Anyone with Scleroderma with secondary raynaud and pulmonary fibrosis and pulmonary hypertension?

[lakemon]

[Hidden]

I have scleroderma with secondary raynauds. I dont have a lung diagnosis, but am not happy about my lung function tests, my DLCO has dropped to 60% after always being ok - do you know what your results are?

[lakemon]

I have been having a decrease over the last two years. The doctors watch three parts of the test: FVC, VC, and DLCO. The results from 9/2010 DLCO was 87, 8/24/2011 was 38, and 1/13/2012 was 45. The FVC was 2010 was 76, 2011 was 31 and 2012 was 53. The VC was 2010 73, 2011 was 43 and 2012 was 55. I was in the hospital for 1 month and then rehab for 2 months. The rehab really helped bring the scores up. I am on oxygen 24/7 but I can still do some of the things I always did but SLOWLY. Just keeping plugging away. As you can see my test were pretty good until the spring/summer of 2011. Raynauds is not really bad, just bothersome. My advice is to make sure they watch your PFT test and try to have them do it pretty often.

[Hidden]

Thanks for that, the other parts of the tests (equivalent to yours) were 125% and 126% last May, then in my tests this Feb, one had dropped by 15% and the other to 91%, which is a 30% reduction - i thought that a big reduction. Not very happy about my care and getting a referral elsewhere. I feel for you being on oxygen as the lung probs scare the hell out of me! Hope you stay stable.

[lakemon in reply to Hidden]

I just take one day at a time. The progression is pretty quick but I am starting on a new drug for PH. Keeping a positive attitude is the best way to keep going so just hang in there and keep pushing until you find the right doc. By the way I live in the US in Virigina

[dawgjones]

I have Scleroderma with secondary raynaud's syndrome and pulmonary hypertension. I do not have the pulmonary fibrosis. I had a lung function test the last visit with my doctor and he said my lungs were solid. I just have the problem with the PAH. I have been on oxygen since my doctor found I was unable to maintain an O-sat level above 90 percent on my own. I take Tracleer, Revatio and Tyvaso for my PAH. Since being on the Tyvaso, I have improved to where I don't feel like I'm going to pass out before I get my oxygen back on when getting dressed. I am closing in on my one year anniversary of retiring on disability.

[lakemon]

I am so glad you don't have pulmonary fibrosis. It is really does slow you down. I have been on mycophenolate for 5 months but we don't see any benefit. I am starting Letairis next week. This is supposed to help the PAH. It is a very expensive drug but the Dr. was part of the program where I can get help with the cost. Do you take all three drugs now or have you taken them seperately? I am one year into oxygen and I too can't do without it. Using 3 liters. Good luck and keep up your spirits. Jane (Lakemon)

[Thelma]

Hi LakeMom: I too am on Revatio and Letairis, and have improved over the last couple of years. Have been on oxygen 24/7, then night time, then 24/7 again, and now mostly for sleeping. I put forth a lot of efforts for helping myself to feel comfortable, having a positive attitude, engaging with positive people, prayers, and kind support. My schedule is rather regimented and trying to keep my stress levels as balanced as possible helps a lot. I practice Yoga, meditation, weight training (and even through some pains), moderate paced outdoor walking when the weather permits. I had been a very healthy person throughout my life; little symptoms were happening to my body beginning in the early 90s, and then I came down with severe pneumonia in 2005 w/a 17-day hospital stay, fighting for my life; then I was diagnosed w/Scleroderma and Raynauds in 2008, w/lung surgery that year also. Was on life support in a coma after the surgery for 5.5 days. Then had 7.5 weeks of round-the-clock care-giving after the hospital release. I've come a long way, and I continue to try to do the best that I can. I believe it is essential that we are regimented with our schedules for our meds, our meals, and try to stay around positive environments and positive people. Added stress can take a toll on any one, and especially if there are noticeable and diagnosed medical conditions. Some days are more challenging, but overall many miracles have happened for me, and just to have survived through this last ordeal, and to come back to life with having a memory, and to be able to function again. I remain amazed and so aware of these awesome blessings. I continue to keep us all in prayers. GOD bless you.

