IPF – the final stages

David's story deals with the subject of dying in an honest and open way.


David wrote this story in 2014, after being told he was approaching end of life.

Although I had a cough and minor problems with my breathing early in 2008 and some ‘crackling’ sounds in my lungs, it was not until later that year it was identified as idiopathic pulmonary fibrosis following a CT scan.

My consultant explained the sad reality facing me: there is very little that can be done for this disease and there is no known cure. While the BLF now has a whole section of its website dedicated to the latest IPF developments, back in the days of my diagnosis, there was very little information available online.

But I read everything I could find, sometimes not understanding half of it. I felt very alone knowing there was there was so little help and support of value available to me.

Exercise makes a difference

The following year, I was offered a place on an exercise class run by the local respiratory team.  I went for it! The physiotherapists and nurses were really good and the class of a dozen loved it, even the more strenuous bits!

And it also gave us a chance to spend time talking, getting to know about each other and sharing our daily challenges.  It was quite enlightening.

I was upset at how the people with COPD struggle and they were taken aback to find that the IPF prognosis was death just a few years after diagnosis.

It was a very positive experience, but the exercise course only lasted 9 weeks and any following courses would only last 8 weeks.  After that, there was nothing! When we ended the course, we all agreed that more could be done.

So one day, I went to the local authority sports centre to enquire about the possibilities. It turned out that the manager of the gym and the lead physiotherapist at the pulmonary rehabilitation unit knew each other, and everything slotted into place.

They worked to make sure the group could continue their exercise and others could join too. Not only were we moving again, we were also talking and looking after each other when the going got tough. Sadly it only lasted a little over a year. Access to this class was by referral only, from a doctor or respiratory nurse, and the facility was withdrawn because there were no new referrals -  and of course sick people drop out over time!  Can you believe it?

In my experience, if you have a lung disease, make sure you exercise, even though it’s probably the last thing you want to do when you have difficulty in breathing. Don’t just rely on drugs, particularly in the early to mid-stages.

Progression

Well here I am now, five years on. My condition worsened badly in February, like falling off a cliff. I found difficulty doing anything at all. General panic all round.

I developed secondary conditions like mucus creation and coughing.  Regular bodily functions were disrupted. I went through a series of drugs.  But after a while, I had had enough and I was tired of living with a cloudy head, so it was goodbye to the morphine.

Live, laugh, love

I have found the best possible way to deal with this extremely unpleasant disease is to laugh at it. Clint Eastwood famously said: ”a man’s gotta know his limitations”.  Too true with IPF. So now, in the words of A. A. Milne, I largely ‘sits and thinks, and sometimes I just sits.’ 

I have been outside to the garden once this year. I use a commode. I never walk more than five paces and that is to get into and out of bed. My wonderful wife dresses me and washes me and generally holds me physically and mentally together when it all seems too much and perhaps it is.

End of life

I know that my medical notes say this is the ‘end stage’ and that I am a dying patient. Well, not yet matey, I’m not - try again next week. But while I am excited and curious about what comes next I have no fear about it. 

I am also curious about what comes after, especially as I face the end! I find that few people want to talk or contemplate dying, death and after death.  Yesterday I told the plumber with a big smile I was moving towards the end and doing just fine but the body was a bit of a bind.  We ended up laughing.

But I am interested in how I will die.  Will it be painful, traumatic or gentle and quiet? I have wondered and felt distinctly wobbly when I have heard doctors say bluntly I will die of suffocation. The most I know is that somewhere in our house there is a sealed bag that contains drugs that can only be administered by a qualified person.

These drugs that sit quietly waiting their turn are not to speed the end, they are to carry me through the inevitable end process of this disease in ‘comfort’ rather than in deep distress. But there remains the question: not when it will be but how it will be?

Coming to terms

We all know idiopathic pulmonary fibrosis is a pretty rare disease. So it is rare to know anyone near you who has it. It just so happens that the father of one of my carers died about five years ago and rather than have him taken into hospital, she nursed him through it to the end with her mum.  Until she came here and we talked, she wasn’t sure exactly what he died of.

But the more she talked, the more it became clear the symptoms and events matched mine.  He had died of IPF. He had struggled through all the problems mentioned above attended constantly by a nurse in the last week until close to the end he said he didn’t think he could stand it anymore. The package was opened and the nurse injected the drug.

