A sports star and a cook

At the Redwing Health Clinic in Redwing, MN, USA, a faded picture hangs in Scotty Redford’s room. A young, handsome man in a football uniform beams at the camera. Now 33, Scotty misses those days (figures). Once a teenage football sensation, he was tested and diagnosed with Huntington’s disease more than 15 years ago, aged 18. After watching his mother deteriorate, and eventually die, because of the disease, Scotty is now faced with his own vanishing muscle control. Although still handsome and smiling, he is confined to a wheelchair, and has difficulty speaking and eating. Scotty is one of about 30 000 Americans who have the disease, an adult-onset, dominantly inherited disorder, caused by the death of neurons that control movement. Involuntary muscle movement, cognitive impairment, and behavioural abnormalities characterise Huntington’s disease. Many of these features are evident in Scotty. As a young man in Tuscaloosa, AL, Scotty was employed as a caution driver. He took for granted the quick motor coordination and sharp cognitive skills that he depended on for safe transport of heavy vehicles and massive equipment. However, as his uncontrolled movements got progressively worse, Scotty became aware that his performance was deteriorating to the point where work was no longer possible. Even more shattering was the realisation that the former sports star, attuned to physical activity, would be unable to throw a football or to swing a bat again. Next to his football picture, there is a newspaper clipping showing Scotty in a tuxedo at his highschool prom. With his arm hooked around his sweetheart, he is the very picture of youth and vigour. However, his deteriorating muscle control made social interactions difficult, and, before the age of 20, Scotty vowed not to marry for fear of passing on the gene that had ravaged his mother and now himself. Nancy Elaine Mitchell, 42, another resident of the Redwing Health Center, also has Huntington’s disease. Like Scotty, Nancy watched her mother die from the disease. With a 50% chance of passing the gene on to a child, affected individuals must decide either to abstain from raising a family, or gamble with the fact that one or more of their children might inherit their disorder. Nancy chose not to confirm a positive diagnosis through genetic testing. However, because of the possibility of transmitting the faulty gene, she too decided against having any children. Although she believes that hers was the right decision, she is still angry at having to make the choice. Those with the disease often live with misunderstanding. Nancy, a cook in her hometown of Indianapolis, IN, was capable, dependable, and fiercely independent. However, at age 32, she began to show mild signs of the disease, including erratic movements and slurred speech. The gradual onset of her symptoms was misleading, and her employer abruptly fired her for being drunk. There was no discussion. 10 years after she was diagnosed with her disorder, Nancy lost the coordination skills that enabled her to walk; she now uses a wheelchair. For a woman longing for and clinging to independence, this development dealt a staggering blow. Her disease has taken her from home to, what seems to her, an alien world. She never imagined that, so early on in life, she would be forced to live in a long-term care facility, without privacy, and where her every move would require assistance. Without more effective therapy than is available, neither Scotty nor Nancy is expected to live more than 5–10 years. They have different attitudes towards the development of new treatments. Having lived for more than 15 years with his disease, Scotty is not optimistic about the chances of an effective therapy becoming available soon enough to help him. However, consistent with his cheerful disposition, he has channelled his energy into a commitment to help others with the disease. With the aid of Jeannine Morgan, his caregiver, Scotty has ensured that his tissues will be available for research, hoping to help cure the disease that has crippled him. Nancy is more hopeful, although she too wonders when a therapeutic breakthrough will occur. In the meantime, she is frustrated by the fear and the lack of understanding that surrounds Huntington’s disease. When in public, strangers laugh or stare; others believe the disorder is contagious. Thus, Nancy is committed to educating her community about the disease. She is active in local community groups, and her campaign has received local media coverage. She and Scott, with the support of Jeannine and their families, are lobbying for an appearance on national talk television. They hope to use the platform to provide a better understanding of their disease. Their actions speak clearly of their dignity and grace, even if their words are difficult to understand. Scott Redford at age 18 and 33 years