Speed of Progression of Systemic Sclerosis

I was wondering if anyone could comment on the speed of the progression of their systemic sclerosis.... particularly if you have lung involvement.
I ask because I was just diagnosed in July of this year and the progresion has been much faster than anything I can find on any website.
When I first saw a pulmonologist (back on May 3, 2012), I just assumed that I had exercised induced asthma as my exercise tolerance went down very fast but I was fine at rest. As a matter of fact, in january (5 months before I saw the pulmonologist) I was running 5 miles a day and biking about 30 miles a day, and now am wearing oxygen 24/7 and still having shortness of breath.
My initial DLCO was 35% and is now down to 20% and when I did my first 6 minute walk test, I was a little short of breath but did okay, and when I did it the other day, the distance was less, I was on 6L of oxygen and my oxygen level still dropped and stayed at 80%.
I did just complete a lung transplant evaluation at Columbia in NYC and they said that I meet all of the requirements with the exception of my required assistance with activities of daily living, as I am still working full time.
So, any information you can provide would be very helpful so I can put this in perspective.

Just for completions sake, I am currently taking Cellcept 500mg (3 Pills), twice a day, prednisone 10mg/day, and Bactrim DS three times per week.

Thanks again andI look forward to reading the responses!!!
Sam.

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Hi Sam,

While I don't have experience with it myself I have read some other accounts (on this site, I think) from people with rapidly progressing lung disease. It sounds like you're getting good medical care if you are on Cellcept and have been evaluated for a lung transplant. I read one of the personal accounts on the Scleroderma Foundation website and she described her experience with a double lung transplant -- it sounds like a very difficult process, but after her recovery she has an incredible improvement in her symptom and her disease has not progressed. If you have not read it yet, go to the Scleroderma Foundation website and look through their stories. There are also a few people on this board who have been evaluated for lung transplants so searching through the archives might bring up some interesting discussions and allow to get in contact with people who've been in a similar position.

Have you been evaluated for a stem cell transplant? I'm not sure what their cut-offs are in terms of DLCO, but it's worth investigating if you have not already. The procedure is offered to those with rapidly progressive diffuse disease (onset within the previous 3 years, diffuse skin disease, and lung fibrosis).

Finally, do you have a scleroderma specialist? If not, I'm sure someone on here can suggest the best one in your area. Sometimes a scleroderma specialist will understand certain subtleties about treating the disease that a regular rheumatologist doesn't.

Good luck!

Zoe

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The best way to put this into perspective is to understand that scleroderma is a very individual disease, and everybody's progression is different. There really isn't a "typical" pattern. I think I've had some features of scleroderma for all of my 60 years, so mine is a very slow version. Yet some people develop it quite suddenly and progress quickly. Whatever your experience, though, you're sure to find at least at a few others who can relate.

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Is the reason you are taking Bactrim lowering your PFT scores and O2 sats?

Do you live alone and that is the problem they have with your ADL's.

I know the transplant procedure is overwhelming. Have they assigned you a Social Worker to assist with finding help with your ADL's (Acitvities of Daily Living). A social worker can relieve a lot of stress by advocating for you so that you can concentrate on breathing, if that is the only criteria you are lacking.

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Hi what kind of lung involvement do you have? Everyone's progression varies due to environment, lifestyle and disease. I can commit better if I knew your lung prognosis. Thanks

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Wow!!! Thanks for the responses. I will try to answer all of the questions.

To erayf: The reason I am on the Bactrim DS is because I am consistently in a high risk environment for aquiring funky infections (I work as, ironically enough, a Respiratory Therapist, primarily with pediatric and neonatal patients). Specificially, the reason for the Bactrim is that I am high risk for PCP pneumonia.
Yes, I do live alone, but with a strong friend based social support system as I have a very good friend and his family living accross the street along with a slew of other people, almost all of them nurses or respiratory therapists whom have committed to helping me with whatever I need, including bringing me to appointments or helping me run errands. But, the problem they tell me is that I do not have a family member living with me, and, to be honest, that is not logistncally possible for my family situation.... still trying to figure this one out. I did make an appointment with the social worker again to discuss this in more detail. I agree that, if the fact that my social support is not typical is my excluding factor, that would be a big dissapointment.

to miraclebaby1994: my lung involvement has been rapidly progressive and primarily of a restrictive nature, that is my total lung volumes have been rapidly decreasing and the diffusing capacity (DLCO) has been decreasing rapidly as well. I had a routine pre-surgical PFT back in 2010 and everything was completely normal, nothing even slightly abnormal. Then, my first PFT with the pulmonoologist when I had symptoms showed a DLCO of 35% of predicted and a total lung capacity (TLC) of 50% of predicted (May 2012) and has since decreased to a DLCO of 19% of predicted and a TLC of 40% of predicted on my most recent PFT (Nov 2012).
Symptom wise, back in January of this year, I was running 5 miles a day and biking about 30 miles a day, while now I am wearing 2L of oxygen at rest and about 4L with activity and there is still noted oxygen desaturations and shortness of breath with any sort of activity that either involves in incline or stairs or is prolonged more than a few minutes.

I hope this answers the questions. It is also quite a change for me to go from being the healthcare provider to being a patient, particularly when my issue is so close to my area of specialty, yet so unrelated as we do not see this in kids.