She said the effect on her dad was immediate.  Where he had been struggling desperately to breathe he just relaxed on the bed and appeared to be in a gentle sleep, breathing lightly. He died peacefully the next day. My relief at hearing this was palpable. My carer was so pleased she had been able to help in this way.  Also, unwittingly, I had given her the opportunity and a safe place to let it all out which was of some therapeutic value for her.

So we have gone on talking about it and I continue to learn. I hope something in all this may now be of help to others. Meanwhile, keep on truckin...

If you or someone you know is facing the end of life with a lung condition, we’re here to help. Contact our helpline on 03000 030 555 between 9am – 5pm, Monday to Friday and one of our qualified nurses will be on hand to offer you support. You can also view our list of organisations who can offer end of life support.


Comments

Thinking of you and hoping that you're doing ok? x
Hi David To have a positive determination after being told you have IPF can be hard but if there is someone you can share your journey with it will be of great help. My wife felt ill in mid January 2014 and after CT scans and many blood tests she was diagnosed with IPF in April. The devastating news that she had only three or maybe more years to live made us both work hard together to make life as interesting and pain free as possible unfortunately almost immediately she was on oxygen 24/7 and she deteriorated quickly but together we made best use of her ability for outings and home entertainment. These activities were emotionally important for her and now for me as she sadly died in July and I have those last happy memories of us enjoying life together.
So glad to see more attention paid to "our" disease. I was diagnosed in May 2008 - having been fit and active all my life! -and was given the standard 5 years. Well, that - my "sell-by date" as I call it - was 15 months ago, so I am WINNING. I am past the stage where exercise as such is possible, but I still DO as many ordinary little things as I can. A recent fabulous breakthrough for me was a shot in the dark by my wonderful consultant - 4mg of Dexamethasone per day. Suddenly my appetite came back, I could get up at 07.15, I could cook |(my hobby - my wife does everything else...) and some energy has come back. Compared with how I was 5 weeks ago I feel great halfway through my 80th year. Breathing still a major problem, and we've just had to cancel a very expensive cruise, but we are determined to make the most of the rest of my life. Good luck to you, Dave. Don't give up! Derek
my husband has ipf and his health has gone downhill rapidly since diagnosed last year,was on fluimucil a drug which gave him terrible cramps, his breathing is bad cant walk five yards without stopping for relief,his is on supplemental oxygen and is completely housebound except for doctors visits for which we bring a wheelchair. he is having a colonoscopy and endoscopy as his blood count is too low and doctors suspect an internal bleed. he has been an avid fisherman and does miss his days on the lake however we feel so lucky to have him at home regardless of any problems, take care and you will be in our thoughts, maeve
We have a dear friend with IPF so I was glad to know more about the disease & how it progresses. As a fellow copd patient I know how hard it is to breathe sometimes. It is so sad there is no real treatment & I pray with more awareness there will be more research & treatments in the future. Keep exercising, even if it's just sitting in your chair or on your bed. And stay positive until that final injection & final breath.
Hi David, what a unique story, I have suspected fibrosis not yet definitely diagnosed having been very short of breath since march when I developed a cough during the week that we were hit with very bad air pollution, since then I have been treated for Chest infection, age onset Asthma, blood clot on the lungs(turned out not to be), fluid on the lungs due to heart failure. Just yesterday I was told it looked more like fibrosis and need to see a lung specialist. It is very scary and wish I hadn't googled it but too late now, I can only hope it is not, I am 67 and have the most loving husband and hate the thought of leaving him and our 4 children and all our grand children. I am trying to stay positive, exercise is not easy for me as I have a problem with 2 discs in my lower back and have to use two walking sticks, thank goodness for places like this where people can just waffle on to get it all in perspective. Linda
I found out January 22, 2014 that I have IPF. I went through 36 visits at a pulmonary rehab exercising to get my strength built up to help take care of myself. I'm on 6 liters oxygen at rest 8-10 walking and exercising . You have answered some of my questions but found none yet. In 2008 they diagnosed me with just interstatial lung disease take prednisone ad air and albuterol then September 2013 I asked for supplemental oxygen it was 3 liters then. Thanks for your story may your days be peaceful and prayers for you and your wife and family. Kathy Cox
