Sharing Your IPF Story: How Do You Feel About It?

[Karen Martin]

I am 69 and the first thing I said to my pulmonologist on a visit just after my diagnosis was, “I don’t want this.” Of course not! I have been fortunate with my general health in the two years since that diagnosis and life goes on. However, yesterday I got a call from the transplant team at Duke to set up a consult week to help me understand what is involved in a transplant. The very nice woman told me that they like to have their patients informed as they “approach the window of transplant” so they don’t let it pass. It was the single most devastating thing I have ever heard!! Because if that window passes and I haven’t done anything, I presume I know what comes next! I admit I am ambivalent about the idea, but my daughters have urged me to go and find out all I can before coming to any conclusions.
I have friends who feel sorry for me needing oxygen. I have friends who think I am strong and brave. I have friends who think I have a great attitude about life in general and see me as always a happy person. What I can tell you is that at this moment, I feel the most confused and conflicted I have ever felt in my life. Karen

[Miltont]

I am a very pragmatic person. I face up to and handle reality very well. I don’t mind sharing. However, when I got the news and was told that at 79 I had an expectancy of 1 – 3 years without a transplant and I found that the oldest transplant approval was aged 72 I was deeply shocked.  My wife was in the same hospital having just had a major section of bowel removed because of cancer. I went from Emergency to visit her but there was no way I could tell her. It took weeks. I did not tell my kids and grandkids for 3 months, after the next lung function test showed that I had stabilised at 75%, so I felt that the prognosis may have been a bit severe. The question was how to tell them. At a family gathering was deemed inappropriate as it would destroy the occasion. That said, they had to find out virtually at the same time so no-one would be offended. Eventually I got on the phone. Very difficult. Sharing with associates was heaps easier but last week at a dementia sufferers’ group that I lead I was,asked to explain my emotions – how I felt when I got the news. Thst was very hard and depressed me for two days. That is enough from me.

[Tom Nicholas]

I was not sure if I wanted to respond to this thread. After thinking about it awhile, I remembered how either talking or writing about things was good therapy. Maybe my story might encourage others to share their stories, and we would find, we are not so all alone in our thoughts nd our journeys.

My IPF story started almost 4 years ago. We live in the mountains of Western North Carolina, at an altitude of about 3,000 feet. My wife nd I daily, would hike these old gravel, logging roads, 2-4 miles a day. It was late fall and although I am very healthy, I had suffered cold, and couldn’t quite shake it. As the weather turned in winter, my shortness of breath became worse. Yet we were climbing steep inclines in my late 60’s…maybe this was just part of the “aging process”. The SOB continued to early spring, and I remember carrying a 40 pound bag of top soil. After just a few feet I was totally out of breath. This wasn’t “right” so I scheduled an appointment with my doctor. Who listened to my lungs, thinking I might have pneumonia, I was sent to the local hospital for a chest x-ray. Later that day, my doctor’s FNP called me and said the x-ray did show something and I would be treated for pneumonia for 10 days, if it did not improve, I was to return. The FNP and I had known each other for several years and were close. When I asked what the x-ray showed see was real emotional and almost in tears and said we, “we just don’t know, and you’ll probably need some additional tests.”

I am very proactive regarding my medical history, and I belong to several “portals” for my medical information, including the portal for the hospital. So, I pulled up the notes on my chest x-ray. There it was, three words which changed my life forever. Three words which suddenly unlocked thoughts and memories I put away over 20 years ago. Three words: “potential pulmonary fibrosis”. My mother died of pulmonary fibrosis over 20 years ago. The last year of her life, especially the last months in hospice. There is an another entire journey in those times, not for here. My mother was an RN. She raised 5 children One of them me! Me alone would be a challenge for any mother. My mother was a strong woman, yet this disease dictated otherwise. Twice during hospice, with my wife sitting next to me, my mother asked me to do the unconscionable. The first time she said I should take her pillow and help her to heaven. I could not believe my ears. The second time, she was on morphine. She asked me why I was always fighting her. If I really wanted to help her, I should make her a strong cocktail (morphine) and no one would ever know. Of course, I did not. By some luck, and blessing by our maker, I returned by myself about 3 weeks later. I noticed my Dad had not put anything in her “log” for 3 days. I asked him about it, and he said she’d been sleeping a lot the past 3 days. I looked at her closely and she was sleeping with her mouth open, so I tried to close her mouth, it was rigid. She was definitely in a coma. My Dad went off to the den to write a few emails. I took my mother’s hand, and told her she had fought the good fight. I was going to be there for the duration to be there with Dad and stay with him for awhile. I told her I loved her and told it was ok to let go. Less than 5 minutes later, her breathing stopped and she had passed on. My father, my mother’s care giver, died 4 months later of a massive stroke.

