SSc-ILD – 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: The 39% Imposter Syndrome

It was the third day in the Intensive Care Unit following my lung biopsy. I was sitting up in the bed, rocking all of my tubes but finally off the high flow machine, drinking a Starbucks latte and chatting with a visitor. The nurse came in the door and stopped short, taking in the scene of me, the Starbucks, and my visitor. “I don’t believe it!” she exclaimed. “They just told me all the things that are wrong with you, and I walked in here expecting to see a gravely ill person. Instead… here you are! Not what I expected!”

There I am, day three post-surgery after ditching the high flow machine for a regular oxygen line.

“Yeah, I get that all the time,” I told her. It’s the curse of chronic illnesses… you adjust and stop acting sick. Your condition is invisible, and it is too exhausting to explain to people what’s wrong when the list of diagnosed conditions is… well… exhausting. Seriously, if you say more than a couple of things it starts to sound like you’re just making things up. It doesn’t help when the diagnoses sound fake to begin with (systemic sclerosis associated interstitial lung disease… it’s called SSc-ILD for short, but still… sounds like something invented in a science fiction novel, right?). It invariably gets worse if people start to share back some rheumatic or autoimmune conditions that they know of (like fibromyalgia or Raynaud’s), and you are compelled to say… “Oh, I have that too…” See what I mean? Attention-seeking hypochondriac is sure to cross their minds. I mean, I would wonder that… It is much easier to cover up as much as you can and act normal.

I’ve been thinking about this a lot. Several patients in the support groups that I belong to have been struggling with family and friends who are unable/unwilling to accept the diagnosis. They don’t want to hear about it, they don’t want their lives to change, and there is no support. There has been heartbreak and tears, to be frank. Others in the groups have shared that their families also refuse to accept the seriousness of their condition(s) because there have been years of struggle to obtain the diagnosis, and after so many doctors have said that it’s all in your head, or, you need to control stress better, they kind of believe that. From the perspective of these family members, their scleroderma family member is just trying to milk the diagnosis for attention. To make it worse, some patients (and this is exhausting, believe me) start to list all of their symptoms as if to justify their diagnosis. It is a little desperate, and more than a little heartbreaking.

Hannah: the Mother of Cats is making a new patient in her Zoom support group an emotional support chicken!! Her family is unwilling to become involved as she grapples with the implications of her new diagnosis.

Then there is the more insidious problem: what did you do to make this happen? I have been asked that, and it usually is followed by advice about things that I can do to control my scleroderma and the resulting lung/heart complications or even how to make myself get well. The implication is, if you don’t do this (herbal treatment, sunshine, exercise, wacky stem cell injection), it is your own fault that you are sick!! It happens to a lot of us. It eats away at self-confidence, and makes me, and other patients question just how sick they really are.

I, and a lot of others in my extended scleroderma community, feel like fakes a lot of the time. Like, almost all the time!

I mentioned that to my pulmonologist last week on the phone, and he was really direct in his response: do not fall prey to imposter syndrome!!! This is really happening to you, you are dealing with it very well, but you need to remember to take care of yourself!

I had never thought of imposter syndrome in this way, but I think that his point is well made. Maybe I should have a poster made with this written on it. With some sunflowers and a tuxedo cat for interest. Every person diagnosed with a serious chronic condition needs this, because it is easy to just trick yourself into pretending that you are “better” and then the bad decisions get made. Like going off your meds. Or partying like it is 1999. Or eating all of those things that are essentially forbidden at once! There has been a rash of wailing patients in my online support groups who say things like… I went off my meds and started treating myself with natural remedies, and now [insert new symptom that has them terrified] is happening. Yep. That is imposter syndrome, hard at work. No wonder my doctor was so firm with me.

Because the point that he was making is that I am pretty ill, and I need to own it. I’m in a very small group of scleroderma patients who have the full battery of the more serious systemic sclerosis complications: pulmonary hypertension, interstitial lung disease, and diastolic dysfunction. I am being treated aggressively for those conditions, and I am doing really well, but they haven’t gone away.

I finally pulled myself together to check what the survival rates are for patients with two of my concurrent conditions (SSc-ILD-PH) and discovered that there are recent studies that looked at patients being treated with the same drugs that I am on.

This study, done in 2009 with patients on the same drug regimen as mine, showed the 3-year survival rate to be 39%. Oh, oh. Not an imposter any more.
This study, published in 2011 has a 3-year survival rate of 47%. Oh. That’s better.
A huge study done in Germany and published this year, shows that scleroderma patients with ILD-PH had the worst outcomes: the 5-year survival rate was 79%. Gosh, that is a good-looking number!

