Cosmetics

The chemotherapy has settled into a regular routine now, 21 days of Ninlaro, Revlimid, and dexamethasone, followed by one week with just dexamethasone. So far, this seems to be working in the sense that the kappa (that's the protein produced by the tumor cells) levels in my blood continue to decline. There's still about 7 times as much kappa as lambda (produced by properly working plasma cells), though, so there's plenty of work left to do. While all this is happening, I feel good most of the time, and I've been able to get out on my bike quite a lot during this weirdly mild winter.

When I don't feel great, it mostly seems to be something related to the drugs. Besides the chemo trio, there is a set of old-lady-who-swallowed-the-fly companion drugs, to prevent or treat possible consequences of chemotherapy: an antibiotic (doxycycline), an antiviral (acyclovir), aspirin (to prevent blood clots), prilosec (to prevent heartburn induced by dexamethasone). And stool softeners and laxatives for the usual reason (also a consequence of dexamethasone). All this is just now a part of the daily cycle.

There are a couple of things that are puzzling, and we're trying to figure them out, if only out of curiosity. I've already mentioned the rash, which I blamed on shrimp. It turns out that even now that I am avoiding shrimp, the rash still occurs, then fades after a week or so, seemingly in relation to the chemotherapy cycle. Another cyclical feature is an obnoxious rosacea or acne-like condition that will overtake my nose and surrounding skin very quickly and disappears just as quickly. The rash is still mysterious, but the acne seems to be following a predictable pattern at this point, peaking during the last 7 days of the 28 day cycle, when I am taking only the dexamethasone. On the first day of the next cycle, I take the Ninlaro and Revlimid, and the sores disappear rapidly. Of course, I'd like to know which is doing the job, but what I'd really like is to find something I could use during that week to control the nastiness.

Less annoying, though peculiar, is the fact that my hair has become curly. I'm happy to have it back, and the novelty of having curly hair is fun, although I have no idea how to deal with it. Liz has helpful advice, of course--in fact, my hair looks a lot like hers now. We've been speculating about how this happens at the cellular level. It doesn't seem possible for it to be a genetic effect, because that would be confined to one or a few follicles. Instead, it seems that all the hairs on my head decided to become curly in unison--so the follicles must be talking to one another. Maybe there's some literature on this out there somewhere.

Full disclosure: I've had curly hair before, sort of--a mid-70's perm, which explains why I didn't get many dates in college. I blame Mike Schmidt for this epic lapse in judgement. Never mind.

The return of the shrimp

So I'm most of the way through the first cycle of post-transplant chemo, have my hair back (I promise to post a new photo soon), even had a haircut the other day. All the lab numbers are looking good enough--I could wish for a little more hemoglobin, and the kappa could go down further, but I am not complaining and feel pretty good. Almost all my current complaints are related to my old friend dexamethasone, and even those are greatly reduced by the lower dosage.

So it was a little alarming to find my torso, neck, and arms covered with hives a couple of days ago. It didn't feel like much, but it sure looked like something was going on. Fortunately, I had my monthly oncologist visit scheduled for the next day, and we figured we could discuss whether this was some kind of adverse reaction when we came in the next day. So we reported for duty at 7:50 AM, barely caffeinated, and went through the prelim paces (draw blood, insert IV tube, wait for labs, check vitals, check the med list, are you in any pain), and then I mentioned to the nurse that I had hives, just like I used to get when I ate shrimp . . . and Liz and I looked at each other and I don't know whose draw dropped lower.

I had been allergic to shrimp and other crustaceans since I'd had an arthrogram (involving a largish injection of radioiodine) at age 16, but the allergy had faded over the years and I had enthusiastically welcomed shrimp back into my diet, especially in the last couple of years. A great sustainable, highly nutritious source of protein, really easy to cook and mighty tasty to boot. Two nights before the visit, we'd made a (gluten-free, for those of you keeping score) pasta with a cilantro-jalapeno-lime-cream-shrimp sauce. It was very good, and I'd eaten leftovers for lunch the following day. What's more, the last night we were in Maryland after Dad died, I'd had shrimp and grits at a great restaurant in Frederick.

I will probably have no more. I'm guessing here, but I'm pretty sure that the stem cell transplants have reset enough of my immune system to have restored the old shrimp allergy. It's a minor thing, in context. I will remember those shrimp fondly.

postscript

We have been sifting through the memorabilia since my father died, and it's hard to keep straight what has been posted where. Just for the record, I want to put this photo here. It must have been taken when Dad was in high school (1944 or 1945, I guess). I think it has that mythic quality of old photos: all the thousands of images we can generate now, cropped, shopped, auto-tuned ad infinitum can't hold a candle to it.

In Memoriam

My father died last night. He had been declining for a long time, heart, kidneys, and brain more or less in sync, and while at the end the turn for the worse seemed sudden, I think it was really just the result of a long slow descent, like an airplane landing (safely) in the fog.

