Culture

The Upside of Down Syndrome

Meet Dr. Brian Skotko, champion of extra chromosomes.

The first thing one notices about Brian Skotko is his winning smile. The second is his pants. They are, I think it is safe to say, rather absurd. Colorful and loud, and often paired with a conservative dress shirt and tie, Skotko’s pants invite mockery.

And that’s the point. They are part of a campaign the 35-year-old physician launched called “Mock my pants, not my sister.”

In 2011, John B. Thompson, writing in GQ magazine, ridiculed Boston men for being, in his opinion, the worst-dressed people in the country. “Boston suffers from a kind of Style Down Syndrome,” he wrote, “where a little extra ends up ruining everything.”

As the brother of a woman with Down syndrome (DS), Skotko took exception to the notion that an extra chromosome ruins everything — or anything, for that matter.

Go ahead and mock my clothes, Skotko, who is based in Boston, wrote in an open letter to GQ that he posted on his blog. “But, whatever you do, do not mess with my sister.” Skotko then schooled Thompson on what “Style Down Syndrome really is.” He wrote: “It’s smiling when everyone prefers to frown. It’s spending three summers, in sheer determination, learning to ride a bike because you want the freedom to be like everyone else. It’s singing tunes from Grease at the top of your lungs with your friends. It’s celebrating a third-place victory at a swim meet with as much gusto as a gold medalist. Style Down Syndrome is strong-willed, persevering, and forgiving — because it has to be.” (GQ later apologized privately but refused to do so publicly.)

“Mock my pants, not my sister” provides a glimpse of the passion with which Skotko advocates for his sister and all people with DS. But Skotko is much more than an advocate. He is also one of the country’s leading medical clinicians, geneticists, and Down-syndrome researchers.

The goal of his work, as he explained it to me when I visited him at Massachusetts General Hospital, is to make “tomorrow better than today” for people with Down syndrome. Put another way, Skotko is using his extraordinary talents to help move society beyond mere tolerance for people with DS and toward a celebration of them.

Skotko grew up in Strongsville, Ohio, a suburb of Cleveland, as the only son of an industrial-supply salesman and a grade-school teacher. Skotko has two younger sisters, Allison Hanselman, 32, and Kristin Skotko, 34, who has DS.

Skotko is, by all accounts, an exemplary brother and son. In an e-mail, his mother, Sharon Skotko, told me that Brian “has always been the perfect son. We have been so blessed. There was never one instance where he ever disappointed us.” Sharon told me that Brian has called home every day since leaving for college nearly two decades ago.

Brian has a special bond with Kristin. “I wouldn’t be doing what I am today had it not been for my sister Kristin,” he said in a 2013 interview on the Today Show. When I asked him to elaborate, he said: “Kristin fills in the missing pieces in terms of, when I am selfish she reminds me not to be. When I want to give up, she reminds me to keep on going. When I get frustrated when I didn’t achieve a goal, she reminds me of all the smaller steps along the way that were successful. When I lose sight of the big picture, she reminds me to focus on the goodness that’s there. When I am stressed, she reminds me that it’s going to be OK.”

Kristin grew up in the 1980s, when people with DS were often referred to as “mongoloids,” and when their life expectancy was only about 25 years. But Kristin’s parents didn’t dwell on her condition. “We always discussed her Down syndrome,” Skotko said, “but it was never the centerpiece of dinner conversation.”

Because there were few resources available to families raising children with DS, the Skotkos helped launch a parent support group, one of the first in their area. A group of six to ten parents of children with DS would gather once a week for dinner and fellowship.

Brian excelled in school, and after becoming valedictorian of his class at Saint Ignatius High School and a USA Today first-team academic All American, he enrolled at Duke University.

He had discovered his calling a few years earlier while caring for his grandfather, who was dying of cancer. “I spent a lot of time with him as he was going through the dying process,” Skotko said. “And I got to appreciate the importance of compassionate care and palliative care.”

At Duke, Skotko soon sharpened his focus and decided to specialize in Down syndrome. “All of a sudden things just clicked,” he said. “It felt right. I knew what I wanted to do. It combined all of my advocacy and my passion for my sister, with this interest for the healing powers of medicine.”

Skotko smiles often, and his smile broadened when I mentioned Allen Crocker, photographs of whom Skotko has posted throughout his office. Crocker was a developmental-behavioral pediatrician who taught at Harvard and directed the Developmental Evaluation Center at Boston Children’s Hospital for many years.

