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.

53 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.

  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!!!

  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

      • 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

  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.

  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.

  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.

  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 O@ 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.

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