The Scleroderma Chronicles: Getting My Mojo Back!

This post is going to be something of a mess as I’m having trouble distilling all of the issues I’m having with my disease(s) into specific themes. I’ve been pondering and trying to make sense of everything for the last couple of days (while knitting, of course!) and here are the main threads of thought.

    • When you are chronically ill it is hard to be assertive with medical professionals upon whom you depend.
    • In a life with constant symptoms and annoyances, when is it time to insist that something must be done to help you?
    • How should I respond when medical professionals insist that test results prove that the symptoms I’m experiencing aren’t really happening?
But before I start whining about all my woes I would like to offer up this picture of the bee party going on in my garden. I definitely need to plant more of these plants!!

I’d like to set the stage for the little adventure I’ve been on for the last 6 months. Actually, I need to go back farther in my odyssey for this to make any sense. It has been 5 years since I was first diagnosed with systemic sclerosis (scleroderma), Sjogren’s Disease, and fibromyalgia. For the last 3 years I’ve been experiencing painful and burning muscles, joint pain, and swollen bursa. I am just miserable and struggle to walk on some days, and I’ve noticed that I have many other symptoms on these bad days: fatigue, brain fog, edema, eczema, GI woes, hair loss, and shortness of breath. I keep checking to see if I’m running a fever, and I feel so sore and sick it feels like I must be coming down with influenza. I call these surges of symptoms “flares”.

If I break down and take an NSAID (ibuprofen) , a drug I’m forbidden to use as it might damage my kidneys, I can buy myself 2 good days as my flare symptoms fade into the background. Other supplements and medications that I’ve taken also make my symptoms subside, and when I checked they all had anti-inflammatory properties (doxycycline and tart cherry extract, I’m talking to you!). Hmmm… it’s almost like I’m struggling with inflammation, huh.

Because of my observations I’ve been suspecting that I’m in the grips of some type of systemic inflammatory event, and for three years I’ve been consistently reporting to my rheumatologist and others that I’m struggling and that I need help. Mostly I’m told that I have a chronic illness and that there is nothing that can be given to me that will not cause damage to my stomach or kidneys. I’ve cried. I’ve been bounced between doctors. I finally got a referral to physical therapy but I continued to struggle. Mostly I just maintained and accepted my fate, trying to make the best of things. My world closed in as I retreated to my “safe house” to manage my symptoms.

Time for another trip to my garden, don’t you think? One of my mini-roses, attacked by spider mites and reduced to a couple of twigs, has recovered and started blooming this week. It just goes to show, it is not unreasonable to wish for improvement!

In February I tried to raise the pain issue with my rheumatologist again, and she just snapped at me that I was on the best medications available, I didn’t have inflammation because my C-reactive protein was normal, and that I was always complaining, and that maybe she should prescribe me some anti-depressants! Simultaneously outraged and heartbroken, I refused. I got out of there and cried in the car. I clearly needed a new doctor, but I didn’t know of a better one, and I needed more than my own observations to press my case.

Here’s the deal: I used to do medical research on, ironically, scleroderma. I’ve sat through lots and lots of presentations by physicians struggling to treat patients with rheumatic conditions. I know to be skeptical of cookie-cutter treatment plans, and to always follow the data wherever it takes you, even if it seems to be unreasonable at first. I know to remember that even if 90% of patients respond to specific medications and treatment plans, there are still the others who need something different.

Struggling to walk, in pain, and short of sleep, I made appointments with other doctors in my care team. I also began to collect data to support my inflammation hypothesis. I also began to hunt for another rheumatologist.

