[BobbyB]

Middleborough welcomes state registry of ALS cases
By Christine Wallgren
Globe Correspondent / January 20, 2008


MIDDLEBOROUGH - The town, which has a cluster of cases of Lou Gehrig's disease, is welcoming the start-up of a state registry to track occurrences of the nearly always fatal disease.

more stories like this"We feel blessed," said Suzanne Dube. The first-in-the-nation registry will chart cases of the illness known officially as amyotrophic lateral sclerosis, or ALS. Dube lost a cousin to ALS in 1997 and an uncle last summer. Both worked in Middleborough, in Everett Square.

Dube heads a committee established by Town Meeting voters a year ago to look into the high incidence of ALS in town, and any possible connection to industrial hazardous waste sites nearby.

The registry will provide health officials with a record of how many cases have occurred in a particular area. Common denominators may surface that would help lead experts to the causes of the illness and, hopefully, a cure.

"What's happening now is very gratifying," said Middleborough resident Victor Sylvia, who, with his wife, Marion, has been tracking cases of ALS locally for about 13 years and forwarding their findings to state health officials.

As a first step, state officials this month will send out questionnaires to all neurologists and ALS clinical centers, requesting information on ALS patients. Participation in the survey will be mandatory.

A team of specially trained nurses will evaluate the records, and an epidemiologist will oversee surveillance of cases across the state.

"Surveillance data is critical in providing a picture of the disease burden across the population. The ALS registry will lay an important foundation for future research efforts and intervention activities," said Suzanne Condon, director of the state Bureau of Environmental Health.

Because ALS can be difficult to diagnose, the state's medical team will do follow-up checks whenever a diagnosis is uncertain. All data will be entered in a secured Department of Public Health database.

Information on patients will include name, age, gender, where a person lives, has grown up and worked, family medical history, and what symptoms they show.

"It's about time," Sylvia said of the registry start-up.

"Now it would be a great help if other states did the same thing."

ALS is a neuromuscular disease whose initial symptoms include clumsiness, tripping, trouble gripping and sometimes slurred speech, according to the ALS Massachusetts chapter's website. Within an average of two to five years of ALS onset, victims weaken to a state of paralysis, although mental capacity remains normal.

There is no known cure.

State health officials have already identified Southeastern Massachusetts, and Middleborough in particular, as hotspots for the illness. A recent state study focused on possible connections between hazardous waste sites and ALS rates in Middleborough, as well as in Hingham, Rockland, Abington and Weymouth, which border the former South Weymouth Naval Air Station.

Rates of multiple sclerosis, another neuromuscular disease with possible environmental connections, was also reviewed. According to DPH spokeswoman Donna Rheaume on Monday, the report is still undergoing peer review. Results are expected to be made public shortly.

According to prevalence estimates developed by the Department of Public Health and presented to Middleborough residents last fall, there are 4 to 6 cases of ALS per 100,000 statewide, compared with 9.5 per 100,000 in Plymouth County and across Southeastern Massachusetts. The figures were based on a study of data on cases occurring from 1998 to 2003.

Because there was no registry to tap, state health officials had to send out personnel to go through records at doctors' offices.

The high incidence rates reported last fall came as no surprise to Middleborough residents, who have been long concerned over the number of ALS cases in town. They say most of the 27 cases they have counted are clustered around three hazardous waste sites near Everett Square and Plymouth Street.

Eileen Bendiksen, another member of the Middleborough ALS committee, said her panel is reviewing reports of cases that date back to the mid-1980s.

Dube said her committee will try to secure a grant to cover the cost of hiring an epidemiologist who would interview local ALS patients and family members, and put together their information for the registry.

The head of the Massachusetts chapter of the ALS Association welcomed news of the registry.

"I'm personally gratified that for the first time ever we'll have a statewide system to record and track ALS," said Rick Arrowood, president of the nonprofit organization, which provides direct patient services as well as research funding. "We're out there in the front line. Currently we have a 24-year-old patient. It's sad there is no cure. But maybe, just maybe, this registry will put us on that path."

Christine Wallgren can be reached at CLWallgren@aol.com.
http://www.boston.com/news/local/art..._of_als_cases/