Those Crazy Days of April, 2022

April went by in a blur for me. I entered the month feeling pretty sick. I had just had an appointment with my pulmonologist, and he had referred me to a surgeon for a lung biopsy. Well, not what I was looking for, but necessary to definitively diagnose what type of interstitial lung disease I had. There are several types, and the treatment plans for each are different. The surgery was set for May 2nd, an entire month away. Annoyed, upset by the wait, pretty darn sick, it was hard to wait all through April.

April rose to the challenge. Never was there such an ill-behaved month. For some bizarre reason my Amazon account reset to Spanish. Do you know how hard it is track orders and make changes to your account settings in a language that you don’t read well? Then email in Italian began arriving. Someone tried to hack into my PayPal account, setting off a flurry of new password and security settings on my part. Then my Facebook feed was flooded with pictures of… owls.

Umm… the universe wants me to learn a new language? Maybe the next BioGeek post should be about owls? This is really strange. April was getting off to a rocky start…

I kept struggling with my health as the month advanced, taking care of essential items in preparation for months of difficulty ahead if I was moved into a 6-month course of chemo. I filed my taxes. I took Matao in for his vaccinations. I filed the paperwork to be excused from jury duty. I got the last vaccine I needed (shingles) before the surgery. A neighbor installed the new outdoor lighting that I had purchased before Christmas, and another neighbor will arrange with her grandkids to take care of my lawn. I ordered more oxygen cannulas, and in keeping with April craziness, 4 cases of tubing arrived instead. I made arrangements to be tested for a portable oxygen unit… first appointment is in June. April, you are killing me here! One of the side windows of the car shattered without warning. I continued to get sicker. I was sick and tired of April, too!

My wild bunny moved into the front yard where I could see it each time I went out of the garage. Very used to me now, it never runs away when I go outside. Bunny support on sad days.

As the month wore on, I began to sleep a lot. Like 10-12 hours a night. I had to use oxygen during the daytime. I was losing weight. I wrote my doctors an email towards the middle of the month telling them that I felt that I was declining, and that I was worried it was taking too long to begin treatment. I was scheduled for a series of additional tests and had appointments with the pulmonologist and the cardiologist. Oh, good, April. Now I have to drive all over town without portable oxygen.

How to respond to a worsening situation that I have no control over? I bought happy new shoes in an outrageous color.

These are the shoes that are going to walk me into the hospital!!

And then I cast on new socks that would do the shoes proud.

This yarn’s name is “Squad Goals” and dyed by Hue Loco.

The results from the heart, lung, and kidney testing came back and they all showed that… I was getting worse. My doctors conferenced back and forth, debated different treatment modifications, and came to the consensus opinion that I should have the biopsy done May 2nd. I got that email late on Friday before the surgery. Checking the physician notes attached to my last appointment I found the full text of all the test results and the email chain of discussion between my doctors. I felt very lucky: what a great team! How good I feel that the decisions about drugs and treatment is being arrived at through this interdisciplinary collaboration by my doctors, who have chosen to make this all visible to me. Knowing that, however, did not change the fact that the upcoming surgery is now a little risky.

So, I cleaned the house, did laundry, paid the bills, and got in groceries. I planted flowers in the front tubs and along the walk. I also cast on some baby booties for my next-door neighbor who just brought home a new little one. This pattern, extremely versatile, is our family recipe passed down through three generations over almost three quarters of a century. It is the same as this one posted on Ravelry.

And just like that, the month was done. I had made 2 hats, 4 PICC line covers, 2 pairs of socks, and used up 3.38 skeins of yarn. I read 6 books. I did work on a sweater, but since I’m now in the colorwork portion of the knitting I put it on hiatus to await better days.

Goodbye April, you crazy, bad-boy of a month! I want you to stand in the corner and think about what you have done!! I expect a whole new attitude when I see you next year.

The Scleroderma Chronicles: It’s okay if you cry…

My fatigue lately has been off the charts. I struggle to get the simplest of tasks done, and to be honest I just don’t feel like getting out of bed for days on end. I have been slowly, slowly sewing on a quilt top over the last few weeks. It is soooo exhausting to pin two fabrics together, guide the fabric through the sewing machine, and then to stand up to iron the seam. I handled all of this by 1) sewing only for an hour a day, and 2) lowering the ironing board so I didn’t have to stand while ironing. Take that, you nasty, exhausting fatigue!!

The quilt has a panel in the middle with really cute pictures like this one. Super cute, right?
Here’s the finished quilt top. This baby has really simple quilting in one border and then simple, simple borders around the central panel. Yeah. This took over two weeks to complete. Thanks, fatigue.

When I noticed some strange terminology on my last heart imaging test report, I contacted my pulmonologist about it, and he ordered a CT scan of my lungs. (You can read about that adventure here.) I knew that something was up when I got a call from his office telling me the date and time of the earliest possible appointment with this doctor. The nurse had intervened and made the appointment for me ahead of time. Then there was a call from the cardiologist’s nurse that was the same; an echocardiogram and appointment with that doctor had also been scheduled for me in order to secure the earliest possible appointment. Kind of the harbinger of a tough appointment, right?

