Author: Mark Kleiman

Did I say “boring”? Well ….

1. The X-ray taken the same time the bad blood test was taken showed fluid accumulation in the right lung, with the lung itself partially collapsed. That may explain the lack of exercise tolerance. (And would be a more favorable explanation than worsening heart disease.) Next step is a CT scan tomorrow. Step after that is having the fluid removed with a needle (a procedure than can be done under local) and the lung re-inflated (not sure how that works or how stressful it will be).

2. Still not word from the Columbia lab about their screw-up.

3. A funny thing happened to me on the way to the transplant. The colonoscopy report was fine, though I’m having a devil of a time getting it transmitted to the transplant team; the various physicians’ offices seem incapable of communicating with one another except through me, and the full report with the biopsy results (seven adenomatous sessile polyps, largest 12mm, the rest all under 8mm, none cancerous) was sent to me by snail-mail. Since I don’t have a fax machine,Dr. Sulica’s office was good enough to fax it to Nikki Lawson, the transplant co-ordinator, this afternoon, but as of 6pm she report that she hasn’t seen it.

But that’s just an annoyance. The problem is that the report from the radiation oncologist –which I’d been told would be “He had a completely curable cancer and we gave him the definitive treatment for it” –turned out to be more complicated, and less encouraging, than that. The recurrence risk for the cancer I had (a squamous cell carcinoma of the glottis) is about 6-8%. (All I’d been told was “less than 10%.”) That doesn’t sound so bad. But what I didn’t know is that the consequences of recurrence would be very bad indeed. I’ve already maxed out on radiation, and the paralyzed vocal fold means that the only surgical option would be a complete laryngectomy, with a mortality risk of about 30%. And of course even if it worked I’d have no voice box left. Apparently there’s a way to redirect the breath to give me some simulacrum of a voice, but the whole thing sounds pretty damned grim.

The good news is that we’re now almost 6 months out from the radiation treatment, so about half of that 6-8% risk should be behind me. In addition, Dr. Sulica, who examined me today, reports that the tumor site looks entirely clean and that the residual radiation damage is less than might have been expected; both of those findings seem to him like favorable prognostics. In any case, I’ve faced worse odds of worse outcomes before.

The bad news is that the 6-8% figure doesn’t allow for any additional risk due to immunosuppression. How big is that risk? No one has a clue. My cancer was non-standard (most people who get it are smokers, while mine is probably the side-effect of the radiation I had twenty years ago), and the combination of this particular cancer caused by radiation with kidney failure is sufficiently rare that there’s simply no relevant research. (“The n would be very small,” said Dr. Sulica.)

So that leaves the transplant team guessing. They can wait until the recurrence risk goes down even more, but then I’m likely to need dialysis before they can do the transplant, which substantially worsens transplant outcomes. (I’d also have fairly debilitating heart disease in the meantime, because the kidney problem means that I can only get about half the indicated dose of the heart medication.In addition, my sister’s life is on hold until she knows whether and when she’s flying to New York and being out of action for at least a week.) Or they can choose to roll the dice.

I’m pretty sure I know what I want them to do, but I don’t have a clue about what they’re actually going to do.(Except that Dr. Montgomery, the one time I met him, boasted about how aggressive he is in doing transplants others shy away from.) Apparently the whole thing is run on the principles of a firing squad, to make sure no individual feels responsible for anything. The decision is made at a meeting of 10 M.D.’s (five transplant surgeons and five medical nephrologists). The meeting happens Thursday, and I’m promised results as soon as it’s over. You’ll know when I know.

It’s been a month since my last update. My only excuse is that everything in between has been boring. For reasons not clear, my exercise tolerance has declined –even walking a couple of blocks is tiring –so I’ve mostly been staying home and getting a gratifying amount of work out the door.

There was one bit of comedy last week. I went in for routine bloodwork Wednesday: Dr. Weiss wanted to consider adjusting my dose of diuretic but wanted to see the numbers first. (He also wanted a follow-up chest X-ray to see if the pneumonia was lingering.)

