Pressure to laceration Prophylactic therapy: • Children age 1-2 receive PO factor 8 replacement on a regular schedule if frequently symptomatic • prior to surgery, dental work

39. Parental Education • Injury Prevention • Promote oral hygiene, up to date immunizations • No aspirin • Avoid activities that induce bleeding • Provide activities for normal G&D • Administration of factor replacement

40. Von Willebrand’s Disease • Most commonly inherited bleeding disorder, autosomal dominant (Males and Females) • Lacks production of VWF • Inability of platelets to aggregate • Varying degrees of disease • VWF is deficient to defective

41. Signs & Symptoms Can be so mild that disease is undiagnosed • Epitaxis • Prolonged bleeding from cuts • Excessive bleeding following surgery • Bleeding from gums

42. Management • Replace dysfunctional factor in blood • Treatment of Choice: DDAVP • Prophylactic therapy • Injury Prevention

43. ITP (Immune Thrombocytopenic Pupura) • Autoimmune disorder (antiplatelet antibody) or cause is unknown (idiopathic) • Occurs most commonly at age 2-4 years • Reduction in and destruction of platelets • Typically seen 2 weeks after a febrile, viral illness • Congential: if mother had ITP during pregnancy

44. Signs & Symptoms • Excessive bruising and petechiae • Epitaxis • Bleeding into joints • Tourniquet test: shows many petechiae after inflation of BP cuff

45. Diagnosis Labs: • Platelets < 150 (Marked thrombocytopenia) • PT and PTT: Normal

46. Management • Prednisone • IVIG (IV immunoglobulin) • PLT transfusion (only a temporary solution) • Most cases are self-limiting • Avoid when possible: • administering intramuscular injections • aspirin, aspirin-containing products, and nonsteroidal antiinflammatory medications (e.g., ibuprofen) • taking temperatures rectally • perform invasive procedures with extreme caution

47. Aplastic Anemia Bone marrow stops production of RBC, WBC and platelets (pancytopenia) Congenital • inherited autosomal recessive • Fanconi’s Syndrome: pancytopenia plus skeletal & renal abnormalities, hypogenitalism, short stature Acquired • Idopathic • Excessive exposure to radiation or drugs causing bone marrow damage

48. Signs & Symptoms Mean age is 6-8 years • Bruising • Anemia • Pallor • Weakness • Petechiae • Epitaxis • Multiple infections • Cardiac decompensation

49. Management: • Bone marrow transplant (if donor available) • Platelet and RBC transfusions • Maintain platelets > 20,000/mm • HBG >7g/dl • Suppress autoimmune response • Cortiosteroids • Testosterone Parent education: Prevent infection, protect from injury

50. Oncology • Cancer in adults • abnormal cell is transformed by genetic mutation of its DNA • usually as a result from exposure to a tetragon • Cancer in children • usually arises from chromosomal abnormalities, genetic mutations and proliferation of embryonic cells