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Grand Rounds | Clinician's Corner

Adult Cystic Fibrosis

Michael P. Boyle, MD
JAMA. 2007;298(15):1787-1793. doi:10.1001/jama.298.15.1787.
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Cystic fibrosis is a multisystem disease characterized primarily by chronic pulmonary infection and bronchiectasis, pancreatic exocrine impairment, and elevated sweat chloride. In the last 4 decades, new treatment strategies and aggressive nutritional management have resulted in a significant increase in expected survival, with median predicted survival in cystic fibrosis now to older than 35 years. This increase in predicted survival has also been aided by a greater appreciation of the potential variability in the presentation and severity of cystic fibrosis, resulting in identification of a growing number of mild cases. As it is estimated that within the next decade more than half of all individuals with cystic fibrosis will be aged 18 years or older, adult medicine caregivers are increasingly likely to encounter patients with cystic fibrosis and be exposed to their unique medical management.

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Figures

Figure 1. Forced Expiratory Volume in the First Second of Expiration (FEV1) for Mr Y From 2000 to 2007
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The large plot shows FEV1 percent predicted in the month spirometry was performed. In August 2003, spirometry was performed 3 times; these data are shown in the inset. The patient was taking only intermittent oral antibiotics from 2000 through July 2003. In August 2003, the patient was treated with intravenous tobramycin and meropenem and subsequently began aggressive chronic therapy for cystic fibrosis in September 2003. The percentage predicted of FEV1 function was calculated using Goldman equations.1

Figure 2. Cystic Fibrosis Median Predicted Survival Age From 1985 Through 2006
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The median predicted survival was 36.9 years in 2006, which represents the age by which half of the current Cystic Fibrosis Foundation Patient Registry population would be expected to die, given the ages of the patients in the registry and mortality distribution. Data are from the Cystic Fibrosis Foundation Patient Registry. The error bars indicate 95% confidence intervals.

Figure 3. Percentage of Adults Among Individuals With Cystic Fibrosis
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Forty-three percent of individuals with cystic fibrosis were aged 18 years or older in 2005. Data are from the Cystic Fibrosis Foundation Patient Registry. Beginning in 1987, the percentage of adults was reported. Earlier adult percentages are calculated from available Cystic Fibrosis Foundation Patient Registry data.

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