The Scleroderma Chronicles: Rare Disease Day 2018

It seems like I just wrote a “rare disease day” post just yesterday, but it has been a whole year already. Actually, I think that I was really lazy last year and just re-posted the previous year’s blog post. Anyway, tomorrow (February 28th) is World Rare Disease Day. This year the motto is “Show Your Rare”, which is a little scary as it sounds vaguely like how I might order my steak, but I’m game. I’m rare, and here is my story.

First things first. Let’s talk about rare diseases. Here in the United States, a rare disease (also known as an orphan disease) is one that affects fewer than 200,000 people in the US. The bad news is that there are a lot of rare diseases; over 6,000 different diseases that impact about 25 million people just here in the US. Many of these are genetic in nature, and they tend to be chronic (more about that later).

I joined the rare disease club the day that my systemic sclerosis (a form of scleroderma) was diagnosed. With only about 100,000 cases in the US, we are definitely rare. Here’s the scoop on my disease: it is progressive, disabling, chronic, and possibly fatal. It shares some characteristics with cancer cells. It is also mostly invisible to other people, and people have no frame of reference to understand an illness that they have never heard of before, so they tend to be dismissive.

Scleroderma hand
One of the most obvious features of my condition is my thick skin. My legs and lower arms are literally hard, and here on my hand you can see that the skin folds kind of strangely. My skin is thick with scar tissue that has built up in response to inflammation set off by my immune system’s attack on normally healthy tissue.
You can really see it on this finger. My finger looks pale since the scaring is so bad it cuts off circulation when I hold it out straight. There is a silver lining to this: I will never have “old lady” hands.

My hands look strange, but I am doing really well all things considered. I have good circulation and flexibility which my doctors think is due to knitting. “Knit as much as you can!” was the recommendation. See, every setback in life has a silver lining.

What can’t be seen is my astonishing fatigue, muscle/joint pain, and brain fog. So much brain fog. I’m also accumulating organ damage as the months pass by. Digestive system damage, kidney damage, lung damage, and blood vessel damage that is slowly increasing the pressure in my heart.

Scleroderma face
All that redness on my face is due to blood vessel damage, and you can see the thick skin around my eye. Collagen build-up has given me chipmunk cheeks. Special. Thank heavens for red-cancelling make-up.

It’s like a scene from the movie, The Terminator. You know… if you substitute this line about the Terminator from the movie with the word scleroderma it would be… [Scleroderma] can’t be bargained with. It can’t be reasoned with. It doesn’t feel pity, or remorse, or fear! And it absolutely will not stop, ever, until you are dead!

Mortality rates are down for my form of the disease due to new drug interventions. The other good news is that it is moving really slowly in my case. I think that I have had this disease for decades, and only now, in my 60s, is it catching up with me. I’m on the best drugs available, I love my doctors, and they have slowed the disease progression down tremendously over the last couple of years.

But I’m still dealing with a serious chronic illness, as are many other people with rare diseases. For me, when trying to explain my illness to others, it is the concept of “chronic” that causes the most trouble. People tell me to get more sunshine, more exercise, better food, use essential oils, whatever, so that I can get better. That’s the way it is for many health conditions, but not for a chronic illness.

There is no better, I tell them. Only this. Nope. They will insist that I can get better if I just try, and take it as defeatism when I calmly explain that some of this damage can’t be reversed. It is possible to get better without getting well, they will say. You look so great! It’s like it’s a personal affront that I can’t just be cured. If they haven’t seen me for a few months they will express surprise that “this is still going on”.

It is the concept of “chronic” that is the problem. To accept that you are sick is not weakness or “giving up”. It is coming to terms with the nature of your enemy.

So here is my Rare Disease Day request for anyone who has managed to read all of this. Accept chronic. Ask the person you are talking to about how they are doing. Ask what their doctors are telling them. Accept that they are in a situation that they can’t escape from. Don’t shy away from the fight if you care about that person.

If you know the movie “The Terminator“, you know that the heroine of the tale, Sarah Connor, was a hell of a fighter. She never, ever gave up, and in the end she got the best of the monster trying to do her in.

I’m channeling Sarah Connor.

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.

19 thoughts on “The Scleroderma Chronicles: Rare Disease Day 2018”

  1. HELPLESS, IS MY FEELING! I sit back in my own little world. I’m knitting with stiff fingers, but it is nothing compared to you. My stomach is upset, but that is my own undoing from overeating all day. I am fortunate to be blessed with good health (so far in life) and feel guilty that my life is so easy.

    I feel helpless as a friend and caregiver. I want and should be able to do something to make life easier for you, but am at a loss of what that might be. I look to you to give me that answer, but really is there an answer ? I was trained as a nurse, there must be something I could do to make your life easier, but do not have a clue what that might be, my brain and training fail me.

    Day by day you see your life changing slowly before your eyes. I do not see that change, but feel helpless that there is nothing I can do personally to improve your day. I want to run away and not face your reality, so that I can go on living in my padded little world.

    But I have chosen you as a friend, knowing what your life holds for you and that there will be little I can do. I thought long and hard before I chose the path that leads to friendship. And you have chosen me as a friend also, so there must be something good about me. We have common interests that we can share and talk about and find joy in.

    Right now I am a shoulder for you to lean on emotionally. That seems to be all that I can offer you. One day maybe I can be that shoulder that you lean on physically.

    1. Hey, you are the best friend ever, and I am so grateful that you decided to take a chance on me. Thank you for taking me as I am and being willing to give me your shoulder when I need it; meanwhile, let’s do lunch soon and go to a great yarn store road trip. 🙂


  2. Big hugs. As a fellow spoonie, I know how chronic illness feel. I try talking about it more, but I’m always afraid to bore people to death. I didn’t know it was rare disease day, but I just started writing a book today about the topic, and how to handle it on a daily basis. Crazy synchronicity.

  3. Hi Midnight,
    I came across your post while looking up pictures of finger pitting, and there was a picture of your hands with the abnormal skin folds. I have that on my pinky and ring fingers on both hands. If you let your fingernails grow long, do some of them grow sideways (the only way I can describe it), or in a different manner than how they used to grow? I have Limited SSc with organ damage as well, including ILD. I’m 58, and was diagnosed just over 10 years ago. I appreciate your eloquently worded post. You hit the nail on the head about people not understanding that this can’t be exercised away, or cured by diet and supplements.

    1. Hi KC! I also have limited SSc with organ damage, and I was diagnosed in 2014 but I think that it had been chugging along for a few years before that. I haven’t noticed that my fingerails are growing sideways, but my index and middle fingers are. How crazy is that? My fingernails do have ridges and some of them are crazy deep.

      I think that people struggle to accept the realities of chronic illness because they need to believe that something like this will never happen to them, or they are unwilling to become involved and use the (it’s your fault that you are sick) rationale to justify their distance. I’m empathetic, but it’s toxic and hurtful.

      Hugs to you, fellow sclero warrior! I’m so glad to hear from you.

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