Cystic Fibrosis

T/F: Cystic Fibrosis is an inherited disease caused by defective CFTR protein within the epithelial cells

• True

• False

Patients with cystic fibrosis will have what type of secretions (sweat, tears, saliva, mucous)?

• Thin

• Thick, Sticky

Cystic Fibrosis leads to Multiple Organ Involvement. The integumentary presentation will show

• Salty skin

• Dehydration

T/F: Patients with Cystic Fibrosis require higher caloric intake to prevent malnutrition.

• True

• False

How should pancreatic enzymes (amylase, protease, lipase) be administered?

• On an empty stomach

• With a meal

CF - Related Diabetes (CFRD). Primary defect is insulin insufficiency. Mucus leads to scarring in pancreas but still able to make some insulin. What is the primary treatment for CFRD?

• Metformin

• Non-Insulin Therapy

• Insulin

T/F: Give bronchodilator (albuterol) after hypertonic saline to prevent bronchospasm

• True

• False

T/F: High-dose ibuprofen has been demonstrated to slow disease progression

• True

• False

Select the antibiotic that is NOT commonly used in treatment of CF bacterial infections.

• Azithromycin

• Amoxicillin

• Tobramycin

• Aztreonam

Ivacaftor (Kalydeco) is a CFTR potentiator should be taken

• on an empty stomach

• with fat-containing food