Wrestling with the End Stage of Pulmonary Fibrosis
One of my frustrations as a pulmonary fibrosis patient is finding solid information about what to expect as my PF progresses. I’ve asked several doctors what to expect during the end stage of PF, but often get the same answer: “We’re not there, no need to talk about that now.”
I want to talk about the end stage
I’m the kind of person who is comforted by knowing what to expect, so I can prepare, even if it is bad news. However, I’m sure for some people, getting specific information would be too distressing.
I decided to write this column last week, when someone in a PF Facebook group asked, “Do we continue with weakness till we cannot do anything or do we keep losing lung function till we cannot breathe and die?”
I answered, “Your frustration is so normal. There are many other diseases where symptoms and progression is known. PF is different, and its progression is different for everyone. There are so many factors: etiology of PF, age and health of patient, and a whole lot of unknowns, which is very frustrating. My experience over the last 3 1/2 years has been periods of stability, and slow periods of decline. My understanding (in general) is that it slowly gets worse until we become bedridden and pass away. For some, there is the possibility of a lung transplant. I’m sorry to lay out a stark answer. I wanted to answer your question honestly.”
Discuss what to expect during the end stage of pulmonary fibrosis in the PF News forum
I’m not sure how helpful of an answer this was, so I decided to do some research to better understand what we can expect during the end stage of PF. Most of the information I got from caregivers who shared in online groups what their loved one’s end stage was like. As you can expect, one size doesn’t fit all, and there are a lot of variations. I am not a doctor. I am relating what I’ve been told by about 50 caregivers.
Here are the themes I saw
- A gradual decline in lung function over time, although for some this can be sudden.
- Patients require more oxygen over time when at rest and especially when moving.
- Over time, it becomes harder to walk and go out because of shortness of breath.
- More fatigue, more sleep.
- The heart works harder to get oxygen to the body, which may result in the development of pulmonary hypertension. Leg swelling may occur as a result.
- Patients may eat less and experience weight loss.
- Sometimes increased anxiety and depression are experienced.
- Some caregivers reported a peaceful and calm passing, while others report pain and anxiety the last few days.
Here’s what I learned
Everyone experiences a different pattern of progression. Some people remain stable for years before worsening begins. Others seem to have a rapid progression from the day they are diagnosed. Most people fall somewhere in between, with periods of stability alternating with periods of progression.
I’m pursuing palliative (supportive) care. This treatment is focused on relieving and preventing symptoms that are distressing. I was glad to hear we can still receive “curative care” at the same time. Curative care refers to participating in research studies, being evaluated for lung transplantation, and considering new medications. This is not hospice care, which is treatment administered during the last months of life. Palliative medicine involves a team of healthcare professionals who consider physical, psychosocial, and spiritual factors in their treatment approach. I’m meeting with someone next week and will let you know what I discover.
Ask for hospice as soon as you can. Your doctor can order it if he/she believes you may pass in the next 6-12 months. Dr. Molly Bourne shared at a recent PFF summit that patients with advanced lung disease live on average nine months longer. Now, that sounds good to me! Hospice provides a great deal of support to you as a patient, as well as to your caregivers and loved ones. They really need the support, too.
Talk with your loved ones ahead of time about what you want treatment-wise. For instance, I’ve already told my husband and my doctor I do not want a ventilator and have signed a DNR (do not resuscitate) order. I also want to use whatever medications would be needed to help manage pain, and also use anti-anxiety medications if needed.
This was hard to research and write about
I hope it was helpful to you. This knowledge gives me a way to be proactive, for which I am grateful. After I wrote this column, I felt kind of down. I made yummy chicken vegetable soup, and it was soothing to my body and soul. Here’s the recipe, if you’d like to try it 🙂
I’d love to hear from you!
What stood out to you from this column? Do you have any other knowledge of experience about what end-stage pulmonary fibrosis is like? What brings you hope?
Note: Pulmonary Fibrosis News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website. The opinions expressed in this column are not those of Pulmonary Fibrosis News, or its parent company, BioNews Services, and are intended to spark discussion about issues pertaining to pulmonary fibrosis.
I am so glad you wrote this. I believe I am starting into the end stage of this awful disease. I have recently had to increase my O2 significantly after a long stable period. I had to have five stents put in to open up some arteries in preparation for transplant but have to stay on blood thinners for 6 months, I'm not sure if I will last that long and then there is the wait for lungs.... I didn't realize you can ask for palliative care while still treating and still fighting. I have also told my hubby that I do not want to be on a ventilator if a transplant is not imminent. Thank you for your honesty with a difficult subject
This is so difficult for me and my sister's to whack as my dad is a busy body and do want help how can we help him
Thanks so much for your comment. I'm so sorry you are in such a difficult place. I'm glad my frank honesty is a help to you. Blessings and hugs to you...
I've seen a number of comments here about predisone. PLEASE be VERY careful if you take any statins if your doctor asks you to take prednisone. If you take the two together, you are HIGHLY at risk for something called fluoroquinolone toxicity, which will affect your tendons and muscles. DO NOT TAKE THE TWO TOGETHER! this is also possible if you have to take prednisone and levaquin together, but much more of an issue if you have to take prednisone if you are already taking a statin (for cholesterol or heart issues)!!
what happens when prednisone and a statin are taken together? Isn't fluoroquinolone an antibiotic?
I am now very nervous now as I have been taking 5 mg of prednisone and 40mg pravastatin together for over 9 years and this is the first time I have heard not to take them them together.`I will be inquiring soon about this.
If you've had no problem in 4 years, why worry about it? I know many people who are on prednisone and take cholesterol medicine. If you were going to have problems with it, surely it would have surfaced by now.
I also have been on stations for 8 years and take 10 mg predisone, during celebrations in on as much as 80 mgs. No one has ever told me that, thanks
What about when PF post was brought on by Ards and sepsis and person is on 4-5 liters of o2 24/7 and tries to still work at 70 ish and o2 drops into 83-85 while brushing teeth until put nasal cannula back in nose does not have a cough or wt loss but extremely fatigued all the time . No meds for seeing as how it was caused by ards .Dnr in place .
That's a good question for your doc who has your history. However, I spent several years living in NM at 6,000ft and had PF but wasn't diagnosed yet. The ground glass was on the X-rays but my doc didn't realize what that was a sign of. At that altitude, I was lucky to see my O2 level get to 92. Mid to high-80s was normal for me. Low 80s when I was hiking. I dropped below 80 more than once and knew enough to go to bed. I lived in the country and spent my days hiking and exploring and taking pictures. I wasn't on oxygen. I have Asthma and my doctors blamed that and the altitude. It wasn't till I moved back to CA that I was finally diagnosed with PF. I am a mile from the beach and my normal O2 level is mid 90's. My only change is constant fatigue and now weight loss.
Thank you so much for this column; I have just been diagnosed with IPF. Have a health directive, but wanted to update it with the recent diagnosis. Your column, and the replies, have eased my mind about decisions regarding a ventilator, etc. The comments about palliative care were especially reassuring, and came at just the right time for me.
I'm so glad this column was helpful to you. So sorry about your recent PF diagnosis. We're all here to help :) I wanted to let you know I have a book coming out on 11/15/18 called Pulmonary Fibrosis Journey: A Counselor and Fellow Patient Walks with You. I wrote it in hopes of making this journey a little easier. It's the book I wish I had. I hope it will be a big help to you. Many blessing to you.
I have been diagnosed and i am afraid to be honest I also have Rheumatoid arthritis and does how I ended up with Pulmonary Fibrosis. I am looking for a support group to talk to and share thoughts, reading all of this comments gave made me feel better I guess just learning more about what to expect.
I also feel very nervous and depress I have lost lot of weight I do not want to be weak handling my illness I need to be stromg for my husband, kids and grandkids. It is so hard at times.
I am sorry I feel Iam all over even writing this.
Thank you I would love to hear from some one that I can talke to.
God Bless ever single one of you and Let our Jesus be with us all the way.
I was told in 2014 that I have pulmonary fibrosis it has advanced at different times in my last check up my doctor told me I lost 10% of my lung capacity he said my time left may be approx. 3 years I have already signed a dnr and have made all arrangements for my spouse and have accepted the out come when it happens I cant do much of any thing im fine setting around but if I try to do any work at all I cough all the time and have a very hard time breathing as of the last report from my doctor i only have about 50% of my lungs left so I know my time is short but ive accepted that I I will keep going till the tile comes I will not quit I hope in some small way this will be a help to any one who gets this terrible virus to all please take care
thank you all for sharing openly & honestly your experiences , my mom was diagnosed with PF 6 years ago and has been on 24/7 oxy for 4 years unfortunately she got covid and has come home to live with me on hospice (the hospice team has been amazing ) i have recently experienced a very quick onset after several episodes of high anxiety (pulling canula off ox dropping to 62-70 her norm at rest is great 85-93) my mom unable to make sense when speaking or in her thoughts? i've noticed a quick decline in her ability to make sense the content of conversations are off and just out there , i'm wondering fearing maybe she had a stroke or could this be a result of hypoxiam ? could the repeated episodes of lack of oxy to brain have caused this ?
Lester W Willis (Will)
I too went through the 80 different tests to qualify for transplant. All the Drs met and decided I was nearing the (too old) age but still very healthy. Than when they told me about all the negatives, I decided against it. Rather just live out my life and leave early maybe.
I've had a wonderful life, have a wonderful wife and family, and am very happy with all. I'm in a very happy place now and hope the next place will be as good.
Thank you for all you do for us. Keep up the good work and keep the faith!😁
Kim: I think you have done a nice job with a difficult topic. I would like to add that in addition to the lack of appetite, one of the driving forces behind the weight loss is the hyper-metabolism generated by increased work of breathing. For this reason, nutrition; PROPER nutrition, meaning high-calorie, lower carbohydrate foods (lean proteins and healthy fats) are crucial for maintaining weight and strength.
Thanks Dr. Noah! I appreciate you adding some very valuable information :)
Is there anything to be done to prolong life with end stage disease?
I'm a carer of a patient in the earlier stages of IPF and it was hard to read so I can only imagine how hard it was to write and research. I think you are very brave and thank you for doing it for me.
Wayne and Wendy Dixon
My wife and I are not sure if this is going back to the author of the above article. But we to are frustrated beyond belief. I had to find out what fibrosis was via the internet. My family doctor kept telling me each time I had a x-ray or C.T. scan that my fibrosis was getting worse, but never explained what it was. So I thought that I would end up on an inhaler somewhere down the road. It was only when I became symptomatic that I checked to see what it really was. We were shocked. And getting any information since has been like trying to get water out a stone. We are completely frustrated with the entire system...Thank you for your info and insight. This is probably the most informative information we have gotten to date, and honest. Thank you. Please continue to be honest with yourself and others..Not sure where you are located, but where we are facilities for palliative care and transplant are thousands of kilometers away.
You are so right, I have spent 2 yrs looking for this type of Blog to share and learn, had to find on my own?????? Financial, personal, emotional, family, research, so many questions and no where to go for answers, very frustrating, but now allast.. I will be on this every day!!!
My husband was diagnosed in May 2015. His progression was quite slow the first year. He was put on Esbriet very soon after diagnosis. As it progressed our pulmonologist suggested we try for transplant. We were turned down four times due to his situation being “too complex”. He is 72 now also. He was put on oxygen January 2017 after his second pulmonary embolism. Started at 2-3 liters. He had progressed a lot since that time and is now on 8 liters 24/7 and is somewhat housebound, but does still get out. Last month the Dr told him he should no longer drive. A few weeks ago he started hospice comfort care. We feel like this care has helped a lot and they provide a lot of help, although we still cannot get a clear prognosis. We know he is terminal, know the progression is moving along now, and see the signs like e tea oxygen, more sleep, harder to focus, etc. I may be in denial, but I still am hoping to keep him around for some time . It’s a miserable disease.
I wish I had known about this site but like you we didn't find out off our doctor he said he wasn't a chest specialist , we found out my husband had this when he was admitted to hospital and collapsed there , he was ventilated and put in coma he pulled through and the info was never clear on how bad he was , they sent him home he was on 4 oxygen he had 2 generators an 4 back up gas tanks because if he moved he couldn't breath and I had
To turn the oxygen up I even had to change his masks , was him he was bedridden , not a soul came near to help us , I had never looked after anyone sick before , my husband went back into hospital 5 days later , after 2 days in hospital we left him on his iPad at 8.30 pm and at 11.00 pm my daughters mobile rang it was her dad but he didn't speak a nurse took the phone off him and said your dad wants you , we flew to the hospital and was put in a side room being told we are making him comfortable twenty minutes later they came and told me my husband had died , devastated , to know we were there but couldn't hold him and tell him we loved him is the worst feeling ever, I miss him so much he was only 53 yrs old, the more people know about this terrible illness then the more knowledge they can gather unfortunately for us it was to late
I am so sorry for your loss. My heart is breaking for you. This is a horrible disease. My 84 year old mother has had it for approx. 3 years. She really has been tired a lot the last week or so. I know I will lose her in the near future and am trying to prepare myself for it. My dad is at the beginning of the last stage of Parkinsons. We also thought my dad would die first but I don't know at this point.
It is tough...we have a lot in common Julie. My almost 84 year old father has had IPF for over 7 years and has done remarkably with a gradual decline over the years. This year however, he experienced an exaserbation of symptoms and his decline is progressing rapidly. He was hospitalized for steroid infusions, is now on Hospice and continues on 30mg of prednisone, lasix, and a small amount of morphine during the day which helps his breathing. He lives in an independent senior facility and with Hospice allowing him to stay for the time being which is important to him. He gets around the facility on a scooter, with oxygen tanks in tow. Ironically, his wife and my 83 year old mother just passed in February from complications of Parkinson's. We always thought he would go first, but it is a blessing it happened this way. We actually had to get him discharged from his hospital stay ASAP in order for him to be with her when she passed. It's hard to face the loss of both parents in such a short time.
Hi Julie, my 84 year old Mum has had a rapid decline in the last two months and was admitted to hospital last night. I live interstate and it's very distressing not to be close by. We are waiting to hear the outcome of her 48 hours assessment. I'm flying up on the weekend. I am NOT prepared and very upset at what's coming. She's frightened of what is to come and expressed her worry that she will just suffocate. But knowing palliative care will be there eases that terrifying thought. :(
I watched my father die of pf at the ripe age of 91. I visited him in the hospital during late afternoon Little did I know he was dying. He kept asking me for a fan. Struggling for breath. Saying there is no air wants a fan. Well now at 70 I have been told I have pf. I can’t forget the struggle he went through the last 24 hrs. I too will gasping for my last breath. Disturbing.
I got pulmonary fibrosis from a motor vehicle accident in 2014. In the accident, I was in a coma for several months and the ventilator breathed for me. In doing so, it caused me to have severe scarring and pulmonary fibrosis. I had 2 collapsed lungs, 8 broken ribs, just a mess in my chest. I remember being on 32 liters o2 and had a trachea. I had to be weaned off of it and went to nasal canula. I, recently have noticed that it seems to be getting worse . I want to know if my case is different and can my condition improve..ANY? Mine is not from smoking or copd...or emphysema. Mine is from the accident and the ventilator. I am 45. A male. Caucasian. Never smoked. I have been recommended to get a lung transplant but I just feel like I was healthy before the wreck, an athlete. Now...it is like I am a frail car that gets winded just from drying off after a shower.
I wish I'd come across this site years ago. My husband was diagnosed in 2009 & passed away 2 years ago in 2016.He died at home which is what he wanted but I didn't realise it was the end we'd had the GP down the week before & he organised palliative care. Friday he was in the lounge having a drink watching TV & Monday he was gone.His breathing that day was terrible, I wish I had of known more about the end of the disease & I might have been more prepared for his death
Thank you for sharing. My dad passed away last month from PF in the ICU from a sudden exacerbation. It was unexpected, and we were not prepared, so I understand not realizing the end. Did any of your doctors (PC or Pulmonologist) discuss the end-of-life stages, or let you know any type of prognosis as time went on? I am trying to see if there is some commonality with PF patients/families not being provided sufficient information by doctors. Ultimately, it was an end that could have gone differently had we been more adequately prepared.
yes I have learned more over the internet than from the doctor,,,it is hard to hear but very important also THANK YOU SO MUCH FOR ALL WHO SHARE
Pulmonary fibrosis from what I’ve read is very hard to diagnose. One because the doctors treat it as a cold, sinuses, then pneumonia, etc., and also because of the patients denial. Well after almost 2 1/2 years I convinced my mother that what she was doing was not normal. It was not a cough or a cold. It was not normal! One day when the specialist ran the tube down her throat for testing, he came back in and told my mom sister that he would be changing her medicine. My aunt said will that stop her non-stop coughing? And the specialist said well that’s the first I’ve heard of this. Boy, he got busy And shortly there after he diagnosed her with pulmonary fibrosis & recommended that she have a second opinion to find out what type she has. Eight months later we finally get to go to our appointment at Barnes Jewish Hospital in St. Louis Missouri on January 25th, 2019 and as of today, February 15, 2019 we still have not received any news. Nor have any of her specialist in Jonesboro. This is so frustrating. I listen to her cough night after night and throw up all night long. And this has been going on a good 3 years now. Her oxygen level is at 90 and 91. She gets choked on everything she eats. Her general practitioner doctor told her Friday that they still have no results from Barnes Jewish Hospital and he could start her on steroids and then onto morphine. I’m sorry but morphine sounds like the end to me. Can anybody give me some insight here? I just wanna know how much time I have left with my mom and I know no one can tell me that but our Almighty God. I live with my mother. She wakes in the morning and needs to take a nap. And has for a couple of years now. That was one of the red flags for me. Because that is not my mom. The coughing has been going on three years now and for the last year and a half she is throwing up/spitting up brown phlegm, sometimes it’s clear but mostly a dark brown. Her lifestyle is so limited because just walking through the house makes her out of breath. Somebody please tell me something. I already know she’s going to die. Help… Please email me with any info and/or insight at [email protected] Thank & God Bless you all.
