Rare Disease Day

A rare disease

Yesterday and today I've been feeling weak from Monday's chemunotherapy. I've been a little better than on the corresponding days last round, though, between knowing what to expect and some additional anti-side-effect medications. For now I'm mostly just lying flat and waiting for another day or two to go by. 

I wanted to get a post done today, though, because it is the eighteenth annual Rare Disease Day, a day observed worldwide to raise public awareness of rare diseases and to advocate for rare disease research and patient support. 

Any given rare disease is, by definition, rare. In the United States, a rare disease is defined as one that affects fewer than 200,000 people in the US, which comes out to about one in 1,650 people. Other countries use their own metrics.

The thing is, though, that the combined effect of rare diseases is not so rare at all. There are 5,000 to 8,000 such diseases, and it is estimated that 300 million people worldwide have them. But because each one of them is rare in itself, progress in developing treatments and cures tends to be slow and underfunded.

Mesothelioma is categorized as a rare disease. In 2021 (the most recent year for which numbers have been fully processed), there were only 2803 new cases of mesothelioma in the United States. (I always knew I was special.) 

Recently I was asked to provide a patient's statement for a panel convening in Europe this week that is deliberating on the approval of Keytruda (my immunotherapy drug) for a wider range of uses in mesothelioma there. I said, in part, that "to fight mesothelioma we need as many weapons in our arsenal as possible. We need to be able to use one weapon to push the cancer back as far as we can and then use another weapon when that first one fails. We need to keep pushing the cancer back until a cure can be found. Our lives depend on it."

Sadly, the US Food and Drug Administration (FDA) has postponed its events surrounding Rare Disease Day this year to an unspecified date in the future.

Heartfelt thanks to all out there who are doing their best to fight mesothelioma and other rare diseases.

Sleep and Signs of Spring

Thanks to everyone who has sent messages of support in the last few days!

It's now been two days since my second round of "chemunotherapy," and so far I am doing OK. I feel a bit weird and weak, and I have a metallic taste in my mouth, but all in all things aren't too bad yet. Tomorrow and the next day are expected to be the worst, though, so we'll see how things go from here. This time I have my new ultra-soft toothbrush, so hopefully my gums won't start bleeding again. 

I looked at the numbers for my blood counts. I wouldn't have been given the therapy on Monday if they had been too bad, but now I look at them myself, to my inexpert but increasingly informed eye they look pretty good. Not perfect (which would be too much to ask), but pretty good for a cancer patient.

On Monday my oncologist gave me an additional medicine for nausea. (I do find it notable—and useful—how willing doctors are to prescribe just about anything if one has cancer.) So far I have felt almost no nausea, which already puts me ahead of the last round's experience by several hours. The new stuff has also has the positive side effect of making me sleepy. I was expecting extreme insomnia the night after the treatment but instead slept quite well. Then I have taken long naps the two following afternoons. This seems to me to be a good way of spending time post-chemo!

The weather being absolutely lovely this afternoon, I tottered outdoors to check on my crocuses (pictured). These signs of spring bring me great comfort.

Welcome signs of renewal 

Two Done!

I've just come back from my second round of chemunotherapy. Many, many thanks to all who sent me encouraging messages last night or this morning. They really helped!

Pumped full of offsetting meds, I don't feel too bad yet from being poisoned, just kind of weird. 

This time I asked to have my feet elevated before they tried to draw blood from my port (which they do to make sure I'm healthy enough to get the chemo drugs). So I didn't nearly pass out. Instead... nothing happened. The blood wouldn't even come out. They asked me to breathe deeply, raise my arms over my head, hold my breath, and stand up. Nothing worked. Apparently this happens sometimes. The port can clot up, which calls for the injection of a local clot buster, which delays the whole treatment by an hour or so. We were already running late from my pre-treatment appointment with the oncologist, so at that point I let them draw the blood from my arm instead and let the clot buster work to get me ready for the infusions while they were analyzing the blood.

A friend had thoughtfully sent me a fleece chemo jacket, shown in the photo. These jackets have zippers in the sleeves and chest to allow access to either veins or ports on either side. Warmie kept me company as a comfortable armrest throughout, and Anand and Gita took turns providing further support (they only allow one human at a time, and Anand had to go teach halfway through). 

My chemo jacket—the new hot style

It all took way longer than originally scheduled, but it's done. The offsetting meds are still pumping through my system, so I'll have insomnia tonight, for which I have some audiobooks checked out and ready. They've given me more anti-nausea medications for the transition off the IV drugs this time. So the insomnia is expected and prepared for, and the nausea may be better dealt with. The fatigue will just happen. And the strange body odor and the need to use a separate toilet for a few days. There are other things I hadn't realized were the chemo, like itchy eyes and a runny nose. This is apparently because of the loss of fine protective hairs in the nose and around the eyes. I haven't noticed any scalp hair loss yet, but the oncologist says to expect some with this treatment. 

