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Pulmonary Fibrosis


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#1 Lauriejill

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Posted 16 October 2007 - 08:36 AM

Hello everyone,

ts been a long time since I have posted but I need some help. I just found out that my PFT showed an FRC of 60% which is down from 80% a year ago. My Doctors - Pulmonary and rheumatologist say that my interstitial lung disease is moving moderately fast and needs to be treated. Cyclophosphamide (and possible prednisone) has been suggested. Does anyone have any experience with this drug and has it slowed down progress, side effects etc. Thanks so much ... I am feeling quite stunned and scared about this next step.....thanks in advance.

Laurie jill



#2 TJ903

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Posted 16 October 2007 - 09:12 AM

Can't realy help you with the long-term stuff, I was on cyclophosphamide for 3months, 'cause I was trying to get into the SCOT trials. However, I did the cyclophosphamide and my only complaint was an upset tummy (taking it with food helped that), and hair loss (that's just from the disease, but came out faster). There are others who will give you more accurate info on their side-effects. I just hope that this works for you.

Sometimes we have to suffer the bark to lessen the bite. Doesn't make it any easier though. Prednisone is a different dog altogether for me. Haven't done that yet.

I can't remember who it was on this site, when I was diagnosed in Jan(very aggressive form) of this year and scared to death about my ILD, that we can survive on 10% lung capacity if we had to. So try not to stress too much you are in my thoughts and big hugs are coming your way!!



#3 peanut

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Posted 16 October 2007 - 10:21 AM

Hi laurie jill,
I too have interstitial lung disease caused by scleroderma. A year ago I did 6 months of IV cyclophosphamide, which is low dose chemo. I was lucky. My hair thinned a little and I had very little nausea but that is not always the case with everyone. After 6 months the progression seemed to slow. The goal is to use cyclophosphamide to stop or slow the progression, then to put you on something less toxic that will stabilize you. We tried Imuran after my 1st round of cyclophosphamide and it didn't go very well, so currently I am starting my second round of IV cyclophosphamide

For me the major side affect is chemo brain. I'm a total air head. I remember nothing. But no worries, its just temporary. It all comes back after the cyclophosphamide is over.

Not everyone handles cyclophosphamide well. It all depends on your body. cyclophosphamide is also not a very good long term option. Oh and if you're of childbearing age or might think about having kids - cyclophosphamide may make you sterile. Look into getting a Lupron Depot shot. I did this. It puts your ovaries to sleep/temporary menopause which protects them from the side affects of the cyclophosphamide. After there's a 90% chance they will come back to normal.

If you can handle it it's really not too bad. If you schedule your appointment on a Friday then you have Sat to rest and recoup in case you don't feel well.

Hearts,
peanut



You can deprive the body but the soul needs chocolate
my HMO makes me wear a helmet...

#4 Clementine

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Posted 16 October 2007 - 11:36 AM

Laurie Jill,

I am so sorry to hear about your lungs. I also have ILD. My TVC is 61% and my DLCO is 51%. Since beginning treatment for my lungs two years ago, I have really improved as far as feeling any symptoms. Technically, I have not improved but I feel better.

My question is was this last pft the only one you've had since the one a year ago? Are your CT scans showing progression? I would be wondering if the 20% change was truly a change, and not just a fluke, before I'd try something like cyclophosphamide. Also, are you going to a Scleroderma Clinic/Expert?

I have not tried cyclophosphamide. My Dr. and I decided to try Cellcept to see if that would work for me, before we tried the more toxic route, cyclophosphamide. Guess what....Cellcept worked for me!

Having ILD is pretty depressing but you have us to talk to now. Peanut rocks, although her memory has left us. smile.gif

xo,
Tangelo



#5 Lauriejill

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Posted 16 October 2007 - 03:47 PM

Thanks ladies....for replying to me. I am having another CT (last one about 7 months ago) sometime soon. The PFT was the fourth I've had in the last two years each one showing a little decline from the last.

My rheumatologist is pushing the cyclophosphamide, as he did a year ago when I refused to take it then.....all of my other MD's thought it was a bad idea ...but now the Pulmonary MD says it is time to start treatment as I am clearly getting worse. He called it a "moderate change".

My pulmonary MD told me about an experimental drug called pirfenidone that is in clinical trials as an anti-fibrotic and is doing very well for patients but not approved yet by the FDA. I am trying to find out if there are any trials for scleroderma patients....all I have found are for idiopathic lung fibrosis.

I will be sending my recent PFT and echocard. to Dr. Furst at UCLA as he is my scleroderma specialist/advisor.

But no one likes to hear the words "getting worse" ...."cyclophosphamide" ....so scary to me and it helps to hear from others who have advice and experience. Thanks for your replies. I will ask about cellcept too ......

