systemic sclerosis – Page 3 – Yarn, Books & Roses

The BioGeek flirts with Long-Hauling

It’s been over a month since I first developed Covid-like symptoms and began to spend my days in bed. Like, my bed became my little nest with the detritus of a prolonged illness littering the floor and shelves around it. Outside the world moved through the end of summer; my tree lost all of its leaves, and the garden moved into dormancy. The birds disappeared. The squirrels have remained on the crazy side, but the crickets are now silent. The cats are chasing the last few grasshoppers of summer, but they are almost too cold to hop anymore.

Mateo has used my down time well, destroying his cat tree, removing push pins from the wall hangings, and just being an agent of mayhem in general. Hannah is not impressed.

While the symptoms of “whatever the heck that Covid wannabe virus was” are mostly gone, I’m still struggling big time. Everything, and I do mean everything, hurts. Tendons that I didn’t even know I had are now too painful to touch. Random shooting pain and muscle cramps have become routine. Did I mention the chest pain? I can’t concentrate enough to read; brain fog is driving the bus these days. I can’t knit for more than a few rows at a time as my arms get too heavy to hold up. I am kind of weak and shaky. I’m starting to stare down the depression monster for the first time in years. Did I mention that the fatigue is unreal? Ugh.

Hannah has been supporting my knitting efforts. I’m slowly making progress on my Weekender Crew sweater.

So, there hasn’t been much blogging. There has been, however, some medical testing and trips to doctor offices going on.

Yesterday I finally saw my rheumatologist. “This is just awful,” I told her. “This is fibromyalgia!” she replied. In her opinion, what I’m experiencing is a pretty severe episode of fibromyalgia. I’d already been diagnosed a few years ago, but I’ve never experienced symptoms as bad as this. Sigh. Remember the 15% rule in scleroderma? It says that about 15% of patients with systemic sclerosis (the type of scleroderma that I have) will also have a second autoimmune disease called Sjogren’s. Check, got that. Since Sjogren’s symptoms can look like fibromyalgia I always took that diagnosis with a grain of salt. Now it’s pretty clear that it is a separate condition, and my fibromyalgia is running wild. Like a squirrel. Or Mateo.

Feeling particularly unlucky I drove home thinking about writing an autobiography called Outlier: My life as a singular data point. Seriously, who in their right mind would ever want to be so far off the bell curve? Then the BioGeek emerged, and I thought about Covid long-haulers. I have met some people who have been diagnosed with Long Covid, and I did think at the time that what they were dealing with was an awful lot like one of my flares. Like, an awful lot. Yep. According to this article about a third of Covid long-haulers have “FibroCovid”, a condition that pretty much looks like fibromyalgia. Fabulous. This article suggests that Long Covid is just another name for fibromyalgia. Another article explored the benefits of applying lessons from fibromyalgia research to Long Covid. Thanks, nasty virus that acted like Covid but refused to test positive. Thanks for acting like a nightmare houseguest who leaves a huge mess behind…

This is crazy, right? Covid shares similarities with systemic sclerosis, and long Covid shares similarities with fibromyalgia. The BioGeek in me is interested in all of this, but let’s hope this doesn’t drag on for months.

My rheumatologist has started me on a course of narcotic painkillers as that sometimes pops people out of fibromyalgia flares. I’m taking magnesium as that can also help. My rheumatologist has some more tricks up her sleeve if this doesn’t do it, but I’m relieved to finally have a name for what is happening.

The dedication page on the book I started reading this weekend. I haven’t gotten very far because… brain fog.

Time to kick this thing to the curb (like a zebra!) so I can get back to knitting!!

The BioGeek does Covid

It’s been a while since I’ve posted. I know, I know… lazy, lazy, lazy. This time I have a really good excuse.

I caught Covid!

I think that almost everyone who follows my blog knows that I have a type of scleroderma called systemic sclerosis; this is a progressive autoimmune disease that involves damage to blood vessels, lots of inflammation, and then scarring (fibrosis) of organs. In my case, the worst damage is occurring in my GI tract, my heart and my lungs. Because of the lung complications that I have developed I’m pretty high risk for a severe case of Covid-19, so my doctors have encouraged me (strongly) to get updated Covid-19 vaccines as soon as they are available. Because of my interstitial lung disease, I don’t halt my immunosuppressive drug for these boosters anymore.

So, as soon as the new Covid boosters were available I made an appointment, and on October 12th I got my shot. Yay! I was so excited to get it as I usually feel great for a few weeks after the vaccine. Like… my systemic sclerosis symptoms improve, I get more energy, things stop hurting, I can breathe better… you know, better! I know, I know, this doesn’t make any sense, but it is a well observed phenomenon in the systemic sclerosis community. It’s like there is some crazy connection between that vaccine and our autoimmune condition. It does not happen with any other vaccines.

Vaccines always knock me on my butt at first, though, so I wasn’t surprised that I developed muscle/joint pain, chills, fever and fatigue. I headed to bed expecting to feel better in the morning. Nope. I began coughing, got a sore throat, developed a lot of congestion, and GI symptoms arrived. Three days later I began to suspect that maybe this was actually Covid, and that I had unluckily gotten ill on the same day as the booster. I took an at-home Covid test, which was negative.

