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Sara P: CREST Syndrome

"Just answer me this, am I going to live to be an old lady?"

Hi! My name is Sara, and I have CREST.

I have been very healthy all of my life, then in the summer of 1997 I started getting arthritis-like pain in my joints. I thought it was because of the Atkin's Diet I was on, so I went off the diet, and the pain went away.

Then in the winter of 1997, my fingers and toes started turning white and numb in response to the cold weather. I thought it was just some weird virus or something, so I did not think too much of it.

When winter of 1998 came, and the white fingers and toes returned! Something is not right here!

So I went to my OB/GYN and told him about this. He said it sounded like Raynaud's. He ran an arthritis panel, and the anti-centromere pattern showed up. It said,"Anti-centromere antibodies detected, characteristic of the CREST, a sub-set of Scleroderma. Prognosis for such patients is relatively good."

I had never heard of CREST, or Scleroderma. When I saw the prognosis, I thought, well, it isn't the end of the world, I guess it is something I will have to deal with. My doctor sent me to a rheumatologist. My rheumatologist ran all the tests again. He called me a week later, and said that I have the anti-centromere pattern, which "gets his attention", and only one symptom. So at this point, I am only at risk for CREST.

Okay, I can deal with this. Well, then I got on the Internet and read stories about people with CREST, and how they are having pulmonary hypertension, and they are in and out of the hospital, and all this stuff. I was really getting scared! I was looking for anything that told me I have a good, or very good prognosis (as opposed to Diffuse Scleroderma.) I found a few articles that made me feel better, but of course there is no guarantee.

But the stories of all the problems some people with CREST are facing were frightening!

I called my doctor, panicked! He told me that I may never get any worse than I am right now…or I may…but that most of his CREST patients do fine.

I said to him, "Just answer me this, am I going to live to be an old lady?" He kind of laughed and said, "Yes, you're going to live to be an old lady."

Whew! That made me feel a lot better. Okay, I can deal with this now. It is almost winter of '99 now, and I am starting to have swallowing difficulty, and acid indigestion—there's my second symptom which gives me an official diagnosis.

I take Plendil for circulation for the Raynaud's. I am now on Prevacid for the heartburn. They both work great. I have no skin hardening at all.

I believe I have Fibromyalgia because I am always tired and achy. I get depressed and was taking Elavil for a few years, but stopped it because I was feeling groggy and in a fog during the day, even when I took a tiny dose.

I do Tai-Bo for exercise, and it really helps me with the depression and is great for circulation and keeping fit (my depression is not severe).

I am going back in for my yearly check-up this month. He'll want to do tests on my lungs, heart and kidneys, just to see how they are. I do not have shortness of breath or anything like that…yet. Every now and then I get a little panicky about this. I mean, I have two young children, I do not want to be sick, let alone die!

Then I feel better knowing it's only CREST, and not Diffuse. But then I get scared when I read some of these CREST stories!

I have a very deep religious faith. I know God will never give me more than I can handle, and he'll allow me to raise my kids, and even help with my grandkids someday. So I try to find peace and comfort in knowing that I am truly in His hands, and whatever comes my way, He will be there to help me through it.

~Update 01-09-05~

I have remained stable since my previous entry. I do have a problem with fibromyalgia and joint pain, especially in the winter months. In the summer, I seem to be fine, almost like there is nothing at all wrong with me. I pretty much forget I have an illness until around December when winter starts to set in. I live in Southern California, which is the perfect place to live.

The only difference I have noticed, is that during the winter of 2003, I started having problems with pleurisy. My left lung seems to get inflamed easily with cold, wet weather or stress. Stress, I noticed, is a huge trigger for my symptoms.

My thirty-nine-year-old brother had a major heart attack last year. He survived, but the stress of it made me so sick that it took me six full months to recover. I was on the verge of pneumonia, which is when the pleurisy started, as well as a lot of widespread pain in my joints and muscles, and such a weakness in my soul.

There were times I thought that this was it. That the CREST would take over and just spiral me down. But I did recover and am doing fine now. I hope my symptoms never get worse, although I do worry about the pleurisy.

I found a new rheumatologist, at Loma Linda Medical Center here in southern California. He is cutting edge. But I have not been to him yet because I have been so stable; I think now I should call for an appointment just to be checked. I did not care for the one I was going to, so I have not been checked in a few years.

I am tired most of the time. This is something that I find very hard to deal with because there is so much I want to do and I am always fatigued. I have noticed that when I exercise (not too strenuously), I feel much better. I also have to watch what I eat. No spicy or acidic foods or alcohol.

I just continue to plug along with this and am so thankful, because I could be a lot worse. I welcome any e mails from anyone out there. Take Care.

