A Newtown Family Joins The Fight Against Cystic Fibrosis

By Kaaren Valenta

A cheerful, happy baby, Cameron Powers celebrated her first birthday this week, unaware that she has cystic fibrosis. The daughter of Patty and Steven Powers of Newtown, Cameron was 5½ months old when she was diagnosed with the genetic disorder that affects approximately 30,000 children and adults in the United States.

“You couldn’t tell by looking at her that she has cystic fibrosis,” her mother said. “She is basically very happy.”

Cystic fibrosis occurs in approximately one of every 3,900 births in the United States and while no cure has been found, advances in medicine have extended the life expectancy of a disease that used to claim children before they were teens.

“The average life expectancy is now 32,” said Mrs Powers. “We are really hoping to come up with a cure. Researchers have come up with the cause. Ninety percent of the money raised for the Cystic Fibrosis Foundation goes for research.

In cystic fibrosis, a defective gene causes the body to produce an abnormally thick, sticky mucus that clogs the lungs and leads to life-threatening lung infections. These thick secretions also obstruct the pancreas, preventing digestive enzymes from reaching the intestines to help break down and absorb food.

More than ten million Americans are unknowing, symptomless carriers of the defective CF gene. An individual must inherit two defective CF genes — one from each parent — to have CF. Each time two carriers conceive, there is a 25 percent chance that their child will have CF; a 50 percent chance that the child will be a carrier of the CF gene; and a 25 percent chance that the child will be a noncarrier.

“One in 29 white people have the gene,” Mrs Powers said. “They have to marry someone else who also has the gene for a child to get it and even then there is only a one in four chance.”

About 1,000 new cases of CF are diagnosed each year. More than 80 percent of patients are diagnosed by age 3; however, nearly ten percent of newly diagnosed cases are age 18 or older.

Cameron was about 5 months old when she got a cold that turned into pneumonia.

“We kept taking her to the doctor, then twice to Danbury Hospital,” Mrs Powers said. “She wasn’t tested for CF initially because most CF children are underweight, and Cameron was so plump.”

Once the diagnosis was made, on Columbus Day, Cameron was transferred to the Children’s Medical Center in Newington where she was treated for both CF and pneumonia. After two weeks in the hospital she was sent home with a shunt in her arm to continue IV treatment.

“It was a very scary time until she was over the pneumonia and until the tests were done on our son, Shane, who is 3½,” Mrs Powers said. “He does not have cystic fibrosis.”

People with CF have a variety of symptoms including persistent coughing, wheezing, or shortness of breath and poor weight gain no matter how much they eat. Symptoms vary from person to person due, in part, to the more than 1,000 mutations of the CF gene. The treatment also depends upon the symptoms and the stage of the disease.

“The key is the treatment,” Mrs Powers aid. “Cameron gets albuterol through a nebulizer twice a day, much like children who are asthmatic. She has to take enzymes to help digest the food. We also do chest physical therapy for 10 to 15 minutes twice a day.”

Chest physical therapy is a form of airway clearance done by vigorous clapping on the back and chest to dislodge the thick mucus from the lungs. “We do a lot of bouncing on an exercise ball, too. She loves it,” Mrs Powers said. “The more active, the more sports, the better. Swimming is a great exercise because it helps the lungs. Her mommy is athletic so I hope she will be, too.”

Patty Hensel Powers and Steve Powers are both Newtown natives. She teaches fifth to eighth grade physical education and health in the Wilton schools; he works for Home Depot.

“After we got married, we lived in Southbury, then sold that house and moved in with my mother at the house on Philo Curtis Road, where I grew up, while we built our house, a log cabin, on Lincoln Road in 1999,” Mrs Powers said. “Now she is going to move in with us.

“Steve and I worked out our schedules so we don’t have to use day care,” she said. “That’s especially important now.”

The middle school where Mrs Powers works sponsored a “Shoot for the Cure” event that raised $21,000 for the Cystic Fibrosis Foundation.

On Sunday, May 16, there will be a fundraising walk, Great Strides, for the CF Foundation, beginning at the Danbury War Memorial at 9:30 am.

“It’s a five-mile walk but you don’t have to walk all of it, or any of it, to help,” Mrs Powers said. “I am organizing Team Cameron and invite everyone to join us. Call me at 364-1880.”

Information about CF and the CF Foundation national and Connecticut chapters is accessible on the Internet at www.CFF.org. Persons who want to contribute, but not walk, can send checks made out to the CF Foundation to Mrs Powers at 24 Lincoln Road in Newtown.