Respi CF

2. CYSTIC FIBROSIS • A hereditary disease that affects the lungs and digestive system. • The body produces thick and sticky mucus that can clog the lungs and obstruct the pancreas • A person must inherit a defective copy of the CF gene (one from each parent) to have CF. • This multisystem genetic disease is usually diagnosed in infancy or early childhood but may be diagnosed later in life. • Respiratory symptoms are frequently the major manifestation of CF when they are diagnosed later in life. • A hereditary disease that affects the lungs and digestive system. • The body produces thick and sticky mucus that can clog the lungs and obstruct the pancreas • A person must inherit a defective copy of the CF gene € (one from each parent) to have CF. CYSTIC FIBROSIS – PATHOPHYSIOLOGY • CF is caused by mutations or dysfunction in the “protein cystic fibrosis transmembrane conductance regulator” (CFTR), which normally transports “chloride ions” across epithelial cell membranes. • Gene mutations affect the transport of these ions, leading to CF, which is characterized by thick, viscous secretions in the lungs, pancreas, liver, intestine, and reproductive tract as well as increased salt content in sweat gland secretions. • More than 1500 mutations of the CFTR gene have been identified, thus creating multiple variations in the presentation and progression of the disease. • The hallmark pathology of CF is bronchial mucus plugging, inflammation, and eventual bronchiectasis. • Commonly, bronchiectasis begins in the upper lobes and progresses to involve all lobes. CYSTIC FIBROSIS – CLINICAL MANIFESTATIONS • The pulmonary manifestations of CF include a productive cough, wheezing, hyperinflation of the lung fields on chest x-ray, and pulmonary function test results consistent with obstructive disease of the airways. • Colonization of the airways with pathogenic bacteria usually occurs early in life. • Staphylococcus aureus and H. influenzae are common organisms during early childhood. • Upper respiratory manifestations of the disease include sinusitis and nasal polyps. • Nonpulmonary manifestations include gastrointestinal problems (e.g., pancreatic insufficiency, recurrent abdominal pain biliary cirrhosis, vitamin deficiencies, recurrent pancreatitis, weight loss), CP-related diabetes, genitourinary problems (male and female infertility, and “clubbing of the digits” (fingers and foes). CYSTIC FIBROSIS – ASSESSMENT AND DIAGNOSTIC FINDINGS • The diagnosis of CF requires a clinical picture consistent with the CF phenotype and laboratory evidence of CFTR dysfunction with key aspects: 1. Chronic sinopulmonary disease as manifested by chronic cough and sputum production, persistent infection consistent with typical CF pathogens, and x-ray evidence of bronchiectasis and chronic sinusitis, often with nasal polyps 2. Gastrointestinal tract and nutritional abnormalities (pancreatic insufficiency, meconium ileus or distal intestinal obstruction syndrome, failure to thrive or chronic malnutrition) 3. Male urogenital problems as manifested by the congenital bilateral absence of the vas deferens and obstructive azoospermia. CYSTIC FIBROSIS – MEDICAL MANAGEMENT • CF requires both acute and chronic therapy. • Because chronic bacterial infection of the airways occurs in CF, control of infections is essential to treatment. • For acute airway exacerbations, aggressive therapy involves airway clearance and antibiotics based on the results of sputum cultures (three to four times per day). • Most patients are colonized with Panine, and antibiotics are usually given for 14 to 21 days. • For chronic disease, treatment includes airway clearance measures mucolytics dornase alpha, nebulized antibiotics, oral antibiotics, inhaled hypertonic saline, nutritional support, and exercise. • Airway clearance techniques include manual postural drainage and chest physical therapy. • Dornase alpha is a nebulized medication administered to degrade a large amount of deoxyribonucleic acid (DNA) that accumulates within CF mucus • Nebulized antibiotics include tobramycin, colistin, gentamicin, and ceftazidime. • Acute infections are treated with a variety of antibiotics. Antibiotics such as macrolide azithromycin may also be used. • Approximately 90% of patients with CF have pancreatic marine insufficiency and require oral pancreatic enzyme supplementation with meals. • Supplements of fat-soluble vitamins. A.D.E and K are necessary • Supplemental oxygen is used to treat progressive hypoxemia that occurs with CF. CYSTIC FIBROSIS – NURSING MANAGEMENT • Nursing care includes helping patients manage pulmonary symptoms and prevent complication of CF • Specific measures include strategies that promote removal of pulmonary secretions, Chest physiotherapy (Including postural drainage, chest percussion, and vibration) and breathing exercises which are implemented and taught to the patient and family when the patient is very young. • The patient just reminded of the need to reduce risk factors associated with respiratory infection (e.g. Exposure to crowds or people with known infection) • The patient is taught the early signs and symptoms of respiratory infection and this is progression that indicates the need to notify a primary health provider. • Nurses emphasize the importance of adequate fluid and dietary intake to promote the removal of secretions and to ensure an adequate nutritional status. • As with any chronic disease, Palliative care and end of life and concerns need to be addressed with the patient when warranted. • For the patient whose disease is progressing and who is developing increasing hypoxemia, Preferences for end-of-life care should be discussed, documented and honored. • Patience and family members require support as they face a shortened lifespan and an uncertain future