Ageing in cystic fibrosis and long-term survival

Abstract

The demographics of cystic fibrosis continue to change with adults outnumbering children in most developed countries. Median predicted survival is now over 40 years and 7.6% are aged >40 years. Patients surviving beyond 40 cover the full spectrum of disease from homozygous F508del to single organ disease. Differences in the characteristics of older patients are recognised, but generally patients diagnosed in adulthood are still at risk of accelerated lung function decline. Improved survival brings new challenges, including a rising rate of CF co-morbidities such as diabetes, in addition to other medical problems such as renal impairment and ototoxicity.