Starting the process for transplant... get listed or wait for Kalydeco/VX-809?

[LindseyRose]

And so it begins....

I have my first appointment to start the evaluation process in about a month. Up until this point, I have always known that transplant was something I would definitely want if the time came. I am now starting to wonder if in fact it is something I want. I feel like I am in a delicate situation. I am double DF508, so all this talk about the new meds coming up are really exciting to me and I am staying cautiously optimistic. My lung function is currently at 27% on a good day, and I have been holding steady in the 25-30% range for probably 2 years now. I still work full time, I maintain a somewhat normal lifestyle (obviously have some limitation due to shortness of breath, tiring easily), and only need oxygen at night or when I really wear myself out. My CF doctor agrees with me that on paper, as far as numbers go, I should definitely be on the transplant list. But as far as my functionality and my every day lifestyle, I do not need the transplant yet.

Part of me wants to wait it out for the Kalydeco/VX-809. I feel like I could maintain my current function until it comes out. I just worry that if I get a bad infection it could do me in and really drop my numbers significantly. What I don't want to happen is I go through with getting listed, my time for transplant comes, I get new lungs, and then this med gets approved. As much as I would love to have new lungs and have a shot at really knowing what it's like to be able to breathe with no problems, I'm really scared that something will go wrong and I'd be taking the chance that I wouldn't be a success story. I realize that these new meds probably aren't going to give me super high lung function. But I am living a decent/happy life with what my function is now. I would much rather have Kalydeco/VX-809 allow me to live the rest of my life at just a little higher than what I am now, than to jump the gun with a transplant, have the much desired high lung function, but only survive a few years after.

I would love to hear thoughts from anyone else in this situation, or anyone who has gone through with the transplant already. Knowing how your life has changed, would you take the chance on transplant if you knew these great things were looming on the horizon?

 

[markinohio]

I am not in your exact situation, but close. I only have one copy of 508. I don't know if any of the new drugs coming out will help me before I get worse. I know I have alot of lung damage and I don't think any of the new drugs being tested will reverse lung damage (I could be wrong). My lung function is in the mid 30's and I am on O2. I have been evalutated for a transplant, but am not listed yet. I know a transplant would improve my quality of life, but I think the odds are I wouldn't be around too many years after the transplant. I also have cepacia. I am going to keep doing everything I can to keep from declining and hope something comes along to improve my baseline. I will try to hold off the transplant for as long as I can.

Mark

 

[LindseyRose]

Thanks for your reply Mark. You bring up a question I have often wondered. While I don't think that the meds themselves will reverse lung damage, I wonder if over time, since they supposedly "stop" the progression of the disease, will the lungs start to regenerate? When people stop smoking the damage to the lungs improves, so my thought is the same would be true if mucus is no longer eating away at the lung tissue.

 

[cftransplant]

Lindsey: Delay as long as possible. TX is a one way door, new issues, new time line, new clock ticking down. Just don't delay a day too long.

In 2000 I had zero options and no regrets. From the past, new drugs that will offer a "cure" have been around the corner for at least the last 25-30 years. Hope the new med works, but from the contra indications, long term use may well have other issues. Perhaps using the new medication will prolong the choice for the final TX option, ask the doctors and see if you can try and delay the TX options, I sure would research and try this pathway.

Mark: reasoned thinking, delay and keep your conditioning up. What is your definition of you would not be around too many years post TX?

CF TX 62 cepacia (since 1994) 12.5 years out (on a limb hearing chain saws)

 

[Aboveallislove]

I'm really not familiar with all the ins and outs and can only imagine how hard this is for you. I thought, though, from reading lots of other posts that you can be evaluated for transplant and then "not active," but then that you could go active if need be. I "know" someone who was listed and then went "not active" (or whatever it is called) after being on Kalydeco (she's g551). It would seem that doing this might be the perfect "middle ground." hugs and prayers.

 

[markinohio]

cftransplant- my reasoning for saying that I would probably not survive long after transplant is due to the many articles and findings on the internet regarding cepacia and survival rates, predominately cenocepacia. I have cenocepacia.

