The Scleroderma Chronicles: World Scleroderma Day, 2022

Wow, it is that day of the year again. The Niagara Falls will turn teal, Scleroderma organizations around the world are sending out messages and videos, and patients with scleroderma like me are wondering how best to showcase our conditions in a meaningful way. Here in the US the theme is Know Scleroderma. In Australia it is Shine Like a Sunflower.

The whole idea is to educate the public about this rare disease that pretty much flies under the radar to help secure support for patients, funding for research, and awareness of treatment options. Patients are encouraged to tell their stories and to do what they can to expand scleroderma awareness in the public eye.

Well, shoot. I do that all the time! I wrote about World Scleroderma Day last year and I kind of like what I wrote. I talked about what was going on in my illness and the progress that I was making in getting diagnosed and treated for the significant organ damage that was underway in my lungs and heart. I also mentioned the similarities between Covid-19 and systemic sclerosis (the type of scleroderma that I have), and the fact that people like me are still dealing with lockdown. You can read that post here.

So, what has changed in the last year and why am I typing away on my computer once again about World Scleroderma Day? Well… awareness and support are the messages that I’ve been urged to put out, but I’ve been reflecting on what I’ve learned this year and how it might be useful to others. This was a huge year for me… I was diagnosed with pulmonary arterial hypertension, a complication of systemic sclerosis that is developed by about 15% of patients, and I was also diagnosed with interstitial lung disease, another complication of systemic sclerosis that is also developed by about 15% of patients. These serious complications develop so routinely around the 15% mark that there is now a rule of thumb about it in treating patients with systemic sclerosis. There are other conditions that fall into the 15% rule, and I have two more of them: Sjogren’s Disease and diastolic dysfunction.

MacKenzie and I from a posting a few years ago.

So, I am getting a lot of experience in dealing with being sick in a way that is not visible to the public and is not the first thing considered when you head into a doctor’s office seeking help for debilitating symptoms that have no obvious cause. I have been successful this year in becoming an active participant in my own health care and I love my team! Here are my lessons learned:

  1. Physicians tend to diagnose with the most common condition that matches your symptoms. You know, if you are struggling with fatigue, it must be depression or sleep apnea…
  2. They also tell you to stay off the internet.
  3. That works great up to a point. Get onto the internet!!!! Look up the symptoms and treatment options for the condition/illness that your physician is talking about. Do they really fit? Go ahead with the testing that your doctor orders but continue to educate yourself. Get the full text of any testing reports (those are your tests on your body, so do insist… nicely…) Look up crazy words that you don’t understand.
  4. I should include here…DON’T PANIC… about any crazy-ass, scary condition that you run into on the internet that you think you might have. I mean, what could happen? You already are sick, and you won’t get magically worse overnight once you get a name for it. You might, however, get some really helpful treatment that could turn things around for you. That is, if you have that crazy-ass, scary condition that you really, probably, don’t have. DON’T PANIC!!
  5. What if you get test results that say “you are fine” and your doctor pretty much is ready to stop there? Um… go right back to specific test results and symptoms to reset the conversation. You know, “my face is still blue, and the latest CT scan showed that I was losing tissue in my lungs. What other testing can we do to figure out what is going on?” is exactly where you should redirect the conversation.
  6. Make a list of your symptoms and track them in a journal or on a calendar. Document stuff and then contact your health care provider (email works great!) with your concerns and the symptoms that you are noticing. Specific data helps a lot, and the written record makes you more credible and your health provider more accountable.
  7. Do not let a medical health professional dismiss or disparage you. Kick them to the curb and get another. On the other hand, don’t go doctor shopping to get the diagnosis that you want; that is not productive for you or anyone else involved in your daily struggles.
  8. Ask your physicians to communicate with each other and make sure that they include your primary care physician in any messaging.
  9. Remember to be kind to others: your doctors, the nurses, your family, your friends, and yourself.
  10. DON’T PANIC!!! at any time. Remember, feeling powerless and not knowing what is happening is stressful. Learn everything that you can, do everything that you can, and then sleep well at night. Hugs to anyone who finds this meaningful.
More lavender for my garden!

There. Those are the best, most excellent lessons that I learned this year. Today I am out of the serious flare of the winter and feeling pretty darn great. I am on steroids, and my immunosuppressant drug’s dose has been doubled; I feel more like myself than I have in a couple of years. I headed out on errands this afternoon, bought a Starbucks, and signed up for a Tour de Fleece team at my local yarn shop. I bought some new lavender plants that I am going to put into the ground this evening and I am cleaning up the spinning wheel to see if I can get some paco-vicuna spun next month before my steroids get stopped.