    • My physical therapist told me my joints were too swollen for me to go to the gym. Or to do yoga. Maybe I could do a stationary bike… carefully.
    • The dermatologist, shocked by my swollen face, immediately prescribed an anti-inflammatory ointment and doxycycline, an antibiotic with anti-inflammatory properties. Wow! Big improvement. Better than ibuprofen!
    • My internist stepped in and prescribed inhaled steroids and another anti-inflammatory gel to put on my hip. Wow, wow, wow!!! Huge improvement. I can breathe again!! Brain fog went out the door, energy came up, and I had less joint and muscle pain. My hair started to grow back in and my stomach pain stopped. This was just darn thrilling, people!
    • This week an orthopedic hip specialist that my internist referred me to told me that I have IT band syndrome and bursitis.  I now need to double down on the anti-inflammatory gel, there is more physical therapy in my future, and injections of steroids into that hip bursa will happen if my immune system, already suppressed, can handle it. Because I’m on high risk medications to crush my immune system into submission these things have to go slowly, but the process is now in motion.

I don’t know about you, but it sure seems crystal clear that I’m dealing with inflammation, right? Imagine my shock (NOT) to read a research study last night that showed that C-reactive protein, the inflammatory marker that my rheumatologist uses to assess my disease, does not become elevated in scleroderma’s fibrotic process. You know, the process that made my IT band become so thickened and inflamed that it is clearly visible on my x-rays and responsible for causing my bursitis. “We can’t be sure that scleroderma caused this,” said the orthopedic specialist. “Well, I’m pretty sure I didn’t get this from knitting,” I snarkily replied. We also discussed if I might have bursitis on the inside of my hip joint. “Well, that would be unusual,” he said. “Please, you’re talking to someone with a rare disease,” I replied. He acknowledged the point. This is how normal doctors talk with to their patients.

Shame on my rheumatologist who doesn’t let me talk to her, who used inappropriate data to dismiss my symptoms, and then finally disparaged me as I pressed for acknowledgement and help. I don’t know if an earlier intervention could have saved me from bursitis and scarred tendons, but now I have a clearly established track record that documents inflammation and I feel empowered to move on to another doctor. I talked to some patients active in our local Scleroderma Foundation chapter, and I have the name of a rheumatologist who is treating several other patients with my battery of conditions.

Which takes me back to my original points that I have been pondering for weeks:

  • When you are chronically ill it is hard to be assertive with medical professionals upon whom you depend. Yes, it is. I forgive myself for crying in the car. I was blindsided and ill-prepared to argue with a specialist who deliberately places patients on the defensive to fend off requests for help. Who could have imagined such a thing? I now recognize that this is a defensive tactic by the physician, it is inappropriate, and I will no longer tolerate it. She took advantage of my dependence upon her to victimize me. If her test results do not account for my observed symptoms, then we need other tests and different thinking. That’s what I learned as a scientist, it is valid, and I will stick to my guns if this happens again. But not with this doctor: I’m moving on!
  • In a life with constant symptoms and annoyances, when is it time to insist that something must be done to help you? Sadly, things are not rosy when living with a chronic disease that has no direct treatment and piecemeal strategies for symptoms. When you can no longer tolerate the life you are living it is time to speak up!!
  • How should I respond when medical professionals insist that test results prove that the symptoms I’m experiencing aren’t really happening? Well, that is poppycock, right? If you are vomiting blood, so to speak, it doesn’t matter what their little image is showing them on the computer! I responded by going to other members of my medical team  and securing medical interventions from them (and thereby building a cascade of supporting evidence), getting full copies of all my test results, doing a literature search on the drugs and tests the rheumatologist was relying on, and activating my network to get the name of a doctor more appropriate for my medical circumstances.
Another rose break.

Today I am 5 days into the increased dose of anti-inflammatory gel to get my bursitis under control and I’ve started my physical therapy again. I am better: I actually lifted my leg to get into the car without assistance! Once again I need to wait a few weeks/months to make sure my kidneys and cell counts are stable before adding another drug like a cortisone injection into that bad-boy hip bursa, but I am making progress and am comfortable with what is happening now.

Believe it or not, after all of this I still don’t have a title for this post. Ugh. What was this about?

Refusing to be a victim!

Manipulating the health care system for fun and (pain-reducing) profit?

Fighting for your mojo when no one else can?

That’s it. Fighting for my mojo. Because, at the end of the day, that’s what I did, and my mojo is back!!

