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Diffuse Systemic


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#1 oddone

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Posted 14 February 2007 - 07:05 AM

I know everyone is different in this disease, and that no matter what you read, it will not ever be case "specific", but I have a few queries/comments.
Its frustrating to do alot of research and have all the knowledge sink in, but in the end it never really gets you anywhere.
I am happy with my husbands status, he hasn't progressed to internal organ involvement, but skin score went from 25 to 29.5 in 3 months. He feels good and his attitude is great (cause he's no longer reading the internet).
It bothers me that there is information out there stating that the 10 yr survival rate for DSsc is 20%, yet the stats for internal organ involvement within 5 years of diagnosis is 1/3 of DSsc patients. Does anyone understand this? It does not really make sense to me, and these websites that this information is on seem to be quite reliable.
I have read that if the internal organs do not become involved by the 5 year mark that the odds of them becoming involved is very low.
Can we do a survey? How many males with Diffuse Systemic there are(I know there aren;t that many), when diagnosed and at which stage of progression they are currently at? Im not trying to be nosey, but trying to rationalize the "so called stats" with actuality?(If that makes sense) and perhaps do a little random data collection.
Its all so interesting and vague all at the same time.
Oddone - Spouse - 38 yr old Husband diagnosed October 2006, Diffuse Systemic Scleroderma
Normal PFT(July 06), ECG(Nov/06)

#2 Shelley Ensz

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Posted 14 February 2007 - 08:29 AM

Dear Oddone,

I understand what you mean, but as it happens, we are not set up to do medical surveys on this site. There are hundreds factors that go into valid survey design, as well as laws regarding medical privacy...and we are not doctors nor researchers.

It really is true that every case of scleroderma is different. Some people will survive longterm, and some will not, but there is no reliable way to predict exactly who will develop complications early on, and who will not.

Besides, even if 99% of the men with diffuse scleroderma who were surveyed lived to the ripe old age of 100 with nary a complication, that would mean nothing at all for your husband's particular case, as he might be the 1 in 100 who develops problems (or vice versa).

Each research study on survival needs to be read very carefully before even trying to generalize the results. Basically, diffuse scleroderma is considered to be the worst and most fatal form of systemic scleroderma. Extent of skin involvement tends to be related to the amount of internal damage that eventually occurs -- but not always. Conversely, people can have a lot of internal organ involvement without any skin involvement, too.

Its usually quite a good sign to make it to the 5 year mark without extensive internal involvement. But stuff can happen, and it doesn't necessarily mean that a person is "home free" at any stage with any type of systemic scleroderma (limited or CREST, or diffuse).

Your husband has a very serious disease. But with both good care and good luck, he may beat the odds. The thing is, there are no guarantees -- for anybody, even people who are perfectly healthy have no idea how long they have left on this earth. That's why it behooves all of us to make the most of every day.

Which is getting me entirely off topic of the fact that you can read all you want about prognosis and mortality for systemic scleroderma, and the most you can conclude is that you will need to be extremely flexible -- plan for the worst and expect the best sounds contradictory, and perhaps someone else can explain it better.

My husband has a disease that is usually fatal, but he is beyond the bell curve yet still quite happy and very alive.

With great doctors, superb self-care, and an abundance of happiness and delight, your husband might also outlive all the statistics. And even if he doesn't, if you have filled every moment with love and joy, he will still be one of the luckiest men in the world...and you will be his lucky lady!
Warm Hugs,

Shelley Ensz
Founder and President
International Scleroderma Network (ISN)
Hotline and Donations: 1-800-564-7099

The most important thing in the world to know about scleroderma is sclero.org.

#3 oddone

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Posted 14 February 2007 - 08:56 AM

Thanks Shelley, you always know the right things to say.
I wasn't necessarily considering an official "survey" per say.
I had just finished re-reading the "Getting to know you" topic
and found it so interesting and informative.
Reading real peoples perspectives, people that actually live with this disease.
Rather then the medical journals and articles.

I guess no matter what you do or learn, nothing is ever going to be concrete.
This is where I have to just take a step back and wait and see(the hardest thing in the world for some people to do!!!! - Like me)
My inquiring mind - I wish I could turn it off
Oddone - Spouse - 38 yr old Husband diagnosed October 2006, Diffuse Systemic Scleroderma
Normal PFT(July 06), ECG(Nov/06)

#4 Gidget

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Posted 14 February 2007 - 09:09 AM

Oddone,
Your husband's doctor, if he or she is a sclero expert, will be able to give you some sense as to where your husband is in the disease bell curve and what would be the "normal" progression of your husband's disease should it follow the normal pattern. I believe that there is only a small percentage of the population where the average bell curve will either be shorter or extended. Obviously for us with diffuse, hitting the 5 year mark is an important milestone however, I believe that the proper medical care and right attitude does make all the difference in the world. Your husband is very lucky to have someone so supportive. Wishing you both the best. Gidget

#5 Shelley Ensz

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Posted 14 February 2007 - 09:38 AM

And for those who would like to see the nitty gritty of the research studies in regard to prognosis and mortality, our link is Systemic Sclerosis: Prognosis and Mortality.

