This has been a kind of strange week. The Mother of Cats came home on Monday after spending ANOTHER WEEKEND WITH THOSE KITTENS!!! and then she tried to make it up to us by being really nice all week. She fed us tuna twice a day and played with Mateo with the laser light more than usual. Like that would make up for sneaking off to spend time with these furry little guys instead of US.
Okay, they are kind of cute. Still, they aren’t as cute as Mateo and I were at the same age. Just saying…
Anyway, back to the strange week. The children in the neighborhood all went back to school (whatever that it…) this week and it suddenly is quieter in the backyard. A huge flock of geese flew over the yard yesterday, and today the Mother of Cats noticed that some of the berries on the bushes are turning red. The squirrel is more active than usual, and the moon was out in the daytime. Crazy, right? Here are the pictures.
The Mother of Cats is also excited about something called Pumpkin Spice. Is that a type of tuna?
Anyway, let’s now discuss the Mother of Cats and her COMPLETE ABANDONMENT of the Sharon on the Trail MKAL. She didn’t even bother to open up the emails with the pattern updates. She says that she hates her yarns, and she doesn’t love the pattern, and she just wants to knit the stuff she already has and to make some little knitted kitties to give away. I’d be concerned about her, but she is really happy with the stuff that she is knitting now, and I have to admit that it is looking good.
She put her sweater back onto the needles this week and got several inches of the body knitted. Looking good! She says that the funky looking new stitches are that way because they aren’t blocked yet. Mateo isn’t convinced.Mateo: I’ll reserve judgement until I see that new knitting blocked. Call me Doubting Mateo.
She really spent some time on the new socks that she is making, and she really likes the way they are coming out. I think that has something to do with how she just UP AND ABANDONED Sharon from Security in her quest to apprehend that villainous Keith…
Aren’t these fun looking socks?
She likes these socks so much she is already dreaming of which yarns she will use to knit the next pair with. Poor Sharon, I’m pretty sure that the Mother of Cats will never return to the trail. She has her eye on some purple yarns for the next pair of socks…
Well, that is it. I hope that you all had a wonderful Caturday.
The abandoned MKAL is Sharon on the Trail by Casapinka. I have decided to wait for the finished shawl before I put any more knitting time into it.
The weather has been playing hell with my breathing. A couple of lows tracked right over the state and in the low pressure I don’t do well at all.
Do you see the low? That’s the place where all the wind arrows are swirling together just east of me in the state (I’m located just east of Denver). Fun and games for people with lung/heart conditions!
Behold Hannah and Mateo as kittens… they were pretty cute!
I was a biology teacher in the time of the Human Genome Project. This week, with the coming release of the newest movie about Oppenheimer and the development of the atomic bomb, I’ve been thinking about the DNA and genes again (I know, it is a biogeek thing…), because one of the candidates running for the Republican nomination in the US wants to cut the Department of Energy if elected.
Kind of a loose chain of threads, you’re probably thinking. Am I right? Well… the Department of Energy is the agency that is responsible for the regulation of the nuclear energy industry in the US. There’s a lot of waste coming out of those nuclear reactors, and there was some concern about how much mutational damage was being done to DNA through exposure to radiation. Well, to figure that out, you need to know what undamaged DNA looks like. The initial drive to figure out what the human genome looks like came from that agency and once the results came in early this century the world completely changed. Like a big change. Like an atomic bomb level change. Like, there are now sites that have huge depositories of biotechnical data and tools to aid in research.
Hannah’s World: no big changes here!
In the classroom we biology teachers began to teach about the Human Genome Project and also did a week-long unit around the ethical problems associated with this new knowledge (bioethics, if you will). The kids grappled with dilemmas like… if you had the gene for a fatal, untreatable illness, would you want to know? If you were a child at risk for this gene, would it be okay for your parents to have you tested for it before you are 18? If your unborn child tested positive for this condition, what would you do? Would it be okay for human organs to be grown for transplantation? Who should get the transplant… a single father of 4, or a 16-year-old student in your high school? Should your employer allow you to continue in your airline pilot job if a genetic test shows you are high risk for a sudden cardiac event. Should genetic test results be private? Whew. Lots to grapple with in this unit.
