May 9, 2022 at 10:43 am #31995
Hello Everyone! I am new to the forum and I thank you for the great information I’ve received from everyone’s experiences. I am glad to have found you guys!
I was diagnosed with Scleroderma – ILD August 2021, It’s in its early stages. So far my scarring on the CT has not progressed and my spirometry has had only a slight decline. I am taking Cellcept, Montelukast, Wixela. I just asked my doctor about Ofev. My oximeter is usually around 97%. I am 47 y/o.
I have a few questions surrounding the sensation of dyspnea and what you may do to help with these feelings.
Does anyone have a lot of anxiety surrounding the fact that you can feel your breathing slowly getting more difficult with every passing day?
How do you deal with that daily thought?
Do you hear rales when you breathe in, does your upper chest and upper back hurt when you breathe?
Is it hard for you to focus on working? With the bleak prognosis I’ve read about online, I sit at work every day and think “what the heck am I doing here”… I should be at the Amalfi Coast or sipping a cocktail in Bora Bora.. 🙂
Do you feel if you focus on your lack of ability to breath well, it actually does get more difficult?
Does anyone take anxiety meds for their lack of ability to breathe? Is that uncommon?
When have or when is it time to request oxygen from the doctor? Is there a standard or marker on one of the test you’ve taken?
I used to run 8 miles a day, 6 days a week, now I feel like I am half alive most days because of this constant inability to catch a full breath, yet I am walking/running 3-5 miles a day. I know I am not going to die tomorrow, so I need to figure out how to handle dyspnea.
Is anyone else afraid of suffocating to death? How do you handle this thought?
I appreciate your feedback, thanks in advance for your help!
- This topic was modified 1 month, 2 weeks ago by Christie Patient. Reason: formatting visible
May 9, 2022 at 7:12 pm #32002TeresaParticipant
<p style=”text-align: center;”>I have Interstitial Fibrosis with Ground glass opacities. I get short of breath very easy. I am only on Prednisone. Are there any exercises or any thing I can do to help my lungs. I also would like to know how to deal with dyspnea.</p>
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May 12, 2022 at 3:05 pm #32041
I have been diagnosed with something called PPFE in 2019. Pleuroparenchymal fibroelastosis (PPFE) is <b>a rare lung disease, usually associated with scarring (fibrosis) of the lungs</b>. It tends to affect the upper lobes of the lungs and cause scarring to the area beneath the lung lining (the pleura) and the lung itself. This usually mimics IPF but it different as to our scarring and may be a little slower in progression. My first Pulmonologist who I love in the one who named this disease and after many tests he decided I had PPFE caused by Systemic Scleroderma. He put me on Cellcept and that was a life changer for me. I immediately began breathing so much better with all kinds of my GI symptoms and throat problems getting better and more manageable.
I am also on Ofev along with 2000mg of Cellcept a day. I saw a Rheumatologist and he disagreed with Dr. Frankel and changed my DX to IPAF. IPAF is basically Interstitial lung disease with auto immune features which explains alot of the weird symptoms I get.
I wanted to attempt to answer some of your questions.
Yes I do feel my dyspnea getting worse on some days and just when I despair that I’m declining, the next day or so gets better and is always associated with how much I’ve been doing and usually its way too much ha! The hardest thing for me and what stood out with your post is that you said you used to run 8 miles a day!! Wow! I also used to run (never that much) but on a regular basis and still exercise now. I am reduced to a run/walk now 4-5 miles. That’s where I get down ,knowing I can’t speed up and it won’t get better. I did a 5K Saturday and finished in 45 min. That would not have been acceptable to me in the past at all! But I heard this the other day and it’s what I refer to all the time now and read it everyday I can.
“Do what you can, with what you’ve got, right where you are” At least we are still trying and doing our best. I’m thankful to be able to still exercise and try hard not to compare myself to my husband who did 15 miles the other day, eye roll.
