Pulmonary Arterial Hypertension – Yarn, Books & Roses

Thoughts on the Night of the Waning Cresent (Beaver Super) Moon

The bright moon that I watched rise through the trees to the east almost two weeks ago is now just a shining sliver in the western sky, invoking a glimmer of joy before it dips behind the Rocky Mountains. Goodbye, Beaver supermoon. You were really special.

I blogged about the Beaver supermoon here, and in that post I wrote about beavers, my burst of energy and surge of creativity as I worked through a pause and found new projects and books to read. I mentioned at the end about my community work making chemo hats. port pillows, and zipper pouches, and hopefully wrote: “Like the beaver, I hope that my work will ripple out and bring change in my community around me, supporting lots of new life.”

In the two weeks following that post there have been returning ripples and glimmers that were so intense that they were more like flashes of light akin to a lightning strike. Feedback that left me stunned and in tears. There’s a whole backstory here, so it will take a little to explain it all to you. Maybe you should grab a cup of tea and find some cookies. Ready? Here we go.

In 2014, after years of medical gaslighting, I was diagnosed with systemic sclerosis (a form of scleroderma) and Sjogren’s Disease. I was started on some medications, lots of tests were ordered, and just like that, my view of my future changed forever. I learned that there was a 50% fatality rate for my disease. I failed the first two drugs used to try to slow disease progression. Follow-up testing after a year showed that I had declined 27% in my lung function, and I was referred to palliative care. I was in grief. I began to compulsively knit. Overwhelmed, unable to cope with actually creating a garment that would fit, I made shawls. Lots of shawls.

I was moved to new drugs. I started a third immunosuppressive drug, one that was off-label and required a fight with the insurance company, and I began to slowly improve. Palliative care discharged me. I found more beautiful yarns to love, and more shawls to knit. The shawls began to pile up along with the number of diagnosed complicating conditions that were linked to my underlying autoimmune diseases, but I was okay; I had essentially knitted (and blogged) my way through grief, and I was now ready to take things on. I found new doctors who became collaborative partners in my care and faced down the monsters of new complications. Today I am much, much better than expected; my latest lung testing shows that my lungs have regained more function, and my PAH is under control. My cardiologist rarely mentions heart failure when he talks to me, and I am off oxygen.

As I got better, I began to knit sweaters. Lots of sweaters. I began to look for a home for the shawls. Last spring a friend mentioned the needs of patients at a rehab center in Estes Park, Colorado that she worked with. People often arrived there precipitously with little more than the clothes on their backs, and they needed warm clothes. She was thinking hats, mittens, and scarves, but I sent about 10 shawls.

I thought maybe someone would be able to use them.

Saturday, I asked her what had happened to the shawls. The rehab center has the shawls all displayed on quilt hangers that they installed, and patients take them to wrap up in when they go to meetings or whenever they need the comfort of yarny goodness. Instead of going to just a few patients, they are there for all, part of their recovery journey. Evidently, they are popular, and the center could use more. I was stunned, struck by a glimmer so intense that it was a bolt. I started crying. Those shawls, those things that brought me through a really bad time, are now doing the same for others. I had hoped that my work would ripple out a little, but this was so, so much more than I expected.

I have bundled up all of my remaining shawls, keeping only three back for myself, and I plan to send the rest up to the rehab center before the end of the year.

Shine on, Beaver Supermoon, shine on.

Footnotes:

Another glimmer: my son’s three cats were rehomed together to a wonderful lady who had lost a beloved cat. All three kitties are now happy in their new home, piling on and cuddling with her while she crochets in the evenings.

Look! Tachycardia!! I was reading a book when this happened.

My medical adventures continue, but after conferencing with my doctors following the latest round of testing, we have all decided to delay starting a third medication to treat my PAH (that’s pulmonary arterial hypertension if you are new to this blog…). That is kind of huge. I have SSc-ILD (interstitial lung disease associated with systemic sclerosis… do you see why they use acronyms?…), but I am not putting down scar tissue (fibrosis), and that is even more huge: it is rare to have one without the other. Do you see the glimmer? My prognosis for this condition, the leading cause of death for patients with systemic sclerosis, is stabilizing into the “she’s doing really well” column, and that is why we can afford to delay this drug.

My wrists and knee (the one that was injured in a fall this summer) concerned my rheumatologist, and she has ordered specialized testing, but all things considered, I am doing really well.

Glimmers and ripples.

The best two weeks ever.

Did you enjoy your tea and cookies?

The Scleroderma Chronicles: This is World Scleroderma Day!

A couple of weeks ago I went in for a routine blood draw at my local Kaiser clinic. I gave the phlebotomist my ID card and told her my name and birthdate: for some reason they like to verify these things, right? Anyway, what happened this time was… she exclaimed that didn’t look my age. (Well, yeah. That may be one of the bigger red flags for scleroderma… no wrinkles.) “It’s just my disease doing that,” I assured her. “I really am that old.”

