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!

The Scleroderma Chronicles: Rare Disease Day, 2022

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

You know, I feel like I should represent.

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

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

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

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

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

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

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

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

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

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

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

****************************************

My scleroderma-related diagnoses:

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

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

Happy Rare Disease Day, everyone!

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

The Scleroderma Chronicles: And Do You Exercise Regularly?

It has been an eventful week in big and small ways. I had been mostly in bed for most of a week as I struggled my way through two snowstorms with significant air pressure drops. Ugh. I had chest pain, coughing, heart palpitations, and more sleep than I want to admit to. Towards the middle of the week, I went off my immunosuppressant drug and the flare of my disease(s) arrived over the next two days. Ugh. So predictable, but still discouraging. I dragged myself together on Monday, double masked, and made it to the pharmacy where I had an appointment for a Covid-19 booster that afternoon.

Mateo: Do I need a booster too? Here’s my arm…

I have this really wacky sense of humor. The entire experience just kind of cracked me up. You see, I got the appointment at my local grocery store’s pharmacy. Here are some of the highlights:

  • I have had so many shots at this point that they had to use the back of my vaccine card. At this rate I will need an accordion-like pullout for the vaccine information in a few months.
    • Why so many shots? I’m immunosuppressed. I went off my drugs this time to give my immune system a better chance of responding to the vaccine.
  • The staging area for the shot was at the Fritos display across from the pharmacy. Seriously, the pharmacist said, “Go stand with the Fritos and wait your turn.”
  • The shot was easy, peasy. I think that the syringe was spring loaded it was so fast. “Go walk around the store for 10 minutes before you leave,” I was told.
  • So I waved goodbye to the Fritos and walked around the store. Mostly I just looked at the empty aisles for the 10 minutes feeling sorry for myself. No milk. No Snapple. No cat food. No guacamole. NO GUACAMOLE!!!! Oh, yeah. Genius me scheduled the booster shot during a grocery store strike by the competing chain’s employees and this store was basically stripped of essentials by the descending horde of shoppers who didn’t want to cross the picket line. As they shouldn’t. But they could have left me a little guacamole, don’t you think?
I was able to get the cats a Boston fern to replace the palm that was chomped to death by… I wonder who could have done that? Hmmm…

I also got a Starbucks. Not the worst trip out of the house. I ended up with a sore arm and was so exhausted that I slept for 12 hours.

Today, 48 hours after the booster shot, I feel great!! The flare is gone. I haven’t felt this good in weeks. This happened to me the last time I got a Covid booster. I think that it must be the increase in antibodies or something; I’m so immunosuppressed that my gamma globulins are way too low (a medical condition that my doctors are just ignoring because I do have enough white blood cells). Maybe the boost in antibodies following the shot actually makes me feel better somehow. Maybe my white cell count goes up. It’s a mystery. I’ll take the win!

And in that winning mood I went to see my cardiologist for the first time since my trip to the cath lab last fall. I was a little short of breath but was walking okay when I got to the office. I received an EKG test and the nurse checked me in:

Nurse: “And do you exercise regularly?” (in a judgmental tone of voice…)

Me: “Oh, please. Let’s not even pretend that I am able to exercise!”

Nurse: “Oh. I’m so sorry that I asked you that…” We both started laughing, but I meant it! I am so over feeling defensive about being unable to exercise. In fact, trying to exercise with my condition was damaging my heart.

I also think that I had my snark on under my mask.

Then the doctor came in!

My cardiologist is freaking awesome. He asked lots of questions about how I was doing. (Face now mostly not blue. Yay! Some panting and chest pain, but so much better. I went up a couple of flights of stairs with no problem.) We discussed the fact that I don’t fit the usual diagnostic model for pulmonary hypertension but based on physiological changes consistent with PH and my dramatic response to treatment with a PH drug, he made the call and entered the diagnosis. The matter is now settled until new data comes along.

Exercise-induced pulmonary hypertension. As in, you look perfectly normal when you are on the table getting your lung/heart tests, but the minute you exercise all hell breaks loose in the blood vessels of your lungs. Fabulous. There is a really invasive testing protocol that I could be subjected to, but there is enough evidence now to establish the diagnosis without it. The diagnosis became part of my medical record today. At last.

It has been a long time coming!!

It has been almost exactly 5 years since the BLZ began her journey to find help and answers. This has been really, really hard, but I made it.

We discussed the pros/cons of more testing. We talked about the risks and benefits of adding a second drug to the one I’m already on. We talked about who will take over management of my PH (he will) and how he will integrate with my rheumatologist. We talked about how important it is to be comfortable with “out of the box” thinking when dealing with a patient who is basically at the far end of the bell curve… in other words, a zebra. Oh, I like this guy!!

