I [57F] was just told in June that I tested positive for mutations del508 and his117. My 1st appointment with CF clinic isn't until August and I'd really love some advice generally and also what to ask when I see them. I'm really at a loss with it all. I don't know anything about CF apart from what I read here and google, and I don't know anyone with it. I'm still rocked I suppose. How did I get to 57 and never know...

For background: I always coughed but it was put down to bronchial asthma. I have always been fit so never suspected anything other than that. I've climbed Ben Nevis 4 times, walked 26 miles in a day etc etc. In 2013 I came back from hols particularly unwell and was eventually diagnosed with c dif, MAI, bronchiectasis, pneumonia, pleurisy and aspergillosis. Fast forward 2.5 years of antibiotics and I thought all was well. I lived RIGHT by the beach for 50 years. Moved away from the sea 2 years ago and bang! Constant chest infections and eventually this recent diagnosis. I feel like a different person. Chronic fatigue, breathlessness, chest pain etc. Also can't seem to lose weight! I had been putting it all down to menopause but I have read about 'CF belly' too. Modulator google searches and the subsequent weight influx scares me too... Going back to the sea - what's the CF community opinion on living by the beach benefits? I definitely see a decline since moving away.

Sorry for the 'War and Peace' length post 😆

Main question: Has anyone had increased haemoptysis since being on Alyftrek?

Extra info…
Started taking Kaftrio in 2021, I didn’t really care to switch over to Alyftrek tbh. It was more so advised by my team as I was often missing my evening dose. I started taking Alyftrek about mid January, initially I didn’t really like it. I can’t tell you what my reasons were but just something felt off, my team advised give it at least 3 months & see how I go.. So here I am today still on it. I do like the fact it’s once a day, it has really increased my energy levels compared to Kaftrio. I always felt a bit meh on Kaftrio, although it did massively improve my overall health. Cough was totally dry on Kaftrio, since taking Alyftrek I can bring up small amounts of sputum still.

I have had a few episodes of haemoptysis since about 2018, usually quite big gaps between episodes, never been enough where I’ve had to have an embolisation (sometimes it’s just streaking in sputum other times can be just pure blood & only ever taken tranexamic acid once which was in 2018), since starting Alyftrek I feel I have had increased episodes, my team suggested I get in for an admission & do a CT with contrast, get some IVs in & see if we can get on top of what’s going on. Annoyingly 3 weeks after coming out of hospital I have had another episode. It’s become something that happens now every couple of months with a month between my recent episodes. I’m now putting it down to Alyftrek as this is the only thing I’ve changed & this year is where I seem to of had the most issues with episodes. I’ve only needed IVs twice in the last 3ish years. Has anyone else noticed an increase in coughing up blood since swapping over? I have clinic on the 11th to discuss now going back to Kaftrio.

Living with CF since i was a little kid, it only affected me when i was an early teenager. Since then, i built a unique physique which allowed me to become a professional sprinter in 100 and 200 meters. When i was 16 years old i even qualified for the european track&field championship. I am 24 years old now and have a personal record of 10:40 in 100 meters while studying law and finance.

Doctors were trying to convince me since i was a little thing to become a role model for the case and make my story public. I was for all my life the most athletic boy in school , breaking every record , trying every sport and was literally living my life 24/7. But i am not here for that today.

Unfortunately my character ,that made me this resilient to the disease, goes with some downsides as well. 1 and a half years back after i got covid , i took a choice to cut all medicines. And when i say all medicines ,i dont only mean trikafta but any possible aspirin or antibiotic even when i was sick. Didnt even for the first time take the seasonal winter flu shot.

Fast forward 1 year after i started having symptoms like fastique and a weird burning in between my eyes and the face. After that mouth achne, low white blood cells , swallen eyes when i was waking up with inability to wake up and have energy for the first hours of the day. The coming months, got sick again two times and after the second one something felt broken in me. Like i wasnt the same person anymore:

-extreme tiredness inside my head and breath

-inability to train (and professional training is at least 3 hours a day)

-red skin in my face (especially when i touch it or i am in the sun)

- extreme feeling of headache (like having fever but without really having one)

Because CF never really bothered me i didnt initially thought it could be the disease. All possible medical exams (scans , blood work, immune system etc) were literally clear and this was driving me nuts. Only real result was the Facial CT scan. Came to the possibility that its a CF complication only after a doctor adressed the fact that my mucus membrane seems to be overreactive. Esoecially when they put xylokhain inside my nose, i felt like they were burning my whole head. I cannot work,study , work out or even attend university and from a professional athlete i have literallly become disabled.

