About Cystic Fibrosis

Why Pancreatic Enzymes are absolutely mandatory for optimal growth in Cystic Fibrosis

Cystic fibrosis is a hereditary, autosomal recessive disease that specifically targets the exocrine or mucus glands of the lungs, pancreas, liver, intestines, sinuses and sex organs, thereby causing chronic disability that leads to respiratory and digestive system problems. This disease causes the epithelial cells of the body to produce unusually thick and sticky mucus. The thickened secretions block airway passages and flow of enzymes in the pancreatic ducts. The latter disables proper digestion and absorption of food.

Pancreatic Secretions and Functions

Normally, the pancreas, which has an endocrine function, also acts as an exocrine gland that secretes pancreatic juices containing digestive enzymes which aid in the digestion and absorption of food nutrients in the body.

Two secretory products, essential for proper digestion are:

The first ones function to hydrolyze or convert food molecules to simpler forms for faster absorption. Bicarbonate and water are essential for neutralizing the acid that comes from the stomach going to the small intestines.

Digestive enzymes needed for efficient digestion are protease, lipase and amylase.

The cystic fibrosis problem in the pancreas

In CF, pancreatic enzymes cannot reach the small intestines due to thick and sticky mucus. The food is not properly broken down in the intestines and thus leaves the body undigested. This eventually leads to malabsorption and results to nutrient deficits and the occurrence of:

The patient will have abdominal discomforts and will show poor growth rate, which is known to have a negative outcome on his life.

Pancreatic enzyme replacement therapy

Fortunately, digestive enzymes pills are prescribed to patients with cystic fibrosis to improve digestion and absorption of proteins, fats and carbohydrates. Examples of enzyme replacement pills include Creon, Pancrease, Cotazym, Ultrase and Pancreacarb. These are available in tablet, powder or capsule form.

Because stomach secretions can inactivate pancreatic enzymes, tablets are usually utilized with enteric-coating. Enzyme supplements and extracts are generally well-tolerated by cystic fibrosis patients and do not have any significant side-effects.

Disclaimer: This website provides general information about cystic fibrosis and in no means should be taken as a medical or health advice. Please consult your doctor before acting on any of its information.