The Scleroderma Chronicles: Year 3.0

My, how the time flies. Not that I’m having a good time here, but it is hard to believe that it has already been three years since my diagnosis of limited systemic sclerosis (AKA scleroderma). I’ve been reflecting on the last year while planning this post, and decided that I should start out with a little info about my disease, share the highlights of my three years, and then give unpack this year a little.

Butterfly
But first, a butterfly picture! Butterflies have hard skin, and they are doing OK. Be like a butterfly, I tell myself. This little lady was part of a massive migration of butterflies that came through our state in the fall. Seriously, there were so many of them that they showed up on the weather radar and the NWS put out a bulletin asking the public to identify the bird species: not birds, but butterflies.

Scleroderma is an autoimmune disease that is chronic (no cure), progressive, disabling and possibly fatal. It is rare, which is why you probably never heard of it. The name itself means “hard skin”, and that is one of the most distinctive features of the condition. The widespread scarring and buildup of collagen protein that causes the hardening of skin also occurs in internal organs in patients with the “systemic” form of the disease like me. Most of the damage is hitting my intestinal tract, but my lungs, kidneys and heart are also sustaining damage. In the background, hard to see, but never to be ignored, is damage occurring in blood vessels that can cause blood pressure to soar and places me at risk for blood clots.

I have collected several doctors over the last three years as damage continues to slowly accrue in my lungs, kidneys and intestinal tract.

Here are the highlights of my first three years:

  1. My first year was one of shock and horror. I was so worried about tightening skin and the use of my hands that I didn’t ever think about the bigger picture. I was started on drugs, stabilized, and felt much better by the end of the year.
  2. The bottom fell out my second year. I was using my hands okay, but I developed breathing problems, had to be placed on oxygen, and my heart started to misbehave. Adjustments were made to my medications to compensate for my lowering lung volume and to slow the rate of lung damage. At the lowest point I was sent to palliative care and told to make final decisions.
  3. This year, the third, has been one of highs and lows. The new medications kicked in, I came off oxygen and my chest pain stopped. I was discharged from palliative care. I developed gastroparesis and had to move to a very stomach-friendly diet. I stabilized and sailed through the first rounds of appointments in the spring only to develop kidney problems in the summer along with higher pressure in the artery that goes from my heart to my lungs. This blood pressure, which is called pulmonary arterial hypertension, is extremely damaging to the heart and will need to be addressed if it gets any higher. Fabulous. Another doctor.

My summer this year was really hard. I got very dizzy, developed joint pain and sore muscles, and eventually got so brain fogged that I was afraid to drive. My knees were swollen and developed sharp, shooting pains; I will need to buy a new car if this keeps up as I can’t manage the clutch much longer. I struggled on the stairs and my face turned blue on a regular basis. My neighbors stepped in and took over the yard work for me, and my knitting buddies began to drive me to all fiber related adventures as I wasn’t sure I should be trusted on the road. See, highs and lows. My illness is kicking my butt, and the people around me are stepping in to make sure I’m OK.

Early this month I met with my new internist to see if there wasn’t something that I could take to beat some of these symptoms back. OK, I’m going to be honest here. I cried. We agreed that I would start the tart cherry extract again, but at a much lower dose than I took previously in the spring. (Tart cherry has anti-inflammatory properties and is easier on my stomach than NSAIDs. Unfortunately, my kidneys were damaged the first time I tried to take it.) I’m going to have my kidney function checked every month, but I’m already so much better (brain fog, goodbye!) that I’m really hoping that I can tolerate it OK. In the meantime I’m getting lots of chores done in this golden period while I feel so much better. I’ve moved furniture, completed some projects, and have driven to many, many stores that were ignored last summer.

Wizard Hat
And I made a crocheted had for my niece to wear this Halloween. Really, I’ve been a bundle of energy the last couple of weeks.

