Jump to content


Biomarker for Diffuse Scleroderma skin has been discovered!


Photo

New diagnosis. how advanced is my disease


  • Please log in to reply
19 replies to this topic

#1 Morgan

Morgan

    Bronze Member

  • Members
  • PipPip
  • 11 posts

Posted 24 June 2009 - 05:26 AM

Hello everyone.

I am newly diagnosed, this week. I can say by my symptoms I have had this disease for many years. I have recently had pleurisy several times and have other symptoms that to me are severe. Besides my dry eyes and mouth, fatigue, and chronic pain, I am concerned about how advanced my disease might be. My doctor does want to start Plaquenil this week. I guess what I need to know is what tests or questions should I be asking in order to know if I am starting on the right treatment path. Has this caused harm to my body that I am unaware of. How would I know. Any suggestions would be helpful.

#2 TJ903

TJ903

    Silver Member

  • Members
  • PipPipPipPip
  • 109 posts
  • Location:Merritt Island, Florida

Posted 24 June 2009 - 07:05 AM

Sorry to hear about your situation, but you have come to the right place. Not only will you find a wealth of valuable information, but you will get a wealth of love and support. We know or have been where you are now and those of us who were we you are now will tell you, not to overload on researching. You will tend to fixate on the bad and the stress will not be good for you.

Find a Rhuematologist that you are comfortable with and one that will listen to you and address your concerns. You will need to be an informed patient as well. Gather your support team around you and take charge.

I have been there and obsessed over the outcome rather than finding a good doctor who understood this disease and the different ways it presents itself. I am now in my third year and still taking charge. Everyone on this site you can count as a friend and don't be embarassed to unload. Many times I've needed extra support and virtual hugs and always found them here.

I'm sending warm, gentle hugs your way and would consider it an honor to be one of the first members of your sclero.org buddies.

TJ903

#3 Amanda Thorpe

Amanda Thorpe

    Platinum Plus Member

  • ISN Senior Support Specialist
  • PipPipPipPipPipPipPipPipPipPipPip
  • 2,927 posts
  • Location:U.K.

Posted 24 June 2009 - 09:24 AM

Hello Morgan

Welcome to this forum!

You've already been given sound advice by TJ903, you need a rheumatologist knowledgeable in scleroderma and hopefully one you can have a good rapport with as it's important to engage in you treatment process.

Usually there are various conducted such as lung function test, CT scan, ECHO, blood tests etc basically to get an idea how your internal organs are functioning and whether there are any scleroderma induced changes. Usually the tests are conducted then a course of treatment decided upon taking into account test findings and symptoms.

I am not familiar with plaquenil but we do have a page about it here on this site that may be of use to you. There are a number of drug options and some of us, such as myself, end up on immunosuppressants and again we have information about these.

You'll want to talk to your doctor/rheumatologist about what tests you need and thereafter what treatment is best for you remembering that you're fully entitled to partake in this discourse, asking whatever questions you have and expecting answers.

Please also take a look at out page on emotional adjustment, it's one thing to live with symptoms but quite different when they are named as a specific disease.

Finally make yourself at home here, post your questions and source your scleroderma information right here.

Take care.
Amanda Thorpe
ISN Sclero Forums Senior Support Specialist
ISN Video Presentations Manager
ISN Blogger

(Retired) ISN Sclero Forums Assistant Manager

(Retired) ISN Email Support Specialist
International Scleroderma Network (ISN)

#4 nan

nan

    Gold Member

  • Members
  • PipPipPipPipPipPip
  • 649 posts

Posted 24 June 2009 - 10:11 AM

Welcome Morgan! Did you doctor let you know what type of scleroderma you have, limited or diffuse? I do know that scleroderma affects each person in a different way. You have definitely come to a great place for support. It looks like Amanda gave you great sites to look at.
Take care,
Nan