Makes your head hurt, doesn’t it. Check out my knitting progress this week!
So, shit kind of got real this week. One of the members of an online support group for pulmonary arterial hypertension (a progressive and fatal heart/lung condition that I have thanks to systemic sclerosis) has just been identified with a gene (bpmr2) that causes the condition; her PAH is caused by this gene and can be inherited; she has a different type from me, but it is still PAH. Oh, boy. This is not good at all. The life expectancy right now is up to about 7 years, but you only need one copy of the gene to be at risk for PAH… there is a 50% chance for each of her children that they inherited the gene. Only 20% of people with the gene will develop PAH, but that is still a big risk.
Should she tell her two children? she is asking in the forum. They are in their late 20s. If she does, and they get tested, should they have their own children if they have the gene? Her heart is broken, literally.
Life expectancy has greatly improved over the last few years with new medications being generated in the modern climate of expanding cellular and molecular biological information. Untreated PAH (and PH kind of gets lumped together with it according to my pulmonologist…) has a life expectancy of about 2.8 years… not good.
Rose break! By the end of the discussion thread, she was leaning towards telling the kids.
Which brings me to the next shitty bioethical item that occurred this week. One of the members of another support group caught Covid and had to go off her drugs while fighting the virus. She has been slow to recover, and still feels pretty bad, but she took a pregnancy test and restarted her medications again a few weeks ago, only to discover this week that she is actually 12 weeks pregnant. Why did she have to take a pregnancy test before restarting her drugs? Because some of the drugs used to treat PAH can cause extreme damage to a human fetus. The enrollment process is very strict, and every effort is made to keep patients from this situation.
I am in grief for this woman. The doctors think that the baby has been spared the worst of the drug toxicity, but now she is working her way through whether to abort or not, to restart her drugs, or not. To risk death to save the baby, or to abort and restart treatment. If the baby is born in good health, will she live long enough to see it enter kindergarten. If the baby is born with health problems, her medical burden is increased. She is young. This is a horrible mess, and she is already too far along to get an abortion in many states in the US. I don’t know if she has other children, or what her support structure is…
This week I heard that some states are demanding private health records to identify any out-of-state abortions or transgender care that has happened in another state. I hope that this woman lives in another country…
Then I heard that a popular hamburger joint near my home is now going to fire employees who wear a mask. Say, WHAT?! Let me tell you, any person who has a serious lung/heart condition like mine wants to wear a mask, and they are so grateful if the person at the service window is also wearing a mask. Sometimes people offer to put on a mask when they see that I am wearing one. Now that person can be fired for putting on a mask… remember the young PAH patient whose nightmare began with catching Covid? Truthfully, any random virus can cause serious damage to patients with PAH, especially if they are also immunosuppressed.
So, there are a lot of bioethical dilemmas here, and the unintended consequences of people who want to make sweeping decisions without understanding all the interlocking systems involved and the potential ramifications are staggering. NO, you can’t just disband the Department of Energy, and sweeping, inflexible decisions about reproductive issues (that seem to be smugly self-righteous to me) can be disastrous. It is easy to order up genetic tests, but what happens once you have the information can be life-altering.
And don’t get me started on this animosity towards mask wearing…
I can’t help but think that no one should attempt to enact legislation without suffering through experiencing something like the bioethics unit that was taught at the high school where I used to work. I keep wondering, do these legislators actually understand nuclear power and weapons? Have they heard of the Human Genome Project? About gene testing? About rare diseases? They absolutely need to go see the Oppenheimer movie, maybe, and then write me a report about the Human Genome Project. I’m pretty sure that they would struggle with epigenetics, but it would do them good if they looked into it. They can get extra credit for a summary of pulmonary arterial hypertension. I would like to give them a book list of summer reading to get through on their breaks, because only the well-informed and educated should attempt to make decisions about these issues in the seven levels of bioethical hell that is the life of patients like me.
Because this week was a really hard one; for too many people this shit is real.
PS: Have you seen the show House? I kind of think that he could use a little bioethics sensitivity training, too.
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!!