I have some really serious conditions that carry a significant risk of a poor outcome. And yet, I feel a little like an imposter as I laugh and interact with other people that I encounter. The man at the drive-through window at Starbucks traded cat photos with me. The lady at the yarn store and I laughed and talked about spinning wheel misbehaviors; are the wheels worse if you name them? Behind the mask, I am still me, the old me; I may have scleroderma, but it doesn’t have me. I am kind of the poster child for what an invisible illness looks like, and that’s why there is this campaign today to “Know Scleroderma.”

Those serious conditions that I mentioned… they are complications of scleroderma, but they happen for other reasons, too. Some are rare, but some are not. Knowing about scleroderma can help with research efforts into these other conditions (sadly, some are now more common because of Covid long haulers), and perhaps the lessons I have learned will help others in their efforts to secure empowerment and medical treatment.

This is World Scleroderma Day.

Shine Like a Sunflower!

Hannah and the CoalBear: Caturday, 6/25/22

Hi. CoalBear here.

I’ve been entertaining the Mother of Cats all day because, frankly, she is in a slump.

Well, now that I think of it, she should be in a slump. The only day this week she was frisky was Tuesday and look at what she did to me!!

She took me to the vet!!!

People, this was the most horrible thing that has ever happened to me. I got stuffed into the carrying crate and before I knew it, I was in the car. We ended up in a strange building with other people and DOGS and I kept crying, and the Mother of Cats just ignored me and took me to a little room where I had to COME OUT OF THE CRATE!!!!! I got weighed, physically cathandled by a strange lady, and then there were the SHOTS that I absolutely did not deserve because I am the best boy ever. You want to hear the worst thing about all of this…. Hannah exercised her right to refuse and didn’t have to go the vet. I think that she was laughing when we got back home again.

Okay, back to the week. The Mother of Cats is taking lots and lots of new pills now and parts of her don’t feel good because of the side effects (let me tell you about the side effects of those SHOTS!) so I am doing my best to be cute.

She finished her little quilt and hung it up on the wall behind her knitting chair.

The Mother of Cats used clear plastic push pins to secure the tops and corners of the quilt. I have been pulling the pins out of the bottom of the quilt and taking them upstairs to play with. I left both of them on the bathroom floor where she would be sure to find them this morning so she can put them back into the quilt again. Wasn’t that good of me?

I’ve also been trying to help her with her knitting, but she seems to be in a slump. She isn’t knitting much, and the stuff she has going is… boring.

Do you see how boring this knitting is? The pink blob is a sweater… maybe. The blue is going to be a pair of socks someday, and that pastel smish of funny colors will someday be a hat. Where are the cat toys? Hello? How can I be cute and entertaining if I don’t have all the toys in the world…

Although, I think that I am pretty darn cute with the toys I have now!!!

Well, I guess that is all for now. It is almost time for the baby bunny to come out in the back yard. Show time!

Mateo the CoalBear

Notes from the Mother of Cats:

  • I did get Hannah into the crate at least three times, but she was too strong for me and managed to push her way back out before I could get it zipped closed. She has another appointment next month and I’ll try some new strategies.
  • I’m in the middle of a big drug push to get my lung disease under control; I am breathing much, much better and I have more energy by far than I did a couple of months ago. The downside is… side effects. My doctors are running frequent blood tests to monitor my progress, adding more drugs to control symptoms that are concerning, and so far, things are going well, but I am pretty much homebound with pain, blurred vision, and dizziness. Two more weeks of the steroid push to go and then I start to get tapered off. Yay! I’m dealing with a lot of tendon pain as my immunosuppressant dose is doubled, but that has happened before and I’m hopeful that there is an end in sight.
  • What type of side effects, you ask? I gained 12 pounds in two days and my blood sugar soared into the high 100s. Opps.
  • I am getting out into the garden a little and there is a new rose bush waiting for me to plant it. Someday soon, little guy.

The pictures above are my morning pills, the braces that I now am back in because of tendon pain, and my new, beautiful rose. The name of the rose is… Easy Does It!

Just the rose that I needed for the week.

