Cystic fibrosis a daily struggle but one with hope for Marysville family

Published 12:08 am Sunday, March 8, 2009

MARYSVILLE — From the moment she wakes up each day, 6-year-old Brenna Krug faces an hour and a half of medical treatments — a routine she repeats each night before she goes to bed.

There are two antibiotic pills to swallow.

There’s a black vest that she straps to her chest, connected by a plastic tube to an air compressor. Its rapid, jack-hammerlike compressions shake her 45-pound body like a leaf in a strong breeze, loosening the mucus that threatens to clog her airways.

It’s just one of the medical problems caused by cystic fibrosis, a genetic, ultimately fatal disease she was diagnosed with when she was 10 months old.

While being jiggled by the medical vest, Brenna holds a plastic tube to her mouth, a sophisticated vaporizer, breathing in the medications carried on its steamy vapors.

Before Brenna can eat, she takes three enzyme pills to help overcome the digestion problems also caused by her disease. And there are vitamins to help ensure her nutrition.

“If you count all the pills she takes, it’s well over 20 pills a day,” her mother Brandy Krug said.

Even though one out of every 28 people carry the gene for cystic fibrosis and it affects 30,000 children and adults nationally with about 650 in Washington, it doesn’t have the kind of public awareness — and understanding — of diseases such as cancer or leukemia.

That’s something those affected by the disease hope to change, in part through a March 15 event at Comcast Area at Everett. Singer and songwriter Barry Manilow is donating net proceeds from his 8 p.m. concert to the Cystic Fibrosis Foundation.

The concert is part of a long-term commitment by Portland businessman Harvey Platt to cystic fibrosis research. Platt is chairman of Platt Electric, with branches in five Western states, including three in Snohomish County.

Platt said his first fundraiser for cystic fibrosis was in 1989, initially drawn to the cause by an employee whose son was diagnosed with the disease.

Through what he candidly describes as a strategy combining begging and being “pleasantly annoying,” Platt was able to talk first to business associates of Manilow and then Manilow himself about a 2007 benefit concert in Portland.

In August, when Manilow heard that Platt was trying to organize a similar concert in the Seattle area this year, Manilow quickly agreed to do it, Platt said.

As of late last week, about 4,300 tickets had been sold for the March 15 concert, Platt said. With an expected last minute push of about 1,000 more tickets this week, he estimates the event could net close to a quarter million dollars for cystic fibrosis.

Platt said that Manilow, who often is involved in charity causes, is an unusually humble performer.

Manilow displayed his self-effacing humor during a recent interview with Seattle radio station KRWM about the Everett concert.

After the interviewer noted it was a benefit for cystic fibrosis, Manilow joked, “Even if you don’t like me, you should come.”

The Cystic Fibrosis Foundation helps fund research at medical centers throughout the country, including the Seattle Children’s Research Institute, where scientific studies also are funded through the National Institutes of Health.

One of the breakthroughs pioneered there is the use of inhaled antibiotics to treat lung infections.

Cystic fibrosis leaves Brenna vulnerable to common wintertime viruses, despite having the strength and energy to participate in weekly gymnastics classes.

“By looking at her, you couldn’t tell she was sick,” her mom said of her brown eyed, energetic daughter.

For cystic fibrosis patients, just catching a common cold can result in a trip to the hospital, said Dr. Bonnie Ramsey, a cystic fibrosis researcher at Seattle Children’s Research Institute.

“It creates … an environment where bacteria can grow,” sometimes triggering bronchitis or pneumonia, she said.

In fact, Brenna has been on antibiotics since April of last year, after being treated at Seattle Children’s Hospital for a bacterial lung infection.

Her mom said she worried about Brenna’s health nearly from the day she was born. There was wheezing when she breathed. Lower than usual amounts of oxygen in her blood. Abnormal sleeping problems. And a voracious appetite that had her devouring 16 ounces of milk at one feeding, four times the amount for a typical newborn.

“We ended up at the hospital emergency room several times,” Brandy Krug said, but medical staff couldn’t pinpoint the problem. “All the doctors thought she had a viral infection or a cold.”

Finally, a Snohomish Health District nurse who came to their home heard Brenna’s wheezy breathing and told her mom: “This isn’t normal.”

Her daughter was referred to Seattle Children’s Hospital, where her problems were finally diagnosed.

Washington began screening all newborns for cystic fibrosis on March 15, 2006.

For Brenna, the disease intrudes in ways beyond the morning and evening rituals of medical treatments.

It prevents her from having girlhood sleepovers.

To leave, even for a day, means packing up two to three bags of equipment and medications. “It’s like I’m going away for a week,” Brandy Krug said.

And at picnics, “if someone tries to give her a cookie, I’m running after her saying, “No way, you have to have the enzyme pill first.

“The other thing we have to steer clear of is sick children,” her mom said. It’s often misconstrued by those who don’t know about her disease as being overly protective.

Medical advances have dramatically changed how long cystic fibrosis patients are living.

When Ramsey, the Seattle cystic fibrosis researcher, first began her career 30 years ago, “most of these patients lived to be 12 or 13, she said. “Now it’s close to 40.”

Researchers are at an exciting time of transition, Ramsey said, “where we could see a dramatic change in the health of these patients.”

Tests are now under way on medications that, if ultimately found to be effective, could allow cystic fibrosis patients to extend their life expectancy and live a normal life.

“We have made a phenomenal difference in these people’s lives,” she said. “But to us a cure means their life span is the same as anyone else’s.”

While progress toward this goal is being made “we’re not there yet.”

Reporter Sharon Salyer: 425-339-3486 or salyer@heraldnet.com