~ Update 03-26-08 ~

I just wanted to update everyone and say that I am still doing just fine. I remain pretty much the same as I have since first diagnosed eleven years ago…boy does time fly!

My children are in high school and college. I lead a very normal life. The symptoms I continue to deal with are: Raynaud's, red spots on my face, acid reflux, fibromyalgia. I have arthritis in my toe joints and it is starting in my hands.

This seems to be progressing as time goes on. But so far I still have no internal involvement and my lungs are doing fine. I recently read somewhere that in CREST, the internal organs are usually spared…this is great news! I believe that I have a mild form of the illness and hopefully it'll never get much worse.

I just keep strong in faith. With God I can overcome anything! I welcome your e mails. Bless you all.

~ Update 02-07-11 ~

Well here it is 2011 and I am happy to say I am still pretty much the same. I still suffer the usual symptoms, Raynauds, trouble swallowing (but not too bad), fibromyalgia.

I have arthritis in my hands and feet but my doctor seems to think that is separate from the CREST. I may have had it anyway. I am forty-nine years old and going through menopause. That's been hard for me, some depression, so they put me on Wellbutrin to help with that.

I have a cardiac workup done every two years, and it is always fine. Pressures in the lungs are fine. So I am really no worse with the CREST, just the aging factor and all that it brings. But I feel good most of the time and still continue with my routine, working and traveling a bit.

There is so much hope with CREST, it is not a death sentence and I am proof that you can live a perfectly normal and relatively healthy life with this form of the disease. I certainly welcome all emails. Take Care.

~ Update 04-03-14 ~

It has been three years since I've posted and I am happy to say I am still doing well. I recently had all the tests done, echocardiogram, stress test, endoscopy, and they all came out just fine. I also had my gallbladder out last year and while the doctor was in there, he looked at my internal organs and they appeared to be healthy. I am very grateful I have no serious issues with this illness so far.

That being said, I still struggle with arthritis and fibromyalgia. It seems these ailments are a part of having an autoimmune disease, and it has gotten worse as time goes on. The arthritis is mostly in my big toe joints in both feet. This makes it painful to walk. I take celebrex for it and it does help, but I am unable to walk long distances. When the weather changes, I feel it in my hands and shoulders.

The fibromyalgia seems to be constant but at times I will have a flare up that is just miserable. The pain will be from neck to toes. There isn't much I can do for that other than massages. Over the counter pain killers really don't work for the muscle pain. I have spoken to my doctor about this, but I am hesitant to go on more pain meds; I take plenty of medication already. I have many good days where I am not in much pain so it is manageable. I just deal with it as best as I can on the bad days.

So, yes, I am very grateful that I don't have any "life threatening" issues, but living with the non life- threatening issues can be tough. It is just part of having this disease.

I have received many e mails over the years from people or relatives of people who have CREST/Limited Scleroderma. Some of them are people around 80 years old who say they had the symptoms when they were young. They have dealt with problems over the years but have lived a long life. I fully expect to do the same.

I am still very able to go out and live my life, work, play, travel, enjoy my young adult kids and be part of their lives. I am limited on how far I can walk, my stamina and having to deal with pain at times, but at least I don't need a wheelchair! So glad for that!

I will keep you all posted, and of course I welcome all emails. Take care.

~ Update May 2015 ~

I wanted to keep you all updated as to my progress. I was just diagnosed last week with early stage pulmonary arterial hypertension. This is something that I was hoping I wouldn't get, but I have. I have been mildly short of breath for about two years now. Of course thinking I'm just getting older and out of shape, I didn't bother telling the doctor. But I sort of knew something was going on because having CREST, I know that PAH is a real possibility.

I finally mentioned it to my rheumatologist about a month or so ago. He said it's important that I get the lung function tests. So I did. The DLCO (gas exchange) was severely low. Echocardiogram was perfect, no problem with my heart. So next step was a CT scan of my lungs. No fibrosis, everything was clear except my main pulmonary valve is slightly enlarged. So last week I had a right heart catheter done, where they go in through a vein in my arm, into my heart and to my lungs. I was very nervous about this but it was a piece of cake. I was sedated and never felt a thing or knew what was going on. I'd do it again no problem. (Apparently I'm going to need many more of them.)

My results were that my pressures are high normal. Which means that I am just bordering on normal and PAH. This is a progressive disease so most likely the pressures will continue to rise. My pulmonologist is definitely going to treat me, because this is something we want to catch early. I am thankful that we did catch it early because a lot of people are diagnosed with PAH after its become severe and its harder to treat and prognosis is poorer. So I will be going on one of those insanely expensive drugs that they treat PAH with.

I am very hopeful that catching this early will give me a good prognosis. Twenty or so years ago there were virtually no treatments for this and the prognosis was very poor. They have come up with so many new treatments since then and there are more on the way. Many people live a long time with PAH.