As to when to request oxygen: That depends on your 6 minute walk test and what your O2 saturation is, your MD will help you decide. I honestly feel the best thing we can do for dyspnea is to exercise and not give up doing what we can. I’m in healthcare and 58 yo. I have slowed down at work but my coworkers who understand my disease are super helpful and almost baby me too much. I’m just thankful to be around people who “get it”. Sometimes I’m more short of breath than my patients, then don’t want to hear them whine. It’s the truth.
My sats are good for now but they don’t always reflect my shortness of breath as my scarring is so high up. This is a scary disease but we can’t get inside our own head. It’s a constant battle with fatigue and thoughts that you don’t have long in this world but I have a strong faith that gets me through each and every day and know that the best thing will happen in the end and I will do my best to face it without too much anxiety. It’s ok to get meds for that though I totally support it! I will certainly consider it if my breathing is making me too anxious. I do fear what the end will be like and don’t want to be dependent on anybody but we have to live one day and get through it and find peace in knowing God has got this!
Hope this wasn’t too long just know you’re not alone. One more thing, my Dad is 92 and has interstitial lung disease not sure if its IPF. We have hospice coming to see him once a week. They are wonderful and will see to it he does not suffocate to death. He is currently on 3liters Oxygen. The rest of my family were not on board because of the stigma associated with them but it has been the best thing for everybody.
The end will take care of itself. You are still a vibrant person who has much to offer! Take care!
May 21, 2022 at 12:37 am #32154
THANK YOU for your response and insight, you’ve helped answer many questions where I wasn’t getting straight answers from doctors.. of course they probably don’t have lung fibrosis, so it is wonderful to be able to get an answer from someone who can relate.
It’s interesting that you also have lung fibrosis caused by Scleroderma. I have known I’ve had Scleroderma (which presents itself in the cuticles of my fingers as thickened skin) for about 14 years. I noticed the Scleroderma in my fingers when I developed Raynaud’s. My doctor, who is one of the best Scleroderma focused Rhuematolgist in the US says I have most likely had the fibrosis in my lungs just as long as I’ve had the Scleroderma in my fingers, so about 14 years, and that the lung fibrosis progressed slowly until last year when I hit menopause.
I am also on Cellcept and began Ofev a few weeks back. So far I am tolerating them well, some fatigue. My stomach and kidneys hurt from time to time.
You said your IPAF gives you weird symptoms, what exactly are they?
It’s interesting you said you also feel some days the dyspnea is getting worse and the next day or so it gets better based on your activity level. Thank you for that insight, it was an “aha” moment for me. I admittedly burn the candle at both ends and realize on days when I get good rest and am not stressed, I don’t feel as breathless.
I USED to run 8 miles a day, then 6, then 5… now it’s just run-walking 3-5 a day. This is what took me in to see a Pulmonologist, the fact that could no longer run long distances. They first diagnosed me with asthma and then found the fibrosis. I use Albuterol from time to time prior to going for a run, seems to help.
You said you felt down about running “knowing I can’t speed up and it won’t get better”. I am not convinced of this yet, here is why- I have read of an athlete who runs marathons with COPD, and of another ex-Olympian who has PF and his PFT test actually improved over the years, so I’ve decided to do as much research as possible on people with lung fibrosis who are competitive athletes. I would like to learn from them on how to manage the ILD through exercise. I’ve also enrolled in my local Breathing Center, and joined the Better Breathers club.
I agree that it has been a constant battle with fatigue and thoughts that you don’t have long in this world… but then I get in the flow of my day to day and forget I am sick at all. I agree, the disease has definitely made me lean more on my faith in God, and made me more grateful every day.
Sorry to hear your father is in hospice, thank you for letting me know that they will see to it he does not suffocate to death. I never thought about hospice as being an end of life care taker for me (I’m in my late 40’s so thinking of death so soon is new to me), but yes, I would think they would help lessen that pain, so no need for me to worry about it.