Today I had a pulmonary function test and met with the pulmonologist soon afterwards. We joked about the horrible year we had both had. (He is a pulmonary critical care specialist who has been on the front lines of Covid care for two years now; for me lockdown never ended and the BLZ was running wild.) We laughed at my summation of the year: Crushed by Covid. We decided that “Crushed by Covid” could be the name of a really sad band. Then he whipped out his laptop and had me move over to look at it with him.

He had prepared for my appointment with a spreadsheet of my lung function tests over time and my latest lung scan along with that of a normal person. The spreadsheet showed that I was losing volume in my lungs. The scan of a normal lung was really interesting (old biology teacher here…) and then we looked at mine.

Um… my lungs were really cloudy. Like frosted glass. Like… “Hey. Is that what they call ground glass lung?” I asked.

“Yes. That is exactly what we’re looking at. That’s why I wanted you to come in. This isn’t the type of conversation that you have over the phone,” he replied.

Ground glass is not good, folks. Ground glass is the type of lung imaging that Covid patients with pneumonia have. Covid presents like systemic sclerosis because there is an extreme immune response going on in the lungs; both are aggressively treated with drugs that target the immune system. I also have some honeycombing that is the beginning of fibrosis; first the inflammation (which creates the ground glass appearance), then the fibrosis follows. My ground glass lungs are, in his opinion, absolutely not Covid. It is not likely to be just pulmonary edema. It’s systemic sclerosis at its worst. This is interstitial lung disease.

So, it is not good. On the other hand, this is good. I’m in trouble, but the problem has been identified early on and that means aggressive treatment now may stave off the worst of the fibrosis. I am so glad (and lucky) that I googled those crazy medical terms and then followed through with an email to my pulmonologist.

I’ve been referred to a surgeon for a lung biopsy procedure. Evidently that will land me in the hospital for a few days. Following that, if things go to plan, I will be started on more aggressive immunosuppressive drugs. He is going to talk to my rheumatologist about starting a course of chemo and an anti-fibrotic drug. One of the last things that my pulmonologist said to me was, “It’s okay to cry about this, but we have a plan.” That’s when it hit me that this might be really bad; lockdown will continue, and the fatigue is probably going to get worse. Oxygen 24/7 is right around the corner.

Crushed by Covid plays on. What a sad little band it is.

Luckily for me and the cats it is squirrel season. They will have lots of entertainment while I’m in the hospital and laying around like a slug.

The Scleroderma Chronicles: The shunt hunt takes a left turn…

I’ve been continuing my adventures in cardiology over the last several weeks. If you have been following along on my scleroderma adventures you know that I had a trip to the Cath lab that led to the discovery of a cardiac shunt: a hole in my heart. I also was eventually diagnosed with exercise-induced pulmonary hypertension and started on drugs to treat it, which is a lengthy process as I was slowly titered up on two different drugs while monitoring for side effects. I’ve been mostly living in bed for the last 6 weeks except for short trips out for more testing and blood work. The cats have been loving this, by the way. I’m kind of their captive right now.

This is edema in my arm. I’ve been dealing with headaches, muscle pain, edema, low blood pressure, and extreme fatigue. I cough a lot. Every new weather system is a nightmare. Ugh!

While the whole process has been pretty difficult, I am breathing much, much better and that blue lipped thing has mostly faded away. No more panting!! I haven’t had to put my head down because I felt faint for weeks. This is huge, people!!

I may have to retire the whole BLZ logo the way things are going!

My cardiologist is still hunting for the shunt that was detected in the Cath lab. I have one that they can see (a patent foramen ovale, which is pretty common), but for the really significant disruption of circulation that was detected in the Cath lab the feeling is that I have something much bigger somewhere. I’ve gone through 3 rounds of testing looking for the dang thing, and so far, no joy.

When the test results come in, I always read the entire text and google terms that I don’t recognize. The last imaging of my heart did not find the shunt, and my cardiologist sent an email letting me know that my heart looked pretty good. Umm… okay, but where is that infernal shunt?!!! This is getting a little frustrating, but I am doing better, so I guess I should just roll with it. I did notice this little sentence in the report about the portion of my lungs seen in the heart imaging: “There is mild subpleural reticulation and bibasilar atelectasis.” Say what? I googled and …. bibasilar atelectasis is a partially collapsed lung. I shot off a little email to my pulmonologist to ask if this was something new.

This is why I decided to write this post. As it turns out, this is new. Both of the things that were noted in that test result were significant (subpleural reticulation is evidence of scarring in my lung), and I was immediately sent to get a specialized lung scan that shows I have sustained moderate advancing lung damage over the last 10 months. Oh. No wonder I’m so exhausted. At least they didn’t use the word “severe” in the report. I seem to have developed pulmonary edema and my lungs took a big hit during the last few months; scleroderma is now attacking my lungs. If I hadn’t read that report and then contacted my doctor, no one would have picked up on this. The BLZ may be on hiatus, but the lessons she learned during that drive for the pulmonary hypertension diagnosis really paid off now.

What do you do when you get a sad little lung report? Why, you put on your Catzilla shirt and go start a load of laundry, of course!!

Tuesday I go for a pulmonary function test and then immediately afterwards I will meet with my pulmonologist. I’m kind of thinking that there might be more drugs in my future. Anyway, there is a lesson here that I decided I should share with you all.

