Tag: CREST Syndrome

The Scleroderma Chronicles: Updates from the Sclero Front

It’s spring here in Colorado. The first baby bunny appeared this week, and the plants in the yard are starting to burst out in green buds. The roses are looking like they are going to do really well this year (except for that one that the bunnies ate…) and there was a huge outbreak of my little bulb flowers in the front garden. I have new cushions for my patio swing, and I sit in the sunshine, reading and drinking my morning latte, every warm morning. On chilly mornings I wrap in a favorite shawl and stay out with the cats as long as I can. The first grasshoppers of the year have arrived for the cats to chase, and the air is filled with birdsong. There are even blue jays!! Good days.

The phlox in the front is looking great!

A couple of day have been so warm I couldn’t stay in the sunshine long and I’ve had to deploy the umbrella shade. Then, the next day, there may be snow. This is spring in Colorado: rapid changes and big temperature swings. The same is true for the barometric pressure; look at what happened this week.

My emotional stability has been a little like the air pressure the last couple of weeks as I’ve gone through a round of testing and doctor’s appointments. I was very upbeat when I went in to get my blood draw to check my iron levels, and the bone density scan last month was just routine. I mean, I’ve been feeling soooo much better: more energy, sleeping better, more mentally alert and even my appetite has improved. Suddenly my gut doesn’t hate me, and I am getting out of the house more. I went to my knitting group for the first time in months! I was positive that my test results would reflect the improvement.

Not so much. After weeks of eating iron-rich foods and downing my iron supplement there was absolutely no change in my test results, and in fact, the test that measures the concentration of hemoglobin in individual red blood cells (MCHC) got worse. Seriously? The only gain was one value of 25.5 that went up to 25.6; still too low. After all that red meat, salmon, iron pills, avocado, and spinach all I got was 0.1 improvement? Kind of disheartening as this means that I have to have some invasive testing to see if I’m bleeding in my stomach (it’s a scleroderma thing), and I don’t think my doctor will put it off much longer. (It’s called watermelon stomach) (fabulous) (of course this is rare) (my zebra self is not happy).

Then the bone density scan results arrived. I have somehow developed a fairly serious case of osteoporosis in a short time span; the report says to start immediate treatment. Then I did some googling and found out that the diuretic that I take can cause osteoporosis, and I absolutely shouldn’t have been taking it because I have a strong family history and I’m kind of high risk. (Of course I am) (I stopped the diuretic) (now my feet are swollen) (my zebra self is crying).

I have to be honest. I was kind of crushed by the bad news that I absolutely did not expect. Then I mentally slapped myself around, did my exercises, potted some lavender plants, and went shopping on Amazon for some cheer-me-up jewelry. Just what I needed to pull myself together. One day at a time, right? I already have more than my share of challenges, so I shouldn’t waste any energy on things that haven’t happened yet. Next week I have an appointment with my internist, and we’ll work out a treatment plan for the osteoporosis and next steps for the anemia. (I took another iron supplement) (my inner zebra has pulled itself together again) (the zebra wants to point out that its lips aren’t as blue as they used to be).

I also pulled out the La Prairie sweater and knit steadily through the sadness until the body came off the needles. Gosh. It looks really nice so far. Something has gone right this week…

Today I woke up to a snow/drizzle mixture that was too unwelcoming for even Mateo to go out on the catio. All my joints hurt, and my muscles weren’t sure if they were going to play nice either. Of course, I had to drive across town to get lung testing and a sit-down appointment with my pulmonologist to go over all my results. I put on my cute Weekender Crew sweater, wore my new “in your face, scleroderma!” jewelry, and headed off for the testing. It hurt to breath as I walked into the building. I convinced myself that it was just the cold air; after all, I feel pretty good, and I was overdue for some good news.

The lung testing specialist is now my friend. We laughed and talked and caught up as she got me ready for testing, and then I breezed through all the parts of the pulmonary function testing and the 6-minute walk test. Every single result was cause for celebration as Stephanie (my technician) became more and more excited with the little graphs and data appearing on the computer screen. “This is better!” she kept saying. By the last test she was practically jumping up and down with excitement. “This is great! This is great! she crowed as she walked me to the exam room to see my pulmonologist. “I’ll let him know that you are here.”

I love this pulmonologist. He is the doctor who first listened to me and picked up on the fact that I had a hole in my heart and pulmonary hypertension. He held my hand and told me I could cry when my interstitial lung disease was first diagnosed. He has always been the doctor who was most honest with me; he told me last year after the tide had turned that they hadn’t been sure I would make it a year. He supported me when I halted the anti-fibrotic drug due to quality-of-life concerns. Today he was all smiles as we went over the results and my exam. My lungs have maintained on the scans. I have regained some lung function. The decision to halt the drug was the right one; there is no obvious sign of fibrosis right now. This is the best possible outcome right now; everything that he hoped for. I was the last patient he saw today, and he was pleased to have such a good one. “Best appointment of the day!” he declared as he walked me out. We were both enclosed in a bubble of joyous happiness as we walked.

I took this picture outside the building.

Outside the clinic the parking lot was almost empty. The cold drizzle was steady, the sky was full of sad lumpy grey clouds, and the gloom of early evening was creeping in. Around the building some ornamental trees were just beginning to open their flower buds, but in the shelter of the courtyard on the south side, the sunniest location, one tree was covered in blooms. Kind of a metaphor for the last couple of weeks. Sit in the sunshine and bloom, no matter what is going on in the world around you.

On the drive home, in my mind, my zebra self was dancing for joy.

