A couple of weeks ago I went in for a routine blood draw at my local Kaiser clinic. I gave the phlebotomist my ID card and told her my name and birthdate: for some reason they like to verify these things, right? Anyway, what happened this time was… she exclaimed that didn’t look my age. (Well, yeah. That may be one of the bigger red flags for scleroderma… no wrinkles.) “It’s just my disease doing that,” I assured her. “I really am that old.”
This lady wanted to know what I had done to get rid of the wrinkles. She wanted this magic wrinkle remover too! I carefully told her about scleroderma. “I never heard of that!” she said. Still thinking that I was a lucky person, she drew my blood and I got out of there. I would be happy to have every single wrinkle that I have earned over the years, but no… I have scleroderma. The systemic form with limited skin involvement. Lucky me.
I do have wrinkles around my chin and mouth, but I was wearing a mask during the interaction with the lady at Kaiser. I had to go on oxygen this day (I have lung and heart involvement) and you can see the swollen tendons in my hand and the tight skin over my knuckles.
That’s why there is this thing called World Scleroderma Day. This is a complex autoimmune disease with illusive symptoms (brain fog… trouble swallowing…GERD…swollen fingers…trouble breathing sometimes…fingers that suddenly lose circulation and turn white or blue…) that make the disease difficult to diagnose without specialized testing and evaluation by a scleroderma specialist.
Systemic sclerosis (the form of scleroderma that I have) is thought to start with some event (still unclear) that triggers the formation of antibodies that target specific molecules the nucleus of cells. These antibodies lead to damage in blood vessels and their linings; the damaged blood vessels release chemical signals that trigger a cascade of events that impact other cells of the body. Some cells are related to inflammation. Other cells are tipped over into forming uncontrolled scar tissue (fibrosis). Here is the problem: the tissue engaged in fibrosis is all of the connective tissue. What is connective tissue, you ask? Think of all the ways a person is held together. Tissue that makes your skin stretchy. Tissue that holds your muscle fibers together. Tissue that makes up your tendons, ligaments, and the structure in your joints. Tissue that holds your intestines, heart, kidney, and lungs together. The cartilage in your ribs. All inflamed, under attack, and forming thicker and thicker layers of scar tissue. Some of this damage causes calcium deposition. Unobservable from the outside, the patient is slowly hardening inside. The most obvious symptoms is the skin thickening and then pulling tight over time.
Scleroderma, and especially systemic sclerosis, has the highest fatality rate of all rheumatic diseases. Did I mention that there is no cure? There are treatments for complications that develop like pulmonary arterial hypertension (PAH) and interstitial lung disease (ILD), but they, like the immunosuppressant drugs that many of us take, mostly stabilize and slow progression. (Dear Kaiser lady… that’s why I was getting a blood draw. The drugs that I take are risky, and my doctors need to make sure my kidneys and liver are tolerating the meds okay.)
So, what is the purpose of World Scleroderma Day? It’s to highlight this condition that impacts more people than it should even though it is rare. It’s to raise awareness of the symptoms and hopefully aid in other people getting an appropriate diagnosis. Maybe shining a spotlight on scleroderma will help in getting funding for research, treatments, and even a cure.
Sunflowers are the international symbol for scleroderma.
Here is where you can learn more about scleroderma, it’s symptoms, and treatment.
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 need this picture on a shirt!
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.
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.
The Mother of Cats has been ignoring us for days and days. I mean, our food bowls were almost empty, and she ran out of tuna over the weekend. I seriously question her fitness as a pet parent at this point…
Do you see this? I absolutely did not deserve this either!!!
First it snowed. Like kind of a lot of the wet sloppy stuff. Hannah refused to even go outside on the catio, and I could only stay a few minutes without getting covered in the white stuff because IT KEPT FALLING OUT OF THE SKY!! The Mother of Cats wasn’t letting me out all that much either because for the most part, we couldn’t get her to get out of bed. Seriously, she is the laziest human on the planet. She just stayed in bed listening to books, sleeping, working on her computer, and basically ignoring all of our needs. Finally, it stopped snowing, and the sun came out again. Like magic, the Mother of Cats also started to pay better attention to us. She charged up the laser friend that Hannah likes to chase and ordered me more toys from Amazon. Also, she had some tuna delivered because we needed it DESPERATELY!!
