Health Promotion: Care of the Child With Hematologic and Neoplastic Dysfunction PDF

Health Promotion, Restoration and Maintenance of the Family: Care of The Child With Hematologic and Neoplastic Dysfunction Professor Meghan McCrillis DNP PHNL CNE Quinsigamond Community College NUR201 Sharing...

Health Promotion, Restoration and Maintenance of the Family: Care of The Child With Hematologic and Neoplastic Dysfunction Professor Meghan McCrillis DNP PHNL CNE Quinsigamond Community College NUR201 Sharing and Copyright Infringement Any handouts or postings related to course content is the intellectual property of QCC faculty and cannot be shared by any means to other students or outside entities. Hematologic Disorders Lead Poisoning Sickle Cell Anemia Hemophilia 2 Lead Poisoning Lead Exposure  Typically by hand-to-mouth or lead dust in the environment  Some cultures use medicinal remedies that contain lead  In 1978 lead in paint was banned  Clinical Manifestations  Interferes with the hematologic, renal & neurologic system  Nausea, abdominal pain, anorexia, anemia, glycosuria, proteinuria, ketonuria, ↓vitamin D, distractibility, hyperactivity, intellectual deficits & lead encephalopathy (most serious effect) 3 Therapeutic Management  Blood lead levels – specific actions of therapy depending on lead levels  Chelating agents  Given over several course of treatment  Binds with lead to remove it but does not alter the effects of lead encephalopathy  Monitor renal, hepatic & hematological parameters related to side effects  Ask the right questions and provide anticipatory guidance  Toy safety https://www.cpsc.gov/safety-education/safety-guides/toys 4 Sickle Cell Disease  Most common types  Hemoglobin SS disease (Sickle cell anemia)  Most common form  Hemoglobin SC disease  Hemoglobin Sickle-β- thalassemia 5 Sickle Cell Anemia  One of the most common genetic diseases worldwide  A severe chronic disease that occurs in 2000 newborns yearly in the US.  Affects one out of every 500 Black or African American births  Affects one out of every 36,000 Hispanic American births  Followed by people of Mediterranean, Middle Eastern and Indian decent.  40% incidence in Africans native to West Africa 6  Autosomal Etiology recessive disorder  Sickle cell trait/carrier = Hgb A (normal adult hemoglobin) + Hgb S (sickle hemoglobin)  If both parents have the trait = 25% chance of producing a child with SCA  25% will have normal hemoglobin  50% chance of producing a child with the trait and will be a carrier.  Sickle cell disease = predominantly have Hgb SS 7 When is SCA detected?  Newborns with SCA are asymptomatic until later in infancy  Presence of fetal hemoglobin (60-80% of Hgb F) protects the infant.  Studies have revealed that Hgb F usually disappears from red blood of infants after about 6 months  Clinical manifestations occur when normal adult hemoglobin (Hgb A) is partly or completely replaced by abnormal sickle hemoglobin (Hgb S). 8  SCA results in…….  The rigid sickle-shaped RBC’s become entangled with one another and cause of the microcirculation and increase destruction of RBC’s.  Causing a decrease in oxygen- carrying capacity  Every organ can be effected by vaso-occlusion causing local hypoxia, tissue ischemia and cellular death.  Sickled cells increase the viscosity in the area where they are involved in the microcirculation. 9 Elongated RBC with a shortened life span 10 Clinical manifestations vary greatly in severity and frequency. 11 Clinical Signs of Sickle Cell Crisis  General  Sickle cell crisis is a period of exacerbation of disease process  Symptoms vary greatly in severity & frequency  Increase risk for sepsis  Chronic anemia  Possible growth retardation  Vasoocclusive crisis  Ischemia causing mild to severe pain in the involved area  Sequestration crisis  Pooling of large amounts of blood in the spleen leading to circulatory collapse due to blood volume 12  Clinical Signs of Sickle Cell Crisis Aplastic crisis ↓ RBC production → profound anemia Hyperhemolytic crisis ↑ RBC destruction → anemia, jaundice & reticulocytosis Acute Chest Syndrome Clinically similar to pneumonia → pulmonary infiltrate, chest pain, fever, cough, tachypnea, wheezing & hypoxia  Cerebrovascular accident Occluded blood vessels in the brain causing strokes and variable degrees of neurologic impairment 13  Diagnostic Evaluation  Mandatory newborn screening in all 50 states  Sickle-turbidity fingerstick test (Sickledex) with results in 3 minutes  If Sickledex test is positive, Hgb electrophoresis is necessary to determine if the child has trait or disease. This test confirms the diagnosis.  