The Scleroderma Chronicles: June is Scleroderma Awareness Month

Five years ago the course of my life altered forever when some blood test results ordered by my doctor arrived: the results showed that I definitely had two autoimmune diseases. Specifically, I was producing antibodies that were diagnostic for scleroderma and Sjogren’s disease. I was expecting lupus, so this was kind of a surprise shock. After a quick trip to Google to establish what type of scleroderma was associated with my positive test results I burst into tears. It was worse, much worse, then I expected.

The scleroderma diagnosis was the problem. Through an unbelievable sequence of serendipitous life events I was more knowledgeable then the average newly diagnosed patient, and I knew that scleroderma was a disease that impacted connective tissue, that it was progressive, disabling, and that there was no cure. This was a life-altering diagnosis, and I was in for a long fight that would last the rest of my life.

You see, I used to work in a rheumatology research lab, and I did research in scleroderma. I was a member of the research team that found the first identified antigen associated with scleroderma. I had visited scleroderma patients in the hospital. Later in my life I taught AP Biology and spent years trying to explain connective tissue to students.  It’s a type of tissue that we just never think of, but it is critical in organizing and operating our bodies. Connective tissue makes your skin elastic and strong. It organizes your muscles and makes up your tendons and ligaments. It is a critical layer in your blood vessels, and is part of the essential structure of all of your organs.  In scleroderma all or parts of this connective tissue is under attack by your immune system.

As white blood cells invade my tissues and attack this connective tissue it produces too much collagen in response. The built up collagen produces thick layers of tissue and scarring. My fingers look really swollen, but it is actually very thick hard skin. My skin is also getting really shiny which means that the collagen is hardening up and losing flexibility. Must knit faster!!

You can perhaps see that same thickness on my face, especially on my cheeks. It means that I don’t have wrinkles, but it is also hard to open my mouth, my smile is mostly gone, and I can’t turn my neck well. Crazy, huh.

That is the most ironic aspect of scleroderma: you look pretty darn good, especially if you are a senior citizen like myself, but you actually struggle daily with your illness. For many scleroderma patients their disease just  involves the skin, but for others, the disease is more than skin deep.

As it turns out, my skin is the least of my worries. The rare type of scleroderma that I have, systemic sclerosis, also causes scarring of internal organs. The muscles of my stomach and esophagus have lost function. My kidneys are damaged and I have chronic kidney disease (stage 3). My lungs are scarred and my diaphragm isn’t exactly happy any more. Part of my stomach herniated up into my chest this year… whatever was it thinking of?! My tendons are getting calcified due to inflammation and at least one has partially ruptured. My muscles are sore to the touch and I have bruises everywhere. I have nerve damage and trouble controlling my body temperature. It’s hard to walk. Blood vessel damage affects circulation to my hands and feet and I’m starting to develop open sores (ulcers)… There is a long list of diagnosed conditions linked to my scleroderma, but you get the idea. Pretty much I’m a walking limping train wreck. Well, a knitting train wreck for sure!

There is no cure for systemic sclerosis, but there are treatments that really help a lot. I am taking four different drugs to crush my immune system into submission; it’s a balancing act as I need my white blood cell count to stay high enough to protect me, but low enough to control my symptoms and prevent more damage. I take a drug to shut off the acid produced in my stomach so I won’t accidently inhale it in my sleep since the muscle barrier that usually keeps it in my stomach is now gone. I take a couple more drugs that help control inflammation, and some supplements that help with nerve damage. I’m on oxygen at night. All of these drugs/supplements have made a huge difference for me: my last lung scan showed improvement and my high heart pressure, the most concerning complication that I had, has returned to normal ranges. My kidney damage continues, but it has slowed way down. There is something funky going on with my red blood cell count, but you can’t win them all, right? The main point is that I continue to manage and live independently.

MacKenzie and I last year when I posted this online as part of the “Face of Scleroderma” campaign.

In short, I am a mess. And yet, to the joy of my doctors, I continue to do really well. Okay, I have blue lips, am short of breath, and struggle with tissue damage, but I also continue to thrive compared to other scleroderma patients that they treat. I have had to make many changes to my life, but I have found work-arounds and I still do things that I love. Attitude is all!

Well, knitting, the cat, and the garden are pretty darn essential, too!

So, there it is. What an annoying disease, right? How dare it make you look younger while shortening your life? How dare it do all of this invisible internal damage that makes people think that you are lazy or an attention-seeking hypochondriac when actually you view each day that you are able to leave the house as a personal victory? I’m in several online support groups and there are people dealing with crushing negativity like that. I can see how it can happen; it is so hard to understand something beyond your own experience that is hidden from view.

That’s why there is Scleroderma Awareness Month. It is hard to have a rare disease, especially when it is one that is hard to pronounce (Sclero… what?!). It’s harder still to have one that has no cure and a pretty high fatality rate (hey, with all of the drugs that I’m on my 10 year survival rate is now up to 80%!!). It makes you learn to laugh in the face of terminal complications while forcing you to take every possible precaution to avoid contracting Covid-19. It messes with your head; it gives you power, but it’s also strange and a little lonely.

That’s why we scleroderma patients share our journey with all of you every year so you can get a glimpse of our lives.

 

If you look harder you will see the signs of my scleroderma on my face. The small red spots are called telangiectasia and are symptomatic of my form of systemic sclerosis. The skin of my forehead is tight and shiny, my hair is falling out,  and my dimples are now buried under my thick skin. My upper lip is trying to decide if it wants to turn blue… 

I am the Face of Scleroderma.

Footnote: In addition to scleroderma I also have Sjogren’s Disease and fibromyalgia. The symptoms from these three diagnosed conditions overlap and always make things interesting in sorting out my treatment plan. You can learn more about any of these autoimmune disease by checking out the links in my post.