The Scleroderma Chronicles: June is Scleroderma Awareness Month

Five years ago the course of my life altered forever when some blood test results ordered by my doctor arrived: the results showed that I definitely had two autoimmune diseases. Specifically, I was producing antibodies that were diagnostic for scleroderma and Sjogren’s disease. I was expecting lupus, so this was kind of a surprise shock. After a quick trip to Google to establish what type of scleroderma was associated with my positive test results I burst into tears. It was worse, much worse, then I expected.

The scleroderma diagnosis was the problem. Through an unbelievable sequence of serendipitous life events I was more knowledgeable then the average newly diagnosed patient, and I knew that scleroderma was a disease that impacted connective tissue, that it was progressive, disabling, and that there was no cure. This was a life-altering diagnosis, and I was in for a long fight that would last the rest of my life.

You see, I used to work in a rheumatology research lab, and I did research in scleroderma. I was a member of the research team that found the first identified antigen associated with scleroderma. I had visited scleroderma patients in the hospital. Later in my life I taught AP Biology and spent years trying to explain connective tissue to students.  It’s a type of tissue that we just never think of, but it is critical in organizing and operating our bodies. Connective tissue makes your skin elastic and strong. It organizes your muscles and makes up your tendons and ligaments. It is a critical layer in your blood vessels, and is part of the essential structure of all of your organs.  In scleroderma all or parts of this connective tissue is under attack by your immune system.

As white blood cells invade my tissues and attack this connective tissue it produces too much collagen in response. The built up collagen produces thick layers of tissue and scarring. My fingers look really swollen, but it is actually very thick hard skin. My skin is also getting really shiny which means that the collagen is hardening up and losing flexibility. Must knit faster!!

You can perhaps see that same thickness on my face, especially on my cheeks. It means that I don’t have wrinkles, but it is also hard to open my mouth, my smile is mostly gone, and I can’t turn my neck well. Crazy, huh.

That is the most ironic aspect of scleroderma: you look pretty darn good, especially if you are a senior citizen like myself, but you actually struggle daily with your illness. For many scleroderma patients their disease just  involves the skin, but for others, the disease is more than skin deep.

As it turns out, my skin is the least of my worries. The rare type of scleroderma that I have, systemic sclerosis, also causes scarring of internal organs. The muscles of my stomach and esophagus have lost function. My kidneys are damaged and I have chronic kidney disease (stage 3). My lungs are scarred and my diaphragm isn’t exactly happy any more. Part of my stomach herniated up into my chest this year… whatever was it thinking of?! My tendons are getting calcified due to inflammation and at least one has partially ruptured. My muscles are sore to the touch and I have bruises everywhere. I have nerve damage and trouble controlling my body temperature. It’s hard to walk. Blood vessel damage affects circulation to my hands and feet and I’m starting to develop open sores (ulcers)… There is a long list of diagnosed conditions linked to my scleroderma, but you get the idea. Pretty much I’m a walking limping train wreck. Well, a knitting train wreck for sure!

There is no cure for systemic sclerosis, but there are treatments that really help a lot. I am taking four different drugs to crush my immune system into submission; it’s a balancing act as I need my white blood cell count to stay high enough to protect me, but low enough to control my symptoms and prevent more damage. I take a drug to shut off the acid produced in my stomach so I won’t accidently inhale it in my sleep since the muscle barrier that usually keeps it in my stomach is now gone. I take a couple more drugs that help control inflammation, and some supplements that help with nerve damage. I’m on oxygen at night. All of these drugs/supplements have made a huge difference for me: my last lung scan showed improvement and my high heart pressure, the most concerning complication that I had, has returned to normal ranges. My kidney damage continues, but it has slowed way down. There is something funky going on with my red blood cell count, but you can’t win them all, right? The main point is that I continue to manage and live independently.

MacKenzie and I last year when I posted this online as part of the “Face of Scleroderma” campaign.

In short, I am a mess. And yet, to the joy of my doctors, I continue to do really well. Okay, I have blue lips, am short of breath, and struggle with tissue damage, but I also continue to thrive compared to other scleroderma patients that they treat. I have had to make many changes to my life, but I have found work-arounds and I still do things that I love. Attitude is all!