So, this is the end of the third year. I feel pretty good, I’m getting things done and making plans, and I am making hay while the (tart cherry) sun shines. Next week I get my blood drawn for the kidney function test and after that I see the rheumatologist.

I’ve been thinking about butterflies again. The day after I took that picture of the butterfly it snowed. A lot.  It took a couple of days for it to melt as the temperatures climbed back up into the 60’s and 70’s. I wondered if the butterflies would make it. As I walked out of the office building after seeing my internist (and still recovering from my crying fit in her office…) I found butterflies swarming around one of the shrubs by the parking lot.

Those butterflies. You can kick them, but they come back. Be like a butterfly, I tell myself.

And the fourth year begins.

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The Scleroderma Chronicles: Year 2.5

Wow. How quickly time flies. It has now been two and a half years since I was diagnosed with the autoimmune disease limited systemic sclerosis (AKA scleroderma), and I joined the rare disease club. When last I reported on my progress I was at the end of a tough year; lung disease, oxygen, a referral to palliative care, gastroparesis, and constant pain. I was having trouble walking and my stomach was really acting up; I was losing weight at the rate of a pound a week. Ugh. I was somewhat upbeat by the end of the second year since I was off oxygen, but still, things weren’t really all that great.

Mitts and bracelet
Look at the cute mitts I made in teal, the color of scleroderma. I got that bracelet last fall at a scleroderma patient education conference that loaded me up with lots of great information.

What a difference six months can make. A change in medication made my Raynaud’s much easier to handle. I started doing yoga (carefully) a few months ago and added tart cherry extract as an anti-inflammatory that I can tolerate better than traditional NSAIDs. I bought shoes that felt better on my feet (Haflinger clogs) and got serious with stretching exercises for the plantar fasciitis I developed due to tightening on the bottoms of my feet. I switched to a gastroparesis diet that is mostly easy-to-digest, low fiber foods (got to love pasta and yogurt to do this one…) and began to add some blended  fruits and veggies. My weight loss stopped and I started to gain back some weight. My skin has started to loosen up on my right arm (scleroderma literally means “hard skin” as the most obvious symptom is thick, hard skin that develops as the result of systemic scarring), which is pretty darn exciting!  Over the last six months my energy has been slowly coming up and I have been managing with less pain; I can even walk normally! I’m breathing better and I rarely need to use the rescue inhaler. There was a Sjogren’s flare, but still, things are better.

I just finished making the latest round of testing and doctor visits, and I have to say, it is pretty exciting to visit with happy smiling doctors. My pulmonologist was almost giddy! My lungs and heart have maintained (scleroderma damage tends to be forever…) with almost no new damage; I am right at the edge of trouble, but I’m not there yet. I have good blood work, and even had the first NORMAL kidney function test in over 5 years. My red blood cell count is back down in normal range, and my muscle strength has improved so much I can now easily push shopping carts.  It seems that the increased dosage of immunosuppressants has turned the tide and I am stable. STABLE!! What a wonderful word that is.

I will continue with all of my current drugs for now and there will be more testing in a year, but it is clear that I have definitely pulled up from the nose dive that they through I was in a year ago.

Life is good.

Back to knitting!!

 

The View from Here: One Year of Scleroderma

It’s been a year since I was first diagnosed with scleroderma. Actually, it has been decided that I have the form of scleroderma called limited systemic sclerosis (the disease formerly known as CREST). Sounds kind of fraught, huh. That’s because it is; no matter what I think I know about my condition this week, by next week things will probably change. It has been quite a journey this year and now that all the dust has settled from the latest rounds of medical tests I wanted to share my thoughts.

There is no question that the first months after my diagnosis were filled with waves of horror and grief. At first I was just stunned to discover that I might lose the use of my hands. Then it dawned on me that my ability to live independently might become problematic; I needed to make plans to activate a support system for myself at some time in the future. Eventually I found out about the fatal complications and the high mortality rate. Well, shoot. During this time I became a driven knitter: endless pairs of fingerless mitts rolled off my needles.

