current issue > winter 2009/2010 contents

Type 1 Diabetes in Our Towns
A cluster or a coincidence?

Steve Maas writer
Kristin Burke/Third Rail Studios photographer

Gus Kreft		Kelley Conley		Ashley Conley

Ann marie kreft’s line was busy when I first called her about the suspected diabetes cluster in her neighborhood. She was on the phone with a parent telling her that another child nearby had been diagnosed with the disease.

That was the tenth case in two years within a two-mile radius of Kreft’s home. Behind the statistic is another family plunged into a dizzying world where their child’s health depends on the fickle fluctuations of blood sugar. The good news is that with proper management, the child can live a long and happy life, doing most anything. The tough news is that management means countless finger sticks, dependence on insulin injections, and a lifelong balancing act with diet, exercise, and blood sugar levels.

Ann Marie Kreft and her son, Gus

Type 1 diabetes—once known as “juvenile diabetes,” because it is more frequently diagnosed during childhood—appears to be on the rise worldwide. The disease occurs when the body’s immune system goes awry. And it’s not to be confused with its more common sibling, Type 2 diabetes, which tends to strike later in life, often in people who are overweight.

Kreft, a gentle, self-effacing woman with a wry sense of humor, has become the point person for families affected by Type 1 diabetes in an area that straddles Wellesley, Weston, and Newton. After her son was diagnosed two years ago, she kept hearing about new cases popping up on her block and in nearby neighborhoods. Alarmed, she put her background in health education, and her contacts within the Massachusetts Department of Public Health, to work.

Thanks in part to her efforts, the state is conducting an in-depth investigation of Type 1 diabetes among children 18 and under in all of Newton, Wellesley and Weston. By the end of the year, investigators hope to have collected enough data to determine whether a cluster indeed exists in Kreft’s neighborhood or other spots within the three communities.

On the surface, a story like this brings to mind other neighborhoods with an unusually large disease outbreak, such as the high incidence of childhood leukemia recounted in A Civil Action. But so far no obvious causes have emerged like the tainted water supply in Woburn. And if a cluster is confirmed, it could take years to figure out why. Even Sherlock Holmes would be daunted by the myriad suspects in the mystery of Type 1 diabetes.

“Anybody who tells you that an epidemiology study is fast is not telling you the truth,” said Suzanne Condon, associate commissioner of the state health department, and Kreft’s key state contact. Condon, who played a major role in the Woburn investigation, is a bit like a Sherlock Holmes of medicine. She relishes the challenge of cluster research, comparing it to assembling the pieces of a puzzle. But her 30 years of experience has taught her to beware of making false connections and to be ever vigilant for missing links.

The Kreft’s: Gus (in gray), Anne Marie (mom), Isabel (sister), and Owen (brother)       The Kreft Family When Ann Marie Kreft and her sister were girls, they performed an unusual daily ritual. They dipped a strip of chemically treated paper into a sample of their father’s urine and checked it against a color chart. Ann Marie’s dad had Type 1 diabetes, and in the 1970s, that was the state-of-the-art way to tell if blood sugar was too high or low. In late summer two years ago, Ann Marie grew concerned about her middle son, Gus, who was seven years old at the time. He was extremely thirsty and going to the bathroom frequently. Running errands one Saturday, “I had to find a bathroom in every single store we went into,” she recalled. “The next morning, when he had asked for a fourth breakfast by 9:30…that was it, I knew it.” “I was scared,” Ann Marie said. She was haunted by nightmares of her father. But diabetes care has made enormous strides over the past couple of decades. Instead of taking shots, Gus gets insulin through a pump about the size of a small cell phone that he affixes to his body. Using a wireless device that resembles a Blackberry, Gus programs his insulin intake depending on such factors as the carbohydrates in a meal and his exercise plans. Gus plays on town sports teams and goes to summer camp with other kids who have diabetes. Under the watchful eye of his parents, school nurse, and family and friends, he manages much of his own care. Says his mom: “All Gus ever wants to do is be active, crazy active.”

“If there was anything we knew about right now, we’d be taking other action,” Condon said of the possible cluster. “There’s nothing that jumps out.” Cautioning against alarm, she points out that if the source was found, it could be related to common lifestyle factors rather than geography.

But potentially the findings could advance diabetes research. To Condon’s knowledge, a residential-based study of Type 1 diabetes has never been conducted before.

But before delving deeper into the detective angle, let’s return to the local families. Their response to the possible cluster—how they turned this geographic anomaly to their advantage—is an equally compelling side of the story.

The Silver Lining
“Diabetes is quite demanding in its 24/7 need for attention, and it’s quite lonely,” said Dr. Lori Laffel, who sees many of the families in the suspected cluster, as chief of the Pediatric, Adolescent and Young Adult Section at the Joslin Diabetes Center.

