With Rabbi Dan Ornstein

By RICKI LEWIS

Huntington’s Disease (HD) is not one of the so-called Jewish genetic diseases, but it ravaged three generations of a Jewish family living in the Capital District. Alan Pfeffer of Glenmont is an attorney and an advocate for HD patients: he champions nursing home care and leads efforts to educate legislators and convince them to improve care.

His book Charise, A Woman With Huntington’s Disease Who Changed the World: Her Poetry and Memoir, pays tribute to his extraordinary daughter, who died at age 43 in 2024. She had lived with HD since diagnosis at age 29. Her mother had been diagnosed when Charise was just 8. Her mother’s father had it too.

 

A Quick Science Lesson

HD is passed from parent to child with a risk of 50 percent. I’m a geneticist and love dwelling on the science, but Pfeffer wisely and accurately summarizes it in a preface, quickly getting to the human story. So I’ll do that too.

HD is one of more than 20 conditions caused by a triplet of specific DNA subunits (cytosine-adenosine-guanine, or CAG) that expands from generation to generation. Most people have 20 or so copies; in HD the number exceeds 40. Charise’s was 48. Genes encode specific proteins, and the too-long gene specifies a too-long protein, huntingtin, which gums up the movement center of the brain.

Genetic testing can detect the condition at any age, even well before the symptoms of uncontrollable movements begin. Testing isn’t typically advised until age 18.

A Gifted Writer

Charise was born in Niskayuna in 1980, and graduated from the Hebrew Academy of the Capital District, Bethlehem High School, and the University of Maryland.

She had a wonderful, if challenging, two years as a Peace Corps volunteer in Turkmenistan, where she taught English and self-esteem to children. She also taught children in Thailand and foster children in Israel, and traveled the world.

Charise was a gifted writer, which inspired her father to publish some of her work. Positivity and humor spring from her clever words.

As a senior in high school, “Fiddler on the Roof” inspired “If I Were a Couch Tater.”

If I were a couch tater,

Yai de dai da dai da daidle ya daidde dadde de

All day long I’ll piddly away the time

If I were a lazy girl.

In sharp contrast is “In the Dry Desert,” published in the 1997 Bethlehem High School literary magazine. An excerpt:

The ancient voices whisper.

Masada shall not fall again.

Hushed echoes of the prayers

Of those who sacrificed

Reverberate off the sharp, fitted edges.

The cracks the heated stone,

Like parched lips,

Speak.

Demand not to be forgotten.

With Alan and her art

An Ode to Odd, Uncontrollable Movements

The disease was once called Huntington’s chorea, named for the characteristic uncontrollable, repetitive, dance-like movements that include fidgeting, squirming, and jerking. Charise describes the mortification of misunderstanding chorea in “At a Restaurant in Lee,” written in  2014.

People stare at me

I want to glare back at them

But I’m too busy

Dropping my napkin

Picking it up

Dropping my napkin

And picking it up — again

And picking it up – again

Attempting to grasp this foreign fork and knife

As pithy pieces of lettuce

mockingly dancing and prancing,

Mexican jumping beans

they leap into the air and onto the counter

One after the other

One after the other

Could swear I just picked this one up

Damned leaves of lettuce all look alike,

without warning a flash flooding of sticky white fluid

splashes

into the bowl

everywhere

nearly falling off my chair

Drunk, they no doubt think

And I want to yell;

What are you looking at?

Lush

She’s had one too many

they no doubt think

I want to demand

Didn’t your momma ever tell you

It’s rude to stare.

Years later, Charise returned to the theme of presumed drunkenness, in a discussion with her rabbi. She related being in the Bahamas, loading her luggage onto a bus, weaving back and forth.

“Some guy looked at me, and started joking, ‘How many Bahama Mamas have you had?’”

“Another guy chimed in, ‘She’s three sheets to the wind!’”

She wished she’d said:

““HD affects your balance.

HD affects your cognitive and motor skills.

HD affects your judgment.

You’re treating me in a way that’s really disrespectful and embarrassing.”

Buenos Aires, 2009

Eloquent Capturing Her Plight

Charise was achingly self-aware. Her words bring the reader into her predicament.

At 32 I find myself trapped

There is no cage and yet every day

the bars grow stronger and stronger around me …

My swiss-cheese-holed memories

Through feelings and brains

As the pen slips through my grasp (yet again) wicked pen!

