Sclerodema – Yarn, Books & Roses

Hannah and the CoalBear: Lazy Mother of Cats

Hi. I’m Hannah.

I would like to lodge a complaint against the Mother of Cats!!

The world outside has changed over the last couple of weeks. The sunshine is bright and warm; I love to sleep in the sunshine, don’t you? There are more squirrels than usual chasing each other through the trees out front, and sometimes they even come right up to the windows. The bunnies are spending lots of time in the yard where the CoalBear and I can see them, and there are birds again. Lots of birds!! All of this change is really exciting, and the CoalBear and I just want to spend all of our time playing. So, is the Mother of Cats spending all of her time entertaining us? Giving us tuna? Letting us outside to play in the sunshine and delivering the kitty cookies right on time?

No. She is not!

The Mother of Cats has been sleeping more than usual (and I’m a cat, so believe me, that is a lot!), reading her books, and knitting. Knitting isn’t too bad if we get to play with the yarn, but nope, nope, nope… once again she is not sharing her toys with us like she should.

Mateo the CoalBear is doing his best to play anyway! He loves the needles, I perfer the yarn myself. He is kind of a weird kitty…

The Mother of Cats has been listening to an audiobook during some of her knitting, so I get to listen along with her while she knits and I help with the yarn management.

This is the book that we listened to last week.

Imagine a man who dreams of waves of energy zooming through the air from a spark of electricity to a device that can detect the waves; the device is like magic, letting messages travel from one place to another without wires. He dreams of all the changes that the wireless messages can make in the world, and also about how much money he can make from the business that installs and runs the devices that make this possible. His name is Guglielmo Marconi. Pretty cool, right. (Can I have some tuna now… all this typing is making me hungry…) At the same time in history, there is a man who works creating and selling “cures” for illnesses. He is married to a woman who is very bossy and demanding (CoalBear… I’m looking at you…) and one day he snaps, kills her, and tries to escape with his true love to America on a ship. His name is Hawley Harvey Crippen.

I have to be honest; I played a lot with toys and this fortune paper from a Chinese cookie while the whole book thing was going on…

Are you tired of the story yet? It gets really exciting now. Chief Inspector Walter Dew of Scotland yard finds the reminds of the murdered wife. The hunt for the escaped murdering husband with his girlfriend becomes a big deal in the newspapers, and the captain of the ship realizes that two of his passengers are the people being hunted by Scotland yard. The captain sends a message back to shore using (what else???) his Marconi wireless device. Chief Inspector Dew boards a fast boat and the chase across the Atlantic Ocean is on, with coordination between ships made possible because of … wireless messages using the Marconi system. Marconi messages keep the press updated, and suspense builds as the public hangs onto every new update and intercepted message reported in the news. Whew! My whiskers were just a tingle listening to all of this! Chief Inspecter Dew overtook the ship with the murderer, and he was apprehended before he could land in Canada. Because of the publicity, Marconi’s business was secured. What a story. What a book!! I absolutely need some tuna right this minute!!!!

Look at how much the Mother of Cats got done while she was listening to the book and knitting.

So that has pretty much been the last two weeks. Sleeping, knitting, and listening to really interesting books. I like the yarn and the books, but I do hope that the Mother of Cats will stop being so lazy… Mateo and I have needs, right?

I’m not lazy… I’m a cat. I’m supposed to sleep all day.

This is Hannah, signing off.

>^..^<

Notes from the Mother of Cats:

The sweater that I’m knitting is the Winter Albina sweater by Caitlin Hunter. I’m really pleased with how it is working up.
I’ve already started another Eric Larson book: The Demon of Unrest.
I’m in another scleroderma flare, and I did go see my rheumatologist for help. I am now in possession of an emergency pack of steroids and narcotics. Whew. It’s good to have an emergency pack!
I’m doing better lately, but the cats are still kind of disgusted with me.
What was that Chinese cookie fortune that Hannah was playing with?

Friday, February 28th, was Rare Disease Day. I found that I was ~~too lazy~~ unable to write another post for the day, but here are some nice ones that I wrote in previous years.

The Scleroderma Chronicles: Supermoon Zebra Update

This morning, I dragged myself outside to the catio and just sat there for a while warming my joints and charging up for the day. Sometimes I wake in stunned astonishment at how bad I feel; this was one of those days. The cats, confused by the change in routine, finally stopped begging for tuna and came out to check out the wildlife. Things were certainly busy in the yard this morning!

The bird feeder was open for business with a steady line of little birds waiting for their turn at the feeder parked in the low branches of the tree. Higher up in the upper branches a blue jay was calling back and forth with the other jays in the neighborhood. A bunny napped shamelessly in the middle of a patch of dirt at the upper end of my yard (why do I have a batch of dirt? Bunnies!!! They dig and roll in this dirt. I love the bunnies more than I love the lawn…), and a pair of doves napped nearby in plants escaping my garden. There were grasshoppers in the catio to Mateo’s delight. Squirrels ran along the fence as I baked and recovered; there was a slight breeze and the day was cooler. Time to water the flowers and go in.