I wasn’t too sick to order more yarn on the internet. Look at this amazing sock kit that I bought from Moonglow!

A little aside: I’m pretty sure you don’t already know that Covid-19 and systemic sclerosis share a lot of similarities. Like, a lot. Damage to the vascular system, the production of a lot of inflammatory cytokines (messenger molecules that travel between cells), and then all those symptoms that can include heart and lung complications that result in fibrosis to both organs. (Covid is a fast-moving firestorm, and systemic sclerosis is more like the relentless slow burn version.) The pneumonia that develops in Covid-19 patients looks so much like the inflammatory lung disease in systemic sclerosis (SSc-ILD), it can be hard to sort out which disease is in play when systemic sclerosis patients like me first arrive in the ER. Okay, things never go well in the ER anyway, so I tend to avoid them. Zebra, right? I decided that I was just in a bad flare and suffered on instead of going to the ER.

Two days later, I retested for Covid. Still negative. Still too sick to consider driving anywhere, too sick to sit up and knit, but not sick enough to call 911. I was kind of stuck in limbo. Maybe some level of hell. Covid hell. Anyway, that booster shot had definitely failed me this time!! I spent my days coughing, sleeping and sadly gazing at my new sock yarn propped up on my bookshelf. I kept taking my immunosuppressive drugs and suffered on.

The cats began to live on the bed with me. Good kitties!

After a week of this nonsense, I had improved enough to consider going to a Kaiser Urgent Care to see if they could sort me out. Oh. The doctor there was of the opinion that I had Covid after all, but now it was too late for anti-virals or an infusion of antibodies. I got a chest x-ray, some nice antibiotics, a new drug for my cough, and headed back home to the cats and my unknitted yarn.

Tonight, 16 days later, I’m better. I can sit up and knit at last. Through all of this my oxygen levels never dropped, and while I was sick, I didn’t develop any really serious complications.

So, what have I learned about systemic sclerosis, the drug that I take to control it, and Covid-19? I mean, this is a total BioGeek moment! My mind full of dancing antibodies, failed Covid tests, and mycophenolate pills, I took to the internet for answers. There are some take home lessons that I’ve decided are so interesting that I’m passing them on.

The similarities between systemic sclerosis and Covid-19 are so significant that the two diseases provide understandings that can help in the treatment of both.
The immunosuppressive drug that I take (mycophenolate) to control my immune system’s attack on my lungs can prevent me from producing an antibody response to the vaccine. Okay, I knew that, but I was too sick to think through the implications… because that means…
Immunosuppressive drugs can lead to false negative at-home Covid tests. ~~Those tests are for Covid-19 antibodies; if you can’t make antibodies, then you may have Covid but test negative.~~ Oops. (Correction! My cousin has set me straight… the home tests are detecting viral proteins (antigens), so that theory is blown out of the water! Double oops! The doctor told me the mycophenolate was the cause of the false positive tests, and he was pretty emphatic that I should stay on it. Now I’m wondering if the false positive tests were because mycophenolate has anti-viral properties, as some sources cited in the Lancet paper below have reported.) (Don’t you just love science?!!! )
The same immunosuppressive drug (mycophenolate) can be an effective treatment for Covid patients reducing their risk of severe outcomes.
Staying on the drug was a good decision on my part. I got lucky! Because I am on this drug, however, it can take longer for Covid to clear my system. It has been 16 days, and it looks like I need to isolate for a few more days.
I never, ever thought that the treatment for my SSc-ILD would help protect me from the severe Covid complications that the same condition places me at high risk for. I’m so grateful that we didn’t go the chemotherapy route last year.

Isn’t all that interesting?

Here’s the links to articles if anyone is interested.

Hannah and the CoalBear: Caturday Updates

Hi. I’m Mateo.

I’m all grown up now. I should be trusted outdoors on my own, right?

This has been a busy week at Casa Mother-of-Cats. The shower broke and she had to get a plumber to fix it. Hannah practically teleported into the closet for that! Then the Mother of Cats had to go get some blood work done for her crazy scleroderma and she has been dragging sadly around the house waiting for the doctor to call. Finally, the Mother of Cats pulled herself together and went to the garden center to buy roasted green chiles: Yeow! Those chiles are stinky!! Hannah refused to go into the kitchen while the Mother of Cats was packing them up for freezing. Late in the week the Mother of Cats got some knitting done and early this evening the doctor messaged her, and she has finally perked up.

Through the whole week she took a lot of pictures, so here they come:

There is a bunny hanging out in the front yard that makes the Mother of Cats happy every time she sees it.

These mums came home from the garden center with the green chiles.

The leaves are starting to change on the trees.