This lady wanted to know what I had done to get rid of the wrinkles. She wanted this magic wrinkle remover too! I carefully told her about scleroderma. “I never heard of that!” she said. Still thinking that I was a lucky person, she drew my blood and I got out of there. I would be happy to have every single wrinkle that I have earned over the years, but no… I have scleroderma. The systemic form with limited skin involvement. Lucky me.

I do have wrinkles around my chin and mouth, but I was wearing a mask during the interaction with the lady at Kaiser. I had to go on oxygen this day (I have lung and heart involvement) and you can see the swollen tendons in my hand and the tight skin over my knuckles.

That’s why there is this thing called World Scleroderma Day. This is a complex autoimmune disease with illusive symptoms (brain fog… trouble swallowing…GERD…swollen fingers…trouble breathing sometimes…fingers that suddenly lose circulation and turn white or blue…) that make the disease difficult to diagnose without specialized testing and evaluation by a scleroderma specialist.

Systemic sclerosis (the form of scleroderma that I have) is thought to start with some event (still unclear) that triggers the formation of antibodies that target specific molecules the nucleus of cells. These antibodies lead to damage in blood vessels and their linings; the damaged blood vessels release chemical signals that trigger a cascade of events that impact other cells of the body. Some cells are related to inflammation. Other cells are tipped over into forming uncontrolled scar tissue (fibrosis). Here is the problem: the tissue engaged in fibrosis is all of the connective tissue. What is connective tissue, you ask? Think of all the ways a person is held together. Tissue that makes your skin stretchy. Tissue that holds your muscle fibers together. Tissue that makes up your tendons, ligaments, and the structure in your joints. Tissue that holds your intestines, heart, kidney, and lungs together. The cartilage in your ribs. All inflamed, under attack, and forming thicker and thicker layers of scar tissue. Some of this damage causes calcium deposition. Unobservable from the outside, the patient is slowly hardening inside. The most obvious symptoms is the skin thickening and then pulling tight over time.

Scleroderma, and especially systemic sclerosis, has the highest fatality rate of all rheumatic diseases. Did I mention that there is no cure? There are treatments for complications that develop like pulmonary arterial hypertension (PAH) and interstitial lung disease (ILD), but they, like the immunosuppressant drugs that many of us take, mostly stabilize and slow progression. (Dear Kaiser lady… that’s why I was getting a blood draw. The drugs that I take are risky, and my doctors need to make sure my kidneys and liver are tolerating the meds okay.)

So, what is the purpose of World Scleroderma Day? It’s to highlight this condition that impacts more people than it should even though it is rare. It’s to raise awareness of the symptoms and hopefully aid in other people getting an appropriate diagnosis. Maybe shining a spotlight on scleroderma will help in getting funding for research, treatments, and even a cure.

Sunflowers are the international symbol for scleroderma.

Here is where you can learn more about scleroderma, it’s symptoms, and treatment.

PS How was my blood work? Yay, doing okay!! Kidney function is up into normal ranges again!!! How old am I? I was born in 1951. 🙂

The Scleroderma Chronicles: True North

It has been quite a week. Something happened that put me into a snit that made me think about priorities all week long. Actually, several things are happening all at once and it has taken me days to sort things out. Let’s start with the snit, okay?

These are the chicken sisters, and even though they are really cute, they are the focal point of the snit that has made me rethink my priorities.

I started making emotional support chickens to give to people who needed… well… emotional support. People who are dealing with grief, or anxiety, or an uncertain future, or struggling with medical challenges. I want to send something to people like me who are dealing with something significant and life-altering that is also mostly invisible to other people. The chicken says (Ba-BOK!!!) I see you… I am here for you… give me a hug. That is the mission. It falls under the overarching mission of Knit Out the Yarn Stash Before I Die. Hey, I have a lot of yarn, so there is a sense of urgency there for me.

Well, the day I took a chicken to my knitting group seeking to enroll others to knit chickens things kind of backfired. As in, people begged me to make them chickens. Ugh. I couldn’t say no, because all of these ladies are making/giving things for others in the same circumstances as the people I gift the chickens to. I signed on to knit 5 chickens with the understanding that each chicken would require a cash donation to Frayed Knots. I knit some chickens, posted the pictures, and it was a chicken free-for-all as people reserved the chicken that they wanted.

Here they are, the first chickens looking for a new home.

Four chickens were grabbed right away. Then the problem arrived. One of the members of the board wanted the little raspberry and grey chickens, even though they were already adopted out. The words “those chickens are gone” did not work. She absolutely had to have those chickens. It was a close thing, but I did have enough yarn left over to make the two new chickens.

When I sent her the pictures of the finished chickens and told her they were done she was greatly disappointed that I hadn’t also made a black chicken that we had mentioned while she was debating her options. (Listen, she decided on the two chickens instead of that black hen, so this was a shock!) I told her that I didn’t have the yarn to make that chicken in the stash. She told me that she would buy the yarn. I was like… NO!!! Please do not buy me any yarn!