 

Knitting Speed Bump

A few weeks ago I went to see my primary care physician for help with shortness of breath and joint pain. I totally scored! I walked out of there with a tetanus shot (fail), inhaled steroids to control my small airway disease (win) and an anti-inflammatory gel to put on my swollen, painful joints (huge win). What a difference to my life these new medications have made. I can breath! I can walk without pain! I can sleep through the night! This is huge, people. Take that Sjogren’s and scleroderma! Feeling so much better I began to spend more hours up and about, and there was a lot more knitting happening too.

I got some socks finished right up. Here are my project notes.
I made a lot of progress on my Suburban wrap and was lured into knitting more than usual because I just had to get into the next section of the wrap. This is such a fun project with lots of color, texture and lace interest. So addictive. So hard to walk away from…

Oops. Then this happened.

Tendonitis!

One of the problems of getting better is that swelling is going down and the tissue is tightening up on some of my joints. My knees are really tight. My wrists are stiff. When I knit my joints loosen, so I thought I was helping them stay flexible. That is probably true to a certain point, but I guess I now need to be careful to not overdo things. Sigh. I am using the tendons in my left hand the most since I knit continental, and I push the yarn over the needle with my middle finger for each purl stitch. I’m really fast that way, but my tendons have totally rebelled in my left hand and wrist.  To make things worse I can’t take NSAIDS or any other anti-inflammatory drug because of my scleroderma-battered kidneys and stomach. Sigh. My doctor ordered a knitting hiatus.

I want to be a compliant patient, really I do. I read a couple of books, managed to get through a couple of days without knitting, and then I snapped. I must knit!! Want knit now!! Knit, knit, knit. Why go on if I can’t knit? Sniff. Obviously this is totally unacceptable and I am going to figure out how to knit in spite of this bad boy wrist. Really, my left hand is the one having trouble, so maybe I can work around that. I tried to tension the yarn in lots of creative ways before I remembered that lots of people on the planet hold the yarn in their right hands. English knitting. I have never mastered purling English style, but now I’m really motivated!

I was in the last striped section of the wrap when disaster struck; stockinette means I have to purl back every other row. 
I managed to continental knit with the brace on (okay, it is a little loose) and am moving my left hand very little by working slowly and relying on my right hand.
Working very slowly I am also managing to purl back English style. So slow. So awkward. So much safer for babying rebellious tentons.

Last night I finished the stripes and am ready to enter the last section of garter eyelet. Yay. Knit all the way! I can do this! Then it is into the last, ribbed section of the wrap.

Ribbing. That is going to be slow going. I think that I will check out Norwegian purling. Somehow I need to do this without moving my fingers too much. Yay. A new stitch to learn.

I’m on it!

Take that, scleroderma. You are messing with the wrong knitter!!

The Scleroderma Chronicles: Safe House

You know, I kind of view myself as a happy camper. I have more things (ahem… knitting projects) going then I can get finished on any given day or week, books lined up to read, and a “to-do” list that I’m slowly working my way through. Hey, people, I fixed the loose tiles on my kitchen floor last week!! My cat MacKenzie is my constant sidekick throughout the day as I knit, work in the garden, read in bed, and even with me (underfoot, demanding cookies) while I’m cooking. Even on the bad days when I’m pretty much down for the count, I manage small victories. There is just one problem with this picture.

Look at these beautiful coneflowers I just added to the garden. I’m hoping to lure some butterflies into the yard. Every garden should have butterflies, don’t you think?

I just don’t fit in the world all that well anymore. In my home, living the life that I’ve created for myself, it is really easy to forget how much I have adapted to accommodate the limitations of my scleroderma, Sjogren’s, and fibromyalgia. Once I go anywhere else reality hits me hard. Every trip out of my house is going to come at a cost. Here are the worst of the offenders that will lay me low.

