[Robert Lane]

Growing up I kept having “viral pneumonia”. My dad didn’t think I should be having so many episodes and thus took me to an allergy clinic in Charlotte. I did have allergies and took shots from age 8 to 14. On Boy Scout campouts, I’d always wake up in the morning wheezing heavily. No fun! After my teenage years all obvious symptoms, including allergies, went away. Then in 1970, in my mid-20s, my then wife and I were trying to conceive with no luck. My sperm count was zero. A testicular biopsy returned the diagnosis “bilateral agenesis of the vas deferens”. No known cause at that time. Life continued. About 1975 had pretty severe bout of wheezing. X-ray showed spots on lungs. Biopsy found Aspergillis. Eventually resolved on its own. No noticeable problems from then until about 1997 after moving to Houston, TX, where mold allergies appeared with a vengeance. Took shots for several years while there, then overseas then back to Texas. Retired to Western NC in 2015, stopped shots and allergies returned with a vengeance. Allergy doc referred me to pulmonologist who suspected bronchiectasis (spelling?). CT confirmed it. Referred me to pulmonologist at UNC med school. While taking my history, she asked about children. Explained about the 1970 vas deferens agenesis diagnosis. Doc explained that high percentage of men with CF had that condition. Did the sequencing and found f508 deletion plus p.Arg810Gly substitution. The latter is rare, but was seen in patient with vas deferens agenesis. So, in 2018 (when I was 73) I learned why I had the condition first confirmed in 1970!

I started Trikafta in January 2020. Lungs have been ok since then, but I’ve dealt with diarrhea (now controlled by fiber supplements–I think William recommended that–thanks!!) and also lower leg swelling (I have double knee replacements), reasonably well controlled by compression socks. From 2018 up to 2020 my pancreas was ok. But now I have significant insufficiency and take Creon.

Given the stories I read and hear about patients with severe cases, I consider myself quite fortunate to have such a mild one. I am writing this as I’m on a bucket-list trip to celebrate my 80th birthday. I am blessed.

[Robert Lane]

I had just the opposite problem when I started Trikafta–fairly severe and constant diarrhea. This seemed the more logical effect of Trikafta since chloride and hence water crosses cell membranes more easily (I presume INTO the gut). I finally controlled it with a combination of carboxymethyl cellulose (like Citrucel) and psyllium (like Metamucil) fiber supplements. I agree with William that talking to your doctor is paramount, but if it is attributed to Trikafta perhaps causing more flow of water OUT of the gut, fiber supplements may help you. But please talk to your doctor first!

[Robert Lane]

I am 77 years old, diagnosed with one f508 deletion plus a rare one in 2018. Been on Trikafta since January 2020. Have had severe diarrhea with lots of hydrogen sulfide (H2S) odor, plus lower leg swelling (exacerbated by having two knee replacements) every day since, unless (1) I eat copious amounts of homemade sauerkraut to get near-normal bowel movements and (2) I wear compression socks which manages the leg swelling. Commercial kraut or kimchi or probiotic capsules do not help at all. My team say my lung function has improved, but I cannot tell any difference. (Note that my CF is VERY mild compared to many). I did have M. abscessus cultured from sputum, but it disappeared about the time I started Trikafta (I had been nebulizing 7% NaCl for some months before Trikafta, and I think that is what got rid of the bacterium).

I stopped Trikafta just over a week ago, and bowel movements immediately returned to near-normal and lower leg swelling disappeared. I am starting Trikafta back at low dosage, hoping to avoid these problems.

Does anyone know how to manage diarrhea while on Trikafta? I’m ok when I’m home, but am terrified of traveling where I don’t have access to my homemade kraut.

[Robert Lane]

I was diagnosed with CF at age 73 (I’m now just shy of 78). One copy of the F508 deletion; the other a rare mutation. I’ve had asthma/allergies and related problems along with infertility, but no other symptoms I remember. I started Trikafta in January 2020 and immediately began having horrible diarrhea and lower leg swelling. I had never had problems with bowel movements before, though I did have occasional lower leg swelling from excess exercise since my two total knee replacements. I fairly easily mange the swelling by wearing compression socks, but the diarrhea has driven me nuts almost every day since I began Trikafta. I also generated significant hydrogen sulfide (H2S) containing gas. Trikafta really does a number on my gut biome. I can somewhat manage that by eating copious amounts of homemade sauerkraut or kimchi every day. (Commercial kraut, kimchi or probiotic capsules have little effect). I stopped taking Trikafta eight days ago and both the swelling and diarrhea stopped almost immediately.

When I started taking Trikafta, my pancreas was functioning well (fecal elastase 264); but two years later I have very significant pancreatic insufficiency (fecal elastase 85 and 105 in two recent tests). The little info I’ve found on this is that many patients see an improvement in pancreatic function on TK. I’m wondering if anyone else has seen such a dramatic decrease.

I didn’t see much, if any, noticeable improvement while on Trikafta, except perhaps a thinning of post-nasal drip mucous. My docs say I do have some improved lung function, but it has not affected my quality of life, and it really isn’t noticeable to me.

I’m guessing there is scant data on side effects or efficacy of Trikafta in people of my age. Does anyone have suggestions on how to handle diarrhea caused by Trikafta? Any other comment on my observations?

My care team has been working with me on these things, but they are reluctant for me to stop TK. I will start it up again–initially at half dose. We’ll see from there.

[Robert Lane]

Kathy, I am so sorry to hear of your experience of deterioration of care at UNC. I have been treated there in Chapel Hill since December of 2017 (they diagnosed me). My most recent visit was December 26, 2023–the day after Christmas. I have to say that I have always been treated with the utmost care and respect. I leave feeling encouraged, even when I arrive there feeling pretty low. My doc is on top of the research and clinical trials and takes care to explain the latest findings to me (I try to stay somewhat familiar with the research also, as I was once a research chemist who studied proteins and protein folding. I am very impressed with my doc’s knowledge and treatment recommendations.)

I sincerely hope your experience improves. This disease is a nasty business.

[Robert Lane]

Many thanks, Tim, for your quick response and helpful suggestion. I will add the probiotic and fiber supplement.