Cystic fibrosis

Definition

Cystic fibrosis (CF) is a genetic disorder affecting the exocrine glands, leading to the production of abnormally thick mucus. This impacts various organs, primarily the lungs and digestive system, causing severe respiratory and nutritional challenges.

5 Must Know Facts For Your Next Test

1. CF patients often require high-calorie diets with increased fat intake to meet energy needs.
2. Malabsorption of nutrients is common in CF due to pancreatic enzyme insufficiency.
3. Vitamin supplementation, especially fat-soluble vitamins (A, D, E, K), is crucial for CF patients.
4. Pulmonary health in CF can be supported by adequate hydration and proper nutrition to maintain mucus clearance.
5. Routine nutritional assessments are essential for monitoring growth and development in pediatric CF patients.

Review Questions

• Why are high-calorie diets important for individuals with cystic fibrosis?
• How does cystic fibrosis affect nutrient absorption in the digestive system?
• Which vitamins are particularly important to supplement in cystic fibrosis patients?

Related terms

Pancreatic Enzyme Replacement Therapy (PERT): A treatment involving enzymes taken with meals to aid digestion in CF patients.

Mucolytics: Medications that thin mucus, making it easier to clear from the respiratory tract.

Sweat Test: A diagnostic test measuring salt levels in sweat, commonly used to diagnose cystic fibrosis.