Japanese beetles in my roses! The HORROR!!!!

There were Japanese beetles in my roses!! Very cute, and very not wanted. I drowned those guys in a bottle of soapy water. Bad beetles, bad. No matter how well things are going, there is always something to deal with, right? I was completely recovered and ready-to-go by the time I went in to deal with the tuna-starved cats and my latte machine.

Not a bad start for the day, right? Bake in the sun when you need it, enjoy all the pleasant things happening around you, and take immediate action to deal with stuff that is unacceptable.

It has been another tough week. Having gotten through a huge round of doctors’ appointments and testing, you would think I could just rest up for a few days and recover. Not so much. It is stressful waiting for test results, especially since my cardiologist was openly concerned when I saw him at the end of July. I felt dizzy and struggled with vague symptoms like a sore throat and stiff neck for a few days, and then finally began to face the reality that something more than scleroderma might be going on.

What pushed me into action? Well… the very last medical test was MRIs of my bad boy knees, and the technician who worked with me was concerned that the pupils of my eyes were dilatated more than they should be. There was concern and lots of questions about my medications. “No,” I insisted. “I’m not taking any drugs that weren’t prescribed.” I finally got out of there and immediately forgot about the incident because I had just spent an hour in an MRI machine that looked like a giant sandworm from Dune and made some of the same noises, too!

Then the next day, I fell over sideways while combing my hair. I tried to put on my shoes, and I fell over again to the same side. Huh. That is kind of unusual. I checked my eyes in the mirror, and yep… those pupils looked pretty dilatated. I called in to my health provider, and I was sent to Urgent Care. The words “this might be a brain bleed” got my attention, so I filled up the cat food and water dishes, put a phone charger into my purse, and off I went.

Listen, going to urgent care or the ER is kind of risky if you have a rare disease or two. Either the doctor has never had a patient like you before and just refers you on to a specialist, or the doctor is kind of excited to get his/her hands on you because when will another patient like this come along? They want to do something!! Sign me up coach! I’m just trying to represent here!! You can literally see it on their face and in the sparks in their eyes.

The urgent care doctor was the second type of doctor.

Sitting in the exam room I could hear the zebra laughing…

My eyes were fine when he checked them, and we concluded that what was happening was probably a transitory effect caused by one of my meds. It seemed reasonable, so I was okay with that. Great. I’m out of here, no brain bleed for me! Nope. Nope, nope, nope! He had a real, live zebra here, and he wasn’t letting it slip away!! He felt that while I was there, I should have a lot of tests run to rule out a hypothetical infection that was pushing me over the edge and causing all my fatigue, dizziness, headaches, etc. The stiff neck was concerning. No one should fall over while combing their hair… Sigh. It is hard to argue against such reasonable observations… I didn’t even mention that it hurt to breathe, but there was that, too…

I approved some testing, declined others, and off to the x-ray machine and lab I went. Everything was negative, but he decided that I should go though a course of wide-spectrum antibiotics anyway since I was immunosuppressed, and something clearly was not right. Fine. I’ll do that. My whole life is “not right” and antibiotics make my unhappy gut even more unhappy, but he was so earnest about helping me: I took the plunge and started the pills.

I just want to note that it is hard to score antibiotics if you feel sick with vague symptoms, but being a zebra got me special treatment. I’m still processing that because most of the time my symptoms are dismissed. My knees have been hurting for years and doctor after doctor has just blown me off because the joints seem fine. They are swollen, but my inflammation markers are normal, so nothing is done… My knee x-rays were normal, so this time I requested MRI imaging, which is how I ended up visiting that Dune machine, meeting a nurse upset about my eyes, and now, landing me in urgent care. There is some type of zebra paradox here: specialists who disregard symptoms that set off actions in mainstream health professionals, and special treatment that normal people don’t get because… zebra.

Anyway, I took the antibiotic. It was a strong one that I had never taken before… and two days later I was better! Even my gut has improved. The headaches have stopped, and my dizziness is fading into the background. My chest pain is gone. Yay antibiotics. Yay earnest young doctor in the urgent care facility. He’s written me two notes following up on the test results, and I’ve let him know that I am, indeed, much better.

The last of the test results that my scleroderma care team ordered have come in, and it looks like there may be some adjustments to my care in the future. My pulmonary hypertension is under control, but my heart failure has gotten worse. The steroid injection in my hip has made a huge difference, and I am walking better, but my knees hurt more. There is, indeed, something wrong with my knees; I don’t know if they can do anything about it. The words on the report are

Which is commonly known as “runner’s knee”, and is an overuse injury in most cases. Not in my case, obviously, since I can hardly walk! I have edema in the bone, and in the cartilage, and there is calcium being deposited in the soft tissue which is a response to… you guessed it… inflammation.

That dang zebra is rolling on the floor laughing!!!

