Cardiac Shunt – Yarn, Books & Roses

The Scleroderma Chronicles: And Do You Exercise Regularly?

It has been an eventful week in big and small ways. I had been mostly in bed for most of a week as I struggled my way through two snowstorms with significant air pressure drops. Ugh. I had chest pain, coughing, heart palpitations, and more sleep than I want to admit to. Towards the middle of the week, I went off my immunosuppressant drug and the flare of my disease(s) arrived over the next two days. Ugh. So predictable, but still discouraging. I dragged myself together on Monday, double masked, and made it to the pharmacy where I had an appointment for a Covid-19 booster that afternoon.

Mateo: Do I need a booster too? Here’s my arm…

I have this really wacky sense of humor. The entire experience just kind of cracked me up. You see, I got the appointment at my local grocery store’s pharmacy. Here are some of the highlights:

I have had so many shots at this point that they had to use the back of my vaccine card. At this rate I will need an accordion-like pullout for the vaccine information in a few months.
- Why so many shots? I’m immunosuppressed. I went off my drugs this time to give my immune system a better chance of responding to the vaccine.
The staging area for the shot was at the Fritos display across from the pharmacy. Seriously, the pharmacist said, “Go stand with the Fritos and wait your turn.”
The shot was easy, peasy. I think that the syringe was spring loaded it was so fast. “Go walk around the store for 10 minutes before you leave,” I was told.
So I waved goodbye to the Fritos and walked around the store. Mostly I just looked at the empty aisles for the 10 minutes feeling sorry for myself. No milk. No Snapple. No cat food. No guacamole. NO GUACAMOLE!!!! Oh, yeah. Genius me scheduled the booster shot during a grocery store strike by the competing chain’s employees and this store was basically stripped of essentials by the descending horde of shoppers who didn’t want to cross the picket line. As they shouldn’t. But they could have left me a little guacamole, don’t you think?

I was able to get the cats a Boston fern to replace the palm that was chomped to death by… I wonder who could have done that? Hmmm…

I also got a Starbucks. Not the worst trip out of the house. I ended up with a sore arm and was so exhausted that I slept for 12 hours.

Today, 48 hours after the booster shot, I feel great!! The flare is gone. I haven’t felt this good in weeks. This happened to me the last time I got a Covid booster. I think that it must be the increase in antibodies or something; I’m so immunosuppressed that my gamma globulins are way too low (a medical condition that my doctors are just ignoring because I do have enough white blood cells). Maybe the boost in antibodies following the shot actually makes me feel better somehow. Maybe my white cell count goes up. It’s a mystery. I’ll take the win!

And in that winning mood I went to see my cardiologist for the first time since my trip to the cath lab last fall. I was a little short of breath but was walking okay when I got to the office. I received an EKG test and the nurse checked me in:

Nurse: “And do you exercise regularly?” (in a judgmental tone of voice…)

Me: “Oh, please. Let’s not even pretend that I am able to exercise!”

Nurse: “Oh. I’m so sorry that I asked you that…” We both started laughing, but I meant it! I am so over feeling defensive about being unable to exercise. In fact, trying to exercise with my condition was damaging my heart.

I also think that I had my snark on under my mask.

My cardiologist is freaking awesome. He asked lots of questions about how I was doing. (Face now mostly not blue. Yay! Some panting and chest pain, but so much better. I went up a couple of flights of stairs with no problem.) We discussed the fact that I don’t fit the usual diagnostic model for pulmonary hypertension but based on physiological changes consistent with PH and my dramatic response to treatment with a PH drug, he made the call and entered the diagnosis. The matter is now settled until new data comes along.

Exercise-induced pulmonary hypertension. As in, you look perfectly normal when you are on the table getting your lung/heart tests, but the minute you exercise all hell breaks loose in the blood vessels of your lungs. Fabulous. There is a really invasive testing protocol that I could be subjected to, but there is enough evidence now to establish the diagnosis without it. The diagnosis became part of my medical record today. At last.

It has been almost exactly 5 years since the BLZ began her journey to find help and answers. This has been really, really hard, but I made it.

