Hypertonic saline spray offers new treatments, hope for cystic fibrosis patients

SOUTH BEND -- The salty mist that Devon Conley inhales throws him into a red-faced fit of coughing. Sometimes the fit continues for five minutes or more. But it's actually a good thing, said the 17-year-old from South Bend.\n"I can feel I've definitely cleared a lot of crap out of my lungs," he said.\nDevon has cystic fibrosis, a rare genetic disease that causes sticky mucus to clog the airways. It leaves him vulnerable to life-threatening bouts of pneumonia.\nTo say cystic fibrosis affects his life is an understatement.\n"I don't go to school anymore," Conley said. "My doctor took me out because it's too dangerous for me to be around other people for eight hours a day."\nDr. James Harris is hoping that a new, low-tech and inexpensive treatment makes Conley's life easier.\n"This is the single most exciting development I've seen in CF," said Harris, one of two local doctors who treat patients at a special cystic fibrosis clinic at Saint Joseph Regional Medical Center, South Bend.\nIt's called hypertonic saline, which means nothing more than sterile water loaded with salt.\nThe fact that it's twice as salty as seawater is no coincidence.\nHypertonic saline treatment was inspired by some surfers in Australia who, like Conley, also have cystic fibrosis. They told their doctors that what made them feel especially good was a day on the ocean waves.\nResearchers in Australia and at the University of North Carolina at Chapel Hill put the surfers' tale to the test.\nWith backing from the U.S. Cystic Fibrosis Foundation, they had a group of CF patients inhale 7 percent saline solution twice daily for a year while a control group inhaled normal saline.\nThose receiving salty mist had improved lung function and, more importantly, fewer serious lung infections requiring hospitalization.\nThe report appeared Jan. 19 in the New England Journal of Medicine and was communicated to doctors in a foundation bulletin.\nHarris started using it the very next day, starting with Conley and another patient. Both were in the hospital receiving intravenous antibiotics to battle lung infections.\nThe treatment makes sense, Harris said, because cystic fibrosis itself is fundamentally tied to salt.\nIn CF, people lack genes to make a protein that transports salt across cell membranes. Doctors diagnose the disease by looking for excess salt in the patient's sweat.\nMany organs are affected, but with the lungs it's a life-threatening disaster. The surfaces of the air passages, which should have a thin coating of water, dry out and mucus becomes sticky.\nThe passages also become infected and inflamed, Harris explained, which doctors believe leads to the progressive scarring of tissue known as fibrosis.\nBut inhaling saltwater appears to rehydrate those passages and make them slick like they're supposed to be.\nDr. Matthew Koscielski considers the saltwater treatment to be one more useful tool, not a true breakthrough.\n"It's not earth-shattering," he said. "But I think it's helpful"