It has been quite a week. I pushed for more testing, paid expensive copays to get it, and have gotten validation on the bad-boy knees. I understand a little better why the cardiologist was concerned, but the news isn’t as bad as he anticipated, so the outcome (Class 3 HF) isn’t as bleak as it could be. I learned a lesson about not ignoring symptoms, and the zebra outing to urgent care worked out much better than I expected. I turned in 43 hats and 7 chickens this week to my community knitting group, and yesterday I bought a bushel of roasted green chiles. A tough week in some ways, but also a good week. A week of crazy zebra-related events, knitting, and even green chiles.

Tonight is the blue supermoon, a rare event, and kind of special. Knitting in the light of the blue supermoon is the best way to end a day that started baking in the sun.

And if you have a crown, tonight is the night to wear it!

Shine on, supermoon, shine on.

The BioGeek flirts with Long-Hauling

It’s been over a month since I first developed Covid-like symptoms and began to spend my days in bed. Like, my bed became my little nest with the detritus of a prolonged illness littering the floor and shelves around it. Outside the world moved through the end of summer; my tree lost all of its leaves, and the garden moved into dormancy. The birds disappeared. The squirrels have remained on the crazy side, but the crickets are now silent. The cats are chasing the last few grasshoppers of summer, but they are almost too cold to hop anymore.

Mateo has used my down time well, destroying his cat tree, removing push pins from the wall hangings, and just being an agent of mayhem in general. Hannah is not impressed.

While the symptoms of “whatever the heck that Covid wannabe virus was” are mostly gone, I’m still struggling big time. Everything, and I do mean everything, hurts. Tendons that I didn’t even know I had are now too painful to touch. Random shooting pain and muscle cramps have become routine. Did I mention the chest pain? I can’t concentrate enough to read; brain fog is driving the bus these days. I can’t knit for more than a few rows at a time as my arms get too heavy to hold up. I am kind of weak and shaky. I’m starting to stare down the depression monster for the first time in years. Did I mention that the fatigue is unreal? Ugh.

Hannah has been supporting my knitting efforts. I’m slowly making progress on my Weekender Crew sweater.

So, there hasn’t been much blogging. There has been, however, some medical testing and trips to doctor offices going on.

Yesterday I finally saw my rheumatologist. “This is just awful,” I told her. “This is fibromyalgia!” she replied. In her opinion, what I’m experiencing is a pretty severe episode of fibromyalgia. I’d already been diagnosed a few years ago, but I’ve never experienced symptoms as bad as this. Sigh. Remember the 15% rule in scleroderma? It says that about 15% of patients with systemic sclerosis (the type of scleroderma that I have) will also have a second autoimmune disease called Sjogren’s. Check, got that. Since Sjogren’s symptoms can look like fibromyalgia I always took that diagnosis with a grain of salt. Now it’s pretty clear that it is a separate condition, and my fibromyalgia is running wild. Like a squirrel. Or Mateo.

Feeling particularly unlucky I drove home thinking about writing an autobiography called Outlier: My life as a singular data point. Seriously, who in their right mind would ever want to be so far off the bell curve? Then the BioGeek emerged, and I thought about Covid long-haulers. I have met some people who have been diagnosed with Long Covid, and I did think at the time that what they were dealing with was an awful lot like one of my flares. Like, an awful lot. Yep. According to this article about a third of Covid long-haulers have “FibroCovid”, a condition that pretty much looks like fibromyalgia. Fabulous. This article suggests that Long Covid is just another name for fibromyalgia. Another article explored the benefits of applying lessons from fibromyalgia research to Long Covid. Thanks, nasty virus that acted like Covid but refused to test positive. Thanks for acting like a nightmare houseguest who leaves a huge mess behind…

This is crazy, right? Covid shares similarities with systemic sclerosis, and long Covid shares similarities with fibromyalgia. The BioGeek in me is interested in all of this, but let’s hope this doesn’t drag on for months.

My rheumatologist has started me on a course of narcotic painkillers as that sometimes pops people out of fibromyalgia flares. I’m taking magnesium as that can also help. My rheumatologist has some more tricks up her sleeve if this doesn’t do it, but I’m relieved to finally have a name for what is happening.

The dedication page on the book I started reading this weekend. I haven’t gotten very far because… brain fog.

Time to kick this thing to the curb (like a zebra!) so I can get back to knitting!!

The Scleroderma Chronicles: Be a Rose.

So, I got a little testy in one of my Facebook support groups for systemic sclerosis this morning. A member of the group kind of disparaged me and another person for not being positive enough. It was “you need to refuse to let scleroderma define you” in response to the first person sadly saying that she missed her old life, and me giving her an online hug with the comment that if only a positive attitude was enough…

In my defense I had just experienced a Kaiser employee visibly reacting to my lack of wrinkles. As in, wow, that’s great! Like not having wrinkles makes this all worth it.

I lost another 4 pounds at my last check-in, which is concerning, but the nurse was thrilled for me. Me, I was a little teary at the continued loss.

Um, people… do you think that you might be a little shallow here?

