Toast to Hope for Johns Hopkins HD Center

What's more terrifying than being tested for Huntington's Disease? Speaking about it in public. My sister Kim and I shared our family story to fundraise for the Johns Hopkins HD Center. I'll let the chair of the Psych department share his version of the night.

"Last night at Boordy Vineyards in the rolling farmland of Baltimore County, I welcomed a group of 50 or so people to “A Toast to Hope” sponsored by the Hopkins Huntington’s Disease Center Advisory Board. In a rustic 19th century barn that was warm and intimate, we listened to the Pryce sisters, Kim and Erin, who are in their 30s, tell their story. Kim described how she and her three sisters, when they were young, knew that a grandparent, their father’s mother, had Huntington’s disease, an illness that typically starts in early middle age and brings abnormal twitching and writhing movements, decline in cognitive abilities, and depression. The illness usually becomes steadily more severe and is lethal after a course of 10-30 years. The Pryce sisters described how later in their childhood they began to see their father oddly burying his hands between his legs often. Eventually they realized that he was trying to hide the jerking movements that had emerged in his hands, signaling the onset of the illness. He sought treatment at our Hopkins Center. When the sisters were adults they faced the issue of whether or not to take the genetic test that could tell them definitively whether they carried the disease variant of the Huntington’s gene. Each of them had a 50-50 chance of having inherited it from their father. Kim took the test and found that she was negative—she would not develop Huntington’s. Two of her sisters were also negative. Erin, standing before us, looking young and vigorous, was positive. She told us that she chose to live rather than to despair, to have hope rather than to give up. Erin, the youngest of the four sisters, is a scientist herself, and she believes in the power of new molecular tools to unlock the secrets of disease, and to generate treatments and even cures. Kim chimed in with energetic support, and there was something about her older-sister strength and protectiveness that made my eyes water.

Seeing Erin Pryce standing before us bravely laying bare her vulnerability and hoping for medical scientific progress in the fight against Huntington’s, knowing that she is in a fight against time, and having Kim rallying us to commit ourselves to the effort as she buoys her little sister’s spirits in the process—all of that reinforced how incredibly important our work in the department is. To people staring destiny in the face. To people whose fate hinges on our efforts and our success. We bring immense talent, compassion and caring, and limitless dedication to the task. And for this dreaded illness, we are tantalizingly close to delivering."