Idiopathic Pulmonary Fibrosis

Hi all 

My dad has been diagnosed with Idiopathic pulmonary fibrosis. I’m not sure what that is (apart from what I’ve read on NHS Choices) and wondered whether anyone could enlighten me. 

So far, the NHS specialist consultants have simply said that he should get ready to die! Brutal and abrupt, but I guess because he is in his 80’s they have given up on him. 

Therefore, I would like to know where I can turn to get an alternative opinion and or treatment, and what I can do to extend his life as they have given him weeks, months, or a year to live.

Thank you in anticipation of your swift response to my post.

Best

Dennis

35 Replies

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  • " Hello and welcome to our very supportive site... I personally cant answer your questions regarding your dad. But I am enclosing this link for you.... which may be of some help..... Keep strong...Megan"     blf.org.uk/support-for-you/...

  • ... Thank you, that's very kind of you.

  • " Your more than welcome Dennis..I know it can be very scary when given this type of news.. But try and keep positive... and remember..there is always someone in here to help you..if only to talk too...Thinking of you and your Dad...Megan."

  • ... Thank you again.  The article was very informative and I have already made steps to get him onto a pulmonary exercise class to help strengthen the muscles around his lung. Thanks again, Dennis.

  • " At least you wont feel so alone and helpless now you've something to work with..let me know how you and your dad are doing..just lit a candle for you both..Megan"

  • .... Thank you, how so sweet of you.  I will let him know this and I will definitely keep you informed of progress! :-)

  • Hi Dennis,

    Sorry to hear your news about your dad, I am 80 and suffer from IPF, my current specialist sounds a bit like your dad's, I have had IPF for about 4 years but have never been given any medicine to ease my cough which has got much worse recently.

    No list or number for a specialist nurse, which have just found on this site are available, and of course no phone number to whom I should ring if I have problems.

    It seem to me once you get old the concern gets smaller or non existent.

    My daughter got me a oximeter yesterday, and my 02 readings are below 90, which I have been told should carry some concern.

    So when I go see him next week ,I will take a long list of questions, and will expect to get answers.

    I hope your dad gets on OK, and if you want to keep in touch with me, I would be delighted.

    Take care

    Brian.

  • hiDennnis,

    dont seem to be able to contact you,hope all is well with your dad

    regards

    Brian.

  • Hello Brian

    Thank you for your swift response to my post. 

    I guess you are right.  Once you hit a certain age (my dad being 80) the NHS just doesn't want to know and doesn't really care!

    Fight the good fight and keep asking questions and get what you need - after all, you've paid into the system and should be benefiting from it now that you're ill.

    Brian, I am greatly encouraged by your post and would like to wish you well going forward - and yes it would be good to keep in touch, so keep me posted on your progress. 

    Best regards

    Dennis

  • Yes Dennis,

    I will keep in touch with you and let you know how i get on Tuesday week, what does annoy me is when youbsuffer from any illness the last thing weshould need to do ,is to have to fight your corner,but my daughter will be coming with me and i am sure annything i forget she will pick up on, hope you get more help with your dad,keep in touch.

    Brian.

  • Hi Dennis,

    further to my visit on Tuesday,it would seem he cant even write up a precription correctly, firstly wrong date on it,second wrong details on liquid morphine,third, the other item is not even avalible, what is wrong with these bloody people, i give up,my daughter had to.run around getting it all corrected,dont know what i would have done without her,bless her.

    Brian

  • You are entitled to be seen by an IPF specialist,depending on the severity stage there is medication to slow the progress.Look at the NICE GUIDELINES.please join Pulmonary Fibrosis UK on Facebook,hundreds of us on there,always someone with advice.My mum was 80 or so when diagnosed,not much treatment then that was 5 years ago,she didn't die in the end from IPF,.I was diagnosed 3 years ago and the treatment is now very good,but you must be referred to a specialist .Sooki.

  • Hi Sooki

    Thank you for your advice Sooki; I am very encouraged.

    I will, as you have suggested, look at the NICE guidelines to seek out the key recommendations.

    I am so sorry to hear that your mother had IPF and that you have now been diagnosed with the condition.  However, I am heartened to hear that you are receiving good care and attention, and trust that this continues. 

    Keep strong Sooki

    Very best wishes

    Dennis

  • Hi Dennis I am 57 and have had this cruel disease since 2012, I have been on high dose steroids constantly and on oxygen 24/7. There is nothing else as they do not know what causes the disease. Like your dad they told me a few weeks ago to get my affairs in order as I will not live for much longer, I am still reeling from this as you can imagine. Push the doctors for your dad make them listen and keep asking what they are going to do to help him. All the best. Jenny

  • Dear Jenny

    I am so very grateful to receive your message of support. In fact, I am overwhelmed by the messages of encouragement and advice I've received from this site. Thank you so very much.  