I was out on the deck/catio with the cats drinking my morning latte when I hit this emotional wall, and after I had fired off the somewhat testy response, I spent some time in the garden. There were my roses, blooming like the utter champs that they are.

This is my Princess Alexandra of Kent rose.

This rose is looking great this year. Never before has the plant been able to hold up the blooms without the weight pulling the stems over. The problem is our semi-arid climate with hard winters; the plant grows back from the roots every year and it doesn’t have time to put in enough supportive tissue to hold up the blooms. This year, with all the rain and cool weather that we have had, the plant was able to put in enough of this tissue to do the job. The tissue that I’m talking about, a type of ground tissue, is called sclerenchyma. If you’re ever snapped a celery stalk in half and pulled out the strings, you were pulling out sclerenchyma tissue. This tissue is made of dry, hardened cells in the stem of the rose and I suspect that the “scler” part of the word is of the same origin as scleroderma. Look at that. The hardened cells are doing something good for this rose!

In me, not so much. Things have been a little difficult as my poor heart and lungs are not benefiting from the hardening and thickening going on in the cells and tissues of those organs. All of my tendons are seriously pissed off at the moment. Edema has become a problem, and it is becoming increasing clear that I need to stay on oxygen 24/7.

That’s my arm with the imprint of a quilt in it, in case you didn’t immediately recognize it… Edema is kind of a tip off that my heart is struggling and that’s new. The weight loss is also related to my heart/lungs because if oxygen isn’t getting down to my cells like it should, they can’t use energy efficiently, and then, you know, weight loss occurs. The tendon issue is scleroderma actively attacking them and gradually hardening them to bone. Bad scleroderma, bad!!

So, what did I say in my testy response? Reality bites. Some of us have progressed to the point where we have to admit that no amount of positive thinking will allow us to attend that family function that we were invited to, or to visit the annual Wool Market in the mountains, or to even walk to the mailbox. To suggest that we could do things if we just had a more positive attitude is hurtful and not supportive. No matter how much you want to believe otherwise, scleroderma does define me and everyone else who is dealing with it. Courage requires us to face down the monster and to accept the reality of our disease. How we choose to function within that framework is up to us.

I may no longer be as mobile as I once was, and the life that I used to have is now mostly gone, but I choose to continue to bloom in place.

Like a rose.

Updates/Notes from the ScleroFront:

Do you like to wear linen? Those fibers are from the water tubes (xylem) in the flax plant, and made of sclerenchyma.
The bunny-murdering neighbor put her house up for sale!!
My Alpine Bloom sweater is coming right along!

The Scleroderma Chronicles: Rare Disease Day, 2023

Well, here it is again. Rare Disease Day. I kind of was going to ignore it this year because I’m quite frankly worn out by my… wait for it… rare diseases, but I also feel like I should pull myself together and represent for the community again.

People with rare diseases are referred to as Zebras in the medical community. I obtained my zebra status when I was diagnosed with systemic sclerosis in 2014. This zebra was sent to me last week by my *Most Knitworthy Niece* Melissa.

I wrote a pretty darn good post last year about my journey with a rare disease which you can read here if you want. I talked about rare diseases in general, my specific conditions, and the many things that have been said to me by my doctors over the years. I thought about just reposting what I wrote last year, but I’ve been reflecting all morning on some recent events that kind of shine a light on my situation and that of other people who are coping with rare conditions.

I recently managed to go knit with my fellow members of Frayed Knots. This was a big social outing for me because it’s hard to get out of the house, and I have to be having a really good day to go to something like this. Knitting with friends is just “normal” for most people, and it would just be a little part of their day, but for me this was something that I had to prepare for a couple of days in advance, and then recover from in bed the next day. Many rare conditions are chronic, and chronic illnesses can be very isolating by their very nature.
A woman at the knitting group questioned my decision to wear a mask. I started to explain, but she cut me off to say that I was doing it so I could feel comfortable. It was a little condescending and suggested that I was being paranoid. Truthfully, my immune system, crushed by the drugs that I am taking right now, is compromised in its ability to make antibodies. If I catch a viral disease like the flu or Covid, there is a good chance that I won’t survive. My vaccinations have a low chance of protecting me for the same reason. For people with rare diseases, life is fraught and full of difficult decisions. For me, and for many other immunocompromised individuals, simple decisions involve life/death level risk analysis.
Another woman at the knitting table was struggling with long Covid and shared her difficulties with returning to work. She especially felt crushed by the attitudes of her coworkers who seemed to feel that she was “fine” and just trying to get attention. Yep. Been there, done that. Many rare diseases are genetic or largely invisible to others. Invisible illnesses are especially hard to cope with because others tend to question their validity.
I’m in several online support groups, and there are always discussions about what drugs to take, and whether the side effects are worth the risks. Yep. There are no specific drugs for systemic sclerosis, no cure, and treatment can involve a patchwork of risky off-label drugs. The drugs that are used are often non-specific carpet-bombing like approaches. Rare diseases have fewer treatment options because there are only a limited number of patients.