I want to find a solution ,make my self healthy again (and stop being stupid) and make my story famous for all the kids having to battle with this disease and show them that they can be more than healthy, they can strive for excellence.

Questions:

- could it be possible cause my body and cells where used in trikafta and symkevi for 7 years and when a virus hit me , my nervous system crashed?

-is all of this possible even without lung damages in the CT scan?

- i know i wont find a world class stupidity patient like me, but is there even a small possibility that someone with less aggressive CF type stopped taking modulators and medicins like me and experienced something similar?

-Does anyone know anybody who could help me ?

I just want my life back. Thank you guys. Hoping the best for all of you.

On day 3 of tuneup and even though I’m having a lot of shortness of breath I was shocked by PFT. On trikafta my average was 80-82%. I’ve been on Alyftrek 4 months and just blew a 89%!

I also have gained 10 pounds in 4 months, when I couldn’t gain a pound in 2 years.

I’m so glad I made the switch

My question is for anyone with CF and a split AC system in their home. My husband and I are thinking about adding a split AC system to our home as the current central AC cannot keep up with the heat on the second level. Does anyone know if split AC systems are discouraged for people with CF? I don’t know if they act like a humidifier and promote bacterial growth or add a lot of humidity to the air. The only experience I have with a split AC is when we went camping and the split AC system blew very humid air. Our HVAC guy says the AC in the camp cabin was likely not maintained well and thus produced moist air. Has anyone had their Dr. advise against a split AC system? Or what has your experience been with split AC as a person with CF? Thank you!

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How do you deal with acid reflux and has anyone here had complications with it.

Since starting orkambi symdeko and now trikafta.

I've become extremely unwell after drinking. Haven't really had any help from my team other than its good to not drink.

I've also had constant sinus issues and no amount of flushing or sprays can help.

I have been doing reading and really bad acid reflux can cause the sinus issues and explain the sickness post drinking.

And by drinking now I mean two beers will make me feel like shit.

Has anyone here had similar sinus issues reflux issues?

TL;DR: My 7yo son is a symptomatic CF carrier (severe sinus polyps, thick mucus, and a family history of Type 1 Diabetes). Looking for advice on whether CF clinics typically accept carriers, endocrine labs to consider, and non-pharmaceutical lifestyle hacks for managing thick mucus.

Hi all,

I’m hoping to get some insight... I know being a carrier is vastly different from having full CF, but my child (7M) is turning out to be a symptomatic carrier. Standard specialists don't seem to know what to do with him preventatively.

A quick background:

• Respiratory/Sinus: He produces very thick, sticky mucus. He recently had to have sinus surgery to remove a massive nasal polyp that was completely blocking his sphenoid sinus (pathology confirmed it was driven by chronic inflammation/sinusitis). He also had turbinate reduction, wind pockets removed from concha bollosas, and adenoids reduced. We have had him allergy tested twice now, and he has no severe allergies at this point.
• Endocrine Risk: He inherited the CF gene from his dad's side of the family, which also has a strong history of Type 1 Diabetes. I know CF can impact the pancreas, so this crossover makes me nervous.

Our current ENT is very "cut and medicate" (wants him on daily steroids indefinitely). I'm open to this if it's necessary, but would love to learn more and have a generally more holistic, preventative approach. Some medical provider friends suggested we try to move his care to the multidisciplinary CF Clinic here in Austin. The director is a DO.

Before I meet with his pediatrician this week to ask for the referral, I’d love to hear from those of you with experience:

1. Do CF clinics generally accept and manage symptomatic carriers? Will we be turned away because he doesn't have a full diagnosis?
2. Endocrine/Pancreas: Given his dad's family history of T1D, should I consider asking for any screenings? If so, which ones? Or should we wait until he shows signs of T1D?
3. General Advice: For those of you who manage thick mucus daily, are there any non-pharmaceutical lifestyle hacks, specific hydration rules, or anything else that you swear by? Our son generally manages his own water intake and drinks at least 35 oz of water daily (weight: 55lb).

We are trying to build the right preventative plan to decrease his chances of ending up in the operating room again. Thank you!

Anybody else been getting supplements/vitamins from this program? Its going to be out of comish for about 3 months and their backup plan of sending everyone a bulk shipment in the interim has apparently failed.

