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.

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.

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.

Goodbye 2021: Year’s End

This is the last update for the year 2021. You know, 2020 was a pretty bad year for both me and the world, but 2021 just plain outdid itself. THIS HAS BEEN A HORRIBLE YEAR!!!! Seriously, I have been reflecting on all the horribleness of the year, and it is multilayered in the just plain awfulness of it. Here is some of the angst, anger, and sadness in a nutshell.

If you aren’t wearing a mask these days, shame on you!! I have been in lockdown for two stinking years (!) and I am over all your anti-public health nonsense.

If you are one of the doctors who told me that there wasn’t anything that you could do for me (and to not come back), or that I had sleep apnea, or that I needed to exercise more, or that my tests showed that I was fine and you would just continue to monitor my symptoms… shame on you!!! I finally received credible diagnoses this fall (from new doctors) that explained my symptoms. You know, the ones that you ignored or dismissed all those times I came to get help… It is good to get diagnosed, but in this case it is also a mixed blessing as my condition cannot be reversed and management is going to be difficult. Shame. On. You!!!

If you are one of the people who believe that the election in the US was stolen and that the answer is to impede the ability of American citizens to vote, or to create a provision that allows the legislatures of some states to just overturn the results of elections, or to resort to violence… shame on you!!!!

Did you notice that the number of exclamation marks kept growing? That’s how much shame is attached to those targets. There, I got that off my chest and let’s hope that things go a little better in the coming year.

True to its rotten black heart 2021 went out in absolutely dreadful and ironic fashion. First the dreadful.

We are in an extreme drought where I live and everything is brown. The grass, the fields, the bushes and even some of the evergreen trees are now brown.

Last Thursday, December 30th, we had a high wind event in my state of Colorado as a major weather front pushed its way towards us over the Rocky Mountains. In the area around Boulder, Colorado the gusts were extreme (one was clocked at 115 mph) and the sustained winds through the day were around 75 mph. Several fires started and swept through dried fields and brush towards housing developments and towns with horrifying speed. There was nothing that could be done as subdivisions, stores, and hospitals were evacuated; the fire crews set up base in the parking lot of a mall where they could best defend themselves while waiting for a break in the wind. No joy there throughout the afternoon and early evening. There were heartbreaking scenes of burning homes by others covered in Christmas lights. People parked along the major freeway hoping to see if their home was still standing. The historic downtown area of a town I have frequented in the past was lost.

At around 3pm I realized that the worst fire was near my son’s home in northern Westminster. As the evacuation zone continued to grow to within a mile of him we started to make plans to get him and his pets out safely. It was a nightmare as firetruck after firetruck rushed north towards the fire line in the night past his windows. By midnight the winds had died down, the fire stopped its spread to the south, and my son was safe. Daylight the next day showed that the damage was just horrific. Almost 1,000 homes have been lost, thousands are displaced, and the hunt for the missing is ongoing. There is information about all of this here.

Then the storm arrived on New Year’s Eve.

In a cruel twist of irony, after failing to deliver any real snow all fall and early winter, the winter storm arrived New Year’s Eve with snow, icy roads, and bitter cold. Like, we started the day at 3 degrees Fahrenheit yesterday. Serious, serious cold after weeks of warm sunny weather. All those poor people who escaped with only the clothes on their backs in the wind/fire event now have to deal with this. Bad 2021, bad!!

And that, thankfully, was the end of the year.

I also finished my Kevat sweater in the final days of the year.

It is wet and blocking in this photo, but not finished. See the loose ends of yarn?

I did finish the sweater with the ribbing and I-cord to make nice open and clean edges. I decided to block and try on the sweater again before knitting on a little lace edging onto the bottom; if the length is exactly right there won’t be any lace added. I did do a little math, however, and I do have exactly the right number of stitches to do the lace. It’s a sign, right?

Mateo: Happy New Year

Hannah and Mateo (AKA the Coalbear) and I all wish you all a Happy New Year. May things take a turn for the better with the coming weeks and months.

And 2022, you had better behave yourself or I’ll be sending you out on a walk of shame, too. I do have to warn you, however, that I am a little concerned with how you are starting out…

Because this year we are going to learn how to cope with a million new Covid cases a day in the US, and I am going to deal with this whole broken heart thing that I have going on, and we are going to f*cking save democracy. Shape up quick 2022, because this is the tough time, and you had better show some backbone, because I expect you to fight like you really mean it for the things that are really important.

