ATI Template Cystic Fibrosis

ACTIVE LEARNING TEMPLATE: System Disorder Cystic fibrosis DISORDER/DISEASE PROCESS __________________________________________________________ REVIEW MODULE CHAPTER ___________ Alterations in Health (Diagnosis) Pathophysiology Related to Client Problem ineffective airway clearance, impaired gas exchange, imbalanced nutrition pt inherits a defective copy of the CF gene from each parent resulting in decreased secretion of chloride and increased reabsorption of sodium and water across epithelial cells, resulting in thick, viscous secretions Health Promotion and Disease Prevention Genetic testing should be offered to adults with a positive family history of CF and to partners of people with CF who are planning a pregnancy. ASSESSMENT SAFETY CONSIDERATIONS Risk Factors Expected Findings If both parents are carriers, their risk of having a child with CF is one in four (25%) with each pregnancy. Most common in Caucasians. Laboratory Tests Sweat test Fecal lipid level test Deoxyribonucleic acid testing Liver enzyme test Sputum culture Albumin level test Serum electrolyte levels Arterial blood gas analysis Fecal elastase-1 level productive cough, wheezing, hyperinflation of the lung fields, and obstruction of the airways. pancreatic insufficiency, recurrent abdominal pain, biliary cirrhosis, vitamin deficiencies, recurrent pancreatitis, and weight loss, CF-related diabetes, and genitourinary problems (male and female infertility) Diagnostic Procedures Genetic counseling is an important part of health care for couples at risk. The diagnosis of CF requires a clinical picture consistent with the CF phenotype and laboratory evidence of CFTR dysfunction PATIENT-CENTERED CARE Nursing Care Strategies that promote removal of pulmonary secretions, chest physiotherapy, and breathing exercises. Therapeutic Procedures Chest physiotherapy, nebulization, and breathing exercises, Noninvasive positive-pressure ventilation (bilevel positive airway pressure) High-frequency chest compression via vest. Check for allergies Emphasize the need of up-to-date immunizations and an annual influenza vaccines Complications Medications IV fluids,Antibiotics, Oxygen therapy, as needed Beta-adrenergic agonists such as albuterol sulfate for inhalation therapy and with chest physiotherapy, Inhaled hypertonic saline Mucolytics, Anti-inflammatories, Corticosteroids if pt experiences asthma or allergic bronchopulmonary aspergillosis, pancreatic enzymes such as pancrelipase, Vitamin A, D, E, and K supplements, Cystic fibrosis transmembrane regulator modulators, such as ivacaftor or tezacaftor/ivacaftor (for patients with identified G551D mutation of the CFTR gene) to target the underlying protein defect that causes the disease Client Education Breathing exercises, Reduce risk factors associated with respiratory infections, Early signs and symptoms of respiratory infection and disease progression that indicate the need to notify a primary provider Interprofessional Care Surgery: Gastrostomy tube placement and Lung or liver transplantation Respiratory therapist, Nutritionist, Social Services, Psychiatrist Bronchiectasis, Pneumonia, Atelectasis, Pulmonary hypertension, Pneumothorax, Dehydration, Malnutrition, GERD, Hepatic disease, Diabetes, Arthritis, Biliary disease, Liver failure, Clotting problems, Osteoporosis, Delayed sexual development, Infertility, Electrolyte imbalances, Bowel obstruction, Cardiac arrhythmias Shoc, death