[glenn]

“Hey Glenn! I am so sorry that I’m glitching on your end for some reason. Jenny and I have been texting about this, and I am for her sometimes too… So we will get to the bottom of it, promise!

Yes, I have atypical CF. I was once diagnosed as PCD, but that we before I went to a CF clinic. Since then, they actually shifted it to CF (or CFTRD)- so now I’m classified more classically, haha. I am super lucky that my current CF clinic is amazing and seems to have a lot of patients who fall at ends of the spectrum…. Thank goodness! In terms of actual symptoms and bugs, I’m really pretty “typical”. I get culture the usual CF type of stuff. But, for me, my digestive system is impacted much much more than my lungs. What about you? Has it mostly been lungs throughout the years?”
—
No worries Bailey.
CFTRD was new to me.. I did read that you had a lot of trouble with your digestive system. None of the new modulators help with that I suppose? It sucks when your options are limited to surgeries, but if they actually help then obviously it’s worth it. Glad to see you are happy with your CF clinic. That can make a world of difference. I guess I had typical CF in that it was primarily lungs for me with a side of pancreatic insufficiency, though I have no right to complain about my pancreas as all it did was refuse to work. It never caused pain and I never really had any serious digestive issues. As time went on and I got closer to transplant I had a lot more issues as my lungs turned to mush – multi organ failure and a massively enlarged heart that attacked me several times. It all healed after transplant which was amazing…

[glenn]

Sorry to hear about your sister, and sorry we have all that in common. They were telling everyone back then a cure was close. Maybe it was a good thing. Hope was in short supply back then.. Not anymore though!

[glenn]

This is nothing short of mind blowing for me. I’m thrilled it is having such a positive impact for you Jenny, and for others taking it. I’m sure I’m no different than others who can’t help but think of friends and/or family with CF (my sister died at 14) who had very little to no treatment options at the time, and how far things have come.. I remember being told that a cure was 5 years away almost 20 years ago. It was 5 years away every few years. I know Trikafta isn’t a cure, but it’s something substantial. It took long enough to get here, but it does make me happy that it actually works and makes a real difference. I hope you keep posting updates..

[glenn]

I can’t seem to reply directly to you Bailey. Clicking on “reply” sometimes works and sometimes doesn’t for whatever reason.. To answer your question, my kidney function is a bit worse than normal due to the transplant meds, but I still feel pretty well, so I take no notice of it. That’s pretty much it.. I take insulin/creon, but otherwise my pancreas has never really given me any problems/pain,etc..

I read from your articles that your CF is atypical, and actually called PCD. I had never heard of it before. Maybe I’m wrong, but I don’t think CF clinics, which I no longer go to, are really equipped to handle all the related issues that can come along with CF, let alone atypical CF.. You must be playing quite a bit of specialist ping pong, which is the worst kind of ping pong!

[glenn]

Jenny, I’m glad you are in a place with less virus! Hoping it stays that way, and you and your family stay safe and healthy! I’m sure you know what’s best for yourself, but still, be careful 🙂

Yes Claire was hard not to like.. I had wondered why they don’t give blood thinners as a prophylactic after transplant surgery since I think blood clots are not uncommon. Have to ask my dr that question next time.. Like Claire, I also had blood clots after surgery, but oddly enough they all settled near my biceps. I guess they felt I was sorely lacking in that area.

Bailey,
Definitely need sour and sweet to survive! I’ve really enjoyed reading your articles here. They are witty and insightful.

I’m close to 13 years post transplant. I’ve had a few infections in those 13 years but I can count them on one hand. I don’t cough, my lungs feel clear, I have energy most of the time, and I can sleep laying flat! I have diabetes, which started immediately after transplant, but it’s really nothing. Overall it’s pretty unbelievable. I’m lucky as hell. If you’re considering it or have questions feel free to ask me anything!

[glenn]

Hi Jenny! Hate to start my first post here on such a sour note, but do be careful please! I’ve actually gone the opposite route you have. I largely ignored the fear and paranoia.. I’ve left CF behind so to speak (double lung tx) and now another illness was going to isolate me again? nope.. I wasn’t reckless, but I went out, saw family, friends, etc, as much as one could while everything was closed..

Now to the sour part..

Ok I’m pretty isolated again.. It brings back memories. CF can be/is a very isolating disease. At least now most people are somewhat isolated so the pain is spread so to speak. I’ve really never spoken to anyone with CF (save my first girl friend long ago who passed, and we never spoke about CF, which was odd wasn’t it/). I guess like a lot of people I tried obsessively to pretend I didn’t have it, and I guess she did too. 3 things changed me for me after all this time:
1) Now that I’m home all the time I’ve spent more time on online and came across Claire Wineland who I had never heard of until last week.. soul crushing, but encouraged me to be more active in CF..

ok the really sour part..
2) My transplant dr at columbia/nyp, has told me 33% of his transplant patients who got covid have died from it..
3) someone I know recently died of covid. She had CF, and her husband without any symptoms, gave it to her.

So, be very careful please.. Seems like a vaccine might just be around the corner, so keep those lungs healthy in the mean time!