I managed to escape from the catio last night and ran wild through the yard under that supermoon for a couple of hours before the Mother of Cats came looking for me. So. Much. Fun. I looked everywhere for that bunny, but I couldn’t find it. There was a garter snake in the yard last week too, and that sounds like a lot of fun, but I couldn’t find it either. Unable to have really outstanding fun ~~or a bunny snack~~, I just ate a bunch of bugs and threw them up on the bed after I came back in… why does the Mother of Cats get worked up so easily?

Hannah: I was a good girl and stayed on the catio.

Oh, yeah. I almost forgot. There was a lot of knitting that went on this week, too.

This heap of pink mess is the beginning of the new Weekender Crew sweater. I’m sure that it will actually look like a sweater some day.

The sweater is being knit inside-out, so the Mother of Cats tried to pull the knitted tube open so you could see the part of the sweater that will be on the outside. It is totally a mystery to me, but hopefully you will kind of get the idea. The color is nice, right?

The Mother of Cats really made progress on the new arm warmers. The first one is done, and she has started on the second one; they are so long they go up above her elbow. The next time she had a medical adventure they should help keep her nice and warm!

Well, that’s all for now. I’m going to carry on downstairs for awhile to see if I can get Hannah to come chase me.

Hannah: that would be a big NOPE!! CoalBear. It is bedtime for me…

Happy Caturday, everyone!

This is Mateo the CoalBear, signing off.

Notes from the Mother of Cats:

The last few weeks have seen the emergence of new symptoms: my hair is falling out, I have more edema, bruises are popping up everywhere, and I get sudden muscle cramps. Itching has become a problem, and cuts are slow to heal. Fatigue is back in a big way. I finally contacted my doctor, and she ordered some testing to make sure I wasn’t going into kidney failure.

Kidney failure!! Cue the panic sirens!!!!

Seriously, one of the things that I fear most is kidney failure. Last year, when my lung disease was so bad, letting my kidneys go to save my lungs was an actual topic of discussion; I was a hard no on that treatment option. Ironically, a low kidney function test was one of the first clues that led to my scleroderma diagnosis, but my kidneys have improved over the last couple of years to a comfortable stage 3 level of disease. Discussion of kidney failure again seemed like a huge blow, and I have been sad this week. I cast on the arm warmers during the afternoons when I’m stuck indoors on oxygen during the heat.

The message from my doctor today was reassuring. My kidney function has maintained, but I am very low on protein levels in my blood. This is kind of a scleroderma thing too, as digested food doesn’t get absorbed well through fibrotic intestinal tissue, but I’m so immunosuppressed, it might be because I can’t make antibodies. More testing is on the horizon, but I’m not losing any sleep over this. I am, however, going to be much more careful about wearing my mask!

I’m making good progress on those arm warmers… if I hurry, I can cast on another project like maybe those pressed flowers socks early next week. 🙂

Have a good Caturday!

The Scleroderma Chronicles: Thoughts on the Night of the Blue Supermoon…

Did you look at the moon tonight? It is just huge, shining in the night like it knows that it is something special. Well, it is. This is the Blue Supermoon of 2023, my friends. Not to be seen again for 14 years. Just the sight of it makes me feel happy.

This is also the anniversary of the day that I was told that I had systemic sclerosis and Sjogren’s Disease. Actually, it is 9 years and one day since I drove to my first rheumatologist appointment; I was a little emotional that morning as I passed fields of beautiful sunflowers, their faces glowing in the light of the morning sun rising behind me. You’d think that the date would be kind of a bummer, but nope. As it turns out, there are lots of things that are making me feel happy at the moment.

Do you see that black bag with my purse and cane? That is my portable oxygen concentrator!

That’s right, after languishing for 18 months on the waiting list, my name came up for the portable oxygen concentrator that I have been desperately needing. Look at that baby!!! It only weighs 5 pounds, it works great, I can adjust the level of flow on the fly, it can charge in the car, and it is exactly what I need. Yay!!! I got it yesterday on the exact 9-year anniversary of my diagnosis.

So happy, feeling absolutely empowered, I headed to the yarn store after picking up the concentrator. Time to take this baby for a spin, right? I have been struggling for months to make a decision about the yarn for the La Prairie cardigan that I want to knit next. I bought a kit of yarn to make the cardigan, but I’m not happy with it. I needed a new skein to go into the mix…

The yarn picture on the left is the original kit. The one on the right is the new variation that I’m not completely happy with. Feeling hopeful and more than a little determined, I sat on the floor in front of the most likely candidate yarns and, rocking my new O2 concentrator, holding up my phone with the yarns on the screen, I kept looking at different ideas for the fade…

Winner, winner, chicken dinner! I suddenly realized that the Stitch Together yarn (second from the top) was exactly what I was looking for. There it is. I asked others in the store what they thought, and the consensus was that I had nailed down my fade. Yay!!! Finding that yarn made me happy.

So, I also bought the special edition Babe set from Spun Right Round.

I’m going to make that Barbie pink yarn into hats for the community knitting group that I knit for because… wait for it… the color makes me happy!! I had one of the original Barbie dolls, and just the thought of Barbie makes me… happy!

