Idiopathic pulmonary fibrosis

Idiopathic pulmonary fibrosis

Hi my name is alan and 3 months ago I was diagnosed with ipf.

This was the first time I'd heard of this illness. Both myself and my wife were so glad that it wasn't cancer we had no idea how serious this was we came out of the specialist room happy. It wasn't until I googled it that it sunk in how serious this is.

We got married 2 months ago after saving for 3 years and this was a bomb shell we hadn't planed on.I have now had X-rays, ct scans , lung function tests and just had a lung biopsy with vats done. One of my lungs collapsed during the biopsy and I spent 3 days in ICU.

I would like to know why this illness is so unheard of in the uk.

I am now awaiting my biopsy results so I can get on the transplant register for a double lung transplant.

27 Replies

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  • Hi Alan - I do hope you can be offered a transplant, your age is on your side, and from the photo you look pretty fit otherwise !My husband has IPF but is 77, and has other problems like heart disease and diabetes, so that is not an option for him. I too am mystified as to what is going on here with regard to diagnosis/recognition of IPF. My husband's father had it, and when my husband developed symptoms 3 years ago I asked every professional we came in contact with if this could be the problem and was told in no uncertain terms that he had COPD, if he actually used inhalers they would help (he did and they didn't) and that what I now know were problems with de-saturation on exercise was low blood pressure. After 5 bouts of pneumonia in 6 months he finally had a CT scan early this year which revealed the fibrosis. A follow up appt. with the consultant so annoyed me in its negative attitude that I wrote to him - he phoned me and I said if you had told us my husband had lung cancer there would have been all kinds of support - he said most people are just glad it's not cancer, they don't realise the implications when I tell them what they have. Keith's been in hospital again recently and is now on oxygen 24/7, but there is no automatic follow-up from the GP, I even paid for a flu jab at Tescos because one couldn't be arranged before the middle of this month. I keep asking about a care plan, but the plan seems to be 'call us if you think you have a problem'.

    Anyway, my rant over - and yes, you are right, there is nowhere near enough awareness of IPF, despite the number of people who have it.I do wish you both well - there is a lot of support here on the blogs, and the BLF helpline is very good - it is well worth talking to one of their specialist nurses to get an idea of anything which you ought to asking about.

    It's a tough time for your wife too - it will take some adjusting to, but there are several of us carers on the forum. Look forward to hearing how you are getting on. Take care.

    Gill

  • Hi Alan. I'm sorry to hear you have such a disease at your age. I hope you get things sorted in the not to distant future. I have COPD myself so can't offer any constructive advice but there are some more people on here with IPF whom I'm sure will chat to you later.

  • Welcome to the site Alan. I'm sorry you had such news. I have COPD (something else which is not well publicised but is the third biggest killer in this country) so know little about IPF but the BLF helpline is really good and will be able to answer all of your questions and offer advice. Try them. x

  • hello alan, until you said how old you were I thought you were telling our story, we too were relieved when cancer was ruled out, how silly that seems now, my husband went through all the stages before he was finally diagnosed with a definite ipf. He turned 69 in september, the same day that we celebrated our 40th wedding anniversary. He is on 02 24/7, and being well looked after, he has the resp nurse calls, and the district nurses who are giving palliative care call regularly, we have a 24 hour phone number to ring if we need anything, we only have to ask. Our gp has told us not to go to the surgery ( germ factories )but to ring him, and if hes busy at the time he will get back to us every time, either with advice or or a visit. You are lucky to be young enough for a lung transplant, hold on to that thought, you are not alone, the people in this forum are lovely and we all support each other. x

  • Hi Alan, sad news hope you are able to have a lung transplant a soon a s possible. I was diagnosed with IPF in June this year but he said I should have a biopsy to confirm it. No cure and no treatment was the the thing that I couldn't understand. Luckily for me the result of the biopsy was that I had Sarcoidosis and not IPF. There is at least some help with this. Is there a chance that a biopsy will give you the same result?

