Wrestling with the End Stage of Pulmonary Fibrosis

[deborah]

I have been diagnosed with emphysema, bronchiectasis and Usual interstitial pneumonitis lung disease..the diagnosis came dec 2021 but ive been sick on and off for years i just didnt know what was wrong..Usually i would get sick every year and stay sick 2-3 months at a time. But this time i got sick in the end of July and it just kept progressing so i pushed for an xray that started the journey to finding out it was my lungs..

They say mild to moderate and thats like saying theres only a little poop on your kitchen floor..it still smells no matter the size and this well outside of the Lord giving me a miracle this will be how i die.

I havent bounced back as i have before and im more aware of how im feeling and ive retired now and have no health insurance and im on a very limited income..but i need to take time to take care of me and get things in order in my life.

I have a relationship with Jesus so its not that im afraid to die im more concerned i dont know as this progresses how ill be able to take care of myself, my pets and home. Im praying the Lord takes me before it gets bad but its up to him the timing of my death..

I dont think im struggling with this yet. The diagnosis is still to new and maybe im adjusting to realizing whats been wrong with my health all these years..

Sorry for rambling these r just my thoughts on it.

[dougpys]

I assume you live in the USA.  I would think you are eligible for some kind of insurance with all of your lung issues.  Call your county health services and see what advice they could give you.  Good luck.

[deborah]

Hey Doug, ive applied for disability and waiting..but unfortunately i fall into a low income category that doesn’t have health insurance for me. So i wait on the disability decision

[adelaide-campbell]

I’m so with you, Kim.  I want to make plans.  I’m a person who needs to make plans.  Otherwise I worry worry worry.  But I can’t get a straight answer.

I know I’ve only recently been diagnosed, but that doesn’t mean that’s when the PF started!  Not at all, I remember symptoms (SOB, which I dismissed, 2-3 years ago).  This year it’s got worse …. but will it not be so bad in summer?

It took a while to absorb the diagnosis.  Double whammy, this AND old age.  I’m so not ready for either.  Life seems bleak.
So PLEASE, I want to know, two or three or four or five years, or LESS?

I want to look into MAID, but I don’t know if you have that in the US.  I’m in Canada.  My PCP is against and she won’t discuss, but I haven’t talked to my pulmonologist yet …. Getting a rare half hour appt with him doesn’t give much time.  I can make the decision myself, with support from husband and friends, but ….. when?

Ah well, a day at a time.  Addie

 

[jerry-genesio]

Addie: I was diagnosed with IPF two years ago and I’m also wondering about the end stages. I have requested an appointment with a pulmonologist and will update this if I learn much new that might be helpful.
Jerry

[mary-e-gemmill]

I am in year 2/3 of IDF (formally diagnosed), probably longer with increased shortness of breath, cough, etc. Was going along reasonably well until had some chest discomfort and increased shortness of breath. I also have severe Osteoporosis and have within the past month sustained two compression fractures of L2 and T11. The pain has been incredible! Not sure if I have not compressed another vertebrae and today has been rather rough.

I, too,  like to know what to expect -primarily so I can prepare myself and my sons as to what is coming. Have mentioned Hospice to one physician who says I am not there yet. Am on Palliative Care and I suspect have to have a frank discussion with them.

Even if news is not good, better to know and be ready than be caught unawares.

Mary

[richard-martin]

As a current IPF patient I want to thank you for your research and article. Not pleasant but very informative and helpful. I have asked a half dozen doctors the questions you asked and got nowhere as you also experienced.
Again a big thanks for your efforts
Rich

[paul-carey]

IPF is typically difficult to diagnose; in my case the onset of symptoms was experienced in 2015, with the actual diagnosis in June 2018 following a lung biopsy.  Referral to an experienced Pulmonologist is essential – the earlier, the better.  Describing the progression as “mild to moderate to severe” I am currently in what is characterized as the “moderately” impaired state.

My disease does not advance as a “straight” progression, but rather is characterized by a number of “pauses”, for which the actual mechanism isn’t fully understood.  I am currently in a “pause” that has lasted almost two years.

Following my diagnosis, I started Esbriet, which, following a year resulted in a severe Erithroderma, which was eventually controlled by a regimen of Prednisone over a period of six months.  I have been taking Ofev for two years with minor side effects which are manageable.

The important question in my case has been “Am I a rapid progressor, or a slow one?”

On the basis of the past two years I am pleased to say that I seem to be a slow progressor.

The two available anti-fibrotic drugs are beneficial for the effect they may have, in slowing the IPF progression.