Overarching mission: Knit Out the Yarn Stash Before I Die

Which has kind of been on my mind since my heart is kind of acting up right now. I am having sudden attacks of extreme breathlessness with chest pressure and dizziness. My oxygen levels seem to be okay, but my Fitbit has been sending me alarms when it happens. My fatigue has gotten much worse and it kind of hurts to breath sometimes.

The really crazy Fitbit shot with lots of peaks happened when I tried to knit a chemo hat on my little knitting machine. I mean, making hats while trying to control the cats is stressful enough, but the constant Fitbit alerts are just piling on at this point!

See the quality help that I’m getting while making the hats?

I seem to do much better if I wear oxygen while working with the machines, and I get fewer alerts while working on the smaller knitting machine making fingerless mitts.

So, it was a week of internal conflict. The lady who wants a black chicken is still not happy. My heart is not happy. My yarn stash is not shrinking, and somehow the joy of knitting chickens is gone when I have to make them in the exact colors that other people want. It is a chore when I don’t get to be creative, especially when I know that the chicken is going to someone who just wants a chicken, as opposed to someone who needs a chicken.

Hannah: On Friday the Mother of Cats pulled herself together, reset her priorities, and took some action!

Friday morning, I woke up, made my latte, sat outside with the cats, and decided to push the tiller over and return to a course of true north. I will remember my overarching mission statement, and I will do the things that help other people like me: people with chronic conditions that are isolating and mostly invisible to others. I will spend my time doing the things that feed my own creative spirit and my need for a sense of purpose.

I emailed my rheumatologist to ask if she would like fingerless mitts to give to other rheumatology patients. The answer came back in less than an hour: Yes, please!!!! I have my purpose again, and knitting out the yarn stash is back in business.
I emailed my cardiologist to let him know what was happening and attached the Fitbit pictures.
I took down a sweater (La Prairie) that has been languishing for months and started in on finishing the first sleeve. Gosh, it is going to be cute. Time to knit for me again!
I packed up the chickens to deliver to Frayed Knots. Those chickens are the last ones that I will knit for people who want chickens.
Saturday, I handed the chickens over to the head of Frayed Knots and asked her to please explain to the lady who desperately needs a black chicken that I’m done knitting them. The words I used were, “I’m not in the chicken knitting business, I’m in the emotional support business.”
I then sat with another member of the group and helped her knit her way through her first chicken. Suddenly, two other people wanted to learn. Yay! I am there for them!! Eventually, I am going to get some chickens into infusion centers for the patients!!

I have two little bracelets on my wrist with the Fitbit.

What was it that helped me pull things together Friday morning while drinking my morning latte? Those two little bracelets on my wrist in scleroderma teal. The little silver spoon was given to me by another patient, and it symbolizes the balancing act scleroderma people go through to manage our fatigue. We are “spoonies”. I need to remember to be ruthless about managing my own energy and resources. The teal beaded bracelet has a silver strip that says, “Remember Who the Fuck You Are“. Yes. I need to remember to not let other people run me over with their needs, because… limited resources. I need to set my own priorities with my limitations and needs in mind. I also need to remember to be brave, to face down the monsters, and to let my doctors know when new symptoms appear.

Just like that, I found my way again.

True North.

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 Scleroderma Chronicles: A Decade on the Little Teal School Bus.

Here it is again: World Scleroderma Day.

June 29th is World Scleroderma Day. In Australia sunflowers are used as a symbol of scleroderma. Almost everyone uses the color teal for scleroderma.

I used to be a high school biology teacher before I became a scleroderma patient, and I took my students on field trips sometimes. We would all pile on the bus and off we went on one adventure or another: into the mountains for an ecological assessment, or to the Natural History Museum for an anatomy lesson, or a visit to a biotechnology center, or even off for the weekend to study for the Biology AP Exam. It was always exciting, exhausting, sometimes joyful, often a little overwhelming, and at the heart, an educational experience.

Lately I have been thinking about my illness as a ride on a little teal-colored school bus with a crazy rainbow striped zebra behind the wheel. That dang scleroderma zebra is careening down the road on its way to an unknown destination just over the horizon, and would you believe it, he keeps stopping to pick up more passengers along the way.

Don’t make fun of my bus! I changed the colors on some clip art that I found, and putting a zebra behind the wheel was beyond me!! Use your imagination… the zebra is braying hysterically while driving the bus!

When the bus stopped for me, and I stupidly jumped on board, I only had a few symptoms. I had some trouble swallowing sometimes. I had GERD. I suddenly lost circulation in my fingers if I got cold. Speaking of my fingers, they were pretty fat and puffy. I had lots of red blotches on my face, and the skin was pulling tight. It was hard to open my mouth wide…

Still, I was feeling pretty hopeful as I jumped onto the bus. “This will be fun!” chortled the scleroderma zebra. Bad zebra, bad!! Before I knew what was happening, that dang striped miscreant had pulled the bus over, opened the door, and couple of little demon passengers had hopped on board: kidney disease and gastric complications. What kind of an outing is this… have you ever heard a zebra laugh?

