Some of the world’s top scientists don’t understand the genetic disorders that afflict Amish children. At a new clinic in Delaware, Matt Demczko ’08 and Mike Fox ’07 will crack the code together.
When Dr. Kevin Strauss launched a class at Franklin & Marshall College to explore translational medicine – the way cutting-edge technologies could be applied to underserved populations such as the Lancaster County Amish – he found a willing pupil in Mike Fox ’07.
“Mike was in the very first iteration of the course,” Strauss recalls, noting the curriculum was “about bridging the gap between medical science and clinical care ... and making better use of the avalanche of data generation that really hasn’t reached patients in a meaningful way.”
Simply put, he says, that means better outcomes, higher survival rates and lower costs to the consumer.
The class, “Plain People and Modern Medicine,” was taught by Drs. Erik Puffenberger, D. Holmes Morton and Strauss, all of the Clinic for Special Children (CSC) in Strasburg. It focused on attitudes toward modern medicine among the isolated, sometimes insular Plain-sect communities in the Lancaster area and the otherwise rare diseases that are relatively common within the population. It altered Fox’s career path. In fact, he says, it was “a huge reason that I ultimately went into pediatrics.”
A decade and a couple of medical degrees later, Fox and a friend from F&M, Matt Demczko ’08, recently completed fellowships at the CSC. Now they’re set to open their own clinic in Delaware, where they will serve the growing Amish community near Dover. Slated to open in August as part of the Nemours Children’s Health System, it will share a footprint with a Nemours-run primary care practice. It’s designed with the Amish in mind – local art and quilts on the walls, hitching posts in the parking lot.
Another F&M alumnus, Adam Heaps ’08, is executive director of the CSC and helped set up the relationship between CSC and Nemours. And Strauss, who feels like he’s passing the baton to the next generation of healers, is excited about the work of all the F&M grads. Fox and Demczko, he says, “stood out at F&M as people who were very vigorous learners. Very much self-starters, and very inquisitive. [During their fellowships] they exceeded my expectations. Both brought a lot of fresh ideas to the practice, and they both have tremendous initiative.”
Fox spent a year at Lehigh University before transferring to F&M.
“I wanted to go to a school that was known for its strong pre-medicine program,” he says. “I also loved the small feel of Franklin & Marshall, and the liberal-arts experience. It’s a beautiful campus, and it just felt right.”
He graduated in 2007, then went to the University of Pittsburgh School of Medicine, finishing his studies there in 2011. He completed a three-year residency at the Children’s Hospital of Pittsburgh and spent an additional year as chief pediatric resident. Fox joined the University of Pittsburgh School of Medicine faculty for a year, then moved to the Delaware Valley and began his association with the Nemours.
Demczko graduated from F&M in 2008 and went to Philadelphia’s Jefferson Medical College, graduating in 2012. He did his residency and chief residency in pediatrics at Nemours/Alfred I. duPont Hospital for Children in Wilmington before joining the staff there.
“I’ve always had an interest in genetic diseases,” Demczko says. “From the start, I found genetics fascinating ... and I tended to gravitate toward the more complex cases.”
Demczko planned to enter the field of pediatric cardiology. Along the way, he found himself drawn to a broader view of patient health. “I really enjoyed the cases that were multi-organ, multi-system,” he says. “Cases that required thinking outside the box.”
Besides the intellectual challenge, Demczko says the work provides him “a chance to help a population that was not receiving as much attention and care as it deserved.”
Together, they’re eager to open the clinic. “The Amish tend to live off the grid, but if they need help, they will seek it out,” Demczko says. “The genetic conditions affecting their community are often times novel and poorly understood. Some of the most experienced physicians in the world have never heard of the disorders they have.”
While the public perception is that the Amish shun medical care, Demczko says that’s not always true. “Many of them are very open to medicine, and to therapies that can help their children,” he says.
Dover’s Amish population is smaller than Lancaster’s, Fox notes. “It’s more scattered, but it’s definitely substantial. It’s not nearly the tourist attraction that it is in Lancaster County.”