As a volunteer active in Emergency Medicine (as an EMT until 2015), and having lost my father to Emphysema, I can share what I know about the end stage treatment of these diseases. FIRST, however, PLEASE keep in mind that our understanding of how we might treat fibrotic lung disease (or “scarring”) is advancing rapidly, partly due to the similarities of advanced COVID lung disease.  The belief is rapidly spreading that IPF may be widely underdiagnosed.  The outlook for development of effective treatments is better than ever, so, I would say, whether or not they come “in my time” or not, THE HOPE IS REAL.

The effect of advanced IPF is, bluntly, that we gradually suffocate.

Palliative care can spare us from the effect of “struggling for breath”, by the introduction of morphene, in addition to other measures that serve to maintain comfort.  I would strongly urge discussion of your preparation for late-stage treatment with your caregiver(s) at any time you feel prepared to do so.

As a practicing Christian, my Faith leads me to believe this life is but a preparation for the “Greater Journey”, and that it matters not, however late in this life I may have come to believe and trust in the Grace that is offered to all.

My purpose and hope in this posting that each reader may find something useful.  My respect for the dignity of others also prompts me to add that you are welcome to disregard any part that is not useful to you.

With my warm best wishes,

Paul.

[denise-anton]

AMEN Paul. May God bless us all!

[carol-dyck]

I read with great interest the previous writings here and really can’t add too much to what has already been said.  In 2014 I was having an x-ray done in preparation for a shoulder replacement, when my doctor mentioned the ‘scaring’ in my lungs.  At that time I didn’t think I had any breathing problems.

Since then of course, it has been getting progressively worse and I have been on oxygen for walking and OFEV now for about 2 and a half years.  My energy level has certainly decreased over the years, but as long as I am sitting or lying down, I have almost no breathing problem at all.

I too have wondered about “the end” and assumed it will be a ‘smothering’ … which isn’t a pleasant thought.

We just have to take one day at a time and make the best of every day.   Like you Paul, I trust God will care for us when our time comes.

It is comforting to know that I’m not alone in this awful disease and it’s helpful that we can chat and express our feelings to people who understand.

Carol

 

 

 

 

 

 

[rthorntonbresnan-net]

I too have wondered this same thing. From reading all the postings, it seems there are alot of us wondering and not getting any real answers. I have read other reports and it seems that the answer is usually a smothering for the final. I also have 4 different heart conditions, colon ulcers and recently have been diaginoised  with the start of alzheimer. So, which one is going to get me first? Since 1992, I have had 3 heart attacks with 3 stenz installed. Recently a stroke due to one of the heart conditions. So, which way do I want to end it, only haven knows. I wish everyone luck on their continue survival of this terrible disease called IPF

[chris-soper]

Whilst his Inquest is still to take place I can maybe ‘help’ by describing my late husband’s passing.  He died at home in my arms, gasping for breath and begging me to turn the oxygen up.  The machines were already at maximum (2x9L), he’d gone through a whole series of face masks with various limiters and faced with his ever-increasing need for higher oxygen levels I’d asked the oxygen nurse what options we had when he reached the max but needed more.  “Nothing” was sadly the answer other than try to relax as being stressed/anxious increases the need for oxygen.  Very difficult – nigh impossible really – to relax when you can’t breathe….

The oxygen nurse did tell me that tiny doses of oral morphine hourly would aid relaxation and we did have some guidance on relaxation techniques.

Best advice is to try to stay active as long as you can and avoid being bedridden.

There was no warning that my late husband would die when he did.  (He’d been bedridden for seven weeks after falling and breaking his hip, which the hospital had refused to pin….  He’d had a heart attack six months previously and stents six weeks before his fall.  He was on 4L ambulatory oxygen on as/when needed basis when he fell but after four weeks in hospital he came home – all they were doing was giving him pain relief – and by then he was on oxygen 24/7, 12L prescription.)

Mentally he was good and physically he was still strong.   A week before he died he even manoeuvred himself across from one bed to another – broken hip and all, no support, nada and an hour before he died he pulled himself up the bed on his own to aid his breathing.  One minute he was ‘fine’, the next he was panicking/begging for more oxygen and just couldn’t breathe….

Best way I can describe it is when you’re choking and can’t catch your breath.  It happened to me a few months after he died.  Cake crumb? Stress?  Not sure but definitely not pleasant.

Hope that helps in some small way.

Good luck to everyone associated with IPF.  We knew diddly squat about it and here in the UK it takes far too long to diagnose albeit granted the Covid pandemic didn’t help!