The Scleroderma Chronicles: The Blue-Lipped Zebra Gets Her Diagnosis!

At last. I mean, this has been going on for more than 5 years and had reached the point of utter absurdity. If you haven’t been keeping up on all of this, I have been struggling with shortness of breath and sporting blue lips for way too long. I also have pretty significant fatigue, chest pain, and major muscle and joint pain. I’m a mess.

I have a rare autoimmune disease called limited system sclerosis(scleroderma) which makes me high risk for lung and heart issues. People with rare diseases are called “zebras” in the medical community; since I’m sporting blue lips I’m the Blue-Lipped Zebra (BLZ). Got that?

My doctors do routine testing to monitor me for heart and lung conditions associated with systemic sclerosis; each time I had an echocardiogram and a high resolution CT scan the results were that I was… fine. No indications of a major problem.

But I was absolutely, positively not fine. I began to refer to the reassurances that all was okay as medical gaslighting. I transferred to new doctors. I got copies of all my test results, did lots of google searches, read research papers and articles in medical journals, and began to have evidence-based discussions with my doctors. They ordered up more aggressive testing of my heart and lungs. I posted about the my right heart catherization and CPET here if you want to catch up.

Last Monday my cardiologist called and gave me the final diagnosis. I have a type of pulmonary hypertension that is exercise-induced that is being complicated by a cardiac shunt in my heart. At rest, for all those previous echocardiograms and CT scans, everything was fine. When I’m in motion it is another story.

So, what exactly is pulmonary hypertension and why am I, as a systemic sclerosis patient, at high risk for it? In the most simple terms, the interiors of my lung arteries are narrowing due to scleroderma scarring and tissue growth; as the openings get smaller, the pressure of blood flowing through the arteries gets higher.

When we exercise the body needs more oxygen delivered to tissues; arteries constrict to raise blood pressure, your heart speeds up and your respiration rate increases. In my case, that constriction of arteries in my lungs makes the blood pressure in the lungs increase too much; blood struggles to get through the pulmonary arteries, and the downstream pressure in my right heart forces blood to flow from the right side, through the shunt, and into the left. My body’s blood pressure zooms up as the left side of the heart fights to push blood out of the heart past the jet of blood coming in from the right side through that dang hole. It’s a catastrophic cascade that happens in seconds, and the entire phenomenon is being driven by my systemic sclerosis created pulmonary hypertension. “You’re a challenging patient,” my cardiologist told me as we talked about my future treatment. Yep. That’s me. A challenge. I excel at challenges.

Last week the new medication that my cardiologist prescribed was shipped overnight express to me by Kaiser’s National Specialty Pharmacy. Kind of unusual, right? That’s because pulmonary hypertension is rare, so there aren’t that many people taking this drug in the US. If I was a Blue-Lipped Zebra before, I am now a BLZ wearing a crown. A periwinkle crown, of course, for pulmonary hypertension.

Hannah: I should have a crown!!

When I started this scleroderma journey one of my doctors told me that it was good to have a diagnosis, even if it was a shame. This is true. I’ve learned a lot since my first blood tests came back hinting at an auto-immune disease that generated a referral to a rheumatologist.

I’ve learned to be patient. I’ve learned to advocate for myself. I’ve learned to take the initiative to learn about my disease and to become an active participant in my treatment plan. I’ve learned to face down the monster and to go on with my life.

Challenge accepted!

This is Pulmonary Hypertension Awareness month. About 15% of systemic sclerosis patients develop pulmonary hypertension as a consequence of their disease. You can learn more about pulmonary hypertension here.

The Scleroderma Chronicles: The BLZ gets a CPET

The BLZ had some anxiety going into this test…

Last month I had a right heart catherization that showed an unexpected problem with my heart… there is a cardiac shunt that is allowing blood from the left side (you know, the one that is always colored red because that blood is rich with oxygen) to shoot over into the other side of the heart where it disrupts blood flow and adds pressure to the (blue) right side of the heart; that is a little tough on the right side because it isn’t built to handle the extra pressure. Houston, we have an explanation for all that blue panting that is going on.

Cool. Finally we were getting some answers and my doctors were taking my symptoms more seriously. My cardiologist ordered up some more tests to collect data and clarify the issue.