Hey. I know that I’ve been missing for a while (again). In my last post I wrote about my upcoming trip to the cath lab to get a right heart catherization. This procedure involves having a small sensing device threaded into your heart to check (in my case) the internal blood pressure. Normal pressure in the right side of your heart should be about 14mm Hg; mine had measured 44 mm Hg in an echocardiogram so my cardiologist wanted to get a direct reading.
Here I am, rocking my scleroderma symptoms along with the cath wound. Puffy hands, bluish nose and lips, tons of little red dots on my face… That bandage came off the next day and the entry wound healed right up.
The procedure went well. My pressure measured in the low 20s, which was soooo much better than my cardiologist feared. The number was higher than the last time I had a direct measurement, so my pulmonary hypertension has advanced, especially since I am now on medication to treat my condition, but I’ll take it! It was, once again, a really positive experience and I felt well cared for by all the staff. I was pretty exhausted, hungry, but upbeat on the drive home.
Then I went into a flare… I slept for almost the entire week after the procedure. Seriously, like 12 hours a night and a couple of naps. I had to go back onto daytime oxygen. My hair started falling out again. I was dizzy and exhausted. My joints really hurt. A flare.
This week I’m finally awake again and I pulled out the blanket that I’m knitting. I knitted more hexagons and measured the blanket on the bed. I went out and bought more yarn. I have the hexagons to add another row and the blanket is slowly growing…
I think that I’m going to need more yarn… this is the Nectar Blanket by Ysolde Teague.
I’m really happy with the progress of the blanket, but I have to admit that it is becoming a little boring. I dream of other knitting projects as I sew the hexagons together. I have a serious urge to knit a new sweater in some fabulous colors.
Look at this little topper sweater!! It is called La Prairie (by Joji Locatelli) and I seriously want this sweater. I want to knit in cool colors. Did I mention that I live on the edge of the Great Plains? I long to wind yarn and to cast on and to start knitting those waves and bobbles…
I broke down and bought this kit to knit the sweater online a few weeks ago.
Don’t you love these colors?
The trouble is, I just have to dream and fuss about colors before I am happy. I’m not sure about the order in the kit yarn. I’m not completely a fan of the middle yarn, and I feel like the lavender should be in the middle. I’ve been digging in the stash and trying to image the finished product with other blends of color…
Here are three more spins on the yarn. I like the one on the left the most, with the yarn fading from dark purple to the light grey at the sleeves and bottom of the sweater, but I’m kind of interested in the middle and right versions. I have to ask myself… which version will have the most flexibility in the wardrobe? Definitely, it would be the first spin on the kit, the version with the light grey at the sleeves.
I also desperately want to buy yarn to knit a Soldatna Crop sweater, and some new arm warmers, and then there are those PICC line covers and hats to get done… Did I mention that I have been looking at more yarn online and dreaming of sweaters with lots of colors in DK weight yarn? I’m totally on a knitting drive, but my wrists are not on board with all this needle action. It could be that I’m not completely done with the flare…
Sigh. Guess I’ll just cast on another hexagon or two…
PS I’m still dealing with shortness of breath and low on oxygen; lung testing happens next month as my doctors continue to sort me out. 🙂
Well, here it is again. Rare Disease Day. I kind of was going to ignore it this year because I’m quite frankly worn out by my… wait for it… rare diseases, but I also feel like I should pull myself together and represent for the community again.
People with rare diseases are referred to as Zebras in the medical community. I obtained my zebra status when I was diagnosed with systemic sclerosis in 2014. This zebra was sent to me last week by my Most Knitworthy Niece Melissa.
I wrote a pretty darn good post last year about my journey with a rare disease which you can read here if you want. I talked about rare diseases in general, my specific conditions, and the many things that have been said to me by my doctors over the years. I thought about just reposting what I wrote last year, but I’ve been reflecting all morning on some recent events that kind of shine a light on my situation and that of other people who are coping with rare conditions.
I recently managed to go knit with my fellow members of Frayed Knots. This was a big social outing for me because it’s hard to get out of the house, and I have to be having a really good day to go to something like this. Knitting with friends is just “normal” for most people, and it would just be a little part of their day, but for me this was something that I had to prepare for a couple of days in advance, and then recover from in bed the next day. Many rare conditions are chronic, and chronic illnesses can be very isolating by their very nature.