We'll see how it goes. 

Warmie packed up and ready to go home

Platinum-Level Treatment

Tomorrow is my second round of treatment, and boy do I ever have mixed feelings about it! I don't want to voluntarily show up for something that will make me weak and miserable. The alternative is even worse, though, so needs must.

I'm getting a combination of chemotherapy and immunotherapy, for which I have coined the term "chemunotherapy." Along with some medications to prevent the worst of the immediate nausea and inflammation, they'll be giving me three infusions:

1. Carboplatin. As the name suggests, this chemotherapy drug is actually made with platinum. So I am, literally, getting platinum-level treatment. In fact, many cancer patients get one of the family of platinum-based chemo drugs, which reduce DNA repair and synthesis, keeping the cancer from growing. This particular platin is less toxic to the patient than its predecessor, cisplatin, though cisplatin is still used depending on the particular cancer and/or patient profile. Carboplatin does still cause nausea, and it can cause a dangerous drop in white blood cell count. I am unlikely to lose my hair, though it may well thin. No sign of that so far, though.
2. Pemetrexed. This is a more specific chemotherapy drug which is used for pleural mesothelioma and non-small cell lung cancer. It is a folate antimetabolite, which prevents DNA and RNA synthesis, slowing cell division. I take vitamin B12 and folic acid to reduce negative consequences to me, as opposed to to my cancer.
3. Keytruda (pembrolizumab). This is the immunotherapy part of the cocktail. It is a "checkpoint inhibitor," which means that it turns off the cancer's protective messaging that tells the immune system that the cancer cells are me and not an invader. One of the most common side effects—which I have been experiencing—is fatigue. Keytruda has been approved for some kinds of cancer, such as advanced melanoma, starting in 2014 but was only approved by the FDA for mesothelioma in September of 2024. I am therefore getting the very latest treatment while also knowing that oncologists have ten years of experience with it.

Medical side effects to these medications range from the unpleasant to the fatal. A further side effect is a very large medical bill, particularly for the Keytruda (see photo). I have already hit my out-of-pocket maximum for the year and have surely endeared myself to my insurance company.

The eye-popping figures for a single treatment. The $1.20 is almost funny in the context.

The treatments are every three weeks. The general pattern is that week 1 is the infusions and worst of the side effects. During week 2 I can expect to recover from the side effects, feeling a bit stronger with each day. Week 3 comes with further improvement and is not so bad except for the growing dread of the next cycle. I'll get 4 to 6 cycles, depending on how my blood counts hold up.

Through the Looking Glass

Ever since I got my diagnosis, I've had moments of disorientation, especially when I first wake up or I emerge from a book or some other absorbing activity and realize yet again that I have mesothelioma. And each time, that realization makes very little sense against the backdrop of the world I thought I was living in. Instead, it feels like I have somehow slipped into another dimension, or a parallel universe, or Alice's insane looking-glass world. For a brief instant at such times my irrational mind thinks that the key is to somehow get back to the world I know and the way things were.

And then I finish waking up, and I realize there is no going back.

It's going to take time to fully adjust. I catch myself uttering phrases like "my cancer" or "my chemo," and it sounds outright bizarre to me. To my knowledge, I am the first person among my blood relatives to get cancer—other than the mildest skin cancers—since my grandfather's prostate cancer in the 1970s. I guess it's high time for a case, statistically speaking, but I could have wished for a different one!

I guess I'm not the only one to experience the disruption like this, as attested by the availability of the pictured Mesothelioma Awareness T-shirt.

Primate Grooming

My daughter, Gita, studies behavioral endocrinology in primates. Specifically, she studies the stress hormone (cortisol) and the bonding hormone (oxytocin) and how their levels in the body influence and are influenced by the primates' behavior. At least, that's what I think she studies. She might explain it quite differently!

The relevant part here is that one main way that primates bond with each other and help each other manage their stress levels is by grooming each other. They stroke and pick through each other's fur. Gita has had lots of opportunity to observe this behavior.

Macaques Grooming (public domain image)

As I am having ample opportunity to realize, cancer is a major source of stress. There's the pain caused by the cancer itself, there's the anxiety of waiting for the next round of chemo side effects, there's the wondering how much more I'll be able to do with my life, there's concern for my loved ones as they suffer alongside me, there's not being able to help my parents in their own ill health, ... and some other things I think about in the dark of the night sometimes but that I don't want to write down here.