On the way home from the yarn store, still sucking down oxygen in the car and feeling pretty good, I stopped at the grocery store to buy some tiramisu because I was absolutely having a tiramisu kind of day, and there at the front of the store were sunflowers. You know, it is the end of August, and it is sunflower time. Shine like a sunflower!!

Pretty good looking, huh. Sunflowers are used as a symbol of scleroderma hope in some parts of the world (Hello, Australia! Talking to you!), and you know I bought these too.

By the time I got home I was tallying up all of the things that made me happy. The leaves are starting to change on the trees in town, and the ornamental grasses are covered with rich golden plumes atop bright green stems. That makes me happy. Pumpkin spice is back at Starbucks. The kittens loved the new toys that I bought them. I ordered new clothes this week that fit great even though they are a smaller size than usual. I found new shoes to wear that are really helping a lot. Happy. I am happy.

The shoes are shaped like walking boots on the bottom, but inside there is great arch support and a cushy insole. I am walking now with much less pain. Did you notice the purple detailing and the silver loops for the laces? Happy. These are happy shoes.

Once home, I headed outside to the catio with my yarn, the tiramisu, and a cup of coffee from my new Keurig machine (yep… happy), and as I set the plate and coffee cup on the table, a pair of adult cottontail rabbits raced around the corner of the deck and zoomed under my side gate. Looks like I will be having baby bunnies again. I am happy.

Nine years ago, I asked that first rheumatologist what my life would be like in five years time. He refused to answer, and it was my first clue that I might be in some trouble here. 18 months ago, my pulmonologist told me it was okay to cry when the first lung scans showed serious interstitial lung disease. One year ago, my pulmonologist told me that they were very worried about me after my lung biopsy… nothing was working, and he wasn’t sure I’d make it. Last month, the technician who did my latest pulmonary function test told me that I was too bad to walk any longer without portable oxygen. I came home, looked at the bottles that are too heavy for me to carry, and cried.

Tonight, under the blue supermoon, with sunflowers on my table, I am happy. One month later, I have my portable oxygen. 18 months later, my lung disease appears to be stable. 9 years later, I’m still here, rolling with the punches of new complications, facing down the monster, and finding ways to shine.

I am happy.

Shine on supermoon, shine on.

Hannah and the CoalBear: Trail? What Trail?

Hi. I’m Hannah.

This has been a kind of strange week. The Mother of Cats came home on Monday after spending ANOTHER WEEKEND WITH THOSE KITTENS!!! and then she tried to make it up to us by being really nice all week. She fed us tuna twice a day and played with Mateo with the laser light more than usual. Like that would make up for sneaking off to spend time with these furry little guys instead of US.

Okay, they are kind of cute. Still, they aren’t as cute as Mateo and I were at the same age. Just saying…

Anyway, back to the strange week. The children in the neighborhood all went back to school (whatever that it…) this week and it suddenly is quieter in the backyard. A huge flock of geese flew over the yard yesterday, and today the Mother of Cats noticed that some of the berries on the bushes are turning red. The squirrel is more active than usual, and the moon was out in the daytime. Crazy, right? Here are the pictures.

The Mother of Cats is also excited about something called Pumpkin Spice. Is that a type of tuna?

Anyway, let’s now discuss the Mother of Cats and her COMPLETE ABANDONMENT of the Sharon on the Trail MKAL. She didn’t even bother to open up the emails with the pattern updates. She says that she hates her yarns, and she doesn’t love the pattern, and she just wants to knit the stuff she already has and to make some little knitted kitties to give away. I’d be concerned about her, but she is really happy with the stuff that she is knitting now, and I have to admit that it is looking good.

She put her sweater back onto the needles this week and got several inches of the body knitted. Looking good! She says that the funky looking new stitches are that way because they aren’t blocked yet. Mateo isn’t convinced.

Mateo: I’ll reserve judgement until I see that new knitting blocked. Call me Doubting Mateo.

She really spent some time on the new socks that she is making, and she really likes the way they are coming out. I think that has something to do with how she just UP AND ABANDONED Sharon from Security in her quest to apprehend that villainous Keith…

She likes these socks so much she is already dreaming of which yarns she will use to knit the next pair with. Poor Sharon, I’m pretty sure that the Mother of Cats will never return to the trail. She has her eye on some purple yarns for the next pair of socks…

Well, that is it. I hope that you all had a wonderful Caturday.

This is Hannah, signing off.

Notes from the Mother of Cats:

The sweater is Alpine Bloom by Caitlin Hunter.
The socks are Pressed Flowers Socks by Amy Cristoffers.
The abandoned MKAL is Sharon on the Trail by Casapinka. I have decided to wait for the finished shawl before I put any more knitting time into it.
The weather has been playing hell with my breathing. A couple of lows tracked right over the state and in the low pressure I don’t do well at all.

Do you see the low? That’s the place where all the wind arrows are swirling together just east of me in the state (I’m located just east of Denver). Fun and games for people with lung/heart conditions!

Behold Hannah and Mateo as kittens… they were pretty cute!

