NCLEX 준비 - Cystic Fibrosis

안녕하세요 예비 미국 간호사 선생님들
이번에는 Cystic Fibrosis 에 대해 공부해봅시다.
이 문제는 보통 passing level입니다.

Cystic Fibrosis란 thickened secretion due to impaired Cl- and Na+ channel regulation that causes exocrine gland dysfunction.

Rule #1. Respiratory care

- Potential complication:

• Chronic hypoxemia
• Frequent respiratory infection

- Teaching

• -Manual chest physiotherapy
  • Bronchial hygiene
•  Physical activity is encouraged to help thin secretion and remove them from airway

Rule #2. GI care

- Vitamin deficiencies

• Thickened secretion obstruction the release of pancreatic enzyme, causing GI malabsorption of fat-soluble vitamins (A,E,D,K)
• GI malabsorption causing weight loss

- Teaching

• Give pancreatic enzyme with meals and snacks (GI)
• Increase salt and fluid intake when playing outside during hot weather (GI)
  • GI malabsorption of Cl- and Na+
• High calories diet in protein and fat
  • Damaged tissue recover

Rule #3. Diet
- NO Chew any thing !!

• can cause oral mucosa irritation

- Ok to open the capsule and sprinkle the Meds on applesauce

• Sprinkle with acidic substance such as apple sauce
• Do not take the capsule with milk 
  • 응고 가능성

- High protein, High calories, and High fat

• Defective Protein responsible for transferring to Na+ and Cl-