It is, obviously, an upsetting subject to read about for many of us. As with everything on this forum and in our website, please just avoid anything you don't feel ready for yet, particularly if you are in the throws of depression or anxiety.

Sooner or later, curiousity gets the best of most of us, and I can't say I felt any worse knowing the facts than simply guessing at them. Especially since years ago, doctors painted such a grim picture of survival, I found the actual facts to be a tiny bit less daunting, in some respects.
Warm Hugs,

Shelley Ensz
Founder and President
International Scleroderma Network (ISN)
Hotline and Donations: 1-800-564-7099

The most important thing in the world to know about scleroderma is sclero.org.

#6 oddone

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Posted 14 February 2007 - 09:49 AM

Yes I too find the facts less daunting then some articles that you read (some from years ago, yes they really did sound grim back then).
Thanks for the thread.
I hope others know that there IS just so much study(trial) going on right now.

Patience is a virtue......(Im not very virtuous...... :lol: )
Oddone - Spouse - 38 yr old Husband diagnosed October 2006, Diffuse Systemic Scleroderma
Normal PFT(July 06), ECG(Nov/06)

#7 Margaret

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Posted 14 February 2007 - 10:07 AM

Hi Oddone ,

Your questions are very much like mine. Gareth is male, 18 years old, diagnosis'd with sine Scleroderma in Dec. Having the positive Anti RNA Polymerase test is VERY depressing for me because of what I read. BUT......with the medical knowledge of today and keeping up to date on blood work, kidney function, etc, I am optimistic about his future. Since he hasn't got the skin problems, his symptoms started with the diagnosis of esophageal dismotility....already one strike against him.
Take care, Everyone.
Margaret

#8 peanut

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Posted 14 February 2007 - 12:57 PM

Hi all,
I was diagnosed about 6 months ago. Mortality rate was one of the first questions I had; how long did I have? I found the reports to be grim. I also have lung complications so that didn't really help. Like oddone said, I met real people – men and women at my local support group. It was encouraging to meet some who have managed scleroderma for 20+ years. I know it isn't easy, but I realized that if they can do it, I can do it – or at least I’ll give it my best shot!

peanut

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

#9 Lori-Scleroderma Spouse

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Posted 14 February 2007 - 03:22 PM

My hubby is 40 years old and just met the one year mark. That may not seem much but it counts. That was our first question as well. I had read all the research on the net before we got to the spealist. The rhummy that diagnosis it was not very positive, but he gave it to us straight. The speacialist that my hubby sees now is very positive alsmost too positive. He told us that the research that I was finding on the web was old and many advances have been made in the research. He did not think the 5 and 10 year mark were still true. He also said that being able to recognize and treat the complications now had improved the survival rate. But, like I said he is too positive sometimes. He did not think my hubby would progress as rapidly as he did. My hubby now gets the Cytoxan treatment once a month to help slow the progression. We think it is working because he seemed to be doing better until his treatment was delayed by 2 weeks. He lost the gain he had made. Good luck to you and your hubby. I completely understand. This is tough on the entire family. You and your family are in my thoughts.
Lori
Scleroderma Spouse

#10 sadie20

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Posted 15 February 2007 - 07:21 AM

For Lori,
Just wondering what type of Sclero and symptoms does your hubby have to be get IV Cytoxan?? Thanks

#11 americanmike

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Posted 15 February 2007 - 06:30 PM

Hi Oddone-- watch out what you read on the internet. there is alot of misinfo out there. Even from good doctors and good sources. People are not perfect. Reports have errors. Etc.

I too have seen numbers like 20% 10 year survival and it made me very depressed. But keep looking because the real data is where the truth lies.

Shelley gave you a post to survival. Find the study where they retroanalyze like over 2500 cases and compare DSSC to LSSC and different autoantibody profiles.

I was happily shocked to see survival raging from 64% to 80% for diffuse patients. That study was 2000+ cases, real numbers. And it was over 10 years ago. So going forward should be even better!

Articles like that one can really make you feel better, and you know such data is better than some doctor spouting off a statistic from the hip. It's a real study with real people. (actually better than the survey you wanted to take here on line. just kidding).

You must keep telling yourself and your hubby this is not a death sentence. Certainly not with stats like the one I'm mentioning here. And certainly not with his slow progression, and certainly not with the unbelievable new research and therapy on the horizon. Trust and stay optimistic!

