The number of patients who seek treatment at Deaconess Medical Centers cystic-fibrosis clinic has risen in recent years, but those who operate the clinic say the increase in activity is occurring for the right reasons.


Many of those who suffer from cystic fibrosis are being diagnosed earlier than they had been in the pastand before severe lung problems have developedand are receiving more aggressive treatment, says Dr. Michael McCarthy, a pediatric pulmonologist and pediatric allergist and immunologist who serves as the clinics medical director. Consequently, some patients are living longer than they might have if they hadnt begun receiving treatment until later, McCarthy says.


H says the median life expectancy for cystic-fibrosis sufferers currently is 33 years old. Thats a dramatic improvement compared with 20 years ago, when the median life expectancy was 18 years old. Some cases have been reported in which people lived into their 60s with mild forms of the disease, though McCarthy says he has never encountered such patients.


Karen Harrington, the centers nurse coordinator, says, We have patients who are adult professionals. They were told, Youll be lucky if you can get to 7 or 10 (years old).


Cystic fibrosis is a genetic disease through which a defective gene causes the body to produce unusually thick mucus that can clog the lungs and lead to fatal lung disease, according to the Cystic Fibrosis Foundation. The disease also can lead to obstructions in the pancreas, preventing digestive enzymes from reaching the stomach, and can block the bile duct in the liver, leading to liver disease, the foundation says.


The primary concern, McCarthy says, is taking care of a patients lungs and working to minimize inflammation and infection in them.


Despite the gains, nearly all cystic-fibrosis sufferers die due to complications of the disease, McCarthy says. Most of the time, those patients die of lung problems, but in a small percentage of cases, they succumb to liver disease.


At the Deaconess CF clinic, which is one of 117 nationwide accredited by the CF Foundation, two-thirds of the clinics 126 regular patients are minors. The clinic, which is located on the fifth level of Deaconess main hospital building, is designed to be comfortable for children, with childrens themes, such as cartoon animal paintings, throughout its examination rooms and hallways.


Patients routinely come to the clinic at intervals of anywhere from once every three weeks to once every three months, depending on the severity of their disease, Harrington says. She says that for those routine visits, a clinic day is held every Thursday at which each patient meets with a physician, a respiratory therapist, a nurse, a dietician, and a social worker. On those days, the clinic typically sees 16 to 20 cystic-fibrosis sufferers.


Nearly two-thirds of the clinics patients live outside of the Spokane area, Harrington says, coming from elsewhere in Eastern Washington and from Central Washington, North Idaho, and Western Montana.


If patients didnt have this clinic here, theyd be going to Seattle or Salt Lake City, she says, adding that some otherwise might go to Great Falls, Mont., as well, because a physician who specializes in cystic fibrosis recently moved his practice there from Denver.


The Deaconess clinic has operated for 25-plus years, and McCarthy has served as its medical director for much of that time. He had moved his practice to Sacred Heart Medical Center, of Spokane, for a short period of time last year, but moved back to Deaconess about six months ago.


Deaconess closed some of its pediatric programs earlier this year, and Sacred Heart picked up those programs. McCarthy says, however, Deaconess intends to keep the CF clinic.


Treatment and severity


While cystic-fibrosis sufferers frequently visit the clinic, day-to-day treatments at home are intensive as well. Harrington says a person with cystic fibrosis is advised to take daily breathing treatments, in which they use whats called a nebulizer to convert liquid antibiotics into a breathable mist. Such treatments typically take 20 minutes to one hour to complete, she says.


Patients also undergo chest percussion, which can loosen and thin mucus in the chest.


Cystic fibrosis generally progresses gradually, McCarthy says, but its unpredictable in its degree of severity from patient to patient.


A person with a mild form of cystic fibrosis can have a lung capacity thats close to normal, and as a high-school student or young adult can play sports or perform manual labor. Some such cases go undetected until later in life.


With a severe form of the disease, a person might experience lung damage at a young age and might need to be on oxygen continually.


Cystic fibrosis occurs because of a mutation in a specific gene, and there are 1,200 variations on that genetic mutation that can lead to cystic fibrosis. Theres no apparent correlation, however, between the specific type of mutation and the diseases severity, he says. In other words, he says, two people can have cystic fibrosis caused by identical genetic mutations and still suffer different severities of the disease.


Patients with cystic fibrosis generally are living longer because of earlier detection and treatment of the disease, and because of the improvements in some longtime treatments, specifically enzymes and nutritional supplements that improve pancreatic function and antibiotics that treat lung problems. Also, medicines have emerged over the past decade or so that have made a significant difference. For example, Dornase, an inhaled prescription drug, is used regularly to thin mucus in the lungs.


McCarthy says a number of efforts are under way to bring to market gene therapies that could help cystic-fibrosis sufferers.


Some are far along in drug trials, he says. Within five years, we hope to have some available on the market.


According to the Cystic Fibrosis Foundation, about 30,000 people in the U.S. have the disease, and it occurs in one of every 3,500 live births. The disease occurs equally in males and females.


Cystic fibrosis can occur when two people who have the defective gene that causes the disease have offspring. If both parents carry that gene, theres a 25 percent chance that their baby will have the disease.


About 10 million people in the U.S. are unknowing, symptom-free carriers of the defective cystic-fibrosis gene, the foundation says.