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.

The Scleroderma Chronicles: Invisible (Star) Wars

I’m a big fan of the movie Star Wars. Seriously. I remember the first time that I saw this movie; we stood in line for hours, laughed at the droids, were amazed by the special effects, wished we had the Force, held our breath as the tension at the end of the movie mounted, and cheered wildly when the Death Star blew up. Over the next year we watched the movie 13 times in the theater, memorized the sound tract, and to this day I hear snippets of dialogue echoing in my mind…

Star WarsLike this line said by the droid C-3PO to his buddy R2-D2 as they escaped from their badly damaged and captured ship to carry out a secret mission as directed by Princess Leia:  “That’s funny, the damage doesn’t look that bad from out here.”

Author
Bundled up in wool and staying warm. You can’t see my stage 3 kidney disease, interstitial lung disease, struggling gastric and intestinal organs, burning muscles, painful joints, brain fog, and my truly astonishing fatigue.

That’s me. Badly damaged on the inside, not that bad on the outside.  The curse of all people who have an invisible illness. People often say, when they learn about my illness, something along the lines of… “Well, you look great!” It’s nice to hear, but it also suggests that I’m not really that sick.  I can’t help but feel that they think that I am an attention-seeking hypochondriac. Sigh.

Raynaud's
You have to admit, the visible symptoms are really subtle. It can be as little as lost circulation in a finger: Raynaud’s phenomenon. What can’t be seen is that I’m also experiencing circulation loss in my lungs, kidneys, brain and other organs.
Edema on arm.
A poor dietary decision can hit me hard: dizziness, muscle pain and edema in my arms. I ate cheese enchiladas at my favorite restaurant this time.

It is amazing, really. How can the damage not look that bad from out here? I struggle for air. I often lack the energy to get through basic tasks. Pain stalks me waiting for a poor decision on my part that will give it an opening.  An incoming weather front pushes me over a cliff. Holidays can be the worst as I struggle to manage my energy resources, diet and exposure to cold. I can get through the Christmas dinner okay, but the next two days are spent in bed sleeping myself back to functionality.

And yet, I’m starting the New Year feeling pretty darn chipper, well… as chipper as you can after a 12 hour nap and a strong latte to launch myself into motion. See, I can get back to functionality. I’m on great drugs and I have wonderful doctors. My latest round of medical testing shows that I am tolerating my drugs well, and my disease progression has virtually ground to a halt. I am making some gains. I spend a lot of time managing my symptoms and hoarding energy resources, but I am not getting worse. Some really scary words on my chart have gone away over the last year: chronic respiratory failure, pulmonary arterial hypertension, and severe kidney disease are no longer there. I have wonderful friends and supportive family members. I am still independent and can get out to social events.

MacKenzie the cat.
I have the world’s bossiest cat to tend to me…
Rose.
and my indoor roses are blooming.

The rose bush is also struggling and covered with mildew. Sigh. It’s like a metaphor for my life. Bloom where you can, and pretend that all this other damage isn’t happening over there… Jedi mind tricks can be useful when dealing with implacable enemies like scleroderma and mildew.

Use the Force! I hear in my mind. If you know the movie Star Wars and the other movies in the series, it is a tale of heroic underdogs battling against great and evil foes, desperate times, hope and change.

I don’t have a light saber, but I have knitting needles.

I don’t have the Force (an invisible energy field created by all living things), but I have an online and real network of people and other living things that connect to me and support me. I have science and time on my side: new drugs are on the way and some of them are in new rounds of clinical trials. Remember the movie poster? A New Hope. Yep. Every year brings me a new edition of hope.

My illness is mostly invisible. The battle is real. Bring it on, 2019, I am ready for you!

Cat

Footnote: Perhaps you are wondering… whatever does she have? I was diagnosed with Limited Systemic Sclerosis and Sjogren’s Syndrome in 2014, and those two conditions carry with them a host of complicating conditions such as Raynaud’s, colitis, gastroparesis, interstitial lung disease, kidney disease, heart disease, oh my lord, and a partridge in a pear tree. In 2016 I was referred to palliative care and told to make final plans; Myfortic (CellCept) saved the day and I was discharged from palliative care a few months later. In 2018 my rheumatologist added fibromyalgia to the list and there is an ongoing discussion about dermatomyositis. Why do these autoimmune diseases throw parties and invite all of their friends?  It’s like the bar at Mos Eisley Spaceport with all the strange aliens.  As Obi-Wan tells Luke, “You will never find a more wretched hive of scum and villainy. We must be cautious.” I don’t have a blaster, but I think that in this case the drug Myfortic will do the job!

Indoor Roses

It’s been a long time since I’ve talked about the garden. Over the last couple of years, forced indoors because of my scleroderma, I have had to settle for some potted plants out on the back porch. My favorites among these plants have been surviving in the garage during cold snaps and snowstorms, but we have finally reached a point where the cold is too prolonged for that strategy to work any longer. Time for them to come indoors! I gave them all a little spray of neem oil to kill hitchhiking pests, dragged them inside, and put them near windows. Obviously that wouldn’t be enough light for the little rose bushes. You know, the kind that you buy at the grocery store when you meant to just buy bread, milk, and ice cream. I have several pots of those roses and those babies were growing in bright sunshine all summer! Last week I headed on over to my favorite garden center to see what I could do to help my floral buddies survive over the winter in the house.

Light on roses.
I found a nice little grow light and a stand that, with a little ingenuity, can provide lights for the roses on my bedroom dresser. 

While I was unpacking the grow light and putting it into the stand I noticed that the light fixture itself was designed to also be attached below ceilings, shelves, cupboards, or some other solid feature. There are two little metal brackets and two screws involved. Hey, this is something I can do. I have more plants needing light! I headed back to the garden center.

Grow light on shelf.
I have a cheap set of wooden shelves in my sewing room for plants. Behold! Lights for my orchids! That pink flowering plant has a scent, and the blooms make me happy. My sewing room is really inviting now.

I’m pleased with how the plants are managing with the new light, but there is an obvious readjustment going on with the little mini-roses. They are undergoing a transition with their leaves…

Yellow rose leaf.
All of the summer leaves that grew in full sunshine are turning yellow and dropping off. Seriously. ALL of the leaves.
New growth on the rose bush.
New growth is bursting out all over the stems following the dropping of the summer leaves. Crazy, huh. I am pulling off yellow leaves and pruning almost every day as the rose plants go through this process. 
Rose buds.
The new leaves look healthy and glossy, and the buds are continuing to develop just fine.
Rose blooms.
The plants are even managing to bloom while they are adjusting to the new lights.

I’m pruning the little bushes down to a lower height as they finish up with the last of the summer blooms and the old leaves drop off. There is so much new growth on the stems I’m pretty sure they are going to be fine.

It looks like I’m in business. Winter roses. I may not be able to get outside all that much now, but with the new full spectrum grow lights and the indoor roses it’s like I’ve brought the garden indoors.

Once again, I am knitting in my garden surrounded by my roses.

Take that, scleroderma!