Just before midnight, the phone rang. It was the lab. My hemoglobin, which had been 11.5 (slightly anemic, but not enough to warrant intervention) in January, hadplunged to 5.4, which apparently is a life-threatening level. The guy from the lab wanted me to head straight to the nearest E.D. for a transfusion, but since I had no symptoms other than the limited stamina –wasn’t pale, or feeling week or dizzy –I decided (with Gary Emmett’s advice, given from Egypt where he was on vacation) to let it ride until Thursday afternoon, when I had to go in to Weill Cornell for a routine visit with my radiation oncologist, saving me a trip.I sent an urgent email to Dr. Weiss to make sure he approved, but didn’t hear back. Thursday morning the phone rang again. This time it was Dr. Bomback, the nephrologist. He’d seen the same results and was extremely concerned, saying that at that level of anemia I was at severe risk of a heart attack or a stroke. He reluctantly agreed that a transfusion could wait until the afternoon.

So I did the work I needed to do in the morning and then headed over to Weill Cornell. Saw Dr. Sanfilippo and told him that my throat was still scratchy, myvoice still weak, and my cough still persistent. All he could say was “Too bad.” Since if there’s a recurrence of the cancer Dr. Sulica (the ENT) would be able to spot it, it’s not clear what the purpose is of seeing Dr. Sanfilippo. In any case, he pointed me to the E.D.

Headed there. E.D. was less chaotic than I might have expected. I was interviewed and examined by various folks, all of whom seemed puzzled that someone with a hemoglobin of 5.4 wasn’t obviously sick. I was obviously going to need a transfusion, and maybe more than one unit, so they decided to admit me overnight for observation, which I had not been ready for and had not packed for. Fortunately, Richard bailed me out by grabbing what I needed for an overnight from home, including my meds.

Just to make sure, the E.D. folks repeated the blood test. They then put me on a gurney, next to an incessantly bonging heart monitor, to await the results and the transfusion to follow.

We’re now about four hours (and a not-so-bad hospital dinner) into the story. At this point of of the several M.D.’s who saw me came back and said, “We took two separate blood samples and tested them both. Hemoglobin came in at 10.4 from one sample and 10.5 from the other. The result from Columbia must have been a mistake. We’re sending you home.”

Question: Did someone at Weill Cornell call the Columbia lab and tell them they’d screwed up badly? I’m prepared to be the answer is no.

At that point I was very glad that Richard’s CARE package included some books, because actually getting me out the door took another two hours.My blood pressure and heart-rate, which had been normal when I walked in, were both elevated, and the nurse who took my vitals was concerned; I attributed those effects to having to listen to the heart monitor. (For some reason, hospitals consider earplugs a luxury item.)

So I went home. Friday morning I called Dr. Weiss’s office again and left a message for him to call me. I’m still waiting for that call, but today I got a call from the Columbia diagnostic radiology department asking when I wanted to come in for my CAT scan (first I’d heard of it). Presumably that means Dr. Weiss didn’t like the look of the X-ray. Left a third message for him. If he ever calls me back, I’ll know more.

And then the good news. My sister called from Chicago. After weeks of fooling around and lots of repeated tests, the transplant team has cleared her as a donor. Apparently whether or not she’s a match for me is the call of the transplant team rather than the donor team,but if not the problem can be finessed via a “daisy chain” in which volunteer-recipient pairs who don’t match are shuffledinwith other non-matching pairsuntil every patient matches with some donor.

There’s no great urgency –my creatinine has crept up a little, but it’s still below 7 –but on the other hand sooner is probably better because until the transplant is done they can’t really treat the heart adequately. I’m hoping –how realistically I don’t now know –for later this month. Keep your fingers crossed.

The good news is that I’m now cleared for a transplant, after a –yecccchhhh –colonoscopy. They found eleven polyps, the largest 12mm (in the mid-sigmoid colon), another 8mm, and the rest 4-6 mm. Biopsies should be back tomorrow. Given the number of polyps, Dr. Rubin wants to see me in a year to decide whether I need another colonoscopy. Here’s the full report.

Even better news: one of my potential donors passed initial screening and will be coming to NY a week from now for further testing, including a test where a donor blood sample is mixed with a sample of my blood to see what happens. (This sounds seriously weird. Does the person doing the test have to recite a spell? Do the samples have to be stirred counterclockwise and left-handed?)

In addition, there’s a new back-up volunteer. So I’m optimistic that someone will make it through the process.