#5 Sheryl

Sheryl

    Platinum Member

  • ISN Support Specialists
  • PipPipPipPipPipPipPipPipPipPip
  • 1,029 posts
  • Location:Brooklyn, Michigan

Posted 24 June 2009 - 11:31 AM

Hello and welcome to International Scleroderma Organization forums Morgan. Many of us on the forum go to a rheumatologist whom specializes in Scleroderma or other autoimmune diseases. A couple of the tests that I was first given were a PFT (pulmonary function test). Checking your airways and lung capacities looking for interstitial lung disease or other breathing conditions. Another test might be an EKG looking for any heart irregularities. If you have acid reflux you may have an endoscopy done to get a base line study of how your esophagus looks today and what changes are occurring over the next several years. If you are having any bowel issues a colonoscopy is also preformed. If you have Raynaud's then some doctors check the capillaries in your fingernail beds to see the pattern or disruptions and can prescribe medicine to help with poor circulation or the Raynaud's attacks. I hope this little bit of information helps you out. Ask your questions and we will do our best to direct you to the proper information or tell you how something has affected us. You have come to the right place to seek support.
Strength and Warmth,
Sheryl

Sheryl Doom
ISN Support Specialist
(Retired) ISN Chat Moderator
International Scleroderma Network (ISN)

#6 Morgan

Morgan

    Bronze Member

  • Topic Starter
  • Members
  • PipPip
  • 11 posts

Posted 24 June 2009 - 12:37 PM

Thanks for taking the time to answer my questions. I guess I should say that I am a nurse and because of this the rheumatologist just assumes that I know more than I do. As I said we already suspected that I had Sjogrens and possibly lupus (SLE) before my lip biopsy last week. On Tuesday he calls me with the results and a prescription for the medications (which he did give me a choice to start or not). I have been as you said doing a lot of research and putting the pieces together since then. Knowing what I do know from my nursing, I am worried that I might be further in this disease process than we know. The doctor since last week has not talked to me about all of these questions that I now have. I am 38 years old have three children, one daughter. I have questions about genetics, the disease process, my pain control. I guess maybe I am overwhelmed also. I have been looking for someone who can relate to my illness. Once again thanks for everyone's support.

#7 Jeannie McClelland

Jeannie McClelland

    Senior Gold Member

  • ISN Support Specialists
  • PipPipPipPipPipPipPipPipPipPip
  • 1,706 posts
  • Location:in the Rocky Mountains of the USA

Posted 24 June 2009 - 02:40 PM

Hi Morgan,

Let me add my sincere welcome and a warm hug. You'll get bunches of those if you stick with us. We're a real huggy bunch - great medicine, no side effects, and all insurance plans cover a good hug.

OK, just for general drill, let me give you an idea of what my work-up was and what the diagnosis and progression has been.

I had my initial appointment with a pulmonologist at a center of excellence and 3-1/2 months later saw the rheumatologist and received the diagnoses of systemic scleroderma sine scleroderma, pulmonary hypertension, pulmonary fibrosis, and esophageal involvement, plus Sjögrens and Raynaud's. That was just about 3 years ago.

The initial work-up consisted of all the usual blood and urine tests. I had chest X-rays, extended Chest CT, bronchial provocation test, laryngoscopy, PFT's, nuclear diffusion test, arterial blood gases, 6-minute walk, 'max ex' exercise testing, 24-hr pH study, diffusion testing, allergy testing, CT of sinuses, esophagram, tailored barium study, echocardiogram and bubble echocardiogram. A bit later they did a right heart catheterization.

You can see where they were going with all this, but since I presented with shortness of breath and a persistent non-productive cough and was challenging a diagnosis from elsewhere of asthma, it was definitely the right course to take.

Not every patient warrants all these studies and even most people who do will have them over a longer period of time. I was very, very lucky to fetch up at a research center with wonderful doctors and absolutely amazing in-house testing facilities.

