Jan 19 2006
Two teams of scientists working half a world apart, have identified what they believe is a simple, effective and inexpensive treatment to reduce lung problems associated with cystic fibrosis.
Cystic fibrosis is the leading fatal genetic illness among whites, and victims suffer from a mucus build-up in the lungs that makes breathing difficult.
Cystic fibrosis appears on average in one of four children of parents who both carry a defective copy of a gene known as CFTR.
Children born with the disease soon develop chronic lung damage, since their lungs cannot clear the excessively sticky mucus by sweeping it to the mouth, where it is swallowed and eliminated.
Respiratory failure is the leading cause of death in CF patients.
The scientists say that salt water can ease the condition, and by inhaling a saltwater aerosol solution for between 10 and 15 minutes at least twice a day, young patients should be able to avoid a significant part of the damage the disease causes to their lungs.
The new therapy also appears to be safe and easy to take.
According to the researchers the aerosolized saltwater restores the thin lubricant layer of water that normally coats airway surfaces.
Apparently the water layer promotes the clearance of the naturally occurring mucus the body uses to trap harmful bacteria, viruses and other foreign particles.
The teams from the University of North Carolina at Chapel Hill School of Medicine and UNC Hospitals, worked with the Royal Prince Alfred Hospital, the University of Sydney and the Woolcock Institute of Medical Research, all in Sydney.
The scientists say they are excited that such a simple and inexpensive therapy appears to be so effective and well-tolerated in patients with CF.
They are optimistic that the therapy will reduce how often patients are ill, and will slow the decline of lung function over time and extend their life span.
The UNC study involved 24 CF patients who each inhaled the salt solution with or without pretreatment with a compound known as amiloride over two-week periods. Analysis of mucus clearance and lung function showed that the high-salt aerosol alone worked best, which surprised the medical scientists.
The Australian research team then used a comparable protocol to study another 164 patients for a longer period of almost a year.
During that extended period the Sydney researchers also found fewer lung problems with the concentrated saline than with normal saline, less need for antibiotics to treat lung infections over time, and improved attendance by patients at school, work and other activities during the 48 weeks.
The researchers say that as salt solutions are so cheap to make, the new therapy will produce clinical benefits at a minimal treatment expense, in contrast to many other available therapies.
The studies were supported by the Cystic Fibrosis Foundation and the National Heart, Lung and Blood Institute.
The final reports on both studies, were collaborative and complementary, and appear in the Jan. 19 issue of the New England Journal of Medicine.