She needs to be put on oxygen, when she is moving around like the 6 minutes walk her oxygen level probably drop below 88, so she need oxygen. When she start to cough a lot turn the oxygen up to higher level like 6-8 the cough will settle after a few minutes, then turn it down to 2 or so whenever her normal level is. She may need small dosage of prednisone for her to function better during the day and control her cough and give her a boost of energy. Hope this will help.
My husband was diagnosed with ild feb 2017 was put on oxygen, 2 liters. Had to continue to turn up when moving around. Last year had septic arthritic hip, was in hospitals for 8 weeks and is now bedridden in a nursing home since January. Waiting for the other shoe to drop. He has a lot of head congestion. But is and has been relatively stable. I appreciate everything I read on this, knowledge is power.
Hi Denis, I live in Canada so far from my mum. How is your mum doing now?? My mum diagnosed in 2013, she has had an amazing few years, but now declining. I spent one month this past May of 2019 and found her so be tired most of the time even when she brushes her hair after showering it takes all the breath out of her. She will be 76 this month. She was always so active and walked blocks up till 2years ago when she found herself being out of breath to much. Now the walking has stopped. My sis who lives close to mum tells me I should now go back and spend quality time and help out with household cleaning and support my dad who has taken on everything in the house as mum just can’t do anything she’s so tired and her breathing is rapid. I think she has anxiety and is preventing her from sleeping, my sister is in denial and doesn’t like to educate herself on how the end will be for mum, I however is different and I don’t like surprise and the unknown. My mum and dad are too proud to have anyone come in to clean and certainly will never agree for any home care. I don’t know what to do. Do I fly out in two months spend two months then leave. Or wait till she bedridden and spend that time. I’m on the other side of the world which sucks!! Also mum was allergic to something environmental which her lung doctor said but can’t do biopsy as she is a Diabetic and a little late in the game now. She was also a smoker of many years but had quit 17years ago. Lung specialist assured that it’s not the result of smoking. It’s damage from something environmental. Like asbestos or other.
Did your mum get her diagnose?? Is it fibrosis? I thought fibrosis is a min productive cough though. Is that what the X-ray read? My mum oxygen is or was 91, which is good and doesn’t require oxygen. At least that is what mum tells me her results are, agin I’m in Canada mum in Australia and my sister has not gone to an appt with her ever. But will in March of 2020 with her lung specialist. Don’t know if he will order any more X-ray going forward where mums X-ray that I’ve seen are pretty bad like 75% percent blackened which I’m assuming is the fibrosis. I’ve had to pull out all her xrays from the past 6years and to see those pictures were heart crushing. Good news is that mum has surpassed the 3- 5years life expectancy,, it’s different for all cases. I m worried that this coming year which marks 7 years will be the worst and I pray not the last, but also don’t want mum to suffer so much. Hugs to you Denis, if you want to talk please reply I would like to know how it’s going for you now. I think the person who wrote this article about the experience is amazing and so strong it’s a comfort to all.
I'm 76 and was diagnosed 4 years and two months ago I have watched myself become so weak and fragile everyday I wake up I'm not the same person. Ipf is not nice and plays no favorites. I used to cough until I coughed up blood going to bed I hated it because I coughed even more all night until I could not breathe like 10 hours until daylight. I found out over 6 months ago after taking a swallow study test that my water and food was going into my lungs. I have a dry drown several times. I had sinusitis all the time I was always sick it was the water and my food causing this. I did not know that GERD could actually caused ipf. I have to take exercise every day for the rest of my life which will be short I'm sure this is my last year so that I can continue to swallow some food it's very hard because I choke throw up and my plate can't drink water it's embarrassing and horrible. I'm losing all my hair I can't walk very far without having breathing difficulties can't cook any more I can't do housework I basically can't do anything. I am responding because you mentioned that your mother throws up at night all she coughs all night what is her food maybe that's causing her that the same problems that I have you might want to have that checked. It's very serious when your food and water goes into your lungs. And the doctors don't tell you anything they give you a death sentence send you home to die you have to learn the hard way with your suffering from. I am responding because you mentioned that your mother throws up at night why she coughs all night what is her food maybe that's causing her that the same problems that I have he might want to have that check. it's very serious when your food and water goes into your lungs. and the doctors don't tell you anything they give you a death sentence send you home to die and you have to learn the hard way with your suffering from. I guess so cold most of the time even in the summer I have to turn the heater on. I'm always congested in the throat from where my water stays on top of my vocal cords lose my voice. I've lost so much weight I don't even look like the same person anymore. Suffering a lot of pain and I know that I'm in my in stages are very close to it I see my doctor next week most of the time but even in the summer I have to turn the heater on. I'm always congested in the throat from where my water stays on top of my local cords and I lose my voice. I've lost so much weight I don't even look like the same person anymore. suffering a lot of pain and I know that I'm in my in stages are very close to it I'll see my doctor next week and I'm going to confront him after the things that I have read here. I feel very bad my heart goes out to each and every one of you but has this horrible disease. May God bless each and everyone of you and may you find peace an acceptance.
My mom was just diagnosed with PF, but it has progressed so fast in just the last month. She spent a few nights in the hospital with pneumonia and is home now, but she keeps declining. In 2 weeks she went from 3 on oxygen to 5. She is anxious and sad and just wants to feel better, but I don’t know that she ever will feel any better. It’s breaking my heart. Thank you for the article, at least I have a little more info now on how I can help her in the end.
I can relate totally with you Wayne and Wendy - My Dr said " it's very serious but you don't have cancer" I was so shocked I didn't ask any
more- - she gave me a copy of my CT scan & I went home and googled it - I've been trying to find out what to expect - I live alone and am in my 3rd year - prayers and cheers from Australia ????
Wayne and Wendy Dixon
My wife and I are in this alone as well. Our lung specialist told us the last time we met that there was no need to see her anymore because there was nothing she could do that the palliative care team or our family doctor couldn't. We left feeling abandoned and very upset. So we can relate to your situation. Our family has gone missing in action, either because they can't face the situation or they are too busy with their own lives, so here we are like you not knowing what to expect. From what we have read it's not very pleasant. I to am in my third year and require oxygen upon exertion. Our thoughts and prayers are with you as well and take care...
I was also told that at least it wasn't cancer. However, this year I did get cancer and it was treated quickly and hopefully is completely gone. I think the Dr thought I was nuts when I said I think I would rather it be cancer when first diagnosed. It really is hard to get a Dr to tell you what to expect. I'm in 'end stage' and still don't really know what that means although I believe my new Dr will explain it all next time I see her.
Thanks for your comment. I totally understand having to deal with both PF and cancer. It's tough! I can tell you are a fighter and very brave. I write more about the end stages and preparing in a book I just released, in case this could help you. I hope so. In case it’s of interest it’s called Pulmonary Fibrosis Journey: A Counselor and Fellow Patient Walks with You. Blessings to you.
My wonderful Father was diagnosed with IPF last April after 2 stays in hospital and major organ failure. He is in bed constantly now. We have seen a gp and respiratory specialist at home once in 5 months. Palliative care have not been for 5months. My Father is a feisty tough 81 year old who was superfit and independant until 14 months ago. This disease is horrific. Godbless you all and praying you stay calm and pain free. Love from England. Xx
Where can you purchase this book?
Totally agree with what you are saying, information from my consultant and g.p. Is like getting blood from a stone. This is a good thing this site, thank you
I found out about my possible IPF checking my emails!!. I was being treated for what I thought was pneumonia I couldn't shake. My chest xray results were available online as well as bloodwork. Of course the results from the radiologist was also printed where he wrote'probable ipf'!!! Never heard of it, looked it up: no known cause or cure and possible short lifespan. What a nice way to celebrate my 30th anniversary!
I am a 67 year old woman who was diagnosed with PF in Jan of '17. Decrease in energy and shortness of breath plus occasional pain in my right side are the only symptoms so far. I had a brother who passed away in 2008 from PF and pulmonary hypertension. He passed less than a year after diagnosis. I feel he must have had PF a lot longer though. He was on oxygen the last 6 months and was put on life support at the end. I would never do that for myself and would not recommend it to anyone. I wish the the best on your journey!!!
I'm in the end stages and have recently started having pain in my right side. Can you tell me what causes that. You're the first person who ever mentioned it. Thanks!
I used to have pain in my right side too...Sharp pain. My doc didn't know, but I figured out that it was from being inactive, that my muscles were tight. When I made a movement the pain was a spasm. I started doing stretches and the pain has gone away! I hope this is a help to you :)
Hi. My name is Brandi, I was finally diagnosed at age 37. I am 43 now. I became very sick and got pneumonia then ended up on life support 4 times in the first 2 years. I am a nurse, but on disability because of PF and I got PF because I have Rheumatoid Arthritis. I went through a lot of testing and then open lung biopsy sent to Mayo Clinic confirmed it. I am on 5 liters 24/7. It is a lot to learn about and deal with. I went through all the stages of grief. Denial, Anger, Bargaining Acceptance then in the future there will be death. I am finally in the acceptance stage. My faith in God and support of my family has gotten me to today. I am steadily getting worse. I can tell you the pains in your chest are called blebs. This is similar to pleurisy.... I have learned the main thing is to stay as healthy as possible and stay away from germs... BIG TIME! My Dr told me the first winter that getting the flu really bad could take my life. So, I don’t go around big crowds and I pay attention to what my body is telling me and act immediately. I am involved in at least 3 Facebook groups for patients with IPF/PF. There is one that is private patients only then others for care givers also. You all definitely need to sign up. It helps so much to talk to others that know exactly how you feel. We bounce ideas off each other and some days we just vent, cry and get it all out and end up feeling much better. Like I said, please, all of you come join in with us. I think the one group is called “IPF/PF patients only!” A lot of drs just don’t know much about PF and don’t know what to say to a 40 something divorced sick female so I can so relate to how you feel about having to educate yourself. And please keep doing so. It’s best if you know as much as you can about this disease because as you know there is no cure, transplantation is an option but it’s a long process and you need to start it ASAP. I myself am a DNR. I am on palliative care and I am wel taken care of mainly by my family doc. She’s great. Keeps me comfortable and listens like a friend. I feel like I’ve written a book here, and was just going to refer you to our support group! Haha! God bless you all. Looking forward to talking soon.
And... thank you so much for taking the time to start this site and writing this article. It is very helpful!
Hi, I have a friend who is 39 yrs old and went to the ER because her heart rate has been very high.
When they did the CT scan for what it was supposed to be cardiac issues they told her that she might have PF.
Today is hey told her her heart is fine and that the tachycardia could be related to something else.
Tomorrow she is going to see the pulmonologist to talk about the CT scan results and what they saw there.
Are CT scans a good test to diagnose PF? Should she go and get a second opinion?? Or do other tests?? Thanks for any info.
Thanks heaps for the very informative post! It helps to read this and relate it to my situation.
Thanks so much Jason. So glad it was helpful to you. I have a book coming out 11/15/18 for PF patients and their loved ones that hopefully will be a big help to you as well. It's called Pulmonary Fibrosis Journey: A Counselor and Fellow Patient Walks with You. It will be available on amazon.com on 11/15//18.
What is the name of your support group? Thank you.
Hi Brandi, I just read your comments on PF and I can totally relate to what you’re saying. Most people don’t know about this disease, even ER doctors. I’ve had it about 2 years now and it has gotten progressively worse since I had a lung biopsy. I didn’t need O2 much at all unless on feet a while but after the lung biopsy, and my diaphragm on right side was snipped, I started going down really fast. I’m on 15 ltr. O2 24/7 and am basically home bound. I’m lucky to have wonderful friends, family, husband to take care of me and a super family dr. The Pulmonary dr. said there wasn’t anything else they can do for me which to me says it’s over. I’ve gotten a lot of info from this website and the book written by Kim Fredrickson. I wrote her a long letter and it came back via email. I then learned she passed in June and I was devastated. I cried for over a hour. She gave me so much information that I needed and I’m eternally grateful to her. What a amazing soul!! I hope you can get her book too, my friend was researching the web and found out about it for me. I’m thankful for this life I’ve had, I just wish it could be longer to watch my grandkids grow up, then I say I’m lucky that I have them because I didn’t expect to ever have any. Many blessings to you and everyone reading this and needing to reach out to others with PF.
Hello Brandi, I'm very interested in the group you mentioned. I have IPF and desperately need to talk to others. Thank you
Noah I have lost lot of weight and I would like to know what to do to gain some back I have IPD which is pulmonary fibrosis I am also very anxious and depress. I have rheumatoid arthritis and that is the reason my lungs ended up with pulmonary fibrosis. I take Plaquanil for rheumatoid arthritis and metroprolol for hypertension and northriplyne for anxiety I really do not take anything for my PF I have not been given anything,
Thank you so much and God Bless
Do you have any suggestions that can help me please I feel scared ,
Thank you.. Sandy
Like I said before I am very appreciative that you wrote this and keep updating and sharing information. I come on here whenever I need an answer or an explanation because I cannot sleep just thinking why? My grandpa was diagnosed in May/2018 after being hospitalized I search for as much information as I can so, I can have a better understanding of what is going on; I find relief in that. The past 2 days when I call my grandpa he would talk to me for no more than 10 mins I found it strange so I asked him he says he feels tried and I was starting to think it may be the morphine the dr Rx him and, tonight he sounded better, however, he mentions pain on his right side. He assures me that he better now he went to church had an appetite (he always says he's better and doing fine trying to comfort me). Soon as I get off the phone with him I come on here in the hopes I will find an answer and I always do either in your blogs or in the comments. You are amazing for all that you are doing and because of that, I can sleep better without my mind thinking of the whys!
Thanks so much for your encouragement. I appreciate it so much! I can't remember if I told you yet or not that I have a book coming out on 11/15 for PF patients and their families. "Pulmonary Fibrosis Journey: A Counselor and Fellow Patient Walks with You." The e-book version is available on amazon.com for pre-order now for only $4.99 (1/2 price). The paperback version will be available to order on 11/15/18. I hope it is a help to you :) Just put the title or my name on amazon's website and the book will come up.
Thanks for the info. I am now at 1 1/2 years since Dx. 6L of Oxygen when sitting still, 15L when I am moving around and I still pant for air with that. My doctor tells me the same thing about being "not there, yet" I do not want to one day up and pon day suddenly almost bedridden. I too want to know as well as I can. I want to know when I am in end stage so I can finalize all things I need to.
Hi, 15L of O2 when moving around? My concentrator only goes up to 10. They tell me that’s the highest you can get. Are they not being straight with me? Now I wonder if they say that because they cannot get a machine that will produce 15L. Do you know?
Home concentrators only go up to 10L. You need to use an oximizer instead of a standard cannula. When up and walking.
You will need to get E Tanks with oxygen regulators of 15L. Most are equipped with only 8. My supplier had to order these.
You should now always have a second tank with you even on short trips out of the home.
I have my tank on 10L and again have an oximizer attached. As soon as I'm in the car, I start to reduce the oxygen flow. This is important because at 10 liters per minute the tank will only last for 20 minutes.
Often within a minute of being in the car seat I'm at 3L. A minute later I'm at 2L.
I sleep with 4L attached to home concentrator with standard cannula. I have a wheeled tank with oximizer at bedside which I use when travelling to bathroom. It is set at 10L with the additional 5L from the use of the oximizer.
Showering is done with Home concentrator at 10 L and standard cannula. The E tank is set at 10 L and has what's called an oxi-mask attached.
Showers are brief and require managing two cords.
Hope this helped.
Thanks I was diagnosed 10 months ago. I use 3L sleeping and 2-3L when out. I have no idea how long I have left. I am 76 with seasonal allergies,duabwtis and 100 kbs. overweight.
Diana Moiseff Marcotte
In addition, my husband had to concentrators set at 10L each, which were hooked up to a "Y" adaptor.
I am caring for my 84 year old mother in my home. She has two, 10-liter concentrators Y'd together. She is on 8-10 liters round the clock and we bump her up to 14-16 when she walks to the bathroom.
Thank you for this blog...I have looked everywhere for information on what to expect.
Pretty much my arrangement too.
Two 10 liter high flow concentrators can be hooked together with “Y” tubing thus providing higher flow for the patients that need it.
This is what I do. Insurance only covered one 10L concentrator so I purchased the second. I use the "Y" connector and a high flow cannula. I use the oximizer cannula when I'm out and using the E tanks.
Can you get hose with a larger bore or inside diameter? I guess the hose connector on the machines would have to be larger for more volume. Lots of luck.
yes. It is high volume hose & it is for over 6 LPM.
Cheryl and others
Is anyone aware of a "Y" connection that can support flow from two machines - 10 liter and 5 liter with a value that can turn off flow of 10 liter when at rest?
Larger hose barb and hose inside diameter should help. I guess I'll ask about that with the concentrator mfg. Cheers!
My husband has two concentrators joined together to give him 15 LPM.
The home oxygen concentrators do only go up to 10 liters per minute but if your oxygen prescription requires you to be on 15, your oxygen provider can bring two units to your home which will get you up to 15 liters per minute. Your insurance should approve. I require 8-10 walking and 15 doing Pulmonary Rehab, so I have two units in my house. I'm only 56 years old and never smoked, prior United States Marine Corps and healthy. The doctors stated I got it from "somewhere". I'm undergoing lung transplant evaluation. I hope this helps to answer your question about the home units, but do try your best to introduce, if you're not already, rehab in your daily routine with a treadmill. But consult your Pulmonologist before exercising and monitor your blood pressure and Spo2.
I was diagnosed with I.P.F. from lung biopsies that were taken from the upper, middle and lower section of my lungs and confirmed by John Hopkins Pathology and by two transplant centers with the final diagnosis as P.F. with an auto-immune disorder. It all gets a little confusing because you have I.P.F. and then P.F. I see it all,if they can not pinpoint what has caused the Fibrosis, meaning idiopathic, would be considered to some degree I.P.F. or a form of that falls under a type of I.L.D. It's all considered interstitial lung disease and rare and incurable.
I have IPF but it was caused by a known trigger. Even though called "idiopathic", the cause is known in my case. It behaves like, and has the pathologic markers for IPF, so the treatment is the same as the treatment for IPF: OFEV, Oxygen, etc. I am being worked-up for transplant...but that is an uncertain pathway at best. Yes, it is a type of ILD and incurable. The semantics aside, it is a bad disease. I wish you all well in your personal journey, which is quite individual, from what I have read. Carpe diem!