Progression and prognosis. I've had worsening Sjögren's and Raynaud's, but am essentially stable with regard to the PH and PF, thanks to a good response to medications. I think I'm starting to show some skin hardening and stiffening in my hands. All in all, I'm doing great, though, proving once again that statistics can't be reliably applied to an individual.

Now, for your big concern: "I am worried that I might be further in this disease process than we know." The current biggies that affect prognosis are in this order (I'm pretty sure I got this right) pulmonary hypertension, pulmonary fibrosis, renal involvement (especially renal crisis), and GI involvement. I'd think any symptoms pointing at any of those would warrant further investigation. One really reassuring thing is that every test imaginable is now very sensitive. Think about the huge advantage a CT has over a chest X-ray. ;) Some baseline screening might be a good idea and you may have already had that.

Pain and fatigue - I take Plaquenil for that and also to bolster the other immunosuppressant I'm on. Actually, I take Plaquenil, an anti-inflammatory, and a muscle relaxant and wowee! That combo has helped enormously. You've already been given a link to Plaquenil. It was one of the few medications I had no qualms at all about taking. Steroids are often prescribed but can be hazardous to us - they are known to sometimes provoke a renal crisis.

Genetics. There are some studies that point to a higher incidence in certain racial, ethnic, or geographic areas. There is also a very rare familial type, but generally speaking, your kids probably won't get it, at least that is my understanding. There is a tendency for some families to have a variety of autoimmune diseases, as you'll see if you read some of the personal stories and past Forum posts. Also some people will have more than one autoimmune disease. I gave a blood donation to a DNA registry and the coordinator I spoke to said they were thinking along the lines of having to have a combination of certain genes plus an environmental trigger. Every additional requisite for developing the disease would lessen the chances of our children getting it, and as a mother of four, that's good news.

Hmmm, what else can I say? Well, it's always useful to repeat each and every person has a different experience with the disease. There is much research being done now, more than ever before. The treatments for the complications are getting better and better and also more available.

My best advice? Don't develop cyberchondria! I did and nearly scared myself to death at first. Temper what you read with what you know, especially what you know about yourself.

So, there you are. I don't know if any of that was helpful to you. You are in the worst phase of the disease right now - newly diagnosed.

Warmest of wishes and another big hug.
Jeannie McClelland
(Retired) ISN Director of Support Services
(Retired) ISN Sclero Forums Manager
(Retired) ISN Blog Manager
(Retired) ISN Assistant News Guide
(Retired) ISN Artist
International Scleroderma Network

#8 Sweet

Sweet

    Platinum Member

  • ISN Support Specialists
  • PipPipPipPipPipPipPipPipPipPipPip
  • 2,892 posts

Posted 25 June 2009 - 05:10 AM

Hi Morgan, and welcome!

I'm a bit late in chiming in here. It looks like you've received some great links and answers already.
I've been diagnosed for 9 years now. I also am a nurse. I have to say however, that as a nurse when it comes to our OWN bodies, we are sometimes not the best judge, because there are too many emotions there! I've on more than a million occasions, jumped to the wrong conclusions and then am reassured by my doctors that whatever I was worried about is nothing to worry about. Knowing I'm a nurse, they don't just tell me that, they do tests etc. to alleviate my anxiety. Although, I've been right a few times too.

Try to take one day at a time, one step at a time, tell your doctors everything you are feeling, write it down in a journal so you are prepared for your next visit and then take it from there.
Warm and gentle hugs,

Pamela
ISN Support Specialist
International Scleroderma Network (ISN)

#9 debonair susie

debonair susie

    Platinum Member

  • ISN Support Specialists
  • PipPipPipPipPipPipPipPipPipPip
  • 1,638 posts
  • Location:(United States)

Posted 25 June 2009 - 05:41 AM

Hi Morgan,

I'm also glad you've found "our family"!

I may have missed it, but before you go to your rheumatologist appointment, have all of the questions written down, so you don't miss asking anything.