Well, knitting, the cat, and the garden are pretty darn essential, too!

So, there it is. What an annoying disease, right? How dare it make you look younger while shortening your life? How dare it do all of this invisible internal damage that makes people think that you are lazy or an attention-seeking hypochondriac when actually you view each day that you are able to leave the house as a personal victory? I’m in several online support groups and there are people dealing with crushing negativity like that. I can see how it can happen; it is so hard to understand something beyond your own experience that is hidden from view.

That’s why there is Scleroderma Awareness Month. It is hard to have a rare disease, especially when it is one that is hard to pronounce (Sclero… what?!). It’s harder still to have one that has no cure and a pretty high fatality rate (hey, with all of the drugs that I’m on my 10 year survival rate is now up to 80%!!). It makes you learn to laugh in the face of terminal complications while forcing you to take every possible precaution to avoid contracting Covid-19. It messes with your head; it gives you power, but it’s also strange and a little lonely.

That’s why we scleroderma patients share our journey with all of you every year so you can get a glimpse of our lives.

 

If you look harder you will see the signs of my scleroderma on my face. The small red spots are called telangiectasia and are symptomatic of my form of systemic sclerosis. The skin of my forehead is tight and shiny, my hair is falling out,  and my dimples are now buried under my thick skin. My upper lip is trying to decide if it wants to turn blue… 

I am the Face of Scleroderma.

Footnote: In addition to scleroderma I also have Sjogren’s Disease and fibromyalgia. The symptoms from these three diagnosed conditions overlap and always make things interesting in sorting out my treatment plan. You can learn more about any of these autoimmune disease by checking out the links in my post.

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 an exceptionally spoiled kitten.

24 thoughts on “The Scleroderma Chronicles: June is Scleroderma Awareness Month”

  1. Yes, I ‘LIKE’ this post ! It opens up a whole world of things I don’t have to whinge about. It tells me that people who are suffering are the best people there are; because their suffering causes them to really look at life and really do their utmost to participate in it – far more so than do fat (morbidly obese !) old ladies about to turn 77 and feeling sorry for themselves for what can now be seen to be totally unimportant side issues ..
    You and I share the same interests – our cats, our crafting, our gardens, our ‘books’ – and now we are both worried about our continuing ability to craft: you because of your skin-thickening and I because of finger-bending caused by arthritis. Your top lip is thinking of turning blue ? – I have varicose veins in my lips ! There: I win that one. [grin]
    Listen, O nameless one: just go on the way you’re going, and keep us entertained. As for your diseases, I cannot imagine why anyone believes there is a ‘god’.

    1. Dealing with something like this, or your own arthritis, really make you re-evaluate your priorities, doesn’t it! I am just stunned to hear other people complain about things like haircuts or gaining some weight. Seriously? Get over it! My tax lady was just stunned a couple of years ago because I had closed my little online business and wasn’t attempting to make money anymore. Yep. Like I care about money any more… I have enough to buy all of the yarn and books that I want, and what else is there. Travel? Don’t make me laugh! On the other hand, I am so happy and pleased by small things like sunshine and new kittens. 🙂

      I’m sorry that your hands are threatening your crafting. I understand how hard that is. Contractures are a thing with scleroderma, and I also fear my fingers turning into claws. So unfair and so much grief. I hope that your hands behave themselves!!

      Okay, your blue lip wins! Mine is due to that issue with my red blood cells and should get better. Please don’t try to get creative and develop an even bigger whopper ailment!

      I’m Marilyn. Stay safe!

      1. It’s turning out to be quite a journey. I knew about systemic diseases, but never really understood what the big deal was. Now I do! If you meet another person with something like lupus, figromyalgia, rheumatoid arthritis, or maybe even scleroderma, be kind to them!

  2. Thank you for sharing the information about this. I didn’t know anything about Scleroderma before I started following you at all. I am so sorry that you are going through this, but glad that your fight is having some success.