I just found out today that they said my xrays showed I had it since 2014 an never was told till June this year 2018 an today my oxygen was only 81 so he says it's critical an said he's transering me to a surgeon to have a wedge biopy.has anyone had that done.i had a car wreck in 87 an was in a coma an on ventilated an 6 chest tubes from a car flipping over on me an it's what cause my scar tissue the pulmanary fibrosis do you know what to expect wth the wedge biopsy he just said it was a 6 to 7 inch I so soon an could be in chest sternum or in thoratic an more chest tubes I read on line can you tell me more about that.last month I was riding my bike an breathing fine then this month I can't walk to kitchen an get out of breath.my phone number is 573-820-1054 I sure wish I had someone to talk to about this it's hard not having a support team.my name is shelly if you would like to call an thank u for your post
Hi Michelle, Thanks for your comment. So sorry for what you are going through. Sounds so scary and confusing. I would ask the surgeon for all the possible ways to do a biopsy. They may be able to do a biopsy in a less invasive way. Don't hesitate to get a second opinion. Glad you shared.
It depends on each country I guess. On international IPF Facebook pages I see some with 25L at home! In the Netherlands it goes up to 15L (2 concentrators of 8L combined).
I’m on 15+ 02 and I have had to get another concentrator which the two are “ y” together. This will allow you to go much higher. I’m on 16 flow when active.
So please talk to your 02 supplier and get them to bring another one.
Take care and good luck.
Often times people must use two concentrators using a special y connector. When my husband needed 10 for sitting and 15 for walking to the bathroom he plugged the second concentrator in to a plug that he could control remotely with his iPhone. He would turn on the second (both were located in an extra bedroom) concentrator as needed. As they were preparing to send him home on very high flow he had a device - one of the first to use it - that warmed the oxygen before it got to his nose. (The tank was liquid oxygen, located on our porch and was about 6 feet tall and 30” in diameter).
My mother is further than you are. They can give you a second 10 unit and link it together to give you 20liters. Hospice services provided this. Hope this is of some help.. Prayers for you!
I too am on 6 litres at rest and 15 up walking. I can only walk about 10 feet and have to sit and rest. I need 15 with a re-breather mask to go up 6 stairs and sometimes panic from air hunger. They tell me I am end -stage IPF. Your doctor can order up to 15 litres of oxygen. The oxygen supplier will give you two concentrators which you set at 7.5 & 7.5 < 15.
Thank you for this column. I have learned more reading this than any other site. What type of portable do you have that goes to 15L? Leaving hospital today and mom needs 10 to 12 when up moving. We are going home with 10.?
What she already has at home. She has liquid and a concentrator that takes in air and cleans it . It goes up to 10L . Trying to prepare for her needs when we get home.
Thank you so much and want your book!!
Hi Tonya, so glad my column is a help to you. Yahoo! So glad you have liquid at home. It makes things so much easier. I have portable strollers that go up to 15L, It is a High Flow Caire Stroller. I hope my book will be a blessing...less than 2 weeks until it's released!
I my dad has ipf and his concentrator also goes to 15. He unfortunately is in end stage. He still tries to get up to use a commode and he goes through an entire tank. He did just have a catheter put in because he just doesn’t have the strength. He is on a dilaudid pump and lorazepam for the anxiety. I think for the family this is the worst part to watch.
Yes Debby I share your frustration and sadness about finding out last minute!!! I just found this site tonight, God bless you and you will be in my prayers. I'm trying to close out all my affairs as well and its confusing...
Hi, I’m so sorry that you have this terrible lung condition. I thought patients could only get up to 10L? Please share how got receive 15L?
Thank you and blessings !?
Hi Mich, I use liquid oxygen and the portable containers (strollers) go up to 15. It really makes a difference!
I think this was very helpful. I have had IPF since 2003. To a lot they would think how wonderful. Well in very many ways it was. I got to see my son graduate from high school. I saw my daughter get married and have 4 beautiful grandchildren ages 12- 1.
You are the first person I've come across who has been at this longer than me. I was diagnosed with hypersensitivity pneumonitis in 2007 and went on oxygen 24/7 in 2012. At that time I was told I had 2 years without a lung transplant, which I didn't qualify for.
Well, I'm still here.
My oldest granddaughter graduated from high school this year. My other two grandkids are 5 and 8. While I'm declining now, my overall reaction is boy, did I show them!
Congrats to you and your staying power.
Don't let the doctors tell you you don't qualify for a lung transplant. I was told that over 2 years ago, so I decided to go with ofev. I had horrible side effects from it so doctor took me off of it. I have researched this disease extensively, and it looks like the only cure is a lung transplant.Maybe if I had done research sooner, I might be on the list now.I am getting tested for a transplant now, and was told that if I pass the other two tests, that I should go to the top of the list. No way of knowing if I will get lungs in time. I hope everyone keeps trying for the cure.
My dad was diagnosed with IPF in 2005 at 70. He was very healthy when diagnosed, minke coughing, fatigue until 2017 when had to go on portable oxygen. October 2019 he started falling a lot, oxygen was not enough, needed to move up to higher level of oxygen & has become home bound. Disease has been progressing quickly, he’s in wheelchair, the slightest movement causes his oxygen to drop below 75 & he has a seizure. He has hospice care, checking his vitals, showering him. I’m hoping he’s going to pass soon. My dad & mom who cares for him are suffering terribly. He wakes several times a night. This is such a horrible disease.
Dad died year 2000 with asbestos worked with asbestos in belfast shipyard my sister died with PF 2011. I have been diagnosed with IPF 2016 any connection
But now my oxygen has increased from 2 to 5 but with movement 7-8. I get so tired just to go watch their activities and shopping even with a motor wheelchair is hard work and no fun at all. PFT and timed walk is next week. Should be interesting since my numbers go to 70s and heart rate about 160+
Sounds like fun huh
I have pain from side effects from years of steroids. I have stress and compression fractures in my back, chest pains from my cough. I’m 55 feel much older and as you stated good and bad days. I will know more when all tests are done and I see my dr in December. Don’t think it will be good news but just have to wait n see. My prayers go out to all my IPF warriors and breathing buddies. Stay strong and make every day special. And always end with I Love You to those who mean the most. Love to all of you
You are amazing and I wish you strength. My prayers are with you.
Thank you so much KL. I can’t believe how many people are affected by this. One of the worst and annoying parts are that no two are alike. But thank you for your kind words. I pray for all that have any part with this demon disease. My biggest request is for all to beat this anyway they can. God bless you all tonight and always.
Thank you Kim. You're strength to face forward and understand what may be ahead is an example of courage. Thank you. Sometime, I'd like to speak with you about your success in keeping liquid oxygen.
Kim, Thank you for writing about this and all the other articles you share with us that are Dealing with IPF. I appreciate this information very much. <3
I was diagnosed March 2017 with IPF. After two high resolution CT scans and one regular CT San, my pulmonologist confirmed the diagnosis. I started Esbriet in May or June. The actual disease is stable, no progression. But, secondary issues - bronchitis, pneumonia and rhino virus (common cold) have put me in the hospital twice! One month ago, my pulmonologist said I'm "at the six-month window". Now, I am on palliative care with every other week visits from hospice. I also made my wishes known to my adult children, my doctor and my pastor regarding no ventilator, DNR status, etc. At my request, my pulmonologist met with my children to explain the disease and answer any questions they had. He told them this has been very aggressive with me. The only way I am able to cope and accept this is my faith in Jesus Christ and knowing when I accepted Him as my Saviour is what is taking me to heaven. The Bible says "to be absent from the body is to be present with the Lord". When I pass through death's door, I will immediately enter heaven. I am a little apprehensive about the very end, but again trusting the Lord for the grace to see me through. My daughter and son's, their spouses and grandkids have been so supportive and helpful. I have moved to my youngest son's home, where I have almost 24/7 care, which is a comfort!!
Hi Sandy. I'll pray for strength and peace beyond all reason or understanding. I too have this disease and it scares me sometimes. I'm 52 and was misdiagnosed 2 years ago and finally properly diagnosed a year ago.
I am praying for all of you. I am 46 years old, having had been diagnosed with IPF Among other things. I have been treated with high dose steroids which initially helped greatly, yet no longer give me much if any relief. I have been taking 2 immunosuppressive oral meds for the past year without any noticable changes. I have been painstakingly researching as have the rest of you. I am going start taking over the counter enzyme package of Trebinase ,nattokinase package wich basically consists of enteric coated enzymes along with addition supplement such as vitamin d3 , Nac, etc. There are multiple companies that offer very similar packages / supplements. Very pricey costing close to $280 /month if taking all 5 suppliments which can be reduced at the very least by taking the 2 most important being serrapeptase and nattokiNase Amazon has multiple brands available for 1/2 the cost or less , however the importance of a great quality controlled product with a great enteric coating is crucial. There are claims of enzyme therapy reversing scarring in lungs and throughout the body ( to what extent I am not sure ), increased oxygenation leading to a possible reduction in the liters required for daily functioning, deeper breaths , increased levels of funtioning etc , treatment for cancer etc. The only major concern or warning I have come across is that the use of serrapaptase enzymes should be monitored by a physician if taking blood thinners Coumadin aka warfarin, aspirin etc. It does not mention that it is contraindicated if taking coumadin , it states it needs to be monitored closely by a physician. The hurdles we are facing is that the suppliments are not fda approved and that translates into most physicians not endorsing the supplement use for lack of clinical trials etc. I respect the physicians rationale, however I am willing to seek alternative treatment to beat this disease. There is a major health system in California that uses enzyme therapy within their holistic treatment center along with acupuncture for cancer treatment. .. I feel extremely positive about this treatment specifically because it describes them ( serrapeptase , nattokinase, and other enzymes ) as being a fibrinolytic ( fibrin dissolving) enzymes and anti inflammatory. This is also the rationale given for taking a relatively newer med approved in the US for tx of IPF - “Esbriet /pirfenidone” Both Esbriet and Ofev are antifibrotic drugs. Serrapeptase and nattokinase are antifibrotic. Inflammation is largely a result of an autoimmune disorder that manifested into something larger such as ILD ,IPF, rheumatoid arthritis and the list goes on and on. There are claims that patients have reduced the amount of steroids and/ or stopped taking them altogether after finding pain relief and decreased inflammation from the enzyme treatment. Serrapeptase treatment boasts that it can help treat autoimmune disorders, inflammatory diseases, and too many to list. Please be your own judge and make an informed decision. I am hoping to update you all in several months how the enzymes are working. In addition, please consider listening with headphones - to positive affirmations, healing hypnosis cd’s ,apps, daily or several times daily. What Messages are we sending our tissues and body? We become our thoughts. Stay strong. Stay positive. God bless.
Your story sounds almost identical (even down to the dates) for my mom. She is now under hospice care in my home. She, like you, has placed her faith in Jesus alone, and is at peace. She is frightened of the death, but not of dying...she knows that her last labored breath here on earth will lead to a clear, fresh breath of celestial air in heaven.
God be with you!
My Mom passed away from this in January . My heart goes out to you and your Mom. I take comfort in knowing that when my Mom took her last breath here on earth she took her first breath in Heaven . God bless you both. You touched my heart deeply .
Cheryl J Staten
Amen. There is such peace knowing you will healed and set free when in the presence of our Savior. My husband is in the end stages. Knowing there was no cure from the onset, we chose to not spend all of our time in doctors offices but enjoying life. I'm thankful we made that decision.
You are right ..the progression to the end is different for people. My sister went peacefully but it was a process of weeks. She was never uncomfortable.
Thank you brave girl. I don't know you, but I love you.
I continue to benefit in so many ways from your honest reflections on the greatest problems we face as we each navigate the challenges we face with IPF.
I look forward to hearing about your pursuit of palliative care.
I was evaluated for transplant and accepted in February of this year. I was 70 at the time of acceptance.
Since then the condition has worsened and I now required 15 liters per minute when moving and 4-5 liters at rest.
I'm continuing the challenges of attending rehab in order to maintain my strength.
Thanks again for all you do
Kim, Thank you so much for shining a light on many of the questions that I have about IPF. We're just coming to terms with it now as my husband was diagnosed this summer. Have found our physician is not as forthcoming with status / information as we would like - is this so we don't worry?!
Something I want to check into is body donation, starting to figure out how it is done etc. so far I have to pay for transporting body and cremation. Not sure if I can afford that, but I will venture on.
Thanks for your column.
Just found this the other day and it looks promising www.sciencecare.com You can sign up and get all the info online. Good Luck and Gods grace and blessing be with you!!!!
My GP said when I was near the end of life, and if struggling, morphine would allow me and my husband peace from the stress on both of us. I thanked him for this because that had weighed heavily on my mind.
Me too, I am in stage two on oxygen and was dx, last year,
however have had it a long time. In Sept, of 2017 I was
hospitalized and had to come home on oxygen, I want to know
what to expect and that helps me cope, and prepare. I have
gotten so much from the IPF community. I also have severe
persistent asthma complicating things. My MOLST medical orders of life sustaining are signed, sealed and delivered.
At 73 with no dependents I make my own decisions. I want
to be comfortable and go in peace. Knowledge helps me. Thank you and God bless.
Morphine helped my husband- he self administered so was sleepy but aware right up to the very end.
My sister is in trouble in SNF and very anxious. Staff not able to coordinate her care and often allow oxygen sats to fall to 50's. Weekends are the worst. I read that Morphine may alleviate some of her anxiety and breathlessness, but I cannot find the info on the net to send to her son. She was diagnosed one year ago when she ended up in ER and treated for RESPIRATORY FAILURE. She did not even know she had lung problems until then. Failed by her eight specialists. Thank you for any info you can share. Maureen
Went to lung Dr today heart, is good I'm going on 1 1/2 years of IPF, but I also have COPD ( 2 yrs ) Last month diagnosed with Rheumatoid Arthritis already very painful and starting to deform, the Dr said what is going on with my joints is also going on with my lungs. Scarring has increased. Right now I'm having a difficult time !! HOPE I'll be my up beat self soon. I'm going to chiropractor tomorrow for back out of place. I haven't been in 5years so I have to start as a new patient. I told him that was good cause that old one died and left me
Thank you so much for writing/researching this. I look forward to what you find out about palliative care. I am like you in wanting to know as much as I can, so I can prepare. Would like to have my ducks in order before I am unable to do things myself. Also, want to have built-in people contact when or if I reach stage when I can't go out much. Thank you again.
The elephant in the room. Thank you for writing this article. When my husband was diagnosed this was a big fear/question for both of us. When a member of our support group asked the question following my husband’s death I was more than willing to share our experience and almost everyone in the group entered into the conversation.
Thank you so much. None of us want to hear how bad it could get but all of us want to know, not only for ourselves but for our family and caregivers sake. I'm pushing onward but know that with my oxygen needs and significant lung function decrease, my time is short. I am fortunate not to have other health problems other than IPF which was finally diagnosed in December 2016 after years of mis-diagnosis. I have met with and am working with a palliative doctor to assist in coordinating with my curative doctors and addressing the emotional needs. I thought palliative care was hospice but they explained that is not what their role is at this point. They will assist when hospice is needed and the curative doctors are no longer in the picture. I told her I need her to help me understand when that time is comes. I think in some degree, I'm still in denial that this disease is actually going to cause my death. I get up, take a shower, get dressed and try to find something to occupy my day. I stopped working in May due to my O2 requirements being so high, 15-18 liters when active.
Thank you for the honest and transparent way that you speak about your disease and its effect on you and others. You a remarkable person. You truthfully tell it like it is, then offer your readers comfort in the form of a recipe for chicken noodle soup. I appreciate your thoughtfulness and your remarkable ability to balance truth and grace under such challenging circumstances.
We will keep you in our prayers, asking our great God to continue to give you His strength when you are weak, courage when you are down, and above all, peace in knowing that the final victory is already won, through Jesus Christ our Lord. Your writing is a blessing to others, including us.
Is it possible for me to send you a devotional? It's by Joni Eareckson Tada called "A Spectacle of God's Glory, God's Light Shining through Me Every Day." Perhaps you already have it, but if not and you would like a copy, I will send it out right away if you let me know where to send it. My way of offering encouragement to the encourager. : )
I think you are one amazing human being. Thank you for sharing your story. I can't wait to make the chicken soup from your recipe. Chicken soup is for your soul !
Stay strong, and remember so many people care about you, including me.
God bless you
My mother has PF and it has been a great struggle for us to deal with as a family. I regard my mother as my life and I truly feel as I wont be able to live on without her. I am 23 and my mother is 54 and I've seen my lively, active, never sitting down mother, deteriorate to barely being able to walk without her oxygen dropping to 60s. We have been rejected by a transplant program with the hospital stating her case risk is to high but that did not stop us from seeking other hospitals that offer lung transplants. One year later after being rejected we have found another hospital willing to evaluate her case for a possible transplant. This still doesn't change how sickly my mom is but this gives us hope to keep on trying. But I truly feel anybody suffering with this condition should never give up hope and believe in the miracle of god. Science and medicine is backed with fact and evidence but there is still so much in the world that us humans cannot provide explanations for and that is where I truly believe is where gods miracles are. I send my love to all of you dealing with this hard time and please keep strong. I send my prayers and I ask god to give all you prolonged lives to stay with your loved one.
Thank you so much for this article. I have had stable PF for 4.5 years. It was found on a routine chest xray. I'm not on oxygen, but do get some shortness of breath with exercise. I'm still up and around, I'm 66 yo. I pray that my PF will stay stable, but no one really knows. I appreciate all you do on this site to keep people aware!
Thank you very much for your article and honesty and thank you for all of you who share your own stories in dealing with this horrible disease. I feel like I have learned more about this disease with my own research and forums then what the doctors have been providing. My 63 year old father was diagnosed with IPF in March 2017. He has since been checked in the hospital 4 times for pneumonia or other breathing issues. He is on 5L of oxygen. Just this past week the doctor started him on the OFEV medicine and I was wondering is anyone in this thread has been taking this and if they have had any positive results?? Mentally my father is not accepting of what he has and is upset that the doctors cannot get him better as he still feels strong physically. Since the one constant thing I have heard about this disease is that it affects everyone different in the progression of it. I am praying and hoping that we still have my father for years to come but we are also scared of the worst. Please continue to share any info or experiences you have. I wish and pray for the best to all of you as I can see see there are many brave and caring people dealing with the same things my family is dealing with. Thank you.
