In a rural Wisconsin village, the doctor makes house calls and sees some of the rarest diseases on Earth – Milwaukee Journal Sentinel

It is 5 degrees below zeroand a light powdering ofsnow swirls across the roads of Vernon County.Afew horses and buggies clop through the chillmorningair, but Perry Hochstetler leaves his buggy at the family farmand has a driver take him to his doctors appointment.

TheHochstetlersare Amish. With no health insuranceanda modest income, they cannot afford most doctors.

They can afford James DeLine, once the lone doctor in the western Wisconsin village of LaFarge. Population 750.

When he became the village doctor in 1983, DeLine had no experience treating the Amish and no idea the crucial role they would play in his work. Today, about 20% of the doctors 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 adherence to a simple lifestyle and unadorned dress.

Something of a throwback himself,DeLine, 65, is a short,bespectacledman 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 whos beenborn,andcalls on the mothers and infants to make sure they are healthy. He knows whos 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.

Before he goes on vacation, DeLinetakes out ads in the weekly newspaper so that his patients willknow.

As a young doctor,DeLine never imagined he would find himselfsomedaywith one foot planted solidly in medicines past, the other in its future.

The doctor who makes housecallsalso collaborates with English and American geneticists studying some of the rarest diseases on Earth. Some occur at much higher levels among the Amish, Mennonites and other closed communities that dont allow marriage to outsiders. This prohibition increases the likelihood that when a rare, disease-causing mutation appears in the community, it will take root and pass from generation to generation.

Scientists use a special term, the founder effect, to explain how some variations in the human genetic sequence appear more often in groups that are geographically or culturally isolated.

It has taken DeLine and his staff years to gain the trust of Plain People, some of whom are wary of medicine and technology.Often,theyfear that going to a hospital or clinic will mean surrendering the decision-making to doctors who neither respect their beliefsnor 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. As a result, the clinic has become a magnet for Plain People. Some travel eight hours from Missouri or Iowa just to see him.

We have to pay our own hospital bills, so we kind of shy away from big hospitals as much as we can, Hochstetler says. Ive known Dr.DeLinefor a long time. Mydadsworked with him. Hes always looking for a way to get the cost down.

At first glance, 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,DeLinesays.

Although he does not smoke, Hochstetlers blood vessels are so blocked that if I lift heavythings,I just cant get my breath, he tells the doctor. At one point, it got so bad I could barely walk.

He inherited the genetic mutation that causes an illness most people 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 Hochstetlers 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. A normal level of sitosterol is no higher than 5; three years ago, Hochstetlers level topped 250.

Since diagnosing the disease,DeLinehas treated Hochstetler with a cholesterol-lowering drug called Zetia. His sitosterol level has dropped to 59. Treatment has also melted away another symptom, the bulbous yellow growths called xanthomas that jutted out from the farmers elbows and knees.

Without diagnosis and treatment,Hochstetlercould by now havesuffereda heart attack, a trauma that Zetia should delay, thoughfor how long isuncertain. There is no cure for sitosterolemia.

While DeLine explains his progress and prognosis, Hochstetler remains calm, even jovial.

Im not afraid, he says. If I die young, I guess Im going to die young. I cant do much about it. I cant say I ever get low and have the blues about it.

Amish farms are clustered together along Highway D between Cashton and La Farge.Mark Hoffman / Milwaukee Journal Sentinel

A blizzard almost kept the doctor and village from their appointment.

It was February 1983. DeLine drovehis familyover hilly country roads, staring out the windshield into flurries and fearingtheir carmight not makeit to LaFarge. The green 1972 Dodge Coronet was, he says, a miserable piece of junk with bald tires.

DeLinehad just completed his residency at the Wausau Hospital Center. Now, a10-membercommitteeof localswas recruiting him to fill LaFargesvacancy for a doctor. Thevillage had beenwithout one for a couple ofyears.

In the passenger seat sat the doctors wife, Ann, who was pregnant; in the back, their 15-month-old son, Michael. The snow kept falling and thecar struggled up each hill. When it took five minutes to climb one, DeLineturned to his wife and said it was no use. They would have to find the nearest town where they could buy snow tires.

Later that day, the Coronet, rolling on new tires, finally arrived in LaFargeand theDeLinesdined with the recruitment committee at the home of one of the members.

The doctor liked the friendly villagers, a welcome change from the suit-and-tie types hed interviewed with in other places. He and his wife had grown up in small towns. Were not fancy people, he said.

But asidefrom a library, he saw little in the center of LaFarge just a burned-out building next to a bar. Driving back to Wausau after dinner,DeLinewondered aloud about the villages recruitment effort: How are they ever going to find anybody?

