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!

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The Scleroderma Chronicles: Rocking the Spoonie Clock

I, along with a lot of other people with chronic illnesses, consider myself to be a “spoonie”. If you’ve never heard of spoon theory, it is a metaphor that describes the intricate bargaining game that those of us with limited energy resources play everyday to balance our activities with the little gas in our tanks.  Christine Miserandino developed the spoon theory and described it in an essay on her blog in 2005; trying to explain to a friend how she managed fatigue with her lupus, she grabbed spoons and used them as props to represent discrete energy packets. If you only have a limited number of energy units (spoons) to use in a day, you are painfully aware of how many spoons everything that you do costs. A shower? That is a spoon. Walk to the mailbox to pick up the mail? Another spoon. If you want to grocery shop, plan ahead. When the spoons for the day are gone, you are done. If you planned badly, you are basically toast. Get ready for some bad days ahead.

Owner and her cat.
Wrapped in layers of knitted goods, struggling to stay warm, MacKenzie and I enjoy a late night purr break at the height of my flare in December.

Early last December I ran through my spoons and just kept going for another couple of days. I knew I was headed for trouble, but I was in a situation where there were no other options for me. Take care of today and deal with tomorrow when it comes, I told myself.

Oh dear. The flare arrived and fatigue descended with a vengeance. I slept 10-12 hours a night and collapsed in exhaustion for a nap at least twice a day. If energy is counted in spoons, I was down to only about 10 for the day. Not only were the spoons my problem; it appeared that as soon as I got onto my feet and went into motion an internal clock started running. No matter what I did, I could not go more than 2.5 hours without a nap.

Christmas shopping!! AARRGGG!!!

Thank goodness for the internet. I made plans and checked the availability of items I wanted to buy using my phone while in bed. I made sure stores close to me carried the items that I wanted. I made shopping lists in a little spiral notebook that I carry with me (hello… brain fog!!) to help me remember what I’m looking for while in the stores. I planned shopping routes that were short loops that would take me to three stores/stops max and then get me home before my time and energy ran out. I planned the trips for times when the parking lots would be almost empty so I wouldn’t have to walk far.

So, one day I planned and cooked food for the week. Who knew making spaghetti was so exhausting? Before I could get the dishwasher loaded the timer on my spoonie clock went off and I was done.

The next day I made one of the loops. Whew. I got right up from the nap, started up the car and headed off to Kaiser for my monthly blood testing. Then on to Target… hurry, hurry, it has already been an hour. I finished that loop at the book store where I shared a laugh with a mom who was making the same stops as me on her own shopping route. She had also gone to the public library, though. She looked at me kind of weird when I said I had decided to not go to the library as it was too far to walk in from the parking lot there. Ugh. No way was I going to explain about the spoons to her. No time. My clock was ticking; I was at the 2 hour mark and had to check out and drive home. Hurry, hurry. I’m now having trouble walking because my legs don’t really want to go… Brain fog is starting to close in and my head feels buzzy…

Home. Nap. I didn’t even take the packages out of the car until after the nap.

Two more days, two more loops, and I had gotten everything and even got stuff mailed out on one of the loops. Careful planning, lots of patience, and an acknowledgement of my limitations went a long way in getting me through everything. Did you know that if you go grocery shopping late at night there is no line. True fact!

For more than a month I was careful with the spoons and never ran down my spoonie clock. I knitted in bed. I ate my little pre-planned meals and ordered things off the internet when I could. I took lots of naps, kept up on my meds, and did everything I could to manage my symptoms. The Turkish Hell socks lengthened as the list in my notebook got items crossed off.

Today I am through the flare and I must have at least 20 spoons a day. Maybe even 25. I’m rich!! The spoonie clock is up to 6 hours. That’s pretty darn good.

You know, people are always telling me how great I look.

If only they could see my spoonie clock ticking away.

 

 

 

 

The Scleroderma Chronicles: My Second Year Report

Well, here I am at the end of my second year since my diagnosis of systemic sclerosis, a life-threatening form of the autoimmune disease scleroderma. Last year I blogged about my illness: I had come through the worst of the grief and horror at the initial diagnosis, had made my way through some scary incidents that sent me flying across town to emergency centers, and was pretty upbeat about where I was in the progress of the disease.  I was sick, but I hadn’t developed any of the most serious, life threatening complications. My heart and lungs were fine. I had just been started on some serious immunosuppressant drugs (the same ones that are given to kidney transplant patients), and while they are risky, I had been told that they could really make a difference in my 10 year survival rate.

Hey, you roll the dice and you take your chances.  No sense worrying about the unknown future. I drugged up and slept like a baby at night.

Star Trek Meme
The Kobayashi Maru test, as all Star Trek buffs know, was a no-win scenario;  it was meant as a test of character. Systemic sclerosis, an incurable,  progressive, disabling and potentially fatal disease, can  also be considered such a test.

Ready to hear about my second year? Let me give you a hint: buckle your seat belt, because we are in for a bumpy ride.

