The Scleroderma Chronicles: A Trip to the Cath Lab

The BLZ has been waiting a long time for this…

Five years ago I came down with the flu and ended up in Urgent Care struggling to breathe. I scored some antibiotics, steroids, and cough medicine. To fight the virus I was told to go off my immunosuppressant drugs for a few weeks until I got better, stay in bed, and load up on chicken soup: it took a couple of months but eventually I got back on my meds. Except… things weren’t quite right. I panted for air every time I came up the stairs and I noticed that my lips were turning blue. My blood pressure was too low and I had to stop taking my hypertension medicine. I felt dizzy and light headed sometimes.

I was a newly diagnosed systemic sclerosis patient and my doctors began running tests to see if my disease was impacting my lungs. Nope. Not my lungs. Tests were run to see if scleroderma was attacking my heart. Nope: my heart seemed to be normal. There were some anomalies, but my doctors decided to just monitor me through routine testing and see if things changed down the road. My red blood cell counts were way too high, and I had nocturnal hypoxia, so I was started on overnight oxygen. I was tested for various conditions that could account for the weird test results, but I always had a normal result.

I struggled on, battling for more testing, as my doctors kept reassuring me that I was okay. Hey, I had a blue face and panted for air when I climbed stairs; vacuuming could put me on the floor. How could this be “normal”? Ugh. Welcome to scleroderma, I thought.

I began to think of myself as the Blue-Lipped Zebra (BLZ). If I didn’t have a rare disease confusing the issue I would be getting better health care, it seemed. If doctors didn’t tend to apply most-common-cause thinking to my condition they might get to the bottom of things faster. It was, in my mind, a huge complicated mess as my doctors applied best practice (and rigid) diagnostic parameters to my symptoms or zeroed in on specific complications of my scleroderma and ignored other possible (and to be fair, rare) causes for my symptoms. I worried that my doctors had just parked me in a holding pattern as I slowly got worse; it is hard to advocate for yourself when you are sick and dependent on your doctors for help, even if you think that they are dismissive and borderline disparaging.

I struggled on as my face became more blue, my red blood cell count higher, my panting for air more common, and the occasional near-fainting event left me collapsed on the floor. I began to ask for a right heart catherization procedure to directly measure the pressure in the right side of my heart. “Oh. You don’t want that,” I was told. “That is really invasive testing.” Umm… I think I do, I would reply. Nope. Nope, nope, nope!! “Not even on the table,” one pulmonologist said.

During lockdown last year I got much worse; ironically lockdown also gave me the opportunity to reboot, fire my old doctors and acquire new ones. My new team of doctors this spring ordered up testing that showed definite issues with my heart and lungs. I am now a heart failure patient (the wall of my left ventricle are too stiff and scarred to beat well) and there were concerning findings that suggested that I had developed pulmonary arterial hypertension (PAH): there are areas of cell death in my lungs and my pulmonary artery is too big. There is too much fluid around my heart, a suggestion of ongoing inflammation. I have a hole in my heart between the atriums (a cardiac shunt) that is impacting blood flow. I was gently prepared for the PAH diagnosis, assured that there were great drugs that could help me, and a right heart catherization was ordered by my new cardiologist.

Yay!! About time!!!

Finally, after 5 years of struggle, I was yesterday wheeled into a procedure room to a waiting team of specialists who hooked me up to equipment and took me though testing to get a better look at my heart: I learned in recovery that this team calls themselves “the pit crew”, and that is exactly what it was like. Within 5 minutes I had completed a breathing test, was on oxygen, wired up to a heart monitor, hooked up to an IV, medicated, prepared with surgical drapes, and swathed in warm blankets with a heater by my feet. There was music playing and the crew was cracking jokes as they darted in and out from the table getting me ready. I was knocked out for the echocardiogram imaging of the back of my heart that was done using a probe in my esophagus, but they woke me up for the main event: the right heart catherization. My cardiologist inserted a probe into the carotid vein in my neck and threaded it into my heart by way of the superior vena cava (blue side of the heart diagram above). People, this was the most amazing experience ever! There was a huge screen showing the progress of the probe and I could watch and ask questions as the line snaked through my heart; there was absolutely no pain. “Well, this is interesting,” said my cardiologist at one point, and there were more measurements happening and a flurry of new activity from the team. The BLZ felt a surge of elation: they had found something, and it was NOT what they expected.

Back in recovery my cardiologist caught up with me again. I had done great, he said, and he just beamed as he told me that I absolutely did not have PAH. This is great news, he assured me, great news!! There is another circulation problem in the lower part of my heart, between the ventricles. There is blood coming in from the left side of my heart and mixing with the blood on the right, disrupting the flow through the heart and robbing me of oxygen to my body. I have a second, more serious, cardiac shunt, and now it is a question of locating that pesky little guy and doing something to fix the problem.

It has been FIVE YEARS, people. If I hadn’t had my trip into the cath lab yesterday my doctors would still be nagging me to get more exercise (the BLZ just barks in laughter), offering me antidepressants, or insisting that I must have sleep apnea. I feel so validated!

My cardiologist is now going over my previous imaging to find the hole now that he knows what to look for. I was told that I may need to go through more testing to definitively characterize the opening, but this is huge forward progress. I suspect that I am facing open heart surgery down the road, but I am elated that the progressive and eventually fatal diagnosis of PAH is now off the table. Things are looking up since my heart failure will now be much easier to treat.

I am reminded of Elizabeth Zimmerman’s admonishment: “Knit on with confidence and hope, through all crisis.” Also, when the going gets tough, get a kitten!

Today I am waiting to hear back from my cardiologist who is going to email me with follow up instructions after he has finished going through the data and past test results. I’m on oxygen, knitting, and feeling pretty calm about all the new developments.

Almost exactly 7 years ago (August 28th was the anniversary day) I was diagnosed with Limited Systemic Sclerosis and Sjogren’s Disease. I have learned a lot along the way, but the best, most important lessons have been about self-advocacy, facing down the worst case scenarios, communicating with your doctors, and maintaining a good attitude.

Yesterday this all paid off for me big time.