I agree the end will take care of itself! Thank you for your wisdom and kind words, God bless Lystra.
May 21, 2022 at 7:12 am #32156Christie PatientModerator
Hi Pamela, I just wanted to interject and point you toward a columnist on my team who writes for Sarcoidosis News. Sarc is not PF, but does have similar features in that involves inflammation and change in lung tissue (granulomas in that case). Calvin has pulmonary sarcoidosis and is a runner. He is training for a marathon right now 🙂 His training was derailed by contracting COVID in January, but he recently ran 9 miles of the Brooklyn Marathon as part of his training. Exercise, as much of it as we can tolerate, is a great help to maintain pulmonary health. Anyway, I like his wisdom about living with chronic illness alongside his athletic pursuits. You might enjoy as well.
June 3, 2022 at 8:27 am #32292Paul CadenParticipant
Hi Pamela I am in the same position as you but my strong belief in evolution and the good of other people as proven in sociobiology helps me face life with this dreaded disease and not have to believe in one of the 2,800 man-made gods of History
May 21, 2022 at 1:59 pm #32160
Thanks so much for your reply! Very much interested in your research for people who have PF and run. You sound like me we are willing to do whatever we can to keep running and stay active. You asked about the odd symptoms that I attribute to IPAF. I find that after I eat or get short of breath I can’t talk. My vocal cords lock up somehow and I can’t get my words out. Doesn’t happen all the time but Cellcept improved this greatly. Also I feel it contributes to Reflux. I will skip Ofev for lots of reasons because it plays havoc with nausea and I have to take a Zofran every day but I won’t skip a dose of Cellcept and I’m counting on going up on the dose if symptoms get worse.
Maybe I can find a Better Breathers club here and meet other people like me. Sorry that you got this so young. Please keep in touch so we can encourage each other.
June 1, 2022 at 11:42 pm #32279
Thank you Christie for the article, I appreciate it very much!
<div>You said that the dyspnea gets worse somedays when you burn the candle at both ends, then you bounce back. Has your Spirometry/PFT tests had a decline, or has your scarring on your CT scan progressed at all since your initial diagnosis?</div>
<div>Do you feel the need for oxygen sometimes? Do you take a daily or weekly oximeter reading at home? Mine ranges from 96- 99%, depends on the day.</div>
<div>Lately I’ve been feeling the base of my lungs ache a lot, and I hear rales more often on inhalation. I started Ofev about a month ago, have also felt my kidney and my stomach start to hurt a bit. I read these are a possible side effect of Ofev.</div>
<div>Hopefully you’ll find a Better Breathers club in your area, they are sponsored by the American Lung Association.</div>
<div>Thank you again for insight surrounding dyspnea, I will keep you posted surrounded my research on working out/running with an ILD.</div>
June 2, 2022 at 4:49 pm #32288
My next CT and 6 min. walk test will be in Oct. of this year. My PFT’s have stayed pretty steady and I’ve had a really good month of May! For that I am forever grateful. My sats stay in 98-99 range. Yes Ofev will cause that awful stomach cramping . I experience a moderate amount of rib cage pain myself. That may be due to PPFE , we have a significantly smaller upper body mass.
I still need to work on finding a Better Breathers Club but in the meantime this little thing on Amazon called The Piper Breathing Exercise Device has really helped me to work out my lungs and a great reminder to take deep breaths. I tried to link it but couldn’t.
This is a tough disease to live with everything single thing we do depends on how easy we are breathing for that day! Pushing myself has made me better in other areas and a more sympathetic nurse too. We just have to remind ourselves that there will be good and bad days. I’ve told myself if I ever have 3 in a row I will be concerned but as long as rest fixes it then I’m ok. It’s just that we have to know our limits, and I’m working on that one!
