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.

Then the doctor came in!

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 a long time coming!!

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!

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.

26 thoughts on “The Scleroderma Chronicles: And Do You Exercise Regularly?”

  1. That is good news you finally have official diagnosis and current plan is working well for you. Yay to your cardiologist. All those numpties for so many years not diagnosing you correctly. What a waste of money…even if it’s the insurance company’s.

    1. They were numpties!! To be fair, PH is really rare, and I now belong to a subset of patients who are abnormal in their presentation. It’s kind of an emerging diagnosis and the drug that I’m having added on is one that has been used in research trials with systemic sclerosis patients who have exercise induced PH. On the other hand, those other doctors are numpties because they just dismissed what I was telling them and suggested that I should be placed on antidepressants.

  2. Your new doc sounds priceless. It’s good to hear you are on a better path with your illness. And no, I haven’t been exercising regularly. I typically hole up in the winter. Not an excuse, I know. My Dad, who was a doctor, used to give me gamma globulin shots when I was a kid. I had forgotten about that until you mentioned it.

    1. It is so validating to have a diagnosis that matches my symptoms and actual treatment for the condition. The exercise is a big deal because I was being encouraged (hounded) to get more exercise at every doctor’s visit which kind of carried the message that I was not taking my health seriously and that I was partially to blame for what was happening to me. In truth, I was hurting my heart as I struggled to get more exercise. Life at the edge of the bell curve…
      I got a gamma globulin shot once as a child to prevent me from contracting measles after exposure. It worked! I never got sick but I do have immunity. I bet you had a healthy childhood. 🙂

  3. A diagnosis is a good thing! And I learned from (secondhand) experience that heart surgery isn’t always as scary as it sounds. (A family member had one a few years back, and made a full recovery with a lot less fuss than all of us had expected.) I hope whatever additional steps you need to take go smoothly!

    At least your store had some Fritos for company, I guess? Pity about the lack of guacamole, though.

    1. I still want guacamole. Sigh. I just talked to the cardiology nurse about the upcoming testing. It has to be scheduled at a hospital downtown, so it will be another couple of weeks. Still, forward progress is good. 🙂

  4. So glad you have an official diagnosis now! You have had a long and difficult journey, but finally there’s progress 🙂 Thank goodness for your wonderful sense of humor and for your beautiful cats 🙂

  5. I’m glad you have a good plan going and finally found a good doctor! I have to say I haven’t found the right cocktail of meds yet for my RA after a year but still working at it. And that pesky year old long covid lung problem, researchers are now finding lung damage that can’t be seen on CTs (2 years after the start of the pandemic), I’ve been saying that all along

    1. I think that RA is just really difficult to treat. I don’t know anyone who is happy with the way things are going (that kind of goes for lupus, too). I’m guessing that there is a lot of diversity in symptoms and how people respond to treatment.
      My lung issues were finally picked up on a high density CT scam done with no contrast. I absolutely know how frustrating it is to get a diagnosis. Covid is the gift that just keeps on giving for an unlucky few. I’m so sorry to hear that you are dealing with long Covid. There are several new patients arriving in my pulmonary hypertension and scleroderma support groups online who are long Covid patients. I wish that there was more press about that aspect of the pandemic.

      1. yeah that’s what I’m finding out unfortunately, and know no one with RA. I had a good med that worked but caused worse lung problems oyyyyyy!!! I’m fortunate that my lungs are a little better, just not near precovid levels. My last CT scan was high density no contrast I believe but I’ll have to check—showed some scarring but the pulmonologist says it wasn’t bad enough to cause problems. He may be the doctor but I also have had consistently elevated carbon dioxide problems and restricted lung on breathing tests. It’s a definite lung problem (I say that) but I’m not “too abnormal”, meaning abnormal but not abnormal enough so he just treats it like asthma. My rheumatologist gave me a med that helps RA connected interstitial lung disease as an experiment because I had prior undiagnosed lung problems—covid just mad it 100x worse)— so it’s interesting it’s gotten a tad better in the time frame the med should take effect (but she said it wouldn’t help with covid damage). A lot that talk about with her, she’s really awesome. Having another CT next month.

