In a Wisconsin village, the doctor makes house calls — and sees the rarest diseases on EarthPosted: Updated:
It is -5 degrees and a 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 and, with no health insurance and a modest income, they cannot afford to visit most doctors.
Enter their hero: James DeLine, once the lone doctor in the western Wisconsin village of La Farge, population 750.
When he became the village doctor in 1983, DeLine had no experience treating the Amish and no idea how crucial he would be to their well-being and his work. Today, nearly 20% of the doctor’s patients are Amish or Old Order Mennonite, part of a Christian population called Plain People. They are known for their separation from the modern world and strict adherence to a simple lifestyle and simple clothing.
Dr. DeLine is familiar with 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 their pain.
When he began his career as a doctor, he DeLine never imagined he would find himself with one foot planted solidly in medicine’s past and the other in its future.
Dr. DeLine collaborates with English and American geneticists who study some of the rarest diseases on the planet, some of which occur in very high levels amonst the Amish, Mennonites and other closed communities that do not allow marriages outside their clans to occur. Because of this, the likelihood of rare, disease-causing mutations can be rife in the communities, and will take root, passing from generation to generation.
It has taken DeLine and his staff years to gain the trust of Plain People, some of whom are wary of medicine and technology. These people do not visit the hospital or clinic often, because they are afraid that they will surrender the decision-making in their diagnoses 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; he has always viewed them as the ultimate decision-makers.
At first glance, Perry Hochstetler seems an unlikely candidate for a rare disease or a health problem of any kind. Work at the local sawmill and his family farm has given the 26-year-old father of two a lean muscular frame. Beneath the skin lies another story.
“He has the vasculature of an 80-year-old smoker,” DeLine says.
Hochstetler inhereited a genetic mutation that causes an illness most people (Amish or not) have never heard of: sitosterolemia. Only 100 cases have been described in the medical literature, but DeLine has 13 patients with the condition, including four of Hochstetler’s 10 siblings and their father.
The disease prevents the body from getting rid of lipids from vegetable oils and nuts, causing them to build up and clog the arteries.
Since diagnosing the disease, DeLine has treated Hochstetler with a cholesterol-lowering drug called Zetia.
Without diagnosis and treatment, Hochstetler could by now have suffered a heart attack, a trauma that Zetia can delay, though for how long is uncertain. Unfortunately, sitosterolemia has no cure.
“I’m not afraid,” he 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.”
The road to becoming a country doctor
A blizzard almost kept the doctor and village from their appointment.
It was February 1983. DeLine drove?his family?over hilly country roads, staring out the windshield into flurries and fearing?their car?might not make?it to La Farge.
DeLine had just completed his residency at the Wausau Hospital Center. Now, a 10-member committee of locals was recruiting him to fill La Farge’s vacancy for a doctor. The village had been without one for a couple of?years.
The doctor liked the friendly villagers, a welcome change from the suit-and-tie types he’d interviewed with in other places.
He was 28 years old?with a bad car, a growing family and $30,000 in unpaid student loans. The average salary for a family doctor in America was then around $80,000, enough to settle down and begin paying off his debt.
But the people of La Farge wanted DeLine — needed him. Their offer was considerably lower than the going rate: $20,000.
That would have to cover DeLine’s annual salary, the salary of an assistant to answer the phones and handle billing, plus all the clinic equipment and?expenses. .
DeLine took the offer.
Medical school was 'meant to be'
DeLine grew up in New Lenox, Illinois, a farming community outside Joliet.
The village of 1,750 consisted mostly of cornfields. DeLine remembers it as the kind of place where children grew up building forts during the day and watching bonfires at night. DeLine had twin sisters five years younger than him. Their father owned a restaurant.
From the time he was young, though, “it just seemed like I’d be going to medical school. It was meant to be.”
DeLine recalled nights when he could hear his mother struggling to breathe. He could hear his father, too, trying to persuade her to go to the hospital.
She had rheumatic heart disease and took blood thinners starting in her 30s. She sometimes joked about needing “a valve job.”
DeLine was 17 when his mother went in for the procedure.
He saw her once after surgery “but I didn’t like how she looked.”?After three days, his mother suffered cardiac arrest. Doctors were able to resuscitate her, but she had sustained a severe brain injury. Days later, the family shut off life support. She was 42.
One week after her death, James DeLine set out to become a doctor, leaving home for the University of Illinois in Urbana-Champaign.
A demanding schedule
University life was difficult. The death of his mother grieved him tremendously; he remained so mired?in it that when he ate, he suffered terrible stomach pain and had to lie on his belly for relief.