Note: The fabulous BLZ graphic was made for me by my exceptionally knitworthy niece Melissa and her beautiful and talented daughter Eleanor.

The Saturday Update: Week 24

June continues to misbehave: we had three days above 100 degrees Fahrenheit this week and several severe thunderstorms with tornados! My arm is still swollen and painful but the numbness is starting to improve; as if in solidarity one of my knees started to complain just today. Sigh. Knock it off, you guys! My new refrigerator came today and I am so happy to have dependable food storage again. Besides, it is just beautiful and shiney! The flowers in the garden are starting to bloom and the roses in particular are looking mighty fine. I have started to knit again every night and I also found some interesting books to read. I bought new glasses and ordered a bunch of fun stuff from Amazon. (Do I need purple and gold washi tape for my journal? Why yes, yes I do. I also got pink and gold replacement bands for my Fitbit and a pair of pink/grey comfy pants.) I went to the yarn store and bought more yarn… that may have not been wise, but I want to believe that I will return to full knitting capacity soon. 🙂 Okay, June isn’t being all bad… it was Hannah’s one year adoption anniversary this week.

Hannah on the 15th (her adoption anniversary date) and little kitten Hannah on the day I brought her home last year. I tried to recreate the picture from her first day home but she wasn’t all that interested and I was lucky to get the shot that I did. To celebrate her anniversary she got crispy pink paper to play in (that is always a big hit with her) and some bows to drag around. Last night I applied to adopt a kitten companion for her from the Cat Care Society and today they called me to approve the request and put us on the list for kitten alerts. Hannah was adopted from this shelter and they consider it a priority to get her a little friend. Yay!

I’ve requested a male, orange tabby kitten. We’ll see what happens. Hannah can’t wait.

Knitting

I’m working steadily on the mitts for my knitworthy niece who is a huge fan of all things Mandalorian. I am now up to the fingers of the second mitt.

Aren’t these the cutest things ever?

I need to do some duplicate stitch detail work and then there are all of the ends to weave in, but the end is definitely in sight now. I’m almost tempted to make a second pair but I have soooo many sweaters and other projects that I need to get done. This pattern is Mando (Natela Astakhova).

Garden

The roses are blooming! The roses are blooming! The roses are blooming!

From left to right, these roses are Hot Cocoa, Princess Alexandra of Kent, Cinco de Mayo, and Home Run. We had lots of rain this spring and I have been watering every day since then; this is the best crop of roses I’ve had in quite a while. I really was responsible this time with the roses as I covered them with frost clothes if there was even a chance of a hard freeze and I fertilized them early in the spring. This week they got more fertilizer and they are all looking good with lots of buds on the plants still waiting to bloom. Yay, garden!!

Books

I have two books going right now; one is text and the other is an audiobook. They both are really holding my interest and curiously they even look similar.

How crazy is it to be reading icy blue books when trapped in an heat wave. I’m only half way through The Doors of Eden and even less with The Cold Millions, but both books are thought provoking and really interesting.

And that’s all I’m going to say about them right now. 🙂

Have a great week everyone.

Read a little, knit a little, and garden like your heart can’t live without it.

Scleroderma Update: My rheumatologist had my hands x-rayed this week and I have severe arthritis in both of them, but especially the one that is now acting up. I was kind of surprised and I’m hoping that they can do something to help with the swelling and numbness. My echocardiogram results also came back and I do have a hole in my heart, but an email came from my pulmonologist yesterday saying that it isn’t big enough to account for the whole panting/blue lips thing and he wants to do some more testing. Fun. We have an appointment next week.

The Saturday Update: Weeks 22 and 23

I’m going to be honest here: June has been really challenging so far. My entire right arm is numb and in pain and there isn’t much knitting going on. My appointment book is completely filled up with medical appointments and the bruises are collecting at an alarming rate; did I mention that June is Scleroderma Awareness Month? Yep. I’m aware. Scleroderma has gone into overdrive this month. Gee, if it wanted more attention it should have just asked!

In solidarity with scleroderma there have been other disasters this month. The car that was bringing me home from GI testing this week developed engine trouble (I ended up in an Uber, hopped up on drugs, leaking fluids from an unmentionable part of my nether region… best ride across town ever!) My refrigerator started shrieking in the night as one of the fans failed putting my stash of cheese at risk (yes, I have seriously cornered the market in… cheese). Oh, did I mention that I also had a scary close encounter with my neighbor’s pit bull? Seriously, it has been challenging to do anything except deal with all of this stuff. On the other hand, I have handled things; a new refrigerator is coming (I must save the cheese!), my neighbor has implemented more safeguards to contain her dog, and I have learned how to use Uber!! I’ve completed physical therapy and am walking much better. The car that broke down was repaired at little cost. My arm is in a brace but I am rocking voice-to-text on my phone. I’m knitting again… slowly.

Still, I am warning you, June, knock it off!! I have knitting and stuff to do.

Hannah: June would be a great time to get me a little kitten companion!!

Knitting

I finished my Noncho (Sharon from Security and Casapinka) this week. What a nice, versatile addition to my wardrobe this will be. I also made some progress on a sock but really the big accomplishment this week has been the Noncho. My project notes on Ravelry are here.

Garden

It’s gotten hot and the garden has taken off. I’ve been weeding steadily and it is really exciting to find flourishing plants and emerging flower buds. Look at what is going on right now:

Seriously, the garden is starting to explode with color. My rose plants are covered with buds and the main show is going to start in just a few more days. We had so much rain earlier this year the rose bushes grew really well and things are looking good. Okay, June is picking up a little.

Books

I’m enjoying The Cold Millions while I work on my second sock. So far it is pretty good and applicable to economic issues that we are dealing with today in the US.

Have a great week everyone.

Read a little, knit a little, and garden like your heart can’t live without it.

PS: The gastroenterologist told me that I can’t eat cheese any more. Or chocolate. Or caffeine. Or carbonated drinks. Alcohol is absolutely forbidden.

I’m pretending that I didn’t hear that part about the cheese.

The Saturday Update: Weeks 11 & 12, 2021

Life has been busy and I’ve really gotten behind on everything. Hannah had her first birthday last week:

Hannah: I’m a big girl now!!

