Recent Developments

This past week the oral surgeon received the results of the CTX test, so I travelled to his office to discuss the situation with him. My score on the test was 175; anything over 150 means that the risk of ONJ is minimal. He is therefore willing to extract #27. We discussed the replacement options, all of which strike me as unpalatable (pun intentional), other than the implant. Of course an implant carries with it risks of its own: the bone may not bond with the implant; the gum may not heal and/or may become infected; nearby teeth, nerves or muscles may suffer collateral damage. Having watched the educational video on the subject, I scheduled the extraction for August 1. Gulp.

Meanwhile, the oncologist has changed my chemotherapy regime. We discussed the concerns about prolonged Revlimid exposure leading to additional hematological cancers, and he offered an alternative protocol involving a lower dose, in exchange for giving up the one break week in four. I'm going for it. I'm hoping that eliminating the break week will allow my digestive tract to figure out what it is supposed to be doing; after more than a year, it has never gotten used to the switching on and off. The old 25 mg Revlimid capsules were large white oblongs; the new 10 mg capsules are smaller, half blue-green, half pale yellow.

The oncologist took the opportunity of this discussion (and the recent two-year anniversary of my diagnosis) to remind me that, although the trials establishing Revlimid as a maintenance therapy showed significant delays to post-transplant progression, they could say nothing about its ultimate effect on survival, vs. waiting for progression before hitting the cancer with it. That's because they stop too soon to show that. One would have to drop Revlimid on the patients in the placebo group after they showed progression, and then continue to follow the entire group until death. No one can wait that long to find out the answer, so I am part of the de facto clinical trial that will ultimately determine it.

The CTX Test

Having successfully petitioned the oncologist to suspend the Aredia (IV bisphosphonate) therapy, earlier this week I presented myself in the office of an oral surgeon, who concurred with the endodontist's diagnosis of external root resorption in tooth #27. If he is to perform an extraction, he insists (as the endodontist predicted he would) on a CTX test to gauge the risk of osteonecrosis of the jaw (ONJ) arising from the bisphosphonate therapy. Of course an oral surgeon has the power to write a prescription for this test, so he sent me home with such.

Ordinarily, if an MD gives you a script for a blood test, say for a lipid panel (cholesterol check), you just take it to a local outpost of LabCorp or Quest Diagnostics. After a little dance involving making copies of your medical insurance card and a photo ID, and signing a form agreeing to pay if the insurance company for some reason declines the honor of doing so, your blood is eventually drawn into one or more little tubes, and some days later the results are transmitted to the doctor's office. That's the basic business of these giants of the medical-industrial complex.

Things are a bit more complicated for a cancer patient. I once tried taking my monthly script to the nearest LapCorp site. Of the three people who happened to be working there at the time, only one claimed to have any idea what the script was talking about. He happened to have worked in a cancer clinic. He said, "Yes, I'm pretty sure we do that, but...". But the logistics surrounding getting the blood sample to the right test lab were such that I could be given no reliable ETA for when the results would reach the oncologist's office. No sale. Instead, although it's a longer drive, I always take this script to the lab in the cancer center; for them, this test is routine, and is reliably turned around within 48 hours.

The cancer center lab waved off the CTX test, which surprised me a little; but then, the oncologist had told me that he has no use for it in his practice. The oral surgeon was pretty sure that Quest could do the test, which I confirmed with a phone call to the nearest Quest site. However, when I arrived there, to my surprise, my insurance card was rejected; the test would be done only if I presented a credit card. So: One of the largest providers of medical diagnostics in the country won't play with one of the largest providers of medical insurance in the country. Love this system.

On the way home, I stopped at the LabCorp site. "I've worked here for seven years, and I've never heard of this test." A phone call to LabCorp customer service confirmed the technician's fear that he couldn't do the test.

My next thought was to discuss the situation with the medical insurance company. What would happen if I paid Quest to do the test and then submitted a claim? But before I did that, another question occurred to me: What if the CTX test really travels under a different name -- a more technical-sounding name that people preferred to abbreviate as CTX? A few moments with Google provided the answer: The CTX test is really the "C-telopeptide serum" test. There is an even longer, more technical-sounding name (the name from which "CTX" actually comes), but I took "C-telopeptide serum" to the test menu on the LabCorp web site and, lo and behold, there it was. Just to be certain, I verified with the oral surgeon's medical assistant that this really is the test I was supposed to get.

