One in a Million

I experienced a life-altering event in 2008. It’s too complicated to include in a getting-to-know-you piece, and would likely keep me from going on Jeopardy! even should I someday pass their wretched test.

In 2002 I was diagnosed with a rare neurological condition called Hemifacial Spasm. HFS is caused by irritation to the seventh cranial nerve. This may be due to a tumor, multiple sclerosis, or as in my case, a blood vessel coming into contact with it and wearing away the myelin sheath. The irritation causes nerve impulses to backfire and the affected side of the face twitches, intermittently and uncontrollably. This is disfiguring, disconcerting and exhausting.

The estimated incidence of hemifacial spasm is one in 100,000. There are three treatment options: medication, Botox, and surgery.

The medications for HFS are also used for epilepsy, the theory being to calm nerve impulses. Unfortunately, drug therapy is notoriously ineffective in controlling hemifacial spasm. I tried three or four different medications. None gave more than slight relief and all had bothersome side effects. To be honest, one of them did cause me to lose a significant amount of weight, which was actually welcome, but it also caused my fingers and toes to go numb and my lips to turn purple. The one that was best in taking the edge off the spasms quite literally made me stupid. Mid-sentence I would be unable to come up with the next word. My neurologist tried to convince me that it due to the natural aging process, but I knew otherwise. For a writer, this was a very sorry state of affairs.

Most people know of Botox as a wrinkle reducer, but it also has therapeutic applications – for example, helping children with cerebral palsy deal with muscle spasticity. Technically a poison, Botulism toxin works by paralyzing a muscle so that it can’t react to the nerve impulses it receives. In the case of HFS patients, injections into affected muscles around the eye, cheek and mouth gives three months or so of relief from spasms, when properly administered. A little too much, though, and the patient will look like he or she had a stroke until the effects of the toxin wear off. Despite the drawbacks, many HFS patients are satisfied with Botox treatments, although over time the body develops a tolerance and the time between treatments will become shorter.

I was petrified of a bad experience, but once my spasms reached the point of being the first thing I felt in the morning and the last thing I experienced as I fell asleep at night, I decided to give Botox a shot (pun intended). On injection day, I expressed my fears to the neurologist. His response? I read too much and once I had the treatment I would be entirely satisfied and live happily ever after. Just before moving into the procedure room, he asked me to sign a waiver that I would not hold him responsible should any of those unfortunate incidents, or a few others I had not even thought of, occur. He refused to recognize the irony in this, even when pointed out.

Did you know that Botox shots really hurt? (Seriously – I now possess a grudging admiration for celebrities who subject themselves to periodic  punishment in the name of youth and beauty.) I got four or five injections around my eye, mainly in the upper lid, one in my cheek and one by my mouth. Then the doctor told me that I would see the effect in about two weeks and it should last for three months. The next afternoon I was reading a book when I noticed that my left eye was itchy. I went to the bathroom to check it, and discovered that I COULD NOT BLINK THAT EYE. So much for two weeks. Over the next few months the muscles that were injected did stay calm. However, the surrounding muscles twitched in their stead. One muscle in my forehead was so active that it gave me what to this day I fondly call my “Botox wrinkle.” On the advice of my ophthalmologist, who did not like what dry eye could do to my vision, I cancelled my three month follow-up.

After six years of intensifying spasms, brain surgery started looking better and better. I saw patient after patient on the web support group I belonged to report excellent results. Because HFS is a rare disorder, there are very few surgeons who specialize in the microvascular decompression (MVD) surgery to repair it. I selected a neurosurgeon, “Dr. C,” who had trained under the doctor who pioneered the surgery and had significant experience but wasn’t yet approaching retirement age. Miraculously, my insurance company approved my out-of-network request right away.

My surgery took place June 2, 2008. I awoke to a raging headache and extreme nausea and knew even before touching it that the left side of my face was paralyzed. I was also deaf in that ear. Dr. C reported that everything went reasonably well during surgery and he padded two arteries and half a vein before he felt he had to quit. He described my nerves as “unhappy” during the surgery but noted that the facial nerve was responding to stimulation during the entire procedure. Usually that is good news, but he somberly stated that he had a “bad feeling” about my facial nerve. He said that after three months I could consider a nerve graft, and recommended a surgeon at the University of Missouri if I wanted to stay closer to home.

At my pre-op consultation, Dr. C had told us that about one in ten patients comes out of the surgery with some degree of facial weakness or paralysis. At my  post-op exam, he added that about one in ten of that group sees no recovery from the paralysis. After four years, I estimate that about 65-70% of my facial function has returned. That’s the equivalent of “D-” on an exam, but on the bright side, I have regained “symmetry at rest,” meaning that impairment is not noticeable in a neutral expression. I can manage a small smile, but my former toothy grin is gone forever. My mouth looks funky when I talk, but I have discovered that one-on-one, people tend to focus on my eyes rather than my mouth. In fact, most people do not notice anything wrong. My blink function is not normal, but I can close my eye and even wink if I concentrate. I need to keep lubricating gel on my eye at all times, but I have adapted to the half-fuzzy vision. I am very thankful that I had LASIK surgery pre-MVD, because I would not be a suitable candidate now. I have recovered about 85% of my hearing and these days only notice the deficit in crowds and large spaces.

That is how I literally became one in a million. One in 100,000 for hemifacial spasm and one in ten for facial paralysis following surgery. I am thankful not to be the one in ten million who never recovers any facial function.

Romans 8:28 tells us that “in all things God works for the good of those who love Him.” At first I had a difficult time believing that anything good could come from facial paralysis, but I have been blessed to meet some wonderful people who share the same fate. Some, like me, had hemifacial spasm, while others were diagnosed with another rare neurological disorder called acoustic neuroma.

Next week I will introduce you to one of them.

3 Responses

  1. I am so sorry you have had to go through all of this, Sara, but I hope you know that you are so much more than this–intelligent, witty, kind, loving, talented–none of which has anything to do with your still-lovely face. Romans 8:28 will continue to work itself out in this situation.

  2. Good Lord Sara. What a harrowing experience. Some years back I had Bell’s Palsy. Nothing like your challenge of course, but I remember not wanting to even leave the house. Since I couldn’t blink my right eye, I had to keep it salved and covered with a patch. Drool would sometimes escape the right side of my mouth. This could all be characterized as a slight inconvenience compared to what you had to endure however. I’m glad you came out of it, perhaps a bit battleworn, but out of it nonetheless.

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