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Does the progression seem like Diffuse?


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#1 Kimberlygolden

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Posted 16 August 2015 - 04:11 AM

Hi ,

Thank you for taking an interest in me.

I appreciate you taking time to read.

I don't have a diagnosis but I really feel I'm heading there.

I'm stressed because I'm wondering if I sound more diffuse than limited.

Never really understood " how fast" diffuse really manifests.

Any thoughts?

2009- hashimotos

2010- muscle fatigue

2012- raynauds in one finger ( 4 episodes) at the age of 41

Joint pains comes and goes in flares.

Jan 2014- raynauds in both hands ( since then till now only 7 episodes) Strange huh?

May 2014- chronic hives
( lasted 6 mos)

June 2014- carpel tunnel ( comes and goes)

Aug 2014- esophageal spasms
( 4 episodes )

Had manometry - was told it was within normal limits.

Sept 2014- hiatal hernia

May 2015- present - pruritis everyday. Intense itching , feels like bugs crawling ( still have it)

June 2015- finger numbness ( lasted 1 week both hands)

July 2015- finger stiffness began and fingertip sensitivity.

Aug 2015- heartburn began

Dry eyes and mouth
Fatigue throughout the past two years.

I have been seeing a sclero specialist who repeats ANA by IFA every 6 months ( I've had 11 ANA tests done. Also ran separate antibodies.
Scl70
Pmscl
RNA polymerase 111
U1RNP
Sjogens
All lupus
You name it!!
ALL negative.

C3 and C4 were low on 2 occasions but then normal when repeated.

Nailfold cap testing every 6 months have remained normal.

I know AT THIS time I do not have enough disgnostic criteria for a diagnoses ( it is UCTD at the moment ) BUT my question is does diffuse Scleroderma present this way?

Or is this considered fast progression?

Thank you.



#2 Joelf

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Posted 16 August 2015 - 10:39 PM

Hi Kimberly,

 

Welcome to these forums!!

 

I can understand how confused and worried you're feeling, especially as you have not had a concrete diagnosis. The fear of the unknown is one of the worst fears of all.

 

I've included a link to our medical pages on Diffuse and Limited Scleroderma and also the classification of both types of Scleroderma, which I hope you'll find helpful and informative.

 

Diffuse Scleroderma tends to progress more quickly, with more widespread skin involvement, meaning that the skin thickening may also spread to the upper limbs or torso (having said that there is a type of Diffuse Scleroderma, Sine, without skin involvement). Limited Scleroderma tends to be slower in onset, with less skin involvement; however, it does vary from patient to patient. I have Limited Scleroderma with very little skin involvement, merely the tips of my fingers.

 

You can see from the above that reaching a diagnosis is quite complex, not less because Scleroderma affects everyone differently and it would be impossible to predict the prognosis of the disease at the present. However, as your consultant is a Scleroderma specialist, he will hopefully be alerted to any more positive signs that the disease is progressing.

 

Please do keep posting and let us know how you are faring.

 

Kind regards,


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#3 Kimberlygolden

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Posted 17 August 2015 - 07:18 AM

Hi Joelf,

Thank you for taking time to read my post and for replying.

I hear where you are coming from and believe me, I have read, a lot. I do understand that no two people are the same but I'm hoping that those with diffuse might share their experiences as to the time frame of progression as well as those with limited and that may give me some idea.

I know that all my symptoms point to sclero-

I just woukd like some opinions on those who've been there and officially diagnosed, do my symptoms seem to point more toward limited or diffuse (assuming I will get a diagnosis.).

The timeframe and all.

Would diffuse be quicker meaning Raynauds, heartburn all at once!

Would I have seem some skin hardening or tightness by now being diffuse is so rapid?

How rapid is fast progression when referring to diffuse.

ANY info,

Many thanks.

#4 Shelley Ensz

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Posted 17 August 2015 - 11:23 AM

Hi Kimberly,

 

Welcome to Sclero Forums. I'm sorry you are sick and send my best wishes to you.

