The Scleroderma Chronicles: World Scleroderma Day

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.

Rather than flash my smile I have chosen to show this picture of me and MacKenzie, wrapped in handknits, fighting my way through a flare one frozen January. Good times! See the light in the background? It reminds me that every flare comes to an end.

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.

Vascular tissue damage causes circulation loss to my fingers and toes (Raynaud’s Phenomenon) which I fight by wearing fingerless mitts and wool socks. Works great and the Zen of knitting brings its own benefits.

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.

Science and the Scleroderma Girl: The AP Therapy Rant

Antibiotic Protocol is an alternative medicine therapy for autoimmune disease. It certainly has a following. Check this out.  There is more information on it at the Arthritis Foundation. It seems to be loosely associated with “Leaky Gut” as it links chronic autoimmune illness to microbial sources originating in our intestines. The shaky model and vague language involved in leaky gut has landed it on the pseudoscience list at Wikipedia.

This has been a really hard post to put together. I have all these little memories and reactions rolling around in my mind, and I struggle to link them together in a meaningful way. Here are some of the things that have happened and my gut reaction (did you see what I did there?) to them:

  • I have a friend with lupus. She has been struggling for years, is on disability, and is clearly in trouble. She is losing weight, her hair is falling out; she is in the care of a nutritionist who has her on severe dietary restrictions and antibiotics to treat her condition. She eats no dairy, gluten, meat, alcohol, or sugar, and continues to take her antibiotics without fail. She is not getting better. My heart breaks every time I see her.
  • My dermatologist placed me on doxycycline (one of the antibiotics used to treat scleroderma in AP Therapy) right after my diagnosis. A year later my lung disease was moving so quickly that I was actually referred to palliative care. I stopped the doxycycline, my rheumatologist boosted the immunosuppressive drug dose,  I was given a new drug for my vascular disease, these new drugs kicked in, and today my lungs look great. Good bye, palliative care!
  • I visit an online support group for scleroderma patients where some people are just devoted to antibiotic therapy. They can be very aggressive in promoting their message and have even private messaged me trying to convince me to switch treatment protocols. Huh? This is kind of extreme. I’m always wary of fanatics because it is more about “winning” and gaining converts…
  • Cat face.
    The Mother of Cats has an active early warning system for fanatics. There is no evidence on Earth that will change their minds…

    So, I already have my back up about AP Therapy. The diagnostic antibodies that I have are to a specific region on my chromosomes (centromeres and kinetochores) that is involved in cell division. Bacteria have their genetic machinery organized very differently from me; why would the defining antibody in my illness be towards an antigen not found in bacteria? (Goodbye leaky gut… it was nice to know you!) That doesn’t mean that I can’t have the medical condition of increased intestinal permeability; correlation doesn’t necessarily mean causation. It also doesn’t mean that antibiotic use doesn’t help some people as the drugs also have anti-inflammatory and cartilage protecting properties, and there is research that shows it helps some patients with rheumatoid arthritis.

Here’s the deal: there are better drugs and treatment options. If you have a mild form of the disease, maybe AP Therapy is what you need. On the other hand, systemic sclerosis is a potentially fatal disease and if it is advancing rapidly the correct response is to go big: use the drugs that have the highest success rate. I feel like I’m a walking poster child for the success of current treatment options. The three drug cocktail (Myfortic, Plaquenil, Losartan)that my doctors are maintaining me on has reversed my lung and heart damage.

Crap, do I sound like a fanatic? Darn. I was worried that this would happen.

I told you, this is a hard post to write. Let me just say I would never, never PM someone insisting that they need to change their treatment plan. Who am I to project my reality onto another person?

What I really mean to say is to think like a scientist: collect data, keep a symptom log, ask questions, assemble a team of doctors to help you, and pursue treatment options that have solid research-backed results that you are comfortable with. Keep collecting data, and don’t be afraid to change if things aren’t going well. There is no best one-size-fits-all treatment plan, but play the odds that are the highest until you get better information. If what you are doing is working, keep going!!

Tomorrow is World Scleroderma Day.

This is a hard, hard disease. All of us with this disease face hard choices.

Hugs to all.