Gary D Chace
Adam, I was diagnosed with IPF in May 2014. I started on Ofev in Oct. 2014. My cat scan in May of 2015 showed some progression but not as much as they expected, the next two, each a year apart, have shown no progression. It appears the OFEV is doing what it is supposed to do. I do understand that not everyone has the same results. I'll pray that your father has the same results as I have. God Bless
Thank you for your reply and thank you for providing us with hope for my father. I wish you all the best and that the medicine continues to work for you as it has been. If I may ask...do you require oxygen? And if so how much? Thanks. No worries if you don't want to answer.
Gary D Chace
Adam, while just sitting and reading or watching television I don't need oxygen, my levels stay in the low 90's, but as soon as I get up and move around my oxygen levels fall to low to mid 80's
and I need to be on 3 or 4L of oxygen. If I'm speaking or eating I need to be on oxygen at 3 or 4L, when I go for a walk or when I go to pulmonary therapy I'm on 5 or 6L. I also need oxygen when bathing or brushing my teeth etc. I hope this helps, if you have any other questions please feel free to ask. Gary
Thank you for the Author of this story. It's really helps. Hello Gary D Chance: My Father has IPF. His oxygen is mid 90s. However, he walk 10 ft. He is out of breath. His Mornings are worst. It takes him about 15 mins to get out of bed. He get oxygen, However he states he does him any good. He has a Vest that thumps his chest. Helps the lungs Not to get pneumonia. I just don't what stage he is at, since his Oxygen is at mid upper 90's. His chest hurts when he lays on his side. He lays on his back. He weight is 98 lbs.I just don't what to expect if he is in final stage or last days. I'm looking at 6 months to a year? Thank you. If anyone can enlighten me on this horrible disease.
My father took Ofev for about three years during his battle with PF. I believe it helped slow the progression of the disease. However, he was older, about 75, when he was diagnosed and ended up with other complications as a result of PF. He developed Congestive Heart failure and massive pulmonary hypertension. We believe it was his pulmonary hypertension & it's effects that ultimately caused him to pass on Aug. 9, 2017.
Has OFEV helped him and is it expensive?
We watched my 63 year mother-in-law pass from pulmonary fibrosis in 2011. In April 2017, my 53-year-old husband was unexpectedly diagnosed with it. His case was severe (he had what we now know was an acute exacerbation in March). We had no illusions where this ends up. He doesn’t want a lung transplant (lung transplant doc said in June he has 12 months) and is too severe for the new drugs to work. He is just happy with to fish and be with his family and friends on oxygen. But I have to prepare for what happens before the end. He decided to move to our favorite lake, four hours away. I am stuck at my job for health and life insurance. Our sons who are in middle and elementary school are up with their dad. I call daily and come up on weekends. I am constantly on alert for signs that the end is near (he has lost 30% lung function since April, and now doesn’t want to get milk at the grocery store because he will be too fatigued carrying it into the house). I don’t want to over-burden our eldest son, but the Family Medical Leave Act is only for 12 weeks. That can go by so fast, I don’t want to start too early. So knowing more about the end signs is super important. My findings are they same as yours- it’s variable. I will be entering a cloud soon. I don’t know what it looks like inside, but I just have to keep going through it. I know what the other side looks like- it’s just me and my sons.
Hi I'm Lyle Romans and have fought this for 3 1/2 years I have lost 90% of my lungs to this disease and I'm 24 and I am in end stage Pulmonary Fibrosis I am to not able to get a transplant but because I'm young and a very rare case for doctors I get to donate my body to science I have become a motivational speaker who in my process have helped many but with the progressive of my Pulmonary Fibrosis I can now longer physically make it to shows anymore now I sing everyday it's not healthy for my lungs but I'm a fighter and will fight until my last breath
My father passes away on Aug. 9 2017 from PF at the age of 78, one month and day before his 79th bday. He was initially diagnosed with COPD and then PF in 2014 where he would use Oxygen as needed. Within the year he was on 24 hr Oxygen but low liters. His energy decreased and he had lost some weight. He could no longer take showers because the steam made it difficult to breathe even with the oxygen. My mother would have to give him sponge baths and wash his hair in the kitchen sink for him. Through the following year we could see a big difference in weight & energy. He increased his Oxygen level to 2-3 liters. He also experienced chronic diahrea. We aren't sure if it was from the meds in which he was on Ofev for this disease. He was then diagnosed with Congestive Heart Failure. He seemed to decline quickly after that. He had gone for cardio physical therapy for a few months before he got worse. He could only walk from the living room down the hall to the guest bathroom. My mom would have get him his clothes from the bedroom everyday. Walking to the kitchen or dining room became difficult as he would be very winded and experience a flushing feeling but not lightheaded. We aren't sure what caused the diharea and flushing but they suspect it was a side effect of the meds coupled with his disaeses. My mom was the primary caregiver and I would help when possible. It was a very difficult to watch a strong man and your role model become so frail and helpless. He'd sleep in the living room in the recliner because he could not lay flat and breathe comfortably.This was even before He could no longer walk to the bedroom. Then we found a pillow wedge for the couch. He would take daily naps and then not sleep until midnight or later for only four hours or so. He couldn't sleep well even with Ambien. He experienced anxiety from thinking about his illness and took Xanax when needed. I believe he became depressed as well. He then began to take Xanax to help him nap during the day. In June 2017 he had gone for a heart cath and was informed that he had massive pulmonary hypertension caused by the PF. Experimental drugs or test programs were the only option. The doctor didn't know how long he had to live. She said it could a year, several months or several weeks. He chose to try the experimental drugs but e had to wait for the health ins company to approve the drug; however, they were able to give him some samples to last about 3 weeks. They also ordered a home health nurse out at least once a week and a physical therapist out a few times a week. We had to get him a bedside portable potty for the living room as He couldn't walk to the bathroom anymore. He also began eating in the living room and not going to the table anymore. However, his spirits and energy seemed lifted. We met with hospice but were told he could no longer see his doctors and possibly not be able to get his same meds. Also, if he went to the hospital he could not continue hospice...insurance wouldn't cover both. His doc told him that he didn't need hospice since my mother and I were there to care for him and he could still get out of the house. However, it was a struggle and took a toll on him to leave the home. After the three weeks he ran out of the experimental drug and insurance still had not approved the meds. He began to decline...lack of appetite, taking more naps during the day and his Oxygen level was 4.5-5 liters which was the max on his home concentrator. He had seemed depressed so I tried to get him out of the house one day to see my new house that I had being built. He had been promising me for months that he'd make it there one day when he felt better. But that day he looked at me with a long face and in a disappointed tone told me he was never leaving the house again; and apologized. I knew something was wrong but was in denial thinking he was just having a bad day. Two days later I said good night and "I love you" as I did every night not knowing it would be my last time. (Note: my kids and I had been living with my parents since Jan) My son came down and woke me up around 1am and said my mom needed me. I went upstairs and found my mom trying to hold my dad up. He was slouched over on the chair and not responsive but gasping for air. I called 911. He came to and said he was fine and he didn't Want an ambulance and wasn't going to the hospital. We still called. He had gone in & out of consciousness again but came back. They came and got him prepped to go. Said fluid was building up in his lungs. They had to perform CPR in the ambulance and satblize him before they could leave. He didn't want to be resuscitated but never got around to doing his living will so the medics.told me they had to do it. They continued at the hospital but after an hour they had to stop. We lost him. They don't really know if it was a heart attack or what; but I know he was exhausted and his frail body couldn't take it anymore. I believe he knew it too when he told me just two days prior he was never leaving the house again. At the hospital as they worked on my dad, my mom told me that my dad had been coughing a lot more than usual and he told her it felt like he had fluid in his ears and he was trying to clear it. Hearing that made me think his heart was not able to pump the blood as it should (i.e. the pulmonary hypertension) and his body began to shut down causing organs to not function and fluid to fill his lungs/body. I could be wrong, I'm no doc. Ironically yet sadly we got a call from the insurance company saying they approved the experimental drug for my dad. I don't know if it would have even worked. Yes, he seemed to improve while he took samples of it; but I've read that often before people pass who habe a chronic disease they seem to get better for a period. We'll never know; but I do know my dad fought a hard fight. His mind was still sharp til the end and before they took him out to the ambulance he was still there. I held him in my arms trying to keep him sitting up and holding his hand. At one point I asked if he could still hear me and squeeze my hand if he could. He squeezed. This is a nasty disease and they never could tell us how he developed it...whether from having been around a farm & having birds/chickens, smoking when he was younger, asbestos in old buildings & factories where he worked while growing up, etc. I pray they find causes for this disease and a cure! My heart and prayers go out to all affected by it!
Thank you for sharing your story. It has helped me prepare.
I'm glad it helped and I'm so sorry you and/or tyour our loves ones are having to live with this disease. Every story seems to be different because we are all made different; but if this was able to help you in any way, I'm glad. My prayers are with you!
I am so sorry for your loss. My dad is dealing with late stage PF and it is heartbreaking to watch. Like your father, he was so vibrant and active. In a matter of months I've seen him become frail and homebound. I very much appreciate you sharing your dad's story. Prayers and love to you and your family.
Hi Linda. My dad is in exactly your dad's situation right now (end stage) except that I live far from him and so he had to be in a nursing home. I will stay with him this entire summer - although he is not likely to see August. He has religiously taken OFEV for over two years. His decline is now rapid. Up to this point he has not been in any pain from the IPF. His pulmonologist tells me that most of his patients never pass away from IPF directly but from accompanying problems like pneumonia or in my dad's case it is likely to be from CDiff contracted from being given a notorious CDiff - causing antibiotic - clyndomicin given to treat a toe infection almost a year ago. Beware any of you whose loved one is given antibiotics when they are already compromised. A good probiotic taken daily is an excellent preventative idea (at least 30 billion cfu's... colony forming units)!
Some things I learned IN HINDSIGHT...
It is really important for IPF patients to keep active as long as possible. My dad was always active. Yet, my dad was rushed at one point to a local hospital (AFIB complications) and in addition to his IPF they noticed a small blood clot behind his knee. They had him on bed rest... A REAL NO NO as patients are no longer kept on bedrest. When he was finally transferred to a top notch hospital they were very upset that he was not kept ambulatory during this two week time. This immoblility started his downfall because two weeks of inactivity coupled with his lung disease made it impossible to get back on his feet again despite physical therapy. And this immobility caused a cascade of other issues.
BEWARE of prednisone. My father never took prednisone ... he was on OFEV. But when he started to decline and had more difficulty getting around (he had an active lifestyle), a doctor decided to give him a short period of prednisone. This led to a host of problems much worse than simply not being able to get around as much as he had been used to. The tapering off this medication posed serious danger to his life at one point (even the slow tapering). So, if your loved one is about to be put on prednisone for any reason, really inquire and research and decide if it is worth it.
BEWARE of CDiff. It is a killer and a horrible one at that. It starts with antibiotic treatment and a weakened immune system enables it. The bad bacteria take over and diarrhea is intense. It never is erradicated. You get over it then it comes back. Fecal transplant can be done but one has to be strong enough to be able to prepare as if one is having a colonoscopy and having a procedure that almost parallels the colonoscopy process. One can also swallow 20 pills (yes filled with prepared fecal matter) but insurance won't cover it and swallowing ability is a factor. In any hospital or nursing home, if you ever notice an aide or nurse handling a diaper gloved but not taking off those gloves and touching other surfaces or not washing hands, SAY SOMETHING. Proper hygiene is SO TYPICALLY not followed and is a likely cause.
If your loved one does have to go to rehab or a nursing home, MAKE SURE ALL STAFF TREATING AND CARING FOR YOUR LOVED ONE understand this lung disease is not COPD and that the correct liters of oxygen needs to be adhered to. My dad needed 6 liters and several staff initially wanted to put him on 2 liters (mistakingly thinking it was the same as COPD and higher would be dangerous)!!!
For any of you wondering what end stage is like... I know it is different for all but I will tell you that my dad is not in pain from breathing difficulties. His pain is from his severe weight loss and not being able to lie in a comfortable position.
Thank you so much for sharing your story. My Dad just past away last month due to PF, and your story was 90% the same as how his last several weeks were. It is "comforting" to hear that what I experienced is shared with others.
My husband was diagnosed with IPF in May 2013, he started Ofev in December 2014. He was stable until this last 6 months, he’s gone from 4 lmp to 6 lmp, 8 with exertion. His Pulmonologist said he had to try for a transplant now before it gets worse, even though he was about 20 lbs away from required BMI for a transplant. The transplant team decided to send him for pretranplant testing now so when he dropped that 20 lbs he’d be ready to go. Well, as a result of some of the tests done we found out there was scarring on his liver, he went for a liver biopsy where he was then diagnosed with stage 3 liver disease. Now all hopes of a transplant are gone. He’s definitely progressing, I’m just not sure where to go from here. I will call his doctor tomorrow, but I’m assuming he should stop his Ofev because it may cause more damage to his liver. How will I know when it’s time to get Hospice involved? We’re just both so disappointed.
First THANKS for the items you write. They are very helpful.
I am the caregiver for my wife diagnosed with IPF in March of 2015. She is on Esbriet and doing much better than we ever expected. Right now she is still active and on O2 at 2 ML. I do want to learn more about the future with this condition so I can be prepared. Are there websites or blogs where I can learn more about the progression of this condition. The PFNEWS articles on increased oxygen usage and possible solutions (liquid O2, Y tubes to double [email protected] output from 2 O2 concentrators etc) have been very helpful.
Hello to everyone who is here.
I have been diagnosed two years ago with what my specialist called Interstitial Lung Disease. Both my lungs are affected and it appears to have started at the base of my lungs in the alveoli and now is halfway up my lungs. I was taking Methotrexate until six weeks ago for my auto immune disorders but my Rheumatologist said it was making my lung disease worse. I have Sjogrens Syndrome and Lupus and Hashimotos. I live in Western Australia but don't know whether what I have is the same as Pulmonary Fibrosis. Can anyone advise me please. I know my shortness of breath is becoming worse every week. I hope someone will read this and give me an answer please. I read your column and have great admiration for you and everyone with this horrible disease.
I was diagnosed with Rheumatoid Arthritis Dec 2016 which also caused my Interstitial Lung Disease with scarring.
I'm not a Doctor, but It looks like ILD is one of the multitudes of lung diseases identified under PF. They both create scarring and similar lung issues, O2, shortness of breath, coughing, fatigue etc. The pain and progression of the RA just make pushing through the ILD symptoms that much worse.
Just so I'm sure I've got enough challenges to deal with my WBC, RBC and platelets are well below the bottom of the acceptable ranges.
I read PF news regularly because it is the very best site I have found for real, clear experiences to hear from those living with this disease. It is a wealth of knowledge so keep researching topics and be sure to enroll in the newsletter.
Im not sure if ILD is a subset of PF or not but the symptoms and progression appears to be the same so I gather info from this site.
I am being treated with infusions of Rituxin ever 4 to 4 months. Im am getting my third infusion in 2 weeks. Within 3 days of my first infusion, my joint pain dropped from 8 to 4, my muscular pain from 7 to 3 and my lung pain from 6 to 1. Rituxan is also supposed to prevent or slow lung scarring. So far it's working better than I expected.
I hope this is some help and encouragement to you.
This site is so refreshing in that it doesn't use doctorspeak, and it is much appreciated.
I also am looking for candid, end-stage information. It's not an uplifting topic and I apologize to anyone who my question may upset but I need to know so I can plan.
RA with ILD
Not on O2 yet
I can cope with this disease because I have no choice, so I just keep on keeping on. What I need help with is coping with the end stage. It is not the fact that there WILL be an end that I'm afraid of. My concern is breathing.
I understand every situation is unique to each individual; my general question is, as one progresses through the final stage will I just lay there suffocating, struggling and gasping for each breath along with all that that entails,( unless some other complication gets me) or will I be able to breathe?
Thank you all for the help your responses provide.
George I too am trying to find out what the very end is like. No one seems to want to speak of it. I think my days are numbered. I was diagnosed with end stage IPF in February 2016 and hozpitalized for 2 weeks. My faith in God keeps me going as does my family. I'll regress. In late 2008/mid 2009 I was diagnosed with the following. P-Anca Vasculitis, Neuropathy, Polymysiosis, and then also informed I tested positive for RA, Lupus and Srojens. Forgive my spelling of these diseases. While I was hospitilized I was not only diagnosed with IPF (due to the Vasculitis), i was also informed I now also have pulmonary hypertension, type 2 diabetis insulin supported, osteoporosis, losing my hearing, have broken 4 vertebrae, and there's more but it's depressing. I have and take enough medicine to start my own pharmacy. Just kidding but it's a lot. I spent most of 2016 at the Mayo clinic for a double lung transplant evaluation, only to be told I'm high risk so I dont qualify. We were nervous about a transplant anyway since it had to be a double. This is a nasty disease and I see little out there on it or donations for research. To all of my brothers and sisters dealing with this nasy disease and that includes caregiver and family, I will pray for you. I wish you only the best and that God will lay his hands upon you. Luan
P.S. Kim good job on getting the ground work together for us to reach out to one another.
Luan, Thanks for sharing your story. You are dealing with so much, and are so brave. My faith sustains me as well during such difficult times. Thanks for your prayers, they are much needed and appreciated. Thanks for your encouragement about my column. Just said a prayer for you.
Oh Kim thanks so much, you are a brave spirit and I have been looking so long!!!
yes i'm with you Luan, bless her for starting this, it's so badly needed for patients to share. And yes I too find it hard to get honest answers to the end stages. I would communicate with you if there is a way.. So much more to say and ask.
Standard oxygen equiptment like INogen 2 with 6lpm can no longer keep my SPO above 90 so I think this is when we are getting to the end, but my doctor won't tell me. Clinical studies aren't very helpful either they are so hard to find and get into, I have lost all encouragement...