Over the last year my wonderful team of physicians have been suggesting that I am really unusual and have been extremely responsive to my emails. They clear an hour for appointments with me. I’m one of the very lucky zebras who has managed to get diagnoses, secured treatment, and am benefiting from a team of collaborative, interdisciplinary physicians who actively communicate with each other and with me; just last week my rheumatologist told me that for a patient with my status this is the only way to deliver care. I’m so grateful to have secured this level of medical attention, but I also feel a little nervous about it. I spent some time this morning trying to work out the probability of one person having the several medical diagnoses that I’ve racked up since 2014. Like, just how rare am I?

The National Organization for Rare Disorders estimates that there are 100,000 patients with systemic sclerosis in the United States. That’s rare, but still, a big club, right?

The 15% Rule is a general measurement of the risk of severe organ involvement in systemic sclerosis. As it turns out, quite a few of the major lung, heart, and kidney complications associated with systemic sclerosis happen about 15% of the time. I have Sjogren’s Disease overlap with my systemic sclerosis, which happens in about 13% of patients. Suddenly, I’m in a much smaller group of about 13,000 patients.

My most worrisome complicating conditions are diastolic dysfunction (a type of heart failure), pulmonary arterial hypertension (PAH) and interstitial lung disease (SSc-ILD). I looked up the risk of having each of these conditions using the 15% rule data, and it turns out the risks are 16% (diastolic dysfunction), 15% (PAH) and 35% for the SSc-ILD. Did you notice the the ILD doesn’t fit the 15% rule? Yep. It’s much more common and is the leading cause of death in systemic sclerosis patients. I found that risk factor here.

I brushed up on my probability math (you multiply the probabilities of independent events…), and after running the numbers:

100,000(13/100 x 16/100 x 15/100 x 35/100)

I came to a grand total of 116 other patients in the US who share my set of diagnosed conditions.

Oh.

See, I have lots and lots of stripes. Stripes in purple, teal, periwinkle, red, green, and blue: these are the awareness colors for my conditions.

I just ordered that rainbow zebra unicorn shirt! I plan to wear it with my mask on my next social outing…

You can learn more about Rare Disease Day or my conditions at the links below.

The Scleroderma Chronicles: Rolling Dice with the Reaper

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

An early summer echocardiogram last year showed that I had developed a type of heart failure called diastolic dysfunction. Scleroderma is causing scar tissue to form in the muscle of my heart.
On September 1st I had a right heart catherization that showed that I had a hole in my heart that was disrupting the flow of oxygenated blood to my body. Oh. That explains those blue lips I’d been sporting around town.

Follow-up testing showed that I had exercise-induced pulmonary arterial hypertension. I was started on drugs to treat the condition.
I went into a flare of my systemic sclerosis (scleroderma) symptoms because of the increased blood flow due to the drugs.
Testing in February to hunt for the cardiac shunt (hole in my heart) revealed that there was something going on with my lungs. A subsequent CT scan in March showed that I had developed autoimmune pneumonia in both lungs, a condition called interstitial lung disease. The cause of the ILD was confirmed by a lung biopsy in May. Yep. It is scleroderma related, but I was still forced to get rid of all my down products in the house. I miss my down comforter…
All summer long I have gone through a staggered course of drug interventions as my doctors worked to get my lung disease under control.

This sunflower is a pretty good metaphor for how I’m doing at the moment. I’ve taken a beating, but I’m still blooming, by golly!

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

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

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

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

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

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

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

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

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

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

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

Take that, reaper! I win this roll!!

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: Matters of the Heart

The Blue-Lipped Zebra (BLZ for short) has been busy the last three months (once she was fully vaccinated for Covid-19) and lots of testing and doctor appointments have happened. Lots of diagnostic hypothesis have been pursued and tested; the BLZ has received several emails from doctors that let her know about good news: you don’t have pneumonia!! your heart looks good!! your kidneys are maintaining!!

All is good. Go visit your baby bunny and don’t worry about it…

Hello. BLUE-LIPPED Zebra. Chest hurts. Zebra is dizzy. Zebra pants as soon as she moves around. Zebra is absolutely sure that everything is not fine at all!

Did I mention that the BLZ got fully vaccinated? Ever since that happened (and the BLZ got a steroid injection for her ill-behaved hip) her fatigue and brain fog have receded into the background. Quite frankly, the BLZ is feeling pretty frisky and clear-headed these day between bouts of dizziness and panting episodes. She has decided that enough is enough and she is on the move to get to the bottom of what is going on!

She contacted her doctors and health providers and had them forward her the entire text of her test results. She read these results carefully and then spent some time consulting with Dr. Google to figure out what some of these words meant. The BLZ is so grateful for that biology degree and years of related job experiences.

