The Scleroderma Chronicles: World Scleroderma Day, 2021

Here we are again… World Scleroderma Day.

I’ve written about scleroderma on this date for several years now. I just went back and read what I wrote last year and decided that I did a pretty good job. I talked about getting diagnosed, the complications that are being caused by my disease, and what that means in my life. Here’s that post if you would like to check out my take on things last year.

This is one crazy-ass disease and it certainly has complicated my life. In the year since I wrote that last post I have hung in there (hey, staying home for a year on oxygen slowly improving was actually good for me) and then emerged from strict lockdown to get lots of testing to try to sort out what is the cause of my blue-lipped status and the source of other little issues that I have going on. While thinking about what to write today I thought of a few things that I haven’t written about before and some things that are new since the last post.

The correct name for the type of scleroderma that I have is limited systemic sclerosis. Systemic means that every part of my body is being impacted by this disease, and the word sclerosis means that scarring is happening in tissues all over my body. What’s happening to me is not obvious to the outside viewer, but it is insidious and ongoing all the same. Somehow, by some mechanism that isn’t clear, my systemic sclerosis is being driven by antibodies that I’m producing that react with the centromeres of my cells.

What’s a centromere? I’m shocked, shocked I tell you, that you just don’t know that! Seriously, the centromere is that little place in the middle of a chromosome that can be seen when cells are getting ready to divide. This is a little tricky because the only time we can see chromosomes is when cells are getting ready to divide and they have already copied themselves: the little pinched waist in the middle of the chromosome where they are connected is where the centromere is located.

Got that?

This image belongs to the NIH; as a taxpaying citizen I hope that it is okay for me to use it here. 🙂

Somehow something happened to a protein located in the centromere area of the chromosomes in my body that made it look “different” and my immune system responded by making antibodies against that protein. The antibodies that are produced are called “anti-centromere antibodies” and their presence is highly suggestive of my form of systemic sclerosis. The alternative explanation is that my immune system just went berserk, decided to attack my own cells, and began making these antibodies on its own. I personally think that something changed and then the antibodies were made as a response. My money is on a virus.

It really doesn’t matter all that much since, once the immune system is triggered, the immune response can’t be turned off and one day you are sat down in some rheumatologist’s office and carefully told about what is happening to you and what to expect in the future. Your skin will get thick. Your blood vessels will be so damaged that they will thicken and spasm shutting off blood flow to parts of your body unexpectedly. The scarring tissue will build up in your digestive tract and damage the smooth muscles that you need to move food along. Your kidneys will lose function. Your nerves will be damaged. Your lungs need to protected since they are especially vulnerable to damage from accidently inhaled stomach acid. If your disease flares badly enough you can develop autoimmune pneumonia. Your tendons, muscles, and joints are all in trouble. You are in trouble. The damage will progress and there is no cure.

“This is really serious,” one of those early doctors told me. “This is like a diagnosis of cancer, but of course, some cancers can be cured…”

Well, shoot. Good thing there are drugs to help control symptoms and to slow the progression of the disease by dialing down the immune system.

Yay for drugs!! I am on drugs to shut down my stomach acid production (my lungs are doing pretty well) and on drugs to crush my immune system into submission. I’m on anti-inflammatories to control other cellular pathways activated by those ill-behaved antibodies. I get steroid injections to help my damaged joints and inflamed tendons. I have strict dietary limitations. I do lots of physical therapy. I’m on oxygen overnight to keep my red blood cell count in a normal range. I dress in layers to help control the spasms of my circulatory system, a phenomenon called Raynaud’s. I knit almost every day to keep my fingers from stiffening up and contracting. I now have a shiny purple cane to help me walk.

Every day is a challenge, but I am fine.

So what is new this year?

Scleroderma has damaged my heart. They are still testing to clarify exactly what is going on, but so far they have established that there is scarring in the heart muscle that is making my heart “stiff” and that somehow I developed a hole in my heart. There are suggestions of pulmonary hypertension, but I need more testing for a definitive diagnosis. No wonder I turn blue in the face and pant when I walk.

Oh, yeah, there is also a pandemic going on.

One of the greatest ironies of the last year is that serious cases of Covid-19 share similarities with the clinical symptoms of scleroderma crisis. Immune system-mediated pneumonia caused by an overreacting immune system is a hallmark of both conditions. Then there is this… Remember those drugs that I take to crush my immune system into submission? I take two drugs for that purpose. One of them, hydroxychloroquine, was (irresponsibly) politicized by influential individuals early in the pandemic and therefore became short in supply; my muscles and joints immediately rebelled when my supply lapsed early in the lockdown. The other drug that I take, mycophenolate mofetil, is linked to poor response to the Covid-19 vaccine. I am vaccinated and I am making anti-Covid antibodies, but since my total antibody count is very low my doctors aren’t sure I can fight off a Covid-19 infection fast enough to stay out of the hospital. I’m advised to continue to mask and isolate because… blue face, hole in heart, stuff like that…

For me, lockdown goes on.

