For the past 35 years, a relentless neurological disorder has taken over my body, causing often-painful muscle spasms that make it hard for me to walk and write and that cause my speech to be garbled enough that people often can’t understand me.
I can live with my bad luck in getting this condition, which showed up when I was 10; what’s harder to accept is that I have passed on this disorder, carried in my genes, to my 11-year-old son, Liam.
As a parent, you hope that your child’s life will follow an upward trend, one of emotional and physical growth toward an adulthood of wide-open possibilities where they can explore the world, challenge themselves emotionally and physically, and perhaps play on a sports team. And you hope that you can pass down to your child at least some of what was passed down to you. Yet my generalized dystonia, as my progressive condition is called, was one thing I had hoped would end with me.
When my wife and I started thinking of having kids, the statistics were fairly reassuring: There was a 1-in-2 chance that our child would inherit the gene that causes the disorder, but most people who have the gene don’t go on to manifest dystonia. We wanted a family and rolled the dice — twice. Our daughter does not have the gene.
Like any loving parent, I don’t regret anything about who my child was born to be. Dystonia has been cruel enough to me that I’m angry only at the injustice that Liam will have to relive this struggle in his own way. He sometimes asks, “If you had known, would you have had me?” My heart breaks 80 million ways that he would even ask that.
My heart also breaks when I see his foot brace lined up with our family shoes in the front hall. He wears it now to deal with the creeping effects of the condition. Just as with me, his symptoms began to show at age 10. Liam can no longer balance on his left foot or stand on his toes. He has difficulty writing and playing the violin. His symptoms — his falls, his need to hop at the end of the day because of muscle fatigue and cramping — will continue to worsen.
Life becomes a series of losses with any progressive disorder, and these small deaths are especially hard to witness in a child who has just moved into middle school with 800 other kids. He will have to both advocate for himself and educate his new teachers and peers about this genetic disorder. Although I never limped the way Liam now does, I stood out when my arm tightened oddly while writing. A few older kids, the crueller ones, would imitate my arms and speech and act as if I were cognitively delayed, too. Will ignorant classmates give our son a hard time when he drags his toes and is already asking for a cane?
Most people who have dystonia take medications to help them slow the condition and improve their symptoms; I’m on three of these drugs. The side effects are dizziness, slower reflexes and loss of balance. Not the best cocktail if you want to drive or keep your liver and kidneys clean, but they do help me. But because my son has dystonia, I’m looking at another treatment, riskier but with the possibility of great reward, a treatment that involves implanting electrodes deep in my brain.
I had planned on waiting another five years to try deep-brain stimulation, or DBS. I had felt that life could be manageable for that long — especially after I retired from a teaching career of 20 years — and that by then maybe the risks would have gone away. But I am typing this article using only my left thumb, and I cannot write with either hand. And my speech has rapidly worsened: Imagine a Wookie trying to speak English. That is who I am now.
It is possible that DBS might actually worsen my speech and not improve my movement. So why have I decided to do this now? While one study says I might get my body back and be able to dump the medications I take for dystonia, the biggest reason for courage is Liam. I hope he will see the surgery improve the coordination in my arms and legs. I hope to build his own courage about DBS.
For myself, trying DBS carries the fear of tinkering with my brain — my persona, which to me represents part of my soul. Oddly, though, when I discussed the surgery with Liam, he called me “a coward” for doing it. I was confused by this, but we talked this through. Liam is afraid that if I get better, I will abandon him to suffer alone.
Would my success expedite his decision to get the same procedure, I asked him. He may need DBS to walk in the near future; I am going first so he knows what to expect. So part of my taking this risk is that I will be his guinea pig.
Here is what I can expect with DBS at the University of Minnesota hospital where I will have the procedure. After some local anesthetic, doctors will screw a cage to my outer skull, to secure the position of surgical tools. Then they will give me general anesthesia and drill a hole the size of a nickel into my skull. Sedation then will be withdrawn so that the surgeon, with guidance from the neurologist who will be interacting with me, can insert a recording electrode into the basal ganglia, the section of my brain where my dysfunction occurs.
This electrode records neural activity that the neurologist will see on a monitor and hear. The recording device will pinpoint a tiny area that controls body motion on my right side, which is more impaired than my left. Once that spot is found, the doctors will insert a wire containing four electrodes that will essentially deactivate the part of my basal ganglia that has been causing the dystonia.
I will be awake throughout the procedure — it’s expected to last five to seven hours — to help the doctors assess what happens when the electrodes begin emitting electricity. The doctors will ask me to perform everyday movements, such as writing with my right arm. (I haven’t done that since I was 12 years old.) They may check to see if they also hit the wrong spots, causing my speech to suddenly worsen or triggering a random muscle twitch. The better the placement, the better my coordination will be.
What terrifies me most about being conscious through the procedure is not pain — there are no pain nerves in the brain — or the physical pressure; it’s being aware that there is a 2 percent chance that the world I see, with my family in it, will suddenly vanish because of a brain bleed caused by what is essentially elective surgery. I will try not to fixate on that.
About a week after the surgery, there will be a second, easier procedure, to implant a battery into my chest and attach it to the electrode wire in my brain. Once my brain has healed from the first surgery, the battery will be programmed to send electrical charges to the electrodes. The neurologist will decide which pulses of electricity are best for controlling my dystonia. Eventually, I’ll have some control over those patterns, too, by using a remote-control device.
Doctors say it might take months or even a year for my brain to essentially rewire itself. If the surgery does work, the same procedure will be done again to tackle the dystonia on my left side.
Liam suffered a new loss this summer. He is beginning to lose, as I did at age 11, the ability to use a pencil. Because he was quite admired among his peers for his drawings of his original heroic characters and the action that surrounds them, this was quite a blow; he met this deterioration with much anger. But then he eventually showed grit. It took him multiple attempts at one drawing to learn on his own, much as I had, that bending your elbow in to your stomach will steady your arm so that you can write and draw. His persistence was even more beautiful to me than the fabulous drawing he made of a sports car.
There’s an odd gift that dystonia has given me: the knowledge that any attachment in this life, whether to a toy or even a skill such as drawing a beautiful picture, is temporary. But humans can adapt, which I believe is our species’s greatest strength. Just as I did, Liam will have to find innovative ways to adapt his body to suit his desires. As I recently said to him, “You can’t play the violin, but you can still sing.” And he does.
In the future, my wife and I have no doubt that Liam will find new ways to reflect his life through art, music, sports or dance. These ways might not look exactly like what a parent expects when they first swaddle their newborn. But they will be Liam’s ways. I believe he will be a good person, and wiser for having learned to think outside the box.
In the meantime, I will be brave for him. If all goes well with my brain surgery, Liam and I — and many others like us — may have an easier road than we thought. And Liam is building his courage for getting this procedure soon — unthinkable bravery when I was his age.