The BLZ has limited systemic sclerosis. What she learned was…

A general rule of thumb, the 15% Rule, can be used to describe the number of systemic sclerosis patients with serious complications associated with their illness. For example, 15% of patients will have Sjogren’s Disease, or digital ulcers, or lung disease, or maybe pulmonary arterial hypertension. These complications are sometimes rare in the general public, but for systemic sclerosis patients they can be common.
A large European study found that the majority of systemic sclerosis related deaths were from heart complications (26%) or were pulmonary arterial hypertension (26%) related.

Time to stop and smell the roses. This is a little disappointing… my doctors have been reassuring me that all is fine because they are focused on lung disease. There are a lot of bread crumbs in the test results that suggest heart problems.

Then then BLZ made an appointment with her internist (the primary care physician) to go over the test results with her and to help her prep for her cardiologist appointment next week. Don’t you think that was smart?!!!

Here’s the summary of my appointment with my wonderful internist. My face was blue and I struggled with dizziness in her office: she entered a new diagnosis into my chart that says I’m cyanotic and told me to press the cardiologist for a prescription for day time oxygen so I can carry portable oxygen with me. (“Now we’re talking!!!” barked the BLZ.) She read the test results for my CT lung scan and echocardiogram and agreed with my understanding of what the test results were saying. She told me what tests to ask for from the cardiologist at my appointment. Here’s the summary:

I have physical findings in my lungs that consistent with pulmonary arterial hypertension. The summary results of that test say “mild to moderate” and even say that the loss of lung tissue and an enlarged pulmonary artery are due to PAH. Huh. Look at that. (“I’m just shocked, shocked!” snarks the BLZ).
The tissue of the heart (the muscle) is scarred and too stiff to beat well. This condition is called diastolic dysfunction and is a type of heart failure. The echocardiogram states that my diastolic dysfunction is Grade II, which is moderate. Scleroderma is attacking my heart; 15% of systemic sclerosis patients have diastolic disfunction. While there are lots of reasons why people develop diastolic dysfunction, for me the picture is different as it is a common complication of my systemic sclerosis and not a result of say… uncontrolled high blood pressure.
The estimated pulmonary pressure from the echocardiogram is difficult to measure in my case (Dr. Google had to teach me about incomplete TR jet and other obscure heart-related terms) and is most likely being undermeasured. The number now is the upper limit of normal; twice in the past it couldn’t even be estimated.
I have a newly developed hole in my heart called a cardiac shunt.
My heart is ~~broken~~ damaged by scleroderma.

So what should the BLZ do about all of this? The internist and the BLZ hatched a plan in which she should ~~insist~~ request direct measurement of the pressure in the right side of her heart (right heart catherization) and another echocardiogram that looks at that cardiac shunt while she is exercising standing up. Like, maybe the BLZ needs to be climbing stairs… The BLZ is just thrilled… Also, the BLZ wants day time oxygen-to-go. Yes, please. Right now, please.

Also, the BLZ is considering taking someone with her to the appointment and will have the cardiologist send the internist his notes following the appointment.

Also, when life get tough, get a kitten!! BLZs love kittens!

So, this is an adventure in progress, but I do have some gems to share with others struggling with their own medical misadventures. Get your own copies of your test results and physician notes after appointments. Google like crazy to learn what the obscure medical terms mean. Educate yourself about your illness/condition. Stay off social media as you do this and read journal articles from legitimate sources like the Rheumatic Disease journals and articles posted by the NIH. Ask another knowledgeable person to review your test results to help clarify/validate your thinking. If my journey here can serve as a roadmap for even one other person battling their way to a diagnosis, then this post was a success.

And remember to be brave.

It is good to have a diagnosis, even if it is a shame.

Zebras are brave!!

The Scleroderma Chronicles: World Scleroderma Day, 2021

Here we are again… World Scleroderma Day.

I’ve written about scleroderma on this date for several years now. I just went back and read what I wrote last year and decided that I did a pretty good job. I talked about getting diagnosed, the complications that are being caused by my disease, and what that means in my life. Here’s that post if you would like to check out my take on things last year.

This is one crazy-ass disease and it certainly has complicated my life. In the year since I wrote that last post I have hung in there (hey, staying home for a year on oxygen slowly improving was actually good for me) and then emerged from strict lockdown to get lots of testing to try to sort out what is the cause of my blue-lipped status and the source of other little issues that I have going on. While thinking about what to write today I thought of a few things that I haven’t written about before and some things that are new since the last post.

The correct name for the type of scleroderma that I have is limited systemic sclerosis. Systemic means that every part of my body is being impacted by this disease, and the word sclerosis means that scarring is happening in tissues all over my body. What’s happening to me is not obvious to the outside viewer, but it is insidious and ongoing all the same. Somehow, by some mechanism that isn’t clear, my systemic sclerosis is being driven by antibodies that I’m producing that react with the centromeres of my cells.

What’s a centromere? I’m shocked, shocked I tell you, that you just don’t know that! Seriously, the centromere is that little place in the middle of a chromosome that can be seen when cells are getting ready to divide. This is a little tricky because the only time we can see chromosomes is when cells are getting ready to divide and they have already copied themselves: the little pinched waist in the middle of the chromosome where they are connected is where the centromere is located.