Stan, the numbers on an Inogen are NOT lpm, they are just numbers where 1<2<3<4<5<6. An Inogen 2 likely delivers 1-2 lpm . There are many alternative pieces of equipment to get more lpm (15 lpm or more) to keep your SPO above 90. Tell your doc you need MORE O2. If he/she can't/won't help, get another doc that will.
I would think at that stage you would be with hospice care. They can provide morphine order by doctor and it helps you breathe easier and peaceful until you take your last breath here and first one in heaven. My husband diagnosed Dec 2017. Seems to be decreasing energy quite fast. No oxygen yet. In pulmonary rehab two times a week. Trying to keep muscles stronger and pulmonary function stable. I am s nurse and I've researched this disease a lot. The doc can't tell anyone much because it effects everyone so differently. Ask God to grant you his peace! Antidepressants and Ativan for anxiety have helped him a lot. Prayers for you all.
HiRA:I'm 76 and later stage than you. 2.75 d/n. Find a comfortable recliner to sleep in or a bed lift 7", My chair sleeps at 30 deg. or so angle. Have you done the night pulse ox test? The Ofev is very expensive, I can't say it's done much for me. I gather that some other opportunistic bodily malfunction will be what does us in. Best, Dave
Thanks so much everyone for your encouraging comments to me about this, and other columns. That encourages me so, as my column is a gift of love to my readers. Thanks too for sharing your stories...they are so valuable, and we are learning so much for one another. I wish I could respond to each comment, but I don't have the energy to. I value and appreciate each one of you!
Thanks George Clayton for your reply. Knowing that others are also coping with an illness that is least understood,I feel less alone.
I M Mayariwala
Diagnosed in 1999 at the age of 18 , much struggled and reached 2017 lols, in all those years I just found that when in depression or over thinking or getting angry , the health become more unstable and with being happy or happy surrounding and positive talking health still remains bit stable, let's pray May god bless us all good health and some day again I can walk . It's not possible I know but the positive thinking brought me till here and hope it will help me again .
Wow finally found what is needed and looking for since diagnosis 2years ago. THANKS TO ALL SHARING THEIR OWN EXPERIENCES AND CONCERNS!!!!! This needs to be interactive to be able to comment directly to relevant comments. Some will help some won't, but finally i'm not alone!!! My doctor didn't give me this info on this site although I asked, I just kept searching, a God send!!!!!!!!
My nan is 92 and she was diagnosed with IPF over 5 years ago. It has taken those years for her to suffer with severe breathlessness, weight loss and fatigue, but still goes out shopping, and only uses small amounts of oxygen when required. She has just been prescribed morphine for her breathlessness and associated anxiety - which makes it so much worse!
Don’t give up hope, just because the life expectancy says a certain time after diagnosis, it doesn’t mean that. Make the most of every day, and try and remain positive, for yourself and for your family who will always be with you on the same journey.
Take care all.
Hello everyone, so glad I found you. My dad was diagnosed with PF about 6 years ago and didn't tell us. We didn't find out until mid-2016 when my mom went to his doctor appointment with him (they've been divorced 35+ year but remain friends). Dad is 75 now and has been on oxygen about a year and a half. He has been in the hospital twice since Thanksgiving for pain in his sides. He thought he was having a heart attack, but they said it's just the progression of the disease. I asked his doctor to talk to me in the hall because I feel like my dad doesn't tell me things because he doesn't want me to be upset. I asked her how long he has. She said he is a candidate for hospice. I said what does that mean. She said they have to believe he has less than 6 months left to be a candidate, and he qualifies. Dad has been on hospice since December and he also has a person that stays with him during the days. Mom and I switch days and go there with him until evening. He's still stubborn and won't let someone stay with him overnight. He's lost over 100 lbs in the last 2 years and with the cold weather he doesn't leave the house. He's also on Esbriet for about a year now. We were worried they wouldn't let him continue taking it on hospice, but they said it's not a treatment, it's for comfort. His pulmonologist said it's not really doing anything for him. It's more of a mental thing that he thinks it's helping. It's more for someone that has just been diagnosed, not for someone that has end stage disease. I'm glad they let him continue though.
I'm just overwhelmed right now. He's my dad. He's not supposed to get sick. I know he wants to cry, but he won't cry infront of me. He wants to be strong. I feel horrible for him. He worked hard for so many years to make sure when he was gone he could leave things for his grandkids. He wanted to teach them things, show them things, go places with them. But he worked every day, all day, and now he is out of time. A while ago he would tell me "when I get better I'm going to take the kids here or do this". Now he tells me "I wish I felt better I would do ...". Ugh.
He seems to be at a plateau right now. I don't know how long it will last, but we will be there for him.
I'm so glad you found us too. You must be in shock, and grieving so. Very normal, and oh so hard. Make sure you get support for yourself. You are going through so much too. It's so hard as a patient to go through all this, but even harder to watch our loved ones suffer too. So glad you shared with us today.
Thank you Kim for your great story and updates and being truthful. That isn't easy, but those of us with IPF need the truth. I am 79 yrs old, diagnosed with IPF in May of 2017, began Esbriet in June and do feel it has stopped a lot of coughing. I use 2l of O2 most times, turning up to 3 when walking. Use a walker to carry oxygenator & support. Feel good when I read the above stories and realize I am lucky to have been healthy for this long. I was doing exercises 3x a wk and eating healthy. No longer able to do many exercises but do Respiratory Rehab ones. They are great & help lots.
Thanks for your comment Joyce, and for sharing about your PF journey. Love that you do all you can. Thanks so much for sharing with us!
My Dad is 75 years old and has PF. He was misdiagnosed with COPD despite failing an overnight pulse ox study and having pulmonary function test that demonstrated no restrictive issues but did clearly demonstrate absorption issues. Once the words PF came to light, his symptoms made more sense. He does not wish for any treatment as he also suffers with mild/moderate dementia and chose hospice care. I agree that speaking of whats to come seems to be taboo, even with hospice. Im a 20 plus year veteran nurse. I have found navigating PF and what to expect to be nearly impossible. I have watched him steadily decline since May of last year. May we had a vacation with no oxygen and just a little shortness of breath. August we had a vacation with 24 hour oxygen and a bit of shortness of breath with extended walking. In November we had a vacation with 4 liters of continuous oxygen, markedly slower pace, increasing shortness of breath and the need for more rest. And now, going out to dinner seems to almost be too much and he naps 3-4 times a day.
He is a very kindhearted and stoic gentleman. He tries his best to hide his decline from these old nursing eyes. He doesn't want to be a burden and he will never be a burden for me- I'm here for this journey with him.
As a nurse, my brain wants to know what my heart doesn't. I ask the hospice team- where are we on this journey? They seem to panic or tell me how much they love my father and he is their favorite patient. While that is all wonderful and they mean well, it is so not helpful. Its like the white elephant in the room. Lots of information and professionals to talk about other disease and stages and what to expect- why is this so different?
Thank you for your bravery and going where no one wants to go.
Thanks for your comment. I'm so sorry to hear of how your Dad is struggling with PF as it progresses. So hard for him to go through, and so hard for you to watch happen. I so appreciate your encouragement about my column. It is so frustrating to not be able to get this information easily. I'm glad my column helped. Blessings to you and your Dad.
Thank you so much for the info. My brother has this and it is an Awful thing to deal with. We are just now learning more about the IPF again it is awful. God Bless you Kim. And all the others.
I am happy to find this site. God bless you all on this journey. My husband also was just diagnosed a couple of week ago after losing 25 lbs in a month and was very tired and week. The dr first diagnosed him with pneumonia and ordered antibiotics when he got worse he said it must be a virus. He continued to decline and after X-rays, breathing test aNo now a CT scan he confirmed PF. We have a appt with pulmonary dr next week. My husband is 74 with heart disease; had a stent in 2001, carotid artery open in 2012 same artery reopened in 2014, hi cholesterol. He has not been able to walk more than 1/2 block without having to stop and catch his breath and his hips hurting for about 3 years (I am not sure if this has anything to do with it). He is on oxygen at 4 on the home concentrator. After his appt with pulmonary dr we may know more but it is very difficult not knowing what to expect. I am a caregiver at my job so it helps. Thank you for all of your stories as they have answered some of my concerns.
Thank you for this forum. My sister is 51 and was diagnosed with IPF for now 10 years. She is in end stage. I have watched my grandfather, my grandmother, my aunt, my mother and now my sister die of this disease. Each one has had a different experience with this disease some taking years, some taking months. We definitely have a familial component to this disease. I am saddened because my sister went through all the testing to determine if she was a candidate for a lung transplant, we were told she had been listed, but now they have removed her denying her the only possible solution to this horrible disease. We are coming to terms, but it is very difficult. There needs to be more research done, new medicines, new screening techniques, new ways to combat this and eliminate it. I was told that more people are diagnosed with this than breast cancer and is second to heart disease. My sisters and I are diligent by asking are there any changes in our lungs. Most of us have GERD and there seems to be a connection i.e. microscopic gastric droplets being inhaled causing scarring, so we are all taking proton pump inhibitors. There was also a report published implying a connection between dental dust and IPF. I am looking for answers and causes, but I know I will not find them. My heart if filled with grief not only for my sister, but to all of you, both patient, family and caretaker. I ask for God's strength and pray for you too.
Thanks so much for sharing your story and words of encouragement. I have no words to respond to what you are dealing with. I can't even imagine what that is like to see family member after family member succumb to this awful disease. Thanks so much for your empathy for patients, family and caregivers who are walking this difficult road. I (and we) appreciate your prayers. Just said one for you and your sister too.
God Be with you all, hello my name is Catherine and my husband of 41 years was diagnosed with IPF after a diagnosis of severe Aortic stenosis. He received a new valve in Dec of 2016. January 2017 he was hospitalized for sepsis. That’s when the IPF came about. He went to Cardiac rehab for 10 weeks and had to use oxygen therapy. Then the oxygen was added during sleep 1 ltr. Pulmonologist put him on Esbriet and for the first 3 months all was good. Then we started to notice he had no desire to do anything but work and exist! He was clinically depressed and losing weight daily. All in all He had a good 2017 being oxygen dependent daily until Christmas Eve. He was diagnosed with double pneumonia. Dr sent him home on strong antibiotic even though his o2 was at 78% resting. Turn up the o2. He passed out on Christmas Day! Made the excuse he got up to quick. That night I called an ambulance. He spent 8 days in the hospital. Dr discontinued the Esbriet said is was causing him more harm being depressed. It has been down hill since. He had no desire to do anything but work at his computer and watch tv. He started falling and this week he fell and broke his ankle. Called an ambulance. He has a new diagnosis of Hypoxia with Syncope (fainting). Congested heart Failure. They gave him Lasix and 6 lbs decrease he began to feel better. But now he has a good excuse not to move around. He has to have the ankle surgically repaired this coming week. He is still weak. No one ( Dr) wants to tell the family what is happening. I’m so glad I found you all. There is so much to this hateful disease we need to know. God be with us all.
I was diagnosed only a few weeks ago. This was found completely by accident as I thought my shortness of breath was related to smoking, which i quit before the diagnoses.
I didn't tell my doctor because i didn't think it was important.
I had stomach pain so i went in for an abdominal cat scan. They found nothing to explain the abdominal pain but they did spot two nodules on my lower lung. 1 @ 3mm & 1 @ 5mm. My doctor didn't seem too concerned about it as we know I'm prone to them from connective tissue issues. But, he ordered a hrcs of my chest & when i asked on our next appointment about the results he flipantly said ild .. "We'll visit this again in 6 months".. I searched it right there and asked well what is it. He said scarring happens in the lungs. But as he was typing what he was saying out loud was reason for concern. I left feeling stupid.
I came home & searched & searched & got angry & mad & searched some more...
What is this? Then i remember did he say... "Fibrosis"?
Ipf? Noooo maybe i heard it wrong.
Talked to my husband Who hunted and found a doctor 200 miles away that specializes in this & lung transplants.
I'm freaked out. I'm 49 & the breathlessness has gotten worse.
I go in 2 weeks to this specialist & I'm still freaked out.
Thank you for this blog.
I am the daughter of a pulmonary fibrosis patient. He was diagnosed in 2015 and basically went on about his business not seeing a doctor or doing any follow up. Now, 3 years later, it has reared its ugly head with a vengence. Suddenly he is using oxygen all the time. We are now seeing a pulmonologist and are praying he will have some things to try. My dad sounds like so many in that this disease suddenly went on the rampage. I know he should have been seeing someone all along, but my family couldn't convince him. My father is rather advanced in years, but is otherwise in remarkably good health. I am really scared of what the future has in store especially with our mother in such poor health. I am glad to have discovered this forum.
I know what you are talking about with the doctors saying we are no there yet! I heard that on the 21st of March and my husband passed on March 30th. I am angry because I don’t think they know any more about the disease than what I have researched on the internet. My husband was diagnosed in November of 2016. He did just fine until he developed pneumonia in December of 2017. Then he started having Hypoxia with Syncope episodes. He broke his leg in his last episode and had surgery to repair the break on Wednesday and Friday morning he was gone. My prayers are with you and your family. Be strong and demand answers. Remember you hire doctors for their service.
Prednisone has made a difference. But one cannot take that indefinitely. Both of my parents are starting to realize that this whole scene is quite serious. Are there other drugs out there that aren't a steroid like Prednisone.It seems there should be something that is as effective that can be used on a more regular basis. Has anyone been treated with something that has given them some relief and energy? I have researched other drugs but have only found a couple.
Can somebody help me understand if UIP (usual interstitial pneumonia) and IPF are the same diagnosis? My mom was diagnosed with UIP in October 2017 from a lung biopsy. She is on 2l of O2 at night and started Esbriet in January. She seemed to improve for a bit but symptoms have returned. My mom was given prednisone in October, however she had a severe reaction to it causing her to go into a catatonic state, along with hallucinations. She was in and out of the hospital and even held against her will (5150) for 36 hours then suddenly released when the psychiatrist said it wasn’t a psych issue, it was a medical issue (side effect from prednisone). Since then, Mom and I are having a hard time trusting doctors/medications. When we ask what to expect when the disease progresses, Dr response is “she’ll know when it’s getting worse”. We were also told that even if she were approved for a lung transplant, that she would be on medications after transplant, one of them being prednisone. After her experience with that, we are now feeling discouraged now that a lung transplant is off the table. Has anyone had a similar experience?
I also forgot to mention that she is having side pain and back pain. Her pulmonologist says it’s not related to her disease. Does anyone experience pain similar to my mom?
Hi. UIP is pulmonary fibrosis and idiopathic pulmonary fibrosis is another form of this disease. Idiopathic is not known what caused the disease and UIP is usually by environmental factors. Saw dust plaster dust smoke concrete dust things like that are believed to cause UIP. Not sure if this is the real reasons. Since there is no cure for either. It starts in the bases of the lungs and honey combs up th lungs and as it does this it's shutting airways off. Yes prednisone is one of the drugs after transplants. There are different corticosteroid so I'm not a doctor idknif there would be another she could tolerate . I just keep researching this disease. My husband has it. Lung biopsy did not show it. CT scan show it. Lung surgeon said he seen it as he was doing biopsies. So his is in early stages had not hot deep enough in tissues yet or they got good tissue on biopsies
Ask your Mom's doctor about muscle spasms specifically. They are common in some cases of IPF. Direct massage of the area may relieve your Mom's pain. I get rapid-onset spasms sometimes in a hand, foot and occasionally in my upper right abdomen. I can break the spasm with stretch of the affected muscle and massage. It's worth a try and limited risk. It works in under a minute, typically.
Now, UIP is a pathologic diagnosis (made from biopsy tissue on a glass slide) and used by radiologists to describe characteristic patterns of honeycombing in the lungs on CT. IPF is a clinical diagnosis made from the whole package of symptom presentation, High Definition Chest CT, physical findings and Pulmonary Function Tests (PFT in a booth, 6 minute walk, etc)...and biopsy, if performed. This linkage (UIP and IPF)is very confusing, so you are not alone. UIP usually implies IPF...but they are not the perfectly interchangeable terms. NSIP implies other causes or a poor sample on the biopsy, missing the UIP areas.
When reading articles, always look at the date of the medical article you are reading, as this is an area of rapid learning and consequent evolution and terminology changes over time. Read a medical journal, online from ~1930 and you will be stunned by the clunky assessment, exam, interpretations and conclusions. That was still the best that they had at that time. Judgement is key in interpreting the info. Remember, medical journal articles are meant to be read by people who have spent years learning context,fine points and interpreting data, not necessarily by patients . It is really easy to misinterpret medical reports if you are new to the process. Bottom line: medicine is inexact. It is the best interpretation in 2018 (or 1930) of complex, detailed information...but not exact in the sense of irrefutable truth.
If you don't fully trust your Mother's doctor, find another. You must, in the end, trust that you Mother's doctor (Pulmonologist) is looking out for you Mother's best interests in an area frequently riddled with contradictions. No doctor "corners-the-market" on truth. So, focus on your Mom, her findings and symptoms. Make her comfortable if possible. Remember, muscle spasms are common in ILD's like IPF and can come on quickly with certain movements. You may be able to provide relief with simple stretch of the muscle (massage). That would help your Mom. Have the steroid-reaction conversation with your Mom's doctor. Specifics are important. What dose of Prednisone was she on when she had the bad reaction. Steroids are well known to produce abrupt psychosis in susceptible individuals at high doses (like 40mg-100+mg a day). Yes, she will need steroids if transplant is done. You have a lot of concerns. Your Mother life is important. Get facts, ensure that her doctor and you are on the same page and stay optimistic. Hope springs eternal.
Kim, I want to join everyone else in thanking you for your articles. Why are doctors such wimps? I just wanted to know what to watch out for. How might this disorder progress? In 2012, I was told to expect about 2 years without a lung transplant, which I didn't qualify for. Well that didn't happen. Still here and filled with questions.
I was diagnosed with a variant of IPF in 2007. I was told it was environmental and would go away when the source was removed. That's pretty much happened until it came back in 2011. I went on oxygen 24/7 in 2012. I worked bi-coastal, flew with my Inogen One G2, went on a couple of cruises. The oxygen was annoying, but really just a distraction.
