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!!

Author: Midnight Knitter

I weave, knit and read in Aurora, Colorado where my garden lives. I have 2 sons, a knitting daughter-in-law, a grandson and two exceptionally spoiled kittens. In 2014 I was diagnosed with a serious rare autoimmune disease called systemic sclerosis along with Sjogren's Disease and fibromyalgia.

16 thoughts on “The Scleroderma Chronicles: Matters of the Heart”

  1. Many hopes that the physicians will schedule the tests and the particular treatments that you need! And that you will get more relief! This is all excellent advice. When I became ill with a rare disease in the early 90s, we found we had to request copies of the office notes and test results ourselves, go to medical libraries and read medical papers on a broad range of topics, and borrow a nursing school textbook from a friend. My husband had an MA in EE so his science background helped. When we got to the sub-specialty clinics and got definitive diagnostic tests done, we got those copies too and we keep them safe. They likely aren’t available any more as the doctors moved to other clinics. The specialist doctors, and a support group, encouraged us to research and welcomed the help. Those early doctors who didn’t know what was going on, not so much. Adorable kitten (and your other kitty too) and bunny, and the kitties and I wish you all the best!

    1. I am so sorry that you have gone on a similar journey, but I’m happy to hear that you were able to take control of your own medical data and work proactively with your specialist doctors. There are laws now that make it easier to get access to data, but I still had to message one of my doctors to get the echocardiogram. I have also “fired” several doctors and found new ones in the last two years because they weren’t listening to me or were gaslighting me. You know, you describe your symptoms and then the doctor explains that something else actually happened. Say, what? Anyway, my current doctors are great, all know each other, and are very supportive.
      My new rule of thumb is if they tell me to not research my condition on the internet or offer my antidepressants they get fired!
      Hugs to you. I hope that your rare disease is either long gone or behaving itself.

  2. You are a woman after my own heart. Back when I asked to see my own records, I was allowed to see very little and only in the presence of the medical records clerk. Had to get an attorney. If all patients could advocate for themselves, learn their rights and not be intimidated by people who “practice” medicine . . . Well, the world would be in a better place. I still cringe and sometimes cry when I remember how I gave over my power to doctors, none of whom had my best interests at heart. We know our bodies better than anyone. Doctors need to learn how to listen and allow patients to have an active role in their own care.

    I have great expectations for you. Stay strong. Be well.

    1. You are my displaced sister, obviously. That is my medical data and how dare it be withheld from me. I’m so sorry you had to lawyer up. I do understand that doctors have to deal with lots of crazy alternative medicine requests from patients, but I’m for a well informed and educated patient population that can have meaningful discussions about their care with their doctors.
      I am feeling pretty strong at the moment and have high hopes that we will have a handle on my medical status and an action plan soon. I have dreams of being a patient advocate to help other people with complicated medical profiles navigate the system.
      I have been warned that there isn’t much to be done, but I’ve heard that before and I actually got better. It’s better to know and to deal with things head on in my opinion.
      Be well!

  3. You are so brave. Knowledge is power, and the more you know the better you are for it. I am so sorry you are struggling. I will pray for you. You are a very strong woman and you have many people thinking and praying for you.

    That diastolic dysfunction “magically appeared” during my bout with long covid but no one thinks it’s a big deal (I’m 56 and do have HBP but it’s treated). One doctor said covid didn’t cause it, another said it could have been developing. I’m a researcher by occupation and read about inflammation and RA, that it is quite common in those patients. I had some high risks that were controlled by meds but I’m blaming both the covid and RA because both are inflammatory conditions, it’s too coincidental. The body can only take so much and it’s sure suspicious. So two doctors tell me it’s no big deal, and I don’t need to see a cardiologist. My rheumatologist says if my yearly echos stay stable then don’t need to see one. I usually follow her advice.

    There’s other stuff going on I won’t bore you with but I really appreciate all of your advice!

    Thinking of you and virtual hugs from across the miles!

    1. Oh, I am so suspicious about long Covid; the symptoms are so much like my own experiences with long term inflammation. I get just crazy when doctors tell me that I’m okay and that they will just monitor my condition through my ongoing bloodwork. Say, what?! I am now calling this medical gaslighting.
      My issues all started in the aftermath of a flu-like illness that went on for almost 6 weeks. I was forced to go off my meds during that time and then when I recovered the blue face/panting/problems with blood pressure stuff started. After years of struggling to keep my hypertension under control I had low blood pressure and ended up on the floor if I took my meds. Crazy, huh. I called it in, and was told to stop taking my BP meds, but in retrospect I should have pushed harder to find out what was happening. To be fair, it is so hard for doctors to sort things out when there is so much going on (scleroderma, Sjogren’s, fibromyalgia, asthma, hypertension, partridge in a pear tree, …) but I should have pushed more. Your rheumatologist is probably correct about the yearly echos (it’s the same as what mine say) but if your symptoms get worse do ask about different types of echocardiograms. My DD was discovered in the latest, upgraded echocardiogram that was ordered by my pulmonologist, and he told me that there is another version that should be done now. Who knew?

      1. Thank you for the advice!
        You poor thing. Yeah my RA happened a little over a year after a bad bout with the flu (5 weeks)—-viruses are notorious for instigating autoimmune issues! Actually lung issues started six months after that as difficult to treat asthma (it wasn’t difficult before that). My rheumatologist and former asthma doctor (can’t treat me because of long covid whatev) are worried it’s an autoimmune lung issue and so am I because there’s definitely what seems to be a permanent restriction in my lungs. We were working on figuring it out and then covid hit and ughhhhh. I have lung issues and exhaustion, no other symptoms but it repeats itself over and over like covid. Anyway I’m seeing a new pulmonologist who treats a lot of my rheumatologist’s patients in September. The long covid doctor did say I’m in an inflammatory state -well times two I guess! You are right it’s hard for doctors to sort things out but ughhhhh so annoying
        You hang in there, thinking about you , appreciate all of the advice!

      2. I have had a big gap in care because of Covid too, but at least I don’t think that I had to deal with an infection. I had difficult to treat asthma that was caused by my Sjogren’s disease… evidently one of my antibodies is associated with small airway disease. You can ask your rheumatologist about it. I’ve been living on Sleepy Time tea when I get bad as it contains theophylline which helps with asthma. Long term inflammation is just awful. I was sore all over, had crushing fatigue, and then of course there was the brain fog. I sure hope you manage to get out of that soon!!
        Did I mention tart cherry as a supplement? I got approval from my docs to take it (kidney issues) and it had a major impact on inflammation; my stomach couldn’t tolerate the juice so I gulp down capsules from the health store. I also take turmeric supplements.

  4. Good for you standing firm in getting answers and taking such an active role in managing your care! Thank you for sharing your experience and advice – it will surely be invaluable for others. Glad your internist is helping you with this and advising on good next steps. Hoping for good results going forward! Finally, thank you for sharing pics of Mateo and your lovely flowers 🙂

    1. I keep thinking of that phrase that Maxine Waters used during a congressional hearing: “Reclaiming my Time”. I am so trying to reclaim my life and am no longer willing to be the passive participant in this medical misadventure. I am so lucky that I have managed to secure a medical team that is also onboard.

      All of my doctors are pretty young, which is probably important in how they are dealing with me. 🙂

      Mateo is growing at an astonishing rate right now. Kind of scary to see him in the mornings as he seems to get bigger overnight.

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