My question seemed to make everyone relax. That made me, at 10, the person I wanted to be: In control, competent, helpful. This was difficult for them. It was a good thing for me to recognize and care about. But it was also an ability that helped me avoid feeling much of anything about the word “degenerative.”

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By the time I was ten in early 1976, my father finally found a job he seemed likely to keep and we settled into our second tiny Connecticut town. I’d been to three public schools during fifth grade and was wearing the Milwaukee back-brace 23 hours a day. 

I’d started being seen in the pediatric neurology clinic at Newington Children’s Hospital, up by Hartford. (It had been formerly known by the delightful names Newington Home and Hospital for Crippled Children, as well as The Newington Home for Incurables.) Dr. Russman and Dr. Drennan, my neurologist and orthopedist, respectively, questioned my diagnosis of cerebral palsy atypical but continued the familiar routine of x-rays and exams every 3 months to monitor my always-worsening scoliosis.  

After growing the tissue from two nerve and muscle biopsies taken from my upper arm and calf (an experience that included my first overnight in a hospital, my first pre-procedure Valium, and hearing the words, “Skin stretcher, please,” uttered in connection with my own personal calf), they scheduled an appointment for the verdict. I was interested in a detached way; I was comfortable with not really being one thing or the other, medically. 

Because this appointment was more momentous than the usual quarterly visit, both my parents were there. We weren’t in an exam room, though, or the brace shop in the hospital basement. We were in someone’s office, a small, sunny place with exactly enough room for four people to sit.

This left Dr. Drennan, the last to arrive, leaning against the cinder-block wall in his white lab coat. He was tall and had sandy hair, and he carried a plastic protractor in his breast pocket. As there were no x-rays of my spine to be examined today, the protractor would not make an appearance. Dr. Russman, whose work could be as subtle as Dr. Drennan’s was concrete, and who was dark-haired and urbane, was sitting back easily in a desk chair.

On the whole, I felt well-treated by these two doctors, particularly in comparison to some of their predecessors, who had been prone to such jackasseries as referring to me as “it,” and calling my mom “Mother.” We might have all been gathered cozily around a puzzle — my disorder — fitting little areas in, knowing it wouldn’t be done for a long time. 

I wasn’t aware until years later that I was the only one who hadn’t yet heard the results of the biopsy. Dr. Russman had called my parents the week before, explaining what they’d found, and asking how much they wanted to tell me.

I didn’t, in fact, have cerebral palsy atypical, a stable and non progressive disease. I had, as far as they could tell, some kind of muscular dystrophy, a progressive and degenerative neuromuscular disease. They couldn’t really say what kind but I most closely resembled the type called Dejeurine-Sottas. A diagnosis, yes, but not a terribly descriptive one.

My parents were well on their way to divorce at this point but they were in accord as to what I should know from my doctors: Tell her everything but keep it simple and don’t dwell on what you don’t know. If she asks you about something that’s unknown, just say you don’t know and leave it at that.

“We grew the cells from the biopsy as we described,” Dr. Russman began. Fan-like wrinkles at the corners of his eyes gave away that he was a little tense. Sitting between my parents, I assumed he was talking to all three of us but he was really speaking to me.

He leaned forward, hands clasped. “As we suspected, you don’t have cerebral palsy. You never fit the diagnosis, even as an atypical. I still don’t know why your other doctors went with it, especially after the EEG was normal. But I don’t think it’s negatively affected your treatment.”

He seemed to be waiting for me to say something.  I nodded.

“Okay,” he repeated. “It looks like what you most likely have is a type of muscular dystrophy named for the doctors who identified its key characteristics in the nineteenth century.” He rattled off the two French names and started explaining something about Charcot-Marie Tooth disease.

I repeated back what he said as best I could. He explained that it was a disease that required recessive genes from both parents, and that having even one parent with this recessive gene was rare. Having two was extremely rare.

Some people got lied to just because they were kids. That made me really mad, the idea that I couldn’t handle something difficult. Or to be left out of something that was about me. I watched Dr. Russman very closely, which I knew they noticed, but I kept watching Dr. Drennan and my parents as well, how they shifted and when, and when their silence was suddenly more silent than before.  