Rikki Conley knows about both the 24/7 demands and the loneliness. Her two daughters have Type 1 diabetes. Ashley was diagnosed 12 years ago, and her younger sister, Kelley, two years later. At the time, they were aware of only a handful of cases in the area. Every night, she interrupts her sleep to check her daughters’ blood sugar levels and, when the numbers are low, slips a straw in their mouths to sip juice.

The Conley’s: Kevin (dad), Rikki (mom), Kelley (gray shirt), Ashley (pink shirt), and Colby (shirt and tie)   The Conley Family Phone calls interrupt a visit with Rikki Conley and her daughter Ashley, with updates from the track meet where younger daughter Kelley is competing. Both Ashley, 14, and Kelley, 12, have Type 1 diabetes, but neither lets it get in the way of their lives. “You kind of get used to it. All my friends know about it,” says Ashley, who was just back from an overnight school trip to Canada and is a “huge soccer player.” Ashley was diagnosed before the family moved to Weston; her sister several years later. While Ashley takes the diabetes treatment in stride—“It’s just another thing to do. Like some kids take medicine”—a complication of the disease has made her life a bit more challenging. Two years ago, she was diagnosed with celiac disease, which requires a wheat-free diet. Rikki appears amazingly calm as she talks about her family, sitting in the spacious family room of the house she designed for the family. Her husband, Kevin, is chairman of the board of the Joslin Diabetes Center. “You almost have to be super flexible or [the diabetes] will run your life instead of your running your life,” Rikki says

For six years running, Ashley and Kelley’s Army has topped the family category in raising money at the Boston Walk to Cure Diabetes, which benefits the Juvenile Diabetes Research Foundation. Last year, the family’s total was $53,000. Conley credits the “unbelievable” support of the Weston community. But the Army’s streak may end with the 2009 walk,” she said. “There are so many other families in Weston” with Type 1 diabetes asking for community support.

Because Kelley Conley was best buddies with Kreft’s daughter, the two mothers became friends as well. It was to Conley that Kreft turned when she suspected her son had Type 1. Such connections among families helped surface the possible cluster and laid the foundation for a support group. Now the families meet monthly to swap advice, share news of the latest treatments and research, and vent their frustrations with the only other people who really understand what they’re going through.

Surprisingly, the cluster mystery is not at the forefront of their concerns.

	Soccer fan Ashley Conley

“Do we want to find out what’s going on and why this is happening to so many people? Absolutely,” says Conley. “But we have to step away and have our daily life. And the life is: Keep these kids healthy.”

At one group meeting a college student told parents what to expect when their youngsters reach adolescence, admitting how she at first undermined efforts to manage her diabetes before facing up to the fact that she was putting her own life at risk. Sometimes, meetings are devoted to welcoming a new family into the fold, answering the flood of questions that come with diagnosis.

“It’s a loving environment, a safe haven, kind of like Cheers, where everyone knows your pain,” said Shannon Allen, whose three-year-old son, Walker, was diagnosed while dad Ray, the Celtics shooting star, was competing in the 2008 NBA finals. “Everyone gets it. We’re all kind of these frazzled women who walk around all day with no sleep and constant worry on our faces.”

The families also get together for picnics, call one another for reassurance, and pass along e-mails with suggestions for, say, a sugarless Easter. Kreft alerts the state when she hears about new cases, and updates the group about developments in the cluster investigation.

Finding strength in numbers is particularly important with a disease like diabetes. Patients and their families play a much bigger role in their own care; it’s not a simple matter of taking two pills a day. “You check in with your doctor every three months, but you’re doing the day-to-day decisions,” said Colleen Kettle, who developed Type 1 diabetes as an adult, and whose daughter was diagnosed at age 13. “You have to make decisions based on many factors, most of which are constantly changing.”

Playing Pancreas
All those decisions are supposed to be performed automatically by the pancreas. But if you or your young child has Type 1, you have to do the job instead. The disease is the result of friendly fire, the body’s immune system attacking one of the organs it is supposed to protect. Antibodies destroy the cells in the pancreas that produce insulin, the hormone that transforms glucose from food into energy.

Without insulin, glucose—also known as blood sugar—builds up in the body, where unchecked it could lead to heart disease, kidney failure, amputations, blindness, and a host of other horrible consequences. Meanwhile, the body, starved for usable energy, starts breaking down fat and muscle, setting off another possibly lethal chain of events.

Kelley Conley in the library, a favorite corner to curl up and read.

A huge industry has evolved to help diabetics manage their disease. They can test their blood sugar levels with a prick of the finger or by wearing a monitor. They can inject themselves with various forms of insulin or wear an insulin pump that they can activate as needed. But despite all the advances, the disease is all-consuming.