  • Hello Alan,

    Firstly, sorry to hear that you have had such a diagnosis and understandably whatever life changing diagnosis anyone is given it feels as thought the ground is shifting under their feet for a while, until they come to terms with it. It takes time to adjust to an illness. So if you can, look after yourself, keep as well as you can, use the BLF help line they are marvellous. Many folk on this forum are so supportive, 24/7 so anytime you need to - I would encourage to to use it, and your local Breathe Easy group. In my local group many people there are much older than me and much more ill than I am, so they help me by being there and I hope that I can help to support them a little. Best wishes to your wife and she needs to take care of herself as well.

    All good wishes,

    Pauline

  • Hi Alan

    My name is Vicky, I am one of the respiratory nurses at the British Ling Foundation, can I start by saying I am sorry that you have been given this diagnosis. I was wondering if you have watched out webinar on IPF? my advice to you would be if you have any questions call us on the BLF helpline on 03000 030555 and let us answer your questions. Also I wanted to let you know that we also have a counsellor who can provide support

    Regards

    Vicky

  • Wow thank you all for your kind words were still getting our head round it all at the minuet . And Vicky I will ring the blp at some point. I don't know what support groups if any are in the hull area.

  • Hi Alan, I just wanted to tell you I had a lung transplant eleven years ago. If you are able to have one if you have ipf them please please go for it. I wouldnt still be here if I hadnt had mine seen my beautiful grand daughter and had eleven happyier years.

    Regards

    Angie

  • Hi Alan, my wife was diagnosed as having IPF about 6 years ago, our reaction was just as you describe, the prognosis was not good but as a result of wonderful ongoing treatment at the Royal Brompton, & pure willpower, we still fight on. My wife was too old for consideration of a transplant (over 60 ). Knowing how this condition progresses, we would urge you to fight for a transplant . Good luck to you & your family

  • Hi Alan, I am quite new to this site too and I also have IPF. I am 63 and received the diagnosis 3 years ago following various lung function tests & CT scan - no biopsy suggested though. The consultant at my local hospital appeared quite "uninterested" really just telling me there was no cure, no real treatment and nothing I could do to help myself! He did tell me to come back though if I felt worse in the future, so that's OK then (sorry for the sarcasm). I agree with you that there seems very little known by GPs, local hospitals and the general public about this disease. I did find the Webinar on the BLF site useful though and following all the wonderful reviews on here I am going to try to get seen at the Royal Brompton even though I live in the North. I'm sure you and your wife must be feeling as scared and helpless as my husband & I but I'm sure everyone on here will do their best to support us. If I find out anymore I will post it on here. Regards,

  • Hi Alan. Sorry to hear your news. My other half was diagnosed in September, based on symptoms and CT scan. We were offered no support and told the only treatment would be high dose of steroids for a year or more (with all side effects so not recommended by him), but only when the condition got worse. For now come back in the new year when they will do another lung fuction test.

    He has agreed to refer us to Papworth when I started asking about possible new treatments/drugs and we are waiting for an appointment. I don't know why he suggested Papworth as on this site everyone speaks of the Royal Bromptom in London as being the hospital. Hopefully we might get some more support at Papworth than our local hospital.

    A question for everyone is that can anyone tell me anything about or has any experience of Papworth for IPF suffers. I want to get the best I can for him and it seems you have push where ever you can.

  • Hi carer999

    I am sure you will find Papworth to be an excellent hospital for IPF- they are one of the recognised 10 specialist hospitals for this condition. i go to the Brompton myself, but this is just by accident. If I had known of Papworth I would have asked to go there since it is much nearer and has an excellent reputation. The latest drug being used with some effect at slowing down syptoms for IPF is pirfenidone. My PF is not ideopathic but related to my auto-immune disease, so pirfenidone is not suitable for me. I think it is important with this horrible disease to take every opportunity for treatment that you can - many local hospitals just don't have the expertise or patient numbers - many GPs will not have come across it. It is still a very rare disease.

