[Lystra]

Hey Pamela,

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!

[Lystra]

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.

[Lystra]

Hello Pamela,

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!

[Lystra]

Lee, this doesn’t have anything to do with Laser Therapy…but I just bought your book! It is very inspiring and you are a diehard exercise dude!! You have been inspiring me so much. I don’t have IPF but it’s slower cousin something called PPFE, Hoping to put some of your great suggestions to use. I have exercised all my life, it’s hard to get slower but not on oxygen yet. I will ask my MD about laser therapy and some of your other suggestions. Haven’t finished reading the book yet but it’s alot of information that is. valuable so taking it slow. Thank you for getting it out there!

[Lystra]

Elaine,

I struggle with GI issues also.  Currently just taking one Prilosec 40mg a day.  Do you feel the 2xday is helping?  I just stopped Ofev and started Esbriet.  I’m also on Cellcept 2000mg a day.   Sorry for the appetite issues you’re having.

Lystra

 

[Lystra]

Sue,

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.