Bouncing down the road, suddenly screeching to a halt periodically to pick up a new passenger, the zebra continued the crazy outing in the little teal school bus. The little demon passengers kept piling into the bus, and those little monsters even started to sing “the wheels on the bus go round and round…” while laughing and clapping. What kind of a field trip is this, anyways?????

The demons all wore little nametags:

Gastroparesis
Pericardial effusion
Chronic respiratory failure
Diastolic dysfunction
Pulmonary Arterial Hypertension
Interstitial Lung Disease
Heart Failure with preserved ejection fraction

“STOP THE BUS!!!!” I shouted at the zebra! “I absolutely did not sign up for all of this sh*t!!!!” Nope. Evidently there is no stopping the bus. Ten years on the road, and we are still on our outing. Somehow some extra demons that don’t even wear nametags snuck onto the bus, but they are certainly annoying as they are making all my tendons hurt and what is up with all this edema!!!! Did I mention the fatigue? Always, always there is fatigue. That fatigue demon is sitting on top of the bus blowing raspberries at all the other people on the road…

It has now been a decade for me on the little teal school bus: always exciting, exhausting, sometimes joyful, often a little overwhelming, and at the heart, an educational experience.

I learned about prioritizing and not worrying about things that haven’t happened yet. I learned to advocate for myself, and I have maneuvered myself into the care of some great doctors. I have learned to build for myself a network of supporters. I pretty much have lost interest in making money, but I’m highly motivated to help others. I’m keeping notebooks and collecting souvenirs while on this field trip, and to be frank, it is the outing of a lifetime. Believe it or not, I’m now singing along with the little demons on the bus with me…

The wheels of the bus go round and round… and the zebra is still laughing its head off… and I’m okay.

Happy World Scleroderma Day.

Note: Scleroderma (systemic sclerosis) is a rare autoimmune disease that is chronic, progressive, and often fatal. It has three main hallmarks: damage to blood vessels, the development of autoantibodies, and subsequent scarring of tissues and organs. Right now, while there are many excellent treatments emerging to handle the serious complications due to the underlying disease (like my pulmonary arterial hypertension and interstitial lung disease), there is no cure. You can learn more about scleroderma and systemic sclerosis in the links below.

The Scleroderma Chronicles: The FDA, the Supreme Court and Unintended Consequences.

The United States Food & Drug Administration has really been on my mind this last week. Let me set the background: I have a rare, progressive autoimmune disease that at this date has NOT ONE SINGLE DRUG that can directly treat it. Not one. There are drugs that target symptoms and the complications of my disease, systemic sclerosis, but none that can shut the disease down.

Over the last few days two alerts about new treatment developments for systemic sclerosis hit my newsfeed. One of the drugs, Certa Therapeutics’ FT011, is designed to treat chronic fibrosis and was just granted FDA Fast Track status. After a 12-week trial 60% of the systemic sclerosis patients had clinically significant improvement: I suspect that they are talking about lung function here. This is huge! This is the drug that I have been waiting for ever since I quit the anti-fibrotic drug OFEV last summer due to intolerable side effects. Fast track status means I may get this drug in another year or so. THIS IS HUGE, PEOPLE!!!! HUGE!!!

How about a break? Here is my monster orchid 4 years ago today.

Just a couple days after the news about FT011 another news alert, even bigger news, came that Cabaletta Bio’s CABA-201 drug had been granted Orphan Drug status by the FDA. I’m not completely sure, but this seems to be a type of CAR T-cell therapy that would provide an immune system reset: a cure. Did you catch that? A CURE!!!!! The disease that I live with, systemic sclerosis, could be stopped dead in its track if this works. Orphan Drug status provides some financial incentives and helps in bringing the drug to market, but it doesn’t speed up the process like the Fast Track status will for FT011. Still, this is good news arriving all at once. I have a sense that momentum is building as these new, very sophisticated drug treatment strategies come to market based on specific molecular interventions in the patient.

Anyway, none of this going to happen overnight because the FDA approval process is very slow and painstaking. This is the way it needs to be to develop drugs safely. Drugs are first visualized based on knowledge of the regulation and complexities of biological systems. “Oh, that’s a good idea for a drug,” some scientist tells themself, thinking about a regulatory pathway in humans. They follow through on their idea and then see if it works in a specific science-based process that tests the drug in lab, animals and then finally humans to see if it will treat the disease/condition.

