Being really unique

Uniqueness isn't necessarily a good thing. Consider this tableau from an MGH exam room yesterday. On the right is my neurosurgeon. Centre is Yours Truly. On the left is a surgical resident. We are all looking--staring, really--at an MRI image of my brain. All of us are trying very hard to maintain clinical decorum, and not to say "what the FUCK?"

For those who haven't taken anatomy and physiology, I must explain that trigeminal nerves come in pairs, one set on the left and one on the right. This is why most people who get to entertain this disorder have it on one side or the other. Only a very unfortunate few have bilateral TN. Just as the experience of unilateral TN is beyond the imagination of most people, bilateral TN is beyond our ability to imagine.

Last Saturday's MRI was meant to determine whether my left side TN was indeed idiopathic, or whether it was the result of vascular compression of the primary trigeminal nerve. With the improvement of imaging and other diagnostic tools, the latter has been found to be the source of TN in the majority of cases. The neurologist was therefore confident that he would discover vascular compression of my left trigeminal nerve.

The MRI showed, instead, compression of my right trigeminal nerve. Exactly what this means is unclear, hence the WTF moment for surgeon, resident and patient. The patient is hopeful (desperately so) that what we have here is an instance of neurological gymnastics and the source of my left TN pain, however odd that might be, is the right vascular compression. The alternative is that bilateral TN is somewhere in my future, and that does not bear thinking about.

At any rate, the next step in the decision process is put off until January. The neurosurgeon initially wanted to see me in December, but I suggested January, because the odds that the Beast will be back in full cry are better then. So January it is. Umm, on Friday the 13th. Fortunately, I'm not superstitious, much.

Next steps

I began this dog and pony show around the time we were driving my kid's car across company to her clinical affiliation in California. Now it's a few years later, she's back here, and still in the brain business. My TN is sort of a hobby with her, and she noticed a posting where she works now about a new minimally invasive surgical treatment for TN. She spoke of it as "Botox." OK, logical, since migraine treatment with Botox now has a good deal of traction. As it turns out, Botox is just neurosurgical slang for phenol (or some other) injections of the trigeminal nerve, and the procedure has mechanical similarities to the Botox treatment for migraine. I understood that it wasn't perfect (what is with the Beast?) but could relieve dependency on a bagful of drugs, and didn't seem to come with the after-effects or lengthy recovery associated with other surgical treatment.

Eventually I ended up with the TN neurosurgeon, and went through my usual protocol with surgeons. They ask what I do. I say "medical coder and surgical auditor," and one foot comes out of the stirrup on their high horse. When this one asked how I had heard of his work, I said my kid's a doctoral fellow working with one of his colleagues, and both surgical feet came off the high horse: play the cards you have when gentling surgeons. If you can, show that you're in "the club." It's unfair, but, when they are patients, clinicians and allied health people are generally treated better and get more precise information than ordinary mortals.

At this facility, they have stopped the phenol injections because, to quote this surgeon, "we can't always tell where it will end up." One place it can end up is the heart, which then will stop for good. I would love to find a neurosurgeon who has TN. Then one wouldn't have to explain that that would also be a welcome treatment. One wouldn't have to explain that ending one's life is just another treatment option, so having it done for you is an improvement.

The Good News

My surgeon (cough) buddy has two favourite treatments. Today, with better imaging technology, it has become clear that vascular compression (an artery squeezing the trigeminal nerve root) is the cause of four TN cases out of five. With this in mind, microvascular decompression (or MVD: surgery to relieve the pressure on the nerve) has become much more sophisticated. If a new and better MRI indicates compression, he's ready to cut.

My first MRIs indicated that my TN is ideopathic: a condition without a clear cause. If I come away from the next MRI as ideopathic, then he is a fan of rhizotomy: a partial destruction of the trigeminal nerve root that effectively blocks the pain signals. I'm possibly a candidate for the first of these,  and definitely a candidate for the second. MVD has a 90 % success rate with a low rate of recurrence. Rhizotomy's numbers come under the next heading.

I'm picking this up after the latest MRI. As I've commented here in years past, no MRI is for the squeamishly claustrophobic, and a brain MRI is a notch or two up the scale. So far, my three MRIs have each had distinctive features. The first was like lying under a steel deck whilst someone was dumping 100 tons of scrap metal on it. The second was a decidedly hallucinogenic experience accompanied by sounds similar to post-modern music.

Props to MGH's North Shore Imaging unit. I've always met and chatted with the techs, but this time I got to meet and chat with the radiologist as well. I mentioned the silly advice I got before the first one, that I should bring along a CD of music that I liked. We all chuckled over that, and the tech said "sure. You can hold it."

This experience was well up the decibel scale, but this time, for sound, we had a blend of the heavy metal and the post-modern. My impression was that it was much more intense than either of the previous two. This may have been a hallucination, but I felt certain of being physically smacked around by the pulses at several points, as if my head was actually jolted from one side to the other.

I'll have to wait until Monday's E&M with the Neurosurgeon for the results. My medically naive wife thought I should get an autographed copy of the films. nah-uh. In the latest iteration of HIPAA, you have practically to qualify for a security clearance to walk around with your films. And, of course, they're not films at all, but digital images which go to the common Mass General database in real time.

The Bad News

One of the reasons that rhizotomy fell out of favour as a TN treatment is that patients, while they did get relief from the pleasures of the disorder, also lost most or all feeling in the treated side of the face. They also ran the risk of ending up looking like Tex Avery's Droopy.

MGM Cartoons

Understandably, many patients objected to this. In the modern history of treating TN, this problem has dogged patients and clinicians alike. Nothing can really be worse than the disease, but many treatments have left patients wondering what they let themselves in for.

Supposedly, we now have newer and friendlier facial rhizotomies, which don't leave one with sagging jowls. However, rhizotomies still are likely to leave numbness in their wake. Worse, one goes to the trouble and expense, only to have the Beast back in a few years, leaving the surgeon to do it all over again: the success rate is around 40 percent and there aren't warranties. All these things are the focus of my rhizotomy questions tomorrow.

MVDs are cranitomies: significant brain surgeries that carry numerous risks with them, no matter how well done. Even if everything goes flawlessly, I face a staggering recovery period:  no work, no driving, restrictions on practically everything (Did I mention no drinking?), stretching on for months. As I approach 70, I'm aware of how few months I have left to throw away.

I don't like it. One of the main ways, besides medication, that I currently attempt to contain The Beast is restrictions on what doctors euphemistically call "quality of life." I've had to give up winter sports, especially skiing. I can't eat anything more solid than stew. I can drink little to nothing. Hell, when my sickness is on, my trips outside consist of dashes from house to car to whatever building I must visit. MVD seems to trade one set of quality of life restrictions for another: a treatment that creates a dilemma is perhaps no treatment at all.

Tomorrow I see the neurosurgeon, and my PCP as soon after that as I can manage. Stay tuned.

Tomorrow has come and almost gone. I'm off the hook until January. More directly. That was a bundle of news for all concerned.