I see a trend. Do you see a trend? It looks like things are getting better and that survival rates are going up. I have to admit, when I saw that first 39% I almost panicked and stopped searching, because… I was diagnosed and started treatment for pulmonary hypertension and diastolic dysfunction exactly 3 years ago. The ILD diagnosis came in a few months later. The clock is ticking, and I am one of the 39%… talk about imposter syndrome: how can I be this functional when the majority of patients like me are already dead?? I’m a fake, an imposter!! That can’t be true, it is not true, and that new data shows what is happening: it looks like the treatment plans of today are really making a difference. My pulmonologist feels that the drug that I’m taking to control my lung disease is a game changer, and that drug was only approved for use with scleroderma patients within the last decade.

For some reason the term 39% Imposter has stuck with me. That’s me. I’m not really all that sick, because only 39% of me is pretending to be sick. Or well. Whatever works today. I’m thinking about a tee shirt with the slogan. I’m rocking the imposter persona. Maybe a shirt that says 39% Imposter and 100% Survivor…

I mailed back the heart monitor last Monday and now I’m waiting for the results. It has been a difficult three weeks because the flare just won’t go away, my wrists and knees are being bad boys, and now I have painful heart palpitations. Sigh. It is always one thing after another.

And yet, in my heart, I don’t feel sick at all.

Imposter Syndrome.

But only 39%.

Hannah and the CoalBear: Signs of Fall

Hi. I’m Hannah.

There was a huge flock of blackbirds that hung out in the backyard this week; there were so many that the CoalBear was a little worried about going outside. The days are getting colder and there are dead leaves in the catio for Mateo (AKA the CoalBear) to chase. The grasshoppers are all gone, and the squirrels are stealing food from the birdfeeder and storing it in the planters on the deck. Those squirrels are really getting out of control: one of them now barks at us from the garage roof, hanging off the rain gutter, and when that happens, we’re too scared to go outside. Bad squirrel, bad!!

Look at what’s happened to our tree!!! It is a kind of funny colored…

It was sort of a busy week. The Mother of Cats had two appointments and left us alone for hours. Then she was busy most of the rest of the week finishing up knitting the sweater that she has been working on. I love this sweater! It is fun to sleep on, and chasing the yarn is great. The Mother of Cats, however, was not very good about sharing the sweater, and then she took it off the needles, tried it on, and then packed it away on a shelf where I can’t reach it. Why does she do these things?

Today she blocked the sweater, and it is covered up with towels so I can’t lick the wool which is one of my very favorite things to do. As soon as it comes out from under the towels, however, I will have my way with it!!!!

In the meantime, the Mother of Cats is back to working on her blanket that was packed away for months and months and months. Look at how cool this blanket will be!

The Mother of Cats says that this is the next big project that is getting done.

So, that was kind of the whole week: abandoned for hours, knitting, and crazy animals that make going outside kind of scary. I’m going to go pester the Mother of Cats for some extra tuna and then I’m going to go take a nap.

Mateo: I’ll hang out with the Mother of Cats in the indoor garden while Hannah’s asleep.

This is Hannah, signing off.

>^..^<

Notes from the Mother of Cats:

The sweater is La Prairie by Joji Locatelli. This was a challenging knit with lots of elements to juggle at the same time: a charted pattern, color changes of yarn, and lots and lots of bobbles!! I had to make modifications to the sleeves as I worked to conserve some of the yarn colors, and I still may need to rip back the cuffs and knit some more to make the sleeves longer; I’ll know the sleeve length better after blocking is done. This is absolutely an individualized knit.
All my sweaters are safely put away in plastic lock-top bins. Sorry, Hannah.
The blanket is the Nectar Blanket by Ysolda Teague. It keeps growing on me, and I’m now thinking about hunting around in lace books to look at different options for the edging. The original pattern calls for a garter stitch edging, and I keep thinking that I need some more lace… I may need more yarn, too…
The appointments were to get my pulmonary function test done, and then a follow-up appointment with the pulmonologist. I have gained back more lung volume, and he is really happy with my progress.
I took my little purse-sized emotional support chicken with me to the testing appointment. Then we went to the grocery store and Starbucks.

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: 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!!

These are a few of my favorite things…

Hannah is taking the week off, but she would like to remind everyone that they should have a good Caturday. Go outside and listen to the birds! Roll around and coat your fur with some nice dust to take the oil off. Look for a bunny. Demand tuna!! Run wild through the house, chase your brother, and sleep in a sunbeam.

I’m still struggling with fatigue, and even though I’ve been trying to eat iron-rich foods, my doctor has decided that I need supplementation. Ugh. I’ve discovered that iron supplements are pretty much evil. Seriously evil! Like, the awful goes on for at least three days after each supplement, but I am starting to feel better. I sleep better. I have more energy. My GI symptoms are starting to calm down. I’m more mentally alert and have an urge to write. Huh. Anemia. There is another post in here somewhere, but not today. Today is about my happy place, the crafting room.