Mateo: Hang on, everyone. The Mother of Cats is going to go all science geek now…

For example, one of my drugs is called Letairis. It is designed to treat pulmonary arterial hypertension, and it is an endothelin receptor antagonist. What the heck is that, you ask? As you might, because who in their everyday life would need to know about this stuff, right? Maybe you should skip this part if you are feeling sleepy… You’re still reading? Wow! I’m so impressed and grateful for your trust… Well, anyway, here is the very short version at the Midnight Knitter level of understanding: endothelin is a small protein produced by the cells lining the inside of my blood vessels that causes blood vessels to constrict. The drug that I take, Letairis, is a sneaky molecule that mimics endothelin; it binds to the receptor on the target site and blocks endothelin, keeping it from attaching to the receptor. The drug prevents my blood vessels from constricting and keeps my blood pressure in my lungs low. Yay!

Anyway, some scientist long ago had an idea that maybe blocking the action of endothelin would be a good way to control pulmonary hypertension. This idea was tested in the lab, then on animals, and then if all seemed okay it was tested on a very small group of humans, and then larger groups of humans. Data is collected and analyzed to look for the efficacy of the drug while also identifying all the possible side effects. There is a lot of risk/benefit analysis before the drug is released to market. After that more data is collected to hunt for bad side effects once the drug is being used in this much larger market.

I guess my point is, this is a long, long process with lots of safeguards along the way. The FDA is the agency that provides the scientific guard rails that protect me and every other drug consumer in the US from bad information, harmful drugs, and unscrupulous people who push pseudoscience treatments in order to make a buck. Thank you, FDA, for providing this essential service for me and every other American whether they appreciate it or not. I’m glad that you do this, even if it means I have to wait for my new drug that is slowly working its way through the process to come help me.

Mateo: Now the Mother of Cats is getting political. Watch out everyone!!

Today the United States Supreme Court heard arguments about the abortion drug mifepristone that centered around its approval by the FDA and the decision by that agency to allow it to be delivered by mail. I’m convinced that the issue has been raised solely because this drug is used for abortions, but the arguments brought before the court are suddenly extremely pertinent to me and my own situation.

I’m pretty sure that not one of the justices on the supreme court is qualified to make a judgement about the scientific process used to develop this drug and the analysis that was made about its safety. Just as I wouldn’t allow one of the large pharmaceutical companies to rule on a matter of law, I am alarmed that the courts are now going to second-guess a science-based agency.

I am also extremely concerned about the court deciding whether drugs can be sent to patients through the mail. It has to do with the ancient, mostly forgotten until now, laws on the books about drugs that can be used for abortions or contraception being delivered by mail.

Remember my drug Letairis? I need it to control my life-threatening pulmonary arterial hypertension that was gifted to me by my systemic sclerosis. This drug has a rigorous enrollment process and requires female patients to use two forms of birth control and to take a pregnancy test every month before they can get the next 30-day supply. Each month this drug is delivered to me by overnight express from a pharmacy in another state. This drug can harm an unborn child and may create the need for an abortion. Suddenly the arguments that were made today before the US Supreme Court threaten me and my access to medical care.

I sure hope that Certa’s FT011’s progress on the Fast Track isn’t affected by all of this. An upended FDA approval process could be disastrous for me and a lot of other people waiting for a new drug to arrive to save their butt.

Unintended consequences are a bitch.

Update 3/27/2024: While I was writing this post yesterday, the FDA approved a new drug for pulmonary arterial hypertension (WHO Group 1). The relief and celebration in the online support communities this morning was pretty amazing. This is good news for me, too, as my PAH is in Group 1. Yay science!!

The Scleroderma Chronicles: Rare Disease Day 2024

Rare Disease Day is tomorrow, but since I’m going in for a lung scan on the 29th, I thought I would post this now.

Well, here it is again. Rare Disease Day. This is my 9th year posting about rare diseases: on August 28, 2014, I was diagnosed with a form of scleroderma called limited systemic sclerosis. This condition, autoimmune in nature, is progressive as multiple organs, blood vessels, and the skin of the patient (that would be me) stiffen and harden due to scarring (fibrosis). It is also considered rare, since fewer than 200,000 people in the US are currently diagnosed with it.

So, what’s up with the zebra? Well… in the medical community doctors and other health professionals are trained to focus on the most common cause for the symptoms that they are seeing in their patient. The saying goes: when you hear hoofbeats, think of horses, not zebras. Certainly, that makes a lot of sense in terms of patient care and cost control. It works most of the time.

Unless, of course, you are a zebra.