Hannah and the CoalBear: It’s Caturday Again

Hi. I’m the CoalBear (AKA Mateo)

It is sooooo hot today. It is almost 100 degrees outside, and Hannah and I are staying cool in the house. The Mother of Cats has all the windows closed and we’re too hot to look out the windows anyway. Do you notice that the Mother of Cats has been brushing out all of my downy fur? I used to have a huge ruff around my neck, and now it is almost all gone. I look a whole size smaller now. I don’t like getting brushed, but today I guess I am glad that she did it. She also cuts my nails off and I will never get used to that!!

The Mother of Cats has been knitting away on her sweater this week and look at how far she’s gotten! She is done with the first sleeve and halfway down the second one already! She has also been sewing on the quilt with Hannah.

The quilt is coming right along and should be done in just a few more days. The Mother of Cats can’t wait to get it done. Hannah kind of hopes that she is going to get to keep it for her new bed, but I think that the Mother of Cats is planning to hang it up on the wall behind her knitting chair. I ripped the old quilt down a couple of days ago (Hey! There was a moth!) and she didn’t even bother to hang it back up again. Okay, the old quilt was a Christmas themed one, so maybe she’s ready to put it away now anyway. I’m such a good boy; see how I did her a big favor?

A scary man came this week and messed around with the Mother of Cat’s oxygen machine. When he left there was new stuff left behind including this new green bottle with a snazzy carrying bag. Now the Mother of Cats has oxygen she can take with her when she goes out on errands, but mostly she stays home with us because we are so cute! She has been working on her gardens this week and they are starting to look pretty good. The first rose bloomed this week which made her really happy.

It’s her first English rose of the year!
Well, that all I have. It’s still stinking hot outside and all of the animals are asleep, so I am going to take a little nap too.

See you next week!

>^..^> CoalBear

Notes from the Mother of Cats

  • The sweater is GoldenFern by Jennifer Steingass. Hopefully I will have it done in another week or so. I’m really anxious to start another sweater but so far I’m sticking to getting this one done first.
  • I know that isn’t a good shot of the quilt, but hopefully I will have a nice picture of the completed quilt to show off next week. Hanging on the wall, where I hope it remains as long as no moths land on it. 🙂
I’m listening to this audiobook while I quilt and I just am loving it!!
  • Not only did I get oxygen-to-go this week, but I also got my injections of Evusheld, which is a combination of two monoclonal antibodies that will give me protection from Covid for at least 6 months. This is huge piece of mind for me since my doctors are dialing up my immunosuppression drug dose this month and have started me on a new immunosuppressant drug at the same time. Yay, Evusheld! With the higher dose of drugs, I won’t be able to make many antibodies of my own, so I’m glad to have the Evusheld antibodies.
  • That rose is Princess Alexandra of Kent, and it is pretty much my favorite rose in the garden right now.
  • Hannah and Mateo don’t know it but they are heading to the vet for their vaccinations and checkups next week. Sad cat day is on the way…

The BioGeek Memoirs: Sunflower

Okay, I need to be complete upfront about this: this is a crossover post. It is going to be a total amalgamation of the Scleroderma Chronicles and The BioGeek Memoirs because I just couldn’t come up with anyway to make them separate posts. Hey, I’m a biogeek with scleroderma. It was bound to happen eventually…

So, let’s get this ball rolling by talking about bean plants. That makes a lot of sense, right? When I was a biology teacher struggling to make plants interesting and to help students understand experimental design, I came up with the genius idea of letting the students design an experiment looking at the effect of fertilizer concentration on the growth of bean plants. The students had solutions with different concentrations of Miracle Gro fertilizer available to them, and then they had to struggle with planting and growing 6 bean plants while holding all the other variables constant. The plants grew, the students measured their growth, and then they charted the growth to make decisions about the best fertilizer amount.

I had the hot idea of using an Excel spreadsheet to display the student data to the whole class. That worked great! I then combined the data from all 5 classes together and… it was a huge mess. The plants were all different heights depending on which class was collecting the data. The students weren’t making any errors; the bean plants were raising and lowering their leaves each day in circadian rhythm. Depending on the time of day, the plants were a different height. Oh. Plants can move!

Sunflowers have been on my mind a lot recently. Beautiful sunflowers, whose faces turn throughout the day to follow the sun. My cousin grew enormous sunflowers one year that towered over the other plants in the garden. Sunflowers are the symbol of Ukraine. The sweater that I am knitting right now is in the colors of a field of sunflowers with their faces in the sun.

Those aren’t sunflowers, but the colors remind me of all the “Support Ukraine” knitting that is going on right now.