Be proactive! Read your test results and ask questions of your doctors. Google is your friend, and those online portals that let you shoot your doctor an email are priceless! Use them!

And now readers, back to the shunt hunt…

Mateo: and now readers, back to my nap! After that I’m going to go swat some more helicopters!

The Scleroderma Chronicles: Rare Disease Day, 2022

Well, this is a hard topic to write about. Quite frankly, I have been getting my butt kicked lately by my (wait for it) rare diseases. Still, I am trying to respond to the calls for publicity about rare diseases along with other members of the scleroderma and pulmonary hypertension communities.

You know, I feel like I should represent.

People with rare diseases are referred to as “zebras” in the medical community.

So, what’s a rare disease? A rare disease is classified as one that impacts a small percentage of the total population. Here in the United States that means fewer than 200,000 people diagnosed with the condition/disease. Perversely, there are a lot of people with rare diseases as there are almost 7,000 different rare diseases!  Some of these diseases are common enough that you may be familiar with them: albinism, achondroplasia (a type of dwarfism), and autoimmune hepatitis are examples. Others are very rare. Most are genetic in origin, and half of them impact children. More than 90% of rare conditions have no drug treatment.

The type of scleroderma that I have, limited systemic sclerosis, is considered rare as there are about 100,000 people in the US with this diagnosis. The latest diagnosis added to my medical history is of pulmonary arterial hypertension, another rare disease, and one that is a consequence of my scleroderma. Well, I am really rare now! I have struggled to explain my scleroderma to people when they ask; how can I explain in just a few sentences something that is just frankly causing horrific damage to my body and generating an ever-growing list of diagnosed conditions. Here’s my best answer at the moment:

Scleroderma is a chronic, progressive, uncurable, and often fatal autoimmune disease that causes scaring and damage to blood vessels, skin, internal organs, and muscles/joints. It is controlled and treated through the use of immunosuppressants and drugs that address symptoms. It is a life-altering diagnosis. It is my life.

So, I have blogged about Rare Disease Day several times in the past. Here’s what I wrote a couple of years ago, and what I wrote in 2018. In the past I have written about my symptoms and the struggle of living with a rare disease. It is pretty isolating. It is hard to get diagnosed and treated. I have also written about the difficulties to get funding for research for rare diseases and conditions, and the lack of treatments and cures because the patient population is so small.

This year I thought I would share some of the things that doctors have said to me since my scleroderma diagnosis. I’ve tried to organize these into chronological order to better reflect my journey.

  • My internist: It’s good to have a diagnosis, even if it is a shame.
  • My rheumatologist when I asked him what my life would be like in 5 years: Let me run some more tests, and then we can talk.
  • My ophthalmologist: Do you have a will?
  • The physician at the regional acute diagnostic center: This is a diagnosis like cancer. Of course, some cancers can be cured.
  • A physician speaker at a scleroderma support group presentation: this drug [the immunosuppressant that I take] can really give you a chance, as long as you don’t contract an infection.
  • My old rheumatologist: All you do is complain. Maybe I should order a sleep apnea test or prescribe antidepressants.
  • My internist, as I begged for an anti-inflammatory drug: I’m sorry. There isn’t anything that I can give you that won’t hurt your kidneys.
  • My dermatologist, as she prescribed an anti-inflammatory topical gel: This is unacceptable, and I am putting a stop to it now.
  • My new rheumatologist as she orders more testing on my painful joints: Why has no one followed up on this?
  • My rheumatologist two days later: You need to get a steroid injection in your hip joint as soon as possible.
  • The hip specialist: There is nothing more that I can do for you because your scleroderma is attacking all of your tendons and ligaments. You need a hip replacement, but it will fail.
  • The physician at urgent care: I don’t think they understood how complicated your medical status is when you were referred here. You need to be hospitalized because we can’t do the testing that you need here.
  • My pulmonologist as he walked me back to the waiting room: I really admire your attitude.
  • My cardiologist as I was being sedated for a right heart cath: Don’t worry. I’ll take good care of you.
  • My cardiologist as he started me on medication for pulmonary arterial hypertension: This is challenging, and we will need to be comfortable with “out of the box” thinking.
  • My rheumatologist last week, referring to herself, the cardiologist and the pulmonologist: We are your team!
  • My rheumatologist, also last week: We need to add a gastroenterologist to the team.

You can see how rocky the start was. There is a lesson here, I think. To be rare, to be a zebra in a medical community that is designed to identify the most likely cause of symptoms in a herd of horses, is hard. It is really challenging to secure the care that you need when, no matter how hard doctors try, you do not respond to the usual treatments, and you never fit the usual profile. It is easy to be seen as a problem. It is hard to keep insisting that there is something wrong when all the test results say you are okay.

Even when you are blue-lipped and panting it can be hard to convince doctors that there is a problem.

And yet, it is possible to get there. Over time, with great determination and persistence, I have A TEAM of doctors who view themselves as active collaborators in my care. They message each other to discuss test results and possible drug interactions, and they loop me into their discussions. It is only now, newly diagnosed with a terminal condition, that I feel confident and hopeful about my care.

Today I went in for a blood draw and a little jaunt through the local bookstore. The sun was shining, I bought a Starbucks coffee, and it was a good day.

