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Pediatric Hematological Disorders

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

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

  2. 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

  3. 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

  4. 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

  5. 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

  6. Iron Deficiency Anemia • 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 • 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

  8. 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

  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 • Hgb A is partly or completely replaced by Hgb S • 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: • Exercise intolerance • Anorexia • Jaundiced sclera • Gallstones • Chronic leg ulcers • Growth retardation

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

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

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

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

  16. 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)

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

  18. 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

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

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

  21. 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

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