How did your decline go?

[Lena Bean]

If no one wants to answer that's fine. I'm honestly just curious. How did your decline leading you to need a lung transplant go? I am moderately healthy for a CFer, and have never really known anyone go from being healthy to transplant, only ever met people while they were waiting for the transplant or had already had one. I always wonder, like where were your PFTs 1 year before, 5 years before? I just it's hard for me to wrap my head around sometimes. I mean I've had my own decline, but I'm no where near transplant yet thankfully, and I just always wondered how drastic or fast it is? Or can it happen quickly or slowly? I just don't understand. lol. I'm sorry if this offends anyone, I'm sure it's a touchy subject, but I would appreciate any answers.

Sorry if this post was rambly or didn't make sense. I am anxious asking this.

 

[Kyrav]

I was actually really surprised at how fast it happened to me, I had never really heard of anyone all of a sudden just getting way worse so I think it's a fair question to ask.


In march last year my baseline was in the mid 50's and I was not on oxygen. In April I had a really bad exacerbation and started having to wear oxygen at night. Then every month after April I was being admitted and during that time my baseline dropped to the low 30's. My cf drs told me they start discussing transplant when pft's are around 35% and put me on oxygen all the time. So in January I decided to start the evaluation. I just had my last test for eval on Monday so now I just have to go back to my transplant center and meet with some more people and wait to hear if I made it on the list. Oh and I just had pft's last week when I got admitted and they were 29%.

 

[cfowler]

I think its different for all CF patients, some drop quickly, others drop over a period of time. Me, I have been between %25 ad %30 for the last 8 - 10 years. I have never been on Oxygen and did a tuneup about every 6 months. As of last year, my PFT's dropped to %24, I am now on Oxygen and as of April 16th this year, I am now on the transplant list. My PFT's last month were %18, but they jump back to above %20 after a tuneup, but I now need a tuneup every 2 to 3 months instead of 6. But I was stable and fairly healthy for those 8 - 10 years at %25 to %30. Even now, I struggle to walk up stairs, but I can still get around and I still work a fulltime job, but I am on 2L of oxygen 24/7.

 

[keefer11]

To be honest, I'm curious as well Lena Bean, as I hear stories of people going in for "routine" clean outs and then coming out of the hospital with severely reduced lung function or don't come out alive. I just wonder do the people who get admitted and then pass away in the hospital have low FEV1's to begin with? What happens? It all confuses me, too.

I'm not answering for myself as I am not near the transplant stage, but I can explain how it happened for my sister. Her decline was somewhat rapid. She was 23 She had gotten a nasty flu and simultaneously was starting to exhibit symptoms of CFRD. Her doctor was ignorant and even though she had a random glucose test that had a result of 180, he refused to believe she had diabetes and wouldn't test her nor treat her for it. So basically she was on IV's that weren't being effective for the secondary infection she got from the flu and she had Diabetes that wasn't being treated. (I'm not sure if the flu caused so much damage because she hadn't treated it right away or if it was just a nasty virus that would have damaged her lungs no matter how fast she got treatment) From that combination her FEV went from 70 to 40's. Because I started testing her blood sugar(I was diagnosed with CFRD at 13) we convinced her doctor that she had diabetes and he finally started to give her medication so she could control it. Once that was stable and she finished off the IV's she was able to keep her health relatively stable for about 1-1.5 years and then started the evaluation for transplant. She was transplanted about 3 years after her mishap with the horrible flu/undiagnosed Diabetes. I think her FEV1 right before transplant was mid 20's. So in 3 years she went from lower 70's to mid 20's.

My doctor tries to tell me that it's "normal" to lose about 4% FEV1 every year once you are an adult....but it still makes me paranoid!

 

[kyeev]

If no one wants to answer that's fine. I'm honestly just curious. How did your decline leading you to need a lung transplant go? I am moderately healthy for a CFer, and have never really known anyone go from being healthy to transplant, only ever met people while they were waiting for the transplant or had already had one. I always wonder, like where were your PFTs 1 year before, 5 years before? I just it's hard for me to wrap my head around sometimes. I mean I've had my own decline, but I'm no where near transplant yet thankfully, and I just always wondered how drastic or fast it is? Or can it happen quickly or slowly? I just don't understand. lol. I'm sorry if this offends anyone, I'm sure it's a touchy subject, but I would appreciate any answers.

