Cystic fibrosis mortality rates improving

Associate Professor David Reid, from the Prince Charles Hospital, Chermside, and co-authors analysed information on mortality events (death or lung transplantation) among Australians with CF between 1979 and 2005 to determine whether there had been any improvements in survival rates.

Professor Reid said that between 1979 and 2005 the mean age at death increased from 12.2 years to 27.9 years for males and from 14.8 years to 25.3 years for females.

“Survival has increased since 1979, but females continue to have reduced length of life compared with males,” Professor Reid said.

In another study published in the MJA, Associate Professor Scott Bell, from the Prince Charles Hospital, Brisbane, and co-authors conducted a cross-sectional study to determine the current demographics, clinical features and outcomes among people with CF in Australia.

The authors found that CF remains a progressive respiratory disease associated with multisystem complications and that the acceleration in disease severity in adolescence and early adulthood suggests that better treatment at these particular life stages is required to further improve survival.

In an accompanying editorial in the MJA, Professor Kevin Gaskin, from the Children’s Hospital at Westmead, Sydney, and co-author said that although both studies recognised improved survival among people with CF, reasons for the improvement remain unclear.

“Many factors probably contribute, including improvements in medical care, such as anti-pseudomonas antibiotics and better chest therapy in general, and the proven benefit of a high-fat diet for maintaining normal growth and nutritional status,” Professor Gaskin said.

“In addition, a fall in the number of early deaths from CF with the advent of newborn screening has been clearly demonstrated.

“The databases used by the Australian investigators ... have provided invaluable outcome data for comparison of Australian clinics,” Professor Gaskin said. “It is essential that governments and the community ensure the sustainability of clinical registries.”

The Medical Journal of Australia is a publication of the Australian Medical Association.

The statements or opinions that are expressed in the MJA  reflect the views of the authors and do not represent the official policy of the AMA unless that is so stated.

CONTACT:            Associate Professor David Reid                        0407 272 931
                           Associate Professor Scott Bell                          0401 718296
                           Professor Kevin Gaskin                                    0408 466 053