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News, People

The Irish Risk for Tay-Sachs Disease

Cathy and Jeff Mitchell with a photo of their late son, Harrison, who had Tay-Sachs.

When Cathy Mitchell’s son, Harrison, was diagnosed with Tay-Sachs disease, she and her husband, Jeff, were devastated–and more than a little confused.

“I thought Tay Sachs was a Jewish disease,” says Cathy. The Mitchells, who live in Langhorne, then learned that this deadly inherited disease, which cripples and kills, is common among people of Irish descent. As many as 1 in 50 Irish Americans is a carrier.

Children can only inherit the disease when both parents carry the trait; with every pregnancy, a couple runs a 25 percent chance of having a child with Tay Sachs and a 50 percent chance of having one who is a carrier but doesn’t have the disease. Cathy and Jeff Mitchell are both carriers.

“I didn’t even know I was Irish,” said Jeff Mitchell, who, with his wife and another couple–Aaron and Kathryn Harney of Downingtown—manned an information booth on Tay Sachs at the recent Irish Festival on Penn’s Landing. Mitchell hadn’t been close to his father’s side of the family and only learned after Harrison was diagnosed that his grandfather was Irish.

Like other children with Tay-Sachs, Harrison seemed like a perfectly normal baby when he was born. But at six months, he began showing troubling symptoms. “Harrison didn’t sit up. His muscle tone was weak,” says Jeff, a shop foreman at a truck equipment company. “When you would pick him up he would fall over. He startled to loud noises. He rolled over once and then never rolled over again.”

When Harrison seemed to have trouble seeing toys in front of him, the Mitchells took him to their pediatrician who, says Jeff Mitchell, “kept pushing it off on the fact that Harrison was born a month early,” implying he was experiencing normal developmental delays. Eventually, the Mitchells took their baby to a pediatric opthamologist who saw a cherry-red spot on his retina, a physical marker of Tay-Sachs.

The disease is a particularly cruel one. New parents come home with an infant who appears to be healthy and normal, but is lacking an important enzyme, hexosaminidase (Hex-A), that helps clear out fatty protein and other substances from the tissues and nerve cells of the brain. That regular housecleaning allows the infant to develop vision, hearing, movement, and other vital functions. For a few months, the Tay-Sachs babies grow and develop as babies do—cooing, reaching for toys, smiling, laughing, turning over.

But as those proteins build up in the tissues and nerve cells, a relentless deterioration of physical and mental abilities begins. Children lose their sight and hearing. Their muscles atrophy and they become paralyzed. Eventually, they’re unable to swallow, and they develop seizures and dementia. Most children born with Tay-Sachs die by the time they’re five. “They just slowly fade away,” says Jeff.

In 2010, Harrison Mitchell died just shy of his sixth birthday.

Kathryn and Aaron Harney of Downingtown, with their son, Nathan, who has Tay-Sachs.

Unfortunately, most doctors don’t encourage any but their Jewish patients—particularly Ashkenazi Jews–to be tested for Tay-Sachs. They also may not recognize the symptoms when they see them in children like Harrison. Like the Mitchells, Kathryn and Aaron Harney saw doctor after doctor before their son, Nathan, now 18 months old, was diagnosed—again, by a pediatric opthamologist. “A lot of doctors looked and us and said, ‘Well, you’re first-time parents. . . .,” says Kathryn Harney, Nathan slouching on her lap, his hazel eyes wandering, unfocused.

Like the Mitchells, they Harneys didn’t know that Tay-Sachs is common among the Irish. After they were tested, Aaron Harney learned that he carries a strain common among French Canadians. “I didn’t realize I had French Canadian ancestors,” he says. Tay-Sachs is even more common in French Canadians and Cajuns than in the Irish—an estimated 1 in 27 carry the trait, the same as Ashkenazi Jews. Nathan also has a rare form of Tay-Sachs that more often occurs in older children. “That gives us some hope that he might survive longer,” says Kathryn.