        Oh boy—-yeah statistically it’s really bad, there are so many with it. You are right about that. It’s sad.

        Hang in there, virtual hugs!!!

      2. I wonder if you were on methotrexate. I was on that for a few months and it really helped except… I developed costochondritis and that was the end of that. The big concern in the ER was that I had lung damage from the drug and I wondered why was I ever put on it!! I do have a little interstitial lung disease going on so I totally understand the whole lung testing merry-go-round. I am so sorry that you are on it now. I hope that new drug does wonders for you!! My cardiologist pushed through an urgent referral to get me started on another drug for my pulmonary hypertension (well, the whole Kaiser team jumped on it… there were 4 phone calls and some paperwork that was emailed back and forth to make it happen) but it’s another “it will take a few weeks or months for it to make a difference” type drug. I’m glad that you have a doctor now that is awesome. Hugs to you! I know how exhausting this is, especially when you are struggling to breathe.

  6. It isn’t fair that they took all of the guacamole and you couldn’t have a celebratory chip and guacamole dinner after your booster. I’m so happy you have a doctor that cares and listens now. I feel like all of your blog knitting friends would happily send a blanket square to you to sew together to give to him to thank him for taking care of you.

    1. I got guacamole last night and today I went back onto my immunosuppressant drug. Wow, that is so sweet to mention the blanket. I told him that I the last thing I remembered before I fell asleep in the Cath lab was someone telling me that everything would be okay, he was going to take good care of me. It was him, evidently.

  7. I’m pleased to read your update and to know that you are at long last in good hands. I loved your comeback to the nurse, too. I wonder if you can report your uptick in energy after your booster to the CDC. It would be interesting to know if your booster could be a finding that helps others. Mateo is adorable. I’m so glad you have two kitties to keep you company, and to entertain each other when you need rest. Hugs

    1. You know, there is another person who I met in the support group forums on Facebook who also had a marked decrease in symptoms after her booster. It makes me think of Long Covid and other interesting things about this pandemic like how the pneumonia looks and acts like the systemic sclerosis version of pneumonia. It was when they started treating it like an autoimmune condition (steroids!) that things got better. So interesting, but also sad. I know that after the immune response is calmed down the scarring happens. The nurse in cardiology said that the lung transplant list is really long right now.
      I don’t know what the CDC could make of this for the general population. I’m in such a small subgroup of the population. I am participating in the genetic systemic sclerosis study at 23 and Me hoping that they find something helpful.
      The kitties are the best!
      I’m feeling a little invincible right now, one week out from the booster and not yet immunosuppressed again. Thinking about putting on a mask and going on out there for a hair cut!! Take that, Covid. 🙂

      1. Take that, Covid indeed! I hope you ventured out for that haircut. I find a good shampoo and cut to be a day-maker. You feel great, you look great, and you can take that to the emotional bank. I’m glad to hear you’re part of a 23 and Me study. I hope they find something that can really help.

      2. I never did get the haircut because the weather turned really, really cold here and no haircut is worth venturing out into the subzero air. Maybe today as it is nice and we are in a meltdown. I am really hoping that the 23 and Me finds something. My scleroderma is rare, but fibrotic disease is really, really common and something that can help reverse the damage would be huge! Ironically, that is one of the huge problems with Covid and there may be some applications there.

      3. I wouldn’t be motivated to go out in the freezing cold either. We are quite spoiled here in California. Even our coldest days are mild in comparison. I’m routing for you.

      4. I got the haircut finally yesterday. They also lifted the mask mandate in my county so that is the last haircut for some time. I used to live in the San Diego area and I do miss the balmy weather on really cold days.

      5. I’m glad you got your hair cut, but I’m sorry to hear about the lifting of the mask mandate. I personally feel that it’s far too soon, and I will continue wearing mine for sometime to come. I’m sorry that other’s actions will now limit your time outside of home. Grrrrrr….

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