We discussed the pros/cons of more testing. We talked about the risks and benefits of adding a second drug to the one I’m already on. We talked about who will take over management of my PH (he will) and how he will integrate with my rheumatologist. We talked about how important it is to be comfortable with “out of the box” thinking when dealing with a patient who is basically at the far end of the bell curve… in other words, a zebra. Oh, I like this guy!!

I agreed to start the additional medication which will be added to the one that I’m already taking. There will be more side effects as this second drug kicks in and I will be getting several phone calls to check on me as I start it. The plan is to try to slow down my progression before I develop full blown PH.

Next up: more testing to hunt for that dang hole in my heart. It’s like a snipe hunt, but so much less fun. As in, heart surgery anyone?

Hannah: Don’t worry mom: you’re got this!

Goodbye 2021: Year’s End

This is the last update for the year 2021. You know, 2020 was a pretty bad year for both me and the world, but 2021 just plain outdid itself. THIS HAS BEEN A HORRIBLE YEAR!!!! Seriously, I have been reflecting on all the horribleness of the year, and it is multilayered in the just plain awfulness of it. Here is some of the angst, anger, and sadness in a nutshell.

If you aren’t wearing a mask these days, shame on you!! I have been in lockdown for two stinking years (!) and I am over all your anti-public health nonsense.

If you are one of the doctors who told me that there wasn’t anything that you could do for me (and to not come back), or that I had sleep apnea, or that I needed to exercise more, or that my tests showed that I was fine and you would just continue to monitor my symptoms… shame on you!!! I finally received credible diagnoses this fall (from new doctors) that explained my symptoms. You know, the ones that you ignored or dismissed all those times I came to get help… It is good to get diagnosed, but in this case it is also a mixed blessing as my condition cannot be reversed and management is going to be difficult. Shame. On. You!!!

If you are one of the people who believe that the election in the US was stolen and that the answer is to impede the ability of American citizens to vote, or to create a provision that allows the legislatures of some states to just overturn the results of elections, or to resort to violence… shame on you!!!!

Did you notice that the number of exclamation marks kept growing? That’s how much shame is attached to those targets. There, I got that off my chest and let’s hope that things go a little better in the coming year.

True to its rotten black heart 2021 went out in absolutely dreadful and ironic fashion. First the dreadful.

We are in an extreme drought where I live and everything is brown. The grass, the fields, the bushes and even some of the evergreen trees are now brown.

Last Thursday, December 30th, we had a high wind event in my state of Colorado as a major weather front pushed its way towards us over the Rocky Mountains. In the area around Boulder, Colorado the gusts were extreme (one was clocked at 115 mph) and the sustained winds through the day were around 75 mph. Several fires started and swept through dried fields and brush towards housing developments and towns with horrifying speed. There was nothing that could be done as subdivisions, stores, and hospitals were evacuated; the fire crews set up base in the parking lot of a mall where they could best defend themselves while waiting for a break in the wind. No joy there throughout the afternoon and early evening. There were heartbreaking scenes of burning homes by others covered in Christmas lights. People parked along the major freeway hoping to see if their home was still standing. The historic downtown area of a town I have frequented in the past was lost.

At around 3pm I realized that the worst fire was near my son’s home in northern Westminster. As the evacuation zone continued to grow to within a mile of him we started to make plans to get him and his pets out safely. It was a nightmare as firetruck after firetruck rushed north towards the fire line in the night past his windows. By midnight the winds had died down, the fire stopped its spread to the south, and my son was safe. Daylight the next day showed that the damage was just horrific. Almost 1,000 homes have been lost, thousands are displaced, and the hunt for the missing is ongoing. There is information about all of this here.

Then the storm arrived on New Year’s Eve.

In a cruel twist of irony, after failing to deliver any real snow all fall and early winter, the winter storm arrived New Year’s Eve with snow, icy roads, and bitter cold. Like, we started the day at 3 degrees Fahrenheit yesterday. Serious, serious cold after weeks of warm sunny weather. All those poor people who escaped with only the clothes on their backs in the wind/fire event now have to deal with this. Bad 2021, bad!!

And that, thankfully, was the end of the year.

I also finished my Kevat sweater in the final days of the year.

It is wet and blocking in this photo, but not finished. See the loose ends of yarn?

I did finish the sweater with the ribbing and I-cord to make nice open and clean edges. I decided to block and try on the sweater again before knitting on a little lace edging onto the bottom; if the length is exactly right there won’t be any lace added. I did do a little math, however, and I do have exactly the right number of stitches to do the lace. It’s a sign, right?