    Good luck

    Maggie

  • Maggie

    Thanks for this. I feel a bit more reassured that Papworth will know more about this condition. I just feel so helpless and am trying to do the best I can and did want to miss an opportunity of an hospital with greater expertise or knowledge that may be able to help him more. If Papworth is as good as Brompton then I am happy with the consultants decision to refer to Papworth

  • Go to Papworth if you can its good, and you are treated as a friend not as a patient. I've had my lung bio and recently lung opened and a bulla removed after my third lung collapse ,also contact the Papworth IPF Support group , look for Action for ipf support ,think that whats its called and email a lady called Marilyn for information.

    Malcolm

  • Hi Alan

    may i congratulate you and your wife on your wedding and wish you a long and happy marriage.I to was diagnosed with ipf in feb this year and agree with your statement in regards to lack of information on this decease i have also found it very hard to come to terms with at one point i found it unbearable but rang BLF who were very understanding and as helpful as they could be.

    I wish you both good luck for the future keep fighting Julia 65 Birmingham.

  • Julia sorry to hear about your diagnosis. I live in Birmingham and have had IPF for 5 years. At first it didn't effect me much, apart from people telling me to get that cough seen to. I attend the Lung function and sleep department at the QE under Dr Thickett. He told me I had 2 to 10 years. I have been on perfenidone for approx 3 years but it is being taken from me as my lung function is below 50%.I don't know how effective it has been because the last 3 months I seem to have gone over a cliff leaving me on 15 hours per day oxygen and an inability to walk any distance at all! My advice to you would be to cram as much into the coming years as you can

  • Hello and welcome to the site!

    First of all, I would just like to say that having been through the rollercoaster of a ride that you and your wife are just starting on, there can be light at the end of the tunnel and whilst I know you will both be feeling completely devasted right now, believe me, it will subside and you will get very pro-active and fight this dreadful disease.

    My dad is 65 and has IPF and not wanting to go through his history right now, you are more than welcome to read back through my blogs about dad if and when you want to. What you must focus on right now is, you are young and you have everything going for you. There are some new treatments out there that are starting to show promising results for stabilising IPF and as you have said there is the transplant option. It was lovely of Angie to say about her transplant and that must give you loads of inspiration and hope. It does me, as we have a meeting with dads specialist Friday to talk about a possible transplant.

    Have a good cry, scream, shout whatever makes you feel better. I was angry when we first found out and I found that spurred me on to do everything I possible could for dad and I had to know the ins and outs of IPF so research was my saviour.

    I hope we can all help you here, they are a good bunch!

    Take care both xx

  • Hi Alan,

    I joined the BLF about 3 weeks ago l have been desperate to talk to other people as l am the only one at our GP surgery and l think only 4 at my local hospital unless a few more have been diagnosed since, and l have found it very helpful and would love to chat with other people with IPF unfortunately l am unable to tolerate the high doses of drugs so I just take steroids to reduce the inflammation. l have been diagnosed since 2003 but had it a few years before that and since last October it started kicking off. l'm not at the Brompton but l can't fault my local hospital l have a gem of a lady consultant who has tried to help in every way, l know how you feel in total shock as l was told what is going to happen, you can always ask any questions here and we all will try and help.

  • Hello Alan

    Sorry to hear of your diagnosis - it is a very hard message to be given. I was diagnosed two years ago and told that I had just a few yearsto live. As it happens, it turned out I didn't have IPF because the cause is known - my own immune system is the cause. This means the treatment and out come are slightly different but sadly still no cure available.If you get transferred to one of the specialist hospitals they may begin to treat you with one of the new drugs that may slow down the progress

    ( no cure available, but slowing the scarring down will keep you in better shape until the transplant becomes available). I met a chap with PF at Pulmonay Rehab classes who was younger than me (I'm now 68 so transplant not an option) he had a transplant last spring and when I last spoke to him was feeling much better and working hard to get his fitness level back to where it was. When you have both got over the initial shock ( not easy and several stages to go through) It will be important to ask questions about what treament is available and take every opportunity offered. Talking to someone at the BLF helpline may be useful.

