The Reality-Based Community

Some good news, and some bad news.

On the bad side: Two of my four volunteer donors have washed out of Donor Boot Camp, one for a pulmonary problem and the other for insufficient kidney function. Especially regrettable because one of them apparently was good immunological match with me. Still have two left; keeping my fingers crossed.

Also on the bad side: My cough isn’t going away, and my voice, which came back to some extent, is now coming and going more or less at random, with no clear trend. It tends to wear down over the course of a day, and any extended talking tends to bring on very intense coughing. There’s also some audible wheezing at bedtime,sometimes loud enough to keep me awake. The sore throat is mostly gone, and I’m no longer constantly chewing cough drops, but Dr. Sulica (the ENT specialist) isn’t making any promises about continued improvement. If the cough continues for another month, he’s going to order another chest X-ray; maybe the zithromycin didn’t cure the pneumonia, or maybe it came back. Or maybe, as he said, I’m simply now a guy with a cough.

All in all, I’m regretting the decision to go with radiation instead of surgery. (Though radiation had one big advantage: it was more or less certain to work, while there was some risk that the tumor would turn out to be unreachable surgically.) My understanding going in was that surgery would, and radiation would not, threaten permanent damage to the voice; that apparently was not the case. Dr. Sulica explained that radiation can damage the salivary glands, leaving thicker, “ropy” mucus. That’s the first I’ve heard about that.

Dr. Sulica scoped me again and verified that the tumor mass had disappeared; he will write the letter the kidney transplant folks wanted, saying I’m officially cancer-free.

Despite the cough, I’m basically comfortable. Energy seems to be returning to some extent; I walked to work (just about one mile) this week, without much strain. Traffic in New York guarantees frequent pauses at traffic lights, so that’s not really a mile of continuous walking, but it’s way more than I could have done even two weeks ago.Stairs are still a big challenge, a big problem if you’re as dependent as I am on NYC subways. I’m learning bus routes as an alternative, which is OK except at rush hour or for long distances.

The kidney transplant folks also wanted a cardiology workup before they’d clear me, so I had a trans-thorasic echocardiogram (quick and painless) followed by a visit with a cardiologist, Dr Tharaon. He was quite upbeat; the TTE showed nothing alarming, and he sees no impediment to surgery.

Dr. Tharaon also explained what a “left bundle-branch block” is; it means that two of the three nerves that drive heart function are out of action. (I thought I’d been told that it was a problem for reading EKGs but without functional significance.) Hethinks a pacemaker would help, and explained that installing one is a fairly trivial outpatient procedure; there’s some doubt whether my current function is bad enough to warrant one. We discussed the problem of theburden Entresto places on the kidneys, and he was impressed that I’d found a nephrologist flexible enough to all me to be prescribed any of it. Once the new kidney is in place, he’s convinced that I can be restored to full heart function.

I mentioned the mistake I’d made in continuing to take isosorbide dinitrate and hydralazine the first day I was on the Entresto, and how great it made me feel. Dr. Tharaon said that made perfect sense, because those two work on mechanisms different from the two drugs that make up Entresto, and the gains are more or less additive. So he wrote me a new prescription for those two drugs, but told me to hold off on actually taking them until he’d checked back in with Dr. Weiss, who had taken me off them when I went on the Entresto. He did so, and sent a note saying that Dr. Weiss had ruled against going back on isosorbide and hydralazine and instead wanted to double my dose of carvedilol (to 6.25 mg. twice a day). No explanation about why; I’ll have to ask Dr. Weiss why the next time I see him.

The only other hurdle I need to jump before the transplant –other than finding a donor –is a colonoscopy (yech!). Apparently the rule is that you need one no more than 10 years old before a transplant, because if there’s any cancer present the immunosuppressives will set it loose. Just my bad luck what my last colonoscopy –which at the time I swore would be my lastcolonoscopy (it’s easily the most uncomfortable and disgusting test I’ve ever suffered through) turns out to have been in 2006. I’m tempted to get the report from UCLA and use White-Out to change the “6” to a “9,” but I doubt I’d get away with it.

My appetite seems also to be back a little bit, which is no doubt a good sign but definitely not what I want right now. Apparently,the less fat I carry into the transplant, the less fat they have to cut through and the quicker the healing process, so I’m hoping to lose some additional weight, and a restored appetite –coupled with lack of stamina for exercise –isn’t going to make that any easier.

Today was my monthly appointment with Dr. Bomback, after going in for labs yesterday. Mostly good news:

-Creatinine is back down to 5.6, which means it’s been roughly stable for about three months after jumping this summer.

-C-cystine, which is the more relevant reading in my case, will take a week to come in.

Nonetheless, the course of the disease (IgA nephropathy) is not predictable, and Dr. Bomback wants to push the transplant process as fast as it will go, to ensure against my having to go on dialysis before the transplant.

-Blood pressure (averaged over three readings) about 120/80 (I didn’t write down the numbers. At Susie Lew’s suggestion, I brought my home BP cuff to calibrate it, and it came in right about on target.

