Hi Charlene. I’m on Cellcept but my diagnosis isn’t HP. It’s PF caused by an autoimmune disorder — dermatomyositis. I started the Cellcept (I’m now at 3,000 mg) and Prednisone last fall and saw some improvement in oxygen levels and breathing tests after about four months. In the past week, however, I noticed a drop in my O2 levels. I’ve… Read more

Good luck, Robert. Sounds like you have a great support system there and MGH is first rate. (My pulmonologist came from there last year, and I think the world of him.) Keep us posted.

Thanks Mark. I got a friend request from Johnson too.

Charlene, I’m sorry to hear you’re still feeling sick. Hope you’re on the mend soon.

I think it takes at least two months for Ofev to start working.

I have an autoimmune-related pulmonary fibrosis (dermatomyositis) and am currently on Cellcept and Prednisone.  I saw my pulmonologist yesterday and he said he thinks within the next six months Ofev will be approved for ALL forms of PF that are progressing. He thinks I should add the Ofev when it’s available to me. I was on Ofev for five… Read more

Thanks. I was on Ofev for a month before my diagnosis changed to autoimmune. I didn’t have any serious side effects, although my appetite and sense of taste seemed off. I think if I’m offered both I’ll do it.

Now that Ofev has received approval for several forms of autoimmune-related pulmonary fibrosis, many patients are starting to take it in addition to the mycophenolate (Cellcept) they had already been taking. My pulmonologist thinks Ofev will be available to me relatively soon. I’m a little concerned about taking two such strong drugs, but… Read more

@caneelbay1

Your doctor didn’t suggest Cellcept? It’s pretty effective in treating autoimmune-related pulmonary fibrosis.

They believe it’s dermatomyositis and Raynaud’s.

Last winter my hands, especially around the thumb and index finger, got very red and cracked. It was quite painful. I thought it was the dry winter air and tried treating it with hand creams and mositurizers. A biopsy showed it positive for connective tissue and it’s what they call “mechanic’s hands.” Some 70 percent of people with this rash… Read more

I do worry about that and wash my hands a lot. I also carry wipes around with me to clean surfaces. And I don’t greet people with hugs or hand shakes anymore. I stay away from hospitals and schools. I’m up to date with all my vaccines. But my doctor tells me not to let it make me crazy.

I’m also 72 and was diagnosed last spring with pulmonary fibrosis. At first they called it IPD (idiopathic pulmonary fibrosis) but later determined it was caused by autoimmune disorders. I started at 1,000 mg cellcept a day and am now at 3,000 mg. I’ve been on it for almost three months, which is how long it takes to start working. I’ve also… Read more

That IS exciting. Thanks for sharing that.

Also, I live in RI too, and go to a support group the the third Thursday of the month in East Providence. Let me know if you’re interested in that. There are people there who are also pursuing transplants.

Hi Pamela. I assume you’ve looked into Brigham and Women’s in Boston. I’ll be 72 next month and am considering starting the process at either B&W or Mass General. Did they put you through a lot of tests before telling you you weren’t eligible? That’s what I’m dreading. Can I ask what they decided made you ineligible? Only if you’re… Read more

It looks gorgeous. Have a wonderful trip.

Have a wonderful trip, Charlene. I look forward to hearing how you handle any challenges you encounter. I haven’t traveled since getting my diagnosis and supplementary oxygen, so I’m particularly interested in how others do it.

This is potentially huge — and good — news for those of us who don’t have IPF. This approval is for those who have Scleroderma, but according to my nurse practitioner other clinical trials are showing equally encouraging results for pulmonary fibrosis caused by other autoimmune disorders. She expects FDA approval will get pushed… Read more

I share your ambivalence about transplantation at my age. I’m going to see how I am after the holidays and make a decision then whether to pursue that option. I’m not sure I’d even be eligible. Right now I’m hoping the drugs I’m on will stabilize this disease. Mine is autoimmune-related and my doctor says sometimes the CellCept is able to put… Read more

Thanks for sharing that, Annette. The unpredictability of this disease scares me to death too. Did you ever look into transplantation? Can you tell me a little more about your current condition. Are you on a lot of oxygen? Are you able to care for yourself and do some socializing? I’m curious why your doctor thought you were ready for… Read more

It’s possible you might feel better after starting the immune suppressive drugs if you have inflammation. I just started prednisone and CellCept two weeks ago and my pulmonologist is hopeful that some of what he sees in my lungs is in fact inflammation, which can be eradicated. The scarring, of course, is irreversible, but hopefully the drugs… Read more

Mark, thanks so much for letting us know about this webinar series. I just listened to the June “Ask a Doc.” It’s excellent.

@peter

I’m surprised your doctor waited so long to put you on oxygen. It sounds like you might have needed it earlier. I was put on oxygen (2L) for exertion and sleep right after the diagnosis in May. I can definitely feel the difference. Even walking around a supermarket makes me dizzy and tired if I don’t use it.

I’m on 1000 mg 2x  a day of Cellcept and 40 mg of Prednisone. One or both of them are giving me terrible insomnia. I do feel tired during the day.