He was 28 years oldwith 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 beginpaying offhis debt.

But the people of LaFargewantedDeLine needed him. Their offer: $20,000.

That would have to coverDeLinesannual salary, the salary of an assistant to answer the phones and handle billing, plus all the clinic equipment andexpenses. The clinic itself was an empty, dilapidated building with orange carpeting on some of the wallsand a couch that looked like it had been sitting in someones garage.

DeLine took the offer.

At his house a few minutes drive from the clinic,DeLinehas kept the same photograph taped to the refrigerator for the last 30 years: a portrait of a heavy-eyed doctor in white surgical scrubs leaning against a counter, cup of coffee in one hand, cigarette in the other.

The photograph, given to him by a favorite medical student, was taken by the legendary W. Eugene Smith and published in a Life magazine photo essay from 1948: Country Doctor. The photos told the story of ErnestCeriani, the lone doctor inKremmling, Colorado, population 2,000 about three times the size of LaFarge.

Other photographs in the essay showedCerianiwalking across a field with his black medical bag, helping carry the stretcher bearing a ranchers son whod been thrown from a horse, and administering morphine in a car to a woman stricken by a heart problem.

But its the photo of an exhaustedCerianiclutching a cup of coffee that best capturesDeLineslife as a country doctor.

The photo of country doctor Ernest Guy Ceriani, made famous in a groundbreaking Life Magazine photo essay by W. Eugene Smith, hangs on James DeLine's refrigerator door at his home in La Farge.Mark Hoffman / Milwaukee Journal Sentinel

During the first five or 10 years,DeLinesays, I did all my own night work and emergency work, rarely using the emergency room.Sowhen 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.

If it was chest pain, Id bring my own EKG machine and cardiac defibrillator, start an IV and run infusions of medication in the back of the ambulance.

These days it is rare to find a doctor like Ernest Ceriani or James DeLine.

The mandatory use of electronic health records that got rid of the country doctor as we knew it,saysTherese Zink, author of the 2010 book, "The Country Doctor Revisited: A Twenty-First Century Reader," and formerly a doctor in tiny Zumbrota, Minnesota. Adopting an electronic health form system is not a small investment.

These changes come out of huge health care systems like Kaiser Permanente, says Byron Crouse, who retired last September from his job as associate dean for rural and community health at the University of Wisconsin-Madison. How do you scale that down to a small, rural practice?

In some cases, the cost of installing an electronic medical records system has forced country doctors to affiliate with hospitals or larger clinics. But the business model at such places discourages house calls. A doctor can treat three or four people in his office in the time it takes to drive out to a patients farm.

Over the years,DeLinemanaged to take in enough money to sustain the LaFargeMedical Clinic. In 2003, the clinic was purchased by Vernon Memorial Healthcare, 15 miles west in the town of Viroqua. Today he works with two other doctorsin LaFargeand a total staff of 25.

The new ownership, theexpandedstaff, the addition of electronic records these have not changed the kind of medicine DeLine practices.

Hes maintained the Norman Rockwell bedside manner skills, and yet hes working on the cutting edge of 21st-century medicine, the very newest science, Crouse says. I cant think of anybody else whohas that ability.

Four years ago, DeLines clinic opened its Center for Special Children, which focuses on the diagnosis and treatment of children born with rare genetic and metabolic diseases. Many, but not all, of the centers children come from AmishorMennonite families.

At least two-thirds of the money needed to run the center comes from benefit auctions at local barns, where handmade Amish quilts and furniture are auctioned off,along with flowers, maple syrup and other goods. Much of the remaining money comes from corporate donors.

And some comesfrom the doctor himself.

At an auction in June, hundreds of locals filled a barn innearbyCashtonand there stood DeLine, bidding away, accumulating five brick-sized blocks of cheddar cheese, a broom, baskets, flowers, a rocking chair, a handmade wooden toy and 300 pounds of beef.

All but the beef, the broom and one block of cheese wentto the clinic.

Country doctor James DeLine talks about his work with the Amish

In 33 years at the La Farge clinic, Dr. James DeLine has gained the trust of many Amish. He understands their beliefs and their financial limitations, and he leaves the medical decisions to the families.

Mark Hoffman, Milwaukee Journal Sentinel

DeLinegrew up in New Lenox, Illinois, a farming community outside Joliet.

The village of 1,750 was mostly cornfields. DeLine remembers it asthe 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 age of 13,DeLineworked at the restaurant six days a week, doing everything from washing dishes to cooking.

I dont remember much of high school, he says.

From an early age, though, itjust seemed like Id be going to medical school. It was meant to be. Although he remembers no single point when he first considered becoming a doctor, the idea may have crystallized at a crucial moment between childhood and adulthood.