There are enormous fields of sunflowers near the airport in Denver that are just spectacular in the late summer. Early one morning in late August,2014, I drove past them on my way to my first appointment with a rheumatologist; my primary care physician had referred me to a specialist after some concerning bloodwork results. I was pretty sure that this morning was going to be a turning point in my life, and I was nervous and kind of fighting off tears. Behind me the rising sun poured light onto the glowing faces of sunflowers ahead of me as far as I could see; the sight was just thrilling, and I settled right down. An hour later the rheumatologist explained that I had limited systemic sclerosis (a form of scleroderma) and Sjogren’s disease. I was prescribed medication, sent for more testing, and told to stay off the internet. I looked for the sunflowers as I drove home that afternoon, but I couldn’t see them; the fields were too far from me as I drove east. Still, just knowing they were there sort of helped. Sunflowers. They were kind of a symbol of hope and the promise that I could handle anything.

Are you ready for this? The sunflower has been chosen as a symbol for scleroderma by Scleroderma Australia. Shine like a Sunflower is their campaign this June to bring scleroderma into the light of awareness.

Just like that the sunflower became an international symbol for scleroderma. I swiped this shirt image off of Amazon.

Why a sunflower? Well, like sunflowers, we scleroderma people follow the sun. Strong sunlight is actually a problem, but the warmth… bring on the warmth! For the last few weeks, I have been recovering from surgery and waiting for my biopsy results. I have been sitting outside on my deck out of the direct sun, soaking up the heat and light. Day by day, I have been improving and no longer need daytime oxygen support. My cardiologist has restarted the medication that was halted while I was in the hospital, and it hasn’t even caused a bump in my recovery. Heat and sunlight are really making a difference.

My biopsy results arrived on the first day of June. I have developed a type of interstitial lung disease that presents as hypersensitivity pneumonia. I also have the characteristics of what the report called a vascular/collagen autoimmune disease, which is pretty much a descriptor for scleroderma. Yep. What my pulmonologist prepared me for. This is interstitial lung disease associated with system sclerosis (SSc-ILD) and I am going to get started on an increased dosage of immunosuppressants and a new drug to prevent scarring in my lungs called OFEV. This drug is really new; it has been developed in the years since my diagnosis, and now it is here just when I need it.

June is Scleroderma Awareness Month. Here in the US the theme of the campaign is Know Scleroderma. Oh, I know scleroderma, and so do some of you through my blog. Let’s put scleroderma aside for the time being and go back to sunflowers. And science. Remember that this post started with a little story about doing a science experiment with bean plants and my students? As simple as that was in my classroom, the heart of that process, curiosity, scientific experimentation, and data manipulation, is serving me well now. Ironically, new therapies and treatment approaches are being developed because of the lung scarring caused by Covid-19. Science. It rocks!

Today I planted these sunflowers along my side fence.

This afternoon I am once again outside in the warmth and light, knitting on my new sweater in the colors of sunflowers against the sky, admiring my beautiful newly planted sunflowers. They have their little faces angled to the southwest, following the sun as it starts to dip towards the Rocky Mountains.

Beautiful, tough, follow-the-sun sunflowers, reminding me to also follow the sun and to shine when I can. They remain a symbol of hope and a promise that I can handle anything.

Shine like a Sunflower.

June is Scleroderma Awareness Month. You can learn more about scleroderma at these links.

The Scleroderma Chronicles: Lung Biopsy Story

Last Monday, May 2nd, was the date of my lung biopsy procedure. My pulmonologist had diagnosed me with interstitial lung disease a couple of months ago and the biopsy was required to definitively diagnose the type of ILD I had. This is kind of complicated, but the simple reason for the biopsy is that I don’t really fit the profile of the usual scleroderma ILD patient, and the treatment is expensive and somewhat risky. Biopsy time.

At 4:30am Monday morning my son drove me through the rainy dark to the hospital where I was going to be admitted for the surgery. I had on my new raspberry clogs for good luck, and I was exhausted after being up most of the night completing pre-op tasks at home. I was fighting off a sense of impending disaster…

Image retrieved online from Wikipedia Commons at https://en.wikipedia.org/wiki/Respiratory_failure

The day I met with the surgeon he drew a funky little drawing on his white board to explain what he was going to do. Check out the diagram above: your right lung is actually different from the left with more tissue and three lobes. My surgeon planned to use special instruments guided by a tiny video camera (VATS) to go through my chest wall and get the tissue samples. This is a minimally invasive procedure that will only take a few minutes. The catch: the right lung will be deflated before he takes the samples.