Anyway, thanks and, as always, I look forward to reading your responses.
Sam.

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I can understand having someone dependable there 24 hours a day in case of rejection or acute SOB. Hopefully some transplant patients will chip in with some info. That is a doable fix.

The progression is a head scratcher, but I am just a simple patient. I had low PFT's from the start, so when they hit the 30's they started the checklist. I stopped working and gave up stressing on things I could not control at the time and I stablized. But we are all so different. You seem to be in good hands and I pray for some positive changes for you.

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Do you work NOC's or 10 or 12 hours shifts? Try tweaking you enviroment. It's not like you are doing a mind numbing job. Dose calculations themselves can be stressful enough for a healthy person. And then you throw in a freaky storm. It might be worth it to mention these things to the social worker also.

Sometimes the big picture is bigger than we can see at the time (O.K. I am not Confucius). You are very bright and you are looking in the right direction.

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As to your query regarding rapid progression, yes, mine too has been very rapid. I have sy sc that started in summer of 2011 and I have hypertension, kidney damage, heart issues and I'm praying NOT to have pulmanary hypertension since my last chest xray showed the right side of my heart to be larger than the left. I see my heart dr and rum next month to see about this. I don't have lung involvement and my rum told me with my antibody I may not.......just everything else. When I ask Maureen Mayes if rapid progression meant a worse outcome overall she said, "no, it could actually go the other way." It's just a wait and see and take care of each issue as it comes up. Best wishes in all areas.

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Sounds familiar.....I was also working out and in pretty good shape when my pulmonary functions dropped dramatically. The local hospital didn't have a clue other than pulmonary fibrosis. Basically they were expecting me to die.

My wife insisted that the local hospital transfer me to the University of Iowa Hospital in Iowa City, IA. Boy am I glad she did! I was put on Cellcept and saw immediate improvement. My lung involvement was diagnosed as BOOP. Eventually my diagnosis moved to systemic sclerosis.

It took about six months for me to move off oxygen and return to working out. So far I have seen no additional lung involvement (knock on wood). I am still on Cellcept and have regular checks for liver and kidney functions. My life has returned to normal.

Stay as positive as you possibly can.....I wish you best in your fight!!

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Sounds familiar.....I was also working out and in pretty good shape when my pulmonary functions dropped dramatically. The local hospital didn't have a clue other than pulmonary fibrosis. Basically they were expecting me to die.

My wife insisted that the local hospital transfer me to the University of Iowa Hospital in Iowa City, IA. Boy am I glad she did! I was put on Cellcept and saw immediate improvement. My lung involvement was diagnosed as BOOP. Eventually my diagnosis moved to systemic sclerosis.

It took about six months for me to move off oxygen and return to working out. So far I have seen no additional lung involvement (knock on wood). I am still on Cellcept and have regular checks for liver and kidney functions. My life has returned to normal.

Stay as positive as you possibly can.....I wish you best in your fight!!

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Did they ever try Cytoxan infusions? That is typical for pulmonary fibrosis in its acute inflammatory phase.

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To Erayf: I work evenings actually.... Usually 3 to whenever I finish. It sounds like a WIERD schedule, but I have been in management for a little while now so I actually supervise 5 departments after their supervision leaves. The schedule works well for me as I am not sure I could do 12 hour shifts anymore and it still gives time for my plethora of Dr. Appts each week! Thanks for the encouragement though. We will have to see how it goes.

To FFS: I am sorry to hear about your rapid pace of progression. It is funny (not in the ha ha sense) to see how this progresses in different people. I say this because it appears that we have exactly the opposit organ systems involved. I have no cardiac or renal involvement as it is all limited to my pulmonary system and GI tract. No wonder it is so difficult to diagnose and treat if it can present in one of fifty different ways. Good luck in your upcoming appointments!!!

To: Thegoodfight: we had talked about switching to Cytoxan from cellcept but so far it has only been talk. From what I read it is only beneficial if their is an active severe inflammatory process and there is a question as to whether mine is currently active or has subsided but left severe fibrosis. I, personally, have trouble believing that this has went from nothing to fibrosis this quickly as this process normally takes a few years. But we will have to see. I am advocating that something in the care plan change as simply staying status quo is simply not working for me right now. It's possible it's just because of the sudden onset, but I refuse to accept that I'm 29 years old with these set of severe limitations.

Thanks for the insight everyone and any other thoughts would be greatly apprecieated!!!
Sam

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Try rewording your posts or maybe visit the pulmonary fibrosis foundation support group.

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I would push for answers with the Cytoxan infusions. When I was first diagnosed, I went from active to SOB and needing O2. They gave me 6 monthly infusions of Cytoxan and then started me on Cellcept. This has held my DLCO at the mid 50 range for 3 yrs. it would seem to me that if you are having those significant changes that quickly, something else needs to be done, NOW. Please get those answers......

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Thanks everyone for all of the information. After having another followup appointment with the pulmonologist and phone call with the rheumatologist, they have decided to start Cytoxan IV next week. I certinly hope that it will show to be more effective than the cellcept has been thus far in reversing at least some of this.

Thanks everyone!!!

Sam.

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I am happy and relieved to hear that. I will keep you in my prayers. God bless.

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Good luck Sam. I hope the cytoxin works for you.

Ed

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