11/13/2012 03:59 pm ET Updated Jan 13, 2013

Huntington's Disease and the Inspiration Behind The Shortest Way Home

I first met Sue Koehler when I was living in Seattle in the mid-1980s. We'd both been in the Jesuit Volunteer Corp -- sort of a Catholic City Year -- and had jobs working in homeless shelters. Both in need of a roommate, we shared an apartment for a year.

I soon learned that Sue's mother had Huntington's Disease: "A devastating, hereditary, degenerative brain disorder for which there is, at present, no cure ... HD slowly diminishes the affected individual's ability to walk, talk and reason," according to the Huntington's Disease Society of America.

If one of your parents has HD, you have a 50 percent chance of inheriting the gene for the disease. Onset of symptoms is most likely to occur in your 30s to mid-40s. Sue's mother's symptoms began with severe mental disorientation at the age of 35. With two young children, "devastating" seems like too mild of a word to describe it.

At the time that Sue and I lived together in our 20s, there was no test for the disease, as there is now. She had no way of knowing if she would begin to lose her physical and mental capacities in 10 years... or never.

She went about her life with passion and a great sense of humor, but didn't seem to have a long-term plan for her life, throwing herself into her work at the Martin De Porres Shelter for homeless men without much thought for a career. She never seemed to date anyone that she would ever really marry. However, I didn't get the sense that she obsessed about HD all the time, either.

Personally, I couldn't stop thinking about it. What would she do if it turned out she had HD? And how would she feel if she didn't have it, and had lived a sizable chunk of her life basing important life decisions on what turned out to be a non-issue?

Many years later, this latter question continued to haunt me, and became the basis for my latest book, The Shortest Way Home. The novel is not based on Sue, but it is connected to her by virtue of how her true story has always stuck with me. What follows is a little more about that true story, in Sue's own words.

How did you first learn about Huntington's Disease?

My mother had been in the hospital for over three months (something that could never happen today!) in part because her doctors were trying to figure out what was wrong with her. I was in deep distress because her personality had unraveled, and no one seemed to know why.

One night, my father told my brother and that we would be going to our grandparents' house for some news. We never just "went to our grandparents' house," so I knew something was up. I was 13 at the time and my my brother was 11. My dad took us out on the back porch and blurted out, "Your mother has Huntington's Disease and you each have a 50/50 chance of getting it."

Although I knew nothing about the illness, I knew my mother had become unrecognizable to me. That, in and of itself, was bad enough. But to be told in the same breath that my brother and I could also have the same fate was overwhelming.

How did that knowledge impact you as you were growing up?

At such a young age to find out that my own life could be virtually over by the time I was in my 30s had a profound impact. I always presumed I had the illness. So I never expected to marry or have children. And because of this, I did not allow myself to contemplate what my life could be as a mature adult. This caused a lot of short-term thinking and some terrible relationship choices. I only allowed myself to be involved with "unavailable" men. Not a good plan.

Living under the cloud of Huntington's did offer one incredible gift: Without the guarantee of a long, healthy life, I embraced each day as fully as I could. I also developed an extraordinary faith in God. With my mother unavailable due to illness and my father unavailable due to not being able to cope with my mother's illness, I looked to God for solace, strength and direction and found it!

Did you think about HD on a daily basis?

HD was the major preoccupation of my young life. Every major choice was viewed through the lens of likely having HD. In my 20s, I had a wonderful man -- with some "availability issues" of his own -- who wanted for us to seriously explore marriage, but I was simply not able even to consider a life with him because I was frozen inside by the fear of the illness.

So many people who are at risk for HD decide not to get tested, but you wanted to. Why was that?

After my mother died of HD when I was 28 years old, I woke up and realized I could never freely make a choice about relationships and marriage unless I knew whether or not I had the illness. I needed to be free.

How does HD continue to affect you?

On March 20, 1992, due to the extraordinary work of doctors like Ric Myers at Massachusetts General Hospital, I found out that I did not have HD. However, by that time, my brother, Dave, was already showing signs. A few years later, he was diagnosed with HD, and I have been his main support since then. For many years he lived with me, until it was no longer safe for him to be alone while I was at work.

Today he lives at the Laurel Lake Center in Lee, Mass. Each time I visit him, my heart breaks a little bit more. I have done some advocacy work, public speaking, and letter-writing to try and ensure that people with HD get the care they need. For me, it still takes a tremendous amount of courage and faith just to walk through the door of Laurel Lake and face HD again. But living with HD takes even more courage. Seeing my brother and the other residents keeps my heart soft and teaches me compassion. The trials I have had to face in my own life and observe in my brother's life have given me an especially soft spot for others facing or caring for someone with any kind of dementia, something I see nearly every day in my work as a hospice chaplain.

Sue is now happily married and lives in New York with her husband and son, who is smart, talented, polite and handsome. I can brag about him because he's my godson, raised by an extraordinary person and friend.

For more by Juliette Fay, click here.

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