Pediatric Hematological DisordersWhaley and WongChapters 35, 36

Components of the BloodBlood:Plasma water, albumin, electrolytes, clotting factorsCellular ComponentsRBCs, WBCs, PlateletsAll formed in the red bone marrow (after birth)In utero- spleen, thymus, liverlymphatic system regulates maturation

ErythrocytesRBCs carry hemoglobin which is attached to oxygen- provides O2 to the tissueslife span 120 daysmanufacture regulated by erythropoetinNormal Hematocrit- 35-45%Normal Hemoglobin- 12-16 grams

Problems of Erythrocyte ProductionAnemia 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 AnemiaNutritional deficiency iron, folate, B12Increased destruction of RBCs sickle cell anemiaImpaired or decreased rate of production aplastic anemiaExcessive blood loss - hemophilia

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

Iron Deficiency AnemiaSigns and Symptoms: due to tissue hypoxia > lack of energy, easy fatigability, pallorDiagnosis: CBC with diff, red cell indices (MCV, MCH, MCHC), iron studies, physical examMedical Treatment: supplement with ferrous sulfate (dosages vary with age), dietary counseling

Iron Deficiency AnemiaNursing 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 AnemiaCauses: genetic transmission, 2 parents with the trait have 25% chance of having child with SCD, found primarily in Blacks, occ HispanicsHgb A is partly or completely replaced by Hgb SWith dehydration,acidosis, hypoxia, and temp elevations, Hgb S sickles

Sickle Cell AnemiaPathophysiology: - 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 AnemiaSigns/Symptoms:Exercise intoleranceAnorexiaJaundiced scleraGallstonesChronic leg ulcersGrowth retardation

Sickle Cell AnemiaDiagnosis - Sickledex - Hgb electrophoresis - Stained blood smearVaso-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 AnemiaNursing care:Minimize tissue deoxygenationPromote hydrationMinimize crisesPain managementAdministering blood transfusionsEncourage screening and genetic counselingParent education

ThalassemiaAutosomal recessive disorder Greeks, Italians, SyriansSigns/symptoms microcytic anemia > splenomegaly,jaundice,epistaxis, goutDiagnosis Hgb electrophoresisMedical Treatment transfusions, chelation

HemophiliaFactor 8 or factor 9 deficiencyprolonged bleeding any where in the body!Cause: X-linked recessive disorder, defects in platelets and clotting factorsDiagnosis: history of bleeding episodes, evidence of x-linked inheritence, labsMedical Management: Factor VIII concentrate, DDAVP (vasopressin)

HemophiliaNursing care:Prevent bleedingRecognize and control bleeding (RICE) - Rest - Ice - Compression - ElevationPrevent crippling effects of bleedingClient education

Idiopathic Thrombocytopenic PurpuraCauses: acquired hemorrhagic disorder of unknown origin, probably an autoimmune response to disease-related antigens Diagnosis: platelet count < 20,000, abnl bleeding time and clot retractionSigns and Symptoms: petechiae, bruising, bleeding from mucous membranes, prolonged bleeding from abrasionsMedical management: supportive, steroids, Anti-D antibody, splenectomy

Idiopathic Thrombocytic PuerperaNursing Considerations:Client/Parent teachingNo contact sportsNo aspirinPrevent infection

Blood TransfusionComplications:Hemolytic reactions - chills, shaking, fever - dyspnea - flank pain - progressive signs of shockFebrile reactionsAllergic reactions - urticaria, flushing - wheezingCirculatory overload

Blood TransfusionsNursing CareTake VS BEFORE administering bloodCheck ID of recipient with donors blood typeAdminister 50 mL or 1/5 volume SLOWLY STAY WITH THE CHILDAdminister with NS on piggyback set-upUse appropriate filterUse within 30 mins infuse within 4 hrsIf reaction suspected: Stop the transfusion, maintain patent IV line with NS, take VS, notify practitioner