Once she was back into the kitchen, she made more of that crazy squash soup that she likes and spent some time playing with the lemons that her niece sent her. She cut them all up and put them in the countertop oven on the dehydrate setting for hours. Kind of a stupid thing to be doing when I NEED TO GO OUTSIDE AND SEE BUNNIES!!! Luckiy, she had taken care of the tuna issue already… I’m a good boy, but I do meow in emergencies!
Now she can put lemon slices into her tea while she is being lazy in bed and ignoring our needs. Thank heavens the new toys arrived, and the laser friend kept working because I never got to see any bunnies or squirrels or anything for the whole weekend.
The only other thing that she did last week was to set up an experiment with dirt and seeds and stuff.
The Mother of Cats collected the seed pods from her roses last fall, and they have been out in the cold of the garage ever since. She carefully planted half of the seeds and put the trays onto a warm pad on the floor because Hannah pushed the trays onto the floor from the table in the craft room. Silly Mother of Cats, doesn’t she understand that they needed to be on the floor in the first place?
Hannah: I was just trying to help out! I take the covers off the planter trays too so I can keep my eye on things.
Now the rest of the seeds are chilling in the refrigerator that I like to shove my toys under where they are going to stay for another few weeks. They will get planted then if the first seeds haven’t sprouted. She also put milkweed seeds into dirt that is out in the cold of the garage or outside under the snow: another experiment! She likes to do stuff like that. I think that she would be happier if she played with chirpy toys instead, but what can you do. I’m only one cat, and she is a lot to handle, let me tell you.
This is Mateo, signing off.
Note from the Mother of Cats: The barometric pressure really dropped quickly when the snowstorm came in, and I evidently developed a bit of pulmonary edema when that happened and gained almost 8 pounds in two days. Friday night I realized that I was dizzy and having trouble breathing: diuretics to the rescue!! It has taken several days to get the weight back down and I’m breathing a lot better now, but the cats sure were neglected while I was in bed on oxygen dealing with it. Just another scleroderma adventure…
Also, Amazon delivered the tuna and the chirpy toys overnight. Yay, Amazon. It is good to live in a city with a fulfillment center…
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…
Bingo! I found my yarn!
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.
So, I got a little testy in one of my Facebook support groups for systemic sclerosis this morning. A member of the group kind of disparaged me and another person for not being positive enough. It was “you need to refuse to let scleroderma define you” in response to the first person sadly saying that she missed her old life, and me giving her an online hug with the comment that if only a positive attitude was enough…
In my defense I had just experienced a Kaiser employee visibly reacting to my lack of wrinkles. As in, wow, that’s great! Like not having wrinkles makes this all worth it.
I lost another 4 pounds at my last check-in, which is concerning, but the nurse was thrilled for me. Me, I was a little teary at the continued loss.
Um, people… do you think that you might be a little shallow here?
I was out on the deck/catio with the cats drinking my morning latte when I hit this emotional wall, and after I had fired off the somewhat testy response, I spent some time in the garden. There were my roses, blooming like the utter champs that they are.
This is my Princess Alexandra of Kent rose.
This rose is looking great this year. Never before has the plant been able to hold up the blooms without the weight pulling the stems over. The problem is our semi-arid climate with hard winters; the plant grows back from the roots every year and it doesn’t have time to put in enough supportive tissue to hold up the blooms. This year, with all the rain and cool weather that we have had, the plant was able to put in enough of this tissue to do the job. The tissue that I’m talking about, a type of ground tissue, is called sclerenchyma. If you’re ever snapped a celery stalk in half and pulled out the strings, you were pulling out sclerenchyma tissue. This tissue is made of dry, hardened cells in the stem of the rose and I suspect that the “scler” part of the word is of the same origin as scleroderma. Look at that. The hardened cells are doing something good for this rose!
In me, not so much. Things have been a little difficult as my poor heart and lungs are not benefiting from the hardening and thickening going on in the cells and tissues of those organs. All of my tendons are seriously pissed off at the moment. Edema has become a problem, and it is becoming increasing clear that I need to stay on oxygen 24/7.
That’s my arm with the imprint of a quilt in it, in case you didn’t immediately recognize it… Edema is kind of a tip off that my heart is struggling and that’s new. The weight loss is also related to my heart/lungs because if oxygen isn’t getting down to my cells like it should, they can’t use energy efficiently, and then, you know, weight loss occurs. The tendon issue is scleroderma actively attacking them and gradually hardening them to bone. Bad scleroderma, bad!!
But look at how great these roses look!