Air Conditioning I know that almost everyone in the world is grateful for air conditioning in the summertime, but for me it is a royal nightmare. The shock of walking into a refrigerated building on a hot summer day will trigger an immediate Raynaud’s attack. I pull on long sleeves and fingerless mitts as soon as I get into the building, but my lungs know what’s up and I have trouble breathing. The airflow makes my eyes burn; I’ve been reduced to wearing my sunglasses indoors to protect my eyes. Don’t even get me started on the refrigerated cases churning out cold air; you haven’t lived until you’ve had to pull up the hood of your sweatshirt and the sleeves down over your hands so you can score some butter and eggs.  If that wasn’t enough, there are also usually…

Scented Products Almost all buildings use scented cleaning products and sells additional items with scents. Candles. Lotions. Laundry soap. The scented bathrooms are a nightmare. If I’m not already in trouble with my breathing I will be if I have to walk down the laundry detergent aisle at the grocery store: I also start to itch and my face swells. Why do these chemicals even exist? They can’t be good for anyone!

Restaurants These are a special kind of hell for me. All the drinks come cold and with ice, and the entrees are served piping hot. There are other landmines that I need to avoid: salt, lactose, fiber.  I have to carefully select something that is very soft and that will behave itself in my gastroparesis stomach. I can’t have spicy food. I can’t eat fresh veggies. Actually, to be safe, some of this food should go through a blender… I actually once soaked a cut up sandwich in soup so I could eat it…

Walking I am trying really hard to meet my walking goals every day, but I stretch those steps out over the day. A trip to run errands can be just exhausting if I’m on my feet for a couple of hours at a time. I need to always carry water, be aware of the location of bathrooms, and have places where I can sit down if I need to.

Sunshine It makes me sick! Enough said.

Ready for another picture? These paper wasps are building a nest right on the edge of my deck! MacKenzie and I are not amused. Still, it kind of shows how the outside world is full of dangers…

Recently I had a tough talk with myself about pruning down my outings and being more strategic about how I expend my energy. I need fewer outings, and my destinations need to be closer to home. I need to live online. I need to in a safe environment as much as I can to manage my diseases.

My home is my safe house. I have no air conditioning and I minimize air flow. I keep the temperature in the mid 70’s in the day so that my joints and lungs will be happy. I cook all of my own food, I don’t own any salt at all, and everything that I drink is room temperature. Fruits and veggies go through the blender to become smoothies. Every product that comes into the house is scent free. I’m always close to a bathroom or a soft surface to crash onto for a quick recovery if I get dizzy. My stairs have wrought iron rails that I use effectively on bad joint days. Flourishing in my safe house I sometimes forget how sick I am because, well, I have fewer problems.

I planted this yarrow last year in a flowerbed that has killed almost everything planted into it. Not this yarrow!! The secret of gardening, and living with serious chronic illness, is to keep on trying new things, and to match your needs to your environment. Or, in my case, make your environment match your needs.

Tomorrow I have a doctor’s appointment so I listed up some symptoms and issues that I need to ask her about. It is quite a list now that I look at it, and it kind of underscores how chronic illness can trick you into thinking that things that would normally send you screaming into urgent care are “just another day of scleroderma.” Shortness of breath is an almost daily thing. When I glance into the mirror these days I sometimes notice that my face is blue. One hip keeps failing me; okay, I actually have to lift that leg to get into the car. My joints swell so much that I can’t sleep at night.

But I am good, here in my little safe house with my gardens and cat.

Tomorrow my doctor and I will attack some of these scleroderma/Sjogren’s issues. I kind of think that lung testing and a MRI of my hip are in the future, and that there may be follow-up with my pulmonologist. I’ve been gathering up my energy in preparation for these outings into a world that is dangerous for me, knowing that after each outing my garden swing, knitting and latest book will be waiting for me. With a room temperature ice tea.

And a cat!

It is good to have a safe house.

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.

The Scleroderma Chronicles: The Ankle Adventure

The last few weeks have been hard : rain, snow, an exceedingly badly behaved ankle, and all the fun of never-ending chronic complaints. Ugh! The weather fronts just kept rolling in, sending the air pressure swinging wildly and my breathing and joints into their own little crises. “Will it never end,” I asked my ankle and MacKenzie? “Nope!” said the ankle with a little sneer in its rotten little ankle voice. “Don’t count on it,” said MacKenzie as he squirmed deeper into the current knitting project with one paw extended, claw flashing, reaching for my yarn.