Skotko calls Crocker a “legend” of the Down-syndrome community. He was one of the first doctors to encourage parents to have pride in their children with DS instead of pitying them.

Skotko admired Crocker from afar until he entered Harvard Medical School in 2006. On the day he arrived in Boston, Skotko made a beeline for Crocker’s office, where they struck up an instant rapport.

Crocker became Skotko’s mentor, introducing him to the world of nonprofits, advocacy, and research, and encouraging him to pursue his most audacious dreams. Skotko would follow Crocker to the Down Syndrome Program at Children’s Hospital. Crocker died in 2011 at the age of 85. “To the very end, Allen was a friend and a teacher and a real champion for people with Down syndrome,” Skotko said.

While attending medical school, Skotko found time to earn a master’s degree in public policy from the Kennedy School. After Harvard, Skotko did a combined residency in pediatrics at Children’s Hospital and Boston Medical Center. He followed that with a fellowship in medical genetics. He is now an assistant professor of pediatrics at Harvard Medical School.

Skotko’s 40-page CV captures the depth and breadth of his devotion to studying Down syndrome. He has won numerous awards for his research and advocacy work. He has testified before Congress, spoken at the United Nations, made many radio and TV appearances, and delivered scores of lectures at medical schools and advocacy organizations around the globe. He has published two books and worked on more than a dozen research and clinical projects. He has helped launch two nationally recognized DS organizations, and has served on the boards of more than a dozen DS groups. Simply put, whenever the topic of Down syndrome is raised in public, there’s a good chance Skotko will be involved in the debate.

His main focus today is as clinical co-director of the Down Syndrome Program at Massachusetts General Hospital, where he and Dr. Allie Schwartz do research and see patients with DS.

Skotko is so immersed in the Down-syndrome community that on his first date with his future wife, Carrie Liken, he took her to a dance for people with Down syndrome. “He lives and breathes Down syndrome,” Schwartz told me, “which makes him such an incredible clinician and advocate.”

Skotko is both brilliant and driven, but his most valuable asset may be his relentlessly upbeat charisma. “He carries a wonderful light in the world,” is how Mary Ellen McDonough, a clinical-research coordinator who worked with Skotko at Boston Medical Center, put it to me.

Perhaps more vividly, Cynthia Hutchinson, Skotko’s co-author on the book Common Threads: Celebrating Life with Down Syndrome, has referred to Skotko as “Forrest Gump with a real high IQ.” She explained: “Only good things happen to him, and he’ll allow only good things to happen to him. He thinks the whole world is out to do good.”

Down syndrome most often occurs when there are three copies of the 21st chromosome instead of the usual two. The excess DNA causes a variety of changes, including low muscle tone and delayed cognitive development. DS is also associated with health problems such as congenital heart defects, sleep apnea, and early-onset Alzheimer’s.

Today, people with DS have more opportunities than ever before to live long and fulfilling lives — to graduate from high school and perhaps attend college; to form deep relationships and even marry; to live independently and hold down meaningful jobs.

Yet despite these gains, most expectant parents who receive a prenatal diagnosis of DS terminate the pregnancy. A 2012 synthesis of 24 American studies found post-diagnosis termination rates ranging from 50 percent to 85 percent.

The overall birth rate of babies with DS hasn’t dropped as much as those numbers would suggest, however. That’s because only about 2 percent of pregnant American women currently undergo the combination of prenatal screening and invasive diagnosis necessary to confirm Down syndrome. But the number will likely rise sharply as more women take advantage of sophisticated new blood tests collectively known as non-invasive prenatal testing (NIPT).

NIPT arose following scientists’ discovery that pregnant women’s blood contains minute amounts of fetal DNA. Through a blood draw from the mother, the fetal DNA can be captured and analyzed to learn many things about the fetus, including whether he or she may have DS. Some experts think NIPT will soon be diagnostic — meaning that with a simple first-trimester blood test and a signature on one more form, a mother will know definitively whether her child has any of a host of genetic conditions, including DS. Hank Greely, a law professor and bioethicist at Stanford Law School, told me last year he believes NIPT will be diagnostic in less than a decade, a development he characterized as a “revolution.”

This revolution could hold untold benefits for mankind, but it also makes DS advocates nervous. “We are, in the moment, potentially seeing through our society’s choices the elimination of a people,” Skotko said.

