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: The cardiac test results have arrived…

Spoiler Alert: there’s a happy ending!

It has really been kind of a rocky month. Following several episodes of shortness of breath, chest pain, heart palpitations and blue lips my doctors decided that I was due for a full round of testing. My primary care physician ordered up a battery of cardiac testing and sent me off to see my specialists.

Cookies
First up: the pulmonologist. I made these cute little sheep cookies to take to the office staff when I went in for my appointment.

I really like my pulmonologist. She is thorough, direct, and answers all of my questions without sugar coating things. I had chest pain in her office and while I was talking to her my lips turned blue right on cue. Wow. It’s like having a trick pony that performs for the audience! We talked about the possible causes, all of which were pretty serious heart conditions, and she decided that I needed to complete my cardiac testing before I did her pulmonary function tests.

I’m not going to lie, it was sounding pretty serious. She thought that I either was developing heart failure, pulmonary hypertension (a fatal complication of scleroderma), or my heart was being starved due to blood vessel constriction.

Next up: my rheumatologist. We discussed increasing my immunosuppressant dosage to crush my Sjogren’s, which has been pretty active, into submission. She hated to up the dosage unless there was no other choice due to the risk of infection, especially since she thought that it was probable that I was experiencing pulmonary hypertension symptoms, and that meant that the pulmonologist should be the lead on treatment.

What was needed, clearly, was some test results to clarify the situation. And knitting. Lots of knitting.

Knitted fabric.
I knitting like crazy all month on the What the Fade?! shawl and finished it yesterday. There has been research that shows that knitting has calming benefits greater than yoga. Since yoga is out of the question right now, I knit.

Are you familiar with Holter monitors? That’s the test where you are hooked up to sensors and wires that go to a device that records your heart’s electrical activity for 72 hours. 72 long, itchy, forced to sleep on your back, OMG, how did this sensor get attached to my hair, hours. Whew. It was done. The results: my heart was normal. Still short of breath and feeling dizzy, I went back to knitting.

Next up was the echocardiogram.  Dizzy and feeling faint, I went for the test one morning last week and then headed on over to my LYS for some knitting action. Following my BKB Deb around the store looking for the yarn to knit a Tegna sweater I felt faint and ended up sitting on the floor at one point. This was getting ridiculous! Especially since the technician who did the echocardiogram test told me that she thought I would be very happy with the result…

I began to wonder if the problem could be my blood pressure. After years of battling hypertension that was hard to control I was posting some really low numbers at my checks. Maybe I was getting too low?

I did a little searching on the internet, and discovered that there was some research that suggested that it was important to keep diastolic pressure above the 60s. Oh. I was often in the 60s. Maybe I was sending my pressure down too low every time I took my morning medication. My doctors were so happy with the current numbers, but maybe things had changed. Maybe I didn’t have hypertension any more… maybe the pulmonologist was right about the blood starved heart, but it was due to low blood pressure. I decided that I should check my pressure every morning before taking my meds.

Blood pressure.
Tbis was my blood pressure reading the next morning before my medication.

I skipped my meds and started recording my pressure readings several times a day. The chest pain and dizziness disappeared. I sent an email to my primary physician with the BP log attached and we set an appointment to talk yesterday as she had just received the echocardiogram results.

Surprise!! My heart is in better shape now than it was at the time of my diagnosis. My pulmonary hypertension is gone and my heart is now pumping normally. She agreed that my high blood pressure seems to have reversed and that the medications that I have been taking are too much for me now. We agreed to try a quarter dose for a few weeks to see what happens.

The immunosuppressant drug that I am taking has been shown to reverse scarring in lungs, and the high blood pressure drug that I was taking (in too high a dose) also may have positively impacted my heart. My skin isn’t as tight as it was a couple of years ago; it looks like my blood vessels are also now in better shape. Less stiff blood vessels means lower blood pressure. Yay!!

My doctor thinks that the drugs have caused these improvements.

My neighbor, who mows my lawn and prays for a miraculous cure, is sure that God has intervened.

I’m convinced that it was the knitting. 🙂

Next week: the lung testing begins.