Ch 19 ATI

Question 1

R/F for Cystic Fibrosis

Answer 1

Caucasian Ethnicity
Both parents contain the recessive gene

Question 2

What is CF

Answer 2

The respiratory disorder is obtained from inheriting a mutated gene. Can cause increased sodium and chloride content in sweat, and mechanical obstruction of organs via increased mucous viscosity.

Question 3

Expected findings of CF

Answer 3

Family history
Respiratory failure or growth failure
Meconium ileus at birth manifested as abdominal distension. [Earliest manifestation]

Question 4

Respiratory Findings

Answer 4

Early:

Moderate
Dyspnea
Paroxysmal cough
Atelectasis
Obstructive emphysema

Advanced:
Clubbing
Barrel chest
Multiple episodes of bronchopneumonia

Question 5

GI findings:

Answer 5

Steatorrhea [large greasy stools that smell bad]
Voracious appetite early and loss of appetite late
Failure of weight loss or gain
Delayed growth patterns
Thin arms
Anemia
Reflux

Question 6

Reproductive:

Answer 6

Decreased or absent sperm
Thick cervical mucous

Question 7

Diagnostic tests

Answer 7

Pulmonary Function Tests
Abdominal x-ray
Chest x-ray
Stool analysis
Duodenal analysis

Question 8

Gastrointestinal Management

Answer 8

Give three meals a day with snacks
Provide a well-balanced diet high in protein and calories
Administer pancreatic enzymes within 30 min of eating meals
Administer water-soluble vitamins and vitamins [A, D, E, K]
Encourage adding salt during hot weather

Question 9

Pulmonary Management

Answer 9

Airway Clearance Therapy promotes the expulsion of secretions. Avoid before or after meals.

Question 10

Notable Meds for CF

Answer 10

Dornase alfa, decreases viscosity of mucous