Clan Condition - Nez

 

After a playground game of basketball Wayne quickly sat down and removed both shoes. His feet hurt. A lot, and he didn’t know why. They didn’t burn or sting like needles or spikes. They just hurt. His shoes still fit, and he’d been wearing them a couple of months. He had played several basketball games over that time. This was the first time his feet hurt though. He took off his socks and rubbed both feet and didn’t see anything unusual. He tried standing on his bare feet and the pain wasn’t as sharp as it had been. He sat back down and put his socks on and stood again. Still, not as sharp as before. He sat down again and tried putting his shoes back on and stood. Sharp pain was back. So, he sat down again and loosened the laces. That was better, so maybe it was the shoes after all. With loose laces he could walk so he limped home and told his mom he needed new shoes. That would not be the last time Wayne’s feet were that painful. No.

Lillian enjoyed writing stories and was excited when her sixth grade teacher gave the class a short writing assignment about animals. Lillian had a couple of pets, a cat, a hamster and a parakeet and telling stories about them was one of her favorite things. She took paper and pencil and began to write, except that her right hand was painful. The more she wrote the more it hurt and now the pain was moving up her arm. She put down her pencil and cried out. Her teacher came to Lillian to see what the matter was. Through tears Lillian tried to explain her pain. Her right hand was very painful and now her left arm was also becoming painful. The very concerned teacher took Lillian from the classroom and called her mother. Lillian’s mother came and took Lillian home and to the doctor. And that was not the only time Lillian’s hands and arms would experience excruciating pain. No.

One Sunday morning Judy was singing for the church congregation when her left eye began twitching painfully. By the end of the song her eye was twitching so painfully that she ran tearfully off stage. Judy’s mother noticed her sobbing exit from the stage and went to her. After Judy’s explanation and showing her Mom her twitching eye, they went home and made a doctor’s appointment. And that was not the last time Judy’s left eye twitched so painfully. No.

Terry and a buddy were riding bikes when Terry’s right foot suddenly and painfully cramped up. He tried braking but the pain was too much and his left ankle was hurting too. Terry couldn’t pedal and he couldn’t stop so he coasted and simply fell off his bike. His buddy came to his aid but there wasn’t much he could do to ease Terry’s pain. But, he helped a sobbing Terry, up to sit on his bicycle seat, and with Terry’s feet dangling, his buddy pushed him home. Terry’s mother helped him inside and called the doctor. And that was not the only time Terry’s feet and ankles would hurt so painfully. No.

One might think these little anecdotes are unrelated. They are not. Each of these kids, that I grew up with in Lyman, was diagnosed with the same ailment, a Musculo-skeletal nerve disorder that’s a little different from most diseases. And while the condition is not unheard of, it’s pretty rare. It’s called CMT, an inherited condition for which there is no cure. Those who get it have it for the rest of their lives. It manifests itself in families or clans rather than the general population. And it passes from one generation to the next. In this family it manifested in each individual quite suddenly, yet it didn’t afflict every family member. As in this case Wayne and Judy are brother and sister. Their other sister and brother were spared. Lillian and Terry are also siblings and their two brothers were spared as well. These four are children of two brothers, one of whom had the malady in his feet, drop-foot. There was also another brother who suffered from the disease, yet none of his three children got it. Of that older generation of five kids only two had the condition. The disease is not contagious and generally shows up after puberty, and in this family’s case it was localized in each person. It didn’t spread, though it often worsens and can become debilitating. It appears in males and females equally.

All of these kids saw many specialists and the whole family became a medical case study for Seattle hospitals in the 1950’s. Several medications were tried to ease their pain, but they tended to make things worse. When ibuprophen finally came on the market in the 1960’s they discovered it eased their pain better than anything else.

Lillian and Terry suffered greatly throughout their lives as they attempted many different solutions that didn’t work. In time, Lillian had a hand and arm amputated below the elbow, and Terry had a foot amputated, and years later he had the other foot amputated, both just above the ankle. Wayne and Judy worked with their doctors, who did their research, and made personal adjustments rather than trying unproven medicines. Judy still manages to live with her twitching eye, at times quite painful. Wayne wore specially made shoes with extra-high arches. He lost feeling in his feet and walking was sometimes difficult for him.

These four kids and their four siblings produced fourteen children of their families’ next generation, yet only two have become afflicted with the disease. It’s unknown how many American families share this disease. The disease is called CMT (Charcot-Marie-Tooth) named after the doctors who discovered it in the mid-1800’s (two French, one English), and there are several forms of the disease. CMT is caused by a defect in one of the genes responsible for development of peripheral nerves. Early in its diagnosis it was thought to be a type of muscular dystrophy. But it’s an inherited nerve disorder, and not muscular dystrophy, arthritis, polio, nor any other known disease. It’s different and often painful.