This morning, I journeyed back to LabCorp, this time armed with the LapCorp test number for "C-telopeptide serum", and was in and out of there in minutes. All I have to do now is wait for the oral surgeon's reaction to the results.

Rare Cancer Genetics Registry

In the wake of the tremendous progress made in the field of genomics over the past decade or so, research into the genetic underpinnings of the diseases collectively called "cancers" is hot. Genetic flaws -- missing genes, extra genes, transposed genes -- have been implicated in a number of different cancers. Worldwide, researchers are racing to add to this store of knowledge.

Meanwhile, Congress occasionally frets about the fate of victims of so-called "rare" cancers which, collectively, add up to about 25% of cases and deaths. Because of the relatively low number of patients having a given rare cancer, it is difficult to populate clinical trials relating to the disease, and a drug targeting it is difficult to deliver in an economically viable manner. Hence, for example, the Orphan Drug Act, which is meant to short-circuit the ponderous FDA approval process for drugs targeting rare diseases, thus making their production more attractive to the pharmaceutical industry.

A couple of years ago, the National Cancer Institute funded the establishment of the Rare Cancer Genetics Registry, which is meant to be a storehouse of the raw materials necessary for research into the genetics of cancers such as multiple myeloma. The registry contains a database of patient family medical histories, pathology records, and DNA samples. Researchers have the ability to obtain tumor tissue samples, and to contact registrants regarding voluntary participation in studies.

Earlier this year, I received from Johns Hopkins, which is one of the research and recruiting centers of the registry, an invitation to participate. It took me several months to round up all of the required family medical history information (this is how I learned that Grandpa Shaffer's "bone cancer" was really metastatic lung cancer), but a couple of weeks ago I finally sent in the completed questionnaire. Today I sent in my DNA sample, in the form of a tube of saliva.

It is possible, but unlikely, that my entire genome will be sequenced; far more likely that only those portions of it implicated in myeloma will be cracked open. But of course if over time more of it needs to be examined then it will be available for that purpose. In any case, the results of none of this activity will ever be available to me personally. There is no predicting when, if ever, I could be asked to participate in a study, or what such participation might involve in terms of personal commitment. But at least now I'm in there.

Back On My Bike

Before my first trip to the hospital, almost exactly two years ago, I was fairly physically active. I was doing some running (actually running wind sprints) and biking, and doing some light weight training. All this came to an abrupt end, of course, with the onset of my crisis. Since then, once I recovered (more or less) my ability to walk, walking has constituted almost the sole form of physical exercise for me. I use a treadmill or, if walking outside, I continue to use the cane as a safety device. Probably, I will never run again, since that would risk injury to the fragile, disease-ravaged spine. I find the idea of water exercise fairly tempting (I swam for years, back in the day), but fear the chlorine (eventually the irritation it causes the skin becomes unbearable, which is why I stopped swimming in the first place).

But then, there is the bike. This year I was determined to give it a try, once the winter broke. I wasn't completely confident. The muscles used in riding a bicycle are different from those used in walking, and they hadn't been used in a long time. There remains substantial numbness in the lower half of my body, so I wasn't exactly sure how well I would be able to control the vehicle. And once one's feet have left the ground, there isn't a lot of room for error. A fall would of course be fairly calamitous.

The transmission on my bike is 24-speed, so it can be cranked down to a point where, on a level surface, very little force (but a lot of pedaling) is required to achieve forward progress. I assumed that I should begin with this configuration, not knowing how much force I would be able to apply. I wasn't even completely certain that I could remain balanced on the thing. But as it turned out, things went better even than I was hoping. I was able to advance the gearing (but still considerable below the old normal) and ride up and down the street some way a couple of times. I was very happy with the results of this first attempt. I certainly could feel the fact that I was waking up muscles that had been asleep for a long time.

Since then, I have made a lot of progress on this. I can ride several miles, negotiating steep hills, just as before, albeit at a much slower pace. I can feel my strength and stamina improving. I'm not sure it will ever be the same as it was, but that seems hardly to matter. For now, I can just enjoy the ride.

Reefer Madness

Last week, Delaware Governor Jack Markell put his signature to the state's first medical marijuana law. Of course, cancer is at the top of the laundry list of maladies that qualify patients for participation. I guess it's time to go shopping for a nice bong...