Atelectasis is the medical term for a collapsed lung. Both of my lungs were experiencing incomplete atelectasis at the time of my last CT scan, and I’d been getting steadily worse all April. I was a little concerned, but I trusted that all would work out fine in the end. I was prepped, bundled up, and rolled off to surgery. There was the most fantastical robotic surgery machine in there, but before I even got a great look at it, I was on the table, a mask was put over my face and I was gone…

… and I emerged from unconsciousness at the bottom of a rugby scrum with all these faces looking down at me. I was in pain, a lot of pain, and I couldn’t breathe at all. The muscles on the right side of my back were seized up and cramped in what felt like Charlie horses, and I was thrashing around as I tried to get someone to rub on my back muscles. The rugby players in masks (I was later told that there were 8 of them) were attempting to hold me down as they put hot packs under my back, removed my oxygen cannula, and placed a larger oxygen mask over my face. “Do you remember the Xray?” one of them asked. Oh. That’s what set off the muscle cramps. My oxygen was below 75% even on highest oxygen flow available in the recovery room. I got transferred to the ICU.

That right lung refused to reinflate. I couldn’t be given any pain medications or fluids until my oxygen levels came up. New doctors began to arrive and talked to me. To be clear, talking set off coughing and was very painful. I just wanted someone to help me, not talk to me! A pulmonologist arrived to doctorsplain my disease to me and informed me that he was changing some of the drugs used to treat my lung and heart conditions. I told him that I didn’t know him, he couldn’t make any changes to my treatment plan without talking to my doctors, and that I needed him to do something right away to handle my immediate situation (I was in acute respiratory failure). He argued about contacting my doctors. I insisted.

I think that I deserve a huge gold star for standing up for myself while in extremis.

I was placed onto a high flow oxygen therapy machine within minutes and my oxygen levels came up.

I kind of look like I was underneath a rugby scrum, huh. That machine delivers heated water vapor and oxygen at the unbelievable rate of 60 liters/minutes. My oxygen came up into the low 90s on the machine and I finally received oxycodone. Yay!!! The last oxygen number on the monitor before I fell asleep was 94%.

The next day they got me up into a chair (more oxycodone!) and I started respiratory therapy to get the lung working again. On Wednesday my chest tube came out and during the day the flow rate on the machine was reduced until I could come off it.

Day three in the ICU. I am off the high flow machine and finally using a normal cannula. I’m still on 15 liters/minute of oxygen at this point.

That annoying pulmonologist came by every single day that I was in the ICU; my doctors had called him back! After talking to them he made some med changes (and told me that it a shared decision) and his manner completely transformed. The physician’s assistant who removed my chest tube told me that she had also read the care notes with all the data, email traffic, and decision-making by my rheumatologist/pulmonologist/cardiologist team. She was struck by the interdisciplinary care that I was receiving and seemed a little wistful and envious.

Another gold star for the team!!

Over the next two days I was slowly titrated down on my oxygen while doing my lung expanding exercises without fail. Late in the morning on Friday I finally escaped.

I put on my raspberry clogs, black leggings, a little black top, and my raspberry-colored down vest. “How cute you are!” exclaimed the nurse. My son drove me home through the late morning light along streets lined with newly leafed trees. While I was in the ICU the world had turned green.

No matter how lifeless and barren things seem over the winter, spring always comes with the promise of fresh starts and new life. I still trust that all will work out fine in the end.

Hannah is so glad to have me home again.

Now we just have to wait for the biopsy results.

The Scleroderma Chronicles: It’s okay if you cry…

My fatigue lately has been off the charts. I struggle to get the simplest of tasks done, and to be honest I just don’t feel like getting out of bed for days on end. I have been slowly, slowly sewing on a quilt top over the last few weeks. It is soooo exhausting to pin two fabrics together, guide the fabric through the sewing machine, and then to stand up to iron the seam. I handled all of this by 1) sewing only for an hour a day, and 2) lowering the ironing board so I didn’t have to stand while ironing. Take that, you nasty, exhausting fatigue!!

The quilt has a panel in the middle with really cute pictures like this one. Super cute, right?
Here’s the finished quilt top. This baby has really simple quilting in one border and then simple, simple borders around the central panel. Yeah. This took over two weeks to complete. Thanks, fatigue.