Got that?

This image belongs to the NIH; as a taxpaying citizen I hope that it is okay for me to use it here. 🙂

Somehow something happened to a protein located in the centromere area of the chromosomes in my body that made it look “different” and my immune system responded by making antibodies against that protein. The antibodies that are produced are called “anti-centromere antibodies” and their presence is highly suggestive of my form of systemic sclerosis. The alternative explanation is that my immune system just went berserk, decided to attack my own cells, and began making these antibodies on its own. I personally think that something changed and then the antibodies were made as a response. My money is on a virus.

It really doesn’t matter all that much since, once the immune system is triggered, the immune response can’t be turned off and one day you are sat down in some rheumatologist’s office and carefully told about what is happening to you and what to expect in the future. Your skin will get thick. Your blood vessels will be so damaged that they will thicken and spasm shutting off blood flow to parts of your body unexpectedly. The scarring tissue will build up in your digestive tract and damage the smooth muscles that you need to move food along. Your kidneys will lose function. Your nerves will be damaged. Your lungs need to protected since they are especially vulnerable to damage from accidently inhaled stomach acid. If your disease flares badly enough you can develop autoimmune pneumonia. Your tendons, muscles, and joints are all in trouble. You are in trouble. The damage will progress and there is no cure.

“This is really serious,” one of those early doctors told me. “This is like a diagnosis of cancer, but of course, some cancers can be cured…”

Well, shoot. Good thing there are drugs to help control symptoms and to slow the progression of the disease by dialing down the immune system.

Yay for drugs!! I am on drugs to shut down my stomach acid production (my lungs are doing pretty well) and on drugs to crush my immune system into submission. I’m on anti-inflammatories to control other cellular pathways activated by those ill-behaved antibodies. I get steroid injections to help my damaged joints and inflamed tendons. I have strict dietary limitations. I do lots of physical therapy. I’m on oxygen overnight to keep my red blood cell count in a normal range. I dress in layers to help control the spasms of my circulatory system, a phenomenon called Raynaud’s. I knit almost every day to keep my fingers from stiffening up and contracting. I now have a shiny purple cane to help me walk.

Every day is a challenge, but I am fine.

So what is new this year?

Scleroderma has damaged my heart. They are still testing to clarify exactly what is going on, but so far they have established that there is scarring in the heart muscle that is making my heart “stiff” and that somehow I developed a hole in my heart. There are suggestions of pulmonary hypertension, but I need more testing for a definitive diagnosis. No wonder I turn blue in the face and pant when I walk.

Oh, yeah, there is also a pandemic going on.

One of the greatest ironies of the last year is that serious cases of Covid-19 share similarities with the clinical symptoms of scleroderma crisis. Immune system-mediated pneumonia caused by an overreacting immune system is a hallmark of both conditions. Then there is this… Remember those drugs that I take to crush my immune system into submission? I take two drugs for that purpose. One of them, hydroxychloroquine, was (irresponsibly) politicized by influential individuals early in the pandemic and therefore became short in supply; my muscles and joints immediately rebelled when my supply lapsed early in the lockdown. The other drug that I take, mycophenolate mofetil, is linked to poor response to the Covid-19 vaccine. I am vaccinated and I am making anti-Covid antibodies, but since my total antibody count is very low my doctors aren’t sure I can fight off a Covid-19 infection fast enough to stay out of the hospital. I’m advised to continue to mask and isolate because… blue face, hole in heart, stuff like that…

For me, lockdown goes on.

So, here are the takeaway messages from this post. Be kind. There are illnesses that are really debilitating that you can’t see when you look at that person walking into a store from a handicapped parking spot. Don’t be dismissive of conditions with funny names that you’ve never heard of before. I know that it is hard to understand conditions that are beyond your experience and that you can’t really see, but take a moment to let someone tell you about their illness and the daily challenges that they embrace. If you meet a person with an autoimmune disease, they deserve a hug. If they have scleroderma you should give them two hugs! Be understanding of people who are still wearing a mask in public; perhaps they are braver than you can imagine.

Today is World Scleroderma Day.

Go Team Teal!!

Note: If you would like to know about different types of scleroderma you can learn about them here.

The Scleroderma Chronicles: The Blue-Lipped Zebra Gets a Credible Hypothesis

At last.

If you have forgotten about the tales of the Blue-Lipped Zebra, that’s what I’ve taken to calling myself as I struggled over the last few years for some definitive answers to my blue-lipped shortness-of-breath issues. Really, things got pretty darn extreme late in 2019 and I started to get really pushy in finding some answers. Here’s the posts that I wrote then: The Blue-Lipped Zebra Report, The Blue-Lipped Zebra Gets Some MRIs, and The Blue-Lipped Zebra Goes on Oxygen.

Hannah: All of that happened before I came to take care of the Kitten Mom.

