Now Ye're Talking - To a Cystic Fibrosis patient & lung transplant recipient

Boards.ie: Niamh

This week's AMA is from one ouf our members who was born with Cystic Fibrosis. He was placed on the transplant list and was called to London to have a heart and lung transplant in 1995. Unfortunately the heart was not suitable but he received a double lung transplant and returned home to Ireland 10 weeks after his operation. He continues to attend hospital check ups to this day.

brianregan09 is the member who is doing this AMA for us. Since he was diagnosed and ever since, he has been a part of the Irish healthcare system so he may have some interesting insights into the health system as well as into cystic fibrosis and his transplant operation.

Thanks Brian

mocha please!

Hi Brian,

Sorry if this is a strange question, but do you know what happened to your own lungs after the transplant? And did you get to see them outside of your body afterwards?

Do you know anything about the donor(s)?

Medusa22

Hi Brian, thanks for doing the AMA, I really appreciate it. I think it is absolutely brilliant that you are doing so well 20 years after your transplant. I have CF myself and can I just ask if you've had any problems with rejection?

secondrowgal

Hi Brian

I am delighted for you, congratulations!

My nephew unfortunately died before he could have a transplant because he went from not sick enough to too sick very quickly. Do you think that the criteria for getting a lung transplant are too narrow from the point of view of not being "sick enough" to be put on the list?

yellowlabrador

my boyfriend had a liver transplant in 1997. It's great to see people benefit from this life giving procedure. know that in his case, he never looked back and from being on the edge of death, he has had a good and full life.
As a relative, it was very hard and stressfull, yet I had to stand back and let him get on with it. How did your family cope?

brianregan09

Hey all just want to thank Niamh aswell for setting this up and you guys for you're questions so far, I'll try my best to answer them as best as i possibly can for you

Hi Brian,

Sorry if this is a strange question, but do you know what happened to your own lungs after the transplant? And did you get to see them outside of your body afterwards?

Do you know anything about the donor(s)?

Okay so for this one , From what we were told after they were taken out they were medically disposed of , I did ask to see them but was told it wasn't possible and by the time I was well enough to talk (about 5 days iirc) they would have been well dealt with I presume

On the Donor question they have procedure for that as confidentiality and all that , So how it works if we want to contact the donor , We send a letter to the hospital and they send it on to the donor's family and then the ball is in there court, unfortunately for what ever reason they never sent a letter back , The most the hospital could tell us was it was an accident up north (we were placed just outside london about 30 minutes from Heathrow airport) so like when google and the likes came about i did a bit of reasearch and the only accident i could find was a 28 year old police constable up in Elgin in Scotland , So could be him but then again it might not

John_D80

Hello Brian, thank you for your time in doing this. I saw your post in the discussion thread and I was hoping you would be picked.

In your own words, what is Cystic Fibrosis?

In this day and age what is the long term prognosis and life expectancy for a child born today with the condition? Are there varying degrees of severity? How does it affect a sufferer on a day to day basis.

brianregan09

Medusa22 wrote: »

Hi Brian, thanks for doing the AMA, I really appreciate it. I think it is absolutely brilliant that you are doing so well 20 years after your transplant. I have CF myself and can I just ask if you've had any problems with rejection?

Hi Medusa and thanks for the great question , Sucks you have CF too its such an asshole of a disease at the best of times , As for rejection i think i've been lucky enough , While I was over in Harefield I was mid recovery and doing quite well and then had a major setback and rejection hit me quite hard and ended up needing a blood transfusion and all it was quite scary but I fought through it eventually , Since i came home though I've been admitted to hospital 3 times and once out of the 3 was only due to rejection which happened in the summer of 1999 i think and basically it was like a really really bad chest infection and afterwards they rejigged my medication in the Regional in Limerick under advisement from the docs in the UK and its seems to have done the job , The other 2 times were chest infections stemming from the usual cold going on too long and eventually going down into my chest

So anytime i get a cold now i go into lockdown mode lempsips and all that crack and make sure i shake the bloody thing, That doesn't happen too often though just the same as anyone else really I just have to be a little more careful

brianregan09

secondrowgal wrote: »

Hi Brian

I am delighted for you, congratulations!

