Glenorah Helfrich prefers the Latin name for the rare disease that clutches her with pain from globs of fat called lipomas that form beneath her skin, robbing her of energy and strength.
Adiposis dolorosa is “much prettier,” Helfrich said, than “Dercum’s disease,” a syndrome discovered 125 years ago that continues to stump specialists and defy accurate diagnosis.
Dercum’s has neither a known cause nor cure and by some accounts is among the 10 rarest diseases on Earth. Some suspect Dercum’s is more common than perceived but is often misdiagnosed or not diagnosed at all.
Helfrich, 67, doesn’t really know how long she has been afflicted. In 2008, she began having trouble lifting her arms. Within six months after moving from Afton to Columbia to place her husband, Tom, in a local nursing home, rapid weight gain caused her to go from a size-14 to a size-24 dress.
“You have no idea what that does to a woman’s ego,” she said. Forty pounds in six months. “Even Tom noticed that I was gaining weight. And he didn’t notice anything.” She noted the irony in light of her husband’s worsening dementia and his lifelong indifference to hairstyle and makeup.
And there was pain, always the pain, the most universally debilitating symptom of the disease because the lipomas either rest on or press against nerve fibers. In one type of Dercum’s, the lipomas themselves are painful.
Noted Dercum’s researcher and physician Karen Herbst, whom Helfrich visited in May in San Diego, said the best way to understand the pain is to imagine what it’s like when a joint or a muscle hurts. Similarly, with Dercum’s, the fat tissue — and there’s a lot because the disease creates more of it — can be painful.
“Most normal people don’t feel their fat,” Herbst said in a phone interview. “I just don’t think that people understand that in Dercum’s, there’s actually pain in the fat tissue itself.”
A Facebook network of people with Dercum’s from around the world tells the story again and again about the frustrating path to diagnosis and perfunctory medical exams that lead to a common conclusion: “You’re fat. You need to eat better and exercise more.”
Helfrich had the same unhappy experience — a carousel of doctors and specialists, misdiagnoses and misunderstanding, until finally in the fall of 2012, a Columbia dermatologist mentioned Dercum’s.
Looking back, evidence that she had the disorder had been present far earlier than 2008. Those nodules behind her ears, especially that one that pressed on her ear canal, when did they first show up?
Other, more pressing matters at the time had demanded her energy.
In 1999, Tom began having difficulty walking, his handwriting changed and a series of small strokes exacerbated the onset of dementia and erased any recollection of regular falls and missteps that left bruises and broken bones. Helfrich began a 4½-year mission of becoming a thorn in the side of the Department of Veterans Affairs, insisting her husband’s condition was caused by something he encountered during his military service in Vietnam.
Also, before her own piercing pain finally won her attention, Helfrich watched her mother battle cancer. Twice.
Eventually, she made the hard decision to place Tom in a nursing home, where he could get qualified, professional care. She found a way to pay the $4,000 monthly bill until Veterans Affairs stepped in — after bankruptcy — and acknowledged a connection between Tom’s dementia and Agent Orange.
Tom Helfrich was 56 in 2003 when she took him to the nursing home in Festus, one week after spending Thanksgiving with him and other family members. She told him about her decision just before the holiday. “I didn’t know how to tell him. I knew he would be angry,” she recalled. “I knew he would be hurt.”
At that point, a silent scream began to grow within her. It forced Helfrich to mumble when she spoke and to take small bites when she ate, careful to chew ever so slowly, afraid that if she opened her mouth wider the scream might escape.
And then it did.
It was her own anguished, soul-deep reaction to that awful day when she moved Tom into the nursing home.
“It felt like leaving a little puppy at the door,” she said. “Nobody should ever have to go through that with their spouse.”
With tears flowing, Helfrich went to a movie — “I couldn’t go home” — then decided she was out of tears.
“I haven’t cried since,” she said.
Suppressing the guilt she felt for turning Tom over to caregivers and trudging through her own loneliness seemed unbearable.