r/CysticFibrosis

I'm asking people without the F508 mutation as that one is approved for everything.

I'm trying to find out which EU countries have one or both these medicines approved officially for the mutation N1303K. I spent last night looking for the answers online but they're not so clear.

Thanks for letting me know!

So my youngest fighter got approved for Orkambi and is supposed to start this week... insurance called and the copay is over $7,000... wtf? Like thats 2 months income for a 28 day supply... we're waiting to gear back on copay assistance but seriously why is medically necessary medicine costing so much? Is this common for CF meds?

I’m looking for experiences with Natera Fetal Focus. We recently learned that both me and my husband are carrier for the same variant f508delta. I received my Fetal Focus results back today that they are detecting two copies of the variant in the test, resulting in 92% chance that our baby has cystic fibrosis. They made clear that this test is not a diagnostic test and I have an amnio scheduled which will give definitive results. I have concerns that the test is actually picking up my variant plus the baby’s, which would make her a carrier.

The test isn’t scheduled for another 2 weeks. It’s hard not to fall down a rabbit hole, but with this test being so new it’s hard to find any real world instances where this testing has been done and what the ultimate outcome was.

I’m basically just hoping to find people who have had the test done through Natera with a follow up CVS or amnio. I’m stressing and looking for anything to put my mind at ease.

Any input is appreciated and thank you in advance.

Hey all, sharing this for the next person who frantically googles 'alyftrek side effects reddit' ha ha.

Just want to say that I took Orkambi, Symdecko, and then Trikafta. With Orkambi, my legs had a rash for one day. That was the only reaction I had to any of the modulators.

Switching to Alyftrek was more challenging--for the first week I felt WIRED yet exhausted, my right ear blocked up, and I felt so stupid in my head. My, uh, digestion hasn't been great. And I experienced some interesting changes to my libido (those were not bad though *clears throat*). The wiredness was bad, I couldn't concentrate enough to work for over a week. No changes to my breathing, sinuses, blood sugar (I'm pre-CFRD), anything else.

I'm happy to say that:

--I switched to taking Alyftrek at night so I could sleep through the wired/stupid. I take trazadone so I knew that would cover it so to speak. This worked.

--Wired/stupid is now completely gone.

--Digestion is still unpleasant, but pancreatic sufficiency has been my biggest challenge, so I expect this will take some time.

--I saw my amazing ENT about my ears. He said I had middle ear effusion and gave me some prednisone and okayed me doing the valsalva maneuver. It took a few days but it's almost completely cleared.

Important: if you're like me, and you're switching to Alyftrek because it's supposed to make you less anxious...at first you might feel more anxious. Push through as best you can, because I AM noticing less anxiety and that's been great. Sometimes I even feel a sense of wellbeing!

In full transparency I also take daily fetzima, adderall (for sleepiness only), trazadone, lamotrigine, and lorazepam as needed. I have abilify for when I take prednisone, because prednisone makes me hypomanic and I almost become catatonic when I take it. And yes even with all that I still felt hella anxious so I wanted to see if Alyftrek could help with it. So far it seems like a yes.

When my ENT was checking my ears he looked at my sinuses and I was astonished to see them EMPTY. They used to be full of green lozenge-looking things and now they are just empty hallways. What an incredible time to be alive. (We will never abandon the 10% however)

I'm 19 and recently got on oxygen supply, in January this year I was doing fine walking around and doing house work was not too much but past few weeks suddenly it became worse, and it turns out my oxygen saturation was low even tho it had never been before. Idk how to prevent my situation from getting worse, Dr appointed me some oral antibiotics as of now. Are there specific exercises and supplements to prevent this from getting worse. I've been able to be independent as my friends and siblings my whole life uptil now despite my CF being on the bad side since birth. And I have no idea wat to do now. I also don't have access to CF modulator and dont qualify for one.

This post is for all my CF moms… I’m really thinking about having a baby in the future, but I’m really unsure with all the CF, CFRD symptoms and the modulators side effects I’ve dealing with. I’m not as scared of the pregnancy but the afterwards, getting tired easily and sleeping a TON, has been one of the side effects of the modulators, so I’m unsure if I’ll be able to take care of a baby in all his stages.
Also, thinking about all the steps I need to take before getting pregnant, because obviously is not your usual pregnancy…
Would love if you could share your true and honest maternity experience 💗

I’m 22 and living with CF. Just curious if there are any other guys here in a similar position too. I don’t really come across many people who can relate, so thought I’d ask and hear other people’s experiences.

