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Making great strides

Cystic fibrosis sufferers, family members walk to fund research

Cystic fibrosis sufferers, family members walk to fund research

May 06, 2002|BY KEVIN CLAPP

kevinc@herald-mail.com

Kaelii Stout is a growing girl. And for that her mother is truly thankful.

In the first quarter of 2002, Paula Stout's oldest daughter gained weight in an almost unprecedented fashion: Four pounds.

The eight-year-old tips the scale at 49 pounds.

"If she gained a pound we were lucky," Paula says of Kaelii's usual weigh-ins.

Kaelii Stout, small size aside, looks and acts for all the world like a normal girl. She is knee-deep in the third grade; she likes to play with matchbox cars.

She also has cystic fibrosis, an easily misdiagnosed condition requiring the Smithsburg girl to pop enzyme pills with each meal and endure an hour of therapy each night before bed to loosen the thick, sticky phlegm building up in the lungs in her tiny chest.

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A genetic disease, cystic fibrosis causes the body to produce the mucus due to poor transport of salt in cells that line organs such as the lungs and pancreas. Early on, symptoms can mirror those of asthma or recurrent pneumonia.

The mucus clogs the lungs, leading to increased risk of infection. It also obstructs the pancreas, preventing enzymes from reaching the intestines to digest food.

For the next month, the Maryland Chapter of the Cystic Fibrosis Foundation will conduct 14 Great Strides walks, including events in Smithsburg and Shepherdstown, W.Va., on Saturday, May 11, to raise money and awareness to combat a disease that may afflict only 30,000 people nationwide but reaches far beyond its victims.

Kaelii is one of those people. So is four-year-old Faith Stone, a vibrant girl with long blonde hair who entered pre-school this year and takes ballet lessons.

Like Kaelii, Faith is the picture of carefree youth, hemmed in only by the half hour each day spent plugged into her pneumatic vest and her daily diet of medication - 20 pills per day not counting the large orange chewable vitamin stuffed with vitamins A, D, E and K, plus vest time. But her mother, Trinsy Stone, is proud of her littlest one.

"She doesn't let the fact that she has to stop and take some pills impact her life," Stone says.

One in 31 Americans carry the defective CF gene, meaning 10 million people have at least a chance of passing the disease to their children.

When two carriers of the defective gene have children there is a 25 percent chance of having a child with cystic fibrosis. There is a 50 percent probability the child will be a symptomless carrier and a 25 percent chance they will be a noncarrier.

Kaelii's younger sister Regan, 6, and Faith's older sibling Fallon, 6, are both carriers.

Like Kaelii, Faith must take enzymes prior to each meal. Both children also log time in a cumbersome vest about the size of a suitcase.

Strapped in, the contraption shakes each child to loosen mucus from the chest. For a half-hour each night, Kaelii wears the vest. Faith spends 15 minutes in the morning, 15 more at night.

As CF patients get older, it becomes more difficult to control the mucus buildup in the body. Since phlegm traps bacteria, much like insects stuck to fly paper, research centers not only on finding a cure but also on arresting symptoms.

Pediatric pulmonologist Peter Mogayzel, director of the pediatric lung transplant program at Johns Hopkins Medical Center for four years, says advances are coming at a rapid pace.

Advances extend life

Where life expectancy for CF patients once ended in childhood, more are living into their mid-30s. Inhaled medicines help break down mucus, making it less thick and tacky.

A number of early intervention trials are ongoing to test new remedies for use in younger children to delay the onset of any problems. And for the past decade, lung transplantation has become another recourse for patients with advancing CF.

The trade-off in these instances, Mogayzel says, is exchanging CF for the risks of infection and rejection.

"The hope of a transplant is not only that you extend their lives," he says. "But create a better quality of life."

Stone and Stout are hopeful for a cure, but they temper their enthusiasm. A better life, a longer life, is what they hope for their children.

Each walker counts

Great Strides ensures that work towards treatment and a cure continues. According to Trisha Tatam, director of special events at the Maryland Chapter of the Cystic Fibrosis Foundation, all money raised in the state goes to Johns Hopkins, one of several care centers nationwide.

And, since 96 percent of the money raised goes to research, Tatam says the boon to research is enormous. The goal for Maryland is to raise $640,000, which would represent a $90,000 hike over last year.

Stout hopes to generate $20,000 at her Smithsburg walk, organized by Kaelii's mother, Mary Ridenour. Stone's goal is $40,000, though she could care less where the money is raised as long as it is.

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