As you can guess, I’ve developed a nasty mood of my own.

There was a nice week with sunshine that made me and the ankle feel better: I got some yard work done and planted flowers. Then the weather turned on me and once again I was in bed, listening to audiobooks and knitting with my ankle propped up.

In the wee hours of Tuesday morning I woke up to the crack of breaking branches. Six inches of heavy spring snow (the latest storm this bad in 17 years in the Denver area) had done my tree in. No wonder my joints are cranky… even the tree gave up! I took this picture the next day after most of the snow had melted.

What is up with this ankle, you ask? Good question! Three weeks ago it suddenly developed a hard, red, and hot lump that radiated pain ruthlessly. The infection began spreading under my rhino-hide scleroderma skin. I was started on antibiotics, but the pain continued, the redness continued spreading around the outside of my ankle, and a dent in my leg appeared where the redness was. I chatted with a doctor online and she decided that I should be checked for cellulitis. Cellulitis?! Off to urgent care I went, and as soon as that doctor saw my ankle he ordered testing: another blood clot hunt and  x-rays to see if the bone was damaged. Limping and in pain, I headed off to get the testing done. As I drove home from the ultrasound a call came in with the results of the testing: a benign tumor was found, and I was being referred to a dermatologist for treatment.

It’s like they forgot about the cellulitis! “Where are my new antibiotics?” I asked MacKenzie when I got home.  MacKenzie just carried on for attention and cookies, so I made the appointment for the dermatologist to look at my ankle before returning to my knitting and propping the ankle back up. I made a little cage out of a box to put in my bed so I could sleep without the covers touching the ankle… “I have a tumor,” said the ankle in its nasty little voice.

The next day the redness and heat were a little better, but the pain and lump on my ankle remained. I resolved to head back to urgent care if I got worse, and carried on with my knitting. Tumor, whatever. I have scleroderma and I was betting that was what made the lump appear unusual in the ultrasound. I was pretty sure this was just another chapter in the scleroderma adventure.

Two days later I got in to see the dermatologist. Wow. Just wow. I am in love with this doctor. This is the doctor that I have been waiting for since my first diagnosis FIVE YEARS AGO!

Aside: I have been battling eczema for months. All of my doctors have seen my eczema face. Since I have noticed that a dose of ibuprofen (which I’m not supposed to take because of my iffy kidneys…) will give me 24-48 hours of happy joints with no fatigue or brain fog symptoms, and some eczema relief,  I have literally begged for some type of anti-inflammatory drug to help me. Even though I am taking drugs that are crushing my immune system unto submission, there is something else that I need. I have cried in my internist’s office. Evidently there isn’t anything that can be given to me that won’t hurt my kidneys…

This dermatologist entered the office, took one look at my swollen, red and itchy face, and said that this was unacceptable and she was going to put a stop to it. Then she said that she had looked at the ultrasound and that there wasn’t anything that worried her there: scleroderma had caused fibrotic tissue to form. Then she wondered out loud why they had forgotten that I had cellulitis? Then she prescribed antibiotics that are also anti-inflammatory along with an anti-inflammatory cream to get my eczema whipped into shape. I was told to email her in a couple of days if there wasn’t a dramatic improvement to my ankle and face because she was going to make more referrals to get to the bottom of the eczema with an allergy specialist. And that the ankle should continue to be watched.

Look at how well my flowers came through the snow storm! I put a plastic garbage bag over them to keep them warm propped up over a tomato cage. You’d never know that they had 6″ of snow dumped on them! Just like my flowers, I came through the ankle adventure storm looking pretty good!

By the next morning it was obvious that I was better. Much better. Within a week my face was clear and the eczema was gone. My ankle is also much better, the lump is gone, but it continues to ache and carry on when I walk. My brain fog is gone. Fatigue? What fatigue? It’s like I needed an anti-inflammatory antibiotic or something…

My new, most wonderful in the whole world dermatologist is going to maintain me on the antibiotic. I’ve been on this drug before so I’m not worried about my kidneys at all.