Sorry if this post was rambly or didn't make sense. I am anxious asking this.

Essentially the statistics show if you have an FEV1 below 30%, you have a 50% chance of dying within 2-3 years.
Or to put a positive spin on it, you have a 50% chance of still being alive at 2-3 years. Yay!
I found the following info really helpful http://decisionaid.ohri.ca/docs/das/CF_group_a.pdf

Age of course is a factor, if you're needing a transplant in your teens or 20's, then you will have to have had a rapid decline.
If you're in your 30's or 40's, chances are it will be a more gradual decline.
I know that doesn't really answer your question though.

Older patients tend to do better than younger patients after transplant, probably due to less aggressive immune systems although it can vary from patient to patient i.e. older patient's immune system didn't destroy their lungs until they got into their 30's or 40's, so doesn't attack their new lungs as much.

I haven't had a transplant yet, I'm in my late 30's but will definitly need one in my 40's, so I guess I'm a slow decliner.
I'm sure treatment adherence can help some what, getting flu vaccines etc.
But it all really depends on your genetics and predisposed stuff like your immune system activity, cardiovascular strength and maybe even CF centre expertise and access to the latest meds.

 

[markinohio]

I am 44 and was evaluated for transplant about three years ago. I was not sick enough yet. About four years ago my PFT's dropped to the mid 30's. I'm not sure how high it was before that. I held steady in the mid 30's for about four years, then in April I got sick with something and now I dropped to the low 30's. Not much of a change, but enough to tell my breathing and my energy level are different. I also have diabetes and culture cepacia. I think there are many factors involved that determine how fast the decline occurs. I am very compliant with my treatments, I try to eat healthy and stay active as much as I can. I think a positive attitude helps too, up to a point. I may be re-evaluated again soon.

 

[Lauraslungs2]

I have been post-transplant 15 years. (62). I was a slow decliner. About 8 years before my transplant, I had several bouts of lung bleeding and felt I was starting to decline a tad. I also got a very bad cold in 1992 and ended up on oxygen 24/7. My lung functions took a big hit. It took another year to stabilize me. In 1996 I went on a transplant list and waited 2 years and 4 months. My Fev1 was about 30 percent. At the time of my transplant, it was down to 16 percent and felt I was very close to death. I was 47 at the time. You can never believe how different you will feel afterwards. I explain it to people in this way: I jumped into someone else's body. I sincerely hope you are far from transplant and stay healthy for a long time. Best of luck, stay healthy!

 

[CrisDopher]

I have declined fairly quickly. From being a runner with FEV1 in the 50s to being unable to run, FEV1 under 20, and on oxygen full-time has been less than a year. In fact, I was still doing "fine" at my birthday last September, but a few months and a couple of bad exacerbations later, and I was listed December 11th. I'd already long since slipped under the 30% FEV1 mark, but started needed oxygen at that point (around Thanksgiving), and that's what finally tipped me into transplant territory.

 

[azdesertrat]

I went quick. I went from working 45-60 hrs a week to being unable to work in a matter of 3 months. Fortunately, I was only listed for 6 mos. & 1 day before I received my transplant. There is no way I would have made it for another month if I hadn't received my trans. when I did. Unfortunately, it looks like I'm in decline now; almost 8 yrs post-trans. My trans. didn't go quite as good as planned. 3-1/2 yrs. post-trans, I had to have a bit more than half of my rt. donated lung removed. Since then, I've been going around with bouts of GI issues, including most recently a bout with 'C-Diff'. Not sure how much more my poor little body will take. Regardless, I'm tremendously grateful for the additional 8+ yrs. I've been so privileged to have. No one gets outta here alive..