My nephew unfortunately died before he could have a transplant because he went from not sick enough to too sick very quickly. Do you think that the criteria for getting a lung transplant are too narrow from the point of view of not being "sick enough" to be put on the list?

Hi secondrowgal and thanks for you're question and I'm so sorry for you're loss too , this is a hard question , In my opinion CF is a degenerating disease and I think everyone that has it should be entitled to another chance at life, Like speaking from my own experience our doctor is limerick was fantastic at the time from what i remember and from what my parents told me , It was last chance saloon for me as I was slowly getting sicker and sicker and despite his efforts getting trialed medications and all nothing was working and I think (now i could be wrong) that I had under a year left at the time if a donor didn't come through , The thing with CF is there is a very thin line between not sick enough and being too late , As any cold or illness can turn serious because of the total lack of a proper immune system , So to answer you're question from my own experience yes it is a very thin line and should definitely be revisited

brianregan09

yellowlabrador wrote: »

my boyfriend had a liver transplant in 1997. It's great to see people benefit from this life giving procedure. know that in his case, he never looked back and from being on the edge of death, he has had a good and full life.
As a relative, it was very hard and stressfull, yet I had to stand back and let him get on with it. How did your family cope?

Hey Yellowlabrador thanks for the great question and I think this is a funny one in a way I think i coped with it 100% better than any of them did and i was 11 lol , My mam and dad worked at very understanding jobs luckily enough for me so when the call came to go, They were both with me and both of them stayed with me for the duration i was over , My dad from the minute we went over started documenting everything basically writing a book , but the time I had the setback that time with the rejection which i mentioned in my last answer he threw it in the bin and never started up again because he made out that the more he wrote in it the sicker i was getting

Of course i scolded it for him at the time i told him why would ya do that you could sell it and we'd make loads of money ...(even in the darkest times i never lost my sense of humor) and my mam she wears her heart on her sleeve so she often broke down in front of me during the setback (at one point they thought i was feiced) but i constantly told her I was an O Regan and we never gave up (took it from granddad that saying) and no stupid disease was going to take me and by god I believed it as well, So yeah my mam and dad felt every step of the way , Luckily as I began to get better Uncles and Aunts used come to visit for the weekend it was great for me but I can only imagine how relieving it must have been for them at the time to talk to not only a family member but another adult with no white coat or English accent , They had befriended a family with a little girl in the hospital from Sheffield unfortunately though she didn't make it through the recovery so that hit them pretty hard too and i'm sure they thought something similar was coming for me when i had my setback

brianregan09

John_D80 wrote: »

Hello Brian, thank you for your time in doing this. I saw your post in the discussion thread and I was hoping you would be picked.

In your own words, what is Cystic Fibrosis?

In this day and age what is the long term prognosis and life expectancy for a child born today with the condition? Are there varying degrees of severity? How does it affect a sufferer on a day to day basis.

Hi John D okay for this question long term prognosis I think is changing all the time just lately there another drug called released in Ireland even (im not on it as i have a diffrent strain of it ) that is called Kalydeco i think and it has show lasting improvement in patients lung function tests, As for severity i don't know really any people outside of myself any more with CF (the ones i did died when they were kids around the time i got my transplant)

But from hearing on online forums and stuff, tv, radio etc there are people who live quite normal lives with cf (without transplants too) and there are people who go through life with an oxygen mask stuck to there face so ya definitely diffrent degrees of severity

As for me to give whats CF to me in my own words......to me its just a label...A label and a reason I take tablets in the morning and eveing nothing more I don't let it define me at all and never will if I died in the morning I would like people to know that I lead a normal life not led a CF life , and that's probably not the answer you're looking for but that's how i see it anyway

TheBody

Hi Brian,

Thanks for doing this AMA.