There’s no cure for Tay-Sachs, but it can be prevented with genetic screening. The National Tay-Sachs & Allied Diseases Association of the Delaware Valley offers free Tay-Sachs screening to anyone over the age of 18. Researchers at Einstein Medical Center’s Genetics Division in Philadelphia are now conducting a study to determine an accurate carrier rate for Tay-Sachs disease in the Irish and to identify the most common gene changes in this demographic. If you qualify for the research project (you must have at least three Irish grandparents), you will be screened for free and also get genetic counseling to explain your results and discuss your options.

For more information about Tay-Sachs disease carrier screening or to participate in this study, contact Amybeth Weaver, MS, CGC at irish@tay-sachs.org or call 215-887-0877. You can also go to the website and download a brochure that explains the Irish connection to Tay-Sachs.

Eight weeks after Harrison died, the Mitchells went to their first information session to help spread the word about the disease and especially to let others know that the Irish are among the most vulnerable populations. Last March, they manned a stand at the Bucks County St. Patrick’s Day Parade in Levittown (they serve on the parade committee) where they sold T-shirts, hats, and souvenirs to raise money for the parade—and passed out Tay-Sachs pamphlets for free.

“It’s important to us to raise awareness about Tay-Sachs,” says Cathy. “We need to get the word out there that it affects everybody.”

July 2, 2012 by
History, People

Will They Go No More A-Roving?

A scene from the film, "Settling Down."

They’ve had many names: tinkers, travelers, gypsies, Lucht Siuil (“the walking people” in Irish), and Pavee, in their own tongue. There are only about 36,000 of them in Ireland and they’ve traded their distinctive horse-drawn carts for gleaming trailers and, increasingly, houses, just as they’ve given up tinsmithing and seasonal farm labor as 21st life encroaches on their centuries-old itinerant culture.

On Sunday, the University of Pennsylvania Museum of Archaeology and Anthropology will present the short film, “Settling Down,” a look at a small group of Irish travelers in Cork and how their identity and culture has been transformed as a result of bigger changes in Ireland.

Joseph Lennon, director of the Irish Studies Program at Villanova University, will be speaking about the film and the uncertain future facing the travelers. We spoke to Lennon this week.

What’s the film about?

It focuses on pretty localized incidents in Cork and right outside Cork City where the city corporation is trying to negotiate with a group of travelers about keeping some of their camp sites open and creating more open space and fields for their horses. One of the travelers asks many, many times, “Where can we go?” It seems like the big question in today’s Ireland. How are travelers going to find any place they can go? It’s a problem that endures. It’s about land. It’s about prejudice. There’s still a lot of fear of this pretty insulated community and what they do and what they’re about. As much as we know about them and studied them and talked to them, they’re a closed community with their own language, and people are fascinated by that.

Travelers are ethnic Irish. Why is there so much prejudice against them?

It’s half romanticism and half fear. Historically there has been this projection of the settled population, of us, onto this romantic itinerant group that seems to buck the rules of modernity. The truth is, for every incident of traveler theft there are hundreds of thousands of incidents of no theft, but things get magnified both in the media and our cultural imagination. You have to remember too that the population of Ireland had struggled to own their own land for hundreds of years after it was confiscated by the British. It was one of the goals of Irish immigrants to the United States and in Ireland to get land. They have a great passion for that; it’s seen as the most prized possession. For people who never aspired to that, there was a sense that they were the losers in life. Michael Hayes, a scholar in this area, calls it the dropout theory: They couldn’t make it in society so they dropped out. And as the documentary points out, the travelers were left out of Ireland’s economic boom times. They’re considered working class people who don’t have the same ambitions as settled people. They’re not seen as a different ethnic group or lifestyle, but a group that should assimilate and most travelers don’t want to assimilate. Their lifestyle and culture is to be on the road. Ireland has difficulty with multiculturalism. Only in the last 15 has Ireland had any immigrants. The immigrants who are coming to Ireland have brought awareness of the need for advocacy for the travelers.

What are their origins? I’ve read that they’re descended from ancient traveling poets or that they’re descendants of people turned out of their homes during the famine.

It’s difficult to say. There’s no absolute origin story for the travelers. Going back to the mid-17th century, there were these traveling bards or the Filid, people who would travel between districts or kingships as storytellers, bringing news, telling stories, acting as historians, doing genealogies, things that were very meaningful in those societies. It may be that the travelers picked up on that tradition, coopted it if you will, and picked up some of the stories and the oral culture. They are certainly more practiced in orality than, say, people who watch TV all the time, so there may be some truth to it.