I looked to Dr. Drennan. He expression was kind but unreadable. I looked sideways, left, then right, at my parents. My mother had her worried face on, and my father’s chin was practically skyward, his sign of meeting the enemy. “Is there something really bad about all this?” I asked flatly.

When Dr. Russman took a breath before answering, it was clear. “Muscular dystrophy is different from cerebral palsy in that CP is stable and non-progressive. But MD – and there are many different kinds – is progressive and degenerative. Do you understand what that means?”

“It means I get worse,” I said.

There was a fair amount of silence in the room and my instinct was to fill it up. “How do I get worse?”

My question seemed to make everyone relax. That made me, at 10, the person I wanted to be: In control, competent, helpful. This was difficult for them. It was a good thing for me to recognize and care about. But it was also an ability that helped me avoid feeling much of anything about the word “degenerative.” 

Dr. Russman was saying, “Your kind of MD affects the peripheral nerves only, the ones that are called voluntary. Not your brain, not your central nervous system. There’s a sheath around your peripheral nerves that’s too thick, and it’s muffling the nerve impulse going to the muscles that make you move. This is always going on. The muscles get weak as a result, they atrophy much faster than other people’s. We have to make sure you stay as active as possible so that you don’t lose ground.”

This was all true but he didn’t dwell, as my parents would later put it, on the inevitable loss of sensation and strength I would face in my limbs, or how this could affect my heart or my breathing, for example. Nor did he mention the biggest unknown of all: how rapidly this would happen. There simply weren’t enough comparable cases to help develop a detailed prognosis.

In my memory, I see this: I’m looking hard at some point in the middle distance, eyebrows drawn together, my palms pressed hard against my chair. I’m tense, my head turned slightly as if trying to identify an unfamiliar sound. Or whether any sound was missing.

I was weighing two things:  That I believed I was getting the truth, and the still-worried furrows on my parents’ faces. This could have touched off suspicion but worry was such a normal reaction for them that I took it as corroboration that I now knew the score.

I chose somehow to be relieved that it all seemed to be about familiar territory – walking, fine motor skills, reflexes. I didn’t ask whether there were other, less well-defined effects. I didn’t ask about life expectancy or carrying a child, for example, and Dr. Russman didn’t bring either up.

He talked a bit longer about the more clinical aspects, about the relationship between nerves and muscles, and Dr. Drennan unfolded his arms to talk about my back. My father reiterated the genetic aspect of it to me (he was a biochemist and could go on about it in surprisingly understandable terms) until my mother interrupted them all to say, “Give her a chance to ask some more questions, will you?”

There was silence. What was there to say? But I felt I had to say something, I should have been curious, so I recalled myself from where I was listening and sat up straight. “Okay, I get the thing about CP being stable and MD is degenerative. But I remember them saying I was getting better from when I was really little, right?” I gestured to my parents. “How’d that happen?”

“This is true,” my father said. “We were quite encouraged by how your walking improved.” My mother nodded.

“Maybe we should just keep saying I have cerebral palsy,” I said. “I got better when we called it that. Change the name for what I have. Whatever it is.”

But the easy transit to acceptance — because how bad was it, anyway? – halted briefly on the tracks in the back seat of the car going home later, when my parents’ own lack of curiosity in the office lumbered into view wearing lead’s dull shine, visible only after the diamond-mind’s glare switched off.

They had been, as my father would put it, lumpen. They had to have known the results when they walked into the office, and if had not perceived that, there might be more I didn’t know.

Looking at the backs of their heads, I tried to imagine what they knew that I didn’t and ended up amazed by their ability to lie. And to lie so well. They must have known during every minute of however many days before it had been, and I hadn’t seen at all. Looking at them, all I saw were the inscrutable backs of their heads. I hadn’t really considered how scrutable their faces were, or weren’t, before. It was exhausting to think of the burden ahead, keeping such a secret no one suspected there was a secret at all. The fact that I behaved exactly like my parents was lost on me, sharp-eyed as I was, and the person I kept a great deal from was myself. 

“How you doing back there?” my mother asked, turning around.

“I’m okay,” I said.