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: Year 3.6 There is a 911 call…

Yep. It finally happened.

Upset cat
These scary firemen came to the house and took mom away.

It has been kind of a hard six months. I’ve been dealing with a serious flare of my Sjogren’s Syndrome: dry mouth, eye problems (retinal detachments in BOTH eyes), brain fog, and horrible fatigue. Seriously, off the chart fatigue. Bad Sjogren’s, bad!!

But I think that my systemic sclerosis (scleroderma) has been chugging along as well. My chest feels tight, and when I do anything at all I start to breath hard as I catch my breath. Okay, there might be some panting… Sometimes I get dizzy and I have to put my head down. Vacuuming is suddenly a horrific task, but just coming up the stairs or washing out a pan at the sink can also set me off. Sometimes my lips are blue…

Arm with edema.
Check out my edema:  Can you see the details of the quilt that my arm was resting on?

One of the problems with being chronically ill is that you just keep taking things in stride. After all, it isn’t like this is the first time I’ve seen edema like this, or noticed that my lips were blue. I get out of breath all of the time, and I’ve been telling my doctors about all of this for the last 18 months. But somehow, things seem a little worse now. I have heart palpitations and there is a constant pressure in the middle of my chest. I have developed a headache that just refuses to go away, and my muscles and joints are behaving even worse than ever.

So, Friday while I was resting up in bed after the ordeal of making my morning coffee (yep. I go back to bed to recover from getting out of bed…) I decided that I would call the pulmonologist’s office to ask for an appointment. As usual, thinking that I would be on my feet for only a limited amount of time, I planned several little chores to do while I was going to be up.

Arm Warmers
I took this picture of my incredibly cute new arm warmers. This is the Armelitas pattern by knitcats Design, and here are my Ravelry project notes.

Aren’t those the cutest armwarmers? I put them on, and then started the dishwasher, got a load of laundry going, and then pulled on some more warm clothes (ahem… my Cactus Flower socks and Marfa is a Black Elephant shawl over some fleece pants and a denim shirt) so I could sit at the computer downstairs to make the phone calls.

So, I was out of breath when I made the call. I got ahold of the nurse at my pulmonologist’s office to ask her if I should came in for testing right away or if it would be best to make an appointment. As I talked to her my breathing got worse… I just couldn’t catch my breath and I was now outright panting. I began to feel faint.

The nurse called 911.

I ended up sitting on the floor by the front door with the phone on speaker in front of me waiting for the paramedics to arrive. In just a few minutes they had bundled me up and were whisking me out the door leaving a visibly upset MacKenzie watching from the top of the stairs. It was snowing outside; I didn’t have a coat, but I had managed to hang onto all of my knitted items. The paramedics attached me to all of their sensors and then rebundled me in blankets and knitted items afterwards (okay, my hands turned blue. They hadn’t ever heard of Raynaud’s, but they becaume instant fans of wooly warmth when presented with fingers that matched my denim shirt…) My armwarmers were popped back on over the IV line and the oxygen sensor on my finger. They used the shawl as an additional blanket. I never got my breathing back under control, and was still panting up a storm when I got to the hospital, but I was better equipped than most to cope with the cold. I like to think that I was pretty darn fashionable, too.

Into the MRI machine I went, rocking my armwarmers, as once again the doctors went on a blood clot hunt. Every one of my health emergencies has involved blood clot hunts; it’s a scleroderma thing. No blood clot. No pneumonia. No heart attack. After 2 hours sitting on the gurney in the ER my breathing was under control again and my O2 was fine. My best friend Deb arrived with knitting in hand to sit with me. My son arrived in time to take me home again with instructions to not let me drive or stay home alone.

“This is something that needs to be treated by a specialist”, the ER doc tells me. “You need to call your pulmonologist’s office first thing in the morning to get in to see her. You need additional tests that can’t be done here in the ER”.

You think?

It’s hard to not be stuck by the irony of this. I’m back right to where I started. It is kind of known in the scleroderma community that going to the ER is mostly useless because they don’t have the knowledge and experience to treat your condition. I’ll be calling my pulmonologist first thing Monday morning, but this time I will be calling from bed in a well rested condition.

Grooming cats.
with my latte and the cats!

For those of you who don’t know all of the details of my autoimmune bad-boys, here’s what is going on:

  • I have the limited form of systemic sclerosis, which is a subset condition of scleroderma. I have thick skin on my lower arms, legs, face and neck mostly, but the damage is also affecting several of my organ systems. I am considered a classic case with all of the CREST components.
  • I also have Sjogren’s Syndrome, which is fairly common as about 15% of systemic sclerosis patients also acquire this sidekick condition along with the scleroderma. It’s serious in its own right, but not usually life threatening.
  • Systemic sclerosis has a whole basketful of complications: Raynaud’s Disease, interstitial lung disease, pulmonary arterial hypertension, and kidney disease along with the almost universal digestive tract complications. I have all of these, but in my case the words that are used to describe how I’m doing are “mild, early, and moderate”, which are nice descriptors to have if they are being used in the context of lung,  heart, and kidney disease. I’m kind of guessing that one of the heart/lung conditions has taken it up a notch.

Today I’m camped out with my son waiting for Monday to arrive. I’m knitting.