Seriously, I was so busy and worn out over these last two weeks I barely got any reading or knitting done. Adding to the energy drain was my second dose of Pfizer Covid-19 vaccine and three weather fronts that barreled across the state last week. I am grateful that the snow that these storms dumped was manageable this time, but each one took a toll on my joints and breathing. That’s okay though… did you notice that I got my SECOND VACCINE SHOT!!! This is just huge! I am starting to schedule medical appointments right and left as it is finally time to get some attention for my very badly behaved hip and breathing issues. Bring it, Mother Nature. I am ready to take you on!! The Blue-Faced Zebra is emerging from a year of strict lockdown and is on the move again!!

Also, it is now spring and the birds are back and I am so happy to see green shoots of grass again. It has been a really long year, but it is finally getting a little better.

Knitting

I’ve been pretty sporadic with the knitting and I’m kind of limited to simple stuff right now because my hands are NOT HAPPY with all of the running around and weather events, but still I am making some progress.

My Goldwing sweater is getting longer in the body and I am debating how much more to knit before I start the ribbing at the bottom. I often finish the sleeves at this point and then come back to finish off the body as I’m always running short of yarn, but this time I think I can do things in the usual order as I bought an extra skein of yarn.
I’ve also been working on these simple ribbed socks using yarn that one of my sons gifted me with for Christmas. This yarn is Classic Sock by Spun Right Round in the colorway “Hellbent”. I really wanted this yarn, but it looked awful after I wound it… all brownish and muddy looking with some color sadly peeking out. I stuck the wound cake back into the stash to hide it for a few weeks. Needing a small project to take with me on my adventures out of the house, I pulled the cake out last week and cast on socks; it was already wound and I was in a hurry. As I knitted I fell in love with the yarn again. Now the colors sparkle in the mostly grey field and the brown elements have receded into the background. Who knew that would happen? Today, in the picture, the sock looks mostly pink. This sock yarn has been a learning experience for sure.

Garden

Poor garden. It has been so neglected lately, but the plants are still hanging in there. The best thing that is happening garden-wise are the microgreens that I have growing under the lights with the orchids.

I have been eating lots of microgreens these days. Full of nutrients, easy to digest, lower in fiber, this has been a great thing to add to my diet. These greens are broccoli and taste great; I’ve discovered that they work well added to almost everything so I manage to eat some every day. Take that, scleroderma!! I’m growing them in the Hamama system that I got for Christmas and I just reordered more seed quilts because I’m really liking them.

Books

I finished up several books over the last two weeks; let’s just chat about two of them, okay?

I was so in love with World of Wonders when I started reading it. I just loved the way the author blended her joy with the natural world with her family and life experiences. Then as I read more I began to become less charmed as her interactions with nature seemed more forced and some of the magic was slipping away in her writing. By the end of the book I had gotten over her and was thinking that I could do better job writing about my own love of nature and my blended experiences. The author shared experiences from trips around the world; for me the continual moments of joy that have been my encounters with nature that are truly home grown. I was thinking of all of this as I drove east last week and passed a big herd of pronghorn antelope grazing in the Plains Conservation Center near my house. Pronghorn antelope!! I love pronghorns… I could tell you stories about pronghorns… I hadn’t seen pronghorns for at least three years, and there they were, two large groups, just as I was thinking about writing about my lifelong love of all things biological. It’s a sign. Be prepared for some upcoming “Tales of a Biogeek” on the blog.

Now to chat for a few minutes about We Begin at the End. Why did I read a book with a quote on the cover about breaking readers’ hearts? Oh, it also said that it was impossible to not keep turning the pages. Well, that part was also true. This book, constructed so cleverly that even the most astute mystery reader will miss some of the underlying themes, is a tragedy of Shakespearian proportions. The town in this book and the inhabitants are, quite frankly, broken. Badly broken to the point of being almost incapable of functioning, and yet they do somehow. Tragedies in the past have set in motion a series of events that bring unintended consequences that are both catastrophic and ironic for all of the main characters. My heart was broken, but at the same time there was a type of peace and balance in the ending. What a mess these people made of their lives; what a story this book tells. I miss my book group sooo much as this was absolutely a book that should be talked about with other readers.

Hey, did you know that today is National Respect Your Cat day? Yeppers, it is. Here are all the cats in my life in their most “You may now respect me” poses.

Hannah and my Grandkitties Jonesy and Maya will now accept your respect!!

Have a great week everyone!

Read a little, knit a little, and garden like your heart can’t live without it.

The Year Alone: Reflections on Wonder, Luck and Hope

There is a storm on the way. These things look a little unbelievable on the weather forecaster’s computer display, but there seems to be a massive low pressure area cut off from the jet stream sliding relentlessly towards a part of the United States that will set up a big weather event. Snow. Lots of snow. Maybe feet of snow. I’m pretty stocked up but I needed a few items for my weekend cooking, so I headed to the grocery store late in the day to grab them. Oh, oh. The store was packed and the shelves were already emptying out. Shoppers radiated urgency as they raced down aisles disregarding the one-way Covid-19 traffic patterns. New shoppers were pouring in the door as I checked out and there was a whiff of panic as they passed me. This is crazy! How much snow is really coming, I wondered as I loaded my bags into the car and escaped the chaotic parking lot. I hadn’t seen anything like this since the early days of Covid-19 as the lockdown approached…

The lockdown. This week is the anniversary of the first Covid-19 death in my state, Colorado, and in just a few more days it will be the anniversary of the lockdown that started my year at home in isolation. So many people have been sick, and way too many have died. So much has been lost by so many people; this is the greatest tragedy of my lifetime. For me, however, in strict isolation, the year has rolled by with me in my own little world mostly disconnected from the greater world outside; my story is a lockdown story, not a Covid-19 story. I have been disappointed by people who kept me trapped in my home by refusing to wear masks or to comply with public health recommendations, and brought to tears by the kindness of strangers. A year is a long time; looking back now it seems like it passed in a flash even though I had some real struggles along the way.

Last night one of the local news programs had people post the last picture on their phone before the lockdown happened. Here’s mine.