[christie]

@chris I’m so sorry for your loss. As painful as it is, sharing your experience here will be helpful for others. There does seem to be a lack of awareness about this disease, and since each case progresses so differently, it can be hard to predict or describe what the end will be like. It makes it hard to prepare as a caregiver, or even a healthcare provider. It must be hard to come to terms with how his life ended when he seemed so strong one moment and was in crisis the next. My heart goes out to you as you try to make sense of that and grieve. He was lucky to have you there with him in his last moments. We are here for you anytime you want to talk about him.
Christie

[chris-soper]

Thank you Christie.

[christine-mccann]

We all have to die, everyone is different so it is hard to say, all I do know is that when I start to struggle with the idea of smothering I make myself refocus on something else to keep the anxiety at bay, that is the worst feeling in the world to me , I would rather be in pain than to feel what not being able to breathe feels like. The anxiety causes claustrophobia also. I am hoping for a heart failure in my sleep.
I am on Esbriet and have been for going on 5 years, I am fortunate to not have a lot of phlegm or congestion or coughing as, I also have Emphysema and COPD I have had pneumonia and Bronchitis and supposedly some Asthma in the past but, for the most part I feel normal as long as I don’t over exert. In the past few months I have noticed my shortness of breathe has increased. I use my oxygen more now, I fully believe that if I had not been on Esbriet all this time I would already be dead.
It is difficult to come to terms with end of life, We in this country do not prepare for , or even acknowledge it very well. It surprises me how little some Dr’s will explain things to people who really need to know.

[antonia-jensen]

I was diagnosed a year and a half ago with NSIP, fibrotic version, and now use oxygen on exertion. Since I live alone, I have had to think about this question often since both of my adult children live a good distance away. I have already completed my advance directive, basically a DNR, use only morphine. I think at the end we shouldn’t stint on that, since drug addiction would be the last thing anyone would worry about. Hospice has very good protocols for that sort of thing and we ought to trust in it, for no other reason then it can give peace of mind nowadays.

[ron-bishop]

I too am wrestling with an array of emotions as my health declines. Honored and comforted to journey with this compassionate group

[sarah-jayne-saunders]

Hi my 1st post I’m newly diagnosed  but I’ve known a great deal has been wrong with my lungs for at least 6yrs and apparently they saw fibrosis 3yrs ago on my scans etc and didn’t tell me, I can’t tell you how angry I was to be told this. My question  to anyone is does anyone still smoke or did you find it hard to stop im still smoking my brain is angry at me for it but my addiction to it is so ridiculous I don’t know how to stop the pains in my lungs my back and chest are getting worse dose anyone gave any advice not judgment  please  Tia

[patricia-meadows]

Our support group asked the organizer to have someone come in to speak to us about what it is like to die from PF.  We were fortunate to have the head of palliative care in our province (Alberta) address us.  It was one of the best-attended meetings we’ve ever had.  Despite the fact that talking about death is difficult and uncomfortable for many, the speaker’s message gave us the reassurance that at least here in Alberta, those dying from PF are 99% likely to pass away gently and without the panic and gasping that we might envision (and sadly, may have witnessed).  Morphine and/or hydromorphone can be used here to deal with the possible sense of breathlessness or panic that some suffer.

It is always terrible to hear about someone dying in great discomfort.  It’s so awful for them and their loved ones.  Both my parents died from lung disease related to smoking (Dad had emphysema; Mom had lung cancer).  I was blessed to be with them when they died, and in both cases they fell into a deep sleep, then a coma, with one last exhalation and they were gone.  No distress, no gasping or struggling for air.  I wish everyone and their families could have a similar experience.

If you have access to someone in the medical field who can talk to you about the end stage of PF including the dying process I would urge you to do so earlier than later.  As I said, a lot of us in our support group got comfort from what we heard, and now are also better equipped to advocate for our selves and our loved ones for a better experience.

 

[brendacolvin]

Reading these comments have been very informative. I was diagnosed 2 years ago. No medication as of yet but going for CT and extensive blood work. Pulmonologist appt. 17th of May. I am a Christian and I know God has a plan for me. Whatever comes I know he will see me through. But I agree no one wants to die gasping for breath. Hopefully all these trials will produce a medicine to help all of us.

[bob-chiu]

At 74 and using 10 LPM of oxygen, I find it comforting to read Patricia Meadows’ post above, that 99% of patients pass away gently and without panic and gasping.

