Pirfenidone

[Ambiman]

I have been told I have end stage ILP and may..... Be offered pirfenidone has anyone any experience of this drug and does it help are the side effects worth it

What about transplant as an alternative?

[imelda16]

Ambiman Hi

I will be starting Pirfenidone next week and I have IPF my condition is classed as mild to moderate and falls in the NICE Guidlines for treatment . I use oxygen for outside when walking and hope the drug will keep me stable and not go worse transplant has not been mentioned but at my age would not want it. Others on this forum have spoke of the side effects but I think it's got to be up to how each of us feel when taking it. The Respitary Nurse has given me lots of info so it's fingers crossed. Hope this helps.

[Ambiman in reply to imelda16]

Thanks for that imelda 16 I haven't been offered it yet but I am obviously concerned what the side effects will do. I haven't spoken to a Respiratory nurse yet I was told they can give lots of information.

As I am 63 I wonder if I should spend time on the pirfenidone or push towards transplant?

Thanks again and please let me know how you get on.

[jazztrain12 in reply to imelda16]

I have been on Pirfenidone since 2015 yet don't get any of the so-called side effects? They put me on Lansoprazole to counteract Reflux. I used to go to Papworth every 3 months but now I go every 5 months so can't be doing too dad I suppose? I am now 73, take regular coach holidays and still keep the old brain cells active involving myself in different activities.

Hope you do the same.

Kind regards

[imelda16 in reply to jazztrain12]

Jazztrain glad Pirfendone is helping you with your symptoms it does help some I'm now on Nintedanib which has been better for me and only have to take one am and one 12hrs later . We used to go all over on coach trips but being on oxygen 24/7 makes holidays difficult now lots of planning and organisation of supply from Airliquide. Opt for keeping brain cells working and going about when I feel ok These days seem to get more chest infections now so tend to avoid crowds but still enjoy plenty of other things.

[Cherries6]

I believe NICE recommends perfenidone in the earlier stages of the disease. My dad is in the end stage and we saw a fantastic consultant yesterday who did say he would have been willing to consider it if we wanted, but that it would not be of benefit at this stage. Everything I've read confirms this so I believed him, but everybody is different so what's right or wrong for my dad Could be different for you. He is 85 and not well enough for surgery nor would he want any more hospital admissions, but you are young. Are you being seen at a specialist centre? They will discuss everything with you very fully and help you make decisions about what is helpful for you. My dad believes that N-Acetylcysteine (Flumil) helps him and it's the only medication he takes (for his IPF). I hope all goes well for you Ambiman.

[Ambiman]

Hia cherries6 thanks for the reply sorry about your dad I am seeing a consultant in a general hospital at the moment she used a calculator to see if I could have perfenidone that was ok. But I will only get to see a specialist nurse if they put me on it, I am going for rehab on Tuesday though.

it really is the side effects that worry me and should I push for transplant. I don't use any oxygen at the moment.

THanks for your comments hope your dad stays well.

[Cherries6]

Hopefully Ambiman your disease may not be as advanced as you fear, if you are still within the criteria for perfenidone. The specialist nurses are very knowledgeable and seeing them shouldnt be dependent on your taking a paricular medication I would have thought? I found the BLF helpline people very helpful when I rang them with plenty of time to talk so I would suggest you ring them. Sorry I cant be of more help re lung transplant but once you have the information you can weigh up the pros and cons. We think my dad had IPF for a number of years before diagnosis ( it was probably masked by another condition he has) so you may remain quite well (relatively I mean! ) For quite a while and have time to make the decisions you need to. All the best.

[lindacolman14]

Hi

I started Pirfenidone early April and seem to be tolerating the full dose daily. I will be doing lung function tests at the Royal Brompton next March when they will be able to tell me whether or not this drug is working. If it is not halting the progression of fibrosis then I shall be taken off of it. I have been referred to the Papworth for a transplant but there are many hurdles to jump before you are considered for the waiting list. I am 63 and the cut off age is 65. This is a massive operation and I am unsure whether it is the best option for me. If the Pirfenidone works and I can manage on ambulatory oxygen, this may be the best alternative for me.

Keep as well as you can

Linda

[Ambiman]

Hi

Lindacoleman 14 thank you for your reply it has helped a lot it firstly has told me I may be able to tolerate perfenidone and secondly that it may be wise to get on the transplant list in the mean time.

