British Lung Foundation
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Anyone being treated for Usual Interstitial Pneumonitis/Idiopathic Pulmonary Fibrosis?

Hi all,

Please forgive me as I just need to sound off a little to people who might have an idea of what I'm going through, and may have some suggestions.

I'll start with a bit of background....2009 I was taken ill at work with chest pains, I'd already noticed the severe shortness of breath on exertion or getting ready, brushing teeth etc, which I assumed was due to me being in my mid 30s, I'd also had a persistent dry cough for well over a year, I went to my G.P. about the shortness of breath, I had to see a stand in Dr who was covering holidays, and I was told to come back when the cough had gone, I did try and explain the cough had been there over a year, but was still brushed aside by the locum, I went back again and saw a different Dr who immediately arranged breathing tests. I was taken ill a couple of days later with the chest pains, CT scans showed scarring on the lungs, a biopsy revealed significant damage, but didn't provide any idea as to the cause, the biopsy also, apparently, didn't give a definitive answer to what condition it was because it had multiple characteristics so at an Interstitial Lung Disease MDT they chose to "treat it as Desquamative Interstitial pneumonitis", they started me on 30mg of Prednisolone per day, after many months of lung function tests they decided the steroids wasn't working and started me on 50mg of Azathioprine per day, this proved successful in slowing the progression right down, I was on Azt for a couple of years when on a consultation with a different consultant I was told I could no longer take it as it was no longer prescribed for lung conditions due to side effects, at the time I took them at their word, I thought "they're the specialists, they know what's best", they said they wanted to see how my body handled the inflammation, jumping forward a few years, I am still not on any treatment, lung function tests have shown steady continuous decline, although I have always been told I was "maintaining results", then I was seen by another different consultant who is now telling me I am seriously ill and the only treatment available to me now is a double transplant, she also stated that they now know treating my condition "as" DIP was the wrong way, it has now been decided that it is Usual Interstitial Pneumonitis which apparently does not respond to the treatment they tried with me, at my last consultation I was told my VC is 46% and FVC is 13%, I was told that as they now recognise my condition as UIP there is antifibrotic treatment available that would be helpful when the VC is between 80-50%, mine is 46% so therefore I "do not currently meet the criteria for the treatment" and at the last MDT it was decided that a transplant is the only viable option so I have had heart scans, more lung function tests, I am now on ambulatory oxygen as my sats drop to low 80s during activity, resting sats are fine. I'm a little annoyed to say the least, at myself mainly for trusting the decisions of consultants, I now feel like I've been a "wait and watch" case so they could find out a bit more about the condition and how it affects patients who are younger than the general cases they may see (I was 36 when diagnosed, I'm now 44), at no point in time have I ever been told of any specialist centres such as the Royal Brompton, quite the opposite, I was told I was being treated by some of the leading consultants in this area, I was told AZT was no longer available on the NHS for lung conditions, I cannot find any evidence of this anywhere. and now I can't enjoy any of my hobbies without suffering, I have had to stop going to work because I cannot physically cope with exercising and working, and because I look fine on the outside I get looked at as if I'm just looking for a reason to stop working, despite already taking on £10k of student loans to retrain to completely changing career paths to accommodate the condition I managed almost 3 years back in work before I couldn't handle it any more.

Anyway, that's pretty much my experience, what now seems like a catalogue of poor decisions, what do people suggest, what treatment have people had for this condition in the UK?

26 Replies
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Hello and welcome to the forum. You have been through a lot and it may be a good idea to call the helpline on Monday on 03000 555 030. Xxx

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Thanks for the reply, I will give them a call.

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It sounds as though you've had poor treatment. I'm afraid this is all too common, especially for rarer lung conditions. Have you now got a referral to the Brompton? I think their specialist centre for your type of illness is the largest in Europe.

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Thanks for the reply, I only found out about the Royal Brompton today, so will be going to see my G.P Mon/Tue to ask about a referral.

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I go to RBH for different lung problems, they are simply fantastic, & you get the benefit of all the latest research. Plus, all the staff are lung/heart trained, nurses, pharmacists, physios etc so they "get it". I presume you know about actionpulmonaryfibrosis.org/

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Thank you, did you get a referral to RBH from your GP?