So I am just going to take this as it comes, one day at a time, take my pill and live my life. I am very positive about this. My faith with see me through, as always.

I am dreading possible side effects with these treatments, but hopefully I'll respond well and have no side effects. Many people do just fine. I will keep you updated on my progress. I welcome all emails. Thank you!

~ Update August 2015 ~

I have been put on Opsumit for the Pulmonary Hypertension. I have been on it for ten weeks now. I started to notice a tiny difference at around week 7; I felt just a little less short of breath.

At week 8 I noticed it even more. Now at week 10 it's a little more than that. I did the six-minute walk test last week. Before I started the Opsumit, my oxygen saturation was 99% at rest and dropped to 94% when I would walk for six minutes. After being on the Opsumit for 9 weeks my saturation was 99% at rest and dropped to 96/97% when walking for six minutes. So it has improved, but is still not where the doctor wants it. It may take more time, they said this is a gradual thing. So in six months they'll check it again.

I see the cardiologist this week and see what he says. The doctor said over time they'll probably have to put me on other medications along with this one, as it may progress. For now though, I am feeling better and have more energy. It's still not up where I'd like it to be, but I think that will come in time.

I would like to stress to anyone out there that is feeling short of breath, especially if you have scleroderma, to see your doctor immediately. Don't put it off. We have caught mine in the very very early stage, where it has just barely started. That is key to keeping this thing corralled. My heart is in good shape, it has not affected it at this time.

My doctor told me they get so many people in where it has advanced and they can't even walk fifty feet, and their heart is affected. Because I've caught this early I have a fairly good prognosis. There are more medications for this in the pipeline and I bet even one eventually that will stop the progression. Without treatment the prognosis is one to three years. I will keep you posted.

~ Update September 2016

It's been a year or so since I've been put on the PAH drugs. I am doing well. I am tolerating them, have had no side effects from them. I can breathe normally with no shortness of breath with exertion. My doctor tells me that despite what is on the internet, that since we've caught this early and treatment has started, that he feels I'll do just fine with the PAHfor a LONG time. None of this "poor prognosis with two to three years to live" stuff. In fact, since I started the PAH meds last year, there is another one that was just released that is even better. He'd like to try me on that sometime in the near future. There are more being developed and they're working on a way to stop this. I feel very hopeful.

I still deal with the arthritis pain and stiffness, and I've taken NSAIDS for so many years that it is affecting my kidneys so they don't want me to take anymore of those. I am in pain most of the time from this and it is not pleasant at all. It does affect my life in that I can't get out and do much like I used to. I have these "flares" often and it is very depressing. The fibromyalgia added on top of this is very difficult too.

I recently also found out I have osteoporosis. I was shocked because I've always had large and strong bones, no osteoporosis in my mother or in my family at all. I take PPI's, plus I don't absorb vitamins and minerals which may have contributed to this, along with being post menopausal. I am now taking Calcium, D and Vitamin K2. The K2 directs the calcium into the bones and not into the arteries. (This has been a concern with taking calcium). I am hoping I'll feel better with all of this. I'll just keep trudging along and as long as I can keep the PAH at bay, that is a plus. The everyday pain is very hard to deal with, and it's apparently all part of this scleroderma thing. I will update when I can. Thank you!

~ Update February 2018 ~

I am still doing well. I've been stable on the PAH meds, Opsumit and ADCIRCA.

My oxygen saturation fell below 90 on my six-minute walk test. I also noticed I was getting short of breath again and my heart would race when walking. So I am now on supplemental oxygen for exertion.

My doctor increased my medication and I'm waiting the three to four months for it to kick in. I'm hoping I can get off the oxygen. My doctor is very aggressive and he'll add another medication if needed.

The PAH does progress, the meds help slow it down and stabilize it. There are a lot of drugs in the pipeline to treat this that are available to us yearly.

They're working hard on one or two right now that will reverse the progression of PAH. They're about five or more years away from being available to us, but I'm pretty positive I'll still be here and ready for them. I am staying positive about all this.

I do have my down days but I am trusting God. I just put my oxygen on and go about my business as usual and nobody really notices and no one says anything so that's good!

I feel great, otherwise. My heart and all other organs are "golden". All my blood work is normal.

As far as the Crest/Limited Scleroderma, it has remained very stable. Still, after all these years I've tested negative for diffuse scleroderma. No issues other than Raynauds, joint pain, and fibromyalgia. I am very fortunate. My skin is soft and I look perfectly normal, except now for the oxygen.

I do have a lot of telangiectasias on my face. They cover with makeup. They've found that having a lot of them is a predictor of developing PAH.

I hope this helps any of you out there in understanding this disease and the progression. I'll update again in a year or two. God Bless!

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