“I don’t go an hour without thinking about it,” says Kettle, who lives in Weston, “I wake up thinking, what’s my number?”

That number is the blood sugar reading. “The lows are worse than the highs,” says Kettle. “You feel helpless, spacey.” Upon waking, it should be under 100. That’s the “fasting” level, as the body has gone without food for the past eight hours. Depending on the time of day, healthy blood sugar levels vary from 70 to 140. Very low blood sugars could lead to unconsciousness and seizures in the short run, and to brain damage in the long run.

Kettle recalls coming home from, of all things, a Joslin fundraiser, and finding her daughter standing by the door wearing a towel and a big goose egg on her head. She apparently fell while taking a shower in a second-floor bathroom and then wandered down to the first floor. “She didn’t even know what had happened,” Kettle said. As it turned out, she had inadvertently injected herself with too much insulin. Her parents revived her with candy. Sometimes, Skittles and a glass of cola can be good for a diabetic.

Kettle said incidents like that have been rare in her family, but keeping blood sugar within a healthy range is a tricky business. If you are a diabetic, you better not be math phobic. To determine how much insulin to take, you need to count the carbohydrates—which the body breaks down into sugar—in every meal and snack.

Just as with food, stress raises blood sugar levels. Kettle, who has four children, recalled that when she and her husband took a vacation alone, she needed just two daily shots of insulin instead of five. “What that told me was I was taking three shots for stress,” she said.

Exercise lowers blood sugar level. That’s a good thing, unless the levels fall too far. When Kreft watches her son play lacrosse, the numbers that matter aren’t on the scoreboard but on the finger stick blood tests she gives him before, during, and after the game. If the blood sugar remains too low, she gives him candy or glucose tablets.

Despite the risks, all the parents interviewed are determined that their children lead as active lives as possible, playing on sports teams and joining classmates on overnight trips. “These kids have to grow up a little earlier,” says Kettle.

But if the feelings of 14-year-old Ashley Conley are representative, they are under no illusions a cure is around the corner. “It might come in my lifetime, but probably won’t,” says Ashley, who whose fingers are pricked with “dots, dots, everywhere” and wears an insulin pump below her belly button. “There still needs to be a ton of research. Even if they do find a cure, it will take years to test it.”

For now, the families can take heart that their participation in the cluster study could advance the science of diabetes.

The Disease Detectives
Luck also plays a role in science. It was Ann Marie Kreft’s good fortune to have gotten to know Suzanne Condon around the copy machine some 20 years ago at the state health department. So Kreft knew just the right official to contact about investigating Type 1 diabetes. And Kreft couldn’t have picked a better time to do so.

“I wrote back and said you’re not going to believe this, but we’re actually doing this,” Condon said.

For several years, Condon’s office had been tracking childhood asthma statewide, by surveying school nurses about students in kindergarten through eighth grade. Massachusetts’s rate, she said, is among the highest in the nation.

Intrigued by research suggesting that Type 1 diabetes may have an environmental trigger, Condon decided to add the disease to the survey. But to get a real handle on the prevalence of Type 1, she needed to broaden the investigation to include babies through 12th grade. Lacking the resources to do a statewide study, and intrigued by Kreft’s report of a possible cluster, Condon decided to focus on the communities of Wellesley, Weston and Newton.

Collecting the data is painstaking. As a result of the federal Family Education Rights and Privacy Act, schools can disclose only numbers from health surveys, not names. A Weston child, for example, would show up in Brookline’s count if she attends private school there.

“It’s hard to do an environmental study when you don’t know where people live,” said Condon. So her office is sending out letters through the school nurses asking families in the three study communities for their consent to participate. As a check on the school figures, and to identify pre-schoolers and high schoolers, the researchers are also contacting medical centers like the Joslin that provide diabetes care to the area’s residents.