Hannah and Mateo (AKA the Coalbear) and I all wish you all a Happy New Year. May things take a turn for the better with the coming weeks and months.

And 2022, you had better behave yourself or I’ll be sending you out on a walk of shame, too. I do have to warn you, however, that I am a little concerned with how you are starting out…

Because this year we are going to learn how to cope with a million new Covid cases a day in the US, and I am going to deal with this whole broken heart thing that I have going on, and we are going to f*cking save democracy. Shape up quick 2022, because this is the tough time, and you had better show some backbone, because I expect you to fight like you really mean it for the things that are really important.

The Scleroderma Chronicles: The Blue-Lipped Zebra Gets Her Diagnosis!

At last. I mean, this has been going on for more than 5 years and had reached the point of utter absurdity. If you haven’t been keeping up on all of this, I have been struggling with shortness of breath and sporting blue lips for way too long. I also have pretty significant fatigue, chest pain, and major muscle and joint pain. I’m a mess.

I have a rare autoimmune disease called limited system sclerosis(scleroderma) which makes me high risk for lung and heart issues. People with rare diseases are called “zebras” in the medical community; since I’m sporting blue lips I’m the Blue-Lipped Zebra (BLZ). Got that?

My doctors do routine testing to monitor me for heart and lung conditions associated with systemic sclerosis; each time I had an echocardiogram and a high resolution CT scan the results were that I was… fine. No indications of a major problem.

But I was absolutely, positively not fine. I began to refer to the reassurances that all was okay as medical gaslighting. I transferred to new doctors. I got copies of all my test results, did lots of google searches, read research papers and articles in medical journals, and began to have evidence-based discussions with my doctors. They ordered up more aggressive testing of my heart and lungs. I posted about the my right heart catherization and CPET here if you want to catch up.

Last Monday my cardiologist called and gave me the final diagnosis. I have a type of pulmonary hypertension that is exercise-induced that is being complicated by a cardiac shunt in my heart. At rest, for all those previous echocardiograms and CT scans, everything was fine. When I’m in motion it is another story.

So, what exactly is pulmonary hypertension and why am I, as a systemic sclerosis patient, at high risk for it? In the most simple terms, the interiors of my lung arteries are narrowing due to scleroderma scarring and tissue growth; as the openings get smaller, the pressure of blood flowing through the arteries gets higher.

When we exercise the body needs more oxygen delivered to tissues; arteries constrict to raise blood pressure, your heart speeds up and your respiration rate increases. In my case, that constriction of arteries in my lungs makes the blood pressure in the lungs increase too much; blood struggles to get through the pulmonary arteries, and the downstream pressure in my right heart forces blood to flow from the right side, through the shunt, and into the left. My body’s blood pressure zooms up as the left side of the heart fights to push blood out of the heart past the jet of blood coming in from the right side through that dang hole. It’s a catastrophic cascade that happens in seconds, and the entire phenomenon is being driven by my systemic sclerosis created pulmonary hypertension. “You’re a challenging patient,” my cardiologist told me as we talked about my future treatment. Yep. That’s me. A challenge. I excel at challenges.

Last week the new medication that my cardiologist prescribed was shipped overnight express to me by Kaiser’s National Specialty Pharmacy. Kind of unusual, right? That’s because pulmonary hypertension is rare, so there aren’t that many people taking this drug in the US. If I was a Blue-Lipped Zebra before, I am now a BLZ wearing a crown. A periwinkle crown, of course, for pulmonary hypertension.

When I started this scleroderma journey one of my doctors told me that it was good to have a diagnosis, even if it was a shame. This is true. I’ve learned a lot since my first blood tests came back hinting at an auto-immune disease that generated a referral to a rheumatologist.

I’ve learned to be patient. I’ve learned to advocate for myself. I’ve learned to take the initiative to learn about my disease and to become an active participant in my treatment plan. I’ve learned to face down the monster and to go on with my life.

Challenge accepted!

This is Pulmonary Hypertension Awareness month. About 15% of systemic sclerosis patients develop pulmonary hypertension as a consequence of their disease. You can learn more about pulmonary hypertension here.

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: 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 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.