To be honest, I just don't know how to feel about all of this ..... a bit shell-shocked, some denial....what is up ahead?? questions that just cannot be answered.......and sadness. Thanks
Lauriejill



#6 janey

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Posted 16 October 2007 - 04:26 PM

LaurieJill,

It's good that you have a doctor that discovered the downward trend and is ready to do something about it. I had 3 pulmonologist ignore it and finally got one that noticed it and couldn't believe it hadn't been picked up. I was started on oral cyclophosphamide which has a successful study for scleroderma ILD. I was going to be on it for a year, but lasted 4 weeks. I caught a cold then pneumonia so I stopped it. A few months later I was put on cellcept and have been on it for 6 months with no problems. My last pft showed a little improvement in DLCO so it looks likes it's working. You might want to read through some of the studies on our pulmomary fibrosis treatment page. It shows studies with both cyclophosphamide and cellcept.

It's always good to catch things like this early, so please consider it a good thing. A lot of times lung problems are caught late and there's not a lot that can be done. So yes Darlin', it is scary, but not as scary as doing nothing. So please let us know how you are doing what you decide.


Janey Willis
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#7 Vee

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Posted 17 October 2007 - 01:38 AM

LauriJill,

I also have ILD. My last pft was this month. My DLCOis only 37% and FVC is only 58%. And I feel terrific! It has taken me awhile to come to terms with having to do things slower and less aggressively, but I'm still here and plugging along, and plan to be here for a lot longer! You have a great doctor in Dr. Furst. You're lucky to have him. I was put on oral cyclophosphamide last year by a rheumatologist that I no longer see. I switched to a sclero specialist at the 5 month mark and he pulled me off of it. He told me that I was not sick enough to be on such a toxic drug. He also said that he would have sent me for another pft to see if the bad one that I had were just a "fluke", as Jen put it. And I agree also, that you're very lucky to have it caught so fast. So many people go such a long time trying to find out what is going haywire in their bodies. So you hang in there, and keep researching. And no that there is always someone here to talk to.


Happy people don't have the best of everything, they just make the best of everything they have!

Warm and Happy to you! Vee

#8 Gidget

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Posted 20 October 2007 - 04:53 AM

LaurieJill,
I was on IV cyclophosphamide for 6 months. The first 3 dosages were 750 mg per body square; the last 3 were 900 mg per body square.

In my case, I did not find being on the cyclophosphamide all that bad. I drank lots and lots of water and always took it easy the first 3 days after the monthly dosage. On the lower dosage, my white count never came within the normal range. On the higher dosage, my white count came down to the lower normal range.

I took the drug for the lung disease. I am happy that I did. Side effects were thinning of hair, some mouth sores initially and nausea initially. Also tiredness the 14 days after the treatment. I found that my breathing improved and it didn't hurt as much to take a deep breath. Again, I wouldn't have taken the oral cyclophosphamide but with the IV, I felt that I could flush the drug out by drinking lots and lots of water for most of the month.

My last dosage was in August. I am now more breathless and it hurts again to take a deep breath. I have an RX for cellcept but I would have preferred to just get quarterly dosages of the IV cyclophosphamide. Unfortunately, all the doctors feel that the cellcept will be just as effective as cyclophosphamide but without the higher risk of developing leukemia, etc. I feel that Cellcept has not been fully tested -- so who knows -- it could be another HRT drug. So I will start the cellcept and hope it keeps my lungs stable. We shall see. My PFTs while on cyclophosphamide showed a slight drift downward.

Good luck. Gidget



#9 MicheleM

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Posted 21 October 2007 - 08:37 AM

I hope the cellcept works for you Gidget. If this were a game of tag ... tag I'm it! ... I'm going to the pulmo doctor this Wed & expect the usual tests. I saw my general practitioner and he had me visit Dracula for about 4 huge vials of blood & a urine test. Then he handed me a script for prednisone & chest xray. I'm to find out about cellcept and embrel & see which one is better, however, from all I've read on my own and through the many comments on the boards, I'm pretty sure if it's anything it will be cellcept because it's supposed to be less toxic (?) than other drugs used. We'll see how it all develops!
Thanks to all of you for being there for all of us!
Soft hugs your way,

Michele

#10 Janice

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Posted 21 October 2007 - 09:04 AM

I can only comment on prednisone. I have been on prednisone, on and off now, for the past 19 years for RA and Asthma. I find it works rather well actually.

#11 janey

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Posted 22 October 2007 - 05:47 PM

Gidget,
Here is the information that we have on cellcept. From what I've found, their have been a few small studies (less than 30 people per study) for both cellcept and IV cyclophosphamide. There was a phase III study (more than 100 people) for oral cyclophosphamide.

I hope you get some positive results from the cellcept.

Big Hugs,


Janey Willis
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International Scleroderma Network (ISN)

#12 Gidget

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Posted 25 October 2007 - 01:51 AM

Thanks, Janey , or the cellcept link. I did start it and it makes me feel slightly nauseous but I think it is because the directions say to take on an empty stomach. Have fun.

 

Regards,

 

Gidget