Kidding. For a state that trends to blue politically, Delaware is relatively socially conservative, and this is reflected in the fact that the law is substantially more restrictive than those currently in effect in, say, Colorado or California. Patients are not permitted to grow their own, and must obtain their smoke (a maximum of six ounces) from one of only three non-profit dispensaries (presumably one per county). Patients must have an on-going relationship with the prescribing MD; are supposed to have exhausted all the standard alternatives; and must obtain a state-issued ID card. It remains to be seen just how much of a problem there will be with people with nasty hangnails showing up at the dispensaries with ID cards and prescriptions, since there is an escape clause covering individuals with unspecified "chronic" and "debilitating" conditions. On the whole, it all sounds a lot more inconvenient than just driving to certain Wilmington neighborhoods on Friday night, but I guess that's partly the idea.

Huong asked me how interested I am in this. The answer is "Not Very", at least for now. I am not currently taking any kind of pain medication, not even any of the OTC varieties (OK, yes, I'm taking 81 mg aspirin, but that's for the heart, not for pain), and I'm hoping to avoid having to do so for as long as possible. The prescription opioids (e.g. Oxycontin) render me hopelessly constipated. I guess if it ever comes to the point where I'm pretty desperate then I'd consider smoking up. Actually, I'd prefer not to smoke it; I'd probably invest in a nice vaporizer.

Minus Aredia

The oncologist did not require much in the way of persuasion to call off the Aredia treatments for the time being, in honor of my recently discovered need for oral surgery. But he did question the need to order any CTX tests, indicating that it should only be necessary to wait a decent interval before proceeding. That was not the impression I had gained from what the endodontist had told me, so these two titans of medicine will consult, and presumably arrive at some kind of consensus on the subject.

This month, in addition to the usual blood tests (results as usual), I got a 24-hour urine test. This is really just looking for the same monoclonal proteins as the blood test, but is much more sensitive. It, too, failed to turn up anything. I also got a skeletal survey, which revealed little change since the last one I got, around a year ago. So everything is indicating stability at this point.

Recently it has seemed to me that the abdominal bloating has gotten worse, and I occasionally experience pains on the right side. Next week I am scheduled for an abdominal ultrasound, to check into this situation.

An Unexpected Complication

I seem to be specializing in Strange Maladies Of Indeterminate Origin. Last week, I visited my dentist for a routine cleaning and examination. I also got the annual full set of X-rays. The picture of one of the mandibular canines (tooth #27) caught the attention of the dentist, who forwarded it to an endodontist, who delivered a diagnosis of external root resorption. This is a poorly understood (especially by me) phenomenon, in which the tooth appears to be destroying itself from within, "apparently initiated by a peculiar inflammatory hyperplasia of the pulp", by one account. This could be an autoimmune disorder, but it is more commonly believed to be ultimately caused by trauma, where "trauma" is defined to include the wearing of orthodontic appliances -- which I did, back in the day. If the damage is not too advanced, the tooth can be rescued by means of a root canal; otherwise, it must be extracted and, nowadays, typically replaced by an implant. In my case, the endodontist would normally pass me off to an oral surgeon for extraction. But...

But I am receiving bisphosphonate therapy, to counteract the effects of the cancer on my spine. Normally, special cells called osteoclasts clean up old bone mass, making way for new bone created by the counterpart osteoblasts (a process known as "bone turnover"). Myeloma appears to overstimulate the osteoclasts, which begin to outperform the osteoblasts, creating many lytic lesions -- holes -- in the bone. This is obviously a Very Bad Thing, leading in my case to a greatly enhanced risk of spinal compression fractures. Bisphosphonates such as the one I am getting, Aredia, attempt to solve this problem by suppressing the osteoclasts, which is a mixed blessing, since bone turnover is as a result reduced everywhere, meaning that the bones are more brittle and are slower to heal than they should be. Apparently, this is especially a problem where surgical procedures performed on the jaw, such as a tooth extraction, are concerned, leading to a condition known as "bisphosphonate-related osteonecrosis of the jaw". The wounded jaw does not heal, and eventually the bone in the vicinity of the wound dies. This is another Very Bad Thing. So oral surgeons are understandably reluctant to perform extractions on patients receiving bisphosphonates.

The effect of bisphosphonates on bone turnover is typically gauged by a serum CTX test; patients getting bisphosphonates will show much lower than normal CTX numbers. A sufficiently lengthy drug holiday might return serum CTX to a range acceptable to an oral surgeon; a temporary root canal might be necessary to stave off disaster in the interim. The question is whether the oncologist can be persuaded to declare such a drug holiday. I was on track to continue receiving Aredia until at least the end of this year, for reasons of course completely unrelated to dentistry. My next visit with the oncologist should be much more interesting than usual, for this and certain other reasons, which I will discuss in due time.