  • November-December: the drugs begin to kick in and as they beat my immune system into submission my skin starts to harden up. I hurt everywhere!! I can hardly bear to comb my hair. The place where my flu shot went in hurt for weeks afterwards.
  • January: I caught the flu. Talk about insult to injury! Here’s the short version: antibiotics, off the immunosuppressant drugs so my body’s immune system can fight back, and then trouble breathing, chest pain, heart palpitations, and a partridge in a pear tree. What a mess! The month passes in a blur.
  • February: I’m still pretty sick and struggling to breathe. I get bounced back and forth between doctors as the debate about the root cause of my symptoms rages. I begin to pressure my doctors for answers and there is much testing. Oops. All is not well with my lungs and my heart is accruing damage. I get sent to a pulmonologist and she give me an inhaler to help me breath. She also tells me I am in serious trouble and refers me to palliative care. The doctors increase my immunosuppressant drug dosage.
  • March: Why, hello, Sjogren’s Syndrome. We forgot all about you! In the concern about my systemic sclerosis, the bad boy of my autoimmune twosome, everyone forgot that I also have Sjogren’s Syndrome, another serious autoimmune disease that causes dry eyes and mouth. As it turns out, it can also cause small airway disease (think never-ending asthma attack) and it has pushed me into chronic respiratory failure. Hello oxygen machine. You are my new best friend.

    Oxygen machine
    My new best friend!
  • April: new lung scans are back, and while I am diagnosed with interstitial lung disease, it appears that it is only mild. Huge sigh of relief!! There is also consensus that my pulmonary hypertension has not advanced. Both of these diagnosis, while still early and mild, are very serious, and the decision is made for palliative care to continue to follow me. Bummer!
  • May-July: Sunshine! Heat! Burning muscles, aching joints, gastritis, dizzy, dizzy, dizzy, and I notice that my lips are blue. I’m on oxygen 24/7 by the end of July.
  • August: my internist changes my meds to bring my heart rate up, and suddenly I have enough oxygen. The heart palpitations stop and after more testing I come off the oxygen. The 6 month Sjogren’s-driven asthma attack is finally over.
  • Quilt and socks
    Summer quilt and socks for my poor hurting feet.

    September-October: why does it hurt to walk? What is up with my feet? And this whole barfing in the middle of the night is getting downright annoying… My internist tests me to see if I have an H. pylori infection.

  • November: Well, doesn’t this beat all. The H. pylori test came back negative and I am diagnosed with gastroparesis. The muscles of my stomach are too damaged by systemic sclerosis to work correctly; the damage is irreversible. I start eating a very limited diet of soft foods and dairy. Ironically, I can now eat jelly donuts, but not fresh baby carrots. I’m losing weight anyway.
Little Greenhouse
The gastroparesis diagnosis hit me hard even though I kind of  knew it was coming. I stopped to get a little cheer-me-up on the way home.  Check out the little greenhouse I put together for the kitchen window. As always, MacKenzie had to help out with the picture.
Flowers
Here’s the flowers. Aren’t these cute? They were sold at the local nursery to put into “Fairy Gardens”
cat
and how could I resist adding the little cat and the mushroom? Those plants are miniature Kalanchoe that should eventually bloom again.

See, a bumpy ride that is still going, but a year that was also rich in gifts. Palliative care forced me to face the future with more courage and to make end-of-life decisions for my family and to start cleaning out my house of junk. I talked to family about my medical power of attorney. I enlisted one of my doctors to manage the medical team and I began to feel more in control of my basically out of control disease. I began to knit gifts for those I love with a purpose: everything now is a piece of me. In my mind the shawls that I am making for everyone I know are the “Good-bye Shawls”. I am on fire to make as many fingerless mitts for other scleroderma patients as I can. At the end of the day, this year was not one of struggle and heartbreak as I dealt with the endless march of a disease that has no pity or remorse. Rather, it was one of care, giving, creative fire, good friends, and the meditative peace of knitting.

Okay, I do get cranky at times, and there has been some crying.  I get short with annoying salespeople because it is so hard for me to shop. I told my ex-husband I was tired of hearing about his “stupid-ass”  motorcycle. I yelled at the cats. I hate when people say, “Well, you look great!” in a way that suggests that I’m not really all that sick at all. I wish that they were there to hold my hair when I hobble to the bathroom at 2am to throw up that nice meal that I hopefully ate but couldn’t digest. There. I got it off my chest, and I feel much better. Aren’t you relieved to hear that I can be petty and mean from time to time?

Tomorrow is Thanksgiving Day. You know, it is easy to focus on the day: travel, turkey, family and the descent into wild Christmas shopping. Sometimes we forget the history of this national holiday; thanks for a good harvest and the blessing of probable survival through the coming winter. It is also a time to reflect on the bounty of the last year and to be grateful for the gifts it brought.

In spite of all the bumps of the last year, I am grateful for all of the gifts I have received.

Happy Thanksgiving everyone!!