- Why Are They Needed?
- Recommended Dosages
- Danger of High Dosages
- Abdominal Problems and generic enzymes (5/97)
Why Are They Needed?
The basic defect in CF which produces abnormal mucus secretions also blocks the ducts and tubules of the pancreas and prohibits the excretion of certain digestive enzymes into the intestines where food is broken down by these enzymes and passed in the form of nutrients into the bloodstream. The destruction of the pancreas while gradual to some extent is probably 90% complete if you have been diagnosed as pancreatic insufficient.
Later in life, usually in adulthood, the islets of Langerhans may be affected which can cause glucose intolerance and diabetes. Enteric pancreatic enzymes are taken to release protease, amylase, and, importantly, lipase into the digestive tract to make up for the non-functioning pancreas. These enzymes help digest, in particular, carbohydrates and fat. Sometimes an acidic stomach may dissolve the enteric coating on enzymes causing the enzymes to be released prematurely (before they arrive at the spot where food needs to be liquefied), however usually taking a stomach antacid like cimetidine corrects this problem.
Pancreatic enzymes are the foundation of good nutritional status and weight gain in CF and has contributed significantly to overall health.
“A survey of the 10 largest CF centers yielded the following enzyme dosing guidelines:
ù 1,000-2,000 Units of lipase/120 ml (4 oz) feeding for infants ù 1,000-2,000 Units of lipase/kg/meal for older children, with « dose for snacks ù 1,000-2,000 Units of lipase/kg/meal for adults, with « dose for snacks ù add H blocker such as cimetidine if malabsorption 2 persists at a lipase dose of over 2,000 Units of lipase/kg/meal ù ask for a GI consultation if malabsorption persists despite addition of an H blocker” 2 (Foregoing quote from “CF Currents AtùHome” edition), Vol.3, No.1, Winter, 1995, page 3; published by McNeil Pharmaceutical, maker of Pancrease MT capsules).
Danger of High Dosages
The recommended dose (Editor’s Note: predates publication cited above) for digestive enzymes is 1500 to 3000 Units of lipase per kilogram of body weight per meal. Doses above 6000 Units of lipase per kilogram meal, (or 4.5 MT-24 capsules per meal) have been associated with colonic stricture: the ascending colon becomes blocked, sometimes requiring a surgical solution. This has occurred predominantly in young males during the warmest summer months, and may be associated with dehydration. A rule of thumb is one MT-24 for each 18 grams, or 162 calories, of fat. For some people with excessive stomach acid, antacid may insure the enteric coated microtablets reach the digestive tract where the enzymes do their stuff.
Brand names: Pancrease; Cotazym
A First-Person Account
As I’m sure you’ve heard repeatedly, the symptoms and meds needed will vary considerably between PWCF. For the longest time, (my wife) and I were very concerned over what we felt was the excessively large enzyme doses prescribed for each meal. (My child) (PWCF) was taking three to four times the enzyme doses listed in the enzyme instructions as “typical”. We were afraid his digestive system was getting worse and worse.
It took us about a year to come to understand that what works for others (especially the mystical “average” PWCF) isn’t necessarily the best for (my child). Right now (my child), who is three years old, takes three Pancrease MT-20 _and_ one Creon-10 before each meal. He also takes Zantac twice daily to reduce stomach acidity, so the coating on the enzymes breaks down properly to release the enzymes. Before starting the Zantac we were using as many as five Pancrease MT-20 and one-to-two Creon-10 with each meal.
(My child)’s diet is consistently a little higher in fat and protein than I would normally plan for myself. We use ADEKs, vitamin-C, and Omega Oil as dietary supplements, and (my child) (surprisingly) loves raw carrots, celery and fresh vegetable juices (so much that we give him extra as rewards instead of sweets). Sometimes, *when* you give the enzymes makes a difference. Because (my child)’s often a picky eater at lunch, we usually give him one Creon-10 and one Pancrease MT-20 in yogurt just before he starts eating, and then give him the additional one or two Pancrease MT-20 during or after his meal (in the same yogurt), depending on how much he ate. Otherwise we give him the whole dose in applesauce a few minutes before he starts eating. For between-meal snacks we usually give one Creon-10 for cookies or a candy bar or a piece of cake. However, we really try to avoid giving him any sweets between meals, because he doesn’t eat well following that. Instead, we give him a very small amount of pretzel, carrot, or celery sticks, or maybe a couple slices of fruit.
According to our CF doc, we need to give (my child) enough enzymes to break down most of the fat he eats, so that his stools are well-formed and not excessively foul-smelling or light-colored. At least once a year we collect samples of (my child)’s stool for several days, and the CF doc does a fat-content analysis to make sure he’s really getting enough enzymes.
Having said all that, there are children with CF close to (my child)’s age that go to the same center and take wildly different enzyme doses. A good friend of ours also has a three year-old with CF who regularly takes five Pancrease MT-20 and one Creon-10. Some others do well on only two Ultrease (these didn’t work well for (my child)). Your doctor should be the best source of advice for your child’s treatment. He/she is (or should be) educated and experienced dealing with a wide variety of CF patients. It also doesn’t hurt to seek the advice of a second doctor if you have doubts about your regular doctor. We did this once, and it help us better understand the dynamics of treatment.
Abdominal Problems and generic enzymes (5/97)
We have a 7 year old daughter with CF. She was born with meconium ilis and perontintis at birth. She is healthy and doing well. Her major problems are digestive. Last August our insurance changed her to the generic “Pancrelipase” by Pecos Pharmaceutical, Languana Hills, Ca. manufactured by Lini Inc. The pharmacist called and informed me of this changed and I agreed to try it. The change occurred because my plan would only pay for generic and there was now a generic available for Pancrease MT16. He called me back to follow up which I really appreciated because it made me very conscious of her reaction to it.
(My child) complained from the time she began taking the medicine that there was “too much fat coming out.” My husband and I began noticing that it looked like poured cooking oil had been poured in the toilet. After about a week of trying this we went to the mall for lunch. (My child) has been taking her enzymes herself since she was small so this was the first time that I had really looked at the pancrelipase. I noticed that each capsule looked different. That evening at dinner I poured out the entire bottle and found that some capsules were half full, some a third, some three quarters, etc.
I immediately showed them to the pharmacist who was shocked. He reported it to a pharmacy reporting group and suggested that I also call, which I did. Since I had a 90 day supply, I returned all the remainder unopened and still sealed from the manufacturer. I called numerous agencies, the insurance company, and the CF clinic. The Doctors were furious and told me to make a lot of noise, take pictures of the capsules, and we talked with our attorney. This was a clear cut case of the insurance company making the decision on changing the drug and bypassing the doctors. Our doctors also called the insurance company and reported the problem. I filled out a MedWatch form that I obtained from the FDA.
To date I don’t know if anything I did made a difference. We have not heard from any of the groups and neither has the pharmacist except to confirm that our reports were received. We began using my husband’s mail order plan for (my child)’s meds because there is no generic requirement and my goal through all of this was to make sure that she was able to get the “real thing.”
(My child) did lose weight during this time. There were several weeks before her next clinic visit and we were able to get some of the pounds back. The doctors were able to do some of the skin fold tests and determine that there had been some rapid weight loss because of a discrepancy in her muscle mass and prior numbers. (I think I have that right.) She really didn’t have a real significant weight gain until early this spring.