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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. 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 • Bilirubin

  8. RBC: 3.89-4.96 HgB: 10.2-13.4 Hct: 31.7-39.3% Sed: 1-8 WBC: 5,400-11,000 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 Pediatric Laboratory Normal Values: Children age 2-12 Years

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

  10. 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

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

  14. 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 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 • 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 • 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 • 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

  21. 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

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

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

  24. 3 Sequalea of SCA • Vaso-Occlusive Crisis • Acute Chest Syndrome • Splenic Sequestration

  25. 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

  26. 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

  27. Management • Pain relief • Adequate hydration • Adequate oxygenation

  28. 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

  29. Prevent Occlusion • Push PO fluids • IV hydration: 1.5 to 2 times normal rate • Risk for fluid overload

  30. Altered Tissue Perfusion and Prevent Further Sickling • Administer oxygen to maintain saturation of 95% or higher • Pulse oximetry • Semi-fowler’s position • Administer PRBC’s

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

  32. Nursing Considerations • Know the symptoms: • Chest pain • Fever • Cough • Wheeze • Tachypnea • Analgesics • Oxygen • Hydration Incentive spirometry, • Antibiotics • PRBC

  33. 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

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

  35. 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

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

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

  38. 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

  39. Management: • RBC transfusions q2-4 weeks to get Hgb to 10-12 • Iron Chelation therapy • Desferal (deferoxamine) SQ • Splenectomy • Cure: bone marrow stem cell transplant • Estimated 70% do not find a suitable donor

  40. 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.

  41. Compare and Contrast

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

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

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

  45. 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

  46. 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

  47. Diagnosis • Above History • Suspected by Labs: • Platelet level: Normal • PTT: Prolonged (elevated number) > 60 • Confirmed by genetic testing for missing factor

  48. Management of Bleeding 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

  49. Parental Education • Primary Goal: 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 prn

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

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