Here is one I found from 2010.[h=4]RESULTS:[/h]"Two hundred sixteen patients with CF underwent lung transplantation and 22 had confirmed pre-operative BCC infection, with 12 of these being B cenocepacia. Nine B cenocepacia-infected recipients died within the first year, and in 8 BCC sepsis was considered to be the cause of death. Despite instituting a tailored peri-operative immunosuppressive and microbiologic care approach for such patients, post-transplantation BCC septic deaths occurred frequently in those with pre-transplantation B cenocepacia infection. In contrast, recipients infected with other BCC species had significantly better outcomes, with post-transplantation survival comparable to other recipients with CF."

I can see by your signature that you have survived much longer and I know that is possible for me too. I hope to live many years after my transplant, but I see that many people have not.

 

[CrisDopher]

If you're at the transplant stage, it is too probably too late for any Vertex drug to save you from that, even if a perfectly-targeted delta-F508 drug hit the market TODAY. On the other hand, I'm exactly where you're at - just started on o2 yesterday in fact - and could see living 40 more years this way if I could avoid the dangers of rejection. However, my exercise capacity has been greatly reduced and though I think I can build that up a little bit, I worry that I won't be able to exercise enough or well enough to stave off the ravages of time, in the way that exercise is just generally good for everything. I don't want to die at 50 of a heart attack, you know? Even fighting rejection, a transplant gives me back that capacity to fully exercise and become an athlete again. Maybe you have similar thoughts?

 

[Ltlave1114]

LindseyRose: a few months ago I was in the exact same situation as you, besides I work part time. I went for all of the testing to of the lung transplant evaluation, and while I was there, I more or less had an epiphany... I came in contact with another individual who ha CF and he was much worse condition than I was. He was in a wheel chair, on oxygen, and looked like he was truly fighting for his life. I then came to the realization that, although I have difficulty breathing at times and am unable to do some of the things I use to, like walk up a flight of stairs without stopping to catch my breath once I get to the top, I am still able to work a part time job, enjoy socializing with my family and friends, and pretty much have a "normal" life. Not to mention, while I was there, I felt better than I have in a couple of years. So after this experience, I came to the conclusion that I myself was not ready for this challenge because I still have so much I am able to do. Also, the idea of getting a transplant and going into rejection at any point in time, post TX, scares me more than anything in this world, because I would rather try to maintain my lung function and do the best I can with my lungs than be challenged with the chance of my life coming to an end so unexpectedly. And especially with the new drugs coming out I would rather sit around and wait for them, as long as I am able bodied that is what I shall do. I wish you only the best in whatever choice you may make, because it is most certainly a life changing experience.

 

[bigstar]

Well i postponed replying here because i was trying to think this over. Again and i again. What would i do if it was me we were talking about?
New drugs give us hope. Proven and accepted. Transplant is always an option.
-I would start exercising to get my pfts up. I would start exersising to build up my stamina.
-I would consider all the things that im still able to do like mentioned here before.
-I would consult another doctor. Even if mine was perfect, i would get a second opinion and a third and a fourth.
-I would get evaluated -just in case- and i would wait!
But thats just me! Thats what i would do. I hope you make the right decision! We are here for support no matter what!

 

[Cburge02]

And so it begins....

I have my first appointment to start the evaluation process in about a month. Up until this point, I have always known that transplant was something I would definitely want if the time came. I am now starting to wonder if in fact it is something I want. I feel like I am in a delicate situation. I am double DF508, so all this talk about the new meds coming up are really exciting to me and I am staying cautiously optimistic. My lung function is currently at 27% on a good day, and I have been holding steady in the 25-30% range for probably 2 years now. I still work full time, I maintain a somewhat normal lifestyle (obviously have some limitation due to shortness of breath, tiring easily), and only need oxygen at night or when I really wear myself out. My CF doctor agrees with me that on paper, as far as numbers go, I should definitely be on the transplant list. But as far as my functionality and my every day lifestyle, I do not need the transplant yet.