[lakemon in reply to Thelma]

I will be starting on Letairis in the next few weeks. Takes time to get approval. What symptoms/side effects have you had with Letairis? How long have you been on it. I understand it takes about 2 months to see any results. Hopefully this will help with pah since I have trouble breathing even with oxygen 24/7. Take care of yourself

[Thelma in reply to lakemon]

I have been on Letairis since 02/2010 and Revatio since around 03/2010. They really have helped me. I am also on Lasix & Aldactone for I am also a CHF person, and have come a long way w/that condition. Luckily the meds have agreed with me well. I have been able to come off of about 4 meds since 05/2011. I truly believe as mentioned before, that I have come such a long way with recoveries from the multiple conditions and complications with a strong determination to feel as best as possible and to apply mindful health care mentally, emotionally and spiritually with prayer, meditation and stress breaks of quiet relaxation, the moderate fitness walking during good weather, reading, and other hobbies which help with relaxation, favorable music is a helpful therapy too - and being on pretty much a consistent schedule. I arise very early and I retire at night very early - Early? I generally get up between 4:30-5:30 a.m. and retire between 7 & 8:30 p.m. I try to keep my meals and med intake within a 1 hr. window too. When we really think of the little things, like being very nervous and worried, our meals don't even digest properly . . . I'm not in the medical field or a Rocket Scientist, but I would think that our meds wouldn't agree with us as well either . . . just by being super stressed. I have always believed that if we take any kind of meds - even aspirins . . . we must pamper and compensate in other areas. Do you agree? Do you remember some time of experiencing not being able to eat well when your nerves weren't too relaxed too? Not a good feeling. I apply those little trigger potentials to how our govern my illness - and lots of pampering that the good old body likes, I find really helps me - in fact, my endurance to pain (when it occurs) has even improved. - LakeMom, it's nice chatting w/you, but I won't tarry longer for now. You take good care. My other Recovery Friends out here in Cyber, you too take good care. Have a wonderfully blessed day, and we'll chat soon again.

Cheers to You!

[Thelma]

Oh, and yes, I do have the Pulmonary Fibrosis which sometime flair ups can be excruciating w/pain . . . . . it's a little less frequent in the last year; my attacks aren't as extensive at length as they used to be.

[lakemon in reply to Thelma]

what meds are you on if any? Guess we just try multiples to see if they help us continue to move around.

[Thelma in reply to lakemon]

LakeMom, in addition to my response just now, I thought of something else. I was a smoker many years ago - gave it up 20 yrs. this Sept.; however, I was into a health regiment and began my kicked off the New Year of 1981 with a physical fitness program and never left the practice. I read up on health, fitness, subscribed to fitness magazines and so forth - fortunate to grow up in a household who practiced physical fitness - my Dad was a former amateur boxer and martial arts instructor. Anyway, I started taking 400 mg. of Vitamin E a day the last 6 years of being a smoker, and started drinking more orange juice w/calcium. After my first illness in 2005 I cleared my Vitamin intake with my Rheuma., GP, Carido. and other Specialists - I am still taking Vitamin E which assist with blood circulation. A few older family members also have attributed better health to Vitamin E. I was a full time oxygen user, and now during the day when exerted or if I deem oncoming exertion, I use it - and wear oxygen for sleeping. However, since I participated in the lung study rehab program (and I had to wear it during the day during part of that period for my stats were dropping) and emphasis was placed on purse-lip breathing techniques - I paid more attention this time, forced myself to be more mindful about this practice and practice it more frequently, and just generally practice it . . . . even though I'm a little overdue for my checkup . . . . this technique certainly has helped me - and just little ole me is going to say . . . I believe my lungs have more personality these days and are a wee bit stronger. Anyway, like a car needing gas and ya' gas it up . . . hey, that's the scenerio that I use for the purse-lip breathing technique and continuous practice of it. Smiles & Chuckles. Again, we're all here for each other, and I'm wishing you the best LakeMom.

[CWats in reply to lakemon]

Remodulin subcutaneously. I have severe PAH due to scleroderma and I now need a double lung transplant. I am in the high-risk group due to reflux.

[Meda]

I have scleroderma with raynaurd's as secondary and bilateral interstitial lung disease. I haven't head to use oxygen since my long biopsy but for a while I had to carry oxygen with me where ever I went, just in case along with a ventolin inhaler. When it started getting worse,I finally went to a specialist and she put me on cellcept. After six months they did another breathing test and I showed improvements. I'm thankful that I was pursuant with doctors and this was caught early. I can't imagine what it would be like now if out hadn't.

[lakemon]

The oxygen is a life saver for me. I would drop down to 83 with out it. I am on mychophenlate and Letairis since I now have pulmonary artirial hypertension (PAH). Just seems that each new change in this disease requires another drug. I am fortunate to be under the care of one of the top doctors in the US for scleroderma and a great pulmonologist. They have discussed cellcept but so far are waiting. Take care and just keep up a positive attitude (some days that is hard isn't it)