Anxious stress is not only unpleasant but unhealthy. If I'm going to give my body the best chance to fight this cancer (and the best chance of not getting an autoimmune disease from the immunotherapy), I need to manage my stress.

Gita to the rescue! She correctly guessed that imitating the small strokes and picking action of primate grooming on my arms or scalp would be calming. It really is remarkably effective—the stress fades quickly and dramatically. I am therefore embracing my primatehood. Luckily Gita has yet to find any fleas on me!

Chemo Plant #1

A young academic friend of mine buys herself a plant whenever she gets a paper or a grant proposal rejected. This is such a smart way of taking the worst sting out of the rejection that Gita, my daughter, has adapted it for her own purposes: she buys herself a new board game under those circumstances.

Inspired by this system, I have a new scheme of my own: I am going to buy myself a new plant for every round of chemotherapy that I get through. So I have already ordered the first one: a Changshou kumquat tree. It is a relatively hardy, self-fertile citrus that should grow well in a container. 

Those who have been to my house may already be asking themselves the obvious question: Where, amongst all the plants I already bring in for the winter, will I find room for yet another citrus tree?

Room for more?

It's a fair question. But I find I don't really care about being prudent about my plants just now. I want to be surrounded by growing things, preferably ones that fruit. By the time the kumquat comes there won't be too much left of the cold season, so it will soon be able to go outside. If I need to buy some grow lights next fall to support all my plants, so be it.

I have long tended to draw back from purchasing non-essential things (like extra plants) that are likely to lead to the purchase of additional things (like grow lights) and then yet more things (like timers for the lights) until pretty soon you have a Very Expensive Hobby.

But you know what? I don't think I care anymore.

Instead, I'm looking forward to getting my kumquat.

Cause and Effect

I continue to improve after the depths of post-chemo side effects, which leaves me with the energy for the following philosophical musings.

In The Sound of Music, Maria (played by Julie Andrews) sings that "somewhere in my youth—or childhood—I must have done something good." She feels that she must have done something to earn her current happiness with Captain von Trapp (Christopher Plummer). "Nothing comes from nothing," she sings. It's a sweet song, but a dangerous philosophy.

Somewhere in my youth—or  childhood—I must have inhaled some asbestos. The fibers settled deep in my right lung and gradually sank down, like some sort of evil mud monster, into the lung's lining (the pleura), where they irritated and irritated and irritated the tissues until finally, some forty years or so later, the tissues turned cancerous.

That's what caused this. I didn't kick kittens or drown puppies or even dig up a cursed mummy. I simply inhaled. Like we all do, every day.

There are villains in this story (and I may get to them in a later post), but I am not one of them. I reject unequivocally the Sound of Music notion that we earn our good and bad luck. Some things we earn, yes. There are consequences to bad choices and bad behavior. But other things just... happen.

So I am not asking, "What did I do to deserve this?" 

And you, my dear friends out there, shouldn't ask that about your bad luck either.

Because the answer is simple: nothing.

My Team

I am feeling better today, thank heavens! I'm even writing this sitting up, which is saying a lot. 

Now that I can lift my attention from the individual moment, I want to devote a post to introducing my support team. A friend has taken to annotating texts that mention supporting me with #Team Amalia, and I feel a warm glow every time I see it. I do indeed have a great team.

The Medical Team

I have not one, or even two, but three oncologists.

Surgical Oncologist: This is the one I saw first and who put my port in. Mostly he'll be waiting in the wings until after my chemo/immunotherapy to see if surgery is recommended at that time. If it is, I'll be extra glad that everyone says he's the very best.

Medical Oncologist: This is the one I saw second and who is in charge of my treatment and day-to-day care. He is also highly recommended. I am deeply grateful for the level of accessibility that he and his practice provide for their patients. If I have a concern, I get an answer within minutes. He reassured me today that as we get to know how I react to chemo, better countermeasures to the side effects can be deployed. I am greatly relieved!

Research Oncologist: I have also been to see an oncologist who specifically researches mesothelioma at Johns Hopkins and who also comes with the highest recommendation. She helped to tailor my treatment protocol to my health history and genetic profile, which included getting carboplatin instead of cisplatin and getting my vitamin B12 shot a few days before my first treatment instead of during it. She will be able to connect me with the latest treatments and with clinical trials in the future.

In general, it's been gratifying how knowledgeable, attentive, and helpful all the doctors, nurses, and other medical staff have been. The nurses deserve special recognition for their unflagging care in the absence of the prestige with which physicians are compensated. 