The test that I have been the most concerned about was the cardiopulmonary exercise test that was scheduled for last Monday. I hunted for some cool links to explain the test to use in this post and came up with a bunch of technical articles for physicians and specialists. Okay. Let’s not go there. I’ll try to explain what this test was all about. I was hooked up to a bunch of monitoring equipment that tracked my breathing and heart while I was riding a bike. As part of the test my lung function was tested, my heart monitored (like in an EKG), my blood pressure was tracked, my respiration rate, and the actual amount of oxygen I was using and the carbon dioxide that I was expelling for each mL of blood pumped. It was a lot!!

I was a little concerned because of my bad boy hip, but that wasn’t really a problem at all.

I shared my catnip with the Mother of Cats so that she wouldn’t have any trouble with the test.

The test went great for about 7 minutes: then there was an emergency stop. My blood pressure had suddenly zoomed up into the stratosphere at about 6 minutes into the test, and then 30 seconds later I began panting like crazy and the technician pulled the plug. Remember that cardiac shunt they found in the cath lab? When I exercise the direction of blood flow in that shunt reverses and blood from the right side is shooting into the left side where it interferes with the pumping of oxygenated blood to my body. No wonder I turn blue and pant.

The BLZ is both happy and sad at the same time.

The specialist who did my CPET was great at explaining the data to me; when he sent the report to my doctors he also included me in the email group. For the last week I have been reading the back and forth discussion by my doctors about the test and what the next steps for me will be. I am really pleased to be included in this process and feel that this option should be available to everyone; it is also kind of scary as I get a glimpse of what is coming down the road for me later in the testing department.

The gear and set-up for the CPET was pretty impressive. I joked with the specialist about it and he told me that there was an even more extreme version of testing that included a right heart catherization at the same time as the exercise test. Yep. You guessed it. That’s what my docs are considering to do next. It is clear that I have a cardiac shunt but they haven’t located it yet. The BLZ is pretty bummed. The word “profound” was used to describe my symptoms. There has also been some speculation about neuroendocrine tumors… The good news here is that no one is even considering sleep apnea or me needing antidepressants because, you know, I complain too much…

The BLZ is both happy and sad at the same time.

I keep my eye on the Mother of Cats while she reads her email.

So, it was a kind of tough week. I pulled myself together on Tuesday and went to a new yarn store for a little pick-me-up and was so distracted I missed my turn twice and had to detour through Starbucks before I actually pulled up in front of the store. I almost didn’t go in I was so worn out by then, but I did a little mental slapping to put myself into motion, pulled out my cane, and went in…

…to discover a woman dragging out three big bags of yarn that kind of screamed “community knitting” because they weren’t the yarn being sold in the store. “Hey! I want to community knit! Please, can I come play with you guys?” I immediately said.

That is how I ended up in a new local yarn store this morning knitting with the most wonderful group of ladies in the world. They are all current or former employees of the Kaiser medical system that I go to for my care, and they are producing hats for all of the Kaiser clinics that have infusion centers; I know those centers because they are the same ones that rheumatology patients go to. These ladies are all vaccinated and they all wore masks because they knew I was taking a risk to come. They had donuts! I have found a new knitting home when I needed it like no other. I actually cried a little with joy and relief as I drove away at the end of the meeting.

If I hadn’t made all those bad turns and the Starbucks stop this wouldn’t have happened.

My yarn stash is full of yarn that wants to turn into hats. Hats with happy colors and a dash of cashmere. I am on fire with purpose to produce as many hats as I can for other people who are facing down serious medical situations. I want to make them arm warmers and fingerless mitts. I have tapped into the best, most perfect group to produce useful gifts for other people like myself just when I needed to be grounded, inspired, and calmed by the peace of knitting.

The BLZ is happy.

The Scleroderma Chronicles: A Trip to the Cath Lab

The BLZ has been waiting a long time for this…

Five years ago I came down with the flu and ended up in Urgent Care struggling to breathe. I scored some antibiotics, steroids, and cough medicine. To fight the virus I was told to go off my immunosuppressant drugs for a few weeks until I got better, stay in bed, and load up on chicken soup: it took a couple of months but eventually I got back on my meds. Except… things weren’t quite right. I panted for air every time I came up the stairs and I noticed that my lips were turning blue. My blood pressure was too low and I had to stop taking my hypertension medicine. I felt dizzy and light headed sometimes.

I was a newly diagnosed systemic sclerosis patient and my doctors began running tests to see if my disease was impacting my lungs. Nope. Not my lungs. Tests were run to see if scleroderma was attacking my heart. Nope: my heart seemed to be normal. There were some anomalies, but my doctors decided to just monitor me through routine testing and see if things changed down the road. My red blood cell counts were way too high, and I had nocturnal hypoxia, so I was started on overnight oxygen. I was tested for various conditions that could account for the weird test results, but I always had a normal result.