Also, if possible, it helps to have an extra pair of ears and another pen to take notes. If you and another take shorthand...all the better!

I'm sure most of us can appreciate what you are feeling right now. Just please remember...once you get some answers to your questions, it will alleviate some of the anxiety you are feeling.

Knowledge is Power and being your own advocate should be a natural for you!

Keep us posted!
Special Hugs,

Susie Kraft
ISN Support Specialist
ISN Chat Host
International Scleroderma Network (ISN)

#10 Margaret

Margaret

    Platinum Member

  • Members
  • PipPipPipPipPipPipPipPipPipPip
  • 1,001 posts
  • Location:Pennsylvania

Posted 25 June 2009 - 08:05 AM

<<Don't develop cyberchondria>>

Jeannie ,

Oh MY.....a new word!!! I love it!!! I was so overwhelmed when Gareth was first diagnosed that I had a horrible case of cyberchondria --- for him!!!

Take care, Everyone.

Margaret

#11 Snowbird

Snowbird

    Platinum Member

  • Members
  • PipPipPipPipPipPipPipPipPipPip
  • 1,008 posts
  • Location:Canada

Posted 25 June 2009 - 02:29 PM

Hi Morgan

Just wanted to chime in and say hello as well. You've received excellent advice already which is the norm here...the only advice I'de like to offer just now is a recommendation that you do not read everything on the net...not good for you. You have found the place you need to ask your questions and to get factual information. :)
Sending good wishes your way!

#12 Shelley Ensz

Shelley Ensz

    Root Administrator

  • ISN Root Admin
  • PipPipPipPipPipPipPipPipPipPipPipPip
  • 3,274 posts
  • Location:Minnesota

Posted 26 June 2009 - 01:41 PM

Hi Morgan,

You've already received some terrific advice and made some new friends here! So I just want to say welcome to Sclero Forums and send some nice great big warm hugs your way.
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.

#13 epasen

epasen

    Silver Member

  • Members
  • PipPipPipPip
  • 233 posts
  • Location:Lohja, Finland

Posted 26 June 2009 - 07:11 PM

Morgan hi,

welcome. :) I bet you've already seen how willing these people in here are to help you. I also want to say that you've come to a right place.

At first I have to say that I don't have systemic form of this disease and I'm no expert but since I've been dealing with this since I was 6 years old I don't panic anymore. And when you've stopped panicking you can start thinking with a common sense.

The best way to communicate with doctors is to ask directly. They normally don't know the answer if they're not scleroderma specialist so you can always be a little sceptic, but this might give you some very good guidelines. Ask whatever questions you have, don't assume that they assume you to know everything. I've noticed that doctors don't tell anything to me either, and I'm just a 19-year-old girl, not a medical professional.

Secondly, you don't have to know what kind of tests to order. You can simply just write down a list of your symptoms and "weird feelings". Then clearly express your concerns to your doctor, and if she/he is wise enough she/he will order the tests needed according to your symptoms.

You sound like a person who's a "doer": You always do everything yourself in an order to get the best result, just like me, haha. :) But what I've noticed while living with scleroderma is that you can not always do everything yourself. You can search for information, you can learn and that is the best way to get familiar with your disease and yourself. BUT at times you have to let others to take care of you. You have a permission to be scared and confused. You have a right to get the answers you want from your doctor. But remember to breath, relax and smile every once in a while. :)

Take care,
Emmi

#14 mando621

mando621

    Silver Member

  • Members
  • PipPipPipPip
  • 212 posts
  • Location:Wisconsin

Posted 27 June 2009 - 02:22 AM

Hi Morgan,

Welcome to the forum.

I've been going through the process of trying to identify what is going on with me for about 6 years. Just lately, the rheumatologist has ordered lots of the tests for baselines on echocardiogram, pft, skin biopsy, and blood work. He did note that I have abnormal nailbed capillaries and I have dysmotility of the lower esophagus.