-Yes, the hydrobromide in DXM hydrobromide would have been expected to spike the blood pressure.

-No, he really doesn’t want to take the risk of a higher dose of Entresto.

-The 12.5 mg. of hydrochlorothiazide (a diuretic) probably isn’t doing any good given the state of my kidney function, so Dr. Bomback took me off that and switched to another diuretic (the name of which I’ve forgotten) that works on a different principle. In addition to keeping the BP down, this might help some with heart function if the heart muscle has been retaining fluid.

-In addition to the cardiac insufficiency, I’m a little bit anemic: hemoglobin of 10.6, where my normal was about 13. Nothing to be done about it; even if the insurance company would pay for blood growth factors (which it won’t at this level) there are disputed but possibly significant side effects: increased risk of cancer recurrence, increased risk of stroke.

-He has no clue what sort of cardiac workup the transplant folks think they need, after the angiogram; I’ll discuss that with the transplant team next week.

-Yes,as Susie suggested, my magnesium is high andI need to knock off the magnesium supplement. And I shouldn’t go back on to zinc. If I start to cramp again, the prescription is raw almonds.

-On the other hand, I should boost my daily 1000 units of Vitamin D to 2000 units; something about parathyroid function I didn’t quite get.

-Losing 30 pounds was good (I weighed in at 229). Losing more would be better; the lighter I am when I get the transplant, the quicker the recovery.-I should expect to be pretty much out of action for eight weeks post-transplant, though people have been known to go back to work after four. (Fortunately, I can do most of my work from home, assuming I can sit up.)

Next week I go in to the transplant unit to get a blood draw for a cross-match with my potential donor. Fingers crossed.

When Dr. Sulica said that my cough hadn’t been a result of the carcinoma and therefore might not go away, it occurred to me to ask him whether it might be worthwhile to try dextromethorphan. He said there was no reason not to, and that shutting down the cough temporarily might break the cycle of irritation-cough-irritation. Since the liquids taste foul and seem to induce coughing due to the coating they create on the lining of the throat, I ordered a bunch of gel-caps (of DXM hydrobromide)on line, and started taking one 15-mg capsule twice a day. (The bottle says two capsules every 6-8 hours, so I’m at the bottom of the dosage range.) Of course with the combination of healing-with-time and the placebo effect, it’s hard to judge, but the stuff seemsto be highly effective; I’m far less dependent on cough drops, and coughing far less often (really, not at all during the day, and much less at night, though I have been blowing my nose a lot).On the other hand, my physical stamina seems to have gone down, and my blood pressure up.

(BP just came in at 164/87; average since I started on the DXM has been something like 145/85. Hard to interpret because my pressure as measured in a medical office is consistently much lower than my pressure measured at home, but these readings are definitely higher than my home readings had been).

I have no idea how to think about the tradeoff; I think I’ll give it another couple of days and then cut back to one a day, unless Dr. Bomback (the nephrologist) says otherwise when I see him Tuesday.Losing the cough would be a big deal.

Heard from the kidney-transplant folks. They want a colonoscopy (yech!) unless I’ve had one within the last 10 years; I just checked, and my last one (which I swore would be the last one) was 12 years ago. They also want another cardiac work-up, and a letter from either Dr. Sulica or Dr. Sanfilippo that the carcinoma is gone. I also have to come in for additional bloodwork; apparently the question of whether the donor and I are really a match can’t be answered without some sort of test involving both my blood and hers.

Will also get a blood draw Monday in preparation for the appointment Tuesday; that will help guide the decision about how quickly to get the transplant. I’m inclined to push it as fast as the transplant folks are willing to move.

A little bit of medico-economic comedy, just for relief:

-One of the things I’m supposed to do before the transplant is get all of my vaccinations: not just flu, but the second round of the shingles vaccine, pneumonia, and tetanus/diphtheria/pertussis. The theory is that I won’t be able to tolerate live-virus vaccines in the year I’m immune-suppressed. Dr. Weiss’s office had the flu vaccine on hand, but sent me downstairs to the pharmacy to get the TDAP. The pharmacy reported that my (otherwise pretty good) United Health Group insurance explicitly excluded the TDAP vaccine: that will be $96, please.

So I went to CVS, where they said it would be $60. But when I pulled a long face and indicated that I’d go scream at United, they suddenly decided that there was “a coupon” that would reduce the price to $46. The difference between that and what I thought of as the top copayment through UHG of $35 didn’t seem worth worrying about, so I bought it; it turned out that CVS had someone ready to give the injection, so I didn’t even have to go back to the doctor.

I’m still trying to figure out the logic of an insurance company not wanting to pay for what appears to be a routine vaccination to prevent what must be very expensive conditions.

