The Scleroderma Chronicles: The BLZ gets a CPET

The BLZ had some anxiety going into this test…

Last month I had a right heart catherization that showed an unexpected problem with my heart… there is a cardiac shunt that is allowing blood from the left side (you know, the one that is always colored red because that blood is rich with oxygen) to shoot over into the other side of the heart where it disrupts blood flow and adds pressure to the (blue) right side of the heart; that is a little tough on the right side because it isn’t built to handle the extra pressure. Houston, we have an explanation for all that blue panting that is going on.

Cool. Finally we were getting some answers and my doctors were taking my symptoms more seriously. My cardiologist ordered up some more tests to collect data and clarify the issue.

The test that I have been the most concerned about was the cardiopulmonary exercise test that was scheduled for last Monday. I hunted for some cool links to explain the test to use in this post and came up with a bunch of technical articles for physicians and specialists. Okay. Let’s not go there. I’ll try to explain what this test was all about. I was hooked up to a bunch of monitoring equipment that tracked my breathing and heart while I was riding a bike. As part of the test my lung function was tested, my heart monitored (like in an EKG), my blood pressure was tracked, my respiration rate, and the actual amount of oxygen I was using and the carbon dioxide that I was expelling for each mL of blood pumped. It was a lot!!

I was a little concerned because of my bad boy hip, but that wasn’t really a problem at all.

I shared my catnip with the Mother of Cats so that she wouldn’t have any trouble with the test.

The test went great for about 7 minutes: then there was an emergency stop. My blood pressure had suddenly zoomed up into the stratosphere at about 6 minutes into the test, and then 30 seconds later I began panting like crazy and the technician pulled the plug. Remember that cardiac shunt they found in the cath lab? When I exercise the direction of blood flow in that shunt reverses and blood from the right side is shooting into the left side where it interferes with the pumping of oxygenated blood to my body. No wonder I turn blue and pant.

The BLZ is both happy and sad at the same time.

The specialist who did my CPET was great at explaining the data to me; when he sent the report to my doctors he also included me in the email group. For the last week I have been reading the back and forth discussion by my doctors about the test and what the next steps for me will be. I am really pleased to be included in this process and feel that this option should be available to everyone; it is also kind of scary as I get a glimpse of what is coming down the road for me later in the testing department.

The gear and set-up for the CPET was pretty impressive. I joked with the specialist about it and he told me that there was an even more extreme version of testing that included a right heart catherization at the same time as the exercise test. Yep. You guessed it. That’s what my docs are considering to do next. It is clear that I have a cardiac shunt but they haven’t located it yet. The BLZ is pretty bummed. The word “profound” was used to describe my symptoms. There has also been some speculation about neuroendocrine tumors… The good news here is that no one is even considering sleep apnea or me needing antidepressants because, you know, I complain too much…

The BLZ is both happy and sad at the same time.

I keep my eye on the Mother of Cats while she reads her email.

So, it was a kind of tough week. I pulled myself together on Tuesday and went to a new yarn store for a little pick-me-up and was so distracted I missed my turn twice and had to detour through Starbucks before I actually pulled up in front of the store. I almost didn’t go in I was so worn out by then, but I did a little mental slapping to put myself into motion, pulled out my cane, and went in…

…to discover a woman dragging out three big bags of yarn that kind of screamed “community knitting” because they weren’t the yarn being sold in the store. “Hey! I want to community knit! Please, can I come play with you guys?” I immediately said.

That is how I ended up in a new local yarn store this morning knitting with the most wonderful group of ladies in the world. They are all current or former employees of the Kaiser medical system that I go to for my care, and they are producing hats for all of the Kaiser clinics that have infusion centers; I know those centers because they are the same ones that rheumatology patients go to. These ladies are all vaccinated and they all wore masks because they knew I was taking a risk to come. They had donuts! I have found a new knitting home when I needed it like no other. I actually cried a little with joy and relief as I drove away at the end of the meeting.

If I hadn’t made all those bad turns and the Starbucks stop this wouldn’t have happened.

My yarn stash is full of yarn that wants to turn into hats. Hats with happy colors and a dash of cashmere. I am on fire with purpose to produce as many hats as I can for other people who are facing down serious medical situations. I want to make them arm warmers and fingerless mitts. I have tapped into the best, most perfect group to produce useful gifts for other people like myself just when I needed to be grounded, inspired, and calmed by the peace of knitting.

The BLZ is happy.

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 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!!

The Scleroderma Chronicles: World Scleroderma Day, 2021

Here we are again… World Scleroderma Day.

I’ve written about scleroderma on this date for several years now. I just went back and read what I wrote last year and decided that I did a pretty good job. I talked about getting diagnosed, the complications that are being caused by my disease, and what that means in my life. Here’s that post if you would like to check out my take on things last year.