My son’s kitten Jonesy in a tube attached to the cat pillar. He’s a 6 months old kitten in this shot.
Jonesy today as a handsome young adult at 18 months old.

This year of isolation has been 2/3’s of Jonesy’s life and the entirety of Hannah’s life as she was born within the first few days of lockdown in the middle of March. Looking at Hannah and Jonesy it is so obvious how long this year has been. Looking at Hannah and Jonesy it doesn’t seem all that bad, but of course this has been an extraordinarily tough year.

I sewed some cloth masks early on and wore them on the few occasions I had to leave the house: a science geek who had read way too many books about epidemiology, I suspected airborne transmission based on anecdotal reports from the New York City outbreak. That mask picture is the 2nd one on my phone after the start of the lockdown. As the debate about mask efficacy raged in online forums I wore mine anyway and ignored people who made negative comments. Almost a year later I was wearing two masks, one a highly regarded Vogmask, as I got that first dose of Pfizer vaccine.

“Do you trust the vaccine?” asked my neighbor yesterday. “Absolutely,” I replied.

I feel very, very lucky to get this Pfizer vaccine. Through chance I have a degree in molecular biology and worked for years in an immunology research lab. The molecular trickery used in this vaccine to harness my immune system to protect me from Covid-19 is the best thing that happened all year in my opinion.

I’ve been assigning lots of labels to this year spent mostly alone with my little tuxedo kitten, my books, and my bottomless pit of a yarn stash. This has been the year of astonishment. The year of disappointment. The year of living dangerously. The year of setting priorities. The year of realigning values. The year of healing. The year of decluttering. The year of absolute outrage. The year of lies and fake news. The year of masks. The year of Zoom. Finally, today, it is the year of luck and wonder.

I do want to apologize for my use of the words luck and wonder. There is no real luck in a pandemic at all. I absolutely know how awful and devastating this has been for so many people: how profoundly unlucky so many of us are that this happened to us right now in our lifetimes. The mutation and jump to humans of the coronavirus that causes Covid-19 was an event that has been anticipated and feared for a long time. It’s like waiting for an earthquake in California (The Big One) that will be massively destructive. You know it is coming, but you don’t know when or exactly where it will strike, and how devastating the damage will be. You prepare for it and hope that you are ready. How ironic, after growing up in California waiting for The Big One to hit, the crushing event of my lifetime came from a virus. In spite of the basic awfulness of all of this, I am learning to value the little crumbs of luck that came my way during my time in strict isolation while other people recovered so much of their lives and I was left behind.

I am so grateful for the luck, the random chance events, the technology, the human kindness, and the science that helped me get through this year.

My wonderful pandemic kitten was a failed adoption returned to the shelter and rejected by everyone else before I arrived to find her alone standing in a little cat tunnel. The last kitten left in the kitten room; her 6 litter mates had all found forever homes days before. Returned, rejected, all alone: the one thing that I needed. How lucky is that?

That’s little abandoned kitten Hannah on the left, 6 month old Hannah in the middle, and Hannah tonight hanging out with me while I type. Hannah was the one thing that I needed to pull me out of growing sorrow and a sense of abandonment when I realized that the the pandemic was raging unchecked in the USA as the result of a deliberate policy set by my government.

I was diagnosed with nocturnal hypoxia and received the oxygen equipment 5 days before lockdown. How lucky is that? Because of the oxygen I have been steadily improving for months.

I can order anything that I need online and have it delivered to the house in just a few minutes, hours, or days. Seriously, almost anything. A hamburger? It’s on the way!! A case of paper towels? My Instacart shopper is on it! A pair of new sheepskin slippers? Amazon makes it happen! More yarn… yes!! A plethora of indy yarn dyers will ship me my heart’s content. What would have happened if this pandemic hit in the 1990’s? How lucky that the technological infrastructure that allows all of these supportive services to exist is there for me and everyone else who needs them.

Every time I stream a movie on Netflix I feel lucky.

I was raised in the 50’s and 60’s: I can home cook from scratch and have returned to the meals of my childhood. Comfort food in a modern crockpot. How lucky is that?

I have a SMART PHONE that does everything that I can imagine doing. It banks for me. It remotely checks me into my doctor’s appointments. It tells me the route to drive to come home after a long day in a medical center. It connects me to so many other people in Facebook forums. It answers all of my questions: a couple of YouTube tutorials can handle any crisis. I can text all of my friends and family no matter what is happening outside. I feel lucky, people!!

I met with my primary care doctor via Zoom. I feel grateful for Zoom, people, even though most people in America hate its guts by now.

Knitting and reading groups have sprung up online that have connected me to wonderful, supportive, and positive friends from all over the world as we share our books, knitting, and cats. Then there is this blog. I tell you, I feel so lucky that this is all possible.

The Sharon Show, a MKAL run by Sharon from Security, a snarky cat who loves whiskey a little too much…

I can get unlimited ebooks and audibooks (well, as much as my bank account will allow…) instantly delivered to my Kindle tablet. I feel lucky.

In my year of isolation, oxygen, and limited adventures out of the house my painful joints have settled down, my kidney function has improved, and my red blood cell count has fallen into normal ranges for the first time in years. I am doing much better in many ways. Okay, I’m looking at a lot of medical testing to identify the cause of persistent chest pain, and there is still the problem of the severely damaged hip joint, but I’m in much better shape at the moment to tackle this then I was a year ago. I feel lucky.

Winter will end and the garden outside will come back to life soon. I feel lucky!!

I spend too much time wondering about things. Left alone, I have a lot of time for my imagination to run wild as I wonder about everything. I wonder if my roses all survived the dry winter. I wonder if the vet will yell at me for not getting Hannah in for her shots this year. I wonder what would have happened if this pandemic happened 20 years ago. I wonder what if it hadn’t happened at all. I wonder if I should throw away so many of my belongings as I declutter. I wonder why do I have a rare disease (systemic sclerosis) that appears to share some characteristics with Covid-19; what were the chances of that? I wonder when the Big One will come. I wonder if I should write a book. I wonder how I should combine colors of yarn in my next knitting project. I wonder what is happening with the Covid-19 long haulers. I wonder why some Covid-19 long haulers have improved after getting their Covid-19 vaccinations.