The not-so-good news is that my exercise capacity, which had been expanding, has contracted again –the mile walk I used to be able to do is now out of reach, and even a couple of blocks is an effort. On two occasions it’s been hard to breathe deeply lying down at night. In each case a dose of isosorbide dinitrate made the problem go away. On two other occasions moderately vigorous activity left me gasping for breath for a couple of hours,with a blood pressure around 165/95 and a pulse around 88. I’m now on 30mg of that a day, and have also gone from 25mg of hydralazine once a day up to twice a day. If things don’t improve soon I’ll go back to Dr. Weiss.

When I saw Dr. Sulica a couple of weeks ago, I complained about a persistent runny nose, and he prescribed ipropropium bromide .03% nasal spray (23 micrograms of the active agent twice a day in each nostril) which handled the problem nicely.

I still have a nasty, productive cough, which may be a lingering pneumonia. A second chest X-ray showed some remaining mass in the lung but because the X-rays were done in different facilities there’s apparently no way of having them read together to measure improvement. Dr. Weiss wants a third X-ray in a couple of weeks. In the meantime, my voice has stopped improving; I’m audible but hoarse and ragged, and if I talk too much the voice starts to go.

My appetite has returned somewhat, which is presumably a good sign but not a good thing; I’m up a dozen pounds from my low of 227. I’m now being more deliberate about restricting my intake, in hopes of getting back below 230 before the surgery. Dr. Bomback reports that my potassium is borderline, so I’m watching that as well. Unfortunately, that means no more grapefruit, which I think helped with the weight loss.

Creatinine is back up to 6.2; that’s about where it was four months ago, but Dr. Bomback wants the transplant done as soon as possible.

The colonoscopy had its comic elements. Dr. Weiss had referred me to Dr. Moshe Rubin, who is apparently a star in that field. When I called to make the appointment, I’m pretty sure I said it was to get cleared for a kidney transplant, but Dr. Rubin’s office staff didn’t pick up on the fact that I was a complicated case, and apparently Dr. Rubin hadn’t reviewed the chart until I was actually on the table. He expressed some annoyance that no one had warned him about the complexity; he would have wanted to meet with me and examine me first. But he decided the procedure would be reasonably safe, and went ahead.

Instead of the fentanyl/Versed combination I’ve had before, the anaesthesiologist at the colonoscopy center used a sedative called propofol (190 mg.) Either the stuff is very fast-acting or it causes some amnesia, because the last thing I remember before waking up was the anaesthesiologist saying “We’re going to start running the sedative now.”

In the conversation when Dr. Rubin was trying to figure out whether I was in good enough shape to tolerate the procedure, I seem to have given him the –correct –impression that I was both interested in learning about the technical details and unlikely to freak out. As a result, about midway through the process the propofol was discontinued, and when I awoke I was looking at a screen with various images of my large intestine. Dr. Rubin started to explain what he was doing and what he was finding, but with the instrument still in me I was too uncomfortable to pay much attention. When I said so, they put me under again until it was all over.

As usual, the prep was the worst part of the process, and even that wasn’t nearly as bad as it was a dozen years ago; there’s a new prep medicine called Clenpiq, taken in only two doses starting at 6pm the evening before the procedure, after a single day on clear fluids only. It didn’t even taste especially awful. Recovery was reasonably rapid,and I didn’t need any medication afterwards.

Well, at least it’s never dull. I’ll try to be more diligent about keeping you updated.

In the meantime, everything else in my life is going very well. I’m getting some work out the door, and if the quality has declined I haven’t noticed it and everyone else has been too polite to mention it. I think I’ve persuaded a star Ph.D. student about to finish up at the RAND graduate school to join the Marron/BOTEC team; I’ve known him for a long time, and I’m confident that if I’m out of action he can pick up the slack.

A friend writes to remind me that I haven’t updated here in a couple of weeks.

Don’t worry: In this case, no news is good news.

On some dimensions I’m better: in particular, my voice is near normal (just slightly hoarse) at times, though it comes and goes, and I’m limited in how much volume I can produce. Physical energy seems slightly improved: I’ve now walked the mile home from work (slightly uphill) three times, which I certainly couldn’t have done even three weeks ago.

It turns out that Dr. Weiss and Dr. Tharaon didn’t actually disagree about my going back on isosorbide and hydrazaline; Dr. Weiss got the impression that Dr. Tharaon was recommending that instead of Entresto rather than in addition to it. He has no objection to the combination.

I tried it once, about a week ago, with bad results: half an hour after taking I felt dazed and sleepy, and headed right to bed in the middle of the day. Today I’m experimenting with a single dose of the isosorbide; I’m tolerating it well, and (with a hefty dose of hot chocolate) it does seem to have made me somewhat more energetic. I’ll try one dose a day for a while to see how it goes.