I hadn't heard of Action for Pulmonary Fibrosis before.

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Actually no, I was referred by a local consultant who (eventually) admitted he was out of his depth. But I've checked with RBH before, for a friend, and they confirmed they take GP referrals too. It's a specialist centre anyway, so you shouldn't have a problem. Good luck.

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I was referred by my consultant in Chichester. I've been up to the Brompton, lovely place, and the consultant from the Brompton comes down to do a joint clinic a couple of times a year. I have every confidence in both ladies to keep me going

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Thanks for the link to Action for Pulmonary Fibrosis, I'd not heard of it, now on my bookmarks list!

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I have same diagnosis and I'm so sorry to hear your story. Unfortunately, it's not that uncommon and it's a real problem that some areas of the country do not have good enough specialists who are able to make the correct diagnosis. The best specialists are in the centres of excellence and most people do not have access to them at all or until its often too late.

As regards your treatment, the steroid immunosuppressant (prednisone and azothiaprine) approach was the standard for IPF and possibly other ILDs until about 2010 when a major study (PANTHER) showed people on a triple therapy that included these drugs (they were also taking NAC as well) were dying quicker than those on placebo. After that the treatments were phased out and I think its official guideline to not prescribe that any more. If you want some more info let me know. Fact you wre stable on them makes you wonder about accuracy of IPF diagnosis and perhaps original was correct.

The failure to prescribe either ofev or pirfenidone (for what they now think is IPF) on the grounds of you just failing the 50% FVC level is truely shocking considering their possible misdiagnosis and previously, arguably, giving you the wrong meds. I was not certain of your values. Is it really VC of 46% and FVC of 13% or was one of those DLCO or transfer factor?. Anyway if your FVC is 46% then the cut off is 50% and its a disgrace they will not stretch the guidleines as they know there is around a 5-10% error in the measure. Scientifically also it make no sense - the drugs do not stop working once FVC falls from 50 to 49% !!!. Overall it sound like you are being treated by a bunch of jobs worth's more interested in saving money than caring for patients. I would get as much info as you can and consider an appeal against the decision to not give you ofev or pirfenidone and demand a second opinion.

I'd have to say your best hope is a lung transplant and at your age you should stand a very good chance. The assessment can take ages so make sure they are moving along quickly. They can really drag their feet. I've been waiting for a year and half since the first tests started and, for various reasons, and am now on my second application and thats taken 5 months and a huge amount of pushing to even get an assessment date.

Good luck and hope you get some additional help on here.

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Thanks for the reply, it has provided me with valuable information I can use, after the consultant said I wouldn't meet the criteria for the antifibrotic medication I immediately asked for another set of lfts to rule out any underlying infection or anything that could have impeded the results, her exact words were "even if you manage to get 50% I doubt you'd be accepted as your results may not be reliable enough, and it's not solely my decision" I sort of walked away quite shell shocked as I have always been lead to believe I was maintaining lft results, and had increased my exercise regime as I was under the impression that if I maintained the same they would start treatment.

What I get told in a consultation to what is stated on the following correspondence is never truly reflective either, I'm not sure if you can post images on here but I have a letter that on one side state's "shows vital capacity at 46%. I explained to you that antifibrotic treatment is likely to be helpful when the vital capacity using between 80-50%. Therefore currently you do not meet the criteria for the treatment", and on the other side of the same paper it states "shows FVC at 13% indicating that you may benefit from antifibrotic treatment."

This is another reason I posted the thread, to try and gain some information to take to my GP, along with the letter so I can ask for a second opinion as the whole thing is feeling more like an experiment with me on their part.

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What a mess. Gives no confidence in the consultant. My FVC is spot on 50% and my consultant allowed a change from pirfenidone to nintedanib without hesitation to see if it would help.