I wonder why, one week after my vaccination, I feel significantly better then I did two weeks ago.

I am actually looking forward to shoveling some snow this weekend.

In the greatest of tragedies there are still little crumbs of luck and rays of hope.

May our bad times end soon and we all have days of wonder, luck, and hope.

The Scleroderma Chronicles: Coming Full Circle

Systemic Sclerosis is a rare, chronic, progressive, and incurable autoimmune disease that is included in the family of rheumatic conditions. It is characterized by blood vessel damage and the scarring and thickening of skin due to excessive collagen production (fibrosis). The internal organs can also be damaged by inflammation and scarring: the digestive tract, heart, kidneys, and lungs.

There are two main types of systemic sclerosis: diffuse and limited.

I was diagnosed with limited systemic sclerosis 6 years ago.

Hannah: Do you like my circle?

The Circle Starts: In high school I developed a mystery illness that involved intense itching, lots of rashes, sensitivity to sunlight, and swollen digits. I was a mess. It went on for a few years and then the worse of the symptoms faded away.

First Quarter Circle: In my mid twenties I was a researcher working on a scleroderma project for the Division of Rheumatology at the University of Colorado Health Sciences Center in Denver, Colorado. The principal investigator that I worked for was interested in isolating the targets of the autoantibodies that scleroderma patients made; if we found the actual proteins that the antibodies characteristic of this autoimmune rheumatic disease were targeting we would be closer to understanding what they did, and eventually closer to understand the disease process of scleroderma. Let me be clear here: my boss, Dr. Angeline Douvas, was the brains of this little research outfit and I did most of the bench work.

One morning Angie had the hot idea that we should see what happened if we did an anti-nuclear antibody (ANA) test on the polytene chromosomes of the fruit fly Drosophila, a common lab experimental animal. We knew that the antibodies produced by the scleroderma patients were sticking to the chromosomes in the nucleus of cells… what would it look like if we checked this test on the chromosomes of fruit flies which were enormous structures that were easy to look at under the microscope?

After staining we could see that on the entire chromosome a few distinct bands were stained: only a few genes were targeted by the antibodies of the scleroderma patients. What was the function of the genes, and what were those proteins, the clear targets of the antibodies made by these scleroderma patients, doing? Something important that was linked to this disease that we call systemic sclerosis (scleroderma). How was all of that tied to the damaging fibrosis going on in these patients?

Here’s the other interesting conundrum that we kicked around: what changed in these genes/proteins that made them trigger the immune system to make antibodies? There are lots of things that can change genes and the proteins that they make. Things like radiation, chemicals, environmental triggers, viruses…

Hannah: Viruses?! The Kitten Mom and I spend all our time at home hiding from viruses!!!

Second Quarter Circle: Now in my 60s, and diagnosed as a scleroderma patient myself, I began writing letters to my congressman asking him to support the National Commission on Scleroderma and Fibrotic Diseases Act, a bill which would coordinate and organize resources to study the process that is involved in the formation of scar tissue in scleroderma and other diseases/conditions. Two summers ago I met with a representative of my congressman, Jason Crow, with other scleroderma patients to make our case. Scleroderma is special, I argued, because our antibodies are a tool that can be used to unpack the process of fibrosis. Representative Crow did support the bill.

Hannah: Now, are you ready for some really crazy stuff? Hang onto your catnip mice, this gets a little wild!!

Third Quarter Circle: It is 2020 and Covid-19 has arrived. Early on we learned that this is a serious virus that causes an extreme immune system response in some individuals that is life threatening. There is severe lung involvement, blood clots, scarred lungs, injured hearts, failing kidneys… this is no joke if you are already dealing with all of this, so I and other scleroderma patients are avoiding it like the plague and retreating to our online support groups even more than usual. We share observations and experiences in these forums, and it wasn’t very long before we started to notice that these severe Covid-19 cases seemed to be awfully similar to severe systemic sclerosis. We laughed about “Covid Toes” since dealing with blue fingers and toes is a daily struggle for us. Then the news reports about Covid-19 long-haulers started to emerge, and we all started to say to each other… wow… fatigue, brain fog, muscle/joint pains, lung and heart problems… sounds a lot like what we deal with… Then some people started to arrive in our support groups who were newly diagnosed scleroderma patients who were also Covid-19 long haulers. Now every week new people, shocked and frightened by their life-altering diagnosis of systemic sclerosis, are showing up in our forums. “Gee, there are a lot of new patients arriving,” someone wrote last week…

Unbelievable, right? I decided to hunt around online and quickly found that there were a number of reports about Covid-19 and systemic sclerosis. I discovered to my shock that people with severe Covid-19 disease do share a lot of documented clinical features with severely ill diffuse systemic sclerosis patients, and there is a connection between Covid-19 and rheumatic autoimmune diseases. When there was an article in the New York Times reporting that some Covid patients were developing autoimmune disease it caught my eye, so a little more work online found this nicely written overview by the Global Autoimmune Institute that listed specific research reports and the autoantibodies being discovered in Covid-19 patients and Covid-19 long-haulers. There in the reports are listed the same, exact autoantibodies that are the specific hallmarks of my two autoimmune diseases, systemic sclerosis and Sjogren’s Disease. 2020, shame on you. This is really, really bad, even for you.

All of a sudden it is really important to understand fibrotic diseases and how to reverse the damage caused by Covid-19.

Hannah: The Kitten Mom feels like she needs to do something about this!!

The Circle Closes: The genome sequencing service 23andMe has launched a genetic research study of systemic sclerosis patients. They are screening and accepting 1000 diagnosed patients who will donate their DNA for research into systemic sclerosis. I’m pretty sure that this effort to collect more information about the genes of systemic sclerosis patients is in part driven by the urgent need to deal with an emerging flood of new patients with fibrotic organ damage due to Covid-19 infections. Tonight I completed my application to submit my DNA to the study and to participate in all their additional data collection about my disease. Remember those few, distinct genes lit up on the fly chromosome? “Go get ’em, boys!” I muttered to myself as I clicked the submit button.

