The Scleroderma Chronicles: Rolling Dice with the Reaper

I’ve been collecting new diagnoses over the last year like an out-of-control yarnaholic shopper at a fiber festival. Yeah, that bad. That’s how my yarn stash got so out of control… I mean, you never know when you will need that fabulous color in the future, it is one-of-a-kind, and it’s cashmere… Anyway, let’s get back to the topic at hand. The last year has been really eventful, and the new tests, diagnoses, and drug interventions keep rolling in. Here’s the chronicle of events:

  • An early summer echocardiogram last year showed that I had developed a type of heart failure called diastolic dysfunction. Scleroderma is causing scar tissue to form in the muscle of my heart.
  • On September 1st I had a right heart catherization that showed that I had a hole in my heart that was disrupting the flow of oxygenated blood to my body. Oh. That explains those blue lips I’d been sporting around town.
Remember the BLZ?
  • Follow-up testing showed that I had exercise-induced pulmonary arterial hypertension. I was started on drugs to treat the condition.
  • I went into a flare of my systemic sclerosis (scleroderma) symptoms because of the increased blood flow due to the drugs.
  • Testing in February to hunt for the cardiac shunt (hole in my heart) revealed that there was something going on with my lungs. A subsequent CT scan in March showed that I had developed autoimmune pneumonia in both lungs, a condition called interstitial lung disease. The cause of the ILD was confirmed by a lung biopsy in May. Yep. It is scleroderma related, but I was still forced to get rid of all my down products in the house. I miss my down comforter…
  • All summer long I have gone through a staggered course of drug interventions as my doctors worked to get my lung disease under control.
This sunflower is a pretty good metaphor for how I’m doing at the moment. I’ve taken a beating, but I’m still blooming, by golly!

So, I’ve been taking a lot of drugs, and all of them carry some side effects. I became dizzy from one, my blood sugar zoomed up, and my vision became foggy. Another is causing hot flashes. One of them causes muscle pain, and a couple of them are seriously interfering with my sleep. Two drugs cause edema, so I’m taking another drug to combat that. Two weeks ago, I started the last drug on the list, Ofev, which has a list of side effects that made me pause a few days before taking the first pill.

Nausea, diarrhea, vomiting… well, okay. That’s stuff that I deal with all the time because of scleroderma…

Liver damage, heart attack, stroke… say, what? Seriously?

I had to go through a 30-minute phone interview with a pharmacist before the first 30-day shipment of the drug could be sent to me. I will need to get blood work done every single month to check my kidneys and liver before I can receive the next 30-day shipment. I need to be vigilant about watching for bruising and the symptoms of blood clots, including heart attack and stroke symptoms. One of my sons is checking in on me daily.

Time for a baby bunny picture, don’t you think? One of the best parts of the summer. 🙂

So, here’s the deal. I’m in a lot of online support groups where people are afraid to take drugs to treat their systemic sclerosis (scleroderma) because of the side effects. We live in a world where people are afraid to get vaccinated because of possible side effects. We live in a world where people are resistant to wearing a mask because of… well, they will tell you. They have more reasons than I have time to list, quite frankly.

This is kind of nuts, in my opinion. Did you see that list of diagnosed conditions? Yikes. Not good, little BLZ, not good. I’m facing down some really serious stuff here, and I will take these drugs, get through the side effects, and fight this like the hail-battered and grasshopper-savaged sunflower that I am!

Two weeks ago, I gulped down the first Ofev capsule. All those nasty GI symptoms arrived and had their way with me for several days, and then I was through it. I’m now on the final doses of all my drugs, and the sleep disruption is improving, the dizziness is gone, and the muscle pain is receding. I’ve learned to never, ever drink elderberry juice, and that green chili actually improves GI inflammation. I can do this, yes, I can!!

My niece referred to my battles with my disease and the drugs that I take as “rolling dice with the reaper”. I guess that is a one way to look at it. The other way is… you have to play to win. I chose to take the drugs; I chose to play. Hand over those dice, reaper!!

Last week I went to get my first follow-up CT scan of my lungs. “You can take off your mask, if you’d like,” said the technician. Nope! No one with a list of diagnosed conditions like mine should take off their mask in a diagnostic facility attached to an urgent care center. I roll more dice than I should as it is! Grumpy, the technician did my CT scan while I kept my mask on.

The message from my pulmonologist came last night. My lungs show improvement, and there is no new fibrosis.

Take that, reaper! I win this roll!!

Scleroderma: Shine like a Sunflower!