I have the impression that stoicism is having a moment, appropriate for confusing and difficult times, and coincidentally Dad was the paramount example of stoicism among all the people I've ever known. He was famously self-contained. He grew up poor on tenant farms during the Depression, the family abandoned by his father at an early age, finding an escape route by working as a stable hand for posh families on the Main Line.

In spite of circumstances, and his legendary unwillingness to speak, he managed to become Class President and Salutatorian of his high school class (1945). According to legend, he enlisted in the Navy to avoid having to give a speech at graduation. As it happened, the war ended while he was still in training at Pearl Harbor, so he did a brief stint in postwar Manchuria, then a few months in Japan. He was discharged in 1946, but still owed Uncle Sam the best part of a year, and was called up for service in Korea in 1951. There he was attached to a logistics unit, set up to distribute pay to soldiers and sailors invading the North--which service was abruptly interrupted when the Chinese army routed the UN troops and the entire enterprise was abandoned in a mad scramble for the ships. When his term of service was over, he was offered a chance to take Navy pilot training, but was not enamored of the prospect of landing planes on ships, and decided to come back home instead. Not long after, he met and married my mother (BTW, the second most stoic person I know). They were married for 64 years.

His love for and ability with horses was something I didn't really know about until Karen and I were old enough to be introduced to riding lessons when I was 10 or so. I was a sad disappointment to him, lacking both ability and confidence, but Karen took to it. It was only a few years later we moved to a small farm with pastures and surrounding open country, and horses re-entered his life in a major way.

From that point on, I watched the most interesting developments in his life from a distance, although he had considerable (I won't say endless) patience for my insistence on fishing, tennis, ping-pong, horseshoes, and other recreations more compelling to me. Until age became a major factor, he was consistently better at all that stuff than I was, and my adolescent frustration fueled some pretty serious, seriously one-dimensional, competition. It might be that the whole drama was contained within me--an outside observer might have had trouble finding any drama--but I thought it was real at the time.

Dad's competence (and his confidence) was such that he rarely hesitated to quit a job when he had had enough. Mostly he had a new job within a few weeks, and he generally he seemed to feel better off for having made the change. Not always, however: he did a stint as "Mr. Goodwrench" at a local Chevy dealer, and hated the association with a lame but well-known advertising campaign. He quit that job too.

I think his glory days came in the decade or so after he retired (maybe a trifle early, truth to tell). He became a full-time horseman. Between fox hunts, horse shows, and the daily cycle of feeding/watering/grooming/exercising/mucking out, he was fully engaged and extremely good at his work. This was a job he didn't want to quit, and he didn't. He was still working at horses last year, in spite of age, infirmity, and people finding him an easy mark. If that could have been sustained, I think he might have lived forever.

Overdue update

Lots of changes in the last few weeks. I've started the next round of chemotherapy, with a new oncologist, gone on long-term disability, and finally managed to arrange a quick visit to Maryland to see Mom, Dad, and Karen. 

Dad's cognitive slippage has continued, and he needs pretty constant help. While he has trouble remembering lots of things, he is still pretty certain that he does not need to take instruction from his wife or daughter, so professional assistance is the only thing that works (my own feeble efforts are no better rewarded than Karen's or Mom's, and in any case are too infrequent to do much good). So he will be moving into assisted living soon. Mom will stay in their current apartment, which is right across the "street" (really it's a parking lot). I was happy to find a decent last-minute airfare and squeeze in a quick pre-Holiday visit, complete with a mini-Christmas dinner (glorified finger food). It wouldn't have impressed an outside observer, but our little gathering will rank right up near the top of my Christmas memories.

As for me, I am feeling good, and the new chemo routine seems a bit easier to take than previous rounds, partly because the new proteasome inhibitor (Ninlaro or ixazomib) is delivered orally rather than by injection, and especially because the dexamethasone dose is only 1/4 of the pre-transplant dose. I still go in for weekly blood draws, but am responsible for managing all the meds myself. Everything is on it's own schedule, so it's not quite trivial. Quoting from the Clinical Note: 

He will start with Ixazomib 4 mg/week (days 1, 8, 15 every 28 days), lenalidomide 25 mg daily days 1-21 every 28 days, and dexamethasone 20 mg days 1, 8, 15, and 22. Supportive care would include acyclovir 400 bid, aspirin 325 mg daily. 

The Ninlaro is something to behold. A 28-day supply consists of 3 capsules, and according to Express Scripts, they pay a little over $12,000 for those (I think this includes the negotiated discount!). Each $4000 cap comes in its own fancy (cardboard) box, although you might hope for a little gold leaf or at least some decent inlay at those prices. Of course, its true value lies in the unobservable things it does, and I don't want to sound ungrateful--I am depending on this stuff to keep me alive--but still.

Rosie is here for the week, and we have somewhat belatedly got our holiday situation together. The tree is a little on the short side. I swear we weren't (much) slower than usual in our tree shopping, but all the usual sources were mysteriously tapped out. I blame my fellow consumers for being over-eager. It's a cute tree, anyway, and Rosie was able to put the angel on top without benefit of a stool or ladder. 