My brother was diagnosed with IPF 18 months ago, another kick in the gut! So pretty much for sure, our IPF is inherited. Now we worry about our adult children….

Those were the memories which came back crashing down on me. I read the x-ray results several more times with tears streaming down my cheeks. I waited about 3 days before I could even tell my wife. Unfortunately, when I did tell her, she too knew what would eventually unfold.

We still try to be active. There are many stories here left untold. I’ve always been person to never have someone do for me what I could do for myself. Which meant learning many new skills. Asking for help was always uncomfortable for me…a sign of weakness. So, I am getting better at asking for help. Yet, now on O2 for exercise and exertion, I’m beginning to relent. At the same time feeling frustrated I cannot do many things I was able just a few months ago.

On positive note, in a few more weeks we will be able to go out kayaking, me with cylinder. I am NOT slowing down! Ok, maybe a little bit!

I am a candidate for lung transplant at Duke Medical Center. I am “early” in the transplant window. Knowing the alternatives scare me. As I know first hand what this disease does to the patient, their loved ones and friends.

Each of us in control of our feelings, emotions nd thoughts. We can choose how we navigate these trying times. Me, I move forward with a positive attitude.

[Bert Maidment]

I was diagnosed with IPF almost three years ago, however, I suspected that something was wrong due to shortness of breath climbing stairs in our 3 level townhouse and trying to get back in shape to play ice hockey again (I just couldn’t get going and I thought it was just that I was really out of shape).  I have one floor living and am on oxygen 24/7.  I cough intensely most mornings (I passed out one time and often trigger laryngeal spasms that impair inspiration of air). I have an incredibly supportive wife and family (with 5 grandkids) which provide a foundation of strength. Coping with this X-ing disease is exasperating. I qualified for new Phase III clinical trial of a new drug (panrevlumab) and have had 6 infusions so far over the last 6 months.  The coughing has gotten milder but it now just a nagging frustration. I go Pulmonary Rehab twice a week and can exercise (with 8 liters per minute of oxygen) fairly rigorously. I am determined NOT be defined by this disease.  I am a little crazy (as you might have expected after playing ice hockey for 47 years) so I sought out a challenge and found the American Lung Association’s Fight For Air Climb.  I decided to sign up and attempt the climb — 42 flights of stairs! My daughter and 10 year old grandson made a decision to support me and climb with me. So ,,, https://www.kmbc.com/article/<wbr />man-with-lung-disease-tackles-<wbr />fight-for-air-climb/31179883<span style=”color: #222222; font-family: Arial, Helvetica, sans-serif; font-size: small;”>  this link will show you the results!  Now most people do the climb in 15-20 minutes, it took me an hour and 8 minutes. I paced myself with my pulsox and took many rests. I pass this on to let you know that we all can do something to challenge ourselves no matter how big or small that gives us a sense of more control of our lives.  You can still do things, just modify your expectations and be kind to yourself.  I have another infusion next Monday and will continue being a guinea pig in the hope of helping to find a mitigation for this disease…BERT</span>

ps – humor helps:  my favorite song is Stayin Alive by the BeeGees!

[Charlene Marshall]

Oh Karen, your post is so important for others to read but also so heart-wrenching…. I agree, there is nothing clear about this disease, including how to feel! Like you, I’ve spent many days wishing I didn’t have IPF and that it didn’t exist. In addition to how I feel about it, navigating the emotions, questions and sometimes judgements that come from others is also tough. I can tell you the information that comes from transplant assessment is exhausting, and it gives us so much to think about. However, information is power and then you can make an informed decision. Will this meeting still go ahead for you amidst all the COVID-19 chaos? Will be thinking of you and keep us posted.
Charlene.

