"I’m still in here.”
As a child, Sarah Foster didn’t understand why her aunt and grandmother acted the way they did. As history begins to repeat itself, she shares a letter about Huntington’s disease and how it makes her unrecognizable.
Before I became too old to bother with regular visits, I spent a lot of time with my grandmother and her sister - my great aunt. Both of them were living with Huntington's disease (HD).
My grandmother wore house dresses everywhere she went, with her long, silver hair in a braided bun. I remember my mother redoing that same hairstyle every weekend, keeping it neat.
When I was a toddler, the only game my grandmother could play with me was hide and go seek. To this day, I refer to her as "Baboo." That's how she'd pronounce "boo!" when she jumped out from behind the refrigerator to scare me. I thought she’d made up a new language just for our games.
But she didn't have her own language. She had Huntington's disease and was struggling to speak. In fact, "Baboo" was one of the last words she said to me (or so I remember) before she started to decline. The games and mischievous fun tailed off into an alien land of simple yes-or-no answers. A few years later, Baboo's world was one of complete silence.
Related: Not Just Normal Aging – Living with Huntington’s Disease
I had no idea how much HD changes people
Meanwhile, her sister, Aunt Naomi, was known for the impulsivity  that can go hand-in-hand with HD.
The whole town knew about the time she'd fired her shotgun up the chimney to destroy an active beehive. The family took away all her guns, and her doctor prescribed medication to calm her down.
Though she couldn't cause any accidents or damage, her impulsivity came out in other ways. Aunt Naomi began to make repeated, daily phone calls to everyone whose number she could still dial. When we picked up, she'd say, "How are you, Sug?"
Being young and silly and thoughtless, my brother and I laughed at her. Mocked her. Yes, we even prank-called her and others, mirroring the calls we'd get so often. "How are you, Sug?" we'd say in Aunt Naomi's lilting, Julia Child-like voice. We'd then explode in giggles of wicked delight and hang up (the unmistakable hallmarks of a child's prank call).
I thought they were “gone” – even as they sat in front of me
Eventually, the calls from Aunt Naomi petered out. She and Baboo were now struggling with another symptom called chorea. This movement disorder caused their bodies to move against their whims in unpredictable, irregular muscle contractions.
In her kitchen chair, Aunt Naomi's body writhed with slow, twisting motions she was unable to control. The word "chorea" comes from the Greek for "dance," which seems both accurate and cruel, somehow. One time, I swear I saw Aunt Naomi wrap her left leg around the right almost five times. Just like a bullwhip.
I made the whipping sound in my head. It was the only sound in the room.
Chorea exhausted the bodies of Baboo and Aunt Naomi. They'd sit silently in their last days, like twitching shadows in the straight-backed chairs my great-grandmother had caned years ago.
Whereas child-me didn't understand or perhaps care enough, adult-me has written them a letter. It's now decades too late, but I need to say the words even if they can no longer hear them.
Related: Running Away from Huntington’s Disease
To My Aunt Naomi and Baboo:
I didn't know you were in there.
I thought you were bodies who sat quietly, nothing going on in your heads. I thought that's what happened as people got older and were approaching death.
That seemed normal to me.
But now, I know I was wrong.
You weren't empty shells.
You still had your essences in those bodies that couldn't co-operate; your personalities remained unchanged.
You were there as much as I am here now, beginning to struggle with my own speech. I'm sorry I devalued you.
I didn't know you could both hear everything that was being said. But in your silence, you were listening, and you understood. You were probably trying to formulate an answer.
I'm sorry I didn't wait for you to respond.
I didn't know if your expressions were happy or sad. I finally stopped asking either of you if you'd had a good day. I decided on the good and bad days for you both, based on whether you choked on the mashed-up food spooned into your mouths. Now I know that choking when being fed was just one of the many experiences of HD you felt throughout the day.
I remember being so young and not understanding anything. I was boisterous - running and jumping into your laps, firing off questions you never answered, and smothering you with kisses you couldn't return. When you didn't respond, I'd shrug the same way I would at a broken toy, then run into another room for amusement.
Decades later, I can see you were fighting ongoing, silent battles. HD held you captive, making every response you attempted too slow, too weak, too late.
I get it. I've become less able to converse with my older sons when they visit. Nowadays, I struggle to think of anything to say but, "How are you?"
This reminds me: my brother and I lovingly call each other "Sug" in your memory, Aunt Naomi. When we talk to each other, you speak with us: "How are you, Sug?"
But this letter is as much to the entire family as it is to you both. I strive to include myself in their gatherings, even though it feels more and more awkward. It's a million times easier to sit on the couch and write blogs about how bitter I am.
But I need to put myself in their presence. I only have so much time to explain your - now our - experience to them.
Of being trapped by HD.
Of bursting with love for them all, but slowly losing the means to communicate it.
So, before they, like I, become too old to be bothered:
Guys, I'm still in here.
Johnson, P. L., Potts, G. F., Sanchez-Ramos, J. & Cimino, C. R., 2016. Self-reported impulsivity in Huntington's disease patients and relationship to executive dysfunction and reward responsiveness. [Online] Available at: https://pubmed.ncbi.nlm.nih.gov/27892808/ [Accessed 21 February 2022].
NPS-ALL-NP-00529 FEBRUARY 2022