I’m sorry I said all those nasty things to you, ankle. All in all, you were a blessing in disguise.

But anytime you want to stop with the achy hurt, that would be fine!

The Scleroderma Chronicles: Flares and Zebra Nonsense

My last flare started sometime in the depths of December and dragged on for almost two months. It’s hard to begin to describe what is happening to me as my illnesses (systemic sclerosis and Sjogren’s Syndrome) intensify and the walls of my world gradually close in as I descend into the ever-deepening abyss of escalating illness. Always unpredictable, it starts before I am even aware that I am in trouble and before I know what’s up I’m a hot mess. Even now, as I write this, I can’t recall what the first true symptoms are, but I’m pretty sure that in the middle of my usual challenges with pain, fatigue and dryness the other problem children creep in the door and take me down before I even know they are there. As the flare builds momentum new symptoms erupt daily including:

  • Shortness of breath – I literally start panting every time I move
  • Gastritis that makes my stomach burn whenever it is empty, and
  • Gastroparesis that makes my stomach hurt whenever I eat
  • Intense itching and eruptions of eczema
  • Hair loss
  • Crushing fatigue
  • Sleep interruption
  • Swollen joints that won’t bend in the morning, and
  • Burning muscles that are too sore to touch
  • Brain fog and disorientation that makes me afraid to drive and unable to read
Edema on arm.
 Oh yeah. There is edema, too. Check out this arm!

Flares are a test of patience, but they always do come to an end for me. Somewhere towards the middle of February, for no reason that I can put my finger on, I slept soundly through the night. Wow. That was great, I thought. A couple of days later I realized that my energy was coming back and that my stomach didn’t hurt any more. After a week I took a shower and there wasn’t a wad of hair deposited in the drain. My thinking became clear, my driving fearless, and my muscle pain and swollen joints receded to background levels.  The itching stopped, the eczema disappeared, and I stopped using my inhaler. It was over; the storm had passed.

When I mentioned to my rheumatologist last month that I was having these flares things suddenly took a left turn and my whole appointment went off the rails. She first told me that there wasn’t any treatment that was more effective than what I was already receiving. (I know that, my systemic sclerosis is pretty stable, and I am grateful. I think that it is Sjogren’s causing all the trouble, but it has to ride in the treatment back seat since it probably won’t kill me.) Then she reminded me that I always mentioned my fatigue and muscle pain, and that maybe I should be tested for sleep apnea or given antidepressants. Suddenly, instead of talking about the conditions for which I was already diagnosed, we had to talk about depression and sleep apnea. I was defensive and almost in tears. It took days to process what had happened there.

MacKenzie the cat.
The poor Mother of Cats needed extra attention for a few days…

It all boils down to two essential truths. I am a Zebra. I have been betrayed by a medical system designed to treat commonly occurring conditions when I suffered from a rare disease. I have had my symptoms dismissed, disparaged, or ignored for literally decades. For my rheumatologist, who I like and trust, to do this suddenly threw me back into a defensive, victim-like posture. I will not let this happen again.

The other essential truth is that it is insulting and counterproductive to cherry-pick out a couple of symptoms from the entire package that I call a flare, concentrate only on them, and then build a diagnostic hypothesis that addresses only those isolated symptoms. Yes, I do have muscle/joint pain, fatigue, and sleep disruption, but let’s not forget about the gastritis, itching, hair loss, edema and shortness of breath. Once I am out of the flare, my sleep is pretty good and my fatigue is much reduced. I know that my rheumatologist wants to help me, but I’m going to insist that we stick to the data and that logic and reason are employed as part of my treatment plan. Even if that plan includes a clause that says… you are currently receiving the best care available and there is nothing else we can do for you because scleroderma and Sjogren’s sucks. Well, all right then. Give it to me straight, because I can handle that, but don’t hunt for ANOTHER condition that you can medicate without better data.