Part of me wants to wait it out for the Kalydeco/VX-809. I feel like I could maintain my current function until it comes out. I just worry that if I get a bad infection it could do me in and really drop my numbers significantly. What I don't want to happen is I go through with getting listed, my time for transplant comes, I get new lungs, and then this med gets approved. As much as I would love to have new lungs and have a shot at really knowing what it's like to be able to breathe with no problems, I'm really scared that something will go wrong and I'd be taking the chance that I wouldn't be a success story. I realize that these new meds probably aren't going to give me super high lung function. But I am living a decent/happy life with what my function is now. I would much rather have Kalydeco/VX-809 allow me to live the rest of my life at just a little higher than what I am now, than to jump the gun with a transplant, have the much desired high lung function, but only survive a few years after.

I would love to hear thoughts from anyone else in this situation, or anyone who has gone through with the transplant already. Knowing how your life has changed, would you take the chance on transplant if you knew these great things were looming on the horizon?

i am in the same position but I choose to go with transplant.. My doctors said that even if Kalydeco comes out for us (double f508) it won't significantly help.. Our lungs can only heal up so much and with us being in the 20's it's not going to be a huge jump.. When it comes to transplant I'm not sure what your doctors have told you but always keep in mind in the back of your head that there just numbers when it comes to yrs of living after transplant.. People live 20+ yrs after a transplant it all depends on that person.. So keep a positive outlook.. I've talked to many double lung transplant people with cf and they said it was the best decision they ever made.. I haven't heard 1 bad thing.. You get your life back, no more o2, weight gain, travel anywhere, no more coughing.. It's a great choice.. And we are lucky that we can even have that option.. It's going to be the hardest decision you ever make but I'd much rather live 5 great yrs then 1 more out of breath miserable 1-2 yrs.. Remember they only ask you to do this if they know its the last option.. I hope it all goes well

 

[LindseyRose]

LindseyRose: a few months ago I was in the exact same situation as you, besides I work part time. I went for all of the testing to of the lung transplant evaluation, and while I was there, I more or less had an epiphany... I came in contact with another individual who ha CF and he was much worse condition than I was. He was in a wheel chair, on oxygen, and looked like he was truly fighting for his life. I then came to the realization that, although I have difficulty breathing at times and am unable to do some of the things I use to, like walk up a flight of stairs without stopping to catch my breath once I get to the top, I am still able to work a part time job, enjoy socializing with my family and friends, and pretty much have a "normal" life. Not to mention, while I was there, I felt better than I have in a couple of years. So after this experience, I came to the conclusion that I myself was not ready for this challenge because I still have so much I am able to do. Also, the idea of getting a transplant and going into rejection at any point in time, post TX, scares me more than anything in this world, because I would rather try to maintain my lung function and do the best I can with my lungs than be challenged with the chance of my life coming to an end so unexpectedly. And especially with the new drugs coming out I would rather sit around and wait for them, as long as I am able bodied that is what I shall do. I wish you only the best in whatever choice you may make, because it is most certainly a life changing experience.

I think you hit the nail on the head. THERE IS STILL SO MUCH THAT I AM ABLE TO DO! I know that I am blessed to still be able to do so much, and maybe I feel a little guilty for even considering transplant when there are MUCH sicker people out there than me. But, then again, I am also stubborn as all heck so maybe I'm just turning a blind eye to the fact that I am getting closer to needing it. I am really concerned that I will be shooting myself in the foot. I like to think of myself as a generally optimistic person, and like I said, have always known that transplant was something I would pursue. But for some reason I am now doubting that, at least at this stage. I know I would be able to handle the surgery, I have a great support system, and I don't doubt that it would be incredible to breathe without problems. The ONE thing I look forward to the most? LAUGHING again! Oh, how I miss being able to have a good, hearty belly laugh. The kind where your stomach hurts so bad, you pee your pants, and every time you compose yourself you think of what it was that was so hilarious you break out in hysterics again.

 

[LindseyRose]

Thank you all for your replies and experiences. It's great to hear opinions from people who are actually going through this. I have so many people in my life that are very "Team Transplant", and every time I try to voice my concerns and reasons for hesitation they make me feel like I'm just being negative. I will definitely try to maintain (or better yet, improve) my lung function for as long as I can, and remain cautiously optimistic that good things will be around the corner. If not, at least my foot will be in the door with the transplant team if/when the time does come that I truly need new lungs.