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
Systemic coagulation (small vessel occlusion) and consequent hemorrhage. Coagulation pathways are activated and exhausted; factors and PLTs consumed leading to bleeding. EMERGENCY
Disseminated Intravascular Coagulation (DIC)
Hypoxia, sepsis, shock, cancer, burns, trauma, obstetric conditions, heat stroke, transfusion reactions.
Causes of Disseminated Intravascular Coagulation (DIC)
Oozing, petechia, gum bleeding, GI bleeding; tachycardia/hypotension
S/S of Disseminated Intravascular Coagulation (DIC)
Monitor bleeding; monitor CBC and coags; maintain tissue perfusion; administer blood products
Nursing Interventions for Disseminated Intravascular Coagulation (DIC)
1% of all cancer cases. Brain tumors leading cause of cancer death in children ages 1-19. Leukemia increased 55% over past 35 y. Higher survival rates. Certain types of cancer “better”, responsive to tx. Arise from embryonic cells, lymphohematopoietic system (blood cells), or CNS; multifactorial: affect tissue, bone marrow. Presents with fatigue, weight loss, bruising, recurrent infections. Delay in diagnosis
Pediatric Cancer
IV, IT oral. Central line (CVL). Toxic- administration protocols. Dosing based on BSA, very accurate weight/height required. Cell-cycle specific or not, most regimens use a combination.
Chemotherapy
Understanding and monitoring side effects. Normal blood count values. S/s of infection. CVL care. Psychosocial
Nursing Interventions of Chemotherapy
Daily skin assessments. Sunscreen. Fatigue, N/V, decreased appetite
Nursing Interventions for Radiation
Stem Cell (SCT)/ Bone Marrow (BMT). After chemo and radiation. Autologous- child's own. Allogenic- donor. Nursing: isolation, monitor labs (ANC), infection, GVHD
Transplant
Myelosuppression, mucositis, skin breakdown, neuropathy (feet, jaw, constipation), pain, loss of appetite, hemorrhage cystitis, alopecia, toxicity/organ failure, cardiomyopathy, cognitive/ psychological effects
Adverse Effects of Chemotherapy
Most common; 20% of pedi cancers. Males 1-4y; hispanic kids. 80% survival rate. Prognosis depending on age, WBC. Proliferation of lymphoblasts(immature WBCs that do not function properly). Bone marrow unable to produce RBCs and PLTs leading to anemia, petechiae. Bone pain, lymphadenopathy. CNS s/s.
Acute onset: pale, fatigue, fever, bone pain, bruising, hepatosplenomegaly. CBC, then bone marrow biopsy, LP
Acute Lymphoblastic Leukemia (ALL) General and S/S
3 phases, 2-3 years. Induction- reduce # of lymphoblasts in blood. Consolidation- systemic/CNS chemo (IT). Maintenance- chemo, eliminate residual disease.
Nursing: labs; chemo, side effects; procedures, infection, pain, psychosocial
Acute Lymphoblastic Leukemia (ALL) Treatment and Nursing interventions
11% of childhood leukemias; 15-19y. 60-70% survival rate. Excessive # of myelocytes produced by bone marrow. Depletion of functioning RBCs, WBCs, and PLTs. Presentation similar to ALL and subcutaneous nodules. Bone marrow biopsy/aspiration to distinguish this from ALL. Treatment and nursing same as ALL
Acute Myelogenous Leukemia (AML) General, S/S, Treatment and Nursing
3rd most common cancer in pedi; most common in 15-19y.
Survival rate 90-95%. Presentation same for all ages:
*Swollen, painless, rubbery-feeling lymph nodes in neck/supraclavicular region. May have chest pain, dyspnea and cough (mediastinal mass); fever, wt loss, night sweats.
Treatment= Combo: chemo and low dose radiation
Hodgkin Lymphoma
3rd most common cancer in pedi; most common in 15-19y.
Uncontrolled proliferation of abnormal B or T-lymphocytes. Survival rate more than 80 depending on subtype (4 types). Association with weakened immune system. Acute onset; symptoms r/t site of involvement. Can be EMERGENCY, blocked blood flow, spinal cord compression.
Treatment: chemo
Non-Hodgkin Lymphoma (NHL)
2nd most common type of pedi cancer. Survival rate up to 70%. Progmosis depends on location, malignancy and type. Benign or malignant leading to neurological effects in 50%.
S/s: Increased ICP, headache, N/V, ataxia, vision changes, seizure, asymmetry, weakness.
Brain Tumors General and S/s
Depends on type, size, and location. Surgery: Chemo (cross BBB?), radiation. Clinical trials: monoclonal antibodies, vaccines/immunotherapy.
Nursing: pre-op and post-op care; *NEURO STATUS: LOC pupils, orientation, speech, cranail nerves; * PREVENT INCREASED ICP; meds and side effects, pain control; psychosocial. Lifelong care with multidisciplinary team
Brain Tumors Treatment and Nursing Interventions
Embryonal cells of PNS (often begins in utero); no known cause. Most frequent in kids under 5, more common in males. Most common in medulla and adrenal gland but can be anywhere in the SNS (retroperitoneal area, head, neck, pelvis, chest)
Abdominal swelling, painless, firm, nontender; affected bowel and bladder function.