****************************************

My scleroderma-related diagnoses:

  • GI tract: difficulty swallowing, hiatal hernia, GERD, gastroparesis, chronic gastritis.
  • Kidney: stage 3 chronic kidney disease.
  • Lungs: pulmonary arterial hypertension, interstitial lung disease, asthma, partial lung collapse.
  • Heart and circulatory system: grade 2 diastolic dysfunction (a type of heart failure), Raynaud’s phenomenon, telangiectasia.
  • Muscle/Skeletal: fibromyalgia and severe joint damage.

This is scleroderma. I’m a zebra, and these are my stripes.

Happy Rare Disease Day, everyone!

The colors associated with my diseases are teal (scleroderma), purple (Sjogren’s), and periwinkle (PAH). It sounds like the start of a great sweater, huh. ūüôā

The Scleroderma Chronicles: Matters of the Heart

The Blue-Lipped Zebra (BLZ for short) has been busy the last three months (once she was fully vaccinated for Covid-19) and lots of testing and doctor appointments have happened. Lots of diagnostic hypothesis have been pursued and tested; the BLZ has received several emails from doctors that let her know about good news: you don’t have pneumonia!! your heart looks good!! your kidneys are maintaining!!

All is good. Go visit your baby bunny and don’t worry about it…

Hello. BLUE-LIPPED Zebra. Chest hurts. Zebra is dizzy. Zebra pants as soon as she moves around. Zebra is absolutely sure that everything is not fine at all!

Did I mention that the BLZ got fully vaccinated? Ever since that happened (and the BLZ got a steroid injection for her ill-behaved hip) her fatigue and brain fog have receded into the background. Quite frankly, the BLZ is feeling pretty frisky and clear-headed these day between bouts of dizziness and panting episodes. She has decided that enough is enough and she is on the move to get to the bottom of what is going on!

She contacted her doctors and health providers and had them forward her the entire text of her test results. She read these results carefully and then spent some time consulting with Dr. Google to figure out what some of these words meant. The BLZ is so grateful for that biology degree and years of related job experiences.

The BLZ has limited systemic sclerosis. What she learned was…

  • A general rule of thumb, the 15% Rule, can be used to describe the number of systemic sclerosis patients with serious complications associated with their illness. For example, 15% of patients will have Sjogren’s Disease, or digital ulcers, or lung disease, or maybe pulmonary arterial hypertension. These complications are sometimes rare in the general public, but for systemic sclerosis patients they can be common.
  • A large European study found that the majority of systemic sclerosis related deaths were from heart complications (26%) or were pulmonary arterial hypertension (26%) related.
Time to stop and smell the roses. This is a little disappointing… my doctors have been reassuring me that all is fine because they are focused on lung disease. There are a lot of bread crumbs in the test results that suggest heart problems.

Then then BLZ made an appointment with her internist (the primary care physician) to go over the test results with her and to help her prep for her cardiologist appointment next week. Don’t you think that was smart?!!!

Mateo: Very smart!!

Here’s the summary of my appointment with my wonderful internist. My face was blue and I struggled with dizziness in her office: she entered a new diagnosis into my chart that says I’m cyanotic and told me to press the cardiologist for a prescription for day time oxygen so I can carry portable oxygen with me. (“Now we’re talking!!!” barked the BLZ.) She read the test results for my CT lung scan and echocardiogram and agreed with my understanding of what the test results were saying. She told me what tests to ask for from the cardiologist at my appointment. Here’s the summary:

  • I have physical findings in my lungs that consistent with pulmonary arterial hypertension. The summary results of that test say “mild to moderate” and even say that the loss of lung tissue and an enlarged pulmonary artery are due to PAH. Huh. Look at that. (“I’m just shocked, shocked!” snarks the BLZ).
  • The tissue of the heart (the muscle) is scarred and too stiff to beat well. This condition is called diastolic dysfunction and is a type of heart failure. The echocardiogram states that my diastolic dysfunction is Grade II, which is moderate. Scleroderma is attacking my heart; 15% of systemic sclerosis patients have diastolic disfunction. While there are lots of reasons why people develop diastolic dysfunction, for me the picture is different as it is a common complication of my systemic sclerosis and not a result of say… uncontrolled high blood pressure.
  • The estimated pulmonary pressure from the echocardiogram is difficult to measure in my case (Dr. Google had to teach me about incomplete TR jet and other obscure heart-related terms) and is most likely being undermeasured. The number now is the upper limit of normal; twice in the past it couldn’t even be estimated.
  • I have a newly developed hole in my heart called a cardiac shunt.
  • My heart is broken damaged by scleroderma.

So what should the BLZ do about all of this? The internist and the BLZ hatched a plan in which she should insist request direct measurement of the pressure in the right side of her heart (right heart catherization) and another echocardiogram that looks at that cardiac shunt while she is exercising standing up. Like, maybe the BLZ needs to be climbing stairs… The BLZ is just thrilled… Also, the BLZ wants day time oxygen-to-go. Yes, please. Right now, please.

Also, the BLZ is considering taking someone with her to the appointment and will have the cardiologist send the internist his notes following the appointment.

Also, when life get tough, get a kitten!! BLZs love kittens!

