Damocles ======== * Ian A. Cameron ## Abstract Dionysius the Elder invited Damocles to a sumptuous banquet to dramatically demonstrate the insecure nature of grandeur and happiness. Damocles was thoroughly enjoying the banquet until he was asked to look upward. There above his head, hanging motionless by a single horsehair, was a razor-sharp sword. Hesitantly, he closed the car door and walked up the driveway. I noticed that his beard and moustache were grizzled, but otherwise Jack Weber looked just as he had 30 years ago. Bev and I had met Jack and Cathy Weber as far west as you can go in this country and 40 miles farther. We were drawn to the Queen Charlotte Islands to cut our teeth in our brand-new careers and marriages. We worked with Cathy at the hospital, and it wasn't long before I got to know Jack, who was grading logs for one of the lumber companies. We were isolated, far from home, and the Webers became like family to us. We shared the newness, the adventure: fishing, beachcombing, getting lost in the great Sitka forest. One overcast day toward the end of spring, Jack and I hiked through the rainforest to explore the elk barrens, the home long ago of early settlers. At the edge of the barrens we found an old log cabin. Most of the shingles had blown from the roof, the chimney was precariously askew, the verandah had collapsed at one end, and the once robust cedar logs had begun to come apart. Suddenly we heard a strange trumpeting, and from the fringe of the forest a great white bird magestically emerged and then slowly disappeared into the mist. We watched in awe. It was to be our last outing on the Charlottes. That summer Bev and I returned to the east. The Webers remained for a time and then moved to the mainland. We kept in touch with fitful Christmas cards that chronicled the happy and sometimes sad milestones of our lives. Jack had always wanted to visit the east coast and when he had a chance to attend a conference in a neighbouring province he took it. We welcomed Jack and introduced him to our youngest daughter, who gave him a hug and made him feel right at home with her friendly chatter. Soon we were recounting fish stories over a cup of tea, but I couldn't help noticing how restless Jack was on the couch. He seemed uncomfortable. "It was a long drive," I remarked. "Is your back bothering you?" "No, my back is fine." Later, in the kitchen, Jack was talking about grading cedars before transporting them to the mainland. Suddenly he was on the floor. He had slipped. The transition in his sock feet had been so smooth that not a drop spilled from his teacup. He sat there for a moment with just a hint of embarrassment, declined my help, and slowly, awkwardly, stood up. FIGURE ![Figure1](http://www.cmaj.ca/https://www.cmaj.ca/content/cmaj/162/7/1023/F1.medium.gif) [Figure1](http://www.cmaj.ca/content/162/7/1023/F1) Figure. "Where was I? Oh yes, red cedar." The next morning was a bright end-of-summer day. We opted to head down the coast. Along the way I talked about the fishing villages and how the glaciers had affected the terrain. Jack was looking out the car window at stunted, wind-sculptured spruce trees when he said, "Cathy left me two years ago. We had some counselling but it seemed she had made up her mind. There was nothing I could do." I was still putting a sentence together in my mind, some kind of appropriate response, when he continued. "It's been really difficult the last few years. My older brother had a successful business, then all of a sudden he started making very bad decisions. He lost everything. His behaviour became progressively more disturbed, and he had to be committed, just like our mother years ago. That's when they did the genetic testing on him. Then the other six children in the family got tested. Only my sister and I asked for the results. She was negative, I am positive." *The Huntington's disease (HD) gene,* IT15, *is on the short arm of chromosome 4. The RNA transcribed from the HD gene can be found in all organs of an affected person, and codes for a protein called* "huntingtin." The road to the ocean led down through a cranberry bog to a fishing village. Brightly coloured houses were strewn over the barren landscape, some nestled in the lee of huge granite boulders, others jauntily perched on the rocky ground, exposed to all the beauty and fury of the north Atlantic. We drove in silence to the end of the road and parked the car. In the distance the seagulls were wheeling and diving near some fishermen who were transferring mackerel to an offshore cage. The salt air was invigorating. Jack slowly got out of the car, marvelling at the enormous smooth granite headland. Nuggets of mica danced in the rock, the tidal pools shimmered, and the ocean swells surged up beneath brown ribbons of slippery kelp, sending spray over the bald rock. Jack moved tentatively out over the granite toward the water and I followed. *The neural damage in HD proceeds from the caudate to the putamen, resulting in damage to frontocaudal and frontolimbic circuits. HD is classified as a neurologic condition characterized by chorea, dystonia, athetosis, bradykinesia, dysarthria, dysphagia and urinary incontinence. Psychiatric manifestations include mood changes, anxiety, psychosis, personality changes and aggressive behaviour.* The rock surface was uneven with crevices and sheer faces. Moving across it amounted to horizontal rock climbing. We moved obliquely toward the ocean and the sign bolted securely to the rock: DANGER! DO NOT GO BEYOND THIS POINT. The warning was not to be taken lightly. In the past year a young couple strayed beyond the sign when the seas were running high. A moment's inattention, a rogue wave, and they were gone. Jack was out in front of me, heading toward the receding waves. "Jack! It's dangerous down there!" He disappeared behind a ridge of granite. *Death in HD is caused by cardiac and pulmonary complications, suicide and self-destructive behaviours.* I scrambled around the far side of the ridge. There was Jack - waiting for me, camera in hand, ready to immortalize my consternation and the majestic white lighthouse looming behind me. That day we dined on fish chowder and lobster rolls, explored the cove, the fish sheds and the wharves, whose pilings were festooned with barnacles and mussels and rhythmical seaweed. Jack talked to the fishermen and found a shop that sold old glass floats reminiscent of our beachcombing days. Toward the end of the afternoon a piper began playing near the lighthouse. Jack was mesmerized, and at the end of her set he talked to the piper about Celtic music and where in the province he could go to hear the best. The next morning Jack headed out after breakfast, travelling farther east. He returned in three days, full of stories about ceilidhs, amazing fiddlers and virtuoso spoon players. He had not attended the conference on Huntington's disease. "There will be another one," he said. That spring our youngest daughter graduated from high school. She received a card from Jack. The handwriting was resolute. He thanked her for her smiles, her joy, her acceptance of a stranger. He wrote: "May the Lord bless you as you begin your new life."