Hi guys!

My name is Pete. 41, born and raised in Indiana, now in North Carolina. CF since I was diagnosed at 8 months. When I was 13 my doctors told me I probably wouldn't make it past 18 and I shouldn't expect to have kids. I have three. We went the CBAVD plus IVF route, and now there are three small humans in my house breaking everything I own.

I'm also the founder of the Filotimo Foundation. "Filotimo" is a Greek word, that roughly translates to "helping someone and expecting nothing in return" which is what my family raised me on. The foundation exists because medicine caught up with CF and the support system for adults around it did not. We help adults with CF with two things: hardship (rent, copay, meds, when the system is failing you in real time) and family building (IVF, fertility, adoption costs). That's it. Real support, real fast, no red tape theater.

I'm posting here because I've been a lurker too long and the people I most want to help are already in this sub, asking questions I have answers to. I am not here to fundraise (donor stuff happens on the foundation's own channels, not yours). I am here to listen and help.

If you have CF, or you love someone who does, and you're trying to figure out fertility, copay, hardship, adoption, or just what life as an adult with this thing looks like, DM me anytime. Comment here, DM, whatever works. If Filotimo can help, great. If we can't, we usually know who can.

Moderators- if this breaks any rules let me know and I'll edit or delete.

Pete

Hi friends!

I wanted to share something here because this community has meant a lot to me over the years.

I recently had the chance to sit down with Meet the Press and talk openly about what it's actually like to be an adult living with cystic fibrosis. The parts that don't usually get airtime. The waiting rooms. The fight for coverage. The quiet weight of planning a future we were told not to plan for.

For a long time, adults with CF have been the unseen generation. We grew up not expecting to be here. And here we are. Building lives. Raising kids. Showing up for each other.

I just wanted you all to see it. Not because of me. Because of us.

http://youtube.com/watch?v=zvbJdoLMMuE.&feature=youtu.be

And to this beautiful community, if you have ideas, please please reach out. I want to hear them. We move further when we move together.

Thank you for being part of this. You matter more than you know.

Pete

Has anyone else experienced really bad morning coughing with CF?
For me, the coughing is worst during the first 5–7 hours after waking up, then it mostly stops at night and I feel much better. I’ve also noticed it gets significantly worse after eating breakfast. Sometimes I cough so hard after breakfast that I end up vomiting.
Because of that, I started delaying my first meal by 2–3 hours after waking up, and surprisingly the intense morning coughing became much less severe.
Has anyone else noticed something similar? Did delaying your first meal help you too?

Hey all, my(27M) gf(24F) just started Alyftrek yesterday. Her doctors told her to start with half doses because she's had complications with modulators before. As the title suggests she's already coughing up blood before even the 24 hour mark of her taking the first half dose.

She's actually really healthy. She goes to the gym with me, she trains jiu jitsu, she and her sister (also has CF) run long distance together. For whatever reason when she tries modulators (Trikafta and Alyftrek) they always cause her to cough up blood and feel awful.

Is her body rejecting the modulators? Has anyone else experienced something similar? If anyone has any advice it would be greatly appreciated. TIA!

Edit: this probably helps. She is delta f508 homozygous.

So it's been a month now! I've lost 10 pounds. I notice it mostly in my belly. My pants are looser.

Side effects I've had are: nausea, constipation and fatigue. They've tapered off the longer I've been on theed. I'm rarely nauseous now and not taking a nap every day. I was taking a full dose of miralax every day for the first couple of weeks but I'm down to half a dose for the 2 days after my shot. If you struggle with constipation you need to be ready if you start these meds! Even with the miralax I only went #2 three times in the first two weeks.

For a while I didn't get hungry at all and had to force myself to eat even just to take my meds. You want to stay on top your vitamins with this stuff. I started taking biotin too because a lot people lose a bunch of hair whe they start a GLP1. I haven't noticed that so far.

