OU Cystic Fibrosis Center Achieves Top Rating
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Pediatric pulmonologist James A. Royall, M.D., with 9-year-old patient and Prue Armstrong, respiratory care technician. |
When it comes to keeping kids' lungs clear, the OU Cystic Fibrosis Center has every right to boast we're number one!
These three words are the perfect motto for a center directed by a man inclined to make his point with a sports metaphor.
"We are proud that our center has really good outcomes," says pediatric pulmonology professor James A. Royall, M.D., holder of the CMRI-C.R. Anthony Chair, and he offers this explanation for the center's success:
"If you look at a great football team, it's not because there's an amazing quarterback who can throw 80 yards, although that's a good thing to have. It's because everyone on the team applies the basics in a stringent fashion on a continuous basis.
"That, to me, is what helps patients do well versus poorly."
Good care of cystic fibrosis patients means aggressive care, Royall said. "One of the biggest things is to understand that you have to treat each episode of what we call pulmonary exacPediatric erbation, which is an episode of acute bronchitis, to maximum recovery - even if the patient doesn't look sick."
The reason is simple: "You treat aggressively in the present not because he's at risk in the present, but because you need to recover lung function, and you can only do it in the present."
Cystic fibrosis centers that are more aggressive with intravenous antibiotics and hospitalization of their younger, healthier patients wind up with patients who survive to be older, healthier patients, Royall said.
"In principle, that sounds like an easy thing to do. You have a 16-year-old patient whose lung function drops from 100 percent of predicted to 70 percent of predicted, but he doesn't look all that sick. You give a course of out-patient therapy, but if he doesn't improve and is still coughing a little bit, even if he looks fine, you admit him for two weeks and use aggressive lung treatment."
Royall acknowledged it would be much easier for both physician and parents to give in to arguments and hostility from a 16-year-old who wants to go to social events and be treated at home rather than to put their collective foot down and admit the teenager to the hospital.
However, taking grief from the patient is worth it, Royall said. "If you can bring them back to 100 percent, conceptually you've just bought them 15 years."
Children who once would not have lived to the first grade can now have full, productive lives until their late 30s, assuming they receive good nutrition and [respiratory] treatment beginning in infancy. Royall credits the national Cystic Fibrosis Foundation, founded in 1955, for spurring scientific discoveries, for founding and focusing delivery of care around the country's CF centers and for organizing clinical trials of new drugs.
Research milestones came with the identification of the defective CF gene and its protein product in 1989 and later with FDA approval of Pulmozyme®, to thin out the sticky mucus in the lungs, and inhaled tobramycin solution, to fight Pseudomonas aeruginosa, the most common source of chronic lung infections.
Oklahoma is the 11th state to screen newborns for cystic fibrosis, a change expected to result in marked improvement in the health of these children. Recently, the CDC has recommended that newborn screening for CF be universal across the United States.
Because many with cystic fibrosis are missing the pancreatic enzymes that absorb fat, the babies Royall saw in the past were often "scrawny, malnourished, sickly and with lung problems.
Nowadays with newborn screening, they're big, good-looking babies, and we can get them started right off on maintenance.
"Rather than wait for cystic fibrosis kids to be recognized because they're so sick, you recognize them before they get sick and hopefully, they won't get so sick," Royall said.
Royall and the center team cultures their young patients for Pseudomonas at every visit in an effort to hold this dangerous lung infection at bay for as long as possible. "In 90 percent of patients with CF, it becomes a chronic airway infection," Royall said, "but if we can delay its start to 20 years (of age) instead
of 2 months, we've done that kid a heck of a lot of good."
The OU CF Center is considered to be of moderate size. There are 120 patients, but only two physicians currently on staff to care for these and all other pediatric patients with pulmonary problems. Royall works with Nighat F. Mehdi and Hassan A Dbouk in treating pediatric pulmonary diseases.
A full-time center coordinator, a registered nurse, a respiratory therapist, a nutritionist and a social worker - all with a "sense of mission" - round out the center team. Each serves as a combination trouble-shooter, confidant, cheerleader, adviser and friend to patients and families who have immediate access to the centers' physicians, even on weekends.
"One of our secrets is that we're small," Royall said, and everyone on the team understands the importance of vigilance and aggressive care for the center's young patients.