For some reason that I don’t understand, Hannah has decided that I need to spend all my waking hours in the crafting room. She trills and carries on (like, she scratches the woodwork around the doors to make me come running…) until I come back to the room. She is okay if I’m up and moving about, but if I try to read in bed, she intervenes and makes me get up. Okay. Transferring my reading to the craft room… huh… there is lots of sunlight in this room. I have a little loveseat along with the indoor garden, my loom, and a television, so this is actually a good place to hang out while I’m iron-loading. Did I mention that there is a walk-in closet attached to this room with all things crafting inside? Maybe Hannah knows what she’s doing. Over the last week I’ve been gardening and moving things around, and I realized that I really have made a happy place for myself in this room.

Here’s my loveseat with my emotional support chicken, my knitting, and one of my favorite shawls.

Did you want the links for those knitted objects? Just in case you do, here they are. The sweater is La Prairie by Joji Locatelli. The shawl is another Joji creation called Julie’s Wrap, and here is the link for the Emotional Support Chicken.

Do you see all the popcorns on the edging of Julie’s Wrap? It took FOREVER to make all of those, and you would think that I’d learned my lesson, but noooo…. I went ahead and made those popcorn stitches on the La Prairie. Do I regret this choice each time I come to that row on the chart with the popcorns? Why yes, yes I do. And yet…

Look at how cute they are in this cardigan!!!!

If you commit to knitting this sweater, and it is a serious commitment, you might as well commit to the popcorn. I’m really pleased with how this is looking. I’m into the third color of yarn and can’t wait to get to the fourth one. This is going to be a fun light cardigan later on this year.

Back to the happy things. They have pretty much piled up on the table in the room that usually has my fabric cutting station on it. This is what has moved onto it over the last week or two.

That teddy bear is the one that my son gave me in the ICU after my lung biopsy. He’s wearing a hat that I’ve knit for Frayed Knots donation, and the little zebras are ones that my niece sent to me as happy surprises. The Amazon Echo on the table reads my audible books to me while I knit. Happy. This is happy stuff.

Did you notice the green cat tail in the last photo? Here is the knitted cat, in fun spring colors. He’s propping up my current dragon/octopus books and doing a great job at it, too. Those books make me happy! The watercolor painting in the background is one I bought with a girlfriend at an art sale one perfect fall day in Golden, Colorado. My friend died two years ago, but the memory of her and that perfect day lives on in the painting.

Some of the plants from the indoor garden have overflowed onto this table. (Actually, that’s what set off the rearrangement of happy stuff.) This white orchid is a rebloom on a plant from last year (yay!), and in front of it is one of the new little fig trees. To the right, in a little terrarium that MAKES ME HAPPY is an African violet that is blooming its heart out. Above them you can see a bit of the quilt that my cousin made and sold to me a few years ago. Hey, doesn’t this make you happy too?

If you are interested in knitting your own cat, the pattern is Grey Kitten, Calico Cat by Claire Garland. I think that some more things will be joining this assembly on the table. I have some little statues that were put away because… Hannah… that I can now take out and arrange with the flowers. I have some special skeins of yarn that might be fun to display. Maybe I should focus on weaving a little wall hanging on the loom.

Tomorrow it will snow again, but I will be safely indoors with Hannah in the craft room, embracing the popcorn stitch, and enjoying all the little happy things that have been collected in here that are emblematic of friends, family, favorite knits, and happy memories.

There is a little take-home lesson here somewhere. If you are struggling with extreme fatigue, shortness of breath, and everything hurts, just go with it. Make yourself a happy place. Make something beautiful. Hang out with your cat. Embrace the popcorn stitch. Read in the sunshine.

Don’t forget to take your iron supplement, though, no matter how nasty it is!

Hannah wants me to mention that the Chewy tuna fairy visited us yesterday.

p.s. MR, how could I have forgotten to post the picture of the whole quilt? Here it is, a ribbon winner by my wonderful cousin Ruth Ann. Ya’ll, MR is at Downunder with Boodz and sets me straight whenever I wander from the straight and narrow…

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 BioGeek does Covid

It’s been a while since I’ve posted. I know, I know… lazy, lazy, lazy. This time I have a really good excuse.

I caught Covid!

I think that almost everyone who follows my blog knows that I have a type of scleroderma called systemic sclerosis; this is a progressive autoimmune disease that involves damage to blood vessels, lots of inflammation, and then scarring (fibrosis) of organs. In my case, the worst damage is occurring in my GI tract, my heart and my lungs. Because of the lung complications that I have developed I’m pretty high risk for a severe case of Covid-19, so my doctors have encouraged me (strongly) to get updated Covid-19 vaccines as soon as they are available. Because of my interstitial lung disease, I don’t halt my immunosuppressive drug for these boosters anymore.