I retired in 2014 and moved from Southern California to Northern Cal to be near one of my sons, in case I needed help. In 2015, I had a major decline, like stepping off a huge step. Asked my pulmonologist if this was a step towards the end, but he declined answering. Started going out with a walker. 2016 and 2017, it happened again and landed in the hospital. Did the 6 minute walk and oxygen flow prescription went to 7lpm. This has been like a prison sentence. Can't use my trusty portable oxygen concentrator anymore. Can't get the walker out of my car on my own anymore. Had to get a power wheelchair and use a transport service to go anywhere. That's really expensive. So the last 4 months have been really an adjustment.
Got my power wheelchair that breaks down and fits in my minivan. Holds an oxygen tank that will last about 4 hours with the extender cannula. Safeway and Costco have grocery delivery services. Can get restaurant meals delivered too. Just need to find someone to drive me to Doctor appts and errands. So I can continue living by myself.
Then I found your article and I have The first 6 points on your list. I tell my dr. I'm tired all the time and sleep over 12 hours a day. Response: Oh, that's interesting. I say I'm losing weight and not dieting. Response:Oh, that's interesting. See you in 3 months.
You've helped me more than any of my doctors have!!
so thank you.
Thanks so much for your comment. You have no idea how much I appreciate you taking the time to let you know that my columns have helped. My columns are a labor of love and a chance to make something good of such a difficult situation. Your words blessed me so. So sorry for the run around and lack of honesty from your doctor. Here's an idea...print this column and show him next time you go in. It will be interesting to see his reaction.
You are so resourceful and brave to do all you are doing to continue to live by yourself. Good for you. My Dad who is 94 has someone come 3x a week to do light housekeeping, laundry, and drive him to doctor appts and errands. We got a referral from the Senior Center in his area. Maybe that could be an option for you. Blessings to you.
Your story sounds like mind except I’m fortunate to have both paid daytime caregivers plus my husband evenings nights and weekends. I’m essentially homebound.
On O2 continuous , must increase when walking few steps to bathroom . Use portable cobedside commode for voiding only- it’s dtill a dignity thing with me? my main point is I’m 62 , I’ve been evaluated for double lung tax, decided against it when weighing risks v benefit in terms of outcomes and quality of life. I’m on skilled home care and will likely seek hospice sooner than later. Ppl generally seek hospice way too late , they’re a blessing to both patients and families. I’m a retired RN and worked in a hospice and used hospice for my parents.
Hospice typically requires your Dr. Approval that is they provide the order for hospice to the hospice agency. No Dr at any great accuracy can predict a timeline for passing so hospices are very flexible about the time line thing ( example 2 rounds of 90 days plus one round of 60 days) - the skilled home care agency ( for nursing, social worker & PT including pulmonary care) I use also has bridge to hospice so I’d continue getting care from the same multidisciplinary .team Personally I want to do hospice in the comfort of my own home to benefit both my family & I.
One may also leave hospice care anytime.
Hope this info sharing helps feel free to PM for follow Qs.
Meantime hang in there ,make each day count, recognize the nature of our disease is weak days and stronger days. Take no day for granted, eliminate all drama & negativity in your life.
Sending hugs & prayers to all. We’re all in this together.
I’m in n Facebook.
I wanted to update a previous comment I made concerning my sister who has IPF. I have read through the forum that people are concerned about end-stage. You will "know" when things happen and each case it happened quickly. My sister is now in hospice care. It is day 3. She is on O2 continuously and has been prescribed Ativan and Morophine sub-lingual. She is sleeping a lot and does not seem distressed but is comfortable. To all who are concerned...the hospital (for my family members who went this route) and hospice (for my sister) have been wonderful. All four of my family members who had/have this disease have been made extremely comfortable and anxiety free. Please know, it is harder on the family members who have to watch this disease play out. We are at peace, although hard to say good-bye.
Your candid description of the last days is really the first piece of information about the end days that I have come across- really useful and helpful, this has been my worst fears for my husband who has PF diagnosed in oct 2016 . Getting worse but comforting to know he will be peaceful and comfortable when the ends come.
I hope all is well with you and your loved ones.
Cathy in Australia
I stumbled across your page because I was looking for any and all information I could find on IPF. My mom was recently diagnosed she is 74. When she first told me she was so nonchalant about it and I had NO IDEA how serious it was until she gave me an article to read regarding the disease. I was floored...I sat and cried on my couch..all I could see on the internet was 3-5 years and I fell apart. Once I came to grips with her diagnosis I started doing my research. First let me thank you for your candidness, it is greatly appreciated. Some of my family members don't want to discuss it but I needed to know. I have so many questions that my mom cannot answer and she has told me that she needs to do this alone. I told her we need to learn about it together. My mom is a very tiny women and in my eyes seems frail due to her size, however she does eat healthy, she exercise and tries to lead a generally healthy life. She told me she has passed all the tests but her xray showed spots....she cannot tell me if that is what the doc used to make is diagnosis or not. How do I convince her to let me be part of this. She is not an emotionally strong person and I think that is why she wants to keep me a bay...she is afraid to show weakness and fears she will if I am with her. My head fills like it is swimming with all this information..I don't know what to do to help or even begin to start this journey with her. She seems fine, she says she doesn't get winded, she doesn't have a cough and so far the tests they use for checking oxygen have all been A+. I have also ready that autoimmune can make IPF worse, she and I both have Hashimotos thyroidism, does anyone know if that particular immune disorder cause IPF to be worse? I apologize for all the questions but you guys are the first to offer any real answers...the good and the bad ones. Thank you so much and I pray for all of you going through this journey...sending nothing but positive energy to all!
I am a 74 year old woman diagnosed with IPF, like your mum, [in 2013.] I also have Hashimotos Thyroiditis, diagnosed in 1998.
Up to now I have remained fairly well, and have not needed medication.
My Dr, who I visit every 6 months says that I can have the medication but he has had bad luck with it lately as the last 3 patients who were started on it developed nausea and upset stomachs - so I chose to wait as my disease seems to be progressing very slowly so far.
I don't know if there is a connection between the two types of autoimmune disease that we have but one would think there has to be.
I feel that I have been quite lucky to have a slow progression, so do hope your mother is as well.
My guess is your mother doesn't want to put herself in the 'sick role' while she feels ok, and I can understand that - maybe it's a form of denial.
My advice is stay close and be there if she declines but remember that could be years away - it is for me.
God Bless x
Love the information it was better than the doctors gave to me i would like more information thank you
For those curious about the last hours, my husband died at home from IPF in February 2015 ; we had help from 'hospice at home' care (England).
It was very peaceful at the end, his breathing just gradually slowed to nothing. He didn't suffer, he just drifted off.
I don't know if that is typical, I hope it is.
It appears that information from health practitioners is still sadly lacking in terms of what to expect. We, the novices who have to provide the ultimate support, are left to discover the hard way.
Thank you your message is right on point!
Hello to everyone on this valuable forum. And thank you Debbie and all contributors who have helped me understand more about my own recent diagnosis. I have learned a lot in 3 days or so and have progressed from being unconciuosly incompetent regarding IPF to at least the consciously incompetent stage. Will I ever reach consciously competent? Maybe, with all your help. I have passed the from the panic stage through denial and anger. I now accept that it is what it is. The question of why it is does not feel as important anymore. I am no longer in great fear of what lies ahead, but I am absolutely consumed with the impact of this disease on my wife, children and grandchildren. Hey, I'm 81, feel like maybe 40, fairly active (e.g., mow my own lawn, huff and puff, but have learned how to adapt and do everything much more slowly), my mind is still 20 or 30 years old. I feel so terribly for those much younger than I with so much of their lives still ahead. The biggest regret I have is the impact this disease is having and will have on my family.
I will try to post from time to time on my progress if it will help others cope. You have certainly helped me. Thank you.
Hi I am paul. Your article and responses were very informative. My case is different then most I will abbreviate to make shorter I was diagnosed with ipf in Oct of 2017 and In Feb confirmed by Cleveland clinic before I got ipf I was.fighting a few different diseases to include cad (coranary arteriey disease) i have 10 arty stents 2 on last month i have mvd(micro vascular disease) i have sleep apnea i have chf(chronic heart failure) ibs (irritable bowel syndrome) chronic fatigue and pain syndrom since i was 19 in military believe all of it caused by agent orange from Viet nam and now I have ipf i believe for 2 1/2 years my ox is 3l at home and 2 on the road ox does a lot of haunting going from day 92 to 82 and back to 97 all in seconds I had to get off esbriet because of terrible symptoms i am on cbd oil now its oil from the hemp and marijuana plant without the th the ox reading have improved dramatically over the last 3 weeks so I watching my ox numbers they don't lie everybody with ipf should have a pulse ox meter that's a good way to check how your doing hope I helped a little
My mom has Pf also. I have done some reading on cbd oil also? How are you doing now that you have been taking it. Thinking of you and all those out there with this illness.
Do the hands and feet feel cold near the end?
My father just passed away from this two days ago. He was diagnosed in June 2017. He definitely declined rapidly and memory loss and confusion started kicking in about four months ago. He got up to use the bathroom In the middle of the night and wasn’t wearing his oxygen. My mom found him on the bathroom floor. We are devastated. He was 78. He was on 6 L, but was having no pain. Depression definitely was an issue due to being housebound.
So very sorry your father just passed away from PF. What a difficult journey for all. He sure was blessed to have you and your Mom by his side. My heartfelt condolences.
Kim, thank you so much for writing about this disease. My father-in-law has IPF and we have just been told that he will be put in palliative care soon. He had a stroke 2 weeks ago and is in the hospital. Some of the doctors want to send him home but my SIL is fighting for him and so is the respiratory doc.
So sorry your father-in-law is struggling so. So hard to watch I know. Good for you for fighting for his welfare!
I have a cousin that was diagnosed with IPF about 2 years ago. She was immediately started on oxygen and now is in the end stage. She lives a couple of states away from me, so I've only been able to see her twice this year. Plans for me to visit just today were asked to cancel because of my cousin's declining condition. She has one daughter and two grandsons and of course her husband and son in law. They stated that they wanted to help her cross over to Jesus with just this immediate family there. I truly understand. She is taking ativan and tablet form morphine. She's been on the morphine since February. It had become too painful to get up to use a bedside potty and a few months ago, she was in a diaper and had to have morphine in order to withstand the pain of just having the diaper changed. At this point, she is somewhat confused, forgetful, agitated and can barely speak because of lack of oxygen. I know she is on 16L of pure oxygen 24/7. She has been under hospice care since February. I wish there was something I could do. We've always been closer than cousins, more like sisters. We truly have a special relationship and my heart is breaking that I can't see her and most likely will not get to again. I guess I said all of this just to say, "Don't put off until tomorrow what you can do today." Her time is short and I know I did not spend enough time with her the past few years and that heaviness is going to be on my heart forever. Thank you for allowing people to post on this blog. There are very few people that I can talk about this with because they don't understand the disease. I don't understand a lot but I know what she is and has been going through. To all who read this and suffer with this disease, I will be praying for strength and comfort as you suffer with this or are a caregiver. God bless you.
My husband went into hospital with pneumonia the day before his 70th birthday in April,we were told he had a heart problem because bloods taken for analysis were showing signs of a heart attack.
Over the next 5 week hospital stay he lost 2stones in weight. We were still no nearer to finding out what was wrong with him,he was on oxygen and couldn't move far on his own.
He seemed to rally round and came home for about 2 weeks, then heart palpitations put him back in hospital.
From then on he went down hill very quickly, we still didn't know what it was he had.
Within 2weeks my darling husband had gone.
We didn't find out till the last few hours of his life that he had IPF.
So many questions and why's why so quick ? why hadn't he shown signs before ?
Thank you so much for sharing your story. I'm sure this was so difficult to research and write. My dad has not been feeling well for a long-time and has had many "issues" related to blood pressure and heart related stuff. He has had some stents put in over the years. Although he's been fairly private about his doctor's appts, most recently he did tell us he was diagnosed with PF. He didn't say much beyond that other than the words PF. He is a long-time smoker which doesn't help anything. This makes me so sad for him and for me and my sister. My parents divorced when I was four (I'm 43 now) and we've had to endure a long-distance relationship over the years. I'm not sure how long I'll have left with him. Thanks again for your story and many prayers for you.
Thank you for stories. My dad Diagnosed in April 2018. They gave him 3 yrs to live. He has 30% percent lung capacity, rapid breathing, weight 99 lbs, very exhausting for him to walk to the kitchen or the bathroom. Pretty much stationary. Oxygen is 90 in a stationary position. He just started taking steroid prednisone 3 weeks,He does have oxygen uses it about 2-3 hours a day. He don't feel a difference. I don't know what stage he is in, Severe or End stages? I don't see clubbing on his fingers. He seem stable since April not much changes only to the fact the breathing is getting more difficult. Prayers to you and the caregivers.
Thank you very much for the information and for all the comments, I wish you all the best.
My mom (74) was diagnosed about three years ago but because she also had a failing aortic valve and was in rough shape, they could not do a lung biopsy to get to the cause of the IPF, and they could not determine how much of her shortness of breath was due to the heart and how much to the IPF.
Unfortunately after she went through a valve replacement her breathing did not improve at all, in fact it started to decline rapidly. She has been on prednisone since November 2017 and on Mycophenolate for the last three months. The Mycophenolate has not had the effect we hoped for and she has continued to deteriorate. She had a crisis a month ago (suddenly was not not able to get any air in) and ended in emergency for 6 days where they stabilized her. She was on oxygen at the hospital (and has portable oxygen at home just in case) but they told her not to use the oxygen at home if she didn't absolutely need it (something about becoming dependent on the oxygen).
My question is, should she be on oxygen, even if not all the time, to help her? she insists that she shouldn't use it because that's what the nurses told her.
I believe she is in or is very near final stages, her dry cough is incessant and she can not take more than 3 steps or even stand up for more than a couple of minutes without her oxygen levels dropping dramatically. She is practically bed-ridden, has no appetite and gets severe indigestion when she does eat, is getting more depressed each day and her memory fails her. Your thoughts on this would be appreciated.
Thanks so much for sharing. So very sorry your mom is having so much trouble. Super hard on you too. I'd be happy to give you my opinion on the oxygen, although please note that I am not a doctor. She probably does need to be in oxygen. I can give you the guidelines, and you can test her oxygen levels yourself. You can buy an oximeter on the internet, and put it on the tip of her finger. It measures her oxygen saturation and heart rate. Experts recommend oxygen levels not go below 90% saturation. You'll be able to see what her levels are sitting and when she moves. You can adjust her oxygen levels accordingly. If her levels are above 90% consistently she will feel better. It is false information that a person can be dependent on oxygen. You either need it to supplement what your lungs can't provide, or you don't. I hope that helps :)
I lost my mother at age 60 at the end of June. She was diagnosed with end stage Pulmonary Fibrosis just 48 hours before she passed away. She was admitted to ICU on Friday night, and passed away Wed morning. Each day she continued to decline in every way including respiratory effort, memory, aggravation, hallucinations, and oxygen levels. It became apparent that she was not going to respond to treatment. They used the Bipap machine on her at times in ICU, but it stressed her out, and she was unable to communicate with us as well when she was on it. Even with Bipap, her oxygen levels did not stay adequate.The only other thing they could have done for her in ICU was mechanical ventilation, and she had already told us she did not want that. The Doctor assured us that she would not survive despite mechanical ventilation. So, we proceeded with Hospice because we wanted to keep her as comfortable as possible. My mother struggled with major anxiety, so keeping her comfortable mentally, and physically was a huge goal of ours. My biggest fear was that she would feel like she was suffocating at the end. Fortunately, she became unconscious before she had a chance to experience that, and I am grateful for that. Between the hypoxia, and the comfort medication ( hydromorphone and ativan) she was able to remain as comfortable as possible, and not consciously experience much "air hunger". Toward the end, family will have to choose comfort over consciousness...otherwise there is not a way to achieve both, and ensure that the person doesn't suffer. Aggravation, and hallucinations will naturally continue to occur toward the end as the body is deprived of oxygen (despite supplemental oxygen), and the best thing for the dying person is to relieve them of those stressful events...Hospice is not giving up on a person...it is allowing them to die with as much dignity as possible considering the circumstances. I hope this helps someone ?
Thanks so much for your comment and for sharing about your Mom's experience with PF. Thanks for sharing about what she went through and the remedies to relieve her of the pain and anxiety at the end. What you shared is very helpful, thanks so much.
Thank you so much for sharing your story. My Dad passed away last month, and I have been struggling as it as unexpected. I have been seeking other's stories on how the final days and moments were. My Dad went into ICU as well and went on a ventilator and passed five days later. No doctor told us what really was going on or that we should be preparing for the end.I am on here seeking information on if this was a "common" end to PF. Thank you again for sharing.
My dad is at the very end stage of IPF. He’s 81 and can’t get around anymore, even to the bathroom. They are finally starting hospice (should have started months ago). My father is so afraid and his anxiety is overwhelming to him, but he refuses any anxiety meds. He is only about 120lbs or so
- this is a man who weighed over 200 his whole adult life. Air hunger is a terrible thing. Breaks my heart to watch him.
So very sorry for how much pain, anxiety and turmoil you and your Dad is going through. I so wish he would take medication that could ease his suffering. I am praying that Hospice will be able to help him chose this kinder path. So sorry for all you are going through too. So painful! Appreciate you sharing, and am so sorry for what you are going through.
My husband was diagnosed with IPF in June, 2017 at the age of 63. It was clear to us that just being out of shape wasn't the entire answer for his breathlessness. In August, 2018, while in the care of hospice, he chose to end his life (legally). I am now 56 and a widow and I am lost. There's no other way to put it. There are two things I want to share. This condition is on the Compassionate Allowances List for Social Security, and, if you choose the same path as my husband, there is a less expensive version of the drugs called DDMP2. I mean to offend no one in offering this information. It is just information that he would want me to share with others who are diagnosed with IPF.
My 81 year old father in-law has PF. He has suddenly taken a turn for the worse, and is now on oxygen full time. He can only walk a short distance now, maybe 20 feet. He has canceled a scheduled visit, can no longer use stairs, etc. He is famously silent about his health, and is not nearby enough to visit quickly. Can anyone tell me at what stage his health is? To be very blunt, how much longer does he have? Thank you in advance for any help you can offer.