The Scleroderma Chronicles: Bioethical Dilemmas and Unintended Consequences

I was a biology teacher in the time of the Human Genome Project. This week, with the coming release of the newest movie about Oppenheimer and the development of the atomic bomb, I’ve been thinking about the DNA and genes again (I know, it is a biogeek thing…), because one of the candidates running for the Republican nomination in the US wants to cut the Department of Energy if elected.

Kind of a loose chain of threads, you’re probably thinking. Am I right? Well… the Department of Energy is the agency that is responsible for the regulation of the nuclear energy industry in the US. There’s a lot of waste coming out of those nuclear reactors, and there was some concern about how much mutational damage was being done to DNA through exposure to radiation. Well, to figure that out, you need to know what undamaged DNA looks like. The initial drive to figure out what the human genome looks like came from that agency and once the results came in early this century the world completely changed. Like a big change. Like an atomic bomb level change. Like, there are now sites that have huge depositories of biotechnical data and tools to aid in research.

In the classroom we biology teachers began to teach about the Human Genome Project and also did a week-long unit around the ethical problems associated with this new knowledge (bioethics, if you will). The kids grappled with dilemmas like… if you had the gene for a fatal, untreatable illness, would you want to know? If you were a child at risk for this gene, would it be okay for your parents to have you tested for it before you are 18? If your unborn child tested positive for this condition, what would you do? Would it be okay for human organs to be grown for transplantation? Who should get the transplant… a single father of 4, or a 16-year-old student in your high school? Should your employer allow you to continue in your airline pilot job if a genetic test shows you are high risk for a sudden cardiac event. Should genetic test results be private? Whew. Lots to grapple with in this unit.

Makes your head hurt, doesn’t it. Check out my knitting progress this week!

So, shit kind of got real this week. One of the members of an online support group for pulmonary arterial hypertension (a progressive and fatal heart/lung condition that I have thanks to systemic sclerosis) has just been identified with a gene (bpmr2) that causes the condition; her PAH is caused by this gene and can be inherited; she has a different type from me, but it is still PAH. Oh, boy. This is not good at all. The life expectancy right now is up to about 7 years, but you only need one copy of the gene to be at risk for PAH… there is a 50% chance for each of her children that they inherited the gene. Only 20% of people with the gene will develop PAH, but that is still a big risk.

Should she tell her two children? she is asking in the forum. They are in their late 20s. If she does, and they get tested, should they have their own children if they have the gene? Her heart is broken, literally.

Life expectancy has greatly improved over the last few years with new medications being generated in the modern climate of expanding cellular and molecular biological information. Untreated PAH (and PH kind of gets lumped together with it according to my pulmonologist…) has a life expectancy of about 2.8 years… not good.

Rose break! By the end of the discussion thread, she was leaning towards telling the kids.

Which brings me to the next shitty bioethical item that occurred this week. One of the members of another support group caught Covid and had to go off her drugs while fighting the virus. She has been slow to recover, and still feels pretty bad, but she took a pregnancy test and restarted her medications again a few weeks ago, only to discover this week that she is actually 12 weeks pregnant. Why did she have to take a pregnancy test before restarting her drugs? Because some of the drugs used to treat PAH can cause extreme damage to a human fetus. The enrollment process is very strict, and every effort is made to keep patients from this situation.

I am in grief for this woman. The doctors think that the baby has been spared the worst of the drug toxicity, but now she is working her way through whether to abort or not, to restart her drugs, or not. To risk death to save the baby, or to abort and restart treatment. If the baby is born in good health, will she live long enough to see it enter kindergarten. If the baby is born with health problems, her medical burden is increased. She is young. This is a horrible mess, and she is already too far along to get an abortion in many states in the US. I don’t know if she has other children, or what her support structure is…

This week I heard that some states are demanding private health records to identify any out-of-state abortions or transgender care that has happened in another state. I hope that this woman lives in another country…

Then I heard that a popular hamburger joint near my home is now going to fire employees who wear a mask. Say, WHAT?! Let me tell you, any person who has a serious lung/heart condition like mine wants to wear a mask, and they are so grateful if the person at the service window is also wearing a mask. Sometimes people offer to put on a mask when they see that I am wearing one. Now that person can be fired for putting on a mask… remember the young PAH patient whose nightmare began with catching Covid? Truthfully, any random virus can cause serious damage to patients with PAH, especially if they are also immunosuppressed.

So, there are a lot of bioethical dilemmas here, and the unintended consequences of people who want to make sweeping decisions without understanding all the interlocking systems involved and the potential ramifications are staggering. NO, you can’t just disband the Department of Energy, and sweeping, inflexible decisions about reproductive issues (that seem to be smugly self-righteous to me) can be disastrous. It is easy to order up genetic tests, but what happens once you have the information can be life-altering.

