Do you see all this yarny goodness that I’m cuddled up with?
The Mother of Cats has taken down the sweater that was in time out (I wish that Mateo was in time out… he has been annoying all day!!) and started knitting on it again. She was a little worried about coming back to a project after so many months, but it turned out to be kind of easy for her because she had made so many notes on the pattern and even made a big knitting aid to help her keep track of the yarn colors, the pattern, and the decreases on the sleeves.
All the numbers refer to the chart rows for the pattern. The dark boxes are the decrease rows, and the numbers to left side are the yarn colors. Whew! It’s enough to give me a headache!! Maybe some tuna will help…
Anyway, she spent the week knitting away on the first sleeve and got it finished in the middle of the week. Here’s what it looks like:
Pretty cool sleeve, right? The pattern goes down the outside of the sleeve, and the Mother of Cats is soooo happy that she doesn’t have to knit too many bobbles.
She’s now working on the second sleeve and really worried about running out of one of the yarn colors. She keeps weighing the ball of yarn and saying things that I think are inappropriate for kitty ears under her breath. Poor Mother of Cats. I never stress. Well, I only stress a little. Okay, I am in the closet for hours every time a stranger comes to the door, but that is just good sense, right?
The worries about the yarn are pretty bad, so I’ve been hanging out with the Mother of Cats while she knits on the second sleeve.
Anyways, the sweater is coming along well, and I think that it is one of the comfiest knits that she has made in a while. I just love taking naps on it!! Isn’t the color nice? It really makes my coat shine.
This is Hannah, signing off.
>^..^<
Notes from the Mother of Cats: That sweater is La Prairie by Joji Locatelli. I’m now in a rush to get it done before the first snow of the season. Next week is going to stay warm, so maybe I’ll pull this off!
After a phone call and then a few email exchanges with my cardiologist it was decided that I needed to wear a heart monitor for the next 30 days to rule out atrial fibrillation (AFib); evidently that is a systemic sclerosis thing, and I’m high risk to develop it because of other things going on with my heart. I did not see this coming, but it absolutely matches my symptoms.
Look at this heart monitor!!
That heart monitor is just fabulous!! The whole thing is taped on, I can wear it in the shower, and there are no leads to worry about coming loose. The monitor is blue toothed to a phone that I need to carry at all times, and it is transmitting to both the company that owns the monitor and my doctor. If I have another event while wearing it, I can send a message immediately to my doctor on the phone. Isn’t this technology fabulous? Of course, nothing has happened while I’ve been wearing the monitor, but I still have 28 days to go…
Mateo: I am not annoying!!! Hannah should be the one in time out because… hold on… I’ll think of something… she hogs all the tuna!
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.
Cute fingerless mitts, right?
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.
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
Grade II, both knees.
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.
All My Chickens
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!
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.
Shine like a sunflower, everyone!
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.
I am still thinking about resiliency. I went for a drug infusion a couple of weeks ago, and the lady in the chair next to me and I talked about what resiliency means. She is a liver transplant recipient, and she is also taking the immunosuppressant that I take to control my systemic sclerosis. Like a lot of patients in my scleroderma community, she has had to roll with a series of escalating complications tied to her original liver disease. We both now have osteoporosis, walk with canes, struggle with anemia, and are on a lot of drugs. We laughed about the canes and drug side effects: we’re still here, we crowed. Unlike me, she never checks for drug side effects or interactions; if she knew about them, she figured that she would just worry and imagine she was developing them. Instead, she just cruises along and if something comes up, she contacts her doctors.
Resilience.
Welcome to Scleroderma Awareness Month. I’m spending the month organizing my thought about resilience into some type of logical order. I have a Word document going with an organizational table, a ton of links, and everything scleroderma in it. It will be good for me, I think.
This is a pygmy sunflower. How cute it that!
By the way, my new friend also wants an emotional support chicken.
I’ve been thinking about resilience today after this graphic showed up on my Facebook feed.
What is SPIN you ask? It is the Scleroderma Patient-centered Intervention Network.
I’m pretty sure that I was one of the patients in this study. Certainly, I was a participant in a SPIN clinical research study during which I filled out lots of questionnaires about my illness. The focus of the project that I was enrolled in was to see if online educational videos about scleroderma, possible interventions to help with the progression of symptoms, and information about resources could help patients in how they coped. I was tracked for over a year to see how I was doing. How was my pain? How was my sleep? Was I depressed? How severe was my disease, and how did I feel about that? Did I have issues with anxiety? What were my scleroderma-associated complications?
