Is a decline inevitable?

[emmalou1610]

Hey guys hope you're all doing good! I have a question that's been on my mind lately about natural deterioration in CF. Excuse the seriousness/slight morbidity but I am curious. I am 24 years old, I am very compliant with treatments, I try to run 5 days a week and my FEV1 is stable & always high 80s to low/high 90s, I am very lucky. My weight is good & overall I am doing well thankfully. I do experience problems with CF, don't get me wrong, digestive, and pleuritic pain & low energy etc & am starting home IVs tomorrow, but overall doing really well. So my question is this, does anyone know of someone who has been so well with CF, compliant with treatments, exercise etc with high FEV1, who still took a sharp decline? Or am I completely naive in believing that once you're well & you look after yourself 100%, you are always gonna be ok? I'd appreciate honest answers & excuse my ignorance on this matter. I am just curious to know what the future might hold for me.

Thanks you guys & happy new year!

 

[semnle34]

Emmalou,

Well I think the disease varies from person to person. In my 20's I was like you high FEV's, exercised like an animal, had good weight, non-diabetic went in for regular tune-ups 2-3 times a year. I was even a police officer for 10 years. I have been on and off IV's my whole life, tune-up's, CPT, Nebs etc...I think we all know that at some point our disease is going to "decline" to some point. As to what extent or how fast is unknown. After testing positive for Mycobaterium Abscessus several years ago my lung function declined, I've been sick a lot more and had to quit my job. I am still doing really well despite fighting this bug, my FEV's are still up there (they fluctuate) weight is good, still not diabetic. I think you have to be realistic about CF but keep in mind that with all the new research and new medications the outlook for Cystic Fibrosis patients is better than it ever was. I know people who are in the later 40's now who are doing fantastic. I also have 2 friends who have had double lung transplants that are doing absolutely amazing. There will be set backs and then you will need to fight to get back. Keep up with exercising, following your treatments and most important keep your head right. A strong mind can over come more than you know. Don't spend time thinking about what's possibly going to happen, focus on living now and doing everything you want to. I strongly believe in will power and your commitment to be a fighter. If those two things are high on your agenda you will do great and kick CF's butt. Hope this helps. If you have questions or want to chat just inbox me :0)

 

[Aboveallislove]

I can't give you the personal take as I'm the mom and our son is 5.5, but from everything I've read, as semnle said, it varies. It is so great, though, how well you are doing now and how dedicated you are to things. That honestly is going to be so key because, again as semnle said, the meds that are coming out. There are several people who were really really struggling and then with Kalydeco it was night and day. Do you know what mutations you are? If you posted (and you don't know), I can give you a quick update of the meds that are coming around. Since your doing so well, they will really be great for you since you likely don't have a lot of lung damage!

 

[welshwitch]

I wouldn't say it's inevitable, but I also wouldn't rule it out. When I was in my mid 20's I had kind of a "CF freakout" and one thing that really helped me was talking with an older CF adult on the phone. She was in her 50's and also doing really great. One thing I remember her saying to me is "everyone slows down eventually, we're just all on different timetables." I'm 35 and still working full time with a lung function of over 100%. But, I think my CF is mild. I also don't rule out the possibility of things changing on a dime one day. So, for now I am just trying to be healthy, smart, optimistic, and listen to my body. Also, worrying about something that hasn't happened yet isn't productive. It's worked for me so far

 

[emmalou1610]

Thanks guys for your input! It's great to hear of other people doing so well.

It's not something I dwell on I was just curious in general. I am a very positive person & to be honest never really think about my CF & I guess I'm lucky I don't really have to yet .

To aboveallislove I am double delta F508, so I'm really hoping for the new drug! My consultant recently just told me how well I'm doing for those mutations.

You guys are right about positive mindset, I think that is so important in fighting any illness. It's as important as taking meds & exercising etc.

Welshwitch I like how that lady put it to you about us all being on different timetables, it makes sense!

Thanks again guys for your replies, it's so great to have a community of people on here who really understand.

As for me, I'd better get my running shoes on before I go to the hospital this morn to start IVs. The joys!

Have a nice week all!

 

[Ratatosk]

As a parent of a child wcf were told from day one that cf is a progressive disease and eventually the lungs will be affected. Thus the importance of proper lung care (cpt/vest), nutrition --- maintain a routine and being proactive. That said --- I've seen people do everything they're supposed to do and get knocked for a loop from influenza, hospital acquired bugs, issues during routine surgery. On the flip side, I see people who were diagnosed later on in life or who aren't as proactive who have little or no issues healthwise. Sometimes it's just "the luck of the draw".

So as a parent, I try to find balance. It does me no good worrying about what ifs and waiting for the other shoe to drop... That said if ds is a little off, gets sick, I'm a HUGE worrier, in full on freak out mood. Although I do try to logically plan out a strategy, freak out, pause, repeat...

