MS has a genetic component, but neither my parents nor Meredith and I understood that when we decided to have children. I had become aware of familial connections through the years, punctuated by my own diagnosis with MS. Complicated emotions find their way to the surface when I worry about MS hitting my children.
The nightmare scenario does not go away. I feel powerless before my fears. Meredith is right there with me and worries as much as I do. Still, I am alone with my worst fears. That issue was in its usual spot in the back of my mind when I came across an online column in The Washington Post’s Health and Science section in late 2016. My inquiry into hope was just under way, and the prospect of reading about a father passing along a disease to a son was horrifying.
The headline stopped me cold: “‘My Heart Breaks 80 Million Ways’: A Father Passes a Disorder to His Son.” The author, Carl Luepker, was a forty-four-year-old former sixth-grade math teacher in Minneapolis, who described his long battle with dystonia.
Dystonia is a debilitating neurological condition, causing the muscles of the body to contract involuntarily, which results in repetitive or twisting movements. These movements can interfere with the performance of many day-to-day tasks, even some as basic as the ability to use a pen or pencil. Join the club, I thought.
Dystonia generally begins in either the hands or the feet and gradually progresses to the rest of the body. The disease can eventually affect the ability to speak. The psychological impact can be devastating when only a speaker understands what he or she is saying.
When Carl and his wife, Heather, a speech-language pathologist, thought about starting a family, they considered the risks of passing his illness on to another generation. They decided to roll the dice, he told me. “If I did carry the gene, there would be a one-in-six chance that our kid would develop the disorder. Heather said, ‘Well, then you’ll have to coach them through it. We will love them regardless.’”
Their son, Liam, did develop the disease. Their daughter, Lucia, did not. Carl wrote in the Post of how hard it is for him to see his child suffering. “I can live with my bad luck in getting this condition . . . What’s harder to accept is that I have passed on this disorder, carried in my genes, to my 11-year-old son.”
It hurts Carl to see the difficulties his son already has with even simple tasks. Painful memories linger from his own childhood. Carl remembers how his classmates mimicked and made fun of him, and he worries that Liam’s classmates will do the same.
I saw my own life reflected in Carl’s emotions. I related to the pure pain a parent feels knowing a life sentence has been passed to a child. I thought of my own parents and a much younger me. There was the day my mother came to spend time with me at Georgetown Hospital in Washington, D.C., shortly after my MS diagnosis. There I lay, flat on my back in a hospital bed, half my face paralyzed with Bell’s palsy. I had just lost all vision in one eye from optic neuritis.
My mom felt such anguish that she told me she never would have had children if she had known there was a risk they would inherit MS. Great, Mom, I responded with a chuckle. Thanks a lot. I was not hurt; I only felt bad for her. I told her that my life is not a tragedy. I was happy to be alive and doing well on all the fronts that mattered to me. My determination to stay in motion showed no signs of abating.
My father said little, but over the years it became clear to me how much he struggled with the fact that he had passed along his disease. His mother had done the same to him, of course. I think it was guilt that made the Old Man strangely hard on me. He could be cutting, but I let his occasional critical remarks roll off my back.
One experimental treatment for dystonia is a procedure called deep brain stimulation. DBS involves inserting electrodes into the brain, offering the possibility of substantial improvement in neurological function. The procedure does carry risk. There is some chance of a brain bleed, and there is also the possibility that neurological deficits might worsen.
Because of the risks and his feeling that his symptoms were manageable, Carl had originally intended to wait another five years for DBS. But he changed his mind because Liam’s condition was worsening. His son’s neurologist recommended that Liam should have the procedure within a year, in hopes that the surgery could help him before his dystonia left him wheelchair-bound. Carl felt compelled to act, feeling he could set an example for Liam. He knew that for his son, the time was right. He knew he had to go under the knife and endure the drill before he could persuade his son to do the same. Carl made clear that he is more focused on Liam’s health than his own. “I hope that by taking radical, elective interventions . . . my success will embolden Liam’s own courage for the procedure.”
“I’m not afraid of this surgery because I have no choice at this point but to sell hope to my son,” he wrote to me in an email. “It’s hope or bust (death during surgery). I believe hope is the last thing to die, and this hope, expressed as love for family and friends, is all I have left, and now it’s tied to DBS.”
A follow-up email was necessary because, as I transcribed our phone conversations, I realized I could not understand enough of what Carl was saying. His speech was slurred and disjointed, his voice distorted. I could hear what a strain it was for him to speak.
I knew I had to level with him and thought long and hard about just what to write in my email.
Carl immediately responded. “Thank you for acknowledging my own frustration with my voice,” Carl wrote back. “I hope to speak again, and sing again freely. I have a renewed flirtation with the external world.”
The DBS procedure takes five to seven hours and is painless. Though the procedure is arduous, it has a space-age appeal. After the anesthetic is administered, a hole is drilled into the skull through which a wire containing four electrodes is inserted. When the electrodes are activated, the impulses emitted are supposed to deactivate the portion of the brain causing the movement malfunctions.
“I recall being awake and in a head frame,” Carl said, describing the procedure to me. “I was completely aware of what was going on. I remember the dial my neurologist used to advance the electrodes as he mapped where [they] would benefit the most. I knew there would be a follow-up treatment.”
I asked Carl to describe any changes since the initial treatment. We were communicating by email. I was dying to hear Carl’s voice, to hear if he had improved. “My body is upright. It would tend to twist slightly at the neck level and there are less tremors. This overall impact has helped my speech as muscles trigger other muscles (like a dysfunctional network).”