Because I don’t have sleep apnea. I’ve been tested twice in the last three years. I wear a Fitbit that shows that I’m in deep sleep for 1-2 hours a night and that I’m almost motionless all night long. It is time to put that hypothesis to bed and to spend more time talking about things like this lupus-like rash that has appeared on my face…

I’ve also been checked for depression and I’m fine. Yes, I am aware that MOST chronically patients need help with depression, but that doesn’t mean ALL patients require additional drugs.

After all, I’m self-medicating every day with knitting!

I finally decided to write about this experience in my chronically ill life in case it could help someone else. Okay, I also needed to vent a little!

Should I perhaps knit a little something for my rheumatologist? It must be hard for her to treat patients every day who are dealing with painful, progressive and incurable diseases; no wonder she sometimes grasps at diagnostic straws hunting for a way to help.

But if she does it again this Zebra is ready to deal with that nonsense!

The Scleroderma Chronicles: An Autoimmune Zebra

“When you hear hoofbeats, think of horses, not zebras.”  ~medical school proverb

Zebra ~medical slang for an exotic medical diagnosis

Author as 16 year old student.
16 years old: my last year illness-free.

As a teenager I struggled with eczema and was photosensitive; I was started on steroids, told to wear long sleeves, and to stay out of the sun. Without a clear cause for my eczema/rash I told that I was high strung and unable to handle stress; if I could control my emotions I would see my symptoms disappear. Eventually I did get better, the drugs stopped, and my life went on.

In my early 20s I had my first asthma attacks. I didn’t really understand what was happening, it was always in the night, and the doctor couldn’t find anything wrong. I was told to handle stress better. Most of the time I was okay, so I just learned to deal with it.

In my early 30s my knees began to swell and become painful. There were lumps below and behind my knees. I had an ultrasound to make sure the worst lump wasn’t cancer. I was told to do special exercises and to stick to low impact. My eyes hurt and I couldn’t go outside without sunglasses. Clouds of floaters swam across my vision. You have allergies, said the doctor. My finger was too fat for my wedding ring so I resized it.

In my 40s my hands became numb and swelled. I had a lot of pain and struggled to manage routine tasks. Carpal tunnel, said the doctors: it’s a repetitive motion injury and you did this to yourself. I had the surgery on both wrists and my hands did get better, but I still struggled with swelling and numbness, even in my feet. I developed a rash on my face. Rosacea, said the doctor. My knees still hurt. I started to develop high blood pressure. Control your diet and exercise, said the doctor. My fingers became too fat to wear any of my rings. I was tested for autoimmune antibodies associated with rheumatic diseases (anti-nuclear antibodies), but the result was only mildly positive so it was dismissed as not significant.

In my 50s my knees were so bad I had to take ibuprofen every day to function at work. It hurt to breathe in the mornings, and I had several cases of bronchitis that required an inhaler and steroids to recover from. I developed pleurisy. I was also told that there was nothing wrong with my lungs, and that I needed to get better shoes. My face and jaw hurt so much I couldn’t sleep. TMJ, said the doctor. You need to handle stress better. My kidney function dropped to 35%, alarming another doctor. You damaged your kidneys taking ibuprofen, I was told, and you can’t take any more.  I changed my job so I wouldn’t have to stand so much at work. My blood pressure continued to rise in spite of changes in my diet and exercise; I was placed on a beta blocker to control it. I developed Raynaud’s phenomenon; don’t worry, I was told, it is a side effect of the beta blocker. I wondered why I wasn’t getting wrinkles like my friends. The rash on my face was getting worse.

In my early 60s I noticed that my arm was getting “thick” and that I sometimes couldn’t complete a swallow; food became “stuck” mid-gulp. I complained about dry mouth. My fingers turned blue in the cold. I was hospitalized with severe gastritis. I was stalked by fatigue and my knees hurt every single day. My fingers were too fat to make a tight fist. Unable to function at work, I retired early. I developed colitis and rarely left the house. Testing showed that I didn’t have an intestinal infection, and my doctor didn’t pursue things any further.

Struggling with life and my early retirement,  I grew roses, read books, knitted, and launched this blog.