    As you say, some doctors can be very insensitive.  I can see from your mail that you are however a fighter, and that is good to see.  I believe I am too, and I will unlock the doors  in order to get the support my dad needs going forward.

    Good to hear from you Jenny, stay strong.

    Best wishes

    Dennis

  • Hi, Dennis. 

    I have IPF The "I" Stands for not known cause. Pulmonary speaks for itself and the fibrosis is the scarring in the lungs. I have it as well and, my GP told me its rarer than lung cancer. Problem is, is that they don't know how to cure or treat it so, the medication your husband will be given is to help with build up of mucus, I assume. All I have been told is to try and stay well. I feel noticeably better when the wind has dropped and the there is less moisture in the air. Now you've found us all, it's important to sign in to the lung community regularly, because we're all together in this and not alone. Stay in touch and I wish you well. 

  • Hi Alveo

    I am so very sorry to hear you have IPF. I hope you are receiving good care and attention .

    Thank you for your informative and encouraging message.  Yes, now that I have found you all i will definitely stay in touch.

    I wish you all the very best going forward, and thank you again for your kind wishes of support and love.

    Stay strong!

    Best wishes

    Dennis

  • G'day Dennis,

    Brutal and Abrupt,  Pretty much describes how scared some Dr's are when having to tell someone that a loved one is beyond help.

    You are quite right to be offended,to say the least, regarding their attitude towards your father's condition.

    There are too many in the medical profession who act like like amateurs,showing little regard for their patients. And yet there are many good Dr's who have a great,caring "Bedside manner". 

    IPF is a fatal disease,with the medicos still trying to find answers,but, with government funding as it is , it is at the bottom of the money pit.

    The cold facts are that it cannot be stopped, age is not necessarily a setback,but the older you are the less likely you will be able to survive the surgery. So it is not so much "Not caring" but in many cases concern for the sufferer.

     IPF moves differently in patients, some succumb very quickly,other pass as a result of a secondary infection, so the time factor varies.

    Please do as others have suggested, join the IPF site,learn from the testimonials, but please be there for your father and use the knowledge you gain to keep him comfortable.

    There are two scenarios, 

    1. Put your head in the sand and ignore what is happening,hoping it will pass you by.

    2. Learn as much as you can so that you can best help you dad,now and even more as he will need much care in the future.

    Sorry I cannot give you the answers you probably are desperate to hear, but it is what it is.

    I know, as I am watching my dear wife (IPF) slipping, and praying for a donor set of lungs.

    Will (Australia)

  • Dear Will

    I am so very sorry to hear that your wife has IPF and is waiting for a donor; I hope that your prayers are answered.  

    Thank you so very much for taking the time to share your story with me.  I am, as you say, being realistic and thus exploring all possibilities fully knowing at some point he will go. Whilst he is here, however, I am determined that he receives the best care and attention he can possibly receive.

    I send you and your wife my very best wishes.

    Stay strong Will.

    Dennis

  • Thank you for your kind words of support Dennis.

    Like it or not we carers and sufferers are all in this together and hopefully we can glean information as to how best look after our loved ones.

    Best wishes

    Will

  • Hi Dennis -My ex-husband got a dx of idiopathic pulmonary fibrosis--They figured it was from working with asbestos or chlorine--It is progressive and a deadly disease--From the first time he noticed shortness of breath till was dragging around oxygen tank was about 6 months--He just had weeks to live and got the lungs of a 42 yr old woman killed in an ATV accident--so the transplant has given him 8 more years so far-He will be 65 May 9th...-He has to take anti rejection drugs and has no immune system--your body never adapts to  anothers organs--your immune system will attack it--so the drugs he is on destroy his immune system and are in process of destroying kidneys==so is hoping gets a transplant before has to do dialysis--He is a vet--so in this case got taken care of quick--or lucky cuz the right lungs came in shortly after got on transplant list--Some people have to have both lungs and heart transplanted because it damages rt side of heart--Personalkly I could never have done it--and the qualifications are strict--He never smoked so that was an easy one--but very positive and excellent health otherwise--Im thinking your father is older so they may wonder if he could survive a major surgery like this----Maybe it will stall--It is awfully hard to watch I know that----I think there are  many people waiting for transplants so they will take youngest and healthiest --if he is even a candidate--My advice--Hope for the best--Love him--don't let him overdo it--make sure he does any medical treatments etc--and pray for a miracle--they do happen--Blessings to you both--MmeT  write me anytime

  • Dear MmeT

    Thank you so much for your detailed response to my post; I truly appreciate all your kind words of advice and clarity. 

    What a journey you and your ex-husband have been on. I am so pleased he is still here and fighting the good fight, and long may that last.  