And don’t get me started on this animosity towards mask wearing…

I can’t help but think that no one should attempt to enact legislation without ~~suffering through~~ experiencing something like the bioethics unit that was taught at the high school where I used to work. I keep wondering, do these legislators actually understand nuclear power and weapons? Have they heard of the Human Genome Project? About gene testing? About rare diseases? They absolutely need to go see the Oppenheimer movie, maybe, and then write me a report about the Human Genome Project. I’m pretty sure that they would struggle with epigenetics, but it would do them good if they looked into it. They can get extra credit for a summary of pulmonary arterial hypertension. I would like to give them a book list of summer reading to get through on their breaks, because only the well-informed and educated should attempt to make decisions about these issues in the seven levels of bioethical hell that is the life of patients like me.

Because this week was a really hard one; for too many people this shit is real.

PS: Have you seen the show House? I kind of think that he could use a little bioethics sensitivity training, too.

The Scleroderma Chronicles: Be a Rose.

So, I got a little testy in one of my Facebook support groups for systemic sclerosis this morning. A member of the group kind of disparaged me and another person for not being positive enough. It was “you need to refuse to let scleroderma define you” in response to the first person sadly saying that she missed her old life, and me giving her an online hug with the comment that if only a positive attitude was enough…

In my defense I had just experienced a Kaiser employee visibly reacting to my lack of wrinkles. As in, wow, that’s great! Like not having wrinkles makes this all worth it.

I lost another 4 pounds at my last check-in, which is concerning, but the nurse was thrilled for me. Me, I was a little teary at the continued loss.

Um, people… do you think that you might be a little shallow here?

I was out on the deck/catio with the cats drinking my morning latte when I hit this emotional wall, and after I had fired off the somewhat testy response, I spent some time in the garden. There were my roses, blooming like the utter champs that they are.

This is my Princess Alexandra of Kent rose.

This rose is looking great this year. Never before has the plant been able to hold up the blooms without the weight pulling the stems over. The problem is our semi-arid climate with hard winters; the plant grows back from the roots every year and it doesn’t have time to put in enough supportive tissue to hold up the blooms. This year, with all the rain and cool weather that we have had, the plant was able to put in enough of this tissue to do the job. The tissue that I’m talking about, a type of ground tissue, is called sclerenchyma. If you’re ever snapped a celery stalk in half and pulled out the strings, you were pulling out sclerenchyma tissue. This tissue is made of dry, hardened cells in the stem of the rose and I suspect that the “scler” part of the word is of the same origin as scleroderma. Look at that. The hardened cells are doing something good for this rose!

In me, not so much. Things have been a little difficult as my poor heart and lungs are not benefiting from the hardening and thickening going on in the cells and tissues of those organs. All of my tendons are seriously pissed off at the moment. Edema has become a problem, and it is becoming increasing clear that I need to stay on oxygen 24/7.

That’s my arm with the imprint of a quilt in it, in case you didn’t immediately recognize it… Edema is kind of a tip off that my heart is struggling and that’s new. The weight loss is also related to my heart/lungs because if oxygen isn’t getting down to my cells like it should, they can’t use energy efficiently, and then, you know, weight loss occurs. The tendon issue is scleroderma actively attacking them and gradually hardening them to bone. Bad scleroderma, bad!!

So, what did I say in my testy response? Reality bites. Some of us have progressed to the point where we have to admit that no amount of positive thinking will allow us to attend that family function that we were invited to, or to visit the annual Wool Market in the mountains, or to even walk to the mailbox. To suggest that we could do things if we just had a more positive attitude is hurtful and not supportive. No matter how much you want to believe otherwise, scleroderma does define me and everyone else who is dealing with it. Courage requires us to face down the monster and to accept the reality of our disease. How we choose to function within that framework is up to us.

I may no longer be as mobile as I once was, and the life that I used to have is now mostly gone, but I choose to continue to bloom in place.

Like a rose.

Updates/Notes from the ScleroFront:

Do you like to wear linen? Those fibers are from the water tubes (xylem) in the flax plant, and made of sclerenchyma.
The bunny-murdering neighbor put her house up for sale!!
My Alpine Bloom sweater is coming right along!

Knitting after the Cath Lab

Hey. I know that I’ve been missing for a while (again). In my last post I wrote about my upcoming trip to the cath lab to get a right heart catherization. This procedure involves having a small sensing device threaded into your heart to check (in my case) the internal blood pressure. Normal pressure in the right side of your heart should be about 14mm Hg; mine had measured 44 mm Hg in an echocardiogram so my cardiologist wanted to get a direct reading.

Here I am, rocking my scleroderma symptoms along with the cath wound. Puffy hands, bluish nose and lips, tons of little red dots on my face… That bandage came off the next day and the entry wound healed right up.

The procedure went well. My pressure measured in the low 20s, which was soooo much better than my cardiologist feared. The number was higher than the last time I had a direct measurement, so my pulmonary hypertension has advanced, especially since I am now on medication to treat my condition, but I’ll take it! It was, once again, a really positive experience and I felt well cared for by all the staff. I was pretty exhausted, hungry, but upbeat on the drive home.

Then I went into a flare… I slept for almost the entire week after the procedure. Seriously, like 12 hours a night and a couple of naps. I had to go back onto daytime oxygen. My hair started falling out again. I was dizzy and exhausted. My joints really hurt. A flare.