June 8, 2022 at 11:06 am #32321Karen MartinParticipant
The most help I get for dyspnea is through pursed lip breathing and visualization. You need to breath from your belly, not the upper lungs as we do when gasping for breath. Breathe in slowly through your nose as your belly expands and out through pursed lips. It is best to do this when sitting with a very upright posture. As you calm down and your breathing becomes better, it helps to imagine a scene that calms you. Maybe a beach or sitting with a close friend watching a sunset. Whatever it takes to calm you. As for thoughts of how long do we have to live and how much worse will it get? Why go there? Do you wonder what your odds are of dying in a car wreck every time you drive? I’d bet not. My point here is that stressing yourself out is not helping. As someone else pointed out, we do what we can with what we have where we are. I wish you the best as you find your way forward.
June 9, 2022 at 5:23 pm #32328Jerry GenesioParticipant
My oximeter is usually at 91-92 but I don’t feel a lot of anxiety, probably because I’m 83 and have several other serious problems including heart disease and severe spinal stenosis. My VA pulmonologist said oxygen isn’t an option until my lung capacity drops to 88%. Right now it’s at 90%.
I do hear chest rales but not all the time. And of course I’m retired but still do a lot of research and writing so I’m at the computer several hours each day. I have to use a cane when I walk or a Zinger wheelchair if I have to go more than 20 or 30 feet due to the spinal stenosis so the Amalfi Coast and Bora Bora aren’t very tempting. I have traveled extensively in my lifetime, however, including Asia (Okinawa & Japan when I was a Marine), Europe (England, East & West Germany in the 1980s, Italy, France, Croatia, Bosnia, and Russia), Central America (Nicaragua, Honduras, and Guatemala), and Canada.
When I focus on the difficulty I have breathing, it does seem to get worse but it’s probably my imagination. I’m not taking anxiety pills regularly but when I do it’s to keep my BP down to protect my heart. I was told that I should consider taking Esbriet but I declined due to my age and concern about incompatibilities with the other meds I take. At the moment, though physically limited, I have a rather good quality of life and I don’t want to spend months dealing with Esbriet’s many side effects. Ofev is not an option for me because I take blood thinners.
I would much rather drop in cardiac arrest than suffocate when my number’s up but if it’s the latter I hope it’s in my sleep.
I try to do as much as I can but I’m very limited now. Most importantly, I keep my mind occupied with historical research and writing (I’m a historian), reading, and world events.
I wish you well. Please don’t let your dyspnea prevent you from enjoying life prematurely!
June 9, 2022 at 8:07 pm #32333Sue ButlerParticipant
Hi Lystra, I am also new to this forum. But I needed to respond because I also have PPFE, diagnosed in June, 2021. I am 77 years old, and have been incredibly healthy all my life, until now. My doctor tells me there are 200 people in the world, so it is extremely rare. This is the first time that I have encountered someone on these forums who also has PPFE. Most people have never heard of it, and assume you have IPF. So I would like to share my experience with you.
I began on 2000 mg. Of CellCept in July, 2021. The hope was, in consultation with my rheumatologist, that it would relieve the inflammation that leads to scarring. My most recent PFT’s in April indicate decline of over 10% in 10 months, and the the fibrosis is advancing in my upper lungs and also spreading to other areas where there previously was no scarring, according to my most recent HRCT. The doctor says the CellCept is clearly “not holding the fort.” My disease has declined more quickly that he anticipated when he suggested my prognosis was better with PPFE than IPF. I never felt that the CellCept helped my SOB. I was just hoping that internally it was slowing down the inflammation.
My official diagnosis is IPAF with the PPFE being a sub variant. The rheumatologist suspects Lupus is the issue, but calls it undifferentiated. I have been seeing her for 3 years when lupus was suspected, but she tells me it can develop over a period of 6 years. I believe the PPFE is merely a manifestation of the autoimmune issue. Over the course of 15 years or more, I have had other issues – psoriasis, eczema, hair loss, all scarring issues, but nothing was diagnosed until I began experiencing shortness of breath and they discovered the scarring in my lungs. But the disease has been evolving over a very long period of time.