I have suspected Sjogren's for some time now but I haven't had the lip biopsy. My blood work doesn't come up postivie for SSA or SSB but I have extremely dry eyes, mouth, etc. Shirmer's test just done last week showed less than 3 mm of moisture after 5 minutes with drops for numbing the eye. The nurse that took the strips out threw them away but it looked like they weren't even a bit wet to me. The doctor came in and was disgusted that she had thrown them away since he wanted to see it and keep for evidence.

Did you get diagnosed from the lip biopsy? or were there other results like blood work that gave you your sclero diagnosis? I have refused to have the lip biopsy at this time. Seems like it International Scleroderma Organization't the gold standard for diagnosis if they miss a salivary gland.

I'm at a point where I don't have a definite diagnosis, mostly just "connective tissue disorder". I find that I have very conflicting emotions about all this. I'm glad that the rheumatologist. has decided that I have a valid point about things not being right with me, now I just have to find a way to figure out how to deal with the fact that it is all "real" now.

I recently had a life insurance policy application rejected due to dysmotility of esophagus. (only took it out for the auto/home discount and for kicks to see if it would go through). I wasn't suprised that it didn't go through, but I'm frustrated that I don't have a clear diagnosis and yet I am not a good candidate for insurance. Seems like a contradiction to me.

I am not taking any meds, just flax seed oil, Vit. D, and a baby aspirin daily. I'm experiencing more joint pain especially the hips, more hand swelling, and a very tight calf/behind the knee tendon thing on my right leg. I'm not really looking forward to fall and the start of classes, I know I'll be hit with major fatigue again.

My GI doctor and eye doctor both think it looks like sclero and asked me lots of questions for personal reasons. GI doctor has a sister going through something like this, and the eye doctor knows someone in CA with same troubles. It feels a little like a nightmare coming true at this point. I've done way too much research and have gone through the cyberchondria stage, which I think initially turned off the rheumatologist.

Sorry this is all about me. I am with you on having lots of questions, doubts, and fears. I am sending lots of warm thoughts your way. I don't see the rheumatologist again until August, so it is wait and see some more.

Mando.

#15 Morgan

Morgan

    Bronze Member

  • Topic Starter
  • Members
  • PipPip
  • 11 posts

Posted 29 June 2009 - 04:42 AM

I had a lip biopsy to confirm it showed chronic disease. The doctor doing the biopsy said he had never seen such large minor salivary glands! Of course being a nurse I did watch and he explained what they normally look like even when they are positive. He and I work together so he did speculate that day that the test would come back abnormal and two days later it did.

I also had put the biopsy off for longer that I should have but I have been having so many other symptoms that we could not put anywhere. Finally I gave in. I have recently had a endoscopy and colonoscopy.

It is funny that you say have your heart checked out. I took everyone's advice and slowed down on the research. I did not get to the part about the heart yet. Well last night I ended up in the ER with a very rapid heart rate it lasted over an hour. As I think I told you I have had trouble with pleurisy. I think that in the last week I have been developing another case (chest pain). I now have questions to anyone, just how can this effect my heart? Thanks.

#16 Morgan

Morgan

    Bronze Member

  • Topic Starter
  • Members
  • PipPip
  • 11 posts

Posted 29 June 2009 - 06:11 AM

I just got my doctor to order a pulmonary function test (PFT) and a Holter monitor. I am still wondering if I need an echocardiogram and a stress test? I think that the doctors think that I am just too young and know too much and they do not want to order any of these tests on me. Any suggestions? Thanks again.

#17 Jeannie McClelland

Jeannie McClelland

    Senior Gold Member

  • ISN Support Specialists
  • PipPipPipPipPipPipPipPipPipPip
  • 1,706 posts
  • Location:in the Rocky Mountains of the USA

Posted 29 June 2009 - 06:57 AM

Hi Morgan,

We have a really great page on Cardiac (Heart) Involvement in Systemic Sclerosis (Scleroderma). There are articles that explain the various ways the heart can be affected, symptoms, diagnostic methods, treatment, and so on.