[Charlene Marshall]

@bmaidment

Hi Bert,

Thanks so much for writing and sharing a bit of your story with us. As awful as this disease is, and while I wish none of us had it… I find comfort in this forum and connecting with others who truly “get it”. Like you, I’ve heard many who suspected their disease was present long before they were diagnosed due to shortness of breath and fatigue. These things can be suggestive of so many other things that doctors look for first! I’m so sorry to hear about your cough, I know it can be debilitating, although I am glad to hear your wife and family keep you going and give you that foundation of strength. Continue to make it down that you are not defined by this disease – so important, and kudos to you for thinking that way! I loved reading the article you sent me, and I hope others take a peak at it as well re: your stair climb. Keep inspiring!!! 🙂
Char.

[Charlene Marshall]

@tom-nicholas,

Hi Tom,

Thank you for taking the time to share your story — I am glad you did, and I have no doubt it will help others! As you say, sharing our stories (albeit difficult) give us the power to help others feel less alone and I appreciate the time you took to write us.

It sounds like we were diagnosed around the same time, as I am approaching 4 years in April. I can’t imagine how you felt reading the three words on your patient portal, especially having known what your Mom went through, and now your brother’s diagnosis. That must have been so tough! I don’t know whether knowing what IPF is or having never heard of it is worse upon the diagnosis, or maybe there isn’t a worse? It just sucks either way. Imagine the day a cure is found for this disease — what a celebration that will be! I just hope it is in time for all of us to enjoy. I feel it in my bones that it will come someday.

If there is a genetic link, is there any testing your hospital/treatment facility can provide for your adult children? If so, do you think they would do it?

 

I love the comment on your kayaking! I did this last year with my cylinder in my lap … I have a picture of it, I’ll try to remember to email it to you if I can find it. It was for a work team building trip and they always laugh that I went with my oxygen, but I refuse to let this disease stop me from doing the things I enjoy 🙂

Keep in touch and thank you again for sharing your story. I have no doubt it will help others!
Charlene.

[Charlene Marshall]

Hi @miltont,

Thank you for sharing your story with us, and how you ended up telling your wife and family about the diagnosis. This is one thing that I know many struggle with — especially the timing of it all. We tend to want to protect the ones we love from bad news, and then in large gatherings as you say, we don’t want to ruin the mood or the fun. I’m glad you were able to able to tell them, and that they can be there to support you. Absolutely very difficult, as you say. Hope you continue to do as best you can and thanks for being part of our forums community! Charlene.

[Karen Martin]

Hi, Charlene. Thank you for your encouraging words. I have no idea if the consult will happen as a result of the COVID-19 mess. I will say that for now I am just taking life one day at a time and doing my part to stay healthy and positive. I woke up with a song in my head just recently, which happens to me a lot. You are way too young to know it but you probably can google Doris Day’s “Que sera, sera.” Whatever will be, will be. So true for all of us and comforting to me in a strange way. I will let you know if my consult happens and what we decide. You are a great comfort to so many of us with your posts. Big hugs to you! Karen

[Charlene Marshall]

Hi Karen,

Thanks for circling back and letting us know how you’re doing. It’s so tough not to know what is happening with upcoming appointments, isn’t it? Scary really! Your approach of taking one day at a time is a good one, it is all we can do right now (as hard as it is). I just wrote a column about trying to find some good amidst this pandemic, it should be published Thursday. It really lifted my spirits as I compiled some good things happening around the world right now! I will look up the song, but often the sentiment I catch myself saying regularly, which is very similar to the song is: it is what it is. Thanks for sharing, and for your kind words — I really appreciate them 🙂 Thanks for making my day a little brighter! Sending hugs right back to you, keep in touch!
Char.