Still, he took on a demanding schedule. Driven students tended to enter the more advanced honors program in either chemistry or?biology. DeLine, a physiology major, enrolled in both.
He paid for college through restaurant jobs and financial aid.
He went on to medical school, first in Champaign, then at the University of Illinois campus in Chicago. He lived in the city’s Little Italy section on the near west side. There he met his future wife, Ann Doherty, who worked in a print shop.
DeLine graduated from medical school on June 7, 1980. The next day, he and Ann married.
A week later, he started his residency.
He would work a typical residency shift: 24-hour shift on, 24 hours off, then head back for another 24 hours at the hospital. “By the time I’d stagger home for some rest,” he says, “I was sleep-deprived, hungry, with a headache.”
The schedule bothered his wife. She missed him. In his next job, she would see even less of him.
On call 24 hours a day
In La Farge, DeLine worked harder than he had in his residency.
He was on call 24 hours a day, seven days a week. To make ends meet, both for his family and the clinic, DeLine would work five shifts a month at the emergency room at Vernon Memorial Hospital in Viroqua.
There were days he would work 9 a.m. to 5 p.m. at the clinic, then drive to the hospital and work 6 p.m. to 8 a.m. in the emergency room. He would return to the family’s home just in time to shower and get to the clinic by 9.
“There were times when he was tired, but it didn’t slow him down,” Marcia Bader, his now-retired office manager says. “It was that deep-seated caring that kept him going.”
It was his wife, Ann DeLine, too.
The woman who had dreamed of being a mother did everything for the couple’s four children, all born within a five-year span. She spent her days washing cloth diapers, hanging them out to dry, cooking, cleaning, watching over the children, helping with schoolwork and accepted with grace all the times when her husband was called away from holidays and birthday parties.
"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."
A fixture in the community
Villagers embraced their doctor because, unlike other doctors, he did not talk "at them."
In the early years, the clinic struggled financially. Sometimes, people couldn't pay their bills.
The doctor and his wife became fixtures of?community life. They went to their children’s cross country meets and other school events. They attended the annual Kickapoo Valley Reserve Winter Festival.
But it was his presence in the homes of area residents that endeared him to them.
“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.
“That was just unheard of. … 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.”?
The doctor's turn for illness
The villagers that Dr. DeLine serviced loved him, but worried about him, too. From time to time, rumors would spread about how the doctor was sick, maybe even dying. The rumors became fact when, in 2007, DeLine was diagnosed with prostate cancer.
Feeling, as he put it, “reflective, maybe anxious too,” DeLine approached the Epitaph-News editor. He asked to write a series of columns for the newspaper describing his illness and treatment. He would counter the rumors with transparency, and perhaps help people along the way. He titled the column, “From the Other Side.”
“I decided early on that I was comfortable sharing my experience with our community,” he wrote in the first column. “After all many of you have shared your concerns, fears and symptoms with me for nearly 25 years. … Each of us knows that our turn must come for illness and eventually death.”
He discussed his fears about surgery to remove his prostate —?“Would I be able to jog again?” He even shared the frustration of phoning to make a doctor’s appointment and going through endless computer prompts before reaching a live human voice.
His 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,” explains Howell-Sherman, the newspaper editor.
It’s been 12 years since DeLine’s surgery. The cancer has not returned.
Earning the trust of the Amish
Of all the relationships the doctor has built in La Farge, the most challenging involved his Amish patients.
DeLine found his medical work was affected by a deeply held principle among the Amish, expressed in 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 take a back seat?to the good of the community and God's will.
There are some in the Amish community who will visit a hospital and accept modern medicine as treatment for their ailments, but other prefer natural medical techniques such as herbs, vitamins and home remedies. Many of those patients will use those items before visiting Dr. DeLine, to potentially disastrous results.
Such was the case with Abie and Edna Yoder when their 8-year-old daughter, Barbara, first grew sick in spring 2015.
The girl had little appetite and suffered from a terrible stomachache and bloody diarrhea. Barbara weighed 38 pounds —?19 pounds below average for an 8-year-old.
The Yoders took her to a so-called “non-traditional doctor” used by some of the Amish, who didn't have a medical degree. That person looked at her blood under a microscope and told the family that she might have colon cancer.
The parents worried terribly about their daughter’s survival, but worried too about putting her in the hands of a traditional doctor. The scenario that haunted them had happened to a 3-year-old Amish boy with leukemia. The boy was given chemotherapy, they say, despite the excruciating pain and ultimately, the little boy died.