 

[kyeev]

I went quick. I went from working 45-60 hrs a week to being unable to work in a matter of 3 months. Fortunately, I was only listed for 6 mos. & 1 day before I received my transplant. There is no way I would have made it for another month if I hadn't received my trans. when I did. Unfortunately, it looks like I'm in decline now; almost 8 yrs post-trans. My trans. didn't go quite as good as planned. 3-1/2 yrs. post-trans, I had to have a bit more than half of my rt. donated lung removed. Since then, I've been going around with bouts of GI issues, including most recently a bout with 'C-Diff'. Not sure how much more my poor little body will take. Regardless, I'm tremendously grateful for the additional 8+ yrs. I've been so privileged to have. No one gets outta here alive..

Pat, I really like your "No one gets outta here alive" quote. Good one.

CrisDopher, what age were you when this decline happened?
50% down to 20% in 1 year is catastrophic.

 

[Grendel]

I have been post-transplant 15 years. (62). I was a slow decliner. About 8 years before my transplant, I had several bouts of lung bleeding and felt I was starting to decline a tad. I also got a very bad cold in 1992 and ended up on oxygen 24/7. My lung functions took a big hit. It took another year to stabilize me. In 1996 I went on a transplant list and waited 2 years and 4 months. My Fev1 was about 30 percent. At the time of my transplant, it was down to 16 percent and felt I was very close to death. I was 47 at the time. You can never believe how different you will feel afterwards. I explain it to people in this way: I jumped into someone else's body. I sincerely hope you are far from transplant and stay healthy for a long time. Best of luck, stay healthy!

I am scheduling an interview with a TX-Center, and I am at 37%. I think it is a waste of time with my FEV1 so high, but my physician and her PA are strongly urging me to make an appointment now. I've too have had my lungs bleed, one that put me in the ICU for 3 days. I am told that the urgency is due to the lung bleeds, that bleeding alone can put you on the list. The CF docs are telling my wife I need new lungs now, which is creating false urgency from my perspective. Any input on how bleeds affect the process?
-
As for the original question, I had high FEV1 numbers (70-80s) till I picked up multiple parasites in Mexico. After that I dropped to 50% in <12 months. I hovered between 50s and 40s for about 6 years up until the past year and my numbers have dropped due to ineffective tune-ups. So now I am at 35-37%.

Grendel

 

[musclemania70]

For me it was super quick though I knew it would come eventually.
I was compliant 99.9% of the time.
Iwas holding steady at about 35% for a couple yèars with reg hospitalizations and o2 at night.
I went in for my reg iv treatment and suddenly started having severe pain and my body was withholding co2-NOT GOOD.
Within a week they said I needed to go to my tx ctr to try and get listed. Rushed by ambulance at 90 miles ph and tons of morphine later, I arrived. After my eval was completed, I was listed and had new lungs within 2 wks.
They gave me less than 2 wks to live if I didn't get lungs.
The tx nurse said that was the highest # she ever saw a person get listed at. I was on eccmo.

My husband saved my life and kept me alive.

 

[Nicole Emily Buckley]

My BF, Patricks health declined very rapidly. He was in the high 60's in August 2012. In September 2012 his left lung collapsed and left him in the hospital for about 6 weeks....ever since his FEV score has been int he 30s and just recently they have been anywhere between 21-24%...he is currently on the donor list @ USC and has been for almost 3 weeks. He is on oxygen 4L 24/7 and he gets tired very easily! Praying for lungs soon!!!! <3 He is approaching his 27th birthday in 2 weeks...hes got a life left in him...we are ready

 

[BreathinSteven]

Hey Lena Bean! I agree with some of those who have said we're all different - but that doesn't help you much... I've seen people's disease progress relatively slowly - and seemingly predictably - and I've seen people I assumed FAR healthier than I, crash & burn. I was pretty compromised, but kinda stable for 3-4 years, probably between 30-40% capacity. When I was 35, I collapsed my right lung and it never quite came up to near where it was - I would guess my capacity went down to 20-25%. When I was 36-37 I had a bad hemoptysis - that left me on oxygen 24/7 and being listed for lungs. (I probably could have used the oxygen long before - but there's that whole vanity thing...) After that hemoptysis I was hovering around 10-15% capacity. I waited almost 3 years after that for my lungs - though most were waiting about a year. (Simply, my size & blood type didn't come up...) The wait was different in 1997-2000, it was "first come first served" and not the LAS / lung allocation score system we have now, that was put into effect in 2005 and greatly benefits you as you become sicker... I was transplanted 4 days before my 40th birthday. Like so many of us - I never really understood what it ever was like to breathe "normally"... I've had my beautiful, new lungs for 13+ years now and this still blows my freaking mind, Lena... Love, Steve