My doctor is now suggesting adding the anti fibrotic drugs because the CellCept has been ineffective. We have decided to wait until August to see another set of PFT’s for more decline. Like others on their posts, I am reluctant to go down that road, if for the rest of my life, I have no quality of life. They will only buy me more time, not cure my condition.
My oxygen levels are still good. As you described, the scarring is mostly in the upper lung. I have INCREASED SOB and increased heart rate on exertion, but oxygen remains rather stable and returns to or normal quickly after exercise, never dropping below 90%. The key to dealing with the dyspnea is to pace yourself. I used to power through my life, and was extremely efficient. I have learned over the last year that I must take my time and rest for a while when I get exhausted. And I have to forgive myself when I am not as productive as I once was.
So to exercise, my research tells me, as others have expressed, that DAILY planned exercise is the key to being able to keep going. I did Pulmonary Rehab in the Fall, and then began working out with a trainer several days per week,. On the off days, I walk, doing bands and weights and ride a bike, or treadmill for cardio. The key is to do SOMETHING every day. If you become deconditioned, the disease will only get worse. Much of my information has been gleaned from the Pulmonary Wellness Fiundation. They have a comprehensive website and do regular podcasts with experts and Q&A sessions. The exercise guru is Dr. Noah Greenspan. He has written two excellent books on Pulmonary Wellness. I highly recommend them. There us also a group calked PF Warriors who have an active Facebook community and also do informative podcasts with medical experts. I have gleaned a lot of information from these sources. Many of you have probably already discovered them.
Lystra, you mentioned some issues with your vocal cords and I must shAre with you that I had been experiencing ever worsening of dysphonia and hoarseness in my voice, which got very bad this winter. I had my throat scoped and discovered that my left vocal cord is paralyzed. My doctor was fascinated and found one other case in Japan in 2019 where he had PPFE and vocal cord paralysis. You may want to check it out.I have had injections in my vocal cord now, which has helped the dysphonia temporarily. A long term solution maybe a surgical stent.
Another issue I have developed simultaneously is dysmotility in the upper 1/3 of my esophagus. I am still undergoing tests and don’t yet know the treatment, but it is all fascinating to all my doctors that this is all happening in the area of the scarring in my lungs, I am convinced more than ever that I am dealing with a systemic autoimmune disorder, rather than just a lung disease.
But my mindset is good. As some others have stated, I have a very strong faith and am placing this in God’s hands. I am trying to live my best day every day taking cre of myself and spending quality time with people I love and care about. I think I have done a pretty good job of putting my affairs in order in the last year. And I believe something good will come from all of this. When I think of how I may die, I believe that anything I might imagine is likely not what WILL happen. So it is wasted energy. I will accept each day as it all unfolds, and got to bed at night trying to find AT LEAST ONE THING to be grateful for.
Thank you all for sharing on the forum. It has been extremely helpful, and I will look for more helpful information and encouragement going forward.
June 10, 2022 at 4:55 pm #32335
Thank you so very much for reaching out. We may be rare but we’ve found each other! I have the same diagnosis except reversed PPFE with IPAF. There is a chance I have Systemic Scleroderma because of an unusually high blood test for Scleroderma. It is what I believe causes the throat problems and GI issues that I have. For example, today on break I ate some brownies that my friend made and I couldn’t talk clearly afterwards, it was frustrating and I do believe food triggers some of the problems so hearing your experience has helped me. I guess it really doesn’t matter what our exact diagnosis is because the treatment is essentially the same. Cellcept and Ofev. Cellcept definitely helped with my dyspnea and GI issues. If I max out on it then there are a few more autoimmune drugs to try before considering transplant. I love what my MD said to me the first time I saw her for PPFE, she said the clock starts when you get transplanted so keep the lungs you have now for as long as you can. Apparently transplant may be an option for me but I’m not looking to it as a cure.