In many ways, he says, that means the largely agricultural community is poorer and divided even more from the surrounding population. The population emigrated from Ohio, not Lancaster – meaning Dover communities’ risk for genetic disease is similar to other Ohio Amish groups.
“We’ve been working with the doctors in Ohio to learn more about the diseases they face,” he says. “Although the reality is that they’re so poorly studied, from a medical standpoint, that we anticipate there being diseases we don’t know about yet.”
Fox says folks at the CSC discovered a previously unknown ailment in six patients among the Delaware Amish. It’s a peculiar genetic mutation of an enzyme that converts DNA into protein. Because of the mutation, afflicted children suffer a whole host of physical problems. Four of the children have already died from liver failure. They also had problems with their brains, blood sugar, hearing, vision, pancreatic function and growth.
A cure may be in the future, “but now we can recognize the disease,” Fox says. “And knowing what’s wrong with these children – and knowing what to expect – from the disease – gives us a head start. We are hoping we are able to mitigate some of the effects of the disease.”
The doctors have a theory that liver transplants might help manage the symptoms and allow these children to live longer, more productive lives. And even if they can’t help these particular patients, “we might be able to help the next generation of children born with the disease,” Fox says.
“These families have been incredibly generous with their time,” he says. “They have received a diagnosis for a child that is, essentially, a death sentence. But, knowing what is wrong with them, we are better able to be proactive in minimizing some of the impact.”
There are multiple barriers to providing care to a Plain population, Fox says. “The biggest and most obvious barrier is their lack of access to health care. That’s largely due to them not having health insurance.”
“If you bring a child in who could benefit from an MRI, you want to make sure that the test is truly necessary because the Amish are going to get an itemized bill at the end of their hospital stay. They’re going to pay for everything out of pocket.”
There’s also a big difference in the way medical professionals converse with Amish patients and their families, he says. “Generally speaking, Amish people tend to finish schooling around the eighth-grade level,” he explains. “So they don’t have a formal high school education, and likely have never taken a science class. You have to keep that in mind. You don’t want to talk down to them but you also can’t use advanced verbiage. The lingo is different.”
Also, he says, “they tend to think things over more. They don’t just make a decision, say ‘Let’s do this.’ They go back, they talk to other family members and members of their community first.”
There’s a philosophical block, too. Fox says the Amish sometimes hesitate to pursue treatment for a child’s illness if they know it will ultimately be fatal. “That’s another barrier we have to overcome,” he says. “We can’t always offer cures, but we can offer other things, such as palliative care measures by which we can keep the children comfortable. That’s a difficult conversation to have with any child’s family, but when you’re dealing with a population that has always done things one way, that can be quite a hurdle.”
Of course, the Amish have limited access to health-care providers because they travel by horse and buggy. If they need to travel outside the local area for specialized care, they will hire a driver, which can be costly.
“That’s one of the biggest things this clinic in Dover is going to provide,” he says. “We are bringing the care to them – primary care physicians, specialists, the whole team – so they don’t have to travel long distances.”
Strauss says the Strasburg clinic, which opened in 1989, led to the creation of similar clinics serving Plain-sect populations in Indiana, Ohio, Kentucky, Wisconsin and elsewhere in Pennsylvania.
“These guys are going to do great,” he says. “They have been embraced by the Amish population without hesitation.”
Fox and Demczko grew up about 20 miles apart in central Pennsylvania, Fox in Selinsgrove and Demczko near Mount Carmel, but they didn’t meet until F&M, where they took classes together and had overlapping circles of friends.
They joked about working together but had no plans to do so until circumstances brought them both to Nemours and the affiliated clinic in Strasburg.
“Nemours was looking to hire two people. It just happened to be Matt and me,” Fox says.
“People can’t believe the small-world nature of it,” Demczko adds. “Mike and I are very similar in the way we approach problems. I couldn’t be more excited about having a friend along for the ride.”