A woman at the knitting group questioned my decision to wear a mask. I started to explain, but she cut me off to say that I was doing it so I could feel comfortable. It was a little condescending and suggested that I was being paranoid. Truthfully, my immune system, crushed by the drugs that I am taking right now, is compromised in its ability to make antibodies. If I catch a viral disease like the flu or Covid, there is a good chance that I won’t survive. My vaccinations have a low chance of protecting me for the same reason. For people with rare diseases, life is fraught and full of difficult decisions. For me, and for many other immunocompromised individuals, simple decisions involve life/death level risk analysis.
Another woman at the knitting table was struggling with long Covid and shared her difficulties with returning to work. She especially felt crushed by the attitudes of her coworkers who seemed to feel that she was “fine” and just trying to get attention. Yep. Been there, done that. Many rare diseases are genetic or largely invisible to others. Invisible illnesses are especially hard to cope with because others tend to question their validity.
I’m in several online support groups, and there are always discussions about what drugs to take, and whether the side effects are worth the risks. Yep. There are no specific drugs for systemic sclerosis, no cure, and treatment can involve a patchwork of risky off-label drugs. The drugs that are used are often non-specific carpet-bombing like approaches. Rare diseases have fewer treatment options because there are only a limited number of patients.
Over the last year my wonderful team of physicians have been suggesting that I am really unusual and have been extremely responsive to my emails. They clear an hour for appointments with me. I’m one of the very lucky zebras who has managed to get diagnoses, secured treatment, and am benefiting from a team of collaborative, interdisciplinary physicians who actively communicate with each other and with me; just last week my rheumatologist told me that for a patient with my status this is the only way to deliver care. I’m so grateful to have secured this level of medical attention, but I also feel a little nervous about it. I spent some time this morning trying to work out the probability of one person having the several medical diagnoses that I’ve racked up since 2014. Like, just how rare am I?
The National Organization for Rare Disorders estimates that there are 100,000 patients with systemic sclerosis in the United States. That’s rare, but still, a big club, right?
The 15% Rule is a general measurement of the risk of severe organ involvement in systemic sclerosis. As it turns out, quite a few of the major lung, heart, and kidney complications associated with systemic sclerosis happen about 15% of the time. I have Sjogren’s Disease overlap with my systemic sclerosis, which happens in about 13% of patients. Suddenly, I’m in a much smaller group of about 13,000 patients.
My most worrisome complicating conditions are diastolic dysfunction (a type of heart failure), pulmonary arterial hypertension (PAH) and interstitial lung disease (SSc-ILD). I looked up the risk of having each of these conditions using the 15% rule data, and it turns out the risks are 16% (diastolic dysfunction), 15% (PAH) and 35% for the SSc-ILD. Did you notice the the ILD doesn’t fit the 15% rule? Yep. It’s much more common and is the leading cause of death in systemic sclerosis patients. I found that risk factor here.
I brushed up on my probability math (you multiply the probabilities of independent events…), and after running the numbers:
100,000(13/100 x 16/100 x 15/100 x 35/100)
I came to a grand total of 116 other patients in the US who share my set of diagnosed conditions.
Oh.
See, I have lots and lots of stripes. Stripes in purple, teal, periwinkle, red, green, and blue: these are the awareness colors for my conditions.
I just ordered that rainbow zebra unicorn shirt! I plan to wear it with my mask on my next social outing…
You can learn more about Rare Disease Day or my conditions at the links below.
Wow. It has been some time since I last posted. Ironically, the last post was about the Mother of Cats being broken. I felt really good, and I was casting on a plethora of projects and was all over the place with my knitting, reading, projects, you name it. Hannah was complaining in that post that I was broken, and that I wasn’t devoting myself to making her a blanket.