    Take care

    Maggie.

  • Hi Alan I was diagnosed with I.P.F. 18 months ago, but I know I've had it for over 4 years because my doctor at that time said he could hear a noise in my chest that should not have been there but he did nothing about it! I'm 65 they don't do transplants at my age,but they do what is called living lobe transplants you need 2 living donars for this op who are willing to give you parts of there lungs.Hope this info is helpfull,I just tryu to keep doing the same things I have always done because I think if I change my life style to much it could possibly

    make things worse.cheers from John.

  • Tough luck Allan , I too have IPF (diagnosed 007) still on all the usual tablets, oxygen etc etc! Appear to be getting more breathless now. You are very brave going for a double lung transplant. Good luck with that

  • My mother was diagnosed with IPF in 2012, a so-called incurable disease. In March '12 her gasometry went down to 57% (= 89 % oxygen saturation rate). The CAT-scan showed "honeycombing and thickening". She then started to take every day:

    - 7500 IU vitamin D,

    - 640,000 U serrapeptase, an enzyme that cleans scar tissues.

    - 4 grams curcumin + peperin, a natural anti-inflammatory.

    The October 2014 CAT-scan, 6 months into this "treatment", shows things have improved. Her gasometry has gone up to 77% (= 95% saturation rate) with "fewer ground-glass opacities [...], no increasing of reticulation [...] and no honeycombing.")!

    Her pneumologist told her vitamin D was excellent but confessed he had never heard of serrapeptase which, I think, has done the entire job. So don't lose hope. I'm not saying it will work miracles for you but it has shown some effect on my mother, so why not give it a try?

    Feel free to contact me if you have any questions.

  • Hi Remo3

    Thanks for your post re your mother and IPF. I was diagnosed with IPF about 3 years ago.

    From fairly early on I have taken 1 x 40,000 capsule of Serrapeptase - am interested that your mum is taking a very much higher dose. How did you get on to Serrapeptase and how did you determine the dosage?

    I also take one 2,000 FUs of Nattokinase as well. I might up the dosage there as well if and when I can get some guidance.

    I also take 1 x 600mg capsule of NAC - N-Acetyl-Cysteine - en.wikipedia.org/wiki/Acety...

    NAC is the only supplement I have seen that is on the lists of IPF consultants as well as 'alternative' lists.

    RE VITAMIN D3: From around Christmas I developed heart palpitations that frequently felt as if my heart was trying to punch its way through my chest wall.

    I started taking D3 and sunbathed during the summer. This cleared completely the very distressing palpitations and missing and extra heart-beats - and any and all IPF symptoms - for a few glorious weeks. But they have come back again - perhaps I should increase the dose and use a sunbed as well. I might consult this ex-nurse - easy-immune-health.com

    This org might also be useful - vitamindcouncil.org/

    Do you have any source of guidance re dosage of D3 in relation to IPF?

    FRUSTRATIONS RE CAUSE & EFFECT: You and your mum probably, like me, find great frustration in rarely being able to pin down cause and effect. For example are the palpitations in my case caused by the IPF, or does an IPF patient have more than one autoimmune disease? Is the IPF a consequence of the ‘real’ disease - not the disease in and of itself? I went to see an NHS consultant on the issue of autoimmune diseases. All he said was that I don’t have rheumatoid arthritis. What he did say was that as soon as someone has one autoimmune disease they are much more likely to get others.

    The number of autoimmune diseases is astonishing - aarda.org/descriptions-of-d... alveolitis​

    I take curcumin daily but I would like to add peperin I want to add it to my regime - please could you give me a link for a supplier and a suggested dosage.

    Thanks and I hope your mother is continuing to do well.

  • Hello, Sunwalker,

    I hope my message will be able to help you.