Do you know the great Elizabeth Zimmerman? Her famous quote, one that I bear close to my heart on days like this, is: “Knit on, with confidence and hope, through all crisis.”

Knit on, my friends, knit on!

Happy Thanksgiving: Arm Warmer Mitts Pattern

All week I’ve been getting ready for the holiday tomorrow. The turkey is in the fridge (and at exactly 2pm tomorrow afternoon he will slide into the oven!), the pies are on the counter, and the rest of the fixings are patiently resting in the fridge. All the cleaning is done except for the last minute vacuuming (hello… cats!), and even the stash has become organized. Through all of this I have also been churning out long mitts that are also arm warmers. It’s cold now. I need arm warmers!!

Hand in MItt.
Look at how cute these are! This yarn, Western Sky Knits Magnolia Sock, is 10% cashmere. Yum!!

I have Raynaud’s syndrome; when I get cold I lose circulation to my hands, feet and even my face. It can happen really quickly, too. Look at what happened to me while shopping in the produce section of the grocery store last night!

Raynauds
I was picking out fruit and salad from the refrigerated cases when I realized I was cold and my hands were numb. Yep. Raynaud’s attack. I finished my shopping with the sleeves from my hoodie pulled down over my hands.

During the winter I cope by wearing lots of simple layers that can be easily adjusted to adapt to changing conditions. Since I’m a knitter I have lots of socks, hand warmers, and shawls that I can layer on with reckless abandon. Seriously, I’m a walking knitwear advertisement in cold weather. I’m thinking about leg warmers for under my jeans and for my arms… arm warmers!!

The perfect product would be simple arm warmers that could be pulled down over my hands and fingers if I need it (so I put in a slit for my thumb), but could also be worn pulled up my arms to keep my hands free for household tasks like when I’m working with water or cooking (with cold things from the fridge). The warmers also needed to be long enough to pull up my arm, but should also be able to just bunch around my wrist. Snug enough to slide under sweaters, but loose enough to slip over gloves. Multi-purpose warmness. Take that, winter!

So here there are. I’ve made three pairs so far and I have another pair on the needles. I am just rocking these guys.

Arm Warmer Mitts
The yarn with color stripes is Chasing Rabbits Fern yarn. The fern is a little thinner than the Magnolia sock, so those warmers hug my arms better. The cashmere ones are just wonderful to wear while reading (and knitting) in bed. I love these mitts!! My Ravelry project notes are here.

As you can see, I have many things to be thankful for. My hands aren’t great (thanks, scleroderma), but they work for knitting, and because I knit I’m in better shape than most other patients with my condition. I have Raynaud’s, but because I knit I am able to create product that help me beat it into submission. I am knitting in a time of absolutely fabulous Indy dyed yarns that make me happy with every single stitch. The fabulous colors in the yarn mean that even a simple stockinette item looks great. I am thankful. Very, very thankful.

Hey, maybe you would like some mitts too! Here’s the pattern.

Arm Warmer Mitts

Needles: size 1 (2.25 mm) double point or cable needles. I used 2 16″ cable needles and split the stitches between them. Adjust my directions to fit your needle choice.

Yarn: Fingering or sock yarn. These mitts each took about 250 yards.

Right Hand:
1. CO 72 stitches using Old Norwegian CO or any other CO that you are fond of. It needs to be a little stretchy. Join in the round with 36 stitches on each of 2 16″ circular needles. Mark the start of the round.
2. Complete K1P1 ribbing for 12 rounds.
3. Knit rounds in stockinette until mitt length is 4.5 inches from the CO.
4. Decrease: K5, K2tog, PM, K1, SSK, K rest of the round. (70 stitches)
5. K rounds for another 1.5 inches.
6. Decrease: Knit until 2 stitches before mark, K2tog, SM, K1, SSK, K rest of the round. (68 stitches)
7. K rounds for another 1.5 inches.
8. Repeat steps 6 and 7 once, and then step 6 once more. (64 stitches)
9. Thumb opening: turn the work at the end of the round and purl back on the WS to the start of the round. (remove the mark when you come to it). Turn the work again and knit the RS to the start of the round. Continue turning the work and working rows in stockinette (purl on the WS, knit on the RS) until the thumb gap is 2.0 – 2.5 inches long; check fit on your hand and knit until you like the size of the gap. End with a RS row.
10. Return to knitting rounds. Knit one round, closing the gap for the thumb.
11. Complete K1P1 ribbing for 11 rounds.
12. CO in K1P1 pattern making sure it won’t be too tight around your fingers when worn.

Left Hand:
Complete steps 1-3 as for right hand.
4. Decrease: K 62 stitches (10 stitches left in the round) K2tog, PM, K1, SSK, K to end. (70 inches)
5. K rounds for another 1.5 inches.
6. Decrease: K until 2 stitches before the mark, K2tog, SM, K1, SSK, K to end of round.
7. K rounds for another 1.5 inches.
8. Repeat steps 6 and 7 once, and then step 6 once more. (64 stitches)
9-12. Same as for the right hand.

Weave in the ends. Add buttons or other embellishments to mark the tops of the mitts if you wish.

Mitts
The finished mitts are 12 inches long, 4.5 inches wide at the top and 3.75 inches wide at the lower (hand) edge.

Happy Thanksgiving everyone!  May your day be a good one, and stay warm!