The only obvious conclusion from the above is that the consultant is more interested in saving money than patient care and you have to get a second opinion. Brompton certainly has a good reputation and is also a lung transplant assessment centre. Hopefully the consultants there are caring human beings who put patient care over money

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Hi Oxijon, I was diagnosed with IPF when my youngest son was only 3 months old that was in 2000, I was at the time under an amazing consultant how started my on treatment steriods and Azathioprine these really worked and sort of halted my IPF, so I was taken off them. That remand the case until around 2014/15 when I started to get very breathless, my consultant had retired and I was under a different consultant only words I can say about him are waste of space sorry the man had no idea I kept telling him that I new I was getting worse but he just didn't seem to get it. This time last year I got a chest infection and still has it in March this year, lets just say I am no longer under this man. I saw an consultant in late March and was sent for new CT scan this showed that my condition had changed they held a MDT meeting the upshot is I also now need a double lung transplant. I have been undergoing test for this an am being referred to the Brompton. I am also on oxygen 24/7 now, but am still working it is very hard at times but I have to keep my brain active, I take my oxygen with me and the kids at school have been amazing ( I am a Teaching Assistant ) work has made many changes to my role to make this possible, Access to Work have been great as they pay for my taxi's to and from work and are will to come into work and see if there is anything else they can do to make my working life as manageable as possible. I have a great team of people now. I wish you well in getting the right treatment you need, take care and stay warm. :)

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It's terrible really, I've been reading a fair few different things (patient experiences) over the weekend, of different lung conditions, they all seem to be looked at a lot less than other less life limiting conditions, I'm beginning to feel like I've been viewed as some kind of opportunity to see how a relatively young patient's body handles the condition over time without medication.

Have you begun the transplant process? I have no idea what to expect really, I did expect it all to start quite quickly due to the implied seriousness of it, but 4 weeks later and I only received the paper summary of my consultation yesterday, I had a couple of appointments at the hospital I'm under but nothing from any transplant centres, at this point in time I don't even know if my consultant has referred me for assessment.

I hope you receive the treatment you need, and I wish you all the best going forward.

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Hi Oxijon, Firstly please don't think that, I was also in my 30's when I was diagnosed and had 3 very young children one only being a baby. I just think that sometimes we are not sent to the right consultant in the beginning.

I am at the moment going through so may tests as they need to know that all your other organs are good, eg: blood test galore, endoscopy, bone scan, ultra scan of internal organs, heart scan, breathing tests and 6 minute walking test from my oxygen nurse etc: my consultant is sending all of these to the Brompton. They just sent back asking for further blood test. It is now 4 1/2 months but at least something is being done now.

A good thing is that you will have the best MOT of your body if there's anything wrong they are going to find it.

What you have to understand is we are all different, we all deal with things and cope differently. Just stay strong keep as active as you can and try and think positively. Take care and I hope you get seen soon and can then start the process that I am going though. :)

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Unfortunately, lung conditions are the poor relation in the nhs, compared with say cancer or heart disease. I think it's because of the strong association with smoking, though many of us have never smoked in our lives, and even those who have hardly "deserve" to be ill! There are other plenty of ailments related to poor lifestyle choices, but respiratory problems do seem to be discriminated against.

I think you'll feel differently once you get to the Brompton, such a superb hospital, you can relax in the knowledge they really are the experts & you'll get the best care there is. You won't have to fight for everything once you get there. I've been a patient for over 20yrs & they've never once let me down.

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welcome to the world of ipf its a pain i went throu very very similiar my self and ended up having double lung transplant they ask you where you want to go for tplant i chose qe birmingham at leat you get the best treatment goin there if you need to ask me anything feel free one thing i know it hard and i spent time dreading it but you must exercise breathless is fine it will come back over time it does not feel nice to do but its a must i was down to about 6 weeks to live and still did some just cranked up oxygen and got on treadmill walked for 9 mins i know its a six min walk but i over trained to do it like i say anything you want to ask feel free

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I'm pleased to read that you got the transplant in time, did you have to wait long from finding out it was going to be transplant to having it, what is life post op like?

I'm not going to lie, a transplant scares the life out of me, the QE is my local centre as I'm from Birmingham too, but I was told if they refuse me they'll try a centre in Cambridge

Yes, as you say, you dread the exercising, I have upped my routine as much as I can physically handle, I use a road bike on a turbo trainer for 10 minutes, I try to maintain around 10-12 mph. I was doing a few circuits in a local park but the weather change meant that become too difficult, hence bringing it in doors, they did say the oxygen would make me feel like a new man and massively improve my abilities, to be perfectly honest it hasn't, it does make recovery easier after as I keep the mask on for 20 minutes after

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i waited from oct 2015 till marck 2016 about 5 months work on the 6 min walk that is the main test i hated it so hard for me but they do give you a little slac

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Sorry for your trouble. I hope you finally get the treatment you need.