My question is what, if any, exercises or physical activities can you do?

GLaDOS

Hi Brian, thanks for answering our questions I actually worked with a girl doing her PhD on bacteria infecting CF patients.

I don't know if your condition and/or being an organ recipient precludes you from donating organs yourself, but if you could, would you like to be placed on the organ donor list?

Is your condition something you consider in terms of having children? (i.e. because they would have a high risk of of having CF themselves).

brianregan09

TheBody wrote: »

Hi Brian,

Thanks for doing this AMA.

My question is what, if any, exercises or physical activities can you do?

I think for anyone with CF its not a question of what you can and can't do speaking from own experience pre transplant , I still played with all the lads at school and stuff and got up to the same shenanigans but usually its accompanied by a gob of phlegm and coughing...Lots and lots of coughing

But since my transplant my energy levels shot through the roof I used be cycling around with my mates in my teens chasing after birds the same stuff anyone else would do and playing under 16's soccer was never a fan of anything else to be honest, Even as I got older the football continued and i have a junior soccer league medal to my name for me it all depends on how fit i get , Sometimes i get lazy

brianregan09

ScienceNerd wrote: »

Hi Brian, thanks for answering our questions I actually worked with a girl doing her PhD on bacteria infecting CF patients.

I don't know if your condition and/or being an organ recipient precludes you from donating organs yourself, but if you could, would you like to be placed on the organ donor list?

Is your condition something you consider in terms of having children? (i.e. because they would have a high risk of of having CF themselves).

Thanks for a real good question If I could donate organs I definitely would , It would be ironic sort of repaying the favour i think , But i'm not sure I could because of the damage done to me by CF

the kids thing is a hard one for me as males with CF cannot have kids without IVF , Would love to one day but we'll see , There is a difference though in having CF and being a carrier of CF i'm not a carrier i'm a sufferer so it wouldn't be possible for my kids to have it unless my partner was a carrier ..that's my understanding anyway

GLaDOS

brianregan09 wrote: »

the kids thing is a hard one for me as males with CF cannot have kids without IVF , Would love to one day but we'll see , There is a difference though in having CF and being a carrier of CF i'm not a carrier i'm a sufferer so it wouldn't be possible for my kids to have it unless my partner was a carrier ..that's my understanding anyway

Ah I did not realise that about needing IVF, another complication so. Yeah your partner would have to be a carrier. Would you consider asking them to take a DNA test to ascertain if they were a carrier or not before considering kids? Obviously a difficult question to answer dealing with hypotheticals .

spaceHopper

My understanding is that CF genetic and having a lung transplant resets the clock on the damage its done to your lungs but you still have cf is that right?

brianregan09

ScienceNerd wrote: »

Ah I did not realise that about needing IVF, another complication so. Yeah your partner would have to be a carrier. Would you consider asking them to take a DNA test to ascertain if they were a carrier or not before considering kids? Obviously a difficult question to answer dealing with hypotheticals .

No if we could have kids in the morning we'd do it , I'm living with my girlfriend and engaged for the last few years and we have talked about it and yeah we'd definitely have kids straight away and look if our kid did have CF who better to look after him ya know

brianregan09

spaceHopper wrote: »

My understanding is that CF genetic and having a lung transplant resets the clock on the damage its done to your lungs but you still have cf is that right?

Yep that is bang on , Having the transplant for lack of a better term gives the CF a new fresh pair of lungs to start infecting and you have the lingering threat of rejection down on top of that , But i've had it pretty good since the transplant but i still have all the cf traits

spaceHopper

How did affect school what about work and getting a mortgage

brianregan09

spaceHopper wrote: »

How did affect school what about work and getting a mortgage

School it didn't really affect outside of getting time off for appointments every so often which i loved because any excuse to get out of school is a good one

Workwise I went straight from Leaving cert into full time employment and lasted at that up until i was 28 , Then I went on disabilty allowance and I was still allowed work 12 hours a week in the local spar so that's what I'm doing now , Being honest I wasn't able to keep going at the 40+ hours a week I was banjaxed from it constantly and my mother found out I could go on disability allowance and my consultant approved

As for a house ...I got a council house 5 minutes from my mothers house i was on the housing list for about 5 years but got letters from Dublin, UK and Regional in limerick all pushing my claim through and i've been here 3 years and a bit now

vixdname

Hi there, It was discovered that I was a CF gene carrier a few years back and my wife had a genetic test to see if she to was a carrier.
Thankfully my wifes test came back negative so our two kids were born CF free.