A couple years ago, despite vesting 3 times a day, being relatively healthy, ds had a lung scan which showed issues with his large airways. We were pretty sure he was going to end up admitted for a clean-out. Not sure what happened, we did extra cpt and next lung scan things were improved. Not sure why he had issues at the time -- nothing changed, he wasn't culturing anything new... At the time we thought, "here we go, it's that disease progression we were warned about".

 

[imported_Momto2]

In short, Yes. I have seen many people work their butts off, and take a sudden nosedive. It SUCKS. 2 years ago I was training for a sprint triathalon, running 10K 5x a week, swimming a mile every day and biking 15-20 miles 3-4 days a week. Now I have to use oxygen to walk on a treadmill. I havent cultured ANYTHING different (or bad) in my sputum and my FEV (strangely) is still around 55%, as it was 2 years ago. The difference is a continuous severe asthmatic componant. Yeehaw. It has completely changed the quality of my life.

 

[stephen]

emmaliu1610, and others:

At the age of 73, my present feeling is that decline due to CF is NOT inevitable!

A year ago I did not feel this way. I felt myself going downhill. And then along came something I was not expecting - Kalydeco.

For this past 10 months I’ve felt better than I have in years.

Even if Kalydeco does not do for you what it has done for me, there are other drugs in the pipeline. As many are saying, the future does look bright for many with CF, and the turnaround may be right around the next corner. It was for me, thank G-d. Be optimistic.

 

[Deb]

Everyone is different and due to all the different mutations and numerous bacteria we can encounter there is no way of predicting the health of any of us. You are on the right track. Continue to stay on top of your health even though you are doing well. I was like you for years and barely felt like CF was part of my life. Even so, I continued to be diligent with meds and therapy. At 54 I am doing exceptionally well but I have had more than my share of struggles. Losing a lung was tough but I am now doing much better without it. And Kalydeco just got approved for my mutation so my future looks good at this point. Bottom line, age and progression are inevitable but the way you approach it makes all the difference.

 

[Hail2Pitt]

I agree with the main sentiment here - everyone is different. I have seen graphs of of FEV1 over time for the CF population, and it does show a decline. However, if you produced a graph for each mutation combination, you'd probably see a lot of differences based on just that. And, not all patients approach their CF the same way. emmalou, it sounds like you are very compliant with your treatments and are very active, and that's not the norm across our population. If you graphed FEV1 vs time for patients who exercise and are 100% compliant with their treatments, I absolutely guarantee the rate of decline would be less than it is for those who aren't compliant and active.

I was diagnosed eight years ago at age 29, and my FEV1 was about 80%. Eight years later? It’s still 80%, as confirmed by my clinic visit yesterday. When I was diagnosed with CF and M. Abscessus, I read about a patient who received the exact same diagnosis at age 30, and passed away within a year – it of course freaked me out. Though, I have no idea how she handled her diagnosis – did she attack it, do everything her doctors told her to do and more, and work like crazy every day to keep her lungs clear? Or did she take a more passive approach? I don’t know, but by choosing the former, I’ve been able to maintain status quo for several years now.

I’d just encourage you to control what you can control, i.e., work every day to keep kicking CF’s butt, which you’re obviously doing! And for your mutations, know that the cavalry is coming soon – the Ivacaftor/Lumacaftor combo likely later this year, and the Ivacaftor/VX-661 combo hopefully shortly after!

 

[cam]

In my situation with it a big yes the kaldeco has done nothing for me and im very complaint with my treatments and meds juat nothing helps im 35 fev1 of 50 to 55 on a good day has steadily went down over the years but for some with different genes it wont b so fast or maybe not at all but in my life and the cfers I personally know it definitely is but I hope one day it wont b

 

[Gammaw]

Hi Cam. I hope this new drug for you helps with the decline you were experiencing. How long are you supposed to take it and in what form? I'm unclear tho on your statement about Kalydeco.....have you taken it or do you mean it hasn't done you any good since it's not approved for your mutations? I hope you improve rapidly! I've been wondering how you were doing! Keep at it!

 

[cam]

On study for it seems like forever now been on actual drug for long time too decline has been happening for awhile now I hope everyone stays healthy tring to stay positive

 

[donin]

I think a lot depends on your mutations. There are some mutations that are so mild it's difficult to really understand why they are categorized as the same disease as someone who is homozygous for deltaF508.

 

[Cardinal]

Decline is not inevitable because new drugs are on the way - better combination oral drugs that can correct and potentiate more mutations, and better inhaled combination drugs that actually make sense for CF. Drugs that prevent progression instead of treating symptoms! With an oral combo that can move sweat chlorides near the level of a heterozygote and keep them there, patients are stable. If patients inhale the substrates CFTR fails to transport in the right ratios and amounts a couple of times per day then lungs are hydrated and fight infections like normal lungs. This inhaled drug would work for any mutations. Everything mentioned here has proof of concept already in patients. So it is coming. Stay as healthy as you can, all... and hang in there with justifiable hope.