Thanks so much for your comment. I know it must be so hard to see your father-in-law so quickly. I'm sorry it is not possible to answer how long he has. I would encourage you to call his doctor and visit him anyway. He probably needs help but either isn't admitting it to himself and/or you. It's hard for all of us to accept the reality of our diagnosis, especially as we worsen. I hope this helps a little. Just said a prayer for you and your Father-in-Law.
I am very grateful to have found this page, much like everyone else that has commented. My mum who is 83 was diagnosed with IPF around 3 years ago. Like most people here, we had no clue what it was and the shock of being told she had 3-5 years broke our hearts. In hindsight she’d had breathing problems before this and she thought it was her heart.
My mum is now bedridden (at home) and has morphine patches for her pain, occasionally she manages to use the commode that is next to the bed, but that now causes her too much distress as she gasps for breath with 15l of oxygen!
4 months ago she could still move around the house. It has progressed rapidly of late. She has 2 concentrator at 15l oxygen 24/7 and when she’s not moving her saturation is over 90, but the slightest movement to sit up, can take her oxygen saturation to as low as 48. The doctor has just said minimal movement.
My dad is caring for her in every way and he is exhausted, but says he wants to do it. My sisters also do as much as possible.
My mum is depressed and sleeps 80% of the time. She gets confused, nauseous and her appetite is disappearing rapidly. She just says all the time, I hope I’m not always going to feel as bad as this. It’s truly devastating us, we are a very close family. She knows full well the disease has no cure and what the end result is, but she says things like, I wonder if I’ll feel better soon, or if I have a good day, we can maybe get out for a drive somewhere. That makes me cry so much. She can barely talk she’s that tired.
To make matters far worse, she was put forward as a possible candidate for the treatment that can slow the progression of the scarring around 3 months ago when she was nowhere near as bad. But she received a letter last week that said.....
“The imaging sent to the specialist hospital, suggests that you may have Chronic Hypersensitive Pulmonitis possibly the cause of the fibrosis. Therefore you are not a suitable candidate. So palliative care is what’s needed now.
Just like that. Boom.
Suddenly more reading on the internet to find out what that is.
So for 3 years it was IPF, now it’s got a cause, so it’s not idiopathic? Why did it take 3 years to find this out.
So if the CHP had been diagnosed/detected 3 years ago, she could possibly have been treated with steroids, we could have tried to find out what was causing the lungs to inflame and possibly she may never have gone on to develop fibrosis.
I am angry, broken and lost.
She is at end of life stage and none of us can believe what’s happening.
It’s a cruel disease and we are not told anywhere near enough about it.
There is so much to learn about this horrific illness and I pray there is a cure in the near future, so no one else suffers like my mum.
My heart goes out to all the sufferers and their families and I thank you all for your stories that have helped.
I am dreading what happens from now. Her skin recently was very clammy and cold. Does she have days, or weeks and how will she go. I don’t know and the doctors don’t say much at all.
It’s destroying us.
I am so sorry for such a very long message :( I needed to talk about it to people who understand.
Love and strength to all of you.
So very sorry for all you and your Mom are going through. Such a painful process, made worse by a possible misdiagnosis. Ugh...makes everything so much worse! So sorry the doctors aren't more helpful. I hope hospice is a help to you. They are experts at this difficult part of passing on. I know you are heartbroken and am so glad with all of us who understand. Hugs and prayers to you and your Mom.
Can anyone address the financial aspects of running the concentrator 24 hours a day. Is there any way to get help with the electricity bill? I talked to my electric company and they don't have any special programs for people on medical equipment. I'm on Social Security and still work part-time, but don't know how long I will be able to work.
Hi Marilyn, I know it is such a drain financially to run the concentrator 24/7. My electric company has discounted rates for electricity for customers who make below a certain amount. That's the only discount I know of. Great question. Wish I knew of other resources.
I found out in March 2018, that I have IPF I am 76 years old. I also have lung cancer. I just want to know what the end will be like. I can't seem to get the Dr. to tell me. I have a strong faith in God and I pray that he will be with me all the way.
So sorry you are dealing with both IPF and lung cancer. Ugh! I totally know what you need about Docs not willing to talk to us about it. I have a book coming out next week with a whole chapter on this topic that I hope will help you and all of us in the same situation. It is available on Kindle (e-book) to pre-order now, or in paperback next week. https://www.amazon.com/dp/B07K5QP7RT It might help you talk to your doctor and get the help you need. I have a strong faith in God too, and I KNOW He will be with you. Just said a prayer for you :)
I am 45 years old, recently diagnosed with IPF. Three years ago I had an abnormal chest X-ray due to a spot on my lungs. I followed up with a Pulmonologist who did an additional chest X-ray. He advised I had scarring on my lungs, prescribed an inhaler and another medication to help, but never specifically explained what it actually was. A year and a half ago I told my GP my chest felt really tight and that I couldn't breath, and what the pulmonologist had told me. My GP recommended weight loss, even though I specifically said my chest feels tight like when you have bronchitis. In July, I brought it up to my new GP, and showed him my fingernails were deformed looking. He advised it was "clubbing" and immediately sent me for a CT scan. After diagnosing me, he told me not to worry, I could live till I'm 80. My new Pulmonologist hasn't been forthcoming either...no one wants to tell you what to expect. Everything I've learned is from the internet. Also, I figure I've probably had this 5 years now, because looking back, that's when the shortness of breath started. It's disheartening that I don't know how long I have. I'm about to start on OFEV, but am worried about side effects. Thank you for your blog. I know now that I'm not alone in feeling like I'm in the dark. I don't know what to tell my loved ones even.
Dear Kimberly, So sorry you have been misdiagnosed for so long. Ugh! That is horrible. Unfortunately this happens a lot. You are so right that Docs are often not willing to talk to us about what it will be like at the end. I have a book coming out next week with a whole chapter on this topic that I hope will help you and all of us in the same situation. It is available on Kindle (e-book) to pre-order now, or in paperback next week. https://www.amazon.com/dp/B07K5QP7RT I mention it because I think it will answer a lot of your questions, including talking to your family. You are NOT alone! I'm so glad you reached out. I don't know if you are on facebook, but there are some great groups I belong to that give wonderful support. Here's one if you want to check it out. https://www.facebook.com/groups/pfwarriorrs/ Blessings to you.
My husband lived 9 yrs after being diagnosis with IPF. Eight months before he passed he developed blood clots in his lungs, and it was down hill after that. He was told Dec. 29, 2017 he was at the end of his IPF. His oxygen level dropped and he was admitted to hospital, when they were unable to increase his oxygen, my husband had already talked to his doctor that he did not want to be on a respirator. That day they began giving him morphine and anti anxiety meds, starting about 1 pm. He was able to ask for water about 6 p.m. by 8:30 p.m. he took his last breath. He died peacefully as he water.
My husband read this blog faithfully and found it very informative and comforting. I thank all of you for that and wish the best for everyone.
Thanks so much for your comment. I'm so sorry to hear of your husbands passing. I'm very glad he was able to pass peacefully. What a comfort to hear. It touches my heart that your husband read my column and that it was helpful. That is such an encouragement for me to know. Thanks for taking the time to tell me.You husband was blessed to have you by his side.
I am a 62 yr. old Male with IPF. I was forced to leave my job of 23 years because of this illness and getting to this point has been a journey. I was diagnosed with the SRV Virus and that is when it all was set into motion. It took months to settle on the IPF diagnosis, but with the help of National Jewish Health, I am managing. It is 110 to NJH from my home but they have been absolutely Amazing, in all aspects.
I am another looking for the End expectations, like everyone else Medical People don't encourage it until you are near that stage. Also, the subject is hard to find.
I have finally had the courage to join the group and share my thoughts. I have been diagnosed as having less than 4 years so my time clicks away.
Thank You all for your sharing, It is sometimes difficult to read such tragedy but that is a future I must learn to accept. Blessings to All.
Thanks so much for sharing about your journey. I'm so glad you are getting such fine treatment. I've heard wonderful things about National Jewish Health. Sorry you've gotten the run around about the end stage too. I just released a book last week that has more on this topic as well as many other things we need to address as our time comes to an end. In case it's of interest it's called Pulmonary Fibrosis Journey: A Counselor and Fellow Patient Walks with You. Blessings to you.
Thank you for your story, like you said Dr.s are really unwilling to talk about later stages or end stages of IPF, which has been truly frustrating for me...I just happened upon this article...because unfortunately I’ve been coughing until I just about pass out. So I was wondering what to expect.
So Thank You from the bottom of my heart
Thanks for sharing. So sorry you've had the same experience I have. What a horrible experience with your cough. I know it is so awful. I write more about the end stages and preparing in a book I just released, in case this could help you. I hope so. In case it’s of interest it’s called Pulmonary Fibrosis Journey: A Counselor and Fellow Patient Walks with You. Blessings to you.
I lost my mother to IPF this past May. She was 85, but only diagnosed at 82. The doctors gave her one year to live, but she was a fighter! She lived with me, my husband and my 3 children for many years; the heartbreak is just terrible! Towards the end, I thought she just had a bronchial infection. After 7 days in ICU and many many tests, the doctors prognosis was grim, so she asked to just come home and be in her own bed. I stayed by her side, along with incredible hospice nurses, for 10 days straight. She passed peacefully on 20 liters of oxygen (doctor said it was incredible how we made that work) thru both a nasal cannula & mask. In her OWN bed (not a hospital bed) on her own terms. It was a gift to let her go with dignity ♥️ I pray for everyone affected by this awful disease; may you find comfort in knowing some of us will not rest till a cure is found. God bless you all ??
So sorry for the passing of your Mom due to PF. Thanks for sharing her story and how she passed. I'm so glad she passed peacefully. That is an encouragement to all of us. I love that her passing was in her own bed and on her own terms. What a blessing you were to make that happen. Thanks so much for rooting for all of us!
Dear PF warriors,
My grandpa passed away two years ago at the age of 83 from which we thought was IPF, however we found out later it was FPF. My grandpa was diagnosed accidently during an scan for other medical causes, 1,5 year before his death. He didn't have any symptoms during the first 0,5 year after diagnosis.
His PF progressed in periods of rapid decline and stabilisation. The first decline caused him to be dependent on 3 l oxygen while before he didn't use oxygen at all. The disease caused him to give in parts of his independency everytime. Driving his car, doing groceries, showering and during the last month he was unable to go to the toilet.
However he was never an old fragile man. PF is an devistating disease but he never lost his dignity. During the last month 10 l of oxygen caused saturation levels between 50-70 when moved an little bit. Two weeks before his death he decided that he wanted to end his live by 'death with dignity'. The only other option was going to an hospital and die on an ventilator, which was his biggest fear.
The last two weeks were one big party to celebrate his rich and wonderful life. We called lost friends from the army, all his friends and former neighbours. It was extremly hard for him to tell everyone goodbye each time an fried came visiting him, but meant everything for him. My grandpa was my best friend and he told me everything about his life and telling his biograpy gave him closure.
The day he passed away we were giving one last party for him with his children/grandchildren/great-grandchildren. He kissed his wife one last time after 65 years marriage and passed away really peacefully with an big smile on his face.
Since his death two other members of my family passed away due PF, however they were in the beginning stages of their disease and had an acute exarcebation and passed away in hospital.
Thanks so much for sharing about your Grandpa and his experience with PF. He sounded like such a wonderful man. I can tell from your kind heart that you are like him too. He was so blessed to have all of you celebrating his life and friendships. What a honoring way to leave this earth. Many blessings to you and your family.
How brave of you to offer and manage this forum. I admire how you are truly giving back to your community... locally and globally. My dad is 88. He was diagnosed with IPF 7 years ago and given 6 months to live. More than likely his PF is a result to Agent Orange exposure in Vietnam. At any rate, he was doing quite well for a long time and suddenly had to go on oxygen about 7 months ago. He was managing still well after this, maintaining trips to the gym, etc. Suddenly, his decline worsened. He can now barely walk 20 paces without feeling uncomfortable, much as others have described.
At his age, trials and transplants are out of the question and rightfully so. Nonetheless, we are hoping he can have as rich a life as possible in his remaining time. We currently live at 4,000 feet. The pulmonologist has said moving to a lower elevation will definitely help his symptoms. Have others done this?
I'd love to hear about anyone else who has considered changing elevation.
Thanks so much for your kind words. I want to be open that I don't manage the forum - my wonderful columnist friend Charlene Marshal does. She is kind enough to feature some of my weekly columns. It is a privilege to be able to write them. So sorry about your Dad's sudden decline. It is so discouraging when this happens :( I can see how living at 4,000 feet would be very difficult. Anything over 3,000 is really hard for me. I know that must be a very hard decision to make. Thanks so much for sharing!
I so appreciate this web site! If my husband had lung cancer there'd be a panel of doctors, a treatment procedure, and if that fails, predictable steps to death. But with PFF we're just left to cope on our own. One specialist basically said, "You're very sick, I'll see you again in 4 months." I'm now applying for long term paliative care (at home now). We only get broad statistics about longevity from doctors, but we don't know what to expect. Knowing what to expect is important to carers and patients alike. It gives us the choice to face end of life issues together as we want to.
So glad this column and website has been so helpful to you. It's great you're signing up for palliative care. I just released a book on amazon.com that can help you with navigating this disease. If you're interested it's called, "Pulmonary Fibrosis Journey: A Counselor and Fellow Patient Walks with You." I hope it will be a help to you, Kim
I read your book, such a beautiful informative well written book it’s not what I expected (thought it was going to be a collection of people going through IPF and their journey’s) but I was not disappointed, you shared your heart, faith, and fears with us and solutions on how to handle ours. Thank you, I have God’s peace that passes understanding, which I am so blessed and will be eternally grateful and appreciate the Bible verses you shared and they strengthen my faith and warm my soul The chapter on oxygen is helpful at this point of my disease progression I’m on 3L continuously but can’t seem to get them portable oxygen so I’m home bound right now til I navigate the system or buy one myself. I’m sure I will reference your book many more times as I continue on this journey. Thank you again very good book well done May God continue to bless you and give you his peace
Thanks so much for your wonderful comment. I am so encouraged to know that my book was a help to you. Thanks so much for taking the time to let me know. So glad your faith in God gives you peace that passes understanding. It sure makes this difficult journey easier. Many blessings to you ...
Just a few observations from a 76 late stager. Vanderbilt Univ. is doing a genetic study so if this has showed up in the family tree they would like to know. My Dad, Uncle, Cousin all died from this. I was diag. march last year 60% lung capacity so I have had this for quite a while. I think the biggest cause for advancement of this is lung infections.
Get flu shots, prevnar etc. Be careful around large groups of tightly packed people (wear a hosp. mask on the plane train). You will loose weight and your heart rate will increase, look up hypertension. Sleep in a recliner as lying flat makes breathing harder. Get one of those coccyx rings to save the tail bone. Do the night pulse ox sleep test. Look for a caring pulmonologist who will spend time with you answering your questions..
My mother is also having ILD since 5 years,she is suffering more in these days she unable to walk due to breathlessness.she is using oxygen concentrator,but taking 2L ,she is feeling cough or some discomfort,so she wants to continue on 1L,Is there any cause to not able take high level oxygen . Please give suggestions.
My dad is only 58 never smoked, rarely ever drank and he has idiopathic pulmonary fibrosis and pulmonary hypertension. The hypertension was diagnosed first and he had major surgery in 2012 to remove a load of clots from his lungs. Hes been declining a lot recently as hes been on steroids since November and hasn't been taking any meds to slow the progression, should be trying a new one this Friday as the last time he tried in November it put him in hospital. He's been on oxygen for exertion for a while now and his levels have been rather low for quite a few weeks so today he had a review and hes now on 24/7 oxygen and I have a super bad feeling I will lose him within the year. I hate seeing how much this disease takes away your loved ones so slowly and you know there is nothing you can do to stop it. Trying to stay positive for him and we are all doing a fun run to raise money for action for pulmonary fibrosis in June and just wish there was more I could do.
Mel I know what you mean. Your Dad is so blessed to have you by his side. I know it is excrutiationly painful for you. If it would help you, I just released a book called Pulmonary Fibrosid Journey: A Counselor and Fellow Patient Walks with You (Amazon). I hope it is a blessing to you and your Dad.
My dad was diagnosed with pulmonary fibrosis in Feb 2019. He was put on oxygen immediately. He had been given Methotrexate for psoriatic arthritis. This was during what they thought was a sinus infection. He started the med and by the third week he was having increasing difficulty breathing. Was almost passing out, turning white. His pulmonary doc told him to stop taking the med. it was not replaced with anything else, and I am fairly sure his liver function was not tested after starting the med. he got worse and worse and would not go back to the doctor until his next appointment. He collapsed in the bathroom and passed out. Went into ICU, was given steroids and antibiotics. They never got him stable enough to do a biopsy to see what exactly was happening. He lasted about 9 days. He was not in much pain until the last hour, and I would have given anything to trade places with him. I do not know much about this med or this disease, and I don’t even really understand what happened. Not sure if I should contact the hospital doctor and let him tell me what happened or if they even know.