The earlier detection the better  Teach parents how to administer prophylactic antibiotics and signs of infection  Compliance is of the utmost importance  Goal of therapy  Prevent sickling  Treat medical emergencies for crises 14  Supportive & symptomatic treatment for Crisis  Rest- minimize oxygen use  Maintain hydration- hemodilution to reduce viscosity of blood  Electrolyte replacement- prevent hypoxia & metabolic acidosis  Analgesics- usually undermedicated  Fever- Tylenol or Motrin if not contraindicated. Do not use a cooling blanket. 1  Supportive & symptomatic treatment for Crisis (cont’) o Anemia- Blood transfusion o Antibiotics- to treat infection and oral penicillin prophylactic o Vaccines- Pneumococcal, meningococcal & flu o Oxygen- prevent severe hypoxia o Partial Exchange RBC transfusion- out with the bad & in with the good o Splenectomy –life-threatening splenic sequestration 16  Pain Management  Mild to moderate pain  Ibuprofen, ketorolac (Toradol), acetaminophen or codeine  Severe pain  Narcotics are usually the drug of choice for severe pain & administered ATC (PO or IV)  Crisis-IV route is recommended  PCA administration gives patient control  Meperidine (Demerol), a CNS stimulant, is contraindicated  Produces anxiety, tremors & seizures with repetitive dosing 17  Hemophilia  Deficiency of coagulation factor (proteins) in blood  X-linked hereditary autosomal recessive disorder  Transmitted by carrier mothers to their sons.  Affects 1 in 5000 males Factor VIII deficiency (hemophilia A) 80% of all hemophilia cases Factor IX deficiency (hemophilia B) Factor XI deficiency (hemophilia C) 18 Patterns of Genetic Inheritance  Females with one normal X chromosome and one mutated X chromosome generally do not manifest the disorder but are carriers and can pass on hemophilia.  On the other hand, since a male only has one X chromosome, if it is mutated he has no normal copy and will develop the disorder. 19  Diagnosis  Treatment  Hx of bleeding episodes  Factor VIII injection  Evidence of x-linked inheritance  Antihemophilic  Labs factor (Bioclate).  PTT  Assay procedure to detect deficiency type  DNA testing for carrier detection in families in which female offspring may have inherited the trait 20 Clinical Manifestations  Prolonged bleeding to affected areas  Traumatic hemorrhage  Excessive bruising  Subcutaneous & intramuscular hemorrhages  Hemarthrosis  Knees, ankles & elbows  Hematomas  Hematuria 21 Neoplastic Disorders Rhabdomyosarcoma Retinoblastoma Wilms Tumor Neuroblastoma 22 Rhabdomyosarcoma  Softtissue tumor that usually arises from cells that would ordinarily form striated muscle.  Tumoris commonly located in the head, neck, GU tract and extremities.  Tumoris highly malignant and cells spread via the venous or lymphatic system  Diagnosis  Age 2-10 years, often times presents with an asymptomatic mass  Definitive diagnosis = primary tumor biopsy  CT, MRI, bone marrow aspiration, bone scan and skeletal survey.  There are 4 stages  Symptoms determined by involved site 23  Rhabdomyosarcoma  Treatment  Surgical resection of tumor  Treatment determined by the site and size of the tumor with radiation and chemotherapy.  