The Juvenile Diabetes Research Foundation Ashley Conley doesn’t know what it’s like to live without diabetes. The Weston 14-year-old was diagnosed when she was two years old. Ashley says she expects to live with the disease for the rest of her life. The Juvenile Diabetes Research Foundation, which has its New England headquarters in Wellesley, hopes to surprise Ashley. It is funding research in multiple areas, with the goal of perfecting an artificial pancreas and stopping complications of diabetes in the short term, and arresting and reversing the disease in the long run, according to Bill Ahearn, the foundation’s vice president of communications. The artificial pancreas would unite two technologies already available: continuous glucose monitors and insulin pumps. The challenge now is more mathematical than scientific, Ahearn said. The device, which would be about the size of current insulin pumps, would employ “a sophisticated algorithm to dispense just as much insulin as needed when it is needed.” In effect, it would mimic a healthy pancreas. That may sound simple, but consider all the factors that affect blood sugar levels: diet, exercise, stress level, and even outside temperature. Initial units would be for use during sleep, when a diabetic would be least likely to recognize a plunge in blood sugar. Eventually, a variety of units may be available, some entirely automated and others that allow some intervention. “We would like to see a lot of choice and competition,” Ahearn said. “I think within five years or so, we’ll certainly see prototypes.” Meanwhile, researchers are testing a number of protocols in people, that slow or stop the attack on the pancreas that causes Type 1 diabetes, or that restore or replace insulin production. Some compounds, administered to people just diagnosed, “stave off the full impact of the disease for years,” Ahearn said. He said the foundation was also funding research to improve the lives of people with diabetes, and reduce the risk of complications such as eye, kidney, and nerve disease. Scientists hope to develop treatments that both correct the immune system and recreate or regenerate the cells essential for insulin production. “There will be a cure at some point of time or a combination of therapeutics,” said Ahearn. “We’re working hard to help everyone, but certainly for that 14- year- old.” For more on the Juvenile Diabetes Research Foundation, visit www.jdrf.org.

“By end of the year we’ll be able to say what the numbers look like in relation to national and state rates, and whether that cluster really is unusual,” Condon said, referring to the Weston area. If a cluster turns up, she doesn’t expect it to be within a neat two-mile radius, as the number of cases has to be viewed in the context of such factors as population density.

Working closely with Laffel at the Joslin, Condon’s staff is drawing up a detailed questionnaire for families with Type 1 diabetes. They’ll be asked for family histories that might reveal genetic links. (Kreft’s father had Type 1 diabetes, but Walker Allen’s family has no history of the disease). The survey will collect data on birth weights, immunizations, allergies, diseases, and, of course, where and when the diabetes was diagnosed. Parents will be asked about their occupations and hobbies, in case any involved environmental hazards. Children will be asked about the schools they attended, their hobbies and sports, and the parks and playing fields they frequented. “We may find out that a large number of children spent a lot of time at a particular park,” Condon said.

The survey will also detail information about diet, recreation, and cultural practices of the families to determine if the link is lifestyle related.

Meanwhile, the researchers will pull the files on the 436 hazardous waste sites in the three communities. Most of them are small, often closed gas stations and underground tanks. But each will have to be examined to determine the nature and severity of the contaminants. Additionally, the state will examine environmental concerns raised by the parents, such as pesticide and herbicide use. Condon notes that the railroads were exempt from some laws regulating chemical spraying.

Once the environmental and demographic data have been fed into the computer, researchers can begin generating layered maps, to determine any correlation between the homes of families with Type 1 diabetes and hazardous sites.

“We won’t look at Ann Marie’s neighborhood separately until we have the complete picture,” said Condon. “One of the worst things we can do is go in and generate a biased hypothesis. We need the full picture for those three towns.”

If the diabetes study does reveal clusters, but no obvious environmental or lifestyle explanations, researchers may conduct biological studies. That could include testing children for exposure to mercury and to viral infections.

It could take years before an answer emerges to the cluster mystery. “The nature of the work itself is inherently slow,” Condon says. “You have to be meticulous. You have to be comprehensive.”

Of Cow’s Milk and Sunshine
At one time, some scientists suspected a protein in cow’s milk as a trigger for Type 1 diabetes, since they found that the disease was less common closer to the equator, where fewer dairy farms and limited refrigeration led to less consumption of cow’s milk.

Laffel, who is also an epidemiologist, recounts that anecdote as she looks back at the many theories about Type 1. Cow’s milk has been discounted as a factor, but researchers still wonder about a latitudinal link. Finland’s Type 1 diabetes rate, for example, is twice that of the United States. Could exposure to the sun be a factor? Vitamin D is known to influence the immune system, Dr. Laffel notes. But Finnish research suggests acceleration in the number of new cases, not easily explained by exposure.

“Different environmental influences may lead to diabetes in different people,” says Laffel, stressing the complexity of the diabetes mystery. Still, she is excited about the cluster study’s potential.

“If we’re able to confirm a cluster, then we can seek ways to drill down to what could be the factors that are related to the increase occurrence. That will help us understand what causes diabetes.” Laffel then pauses to add the inevitable “but.”

“That’s a big leap. The one thing I never want people to expect is that this will give us an answer. But if it gives us more questions, that’s great.”

For more on the juvenile diabetes Research foundation, visit http://www.jdrf.org/.

For more information about the Massachusetts Department of Public Health study, visit http://web.me.com/amkreft/COUNT_US_In/Welcome_to_Count_Us_In.html.