Life is challenging if you are a zebra in a herd of horses. Doctors screen for the most likely cause of reported symptoms, and then when those tests are negative, you usually get told that you are fine, and then you are pawned off with some pablum like… do these exercises… try to reduce stress…would you like anti-depressants?… Seriously, it is pretty crushing as you start to wonder if you are just an attention-seeking hypochondriac since for the most part you look great. In my case, I didn’t even appear to be aging…

This can go on for years. And years. Autoimmune conditions like mine tend to send patients with vague complaints (I hurt all over…) to the doctor’s office looking for help. Some of the symptoms can be so subtle that you just don’t think to mention them to the doctor because you already feel pretty defensive about complaining after a few borderline disparaging interactions in the past. Why mention that you have trouble swallowing sometimes? Or that your arm is starting to look like Flipper the dolphin in appearance and texture? Some new red freckles have shown up on your face, but why mention them when you really want to get to the bottom of why you hurt all over and what is up with this fatigue????? Sure, there were those carpal tunnel surgeries 10 years ago, and the hospitalization for gastritis last year, but why would you mention them… When I was finally diagnosed, I discovered that I was absolutely classic and presented with all five of the CREST features of limited systemic sclerosis. Oh, one more thing: lack of wrinkles is a red flag for scleroderma…

You can see two of the CREST characteristics here. I’ve lost circulation in my ring finger as a result of Raynaud’s, and the thick (sausage-like) fingers that are trying to contract are examples of sclerodactyly. The other distinguishing symptoms are trouble swallowing, lumps of calcium deposits on some of my bones, and those pesky red freckles that are now appearing everywhere. It was official: I was a zebra. There are a lot of us.

Rare diseases are also known as orphan diseases. They are poorly supported and most of the time there is no treatment. After failing a chemotherapy drug, I was moved to an off-label treatment using an immunosuppressant drug developed for kidney transplant patients. It was hard to get the drug as it was declined by my insurance, then the appeal failed, my doctor filed another appeal, and I went to see the pharmacist with a pathology report showing extreme gastritis. It was a fight, but I got the drug. This drug, Myfortic (mycophenolic acid), has proven to be so effective in slowing disease progression that it is now approved for systemic sclerosis and is a drug of choice along with its close relative CellCept. It has been doing a pretty good job at slowing things down; at the time of my diagnosis the 10-year survival rate was about 50%. Now that there are some better treatment options the survival numbers have improved: 10-year survival is up to 70%.

Two years ago, I developed two of the more serious complications of systemic sclerosis (SSc): pulmonary hypertension and interstitial lung disease: two more rare diseases. Not good news at all; my pulmonologist told me it would be okay to cry as he showed me my lung scans and gave me the bad news. Remember that little word “progressive” that I used to describe SSc? This is disease progression; the inflammation and formation of scar tissue (fibrosis) had hit my lungs and heart, and my have doctors responded with big time drugs. Yes. Big time and pretty expensive drugs. Over the last few years, literally in the time since I was first diagnosed, drugs have appeared that can greatly improve treatment and life expectancy in patients like me, and there are more drugs in the pipeline.

The first drug that can be used to directly treat SSc just moved from orphan drug status to the fast track. This is serious, serious good news. I looked into entering a clinical trial for this drug, but my doctors thought that I wasn’t a good candidate because I have too many complications right now. Seriously, I have been slotted into a diagnosis called SSc-ILD with PH which means that I am a SSc patient with interstitial lung disease and pulmonary hypertension. Good grief, I’m now a walking bundle of acronyms. That’s okay. I can hang on, help is on the way!! FT011, I am waiting for you!!!

So, what is up with all the colored stripes?

Serious medical conditions usually have an awareness ribbon color. It is a little cheesy, but if you are dealing with scary stuff, why not have some fun? Be a zebra, color your stripes in your awareness colors, and get yourself some fun t-shirts while you are at it. Eat Zebra Food (that would be black and white striped caramel popcorn to you non-zebras), get colored medical bracelets, and collect the stuffed animals. Zebras, of course!! My zebra has teal stripes (scleroderma), periwinkle stripes (PH) and purple for the ILD. Lung disease in general is green and blue. I also get purple for my Sjogren’s and fibromyalgia, and don’t forget the red for my heart failure. I’m a rainbow zebra!! Yay!

Okay, time to get serious. What is Rare Disease Day about? Raising awareness in the public about the challenges of living with a rare disease. There are a lot of people who have rare diseases, so by sharing our faces and voices the hope is that it will help make us more visible. We hope that a more educated public will aid in the diagnosis of others with rare conditions. (See above; diagnosis can take years. It would be wonderful if that could be sped up a little…) We hope it will help with funding for drugs, treatments, and maybe even cures. We hope that for those with invisible conditions, they will become more visible and supported. We hope.

To learn more about my rare conditions and others you can go to:

To everyone who battles on against scleroderma or any other serious medical condition, rare or not, I see you. Hugs! Shine on, my friends, shine on!

The Scleroderma Chronicles: Thoughts on the Night of the Blue Supermoon…

Did you look at the moon tonight? It is just huge, shining in the night like it knows that it is something special. Well, it is. This is the Blue Supermoon of 2023, my friends. Not to be seen again for 14 years. Just the sight of it makes me feel happy.