Today there were 225,558 new cases of Covid-19 in the US and 3,499 new deaths.

How many of the Covid-19 survivors will eventually be dealing with a chronic, progressive, and incurable autoimmune disease?

Wear your masks, people!!

Wednesday Afternoon Update: I’ve been accepted into the research program and they have already shipped my DNA collection kit to me. Hannah is so excited!!

Footnote: Did Angie and I find a protein using the scleroderma patients antibodies? Yep. We did.

The Saturday Update: Week 34

Here I am again, posting late on Sunday night. This week was not good. Bad week, bad!! Seriously, it was just multi-dimensional in the badness that went on during the week. I have to admit that it can’t all be blamed on the calendar, although I do think that 2020 continues to behave in a completely unacceptable batshit manner. Fire tornadoes?! Really! Two hurricanes in the Gulf of Mexico at the same time?! There is an asteroid approaching earth? Of course there is! 2020, just stop right now!!

An interesting development of the week was Hannah discovering MacKnitzie on the bookshelf. She’d been up there several times before, but now she thinks that it is fun to drag him down by his tail onto the floor to play with. Such a determined girl, she is undaunted by the crash of falling books…

Incorrigible! I think that MacKnitzie is in for a few adventures…

There was almost no reading this week and very little knitting. Why? Just read on, my friends. 🙂

  • I am fighting an infection, which is always a situation where I first respond with an “oh, oh” and then with a shortly followed word that won’t be shared here. I went onto antibiotics and off my immunosuppressant drugs: race time!! As my immune system revs up and attacks the infection I am also going into a flare of my disease. SO NOT GOOD!! The plan is that the antibiotics will tip the scales to favor defeating the infection before I’m too sick. Ugh. As I got sicker and sicker from my autoimmune buddies I started sleeping through most of the day.
  • We waltzed around with triple digit heat all week. That slight wailing you hear is my crispy garden plants crying in the heat. The louder wailing you hear is me…
  • There are horrible wildfires going on in my state and California that are sending smoke my way.  A lot of smoke. The sun is a dull red ball in the sky, ash is falling, and things don’t look to improve soon. My distress needle is firmly pegged in the red. My lungs think this isn’t optimal. The smoke is affecting my eyes so I can’t read and I’m staying on oxygen 24/7 while this is going on. I know that this is nothing compared to the people fighting the fires and evacuated from their (possible destroyed) homes. The latest news is that Australia is sending California fire fighting resources, and our governor just activated the National Guard. There is another tough week ahead.
  • The nightly news over the last week was also enough to make anyone go back to bed. In the midst of an uncontrolled pandemic that has me trapped in isolation with my adventure-seeking kitten, completely dependent on delivery services, evidence surfaced this last week that the postal service has been sabotaged. No wonder my prescriptions have been wandering around and arriving late and why hasn’t my printer ink come yet!! I’m taking this really personally since now, instead of just messing with the world supply of hydroxychloroquine, a drug that I need to control my Sjogren’s and scleroderma, the agency I need to bring it to my door is also under attack. Instead of knitting I directed energy to writing my congressmen.
  • It is the 6th anniversary this week of my scleroderma diagnosis. Normally this date wouldn’t lay me low, but this week I couldn’t walk, breath, eat anything without a negative consequence, and… you get the idea. Sad anniversary, scleroderma.

Whew! The flaming hot hard lump in my leg is much better today and I went back onto my immunosuppressant drugs. This evening, as I type this, my dizziness has faded away and I feel almost peppy walking on legs that don’t hurt. Yay! Hopefully the antibiotics will manage on their own from now on.

Bring it, 2020! I’m ready for you again!

Knitting

I did manage to get some knitting done through the week. I am now working away on the lace outer edge of my Far Away Dreams Shawl. Look!

My goal for the week was to get the lace edging done, and truthfully I worked into the wee hours of the morning last night to get there, but nope, not yet. It is starting to look really nice (all bunched up on the needles…) and I’m liking that dark color for the lace more and more.

I’m also still working on The Sharon Show. The third clue dropped Friday but I am doggedly knitting along on Far Away Dreams since I am so close to the end and I should be able to catch up with Sharon later on in the week. I did download the pattern and discovered that this third clue had lace sections. WOOHOO!! I love lace. This MKAL shawl is going to fantastic. The knitting goals for the week are now set: finish Far Away and then Clue 3 of the Sharon Show.

Also, I dug around in the yarn stash to find some yarn to make another Sharon Show. This shawl is fun!

Garden

I can’t really go outside too much, but by wearing a mask I’m managing to make short trips out to water plants and pick up mail. I wanted to buy some more bedding plants for the fall to replace ones that expired in the heat, but the way things are going right now I decided to take down the hanging pots and put them on top of the dead planters in the front yard. What do you think?

The heat hasn’t been kind to the plants so I babied them for a week on the deck before moving them out front. There is more shade in the front and I hope that they will continue to recover and bloom.

Books: don’t make me laugh. Smoke in my eyes and all that…

Have a great week, everyone!!

Read a little, knit a little, and garden like your heart can’t live without it.

The Saturday Update: Week 26

Week 26?! Do you realize that we are now at the halfway point for the year? I don’t know about you, but 2020 has been just horrendous so far. I do hope that it decides to straighten up and fly right for the second part of the year…

There has been a lot going on for me this week, but I think that I will just start out with the Hannah update. Hannah, who is almost certainly the last kitten that I will raise, is turning out to be the perfect mix of all the cats I have loved in the past. She is affectionate and attached to me, easy to distract and train, smart, talkative, and fearless. She ignores the plants and hasn’t gone after my knitting all week!!

She is particularly fond of little stuffed toys that she can fling around and carry from room to room in her mouth. 

Okay, it isn’t all sunshine and roses. She climbs into the refrigerator and dishwasher every time I open them.  She started climbing the screen door and curtains today. She pestered me to wake up this morning because she wanted me to turn off the oxygen machine so she could play downstairs… I’m hoping that that doesn’t turn into a thing! Still, I am so happy to have my little buddy now that it appears that Covid-19 won’t be going away any time soon in my part of the world.

