Pedi Hem/Onc Flashcards
Decreased number/volume or impaired function of erythrocytes (RBC’s). Sickle cell disease. Beth-thalassemia. Aplastic anemia.
Types of anemias
Acute Lymphoblastic Leukemia (ALL)
Acute Myelogenous Leukemia (AML)
Types of Leukemia
Hodgkin Lymphoma
Non Hodgkin Lymphoma
Types of Lymphoma
Hemophilia
Von Willebrand Disease
Disseminated Intravascular Coagulation (DIC)
Types of Bleeding Disorders
Brain tumors
Neuroblastoma
Wilms Tumor
Retinoblastoma
Solid Tumors
Types:
Rhabdomyosarcoma
thats it
Soft-Tissue Tumors
Osetosarcoma
Ewing Sarcoma
Bone Tumors
Abnormal Hgb gene = Hgb Sickle gene. Sickle shaped RBC’s that clump together. 60-65%; most severe. 1 of every 400-600 African American births. Autosomal recessive (gene from each parent). Fetal Hgb protects from sickling for 1st 4-6 months.
Sickle Cell Disease General
Physiologic Stress causes: dehydration, fever, hypoxia, hypothermia, acidosis leading to sickling. Sickled RBC’s adhere, clump, clog causing vasoocclusion and thickening of circulating blood. Blood flow blocked, O2 and nutrient delivery blocked leading to pain and tissue damage. Sickled cells die more quickly than normal RBC’s, so low RBC count leads to hemolytic anemia. Free iron from lysed RBCs deposits in organs/tissues causing more damage. TISSUE INJURY/DEATH BY OCCLUSION AND HEMOLYSIS
Sickle Cell Disease S/S and Patho
Can affect entire body. Decreased O2 in tissues causes infarcts at the site of occlusion equalling cellular death and pain. Under 5 years the bones and hands and feet can lead to dactylitis, edematous, erythemous leading to ulcers. Can lead to stroke/neuro injury so CLOSE NEURO MONITORING. Acute chest (pulm vessels)- EMERGENCY. Mimics pneumonia - cough, fever, chest pain, tachypnea, dyspnea, resp distress. Coronary vessels/cardiac muscle. Spleen becomes fibrotic and stops working leading to increased risk of infection. Gallstones and hepatomegaly, sometimes liver failure. Infects kidney filtration and ability to concentrate urine (hematuria). Infarcts affect penile circulation; poor circulation to testes and ovaries causing infertility. Retinal ischemia, detachment
Vasoocclusive Crisis during Sickle Cell Disease
Address what is triggering the sickling. Prevent fever, dehydration, hypoxia (MAINTAIN O2 SAT OVER 93). Pain management- warm compresses causing vasodilation. Blood transfusions to get a higher concentration of non-sickling cells. Prophylactic antibiotics, vaccines; infection prevention. Hydration - 1.5X MAINTENANCE. Hydroxyurea. Teach fam urgent s/s- ER vs PCP.
? BMT - unaffected sibling donor; risk vs benefit
Sickle Cell Disease Treatment
Defective beta chains in hemoglobin. Unstable cell membrane; Hgb molecule easily disintegrates and RBCs easily destroyed. SEVERE ANEMIA and futile erythropoiesis (production of RBCs by bone marrow) Hyperplasia of bone marrow leading to skeletal deformities (frontal lobe, maxilla) and osteopenia and osteoporosis from overworking to make RBCs. Affects people of Greek, Italian, Middle Eastern, and Asian descent.
Beta-Thalassemia General
Hemolysis causes free iron and cellular debris to be released into bloodstream (hemochromatosis). Iron debris and absorption cause damage to:
spleen (splenomegaly)
liver (hepatomegaly, cirrhosis, liver failure)
heart (cardiomyopathy) leading cause of death is HF
Endocrine system (deposits in glands)
Common S/s: short stature, bronze/yellow skin
Manifestations and S/S of Beta Thalassemia
Transfusion therapy- frequent replacement of RBCs; counteract iron overload. Weekly, monthly, every few months (CVL needed?). Goal is to maintain Hgb 0-10 g/dL. Chelation- (remove heavy metals from body) - Deferoxamine IV or SQ (Side effets). Splenectomy. Growth hormone replacement. BMT.
Treatment of Beta-Thalassemia
Blood transfusion/reaction protocol; premed? Post-op care; prophylactic antibiotics. Med teaching: home infusions, reactions.
S/s anemia, bleeding, infection, liver failure, decreased cardiac output
Nursing Interventions for Beta-Thalassemia
Complete bone marrow suppression/failure. PANCYTOPENIA- low absent RBCS, WBCs and PLTs. Congenital (Fanconi) or acquired (autoimmune, infection, exposure, radiation, meds)
Aplastic Anemia
Skeletal defects, GU problems, cardiac defects, growth issues, developmental delays
Congenital (Fanconi) Aplastic Anemia S/S
Resembles leukemia: fatigue, fever, infection, bruising/bleeding
Acquired Aplastic Anemia S/S
Immunosuppressive therapy: corticosteroids; ATG/ALG and cyclosporin. BMT/SCT. Matched donor? Graft vs Host disease (GVHD) (rejection) Failure to engraft.
Nursing: infection prevention! (masks, H20, food)
Treatment and Nursing Interventions for Aplastic Anemia
Inherited; deficit in an essential coagulation factor Type A (classic) - factor VIII; most common; recessive Type B (Christmas Disease)- Factor IX, mild moderate or severe.
Hemophilia
Factor replacement- how often depends on severity (weekly, on demand with injury). Monitor PT/PTT. Rest, Ice, Compression, Elevation (RICE) for bleeding. DDAVP for mild-moderate (increases Factor VIII)
Nursing: S/s bleeding (intracranial, GI, renal), Active ROM better than passive; school nurse, dentist; no contact sports; immunizations SQ instead of IM
Treatment and Nursing for Hemophilia
Deficiency of von Willebrand factor. Autosomal dominant.
Von Willebrant Disease General
S/s: Mucosal bleeding, nosebleeds, gums, excessively large bruises, menorrhagia, telangiectasias
S/s of Von Willebrand Disease
Same treament as hemophilia. Monitor PT/PTT, DDAVP, Factor VIII. vWF on demand. ? birth control for menorrhagia
Treatment of Von Willebrand Disease