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Shelley Ensz

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Posts posted by Shelley Ensz

  1. Hi Mare,


    I am delighted that you were able to consult a scleroderma expert.  I know what it is like to wait for test results, sometimes it feels like half my life has been spent waiting for one result or another!


    This might be a good time to read through forum archives of members who have been waiting for the results of this test or that, or this diagnosis or that, over the years, as many helpful hints for dealing with this phase are lounging about. Most of us will say that the time between onset of symptoms and diagnosis was the absolute worst time for us ever in dealing with the disease. By comparison, it's a piece of cake to deal with any eventual diagnosis.


    I just need to warn you that it is an average of six years from onset to diagnosis of scleroderma.  Sometimes the process is speeded up a bit by seeing an expert right away (which is why we strongly recommend it whenever there is good suspicion of scleroderma).  BUT the disease itself can onset very slowly, can be similar to a great many other illnesses, and can take years and sometimes even decades to firmly declare itself.  It hardly ever happens for a person to see an expert once and then voila, have a diagnosis of scleroderma. It is much more normal for someone to slog through years and years of tests and follow-ups and differing opinions and just general mass confusion.


    I mention this in hopes that this tampers your expectations, both for yourself and for your rheumatologist. They cannot create a diagnosis out of thin air, even when they strongly suspect one. It is just as much a waiting game for them as it is for us. And if their response right now is that it is too soon to tell precisely what you have, please do not be discouraged.


    Many of us here know how it is to be terribly ill, to want and need immediate answers, and to be frustrated at every turn in the road. But in those cases, neither the patient nor the doctor are at fault, but rather those extremely pesky autoimmune diseases which are often extremely challenging to diagnose. I'm not saying you won't get a diagnosis, but only that it would actually be pretty unusual if you get firm answers, this soon after onset.



  2. Hi Stephen,


    Please keep in mind that I am not a doctor and have no medical training at all, and that we cannot diagnose or rule out any condition on our forums. So I'll only give my best guesses and ask that you rely on your doctors for actually figuring out your present health situation, of course.


    As it happens, the systemic types of scleroderma do not cause raised patches or local clusters of bumps, a bit like insect bites.  Rather, the skin changes are colorless and affect both sides of the body at the same time, for example, a colorless skin hardening beginning in the fingertips and going up the hands.


    Morphea scleroderma, which is a form of localized scleroderma, occurs in colored patches of skin, and not matching on both sides of the body. Usually the plaques are reddish with a lighter colored center. Bumps like an insect bite are not a normal description for morphea. Generally, a dermatologist will biopsy unidentifiable skin lesions, and together with the blood tests that might help figure out what is causing your symptoms. Your description -- only of the skin, not your other issues -- sounds a little bit more like eczema than any type of scleroderma. But, I may be wrong -- I often am! -- and of course we are back to that "not a doctor" reminder once again.


    It's possible that you may have several things wrong at once. That is quite common as we age, and although it may seem to "add up" to an underlying disease, sometimes the problem is really an accumulation of age-related issues or the systemic effects of other illnesses, like diabetes.


    You just won't know though until you have all the testing done. Please stay in touch and let us know how things turn out. Please remember that we are always here for you, regardless whether your diagnosis is scleroderma, or not.



  3. Hi Grey,


    UVA1 phototherapy is done in a dermatology office that has UVA1 phototherapy equipment. It is like a vertical tanning booth, and the major side effect is that you get a sun tan. You wear equipment to protect your eyes and/or face, and minimal clothing. The sessions take literally just a few seconds and are entirely painless.


    Normally people are prescribed a certain series of treatments, such as, 2 or 3 per week for 3 months. Then the treatments are assessed and regular skin surveillance is done by the dermatologist. It has been proven to be effective for morphea scleroderma (also other ailments like lupus and psoriasis) and there are reports that it has been helpful for some people with systemic scleroderma, especially with skin involvement. The biggest trick is finding a dermatology office that has the right equipment, so it is usually found in larger metropolitan centers.


    Please review this information with your scleroderma expert to see if it might be appropriate or worth a try in your particular case. For example, some people are on medications that necessitate sun avoidance (like methotrexate).



