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.
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)
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.”
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.
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.
Yarn, Books & Roses 
❤️❤️❤️
I am entirely with him on your admirable attitude
I once sat through a training film at work that had a section titled “choose your attitude” and it really stuck with me. 🙂
Glad you figured out what it is!
Feels like I’ve been holding my breath for you. Am finally exhaling. I admire your persistence. More often than not we must be our own advocate. The road can only be up from where you stand. Be well.
Thank you. I have struggled with how to write about this. I’m pretty disappointed that this took so long as in hindsight there have been doctors who speculated that I was dealing with a circulatory issue or my heart that never made a referral or pursued the issue. On the other hand, it doesn’t change anything and now I just need to focus on moving forward. I am so looking forward to some appropriate treatment and maybe a couple of new drugs! 🙂
I remember when my aunt was diagnosed with Sjogren’s Syndrome. We’d never heard of it, didn’t know anyone who’d ever had it, and we sure as heck couldn’t even pronounce it. That was several decades ago but the doctors at John Sealy in Galveston never back down. Her first symptom was uncontrolled nose bleeds. Auntie has taken part in drug trials that may help others. Her life isn’t easy but she’s living every day that comes her way. THAT takes courage. Moving forward isn’t always easy but it takes courage.
One more thing. I grew up with a cousin who had sickle cell anemia. Everyone spoke in whispers when she missed class because we didn’t know if she’d ever come back. No one ever missed a single day of school back then; we knew so little about the illness. She never talked about it. My cousin died several decades ago but she married and had a son. She realized her dreams. Now there’s a cure. Bone marrow transplant. Perhaps stem cells will be the answer instead of drugs that often do more harm than good. Isn’t it ironic that the cure for many illnesses and diseases are within our bodies? Let us keep Hope alive and help it thrive.
You be well.
Sjogren’s is so difficult. I was diagnosed with Sjogren’s the same day that I got the systemic sclerosis (scleroderma) diagnosis and my doctors focused almost exclusively on the systemic sclerosis in my treatment plan and managed the Sjogren’s with eye drops and mouthwash. After some time I began to realize that the symptoms that were really laying me low were from the Sjogren’s: fatigue, muscle and joint pain, brain fog. Sjogren’s has pretty much taken over my life now: I am grateful for the contributions your aunt has made in developing new drugs. She sounds like a real warrior.
I had a couple of students with sickle cell and began to realize what a horrific struggle they were going through. One, a beautiful girl, had the most amazing attitude and I worked closely with her mother that year to keep her connected with the class and instruction while she was hospitalized for crisis events. Hugs about your cousin. I’m grateful for the advances in medicine that are occurring.
You be well.
I’m glad things are moving towards a correct diagnosis for you, and I hope the treatments will make you feel better. As a person who also has a rare condition, I went through an absolute diagnostic nightmare.
You nailed it: an absolute diagnostic nightmare. It is so hard to insist that your symptoms be taken seriously when dealing with a person whose training and experience is centered around the most common causes of illnesses when you are an outlier with a rare disease. Whew! We made it. Hugs to you, my friend. May we both have more good days then the other kind. 🙂
I don’t know how it works in the US but all those appointments with all those previously useless cardiologists and pulmonologists must have cost either you or the insurance company money that was wasted by their ineptitude and them giving up too easily on finding a condition. These new ones sound much better and I hope the diagnosis is confirmed because I assume once confirmed it will give you a definitive answer but also they can then find a solution. 🤞
I think that it is a failing in our medical system and the training of the medical personnel: it is cheaper to treat people for the most common causes of disease rather then order lots of tests to rule out rare conditions. I am one of the unfortunates who fell through the cracks for too long. I am finally where I am because every single common cause of my symptoms has been ruled out and I kept pushing for answers. Ironically, I may now get great care because I’m “interesting” with an unusual combination of conditions. Bad scleroderma, bad! I’m feeling much better about the doctors I am dealing with now and hoping for some help. Oh, I’m also on the hunt for a new cardiologist…
So sorry that you had to go through all this and be so persistent to get good help, but so glad you persisted! Take care of yourself!
It has been crazy, but I’m finally getting to where I need to be. It is hard to be assertive when you are needy and at the mercy of your doctors, but I got there I think. 🙂
Good for you!
You poor woman. I swear you have to doctor yourself these days. I’m glad you finally found a good doctor.
Life can be very ironic. I used to tell my biology students that it is important to have some background anatomy and physiology knowledge so that they could deal well with medical personnel; I am spending a lot of time researching these days so I can have meaningful discussions with my doctors. You really do need to take an active role in your own health or you are toast if you have something really unusual going on. I am so happy with the doctors that I now have!!
Wow. That was a bomb your doctor dropped. Thank heaven he found the problem.
In spite of all your challenges, you manage to maintain a positive outlook and your knitting is beautiful.
It really was a bomb; I was completely blindsided. Now that I’ve had time to think and to read up on Eisenmenger it seems like a really good possibility for what is going on.
Please keep us posted. Meanwhile, prayers and virtual hugs continue.