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.