Then scleroderma lowered the boom on me. I went into a flare of epic proportions. I hurt all over, I struggled to do just simple tasks, I started sleeping 10-12 hours a day, and I had to go back onto daytime oxygen. I kept heat packs on my sore joints, but I really needed a total body heat pack to cope with what was going on: every single tendon in my body was under attack!! I couldn’t regulate my body temperature and took multiple naps a day because I was too cold to stay awake. I developed chest pain. My brain fog became so bad I wasn’t safe to drive anymore… Yep. Broken.
Okay, I also accidently drank some Miracle Gro fertilizer that I had stored in a Snapple bottle. That is some serious brain fog, people!! (Yes, I did have to call poison control, I did eat burnt toast and I did drink a lot of water, and I don’t want to talk about this anymore…) Yep. Broken.
I managed to get some knitting done in spite of my ill-behaved health. I got some wristers made as Christmas presents and sewed zippered pouches for my sister and cousin.
Wristers and a zippered project pouch.
Sewing was a riot as I had to get the sewing room organized so I didn’t have to stand up very much as I cut, sewed, and pressed fabric to make the bags, but with some creative thinking and furniture arrangements I pulled it off.
I also finished my Emma sweater. I made a hat. I made some socks. I made some slippers. Everything was on big needles and used heavy weight yarn as I got too tired to knit more than a few minutes at a time.
This sweater was knit using the CocoKnits method and I learned some new methods of shaping and finishing. The yarn was chunky weight and is warm and comfy. I got it done early in the flare and it was just perfect for the days to come. The yarn that was left over was used to make a hat and a pair of slippers.
I did read some nice books, but mostly I slept and waited this miserable thing out. In the weeks while I was mostly bedbound my orchids started to grew stems for future blossoms, the first snows arrived, and my son moved in with his cats.
This is handsome Jonesy. Hannah does not like Jonesy, so there is now a chicken wire barricade at the top of the stairs to keep the two groups of cats separated. My son also has a long-haired tuxedo cat who just doesn’t care about my cats. Hannah, however, has a complete meltdown if Jonesy comes near her, so… barricade.
Mateo: I love Jonesy and I go through the barricade every day to rough house with my new best bud while Hannah hangs out with the Mother of Cats. Jonesy is the wild playmate of my dreams!
So, I think that I am out of the flare now as the multiple naps a day have stopped, and my appetite is back. Yesterday we made lasagna for Christmas and here is the present that I got from my son:
Perfect, right?
Can you see my blue lips? The bad news is that the Blue-Lipped Zebra is back in full force. I finally emailed my cardiologist to let him know that I am once again panting for air and feeling light-headed from doing simple tasks like… lifting a pan out of the cupboard or grating cheese. Ugh. I’m pretty sure that I am heading back into the cath lab for another look at my pulmonary pressures, but right now I am glad to be back on my feet and out of the flare. My son is still here (he works from home, so he just moved his operation over to my house) and things are going well in the combined household.
Hannah: as long as Jonesy stays on his side of the barricade!! Also, maybe the Mother of Cats will now settle down and get my new blanket done!!
There was more response than I expected from my last post about my efforts to obtain a better understanding about my chronic conditions, the new drugs I was taking, and how to lower my inflammation. I offered in that post to share the links that I used to figure out what foods impact the levels of cytokines that are important in my systemic sclerosis. I’ve gotten feedback/requests for the spreadsheet with my links that I used in my adventures at PubMed.
I’ve been thinking about this for a couple of days, and I think that the most useful thing to do would be to share my process as I hunted down information about cytokines involved in my systemic sclerosis (SSc), and the cytokines that I needed to pay attention to in my diet.
I started out asking: What are the cytokines involved in systemic sclerosis? I did searches using terms like cytokines and systemic sclerosis. Here are some of the links to papers that I found.
I kept a list of the cytokines that were being measured in these studies and I finally began to get a sense of the four big players that were being measured and considered as targets for therapy: Tumor Necrosis Factor alpha (TNFα), Interferon gamma (IFN-γ), Interleukin-6 (IL-6), and Interleukin-1beta (IL-1β). You can follow the same process to research any inflammatory disease that you are interested in; you may get different results. (For example, C-Reactive Protein, called CRP for short, features in many inflammatory conditions. For little SSc me, not so much… my levels are normal.) Anyway, once I had the cytokines of interest, I did searches on all 4 of them. Here’s my info on TNF-α.