This week I’m finally awake again and I pulled out the blanket that I’m knitting. I knitted more hexagons and measured the blanket on the bed. I went out and bought more yarn. I have the hexagons to add another row and the blanket is slowly growing…

I think that I’m going to need more yarn… this is the Nectar Blanket by Ysolde Teague.

I’m really happy with the progress of the blanket, but I have to admit that it is becoming a little boring. I dream of other knitting projects as I sew the hexagons together. I have a serious urge to knit a new sweater in some fabulous colors.

Look at this little topper sweater!! It is called La Prairie (by Joji Locatelli) and I seriously want this sweater. I want to knit in cool colors. Did I mention that I live on the edge of the Great Plains? I long to wind yarn and to cast on and to start knitting those waves and bobbles…

I broke down and bought this kit to knit the sweater online a few weeks ago.

The trouble is, I just have to dream and fuss about colors before I am happy. I’m not sure about the order in the kit yarn. I’m not completely a fan of the middle yarn, and I feel like the lavender should be in the middle. I’ve been digging in the stash and trying to image the finished product with other blends of color…

Here are three more spins on the yarn. I like the one on the left the most, with the yarn fading from dark purple to the light grey at the sleeves and bottom of the sweater, but I’m kind of interested in the middle and right versions. I have to ask myself… which version will have the most flexibility in the wardrobe? Definitely, it would be the first spin on the kit, the version with the light grey at the sleeves.

I also desperately want to buy yarn to knit a Soldatna Crop sweater, and some new arm warmers, and then there are those PICC line covers and hats to get done… Did I mention that I have been looking at more yarn online and dreaming of sweaters with lots of colors in DK weight yarn? I’m totally on a knitting drive, but my wrists are not on board with all this needle action. It could be that I’m not completely done with the flare…

Sigh. Guess I’ll just cast on another hexagon or two…

PS I’m still dealing with shortness of breath and low on oxygen; lung testing happens next month as my doctors continue to sort me out. 🙂

The Scleroderma Chronicles: Rare Disease Day, 2023

Well, here it is again. Rare Disease Day. I kind of was going to ignore it this year because I’m quite frankly worn out by my… wait for it… rare diseases, but I also feel like I should pull myself together and represent for the community again.

People with rare diseases are referred to as Zebras in the medical community. I obtained my zebra status when I was diagnosed with systemic sclerosis in 2014. This zebra was sent to me last week by my *Most Knitworthy Niece* Melissa.

I wrote a pretty darn good post last year about my journey with a rare disease which you can read here if you want. I talked about rare diseases in general, my specific conditions, and the many things that have been said to me by my doctors over the years. I thought about just reposting what I wrote last year, but I’ve been reflecting all morning on some recent events that kind of shine a light on my situation and that of other people who are coping with rare conditions.

I recently managed to go knit with my fellow members of Frayed Knots. This was a big social outing for me because it’s hard to get out of the house, and I have to be having a really good day to go to something like this. Knitting with friends is just “normal” for most people, and it would just be a little part of their day, but for me this was something that I had to prepare for a couple of days in advance, and then recover from in bed the next day. Many rare conditions are chronic, and chronic illnesses can be very isolating by their very nature.
A woman at the knitting group questioned my decision to wear a mask. I started to explain, but she cut me off to say that I was doing it so I could feel comfortable. It was a little condescending and suggested that I was being paranoid. Truthfully, my immune system, crushed by the drugs that I am taking right now, is compromised in its ability to make antibodies. If I catch a viral disease like the flu or Covid, there is a good chance that I won’t survive. My vaccinations have a low chance of protecting me for the same reason. For people with rare diseases, life is fraught and full of difficult decisions. For me, and for many other immunocompromised individuals, simple decisions involve life/death level risk analysis.
Another woman at the knitting table was struggling with long Covid and shared her difficulties with returning to work. She especially felt crushed by the attitudes of her coworkers who seemed to feel that she was “fine” and just trying to get attention. Yep. Been there, done that. Many rare diseases are genetic or largely invisible to others. Invisible illnesses are especially hard to cope with because others tend to question their validity.
I’m in several online support groups, and there are always discussions about what drugs to take, and whether the side effects are worth the risks. Yep. There are no specific drugs for systemic sclerosis, no cure, and treatment can involve a patchwork of risky off-label drugs. The drugs that are used are often non-specific carpet-bombing like approaches. Rare diseases have fewer treatment options because there are only a limited number of patients.

Over the last year my wonderful team of physicians have been suggesting that I am really unusual and have been extremely responsive to my emails. They clear an hour for appointments with me. I’m one of the very lucky zebras who has managed to get diagnoses, secured treatment, and am benefiting from a team of collaborative, interdisciplinary physicians who actively communicate with each other and with me; just last week my rheumatologist told me that for a patient with my status this is the only way to deliver care. I’m so grateful to have secured this level of medical attention, but I also feel a little nervous about it. I spent some time this morning trying to work out the probability of one person having the several medical diagnoses that I’ve racked up since 2014. Like, just how rare am I?

