1 / 21

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

alpha
Télécharger la présentation

Pediatric Hematological Disorders

An Image/Link below is provided (as is) to download presentation Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author. Content is provided to you AS IS for your information and personal use only. Download presentation by click this link. While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server. During download, if you can't get a presentation, the file might be deleted by the publisher.

E N D

Presentation Transcript


  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

More Related