Summer 2000 ~ Newsletter
How To Tell When Your Child Is Sick
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CF and the Digestive System
by Cary Cavander, MD
The primary effect of Cystic Fibrosis in the gastrointestinal (GI) tract is thick secretions. These thick secretions occur in the pancreas, liver, gallbladder and their passageways. The thick secretions in the pancreas result in pancreatic enzyme insufficiency. The normal healthy pancreas supplies the stomach and small intestines with the enzymes needed to properly digest the food we eat. In CF, thick secretions block those enzymes. Without them, the calories and nutrients from the proteins, carbohydrates, and fats we eat cannot be fully absorbed into the body and used. This is what is meant by the term "malabsorption".
Malabsorption causes failure to thrive or poor growth. Unabsorbed calories and nutrients are wasted in the stool. As a result, the stools are loose, bulky, and foul smelling. Oral enzymes replacement is the main treatment for malabsorption. They should be given before eating so that enzymes are immediately available to act on what is taken in the diet. The dose of enzymes should be between 2000 and 2500 units of lipase per kilogram of body weight. With fewer enzymes that than, malabsorption may occur, especially with fatty foods. With more enzymes than that, scarring of the colon may occur. This scarring develops over a period of months and years. So taking extra enzymes occasionally is OK, but consistently taking to many could cause serious problems to the colon.
As children begin reaching school age, we tend to see a problem called distal ileal obstruction syndrome (DIOS). This used to be called the meconium ileus equivalent. Thick secretions slow motility of the GI tract. If you accumulate thick secretions in the end part of the small intestine, blockage and bloating can result in constipation. Vomiting and dehydration can also occur. Oral enzyme therapy helps to prevent this. Increased activity and inadequate fluid intake can also cause secretions to become even thicker, which contributes to the distal ileal obstruction. It is important to drink plenty of fluids, especially in the summer, when you are outside and active. Taking appropriate amounts of enzymes and paying attention to your normal bowel pattern (normally people have one bowel movement every other day, up to three times per day) is also important. Having less that one bowel movement every other day could lead to obstruction and more than three could cause malabsorption. Drinking plenty of fluids and taking the right amount of enzymes should prevent these problems.
Cystic Fibrosis can also cause gastro-esophogeal reflux disease (GERD), which tends to aggravate the respiratory status and liver disease. Sluggish liver secretions may cause liver scarring and sluggish bile secretions may lead to gall stones. Actigall is a bile salt that enhances liver secretions and protects the liver from scarring.
The treatment of Cystic Fibrosis is a lifelong process and is somewhat like training for a gold medal. You don't just show up and expect to win. It takes lot of practice and lots of involvement from coaches, teammates, and fans to reach the goal. Education is very important. The more you know about your body and your disease the better you will be able to detect problems before they become significant and be able to get help. Despite that, trouble may still come in one form or another. Follow the GI clinic motto, "Be more stubborn than your tummy." Being more stubborn than your cystic fibrosis is important as well since quitting can be tempting. I think kids should be active and involved and not let a label keep them from reaching their goals.