Effects of Gender and Age of Diagnosis on Disease Progression in Long-term Cystic Fibrosis Survivors

Am J Respir Crit Care Med. 2010 May 6.

Effects of Gender and Age of Diagnosis on Disease Progression in Long-term Cystic Fibrosis Survivors.

Nick JA, Chacon CS, Brayshaw SJ, Jones MC, Barboa CM, St Clair CG, Young RL, Nichols DP, Janssen JS, Huitt GA, Iseman MD, Daley CL, Taylor-Cousar JL, Accurso FJ, Saavedra MT, Sontag MK.

Department of Medicine, National Jewish Health, Denver, Colorado, United States; Department of Medicine, University of Colorado Denver School of Medicine, Aurora, Colorado, United States.
Abstract

RATIONALE: Long-term CF survivors (age >40 years) are a growing population comprised of both patients diagnosed with classic manifestations in childhood, and non-classic phenotypes typically diagnosed as adults. Little is known concerning disease progression and outcomes in these cohorts.

OBJECTIVES: Examine effects of age at diagnosis and gender on disease progression, setting of care, response to treatment, and mortality in long-term CF survivors.

METHODS: Retrospective analysis of the Colorado CF database (1992-2008), CF Foundation Registry (1992-2007), and Multiple Cause of Death Index (1992-2005).

MEASUREMENTS AND MAIN RESULTS: CF patients diagnosed in childhood that survive to age 40 have more severe CFTR genotypes and phenotypes compared to adult diagnosed patients. However, past age 40 the rate of FEV1 decline and death from respiratory complications were not different between these cohorts. Compared to males, childhood diagnosed females were less likely to reach age 40, experienced faster FEV1 declines, and no survival advantage. Females comprised the majority of adult diagnosed patients, and demonstrated equal FEV1 decline and longer survival than males, despite a later age of diagnosis. Most adult diagnosed patients were not followed at CF Centers, and with increasing age a smaller percentage of CF deaths appeared in the CFF Registry. However, newly diagnosed adults demonstrated sustained FEV1 improvement in response to CF Center care.

CONCLUSIONS: For CF patients older than 40, the adult diagnosis correlates with delayed but equally severe pulmonary disease. A gender-associated disadvantage remains for females diagnosed in childhood, but is not present for adult diagnosed females.