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.

The Saturday Update: Weeks 19 and 20

The last two weeks have been busy, but not all that productive in the crafting/gardening/reading departments. I’m still busy going to appointments, doing my physical therapy (hey, I have graduated from the walker!!), and putting my yard and gardens back into order. Somewhere during the last two weeks the CDC here in the US changed their recommendations on mask wearing and then my state also lifted the mask mandate in most situations. Evidently we are now going to rely on the honor system to protect people at risk from other people who are unvaccinated and unmasked. Oh, dear. I have been pulling weeds and considering my options…

Late one afternoon I hurried and pruned my roses in the rain so I could get frost cloths over them to protect the new shoots from an overnight hard freeze. The resident bunny, all alone since the Great Horned owl caught his mate, joined me in the rain as I worked. It’s been that kind of week.

So here is my dilemma: what is a seriously compromised individual on immunosuppressant therapy to do? I hit the internet, of course.

As luck would have it, Science Magazine (the journal of the American Association for the Advancement of Science) recently published an article reporting out on the current data from several studies on this very issue. As it turns out, there are some immunosuppressant drugs that are seriously impacting the efficacy of the Covid-19 vaccines, and one of those drugs, mycophenolate, is of particular concern. Rats!! I’m taking that drug to control my scleroderma. I stopped reading the article when I got to the part about only 9% of patients on mycophenolate produced antibodies to the Pfizer or Moderna vaccines. Gosh darn it, I was vaccinated with the Pfizer vaccine. I wrote an email to my doctor and requested an antibody test to see if I was producing anti-Covid-19 antibodies. Late that afternoon I got a note from her nurse letting me know that I could just make the appointment online and go get the test on my own. I immediately did that and scored an appointment across town at a clinic that I could just make that evening if I jumped into the car and drove really, really fast…

So I drove across the Denver Metro area like a bat out of hell with the prevailing traffic flow and arrived at the clinic with 10 minutes to spare. Yay! Um… why is the parking lot so empty?

The clinic closed 20 minutes before the appointment; there was a goof in the scheduling system. I called my health provider from the parking lot and made sure my test request would transfer to another clinic and/or time and then sadly drove off. I bought myself chocolate on the way home. Why is everything so hard?

Thank heavens there is Hannah to balance out the little bumps in life. 🙂

The next day I got the test done after another drive across town and also heard back from my doctor. She recommended that I return to strict isolation because 1) the mask mandates had been lifted and 2) there was a strong chance that I wasn’t protected from Covid-19 no matter what the antibody test result was. Later in the day an email arrived carrying a letter from the Scleroderma Foundation that advised that I and other patients in my situation continue to wear masks and practice common sense safety measures including limiting contacts. Darn. When will this ever end? I’m starting to feel a little picked on here…

The next morning the antibody test result arrived: I have antibodies!!! Yay! About time I was cut a break, don’t you think? I’ll still be wearing a mask and will be really careful, but I think that I am safe to continue to go to appointments and the occasional book store or yarn shop.

Do you see why I’m not getting a lot of stuff done? All of this driving and decision making is just exhausting.

Knitting

The great thing about physical therapy is that you get better. You get to take afternoon breaks with hot packs on your sore muscles. The bad part about physical therapy is that, for some reason, the inflammation associated with building up muscles and loosening my bad boy tendons (I’m finally rehabilitating from a ruptured tendon in my hip) makes my joints misbehave. My hands especially have decided that they want to be babied and they definitely don’t want to knit. Still, I have made a little progress over the last two weeks:

My Noncho (Casapinka) is finally starting to take shape. The simple stockinette knitting in the round is easy on my hands and I can’t wait to get it done so I can wear it in cool offices during appointments. My socks, which are a take on assigned pooling knitting, are more adventurous with the texture stitches and the purl sections not to mention turning that heel and picking up stitches. Still, I am getting somehere with them. I’m inventing the socks as I go and will take time to explain what I did then they are done, but right now I’m just enjoying the fun of an adventurous bit of knitting fun. Don’t those socks make you happy just looking at them?!

Garden

The weather has totally been messing with us the last two weeks. It has rained… at lot. We have had snow followed by really warm sunny days. The the windy days arrived late this week to knock the new plants around under cloudy skies that threatened severe thunderstorms and tornadoes.

Ugh! I am getting lots of weeds pulled, new bedding plants planted, and sections of the lawn reseeded. Really, I have been working, but all I really have to show for my time is…

Hannah and her hydrangea plant that I pulled inside for a couple of days (hard freeze warning). Hannah thought that the plant was for her and was really involved with it for the couple days that she had it. Sorry Hannah. Hydrangeas need to live outside.

Books

I just started this book. Anyone have any feedback on whether I should keep going?

I was really excited about rejoining my book club next week for their meeting about Wanderers. Nope. Guess that isn’t happening as they are meeting at an indoors restaurant and the Covid-19 restrictions are now lifted so dining capacity is back up to 100%. This is a big, heavy book. It hurts my hands to hold it. If it doesn’t get really compelling really quickly it is going back to the library.

Anyone have a book recommendation?

Have a great week everyone.

Read a little, knit a little, and garden like your heart can’t live without it.