After the laughter

Ask anyone who knew my oldest brother, Corey, and without fail, they’ll mention his laugh. It was one of a kind, a tittering chuckle that came easy and often, sometimes escalating to fits of mirth. I first realized something was seriously wrong with him when he stopped laughing.

We were at breakfast the morning after his daughter, Cassie, got married in 2010. Corey lived in Washington and we saw each other only a few times a year. He hadn’t seemed himself that weekend. I’d chalked it up to worry over the wedding until my sister pulled me aside that morning and said, “Watch Corey. Something isn’t right.”

We all tried to engage Corey, asking questions and relating funny memories, but he’d respond only with a pained grin and uncharacteristically empty laugh. The confusion in his eyes suggested he was struggling to understand our conversation. He tried to play along, either unaware something was amiss or, for our sake, pretending it wasn’t.

A few months later—at the age of 50—Corey was diagnosed with early onset dementia resulting from a degenerative brain disorder called Lewy body disease (LBD), also known as Lewy body dementia, a condition that eventually would take his life.

Like most people, my family had never heard of LBD, which is caused by abnormal deposits of proteins—known as “Lewy bodies”—in the brain. It’s relatively unheard of but hardly rare, as it affects an estimated 1.3 million Americans and is the second leading cause of dementia behind Alzheimer’s disease. That’s according to the Lewy Body Dementia Association, a national organization dedicated to raising awareness about the illness, which also notes on its website that many cases go undiagnosed because even doctors remain woefully unfamiliar with the illness. LBD is often misdiagnosed as Alzheimer’s or Parkinson’s disease, and can co-occur with those conditions.

Like those illnesses, there is no cure; symptoms may be slowed in some cases, but never stopped. Further complicating treatment is the fact that some medications commonly used to treat other forms of dementia can actually speed up LBD’s progress.

Beyond dementia, the illness also causes visual and aural hallucinations and progressive loss of cognitive and physical abilities. Life expectancy from the time of diagnosis is roughly six to eight years, because the disease doesn’t just rob those afflicted of their memory, personality and mental capacity, but also eventually affects the most basic bodily functions. After victims forget how to walk and talk, their bodies forget how to breath or swallow. As the body falters, it also becomes incapable of fighting off other illnesses. My own brother’s death last summer was hastened by a respiratory infection.

It’s hard to explain Corey’s cause of death to most people, given that the most common response when I mention Lewy body disease is, “Louie who?” I usually try to explain by relating it to the death of another man also remembered for laughter, albeit on a much wider scale—Robin Williams.

The comedian’s 2014 death spurred some dialogue about anxiety, depression and suicide, for which I am thankful. However, I also am frustrated by the missed opportunity to inform more people about LBD, which autopsy reports show Williams suffered from.

His widow, Susan Schneider Williams, has since dedicated herself to spreading awareness about the disease. She joined the board of directors of the American Brain Foundation, which advocates for those afflicted with LBD and other disorders, and in recent months has traveled the country speaking about her late husband’s illness. She also penned an essay published in the journal Neurology last month, “The terrorist inside my husband’s brain.”

In the article, Susan describes Robin’s rapid deterioration in heart-wrenching detail, and relates many of the frustrations common to LBD victims and their loved ones— incorrect and insufficient diagnosis, negative reaction to medications, and a feeling of utter helplessness.

“Robin was growing weary,” she writes of his final days. “The Parkinsonian mask was ever present and his voice was weakened. His left hand tremor was continuous now and he had a slow, shuffling gait. He hated that he could not find the words he wanted in conversations … At times, he would find himself stuck in a frozen stance, unable to move, and frustrated when he came out of it. He was beginning to have trouble with visual and spatial abilities in the way of judging distance and depth. His loss of basic reasoning just added to his growing confusion.”

The last time I saw Corey was on his 55th birthday. He’d been in a full-time care facility for the past few years and his condition had deteriorated to the point that his children were considering hospice and end-of-life care. My surviving siblings, Chris and Kim, made the trip with me to say goodbye.

Corey had long lost the ability to laugh, walk and talk or to feed or dress himself. I like to believe the briefest moments of recognition I’d detected on his face were real rather than wishful thinking, but I can never know for sure. By the time he took his final breath one month later, I was devastated, but found solace in the fact my long-tortured brother was free of his broken vessel.

A few months after Corey passed, I shared his story with my own doctor. He asked if I wanted to be screened for LBD, to which I replied, “Is that possible?” He seemed surprised by his own answer: “I have no idea.”