Complications  Highly malignant tumor with metastasis  Lung (common site), bone, bone marrow and the CNS 24 Retinoblastoma  Highly malignant tumor of the retina  Dx by age 3 with a survival rate of 90%  Nonhereditary  The mutated cell occurs early in life and occurs in one eye (unilateral)  Hereditary  All the cells in the body have a mutated defective RB1gene  It can either develop after conception while in the womb (75%) or can be inherited from one parent (25%, autosomal dominant)  Unilateral or bilateral Cancer.org 25  Retinoblastoma Diagnosis Diagnostics include: ophthalmologic exam, CT, MRI, US, LP and bone aspiration with metastasis. Often times present with cat’s eye reflex (eye has a white appearance), strabismus, orbital inflammation, vomiting or headache. Treatment Staging will determine treatment A combination of radiation, chemotherapy, laser surgery and cryotherapy 26  Nursing Management Postop care Assess drainage, change  Anticipatory Guidance dressing Protect vision in remaining eye Monitor side effects of chemo Routine eye Follow-up care Eye exams Q3-6 months until age 6 then Prosthetic eye care if needed yearly exams Complications Education Retinaldetachment Genetic counseling (enucleation needed), exams metastasis to lymph nodes, Safety goggles during sports bone, bone marrow & liver Secondary tumors caused by No contact sports with 1 eye treatment Treat eye infections 27 Wilms Tumor (nephroblastoma)  Most common malignant renal & solid intraabdominal tumor of childhood  Peak age is 2 to 3 yrs, slightly > in boys  1-2.5% have a familial origin  Usually only unilateral with 6% bilateral involvement  Maymetastasize, commonly to perirenal tissue, liver, diaphragm, lungs, abdominal muscles and lymph nodes but metastasis is rare 28 Diagnostic Evaluation and S & S  Diagnosis  Thorough history & physical exam  Assess for signs of malignancy: weight loss, liver & spleen size, anemia & lymphadenopathy  X-ray, abdominal US, CT and hematologic studies, bone marrow aspiration to r/o metastasis S &S  Abdominal mass  Abdominal pain  Fatigue, malaise, hypertension, vomiting, anorexia, constipation  With metastasis to lung: dyspnea, cough, SOB & chest pain on occasion 29 Stage I: The tumor is 7 cm or Stage II: The tumor is smaller and is in the kidney larger than 7 cm, extends only. It has not invaded the beyond the kidney but is lymph nodes or distant organs completely resected. of the body & is completely resected. Stage III: Tumor is confined to abdomen Stage IV: Mets to lung, liver, bone & brain Stage V: Bilateral kidney disease 30 Therapeutic Management  Radiation and chemo may be  Nursing Considerations administered before or after  ***DO NOT palpate surgery abdomen: may cause dissemination of cancer  cells to adjacent & Surgical Intervention: ASAP after diagnosis distant sites causing  metastasis Nephrectomy  Tumor must remain intact or may seed cancer cells throughout the  Post-op care same as abdomen, lymph system & for abdominal surgery systemically  Contralateral kidney is inspected  Biopsy of lymph nodes  Teaching: avoid contact  Any involved structures are sports & S & S of GU removed dysfunction 31 Prognosis  StageI & II have a 90% chance of cure with multimodal therapy  Wilms tumor may recur, especially in the lungs  Bothchemo & radiation can induce secondary malignancies, usually in the areas that have been irradiated 32 Neuroblastoma  Most common malignant extracranial solid tumor of childhood  Majority are diagnosed before 5 yrs of age.  Majority of tumors develop in the abdomen along the adrenal gland or retroperitoneal sympathetic chain  Other sites: head, neck, chest, pelvis  “Silent Tumor” – In many cases diagnosis is made after metastasis occurs  Prognosis  Survival rates is about 40-90% depending on staging  In general, younger at diagnosis (< 12 months old) and children with stage I disease = better prognosis 33 Neuroblastoma  Diagnostics  Management  Objective is to locate  Clinical staging to primary site & sites of establish treatment plan metastasis  Systems assessment based on location of tumor  Surgery to remove  tumor and obtain Signs and symptoms biopsies depend on location and stage of disease  Radiologic studies, skeletal  Radiation & survey, bone CT scan, bone chemotherapy with all stages with exception marrow aspiration with of stage 1 which can be biopsy completely resected 34 Dr. Seuss Inspiration of the Day D R. M E G H A N M C C R I L L I S N U R 2 0 1 P E D I AT R I C S 35