Michael in Florida

#12 barefut

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Posted 15 February 2007 - 08:52 PM

Like Shelley said, I "plan for the worst" but I only HOPE for the best because I am not at the point of "expecting" the best yet. (But I am close.) Hopefully someday soon I can get there.

I have Diffuse Systemic Scleroderma with lung involvement (among every other organ) diagnosed just over a year ago. Hey, if I'm gonna get a disease might as well do it up right. I'm SUCH a perfectionist :lol: I read that a person at my stage in the game and with the symptoms I have, has about 6 years.

But those are the statisics and I am not a statistic. I am a single mother of two precious young boys, separated from an abusive husband, trying (and failing) to make a living with my in home preschool and get a divorce.

So, I don't have time for this stinking disease to slow me down in any way shape or form. I've got things to do, places to go and people to see. And I am determined to have a blissful new life before I have no life because I deserve it.

The first 3+ months after my diagnosis were emotional chaos and I still have my moments. This disease has taught me that sometimes I need to come first and that's just tough noogies for anyone who doesn't like that.

It has also taught me to quickly weed out the things in life that don't matter or that really don't need much attention and which things to really focus on. I'm subconsciously re-prioritizing all the time.

Sounds cliche but I am living one day at a time and I am living IN every precious moment, good and bad, and trying to make the good win over the bad. I have a lot of BIG stuff going on and I focus on one thing at a time to get through it all. I have had to learn PATIENCE because all good things take time and I am a "get it all done all at once and right now" kind of person (not to mention, I only have 6 years left! ;) )

Probably hardest of all for me, I have had to learn (and still learning) not to feel judged by all the things I cannot do anymore and to not feel bad about asking for help. I still hear judgemental voices criticizing my yard, my unwashed vehicle, the moss on my roof, and my dusty house but those voices are probably only in my head anyway ;)

*Sigh*.....Wow. Okay, so what was the question? :lol:

Oh yea, one more thing, humor. Gotta absolutely have lots and lots of humor in your life. If you can't find it or it won't come to you, make it up yourself.

Well, I think I'm about done rambling now.
Good night all and be well.
Barefut

#13 Lori-Scleroderma Spouse

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Posted 16 February 2007 - 12:13 PM

Hi sadie

My hubby has diffuse with possible lung involvement. He had a great deal of muscle and nerve involvement. He had been on 4000 mg of cellcept daily with not improvement. His doctor then decided to try the IV cytoxan. He told us that IV was lwss toxic than oral. HE was going downhill quickly. He has seen some improvement since getting the IVIg and Cytoxan. He got the last IVIg treatment today. Hope the Cytoxan does it job.

Lori
Lori
Scleroderma Spouse

#14 sadie20

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Posted 17 February 2007 - 05:42 AM

Lori,
Seems like I've heard good things about IVIG here - good response. I asked my doctor about it and she says I need to try the Cellcept for 6 months first. I know she is the expert, but the thought of "waiting" 6 months while this disease may be progressing is frightening. I see there are several folks here on Cellcept and may don't seem to be improving. As for the topic, the autoimmune diseases are all so unpredictable - especially sclero. This is the most frustrating part of this illness. When I was first diagnosed last fall, I came to the internet to educate myself. I was horrified initially, especially when I thought about the movie,"For Hope". Also, I was told At the beginning of Sept. by my primary care physician(labs) but could not get in to a Rheumatologist for 4 weeks. When I saw her and she examined me, she told me I had CREST and was one of the "lucky ones". I ahd several more labs done that day, and the next day she called me to say,"I am so sorry, but you have Systemic Diffuse Sclero". Based on all the info I had read, I was planning my funeral!! I think this probably happens to all of us. Since that time, I've read from many on this site, talked to folks in my local support group and have focused much on my spirituality and having a positive attitude. According to all I've read and seen, I may improve spontaneously, stay the same or get worse. Although I hate this disease I realize that I have no guarentee from day to day. Prior to this illness, I never thought much about it. It has definitely helped me to live by the mantra,"Live each day as if it might be your last." One of the things I've decided to do is participate in the education to the primary care physician. I will be attending our state sclero board meeting next month. There is a wonderful pamphlet that I was told was made and given to all the rheumatologists last year. My question was why give it to them? It is the primary care physician and GI doctors that need the education. For example, I was diagnosed by my GI doctor 2.5y ago with watermelon stomach. I have recieved treatment 4 times since the diagnosis(Argon laser, transfusions, iron infusions). The first time I saw my rheumatologist, she said the watermelon stomach should have been the RED flag - it is one of the hallmark symptoms of sclero. My GI doctor is considered to be one of the best in the city. After I was diagnosed and told him what she said, he apologized profusely and continues to do so when I see him. I don't blame him. I just want him to educate others. Anyway, sorry to be rambling. Have a good day all. It is snowing agin here(ohio) and this city freaks out and dramatizes it to the max.. no bread, milk, etc for several days prior.