It has been gratifying but very odd to get extremely prompt service, such as appointments within a day or two of requesting them, not the "Dr So-and-So has a free appointment slot in three months" that I'm way too used to in other healthcare contexts. It's yet another way in which having cancer puts you in a new and strange world. I can't help think of the supposed ancient Chinese curse: May you come to the attention of important people.

The Home Team

I couldn't ask for a better home team. The players are

Anand, husband: Anand is driving me to appointments, reading me stories, running errands, and learning about the household finances so as to be able to fill in for (or take over from) me. He's been juggling his teaching schedule at Johns Hopkins remarkably well under the circumstances.

Gita, daughter: Gita canceled her class at Spelman College, closed up her house in Atlanta, packed up her two cats, and moved in to help take care of me. Thanks to her channeling her inner Indian grandmother, she got my caloric intake back on track. She is working on her Emory postdoc remotely this semester.

I don't know where I'd be without the two of them. 

There are also the cats, of which there are now four. We are working very slowly toward integration. For now I'm just glad we bought a larger house than we thought we needed when we moved to Maryland as empty nesters.

The Away Team

These are all the wonderful people who are supporting me and my family in various ways. You know who you are! People have

• texted, emailed, and called with messages of support
• written an actual letter on real stationery
• sent cards or flowers
• come over to visit
• sent useful, meaningful and/or whimsical (pictured) gifts 
• suggested music for my playlists
• shared experiences with cancer and chemo
• coined #Team Amalia

From a member of the Away Team

Ambitions

This morning I had grand ambitions: I washed my hair. It almost did me in, and I staggered back to bed.

Things have been tough. Various people have assured me that the chemo after-effects last only a few days, but... No, I haven't been that lucky. I keep thinking that now, surely, I'm pulling out of it, only to be felled again. Ambitions keep having to be modified.

For anyone who has gotten a text from me assuring them that I'm doing "better" now, consider that if I am up to texting, I am by definition doing better than I am at my worst. Thus I always think I'm telling the truth at such moments, but those moments may be temporary.

I do have some hope for improvement, though. For one thing, more time has gone by, and the side effects do eventually resolve. For another, I've been prescribed a proton pump inhibitor, which can apparently be very helpful. And for a third thing, I have rediscovered the comfort of a traditional South Indian remedy, yogurt rice.

Meso messaging

Meanwhile, my daughter has indulged in some retail therapy on my behalf, including fitting us all out with anti-mesothelioma wear.

In other good news, my bodily fluids are no longer toxic from the chemo chemicals, and family members no longer have to use a different toilet or refrain from touching me if I break out in a sweat. A small win, but we take what we can get.

Flattened

For the past 48 hours I've felt pretty flattened. I have ondansetron and therapeutic sound frequencies to help with the nausea, but that still leaves me flat on my back, limp as a wet rag, and uninterested in ever doing anything again. 

The fact that I am writing this post (though from a horizontal position) is indicative of light at the end of the tunnel, I very much hope. They do say that chemo after-effects mostly clear up after about two days.

Meanwhile, I have worried my cat, Hekla. She's been spending a lot of time staring at me in concern. She is not at all sure that the food that other people put in her bowl is actually the same stuff as what I feed her. Her little world has been upended.

So has mine, Hekla, so has mine!

Hekla is worried.

Musical Ambush

I was going to do a post yesterday, but I felt pretty washed out after a night of insomnia. It turns out one of the very wonderful medicines they give to counteract chemo side effects, dexamethasone, itself has insomnia as a side effect. Today, after less insomnia (and a lower dose of dexamethasone), I'm a little more alert but I'm also starting to feel a bit nauseated. At this point, ondansetron (anti-nausea) is my friend.

Backing up to Monday, I went in to the chemo infusion center in the morning. The center is a field of low-walled cubicles, rather like some work places I've seen, but with reclining chairs and IV drips and nurses' stations around the edges.

My cubicle, with Warmie in the background

I got a place near the window, which meant a cold draft but a better view. At least they give out warmed blankets.

The nurses complimented me on the progress my port was making in settling in (it was indeed feeling better by then) and on the proper preparations I had made, applying lidocaine cream and covering it with plastic wrap. Then they accessed the port for the pre-infusion blood draw, and I nearly passed out. Both the nurse and my oncologist, who stopped by, told me this was just because it was the first access and the actual chemo wouldn't affect me like that. It turned out they were right about the chemo not affecting me like that, though I have my suspicion that my reaction was at least partly because of the difference between drawing out and putting in rather than between first and second times. I tend to get light-headed for blood draws anyway. They reclined my chair, gave me an ice pack for the back of my neck and a glass of ice water, and went off to test my blood and mix up my infusions.