    1. It’s so rare, and hard to see, I’m not surprised. I now look at the world with different eyes… how many other people also carry burdens and physical ailments that we can’t see? If nothing else, this is teaching me to be more patient and kind.

    1. 🙂 Thank you. I first started blogging as I struggled with grief and loss following my diagnosis. I mean, I was so sick and then I found out that I wouldn’t get better or ever get my old life back. Now that is a distant memory, and a lot of things that I thought were important really aren’t. There is a lot to be positive about!

  3. I’m so happy to have found your blog. I’ve learned a lot about Scleroderma from your posts. I have great admiration for the person you are. It sounds painful, exhausting, and lonely to live day to day with something so debilitating. I’m pleased to hear that you’ve found online support groups so that you can share in the day to day of others in the same boat. I loved your words here: “it is so hard to understand something beyond your own experience that is hidden from view.” My younger sister has lived with MS for 20 years now, and as I’ve watched her adapt to the progression of the disease, I’ve also learned a lot about the society around us. It’s now always pretty.

    My request for a commissioned Tessa feels entirely selfish now. You make things look simple when they are everything but. Sending a warm embrace, admiration, and gratitude for the medical folks that can help keep your symptoms at bay. xo Alys

  4. I am sorry to hear about your condition. I don’t comment sometimes because all the things I can think to say seem trite.

    Life doesn’t seem fair.

    I had a brother. His name was Jeffery. He was my best friend. One day he just started to have trouble swallowing. Within a month he was diagnosed with terminal esophageal cancer. He was young. Within a few weeks he couldn’t eat at all. It was so hard to see him like that.

    There was no warning. And then he was gone.

    This world is messed up in so many ways. But, I do see some good things. My brother was a good thing. I thank God for him.

    Meeting new people is also a good thing. I have enjoyed “meeting” you and reading your blog. I enjoy your knitting posts, and especially your posts about your kitties. I also enjoy seeing your flowers.

    Like I said before, I am looking for the day where Jesus said he would make all things new, and when there is no more sickness . . . and when the world is no longer messed up.

    1. I am so sorry to hear that happened to you and your brother. I know that it is really hard to deal with events like that and that the grief and pain goes on for a long time. Hugs!

      It is so good to “meet” new people and I so enjoy the friends that I have made online. I’m glad that you enjoy the stuff that I share; the kitties, flowers and knitting are the little moments of joy that appear out of nowhere in the day, It’s nice to share them because people share back with me. 🙂

      I’m getting ready to post a kitten picture and may through in a rose too. 🙂

      1. Thank you. It does hurt, but I have been blessed to never go through it alone.

        My brother said the same thing the last day I saw him, and I believe he had peace.

        This life isn’t just roses, there are a lot of thorns. But, I have hope of seeing him again.

        I am keeping you in my prayers. 🙂 Hugs!

  5. I so appreciate your candor in sharing your struggles with this diagnosis. I knew very little of the disease, but feel more informed about this now. Thank you. I have so much respect for your attitude! No doubt this contributes to your doctor’s comment on how well you are managing your condition. I am so pleased to have met you here. You will be in my prayers. Love, Regina

    1. Thank you so much, Regina. I once saw a training film while I was teaching about interacting with the public. One of the items was “choose your attitude”, which made a big impression on me. No matter what was going on in my life, I can control my attitude. The blogging is somewhat selfish because it helps me a lot to write about all of the little things going on with my life, the good and the not-so-good.
      I was once sent to palliative care when my lung disease appeared to be out of control. You should keep a journal, they told me. I blog, I replied. Oh, just carry on with that, they said. A few months later my drugs kicked in and I escaped from palliative care. Yay! Attitude is all! I’m sure that it does help me.

Leave a Reply

Fill in your details below or click an icon to log in:

WordPress.com Logo

You are commenting using your WordPress.com account. Log Out /  Change )

Google photo

You are commenting using your Google account. Log Out /  Change )

Twitter picture

You are commenting using your Twitter account. Log Out /  Change )

Facebook photo

You are commenting using your Facebook account. Log Out /  Change )

Connecting to %s