LittleLab4CF
Super Moderator
Welcome to the site and welcome to the club. Late diagnosed CFers seem to have GI dominant issues and for many, it is discovered during a flare that tops all other times symptoms gang up. Fortunately the CF medical community is starting to improve the specialist services needed to address pancreatic and GI CF. For too long the GI tract has been underserved and a talented GI specialist is barely informed on proper enzyme titration, bowel motility and DIOS or Distal Intestinal Obstruction Syndrome or blockages caused by the adhesive, voluminous maldigested feces and swollen, inflamed and sticky intestinal mucosa.
My diagnosis was two positive sweat tests. I was more than a bit thrilled to finally have a diagnosis that actually fit what I had dismissed as a constellation of seriously annoying but manageable health problems. Genetic testing followed and according to my brand new CF specialist, I was just a carrier and didn't have CF.
I didn't have pulmonary CF but by chance the CFTR mutation I have does cause CF in single copies. Typically it is a nasty form of pancreatic/GI CF but it can penetrate into the lungs. Although I have had GI issues my entire life, I hit a kind of wall at age 48 and was too I'll to work by fifty. Strangely, my father had this form of CF and he died at 48.
Being tested for malabsorption will determine how much enzyme activity you still have. CFers usually have small duct disease and this usually means poor delivery of bile at best and a bag of gallstones and damaged liver at worst. Small ducts and ultra thick pancreatic juices slow the flow of this digestive cocktail down and even prevents the flow. Wherever digestive enzymes are stuck for any period, they digest whatever it is in contact with. Pancreatic autodigestion is an experience in pain that defies description and control in many cases.
The misfiring of everything in the digestive process creates havoc and a misery that is never ending. If you don't get things straight soon, I suggest you seek out Dr. Steven D. Freedman with BIDC in Boston. There are more and more places where the CF gut can be scientifically evaluated and optimized but they are still rare, maybe ten places in the U.S.
Now that your diagnosis is known, hydration, electrolytes, enzymes and good bowel habits are vital to the best quality of life with CF. Diet is always important and the needs of a diet changes. Concentrated fats and proteins are needed to bring your weight up or maintain it. A low FODMAP diet helps with bloating and a tender gut. People are often trying and swearing by gluten free and lactose free diets in particular with CF. It is hard to say for certain with gluten and lactose free diets. A tiny percentage of people are in fact intolerant.
Keeping the goals of eating and pooping normally are important to keep the focus. A normal meal that doesn't come back up and a BM that is formed and pliant are possible goals. Don't get lost in the diagnostic minutiae, worry about feeling better. As a side thought proton pump inhibitors can reduce your powerful stomach acid that kills bacteria we swallow and eat. Low stomach acid can act and feel like gastritis or excess acid. A hydrogen breath test is simple and can determine if the acid is adequate or low, resulting in bacterial overgrowth.
Best of Luck,
LL
My diagnosis was two positive sweat tests. I was more than a bit thrilled to finally have a diagnosis that actually fit what I had dismissed as a constellation of seriously annoying but manageable health problems. Genetic testing followed and according to my brand new CF specialist, I was just a carrier and didn't have CF.
I didn't have pulmonary CF but by chance the CFTR mutation I have does cause CF in single copies. Typically it is a nasty form of pancreatic/GI CF but it can penetrate into the lungs. Although I have had GI issues my entire life, I hit a kind of wall at age 48 and was too I'll to work by fifty. Strangely, my father had this form of CF and he died at 48.
Being tested for malabsorption will determine how much enzyme activity you still have. CFers usually have small duct disease and this usually means poor delivery of bile at best and a bag of gallstones and damaged liver at worst. Small ducts and ultra thick pancreatic juices slow the flow of this digestive cocktail down and even prevents the flow. Wherever digestive enzymes are stuck for any period, they digest whatever it is in contact with. Pancreatic autodigestion is an experience in pain that defies description and control in many cases.
The misfiring of everything in the digestive process creates havoc and a misery that is never ending. If you don't get things straight soon, I suggest you seek out Dr. Steven D. Freedman with BIDC in Boston. There are more and more places where the CF gut can be scientifically evaluated and optimized but they are still rare, maybe ten places in the U.S.
Now that your diagnosis is known, hydration, electrolytes, enzymes and good bowel habits are vital to the best quality of life with CF. Diet is always important and the needs of a diet changes. Concentrated fats and proteins are needed to bring your weight up or maintain it. A low FODMAP diet helps with bloating and a tender gut. People are often trying and swearing by gluten free and lactose free diets in particular with CF. It is hard to say for certain with gluten and lactose free diets. A tiny percentage of people are in fact intolerant.
Keeping the goals of eating and pooping normally are important to keep the focus. A normal meal that doesn't come back up and a BM that is formed and pliant are possible goals. Don't get lost in the diagnostic minutiae, worry about feeling better. As a side thought proton pump inhibitors can reduce your powerful stomach acid that kills bacteria we swallow and eat. Low stomach acid can act and feel like gastritis or excess acid. A hydrogen breath test is simple and can determine if the acid is adequate or low, resulting in bacterial overgrowth.
Best of Luck,
LL
Unfortunately with Boston being about a 6 and a half hour drive, idk how I'd be able to see him reguarly. And yea I have problems with my bile already, elevated indirect bilirubin, upper right quadrant pain, bile reflux. None of my tests/scans showed stones, but when I got my hida scan my gallbladder did not show up during the first two hours of the test, not until they added the cck, but they didn't go into detail as to why,