So, this is an adventure in progress, but I do have some gems to share with others struggling with their own medical misadventures. Get your own copies of your test results and physician notes after appointments. Google like crazy to learn what the obscure medical terms mean. Educate yourself about your illness/condition. Stay off social media as you do this and read journal articles from legitimate sources like the Rheumatic Disease journals and articles posted by the NIH. Ask another knowledgeable person to review your test results to help clarify/validate your thinking. If my journey here can serve as a roadmap for even one other person battling their way to a diagnosis, then this post was a success.

And remember to be brave.

It is good to have a diagnosis, even if it is a shame.

Zebras are brave!!

MacKenzie Speaks: Crutches, Mountains, and Squirrels

Hi. I’m MacKenzie.

Do you see this ball of yarn? This is my most favorite yarn at the moment. So soft. So nice to cuddle with. The little flecks of color are my favorite.

The Mother of Cats has been somewhat difficult to work with lately. She has been limping all around the house, and yesterday her physical therapist finally told her she needs to use crutches for the next few weeks. HER CRUTCHES KEEP GETTING IN MY WAY!!! What is up with that? She doesn’t go outside as much as she used to, and she started knitting this simple sweater with just one color of yarn instead of the Koivua sweater that I just love.¬† There are more colors to chomp in the Koivua, and it is now so big she can’t keep me off of it while she knits. Is she working on the sweater that I can sleep on while she works? No! She is not!! She says knitting with the one color is easier to stuff into a bag to haul around with crutches. Whatever. What is up with the Mother of Cats? A little adversity and she totally folds: I’m so disappointed with her!

Except I do really like this new yarn so I’m starting to forgive her for putting the Koivua into time out. This sweater is called “Climb Every Mountain.”¬† What is a mountain?

Today the Mother of Cats finally let me go outside while she took pictures of all of her knitting and we watered all of the outside flowers.

Look at what happened to the blooms on the hydrangea plant over the last few days of cool weather. The flowers have turned pink again! The Mother of Cats was really happy to see the flowers. I was like… meh… laters‚Ķ I have to go have a chat with that squirrel…

While I was chasing the squirrel around the yard she took these pictures of the new sweater. It is starting to look pretty good, isn’t it? It is kind of nice to sleep on…

She is now below the sleeves and getting ready to use her last ball of yarn.

Oh, yeah. Look at the new stitch markers that she made to use while making this sweater. How cute are these? She should use these to play with me!!

Now it is late in the afternoon, the squirrel has left to do whatever squirrels do, and the Mother of Cats, the crutches,¬† and I are back in the house. I just got some yummy salmon to eat and the Mother of Cats is getting ready to knit again. Hmm… that new sweater is getting pretty big now. Maybe I can sleep on part of it while she is working. I’m so handsome and the yarn really looks nice next to my fur. Maybe she will be reasonable for once and will let me do that. IF she loves me I will get a quality nap on yummy yarn.

I’m such a good boy.

Can I have some cookies now?

>^..^<

Notes from the Mother of Cats:

  • The sweater is Climb Every Mountain by Heidi Kirrmaier. That yarn that MacKenzie loves is Tweed DK by Western Sky Knits, and it really is nice to work with. My Ravelry notes are here.
  • How ironic is it that I’m knitting a sweater called “Climb Every Mountain” while trying to master walking with one crutch?
  • The hip has become even more challenging. I met with the physical therapist yesterday who had printed out the notes from the hip specialist that I met with last month. Things are worse than I realized. I have IT band syndrome, inflamed tendons associated with my hip adductors, and then there is the severe hip bursitis.¬† I need to rest the tendons as much as possible as I stretch the IT band and use the topical anti-inflammatory gel that we hope will get the swelling down. Since I can’t walk without limping and the adductors need a rest I’m on crutches for the next three weeks at a minimum. I’m truly a mess as I can’t use a cane (bad wrists), ice (thank you, Raynaud’s), or work in a pool (Sjogren’s, I really hate you right now), so things are challenging, but I’m really motivated since I want to stay mobile as long as possible and I’m a poor candidate for a hip replacement.
  • MacKenzie is learning to stay out of the way of the crutches, but he is taking the whole thing personally. Poor thing. I have started giving him more cookies… I have NOT surrendered any of the sweaters to him.
  • Koivua¬† is waiting for the first snow fall as I rush to finish two transitional sweaters. Winter, take your time…

    Napping MacKenzie_web
    Finally!!

Knitting Speed Bump

A few weeks ago I went to see my primary care physician for help with shortness of breath and joint pain. I totally scored! I walked out of there with a tetanus shot (fail), inhaled steroids to control my small airway disease (win) and an anti-inflammatory gel to put on my swollen, painful joints (huge win). What a difference to my life these new medications have made. I can breath! I can walk without pain! I can sleep through the night! This is huge, people. Take that Sjogren’s and scleroderma! Feeling so much better I began to spend more hours up and about, and there was a lot more knitting happening too.

I got some socks finished right up. Here are my project notes.

I made a lot of progress on my Suburban wrap and was lured into knitting more than usual because I just had to get into the next section of the wrap. This is such a fun project with lots of color, texture and lace interest. So addictive. So hard to walk away from…

Oops. Then this happened.

Tendonitis!