So, as soon as the new Covid boosters were available I made an appointment, and on October 12th I got my shot. Yay! I was so excited to get it as I usually feel great for a few weeks after the vaccine. Like… my systemic sclerosis symptoms improve, I get more energy, things stop hurting, I can breathe better… you know, better! I know, I know, this doesn’t make any sense, but it is a well observed phenomenon in the systemic sclerosis community. It’s like there is some crazy connection between that vaccine and our autoimmune condition. It does not happen with any other vaccines.

Vaccines always knock me on my butt at first, though, so I wasn’t surprised that I developed muscle/joint pain, chills, fever and fatigue. I headed to bed expecting to feel better in the morning. Nope. I began coughing, got a sore throat, developed a lot of congestion, and GI symptoms arrived. Three days later I began to suspect that maybe this was actually Covid, and that I had unluckily gotten ill on the same day as the booster. I took an at-home Covid test, which was negative.

I wasn’t too sick to order more yarn on the internet. Look at this amazing sock kit that I bought from Moonglow!

A little aside: I’m pretty sure you don’t already know that Covid-19 and systemic sclerosis share a lot of similarities. Like, a lot. Damage to the vascular system, the production of a lot of inflammatory cytokines (messenger molecules that travel between cells), and then all those symptoms that can include heart and lung complications that result in fibrosis to both organs. (Covid is a fast-moving firestorm, and systemic sclerosis is more like the relentless slow burn version.) The pneumonia that develops in Covid-19 patients looks so much like the inflammatory lung disease in systemic sclerosis (SSc-ILD), it can be hard to sort out which disease is in play when systemic sclerosis patients like me first arrive in the ER. Okay, things never go well in the ER anyway, so I tend to avoid them. Zebra, right? I decided that I was just in a bad flare and suffered on instead of going to the ER.

Two days later, I retested for Covid. Still negative. Still too sick to consider driving anywhere, too sick to sit up and knit, but not sick enough to call 911. I was kind of stuck in limbo. Maybe some level of hell. Covid hell. Anyway, that booster shot had definitely failed me this time!! I spent my days coughing, sleeping and sadly gazing at my new sock yarn propped up on my bookshelf. I kept taking my immunosuppressive drugs and suffered on.

The cats began to live on the bed with me. Good kitties!

After a week of this nonsense, I had improved enough to consider going to a Kaiser Urgent Care to see if they could sort me out. Oh. The doctor there was of the opinion that I had Covid after all, but now it was too late for anti-virals or an infusion of antibodies. I got a chest x-ray, some nice antibiotics, a new drug for my cough, and headed back home to the cats and my unknitted yarn.

Tonight, 16 days later, I’m better. I can sit up and knit at last. Through all of this my oxygen levels never dropped, and while I was sick, I didn’t develop any really serious complications.

So, what have I learned about systemic sclerosis, the drug that I take to control it, and Covid-19? I mean, this is a total BioGeek moment! My mind full of dancing antibodies, failed Covid tests, and mycophenolate pills, I took to the internet for answers. There are some take home lessons that I’ve decided are so interesting that I’m passing them on.

The similarities between systemic sclerosis and Covid-19 are so significant that the two diseases provide understandings that can help in the treatment of both.
The immunosuppressive drug that I take (mycophenolate) to control my immune system’s attack on my lungs can prevent me from producing an antibody response to the vaccine. Okay, I knew that, but I was too sick to think through the implications… because that means…
Immunosuppressive drugs can lead to false negative at-home Covid tests. ~~Those tests are for Covid-19 antibodies; if you can’t make antibodies, then you may have Covid but test negative.~~ Oops. (Correction! My cousin has set me straight… the home tests are detecting viral proteins (antigens), so that theory is blown out of the water! Double oops! The doctor told me the mycophenolate was the cause of the false positive tests, and he was pretty emphatic that I should stay on it. Now I’m wondering if the false positive tests were because mycophenolate has anti-viral properties, as some sources cited in the Lancet paper below have reported.) (Don’t you just love science?!!! )
The same immunosuppressive drug (mycophenolate) can be an effective treatment for Covid patients reducing their risk of severe outcomes.
Staying on the drug was a good decision on my part. I got lucky! Because I am on this drug, however, it can take longer for Covid to clear my system. It has been 16 days, and it looks like I need to isolate for a few more days.
I never, ever thought that the treatment for my SSc-ILD would help protect me from the severe Covid complications that the same condition places me at high risk for. I’m so grateful that we didn’t go the chemotherapy route last year.

Isn’t all that interesting?

Here’s the links to articles if anyone is interested.

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.