Scattered on the lawn of Cameron and Chelsea Pratt’s white clapboard house in Kentlands are helmets, a soccer net and a tiny pink bike. Inside, parents Cameron and Chelsea sit talking in the living room as Ben, 7, and Lauren, 4, run in and out of the room, laughing and playing.
Watching them, you’d never guess that both children have a life-threatening illness. Ben and Lauren were born with cystic fibrosis, or CF, a rare genetic disorder that causes the body to produce a faulty protein that leads to abnormally thick, sticky mucus that obstructs both the lungs and the digestive system. Children with CF typically tire easily and are smaller than average, but these two are as lively as jumping beans. They are slender, like many CF children, though no more so than any kid who’d rather play than clean his or her plate. Their frequent hacking cough, despite its alarming sound, is welcome in the Pratt household, because it means that Ben and Lauren are keeping their airways clear.
The Pratts were thrilled when they learned that Chelsea was pregnant less than a year after their January 2000 wedding. Both come from large, close-knit families, and they hoped to have several children. Cameron, 34, is one of the 48 grandchildren of Sid Foulger, founder of Foulger and Company, whose iconic project, the Mormon temple in Kensington, was completed in 1974. In the early 1970s, Foulger’s six children and their spouses bought a piece of farmland on Piney Meetinghouse Road in Potomac and built six handsome and comfortable houses around a wide oval lawn. Cameron, the oldest of eight brothers and sisters, all of whom attended Bullis School, recalls an idyllic childhood playing with his siblings and cousins in the nearby woods and fields. “He was the ideal older brother,” says his sister Christi. “Kindhearted and gentle, and at the same time you felt you had someone looking out for you.”
Cameron met and fell in love with Chelsea Ross at Brigham Young University in Salt Lake City in the early 1990s. Chelsea, 33, grew up in San Diego, Calif., with two brothers and two sisters. Her two younger sisters, Sharlie and Lexie, both had CF.
More than 10 million Americans, mostly of Northern European and Jewish Ashkenazi descent, are genetic carriers of CF. Carriers have one copy of the defective CF gene, but do not have the disease or its symptoms. During Chelsea’s pregnancy, doctors assured her and Cameron that it was unlikely they would have a child with CF. The CF gene is autosomal recessive, meaning that although Chelsea had a 50 percent chance of being a carrier the gene would also have to be present in Cameron’s DNA, and there was no history of the disease in his vast family.
“Out of curiosity, more than worry,” Cameron says, he decided to have a genetic test, and was astonished to learn that he was a CF carrier. Chelsea was tested next and, surprisingly, learned that she also carried a copy of the mutant gene. Although there was only a 25 percent chance that their child would inherit both CF genes, the Pratts decided to undergo an amniocentesis. It turned out that their unborn son, Ben, indeed carried two mutant genes and would be one of the 30,000 people in the U.S. with CF.
“I wouldn’t say the doctors pushed us to have an abortion,” Cameron says, “but they let us know it was an option.”
“It’s not something we’d ever, ever do,” Chelsea says, her arms around their 1-year-old, Ethan, who does not have CF. “But especially because of my sisters: How could we say this baby shouldn’t be born because he’ll have challenges? There are many things in life that are worse than CF.”
‘Woe is the child…’
Long before CF was identified as a disease in the 1930s, its symptoms were mysterious and terrifying to parents. “Woe is the child kissed on the forehead who tastes salty, for it is cursed and soon must die,” reads one 18th-century German medical text. Such children could not digest their food and failed to grow, usually succumbing to pneumonia before they reached school age. Chelsea’s sister Sharlie, born in 1980, was sickly for a year before her pediatrician, on a hunch, licked her foot. Its salty taste made him suspect CF, which was confirmed by a sweat test, which measures the amount of sodium released in a person’s sweat. (An inability to retain sodium is what causes the thick mucus of CF patients.) Six months later, Chelsea’s sister Lexie also was born with the disease.
Although antibiotics and oral pancreatic enzymes had increased life expectancy for CF children, the prognosis for Sharlie and Lexie in the 1980s was devastating—14 years. The life of the family changed completely, Chelsea says. All active pursuits were avoided, because it was believed then that exercise led to dehydration, fevers and infection for CF patients. “Anything that caused [my sisters] to get overheated was really scary,” she says. Despite the family’s caution, lung damage caused by repeated infections led Lexie to have a double lung transplant, and she died of complications at age 14. Sharlie, now 28, is the mother of a 1-year-old child, but her lung capacity is about 17 percent. “When you see her X-rays, you can’t believe she’s walking around,” Chelsea says. “That she’s lived this long and had a baby is a miracle.”
Improved therapies, including aerosolized antibiotics that work directly on the lungs, have increased the life expectancy of CF patients to about 37 years, but there is still no cure for the disease. The Pratts are optimistic that one of three drugs currently in the final stage of clinical trials will be able to alter the defective protein that acts like a stuck trap door. Until a medicine can be found that treats the cause of the disease rather than its symptoms, the goal is to protect children’s lungs from permanent damage. “Even if a pill came out tomorrow,” Cameron says, “Chelsea’s sister will always have 17 percent lung capacity.”
So far, the Pratts feel they have been extraordinarily lucky. Since his birth on May 5, 2001, Ben has had to use aerosolized antibiotics only once. Doctors now encourage, rather than forbid, exercise for CF children in order to build strength and increase lung capacity, so Ben plays soccer and lacrosse, and the family goes on frequent walks and bike rides. A generation ago, CF kids were sent to special summer camps until it was discovered that the children, being in such close proximity, were contaminating each other with opportunistic pneumonia bacteria. The Pratt children attend regular classes; Ben at Rachel Carson Elementary School in Gaithersburg, and Lauren at Epworth Preschool, also in Gaithersburg. Their parents remind them to wash their hands often and try to keep them away, as much as possible, from people with colds and the flu. Since they cannot digest their food without the help of supplemental enzymes, Chelsea packs enzyme tablets in the kids’ lunch boxes. “We say, ‘if your friends ask you what the pills are, you tell them,’” Cameron says. “There are different ideas about raising kids with CF. We tend to talk about it pretty openly.”
“I don’t think we’ve ever said that people die [from CF],” Chelsea says when the children have run into another room, out of earshot.
“I’ve told Ben that when he asked,” Cameron amends. “But then I said, ‘But you guys are really fortunate because you’re so healthy.’ ”