-My other business at the CVS was to pick up the second month’s supply of Entresto, the fancy new heart med. When they printed out the paperwork, the co-pay –which was $35 the previous month –was somehow $75 instead. Apparently the stuff is so ferociously expensive (list price is about $500/mo.: weirdly, that price seems to be independent of dosage) that UHG slapped on a high co-pay, hoping to drive patients to various off-patent medicines that don’t cost as much. Having to pay out of pocket $900 per year for a medicine I can expect to be on for the rest of my life seemed like moderately bad news.

-But wait! Entresto, too, had “a coupon.” Except this was one I had to sign up for and print on line. (My AT&T cell phone can’t get service on Fifth Avenue, a block from the Empire State Building.) So I went home and got the coupon printed out; it magically makes the co-pay $10 per refill, irrespective of whether the refill is for 30 days or 90 days. I guess what’s happening is that the pharma outfits and the pharmacy benefit managers are playing offense/defense games: pharma ludicrously over-charges for on-patent drugs, the PBMs defend themselves with absurd co-pays to discourage patients from filling those scripts, and pharma retaliates by absorbing most of the co-pay.

Even if all the medical care I’m getting gives me a normal life expectancy, I still don’t expect to live long enough to see a sensible drug-pricing system, with the government supporting drug R&D through some combination of grants and prizes for invention, and patients paying something close to long-run average marginal cost.

Never a dull moment. Saw Dr. Sulica, and learned many things:

-Yes, the dubious region in that X-ray is new. Likely a pneumonia, caused either by a gram-negative bacterium or an anaerobe. Fortunately, zithromycin is broad-spectrum and excreted in bile, so it puts no burden on the kidney. Will start on that tomorrow.

-No, the cough probably wasn’t anything to do with the carcinoma, even though the carcinoma was discovered looking for a cause of the cough. Maybe it’s continued irritation of the throat by the radiation, though that seems to have died down. Maybe it’s the pneumonia, but probably not. Maybe it’s self-reinforcing; no reason not to try dextromethorphan (that was my suggestion) to see if that breaks the cycle. Failing that, yes, we might even try Advair. But it’s quite possible that I’m simply “a guy with a cough,” with nothing to be done about it. (Still doesn’t answer why I’ve gone from a normal-sounding cough to a sick-sounding one.)

-Yes, the voice might continue to recover. Or it might not. As a result of the radiation, there’s now a little bit of webbing between the working vocal fold and the paralyzed one. (The tumor was right in the crotch between them.) That means they can’t close up all the way. Surgery would have had the same side-effect. No, there’s basically nothing to do about it; I’m going to have whatever voice I have. Since Dr. Sulica is famous for fixing voices, I have to regard that as a definitive judgment. (Someone told me she found my current voice sexy; we’ll see how that works out, but my past performance isn’t encouraging.)

-Yes, he’ll write a letter clearing me for a kidney transplant. He wants to see me once a month for six months; he estimates the recurrence risk at well under 10%, but it’s not zero. For now, as predicted, the tumor mass is gone.

Dr. Sulica is fairly convinced that both the carcinoma and the heart disease are delayed-onset side-effects of the radiation treatment I had for Hodgkins Disease eighteen years ago. That treatment was described to me at the time as “a little bit of consolidation radiation treatment” to put the last nail in the coffin of the HD after six rounds of enhanced BEACOPP had killed it. That makes me wonder how much my risk of having the HD come back (estimated at the time at around 10-12%) would have gone up if I’d skipped the radiation. What I’m sure of is that my oncologist at the time –who was very good about consulting me on treatment decisions, including whether to use standard or enhanced-dose BEACOPP, treated the radiation as a fixed point, and that no one said “This could do nasty things to you two decades from now.” As Dr. Sulica pointed out to me when I was choosing between radiation and surgery this time, radiation oncologists never see the delayed side-effects of their treatments; no one at UCLA is going to find out about my carcinoma or my heart disease. Is radiation over-used as a consequence?

Saw Dr. Sanfilippo (the radiation oncologist) and his team. They weren’t surprised that the voice was coming back slowly, but they were surprised and worried by the persistent productive cough. Recommended a chest X-ray to rule out an infectious bronchitis or pneumonia. Did that; film came back ambiguous. I’m prepared to wager it’s the same radiation scarring that showed up on the X-ray three months ago, but I’ll ask Dr. Sulica to check when I see him Thursday. Dr. Sanfilippo suggested that the cough might be self-reinforcing through continued irritation of the throat, which certainly seems to be what’s happening. If that’s the case, than an asthma inhaler such as Advair might suppress the irritation long enough to suppress the cough long enough to relieve the irritation. Again, a question for Dr. Sulica.

The voice should continue to improve. In the long run, I now learn, it might be a little bit deeper, which would definitely be an improvement. It also might be a little bit softer, but I’ve always had volume to spare.

Yes, Dr. Sanfilippo will write a letter clearing me for the transplant. It’s close to certain that this tumor is gone, but there is some risk (10%?) of a recurrence over the next couple of years, so I’ll have to see him and Dr. Sulica every few months just to get checked out.