David, I got this link from a wonderful person named Taleena, through a message board on Yahoo. I want to thank you for sharing. It brought me to tears. The biggest concern I have is the not knowing, and dealing with the financial concerns to keep my home, while fighting the disease. While I am currently keeping my oxyben levels easily in the 90's, the moment I start moving, I become winded, and my heart goes crazy. The standard diagnosis is 2 to 5 years. I am coming on 5 years this December, and thanks to Social security, I spent 40 months without insurance, during the time I needed most. Finally I have left the county medical system, and with new drugs coming out, I hope I can get better care, and slow the progression before it gets worse. I also have a large amount of weight to lose, having lost 85 pounds so far out of 200 from my heaviest weight. I just wanted to share. Thanks for listening!
What a brave and courageous man. Wishing him and his family peace, love, and God's blessings. Thank you for sharing!
I too was diagnosed with IPF in 2008 and less than a year later, received a double lung transplant in Pittsburgh, PA. In April of 2008 a donor's fate gave me my second chance, and five months later I went back to work in my day job. I wrote a book about the life and medical partners that helped me through the challenges of IPF. Partners 4 Life, is the book and I am the author, with all of the profits going to the research of IPF in honor and in the name of my donor. Check out my blog for the details at www.partners4life.org. Keep The Faith.
Thankyou David,for your strength,honesty and corouge,I have never met nor spoken to you in person,but because we share this disease I feel I will always think of you in the days to come,I am so glad your picture is up there also,I will always remember your kind face. Love and positive wishes to you and your fabulous wife,I too am blessed with a great husband,there you go we have 2 amazing spouses so for that we must be grateful. May god bless you and keep you smiling for many more days to come. Morag.
Thank you for sharing this story. I am going to share it with my online support groups and others I know. What a courageous man. IPF took my mother from me in 2009. While I miss her, I know she is "breathing freely." Bless you David.
David You've answered questions doctors try to ignore whether they are afraid to tell you or simply don't know. My Dad has IPF and is coming to the end as its 5 years (the life span they give to sufferers). Recently he has developed Parkinsons and seems very calm. I am my Dad's 24 hour Carer and have tried in vain to find out what to expect in the coming months as Dad will stay as home. You are a remarkable man David and I hope and pray that when the illness takes its toll it will be peaceful and take you to a wonderful place when pain and breathlessness will never abide. I have never heard of the pain relief that you mentioned as everyone tells me that with IPF the end stage is heart failure...Thank You David you've made a difference. xxx
Just wanted to thank you David for writing this blog, your words had special meaning to me as my Dad suffered from IPF and we experienced his final stages this year. We were lucky in having a community matron assigned to Dad, her support and guidance lead to respite in Myton hospice followed by his palliative care four weeks later. Dad and the family were truly looked after at Myton and I would advise anyone with IPF to consider as an option. At first we were against going to a hospice but after the first respite visit Dad knew it was the place to be. It gave us all a piece of mind as any anxieties were quickly and effectively dealt with. They let Dad take the driving seat, he was in full control right till the end. There is support out there you just have to look for it. I wish you well and hold you in my thoughts David.
Hello Jim, I've been working with David on this post and will be sharing the comments and messages with him soon - if you do have any words of comfort or support I'm sure he'll be grateful to hear them and I'll be happy to pass them on. All the best, Suzanne - British Lung Foundation
Shame we cannot contact David - even to offer just a few words of comfort.
Thanks for sharing your expericence with us I have copd so I hope you cherish every moment you have left with your loved ones
So sorry to here this David I lost my husband also David to lung disease I cared for him just like your wife I was told my husband would need a lung transplant however he didn't make it on the list as he was over weight ;( my husband went peaceful at the end he asked for that injection my heart goes out to you David & your family god bless you x
Hi David t hanks for sharing your expreicine with us I have copd it's good to know there are good nurses out there to help ease our sufferi mg God bless you for your fighting spirit
What a courageous man, thank you for sharing your experience David, I wish you comfort and dignity as you face the journey ahead. Shirley
Thank you for sharing this with us I have copd God bless don't give up the fight it's made me less afraid to know there are people out there Dedicated to helping people with ing diease
How brave you are, and its lovely to know you have kept your sense of humour hope you have lots of happy days with your family god bless you all
My very best to you and yours David. Lesley a fellow weezer. X
you are a brave man, like my husband was facing the same outcome, reading your story is reading ours almost exactly. i wish you peace and your wife strength when the time comes, but till then, I wish you life.
