USA TODAY International Edition
Country doctor at genetics’ cutting edge
Physician deals with rare diseases in Wisconsin Amish community
It is 5 degrees below zero, and a light powdering of snow swirls across the roads of Vernon County, Wisconsin. A few horses and buggies clop through the chill morning air, but Perry Hochstetler leaves his buggy at the family farm and has a driver take him to his doctor’s appointment.
The Hochstetlers are Amish. With no health insurance and a modest income, they can’t afford most doctors. They can afford James DeLine, once the lone doctor in the village of La Farge, pop. 750.
About 20% of the doctor’s patients are Amish or Old Order Mennonite, part of a Christian population known for their separation from the modern world and adherence to a simple lifestyle and unadorned dress.
Something of a throwback himself, DeLine, 65, is a short, bespectacled man with a walrus mustache, a doctor who carries a brown medical bag to house calls. For years, he carried his equipment in a fishing tackle box.
He knows the families on every local farm and their medical histories. He knows who’s been born and calls on the mothers and infants to make sure they are healthy. He knows who’s dying and looks in on them in their final days, sitting by their bedside, talking in a gentle voice, making sure they have what they need for pain.
When he came to La Farge more than three decades ago, DeLine never imagined he would find himself someday with one foot planted solidly in medicine’s past, the other in its future.
The doctor who makes house calls also collaborates with English and American geneticists studying some of the rarest diseases on earth. Some occur at much higher levels among the Amish and Mennonites, communities that don’t allow marriage to outsiders. This prohibition increases the likelihood that disease- causing mutations will pass from generation to generation.
It has taken DeLine and his staff years to gain the trust of Plain People. Many fear that going to a hospital or clinic will mean surrendering the decision- making to doctors who neither respect their beliefs, nor understand their financial limitations.
DeLine, not a religious man himself, accommodates the beliefs of patients and parents.
Hochstetler, 26, a father of two, has a lean muscular frame, but beneath the skin lies another story. “He has the vasculature of an 80- year- old smoker,” DeLine says.
Hochstetler inherited the genetic mutation that causes an illness most people have never heard of: sitosterolemia. There have been only 100 known cases, but DeLine has 13.
The disease prevents the body from getting rid of lipids from vegetable oils and nuts, causing them to build up and clog the arteries. Without diagnosis and treatment, Hochstetler could have suffered a heart attack. There is no cure for sitosterolemia.
“I’m not afraid,” Hochstetler says. “If I die young, I guess I’m going to die young. I can’t do much about it. I can’t say I ever get low and have the blues about it.”
‘ We’re not fancy people’
A blizzard almost kept the doctor and village from their appointment.
In February 1983, DeLine drove his family over hilly country roads, fearing their car might not make it to La Farge. The green 1972 Dodge Coronet was, he says, “a miserable piece of junk with bald tires.”
A committee awaited in the village, hoping to convince him to fill the vacancy for a doctor.
In the passenger seat sat the doctor’s wife, Ann, who was pregnant; in the back, their 15- month- old son, Michael. DeLine turned to his wife and said they would have to find the nearest town where they could buy snow tires.
After arriving in La Farge, the DeLines dined with committee members. The doctor liked the villagers. He and his wife had grown up in small towns. “We’re not fancy people,” he said.
DeLine was 28 years old with a bad car, a growing family and $ 30,000 in unpaid student loans.
La Farge’s offer: $ 20,000, which would have to cover the salaries of the doctor and an assistant and all other clinic expenses.
DeLine loved the landscape. He imagined life as the village doctor. “I think I liked the idea of building something out of nothing,” he says.
DeLine and his wife agreed to take the offer.
At his house near the clinic, DeLine has kept the same photograph taped to the refrigerator for 30 years: a portrait of a doctor in white surgical scrubs leaning against a counter, cup of coffee in one hand, cigarette in the other.
The photograph, taken by W. Eugene Smith and published in Life magazine, helped tell the story of Ernest Ceriani, the lone doctor in Kremmling, Colorado, whose experiences reminded DeLine of his own.
“During the first five or 10 years,” he says, “I did all my own night work and emergency work, rarely using the emergency room. So when a patient developed abdominal pain in the night, I would do what assessment I could at their home or at the clinic, ride with them in the ambulance if needed, admit them to the hospital in Viroqua, administer pain medicine.”
DeLine was on call 24 hours a day, seven days a week. He also worked five shifts a month in the emergency room at Vernon Memorial Hospital in Viroqua.
“It was that deep- seated caring that kept him going,” says Marcia Bader, who worked at the clinic for 31 years before retiring.
It was Ann DeLine, too.
She did everything for the couple’s four children and accepted with grace all the times when her husband was called away from holiday and birthday celebrations.
“The calendar of holidays does not apply,” she says. “He helps people when they need him – like the volunteer fireman races off when the alarm sounds; like the farmer plants and harvests when the ground and weather are ready.
“Life is lived by needs, not calendars and time slots.”
Villagers embraced their doctor. Patients said they were accustomed to physicians who talked at them most of the time; DeLine listened.
The clinic struggled financially in the early years. “Not everybody paid their bills,” Bader recalls. “But the doctor wasn’t going to send them to collection firms, and he wasn’t going to stop caring for them.”