So, what did I say in my testy response? Reality bites. Some of us have progressed to the point where we have to admit that no amount of positive thinking will allow us to attend that family function that we were invited to, or to visit the annual Wool Market in the mountains, or to even walk to the mailbox. To suggest that we could do things if we just had a more positive attitude is hurtful and not supportive. No matter how much you want to believe otherwise, scleroderma does define me and everyone else who is dealing with it. Courage requires us to face down the monster and to accept the reality of our disease. How we choose to function within that framework is up to us.
I may no longer be as mobile as I once was, and the life that I used to have is now mostly gone, but I choose to continue to bloom in place.
Like a rose.
Updates/Notes from the ScleroFront:
Do you like to wear linen? Those fibers are from the water tubes (xylem) in the flax plant, and made of sclerenchyma.
The bunny-murdering neighbor put her house up for sale!!
Oh, boy. I have been having adventures in my ongoing dice game with the Reaper. My (wonderful) pulmonologist started me on a high-risk drug towards the end of August to slow down the formation of scar tissue in my lungs due to my interstitial lung disease. Oh, boy. The side effects were not exactly great as I battled ongoing GI side effects and started to lose weight again.
My doctors get kind of worked up when I lose weight.
What?!!
I know, like this is a bad thing? Here’s their thought process: there is a correlation between weight loss and poor prognosis, so my docs tend to focus on the one factor that I can control. Ugh. I sadly moved onto a diet of chia seed puddings, rice, and bananas while gulping down fiber tablets. After a week the tide seemed to turn, and I was eating more.
This is the new drug, just recently approved for the direct treatment of scleroderma-associated interstitial lung disease. Yay Ofev!
Ofev entered the scene just recently. I first heard about it in the early years of my scleroderma journey, and it was approved for use about three years ago. It took a few weeks for me to get enrolled to receive this drug and I need to go for mandatory blood testing each month before I can get my next month’s supply.
There is a significant risk of blood clots, and the pharmacist really stressed that I should watch for bruising.
A few days after my appetite returned, I woke up with a sore and swollen leg; I was also bleeding from both nostrils. When I looked at my leg there was a large bruise that grew to be about 6 inches across… not good, little BLZ, not good!! Yep. There was an immediate full stop on the drug for a week, and then I was started at a half dose a week later. A week after I had restarted Ofev my pulmonologist called to check up on me. No bleeding, but I was dealing with stomach discomfort.
He gave me the Fairy Dust talk. “There is no magic cure, and this drug is not fairy dust.” he said, “Drug companies can manipulate data to make drugs look better than they really are; I can’t stand for you to be miserable on this drug. There is a case to be made for focusing on quality of life as opposed to quantity of life.” (Well, that’s not discouraging at all, right?!) As he and I talked I remembered my conversation with the pharmacist; she had stressed how important it was to take the pills exactly 12 hours apart to maintain a steady blood level… I was taking the high dose only once a day at that point, so I was probably experiencing a spike every day in my bloodstream. I asked for a script for the lower dose and convinced him that I should try that for a month even though the total amount each day would be more. Over the last few weeks, I’ve had a little bleeding and a couple of little bruises, but nothing like I saw on the higher dose.
I moved to the lower dose twice a day three weeks ago. While that was going on my roses started blooming again.
A week ago, I went for more lung and heart testing. I bought green chili cheese fries and a chocolate shake on my way home because… my GI tract has decided that it loves me and I’m hungry again! Besides… green chile cheese fries!!! The results came in last Friday. My oxygen is better, and I can walk farther than a year ago! My pulmonologist doesn’t need to see me for 6 months. My heart testing and bloodwork was used by my cardiologist in a predictive model that returned a result of… low risk of pulmonary hypertension progression at this time. My heart failure numbers did double, but he isn’t all that concerned; he doesn’t need to see me for another 6 months.
I hung up the phone after the last call and thought to myself… maybe Ofev is Fairy Dust after all, because… I. Am. Better. This has been 2 months of NOT FUN, but the proof is in the pudding.
By the way, chia seed pudding, highly recommended by my pulmonologist, is just plain nasty! Chia seeds, without any doubt, are not fairy dust!!!
To be fair, it probably isn’t all Ofev; after all, it has been pointed out to me that it is not a magic cure. I started other drugs to control my pulmonary hypertension over the last year. I have made a lot of adjustments on my end to handle my lung disease. All of my down products are out of the house. I bought a new humidifier that can be easily cleaned each week. I put a high-grade filter into the furnace and bought an air purifier. I take all my drugs right on schedule and I do gulp down fiber-rich foods that are keeping any symptoms under control (except chia pudding… see above).