thank you David for taking the time to share your experience, I am sure it will help many who are in the same situation. You have shown how talking can help immensely. Cherish every moment you have.
Hi David thank-you so much for talking and sharing, especially the bits we might normally avoid.I like yourself wonder how the end might be?So I am comforted by your words and information.I have severe emphysema and asthma.Sadly my daughter is waiting for her diagnosis they think she has ipf? of course im hoping its not this but the Brompton have said they can see fibrosis on her lungs and think she has copd too.We will take it a day at a time for now.Exercise is the one thing I am lax on,really must get myself in gear its so good to hear people like yourself as its so very encouraging and inspiring.I wish you well David at this last leg of your journey may you laugh and enjoy your loved ones and friends for as long as...Once again thank-you for sharing David. :) Janexxx
Hi David,Thank you for sharing ..All my love and peace to you and your family..God Bless. Laura...
Hi David, Thank you so much for sharing your experience, thoughts and feelings so honestly and openly with us. I hesitated to respond at first as I have COPD not IPF, but there is much we all share in common and we can all learn from and appreciate your words. Wishing love, comfort and peace for you and your family, Vanessa.
Wishing David and his family peace and love in the coming days and weeks. Thank you for bravely sharing this.
Having read about your experience David, I admire and respect your ability to get a handle on what this disease is doing to you and of course the affect for your family.I am at the beginning of this journey and so shall be taking one step at a time. I hope that I shall have the courage and fortitude that will be needed. none of us know how capable we shall be until  we take those first steps.................
Dear David My heart was warmed when I read your story my father passed away from Fibrosing Alveolitis in 2002 and recently I did a 15,000 ft sky jump in his memory on Salisbury Plain raising funds for our local North Devon Breathe Easy Group I hope to have reached £2,000 once all monies are received. I have given a talk to the group about my mad plan and they were just such a lovely bunch of people surrounded by oxygen cylinders and bags and strange noises like Darth Vadar we all had a lovely time. You are amazing David and so is your wife. We nursed my dad to the end although he died after an emergency in hospital we were with him. Live every day lovely man and god bless you both.
My dad was diagnosed with IPF in 2007 but this last year he has deteriated really fast, he's lost over 30 kilos and is on oxygen 24/7 he hardly eats and he sleeps most of the day. He struggles to walk a few steps. It breaks my heart watching him struggle. My mother takes care of his meals but he has a nurse that comes three times a week to shower him and a cleaner to look after the house. Mums struggling to do things herself as she's got rheumatoid arthritis so she's in pain most days. Dad wants to stay home and mum wants him at home but it's hard on them both. I know he's at the end of his life  and it scares me. 
I was told I had this disease in a non caring way, so that is the way I took it, witha grain of salt. Then my first cousin died of this disease, so I started paying attention. My O2 are at 93 but the coughing breathing, energy level has gone way down, just in last few months. It has been 4 yrs since Dx. All I can say to all of us is to look up, look forward and never give up. I will not .
I have only just come across David's story and I hope he is still out there fighting. I was diagnosed in May 2011 and still "raging against the dying of the light" (short poem by Dylan Thomas try and read it) I don't trust averages. You have to fight it all the way but the symptoms do catch up with us all. I am oxygen 24/7 Level 15 in the day 10/12 at night to be comfortable. That's delivered by 2 x 7.5 concentrators upstairs and down stairs with a 50' tubing. Unsteady on my feet now due to muscle decline but I still get up the stairs on my hands and knees and come down on my backside. I still try to toilet, wash, shave, shower (all sitting down) on my own and will keep doing that as long as possible. Can only walk 5-10 yards before needing to sit down and recover my breath. Outside I am dependant on a wheel chair and I also have a type 2 scooter which gives me the independence to get to the local shops I exercise as much as can with the oxygen demands but it does get harder as things just diminish or tighten up. I don't get hung up about it though and just do what I can We still get out and about using the LOX Strollers and the "400" cylinders. That curtails how far you can go and how long you can be out for because it depends on what oxygen you can carry/transport. We still go to the theatre, visit sports venues for cricket, tennis and rugby. I have had to pack up Ballroom/Latin dancing and golf ( which I did carrying oxygen) They were 2 of my favourite pastimes but I intend to take up short mat bowls and in the Spring do green bowls. Trying to see if I can take up swimming again with somebody walking on the pool side carrying an oxygen cylinder We had 3 holidays on canal boats with being on oxygen. The oxygen companies (Baywater and Air Liquide) are excellent in trying to help you live as normal a life as possible. So my advice would be Don't trust averages Exercise as much as you can then plus 5% Live every day to the full Get out and about no matter how hard it can be Dont look back look forwards Be positive Keeeeep breathing everybody regards Terry Whitehouse