To echo the findings at Alberta, one of my doctors told me that air consists of 71% nitrogen and 21% oxygen.  If a person inhales pure nitrogen, he is likely to lapse into unconsciousness and die. As our lungs take in less and less oxygen, naturally more and more nitrogen will fill the lungs.  However, the speed and pace of lung function decline vary from person to person and different people will experience different symptoms during this long process. But at the very end, as more nitrogen is taken in, the person will likely sleep more and more, be weakened by the lack of exercise, and finally lapses into a long sleep and perhaps die during the sleep.

Anyway, it has taken me 19 years to get to needing 10 LPM from the point I found myself short of breath.  I stopped playing golf 3 years ago but I still exercise 3 times a day for a total of 1.5 hours. I feel that exercise is very important in keeping our body strong so that we may have less or minor symptoms.

[barry-dukker]

I have just been diagnosed with IPF I have all the support you could want from the family, and the medication seems to help I try to be realistic but can not get rid of the overpowering fear of suffocation in the end-stage it is in the back of my mind all the time I dream about it and begin to have fear of going to sleep. How do you cope with this, or where do you go for help?

[bob-chiu]

Hi Barry,

If you are being diagnosed just now, you may have many many years ahead of you because the fibrosis can develop slowly. For me it progressed over 15 years and a friend over 19 years. Therefore it is important to live a normal life and not worry too much about what will happen years from now. Current medications are proving to delay fibrotic progress by 50% or more. Who knows, in a few years new and better drugs may be introduced.  In the meantime, besides following doctor’s orders, It’s important to exercise to keep the body strong, and do not be stingy with the use of supplemental oxygen if needed.

[antonia-jensen]

Why do we wrestle with this issue? Why can’t doctors take the time to give us answers? I too wonder just what my end will be like. Of course we all wish that it is without pain and peaceful, perhaps in our sleep. I don’t understand the medical community’s  reluctance to talk about this. Knowing would give us all so much more peace of mind in our daily lives!

[dougpys]

As far as I know we humans are the only creature that can make the present worse by focusing on what may happen in the future or what happened in the past.  While it certainly is easier said than done, I focus on today and the very near future and make it the best I can.  I don’t know how much longer I have as I am on high levels of O2 and very limited to any kind of physical activity, but I try to make the best of each day .   I do hope for advancements in treatment for the future.  In any case I have confidence by docs will minimize my suffering when the end comes.  Aphorisms help me.  “When the going gets tough the tough get going.” “If I get knocked done I’ll get back up.”

[marj]

After reading all of the above I have to say that when my husband was first diagnosed his first words were “I don’t want to die gasping for breath”.    He was diagnosed just over 5 years ago and has remained quite well, just the odd off day when he struggled.  He doesn’t have supplemental oxygen yet.  For the past two weeks he has felt really poorly but couldn’t put his finger on what was wrong.  We eventually saw a doctor last week and he sent him to hospital (in UK) , he was diagnosed with Covid pneumonia.  His oxygen level did drop to 91 but his heart bpm dropped to low 50’s and at one point in hospital they woke him because they fell into mid 40’s.  He was discharged with antibiotics after 4 days and has now finished the course.  He still has a “productive” cough and feels like crap.  We do keep taking his O2 readings which are now back to 97 but his bpm is still under 60.  Maybe this is normal for him?

I’m sure other spouses etc wonder “is this the start of the end”.  I wonder if he even should have been discharged, but as you can’t get onto the covid ward you can’t ask the questions you feel you need answers to.  Getting an appointment with a pulmonologist is virtually impossible – you have to wait for your anniversary appointment.  He does get upset when he has a day that he can’t do things because he can’t get his breath and his nose is constantly dripping.

Apart from the ipf, he has had a catheter fitted for the past 12 months while waiting for an operation to have some prostrate removed – he should have had his pre-op last week but had to cancel due to being in hospital.  I telephoned to get this booked back in and May is totally booked up and they haven’t opened the June appointment calendar yet !

I lie awake sometimes listening to him breathing – his breaths are really shallow – just like a little pant out then every now and again he takes a deep breath.  I get concerned that he isn’t getting enough oxygen during the night.

I don’t know if I over think things – does anyone else’s carer feel like this?

I don’t know if

[dougpys]

Marj, I don’t know what advice I can give you living in the UK—I was first placed on O2 after my pulmonologist had me do an O2 check while I slept.  He found, which is very common in people with IPF at some  stage, that while asleep my O2 went well below 88%. When you sleep breathing becomes quite shallow at time.  That is why they prescribe a continuous O2 machine, as shallow breathing will not always be picked up by a pulse machine.   As a result I was furnished a home O2 continuous machine I used while sleeping.  My O2 provider also furnished me with a portable Simply Go Mini which I didn’t use at first, but after awhile used it when I was active outside the house.