To summarize all of the adventures of the BLZ (that is code for Blue-Lipped Zebra), about 5 years ago I caught the flu and was really sick. I never fully recovered as shortness of breath lingered and lingered, and eventually I noticed that my lips turned blue after showers and when I climbed the stairs. I steadily worsened and began to call (and call, and call…) my rheumatologist for help. He ordered up some testing and referred me to a pulmonologist. My echocardiogram and lung CT scan looked good, but my pulmonary function test showed some asthma. My pulmonologist decided to that I must have asthma because of my Sjogren’s Disease, and maybe… (cue the dramatic music)

Sleep Apnea

so I got tested for that. That test showed that I had nocturnal hypoxemia (like, I was under 90% saturated blood oxygen for over an hour) so I was started on overnight oxygen. All my doctors dusted off their hands, said “Job done!” and that was that. As far as they were concerned the BLZ had been put out to pasture.

My symptoms improved and after 6 months I was taken off the oxygen again.

Over the next two years my symptoms came back and got steadily worse. To further complicate things my systemic sclerosis and Sjogren’s symptoms also worsened. I was constantly fighting a flare of my autoimmune diseases (joint and muscle pain, fatigue, brain fog, hair loss, GI nastiness) at the same time I struggled to walk without stopping to put my head between my knees because I felt faint. I ended up on the floor more than once. I panted like a freight train when going up stairs and now my entire lower face was looking blue at times. I coughed up mucus every morning and it sometimes contained streaks of blood. The BLZ was back and running wild. My rheumatologist ordered tests; once again my heart and lung tests looked good. Disgusted with my complaining, my rheumatologist decided that maybe I should be prescribed antidepressants, or maybe I should be tested for… (cue the dramatic music)

Sleep Apnea

I fired that doctor and found another. I met with my internist, who ordered more testing, and I had a first appointment with the new rheumatologist who had been recommended to me by the local chapter of the Scleroderma Foundation. The new tests showed that I had severe inflammation of my tendons, a destroyed hip joint, a condition called polycythemia (too many red blood cells), and nocturnal hypoxemia again. I was put back onto oxygen and the pandemic then closed everything down. I asked about the possible cause of my polycythemia, but there wasn’t anything that really jumped out to my doctors. The BLZ was ordered into strict isolation for the duration of the Covid-19 emergency.

Now I am fully vaccinated and back into the world and pushing my way though new testing and have acquired a couple of new doctors after ending up in urgent care with chest pain, a blue face, and shortness of breath. The testing early this year showed that I had more fluid around my heart and that the pressure on the right side up my heart was up into borderline high range. Because of my systemic sclerosis I am high risk for a condition called pulmonary arterial hypertension so that high pressure reading triggered an alarm: I was sent to a cardiologist.

The cardiologist was completely dismissive of the two clinical observations that had generated the cardiology referral, was borderline disparaging of my anxiousness about my not-yet-vaccinated status (there are other people who are sicker…), lectured me about getting cardio in three times a week, and insisted that I be tested for… (you know the drill: dramatic music time)

Sleep Apnea

“At least you didn’t offer me antidepressants,” I said. I limped away, fighting tears and panting for air, wondering why I have to keep firing doctors and hunting for new ones. “Suck it up, Buttercup,” said the BLZ as I drove home. “You have a new pulmonologist to talk about this with.”

There I am, waiting for the pulmonologist, minutes before he gave me the unifying diagnostic hypothesis.

Three weeks ago I met with the new pulmonologist, one that was recommended by my new (wonderful) rheumatologist. He asked me lots of questions, dismissed the notion that I have sleep apnea (“What a shock!” snarked the BLZ), and then dropped the bomb. I have a cardiac shunt. Blood from the right, unoxygenated side of my heart, is passing through a hole in my heart and disrupting the flow of oxygenated blood to my body. He ordered a new echocardiogram with bubbles to look for the hole and to confirm the diagnosis. He also wants to check how much fluid is around my heart and is concerned about the right side pressure levels, which were the two reasons why I was sent to that cardiologist (that the BLZ wants to kick in the face…) in the first place.

Hannah: The new echocardiogram is in three more weeks. The Kitten Mom is getting a little anxious while she waits…

As the pulmonologist walked me to the door out of the clinic that day he casually said to me, “I really admire your attitude.”

Oh, oh.

The BLZ’s whiskers started to tingle.

You know I googled for information about cardiac shunts from the parking garage before I even drove away from the appointment. Almost immediately the condition that he suspects appeared on the page: Eisenmenger syndrome. I have every single one of the symptoms that are listed on the page. This is the unifying diagnosis, if the echocardiogram confirms it, that explains the blue face, shortness of breath, nocturnal hypoxemia, polycythemia, and the inflammation that has been driving my two autoimmune diseases, systemic sclerosis and Sjogren’s disease out of control.

Why did it take so long to get here?

Eisenmenger is rare.

The BLZ is barking with laughter.

Me, I’m working on my attitude as I wait for the echocardiogram appointment.