  4. Hi Grey,


    It's very important to see your scleroderma expert and to also get occupational therapy at the first sign of any scleroderma hand involvement, even if all you are experiencing is finger puffiness and/or stiffness.


    It's actually fairly uncommon for things to progress all the way to frozen, curled hands in systemic scleroderma, especially if you receive proper care early on. I had a year of occupational therapy nearly two decades ago and resume the steps I was taught (measuring, warm wax treatments, and hand exercises) as needed. 


    My fingers still have varying puffiness and stiffness, but are still quite functional, with just some reduction in grip (making things like filling the gas tank or opening jars challenging or sometimes impossible.) There are varying results with UVA1 for sclerodactyly, but I have found UVA1 treatments particularly helpful, as well.


    Avoiding and promptly treating attacks of Raynaud's is thought to also help avoid or reduce disease progression in scleroderma, so becoming an absolute pro at avoiding attacks, and having an arsenal of ideas for responding to them promptly, may also help preserve scleroderma hands.


    With the occupational therapy, I had special splints made for daytime and nighttime. The point of them was to mold my hands into the most usable position, should the hand inflammation/fibrosis progress. Every once in a while, it feels like I should go back to using them again, but then things typically relent after a spell.


    I also get "trigger fingers" and that can go in spurts of being very troublesome for awhile, and then improving. For example, my fingers can quickly get stuck in a position, often when carrying a bag or trying to use a knife or scissors. When that happens, I merely stop what I'm doing and wait for it to relax (without ever trying to force it, which can be harmful). And I pay very close attention to ergonomics to reduce the impact of carpal tunnel (and in my case, ulnar tunnel.)


    The most important thing is to try to prevent scleroderma hand involvement, because once it has a good foothold, there is currently very little that can be done for it -- although there are some very hopeful clinical trials in progress right now, aimed specifically at this, such as the Cytori STAR trial.

  5. Hi Angelily,


    Welcome to Sclero Forums!  I'm sorry you have symptoms like that of Sjogren's syndrome, and that you need answers in that regard.


    As it happens, not everyone with Sjogren's expresses positive or definitive antibodies. Other tests that can be done in an effort to diagnose it include Schirmer's test, which is just a strip of paper put in the eye for a few minutes to stimulate tear production; sialogram (an x-ray of the parotid gland, with dye injected); and a lip biopsy. 


    I've had all those tests (I have Sjogren's.)  Given my experience, I'd recommend the Schirmer's first (very fast and easy and painless, typically done by an opthalmologist); then the lip biopsy (a simple in-office procedure and not as awful as it sounds (it is on the inside of the mouth, not on the outside of the lips); and then the sialogram, which is my least favorite of those tests.
    Many people don't find the additional testing worth it, as their symptoms might be fairly mild (considering) and treatment is often the same, with or without diagnosis (such as, eye drops for dry eyes, etc.)


    It's possible, of course, to have dryness problems caused by other things -- especially medication side effects -- without having the underlying disease. Please keep in touch and let us know how things develop for you.



  6. Hi Regalize,


    Welcome to Sclero Forums! 


    I'm sorry you have rapid onset diffuse scleroderma and that you are also encountering some medication issues. Unfortunately most treatments for very severe diseases also run the risk of being quite toxic, so it is a constant weighing of the risk versus benefit.


    Thank you for your interest in Resunab, and for mentioning it. I thought it might be helpful to explain it a bit more for others who are curious about it.


    As it happens, Resunab is just starting Phase 2 clinical trials in the U.S., to see if it might be effective, and safe, for diffuse scleroderma. Pre-clinical studies looked promising for reducing inflammation and fibrosis without suppressing the immune system.


    Unfortunately we have seen many potential treatments complete even phase 3, appearing to be quite positive, before being proven to be of no use for scleroderma. That is because the illness has a natural course of waxing and waning, even without any treatment at all, which usually causes great confusion, making treatments appear effective which in the end are shown have no effect at all. That's not to say this will happen with Resunab, it's just a disclaimer for scleroderma clinical trials in general.


    Although Resunab (by Corbus Pharmaceuticals) has been fast tracked by the FDA in the U.S., the clinical process still remains quite long, especially for those of us who are eager for an effective new treatment right now. Phase 2 for this trial just started recently, and is scheduled to take 18 months. Then Phase 3 clinical trials generally take about 3 years, after that.