My brother is a CF gene carrier also and they have been unable to conceive for many years.
Can you tell me why CF males can't have kids without IVF as I'm wondering if being a carrier of the CF gene has affected him in some way ?

Thanks a million !!!!

John_D80

brianregan09 wrote: »

Yep that is bang on , Having the transplant for lack of a better term gives the CF a new fresh pair of lungs to start infecting and you have the lingering threat of rejection down on top of that , But i've had it pretty good since the transplant but i still have all the cf traits

Wow that is scary. You're very brave. Fair play to you.

All that considered, is there a limit to the number of transplants that a CF sufferer can receive if their condition keeps destroying new lungs?

Or at what stage (if ever) will a medical team decide that they cannot keep giving new lungs to a patient?

Sorry if that comes across as ignorant or morbid.

brianregan09

vixdname wrote: »

Hi there, It was discovered that I was a CF gene carrier a few years back and my wife had a genetic test to see if she to was a carrier.
Thankfully my wifes test came back negative so our two kids were born CF free.

My brother is a CF gene carrier also and they have been unable to conceive for many years.
Can you tell me why CF males can't have kids without IVF as I'm wondering if being a carrier of the CF gene has affected him in some way ?

Thanks a million !!!!

Nothing to do with being a carrier anyway as my uncle in law is also a carrier and they have had kids no problem. Its a part of you're sperm tubing that bring the sperm from the testes up the the surface that's missing so while the sperm is there in the testes it has no way of reaching the surface

brianregan09

John_D80 wrote: »

Wow that is scary. You're very brave. Fair play to you.

All that considered, is there a limit to the number of transplants that a CF sufferer can receive if their condition keeps destroying new lungs?

Or at what stage (if ever) will a medical team decide that they cannot keep giving new lungs to a patient?

Sorry if that comes across as ignorant or morbid.

Well they have told me in the very distant future they may need to replace the left lung in me as it has suffered abit of damage in the 20 years since the last time , If that happens fair enough i've been through it before so it wouldn't worry me at all ,anything to keep life going to be honest , and that alone is why i'll never understand why people take there own lives (i know thats a whole other conversation)

trixiebust

Brian its great to read you're doing so well. Had a brother who died from CF in 1994, also a cousin shortly afterwards, it seems to run in my family. It really is a terrible disease. Do you ever see a day when a cure will be found for it?

brianregan09

trixiebust wrote: »

Brian its great to read you're doing so well. Had a brother who died from CF in 1994, also a cousin shortly afterwards, it seems to run in my family. It really is a terrible disease. Do you ever see a day when a cure will be found for it?

I'd like to think so , the way technology is coming along now with advances in cancer research flying now you got to think in the future a cure will be possible and i'm sorry for both you're losses

Ken.

Other than the kid's thing is their anything you'd like to do/try but can't because of your CF?.

If that's too personal just ignore it.

Dozen Wicked Words

Hi Brian,

What have your experiences been like in the Irish and UK health service? Things you'd like to change if you could?

Medusa22

Hi Brian, thank you very much for answering my last question. I'm glad that aside from some initial rejection that you haven't had too many problems, that is great to hear. I was just wondering what your lung function is like, your fev1? In litres or %?

moleyv

Hello.

I was wondering what support networks and services are in place for people with CF?

Like support groups, gatherings/clubs, one to one and that type of stuff.

And do you think there is enough support for people with CF?