When I noticed some strange terminology on my last heart imaging test report, I contacted my pulmonologist about it, and he ordered a CT scan of my lungs. (You can read about that adventure here.) I knew that something was up when I got a call from his office telling me the date and time of the earliest possible appointment with this doctor. The nurse had intervened and made the appointment for me ahead of time. Then there was a call from the cardiologist’s nurse that was the same; an echocardiogram and appointment with that doctor had also been scheduled for me in order to secure the earliest possible appointment. Kind of the harbinger of a tough appointment, right?

Today I had a pulmonary function test and met with the pulmonologist soon afterwards. We joked about the horrible year we had both had. (He is a pulmonary critical care specialist who has been on the front lines of Covid care for two years now; for me lockdown never ended and the BLZ was running wild.) We laughed at my summation of the year: Crushed by Covid. We decided that “Crushed by Covid” could be the name of a really sad band. Then he whipped out his laptop and had me move over to look at it with him.

He had prepared for my appointment with a spreadsheet of my lung function tests over time and my latest lung scan along with that of a normal person. The spreadsheet showed that I was losing volume in my lungs. The scan of a normal lung was really interesting (old biology teacher here…) and then we looked at mine.

Um… my lungs were really cloudy. Like frosted glass. Like… “Hey. Is that what they call ground glass lung?” I asked.

“Yes. That is exactly what we’re looking at. That’s why I wanted you to come in. This isn’t the type of conversation that you have over the phone,” he replied.

Ground glass is not good, folks. Ground glass is the type of lung imaging that Covid patients with pneumonia have. Covid presents like systemic sclerosis because there is an extreme immune response going on in the lungs; both are aggressively treated with drugs that target the immune system. I also have some honeycombing that is the beginning of fibrosis; first the inflammation (which creates the ground glass appearance), then the fibrosis follows. My ground glass lungs are, in his opinion, absolutely not Covid. It is not likely to be just pulmonary edema. It’s systemic sclerosis at its worst. This is interstitial lung disease.

So, it is not good. On the other hand, this is good. I’m in trouble, but the problem has been identified early on and that means aggressive treatment now may stave off the worst of the fibrosis. I am so glad (and lucky) that I googled those crazy medical terms and then followed through with an email to my pulmonologist.

I’ve been referred to a surgeon for a lung biopsy procedure. Evidently that will land me in the hospital for a few days. Following that, if things go to plan, I will be started on more aggressive immunosuppressive drugs. He is going to talk to my rheumatologist about starting a course of chemo and an anti-fibrotic drug. One of the last things that my pulmonologist said to me was, “It’s okay to cry about this, but we have a plan.” That’s when it hit me that this might be really bad; lockdown will continue, and the fatigue is probably going to get worse. Oxygen 24/7 is right around the corner.

Crushed by Covid plays on. What a sad little band it is.

Luckily for me and the cats it is squirrel season. They will have lots of entertainment while I’m in the hospital and laying around like a slug.

The Scleroderma Chronicles: The shunt hunt takes a left turn…

I’ve been continuing my adventures in cardiology over the last several weeks. If you have been following along on my scleroderma adventures you know that I had a trip to the Cath lab that led to the discovery of a cardiac shunt: a hole in my heart. I also was eventually diagnosed with exercise-induced pulmonary hypertension and started on drugs to treat it, which is a lengthy process as I was slowly titered up on two different drugs while monitoring for side effects. I’ve been mostly living in bed for the last 6 weeks except for short trips out for more testing and blood work. The cats have been loving this, by the way. I’m kind of their captive right now.

This is edema in my arm. I’ve been dealing with headaches, muscle pain, edema, low blood pressure, and extreme fatigue. I cough a lot. Every new weather system is a nightmare. Ugh!

While the whole process has been pretty difficult, I am breathing much, much better and that blue lipped thing has mostly faded away. No more panting!! I haven’t had to put my head down because I felt faint for weeks. This is huge, people!!

I may have to retire the whole BLZ logo the way things are going!

My cardiologist is still hunting for the shunt that was detected in the Cath lab. I have one that they can see (a patent foramen ovale, which is pretty common), but for the really significant disruption of circulation that was detected in the Cath lab the feeling is that I have something much bigger somewhere. I’ve gone through 3 rounds of testing looking for the dang thing, and so far, no joy.

