Today is World Scleroderma Day. Tonight the Niagara Falls will be illuminated blue and white in recognition of the day. All this month (June) there have been walks and outreach to raise awareness of this disease and to raise money for scleroderma research, and scleroderma patients all over the world have been urged to post pictures of their faces (and smiles) to made this disease more real and relatable.
The purpose of this day is to try to raise awareness of an illness that is mostly invisible and relatively unknown.
So what is scleroderma? It is a rare autoimmune disease that is a member of the rheumatic diseases family. The name “scleroderma” means hard (sclero) skin (derma), which is the most visible symptom. Scleroderma is the general name that is applied to a group of connective tissue diseases that may target only the skin, or the skin plus internal organs, or just the internal organs themselves. The words that are used to describe my type of scleroderma (limited systemic sclerosis) are rare, chronic, progressive, disabling, and possibly life threatening. This is a lot to wrap one’s head around, so I’ve decided to break it down.
Rare: Here in the United States the CDC has defined a disease with fewer than 200,000 patients as “rare”. Systemic sclerosis, with about 1-2 diagnosed people for every 100,000 citizens fits the bill. To be rare means your illness struggles for research and treatment funding as the patient population is small and almost no one is personally impacted by the disease in the general population. Hence, Scleroderma Awareness Month and World Scleroderma Day. Thank you, Niagara Falls!!
Auto-Immune Disease: The immune system, designed to protect us from foreign invaders like pathogens, is attacking some of the components of normal cells in scleroderma patients. The exact components can be identified using the antibodies of scleroderma patients and they are essential proteins found in the nucleus of the cell. My antibodies are attacking a protein found at the kinetochore of dividing chromosomes. I can’t help but wonder what the heck has happened here that I ended up with antibodies like these? I also eventually wondered if my cancer risk is increased since there is something off with this critical structure used in cell division (it is).
Connective Tissue: I used to try to explain this type of tissue to my AP Biology students. Connective tissue… well… it connects. It is what holds your bones together to form the skeleton. It gives support and stretchiness to your skin. It is involved in all the organs of your body, and holds the organs in systems together. It is essential to organize your muscles. In systemic sclerosis, all of this tissue can be involved in an inflammatory attack of the immune system and scarring occurs. Skin gets thick; hands curl, joints won’t bend, smiles twist and faces harden. Muscles and joints hurt. The smooth muscle in the digestive tract can be so damaged that organs don’t work right. Blood vessels are damaged and spasm, cutting off circulation to extremities and organs. The heart can get scarred. Scarred lungs prevent oxygen passage. Scleroderma, invisible to the outside viewer, can be devastating to the patient.
Chronic: There is no cure, and it never ends. There are, however, treatments for individual impacted organs that are really helpful.
Progessive: Damage accumulates over time and conditions worsen. My doctors are monitoring my kidneys, heart, lungs and GI system. So far I am doing pretty well, but my GI tract is taking the most damage.
Disabling: Yep. It is getting really hard to walk, and I just don’t fit all that well into the world anymore since my diet is very restrictive, air conditioning isn’t my friend, and sunshine makes me go into a flare. Right now I don’t need supplemental oxygen, but if that comes back it will further restrict my independence.
Life-threatening: It took me months to think this one through, but eventually I did google “life expectancy of systemic sclerosis” and discovered that for me, with my treatment plan and risk factors, survival rate is about 75% for 10 years after diagnosis. Overall the death rate of scleroderma is 50%. Oh. Now I know. Who wants to live forever?
This week I wrote a letter to my congressman asking him to sponsor a bill that would fund research into fibrotic diseases like scleroderma and cystic fibrosis. I haven’t heard back, but maybe if he has heard of one of these diseases or knows someone impacted by scleroderma he will do it.
That’s why we do World Scleroderma Day.
Yarn, Books & Roses 
Thank you for this excellent and informative post MK and for bringing awareness to this “not rare enough” disease on World Scleroderma Day! I had hoped to write a post today but a flare has been holding me down the past couple of weeks, thank you on behalf of your fellow warriors for pulling our weight. Will share with my social media followers and reblog to help spread awarenss. P.S. You have has almost convinced me to take up knitting:)
Thank you for the feedback. I was trying to be informative without saying things like… this sucks, scleroderma has stolen my life, and I feel like punching people who say “I hope you feel better soon.” Yeah, right. Even when we are between flares life is hard. Having said that, I hope your flare lets up soon and you can get back to writing! When I’m bad I can’t even read! Ugh!!
Reblog by all means! Anything we can do to raise awareness.
I’ve never had an ulcer and my hands are way better than they should be. I highly recommend knitting!!
I really appreciated that you skip the negativity in your posts… I feel like the information comes across better and for those of us reading who have scleroderma, we already know it sucks, lol! Plus, I feel like the scleroderma community shares quite a bit already about misery (for good reason) but when I was newly diagnosed it scared me away from learning more about the disease.
Thanks again for shedding light in the Scleroderma Chronicles 🙂
Thank you. If I focused on the negative I would be crushed so I try to not do that to anyone else. It made me angry that right after my diagnosis I was actively discouraged from learning about my situation on the Internet. What?! It is very scary and basically sucks, but if I can put out a positive voice I want to do that.
Hugs!
❤️
Scleroderma is literally killing me. I just had open heart surgery last month. I have PAH and can’t afford the meds.
Oh, no. I am so sorry to hear this. I also have PAH but it is still mild. Hugs to you! This is a terrible disease.
Thanks for sharing your experience and for raising awareness of this rare disease. My sister has MS, so we’ve learned a lot over the years about autoimmune disorders. Her condition is chronic as well, and she has the less-researched form (primary progressive, not relapsing remitting). Like many things, we aren’t that aware until it touches someone we love.
I’m sorry you’ve been robbed of so much quality of life. That’s the worst. I’m glad you have a sweet kitty by your side, and a passion for a hobby that is both practical and relaxing. I hope your hands continue to cooperate. xo Alys
I am sorry that your family is also impacted by a chronic autoimmune disease. My DIL has MS, and it is such a cruel disease. Hugs to all of you.
I’m very grateful for the online community. I struggle with the world, but there are so many resources available to me this way that I dont really feel all that isolated. 🙂
Thank you for sharing this information and your experience. I am so sorry that you are going through this!
Somedays it feels like a lot on my plate, that’s for sure! It’s annoying to have my life so impacted by a disease that no one had heard of that is also mostly invisible. That’s why we’re encouraged to post on World Scleroderma Day. Thanks for taking the time to read!