Information
Source
TNF-α is also a potent inflammatory mediator and apoptosis inducer
Produced by activated immune cells (macrophages, NK cells, T-cells) known to trigger a series of various inflammatory molecules, including other cytokines and chemokines
I did the same for the other three cytokines and it turns out that for me, it is best to reduce all of them in my blood. At this point I made a little chart with the 4 cytokines at the top and began to do searches on different foods that I thought were anti-inflammatory.
Food
TNFα
IFN-γ
IL-6
IL-1β
Chile (Capsaicin)
Tart Cherry
Tomatoes (Lycopene)
Question: Which foods/supplements will decrease these cytokines levels
Now the searching gets easy. I just typed in the questions in the search bar like, “Does capsaicin reduce TNFα” or maybe “Does capsaicin reduce cytokines” and the answer would quickly pop up with some resources. Warning: the search engine will give you the pertinent text and then you are on your own as you search through these papers! If you just go with the fast answers, you will take some risks… I told my sister that tomatoes were bad because I didn’t read the whole text, and I’m not sure if she has forgiven me yet…
Anyway, here are some papers about the foods/supplements above:
If I found a source that said one of the cytokines was reduced by one of my targeted foods, I just put an “X” in that box and moved on. I was no longer recording all of the links in my excitement, and as I found info it sometimes confirmed previous finds, but not always. If I got contradictory information, I was forced to read whole papers. Ugh. Also, you might want to try a different search engine; I used Chrome on my phone and Explorer on the computer, which is another reason for incomplete links on the computer. For the three foods in the table above it ended looking like this.
Food
TNFα
IFN-γ
IL-6
IL-1β
Chile (Capsaicin)
x
x
Tart Cherry
x
x
Tomatoes (Lycopene)
x
x
x
Question: Which foods/supplements will decrease these cytokines levels
I hope this unpacks the process that I used and helps with any searches that you decide to do that are specific to your own needs/interests.
I’m reading a really wonderful book right now that is really speaking to me on so many levels.
This man is a BioGeek of the first order! He interweaves his experiences, patients, memories, and the history of cells together in a way that makes me green with envy. He unpacks the history of our understanding of cells by bringing those scientists to life in a way that makes me care about them; if only I could write that well. He is the teacher that I wish I had been as he reveals to us how cells work together to create complex human systems, and then ties all of that to the treatment of disease. I’m still in the first parts of the book, but I have already filled my kindle with highlights and notes.
This book was published at the exact right moment when I needed it. I have totally gone down the rabbit hole at PubMed over the last two weeks as I have read paper after paper while chasing down the major players in my chronic conditions (why am I sick, and what exactly are these new meds doing…) and how they link to inflammation. Why would anyone do something like this?
Well, it all comes down to this. I’m on high-risk drugs with some serious side effectss, and I want to make informed decisions about whether I continue taking them. I also had a run-in with elderberry juice, and was rescued by green chile; as a BioGeek I was sucked down the curiosity rabbit hole after that whole adventure. What? That doesn’t happen to you? Listen, it has been so bad I haven’t even been knitting!!!
These are the two drugs that I’m trying to understand. In his book Siddhartha Mukherjee argues that our understanding of cells, and how they work, has transformed medicine into the modern miracle that I am currently benefiting from. Drugs that directly interact with the molecular machinery of cells, the signals between them and the biochemical pathways that cells use to function, are the first of the major transformative directions modern medicine is taking in the treatment of so many pathologies such as cancer, diabetes, neurological, and autoimmune diseases such as mine.
Systemic sclerosis is really darn complicated, as it turns out, and the sequence of events that have been happening in my body are so convoluted it’s hard to track them all. It started in the cells lining my blood vessels. As those cells got injured, they sent out signals that activated parts of my immune system. Signals from the immune cells caused other cells to transform and they began to produce scar tissue… scleroderma means “hard skin”, the hallmark of my condition. Whew. Here’s a condensed version of all that if you want to torture yourself and/or fall asleep.