That offered the perfect opening to ask about Carl’s garbled words. What about your speech? I asked. “My speech has improved, although my tongue and facial muscles still contract. I have much less fatigue when talking.”
And what about Liam? How did he react? “My outcome makes him more optimistic for his own surgery.” He left it at that. I felt that Carl was downplaying any real excitement coming from Liam. “As his foot, ankle and calf painfully contract, and I massage it every night, he is in that stage of desperation . . . and social isolation is creeping in. He wasn’t able to bike to school with his friends because his ‘foot would be too tired to make the ride back.’”
I asked if Liam was going to undergo DBS. “He will. It is tentatively scheduled for six weeks from now.” Carl sounded so matter-of-fact. I try to imagine a young boy, strapped down, his head in a cage, knowing that mysterious strangers in gowns and masks are sticking foreign objects into his brain. I think I would be screaming for someone to get me out of there.
“Liam is nervous. He wants to be ‘out’ for the procedure. I am researching DBS for kids and connecting with parents who have children that underwent the surgery. Yes, we are all scared. It’s brain surgery. But Liam can be spared of the progression and that is where we hold our hope.”
I had asked Carl if I could talk to Liam about his hopes for the future. When I called, we spoke first about his reaction to being diagnosed with dystonia. Talking to a ten-year-old about such a serious subject is sobering. “Even though I suspected it, it still was a shock,” Liam said. “I kind of knew it was there, but I didn’t want to believe it. When I was diagnosed, it really hit me hard.”
Though his words were stark, Liam’s voice sounded even and unemotional. I wondered if the boy really was that calm. Carl had told me Liam has an old soul. Maybe that is a side effect of dystonia, because I heard in his voice the same courage and steadiness that children afflicted with serious diseases often develop.
“Hope is what gets me to move forward,” Liam said to me. “Hope that I can be like my dad and get a job and take care of the family—and that I can get it cured. And someday, I don’t know, that it won’t affect me as much as it is affecting my dad.” I enjoyed talking to Liam, and I was very interested to talk to Carl on the phone after his surgery. I wanted to hear his voice and note any changes. I knew how badly he wanted to feel normal and reconnect with the world. I tried for weeks and had trouble reaching him. I began to wonder if Carl was avoiding me. Maybe the improvements had been overstated.
The phone rang late one afternoon. It was Carl. The phone call was stunning. Carl’s voice was clear, his enunciation much improved. The sound was not perfect but perfectly understandable. I found the conversation breathtaking. I asked if he thought his life was going to change.
“Yes,” Carl responded, “but it’s hard to rejoice because Liam is getting worse.” I could hear the pain in Carl’s voice. “Liam is at a point of desperation. There is a lot of pain at school. At the end of the day, if he could do the DBS tomorrow, he would.” Carl paused. “But Liam is scared.”
Liam would be given general anesthesia, which generally is not how the procedure is done. I asked Carl if the patient needs to be awake so he can respond to commands from the doctor. “That’s right,” Carl responded. Then why will Liam be asleep? “That’s a good question,” Carl answered. We left it at that.
But when we spoke again, the news was very good. Toward the end of 2017, I contacted Carl to ask if Liam had undergone Deep Brain Stimulation. He had, and Carl’s email had a new energy. “Liam is biking every day. He used to have to watch the neighborhood kids bike to school without him. Just last week he biked to school for the first time and is using the skateboard he wanted to destroy last year. He’s catching up on lost childhood. We hope it halts any progression (but it appears to be doing that). Now he’s just a smart-aleck seventh grader, who I’m finally optimistic about.” It is heartening to encounter a happy ending.
I remember how small and vulnerable my kids were at Liam’s age. Serious sickness is not for the fainthearted. Many families are drawn close by medical crises. Others are torn asunder. Outsiders cannot guess where the winds will blow. I know only that these are good people, strong and resilient. They are hopeful and committed to one another.
Families always should hope for that. Meredith and I have been fortunate. In 2015, Ben, our oldest, returned from nearly four years of living in China. When Ben had settled back in, as part of his preparation for business school he studied coding, a systematic method for analyzing data and generating insights into consumer needs.
Unbeknownst to us, as his coding project, Ben chose the task of identifying and analyzing handicapped-accessible entrances to the New York City subway system. He went about it quietly, telling us nothing about what he was doing. When he showed us the video of his presentation, we were very surprised. And moved. Meredith and I understood his motivation. Clearly Ben, who is now twenty-nine, hoped he could make a difference for me—since I still occasionally use the subway—and for many others.
Gabe, our middle child at twenty-seven, has also made MS his cause. When he worked as a television reporter in Spokane, before moving to his current job in Seattle, he was interviewed at his station about a local MS walk. After explaining that MS is a progressive disease, he told the interviewer, “It moves forward, and we take a step back. This walk is our chance to go forward. Let MS move back.”
Lily, now twenty-five years old, ran a half marathon in New York City recently to raise funds for MS. Defiance in the face of debilitating illness is fueled by hope. Our children seem to have a clear path to that hope.
In the end, all of us must grapple with hope. “Hope is being able to see that there is light,” Desmond Tutu said, “despite all of the darkness.” A family can provide a soft glow for a struggling member. Sometimes, a harsher beam is required.
In moments when I was hurting and hard on those around me, Meredith would set me straight, telling me I was never alone in that hospital bed. She and our three kids were there with me. We endure together, and when a family member is sick, hope moves to high alert. We hope as one. Nothing more needs to be said among us.