I find I freak out a little when I have bad days. I’m scared to death the disease is progressing. I’m still relatively new to this (I was just diagnosed in May) so I’m not quite sure how I’m supposed to feel. My doctor just doubled my Cellcept on Friday so maybe the fact that I’m not feeling great today is due to that. I don’t know how to… Read more

What appealed to me with the large Simply Go (not the mini) is that it has continuous flow, which I need for sleep. It would make it so much easier to travel without arranging for a concentrator. And sometimes I just want to go for an overnight trip.

It won’t be a magic bullet, but he seemed hopeful that I’d see some improvement. I definitely have some scarring that can’t be reversed, but there were areas on the CT scan where he said it was hard to determine whether it was inflammation or scarring. Given the autoimmune issue, he’s now thinking some of it is inflammation and that the drugs… Read more

Has it halted the progression of your PF?

I consider it a good day when I go for more than an hour without thinking about this disease. Usually there are physical and mental reminders that make forgetting about it impossible. On a good day my chest doesn’t feel tight or heavy, breathing isn’t a struggle, and I have some energy. I get errands done, finish some work, go to pulmonary… Read more

I was first diagnosed with interstitial lung disease in May 2019. Because my blood tests were inconclusive, my pulmonologist decided to call it IPF rather than an autoimmune issue. I went on Ofev. When I had a flare-up of a skin problem on my hands, and the rheumatologist saw it, he ordered a skin biopsy, which proved positive for… Read more

Hi Charlene. Thanks for responding. Yes, the smog could be an issue, but on those days I’d just stay in my air-conditioned apartment. And probably during the hottest part of the day in the summer. But I’ve stayed with my daughter when the temps were in the 90s and it didn’t feel hot to me at all. So different without the humidity.

Yes, that… Read more

A little off topic, but the nurse coordinator/practitioner in your pulmonologist’s office will become your best friend. Mine is very accessible and knowledgeable. She is almost always able to answer my questions, and she’s the one who oversees my oxygen needs.

I can’t get over how the humidity affects me now. We just had three days in a row with high humidity and I felt so lousy I was afraid I was in some kind of downward spiral — coughing, short of breath, fatigue, etc. Last night the humidity disappeared and I woke up feeling 100 percent better. It was so dramatic that I’m now thinking seriously… Read more

The 3-5 year prognosis was last updated in 2014, the same year Ofev and Esbriet were approved. Those two drugs are extending life expectancy.

That’s exciting research. Thanks for sharing it, Mike.

Jill, I’m wondering if the supplemental oxygen might resolve some of your issues, like the headaches. Be sure to get an oxymeter and check your oxygen throughout the day. I was prescribed it for exertion, which I initially thought would be a long walk or yard work, but I’m finding I need it even when puttering around the house, and certainly… Read more

Linda, there are some clinical trials that are ending now combining Ofev and Cellcept for PF that’s autoimmune related. The results have been very encouraging. I’m on Ofev now because my official diagnosis is IPF, but autoimmune issues haven’t been ruled out. The doctors on my team think this combination of drugs could be very effective for me.… Read more

Yes, the shrunken world you describe makes a lot of sense. That’s how I feel too. Everything I used to take for granted now seems harder — or even out of reach. You’d think I be reading more than ever, but like you I’m having trouble concentrating.

I’d forgotten you started a similar column a few months ago. I just went back to it and many of the comments resonated with me. Yes, I did (and still do) have fatigue, but again I chalked that up to my age. Fatigue would be far more noticeable at 28. Fortunately I’m virtually retired now so I can rest for a few hours in the afternoon.

Have… Read more

Some of my symptoms existed before the Ofev so I’m not sure I can blame all of them on the drug. But it is comforting to know others experience them as well. I’m so hoping that I can remain stable for a number of years that I freak out when I start feeling anything that suggests my disease is escalating.

I’ve been told the three things that initially bring PF/IPF patients to a doctor are a cough, shortness of breath, and “I just don’t feel right.”

I was diagnosed two months ago and was put on oxygen (2L) for exertion and sleep. I started Ofev one month ago. I don’t feel awful but I don’t feel “right.” Is this my new normal? I’m always aware of… Read more

I started two weeks ago and side effects have been minimal. A little nauseous for a few days but no vomiting or diarrhea.

My doctor said the efficacy rates of Ofev and Esbriet are the same. It was my choice so I went with Ofev because it doesn’t have the sun exposure issues and it’s just two pills a day.

My team of doctors includes… Read more

Btw, do you clean your cannula? When I’m out and about it sometimes touches surfaces that might be a little iffy. I’d like to use wipes on them but don’t the wipes have alcohol in them? Wouldn’t that pose a danger?

My provider told me to replace mine every two weeks. They supply me with enough to cover that frequency. I suspect that every two weeks is all Medicare will pay for.

They’ve started a free harmonica class where I go for pulmonary rehab. Let me know how you like it.

I started noticing a change in my taste buds before I started taking the Ofev last week, but now it seems worse. I was always an enthusiastic and adventurous eater, and now the only thing that appeals to me are chips and ice cream. I’m forcing myself to eat healthier food, but am not enjoying it.