Hello fellow IPF sufferers on oxygen. We all know how it is important to keep our airways as clear as possible to maximise the efficiency of the oxygen We also know that brings 2 problems 1) A dry mouth all the time. I tried saliva sprays, drinking water nothing was efficient for a length of time 2) Nasal passages getting blocked up that is difficult to shift just by blowing into a hankie. They get blocked with mucus and sometime blood which set like concrete. I tried cotton buds with water, I tried squirting water up my nose, I tried the sinex stuff from chemists ,but I found none if it worked 100% all the time This is what I do , but suggest you speak to your oxygen nurse and get their advice before trying anything 1) I chew Gum (sugar free of course) That stimulates saliva all the time. Also constant chewing exercises the jaw bones and can help with unblocking the Eustachian tubes I found. 2) Get a stool and sit in front of a wash basin. Make sure it is at the right for you to be able to rest your chin on the edge of the sink (use a towel to make it more comfortable if you need to). Fill the basin up to the overflow with whatever water feels comfortable …. Cold, Tepid, Warm, Hot. In the Summer cold can be nice but in the Winter I use warm water. You can add salt if you wish as we know sea water does unblock the sinus’s. Keep the cannula up your nostrils. Bend your head so that your nose is submerged , but leave your mouth above the water level. Don’t panic…the oxygen still flows through the water. It is just like using a humidifier bottle. Stay like that for as long as you feel comfortable. (occasionally just breath in from your nose in small bursts of a few seconds, if you want to just get that bit of extra water into the nose.) I do it for about 5 minutes. Don’t worry if you do breath in some water into your throat, just stop and spit it out and start again. It is like having a mini jaccussi up your nose....not at all unpleasant !!! After you have lifted your head up blow your nose. Frankly I just blow it out into the water, let the plug out and start again with fresh water. After you have finished, dry your nose and then use the KY jelly or whatever you use. I use a steroid nose spray from my GP to ease any swelling. Repeat as often as you feel the need to do so. Sometimes I find I have to do it for 3 refills. I do this in the morning as soon as I get up (as it builds up worse in the night) , at noon, and before getting into bed. Sometimes during the night if it wakes me up, I do the water treatment then as well. I hope this all makes sense and that you might have found my experience and advice useful. Keep breathing Terry
Today my friend died of IPF. I remember when she gave me the news of her diagnosis and how I cried because I did not want to lose my friend. She told me that she had decided to live life to the fullest one day at a time. She was on oxygen 24/7 but still stayed active in her AA recovery program. She was instrumental in helping me to go back to AA after a relapse. I will miss my friend and my heart goes out to everyone who suffers from this illness. I pray for each of you that a cure will soon be found. RIP Gloria M.
I was just  diagnosed in April 21,2016 with IPF and it's been very hard for me. Today is November 11,2016 an I'm very sick and my lungs are full of  mucus and I'm so tired and I don't want to go to the hospital because I'm scared I'm so week and I don't feel the same as I did a week ago . My body just don't feel right even if I'm laying down I still feel like I have been working like for ever and I can't even think clearly. Is this normal? 
You describe my dads disease progression so accurately. You sound like you handle your illness with such grace. My dad was afraid of what the end would look like. No one could tell him as the disease is so rare. He resisted the injectable morphine & medazolam for a long time, but it helped him so much at the end. I believe he imagined his passing would be a traumatic suffocation, the idea of which was terrifying. In the end he drifted peacefully away a week ago today - his fight for breath was over & he was at peace. I hope this reassurance helps you or anyone else reading this. 
Hi David thank you for sharing your story .I to have IPF 2017 I found out the doctor gave me 3 to 5 years. I was wondering what the final stages of IPF was and now I no .I hope you are keeping as well as you can i wish you all the best .
My husband was misdiagnosed 4 years ago and just 2 years ago was diagnosed with IPF. He has always been active and is trying to work, but he is on oxygen 24/7 now and in lots of pain. His O2 level fluctuates rapidly, sometimes it drops below 70 with oxygen. He becomes weaker daily and is requiring more care from us, his family. I always wonder …. How long do we have together. This disease is beyond awful.

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25 July 2014