 

When they are referring to rapid onset of diffuse scleroderma, they are talking about a time period of weeks or perhaps a few months. It is very rare, but some people can get scleroderma and progress all the way to severe sclerodactyly within a few weeks of their initial symptoms.  People with rapid onset are most likely to get diagnosed quickly, because they display all the classic symptoms so fast, and the progression is so drastic and alarming that they often get referrals to a specialist sooner. Whereas people with slower onset with more insideous symptoms (puffy fingers instead of frozen ones, for example) are likely to linger in their primary care or rheumatologist's office for many years before scleroderma is ever seriously considered, by either the patient or the doctor.

 

If you're being evaluated by a scleroderma expert on a consistent, ongoing basis you have very little to worry about, because if anything speeds up or gets worse, you will know right away. The most difficult thing for you may be to realize that a good 50% of people with UCTD never get a more definite diagnosis than that. UCTD is a diagnosis in and of itself.  You can have symptoms of a variety of connective tissue diseases without ever getting the full blown version of any of them.

 

So one thing to do is to try to relax. Your illness might be as bad as it is ever going to get. It may even clear up on its own accord, and that stands true even it if is early scleroderma, because scleroderma can wax and wane even without any treatment at all.  UCTD is a form of arthritis, so you can tell people you have "arthritis" or mild or moderate arthritis or whatever term you think is most accurate at the moment.

 

I mention this because you are in an awkward situation and many people you know will want to know the results of each of your doctor visits. With UCTD, your doctor is likely to discuss lupus at one visit, scleroderma at another, maybe call up ghosts of many other autoimmune diseases depending on how your symptoms change and develop and even clear up.  It is crazy-making for you and your friends and family, and very shortly they will all be looking at you as though you are a hypochondriac, thinking that, of course, if you REALLY had any disease, the doctor would surely have diagnosed it by now!

 

Unfortunately that is simply seldom true in rheumatic diseases. But if you tell everyone you have arthritis, and that there are over 100 types of arthritis so doctors are debating on exactly which one you have or what combination, and likely will be debating the particulars for years, then people get off your case. That's because everyone knows someone with arthritis!  Its a term they get, but they do not get UCTD, and you'll feel personal pressure to get that "undifferentiated" differentiated in one big hurry, even though that is NOT the normal course of that disease!

 

I know it is hard as all get out, but try to think of yourself as already being accurately diagnosed. UCTD is a diagnosis. So is arthritis. You have it. You may always have just exactly that, and it is NO LESS a disease than any other form of arthritis or even scleroderma. People with UCTD can be sicker than people with milder forms of scleroderma or lupus, and vice versa. After all, the entire range of autoimmune symptoms are open to you, whereas with specialized diseases, there is only a certain subset of symptoms that come into play.

 

Also, you are welcome and entitled to go to any support group that primarily deals with your current most pressing symptoms at the time. That means you can settle right in here, and hold your head high. You are a real person with a real diagnosis and nothing to apologize for if your diagnosis remains the same for the next 50 years.

 

Please also see our resources including a great video by our wonderful Amanda Thorpe on the subject of Difficult Diagnosis.


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.

#5 Kimberlygolden

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Posted 17 August 2015 - 12:03 PM

Wow Shelley. Thank you for all that! I surely appreciate your time and thought in that post.
The specialist I am seeing called it UCTD but as soon as I mentioned the constant heartburn that just started a few weeks ago, he seemed alarmed and I knew it.
We spoke about this presenting more specifically as scleroderma and he explained that lupus will " almost always" present with a positive ANA whereas 10 percent of people with sclero will be negative.
You hit it right on the nail with trying to explain UCTD to anyone. Family included. I no longer speak about ANY doctor appointment because as you said, even my own husband will say " oh no, not this again! Stop it! You are fine! The doctor didn't give you a diagnoses of sclero so you obviously have nothing"
I just don't mention anything about my health. Funny you said what you did...... I guess it must be common.
Getting back to the UCTD, the doctor and I spoke and he already feels if it does differentiate, it will be sclero and not any other CTD so with that said, it may sound nuts but I would rather know it is not diffuse as limited has a better prognosis.
I don't really want to hear ( all due respect) that limited CAN be just as bad as diffuse. I do understand that as well but I have small children that need me so understand where I am coming from please.
That's what brought me here- I figured everyone who has been diagnosed would have some idea as to whether my progression is likely to be diffuse or likely to be limited. I don't have any of the CREST except for Raynauds and heartburn so I wonder if it will end up being the diffuse form. I also do not get terrible Raynauds or many episodes so again, I feel it may end up being diffuse.