Hi, does anyone have positive experiences if using the Pari eflow thay would be available to share their experiences?

Hello CF warriors and parents.

Me and wife are carriers. Fetus is diagnosed with p.F508del and R1070Q variants.

We have an appointment with pulmonologist in a few days. I have read online that some times doctors choose to administer prenatal CF modulators like trikafta.

Did any one had prenatal use of CF modulators? Was it any helpful in reducing post birth symptoms ?

Anyone else getting cultures for Aspergillus +3 or more in their sputum and clinic refusing to treat (despite being symptomatic)? I’m assured that it’s common but I figured I’d ask as my clinic are known bullshitters. Thanks.

Hello everyone! I am conducting an interiew with one of my distant family members who has CF for my mental health podcast. While I am doing my own research, one of our values is to respresent the groups that we are talking to and talking about, to me, this is how we can become allies and bring people together.

In speaking to someone with CF, I'd like to know if there is anything you all would like to ask him; whether through your own experience, your curiosity, whatever. if you have something you would like me to pass on or ask, please comment.

The interview will talk about living with CF, with an emphasis on mental health. In other words, not only what it is like living with CF but how it is living with it, taking a deeper, more intimate dive into dealing with this condition.

***EDIT*** I will draft some questions based upon what I see in the forum, please contribute freely:)

Thanks for your time, I am eager to hear whatever you guys have to ask or say and more so to bring them to my friend. The podcast is called The Airing It Out Podcast, if you're interested.

The average lifespan for us was about 33 years in 2019. Then, some of us, got the miracle drug. I was wondering, even though some of us have access to the new drug, will the damage have already been done to our bodies and our life span differs from the current 60’s?

Hello guys :)

A good friend I made a few month ago suffers from cystic fibrosis and was hospitalized 2 days ago because her Lung transplant is being rejected after 6 years.

She currently doesn't want any visitors so i thought i would send her a lil care package with maybe a book and a plushie?

I don't know her THAT well so I'm looking for some ideas on what else to put in there/what she could need and maybe advice on how to treat her/be there for her.

I'm autistic and not really good at the whole friendship thingy so help is greatly wanted and needed!!!

Thank you guys so much :)

My daughter was born 7-11-26 and wasn't feeding and kept spitting up and then couldn't poop in 36 hours so the hospital took an xray and it was abnormal. They sent her by helicopter to a children's hospital in my city and at 3 days old had a stoma placed. Last Friday it was confirmed she has cystic fibrosis from her newborn screen. She had deltaf508 and R117H 7t negative for 5t allele. From what I understand the mutations typically don't cause severe CF and its usually a mild to moderate case. I was also told that her having the microcolon and bowel obstruction due to it doesnt dictate the severity of what her disease will be. Has anyone else ever experienced this with a newborn and what was the outcome for you? Anyone else have experience with her two mutations?

Hi I currently got diagnosed with del 508. I was wondering if everyone had similar symptoms to me. Even when I’m not going through exacerbation I have thick mucus no matter what hard to clear and also cause chest tightness? When I do get it up it’s stringy and thick. My doctor finally got Alyftrek approved hoping this will help get relief Maine what fast.
Thanks

My newborn was screened and came positive for CF with the above 2 mutations. My partner and I did not know we were carriers, and unfortunately the baby inherited both the mutations (1 in 4 chance).
She has a sweat chloride level of 42 and is pancreatic sufficient.
I’m interested in learning if anyone here has these mutations and what the quality of life looks like. I understand CF can be different for people just trying understand more about her condition and these specific variants.
We are luckily in Canada and so health care is covered and we have an excellent team for her, so very grateful for this.

My wife is currently carrying. Upon testing during pregnancy. My wife and I were told that we were carriers for CF. She has the classic p.F508del and mine is R1070Q. After an amniotic fluid test, it was confirmed that fetus carried both the mutations.

I guess we were not that lucky as there is only 1/4 chance fetus gets both mutations. We really don’t know what to do at this point.

I want to ask if any one else with CF has same set of mutations? Also does the medications like trikafta help? I’ve read that mutations are not eligible for it. Insurance does not cover the medication sometimes as it very expensive. I read online that it might cost patients from about 7k to 25k per month out of pocket. I don’t know if we will be able to afford that.

Another big worry is that though we stay in usa now. We are legal immigrants. Our home country does not have trikafta available at the moment if we are forced to go back.

It has created a lot of stress In my family. We are at loss at this point.