This is also the anniversary of the day that I was told that I had systemic sclerosis and Sjogren’s Disease. Actually, it is 9 years and one day since I drove to my first rheumatologist appointment; I was a little emotional that morning as I passed fields of beautiful sunflowers, their faces glowing in the light of the morning sun rising behind me. You’d think that the date would be kind of a bummer, but nope. As it turns out, there are lots of things that are making me feel happy at the moment.

Do you see that black bag with my purse and cane? That is my portable oxygen concentrator!

That’s right, after languishing for 18 months on the waiting list, my name came up for the portable oxygen concentrator that I have been desperately needing. Look at that baby!!! It only weighs 5 pounds, it works great, I can adjust the level of flow on the fly, it can charge in the car, and it is exactly what I need. Yay!!! I got it yesterday on the exact 9-year anniversary of my diagnosis.

So happy, feeling absolutely empowered, I headed to the yarn store after picking up the concentrator. Time to take this baby for a spin, right? I have been struggling for months to make a decision about the yarn for the La Prairie cardigan that I want to knit next. I bought a kit of yarn to make the cardigan, but I’m not happy with it. I needed a new skein to go into the mix…

The yarn picture on the left is the original kit. The one on the right is the new variation that I’m not completely happy with. Feeling hopeful and more than a little determined, I sat on the floor in front of the most likely candidate yarns and, rocking my new O2 concentrator, holding up my phone with the yarns on the screen, I kept looking at different ideas for the fade…

Winner, winner, chicken dinner! I suddenly realized that the Stitch Together yarn (second from the top) was exactly what I was looking for. There it is. I asked others in the store what they thought, and the consensus was that I had nailed down my fade. Yay!!! Finding that yarn made me happy.

So, I also bought the special edition Babe set from Spun Right Round.

I’m going to make that Barbie pink yarn into hats for the community knitting group that I knit for because… wait for it… the color makes me happy!! I had one of the original Barbie dolls, and just the thought of Barbie makes me… happy!

On the way home from the yarn store, still sucking down oxygen in the car and feeling pretty good, I stopped at the grocery store to buy some tiramisu because I was absolutely having a tiramisu kind of day, and there at the front of the store were sunflowers. You know, it is the end of August, and it is sunflower time. Shine like a sunflower!!

Pretty good looking, huh. Sunflowers are used as a symbol of scleroderma hope in some parts of the world (Hello, Australia! Talking to you!), and you know I bought these too.

By the time I got home I was tallying up all of the things that made me happy. The leaves are starting to change on the trees in town, and the ornamental grasses are covered with rich golden plumes atop bright green stems. That makes me happy. Pumpkin spice is back at Starbucks. The kittens loved the new toys that I bought them. I ordered new clothes this week that fit great even though they are a smaller size than usual. I found new shoes to wear that are really helping a lot. Happy. I am happy.

The shoes are shaped like walking boots on the bottom, but inside there is great arch support and a cushy insole. I am walking now with much less pain. Did you notice the purple detailing and the silver loops for the laces? Happy. These are happy shoes.

Once home, I headed outside to the catio with my yarn, the tiramisu, and a cup of coffee from my new Keurig machine (yep… happy), and as I set the plate and coffee cup on the table, a pair of adult cottontail rabbits raced around the corner of the deck and zoomed under my side gate. Looks like I will be having baby bunnies again. I am happy.

Nine years ago, I asked that first rheumatologist what my life would be like in five years time. He refused to answer, and it was my first clue that I might be in some trouble here. 18 months ago, my pulmonologist told me it was okay to cry when the first lung scans showed serious interstitial lung disease. One year ago, my pulmonologist told me that they were very worried about me after my lung biopsy… nothing was working, and he wasn’t sure I’d make it. Last month, the technician who did my latest pulmonary function test told me that I was too bad to walk any longer without portable oxygen. I came home, looked at the bottles that are too heavy for me to carry, and cried.

Tonight, under the blue supermoon, with sunflowers on my table, I am happy. One month later, I have my portable oxygen. 18 months later, my lung disease appears to be stable. 9 years later, I’m still here, rolling with the punches of new complications, facing down the monster, and finding ways to shine.

I am happy.

Shine on supermoon, shine on.

The Scleroderma Chronicles: Bioethical Dilemmas and Unintended Consequences

I was a biology teacher in the time of the Human Genome Project. This week, with the coming release of the newest movie about Oppenheimer and the development of the atomic bomb, I’ve been thinking about the DNA and genes again (I know, it is a biogeek thing…), because one of the candidates running for the Republican nomination in the US wants to cut the Department of Energy if elected.

Kind of a loose chain of threads, you’re probably thinking. Am I right? Well… the Department of Energy is the agency that is responsible for the regulation of the nuclear energy industry in the US. There’s a lot of waste coming out of those nuclear reactors, and there was some concern about how much mutational damage was being done to DNA through exposure to radiation. Well, to figure that out, you need to know what undamaged DNA looks like. The initial drive to figure out what the human genome looks like came from that agency and once the results came in early this century the world completely changed. Like a big change. Like an atomic bomb level change. Like, there are now sites that have huge depositories of biotechnical data and tools to aid in research.