This is one crazy-ass disease and it certainly has complicated my life. In the year since I wrote that last post I have hung in there (hey, staying home for a year on oxygen slowly improving was actually good for me) and then emerged from strict lockdown to get lots of testing to try to sort out what is the cause of my blue-lipped status and the source of other little issues that I have going on. While thinking about what to write today I thought of a few things that I haven’t written about before and some things that are new since the last post.

The correct name for the type of scleroderma that I have is limited systemic sclerosis. Systemic means that every part of my body is being impacted by this disease, and the word sclerosis means that scarring is happening in tissues all over my body. What’s happening to me is not obvious to the outside viewer, but it is insidious and ongoing all the same. Somehow, by some mechanism that isn’t clear, my systemic sclerosis is being driven by antibodies that I’m producing that react with the centromeres of my cells.

What’s a centromere? I’m shocked, shocked I tell you, that you just don’t know that! Seriously, the centromere is that little place in the middle of a chromosome that can be seen when cells are getting ready to divide. This is a little tricky because the only time we can see chromosomes is when cells are getting ready to divide and they have already copied themselves: the little pinched waist in the middle of the chromosome where they are connected is where the centromere is located.

Got that?

This image belongs to the NIH; as a taxpaying citizen I hope that it is okay for me to use it here. 🙂

Somehow something happened to a protein located in the centromere area of the chromosomes in my body that made it look “different” and my immune system responded by making antibodies against that protein. The antibodies that are produced are called “anti-centromere antibodies” and their presence is highly suggestive of my form of systemic sclerosis. The alternative explanation is that my immune system just went berserk, decided to attack my own cells, and began making these antibodies on its own. I personally think that something changed and then the antibodies were made as a response. My money is on a virus.

It really doesn’t matter all that much since, once the immune system is triggered, the immune response can’t be turned off and one day you are sat down in some rheumatologist’s office and carefully told about what is happening to you and what to expect in the future. Your skin will get thick. Your blood vessels will be so damaged that they will thicken and spasm shutting off blood flow to parts of your body unexpectedly. The scarring tissue will build up in your digestive tract and damage the smooth muscles that you need to move food along. Your kidneys will lose function. Your nerves will be damaged. Your lungs need to protected since they are especially vulnerable to damage from accidently inhaled stomach acid. If your disease flares badly enough you can develop autoimmune pneumonia. Your tendons, muscles, and joints are all in trouble. You are in trouble. The damage will progress and there is no cure.

“This is really serious,” one of those early doctors told me. “This is like a diagnosis of cancer, but of course, some cancers can be cured…”

Well, shoot. Good thing there are drugs to help control symptoms and to slow the progression of the disease by dialing down the immune system.

Yay for drugs!! I am on drugs to shut down my stomach acid production (my lungs are doing pretty well) and on drugs to crush my immune system into submission. I’m on anti-inflammatories to control other cellular pathways activated by those ill-behaved antibodies. I get steroid injections to help my damaged joints and inflamed tendons. I have strict dietary limitations. I do lots of physical therapy. I’m on oxygen overnight to keep my red blood cell count in a normal range. I dress in layers to help control the spasms of my circulatory system, a phenomenon called Raynaud’s. I knit almost every day to keep my fingers from stiffening up and contracting. I now have a shiny purple cane to help me walk.

Every day is a challenge, but I am fine.

So what is new this year?

Scleroderma has damaged my heart. They are still testing to clarify exactly what is going on, but so far they have established that there is scarring in the heart muscle that is making my heart “stiff” and that somehow I developed a hole in my heart. There are suggestions of pulmonary hypertension, but I need more testing for a definitive diagnosis. No wonder I turn blue in the face and pant when I walk.

Oh, yeah, there is also a pandemic going on.

One of the greatest ironies of the last year is that serious cases of Covid-19 share similarities with the clinical symptoms of scleroderma crisis. Immune system-mediated pneumonia caused by an overreacting immune system is a hallmark of both conditions. Then there is this… Remember those drugs that I take to crush my immune system into submission? I take two drugs for that purpose. One of them, hydroxychloroquine, was (irresponsibly) politicized by influential individuals early in the pandemic and therefore became short in supply; my muscles and joints immediately rebelled when my supply lapsed early in the lockdown. The other drug that I take, mycophenolate mofetil, is linked to poor response to the Covid-19 vaccine. I am vaccinated and I am making anti-Covid antibodies, but since my total antibody count is very low my doctors aren’t sure I can fight off a Covid-19 infection fast enough to stay out of the hospital. I’m advised to continue to mask and isolate because… blue face, hole in heart, stuff like that…

For me, lockdown goes on.