Not surprisingly, the study referenced in the graphic found that they could break patients into 4 groups based on the severity of their disease (Low -> Very High). They found, for the most part, as patients dealt with an increasing disease burden, they coped less well and struggled with depression and anxiety more.
Except for one group of outliers. They had a pretty significant disease burden, but they were not depressed and didn’t struggle with anxiety. They kept rolling with the punches and finding ways to flourish even within the eye of the storm, securing for themselves a better quality of life than others facing the same challenges. I’m pretty sure that I am one of the people in this group of outliers. I remember that I reported that I was having lots of trouble with pain and sleep disruption, but no depression; my current battery of scleroderma-associated complications should put me into the High disease group. Here is a paper where the researchers further investigated the association between disease burden and mental health and resiliency.
I bought myself little roses last week. Look at how cute they are! They are under a red light, but you can still kind of see that they are a peach color.
So, the big question that the researchers are now pursuing is what determines resiliency, and how can these essential coping skills be conferred to other systemic sclerosis patients through training and support.
So, what is resiliency? That was the big question of the day for me as I worked out front weeding a garden. Working outside is now hard for me, but I dragged out a little chair to sit on, put my tools into a crate that was easy to push, and I slowly worked my way along the front walk cleaning up my garden and pulling out weeds. I had to take breaks to pant from time to time, but by the time I was done the gardens looked great! The weather was perfect, there were lots of birds, and one of my neighbors had music playing while he worked in his garage. After a little break on the catio with the cats (coffee and a lemon bar time!!), I strapped on my portable oxygen concentrator and mowed the front lawn. I’ve had a hard week, and I struggled to get myself outside to work in the yard, but by the end of all of this I felt much better. You might even say I felt happy.
Look at how nice the front pot of flowers is looking! I bought a new rug to put by the door onto the catio, Amazon delivered it today, and Hannah moved right in. Yay! Take that scleroderma!! I win the day!! I am happy!!!!
So, what is resiliency? I’m still stuck on that question. I’m pretty sure that it is a state of mind that allows people to focus on what can be done instead of what has been lost. Resiliency allows people to embrace their changing circumstances and effortlessly employ life hacks to get things done while acknowledging that other things are now too much. It must have to do with an ability to name the monsters, stuff them into a box, and put them on a shelf (in the back of the yarn stash) while activating self-advocacy. It must require faith that you will be able to cope with scary things when they come, while still recognizing that you are in a difficult situation. It must require support and resources. I think it has to be an internalized quality: resiliency gives you the self-confidence and self-worth that’s necessary to face down medical authorities and to ask for demand help and answers.
I look forward to what these researchers find as they continue looking at the phenomenon of the outliers: patients dealing with a severely debilitating and isolating rare disease with grace.
I kind of think that they will discover that these people have engaging interests that allow them to have a sense of accomplishment and purpose. I bet that they find that they have pets and people that they love. I bet that they produce something of beauty in their lives. I am almost certain that they will find that the outliers have found ways to communicate in a positive manner with others about the challenges of life with a progressive, and often fatal disease.
Hannah on the Catio.
Why am I resilient? I have cats. I have yarn. I have curiosity and lots of resources to chase it. I have purpose as I produce donated items for others in difficult situations. I have roses. I have people who love me, and I have people who read my little posts about coping with life on the down slope of systemic sclerosis.
It’s spring here in Colorado. The first baby bunny appeared this week, and the plants in the yard are starting to burst out in green buds. The roses are looking like they are going to do really well this year (except for that one that the bunnies ate…) and there was a huge outbreak of my little bulb flowers in the front garden. I have new cushions for my patio swing, and I sit in the sunshine, reading and drinking my morning latte, every warm morning. On chilly mornings I wrap in a favorite shawl and stay out with the cats as long as I can. The first grasshoppers of the year have arrived for the cats to chase, and the air is filled with birdsong. There are even blue jays!! Good days.
The phlox in the front is looking great!
A couple of day have been so warm I couldn’t stay in the sunshine long and I’ve had to deploy the umbrella shade. Then, the next day, there may be snow. This is spring in Colorado: rapid changes and big temperature swings. The same is true for the barometric pressure; look at what happened this week.
