Nsg: Cystic Fibrosis

Question 0

Sympstoms of Cystic Fibrosis?

Answer 0

1) Earliest:Meconium ileum (Blocked thick mucus in the ileum present by no BM after birth)
2) Sweat Cholride concentration >60 mEq/L

Question 1

What is Cystic fibrosis?

Answer 1

An recessive autosomal disease.
Several clinical features:
-increased mucus viscosity
-elevation of sweat electrolytes. (Chloride)

Question 2

Cystic Fibrosis Dx Tests

Answer 2

1) Sweat Chloride Test (Test sweat for incr. Chloride >60mEq/L) Stimulate sweat with a device, collect sweat on filer paper, measure sweat electrolytes of two samples to ensure accurate results.
2) DNA testing for defective genes
3) Newborn screening (Benefit: Early nutritional intervention)

Question 3

CF pulmonary tests

Answer 3

1) Pulmonary Function Test ( to get baseline at persons best)
2) Chest Xray (to spot any anatomical changes from scarring from any infection) (Baseline)

Question 4

CF Medications

Answer 4

1) *Bronchodilators: To dilate the bronchioles for easier breathing b/c of obstruction from mucus.
2) Mucolytics: To decrease the amount of mucus in the airways.
3) *Antibiotics: To prevent/treat infection in the respiraotory system
4) Corticosteroids: To reduce inflammation
5) *CREON: Pancreatic enzymes to help digest food and help the absorption of nutrients.
6) Fe: Supplement to help increase hemoglobin levels
7) *Vitamins ADEK: Water miscible Supplements to increase vitamin intake.