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

lymphatic system regulates maturation

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

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

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

Iron Deficiency Anemia

Causes - inadequate supply of iron - impaired absorption - blood loss - excessive demands for iron reqd for growth - inability for form Hgb

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

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

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

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

Sickle Cell Anemia

Signs/Symptoms: Exercise intolerance Anorexia Jaundiced sclera Gallstones Chronic leg ulcers Growth retardation

Sickle Cell Anemia

Diagnosis - Sickledex - Hgb electrophoresis - Stained blood smear Vaso-occlusive crisis - mild to severe bone pain - acute abdominal pain - priapism - arthralgia

Sickle Cell Anemia

Medical management Supportive/symptomatic tx of crises - bed rest - hydration - electrolyte replacement - analgesics for pain - blood replacement - antibiotics - oxygen therapy

Sickle Cell Anemia

Nursing care: Minimize tissue deoxygenation Promote hydration Minimize crises Pain management Administering blood transfusions Encourage screening and genetic counseling Parent education

Thalassemia

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

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)

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

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

Idiopathic Thrombocytic Puerpera

Nursing Considerations: Client/Parent teaching No contact sports No aspirin Prevent infection

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

Blood Transfusions
Nursing Care

Take VS BEFORE administering blood Check ID of recipient with donors 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