When it was time to.keder.my refill I got approved to double the dose. I'm not gonna do that! I think a slower increase will be the way to go.

I’m currently with my son at Children’s in the MICU. He has two class 1 mutations, and we will be meeting the transplant team to talk about evaluation and ask questions next week. Anyone who has been at this stage, are there any questions you’d ask or things you learned that you weren’t expecting when you started the process?

Ok so first time posting. I'm 33m with ∆f508 and another gene I can't quite remember. FEVs are around 34% on a good day. I have had a hard time being compliant with meds and have gotten into bad habits such as alcohol and weed. I have bipolar and depression and anxiety. I've been to so many therapists and none have been able to really grasp the severity of cf in general let alone everything else I'm dealing with. I guess my question is has there been any good coping mechanisms that are healthy that you have tried to get away from the stressful life that is cf? I know it sounds like I'm running away from the problems it causes but I'm sure I'm not the only one that needs that "get away from it" feeling. I'm a nerd at heart so I love video games which help but at the same time I guess I'm struggling with my own mortality. Generally I've been fairly healthy throughout my life given the circumstances but I'm also just trying to reach out to people who are similar to my own issues.

CF dad here 32M. So yesterday I had a vasectomy done. Doctor said at first it appeared that I had one Vas Deferens, but then he said it appeared I might have one that was atrophic. According to my urologist this is common in CF carriers. He also mentioned if you are missing one Vas there is about an 80% chance you are missing a kidney on the same side, as they both form together in utero.

I have an ultrasound Monday to see if I have one or two kidneys. Ive never had any serious health issues and am well aware that most people with one kidney live full and normal lives. But, if it is the case I have one, I will definitely be making some lifestyle changes to protect it.

Idk if this is the right place for this. Just wanted to share it might be worth telling your dads to check with their doctor.

Hi everyone! I’m going on a very exciting trip— I’ll be away in Europe for 6 weeks! I am beginning to get all of my things together to pack and am curious if you have any tips on how to pack meds for an extended period of time (while also not taking up SOOO much space in my suitcase). I was thinking about getting a month- long organizer but it’s so bulky.

Any ideas?

Im now 30, and it's all catching up with me, over the past year I've been in and out of hospital far more often, and far too often, and each time they seem to be getting longer and they seem to add on some heavy topic of conversation. The latest and a reoccurring one is the use of oxygen.

I used to be so into the gym and going on long walks, and now if I want to do either they say I need oxygen. But if thats the case, I wont leave the house as im far too self conscious... and I havent been, so have not been doing much exercise for a while. I feel so weak and so fatigued the entire time. When I was going to the gym a bit last year, it was months of no visible progression (stat wise, not bothered about visually anymore/right now).

The exercise I do do, my tolerance is now minor and I need to keep an eye on my blood oxygen and stop when it drops bellow a certain point, which seems pretty quick and after not doing much activity at all. Even just going for a walk down the road the sats reader says I should stop and wait... it's pathetic... I want to be back going on long walks and enjoying life.

It's a lose lose, do exercise, need oxygen, and hate myself due to social anxiety and might not have any benefit anyway, or don't do exercise, continue the decline... then what?

Im always short of breath now, my chest is always achy and im having to rely far too much on constant paracetamol and ibuprofen, particularly in the morning, sometimes having to wait for them to kick in before I can get out of bed.

Im always comparing myself to Covid time, in lockdown I was never better, in the gym doing heavy workouts for 1.5-2h, then an hour or so dog walk, so thats all I compare to... how good I was... everything I see on TV or literally anywhere, all I think about is oh, I couldnt do that now, or I wish I could still do that, or they don't know how lucky they are, particularly when it's someone who has clearly never looked after themselves for a day in their life, and I put so much work into it. Mentally Im a wreck, but no one can tell and I have never shown it. Ive spent my entire life hiding CF and trying to look like, and do things 'normally' so now that it's all becoming too tricky to hide, im really struggling.

Has anyone dealt with this before, and come out the other side in anyway of progression? Ie. in a state where they don't need oxygen and managed to build up some strength? And can return to an active lifestyle?

The doctors sound pretty helpless and out of ideas now, so really trying to find a way forward.

Thank you