    As you rightly say, younger patients are offered more options, and as my father is in his 80's a lot of doors have simply been closed and avenues of possible support not even explored.  Clearly, in my eyes, a little unfair given that he is a very active young looking 80 year old with a wicked sense of humour and a great sense of fun.

    I am much encouraged by the warm messages of support and advice I have found on here, and would just like to thank you for taking the time to respond.

    I wish you and your ex-partner all the very best going forward.

    Very best wishes

    Dennis

  • Dennis,what a lovely letter from you--well my ex has moved on but--I do talk to him now and  then and  as I said--he is waiting for a kidney now--Gosh what a shame--when your dad is fine otherwise--the sense of humor really got to me--as my dad was so funny--just a dry sense of humor that crackd me up--I like to think I got some of that from him--as that is how they live on--seeing them in ourselves-That was a tough letter to write to you--but people want the truth--we spend far to much  time sugar coating and protecting. I think telling one what to expect is far kinder than catching them off guard--I hope your father fares well--There are so many people suffering with lung problems--it makes me sad--Contact me anytime if you want to talk=Bill(my ex) knows a lot about PF so anything you might wonder about I can ask him for you--I did tell him about your dad he said it is the age thing,, Love to you both--Blessings--MmeT

  • ... Thank you again MmeT.  I will definitely keep in touch. Heartfelt wishes to you and yours. x

  • Good morning Dennis,

    We have been lucky with my dad's consultants and specialist nurse. However when we first was told my silly reaction was to type it into Google... Well that was a mistake, it brought me up to the NHS website which stated on the 3rd line " IPF gets worse over time and is often fatal" and that scared the hell out of me. 

    It also said that on average life span from diagnoses was 3years but dad had been for longer than 3years already before diagnoses, however at the bottom there was a comment some had left saying her mother had it for 20years and was still going strong, (every night she slept with the window open and vicks on her chest) and that gave me the flutter of hope. 

    It was at that point that I realised that if that hope fluttered away that things would get an awful lot worse, the wicked sense of humour you mentioned will also help!

    I hope you find an amazing specialist doctor and nurse because there are some out there. It is now a case of taking it day by day making as many memories as possible!

    Suzy (Tenter's Daughter)

  • Hello Suzy

    Thank you for sharing your story.

    My father's care has been disappointing, to be honest. He has simply been neglected. He informed his doctor years ago that he was having difficulty breathing when walking up hills so they sent him on a series of tests, on return from these tests his doctor said that he had nothing to worry about. All they said to him is that he had something on his lung but that they would give him something to 'wash' it away. Hmm! Well, that says it all really. 

    A name was only given to his condition 2 weeks ago. If I had known I would have made sure, he had oxygen therapy, pirfenidone, and pulmonary rehabilitation classes in place. Maybe then his prognosis would be better.

    Now he is only being offered oxygen therapy. Apparently, his condition is too far-gone for other forms of treatment to be of use.

    In reality, therefore, we are facing an unfolding end of life scenario! 

    What incompetence. The duty of care for my father has been wholly reprehensible. It is a case now, as you say, of making happy memories. 

    All very sad.

    Thanks again Suzy. I wish you well going forward. I hope the future is brighter for your father.

    Best

    Dennis

  • Hi Dennis, 

    Sorry, to hear your sad news and your fathers situation. Also, thanks for the wishes of a brighter future, expressed to my daughter Suzy (tenter-spawn)

    Emotions run riot when diagnosed IPF. Currently, 8 months after diagnosis, I am in an anger and frustration state, not with the system, but, with my stupid body. 

    Detection and confirmation of IPF is very difficult, I first started having minor breathing and tiredness problems in 2006/2007 after a virus. This was put down as chronic fatigue and a lung infection. In 2015, I saw respiratory consultant, X-Ray's from 2012 (taken for kidney surgery) showed minor 'bits' on the base of my lungs, and 2015 X-Ray's it was obvious that there were problems with the lungs. IPF was only diagnosed after a lung biopsy was performed, surgery under general anistetic,  6 days in hospital and months for the wounds to heal.

    With IPF the there is little that can be done, the drugs that are available slow progression, but, do not cure. I tried pirfenidone and ended up as a bundle of coughing mucus that only woke up for eating and coughing. The coughing is still there,but, reduced dramatically within hours of stopping pirfenidone. 

    According to the ILD nurse, In the Uk there will be a new NICE approved drug available at the end of April 2016, called  Nintedanib. I am hoping this will help me and not have same side effects, athough there may be complications with my liver, BUT, this may give me the bright future. I am sorry it may be to late to help your father.

    I hope you can find a place to store the happy memories.