Let’s go back to my meds. Ambrisentan blocks a molecule that is involved in making blood vessels constrict and raises blood pressure when it is active. That molecule, endothelin, is getting turned off by the drug, and there is evidence that this will improve my exercise-induced pulmonary hypertension and will also keep it from progressing; it plays nice with my other pulmonary hypertension drug which shuts down an enzyme pathway involved in blood pressure. Ofev is my new (fairy dust) drug, and it disables some of the essential enzymes in the cells of my lungs that are involved in creating scar tissue. Interstitial lung disease is currently the leading cause of death for systemic sclerosis patients; mine is being treated by side railing the process in the cells that are essential players in the pathology.
Yay! Molecular trickery at the cellular level saves the day! I will be staying on these meds as long as I can.
Dancing to the tune of the song of immune system cells are cytokines, the messenger molecules that travel between immune system cells and other cells that they interact with. The dance is complex, with all the different messengers traveling through the blood to target cells in the body, latching on and causing the cells to take actions. Some cytokines increase inflammation, and other will shut it down. Your immune system can get dialed up or shut down, depending on what the messages are. In my travels through research papers at PubMed I focused first on what cytokines were involved in systemic sclerosis, and then I hunted for papers that had measured the levels of these cytokines when people ate different foods.
Foods that you consume can make a big difference, evidently. Elderberry made me much worse (I cried in two different doctor’s offices), and green chile saved the day. I was done doing google searches for “anti-inflammatory foods” and was going after hard data.
.What did you expect? I’m a BioGeek. OF COURSE I made a spreadsheet with the data!
Tumor Necrosis Factor alpha (TNFα) is a big driver in the whole systemic sclerosis story along with Interferon gamma (IFN-γ). They cause an increase in two more cytokines that promote inflammation, Interleukin-6 (IL-6) and Interleukin-1 beta (IL-1β). All four of these bad boys will make my inflammation worse and (probably… I’m guessing here) encourage my conditions to progress. A lot of these foods/supplements will lower the levels of these cytokines, which explains why I feel better when I eat them. ELDERBERRY increases three of these cytokines which is why I felt like death warmed over while drinking it. Google said it was anti-inflammatory… can you see why I switched to research papers and cytokines? Green chile stew has tomatoes and green chile in it (and some yummy pork and garlic!); no wonder it turned things around. I will try to eat as many of the “good” foods as I can, but I’m going to focus on ones that really shut down TNFα and IL-6. I’m ignoring the IL-10 and CRP info because it wasn’t really as well supported as the others, and I know that my CRP (C-Reactive Protein) levels are normal.
My lunch smoothie: tart cherries, raspberries, banana, spinach, yogurt, chia seed (gag) and cranberry juice. For dinner I’m having a green chile cheesy corn pudding thing that tastes pretty darn good.
Wow. Did you read all of that stuff above? You deserve a prize for perseverance.
Here’s your prize. It’s like a “Where’s Waldo” picture, but this one is Where’s Hannah!
So, there is all is. Inside my systemic sclerosis, pulmonary hypertension, interstitial lung disease self, there are all these dancing cytokines, following the song of cells. Scientists who were captured by all of this and who were entranced by the Song of the Cell have developed the drugs that are treating the two life-threatening complications of systemic sclerosis that have come my way. Inside me, the promise of the song goes on.
Time to get back to my book.
Notes:
Okay, I made a whole other spreadsheet with links to all of the research papers that I used to get some understanding about these cytokines, and which were important in my disease. You don’t want to see all of that, right? If you do, say so in a comment and I’ll send some links your way!
I became curious about what is happening with Covid patients and the cytokine storm that can cause severe symptoms. Yep. It’s happening because of TNFα, IFN-γ, and IL-6. If you catch Covid, I don’t recommend elderberry.
Clinical trials are currently underway to see if an IL-6 inhibitor will be an effective treatment for systemic sclerosis.
I’m a lucky, lucky girl. I have a degree in molecular biology, used to work in an immunology lab that focused on IL-1, was involved in a scleroderma research project, and finished up my lab days on a project looking at the impact of capsaicin on rheumatoid arthritis. I can almost understand what I’m reading on PubMed. Almost.
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 