Newswise — Julia Rae Schlucter and Cameron Grant were born with cystic fibrosis (CF), an inherited, chronic, progressive disease that affects respiratory and pancreatic function, as well as other organ systems. Both artists and athletes, they refuse to let the disease define their lives, and consider themselves integral partners in the care they receive at the comprehensive Cystic Fibrosis Center at Rutgers Robert Wood Johnson Medical School and Robert Wood Johnson University Hospital, one of only three accredited CF centers in New Jersey. In 2016, the CF Center ranked highest nationally and in New Jersey in a composite score for lung function and nutritional status.
CF is an autosomal, recessive disease, caused by a mutation in the CFTR gene. 1,000 new cases are diagnosed annually in the United States, where 30,000 people are living with the disease. A major breakthrough came in 1989, when CFTR was identified. As a result, median life expectancy for patients with CF has risen from 3 years of age in 1950 to more than 45 today.
Many CF patients suffer from frequent infections and loss of function in the affected organs. Because early diagnosis has proven to provide a solid prognosis, all 50 states now require that newborns be screened for CF. The CF Center recently partnered with the New Jersey Department of Health, and received a two-phase grant from the CF Foundation that will improve the state’s CF screening for newborns. “This new grant will improve our ability to diagnose CF in its earliest stages, before lung damage occurs, resulting in better outcomes for patients,” Says Thomas F. Scanlin, MD, professor of pediatrics, chief, division of pediatric pulmonary medicine, and director of the pediatric/adolescent program,
Cameron suffered a CF crisis a few years ago when she was 12. She coughed endlessly, lost weight rapidly, and was exhausted from night sweats and high fevers. The specialist she had been seeing since age 4 insisted that she had contracted a virus and that her symptoms were a natural progression of the disease. However, her mother disagreed with the physician and when she took her to an urgent care center, blood work revealed a lung infection. Back home, Cameron’s mother desperately searched the internet, and finding the CF Center at Robert Wood Johnson Medical School, she called. “They were swamped that afternoon, and they’d never heard of me,” she says, “but they found their social worker in the hall. She picked up the phone and listened. She urged me to bring Cameron in to be seen.”
The next day, the Grants met with Dr. Scanlin and the CF Center team. Cameron was immediately admitted to The Bristol-Myers Squibb Children’s Hospital at Robert Wood Johnson University Hospital, where she spent two weeks being treated for a lung infection. “I was so scared in the hospital, but Dr. Scanlin was great: he made sure I had the nicest nurses, and he came to see me all the time,” Cameron says. “He’s so chill.”
Cameron recently began taking Symdeko, a drug that targets the root cause of CF, and she is doing very well. In addition, she follows a strict, twice-a-day regimen of self-administered therapies. She has an overnight feeding tube fitted with an enzyme-packed cartridge that helps predigest her food. Twice a day, she uses a nebulizer to open her airways and wears an inflatable, vibrating vest for 20 to 30 minutes to shake the mucus free from her lungs so she can “huff” it out.
CF has not hindered Cameron from excelling in field hockey. She was chosen for her high school’s freshman team, while remaining a straight-A student and studying violin.
From birth until her recent transition to the CF Center’s adult program, Julia was under Dr. Scanlin’s care—first at CHOP and then at the CF Center in New Brunswick, when he moved there in 2005. “A lot of kids with CF become athletes or artists,” Julia says. And she is both. By age 6, she was performing in musical theater; at 15, record producers were contacting her. Hoping to advance her career, she moved to Los Angeles, where her health declined precipitously. After two hospitalizations with lung infections and a collapsed lung, she returned home and was accepted at Fordham, where she earned a bachelor of arts degree in media and communications.
Dr. Scanlin’s proactive, goal-directed approach complements Julia’s intense motivation to realize her professional goals while living with CF. Her treatment regimen has included twice-a-day airway clearance and the use of older drugs in combination—while taking Symdeko and participating in clinical trials. Her lung function has reached 120 percent of the predicted volume, whereas 60 to 80 percent is more typical for young adults with CF.
Performing professionally as Julia Rae, Julia has won pageant crowns and scholarships since she was 16, the age at which she founded the CF research–focused nonprofit Singing at the Top of My Lungs. She recorded the song “Be That Girl” for the film The Greening of Whitney Brown and, in 2016, surpassed her own expectations by completing the New York Marathon. Currently, she serves as a digital sports reporter for Channel 6abc Action News, in Philadelphia.
Cameron and Julia are role models for thousands of other young people living with the disease. Now CF is at the threshold of better treatments and perhaps, one day, a cure. While already living life to the fullest, Julia and Cameron look forward to celebrating that day.
Robert Wood Johnson Medical School and Robert Wood Johnson University Hospital, an RWJBarnabas Health facility and the medical school’s principal affiliate, comprise one of the nation’s premier academic medical centers. Clinical services are provided by more than 500 faculty physicians in 200+ specialties and subspecialties as part of Rutgers Health, the clinical arm of Rutgers University. To learn more about Rutgers Robert Wood Johnson Medical School, visit rwjms.rutgers.edu.