    All the best, tenter

    PS: The future is bright, the future is reaching the bathroom before the body detects the lack of oxygen

     (please excuse the flippancy, it is one of the life's small pleasures)

  • Hi Tenter

    Thank you for your response to my post.  Yes, I am very disappointed with the way the system has treated my father.  I can't for the life of me understand why.  Nevertheless, going forward, I have asked for him to receive oxygen therapy, so I hope this fools his body into believing he has another year or more and not the weeks suggested by his hapless team.

    Thanks for the information regarding Nintedanib.  I do hope it is something I can get for my dad. Fingers crossed.

    I appreciate IFP may be hard to diagnose, but believe me my dad's medical team have been totally incompetent.  So far I am just not impressed in the slightest. He has a new doctor and even he has struggled with the sheer incompetence of his consultant/secondary medical team.

    Thank you for engaging and thank you for the tip regarding the new drug.  Again, I wish you well going forward and hope you remain strong with your beautiful humour in tact.

    Very best wishes

    Dennis

  • Are you saying he was diagnosed 2 weeks ago with IPF and he hasn't got oxygen yet?

    I don't know what to say, I know some people are diagnosed with IPF and do not need oxygen for several years, this does NOT SOUND LIKE YOUR FATHERS CASE. I can only relate my own experience. I was not allowed out of hospital after my biopsy, which confirmed the IPF, until an oxygen concentrator had been installed at home. My consultant ordered it on Friday and it was installed by the next day, this did surprise the consultant, he thought it might take a few days. I do feel that I have been incredibly lucky.

    Two thoughts 

    1 - getting oxygen may depend on the amount of oxygen (%SoO2) in the blood stream, mine was in the high 80's, dropping to 70's when walking. It is my understanding that prolonged levels below 85 will damage organs. %SoO2 can be measured using an Oximeter that you put on your finger and it measures the amount of oxygen in the blood (cost about £20 online). If, your fathers is low it might help your push to get oxygen.

    2 - in your fathers case, maybe they believe it will have little affect.

    To the best of my knowledge it only takes a form to be faxed from, consultant to the local oxygen company, via the GP. Not totally sure about this, as it was done whilst I was in hospital.

    It is also possible to buy / hire a concentrator, although I do not know how this can be done without having levels set by the respiratory team.

    Have you tried talking to the BLF helpline?    possibly they will be able to guide you or put you in contact with people that can help.

    Not sure if any of this will help and others on the forum may know better, but, keep pushing to get the oxygen.

    Good luck, tenter

  • .... He has been sent back and forth to consultants since 2014.  His then doctor told him back in 2014 that he had something on his lung but that they would give him something to 'wash' it away and that he should not be overly concerned.  He continued to be sent back and forth for tests with consultants here and there and everywhere but was always told he had nothing to worry about.  

    Then two weeks ago and out of the blue, he was inhumanly informed that he should go home and put his priorities in order because he had IPF and his journey in life had come to an end. 

    Now surely when they found something on his lung in 2014 they knew what this was yet they said nothing to him.  They furthermore, made no attempt to help him understand what they had found and hence manage the progression of IPF. 

    My gripe is the incompetence and inhumanity they have doled out to my father.  Why treat someone like that?

    Anyway, I tire....it has been exhausting trying to put this unfolding jigsaw puzzle together. For instance, I sent a letter to his consultant last week Thursday asking for oxygen therapy to be put into place for him, and still I have yet to hear when this will be actioned. So the years of inaction continues - anyway, I will be persistent even though I'm finding it pretty draining.

    In the meantime, thank you for your detailed feedback, I will go forward armed with some very useful information.

    Thank you again - all very helpful!

    Best

    Dennis

  • Hi Dennis--How are you doing and how is your dad doing?? Just thought Id see how it is going--you are in my thoughts---MmeT

  • Hello there

    Good to hear from you.  I struggle on.  I'm just not impressed with the medical care and attention my dad's been given.  So, anyway, I have a meeting with his consultant this Thursday to get answers to questions which are bubbling up inside.  So far all I can see is that they have done nothing to manage his condition, they've just decided to let him struggle on.  In fact, they were treating his cough as an actual cough and not a symptom of IPF so giving him cold remedy.  Incompetence springs to mind and I'm determined at this late stage to get what I can for him.

    So there, that's where I'm at -  frustrated and angry.

    Dennis

  • Hi Dennis--I don't blame you--The medical community is a giant--Corporate as any business--unless you get lucky and find a true humanitarian within it--Just makes me sick-- Im very sorry for you and your dad--You are a good,devoted son so he must have done  something right-Imagine the poor people with no one to fight or advocate for them...-I hope you get some satisfactory answers and some care that goes beyond a common cold--What B.S.!!!!!! Keep me informed--Blessings to you both--MmeT

  • Dennis how is Dad doing?? Hope you are getting  some answers and he is feeling ok for now  xxx MmeT

  • Dennis how is your father doing? Are you getting any better help for him? Thinking of you--MmeT

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