With cobra, Thailand

If her brain is a Swiss cheese, then her whole body is a tree, in the poem Deadining.

Delicately separating the vivid bright green

Thriving living leaves

From the dark decrepit ones

The ones that without a whimper

Without a whisper simply crumble into dust

A tougher task than one might imagine

Turning over the robust stems to reveal

Dark decaying summer

Hidden underneath

Foraging past glorious lush

Vibrant stems

Deceivingly healthy

On the outside

To reveal the mottled yellow-brown parts

Dying on the inside

Must be careful,

Nimble decisive in this endeavor

Attempting to save the precious purple and pink bulbs

As I must to myself am I more jealous of the plants who will live healthy lives

Or those who get to die quickly quietly simply and with dignity.

 

Buenos Aires, 2009

Public Outreach

Father and daughter chose not to hide HD, but to inform others, through public education and advocacy. “My greatest accomplishment was getting the State of New York to agree to pay a higher reimbursement fee under Medicaid for specialized nursing homes treating HD residents,” Pfeffer writes.

For many years he and a social worker trained sheriff’s offices, corrections officers, 911 offices, and police to recognize when a suspect might have HD. He was instrumental in supporting the Medical Aid in Dying (MAID) Act, which the New York State legislature passed in 2025. Charise’s advanced directives were crystal clear: no feeding tube, no antibiotics, no ventilators, and a DNR on file at the nursing home where she spent her final years.

The last entry in the book, “Hell Piece,” stays with me, in its simplicity and profundity.

Charise is a junior in high school, imagining the horror that is her mother’s life, but written in the first person, perhaps envisioning her own future.

In “Hell Piece,” a young woman sits propped up in her nursing home recliner in her room, watching people walk back and forth in the hallway under the constant fluorescent lights. An annoying man hawks a stainless-steel vegetable knife on the blaring TV, on and on. And then an inquisitive fly begins to torture her, as her body betrays her, rolling her head back and forth in the eerie repetitive motions of HD.

After lamenting the gross hairy legs of the buzzing fly, Charise imagines it landing on her trapped mother.

“I wish I had more control. The fly has landed on my thigh! I can see its horrid black front legs rub together in anticipating of biting my pale unsuspecting innocent skin. If only I could move my hand, I would curl my fingers into a fist and come down hard on that thing! Squish! Ugh! That would teach him a lesson, all right. Thankfully, the wretched beast takes off in search of a more ambulatory victim.”

Charise passed away on May 5, 2024, suddenly and peacefully, her heart stopping, before she became completely rigid and unable to move. Her father was thankful for that.

 

Huntington’s and me

I am a science writer and geneticist, with a curious connection to HD. My first ever newspaper article was about the disease. A clipping somehow found its way from Bloomington, Indiana, where I was pursuing my PhD, to Marjorie Guthrie, Woody’s wife. She wrote me a letter that I shoved into a back pocket, and when washing my jeans, I found it. She invited me to meet her the next time I was in NYC visiting my family, and I did – she and Arlo encouraged me to go into science writing.

In my first faculty position at Miami University in 1980, a student, Margaret Wallace, worked in my lab, and then went on to do her PhD research in Dr. Michael Conneally’s lab at the IU School of Medicine in Indianapolis. In 1983, very late one Tuesday night, she called me, excited. In going over data, she realized that her team had discovered the Huntington’s marker in blood samples that researcher Nancy Wexler and her team had collected from a huge family with HD in Venezuela. Having the marker made possible presymptomatic diagnosis and paved the way for discovery of the gene a decade later.

Then in 2014, I began volunteering for Community Hospice in Schenectady. My first patient was a man with HD who was the same young age as Charise when she passed away. I shared music with him on my iPod, nearly every day for his remaining months, he communicating with raised eyebrows to my yes-or-no questions. Shortly before he passed, trapped in his body, the movements ceased, I called Dr. Conneally to ask why my patient couldn’t close his eyes. “Ricki, he can no longer blink.”

A few years later, I worked for the Cure Huntington’s Disease Initiative Foundation, sitting in on private research workshops and writing up technical reports on the brainstorming discussions.

I was excited to review Alan Pfeffer’s book. It is available at Amazon.