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Stephanie Buhr coaxes a smile out of her 3 and a half year old daughter Kylie while sitting at Mill Pond Friday afternoon. Kylie was diagnosed with Cystic fibrosis when she was only three. The two will take part in the Great Strides Walk on this coming Saturday in Silver Lake Park in Rochester.

Living with a smile

Stephanie Buhr and daughter Kylie, who has cystic fibrosis, will participate in Rochester's Great Strides event in May

By Lee Bonorden (Contact) | Austin Daily Herald

Published Monday, April 27, 2009

Cystic fibrosis is an inherited chronic disease that affects the lungs and digestive system of about 30,000 children and adults in the United States (70,000 worldwide).

Every day, Stephanie Buhr and her daughter, Kylie, wake up to dealing with cystic fibrosis’ life sentence for the 3 1/2-year-old child.

A defective gene and its protein product cause the body to produce unusually thick, sticky mucus that clogs the lungs and leads to life-threatening lung infections; and obstructs the pancreas and stops natural enzymes from helping the body break down and absorb food.

Kylie was diagnosed with cystic fibrosis when she was 3 months old, according to her mother.

A few months later, the state of Minnesota authorized newborn screening for cystic fibrosis

Kylie may be one of the lucky ones, if that is the correct word, who have cystic fibrosis. She was born Nov. 19, 2005.

In the 1950s, few children with cystic fibrosis lived to attend elementary school. Today, advances in research and medical treatments have further enhanced and extended life for Kylie and other children and adults with CF. Many people with the disease can now expect to live into their 30s, 40s and beyond.

“It is what it is,” Buhr said. “I wouldn’t trade her for any other child in the world.”

Buhr has lived in Austin for two years after moving here from Bloomington.

Her mother, Karen Buhr, also lives in Austin, and she has a brother.

Kylie Nienow’s father, Brandon, lives in Brownsdale.

The mother is employed by the Austin Daily Herald as a customer service coordinator.

When doctors told the mother her first and only child had CF, “It was shocking. You never imagine something like that can happen to your child, when she was just born.”

According to the CF Foundation, people with CF can have a variety of symptoms, including:

• very salty-tasting skin;

• persistent coughing, at times with phlegm;

• frequent lung infections;

• wheezing or shortness of breath;

• poor growth/weight gain in spite of a good appetite; and

• frequent greasy, bulky stools or difficulty in bowel movements.

Every day of her daughter’s life, Buhr must administer vest therapy to her. “It takes 30 minutes at a time, and we have to do it twice a day every day,” Buhr said. “They say she will have to keep doing it when she grows up.”

There are also nebulizer treatments and the child takes medications to ward off more symptoms.

Statistics

There are other higher profile diseases, but CF statistics speak for themselves:

• About 1,000 new cases of cystic fibrosis are diagnosed each year.

• More than 70 percent of patients are diagnosed by age two.

• More than 40 percent of the CF patient population is age 18 or older.

• The predicted median age of survival for a person with CF is more than 37 years.

“She’s a normal-size child for her age, and she’s active and does things any other child does. It’s just that cystic fibrosis is with her all the time,” the mother said.

But Buhr stopped short of calling it a “burden.” Only something the mother and child must deal with in life.

Since 1955, the Cystic Fibrosis Foundation has been the driving force behind the pursuit of a cure. Thanks to the dedication and financial backing of supporters, patients, families and friends, clinicians, researchers, volunteers, individual donors, corporations and staff, the organization claims it is making a difference.

The foundation invites the public to also make a difference by participating in an annual Great Strides Walk for awareness and medical research funds.

Buhr and her daughter, Kylie, will be participating in a Saturday, May 2, event at Rochester, beginning 9 a.m. at the Silver Lake Park.

Presently the “CF Mom,” as she calls herself, is raising donations and pledges for the May 2 Great Strides Walk.

Her Web site for the walk is www.cff.org/great_strides/stephbuhr

For more information about cystic fibrosis go to

www.cff.org

If people wish to donate at other times, they can donate online or drop a check off at the Austin Daily Herald payable to the Cystic Fibrosis Foundation.

There was no indication the CF defective gene existed on either side of Kylie’s family. As she grows up, the child will have to adjust to her condition.

“You would never know she has cystic fibrosis to see her,” said the mother. “It’s in her genes, so it’s forever.”

The mother’s repeated use of the word “forever” indicates she has reconciled herself to special circumstances for the family.

Twice before she has participated with Kylie in other events on behalf of the Cystic Fibrosis Foundation in the Twin Cities and now the Great Strides Walk is an opportunity to help make a difference.

“It’s just something we want to do. Something I want to do as a CF Mom,” she said.

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