So, here are the takeaway messages from this post. Be kind. There are illnesses that are really debilitating that you can’t see when you look at that person walking into a store from a handicapped parking spot. Don’t be dismissive of conditions with funny names that you’ve never heard of before. I know that it is hard to understand conditions that are beyond your experience and that you can’t really see, but take a moment to let someone tell you about their illness and the daily challenges that they embrace. If you meet a person with an autoimmune disease, they deserve a hug. If they have scleroderma you should give them two hugs! Be understanding of people who are still wearing a mask in public; perhaps they are braver than you can imagine.

Today is World Scleroderma Day.

Go Team Teal!!

Note: If you would like to know about different types of scleroderma you can learn about them here.

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 Scleroderma Chronicles: The Blue-Lipped Zebra Gets a Credible Hypothesis

At last.

If you have forgotten about the tales of the Blue-Lipped Zebra, that’s what I’ve taken to calling myself as I struggled over the last few years for some definitive answers to my blue-lipped shortness-of-breath issues. Really, things got pretty darn extreme late in 2019 and I started to get really pushy in finding some answers. Here’s the posts that I wrote then: The Blue-Lipped Zebra Report, The Blue-Lipped Zebra Gets Some MRIs, and The Blue-Lipped Zebra Goes on Oxygen.

Hannah: All of that happened before I came to take care of the Kitten Mom.

To summarize all of the adventures of the BLZ (that is code for Blue-Lipped Zebra), about 5 years ago I caught the flu and was really sick. I never fully recovered as shortness of breath lingered and lingered, and eventually I noticed that my lips turned blue after showers and when I climbed the stairs. I steadily worsened and began to call (and call, and call…) my rheumatologist for help. He ordered up some testing and referred me to a pulmonologist. My echocardiogram and lung CT scan looked good, but my pulmonary function test showed some asthma. My pulmonologist decided to that I must have asthma because of my Sjogren’s Disease, and maybe… (cue the dramatic music)

Sleep Apnea

so I got tested for that. That test showed that I had nocturnal hypoxemia (like, I was under 90% saturated blood oxygen for over an hour) so I was started on overnight oxygen. All my doctors dusted off their hands, said “Job done!” and that was that. As far as they were concerned the BLZ had been put out to pasture.

My symptoms improved and after 6 months I was taken off the oxygen again.

Over the next two years my symptoms came back and got steadily worse. To further complicate things my systemic sclerosis and Sjogren’s symptoms also worsened. I was constantly fighting a flare of my autoimmune diseases (joint and muscle pain, fatigue, brain fog, hair loss, GI nastiness) at the same time I struggled to walk without stopping to put my head between my knees because I felt faint. I ended up on the floor more than once. I panted like a freight train when going up stairs and now my entire lower face was looking blue at times. I coughed up mucus every morning and it sometimes contained streaks of blood. The BLZ was back and running wild. My rheumatologist ordered tests; once again my heart and lung tests looked good. Disgusted with my complaining, my rheumatologist decided that maybe I should be prescribed antidepressants, or maybe I should be tested for… (cue the dramatic music)

Sleep Apnea

I fired that doctor and found another. I met with my internist, who ordered more testing, and I had a first appointment with the new rheumatologist who had been recommended to me by the local chapter of the Scleroderma Foundation. The new tests showed that I had severe inflammation of my tendons, a destroyed hip joint, a condition called polycythemia (too many red blood cells), and nocturnal hypoxemia again. I was put back onto oxygen and the pandemic then closed everything down. I asked about the possible cause of my polycythemia, but there wasn’t anything that really jumped out to my doctors. The BLZ was ordered into strict isolation for the duration of the Covid-19 emergency.

Now I am fully vaccinated and back into the world and pushing my way though new testing and have acquired a couple of new doctors after ending up in urgent care with chest pain, a blue face, and shortness of breath. The testing early this year showed that I had more fluid around my heart and that the pressure on the right side up my heart was up into borderline high range. Because of my systemic sclerosis I am high risk for a condition called pulmonary arterial hypertension so that high pressure reading triggered an alarm: I was sent to a cardiologist.

The cardiologist was completely dismissive of the two clinical observations that had generated the cardiology referral, was borderline disparaging of my anxiousness about my not-yet-vaccinated status (there are other people who are sicker…), lectured me about getting cardio in three times a week, and insisted that I be tested for… (you know the drill: dramatic music time)

Sleep Apnea

“At least you didn’t offer me antidepressants,” I said. I limped away, fighting tears and panting for air, wondering why I have to keep firing doctors and hunting for new ones. “Suck it up, Buttercup,” said the BLZ as I drove home. “You have a new pulmonologist to talk about this with.”

There I am, waiting for the pulmonologist, minutes before he gave me the unifying diagnostic hypothesis.