2016 has been a strange year in so many ways. I guess I'm ready to take my chances on the next one. What could possibly go wrong?

Thanksgiving

We're back from a follow-up visit to Mayo, with little news to report, which is a good thing--the monsters, while still present, have not yet reawakened. I am feeling good, and have enough hair to have resumed using shampoo. That I take pleasure in this surprises me, but it is what it is. The hair is surprisingly helpful as an insulator, and I am gradually finding myself less in need of extra layers of insulation even though the temperature keeps dropping.

I'm now on long term disability, trying to figure out how to keep being useful and productive, while also trying to figure out how to balance my desire to keep being productive against my wish to enjoy as much as possible of my limited remaining time on earth.

The main point of the visit to Rochester was to plan the post-transplant "consolidation" chemotherapy.

Forgive me for getting slightly technical here, but just in case anyone else can ever use the information, I do want to record the plan we arrived at and the reasoning. A consistent theme from clinical trials is that patients like me with 17p deletions respond better to the class of drugs called proteasome inhibitors (Velcade, Kyprolis, Ninlaro) than to other classes of  drugs used to treat myeloma. So we want one of those, along with dexamethasone, which is both dirt cheap and the best single drug for knocking down the category of cells (lymphocytes) from which the tumors originate. The effect is much broader than you might hope for, and the side effects are unpleasant, but umpteen clinical trials have shown dexamethasone to be better than any known alternative. The last drug in the regimen is called lenolinamide, which goes by the brand name Revlimid. It's a close relative of thalidomide, the drug that got famous for causing horrific birth defects in the 1950s. Its action as an anticancer agent is not totally clear, but it has a great track record in myeloma, and has been responsible for a major improvement in survival. However, it's not as clear that it works well for patients like me.

Last year, a handful of new myeloma drugs were approved by the FDA. Among them were one proteasome inhibitor (Ninlaro), and two monoclonal antibodies (Darzalex and Empliciti). Because they're newly approved, and patented, and this is the U.S.A., they are all wildly expensive. Revlimid, Kyprolis, and Velcade are also wildly expensive, by which I mean a year's supply of any of those drugs will run between $100,000 and $300,000 per year, if your insurance company won't cover them.

And there's one more consideration. Revlimid and dexamethasone are tablets. All you have to do is swallow them. Almost all the rest are delivered by infusion, which means that you go to your local hematology/oncology clinic and hang out for an hour or two while an IV is delivered into the increasingly less willing veins in one or the other arm. If you've had any recent contact with your local health care providers, you will realize that "an hour or two" in the infusion room means a minimum of three hours of real time. Velcade can be delivered by subutaneous injection, which takes less time, but still requires showing up for an hour or two, and leaves ugly red welts all over your belly.

Darzalex may well be a wonder drug, and I definitely expect to make use of it. But it is not a cure, so it makes sense to put off its use until what I'm doing doesn't work anymore. Meanwhile, Ninlaro is a proteasome inhibitor, to which I'm likely to respond well, but I can take it orally, so I will not be tied to an infusion center for hours on end. So we're going with Ninlaro/Revlimid/dexamethasone. It's not a cure, but then again nothing is. And we'll probably need to change it out after a year or so.

Apologies to all for whom that was too much. May we all survive the next four years.

Benchmarks

There hasn't been much to report since we've returned to Louisville, and I have dutifully failed to report. Liz and I have been taking care of business old and new, between filing insurance claims, re-establishing our relationship with the US Postal Service, obsessively checking presidential poll aggregators, and so forth.

The weather has been spectacularly good here, and I became strangely obsessed with rehabbing some outdoor furniture we brought here from Salt Lake that had been sadly ignored. It got a few days of attention, and looks a little better, anyway. I've been working on the bike and feeling better, although I do need to lie down for a while after a ride.

And there has been baseball. I grew up a Phillies fan (it used to be a congenital condition, as no one with any sense would adopt them as a favorite), back when they were still working off the 77 year-long interval before winning their first World Series. When I was in grad school, living in Chicago, it was fun to play hooky on the occasional summer afternoon and take the el down to Wrigley. This was a few years before they put lights in, so all the games were afternoon games. The bleacher seats were $2, the beer was cheaper than that, and a few of my heroes from the 1980 Phillies had been traded to the Cubs. I'll spare you any further details, but it was easy to fall in love with Cubs, especially in 1984. Never mind what happened then. Or that other thing.

So I do have to say: Cubs win! Cubs win! And attribute it to Jack Brickhouse, although it may go back further. He was gone by '84, but a bit easier to take than Harry Caray while I was trying to comprehend the finer points of principal components analysis.

I'm thinking that I will make it a goal to be alive for the next time the Cubs win the World Series. On second thought, that might be setting the bar a little low, since those guys could do again next year. Maybe the Phillies, then. That's definitely a reach.