The cough is still annoying, but a little big less frequent than before; still productive, though. If it doesn’t improve, we’ll do another chest X-ray to make sure the Zithromax killed the pneumonia.

Big problem now is sleep: occasionally I wake up in the middle of the night and can’t get back to sleep for a couple of hours. Even when that doesn’t happen, and even if I go to bed before midnight, I’m sleeping extremely late. Today, for example, I woke up at 10, still felt drowsy, turned over and went back to sleep, didn’t wake up until 1 p.m. Thursday I didn’t bother to set an alarm because my first meeting wasn’t until 1:30, and was started when a phone call woke me up at 12:45.

Scheduled for a (yeeechh!!) colonoscopy toward the end of the month, which is the last test I need to get cleared for a transplant. Just got the instructions in the mail, and it appears that the process is significantly less annoying than it was the last time I did it: first dose of that awful sludge you have to drink isn’t until 6pm the evening before the test. Good news: at my age, this is the last routine colonoscopy; the guideline is one every 10 years, but not after 75.

Another potential donor has emerged, which gets the candidate pool back to three. Still hoping to get the job done in February, but nothing is certain.Still cheerful, comfortable, and reasonably productive. More when I know more. If there’s any bad news, I’ll make sure it gets posted here, so take silence as encouraging.

Cough may be improving a little bit; using a phenolspray before bedtime seems to help.

Below are the results from the echocardiogram. EF is actually 35% rather than 40%,where anything over 55% is normal.

Several other findings sound sorta scary:

-severe mitral annular calcification

-small pericardial perfusion

-paradoxical septal motion

but I have no clear idea what they mean.

Study Result

Impression

CONCLUSION:

-There is no left atrial dilatation (LA volume index 28 ml/m²).

-The interventricular septum is mildly hypertrophied.

-The left ventricle has normal end-diastolic diameter.

-There is paradoxical septal motion. The basal and mid anterior septum, entire septum, and entire inferior wall are hypokinetic. Contractility of all remaining LV segments is normal.

-LV ejection fraction is moderately decreased (35 %).-There is no right atrial dilatation. The right ventricle is normal in size. The right ventricle has normal wall motion.

-There is aortic valve thickening. There is mild aortic stenosis. The aortic valve area, by the continuity equation, is 1.53 cm². The aortic valve area index is 0.7 cm²/m². There is mild aortic regurgitation. Aortic valve gradient is early peaking.

-There is severe mitral annular calcification. There is mitral valve thickening. There is moderate mitral regurgitation. There is MAC with a mitral valve gradient of 5mmHg at a heart rate of 77 bpm.

-The right atrial pressure is normal (0 -5 mm Hg). There is too little tricuspid regurgitation to estimate PA systolic pressure.

-There is a small pericardial effusion. There is a small pericardial effusion adjacent to the RA and RV without chamber collapse.

-No prior study available for comparison.

Narrative

NYU Langone Medical CenterAdult Echocardiography Laboratory560 First Avenue, New York, NY 10016Tel: 212-263-5664 Fax: 212-263-84612D Transthoracic Echo Report——————————————————————————-Pt. Name: MR. MARK A. KLEIMAN Study Date: 12/11/2018 Pt. ID: 13733272 Accession #: 16505925DOB: 5/18/1951 Ref. Physician: 1245564079 VASISHTA TATAPUDI Age/Gender: 67 M Sonographer: AM Height: 185 cm (73in) Fellow: Rebecca Pinnelas MD Weight: 111 kg (244 lb) BSA: 2.34 m²Location: Tisch NIC EPIC Code: ECH10-O—-

Indication: Preoperative cardiovascular exam

MEASUREMENTS:

Value Normal

Aortic 3.5 cm <4.4 cm LVOT 2.0 cm

Root Diameter LA 4.8 cm <3.8 (<4.0)

LVOT Area 3.1 cm² Diameter

LVOT Stroke 60 ml (25LA Vol 28 ml/m² <34 Volume ml/m²)

Index LVOTVmax 0.9 m/s— (rest) LVOTdPmax (rest)

IV Septum 1.3 cm <1.1 (<1.2)

LVOT V max

LVEDD 5.2 cm <5.3 (<6.0)