    The FDA Drug Approval Process explains more. And we have many more listings of current Scleroderma Treatments and Clinical Trials on our main website.



  7. Hi Stephen,


    Welcome to Sclero Forums.  I'm sorry you are experiencing concerning symptoms and hope that you get some answers soon.


    As it happens, systemic scleroderma is most common in women of childbearing age. That said, it can occur in absolutely anyone, from newborns to the very elderly, both male and female. When it occurs in men, it is most often due to occupational or environmental exposure (such as certain construction workers.)  See Causes of Scleroderma.


    It is also possible for symptoms to be caused by aging, and many other diseases, so things like heart disease and diabetes should also be ruled out, which is why your doctor is running tests, of course. Typically, systemic scleroderma skin issues begin with swollen and achy hands, and occurs about equally on both sides of the body; it is not very likely to cause a small patch by the elbow on just one side. However, localized scleroderma (the type that affects primarily the skin, like morphea and linear), occurs in colored (reddish or brownish) patches and does not match on both sides of the body.


    There are also many other skin conditions that could account for the patch, such as psoriasis or eczema or even a callous.


    I know how worrisome it can be to have symptoms that are as yet undiagnosed. I find it helpful in situations like that to assess all the possible outcomes (so I am mentally prepared in case of the worst outcome) and then try to pick the most harmless explanation to focus on during the wait for test results. Now you know about the worst it could possibly be, but because scleroderma is so rare, and even more rare in men, not to mention elderly men, I hope you find that to be a little bit comforting -- by at least reducing the statistical odds -- while you wait for answers.


    The many types of scleroderma can be enormously confusing, so you may want to look at our main site for What is Scleroderma? and also for Diagnosis of Scleroderma.  Please stay in touch and let us know how things turn out for you.



  8. Hi Margaret,


    I'm glad Gareth had an endoscopy and that the cause of his pain was discovered and treated. It is delightful that his doctor has had so much wonderful scleroderma experience at Johns Hopkins. I'll bet you were very pleased to discover that he is now in such great hands!



  9. Hi Mammabear,


    A bucket list is a great idea for anyone, whether they are healthy or sick. 


    It's a very positive approach to take, and also one to revise whenever your abilities increase or decrease. At the top of my To Do list, I always put Rest - Refresh - Relax, so that I always have a sense of accomplishment, come what may, and that alone makes me happy.


    Sometimes you may need to swap out Tour of Europe to Tour of Neighborhood Coffee Shops. Or swap Become Olympian Gold Medalist for Play a Card Game Tonight. What matters isn't achieving any bucket list items, so much as having a list of things that we can look forward to.  Setting a timer to watch the sunset tonight can be just as inspiring a goal as climbing a mountain, if we're doing what is within our present abilities and entirely letting go of self-pity (so that we can wholeheartedly enjoy it.)



  10. Hi Miocean,


    I'm thrilled that you are finally home!  I've found there is a vast difference between hospitals, even ones in the same neighborhood. You may not notice it quite so much if you're in an area of outstanding facilities, or if you go to the really major centers. In the poorly equipped and staffed centers, sometimes the caring of a few staff members really shines brightly, as we are so aware of the exceptional effort they are taking under adverse circumstances. 


    I hope that you settle in okay at home now, and that your home services are helpful, too.



  11. Hi Mammabear,


    One of the things they study in regard to scleroderma is "fear of progression".  It is so easy for us to sink into fear, over anything, not to mention something like scleroderma!  But that's one reason why we all hunker together here, to provide inspiration and encouragement for each other.


    I've long outlived many dire predictions, and many others here have, too. I find it helpful to try to focus on how to make today as fun and happy and joyful as possible. By building many little joys into our daily routine, we don't need to fear the future quite as much, because we have confidence that we can find some way to somehow make ourselves happy regardless of our outward circumstances.


    The most difficult thing for me was learning to not take life so seriously just because I had a serious illness. The illness will be whatever it is going to be. We have little control over that, except for trying to get the wisest counsel and do our best to follow it.  Then for the rest of it, it can help reduce our stress to take things as lightly as possible; to try to find the silly side of ourselves and of humanity.


    I hope your new wonderful friends here will warm your heart this holiday season and brighten your life in the coming year.