So there I was, waiting for the infusions to start, feeling exceptionally vulnerable. I put on my new headphones, started the "Peace" playlist I had assembled, and turned it to shuffle. First up was James Taylor's cover of "You've Got a Friend." Perfect. Then the Duruflé "Ubi Caritas." Excellent. Then the Beatles' "Let it Be." Excellent again. I was beginning to relax, and congratulated myself on the construction of my playlist. The fourth selection was Israel Kamakawiwo'ole singing "What a Wonderful World." I looked out the window and thought, It is a wonderful world. And I want to stay in it.

And suddenly there I was, in tears. I find the quieter songs do this to me sometimes. They just leap out at unsuspecting moments and ambush me. And yet at other times their calming influence is invaluable. I may need to do some heavier-handed curation. Or it may be that the effects of any given song at any given moment may be entirely unpredictable. 

It may be just as well. If mesothelioma doesn't sometimes reduce a person to tears, they're in serious denial.

The first round of chemotherapy-cum-immunotherapy is now two days in the past. Side effects tend to peak Days 3 and 4. Fingers crossed!

One Done!

Very quick update: I made it through the first round of treatment! Granted, the side effects usually only kick in 4 to 48 hours later, so we'll see how that goes.

There are only three to five more of these particular treatments to go, at three-week intervals. One down is therefore a huge step.

Meanwhile, thanks and love to all who have sent supportive messages!

Oh, and I learned what the "fight cancer" plushie at the oncologist's is: a gnome. Tucked under my arm in the photo below is Warmie the Snow Leopard. Or Snow Leopard the Warmie. A very useful combination of heating pad, pillow, and emotional support critter—highly recommended.

Round One

The C-Word

Cancer is one of the scariest words in the English language. But not all cancers are alike, and medical science has made tremendous strides in diagnosing and treating some of them. There is cancer and there is Cancer. And then there is CANCER. In other words, in the absence of metastasis, some cancers, such as testicular cancer, are now very treatable. They even use the word cure in speaking of them, not feeling obliged to stop with the weaselly remission. The more serious Cancers, like leukemia, still claim too many lives, though not nearly as many, proportionally speaking, as they used to. 

And then there are the CANCERS, which still really, really need medical breakthroughs. Which is a polite way of saying they still kill most of their victims. These are cancers of the pancreas, liver, esophagus, brain, lungs, and ... mesothelium.

What's a mesothelium? (Who even knew they had one?) The mesothelium is the lining of various internal organs, including the lung, gut, and heart. The lining of the lung is called the pleura, making my kind of mesothelioma pleural mesothelioma, the most common kind.

I spotted this plushie at my oncologist's office.
I don' t know what animal it was, but I agree with the sentiments.

Another terrifying C-word of note just now is chemotherapy. The very word conjures up immense suffering, which is entirely understandable for a treatment that can trace its origins to the use of mustard gas during World War I. However, since its first uses—and even since I first became aware of it in the 1970s—chemotherapy has become more sophisticated and better targeted at the cancer rather than its victim, and treatments for side effects have likewise gotten much better. 

I start chemo tomorrow, along with immunotherapy, which aims to teach one's immune system that cancer cells are foe, not friend. I am frightened. But I am also eager to get started on fighting back. So far we have done fact finding, strategizing, and defense building, but not yet anything offensive. It's time to fight.

I will bring my headphones so I can listen to whichever of my three specially curated playlists (Peace, Joy, or Fight) I end up feeling I need. I'll bring a blanket and a book. And my loved ones (husband and grown daughter, only one of whom will be allowed in the room with me at a time, but who can spell one another). And, I sincerely hope, my courage.

Meanwhile, my port and I seem to be adjusting to each other's presence, so that's a win!

Port or Starboard?

I'm recovering from the port placement, but from my position here in the calm before the chemo storm it has yet to endear itself to me. It aches, and I find myself unenthusiastic about moving my right shoulder. They say that one's body and mind both adjust to it after a while--the sooner the better, I say!

I almost had the port put in on the left side of my chest, but luckily my husband asked the surgeon if that was really necessary, given that I am left handed. No, said my surgeon, it can equally well go in on either side. So we chose the right side for the reason my husband had identified.  It turns out there is another reason why that was a very good choice: I was already having trouble lying on my right side due to the disruptive presence of seven tumors in and around the lining of my right lung. Having more trouble on the right side turns out to be less disruptive than bringing the left side into the mix. 

So I have a starboard port.