Minto, N.D., man dies of extremely rare brain disease
Brian Misialek’s obituary starts like any other -- the Minto man died age 58, four days before Christmas, “in his home surrounded by his family.”
But the second line is more unexpected:
“Brian was diagnosed in October 2017 with Creutzfeldt-Jakob disease, a rare brain disease that affects only one in one million people,” it reads. “Brian was truly a one-in-a-million man.”
Misialek’s funeral was late last month, drawing a curtain across a tumultuous several months for his family. When he first began experiencing symptoms this summer -- leg pain and stumbling -- a neurologist wrote it off as a result of overindulgence in alcohol, something his wife denies. By the time he’d gotten a spinal tap and an accurate diagnosis, he’d had double vision, personality changes and hallucinations.
“We have to find a cure or a treatment for this disease, but we also have to educate providers about the symptoms of it so they don’t get misdiagnosed like Brian was,” Debbie Misialek, Brian’s wife, said last month. She encouraged donations to the Creutzfeldt-Jakob Disease Foundation, a group researching the disease.
The National Institute of Neurological Disorders and Stroke explains that, worldwide, the Misialek family’s math is right: The degenerative brain disease only afflicts one in 1 million people each year, and about 300 million in the U.S. It’s part of a family of diseases known as “transmissible spongiform encephalopathies,” which leave microscopic images of the brain deteriorated and reminiscent of sponges. The vast majority of cases are “sporadic,” which means they develop unexpectedly, while between 5 and 10 percent are thought to be genetic. Fewer than 1 percent of all cases are caused by transmission -- often through medical procedures.
It is, the NINDS says, “invariably fatal.”
If this sounds familiar, it’s because most people have heard of a similar condition: Creutzfeldt-Jakob disease is part of the same category of illnesses as mad cow disease. A variant of Creutzfeldt-Jakob can be contracted by consuming infected beef, according to the Mayo Clinic, but the there’s no observed link for “classic” Creutzfeldt-Jakob disease.
Michelle Feist, who helps track diseases and their spread for the North Dakota Department of Health, said there have only been a small number of cases in recent years. In a late December email, she said that from 2012 to 2016, only three such cases were recorded.
Debbie Misialek remembers meeting Brian in 1987, when she was working in Grand Forks with his brother. He was quiet, mature and “organized,” she said. They married in 1990 and have two adult children -- Alana, 24, and Logan, 21. Brian, enthusiastic about carpentry since he was 14, ran his own construction business for decades, which he only left as his health declined.
“He was a perfectionist, and he really struggled with people that didn’t have that passion that he did. He didn’t like crappy work -- he strove for quality of work, not quantity,” Misialek said.
The Misialek family is sprawlingly big -- so big that Chris Misialek, an uncle and an organizer of a benefit for Brian and his family is only three years older than him.
That benefit, held on Dec. 15 at the Minto Community Center, served nearly 700 dinners -- all for a nephew he remembers as by turn meticulous and good-natured. Chris Misialek recalls Brian driving a 1960s-era truck on the verge of falling apart, undeterred by its age.
“Every time he would haul shingles out to the dump, and he’d rub the top of it and say, ‘one more payment and it’s all mine,’ ” he recalled. “He was hoping it would make it to the dump.”
His wife remembers that relaxed confidence, too.
“He was just a laid-back, take-it-easy kind of guy,” she said.