The Scleroderma Chronicles: Unforeseen Circumstances and Unintended Consequences

I guess almost everyone in the world knows by now that the United States Supreme Court issued a ruling last month (June 24th) that overturned the constitutional right for a woman in the United States to seek an abortion at any time during the first two trimesters of pregnancy.

This post isn’t really about abortions, okay. This is about the unbelievable unintended consequences of that decision for women who are dealing with unforeseen circumstances. Women who are dealing with medical conditions that they never saw coming, and that they absolutely did not bring upon themselves.

Like autoimmune disease. Like cancer. Like any one of a number of medical conditions that require serious, high-risk medical interventions by the medical professionals who are treating that woman.

My first inkling that there might be a problem with the Supreme Court ruling that was much larger than what was being reported in the press happened the evening of June 29. A woman in one of my autoimmune online support groups posted that she saw a message from a doctor who had a patient reporting that she couldn’t get her methotrexate prescription renewed. I literally couldn’t sleep that night I was so upset. In the days that followed there were more reports, and this is really happening to some women. They are being denied refilling of their methotrexate prescriptions. This drug is a common one used to treat autoimmune diseases like rheumatoid arthritis, lupus, and scleroderma. It is a chemo drug, it can harm an unborn child, and it can also be used to induce an abortion. Here is an article on Health.com, another at msn Everyday Health, and this one from Time.com.

My morning pills.

There are some serious drugs there in my hand. The green pills, Myfortic, can harm an unborn child by causing birth defects or a miscarriage. I take 6 of those every day. The white capsule, omeprazole, should only be used “only if the benefit outweighs the risk to the fetus”. I have to take two of those each day. That little pink pill towards the upper left is the really big problem in my hand. That drug, ambrisentan, required a rigorous enrollment process with paperwork from my cardiologist and myself along with two interviews by pharmacists. I had to prove that I could not get pregnant to avoid taking a pregnancy test prior to each month’s supply of the drug being shipped to me. Even with all of that, I am screened each month before the next month’s supply is overnight express shipped to me. This drug poses a serious risk to a fetus. In my support groups for pulmonary hypertension there are women who are reporting that they are getting dropped from the programs that allow them to get the drug.

I’m okay because I’m beyond childbearing age. Many other women who are dealing with autoimmune diseases that require drugs such as these are of childbearing age. Why? Because the people in the population who are at the greatest risk of developing an autoimmune disease are women of childbearing age, that’s why. Because of the recent decision about access to abortion these women are also now at possible risk of being denied treatment for their autoimmune disease, or in the worse-case scenario, access to an abortion in consultation with their medical professionals.

I literally couldn’t sleep when I read the first reports of the problems with access to methotrexate in my online forums. This is absolutely unbelievable. This is unbearable. How could this be happening to a population of desperate woman, through no fault of their own, who are in this horrible situation? Right now, methotrexate is being targeted, but there are all these other drugs that are used for so many patients with so many conditions. Women, absolutely, are at risk of receiving inferior health care in the US.

This is awful. Have a rose. Right now, I need a rose. And a hug.

This is hitting me hard this evening because I have been dealing with autoimmune pneumonia since last December. My doctors are trying to get it under control with high dose immunosuppressants, but I am coming off one of them right now and I am struggling with shortness of breath and chest pain once again. I’m back on daytime oxygen and I’m feeling a little low. The lung biopsy was the first engagement with my interstitial lung disease, this current drug strategy the second battle, and the third battle is looming on the horizon. This isn’t a disease; it is a war. I never asked for this, and yet, here I am trapped in this unforeseen circumstance, engaged with an uncurable foe. Without the drugs in my hand above, I would already be gone. Yay, science!!

I think that there is another CT scan in my near future, and then there is a possibility that my doctors will move to the Plan B (see what I did there) that they have already discussed with me. Cyclophosphamide (a chemo drug) and OFEV (an anti-fibrotic drug) are on deck if my doctors decide to escalate my care. Both of these drugs can harm an unborn child. I will get access to these drugs. If I was in my 30s, as many other members of my support groups are, it might be a different story. What about women in other support groups that I don’t belong to who just got diagnosed with cancer or an equally serious disease who also need medical treatment with high-risk drugs such as these? Women of childbearing age. Women who may already have children who they need to think of and care for in their health care journey.