When the test results come in, I always read the entire text and google terms that I don’t recognize. The last imaging of my heart did not find the shunt, and my cardiologist sent an email letting me know that my heart looked pretty good. Umm… okay, but where is that infernal shunt?!!! This is getting a little frustrating, but I am doing better, so I guess I should just roll with it. I did notice this little sentence in the report about the portion of my lungs seen in the heart imaging: “There is mild subpleural reticulation and bibasilar atelectasis.” Say what? I googled and …. bibasilar atelectasis is a partially collapsed lung. I shot off a little email to my pulmonologist to ask if this was something new.

This is why I decided to write this post. As it turns out, this is new. Both of the things that were noted in that test result were significant (subpleural reticulation is evidence of scarring in my lung), and I was immediately sent to get a specialized lung scan that shows I have sustained moderate advancing lung damage over the last 10 months. Oh. No wonder I’m so exhausted. At least they didn’t use the word “severe” in the report. I seem to have developed pulmonary edema and my lungs took a big hit during the last few months; scleroderma is now attacking my lungs. If I hadn’t read that report and then contacted my doctor, no one would have picked up on this. The BLZ may be on hiatus, but the lessons she learned during that drive for the pulmonary hypertension diagnosis really paid off now.

What do you do when you get a sad little lung report? Why, you put on your Catzilla shirt and go start a load of laundry, of course!!

Tuesday I go for a pulmonary function test and then immediately afterwards I will meet with my pulmonologist. I’m kind of thinking that there might be more drugs in my future. Anyway, there is a lesson here that I decided I should share with you all.

Be proactive! Read your test results and ask questions of your doctors. Google is your friend, and those online portals that let you shoot your doctor an email are priceless! Use them!

And now readers, back to the shunt hunt…

Mateo: and now readers, back to my nap! After that I’m going to go swat some more helicopters!

The Scleroderma Chronicles: Rare Disease Day, 2022

Well, this is a hard topic to write about. Quite frankly, I have been getting my butt kicked lately by my (wait for it) rare diseases. Still, I am trying to respond to the calls for publicity about rare diseases along with other members of the scleroderma and pulmonary hypertension communities.

You know, I feel like I should represent.

People with rare diseases are referred to as “zebras” in the medical community.

So, what’s a rare disease? A rare disease is classified as one that impacts a small percentage of the total population. Here in the United States that means fewer than 200,000 people diagnosed with the condition/disease. Perversely, there are a lot of people with rare diseases as there are almost 7,000 different rare diseases!  Some of these diseases are common enough that you may be familiar with them: albinism, achondroplasia (a type of dwarfism), and autoimmune hepatitis are examples. Others are very rare. Most are genetic in origin, and half of them impact children. More than 90% of rare conditions have no drug treatment.

The type of scleroderma that I have, limited systemic sclerosis, is considered rare as there are about 100,000 people in the US with this diagnosis. The latest diagnosis added to my medical history is of pulmonary arterial hypertension, another rare disease, and one that is a consequence of my scleroderma. Well, I am really rare now! I have struggled to explain my scleroderma to people when they ask; how can I explain in just a few sentences something that is just frankly causing horrific damage to my body and generating an ever-growing list of diagnosed conditions. Here’s my best answer at the moment:

Scleroderma is a chronic, progressive, uncurable, and often fatal autoimmune disease that causes scaring and damage to blood vessels, skin, internal organs, and muscles/joints. It is controlled and treated through the use of immunosuppressants and drugs that address symptoms. It is a life-altering diagnosis. It is my life.

So, I have blogged about Rare Disease Day several times in the past. Here’s what I wrote a couple of years ago, and what I wrote in 2018. In the past I have written about my symptoms and the struggle of living with a rare disease. It is pretty isolating. It is hard to get diagnosed and treated. I have also written about the difficulties to get funding for research for rare diseases and conditions, and the lack of treatments and cures because the patient population is so small.

This year I thought I would share some of the things that doctors have said to me since my scleroderma diagnosis. I’ve tried to organize these into chronological order to better reflect my journey.