    Yes, my mother has been on very high doses of serrapeptase. In fact, I made an error in my post: I wrote she was taking 640,000 units but, in fact, she's taking 960,000 units (4 capsules x 3 times per day)!

    I found out about serrapeptase on an American website that sells food supplements to cure IPF:

    biomediclabs.com/pulmonary_...

    I saw that they associate 2 types of enzymes (Serrapeptase and Nattokinase) but I did not want to mix too many products and after seeing some testimonies of serrapeptase-users on YouTube I just raised the number of units of serrapeptase to see if the effects came faster. I also read about NAC but it seemed to me that there might be some side effects so I just forgot about it. What I wanted to do was to make the "treatment" as simple as possible.

    You have to be careful when taking serrapeptase: with water only, 2 hours after any food or 1 hour before. My mum takes her capsules at 11am, 6pm and 10 or 11pm as she has lunch at 12 and dinner at 7pm. She takes 4 capsules of curcumin/piperin with her meals at 8, 12 and 7. As for the vitamin D, she takes it with her lunch.

    The level of vitamin D in the blood should reach 50/70 ng/ml = 125-175 nmol/L for people with chronic ailments like cancer, heart problems, multiple sclerosis, etc.

    - ucsdnews.ucsd.edu/archive/n...

    - sciencedaily.com/releases/2...

    - phytosanteverite.com

    By taking 7,500 IU per day my mother had 47 ng/ml = 117 nmol/L in her latest blood test. My mum's GP used to give her 100,000 units every 3 months but the blood test showed that the level of vitamin D decreased rapidly after a few weeks although she lives in southern France where there's plenty of sunshine. That's why it is better to take a daily dose. The American website (mentioned before) advises 10,000 IU/day for IPF.

    You're right; my mum's lung doctor does not know what caused her IPF to develop. He asked her if she had been in contact with asbestos or if she had pets at home, which has never been the case... He says it "might" be caused by gluten but, in fact, he does not know. My mum used to drink a lot of milk until she realised she had a problem with lactose. So she stopped having milk about 5 years ago. Could that have been the problem? Other dairy products like yogurt and cheese are all right, though. The doctor says the IPF probably started a long time ago: it went unnoticed until 2012 when she was diagnosed with pericarditis and that's when doctors discovered her IPF.

    Regarding curcumin, do take some with peperin as it makes it much more efficient.

    The doctor was (happily) surprised when he saw my mum's latest scan exam. He told her to keep taking the vitamin D. As for the serrapeptase, he had never heard about it before! The last time she had seen him (March 2014) he told her he would put her on oxygen if her oxygen saturation rate kept falling... This time (October 2014) he said this was no longer an option since her oxygen rate was much better!

    Her next scan exam is due next year. I'll keep you informed if you're interested.

    Here is where my mother buys her serrapeptase and curcumin:

    - homecures.co.uk

    They're very nice people. If you have any questions you can contact them at:

    - info@remede-naturel.net .

    Do contact me again any time if you have more questions. I'll be glad to hear from you. If my mother has improved, why wouldn't you? She'll be 88 next week.

    Thanks for your message.

    Wish you well,

    Remo3

  • Hi Remo3

    Many, many thanks for such a fulsome reply - I will be digesting it physically as well as metaphorically!

    And thanks for the offer of further help - All good wishes Sunwalker

  • The disease itself isn't really unheard of, but until recently treatment was pretty much unheard of. Since this post was originally submitted, there have been lot's of new developments in the pulmonary fibrosis world. I have a close family member with IPF and he is too old for a lung transplant. However, we have learned that there are lot's of therapies available from pulmonary rehab, to oxygen, to newly approved drugs, among others. I know of a very good IPF blog that is written by a pulmonary doctor. It has been really useful in helping my family understand the disease and treatment options. Here' a particularly good comprehensive article on all the treatment options currently available: pulmonaryfibrosismd.com/idi...

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