The National Organization for Rare Disorders estimates that there are 100,000 patients with systemic sclerosis in the United States. That’s rare, but still, a big club, right?

The 15% Rule is a general measurement of the risk of severe organ involvement in systemic sclerosis. As it turns out, quite a few of the major lung, heart, and kidney complications associated with systemic sclerosis happen about 15% of the time. I have Sjogren’s Disease overlap with my systemic sclerosis, which happens in about 13% of patients. Suddenly, I’m in a much smaller group of about 13,000 patients.

My most worrisome complicating conditions are diastolic dysfunction (a type of heart failure), pulmonary arterial hypertension (PAH) and interstitial lung disease (SSc-ILD). I looked up the risk of having each of these conditions using the 15% rule data, and it turns out the risks are 16% (diastolic dysfunction), 15% (PAH) and 35% for the SSc-ILD. Did you notice the the ILD doesn’t fit the 15% rule? Yep. It’s much more common and is the leading cause of death in systemic sclerosis patients. I found that risk factor here.

I brushed up on my probability math (you multiply the probabilities of independent events…), and after running the numbers:

100,000(13/100 x 16/100 x 15/100 x 35/100)

I came to a grand total of 116 other patients in the US who share my set of diagnosed conditions.

Oh.

See, I have lots and lots of stripes. Stripes in purple, teal, periwinkle, red, green, and blue: these are the awareness colors for my conditions.

I just ordered that rainbow zebra unicorn shirt! I plan to wear it with my mask on my next social outing…

You can learn more about Rare Disease Day or my conditions at the links below.

The Scleroderma Chronicles: So, there was this flare…

Wow. It has been some time since I last posted. Ironically, the last post was about the Mother of Cats being broken. I felt really good, and I was casting on a plethora of projects and was all over the place with my knitting, reading, projects, you name it. Hannah was complaining in that post that I was broken, and that I wasn’t devoting myself to making her a blanket.

Then scleroderma lowered the boom on me. I went into a flare of epic proportions. I hurt all over, I struggled to do just simple tasks, I started sleeping 10-12 hours a day, and I had to go back onto daytime oxygen. I kept heat packs on my sore joints, but I really needed a total body heat pack to cope with what was going on: every single tendon in my body was under attack!! I couldn’t regulate my body temperature and took multiple naps a day because I was too cold to stay awake. I developed chest pain. My brain fog became so bad I wasn’t safe to drive anymore… Yep. Broken.

Okay, I also accidently drank some Miracle Gro fertilizer that I had stored in a Snapple bottle. That is some serious brain fog, people!! (Yes, I did have to call poison control, I did eat burnt toast and I did drink a lot of water, and I don’t want to talk about this anymore…) Yep. Broken.

I managed to get some knitting done in spite of my ill-behaved health. I got some wristers made as Christmas presents and sewed zippered pouches for my sister and cousin.

Wristers and a zippered project pouch.

Sewing was a riot as I had to get the sewing room organized so I didn’t have to stand up very much as I cut, sewed, and pressed fabric to make the bags, but with some creative thinking and furniture arrangements I pulled it off.

I also finished my Emma sweater. I made a hat. I made some socks. I made some slippers. Everything was on big needles and used heavy weight yarn as I got too tired to knit more than a few minutes at a time.

This sweater was knit using the CocoKnits method and I learned some new methods of shaping and finishing. The yarn was chunky weight and is warm and comfy. I got it done early in the flare and it was just perfect for the days to come. The yarn that was left over was used to make a hat and a pair of slippers.

I did read some nice books, but mostly I slept and waited this miserable thing out. In the weeks while I was mostly bedbound my orchids started to grew stems for future blossoms, the first snows arrived, and my son moved in with his cats.

This is handsome Jonesy. Hannah does not like Jonesy, so there is now a chicken wire barricade at the top of the stairs to keep the two groups of cats separated. My son also has a long-haired tuxedo cat who just doesn’t care about my cats. Hannah, however, has a complete meltdown if Jonesy comes near her, so… barricade.

Mateo: I love Jonesy and I go through the barricade every day to rough house with my new best bud while Hannah hangs out with the Mother of Cats. Jonesy is the wild playmate of my dreams!

So, I think that I am out of the flare now as the multiple naps a day have stopped, and my appetite is back. Yesterday we made lasagna for Christmas and here is the present that I got from my son:

Perfect, right?

Can you see my blue lips? The bad news is that the Blue-Lipped Zebra is back in full force. I finally emailed my cardiologist to let him know that I am once again panting for air and feeling light-headed from doing simple tasks like… lifting a pan out of the cupboard or grating cheese. Ugh. I’m pretty sure that I am heading back into the cath lab for another look at my pulmonary pressures, but right now I am glad to be back on my feet and out of the flare. My son is still here (he works from home, so he just moved his operation over to my house) and things are going well in the combined household.

Hannah: as long as Jonesy stays on his side of the barricade!! Also, maybe the Mother of Cats will now settle down and get my new blanket done!!