When I was first diagnosed in 2019 my local pulmonologist was very adamant that I also start Ofev. She felt that the sooner we slow the progression the better instead of waiting until symptoms worsened. It will slow progression by 50% not stop it and as you know many side effects. So this is what I do, I take it as many days as I can and skip it on vacation, and my off days. I workout 4 times a week (with other stretching stuff on off days) and I hate feeling tired for my cardio so I skip it those days. Seems I don’t mind being tired at work. Also I take a Zofran every night to help with the nausea. I truly believe quality of life comes first over any drug and when I first started Ofev my weight dropped and I looked and felt bad all the time so I just played around with the dose and timing. I seldom take it on vacation and tomorrow I have a kayaking trip planned and will definitely skip it then.
You have a great attitude and are very active. I admire that you have done your research and are at peace. You know your limits and your body well. I see that you have other issues to deal with also and that can be a pile on that seems too much!!
I too have been on the Pulmonary Wellness website and love Noah Greenspan! The benefits of exercise are too many to count and laying around always makes me feel worse.
The main thing that is getting me through this is knowing God is in control. I have seen his hand over and over in directing my care and providing help. I have grown as a person in so many ways because of this disease and have learned things about myself that have made me sit up and take notice of every single day. I’ve learned how to help other friends who are suffering and now have a small idea of what to say to other people with a terminal or incurable diagnosis. This is a hard fight, not easy in any way but I’m up for it because giving up isn’t an option. When I don’t feel good I rest and do what I can. I believe the things we say to ourselves is so important so I try not to beat myself up too much for the running and hiking up hills that I can’t do anymore.
If you’re interested there is a PPFE facebook page that is helpful. There is so little known about the progression of this disease it is very individual as to how it goes. I do have strange symptoms and random chest pain also. I was told I have moderate scarring and my sats are stable for now. Will have the CT scan in October and six minute walk test. If I can help you in anyway let me know. I love how you have taken control of your life and realize that imagining the end is scary and not helpful. I do think about dying though and it takes the fear away because I realize my family WILL and CAN go on without me and in the end its God who decides what is best for me and I’m on HIS plan. So I live in peace and know you do too. There will come a time when we will need oxygen to help us live a more independent life and ease our breathing. Our sats may not drop like the normal IPF patient because of our scarring being in the apical region. We may delay getting qualified for oxygen because we may not qualify even though we are short of breath. That is something we may have to deal with when the time comes.
If you can think of anything else feel free to private message me and we can discuss further there. In the meantime so good to find you.
June 11, 2022 at 8:59 pm #32336Bob ChiuParticipant
I was short of breath whenever I climb stairs or steep inclines starting in 2003. It would took me 6 hours to climb a mountain while my buddies too 2 or 3. My oxygen was 97. However, I continued life as normal and continued to play golf actively. It was not until 16 years later, in 2019 that I need to use supplemental oxygen, and began to think about how to deal with the disease (NSIP.)
From what I read it sounds like you are like me 16 years ago. So don’t think too much about it and stress yourself for something that may not affect your normal life for many years. See your pulmonologist regularly, avoid catching a cold/flu/pneumonia/CoVID (for they will exacerbate your condition.) This you can actively avoid.
Meanwhile medical sciences continue to advance and in 5, 10, 16 years who knows what new medications would be available? I am told Ofev is shown to delay lung fibrosis by 50% for many people.
I really don’t want you to let anxiety to affect your normal life . It seems to me you are in an early stage.
June 23, 2022 at 9:00 pm #32418
Thank you for your kind words and your positive outlook!
Even after a year it’s been trying to NOT think about the disease, but I am slooooowly learning how to focus on getting back to my life again.
I found an interesting article about the mind-body connection that has helped me regarding dyspnea, will post it for everyone here:
Also, I’ve started working with a personal trainer, an Accupuncturist/herbalist, changed ALOT of my eating habits, and ran my personal best in over a year recently. Let’s see how things go!
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