Knitting

I’m making some progress on my socks! The first sock is done, and now I’m cruising through the second sock. I’m focusing on small projects that I can quickly stuff into a project bag because… kitten!!

Look at how much progress I’ve made!

I really like the way the knitted fabic looks!

I love the stripes so much that I’ve been daydreaming and trying to work out how to knit tipless gloves by adapting my usual fingerless mitt pattern to put on the half fingers. Wanting to maximize the amount of leftover yarn I dug through the stash and located the purple yarn that I’m using for the heel and toe portions of the socks. I’m pretty pleased with the look, and now I’m wondering how to incorporate the purple into the gloves. My Ravelry notes are here.

Garden

The week has been one of gloomy afternoons and thunderstorms. Luckily I haven’t had damaging wind or hail, and the roses continue to strut their stuff. My Princess Alexandra of Kent rose in particular continues to shine.

This rose is a David Austin English rose and I keep thinking that I should get some more. My neighbors and I fixed the fences this summer, so maybe those new fences should have some climbing roses planted near them. Something to think about. I really like yellow roses…

Books

I finished The Mirror & The Light this week. I hardly know what to say. This is a rich, rich book that will continue to haunt me in the weeks to come and I may need to read the entire Wolf Hall trilogy again. Maybe it is because I am entering my fourth month of isolation, and I have lots of time on my hands to reflect on things, but the richness of the characters and the subtle connections of the past to the present as the story plays out, but never really ends, are just astounding. Cromwell ponders on how images painted in the past bleed through new paint to show in the present as he remembers violent actions in his past.  Memories of his years as a soldier rise as he marches into meetings and dinners. Near the end of his life, imprisoned in the Tower of London, he recaptures that transformative moment, broken and bloodied in the street, when he abandoned his childhood to launch on the path to who he was now. At the start of this book one of the standout lines is, “if you cannot speak truth at a beheading, when can you speak it?” At Cromwell’s own beheading there is a sense of truth and transition again; I was dreading the end of the book, but you know what, it was actually hopeful and a befitting closure to a great life.

After a book like that, what next? Science fiction, of course! I launched right into a fun little space military opera that is a three book series and I’m happily working my way through it.

There are a number of characters who are slowly being developed and connected as the story line progresses. There was a war. One planet lost. The losers are suffering under harsh peace treaty stipulations. There is some type of rebellion brewing. I sniff corporate greed and political machinations on the horizon. Must keep reading…

Quilting

Look at this! A new category just appeared again. This week has been kind of tough on me joint-wise, and I have finally made myself admit that I need to lay off the knitting for a while. Okay, my joints are really kicking up a fuss, and my shoulder is the biggest complainer. Sigh. It’s like all of the tendons and ligaments are under attack at once, and my drugs aren’t keeping up any more. I’m already on a lot of drugs, and in the world of Covid I’m fearful of getting steroid injections into the worse joint complainers because it increases my immunosuppressive load. Scleroderma, you need to behave yourself!! Anyway, I need to lay off the knitting, so I dived into my endless stash of “projects that I want to make someday” and…. pulled out a really cute art quilt!

This is the first block of an eight block quilt. 

This is the first block to build “Calling Me Home” by McKenna Ryan. The picture is built by tracing the pattern onto little bits of fabric that are then fused together. No sewing at all until I get the whole quite top put together. I’m thinking that my shoulder can handle this…

All of these little bits of fabric, to be exact.

It’s like an adventure! All I have to do is figure out which little fabric bit goes with what. Luckily I have that picture to help guide me. This is going to be a little like building a jigsaw puzzle! I hope that Hannah behaves herself while I’m cutting all of the pieces out.

Have a great week, everyone!!

Read a little, knit a little, and garden like your heart can’t live without it.

Surviving the Stay-At-Home Order

So, here we are.

I don’t know how everyone else is doing, but here in my state we were just ordered to stay at home for another 2 weeks. At first they asked us nicely to socially isolate as much as possible; now the gloves are off as things are getting more serious. Many other nations have been dealing with lockdown situations for longer than we have, and for some of you this situation may be coming. Times are really getting tough: an invisible enemy, stress, grief, loss, and economic uncertainty. And now you need to stay home for who knows how long…

Well, as it turns out I have some experience with staying home in self-isolation. I’ve been social distancing for months now; it started last summer when I switched to “night shift” to avoid sunlight, and then I doubled down when the flu season started. Hey, sunlight activates my scleroderma, and I was blue-faced and panting for air, so catching the flu was a really terrifying thought. Rats! I contracted the flu anyway, and just as I recovered I became aware of this looming new coronavirus…  I was motivated to self-isolate, which may be or not be the case for you, but I do have some insights and strategies to pass on to anyone who wants them.

Okay, I just want to acknowledge that this is really, really tough. I absolutely understand that my situation, under no circumstances, should be confused with the stress of a pandemic and the coinciding economic repercussions. Still, in case any of this helps, here it is.

  1. Make lists. Lots and lots of lists! It helps so much to bring some internal structure and purpose to your days. I make lists of things to do each day, and more lists of long-term projects that I think I might like to tackle. Make lists of blog post ideas. Add new things that you think of to your lists, and reorganize them as needed. Keep yourself going, and make plans for the days to come. Really, it helps!

    One of the projects that I put on the long-term list is to knit at least one sock from each of these books. There are a lot of technically challenging socks to choose from: should keep me busy, huh!
  2. Structure your time. When I lost my work-day structure I just didn’t know what to do with myself at first; creating a new structure helps with that. Plan a daily walk, watch a set show each night on the television, create blocks of time for specific tasks (like knitting!), do a puzzle or read each day; don’t forget to stick in yoga, meditation, or journaling if they appeal to you. Just don’t spend all day on one thing that will be finished at the end of the day. It actually is better to chunk multiple tasks over several days so you won’t hit a dead zone.

    Right now I am working on this quilt every afternoon for a couple of hours.
  3. Exercise and get sunshine. Unless of course the sunshine will make you sick. I can’t emphasize how calming and peaceful some time outside can be. Even gardening inside helps. Get some exercise! That can be one of your daily blocks of time, even if it is only your physical therapy and the number of steps daily on your Fitbit.

