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Articles
Wednesday Jan. 23, 2002

NEWS BRIEFS

GOMA, Congo

Residents line up for water in volcano-ravaged Goma; 90,000 left homeless search for shelter

Associated Press

Thousands of Congolese lined up for water at a church and other points around this wrecked city yesterday as relief agencies trucked in food and tents for some 90,000 people left homeless by a volcanic eruption.

Aid workers were setting up water distribution systems and planning to start delivering food today in Goma, where tens of thousands fled the streams of lava that cut through the lakeside city from Thursday's eruption of Mount Nyiragongo.

"We are finally getting some momentum" on distributing aid, said Michael Despine, the head of the International Rescue Committee's operation in Goma.

Earthquakes related to the eruption continued to rattle the region yesterday. In neighboring Rwanda, just across the border from Goma, more than 288 homes and 19 schools have been destroyed since the tremors began last week, the Ministry of Local Government said.

While trucks loaded with blankets and plastic sheeting rumbled down Goma's streets, hundreds of aid workers and U.N. staff from around the world met in a hotel to coordinate the relief effort. More than 30 percent of Goma's residential neighborhoods were destroyed by lava, said Ross Mountain, the U.N. deputy emergency relief coordinator. An estimated 90,000 people are homeless.

Thousands lined up outside a church in Goma to register with aid agencies to receive food. In front of the dilapidated chapel, aid workers from the International Rescue Committee assembled a water system that was soon filled by a U.N. tanker. Water distribution points were set up throughout the town, and people gathered to collect their share.

Mwendo Kambale, an office worker and father of four, said his family was sleeping outdoors in the rain and had run out of food on Sunday.

"The food is arriving very slowly," Kambale said. "I'm not happy because now I have no job, no home, no money and very few clothes."

Tens of thousands have fled the city, seeking shelter with families and friends in other Congolese towns and villages. More than 100,000 fled to villages west of Goma, 12,000 to south to Bukavu and about 40,000 remain in Rwanda, said Laura Melo, spokeswoman for the World Food Program.


p>WASHINGTON

FDA approves first drug to save livers of babies born with rare killer

Associated Press

Children born with a very rare but lethal liver disease - it often kills by their first birthday - have won the first drug that promises to help them live years longer.

The drug Orfadin amazingly is a failed weed killer that Swedish scientists discovered could fight a disease called hereditary tyrosinemia.

"This is a real breakthrough drug," said Dr. Marlene Haffner of the Food and Drug Administration, which just approved Orfadin's sale. She said scientists once referred to the drug as "Lazarene, because these kids were so sick and then they were well."

"This was an absolutely fatal disease until now," added Abbey Meyers, president of the National Organization for Rare Disorders, which is running a program for the drug's maker to ensure children who can't afford the super-expensive new therapy will get free or reduced-cost medicine.

Hereditary tyrosinemia type 1, or HT-1, is a genetic metabolic disorder that causes progressive liver failure and liver cancer in young children. It's very rare - fewer than 100 American children, and a few hundred more worldwide, are estimated to be living with the disease.

It is not among the metabolic diseases that all newborns are tested for at birth, so often infants die undiagnosed, Meyers said.

For babies who are diagnosed, the only treatment is a special low-protein diet. The reason: When the body breaks down the amino acid tyrosine, HT-1's genetic defect causes it to produce toxins that in turn cause liver cancer. Babies can't avoid all tyrosine or they'd never grow, so the diet helps but doesn't stop the disease.

Those who survive infancy rarely live into their 20s without a liver transplant.

Orfadin may significantly improve those dismal statistics: In an ongoing study of 180 patients who began the drug in infancy, 88 percent have survived four years and counting - far better than the normal 29 percent survival rate with diet alone.

It works by blocking formation of the liver-destroying toxins, Haffner said.

Orfadin didn't work for everyone and isn't a cure. Even in patients that the drug helped, no one yet knows if taking Orfadin for life could keep the disease at bay or if eventually they would need a liver transplant. Studies to determine that are continuing.

The drug's cost depends on the dose, which varies with the child's size: $12,000 a year for an infant, up to $60,000 a year for an older child, estimated Bo Allen, vice president of Orfadin's manufacturer, Rare Disease Therapeutics.

That's cheaper than a liver transplant, and thus many insurance companies are expected to pay for it, he said. But for families that can't afford the therapy, his company will provide it for free or at a lower cost in a program run by NORD. To enroll, families may call 1-800-999-NORD.

"We're not going to let any child go without the medication," Allen pledged.

About 10 years ago, scientists at Sweden's Gothenberg University discovered how HT-1 destroys the liver and began searching chemical databases for compounds that might block that disastrous action. They found a failed herbicide created by Zeneca Inc., which eventually donated the chemical for medical use, Allen said.

The tiny Nashville-based company won FDA approval under a special law that allows seven years of marketing exclusivity for products that treat rare diseases.


p>MEXICO CITY

Mexico's Grupo Bimbo buys five U.S. bakeries to double U.S. market revenues

Associated Press

Mexico's Grupo Bimbo, the world's third-largest bread maker, announced yesterday it had bought five U.S. bakeries in a deal that was expected to double the company's U.S. market revenues.

The $610 million agreement includes plants in Dallas, Denver, Beaverton, Oregon, San Francisco and Montebello, California.

In addition, Bimbo won the rights to make and distribute Orowheat bread, Entenmann's pastries, Thomas' English Muffins and Boboli pizza kits in the western United States. Bimbo said the agreement with Canada's George Weston Ltd. also grants the Mexican company the rights to about 1,300 direct distribution routes.

No layoffs were planned, said Guillermo Quiroz, Bimbo's chief financial officer.

George Weston's operations in the western United States, which employ 3,900 people, registered sales of $620 million in 2001 and posted earnings of $56 million.

Bimbo has focused on acquiring bakeries in the U.S. with large and growing Hispanic populations, purchasing in recent years Mrs. Baird's Bakeries in Texas and Four-S Bakeries in California.

With its latest U.S. acquisition, 30 percent of the company's sales, which totaled $3.3 billion in 2000, are expected to come from the United States.

 

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