As I understand, diffuse happens in weeks or months?

Would I have seen the skin manifestations by now?

Thanks so much to you both for replying.

Kindly,
Kim



#6 Shelley Ensz

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Posted 18 August 2015 - 07:39 PM

Hi Kimberly,

Since you are having a lingering onset of something, you would absolutely not have the worst form of rapid onset diffuse scleroderma. It's begging the question, of course, to worry about the subtype when the main type of disease has yet to be diagnosed.

And, no type is scleroderma is really preferential. Diffuse scleroderma often gets diagnosed faster, which is its own form of relief. And limited scleroderma can have fatal complications early on, sometimes.

So I get it that you don't want diffuse. Nobody wants that. You want to live a long time. Join the crowd. The problem is though that you might be run over by a truck tomorrow, or you might thwart all predictions and live to 120, out living your children and all the rest of us.

You see, the primary problem is that none of us have ultimate control, and wishing and hoping or dreading and worrying won't change that for a minute.

So my question to you is, what are you doing today to savor and cherish this time with your husband and children? How can you make them laugh? What special treat can you surprise them with? How can you create a special memory today?

The sooner we ask questions like that, and find answers, the sooner we start healing emotionally, which is the most important project of all, and it's something that can begin now and not some nebulous day in the future when we might be diagnosed.

Here are some warm hugs to tide you over, along with a reminder that we are all better off living the moment we have right now, because it us the only one we have for certain.
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.

#7 Kimberlygolden

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Posted 20 August 2015 - 03:15 AM

Hi Shelley,

Thank you. You are 100 percent right and very compassionate in your words. I thank you for that.

In the end, we have no choice but I am one of preparation. I work better and deal easier with situations when I am not surprised.

Is the rapid onset diffuse that you meantioned the same as diffuse or is that an even more "faster onset" than diffuse ?

Thank you again.

May I ask, what form you have and how long?
Would you mind sharing your story.

#8 Shelley Ensz

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Posted 20 August 2015 - 04:12 AM

Hi Kimberly,

I am 100% with you in terms of trying to prepare for life's challenges. I am always looking ahead, trying to tackle the next challenge.

What took me the longest time to get, was that the illness itself does not matter. What really and truly matters is how we cope with it logically and emotionally. How do we find the wherewithal to love and share joy and happiness, when our own existence is threatened, either directly or by our own often overworked imagination?

By fortifying ourselves by developing our physical and emotional resilience, we are able to make the most of our life and talents. So let's say you do have the very worst form of scleroderma and that your days are very numbered.

What then? On your deathbed, in just a few months let's say, what would you regret? Would you regret not having invested enough time in worrying about scleroderma? Would you regret not having watched enough tv? Would you regret not having made a will? Or would you most regret being so worried that you forgot to hug your child, or tuck a love note into your spouses lunch, or draw wild lipstick hearts on the bathroom mirror.

I totally get it that you are trying to look into the scary future and brace yourself for it. We are all in that same boat around here. But "it is what it is" and neither wishing nor dreading any particular diagnosis is going to alter the course of the disease. However, learning skills for coping with stress may actually alter the course of the disease, because certain types of stress have an adverse impact on the immune system.

That's how, and why, it's good to let go of prognosis questions until you have a more certain diagnosis. It is a mind trap that most of us wish we hadn't fallen into. And trust me, I'd urge you to keep on worrying about it, if it would be of any possible benefit you. However you have already covered your bases by having this support group and seeing an expert, so it is okay to relax your vigilance now, in regard to prognosis. Provided, of course, that you live today so you have no regrets tomorrow.
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.

#9 Shelley Ensz

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Posted 21 August 2015 - 08:58 AM

Hi Kimberly,

 

I apologize I got a little sidetracked on my last email. The primary difference between limited and diffuse systemic sclerosis is that in diffuse, the skin tightness extends above the wrists or elbows, or above the ankles or knees (depending on which definition your doctor uses.)  I think the most common definition is probably that skin involvement above the elbows or knees constitutes diffuse scleroderma. 