(Vals)Inf-Lat 1.1 cm <1.1 (<1.2)

LVOT dPmaxWall (Vals)LVESD 4.1 cm —-AV Vmax 1.9 m/s 1.0-1.7 LVED Vol 52 ml/m² <75

AV PeakIndex GradientLV Mass 107 g/m² <95 (<115)

AV MeanIndex Gradient——

AV Area 2-4 Impedance <3.5(Zva)

LVEF 35 % 50-70%

Aortic 453 msec——-Regurge P1/2——-

RAP, mean 3 mmHg 0-5PASP <35PADP <15 MV E wave 1.0 m/s 0.6-1.3RV-RA

VmaxPA-RV MV Mean 5 mmHgLA, mean <12 Gradient——-

MV Area 4-6——-

Mitral E 97 cm/sMitral A 144 cm/s TV E wave 0.3-0.7Mitral 0.7 VmaxE/A TV MeanDecel 188 msec GradientTi—-

Mitral P 55 msec1/2E’ 7 cm/s >8 RVOT(medial) DiameterE’ >8 RVOT Area (lateral) RVOT StrokeE/E’ 13.8 <8 VolumePV S/D RVOT Vmax

Normal values in parentheses are specific for men; normal aortic root values adjusted for age and BSA. LAVI data reflect 2015 ASE guidelines. PV Vmax 1.2 m/s 0.6-0.9PV PeakGradient—-BP 160/79mmHgHR 76 bpm

TECHNIQUE:Complete 2D transthoracic echocardiogram with color and spectral Doppler was performed. Study quality was good.

FINDINGS:

Left Heart:

-There is no left atrial dilatation (LA volume index 28 ml/m²).

-There is paradoxical septal motion. The basal and mid anterior septum, entire septum, and entire inferior wall are hypokinetic. Contractility of all remaining LV segments is normal. LV ejection fraction is moderately decreased (35 %).

Legend: 0=not seen; 1=normal; 2=hypokinetic; 3=akinetic; 4=dyskinetic;5=aneurysmal

Mitral Valve:

-There is mitral valve thickening. There is severe mitral annular calcification. There is moderate mitral regurgitation. There is MAC with a mitral valve gradient of 5mmHg at a heart rate of 77 bpm.

Aortic Valve:

-There is aortic valve thickening. There is mild aortic stenosis. The dimensionless index is 0.47. The aortic valve area, by the continuity equation, is 1.53 cm². The aortic valve area index is 0.7 cm²/m². There is mild aortic regurgitation. Aorticvalve gradient is early peaking.

Aorta:

-The aortic root is normal in size.Right Heart and Systemic Veins:

-There is no right atrial dilatation.

-The right ventricle is normal in size. The right ventricle has normal wall motion.

-The right atrial pressure is normal (0 -5 mm Hg). There is too little tricuspid regurgitation to estimate PA systolic pressure.

Tricuspid Valve:

-There is trace tricuspid regurgitation. Pulmonic Valve:

-There is trace pulmonic regurgitation.Pericardium and Effusions:

-There is a small pericardial effusion. There is a small pericardial effusion adjacent to the RA and RV without chamber collapse.-

CONCLUSION:

-There is no left atrial dilatation (LA volume index 28 ml/m²).

-The interventricular septum is mildly hypertrophied.

-The left ventricle has normal end-diastolic diameter.

-There is paradoxical septal motion. The basal and mid anterior septum, entire septum, and entire inferior wall are hypokinetic. Contractility of all remaining LV segments is normal.

-LV ejection fraction is moderately decreased (35 %).

-There is no right atrial dilatation. The right ventricle is normal in size. The right ventricle has normal wall motion.

-There is aortic valve thickening. There is mild aortic stenosis. The aortic valve area, by the continuity equation, is 1.53 cm². The aortic valve area index is 0.7 cm²/m². There is mild aortic regurgitation. Aortic valve gradient is early peaking.

-There is severe mitral annular calcification. There is mitral valve thickening. There is moderate mitral regurgitation. There is MAC with a mitral valve gradient of 5mmHg at a heart rate of 77 bpm.

-The right atrial pressure is normal (0 -5 mm Hg). There is too little tricuspid regurgitation to estimate PA systolic pressure.

-There is a small pericardial effusion. There is a small pericardial effusion adjacent to the RA and RV without chamber collapse.

-No prior study available for comparison.

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?