High calorie, high fats (fruits, vegetables, whole grains)
Increase fluid intake
Vitamin supplements (A, D, E, K)
Pancreatic enzyme replacement
Administration of extra salt may be recommended
At risk for hyponatremia & dehydration with exercise
Teens at risk for osteoporosis
Increase calcium and vit. D
Pancreatic enzymes with each meal to aid in absorption of fats, proteins, and vitamins
Diagnostics
Stool analysis for presence of fat and enzymes
Pancreatic enzyme test: lack of trypsin
Sweat test: measures amount of salt in sweat (normal value 40-70 mEq)
Genetic testing confirms diagnosis
Chest physical therapy (CPT) involves percussion, vibration, & deep breathing exercises- aims to loosen mucus from lungs
Surgery to remove nasal polyps to improve breathing
Pulmonary Function Test (PFT)
Nursing Management
high-Fowler's
Chest physiotherapy (consult with respiratory therapist)
Sweat chloride test is most definitive for CF
Monitor for malnourishment due to decreased digestive enzymes
Monitor glucose levels due to pancreatic duct obstruction
Increase sodium in moderation
Vitamins (A, E, D, K)
What is Cystic Fibrosis?
Some things to consider:
Cystic fibrosis is an inherited disorder that affects the secretory glands, particularly the glands that are responsible for secreting mucus, digestive enzymens, and sweat. In turn, CF affects the lungs and sinuses, as well as the digestive organs, including the pancreas, intestines, and liver.
Most common in caucasians
Stems from a mutation of the CFTR gene, which causes absence or dysfunction of the CFTR protein.
CFTR is central to the movement of chloride into and out of the body cells
Also affects movement of salt and water into and out of cells
Ensure pt. is staying up-to-date on immunizations (especially influenza and pneumococcal vaccines)
Do not administer cough suppressants to pts. with CF. Coughing is essential method of clearing mucus from the airways, and if the cough is suppressed, pt. will be at risk for airway obstruction due to mucus in airway
Clinical Manifestations
Nursing Management
Can delay growth
Increased chloride levels
Chronic cough and susceptibility to infection
Chronic sinusitis may occur as a result of accumulation of viscous nasal secretions that become infected and produce inflammation
Development of scar tissue (fibrosis) and cyst formation in the lungs
Pneumothorax also may develop during later stages of the disease
Obstruction of the pancreatic duct may impair the production of pancreatic enzymes, which are necessary for food digestion
Steatorrhea (fatty stools)
Pancreatic duct obstruction may interfer with insulin production, causing impaired blood glucose control
Nutritional deficiencies due to obstruction of pancreatic duct
Meconium ileus at birth manifested with abdominal distentio, vomiting, inability to pass stools
Promoting removal of thick sputum through postural drainage, chest physiotherapy
Bronchodilators, Multics
Pancreatic enzymes with each meal to aid in aborption of fats, proteins, vitamins
Increase calcium, protein, liberal amount of sodium to replace sodium lost through sweat
Increase liquids
Stress importance of preventing respiratory infections by avoiding people with colds/flu