Just in case as a back up although the thought of the operation is frightening .

Thanks again

[Salmo]

I've been on pirfenidone for just over 9 months and would certainly say it is worth trying. You may get side effects - you may not. A friend of mine had no side effects on pirfenidone while I had a lot of side effects (nausea/fatigue) at the start but these have now largely disappeared. Just hope the drug is slowing down my disease progression.

The other drug you may be offered is Ofev/nintedanib. Not much to choose between the two in terms of efficacy in clinical trials or side effects (predominantly diarrhea vs nausea with pirfenidone ?).

I'm surprised your consultant has not discussed a transplant if you have been told that you have end-stage disease. I'd get those discussions started ASAP as from my experience it can take up tor 6 months to even get refered for a transplant after having the first

discussions with your consultant. I'm sure there will also be a long

delay after that before the transplant centres can see you.

As an aside it seems strange to say you have end stage disease given that nobody has a clue about these diseases and how they progress (or how you will respond to drugs).

[Ambiman]

Well I have been on Perfenidone for 2 1/2 years now my figures have now dropped below the criteria so that took if off me 4 months ago. In the time I was on it they were prepping me for transplant assessment and found a cancer in my left lung, didn't hesitate had a wedge resection. I guess you can say I'm fine ....I am ok I'm on liquid o2 only ambulatory on 6/8 lts.....do you know I do feel fine ! My vlc has gone from 84% to 44% and dclo down to thirty something. My motto is keep going and I do. I am on a trial too,double blind so I might be taking sugar.

I'm also proud of my son and fiancé who with help of others raised getting close to £3000 for the BLF specifically for research into IPF.

[Salmo in reply to Ambiman]

Thanks for clarifying things. I had thought from your initial post that you had not been on any medication or had discusssions about transplants.

Had no idea there were any trials on drugs for IPF going on in UK - hopefully whatever you are on is the real thing and will help.

Really good news that your son and fiance have raised so much for IPF research - shocking how little research is done on this disease.

Given that you may not have responded to pirfenidone I wonder if your consultants have considered trying you on nintedanib/ofev when the current trial ends ?. Different mechanism of action and some pre-clinical test tube evidence that some people may respond to Ofev but not to pirfenidone (and vice versa). To be honest I had hoped this would be done for me if my FVC fell by more than 10% a year (or whatever the current criteria is for stopping pirfendione treatment). It seems worth a try and a far better approach than simply stopping treatment.

[Ambiman in reply to Salmo]

Thanks for that but the criteria for nintedtanib from nice is the same as Perfenidone so they won't give me that. It does seem odd that both treatments have the same cut off but I'm sure that is about finance, they think if it hasn't worked by then it won't as the figures decline.

The trial is co- trimoxazole (it's an antibiotic used in the treatment of pneumonia) if I wasn't on that I would be on nothing now so if it helps even gathering information what's the harm. All Pumonary health specialists have the information they need 300 (at heartlands I am 002!) plus I don't think they push it you have to ask, that's all I did, well there was nothing left.

[Salmo in reply to Ambiman]

Many thanks for the additional information. I've have learnt a lot from this post. Glad to here that you are well along the transplant pathway as an option and also on the long term antibiotic treatment trial - seems to make a lot of sense, particularly in patients with advanced disease, and beats waiting a week for a doctors appointment when you get a chest infection.

I'm really horrified to learn that there is a FVC level at which you are not allowed to either start or continue taking pirfenidone or nintedanib. This makes no scientific sense as I'm pretty sure nobody has shown that fibrosis magically becomes resistant to pirfenidone and nintedanib when your FVC falls below 50%. There is even some evidence that pirfenidone is just as effective in IPF patients with FVC below 50% (and/or DLCO below 35%) as in patients with better lung function. I can perhaps understand pirfenidone being stopped if you are still declining when taking it but would have thought that you would be automatically switched to nintedanib in this case. Guess the guidelines must be there for cost reasons as you suggest - certainly no other reason I can think of unless the doctors think nintedanib and pirfenidone are the same drug or have the same mechanism of action. Excuse the rant but the more I learn and experience about the general approach to treating (and studying) IPF the angrier I get.