I struggled on, battling for more testing, as my doctors kept reassuring me that I was okay. Hey, I had a blue face and panted for air when I climbed stairs; vacuuming could put me on the floor. How could this be “normal”? Ugh. Welcome to scleroderma, I thought.

I began to think of myself as the Blue-Lipped Zebra (BLZ). If I didn’t have a rare disease confusing the issue I would be getting better health care, it seemed. If doctors didn’t tend to apply most-common-cause thinking to my condition they might get to the bottom of things faster. It was, in my mind, a huge complicated mess as my doctors applied best practice (and rigid) diagnostic parameters to my symptoms or zeroed in on specific complications of my scleroderma and ignored other possible (and to be fair, rare) causes for my symptoms. I worried that my doctors had just parked me in a holding pattern as I slowly got worse; it is hard to advocate for yourself when you are sick and dependent on your doctors for help, even if you think that they are dismissive and borderline disparaging.

I struggled on as my face became more blue, my red blood cell count higher, my panting for air more common, and the occasional near-fainting event left me collapsed on the floor. I began to ask for a right heart catherization procedure to directly measure the pressure in the right side of my heart. “Oh. You don’t want that,” I was told. “That is really invasive testing.” Umm… I think I do, I would reply. Nope. Nope, nope, nope!! “Not even on the table,” one pulmonologist said.

During lockdown last year I got much worse; ironically lockdown also gave me the opportunity to reboot, fire my old doctors and acquire new ones. My new team of doctors this spring ordered up testing that showed definite issues with my heart and lungs. I am now a heart failure patient (the wall of my left ventricle are too stiff and scarred to beat well) and there were concerning findings that suggested that I had developed pulmonary arterial hypertension (PAH): there are areas of cell death in my lungs and my pulmonary artery is too big. There is too much fluid around my heart, a suggestion of ongoing inflammation. I have a hole in my heart between the atriums (a cardiac shunt) that is impacting blood flow. I was gently prepared for the PAH diagnosis, assured that there were great drugs that could help me, and a right heart catherization was ordered by my new cardiologist.

Yay!! About time!!!

Finally, after 5 years of struggle, I was yesterday wheeled into a procedure room to a waiting team of specialists who hooked me up to equipment and took me though testing to get a better look at my heart: I learned in recovery that this team calls themselves “the pit crew”, and that is exactly what it was like. Within 5 minutes I had completed a breathing test, was on oxygen, wired up to a heart monitor, hooked up to an IV, medicated, prepared with surgical drapes, and swathed in warm blankets with a heater by my feet. There was music playing and the crew was cracking jokes as they darted in and out from the table getting me ready. I was knocked out for the echocardiogram imaging of the back of my heart that was done using a probe in my esophagus, but they woke me up for the main event: the right heart catherization. My cardiologist inserted a probe into the carotid vein in my neck and threaded it into my heart by way of the superior vena cava (blue side of the heart diagram above). People, this was the most amazing experience ever! There was a huge screen showing the progress of the probe and I could watch and ask questions as the line snaked through my heart; there was absolutely no pain. “Well, this is interesting,” said my cardiologist at one point, and there were more measurements happening and a flurry of new activity from the team. The BLZ felt a surge of elation: they had found something, and it was NOT what they expected.

Back in recovery my cardiologist caught up with me again. I had done great, he said, and he just beamed as he told me that I absolutely did not have PAH. This is great news, he assured me, great news!! There is another circulation problem in the lower part of my heart, between the ventricles. There is blood coming in from the left side of my heart and mixing with the blood on the right, disrupting the flow through the heart and robbing me of oxygen to my body. I have a second, more serious, cardiac shunt, and now it is a question of locating that pesky little guy and doing something to fix the problem.

It has been FIVE YEARS, people. If I hadn’t had my trip into the cath lab yesterday my doctors would still be nagging me to get more exercise (the BLZ just barks in laughter), offering me antidepressants, or insisting that I must have sleep apnea. I feel so validated!

My cardiologist is now going over my previous imaging to find the hole now that he knows what to look for. I was told that I may need to go through more testing to definitively characterize the opening, but this is huge forward progress. I suspect that I am facing open heart surgery down the road, but I am elated that the progressive and eventually fatal diagnosis of PAH is now off the table. Things are looking up since my heart failure will now be much easier to treat.