Okay, Reaper. You won a couple of the tosses, but this one is totally mine.
I’ve been collecting new diagnoses over the last year like an out-of-control yarnaholic shopper at a fiber festival. Yeah, that bad. That’s how my yarn stash got so out of control… I mean, you never know when you will need that fabulous color in the future, it is one-of-a-kind, and it’s cashmere… Anyway, let’s get back to the topic at hand. The last year has been really eventful, and the new tests, diagnoses, and drug interventions keep rolling in. Here’s the chronicle of events:
An early summer echocardiogram last year showed that I had developed a type of heart failure called diastolic dysfunction. Scleroderma is causing scar tissue to form in the muscle of my heart.
On September 1st I had a right heart catherization that showed that I had a hole in my heart that was disrupting the flow of oxygenated blood to my body. Oh. That explains those blue lips I’d been sporting around town.
Remember the BLZ?
Follow-up testing showed that I had exercise-induced pulmonary arterial hypertension. I was started on drugs to treat the condition.
I went into a flare of my systemic sclerosis (scleroderma) symptoms because of the increased blood flow due to the drugs.
Testing in February to hunt for the cardiac shunt (hole in my heart) revealed that there was something going on with my lungs. A subsequent CT scan in March showed that I had developed autoimmune pneumonia in both lungs, a condition called interstitial lung disease. The cause of the ILD was confirmed by a lung biopsy in May. Yep. It is scleroderma related, but I was still forced to get rid of all my down products in the house. I miss my down comforter…
All summer long I have gone through a staggered course of drug interventions as my doctors worked to get my lung disease under control.
This sunflower is a pretty good metaphor for how I’m doing at the moment. I’ve taken a beating, but I’m still blooming, by golly!
So, I’ve been taking a lot of drugs, and all of them carry some side effects. I became dizzy from one, my blood sugar zoomed up, and my vision became foggy. Another is causing hot flashes. One of them causes muscle pain, and a couple of them are seriously interfering with my sleep. Two drugs cause edema, so I’m taking another drug to combat that. Two weeks ago, I started the last drug on the list, Ofev, which has a list of side effects that made me pause a few days before taking the first pill.
Nausea, diarrhea, vomiting… well, okay. That’s stuff that I deal with all the time because of scleroderma…
I had to go through a 30-minute phone interview with a pharmacist before the first 30-day shipment of the drug could be sent to me. I will need to get blood work done every single month to check my kidneys and liver before I can receive the next 30-day shipment. I need to be vigilant about watching for bruising and the symptoms of blood clots, including heart attack and stroke symptoms. One of my sons is checking in on me daily.
Time for a baby bunny picture, don’t you think? One of the best parts of the summer. 🙂
So, here’s the deal. I’m in a lot of online support groups where people are afraid to take drugs to treat their systemic sclerosis (scleroderma) because of the side effects. We live in a world where people are afraid to get vaccinated because of possible side effects. We live in a world where people are resistant to wearing a mask because of… well, they will tell you. They have more reasons than I have time to list, quite frankly.
This is kind of nuts, in my opinion. Did you see that list of diagnosed conditions? Yikes. Not good, little BLZ, not good. I’m facing down some really serious stuff here, and I will take these drugs, get through the side effects, and fight this like the hail-battered and grasshopper-savaged sunflower that I am!
Two weeks ago, I gulped down the first Ofev capsule. All those nasty GI symptoms arrived and had their way with me for several days, and then I was through it. I’m now on the final doses of all my drugs, and the sleep disruption is improving, the dizziness is gone, and the muscle pain is receding. I’ve learned to never, ever drink elderberry juice, and that green chili actually improves GI inflammation. I can do this, yes, I can!!
My niece referred to my battles with my disease and the drugs that I take as “rolling dice with the reaper”. I guess that is a one way to look at it. The other way is… you have to play to win. I chose to take the drugs; I chose to play. Hand over those dice, reaper!!
Last week I went to get my first follow-up CT scan of my lungs. “You can take off your mask, if you’d like,” said the technician. Nope! No one with a list of diagnosed conditions like mine should take off their mask in a diagnostic facility attached to an urgent care center. I roll more dice than I should as it is! Grumpy, the technician did my CT scan while I kept my mask on.
The message from my pulmonologist came last night. My lungs show improvement, and there is no new fibrosis.
Yarn, Books & Roses 