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

2. Components of the Blood n 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 n 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

4. Problems of Erythrocyte Production n 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

5. Causes of Anemia n Nutritional deficiency – iron, folate, B12 n Increased destruction of RBCs – sickle cell anemia n Impaired or decreased rate of production – aplastic anemia n Excessive blood loss - hemophilia

6. Iron Deficiency Anemia n Causes - inadequate supply of iron - impaired absorption - blood loss - excessive demands for iron req’d for growth - inability for form Hgb

7. Iron Deficiency Anemia n Signs and Symptoms: due to tissue hypoxia > lack of energy, easy fatigability, pallor n Diagnosis: CBC with diff, red cell indices (MCV, MCH, MCHC), iron studies, physical exam n Medical Treatment: supplement with ferrous sulfate (dosages vary with age), dietary counseling

8. Iron Deficiency Anemia n 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 n Hgb A is partly or completely replaced by Hgb S n With dehydration,acidosis, hypoxia, and temp elevations, Hgb S “sickles”

10. 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

11. Sickle Cell Anemia Signs/Symptoms: n Exercise intolerance n Anorexia n Jaundiced sclera n Gallstones n Chronic leg ulcers n Growth retardation

12. Sickle Cell Anemia n Diagnosis - Sickledex - Hgb electrophoresis - Stained blood smear n Vaso-occlusive crisis - mild to severe bone pain - acute abdominal pain - priapism - arthralgia

13. Sickle Cell Anemia Medical management n Supportive/symptomatic tx of crises - bed rest - hydration - electrolyte replacement - analgesics for pain - blood replacement - antibiotics - oxygen therapy

14. Sickle Cell Anemia Nursing care: n Minimize tissue deoxygenation n Promote hydration n Minimize crises n Pain management n Administering blood transfusions n Encourage screening and genetic counseling n Parent education

15. Thalassemia n Autosomal recessive disorder – Greeks, Italians, Syrians n Signs/symptoms – microcytic anemia > splenomegaly,jaundice,epistaxis, gout n Diagnosis – Hgb electrophoresis n Medical Treatment – transfusions, chelation

16. Hemophilia n Factor 8 or factor 9 deficiency –prolonged bleeding any where in the body! n Cause: X-linked recessive disorder, defects in platelets and clotting factors n Diagnosis: history of bleeding episodes, evidence of x-linked inheritence, labs n Medical Management: Factor VIII concentrate, DDAVP (vasopressin)

17. Hemophilia Nursing care: n Prevent bleeding n Recognize and control bleeding (RICE) - Rest - Ice - Compression - Elevation n Prevent crippling effects of bleeding n Client education

18. Idiopathic Thrombocytopenic Purpura n Causes: acquired hemorrhagic disorder of unknown origin, probably an autoimmune response to disease-related antigens n Diagnosis: platelet count < 20,000, abnl bleeding time and clot retraction n Signs and Symptoms: petechiae, bruising, bleeding from mucous membranes, prolonged bleeding from abrasions n Medical management: supportive, steroids, Anti-D antibody, splenectomy

19. Idiopathic Thrombocytic Puerpera Nursing Considerations: n Client/Parent teaching n No contact sports n No aspirin n Prevent infection

20. Blood Transfusion Complications: n Hemolytic reactions - chills, shaking, fever - dyspnea - flank pain - progressive signs of shock n Febrile reactions n Allergic reactions - urticaria, flushing - wheezing n Circulatory overload

21. Blood Transfusions Nursing Care n Take VS BEFORE administering blood n Check ID of recipient with donor’s blood type n Administer 50 mL or 1/5 volume SLOWLY – STAY WITH THE CHILD n Administer with NS on piggyback set-up n Use appropriate filter n Use within 30 mins – infuse within 4 hrs n If reaction suspected: Stop the transfusion, maintain patent IV line with NS, take VS, notify practitioner