Homozygous for D508, PI but...


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When I hit my early 20's, I stopped having so much trouble keeping weight on! Now at 43, I havent taken any enzymes in two decades and constantly have to watch my diet and exercise every day to keep my weight from going up too high. All tests show my pancreas is kaput. My doc thinks I've colonized special bacteria to digest for me. Has this happened to anyone else?

btw, no GI pain, normal stool, etc. You would never know I had such GI difficulty as a child and teen.


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Hi there......finally, there is someone a bit like our son. He is 13 and was diagnosed early this year. Knowing what we know now, it is clear that he has been PI pretty much from birth (but we did not know that at the time) and he had fairly typical PI stools and a lot of pain when he was little. We ended up giving him lots of probiotics and eventually were told he could not tolerate gluten, so stopped that. When he was finally diagnosed this year (because of lung issues) as a DDF508 like you, the CF team simply did not believe us that he has NO tummy aches, no frequent or smelly or oily stools etc. They tested his stools and found him to be PI. They tested nothing else. They then told us he has to take enzymes which he does now, but it makes no difference that we could name....we just hope he does digest the fat-soluble vitamins better, but we have no evidence of that yet....and like you he has not had problems with his weight (he was always slight, but did thrive), until the lung problems started. We consulted a naturalist doctor and he suggested Lukas is somehow managing to use a different enzyme for digestion.....a bit like yours is saying about the bacteria. I only know of one other patient a little similar, but you are the first I heard of sounding almost the same! So good to read your post.


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This is so interesting! Imported_Momto2 have you take any probiotics or have any unique aspects/adjustments to your diet? Has anyone shown an interest in testing your gut flora? There is so much research starting to happen regarding the importance of gut biome, and I've heard of fecal bacteria transplants to help people with certain health issues (sounds gross but if it helps it's worth it!). I can't wait to see what the next few years of research bring.


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The study of gut bacteria is getting more and more attention. I was fortunate enough to attend the NACFC this year and one of the speakers spoke about microbiota and the current research and attention that is ongoing. His name was Lucas R. Hoffman, M.D., Ph.D. He's a pediatric pulmonologist and microbiologist. He spoke as a part of the Liver-Gut Axis in CF Session. His segment was titled Intestinal Dysbiosis in CF. I found his information very interesting. It might be interesting for you to reach out to him to tell him your stories.

Best of luck!



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Thanks for that post, Alanna. I will collect the papers of this guy together and have a good reading session next weekend. Finally a lead.....


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WOW! The doctor's theory fits well for the guts of many animals. Mammals are augmented with powerful enzymes that suit our omnivorous diets plus bile to break down fats for further digestion. Our food is digested beginning with chewing, and amylase is introduced as it is mechanically reduced.

We begin to extract nutrients in the stomach as HCL is introduced and more mechanical rubbing reduces food to the consistency of pablum. Being PI doesn't stop bile production and removing the gallbladder just ruins timely delivery. Bile is a waste product of the liver and it keeps flowing as long as we live.

As for being totally PI, I'd confirm that with a pancreatic function test. This is a direct measure of what the pancreas excretes when stimulated by the hormone secretin. I assume that you're diabetic? I've dealt with chronic pancreatitis and pancreatic autodigestion for decades. Most of my pancreas is destroyed (~80%) and still it puts out enzymes. The proof is a rapid increase in pancreatic pain when I eat, or feel hungry.

For some reason I just assume that all CFers consume digestive cultures from prebiotics to probiotics of every type from probiotic yeasts, bacteria and other good microbial symbionts. Normally our large bowel is a very active culture medium. All mammals grow probiotic colonies in the large bowel. Most insects would starve if the gut was sterile, they're that important!

By definition, the large bowel mostly draws excess water from the feces and bacterial colonies break down any undigested proteins. The surface area of the thirty feet of small intestine can be required in order to absorb enough nutrition in order to live. Enzymes kill bacteria and to that end as long as a person is producing or taking enzymes, probiotic cultures won't take hold in the small intestine.

This is purely speculation, but it would appear that a probiotic culture is living in near perfect symbiosis with you. One expects this in the large bowel, it would be more than a little interesting to understand what's going on. It could be a help to many.



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Hi, thanks for all these explanations. All still a bit much for me to "digest" as it were :p. I need to do some more reading. Anyway, in answer to your question or assumption: my son just had a glucose challenge test and continues not to be diabetic or even anywhere near. No problems there for now!
Interesting to read about the function of the large intestine: my son's stool test showed lack of water in the stool and the report says this indicates either constipation (which he did not have) or slow passage of the stool ....and I guess thr latter may be what is happening.