My emotional stability has been a little like the air pressure the last couple of weeks as I’ve gone through a round of testing and doctor’s appointments. I was very upbeat when I went in to get my blood draw to check my iron levels, and the bone density scan last month was just routine. I mean, I’ve been feeling soooo much better: more energy, sleeping better, more mentally alert and even my appetite has improved. Suddenly my gut doesn’t hate me, and I am getting out of the house more. I went to my knitting group for the first time in months! I was positive that my test results would reflect the improvement.
Not so much. After weeks of eating iron-rich foods and downing my iron supplement there was absolutely no change in my test results, and in fact, the test that measures the concentration of hemoglobin in individual red blood cells (MCHC) got worse. Seriously? The only gain was one value of 25.5 that went up to 25.6; still too low. After all that red meat, salmon, iron pills, avocado, and spinach all I got was 0.1 improvement? Kind of disheartening as this means that I have to have some invasive testing to see if I’m bleeding in my stomach (it’s a scleroderma thing), and I don’t think my doctor will put it off much longer. (It’s called watermelon stomach) (fabulous) (of course this is rare) (my zebra self is not happy).
Then the bone density scan results arrived. I have somehow developed a fairly serious case of osteoporosis in a short time span; the report says to start immediate treatment. Then I did some googling and found out that the diuretic that I take can cause osteoporosis, and I absolutely shouldn’t have been taking it because I have a strong family history and I’m kind of high risk. (Of course I am) (I stopped the diuretic) (now my feet are swollen) (my zebra self is crying).
I have to be honest. I was kind of crushed by the bad news that I absolutely did not expect. Then I mentally slapped myself around, did my exercises, potted some lavender plants, and went shopping on Amazon for some cheer-me-up jewelry. Just what I needed to pull myself together. One day at a time, right? I already have more than my share of challenges, so I shouldn’t waste any energy on things that haven’t happened yet. Next week I have an appointment with my internist, and we’ll work out a treatment plan for the osteoporosis and next steps for the anemia. (I took another iron supplement) (my inner zebra has pulled itself together again) (the zebra wants to point out that its lips aren’t as blue as they used to be).
I also pulled out the La Prairie sweater and knit steadily through the sadness until the body came off the needles. Gosh. It looks really nice so far. Something has gone right this week…
Today I woke up to a snow/drizzle mixture that was too unwelcoming for even Mateo to go out on the catio. All my joints hurt, and my muscles weren’t sure if they were going to play nice either. Of course, I had to drive across town to get lung testing and a sit-down appointment with my pulmonologist to go over all my results. I put on my cute Weekender Crew sweater, wore my new “in your face, scleroderma!” jewelry, and headed off for the testing. It hurt to breath as I walked into the building. I convinced myself that it was just the cold air; after all, I feel pretty good, and I was overdue for some good news.
The lung testing specialist is now my friend. We laughed and talked and caught up as she got me ready for testing, and then I breezed through all the parts of the pulmonary function testing and the 6-minute walk test. Every single result was cause for celebration as Stephanie (my technician) became more and more excited with the little graphs and data appearing on the computer screen. “This is better!” she kept saying. By the last test she was practically jumping up and down with excitement. “This is great! This is great! she crowed as she walked me to the exam room to see my pulmonologist. “I’ll let him know that you are here.”
I love this pulmonologist. He is the doctor who first listened to me and picked up on the fact that I had a hole in my heart and pulmonary hypertension. He held my hand and told me I could cry when my interstitial lung disease was first diagnosed. He has always been the doctor who was most honest with me; he told me last year after the tide had turned that they hadn’t been sure I would make it a year. He supported me when I halted the anti-fibrotic drug due to quality-of-life concerns. Today he was all smiles as we went over the results and my exam. My lungs have maintained on the scans. I have regained some lung function. The decision to halt the drug was the right one; there is no obvious sign of fibrosis right now. This is the best possible outcome right now; everything that he hoped for. I was the last patient he saw today, and he was pleased to have such a good one. “Best appointment of the day!” he declared as he walked me out. We were both enclosed in a bubble of joyous happiness as we walked.
I took this picture outside the building.
Outside the clinic the parking lot was almost empty. The cold drizzle was steady, the sky was full of sad lumpy grey clouds, and the gloom of early evening was creeping in. Around the building some ornamental trees were just beginning to open their flower buds, but in the shelter of the courtyard on the south side, the sunniest location, one tree was covered in blooms. Kind of a metaphor for the last couple of weeks. Sit in the sunshine and bloom, no matter what is going on in the world around you.