I’ve also read that their language, shelta, can be traced back to that time.

Nowadays people have about 200 to 1,000 words of vocabulary and they mostly use it for bargaining. [Irish travelers trade in everything from dogs and horses to scrap metal.] A part of the language appears to be Norman Romany, a root of the language of Romany and what was to become English. Languages like shelta are actually what are considered “anti-language.” They’re there to obfuscate, to be intentionally not understood, which makes them useful in bargaining so people outside the community would not understand.

What do you think viewers will come away thinking after they’ve seen the film?

I hope they come away thinking that what’s going on with Irish travelers is much more complicated than they had guessed and the problems haven’t been solved for good reasons, including prejudice.

February 29, 2012 by
News, People

Having Fun at Irish Summer Camp

Una McDaid tells the story of Cuchulainn to campers at Club Cultur.

Only three days into Club Cultur, an Irish-themed summer camp at Sacred Heart School in Havertown, the campers already knew that sui sios meant “sit down” and seas su meant “stand up.” While they got that cuinas meant “quiet,” they weren’t as familiar with the concept as they might have been.

Sometimes, they were just having too much fun to be quiet.

Club Cultur was started by four Delaware County residents—three of them Irish immigrants—who thought their little idea of teaching children about Irish culture might rouse a some interest in a community where so many Irish immigrants have settled. “We thought if we got 20 kids we could build on that and it would be good,” says Tina McDaid, a native of Glenswilly, County Donegal. “We didn’t expect the response we got.”

Seventy children between the ages of 5 and 14 were registered for the week-long camp, where they were immersed in the Irish language, geography, mythology, music, sports, and games.

Many of their parents are like Camp Cultur co-founder Una McDaid, Tina’s sister-in-law—anxious to keep their American children rooted to their Irish heritage. “When I first came here I used to hear people say they were Irish but when I asked them what part of Ireland they came from, they didn’t know,” says Una. “I couldn’t have my children not knowing where I came from. This is part of who I am.”

The blueprint for Club Cultur’s program is the curriculum in Irish primary schools. “The kids are learning here all the things they would learn if they went to school in Ireland,” says Tina.

A game that looked like net-less volleyball, for example, was a lesson in Gaelige, or Gaelic, the native language taught in Irish schools. The older girls counted down in Irish as they passed the ball to one another and followed Tina’s directions, spoken in her native tongue. “The children have already learned their colors, counting to 10, how to say thank you,” says Tina. “Our motto is, ‘Better to have a little bit of broken Irish than perfect English.’ If the children can pick up 5 percent and keep it, I’ll be happy.”

The campers also learned about modern Irish culture, including what can only be called Irish English. That was taught by Una who says that her own children understand that when she says she left something in the “boot” they should look for it in the trunk of the car. “But they never call it the boot; they say trunk,” she laughs.

“Can anyone tell me what a vest is?” she asked the crowd of kids at her feet during the lesson where most kids knew, thanks to Irish parents or grandparents, that “bangers” are sausages and that a “footpath” is a street. “A sweater?” one child ventured tentatively. No. “A coat?” another guessed. Una allowed for a few seconds of silence then revealed the answer. “It’s undershirt.” The crowd buzzed.

Later, Una’s niece, Fiona Bradley, who is a McDade Irish dancer, taught the littlest girls a few ceili dance moves, assisted by some campers who’d obviously done this before, while Ciaran Porter, games development officer for the Philadelphia Gaelic Athletic Association and a half forward on the St. Patrick’s Gaelic football team, taught the boys how to pick up a sliotar with a hurley (translation: pick up the hurling ball with the hurling stick).

“When we started talking about this, we realized that between the four of us we had everything—sports, language, dancing, and culture,” says Tina, laughing.

With 70 campers in its premier year, there’s a good chance Club Cultur could become a staple of Delaware County summers. But sheer numbers aren’t the only reason. “Most of the kids were signed up by their parents who were skeptical that the kids would like it,” says Una. “But the kids are really, really enjoying it, so it’s win-win.”

We stopped by Club Cultur on Wednesday morning and took some photos, which you can see here. 

August 19, 2011 by