One night the light went off for me. I had joint pain, kidney damage, intestinal woes, Raynaud’s, a rash on my face, and a positive ANA. I walked in and demanded testing for lupus.

How is it possible, asked my doctor when confronted with the list of symptoms, that you haven’t already been diagnosed? Shocked that I had slipped through the cracks for years, my doctor immediately ordered the complete diagnostic battery for rheumatic diseases. It wasn’t lupus after all, but a couple of relatives: scleroderma and Sjogren’s syndrome, two rheumatic systemic diseases that unified all of my symptoms. The decades-long string of mystery complaints was over, and I was started on aggressive treatment for these two autoimmune diseases.

How was it possible that I hadn’t been diagnosed earlier? Isn’t that a wonderful question! How could I have slipped through the cracks year after year as I struggled with pain, respiratory infections, dysfunctional hands, eye problems, TMJ, and all the other medical woes that had parked themselves at my door? Why was there no diagnosis when, as I was to learn later, I was an absolute classic case of limited systemic sclerosis, a type of scleroderma, and that my Sjogren’s was obvious and well established.

In retrospect, the answer is glaringly obvious: I am not common. I have a rare disease (systemic sclerosis), and even though my doctors were well intentioned, they dismissed my symptoms when I didn’t fit the usual diagnostic profiles. I was a zebra in a herd of horses, ill-behaved and refusing to fall into line with their medical school training. Since I couldn’t be diagnosed with any of the normal causes for my symptoms, doctor after doctor concluded that they must be due to something else, like maybe stress… or lack of exercise… or my choices in pain medication. Time after time, I was assigned the blame for my own illness because of my inability to “handle stress”, repetitive motions, bad shoes, lack of exercise, taking ibuprofen, or any other excuse reasonable explanation that came to mind when my symptoms could not be ascribed to common causes. My doctors had been trained to ignore zebras, and these other causes were more plausible to them.

The other reason this happened was because my symptoms were always presented to my doctors in isolation: chest cold, painful eyes, knees that won’t bend, and so on. Symptoms that emerged over decades, and were presented to different doctors. No one saw the big picture until I finally pieced it together myself and then my doctor was shocked by the list: grouped together my symptoms screamed autoimmune rheumatic disease.

Why am I reflecting (and writing) about all of this? This month, March, is National Autoimmune Awareness Month. My story is one that is shared by many, many other people who deal with autoimmune illnesses. One of my diseases is rare (scleroderma), but the Sjogren’s and fibromyalgia are not. In a way, to have an autoimmune disease is to be a zebra because these conditions are elusive, can present themselves with a battery of symptoms that are seemingly unconnected, and don’t respond to the usual courses of treatments like antibiotics, diet and exercise. They can take, just as mine did, many years to diagnose. For many autoimmune patients, they are, just as I was, zebras crying for help in a herd of horses. Trained to treat horses, doctors don’t always hear the cries. One way to combat the problem is to educate the zebras so that they can, just as I did, recognize and group their symptoms together in a meaningful way to present to their doctors to help them make the diagnosis. Autoimmune Awareness Month is meant to educate everyone who might deal with an autoimmune disease: patients, families, caretakers, and doctors.

In my family we know these illnesses well. My grandfather died from complications of rheumatoid arthritis and my father had disabling allergies. I have scleroderma, Sjogren’s syndrome and fibromyalgia. My son has type-1 diabetes and my daughter-in-law has multiple sclerosis.  In all of these illnesses there is an immune system that is attacking normal tissue in our bodies; for some of us the attack is moderated by drugs that are designed to disable parts of our immune systems, but it continues nevertheless. Unless there is a cure the damage will continue to accumulate in our organs and tissues. These are the words that are often used to describe autoimmune illnesses such as ours: disabling, progressive, incurable, potentially fatal.

We aren’t alone. There are around 50 million Americans who also have autoimmune diseases. There are over 100 different autoimmune diseases and the need for research, support and treatment is endless.

You can learn more about autoimmune disease at these resources: American Autoimmune Related Diseases AssociationNational Institute of Allergy and Infectious Disease  ,   and Autoimmunity.