Three weeks ago I met with the new pulmonologist, one that was recommended by my new (wonderful) rheumatologist. He asked me lots of questions, dismissed the notion that I have sleep apnea (“What a shock!” snarked the BLZ), and then dropped the bomb. I have a cardiac shunt. Blood from the right, unoxygenated side of my heart, is passing through a hole in my heart and disrupting the flow of oxygenated blood to my body. He ordered a new echocardiogram with bubbles to look for the hole and to confirm the diagnosis. He also wants to check how much fluid is around my heart and is concerned about the right side pressure levels, which were the two reasons why I was sent to that cardiologist (that the BLZ wants to kick in the face…) in the first place.

Hannah: The new echocardiogram is in three more weeks. The Kitten Mom is getting a little anxious while she waits…

As the pulmonologist walked me to the door out of the clinic that day he casually said to me, “I really admire your attitude.”

Oh, oh.

The BLZ’s whiskers started to tingle.

You know I googled for information about cardiac shunts from the parking garage before I even drove away from the appointment. Almost immediately the condition that he suspects appeared on the page: Eisenmenger syndrome. I have every single one of the symptoms that are listed on the page. This is the unifying diagnosis, if the echocardiogram confirms it, that explains the blue face, shortness of breath, nocturnal hypoxemia, polycythemia, and the inflammation that has been driving my two autoimmune diseases, systemic sclerosis and Sjogren’s disease out of control.

Why did it take so long to get here?

Eisenmenger is rare.

The BLZ is barking with laughter.

Me, I’m working on my attitude as I wait for the echocardiogram appointment.

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 Saturday Update: Week 4, 2021

It was a crazy, crazy busy week with lots going on. I had medical testing, an online court appearance to give testimony in a neighbor’s custody case, and a nail in one of my car’s tires. My medical status continues unchanged (blue lips, chest pain, shortness of breath), but the machinery to get to the bottom of things is now in motion. The first couple of rounds of testing have generated a referral to cardiology and hopefully that will happen this week. My neighbor won her custody case, and the car tire is now repaired. Whew! I spent the bottom half of the week relaxing with my knitting because I was completely pooped by all of that running around.

The Kitten Mom left me all alone THREE times this week!!

Knitting

I did make some good progress this week in spite of the trips out of the house.

My Geology socks are done!! I’ve decided to try to make at least one pair of socks each month, so these are January’s pair.

I also buckled down and did the blocking and finishing work on my Secret Life of Cats (and dogz) shawl by Sharon from Security (Casapinka). This is the longer shawl version of the project; there were also options to make a cowl or a scarf. Fun color for gloomy days, huh! I’m actually thinking that I may be giving this one away to someone who loves purple and bright colors and a scarf version made with scrap yarn from the stash may be in my future.

It snowed this week I so gave in to the urge and cast on one of the sweaters that I have been dying to get going on. I have wanted to make Goldwing for a long time, and bought the yarn a couple of weeks ago with my stimulus check. Here it is, finally started:

Look at the absolute quality help that I am getting from Hannah!!

The Scleroderma Chronicles: The Pulmonary Hypertension Edition

I few weeks ago I posted about my systemic sclerosis, Covid-19, and my decision to donate my DNA to the 23andMe Systemic Sclerosis Research Project. My DNA has safely arrived and is in the lab getting sequenced right now. I was motivated to contribute because Covid-19 is creating so many new patients with fibrosis that may benefit from this research in addition to people like myself with autoimmune disease or people with other fibrotic diseases.

Monday I had an urgent echocardiogram done and once again an eerie connection between my disease, systemic sclerosis, and Covid-19 appeared. My test was started a little late so I asked the technician if things were busy. He told me that they were very busy because there were so many Covid-19 long haulers who needed testing. After a while, thinking things over, I asked if these patients were getting heart damage. “Well, not their heart muscle, but they were developing pulmonary hypertension,” he said. Oh, oh. That is the very reason I was there getting an echocardiogram; as a systemic sclerosis patient I am high risk for pulmonary hypertension and pulmonary arterial hypertension, and I know that those are serious and life altering/ending conditions. After thinking a little longer I asked him how many Covid-19 long haulers were getting that diagnosis. “It’s in double digits,” he replied…

Double digits. At this one medical center in the heart of Denver. That means that there are potentially hundreds and hundreds of patients getting that diagnosis across my state.

I wished that I had thought to ask him how old those patients were…

The next day my rheumatologist called to let me know that I was being referred to cardiology as my echocardiogram results suggested pulmonary hypertension and that further testing was required. There is also an issue with fluid around my heart… It was what I expected, but not exactly what I was looking forward to. The only problem right now is getting me into cardiology, because, all of those Covid-19 long haulers…

It has been impressed on me that I need to double mask now each time I go out into public. I have a nice N95 level mask, but I’m also putting a medical grade mask on top of it.

Be careful, people!!

Stay safe and wear your masks!!

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.

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!!