As I lay in the same room with my 70 yo mother who has end stage IPF reading the comments on how difficult it is to diagnose PF, it infuriates me. She began this long road of trying to figure out what was wrong with her approx 5 years ago. Every time they thought they had it figured out, her health would worsen. After going through testing to qualify for a liver transplant (mind that the doctors believed her non alcoholic cirrhosis was causing her low O2 sats although her liver scans & liver enzymes were not worsening), they finally found the IPF which then disqualified her for any transplant. It took years for them to finally diagnose her properly delaying any treatment to slow the progression of the disease. In October 2018 she was put on 6L O2 & could function pretty well. This is when they still believed her breathing was liver related. It wasn’t until January/February 2019 they found out she had IPF & by this time she was on 10-12L O2 & too weak for a biopsy. The pulmonologist told her she most likely had less than 6 months to live at this point. The best choice we made as a family at that time was to go ahead & get hospice involved. PF is a progressive disease with poor outcomes for most especially in the later stages. Hospice staff is there to bring meds, assess, consult, educate, & are available 24/7 if a change in status occurs. Since feb/March 2019 here is her progression: for 3-4 months she was able to care for herself & do all ADLs progressing from a walker to a wheelchair. As those 3-4 months progressed we noticed that she would sleep more & more & that the more difficult ADLs like showering would wear her out quicker than before. She engaged in conversation but at times wouldn’t make sense or would forget basic things. While seated her O2 sats would stay in the low 90’s on 12L O2 but activity dropped it into the 60’s & 70’s fairly easily. Mid June 2019 (5 month mark) getting out of bed became too much. Hospice has brought her a hospital bed, bedside toilet, & other necessary supplies for comfort. O2 sats staying in upper 80’s at rest but quickly dipping into 60’s when repositioning herself in bed or getting to bedside toilet. Most times she is confused, speaks too softly to understand, refuses to eat or drink much, and is easily frustrated. But we still share many smiles too. She is off all meds excluding comfort meds like narcotic pain meds & Ativan. She sleeps 20-22 hrs a day. She is dealing with depression & trying to say goodbye. I don’t know what it looks like from here or how much longer we have. My suggestion (if given the opportunity)is to choose a hospice provider sooner than later to reduce ER visits & employ a good caregiver team. Family, friends, etc. This disease is tough on all parties involved. Comfort should be a priority.
I'm currently on 5 l and I seem to be suffering shortness of breath when I exercise after reading your blog it sounds like boosting the oxygens settings will give me more flexibility and movement however I have been told that pulmonary fibrosis is the lack of converting lung oxygen into blood oxygen if that function does not convert from one to the other what does increasing the oxygen in the lung help in any way this seems to be a problem of understanding. if I can get past that I might arrange to get more oxygen. Am I at a dead end at 5 I'm a mechanic show I need somebody that speaks to me in terms of how does what do what. I have no desire to carry on when I have to guess for breath to use the bathroom anybody that can better explain that transitional process from long oxygen to blood oxygen please contact me I really would like to know at this point quality of life is not there JT
My father is in the end stages as they call it of ipf. It’s incredibly hard to watch someone so active and previously fit struggle. He is on 10L of oxygen permanently. The hardest thing as a son for me is how withdrawn he has become. I know he wants to be involved in his grandchildren and families lives but it’s just not possible. It’s as if he’s giving up. I wondered what I can do to at least try and get a smile out of him these days. I am truly sorry if this sound so depressing it’s just a bit tough at the moment.
Diagnosed almost 15 years ago. Pretty stable until the last year and a half. Progression has been pretty rapid, lately. The only final stage symptoms I've not experienced are blue lips/fingernails and clubbing.
Weight loss has finally stabilized, but I force myself to eat. I'm simply not hungry. When I do eat, it's like taking a strong sleeping pill. :) My body can't handle it.
I also like to know what is going on, and doctors get closed mouth about this, for some reason. Very frustrating!! At first, all I heard was false bonhomie and 'dont do this' and 'try that', and 'you'll be ok."
I had to try several doctors to get any answers, at all, and all that doctor offered was a diagnosis. Not even a prognosis or treatment options.
I had to research those on my own.
example: How can you prepare and update wills, prepare family members, etc., w/o prior knowledge?
My mum passed away 4 weeks ago age 55.She had slight trouble breathing a few weeks before and her GP said it looks like she could have fibrosis but wanted her to see a specialist at the hospital. A few days after her GP appointment she was admitted into hospital in an ambulance because she was struggling to breathe, this was on the Thursday. She was then put on oxygen 24/7 and did not eat at all and also had barely ate a few days before going into the hospital. They give my mum intravenous antibiotics to fight an infection they believed she had but did not know where the infection was coming from. They told my mum they would need to do a biopsy on the Monday and a CT scan to see how bad the scarring was on her lungs and to determine where the infection was. Over the weekend my mum found it more difficult to breathe and she was put on the highest oxygen they could give. In the few days that followed she was put into ICU (on the Monday)in an induced coma and paralysed which was supposed to be to give her lungs a rest and fight a supposed infection she had. We never got a chance to say goodbye to my mum before they put the tube down as she deteriorated very rapidly from going from the ward to ICU. We had to turn her life support off on the Tuesday night, 5 days after she had been brought into the hospital. She had suffered multiple organ failure which we believe may be sepis but the coroner has done an inquest into her death as he is not sure what the cause of death is. All of this was so sudden and we had only been informed a few days before from the GP that it may be fibrosis so we were just about coming to terms with that diagnosis and had no idea that when she went into hospital she would pass away so sudden. It has been absolutely heartbreaking and I think pulmonary fibrosis should be spoke about more as we had no idea of this illness and now it has taken my mother in the worst way possible.
Thank you for sharing your story. Unfortunately, my Dad passed away almost 90% the same way as you described with your ICU visit. He was diagnosed five years ago, but we were caught off guard with how quick the end stage was. So much so, that the doctors had never even told us to consider hospice. My dad passed away five days after being admitted to the ICU from respiratory failure. It was so shocking. I am so sorry to hear that you went through this. I am thankful you posted, as I am finding "comfort" in reading the similarities with what happened with my Dad.
William J Torres
I was told in Sept of 2019 that I have PF. So what, it pales in comparison to what my Savior Jesus went through. Get right with God and your future looks bright. I will go through my circumstance bravely as I put my trust in Jesus. I won't run from this adversity and will offer it up to Jesus for His purpose. Better the day of death than the day of birth. Thank you Jesus.
Husband diagnosed with PF six years ago lung cancer diagnosis Dec 6th 18 treatment unsuccessful and made fibrosis worse put on oxygen in April which went up and up in the last month and he became bedridden and unable to breathe passed at the age of 56 on 30/10/19 Heartbroken.
After two years of illness we were recently told that our Mom has pulmonary fibrosis and that she’s reached the end stage. I’m not sure what that quite means, the doctor told us that SATS of 88% is normal and that because my moms SATS have dropped to 40% and she is tolerating it, it’s characteristic of the end stage.
I know that she is finding it more difficult to walk, needs more oxygen (4 litres), eats less, is cold all the time has swollen ankles.
She’s still able to walk with a walking stick, but she’s very slow. I don’t know what the progression of the disease will be, how long she has left.
We’re all so upset about what has happened, and want to make her as comfotable as possible.
I’m trying to understand what will happen next to help prepare us as I think planning to give her the best care is all I can do for her now.
My mum (74) was diagnosed with PF 18 mths ago . She is now at final stage , I’m writing this from the hospital room as it was suggested she may not make the night .
Doctors seem to be afraid to talk to me to let me know what the final stages will be .
Mum was brought into hospital 6 days ago with SEPSIS , she Requested they stop treatment.
Prior to this mum was introduced to oxygen in March and levels slowly increased she’s now on 15l 24/7 , Sats 90 when still any movement drops quickly ,she was able to get about but very slowly , loss of appetite nothing has any taste , slight confusion, no swelling at all.
This is a horrible disease that no one really seems to be able to say what will happen next .
Mums fears are pain and suffocation DNAR signed , she sleeps must of the time now and I’m now just waiting for the final breath .
Thank you for this forum it has helped to know what to expect .
I know this post is old but thank you. My Mother didn’t know she had ILD/PF but last week went into the hospital after a period of weakness, coughing, vomiting and fatigue.
She was admitted January 23rd and told she had pneumonia then by the 26th was moved to ICU and by the 27th she was on 100% hot air flow mask & gasping for air. She died 2am the 28th at 67. It was a horrible sight to see her frail and desperately gasping for air. They had her loaded with steroids and antibiotics. Based on a Lung cancer screening two years prior we were told before she died that she apparently had it then. For a year she was fairly stable, then a progression and then a super fast exacerbation leading to death.
Awful disease. Just horrible. My heart goes out to everyone dealing with it.
Hello, My Mum passed away from PF April 9th. I am on this site trying to understand why we were so ill prepared emotionally. It is so important to recognize the stages of this disease, comfort the patient and acknowledge the impact this decease is having on the person. I read that most were shocked by the ferociousness of the end stage. If your loved one has this disease, take the time to be with them and cherish the time you have aside from the required medical appointments. My mother had PF for seven years and we were in denial of what was happening and now have to live with that.
My daddy passed away on 19th December 2020 from this awful disease. He was 70 and had been diagnosed in 2018. It was very quick at the end as my dad put of going on oxygen fulltime to just a month before he died. It is so hard to watch a loved one deteriorate near the end. Heartbreaking.
Janet E Lowery
I want to thank you all for all I have read here. You are brave and have been through and are going through a lot. I understand. No, I do not have pulmonary fibrosis, my brother does. We live many states apart and do not get to see each other. I spend nearly every day looking for information, praying I will find something of a miracle. Logically I know that will not happen but I believe in God and what He can do. I am a retired RN and worked both in hospital setting and in hospice care. I wish so much that I could be there for my brother as I was for so many patients. I loved them all. Please know how much your family and friends care about what you are going through. Be honest with them because they are hurting also What they imagine is far more difficult for them than what you can share with us. God Bless you all. You are and always be in my prayers. LOVE
I must say it was such a relief to read all your messages. I am in the UK and caring for my husband with IPF. The thing that amazes me is that the hospital recommended steroids to slow the process down. However he refused, due to being on prednisone for 18 years to treat another virus. The use of steroids for 18 years is the cause of IPF. He is now in his 7th year and the cough is gradually getting worse.. You have all answered my question... "what happens after the cough gets worse"... thank you to you all and bless you all.
My comment is short,. I was diagnosed with IPF 5 years ago. Got Oxygen 2 years ago. All my paperwork is in order. Got Hospice 3 months ago. Gradually getting harder to breath. Use morphine every few days. Try very hard to control my anxiety. Mostly successful. Pure willpower. Try to be kind and gentle to my wife. Some chest pain today. Have been almost pain free for the entire time. I think that it is a day or so now. September 23, 2021.
Mrs Jane E Murray
My dad is 84 years of age and has pulmonary fibrosis. He got admitted to hospital for 11 days as he got so weak and had to sort his oxygen levels out. Now he has deteriorated really fast, hardly eating and not able to move about and getting really breathless. I'm now coming to terms that he may only have days left. My poor sons one who us still at home and youngest is in the Isle of Man and now had to warn them both that he has not got long. It's breaking my heart just putting this into words, my poor mum has bern his rock and now she will be losing her husband to this horrible disease. I feel for you all on here we are all experiencing something that is so hard to except.
hi, my uncle recently passed away due to this disease just wanted to know if you're there and staying strong
Mrs Debbie Hardy
Hi everyone I am now 60 last year I was told I have NSIP fibrosis. I don’t really know what to expect. I have been put on mycophenolate to help with the lung swelling. Before this I had pneumonia a few times then put on steroids but that didn’t help. After having X-rays and scans and cameras they found ground glass and fibrosis. I don’t know if this is the same or different than pulmonary fibrosis. It’s all new to me all I know is my doctor said “sorry”. So it must be bad.
Darlene (Constance) Cochran
Just a general comment. I too am a IPF patient and understand the many questions that occur and visit our minds. Just a few thoughts that have come to me as I try to manage my life and my illness. After the initial diagnosis and my understanding of it, and this did not take long as my husband died from it in 2003. I decided to take charge of how I was going to live my life. Next, I reached a point of wanting to live well and then of also wanting to die well. Impossible, not really! But not easy I know. So, the main activity was to be informed. The Pulmonary Fibrosis Foundation, pulmonaryfibrosis.org, became my go to place. My Pulmonologist and I both learn from this, and this keeps me as current as I can be with all the variants that happen with PF/ILD patients. My diagnosis was in 2019, actual fibrosis seen on x-ray as early as 2015. Now it is 2022 and it has been an interesting trek. I have learned about myself, my illness and am trying to use that to live my time left with meaning. I also am helping to prepare not only me, but my family how to accept the grace of my life and death. It is a choice, so find the best medical care you can, learn all you can, live life to your fullest with what you have. I used to go everywhere, do everything and had energy to spare. Now I cannot but I still find that knowledge, volunteer work from home, zoom meeting from my chair, phone conversations with family and friends. Do I have times that I hate this, of course!! But I am not going to give up the joys of my life to my illness. I have to fight for this, but it is worth fighting for. I have a good idea of the end, since I was my husband's caregiver when he was ill and with him up to his death. We do not choose our method of death, but we can choose how we deal with it. I wish to the best of my ability to die with some measure of grace and with a family that has been prepared for it. When my new zip code is John 3:16 I will be at rest, and I want my family to know and understand that. So, at my age, I have come to grips, it is easier when you are already past your normal life expectancy, and you are in your frosting years. When you are younger it is much harder to face. My blessing is that my life is being impacted at a time where my acceptance of end time is much easier. Not any answers in all of this, just sharing a bit of my journey. I suggest that if you can do so take mental charge of your life while you can and set up a plan with some plan b, c and d as well. Share with family, share with anyone who will listen. PF/ILD and IPF and all of the other illnesses in this group need exposure to the light so that care is available, science is involved, and cures and treatments are developed. KNOWLEDGE and AWARENESS are the key to a better future. My prayers are for all with challenges in their life which could be illness or any of a thousand other things. May strength come to you as you travel your journey. Look at the time you have as a gift and treasure it as you move forward. Not everyone has the opportunity to prepare and manage their remaining days. I do Lord and I thank you for that. God Bless you and your families. In the name of the Father, Son and Holy Spirit Amen.
Sandy, give me a call when you want to talk. This is a reply to you note on this PF News site. I'm writing this on 2/23/22. Send me an email with your phone # and we can chat if you would like. We are all in the same boat and trying not to be a burden to those who care for us.
I have been under hospice care for a year. I also started out with 2 & now am on 8
I can't get around very good. I bought a 3 wheel scooter to move around the house.
I'm 85 years old today but know my time is getting shorter. But I'm just leaving my life in God's hand's. There are times when I when I wish it was all over as the coughing is tough.
J Ward, sorry to learn about your dad. I'm 83 and have been diagnosed with IPF since 2019 and I'm limited to about 20' in walking distance and I'm on 10-12L of O2 during the day and 6L at night. I'm not sure you can get a good estimate of when the end is going to be but one thing for sure is to know where you're headed when we leave this good ole earth. Jesus made a way for us and if we believe in that way, we're all set. And let me tell you, when we get to the other side there is no more IPF, that's the good news. For those of left here alone we just need to be ready, so when the time comes, we can be with them again. For now, if we can stay busy, helping others with the rest of our faculties that still are working that is the best we can do. Only God has our number and when He calls it we need to be ready to go. All the best, John 3:16 someone said on this blog is their next zip code, I like that!!
Y connectors should be available from your concentrator supplier. We use 2 concentrators so we can shut one off with the switch and just run one at 6L and then in the morning turn on the second one at 4L. Our supplier didn't want to run one concentrator at 10L all the time so he supplied 2 concentrators at no additional charge. That is very nice and makes it easy for my wife to just shut off one switch at the end of the day. I find that the "D" tanks on a dolly give better reliability especially when there could be a power failure or are away from home and need a back up.
Thank you so much for your time in research and posting it. I have had PF for about 5 years was found in early stage because I insisted on answers and the doctor finally did lung biopsy. Anyway I have slowly progressed and last year went to Duke for testing for a possible double lung transplant. I was approved but made the decision not to go thru it because of the risk at my age. Decided to live the rest of my days enjoying what time I have left. I stopped taking a lot of medicines such as sleeping pills and antidepressants have lost some weight and I am feeling much better about myself. My husband and I started doing some traveling and just enjoying life when he had a grand mal seizure which was worsened his parkinsons to the point that I am now his caregiver. This has taken my mind off myself and I am doing fine. The Lord has given me the strength that I needed when I had to have it. Looking forward to every day we have left together.
Tonya B Worley
When I first was diagnosed I blindly search for answers and found none so just recently I decided to turn to the internet for support groups and forums relative to IPF/PF as a patient myself. I am glad I did after I got over the initial shock of being told and then reading every medical venue that mentioned IPF/PF and saw the broad spectrum of what it is, what to expect, and/or what to do. So broad that anyone in their right mind could not begin to even make sense of it. The real clincher and what truly took me by surprise was to find out 3 years into my diagnosis that almost every doctor I have seen in these 3 years has been totally ignorant to what IPF/PF is and/or the prognosis and even diagnosis is. Of course only one of these doctors admitted to their lack of any knowledge of IPF/PF, the rest of them resorted to covering up their ignorance with arrogance and if pushed this arrogance could become quite insulting and demeaning, so needless to say for this reason and several other reasons not related I have been through about 7 doctors in 3 years and yes, I mean pulmonologist. The one doctor that admitted to not knowing jack about IPF/PF was a primary care doctor, not a pulmonary doctor but the rest were pulmonary and they knew very little some of them nothing at all. On a different site or forum, someone suggested to me that I seek out something called a "Center of Excellence" i.e. "COE" on pulmonary issues. I found that I really don't have any so-called COE close by but a secondary suggestion was Schooling Hospitals such as Shands or the University of Florida in Jacksonville etc... as they are closer to me I will let you know how this goes
Thank you so much! I have pulmonary fibrosis secondary to Sarcoidosis. I, too am a mental heath professional (Clinical Social Work) and continue to provide psychotherapy through a Telehealth service. I purchased your book and really enjoyed it, especially the part about considering a lung transplant as I am in the same situation. I don't think I'm going to opt for the transplant and found your discussion of that choice very helpful. This recent thread has been very helpful and I thank you for your honesty. Keep up your great work for the PF community!!!
Thank you for this website. My husband Russ started on it in 2014 when he was first diagnosed with PF. It definitely was a blessing in his life. He passed away peacefully in 2018 without ever having to be hospitalized. We were able to travel in our RV until 2017 when it got too much for him. Luckily he had a wonderful Dr. in Seattle during that time and he also went through all the testing for a lung transplant, but did not qualify. We had the benefit of hospice care his last 2 months. It has been interesting reading all your stories. I have kept this website available as it helps me keep up with changes that may be happening for people struggling with this disease. PF was unknown to me when we started. Our strong Faith made our journey much less strenuous. Our journey was difficult too with many ups and downs. I keep all of you in my prayers on a daily basis. May God's strength carry you through all your struggles.
Finally an honest answer! Thank you so much!