One of the problems of getting better is that swelling is going down and the tissue is tightening up on some of my joints. My knees are really tight. My wrists are stiff. When I knit my joints loosen, so I thought I was helping them stay flexible. That is probably true to a certain point, but I guess I now need to be careful to not overdo things. Sigh. I am using the tendons in my left hand the most since I knit continental, and I push the yarn over the needle with my middle finger for each purl stitch. I’m really fast that way, but my tendons have totally rebelled in my left hand and wrist.¬† To make things worse I can’t take NSAIDS or any other anti-inflammatory drug because of my scleroderma-battered kidneys and stomach. Sigh. My doctor ordered a knitting hiatus.

I want to be a compliant patient, really I do. I read a couple of books, managed to get through a couple of days without knitting, and then I snapped. I must knit!! Want knit now!! Knit, knit, knit. Why go on if I can’t knit? Sniff. Obviously this is totally unacceptable and I am going to figure out how to knit in spite of this bad boy wrist. Really, my left hand is the one having trouble, so maybe I can work around that. I tried to tension the yarn in lots of creative ways before I remembered that lots of people on the planet hold the yarn in their right hands. English knitting. I have never mastered purling English style, but now I’m really motivated!

I was in the last striped section of the wrap when disaster struck; stockinette means I have to purl back every other row. 

I managed to continental knit with the brace on (okay, it is a little loose) and am moving my left hand very little by working slowly and relying on my right hand.

Working very slowly I am also managing to purl back English style. So slow. So awkward. So much safer for babying rebellious tentons.

Last night I finished the stripes and am ready to enter the last section of garter eyelet. Yay. Knit all the way! I can do this! Then it is into the last, ribbed section of the wrap.

Ribbing. That is going to be slow going. I think that I will check out Norwegian purling. Somehow I need to do this without moving my fingers too much. Yay. A new stitch to learn.

I’m on it!

Take that, scleroderma. You are messing with the wrong knitter!!

The Scleroderma Chronicles: Safe House

You know, I kind of view myself as a happy camper. I have more things (ahem… knitting projects) going then I can get finished on any given day or week, books lined up to read, and a “to-do” list that I’m slowly working my way through. Hey, people, I fixed the loose tiles on my kitchen floor last week!! My cat MacKenzie is my constant sidekick throughout the day as I knit, work in the garden, read in bed, and even with me (underfoot, demanding cookies) while I’m cooking. Even on the bad days when I’m pretty much down for the count, I manage small victories. There is just one problem with this picture.

Look at these beautiful coneflowers I just added to the garden. I’m hoping to lure some butterflies into the yard. Every garden should have butterflies, don’t you think?

I just don’t fit in the world all that well anymore. In my home, living the life that I’ve created for myself, it is really easy to forget how much I have adapted to accommodate the limitations of my scleroderma, Sjogren’s, and fibromyalgia. Once I go anywhere else reality hits me hard. Every trip out of my house is going to come at a cost. Here are the worst of the offenders that will lay me low.

Air Conditioning I know that almost everyone in the world is grateful for air conditioning in the summertime, but for me it is a royal nightmare. The shock of walking into a refrigerated building on a hot summer day will trigger an immediate Raynaud’s attack. I pull on long sleeves and fingerless mitts as soon as I get into the building, but my lungs know what’s up and I have trouble breathing. The airflow makes my eyes burn; I’ve been reduced to wearing my sunglasses indoors to protect my eyes. Don’t even get me started on the refrigerated cases churning out cold air; you haven’t lived until you’ve had to pull up the hood of your sweatshirt and the sleeves down over your hands so you can score some butter and eggs.¬† If that wasn’t enough, there are also usually…

Scented Products Almost all buildings use scented cleaning products and sells additional items with scents. Candles. Lotions. Laundry soap. The scented bathrooms are a nightmare. If I’m not already in trouble with my breathing I will be if I have to walk down the laundry detergent aisle at the grocery store: I also start to itch and my face swells. Why do these chemicals even exist? They can’t be good for anyone!

Restaurants These are a special kind of hell for me. All the drinks come cold and with ice, and the entrees are served piping hot. There are other landmines that I need to avoid: salt, lactose, fiber.¬† I have to carefully select something that is very soft and that will behave itself in my gastroparesis stomach. I can’t have spicy food. I can’t eat fresh veggies. Actually, to be safe, some of this food should go through a blender… I actually once soaked a cut up sandwich in soup so I could eat it…

Walking I am trying really hard to meet my walking goals every day, but I stretch those steps out over the day. A trip to run errands can be just exhausting if I’m on my feet for a couple of hours at a time. I need to always carry water, be aware of the location of bathrooms, and have places where I can sit down if I need to.

Sunshine It makes me sick! Enough said.

Ready for another picture? These paper wasps are building a nest right on the edge of my deck! MacKenzie and I are not amused. Still, it kind of shows how the outside world is full of dangers…

Recently I had a tough talk with myself about pruning down my outings and being more strategic about how I expend my energy. I need fewer outings, and my destinations need to be closer to home. I need to live online. I need to in a safe environment as much as I can to manage my diseases.

