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Transcript

Diagnostic Confirmation

  • Sodium >90 mEq/L
  • Chloride >40 mEq/L in infants less than 3 mo.
  • Chloride >60 mEq/L greater than 3 mo.

Nutrition

  • 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
  • High-Fowler's
  • Stay up-to-date on immunizations

Cystic Fibrosis

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