Metastases- lymph and bone marrow; more common in older children
Neuroblastoma General and S/S
Depends on stage of disease.
Stage 1-2: surgery
Stage 3-4: chemo and radiation
Nursing: pre and post op care; depends on surgical site
Neuroblastoma Treatment and Nursing Interventions
aka Nephroblastoma (in kidneys or abdomen). Most common malignant renal tumor, 2nd most common abdominal tumor. Usually 1 kidney, 10% bilateral. Most cases diagnosed at 2-3y. Survival rate 90%, long term comps
Wilms Tumor General
ABDOMINAL MASS; flank pain, hematuria, fever, hypertension. Pain? Compression of organ? do NOT papate abdomen if suspected; can rupture
Wilms Tumor S/s
Prompt surgery (24-48 hours) (nephrectomy) ? stage: additional chemo and radiation Nursing: Pre-op- Careful handling! (diaper, bathing) * SIGNAGE Postop nephrectomy care: Monitor kidney function, I and O, BP, electrolytes, edema. Incision site/infection, pain, C&DB/IS. Educate re: 1 kidney- oliguria, edema
Wilms Tumor Treatment and Nursing Intervention
Malignant, 1 in 18000. Children under 5y; 95% 5-year survival rate.
Inherited vs sporadic -
Sporadic = genetic mutation, always UNILATERAL
Inherited = UNILATERAL (70%) or bilateral
Most common presenting sign = Leukoria - “cat’s eye” also strabismus
Preserve eye/vision
Retinoblastoma General and S/s
Depends on size and location: intra-arterial and intra-vitreal chemo; systemic chemo if metastatic; removal of eye if unresponsive and recurrent.
Nursing: post-op care; pain management; frequent follow ups in 1st 6 months; prosthetic eye?; protection of remaining eye; genetic counseling when older
Retinoblastoma Treatment and Nursing Interventions
Bone tumor; common with adolescent growth spurt (age 15y. peak). 15-20% with mets to lungs. Pain; swelling, limp, random fracture. Delayed diagnosis due to s/s attributed to sports/injuries.
Osteosarcoma General and S/S
Chemo before surgical resection (increase chance of saving limb) Amputation? (chemo) rotationplasty?
Prognosis depends on sites of mets, response to chemo, complete resection?
Nursing: phantom limb pain; body image; stump care, assistive devices, prosthetic education
Osteosarcoma Treatment and Nursing
Rarer than osteosarcoma. Common in bone (pelvis, spine, chest wall) but can be extra-skeletal. Age~ 15y, less than 30; wider age range than osteosarcoma
Presentation similar to osteosarcoma + fever, weight loss, fatigue. Mets to lungs, bones, marrow.
Ewing Sarcoma General and S/s
Biopsy; combo chemo for 14 days, then resection, then chemo again - more intense than osteosarcoma. Avoid weight bearing until treatment complete. Radiation if incomplete resection. ? Amputation.
Nursing: similar to osteosarcoma
Ewing Sarcoma Treatment and Nursing Interventions
Biopsy; combo chemo for 14 days, then resection, then chemo again - more intense than osteosarcoma. Avoid weight bearing until treatment complete. Radiation if incomplete resection. ? Amputation.
Nursing: similar to osteosarcoma
Ewing Sarcoma Treatment and Nursing Interventions
Muscle/connective tissue (nerve, blood vessels) head, neck, GU tract, extremities, orbit of eye. 3% of pedi cancers. Children under 10. Survival 70% under 15y. 45% over 15y.
S/s: (pain, mass) caused by organ displacement or obstruction
Rhabdomyosarcoma General and S/S
Surgery, chemo, radiation (depends on stage, histology and respectability)
Nursing: post op care; chemo/radiation interventions;
Depends on site! Head/neck/larynx: PATENT AIRWAY; monitor for resp distress
Bladder: good u/o, no hematuria
Eye: vision
Rhabdomyosarcoma Treatment and Nursing Interventions
Type of Oncologic Emergency
Malignant cells break down leading to severe metabolic disturbances:
Hyperkalemia, hypocalcemia, hyperuricemia
Prehydration before chemo, allopurinol (lower uric acid)
Tumor Lysis Syndrome
Oncologic Emergency
Mediastinal mass compresses the SVC leading to cough, dyspnea, headache, upper extremity and neck edema
Superior Vena Cava Syndrome
Tumor Lysis Syndrome
Sepsis
Superior Vena Cava Syndrome
Increased Intracranial Pressure
Types of Oncological Emergencies
S/s: Headache, vision changes, vomiting, seizures
Increased Intracranial Pressure (ICP)
cuts/falls/flesh injuries. Contained within tissues- swelling, pain, redness, ecchymosis. In a joint: swollen, painful, limited movement (hemarthrosis); joint deformity/frozen joint.
Hemorrhages