I am reminded of Elizabeth Zimmerman’s admonishment: “Knit on with confidence and hope, through all crisis.” Also, when the going gets tough, get a kitten!

Today I am waiting to hear back from my cardiologist who is going to email me with follow up instructions after he has finished going through the data and past test results. I’m on oxygen, knitting, and feeling pretty calm about all the new developments.

Almost exactly 7 years ago (August 28th was the anniversary day) I was diagnosed with Limited Systemic Sclerosis and Sjogren’s Disease. I have learned a lot along the way, but the best, most important lessons have been about self-advocacy, facing down the worst case scenarios, communicating with your doctors, and maintaining a good attitude.

Yesterday this all paid off for me big time.

Note: The fabulous BLZ graphic was made for me by my exceptionally knitworthy niece Melissa and her beautiful and talented daughter Eleanor.

The Saturday Update: Week 29, 2021

It was a lovely week last week; hot and sunny with cooling afternoon thundershowers. Perfect. I rested up the entire week recovering from the exceptionally busy week I had the week before that and got lots done outdoors.

The new kitten, Mateo, continues to grow at an accelerated rate and is eating like a bottomless pit. He is now 3 months, 3 weeks old and is much less clumsy but still unable to jump up onto counters. Whew. No rush there. By the way, do those front paws look kind of big to you?

Hannah has recovered from her visit to the vet and all the vaccinations and is returning to her playful self. There is a little problem here, though… she wants to play with the kitten all the time and me or her toys… not so much. I’ve been giving her more attention during the kitten’s naps and she is slowly rebounding to her previous endearing interactions with me and her toys. This has been a big month for her, after all, and she is coming through it like a trooper.

Garden

The garden continues to flourish in the good weather but I have some concerns for it as there is another heat dome building and this time it will be much closer to Colorado. I took pictures of the flowers this afternoon as I’m not too sure what they will look like in another week…

There is a lot of blooming going on with the roses and for some reason the insects haven’t been munching on them as much as usual. Look at how happy and fresh these blooms look! This is the second blooming of the season and I am feeding the roses again this week in the hopes that I can get a third blooming next month.

The garden is full of volunteer plants that are popping up from things I put in the garden in years past. The big winners are snapdragons… they are everywhere! I put in a picture showing all the new buds that are emerging on the plants (2nd from left) and one with dried seed pods that already dropping seeds on the same plants (3rd from left). I am moving some of the seeds to gardens in the back flower beds as I work clearing out weeds and I’m hoping that there will be a new crop of snapdragons back there next year. I’m also seeing cute little Johnny-Jump-Ups appearing in the gardens like the little purple flower in the last picture. It’s becoming kind of a Darwinian garden in this flower bed over time as the plants that are more successful are taking over my flower bed and other places in the yards.

Knitting

I haven’t said too much about it, but I’ve been battling tendonitis in my right shoulder that was putting a huge crimp in my knitting activities for the last six weeks… the pain radiated down my arm and I lost all feeling in my fingers and some function. The concern, of course, was that my knitting days were coming to an end due to joint and nerve damage from my systemic sclerosis. My rheumatologist started me on a new drug and sent me to physical therapy and I am finally, finally, recovering the use of my arm and can knit more than a half hour at a time again. Whew! People, I am back!! Am I buying any more yarn right now… no. Sadly, no.

I got busy on a new sweater and made some good progress this week. I am making the Rock It Tee using some great speckled yarn from the stash and I am so pleased with how it is working out.

The tee is constructed from the top down and you knit back and forth while shaping the raglan sleeves and the V neck in the front. In the picture on the left you can see that I have just joined the knitting below the V neck to allow me to knit in the round (so much easier on my hand) and the picture on the left shows the raglan sleeve shaping. I am almost at the point where the sleeves will be taken off and placed on holders and then it will be simple knitting in the round to the bottom of the sweater. Yay! My next ball of yarn looks a little darker so I am going to start blending it into the current fabric as soon as I’m below the sleeves.

Books

I finished this in just a few days!

The Ten Thousand Books of January was great! I had just finished a book about books, doors, keys, and some crazy-ass magic associated with them, so I was a little worried about this one too. Nope. This was a straight forward book within a book that told a great story. I really enjoyed it and loved every minute of the books, doors, keys, words, and crazy-ass magic.

Have a great week everyone.

Read a little, knit a little, and garden like your heart can’t live without it.