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Farber's disease

Farber disease (also known as Farber’s lipogranulomatosis or ceramidase deficiency) describes a group of rare autosomal recessive disorders that cause an accumulation of fatty material in the joints, tissues and central nervous system. The disorder affects both males and females. Disease onset is typically in early infancy but may occur later in life. Children who have the classic form of Farber’s disease develop neurological symptoms within the first few weeks of life. These symptoms may include moderately impaired mental ability and problems with swallowing. The liver, heart and kidneys may also be affected. more...

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Other symptoms may include vomiting, arthritis, swollen lymph nodes, swollen joints, joint contractures (chronic shortening of muscles or tendons around joints), hoarseness and xanthemas which thicken around joints as the disease progresses. Patients with breathing difficulty may require insertion of a breathing tube. Most children with the disease die by age 2, usually from lung disease. In one of the most severe forms of the disease, an enlarged liver and spleen (hepatosplenomegaly) can be diagnosed soon after birth. Children born with this form of the disease usually die within 6 months.

There is no specific treatment for Farber’s disease. Corticosteroids may be prescribed to relieve pain. Bone marrow transplants may improve granulomas (small masses of inflamed tissue) on patients with little or no lung or nervous system complications. Older patients may have granulomas surgically reduced or removed.

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Smoking
From Topeka Capital-Journal, The, 8/25/02 by Kasha Stoll Capital-Journal

By Kasha Stoll

Special to The Capital-Journal

A blue-collar worker with a high school diploma or GED is more likely to smoke than a college-educated white-collar employee.

According to the U.S. Centers for Disease Control and Prevention, 37 percent of men and 33 percent of women in blue-collar jobs smoke. The numbers for white-collar workers are 21 percent and 20 percent, respectively.

"Despite an overall drop since the 1960s in the number of people who smoke, the rate of decline hasn't been equal for all groups," said Gloria Sorensen, Ph.D., director of Dana-Farber's Center for Community-Based Research.

Sorensen led a study examining workplace cessation methods at 15 manufacturing plants in Eastern Massachusetts. Investigators in the study theorized that blue-collar workers may see job-related hazards as a greater threat to their health than smoking.

The more education a person has, the less likely that person is to smoke. Women with nine to 11 years of education are three times more likely to be smokers than women with college degrees, according to the CDC.

Only 11.2 percent of female college graduates are current smokers.

In the United States, 24.1 percent of all adults smoke. By gender, the figures run 26.4 percent for males and 22 percent for females.

A cultural experience

Rates and habits vary considerably among different ethnic groups.

At 40 percent, Indians and Alaska natives have the highest tobacco- use rates of all major ethnic

Smoking: Cessation has been directed at whites

groups.

Compared with the general population, more than twice as many Indian males use smokeless tobacco.

Among Hispanics, 24.7 percent of men and 13.3 percent of women smoke. They average less than 15 cigarettes per day, or half that of other ethnic groups.

Almost 25 percent of blacks smoke. According to the CDC, this group tends to start smoking at a later age. They are more likely to attempt to quit smoking, but are less likely to succeed than whites.

Dr. Jasjit S. Ahluwalia, chairman of the department of Preventative Medicine and Public Health at The University of Kansas Medical Center, said that the black population had been largely ignored in studies aimed at smoking cessation. More than 93 percent of smokers in earlier Zyban studies had been white, and most were from middle- or upper-income households.

"Most researchers are educated whites," said Neal L. Benowitz, a researcher at the University of California at San Francisco.

"When they put together smoking interventions, they do it in ways that make sense to them. That doesn't work with other groups."

Ahluwalia and his team of researchers came up with educational materials and recruitment techniques designed to reach blacks. Their study proved that blacks can be successful when trying to quit smoking.

Since each ethnic group's experience with tobacco is different, Benowitz said that it was essential for smoking studies to focus on particular groups.

On average, blacks smoke fewer cigarettes per day than do white smokers, but 76 percent prefer mentholated cigarettes. Menthol has been associated with higher cotinine and carbon monoxide levels, both of which increase health risks. The menthol also produces a cooling sensation, allowing smokers to inhale more deeply and hold the smoke in their lungs longer.

In a 30-year span (1957-1987), the rate of lung cancer among black men increased by 259 percent and quadrupled for black women.

In Kansas, 3,800 people die each year from their own smoking, according to a report released by the CDC.

Another 330 to 590 adults, children and babies die because of second-hand smoke or women who smoke while pregnant.

A beginning ...

Every day, more than 6,000 children younger than 18 try smoking for the first time, according to the National Center for Tobacco- Free Kids. The CDC reports that sixth and seventh grade appear to be the peak years for first-time experimentation.

According to a nationwide survey conducted in 1999, roughly one out of four high school students admitted to smoking an entire cigarette before the age of 13.

In the United States, 34.8 percent of all high school students are current smokers. That number falls to 26.1 percent in Kansas, but those 55,000 teenagers buy nine million packs of cigarettes each year, according to the CDC.

"Kids want to be part of the group," said Mary Jane Hellebust, director of Kansas SmokeLess Kids Initiative. She said that smoking is presented as glamorous and cool, and as a way to be different.

The tobacco industry argues that it doesn't target children in its advertising, but Hellebust disagrees. She said children see the advertising more often, and that it is geared toward young people.

"What 35-year-old have you ever seen who is going to take up smoking?" she asked. "New customers come right out of the ranks of our children."

Peer pressure also plays a role in youth smoking.

Hellebust explained that children see things from a different perspective. If one of their friends smokes, they are likely to think a lot of children smoke.

More than a third of all children who experiment with smoking become regular, daily smokers before leaving high school, according to the CDC. The addiction rate for nicotine is higher than that of marijuana, alcohol or cocaine.

... And an end

Almost 75 percent of regular smokers in high school try to quit before graduation but are unable. Only three percent believe they will be smoking at all in five years, but the CDC reports that more than 60 percent still are smoking seven to nine years later.

Hellebust said many children are afraid of gaining weight.

While most people do gain weight initially, Sue Henke, Public Information Director of the American Lung Association, said many people lose the extra weight within two years.

Attempts to stop smoking can be costly, and a survey by the CDC published in November found that Medicaid programs in 17 states didn't cover any kind of smoking cessation.

In Kansas, Social and Rehabilitation Services will pay for one treatment per year for any individual who is Medicaid eligible, said Caleb Asher, spokesman for SRS.

Blue Cross and Blue Shield of Kansas doesn't offer any financial support in smoking cessation, according to Graham Bailey, director of communications and public relations. He explained that "history has shown" most people will need to repeat the programs several times before they are successful.

Even if a person has to cover the cost alone, Ahluwalia said the $160 cost of seven weeks of Zyban treatment is inexpensive compared to the cost of treating diseases caused by smoking.

LISA LAUCK/The Capital-Journal

There are 13 ashtrays available outside the Docking State Office Building in downtown Topeka, but maintanence staff spends time each day sweeping up cigarette butts.

Copyright 2002
Provided by ProQuest Information and Learning Company. All rights Reserved.

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