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Hi Oxijon, I also have IPF. This was suspected 5 years ago but only finally confirmed about 2 years ago. I managed to carry on working for a few years after becoming ill (I was a head teacher) but eventually it became too much and I had to take early retirement on health grounds. My consultant has always spoken of transplant at the right time so I had plenty of time to get used to the idea. Early this year I went to the Freeman Hospital, Newcastle for a 4 day transplant assessment. I was told that I was in the 'window' for transplant and was put on the transplant list. I continue to decline and am now on oxygen all the time. To be honest, transplant now seems a good option although, like you, it seemed quite frightening initially. Talk to your consultant about a transplant referral as it is a long process. If you're not I'll enough they will monitor you but the sooner they know about you the better.

Good Luck,

Helen

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Stay strong Helen6 , you are very kind and thoughtful to help a fellow sufferer when you are struggling yourself.

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Gosh I don't write very often , but Your condition sounds soo similar to what I have ! I don't have the patience to type it all , but in desperation I went privately to the Royal Brompton last month ( for two years and and having resperity failour and in February I was again admited and put in to an induced coma )

After 5 months I got a follow up appointment ! They can't help me and am puting me forward for ( like you ) a double transplant . But I have nodules they can't identify , it's all to long to write

But Brompton were super and have got in touch whit consultant here and g p , !!!!!!!

I will be very interested how you get on

And truly hope it will all be good for you !, this is a helish desiese and my hart is with all of us who have it

Annabel

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I can’t help you but I can empathise with your struggle to get diagnosed. The NHS is in a terrible state and in my opinion representatives are promoting doctors into expert positions which under normal circumstances, they would not fit the stringent criteria.

You are young with your whole life ahead of you. No matter how frightening it may seem, if someone has left you the gift of life, grab it with both hands. Transplant is not easy to come to terms with and you really need expert councillors to help you through this part of your journey. Keep asking questions, at some point you will begin to receive answers. Good luck!

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Hi OxiJon

I have a degenerative cystic lung condition called Lymphangioleiomyomatosis alias LAM..it is a form of interstitial lung disease.

No chance of a transplant for me..the only med we have is an anti cancer molecule called Sirolimus which reduces growth cells but has horrendous side effects for some. I am on 4 different inhalers since June, I think it has made me less housebound than nebs. I am on 10mg of Prednisone a day. The rest of meds are for acid reflux etc

I believe pulmonary rehabilitation and physiotherapy have helped me back on my feet, I now exercise daily and have improved my FEV 1 and the rest..

The only problem I have is trapped air as the cysts block the airways but it is monitored and not evolving so far.

So exercising, physiotherapy, meditation against anxiety and stress have got me out of that black hole I was in xx

Take care x

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Hi Oxijon

Regarding lung transplant you really have to push for this as it can take a huge amount of time to go through process. I started process in May 2016 and finally, got appointment for the assessment a week ago (long story!)

The process takes so long because the referring consultant has to fill in and send off a huge amount of information to the transplant centre. Some keep putting this off due to pressure of work. Once the transplant centre has been contacted they then ask the consultant to arrange a whole barrage of additional tests that have to be done before they will even think about assessing you. Often one of these tests can lead to another test needing to be done - more delay. Overall the time adds up.

In first instance I would be really pushy with your consultant to make sure they have definitely sent in a request and ask when you can expect to hear from the transplant centre. Ask which one - it may well be that you are already being referred to Brompton as they are one of assessment centres. If you hear nothing by the date given perhaps go through your GP to get an answer. I found that my GP was excellent at pushing things as he was aware that the system is under pressure and things often don't get done. It's often a delicate balancing act in how you apply pressure to get things moving. You don't want to alienate the consultant - often their apparent incompetence is just down to huge pressure of work and using the GP as an intermediate can help, especially if you have little faith in consultant and might end up in a counter-productive argument!. I was told it's not that wise to contact the transplant centre directly - busy places and they don't really want to get involved until you have cleared all the pre-assessment hurdles (then they are really helpful!).

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