    Spinning is exercise, right? I thought that an hour treadling my wheel was a good idea. My hip begs to differ…
  4. Create zones in your home to keep you moving around. Right now I have transformed the dining room into a quilting area, and there is a reading zone in the living room with a weighted blanket (and my monster orchid) waiting for me. I have a desk with my computer in the room that used to be my office, and I have a knitting area all set up. The trick is to keep moving, and link your movements to your activities. Maybe staying on the couch works for you for awhile, but it is not a good long term plan, people!!
  5. Plan and make nice meals for yourself.  Oh, look! Another list! Does anyone have any yummy recipes that I can cook in a crock pot?
  6. Record your days and your progress. Keep a journal, or maybe just a day planner. Write on the calendar. Try not to write on the walls, but if that makes you happy, go for it!! Sign up for challenges on places like Goodreads or Ravelry. Maybe create your own challenges. Do it!

    Every night I record my knitting progress into my day planner. It’s kind of cheesy, but it helps me keep going. Last night I cast off the sweater and made sure it would fit me: it fits!!! Tonight I will start on the pockets.
  7. Clean and organize stuff. In a world where we don’t have a lot of control over what is happening, it sure helps to create a nice, clean, tidy environment for yourself. Go after the cabinets and clean the closets! Organize the pantry. Arrange your books, or games, or whatever you have cluttering around in your living area. Clean up your music, photos, or the files on your computer. You’ll be so happy that you did. I cleaned the garage last week and I am still riding the wave of good feeling. Next week: the yarn stash!!
  8. Connect with everyone you can. Talk to neighbors from your doorstep. Chat with family and friends online. My book group is working out how to meet virtually next week. Remember to text to check in with people often. Being isolated doesn’t mean that we need to be all alone.
  9. Don’t forget to shower, people!!

So that is my list. A list! I made another list, look at me go! Take any of this that is of use to you, and absolutely ignore the items that aren’t. Feel free to chime in and add any other ideas that you have to cope with being forced into inaction during a time that screams for action.

Be safe, everyone!

The Scleroderma Chronicles: The Blue-Lipped Zebra Goes On Oxygen

This week I finished up the pulmonary tests to see what was up with my lungs. This was pretty darn stressful and the poor BLZ was just besides itself with the trauma of walking into a major hospital to get admitted for outpatient testing.

This is my son’s kitten Jonesy, named after the cat on the Nostromo, the ship that accidently picked up a deadly Alien while answering a distress beacon on a strange planet… Jonesy is utterly fearless, more than capable of facing down a scary monster alien. This Jonesy is also pretty darn fearless. Be brave, face this down, I told myself while petting Jonesy.

I was brave. On the day my state opened up the first drive-through testing station for the Covid virus, I presented myself at outpatient admissions. I wore my fleece gloves the whole time I was there and followed all of the safety guidelines. I combined two appointments into one so I could pick up the equipment for overnight oxygen level monitoring while I was in the building for pulmonary function testing. The hospital was almost deserted and had bottles of hand sanitizer out for use at every stop on my route, so it wasn’t as bad as it could have been. I literally swerved and walked around anyone in my way, especially if they were wearing a face mask…

In case you all need a reminder, my red blood cell count (rbc) is way too high, and I have blue lips and shortness of breath. My internist diagnosed polycythemia, and once I googled it I discovered that I have every single one of the listed symptoms. The purpose of all of this testing is to uncover the underlying condition causing my rbc to rise. So, let’s just jump to the chase. Here are the results:

    • My lungs are scarred and I have interstitial lung disease, a type of restrictive lung disease. I am stable and haven’t gotten worse when compared to the last testing two years ago.
    • It’s not pulmonary hypertension. Yay! That would have been really bad news. My echocardiogram showed that I am at the upper limits of heart wall thickening, and my pulmonary artery is at the upper limit of normal, but I’m hanging in there.
    • My oxygen level fell below 89% for almost an hour while I was sleeping the night I wore the monitoring equipment. Yay!! Houston, we have found a problem!!

Once the results were in the pulmonologist’s office called for a phone appointment and I got the good news. Well, the sort of good news. I have to go on oxygen overnight to keep my levels up, and the hope is that my rbc count will start to drop. If overnight oxygen doesn’t do the trick I may have to go to oxygen 24/7, but I’m not there yet. (The BLZ is happy but a little sad too. The BLZ hates to wear oxygen, and really doesn’t want to wear it when it goes out shopping… ) Seriously, I don’t appear handicapped until I have to walk with a cane and wear oxygen… then there is no hiding it any more.

Thinking about future appointments and the procedure with the hip specialist (I need an injection of steroids into my bad-boy hip), I asked what my risk from Covid was… (The BLZ was trying to not cry) and my pulmonologist told me straight out that she thought I would survive an infection but that I should put off any more trips to medical clinics for a few more weeks so that hospitals could finish gearing up for severe cases. Well, shoot. (The BFZ is now sobbing…)

Then I throttled the BFZ into silence and headed out to buy the last of the supplies that I needed to get through a few weeks alone. I’m immunocompromised; the medications that I take to control my autoimmune diseases have helped me to become stable, but they also make me high risk for any infection. Nothing has changed, I reminded the BLZ, but now the risk of a serious infection is coming at a time when the medical system may be overwhelmed and unable to take care of me. I’ve been using good judgement, social distancing, and hand washing for a few years now; I will continue for myself and everyone else sharing this scary time.

The truck carrying the oxygen equipment was at my house waiting for me when I returned so I am set up. Hopefully the BLZ will now slowly fade away and I will stop panting for air every time I move.

If this was a race to get new test results into my chart, the underlying cause of my polycythemia identified, and remedial measures into action, I have made it. I have a phone appointment with my new rheumatologist next week and I will ask her about the hip procedure and referral to the foot specialist, but I think that in the current unfolding Covid crisis there isn’t any hurry.

As for the inner voice that worries and feels panicky, my inner BFZ voice, I am going to smooth it into submission with the peaceful zen of knitting. Be brave, be brave, I purr to myself. Just like my son’s kitten Jonesy.

You all be safe out there!!