 

[athletixbc]

My lung function was very good 80% and above until I was 27 years old. In my 28th year I had five hospitalizations within a seven month period to treat a very aggressive double pneumonia. My lung function dropped to about 60% and stayed there for three years. Then I dropped about 10% a year for then next three years. Now 33, and hovering at 30% lung function, I was placed on home oxygen. I fluctuated between 25-30% until I was 37. This is when I had a big scare. I went in the hospital for what was supposed to be one week of tune-up IV antibiotics followed by two more weeks of IV at home. Four days into my tune-up I had a major attack where I just couldn't catch my breath. I remember using the call bell, having several nurses/doctors around me, and then I woke up four days later in ICU. I remained in ICU for nine days, and was told by my doctors that I wouldn't be going home. Instead, they were arranging for me to be fast-tracked for the transplant waiting list and they flew me by air ambulance to the transplant centre. I was classified as a top priority, meaning they thought I could have another spontaneous attack at any time and that I might not survive another one. About six weeks later they found a donor and I was transplanted. It's now been five months since transplant and I'm doing great. Lung function is 106% of predicted!

 

[B-Boo]

Hi, my name is Linda and I'm pretty new to this group. I usually just read and hardly ever post. But thing decline thing kind of caught my eye. My son is 16 and has CF. He has always done great until this last year. His numbers were always good but at our last visit he had dropped from 74 to 57. The doctor says that his small airways are blocked. He is on Clostine rotting with the TOBI podhaler. He hates to exercise. He says he feels fine. We go back in December for check up. Not sure what they will do if his numbers don't improve, maybe admit him over winter break would suck. Just worries me to death.

 

[athletixbc]

He hates to exercise. He says he feels fine. We go back in December for check up. Not sure what they will do if his numbers don't improve, maybe admit him over winter break would suck. Just worries me to death.

He has to find some exercise somewhere or his lung function will likely drop more quickly than it should. Find something physical he likes to do, be it walking, biking, skateboarding, rollerblading, dancing, swimming, etc.. Exercise doesn't mean he has to use a treadmill or stationary bike or go to a gym. It just means he has to get off his butt and get his heart rate up for at least 90 minutes a week, preferably in at least three sessions.

I know you're the one in the forum and not your son, and I also understand that teens often tend to ignore their parents. Do you think it might help if he heard it directly from another CFer instead of second hand through his Mom? I'd be happy to share my Facebook, Twitter, Skype, email with you to pass on to your son if you send me a private message.

 

[CyrilCrodius]

Examples from people I know/knew :
This one kid was doing great until he entered high school, where he became infected with aspergillus, to which he was allergic. If I remember correctly, he lasted a year and a half, maybe 2.
Pneumonia leaving permanent damage
Flu leaving permanent damage

 

[laneyb]

Hi All, I hope you are doing good and those who are sick get well soon. I think that everyone is different and not all CFers decline rapidly or the same at all. There are CFers on this site that haven't had their decline rapidly and haven't needed a transplant and I'd love to hear their stories! As a CF lady in her 30s I do get scared sometimes thinking about getting older and declining!!

I changed my life and my health when I moved from Ireland to France. When I lived in Ireland I was having IVs every 2nd year. Since we moved to France I haven't needed IV's and that was 3 years ago now!! My doctor said my inflammation markers keep declining and all test results have improved greatly since moving to this south of France warmer climate. I like to think that not all CFers need a lung transplant and not all might decline health wise so rapidly. I'd love to hear from any of the older CFers on this site that are doing well and have not had a transplant. I know everyone is different and some decline more rapidly than others but some stay fairly well into their 60s and 70s. Please God there is a break through in CF research very soon. There are amazing things happening now with new medication and trials I know that but please God the day is near when all who suffer with CF can benefit from new research and medication that can stop CF symptoms once and for all!!