Someone Else's Life (39 page)

Author’s Note

Huntington’s disease (HD) is a terminal hereditary disorder of the central nervous system, caused by a faulty (enlarged) gene on chromosome 4. Named after Dr. George Huntington, who first described the hereditary disorder in 1872, HD affects as many people as hemophilia, cystic fibrosis or muscular dystrophy.

Every child of a parent with Huntington’s disease is born with a 50 percent chance of inheriting it. If the child does not inherit the gene, he or she cannot pass it on—it cannot skip generations. If the child does inherit the gene, he or she will, at some stage, develop the disease, if he or she lives long enough. In 1993, the HD gene was isolated and a direct predictive genetic test was developed. The test can accurately determine whether a person carries the HD gene, but not the age at which symptoms will begin.

Symptoms of HD usually develop between the ages of thirty and fifty years old, although they can start much earlier (there is a rare juvenile form) or later and can differ from person to person, even within families. Likewise, symptoms can vary from person to person, but include physical, emotional and cognitive changes.

Physical changes
often include involuntary movements (chorea), stumbling and clumsiness, difficulty in speech and swallowing, and weight loss.

Emotional changes
can result in stubbornness, frustration, lack of inhibition, mood swings, paranoia, aggression or depression.

Cognitive changes
can include short-term memory lapses, loss of organizational skills, difficulty multitasking, and loss of drive and initiative—which may be misinterpreted as laziness.

Symptoms progress slowly over ten to twenty years, with death usually resulting from complications such as choking, infections, aspiration pneumonia (caused by difficulties in swallowing) or heart failure.

Although there are currently about 6,700 reported cases in England and Wales—and 30,000 in the United States—probably over twice as many people are affected. This is because people with HD often hide the condition due to social stigma, or insurance or family issues, and because many cases are never diagnosed. Many people with a family history of HD decide not to be tested, since there is currently no cure, and people with no known family history of the disease are also often misdiagnosed with other conditions, such as dementia or depression.

Though no cure has yet been found, since the discovery of the gene that causes HD, scientific research has accelerated, and much has been added to our understanding of Huntington’s disease and its effects.

There are many ways to manage the symptoms effectively. Medication can be used to treat symptoms such as involuntary movements, depression and mood swings, while speech therapy can significantly improve speech and swallowing problems, and a high-calorie diet can prevent weight loss and lessen symptoms such as involuntary movements or behavioral problems.

Useful Websites

Huntington’s Disease Advocacy Center:
hdac.org

HDSA National Youth Alliance:
huntingtondisease.tripod.com/nya

Huntington’s Disease Society of America:
hdsa.org

Huntington Society of Canada:
hsc-ca.org
Young People Affected by
Huntington’s Disease, Canada:
ypahd.ca

International Huntington Association:
huntington-assoc.com

Acknowledgments

Many thanks …

To everyone whose lives have been touched by Huntington’s disease, either personally or professionally, who have helped me in so many ways, sharing their knowledge, advice, and personal stories, particularly Matt Bower, MS, CGC; Susan Walther, MS, CGC; Phillip Hardt; Stacey Barton, MSW, LCSW; Professor Joseph Boyd Martin, MD, PhD; Andrea Gainey, MS, CGC; Bonnie L. Hennig, MSW, LCSW; Dave Stickles; Christina Barnes; David Harbourne; Bill Crowder; Karen Crowder and everyone in the HDA; Jean E. Miller; Frank Medina’s wife, Gloria; Dave Hodgson; Hugh Marriott; Peter Webb and everyone in the Sussex branch of the HDA; Tracie Tuhill; Jean Morack; Fred Taubman; Jennifer Williamson, MS; Adam Coovadia, MLT (CSMLS), MB, CG (ASCP); Kristin Kitzmiller; Shelby Duffer, MS, CGC; Kendell Aitchison; and especially to the exceptional and inspirational Pat Leslie-Penny and Matt Ellison.

To Colleen Begg for her advice about maternity wards.

To Miss Higgins for encouraging my writing after reading my “Owl” poem.

To Ruth Moose for her wonderful writing classes at UNC, where this story was born, and for introducing me to the great SCBWI.

To the Society of Children’s Book Writers and Illustrators, particularly the fabulous Sara Grant and Sara O’Connor, for running the incredible Undiscovered Voices competition with Working Partners, giving unpublished, unagented writers a chance to climb out of the slush pile and make their dreams come true.

To my lovely editors, Michelle Poploff, Venetia Gosling, Jane Griffiths, Amy Black, and Rebecca Short, for making this dream come true.

To my brilliant and very lovely agent, Jenny Savill at Andrew Nurnberg Associates Ltd., for believing in
Someone Else’s Life
from the moment she first read it—and then helping me to ditch forty thousand words.

To Chris, for his constant love and support for my pursuit of this dream, no matter how penniless I’ve been or how hopeless it’s seemed, and for putting up with me scribbling away beside him at ridiculous hours, and in the craziest places.

To my granddad Charles, a true gentleman, for his selfless love of all his family and for always being so proud of us.

To my lovely sister Caroline, for showing me that joy and laughter can be found in every single day.

To my equally lovely sister Jenny, for her incredible humor, courage, and selflessness whilst proving that while life may not follow the route you planned, it’s what you do with it that counts, for sometimes the greatest happiness lies down those unexpected avenues.

To my gorgeous niece Summer, my little clown, with whom the world is an infinitely magical, hilarious, and wondrous place.

To my wonderful dad, for his endless love, support, wisdom, and humor, and for his ingenious “Moley” stories.

And finally to my amazing mum, for always believing in me, encouraging me, and inspiring me every day of my life.

Thank you all so much, from the bottom of my heart.

KATIE DALE loves nothing more than creating characters—both on page and onstage. She studied English Literature at Sheffield University and spent a year at the University of North Carolina at Chapel Hill, followed by a crazy year at drama school, a national Shakespeare tour, and eight months backpacking through Southeast Asia. She is busily working on a variety of projects, from novels to picture books, occasionally playing stage roles as princesses, assassins, and zombies in between! She lives in England.