DeLineremembers 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.

DeLinewas 17 when his mother went in for the procedure.

He saw her once after surgerybut I didnt like how she looked.About the third day, his mother suffered cardiac arrest. She was resuscitated but had sustained a severe brain injury. Days later, the family shut off life support. She was 42.

One week after her death, JamesDeLineset out to become a doctor,leavinghome for the University of Illinois in Urbana-Champaign.

Physician James DeLine eases into his work day starting at 5 a.m. at his home in La Farge.Mark Hoffman / Milwaukee Journal Sentinel

University life was hard.DeLineremained so miredin grief that when he ate, he suffered terrible abdominal pain and had to lie on his stomach for relief.

Still, he took on a demanding schedule.Driven students tended to enter the more advanced honors program in either chemistry orbiology. 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 citys Little Italysection on the nearwestside. There he met his future wife, Ann Doherty, who worked in a print shop.

Doherty was not a woman who liked touching up her face with makeup, not even for her engagement photograph. She was comfortable with who she was, a quality DeLine found attractive. She already knew what she wanted most in life: to be a mom.

Previously, shed been employed by theCatholic Worker house,a social justice program in Chicago. Like DeLine, her sympathies lay with the underdog.

DeLinegraduated from medical school on June 7, 1980.

The next day, he and Ann married.

A week later, he started his residency in Wausau.

To say I lived at the hospital would be a bit of an overstatement, he recalls, but not much.

He would work a 24-hour shift, take 24 hours off, then head back for another 24 hours at the hospital. By the time Id 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.

Physician James DeLine checks on Dean Pease at Vernon Memorial Healthcare in Viroqua. Pease was admitted to the hospital for breathing difficulties.Mark Hoffman / Milwaukee Journal Sentinel

Brad Steinmetz, one of the villagers on the LaFargerecruitment committee, remembers watching theDeLinesdrive off following thedinnerand thinking, Well never see him again.

Attracting doctors to rural areas has always been a challenge and remains so.

In 2018, the health care company Merritt Hawkins reported that roughly 65 million Americans now live in areas deemed to have a shortage of primary care doctors; two-thirds of those areas are rural.

One reason for the shortage: Young doctors are often steered towardthe kind ofmedical specialtiespracticed in larger hospitals. Obstetrics, cardiology, orthopedic surgery and other fieldspay more and allow physiciansto focus on one set of skills.

Rural medicine is womb to tomb, says William J. Crump, who had rural practices in Texas and Kentucky, and now serves as associate dean of the University of Louisville School of Medicine, Trover campus. Crump enjoyed the variety of country medicine.

I couldnt imagine not delivering babies, he says. I couldnt imagine not caring for babies. I couldnt imagine not helping people die a good death.

La Farge faced an additional challenge in attracting a doctor. Vernon County, where the village lies, depended largely on farming and lumber and had one of the states highest rates of children in poverty about 30% in 1980 and about the same today. DeLine wondered how he would ever build a practice in such an impoverished area.

But he found the landscape breathtakingly beautiful snow as clean as cotton blanketingwoodedhills anddeepvalleys, land dotted with white churches and faded red barns.

The young doctor couldnt get the place out of his head. At night, he dreamed of LaFarge. He imagined life as the village doctor.

I think I liked the idea of building something out of nothing, he says.

He and Ann agreed he would take the job.

James DeLine holds a 1982 photo of him holding his then-infant son, Michael, at Vernon Memorial Healthcare in Viroqua.Mark Hoffman / Milwaukee Journal Sentinel

In late June 1983, four months after the recruitment dinner, James and AnnDeLinearrived in LaFarge, still driving the old Coronet. In addition to 18-month-old Michael, they now had 3-day-old, Benjamin.

We looked a little like the Beverly Hillbillies,DeLinesays.

The family rented a little house across from the village school, 900 square feet for the four of them, $180 a month. Our rent never changed, he says, We saved and saved and saved.

The clinic needed significant work before it could open. TheDeLinesand volunteers from the village spent weeks refurbishing, scrubbing and painting. They reupholstered the old waiting room and stripped most of the orange carpeting from the walls.

The doctor also took a two-day course in Chicago on startingapractice, which offered guidance on insurance, bookkeeping, office layout and personnel management.

A few months before the clinic opened, DeLine was at the post office picking up his mail when a woman approached him. She had applied for a job at the clinic and wanted to introduce herself.

Id like to work for you, said Marcia Bader, who had lived in the village for 12 years.

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In a rural Wisconsin village, the doctor makes house calls and sees some of the rarest diseases on Earth - Milwaukee Journal Sentinel