DOI: 10.1097/01.mcp.0000245713.89632.7c
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Issn Print: 1070-5287
Publication Date: 2006/11/01
Excerpt
I have the pleasure of introducing what I hope you will find to be an interesting and provocative section on cystic fibrosis. The manuscripts introduced below with their wide range of topics demonstrate the many fertile areas of investigation in this disease. Knowles provides an update on gene modifiers in cystic fibrosis in the context of a succinct overview of the various scientific approaches to identify gene modifiers. Lai reminds us of how closely nutritional status and pulmonary disease are intertwined in cystic fibrosis. She makes a compelling case for why clinicians should abandon ideal body weight in favour of body mass index as a metric. Ratjen reviews the important topic of newly acquired Pseudomonas aeruginosa. An aggressive therapeutic approach to the acquisition of this organism has been widely adopted at cystic fibrosis care centres around the world. We eagerly await the results of ongoing clinical trials in the hope that they will provide insights on the optimal approach. Tiddens and de Jong provide an update on computed tomography scanning for the monitoring of lung disease in cystic fibrosis. They point out the striking discordance between the results of pulmonary function tests and chest computed tomography findings in young children. The battle in the lungs begins early in life and it appears that physiologic measures are relatively insensitive to important structural changes. Whether chest computed tomography scanning will be widely incorporated into practice at cystic fibrosis care centres remains to be seen. Smythe provides a timely update on pulmonary exacerbations – one of the core problems that clinicians confront on a daily basis. Efforts aimed at standardizing the approach to exacerbations holds great promise for improving outcomes in cystic fibrosis. Bye and Elkins summarize the rationale for the administration of hypertonic saline and review the results of recent clinical trials. This is a promising new therapeutic option that may have a major impact on the course of the disease. Boyle provides an update on bone disease – an important complication in adolescents and adults with cystic fibrosis. He points out that the currently recommended regimen for repletion of vitamin D [1] is often unsuccessful and suggests an alternative approach that has been more successful at the Johns Hopkins care centre. In the final manuscript, Liou updates readers on patient selection criteria for lung transplantation. The impact of the recent major modifications in the donor lung allocation schema in the United States [2] remains unclear at this time.
In summary, this issue highlights the ongoing progress in cystic fibrosis on multiple fronts, providing a sense of hope and optimism for those affected by this disease and their healthcare providers. The steadily improving survival is tangible evidence of the remarkable progress in this disease. The most recent predicted median survival from the 2005 US Cystic Fibrosis Foundation Patient Registry data is well over 35 years of age [3]. Another marker of success is the growing number of adults with cystic fibrosis. More than 40% of the total number of people with cystic fibrosis in the United States are now 18 years of age or older. Over the relatively brief time span of adult cystic fibrosis care, there has also been a notable change in the health status of patients transitioning from paediatric to adult care. A striking pattern emerges when one examines the Patient Registry data on 18-year-olds over the last couple of decades.
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