Denmark has had a national prenatal screening program for a decade. The program, according to the Copenhagen Post, could make the nation of more than 5 million people “a country without a single citizen with Down’s [sic] syndrome in the not too distant future.”

If the number of people with DS in the United States falls much below its current 250,000, it could be reclassified as a rare condition, and many advocates fear that already relatively paltry government funding and support services could dry up. Moreover, as scientists learn more about the neurochemistry of DS, society will be forced to consider where the line should be drawn between preventing disability and limiting human diversity.

In 2013, researchers at the University of Massachusetts Medical School showed that it is possible, in a single cell in a Petri dish, to shut down the extra chromosome that causes DS. The prospect of a cure for DS worries many advocates, including Skotko. “If Down syndrome were completely cured, the world would lose something from the absence of that culture,” he told NBC News. “There is something positive that people with Down syndrome contribute to the world.” And many people agree. In a recent Canadian survey, parents of children with DS were asked what they would do if a cure existed. Fifty-nine percent said either that they would definitely not use it or that they were unsure.

Stotko insists that the main obstacles for people with DS come not from the condition itself but from society. The antidote to this problem, according to Skotko, is information about what life with Down syndrome is like. Skotko once conducted a study of mothers who had received a prenatal diagnosis of Down syndrome. He found that most were not given accurate information about the potential of people with Down syndrome or contact information for parent support groups.

Doctors often reveal their biases. One survey of hundreds of physicians who delivered prenatal diagnoses found that only 63 percent tried to deliver unbiased diagnoses. Ten percent even admitted to actively urging women to abort.

As a result, parents often make uninformed decisions. In a study of Dutch women who aborted babies with Down syndrome, 83 percent said the burden of raising a child with DS would be too heavy, and 73 percent cited the burden on their other children. Just 21 percent reported having had much doubt about their decision.

But those views don’t jibe with the experiences of people with Down syndrome or their families. “A lot of people don’t accept [people with DS] because they haven’t had the luxury and privilege of having a family member with Down syndrome or getting to know someone with Down syndrome,” Skotko said. In a study Skotko conducted a couple of years ago, 99 percent of the people with DS he interviewed said they were happy, and 97 percent liked who they were. Just 4 percent expressed sadness about their lives.

In a 2011 study of hundreds of siblings of people with DS, Skotko found that 96 percent felt affection for their sibling with Down syndrome, and less than 10 percent were embarrassed by the sibling. Among older siblings, nearly nine in ten felt they were better people because of their sibling with DS, and more than 90 percent remained part of their lives as they became adults.

At least one study has even found that married couples who have children with DS have lower divorce rates than parents whose children are without disabilities.

Skotko spends much of his time trying to get accurate information into the hands of care providers and patients. In 2012, Massachusetts passed a law requiring its Department of Public Health to give pregnant women with prenatal DS diagnoses accurate, unbiased, and up-to-date information as well as contact information for support services.

The law was championed by the Massachusetts Down Syndrome Congress, which Skotko helped transform from a small all-volunteer organization into a million-dollar operation with ten full-time staffers and a legion of volunteers. The MDSC has since created a legislative blueprint for DS groups in other states to get similar laws passed. At least half a dozen states have already done so.

Skotko’s primary focus today is finding treatments that will alleviate the disabling aspects of DS but leave personality and character untouched. Skotko and Schwartz are in the middle of a ten-month clinical trial for a pharmaceutical they hope will improve cognition and memory and stave off Alzheimer’s-related dementia. “[These drugs] won’t change who they are fundamentally,” Skotko said, “and that brings a lot of comfort to family members.”

What also brings a lot of comfort to people in the DS community is the knowledge that Skotko has dedicated his life to advocating for them. Charlie Gadbois, a long-time friend of Skotko’s whose 17-year-old daughter has Down syndrome, said, “I thank God at night that there is someone like Brian there to look out for my daughter.”

“Brian is a guy who could, if he wanted, be a hedge-fund manager and make millions and be sitting on a yacht sipping martinis,” Gadbois continued. “If he wanted to be president, I believe he could be.”

For his part, Skotko couldn’t imagine doing anything else. “I feel very fortunate,” he says, “that I get to wake up every day and can’t wait to get to work. The work that I’m allowed to do is incredibly meaningful, because every day I’m making life a little bit incrementally better for people with Down syndrome.”

— Daniel Allott is a writer in Washington, D.C. 

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