[Sarv Punj]

Hi Charlene,

pits heartening as well as heart rending to read experiences of different IPF patients. I shared the story of my wife yesterday. She was started on Cyendiv 150 mg twice daily ( Same as OFev)  last month in combination with Perfenidone 400 mg three times a day after being hospitalised for 10 days due to sudden sudden exacerbation of her illness . As I had mentioned she had been on low dose of Steroids (max 20 mg)+ Perfenidone 800 mg three times daily.She did fairly well for about 5 yrs , but the breathlessness started increasing with little exertion.However, it’s only last year that she started using oxygen at 2 lpm during day only and was doing well with that. Later the requirement of Oxygen increased and she was put on Oxygen 24×7. The oxygen requirement kept on increasing and after her recent hospitalisation its 5 to 7 lpm. She is yet to feel any difference after starting Cyendiv which costs whopping Rs 66000/- (say US dollars 1000.00) for a month’s supply.Its really hard on me but I want her to live.I have only 2 daughters both in U S and both have been suffering fro Autoimmune diseases , one with Idiopathic Pulmonary Hypertension, and another with Moyamoya and other multiple issues.
I am looking forward to early breakthrough in the ongoing research to at least benefit those who are recently inflicted with this life threatening disease.

Sarv Punj

[Ross Harris]

My 90 year old mother passed away on June 19, 2019, from respiratory failure do to IPF. I had noticed difficulty in doing things that I could normally do with ease. Initially, I dismissed the problems as being allergy related. Finally, in the middle of September , 2019, two things happened that started me on the path to my IPF diagnosis. First, I developed a severe sinus infection that sent me to the doctor for treatment. I was prescribed antibiotics with dosages lasting for 7-8 days. I thought that after the infection went away there would be an end to the matter. Not so. I developed a dry cough afterwards that sent me back to the doctor and that’s when things really picked up momentum. Blood tests, 24 hour urine tests, more blood tests, and two scans were taken before the diagnosis of IPF (November 12, 2019). When I was told of the IPF my first words were “That’s what my mother died of”. I started my first dosage of OFEV on November 29, 2019. I was supposed to have my second appointment at the local lung institute yesterday, March 25, 2020, but was rescheduled to the middle of April because of the Coronavirus. Prior to my recent symptoms I was in very good health and worked out at the gym on the treadmill and weight machines. With my mother’s IPF illness and death, I’ve witnessed the devastation this illness can cause the human body, but I am determined to maintain the exercise routine as long as possible, just as soon as the Coronavirus restrictions are removed and the gym opens again.

[Charlene Marshall]

Hi Sarv,

Thanks so much for writing to us and sharing a bit more of your wife’s story. Gosh I can’t wait for the day a cure is found for this cruel disease! You’re right, it is heart wrenching to hear how many people are impacted by it and how all of our experiences are so very different. Sorry to hear of your wife’s exacerbation – were the doctors able to determine a cause of it? Really keep open dialogue and discussions with your physicians about how she is feeling with the medication combination and doses. It’s a tough balance to find, and compromising quality of life (due to feeling so unwell) often isn’t helpful in the long run. I hope this new combination of medications can help her following her recovery from the exacerbation. I also can’t wait for a research break-through for IPF – what a day that will be to celebrate!

Keep in touch, and do let us know how your wife does as she continues on her road to recovery. Thinking of all of you during this tough time.

Charlene.

[Charlene Marshall]

Hi Ross,

Thank you so much for chiming in to this topic thread and sharing a bit of your story with us. That said, so sorry to hear of your Mom’s passing from this cruel disease, and then your own diagnosis. I can imagine that must have been very frightening! It is so easy for us to dismiss the symptoms of IPF and attribute them to more common issues like the common cold or allergies. How are you tolerating the Ofev so far? It took a bit of adjusting for me but I am really glad I can manage/tolerate this anti-fibrotic medication. I hope you can as well. Sorry to hear of your appointment getting bumped – this virus is just wrecking havoc all over the world, but it is so important we stay safe. Kudos to you for remaining active – this will really benefit you, as difficult as it is. This is something I am working hard at as well.

 

Thanks for getting in touch Ross, and be well!
Charlene.