Cystic fibrosis program grows up

Center established for adults with childhood disease

Molly Pam takes daily steps to control her cystic fibrosis, a disease of the lungs and digestive system.

In her sophomore year at Stanford, Molly Pam had a once-in-a-lifetime chance to board a research vessel for a month long trip in the Sea of Cortez.

Taking Holistic Biology, an interdisciplinary course on the history, biology and literature of Monterey, Calif., and Baja, Mexico, would be cool, she thought. It also would be her longest adventure away from home or school. "I had no phone or Internet access," Pam recalled. "I was very much on my own in terms of my health."

Such a trip would have been unthinkable a few decades ago for someone like Pam, who has cystic fibrosis. At that time, most people with the disease did not live past childhood. But Pam, who is now 21, is emblematic of the change in management of CF patients, who are living well into adulthood.

Before leaving for the Sea of Cortez, Pam consulted with her doctors at Stanford Hospital & Clinics about her treatment plan and how to deal with any issues that might arise. Although her lung function is less than half of normal capacity, she experienced no health complications during the trip.

Cystic fibrosis is a genetic disease of the lungs and digestive system that affects approximately 30,000 people in the United States. "In the 1970s, CF was a fatal pediatric disease," said David Weill, MD, director of the Adult Cystic Fibrosis Center at SHC. Today, he said, average life expectancy is about 38 years.

As associate professor in the Division of Pulmonary and Critical Care Medicine, Weill heads the adult cystic fibrosis program, which opened new clinic space in Stanford Hospital in November.

Stanford's CF center for adults is the largest in California, with almost 200 patients, most of them graduates of the program at Lucile Packard Children's Hospital. By age 18, patients like Pam know the daily drill. She takes Colistin, an inhaled antibiotic, and Pulmozyme, an inhaled mucus thinner, by nebulizer twice a day. That adds up to 20 minutes in the morning and 20 minutes in the evening. Pam also puts on a mechanical vest twice a day, for 20 minutes at a time, which vibrates her chest and breaks up the sputum in her lungs.

The daily skirmishes with CF are aimed at getting rid of the sticky secretions that clog the lungs. Thick mucus can lead to a buildup of bacteria, with resulting pulmonary infections.

David Cornfield, MD, who directs Packard Children's Center of Excellence in Pulmonary Biology, said that having a transitional program, with patients moving smoothly from pediatric doctors to adult CF physicians, "has been the hope and dream of pediatricians since the early 1990s."

Working together, he and Weill determined that the best caregivers for pediatric patients transitioning to the adult hospital would be physicians who had expertise in both CF and lung transplantation. A transplant is not a cure for CF, but it can extend a patient's life for a decade or more, Cornfield said.

For more information about Stanford's cystic fibrosis center, visit http://medicine.stanford.edu/patient_care/cysticfibrosiscenter.html.