Wrestling with the End Stage of Pulmonary Fibrosis

Wrestling with the End Stage of Pulmonary Fibrosis

Just breathe, passionate help for the PF journey
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.

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

  1. A gradual decline in lung function over time, although for some this can be sudden.
  2. Patients require more oxygen over time when at rest and especially when moving.
  3. Over time, it becomes harder to walk and go out because of shortness of breath.
  4. More fatigue, more sleep.
  5. 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.
  6. Patients may eat less and experience weight loss.
  7. Sometimes increased anxiety and depression are experienced.
  8. 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

(Photo by Kim Fredricks)

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.

113 comments

  1. Elizabeth Bushey says:

    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

  2. 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.

  3. Jennifer Jones says:

    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.

  4. Wayne and Wendy Dixon says:

    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.

    • Stan L'Hoste says:

      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!!!

    • Jules g says:

      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

      • Julie says:

        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.

        • Linda Schwartz says:

          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.

        • Mel says:

          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. 🙁

    • Carla says:

      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

  5. Carolyn Lee says:

    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!!!

    • Lynda says:

      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!

      • Hi Lynda,
        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 🙂

  6. Debby Clarkson-Greene says:

    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.

    • Len Charette says:

      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?

      • Bob Wells says:

        Len

        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.

        Bob

        • Rhonda says:

          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.
          Rhonda

      • Cheryl says:

        Two 10 liter high flow concentrators can be hooked together with “Y” tubing thus providing higher flow for the patients that need it.

        • Susan Scott says:

          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.

        • Ron Cade says:

          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.

        • Bob Wells says:

          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?

          Bob

      • Donnie Payne says:

        Greetings!
        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.

      • Fadoua says:

        Dear Len,
        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).

      • Carroll says:

        Hi there
        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.

      • Deborah Herndon says:

        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).

    • Stan L'Hoste says:

      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…

  7. Isabel Mickle says:

    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.

    • Terry Moriarty says:

      Isabel,
      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.

      • Yvonne Ray says:

        Isabel,
        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.

  8. Isabel Mickle says:

    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

  9. Isabel Mickle says:

    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

      • Isabel Mickle says:

        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.

  10. Jan Chicoine says:

    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.

  11. Dana says:

    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

  12. Sandy Cunniff says:

    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!!

    • Vicki White says:

      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.

    • Tiffy says:

      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.

    • Rhonda says:

      Sandy,
      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!
      Rhonda

      • Karen Kelley says:

        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 .

  13. Eileen Lewis says:

    Kim,

    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.

  14. Bob Wells says:

    Kim

    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

  15. Katie Stewart says:

    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?!

  16. Mary Lasowski says:

    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.

  17. Sandy says:

    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.

    • Hesed says:

      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.

  18. Judy krasovec says:

    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

  19. Elayne Lemanow says:

    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.

  20. Debbie Herndon says:

    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.

  21. Susan Scott says:

    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.

  22. Jen says:

    Dear Kim,
    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. : )

  23. KL says:

    Kim,

    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

  24. R Dhillon says:

    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.

  25. Rose says:

    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!

  26. Adam says:

    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 says:

      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

      • Adam says:

        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 says:

          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

    • Renee VanCleaf says:

      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.

  27. Vic says:

    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.

  28. Lyle Romans says:

    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

  29. Renee VanCleaf says:

    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!

      • Renee VanCleaf says:

        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!

  30. Rose Anne says:

    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.

  31. Lee says:

    Kim
    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.
    Thank you

  32. Judi Bailey says:

    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.

    • George Clayton says:

      Hi Judi

      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.

  33. George Clayton says:

    Hi Kim
    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.

    DX 12/16
    RA with ILD
    Immunosuppressant treatment
    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.

    • Luan Smalley says:

      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.

      • Stan L'Hoste says:

        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.

    • Stan L'Hoste says:

      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…

      • Joe says:

        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.

    • Sue Routin says:

      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.

  34. 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!

  35. Judi Bailey says:

    Thanks George Clayton for your reply. Knowing that others are also coping with an illness that is least understood,I feel less alone.

  36. I M Mayariwala says:

    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 .

  37. Stan L'Hoste says:

    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!!!!!!!!

  38. Katy says:

    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.

  39. Jackie Raica says:

    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.

    • Hi Jackie,
      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.

  40. Joyce Douglas says:

    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.

  41. Jay Eula says:

    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.

    • Hello Jay,

      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.

  42. Kathy says:

    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.

  43. Sherry says:

    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.

  44. Debbie says:

    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.

    • Dear Debbie,
      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.

  45. Catherine says:

    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.

  46. Crystal says:

    Hello,

    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.

  47. Bonny Fugett says:

    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.

    • Catherine says:

      Dear Bonny,
      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.

      • Bonny Fugett says:

        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.

  48. Sara Narciso says:

    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?

    • Sara Narciso says:

      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?

    • Sue Routin says:

      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

  49. Terry Moriarty says:

    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.

    • Terry,
      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.

  50. Debbie says:

    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.

    • Cathy says:

      Dear Debbie
      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

  51. Michele Neloms says:

    Hi Kim,
    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!
    Sincerely,
    Michele

  52. Jacqueline McIntee says:

    Love the information it was better than the doctors gave to me i would like more information thank you

  53. Pat Ratcliffe says:

    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.

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