DeLine has taken in enough money to sustain the La Farge Medical Clinic. In 2003, it was purchased by Vernon Memorial Healthcare, 15 miles west in Viroqua. DeLine works with two other doctors in La Farge and a total staff of 25.
The new ownership, the expanded staff, the addition of electronic records – these have not changed the kind of medicine DeLine practices.
“My father was diagnosed with colon cancer in 1994. The thing that always struck me was that Dr. DeLine stopped in to see my mom and dad one night after a basketball game,” recalls Bonnie Howell- Sherman, editor and publisher of the weekly Epitaph- News in nearby Viola. “My mom is going through dementia now and out of all of the people she’s met since she’s been here, he’s the one she remembers.”
Villagers worried about DeLine. At times, rumors spread that the doctor was sick, even dying.
In 2007, it was more than a rumor. He was diagnosed with prostate cancer.
Feeling “reflective, maybe anxious too,” DeLine asked the newspaper editor if he could write columns describing his illness and treatment. “I decided early on that I was comfortable sharing my experience with our community,” he wrote in the first column. “Many of you have shared your concerns, fears and symptoms with me for nearly 25 years.”
The columns took readers through his surgery, recovery and return home.
The way the whole village shared the doctor’s illness and treatment, “that’s part of small- town life,” Howell- Sherman says.
Twelve years after DeLine’s surgery, the cancer has not returned.
A higher authority
Of all the relationships the doctor built in La Farge, the most challenging involved his Amish patients.
DeLine found his work affected by one of their deeply held principles expressed by the German word gelassenheit, which means yielding oneself to a higher authority. Among the Amish, the word encompasses a calmness and patience, as well as a belief that individualism must give way to the good of the community and the will of God.
Though some Amish visit hospitals and accept modern medical techniques, others prefer natural methods and home remedies. It is not unusual for an Amish family to turn to these methods before deciding to see DeLine.
Such was the case with Abie and Edna Yoder when their 8- year- old daughter, Barbara, grew sick in 2015.
The girl had little appetite, terrible stomach aches and bloody diarrhea. Her weight dropped to 38 pounds – 19 pounds below average for her age.
A midwife gave the Yoders an herbal medicine. A “nontraditional doctor” told the family she might have colon cancer.
The Yoders worried about putting their girl in the hands of a traditional doctor. They were haunted by the case of a 3- year- old Amish boy whose leukemia was treated with chemotherapy.
“To see her leave the world,” Edna recalls, “would have been easier than to see her suffer on and on and on.”
The Yoders approached a midwife, who sent them to speak with DeLine.
“Dr. DeLine made it really clear that he would respect our wishes,” Edna Yoder says.
Working with a colleague, DeLine discovered the girl had sitosterolemia – the same disease that would later be diagnosed in Hochstetler. Treatment lowered her sitosterol levels and helped her gain weight.
DeLine and the colleague have since found among the Amish a dozen other cases — the second- largest cluster of the disease in the world.
Almost 200 diseases are found in much higher proportions among Plain People. Scientists developed a special Amish genetics test that screens the blood for more than 120 of them.
DeLine has seen patients with more than 30 of the diseases on the test and has at least two patients with diseases never described in medicine.
One of the rare diseases DeLine has encountered, SNIP 1, causes severe developmental disabilities, seizures and other symptoms. Only 34 cases of the incurable disease have been identified worldwide; DeLine has seen nine.
Across the globe, there have been only 20 to 30 cases of a disease called BRAT1; DeLine has seen six.
Some of the children in the community suffer from an illness called Jalili syndrome, which leads to blindness and a loss of tooth enamel. It has been found in only a few families worldwide.
Although doctors cannot cure the diseases, Amish families say they are glad to have a diagnosis.
“We didn’t know what our children had until we took them to La Farge,” says John Yoder, a farmer unrelated to Abie and Edna Yoder.
Yoder’s son Simon, one of 10 children in the family, is colorblind and experiences tunnel vision. When the boy was 14, DeLine diagnosed him with Jalili syndrome. His younger brother Moses also has the disease.
“It kind of changed my opinion on marrying too close,” John Yoder says of the diagnosis. “Me and my wife are actually related to each other. We’re second cousins. It happens a lot among the Amish.”
DeLine and his staff have learned that Amish view birth and death in ways that differ from much of the population.
“I’m used to giving a lot of verbal and emotional support during labor, talking, massaging,” midwife Amanda DeVoogdt says. “I was kind of doing the same thing, and the Amish woman looked up at me and said quietly, ‘ Shhh.’ It’s a lot quieter.”
DeLine says the same sense of serenity shapes the way Amish accept bad news, even death.
Months ago, DeLine sat by the bedside of an older man dying at home from a lung condition and asked if the man wanted to go to the hospital. “I think I’d prefer to go to heaven,” the patient said. Four hours later, the man died. The doctor turned 65 in September and hopes to keep practicing until 70. DeLine’s patients, especially the Amish, have taught him much about health care.
“We – the Amish and I – come at it from different insights,” he says, “but the end result is the same. We must do our best in every situation, but we cannot expect that all things will go the way we would wish. So, we must come to acceptance.”
“The calendar of holidays does not apply. He helps people when they need him – like the volunteer fireman races off when the alarm sounds; like the farmer plants and harvests when the ground and weather are ready. Life is lived by needs, not calendars and time slots.” Ann DeLine