On the drive home, in my mind, my zebra self was dancing for joy.
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!!
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.
MacKenzie and me from 2018.
To learn more about my rare conditions and others you can go to:
I’ve been pondering the concept of toxic positivity for some time now. You know, people who insist that a person with an uncurable, progressive disease (ahem… systemic sclerosis) can get “better” if they just get some sunshine and exercise. People who suggest that the symptoms of a failing heart and lungs are “just old age”, or that debilitating brain fog is something that everyone deals with. People who keep suggesting possible cures of the herbal or holistic medicine variety, or that if I just try hard enough, I will magically go into remission and get well. People who insist that no one knows what will happen when you disclose serious new complications, or people who over-celebrate a small gain. I know that they mean well, but it is isolating and hurtful: it is a denial of my reality.
I lost my temper and snapped off at one particularly obnoxious person at a holiday function who was arguing that I just needed to stay positive and try harder so I could get better… “there is no better, there is only this”, I told her. She was taken aback, and then doubled down and insisted that people do get better if they try. In my defense, I should mention that she had also just told me that her daughter’s diagnosis of a serious illness was awful because her daughter was young, but what was happening to me wasn’t such a big deal because I was old. Yeah. Toxic. My doctor told me that she was lucky that I didn’t hit her. I love my doctors!
Okay, having said that, I am better these days. I seem to have emerged from the worst of the fibromyalgia flare and the horrible dizziness, brain fog, and pain have receded to background levels again. Yay, magnesium!!
So, why is toxic optimism a problem? Well… toxic, right? It’s a denial of what is actually happening while throwing up walls that distance and protect you from the painful thing that is happening. It’s ensuring that you won’t get involved. Some of those walls are an insistence that the person will get better, or they aren’t actually all that sick, or that this is “normal”, or even suggesting that somehow the person is actually at fault for their own illness/condition. Not helpful, people. I was already thinking about how to frame this in a post when an article from The Atlantic called Tragic Optimism is the Opposite of Toxic Positivity arrived in my news feed several days ago. Okay, this is close to what I’ve been thinking about, but tragic optimism isn’t exactly what I’m trying to achieve here. No tragic, okay? In my mind the process of flourishing in the face of adversity requires grace and courage: walk into the darkness, face down the monster, deal with it (severely), and then continue to thrive and shine (like a sunflower, goddammit!) from within the heart of the storm.
So, it was kind of amazing when I watched American Symphony last night on Netflix.
This is what I’m talking about. It was hard to fight back the tears as Jon Batiste and Suleika Jaouad put into words and action the things that I’ve been thinking about, but so much better. Jon Batiste says… “look into the darkness and despair, face it, but don’t let it consume you.” YES!!! This is what I’m talking about, people. “Accept the possibility that you might fail, but absolutely believe that you will succeed,” Jon says in the film. Without any doubt, Jon and Suleika are at turning points in their lives: they have just married, Jon writes and produces a symphony that is to be performed at Carnegie Hall, and Suleika undergoes a second stem cell transplant to treat her relapsed leukemia. I know people who have undergone stem cell transplants (it is used to treat extreme cases of systemic sclerosis too), and it is a lengthy, horrible process of dancing with death while hoping for life at the end of the tunnel. This is a raw and emotional documentary of extremes; for this young couple, they are navigating the highs and lows simultaneously. The stress is crushing, and the emotional toll is heartrending. In spite of this, they cope, they walk into the darkness and face down the monster, they shine from within the heart of their storm, and the documentary ends with the performance of American Symphony at Carnegie Hall with Suleika in attendance.
In my opinion, every significant battle begins with the thought… okay, this is happening. Recognizing your situation is not a sign of weakness, but of strength. The action plans, sense of purpose, and the ability to keep rolling with the punches comes later. There is no place for denial if you are going to battle! Also, as this couple shows in the film, you need some creative outlets if you’re doing battle. Suleika paints and Jon composes. I take yarn along.
Today I started listening to Suleika’s story of her first dance with leukemia and the recovery afterwards.
I’m not sure that these two would embrace tragic optimism, but they sure are shining examples of the opposite of toxic positivity. I think that I’m going to be learning things from Suleika while I listen to her reading her book, knitting away on my new sweater. With my newly blooming orchids. And my cats. Within my own little scleroderma storm, the monster locked safely away in the cupboard. Pipe down, monster. I’m knitting now.
Yarn, Books & Roses 