My home is my safe house. I have no air conditioning and I minimize air flow. I keep the temperature in the mid 70’s in the day so that my joints and lungs will be happy. I cook all of my own food, I don’t own any salt at all, and everything that I drink is room temperature. Fruits and veggies go through the blender to become smoothies. Every product that comes into the house is scent free. I’m always close to a bathroom or a soft surface to crash onto for a quick recovery if I get dizzy. My stairs have wrought iron rails that I use effectively on bad joint days. Flourishing in my safe house I sometimes forget how sick I am because, well, I have fewer problems.

I planted this yarrow last year in a flowerbed that has killed almost everything planted into it. Not this yarrow!! The secret of gardening, and living with serious chronic illness, is to keep on trying new things, and to match your needs to your environment. Or, in my case, make your environment match your needs.

Tomorrow I have a doctor’s appointment so I listed up some symptoms and issues that I need to ask her about. It is quite a list now that I look at it, and it kind of underscores how chronic illness can trick you into thinking that things that would normally send you screaming into urgent care are “just another day of scleroderma.” Shortness of breath is an almost daily thing. When I glance into the mirror these days I sometimes notice that my face is blue. One hip keeps failing me; okay, I actually have to lift that leg to get into the car. My joints swell so much that I can’t sleep at night.

But I am good, here in my little safe house with my gardens and cat.

Tomorrow my doctor and I will attack some of these scleroderma/Sjogren’s issues. I kind of think that lung testing and a MRI of my hip are in the future, and that there may be follow-up with my pulmonologist. I’ve been gathering up my energy in preparation for these outings into a world that is dangerous for me, knowing that after each outing my garden swing, knitting and latest book will be waiting for me. With a room temperature ice tea.

And a cat!

It is good to have a safe house.

The Scleroderma Chronicles: World Scleroderma Day

Today is World Scleroderma Day. Tonight the Niagara Falls will be illuminated blue and white in recognition of the day. All this month (June) there have been walks and outreach to raise awareness of this disease and to raise money for scleroderma research, and scleroderma patients all over the world have been urged to post pictures of their faces (and smiles) to made this disease more real and relatable.

Rather than flash my smile I have chosen to show this picture of me and MacKenzie, wrapped in handknits, fighting my way through a flare one frozen January. Good times! See the light in the background? It reminds me that every flare comes to an end.

The purpose of this day is to try to raise awareness of an illness that is mostly invisible and relatively unknown.

So what is scleroderma? It is a rare autoimmune disease that is a member of the rheumatic diseases family. The name “scleroderma” means hard (sclero) skin (derma), which is the most visible symptom. Scleroderma is the general name that is applied to a group of connective tissue diseases that may target only the skin, or the skin plus internal organs, or just the internal organs themselves. The words that are used to describe my type of scleroderma (limited systemic sclerosis) are rare, chronic, progressive, disabling, and possibly life threatening. This is a lot to wrap one’s head around, so I’ve decided to break it down.

Rare: Here in the United States the CDC has defined a disease with fewer than 200,000 patients as “rare”. Systemic sclerosis, with about 1-2 diagnosed people for every 100,000 citizens fits the bill. To be rare means your illness struggles for research and treatment funding as the patient population is small and almost no one is personally impacted by the disease in the general population. Hence, Scleroderma Awareness Month and World Scleroderma Day. Thank you, Niagara Falls!!

Auto-Immune Disease: The immune system, designed to protect us from foreign invaders like pathogens,¬† is attacking some of the components of normal cells in scleroderma patients. The exact components can be identified using the antibodies of scleroderma patients and they are essential proteins found in the nucleus of the cell. My antibodies are attacking a protein found at the kinetochore of dividing chromosomes. I can’t help but wonder what the heck has happened here that I ended up with antibodies like these? I also eventually wondered if my cancer risk is increased since there is something off with this critical structure used in cell division (it is).

Connective Tissue: I used to try to explain this type of tissue to my AP Biology students. Connective tissue… well… it connects. It is what holds your bones together to form the skeleton. It gives support and stretchiness to your skin. It is involved in all the organs of your body, and holds the organs in systems together. It is essential to organize your muscles. In systemic sclerosis, all of this tissue can be involved in an inflammatory attack of the immune system and scarring occurs. Skin gets thick; hands curl, joints won’t bend, smiles¬† twist and faces harden. Muscles and joints hurt. The smooth muscle in the digestive tract can be so damaged that organs don’t work right. Blood vessels are damaged and spasm, cutting off circulation to extremities and organs. The heart can get scarred. Scarred lungs prevent oxygen passage. Scleroderma, invisible to the outside viewer, can be devastating to the patient.

Vascular tissue damage causes circulation loss to my fingers and toes (Raynaud’s Phenomenon) which I fight by wearing fingerless mitts and wool socks. Works great and the Zen of knitting brings its own benefits.

Chronic: There is no cure, and it never ends. There are, however, treatments for individual impacted organs that are really helpful.

Progessive: Damage accumulates over time and conditions worsen. My doctors are monitoring my kidneys, heart, lungs and GI system. So far I am doing pretty well, but my GI tract is taking the most damage.

Disabling: Yep. It is getting really hard to walk, and I just don’t fit all that well into the world anymore since my diet is very restrictive, air conditioning isn’t my friend, and sunshine makes me go into a flare. Right now I don’t need supplemental oxygen, but if that comes back it will further restrict my independence.