 

Other than that, both limited and diffuse can affect any part of the body, including all the internal organs. Overall, limited has a better prognosis than diffuse, but there are significant exceptions, because there is a greater likelihood of pulmonary hypertension in limited.  So a person with moderate symptoms of diffuse could easily outlive a person with severe symptoms of limited. And there is no automatic progression of either type. It is very possible for symptoms to stabilize and often even improve, for months or even years at a stretch. The oldest person we're aware of who died with systemic scleroderma was in her mid-90's. One of our volunteers had systemic scleroderma for a full 50 years before she passed away, in her 60's. 

 

So, it is entirely impossible to speculate on what version of scleroderma you have, when you haven't yet been diagnosed with scleroderma, and don't yet have any of the telltale skin involvement, nailfold clues, or specific antibodies. Having mild Raynaud's is an excellent thing, no matter whether you have limited or diffuse, but it doesn't specifically point to one or the other.  Right now, your question is impossible for your doctor to answer, and particularly impossible for us, with no medical training at all, to answer.

 

You are asking for a crystal ball, and unfortunately, we are in exceedingly short supply of those around here. You appear to be experiencing a very slow onset of something. You are under excellent surveillance. Please keep in mind that the process of surveillance does not guarantee that you will eventually have any more severe illness ever diagnosed.  I know people who have been under observation for connective tissue diseases for up to 20 years, who were then given the "all clear".  Meaning, their doctors pronounced them entirely healthy! 

 

Can you even imagine the stress they suffered for so long? Or how many of their holidays were filled with dread that it may be their last?  And, it was all entirely wasted, and suddenly they need to prepare for an old age they were sure they would never see. The thing is, this happens frequently, with surveillance for possible cancers and autoimmune diseases. So I need to answer not only aimed at you, in particular, but also at the hundreds of people who will stumble on this thread with their own worries, who are coping with trying to find a way to put them into perspective.

 

I would like to tell all those people to garner as many facts on their potential illness as they can. Try to absorb the situation but not blow it out of proportion, either. And if they still can't get their head around the fact that their job is to live optimistically (their doctor's suspicions might be wrong, they often are!) after about two weeks of dwelling on it; if they find the question dragging them down or impairing their life or spirits in any way, then they should seek counseling.  Not because they are mentally ill or hypochondriacs!  But rather so they can learn additional coping skills, because uncertainty is going to be a major factor in their life. It could precipitate anxiety and depression that could be more life threatening than any cancer or autoimmune disease.

 

People who are diagnosed often suffer from what is called Fear of Progression. Unfortunately, it can even happen to someone who is not yet diagnosed.  Education on the topic and professional support can help to assuage that, and in some cases, maybe even avoid it.  I don't want you, or anyone reading this to fall into that black hole.  Life either with or without any illness can be pumped full of joy and happiness and satisfaction, when we have the tools and skills and training for it.

 

Therefore, I'm wishing you, and everyone reading this, more joy and gratitude and savoring of life today, as it is, in this moment. Because we can control our thoughts in this moment (and thus influence our emotions), but none of us can control the future, whether by hoping or by dreading. Here are some warm hugs to tide you, and everyone else, over, in this very moment.

 

:hug-bear: :hug-bear: :hug-bear:


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.

#10 Kimberlygolden

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Posted 21 August 2015 - 01:30 PM

Thank you, Shelley.

I truly appreciate all the time you took to respond to me. I don't have enough words for Your compassion so thank you here, goes a long way.

Kim



#11 greypilgrim256

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Posted 24 August 2015 - 12:45 PM

Hi Kim.  Did your ANA come back positive at all?  Not the sclero antibodies, but the ANA?

 

I also noticed you weren't tested for the U3RNP antibody and the Th/To antibody. 



#12 Kimberlygolden

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Posted 25 August 2015 - 11:27 AM

Hi Grey Pilgrim,

My ANA has been on a few occasions negative.

 

They've been done by IFA ( the best method too) but as for the other two sclero antibodies, they have not been done.

 

Do they typically run all of them?

Thanks for reading.