Cystic fibrosis treatments improving

NEW YORK (Reuters Health) - Improvements in the treatment of cystic fibrosis during early childhood have led to fewer serious infections and stable lung function between ages 6 and 12 years, according to a study conducted at Cincinnati Children's Hospital in Ohio.

Cystic fibrosis is a serious inherited disease that affects tiny channels that bring nutrients into and out of cells. The disturbance causes thick mucous secretions in the lung that provide a place for pathogens to accumulate and grow, promoting infection and making it difficult for the patient to breath. There currently is no cure for the disease and the average life expectancy is about 37 years.

"There have been major advances in cystic fibrosis care in recent decades, with improved nutrition, inhaled antibiotics, inhaled (anti-mucus treatments), and oral anti-inflammatory therapies," the research team reports in the Journal of Pediatrics.

To see if these advances have translated into clinical benefits, Dr. Gary L. McPhail and his associates studied the medical records of two groups of children with cystic fibrosis treated at their center before 5 years of age: 74 patients born between 1985 and 1992 and 70 born between 1993 and 2000.

The findings showed that children born between 1993 and 2000 received closer follow-up than the other children, showed better growth, were more likely to receive an anti-mucus drug called dornase alfa, and had a lower rate of potentially serious lung infections caused by a bacterium called Pseudomonas aeruginosa.

Moreover, the children treated more recently showed virtually no drop in lung function over time.

Contributing to the "negligible" rate of decline in lung function at their center is improved infection control, with a focus on early eradication of Pseudomonas infection, McPhail's team says, as well as initiation of dornase alfa in all cystic fibrosis patients at age 6 years and older.

SOURCE: Journal of Pediatrics, September 11, 2008.