Final Exam Advanced Review

Final Exam Advanced Review - Final exam advanced review 1....

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Final exam advanced review 1. Cystic fibrosis – autosomal recessive disease in Caucasians Cause: gene mutation or dysfunction of the protein CFTR – CF transmembrane conductance regulator Symptoms Increased thick viscous secretions in the lungs, pancreas, liver, intestines, and reproductive tract Increased salt content in sweat In newborns: Delayed growth, failure to thrive, no bowel movements in first 24 to 48 hours of life Belly pain from severe constipation, i ncreased gas, bloating, distended abdomen Nausea and loss of appetite, and weight loss Stools that are pale or clay colored , foul smelling, have mucus, or that float Lungs: Bronchiectasis starting in upper lobes going down Productive cough, wheezing, hyperinflation of lungs, PFT Sinusitis, nasal polyps, clubbing of the digits Bacteria: S. aureus, H. influenzae, P. aeuruginosa colonization Recurrent episodes of pneumonia: fever, increased coughing, SOB, anorexia, more sputum symptoms: Infertility Pancreatic insufficiency, pancreatitis, CF-related diabetes Billiary cirrhosis, abdominal pain, vitamin deficiencies, weight loss Tests Immunoreactive trypsinogen (IRT) test is a standard newborn screening test for CF. Sweat chloride test is more definitive diagnostic test for CF. Chest x-ray or CT scan show progressive obstructive disease of the lungs Diagnosis - 3 aspects must be present: 1. Chronic sinopulmonary disease: cough, sputum production, persistent infections, bronchiectasis, sinusitis, nasal polyps 2. Gastrointestinal tract and nutritional abnormalities: pancreatic insufficiency, meconium ileus, or distal intestinal obstruction syndrome, failure to thrive, chronic malnutrition 3. Male urogenital problems, absence of vas deferens and obstructive azoospermia Treatment Treatment for lung problems includes: Inhaled antibiotics: TOBI (tobramycin), Colistin (colimycin), Garamycin (gentamicin), (Ceptaz, Fortaz) ceftazidime Bronchodilators to help open the airways Dornase alpha (Pulmozyme) enzyme therapy to thin mucus and ease expectoration Hypertonic saline inhalation, azithromycin (Zithromax) Flu vaccine and pneumococcal polysaccharide vaccine ( PPV ) yearly Lung transplant is an option in some cases Oxygen therapy may be needed as lung disease gets worse Aerobic exercise or CPT to thin the mucus, chest percussion Treatment for bowel and nutritional problems may include: A diet high in protein and calories Pancreatic enzymes to help absorb fats and protein Fat-soluble vitamins A, D, E, and K Care should include: Avoiding smoke, dust, dirt, fumes, household chemicals, fireplace smoke, and mold or mildew
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Clearing or bringing up mucus or secretions from the airways 4 times a day. Patients, families, and caregivers must learn about doing chest percussion and postural drainage. Drinking plenty of fluids.
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This note was uploaded on 12/12/2011 for the course NA na taught by Professor Na during the Spring '11 term at Abraham Baldwin Agricultural College.

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Final Exam Advanced Review - Final exam advanced review 1....

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