Life-threatening: It took me months to think this one through, but eventually I did google “life expectancy of systemic sclerosis” and discovered that for me, with my treatment plan and risk factors, survival rate is about 75% for 10 years after diagnosis. Overall the death rate of scleroderma is 50%. Oh. Now I know. Who wants to live forever?

This week I wrote a letter to my congressman asking him to sponsor a bill that would fund research into fibrotic diseases like scleroderma and cystic fibrosis. I haven’t heard back, but maybe if he has heard of one of these diseases or knows someone impacted by scleroderma he will do it.

That’s why we do World Scleroderma Day.

The Scleroderma Chronicles: The Ankle Adventure

The last few weeks have been hard : rain, snow, an exceedingly badly behaved ankle, and all the fun of never-ending chronic complaints. Ugh! The weather fronts just kept rolling in, sending the air pressure swinging wildly and my breathing and joints into their own little crises. “Will it never end,” I asked my ankle and MacKenzie? “Nope!” said the ankle with a little sneer in its rotten little ankle voice. “Don’t count on it,” said MacKenzie as he squirmed deeper into the current knitting project with one paw extended, claw flashing, reaching for my yarn.

As you can guess, I’ve developed a nasty mood of my own.

There was a nice week with sunshine that made me and the ankle feel better: I got some yard work done and planted flowers. Then the weather turned on me and once again I was in bed, listening to audiobooks and knitting with my ankle propped up.

In the wee hours of Tuesday morning I woke up to the crack of breaking branches. Six inches of heavy spring snow (the latest storm this bad in 17 years in the Denver area) had done my tree in. No wonder my joints are cranky… even the tree gave up! I took this picture the next day after most of the snow had melted.

What is up with this ankle, you ask? Good question! Three weeks ago it suddenly developed a hard, red, and hot lump that radiated pain ruthlessly. The infection began spreading under my rhino-hide scleroderma skin. I was started on antibiotics, but the pain continued, the redness continued spreading around the outside of my ankle, and a dent in my leg appeared where the redness was. I chatted with a doctor online and she decided that I should be checked for cellulitis. Cellulitis?! Off to urgent care I went, and as soon as that doctor saw my ankle he ordered testing: another blood clot hunt and  x-rays to see if the bone was damaged. Limping and in pain, I headed off to get the testing done. As I drove home from the ultrasound a call came in with the results of the testing: a benign tumor was found, and I was being referred to a dermatologist for treatment.

It’s like they forgot about the cellulitis! “Where are my new antibiotics?” I asked MacKenzie when I got home.¬† MacKenzie just carried on for attention and cookies, so I made the appointment for the dermatologist to look at my ankle before returning to my knitting and propping the ankle back up. I made a little cage out of a box to put in my bed so I could sleep without the covers touching the ankle… “I have a tumor,” said the ankle in its nasty little voice.

The next day the redness and heat were a little better, but the pain and lump on my ankle remained. I resolved to head back to urgent care if I got worse, and carried on with my knitting. Tumor, whatever. I have scleroderma and I was betting that was what made the lump appear unusual in the ultrasound. I was pretty sure this was just another chapter in the scleroderma adventure.

Two days later I got in to see the dermatologist. Wow. Just wow. I am in love with this doctor. This is the doctor that I have been waiting for since my first diagnosis FIVE YEARS AGO!

Aside: I have been battling eczema for months. All of my doctors have seen my eczema face. Since I have noticed that a dose of ibuprofen (which I’m not supposed to take because of my iffy kidneys…) will give me 24-48 hours of happy joints with no fatigue or brain fog symptoms, and some eczema relief,¬† I have literally begged for some type of anti-inflammatory drug to help me. Even though I am taking drugs that are crushing my immune system unto submission, there is something else that I need. I have cried in my internist’s office. Evidently there isn’t anything that can be given to me that won’t hurt my kidneys…

This dermatologist entered the office, took one look at my swollen, red and itchy face, and said that this was unacceptable and she was going to put a stop to it. Then she said that she had looked at the ultrasound and that there wasn’t anything that worried her there: scleroderma had caused fibrotic tissue to form. Then she wondered out loud why they had forgotten that I had cellulitis? Then she prescribed antibiotics that are also anti-inflammatory along with an anti-inflammatory cream to get my eczema whipped into shape. I was told to email her in a couple of days if there wasn’t a dramatic improvement to my ankle and face because she was going to make more referrals to get to the bottom of the eczema with an allergy specialist. And that the ankle should continue to be watched.

Look at how well my flowers came through the snow storm! I put a plastic garbage bag over them to keep them warm propped up over a tomato cage. You’d never know that they had 6″ of snow dumped on them! Just like my flowers, I came through the ankle adventure storm looking pretty good!

By the next morning it was obvious that I was better. Much better. Within a week my face was clear and the eczema was gone. My ankle is also much better, the lump is gone, but it continues to ache and carry on when I walk. My brain fog is gone. Fatigue? What fatigue? It’s like I needed an anti-inflammatory antibiotic or something…

My new, most wonderful in the whole world dermatologist is going to maintain me on the antibiotic. I’ve been on this drug before so I’m not worried about my kidneys at all.

I’m sorry I said all those nasty things to you, ankle. All in all, you were a blessing in disguise.

But anytime you want to stop with the achy hurt, that would be fine!