I think doctors are reluctant to order expensive tests unless they see some sort of clinical indication warranting further investigation. I recently was going through my health insurance records on-line and I was astounded at the amount billed to them for what seemed like fairly simple tests. It also sometimes depends on whether or not a doctor has seen a particular complication in his/her practice often enough to be a bit more cautious than others might be.

I forget what you said your age is (38?), but at a certain point, things like echocardiograms and stress tests become a routine screening thing, like the colonoscopies. (Yechhhh! :blink: ) You'd be early for that, but maybe in view of your diagnosis you can push for the tests you want on the grounds of getting a baseline. (I ought to mention that a routine stress test didn't pick up my pulmonary hypertension.)

And just for a laugh, I'll also mention that the chest pain that I was sure was indicative of a heart problem (2 of the 4 family members of my generation have had serious heart problems and one recently died - he was 2 years younger than me!), turned out to be esophageal spasm due mostly to GERD. :D

Best wishes,
Jeannie McClelland
(Retired) ISN Director of Support Services
(Retired) ISN Sclero Forums Manager
(Retired) ISN Blog Manager
(Retired) ISN Assistant News Guide
(Retired) ISN Artist
International Scleroderma Network

#18 Margaret

Margaret

    Platinum Member

  • Members
  • PipPipPipPipPipPipPipPipPipPip
  • 1,001 posts
  • Location:Pennsylvania

Posted 29 June 2009 - 02:50 PM

<<I think that the doctors think that I am just too young and know too much and they do not want to order any of these tests on me. Any suggestions? >>

Hi Morgan ,

Well, you can tell them that you have met people much younger than you and they have the Sclero diagnosis. My son was only 18 years old when first diagnosed with sine sclero. Now it's UCTD. Emmi is young, too, and in a university (right?!?!?). It's always good to have a baseline of all studies in the beginning. That's the only way to know the progression of your disease.

Take care, Everyone.
Margaret

#19 Morgan

Morgan

    Bronze Member

  • Topic Starter
  • Members
  • PipPip
  • 11 posts

Posted 03 July 2009 - 11:52 AM

Thanks for sending me the info. So how did they diagnose your PH? If it did not show up on basic stress testing did you have a heart cath?

#20 Jeannie McClelland

Jeannie McClelland

    Senior Gold Member

  • ISN Support Specialists
  • PipPipPipPipPipPipPipPipPipPip
  • 1,706 posts
  • Location:in the Rocky Mountains of the USA

Posted 03 July 2009 - 05:55 PM

Hi Morgan,

They suspected pulmonary hypertension as a result of an echo, followed by a bubble echo, plus a thing called a VO2 Max Exercise Test. I was on a treadmill, fitted with a respirator, blood pressure cuff, heart monitor, and a port for arterial blood gas draws. The results looked fairly scary so they sent me to a pulmonary hypertension specialist who then ordered a right heart catheterization (RHC). The great thing about the RHC was that my numbers actually looked better than they had with just the echocardiogram. They follow me with routine echocardiograms on a regular basis and I am being treated with Letairis, which seems to be keeping me nice and stable.

One of the hallmarks of pulmonary hypertension is apparently a fairly sharp rise in heart rate and a drop in O2 levels with exercise and a quick recovery 'at rest'. That's certainly true in my case.

I have to tell you I was scared to death about the RHC, but it turned out to be easier than that Max-Ex test! :lol:
Jeannie McClelland
(Retired) ISN Director of Support Services
(Retired) ISN Sclero Forums Manager
(Retired) ISN Blog Manager
(Retired) ISN Assistant News Guide
(Retired) ISN Artist
International Scleroderma Network