“He begged to be released to go to Jesus,” Edna Yoder recalls.
The Yoders approached a midwife, who sent her husband to speak with DeLine. The husband explained to the doctor the circumstances and the family’s hesitation. Then the Yoders brought their daughter.
"Dr. DeLine made it really clear that he would respect our wishes,”?Edna Yoder recalls.
Their daughter was admitted to American Family Children’s Hospital in Madison. DeLine consulted with a pediatric cardiologist he’d worked with at UW, Amy Peterson.
“Dr. DeLine had noticed that she had interesting looking bumps on her arms and on her legs,” Peterson recalls. “They were deposits of cholesterol. Dr. DeLine and I started thinking along very similar lines very quickly.”
Genetic testing confirmed their hunch. The girl had extremely rare sitosterolemia, the same illness that would later be diagnosed in Perry Hochstetler.
Treatment lowered the girl’s sitosterol levels and helped her gain weight.
DeLine and Peterson have since found among the local Amish a dozen other cases — the second largest cluster of the disease in the world.
Encountering nature's cruelest illnesses
Nearly 200 diseases are found in much higher proportions among Plain People. Scientists have developed a special Amish genetics test that can screen 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.
Across the globe, there have been only 20 to 30 cases of a disease called BRAT1; DeLine has seen six in the Amish community. Babies with the illness are born rigid and are prone to frequent seizures.
“When the baby is born you can’t straighten the baby,” DeLine says. “The eyes are jerking, face twitching. Some moms say they have felt things that suggest the babies might have been having seizures while still in the womb."
There is no cure for BRAT1. Afflicted babies will die within months. “But if we can identify it,” DeLine says, “the family can take the baby home and they take great care of the baby until the baby passes away, and they don’t spend five farms' worth of money.”
Help from geneticists from England
On another spring morning, 50 Amish and Mennonites gather at the La Farge Community Temple, a former Masonic Temple a few blocks from the clinic.
DeLine?and his staff have brought together families with similar illnesses to hear from two of the clinic’s scientific collaborators studying these conditions: Emma Baple and Andrew Crosby, both geneticists from the University of Exeter in England.
To date, Baple and Crosby have identified 75 conditions that were new to medical science, of which 30 are found in higher levels in Amish communities than non-Amish communities. In some of the cases, research into these rare diseases has reached the point where scientists are beginning to come up with potential therapies.
“Our role is to support him and get answers for those families," Baple says. "Our great hope is that we can find something to mend or ameliorate the condition.”
The Amish are appreciative that, although there are no cures for the diseases discussed at the meeting, there is no longer a mystery as to what ails their family members.
“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) who came to the meeting from Fairchild, a village of 550 more than 90 miles to the north. “We were just kind of up against a brick wall.”
Yoder’s son, Simon, one of 10 children in the family, is colorblind and experiences tunnel vision. The Yoders tried fitting him with eyeglasses, but the child’s sight continue to deteriorate.
DeLine and his staff took blood samples three years ago when Simon was 14 and, within two weeks, the child was diagnosed with Jalili syndrome. His younger brother Moses also has the disease. DeLine and his colleagues have found four others who have the illness,?the first cluster of Jalili patients ever discovered in the United States.
John Yoder found the news that Simon and Moses had inherited the illness troubling.
“It kind of changed my opinion on marrying too close,” he says. “Me and my wife are actually related to each other. We’re second cousins. It happens a lot among the Amish.”?
Lessons from the Amish
Over the years, DeLine and his staff have discovered that Amish families view birth and death quite a bit differently from the rest of the population.
Amanda DeVoogdt, a midwife in St. Paul, Minnesota before coming to work at the La Farge Clinic, says her first Amish birth four years ago was strikingly different from any she’d seen in the city.
“I’m used to giving a lot of verbal and emotional support during labor, talking, massaging,” she 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. … They are self-sufficient in their lives so much, and that carries over to labor.”
There are no bright lights in the birthing room. The women do not wish for epidurals or pain management.
“The mothers are in a state of deep restfulness,” DeLine says. “It’s a wonderful thing to observe. It’s very important to help women to get to that place.”
The doctor says the same sense of serenity shapes the way Amish accept bad news,?even death.
Months ago, he called on an older man dying from a lung condition. DeLine sat by his bedside and asked if the man wished?to go to the hospital.
“I think I’d prefer to go to heaven,” he replied.
Four hours later, the man died.
“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.”
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