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:

• Pancreatic enzymes, which are synthesized and secreted by pancreatic acinar cells, and
• Bicarbonate, which is secreted by epithelial cells of small pancreatic ducts.

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.

• Proteases are responsible for protein digestion. Proteins are in fact chains of amino acids linked by peptide bonds. Trypsin and chymotrypsin are two major proteases that are stored in secretory vesicles as inactive proenzymes trypsinogen and chymotrypsinogen. After being converted to their active form, trypsin and chymotrypsin function to break down the peptid bonds that link amino acids together.
• Lipase is in charge of hydrolyzing or breaking down triglyceride fat molecules so they can be absorbed in the intestines as monoglyceride and free fatty acids.
  There must also be an ample supply of bile salts in the intestinal lumen for lipase to effectively break down triglycerides to monoglycerides. Therefore, normal digestion and fat absorption depends largely on normal amounts of pancreatic and liver secretions.
• Amylase functions in the conversion of starch to disaccharide and trisaccharide which are then further converted to glucose. Easily put, it converts complex sugars into simple sugars. Amylase is also available in the saliva, enabling digestion to start in the mouth.
• The pancreas also produces several other enzymes needed for normal digestion and nutrient absorption such as gelatinase, deoxyribonuclease, ribonuclease and elastase.

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:

• Steatorrhea or loose stools containing unabsorbed fats, and
• Foul smelling stools containing undigested protein.

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.