So, here are the takeaway messages from this post. Be kind. There are illnesses that are really debilitating that you can’t see when you look at that person walking into a store from a handicapped parking spot. Don’t be dismissive of conditions with funny names that you’ve never heard of before. I know that it is hard to understand conditions that are beyond your experience and that you can’t really see, but take a moment to let someone tell you about their illness and the daily challenges that they embrace. If you meet a person with an autoimmune disease, they deserve a hug. If they have scleroderma you should give them two hugs! Be understanding of people who are still wearing a mask in public; perhaps they are braver than you can imagine.

Today is World Scleroderma Day.

Go Team Teal!!

Note: If you would like to know about different types of scleroderma you can learn about them here.

Author: Midnight Knitter

I weave, knit and read in Aurora, Colorado where my garden lives. I have 2 sons, a knitting daughter-in-law, a grandson and two exceptionally spoiled kittens. In 2014 I was diagnosed with a serious rare autoimmune disease called systemic sclerosis along with Sjogren's Disease and fibromyalgia.

13 thoughts on “The Scleroderma Chronicles: World Scleroderma Day, 2021”

  1. Thank you so much for sharing this! I hope that the treatments you are receiving continue to help you fight, and also that current research will come up with a way to help you to heal. You are such an inspiration in how you do everything you can to resist this disease and find things to bring you joy – Hannah, your lovely flowers, and your knitting. Sending healing thoughts to you!

    1. Hugs to you (two of them!) fellow ScleroWarrior!

      I was a biology teacher before scleroderma made it too hard for me to work and sometimes I find myself still trying to teach. I’m glad that you think I explained this well. Hugs again.

  2. Many many hugs to you (one to me for RA!), you keep a very positive attitude dealing with everything you do, which sounds incredibly difficult, and are truly an inspiration. I appreciate you sharing your struggles, I wish people understood more about AI conditions. Sending many many healing thoughts to you!!!

    1. Thank you so much. It is so hard for people to understand autoimmune. I’m sure that you have been told, as I have, that I just need to exercise, get more sunshine, and eat better to “get well”. Nope. There is no well, there is only this. I hope that my online journey helps others understand in some way.

      I do try to stay cheerful. I told my rheumatologist that I tend to just keep rolling with the punches and adapting as things get worse instead of running for medical help. “Well, how’s that working for you,” she said. Okay, it may not be the best coping strategy but I do maintain a good attitude by adapting and not panicking and obsessing over things that I can’t change.
      Hugs to you! RA is no joke and you deserve every positive thing that comes your way!!
      I do

      1. Thank you! Well that and long covid I’ve gotten the occasional comment that if I keep walking it will help my stamina—and basically allow me to keep doing it (walking). I have basically said I prefer no suggestions. Outside family don’t seem to get it. And no it doesn’t. Relapses and relapses and relapses from a simple walk around one block occasionally. And RA, my father dealt with his mother who had it, she was severely crippled by it. there are some real negative issues there that I don’t know about — they haven’t really been asking me about it, don’t seem too concerned but it’s just starting to ramp up. My impression is a lot of people felt more sorry for my grandfather but I don’t really know what went on. The poor woman, there were no real meds back then. I am very fortunate to get it now.

        Attitude does help but it’s difficult! Hugs and helpful vibes to you!

      2. Oh, no! So much going on, and I 100% understand. I can go into a flare from… grocery shopping! It is so difficult to deal with the constant slap across the face of conditions like these. Long Covid!! Ugh! I caught a flu-like illness Jan. 2020 and was so sick that I thought I would have to call 911. As I was recovering the first reports of covid started to emerge in the news. Where I’m going with this is that I don’t know if that was covid; since I struggle with fatigue and brain fog all the time and I was in lockdown just figured it was depression and my condition. When I got vaccinated I had a dramatic improvement in fatigue and the brain fog vanished!! Even more crazy, my muscle soreness stopped. If you haven’t gotten vaccinated yet you might consider it.

        I have discovered that the family/friends are not all that interested in hearing about my condition. I realized that there is more concern for people who are struggling to walk or some other obvious disability by not so much for those of us with joint/pain/fatigue/internal organ trouble. It is like if you aren’t in a wheelchair you aren’t really having issues. Whatever! Be kind to yourself and remember that you don’t need to justify your symptoms to anyone. Hugs!!

  3. Got both vaccines, not much difference but did it! Yes, I still can’t grocery shop so curbside, thank goodness! Not exactly like my husband and daughter are good caretakers sad…..

    It is so weird about family/friends and AI. Thank you!
    I will, hugs hugs hugs back atcha!!

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