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



  12. Hi Quiltfairy,


    I'm very sorry to hear about your dog Tony. It's never easy knowing that the end is in sight for beloved pets. I'm sure you'll spoil him absolutely rotten and that his holiday dinner will be just about the best ever!  I'll be keeping you both in my thoughts. 



  13. Hi Miocean,


    Wow, I was not expecting an update like that. I'm sorry all this has happened to you, and right when you wanted to be going on vacation. And now you might be stranded in the hospital for the holiday, too?


    Well, I am thinking of you and sending lots of good thoughts your way. I don't want to even imagine your holiday dinner. Like, yuck!


    Are you at least rid of the crazy roommate?



  14. Hi Tully,


    Oh don't worry, we are all used to always thinking everyone else has it worse than us on some days, and that we are the worst of all, on other days.


    For each of us, our journey and our situation is better than some, and worse or even much worse, than others. So all that really matters is that we tell our stories, and share our journeys, and be there for each other.


    We are all in it together, and our togetherness makes all our collective joys, and sorrows, easier to bear.



  15. Ah, my favorite quote of the day, "Tully, I suggest that, going forward, you give yourself the credit you deserve."


    Couldn't we all, shouldn't we all, give ourselves the credit we deserve?  I hope everyone reading this who is affected by scleroderma or illness or adversity in any way, takes the time right now to pat themself on the back, and give themself more credit, if only for surviving it so far, rather than berating ourselves for not merrily thriving our way through every single second of it.



  16. Hi Amanda,


    Wow, you are being put through the mill!  I'm sorry you have so many complications, and all at once, no less.


    I don't know the answer to most of your questions. PH can be very hard to diagnose. And it can be misleading, because some tests can appear to indicate PH, others can appear to be perfectly normal, but only the right heart cath can sort everything out properly. And, that is quite a procedure to undergo. When is your next cardiology appointment?


    Be sure to read up on Nissen fundoplication before you visit gastroenterology, in case they suggest that surgery to fix it.


    For hydroxychloroquine, my rheumatologist had me take my entire dose at bedtime, so that I would sleep through any side effects. That worked like a charm and I never had had any bothersome side effects, except for the good ones like a very noticeable and welcome reduction in pain and fatigue, and fewer flares of all sorts of things.


    Keep us posted as things develop. It's certainly going to be a cute balancing act to manage all of this at once!



  17. Hi Miocean,


    Oh my goodness. I hope you are able to fly on vacation. But if it is not cleared up in time, it is better to recover at home than to be in dire straits in the air or in another country. Perhaps you could develop a Plan B for vacationing at or near home, one that you could always revert to, if needed.


    I try to always think in terms of alternate plans and keep on adjusting them until they fit my capabilities that day. I know you are great at that, too!  What could you do at home that could be really fun and manageable for both of you?  Game nights together or with friends, a painting spree, museum visits, plays, local sightseeing, or even just a great vacation book? 


    I'm sending lots of good wishes your way in hopes that you mend enough in order to be able to safely travel. 



  18. Hi Jpgoogs,


    If your doctor has prescribed Gleevec for something other than scleroderma (such as leukemia or gastrointestinal tumors), be sure to discuss any plans you may have regarding pregnancy. 


    If they were recommending it for scleroderma, then you might want to get a second opinion, preferably with a listed scleroderma expert, which we strongly recommend.


    Please keep in touch and let us know how things are going for you.



  19. Hi Smileforyou,


    Welcome to Sclero Forums.  I'm sorry you have Sjogren's and send my best wishes to you.  As it happens, Sjogren's is the third most common rheumatic disease, so it is often encountered and treated by the usual rheumatologists.


    If you are developing more difficult or complex symptoms of Sjogren's, then it may be most helpful to go to the largest medical university center in your area (the hospitals where they train doctors).  Such centers are likely to see more patients, and more patients with complex conditions as well.


    Another tip is to see a listed scleroderma expert, because they are also rheumatologists, and because Sjogren's frequently occurs along with scleroderma, so they are more likely to be well versed in it than the "average" rheumatologist.


    I'd like to point out that we serve those with scleroderma and related illnesses or symptoms, worldwide -- including Sjogren's. So we are a great support resource for you for the long term.