Being diagnosed with an autoimmune disease like the ones that I have (systemic sclerosis and Sjogren’s disease) is a punch to the gut. Being denied access to treatment because of your childbearing status is an unbearable second blow. Becoming pregnant while on one of these drugs would place a woman in an impossible situation. Nothing, NOTHING, about this situation is good. Where is the privacy for these women? Where are the HIPPA protections that they are entitled to? The decisions that these women face are absolutely heartbreaking, morally complicated, and ethically challenging. They deserve privacy as they make them along with their health care providers, religious advisors, and families.

This post isn’t about abortion, not really. This post is about unforeseen circumstances and unintended consequences.

I feel a lot better for having gotten this out.

Peace be with you all.

Take this rose with you as you go.

But if you decide to share this post, write a congressperson, join a march, or take some other action to ensure that women have equal access to health care, that would be great.

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.

Those Crazy Days of April, 2022

April went by in a blur for me. I entered the month feeling pretty sick. I had just had an appointment with my pulmonologist, and he had referred me to a surgeon for a lung biopsy. Well, not what I was looking for, but necessary to definitively diagnose what type of interstitial lung disease I had. There are several types, and the treatment plans for each are different. The surgery was set for May 2nd, an entire month away. Annoyed, upset by the wait, pretty darn sick, it was hard to wait all through April.

April rose to the challenge. Never was there such an ill-behaved month. For some bizarre reason my Amazon account reset to Spanish. Do you know how hard it is track orders and make changes to your account settings in a language that you don’t read well? Then email in Italian began arriving. Someone tried to hack into my PayPal account, setting off a flurry of new password and security settings on my part. Then my Facebook feed was flooded with pictures of… owls.

Umm… the universe wants me to learn a new language? Maybe the next BioGeek post should be about owls? This is really strange. April was getting off to a rocky start…

I kept struggling with my health as the month advanced, taking care of essential items in preparation for months of difficulty ahead if I was moved into a 6-month course of chemo. I filed my taxes. I took Matao in for his vaccinations. I filed the paperwork to be excused from jury duty. I got the last vaccine I needed (shingles) before the surgery. A neighbor installed the new outdoor lighting that I had purchased before Christmas, and another neighbor will arrange with her grandkids to take care of my lawn. I ordered more oxygen cannulas, and in keeping with April craziness, 4 cases of tubing arrived instead. I made arrangements to be tested for a portable oxygen unit… first appointment is in June. April, you are killing me here! One of the side windows of the car shattered without warning. I continued to get sicker. I was sick and tired of April, too!

My wild bunny moved into the front yard where I could see it each time I went out of the garage. Very used to me now, it never runs away when I go outside. Bunny support on sad days.

As the month wore on, I began to sleep a lot. Like 10-12 hours a night. I had to use oxygen during the daytime. I was losing weight. I wrote my doctors an email towards the middle of the month telling them that I felt that I was declining, and that I was worried it was taking too long to begin treatment. I was scheduled for a series of additional tests and had appointments with the pulmonologist and the cardiologist. Oh, good, April. Now I have to drive all over town without portable oxygen.

How to respond to a worsening situation that I have no control over? I bought happy new shoes in an outrageous color.

These are the shoes that are going to walk me into the hospital!!

And then I cast on new socks that would do the shoes proud.

This yarn’s name is “Squad Goals” and dyed by Hue Loco.

The results from the heart, lung, and kidney testing came back and they all showed that… I was getting worse. My doctors conferenced back and forth, debated different treatment modifications, and came to the consensus opinion that I should have the biopsy done May 2nd. I got that email late on Friday before the surgery. Checking the physician notes attached to my last appointment I found the full text of all the test results and the email chain of discussion between my doctors. I felt very lucky: what a great team! How good I feel that the decisions about drugs and treatment is being arrived at through this interdisciplinary collaboration by my doctors, who have chosen to make this all visible to me. Knowing that, however, did not change the fact that the upcoming surgery is now a little risky.

So, I cleaned the house, did laundry, paid the bills, and got in groceries. I planted flowers in the front tubs and along the walk. I also cast on some baby booties for my next-door neighbor who just brought home a new little one. This pattern, extremely versatile, is our family recipe passed down through three generations over almost three quarters of a century. It is the same as this one posted on Ravelry.

And just like that, the month was done. I had made 2 hats, 4 PICC line covers, 2 pairs of socks, and used up 3.38 skeins of yarn. I read 6 books. I did work on a sweater, but since I’m now in the colorwork portion of the knitting I put it on hiatus to await better days.

Goodbye April, you crazy, bad-boy of a month! I want you to stand in the corner and think about what you have done!! I expect a whole new attitude when I see you next year.

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!