I agreed to start the additional medication which will be added to the one that I’m already taking. There will be more side effects as this second drug kicks in and I will be getting several phone calls to check on me as I start it. The plan is to try to slow down my progression before I develop full blown PH.

Next up: more testing to hunt for that dang hole in my heart. It’s like a snipe hunt, but so much less fun. As in, heart surgery anyone?

Hannah: Don’t worry mom: you’re got this!

Hannah and the CoalBear: Mateo does Caturday.

Hi. I’m Mateo (AKA the CoalBear).

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

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

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

Happy Caturday everyone!!

May you all have an excellent day!

Notes from the Mother of Cats:

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

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

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

And wear your mask!

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

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 Saturday Update: Weeks 35 and 36, 2021

The heat is hanging on and the cats and I are totally over it at this point. Yesterday we set a new heat record in my state: 99 degrees Fahrenheit. The sun is a sullen red ball in the sky and the sky is slate white with smoke from the west. The heat is unrelenting, there has been almost no rain, and fire season is still going on, and on, and on… Should summer be ending soon?

“Not yet!”, say the plants and wildlife in the yard. The cats have been busy chasing late season houseflies and the trees are abuzz with pulsing cicada sounds. There seems to be wildlife everywhere all of a sudden as the lawn and garden recover from the intense sun (but not the heat) of July and August. I even had to chase a little garter snake out of my garage yesterday! Summer is being especially tenacious, but I am seeing some hints of fall as flocks of geese fly over my house and the first leaves on the trees are turning.

The baby bunny is now hanging out in the front yard entertaining the cats as they watch from the front door. Do you see all of that debris on the sidewalk and street in the picture of the bunny? That is the mess being left by the squirrels as they harvest the Russian olives off of my neighbor’s tree. There is a really cute squirrel with an exceptionally fluffy tail digging holes under my trees to stash food for later… not nice, little squirrel!! Speaking of fluffy tails, look at what is happening to Mateo’s tail!! This kitten is getting a lot fluffier than I thought he would…

Garden

Everything that is pink is just flourishing at the moment.

My Autumn Joy Stonecrop is just covered with bees and other plants in containers on the deck have decided that they want to bloom too. Even the roses by my driveway have decided to get a last bloom in. For some crazy reason it is all of the pink plants… even the hydrangea is putting out some new blooms with pink edges. It makes me happy to see all that color coming back even if it will be drowned out by all the fall colors in the trees and shrubs in a week or two.

Knitting

I had several quiet days following my right heart catherization procedure last week (okay, I also slept a couple of days afterwards) and I ripped right through the next clue on the Snark-O-Meter. If you haven’t been keeping up with all of the snark, the Snark-O-Meter is the latest MKAL shawl by Sharon from Security (Casapinka) and I am having a blast this time.

It is getting too big to show off the fun stitches in this project in one picture so I settled for shots of each clue. The top left is clues 1 and 2, the bottom left shows clue 3, and the picture on the right is clue 4. Look at all of those great and interesting stitches and use of color!! I’m going to get a ton of use out of this shawl.

Since I finished the shawl in just a couple of days after the clue was released, and because Sharon is taking a week off to let people catch up on their knitting, and since I was admiring all things pink in the garden, I pulled out my new Rock It Tee that is being knit in a pinky/rusty colorway called Cinnasizzle. The actual yarn isn’t quite as hot pink as it appears in the pictures, but it is full of clay, pink, and rust colors that are just too fun to ignore. I’ve been binge watching Billions on Amazon Prime and knitting like crazy this last week with a nebulous thought that if I really apply myself I can get this sweater done before Sharon releases the next Snark-O-Meter clue in another week. Ha! This is clearly impossible, but that never stopped me before! To try is all. I’m below the armholes now and it is just knitting in the round for the next 15 inches or so…

Mateo is now 5 and a half months old. Isn’t he getting handsome?

I’m still waiting for my cardiologist to contact me with the next steps in the ongoing Blue-Lipped Zebra project. Things are really slammed right now in the health field as there are lots of Covid-19 patients and Covid long-haulers who need help, and my cardiologist is pretty overwhelmed with the case load at the moment. (How do I know? My pulmonologist, who is also slammed, told me. I feel so bad for them.) It is a little hard to be patient and to wait my turn, especially since some of those people who are bumping me back in the health-care line are the unvaccinated.

Last week I was mocked in public by someone for wearing a mask. Umm… retired high school teacher here. I called him out on it and he immediately stopped.

But still, it’s a little shocking that it even happened.

This is the 20th anniversary of 9/11. What a sad, horrible day that led to unpredictable outcomes that were far beyond my imagination at that time. How sad it is to reflect on all that has happened, and continues to happen, in America.

I plan to knit my way though the reflection, waiting, and sadness this week. You all be safe out there!

Have a great week everyone.

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