In the classroom we biology teachers began to teach about the Human Genome Project and also did a week-long unit around the ethical problems associated with this new knowledge (bioethics, if you will). The kids grappled with dilemmas like… if you had the gene for a fatal, untreatable illness, would you want to know? If you were a child at risk for this gene, would it be okay for your parents to have you tested for it before you are 18? If your unborn child tested positive for this condition, what would you do? Would it be okay for human organs to be grown for transplantation? Who should get the transplant… a single father of 4, or a 16-year-old student in your high school? Should your employer allow you to continue in your airline pilot job if a genetic test shows you are high risk for a sudden cardiac event. Should genetic test results be private? Whew. Lots to grapple with in this unit.

Makes your head hurt, doesn’t it. Check out my knitting progress this week!

So, shit kind of got real this week. One of the members of an online support group for pulmonary arterial hypertension (a progressive and fatal heart/lung condition that I have thanks to systemic sclerosis) has just been identified with a gene (bpmr2) that causes the condition; her PAH is caused by this gene and can be inherited; she has a different type from me, but it is still PAH. Oh, boy. This is not good at all. The life expectancy right now is up to about 7 years, but you only need one copy of the gene to be at risk for PAH… there is a 50% chance for each of her children that they inherited the gene. Only 20% of people with the gene will develop PAH, but that is still a big risk.

Should she tell her two children? she is asking in the forum. They are in their late 20s. If she does, and they get tested, should they have their own children if they have the gene? Her heart is broken, literally.

Life expectancy has greatly improved over the last few years with new medications being generated in the modern climate of expanding cellular and molecular biological information. Untreated PAH (and PH kind of gets lumped together with it according to my pulmonologist…) has a life expectancy of about 2.8 years… not good.

Rose break! By the end of the discussion thread, she was leaning towards telling the kids.

Which brings me to the next shitty bioethical item that occurred this week. One of the members of another support group caught Covid and had to go off her drugs while fighting the virus. She has been slow to recover, and still feels pretty bad, but she took a pregnancy test and restarted her medications again a few weeks ago, only to discover this week that she is actually 12 weeks pregnant. Why did she have to take a pregnancy test before restarting her drugs? Because some of the drugs used to treat PAH can cause extreme damage to a human fetus. The enrollment process is very strict, and every effort is made to keep patients from this situation.

I am in grief for this woman. The doctors think that the baby has been spared the worst of the drug toxicity, but now she is working her way through whether to abort or not, to restart her drugs, or not. To risk death to save the baby, or to abort and restart treatment. If the baby is born in good health, will she live long enough to see it enter kindergarten. If the baby is born with health problems, her medical burden is increased. She is young. This is a horrible mess, and she is already too far along to get an abortion in many states in the US. I don’t know if she has other children, or what her support structure is…

This week I heard that some states are demanding private health records to identify any out-of-state abortions or transgender care that has happened in another state. I hope that this woman lives in another country…

Then I heard that a popular hamburger joint near my home is now going to fire employees who wear a mask. Say, WHAT?! Let me tell you, any person who has a serious lung/heart condition like mine wants to wear a mask, and they are so grateful if the person at the service window is also wearing a mask. Sometimes people offer to put on a mask when they see that I am wearing one. Now that person can be fired for putting on a mask… remember the young PAH patient whose nightmare began with catching Covid? Truthfully, any random virus can cause serious damage to patients with PAH, especially if they are also immunosuppressed.

So, there are a lot of bioethical dilemmas here, and the unintended consequences of people who want to make sweeping decisions without understanding all the interlocking systems involved and the potential ramifications are staggering. NO, you can’t just disband the Department of Energy, and sweeping, inflexible decisions about reproductive issues (that seem to be smugly self-righteous to me) can be disastrous. It is easy to order up genetic tests, but what happens once you have the information can be life-altering.

And don’t get me started on this animosity towards mask wearing…

I can’t help but think that no one should attempt to enact legislation without ~~suffering through~~ experiencing something like the bioethics unit that was taught at the high school where I used to work. I keep wondering, do these legislators actually understand nuclear power and weapons? Have they heard of the Human Genome Project? About gene testing? About rare diseases? They absolutely need to go see the Oppenheimer movie, maybe, and then write me a report about the Human Genome Project. I’m pretty sure that they would struggle with epigenetics, but it would do them good if they looked into it. They can get extra credit for a summary of pulmonary arterial hypertension. I would like to give them a book list of summer reading to get through on their breaks, because only the well-informed and educated should attempt to make decisions about these issues in the seven levels of bioethical hell that is the life of patients like me.

Because this week was a really hard one; for too many people this shit is real.

PS: Have you seen the show House? I kind of think that he could use a little bioethics sensitivity training, too.

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!