  • My internist: It’s good to have a diagnosis, even if it is a shame.
  • My rheumatologist when I asked him what my life would be like in 5 years: Let me run some more tests, and then we can talk.
  • My ophthalmologist: Do you have a will?
  • The physician at the regional acute diagnostic center: This is a diagnosis like cancer. Of course, some cancers can be cured.
  • A physician speaker at a scleroderma support group presentation: this drug [the immunosuppressant that I take] can really give you a chance, as long as you don’t contract an infection.
  • My old rheumatologist: All you do is complain. Maybe I should order a sleep apnea test or prescribe antidepressants.
  • My internist, as I begged for an anti-inflammatory drug: I’m sorry. There isn’t anything that I can give you that won’t hurt your kidneys.
  • My dermatologist, as she prescribed an anti-inflammatory topical gel: This is unacceptable, and I am putting a stop to it now.
  • My new rheumatologist as she orders more testing on my painful joints: Why has no one followed up on this?
  • My rheumatologist two days later: You need to get a steroid injection in your hip joint as soon as possible.
  • The hip specialist: There is nothing more that I can do for you because your scleroderma is attacking all of your tendons and ligaments. You need a hip replacement, but it will fail.
  • The physician at urgent care: I don’t think they understood how complicated your medical status is when you were referred here. You need to be hospitalized because we can’t do the testing that you need here.
  • My pulmonologist as he walked me back to the waiting room: I really admire your attitude.
  • My cardiologist as I was being sedated for a right heart cath: Don’t worry. I’ll take good care of you.
  • My cardiologist as he started me on medication for pulmonary arterial hypertension: This is challenging, and we will need to be comfortable with “out of the box” thinking.
  • My rheumatologist last week, referring to herself, the cardiologist and the pulmonologist: We are your team!
  • My rheumatologist, also last week: We need to add a gastroenterologist to the team.

You can see how rocky the start was. There is a lesson here, I think. To be rare, to be a zebra in a medical community that is designed to identify the most likely cause of symptoms in a herd of horses, is hard. It is really challenging to secure the care that you need when, no matter how hard doctors try, you do not respond to the usual treatments, and you never fit the usual profile. It is easy to be seen as a problem. It is hard to keep insisting that there is something wrong when all the test results say you are okay.

Even when you are blue-lipped and panting it can be hard to convince doctors that there is a problem.

And yet, it is possible to get there. Over time, with great determination and persistence, I have A TEAM of doctors who view themselves as active collaborators in my care. They message each other to discuss test results and possible drug interactions, and they loop me into their discussions. It is only now, newly diagnosed with a terminal condition, that I feel confident and hopeful about my care.

Today I went in for a blood draw and a little jaunt through the local bookstore. The sun was shining, I bought a Starbucks coffee, and it was a good day.

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My scleroderma-related diagnoses:

  • GI tract: difficulty swallowing, hiatal hernia, GERD, gastroparesis, chronic gastritis.
  • Kidney: stage 3 chronic kidney disease.
  • Lungs: pulmonary arterial hypertension, interstitial lung disease, asthma, partial lung collapse.
  • Heart and circulatory system: grade 2 diastolic dysfunction (a type of heart failure), Raynaud’s phenomenon, telangiectasia.
  • Muscle/Skeletal: fibromyalgia and severe joint damage.

This is scleroderma. I’m a zebra, and these are my stripes.

Happy Rare Disease Day, everyone!

The colors associated with my diseases are teal (scleroderma), purple (Sjogren’s), and periwinkle (PAH). It sounds like the start of a great sweater, huh. 🙂

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!

Hannah and the CoalBear: Mateo does Caturday.

Hi. I’m Mateo (AKA the CoalBear).

I’m almost 10 months old now. What do you think of my ruff?

The Mother of Cats isn’t feeling well today so I am hanging around with her on her bed. I’m helping her write on her computer right now! I’m such a good boy!

I’ve been bringing toys onto the bed so she can watch me play with them.
I chewed on the palm plant downstairs to keep up my energy.
I then helped the Mother of Cats with her knitting. I’m such a great helper!!
I groomed my sister so she would let me sleep on the cat tree with her.
I’m the best CoalBear ever!!

Happy Caturday everyone!!

May you all have an excellent day!

Notes from the Mother of Cats:

I’ve gone off my immunosuppressant drug so that I can get my Covid booster on Monday. Sigh. There was a big pressure change yesterday with a cold front that triggered some symptoms along with the predictable flare of my disease that has me back in bed for the weekend.

I’ve been in lockdown for two years and I desperately want the booster because my next round of medical appointments starts in another week. I also want to start knitting with my new-found groups, too. I want some of my life back!!

Please think of me and the other people in a situation like my own (high risk, immunosuppressed or immunocompromised, and struggling with chronic conditions that complicates their lives on the best of days) when you are out in public.

And wear your mask!

ps: I threw out the palm last night. It wasn’t a match for the CoalBear and I was afraid it would make him sick. Next up: a Boston fern.