CARD
My sister, who has a somewhat warped sense of humor, sent me this card about the time that I learned about my risk for pulmonary hypertension, an often fatal complication of limited systemic sclerosis.

Yet, it has been a year and I am still here. I sailed through the medical tests and none of the frightening medical complications have manifested themselves. My lungs and heart are fine (huge woohoo!!). My GI tract hasn’t gotten worse. My hands are very swollen and the skin is getting hard (one of the hallmarks of scleroderma is hard skin that forms due to lots of collagen deposition and scarring; my arms and legs are getting hard too), but they work just fine and are actually much better than they should be. My rheumatologist has advised me to knit as much as possible; what a hardship!  Other symptoms have improved and I have transitioned onto a battery of drugs that have good track records for improving the quality of life and increasing survival rates in patients with my condition.

So, what have I learned this year? Here is my reflective collection of observations as I look back from the one-year viewpoint:

  • People are more important then things. Period.
  • ..but they just don’t get chronic conditions and they tend to underestimate the seriousness of conditions that they can’t directly observe. In general people on crutches or using oxygen activate concern, but if you have flaming gastritis, the dizzy wobblies, and your muscles are on strike it’s easy for them to adopt a dismissive attitude. <sigh>
  • Medical personnel can be appallingly insensitive. Last week the technician doing my lung scan mentioned that I had a lot of doctors because my disease was really rare; it was important that the doctors get to learn as much from me as possible. Good lord, I wondered. Maybe she was raised by wolves…
  • As a corollary of the above observation, I have also discovered that I can’t assume that the medical personnel that I come in contact with actually know about my condition. One nurse thought that “systemic sclerosis” was “multiple sclerosis”. A doctor gave me a pep talk about how other conditions were worse because some of those patients actually can die from their disease. (“Did you want to hit him?” asked my rheumatologist…) One thing I can count on, however, is that they will put me into some scary machine to look for a possible blood clot. What is up with all the blood clot hunts?
  • MedAlert ID
    I do have some wonderful doctors! Because of crazy interactions with random medical people I have decided that it is best to be proactive: last month I put this tag onto my keys and the little card in my wallet has the business cards of my internist and rheumatologist stapled to it. 
  • There are few things as empowering as discovering that you won’t live forever. Why sweat the small stuff when you are facing down the long odds? My drugs have a small chance of a fatal brain infection? Whatever. Hand them over, Sparky, I am on board! I never worry about money (bad when I’m in a yarn store) or running out of gas, and I am taking more risks than I was comfortable with before. I mean, what is the worst thing that can happen? This week I figured out how to identify all of the electrical circuits of the house and rewired and installed a new doorbell. I didn’t worry (more than once) about getting knocked on my butt by an electrical mishap. Ha! In my younger days I hated to even change light bulbs…
  • Stay Calm and Carry Yarn
    Elizabeth Zimmerman was right. “Knit on with confidence and hope, through all crises.” is a motto that I can live with.
  • No, I won’t be skydiving! I’m not that risk-seeking. 🙂
  • It is so much easier to be happy than sad. Plant flowers. Read books. Knit like the wind! Chase bees. Go to lunch with friends (and get dessert!). Watch meteor showers and be sure to catch the Super Blood Moon. Talk to strangers in bookstores and coffee shops. Be kind whenever you can. Never miss an opportunity to take a picture of a great sunset (or a cat). It is really, really easy to be happy, even when you’re in a crazy machine that is looking for blood clots. 🙂

I finally went to my first scleroderma support group meeting a couple of months ago. I’d never met another person with scleroderma; it was a little extreme but good. Like me, they all suffer from cold hands. Unlike me, most of them also talked about the struggle to manage pain in their hands, and I could see that several of them had limited use of their hands because the skin was so tight. Remember all of those fingerless mitts I knitted during the sad times? I think that I have found a home for them.