[Sue Butler]

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.