Cystic Fibrosis



Cystic Fibrosis

Introduction

The lifespan of patients with cystic fibrosis (CF) has been steadily increasing in all countries where it has been measured, and there are good epidemiological reasons for believing that this trend, which has been apparent for several decades, will continue even in the absence of novel treatments directed toward correcting the basic CF genetic and molecular defect(s). Death from CF during childhood is now a rarity in the UK and other industrialized countries, and well over 95% of CF children can expect to enter adult life, with a corresponding need for appropriate medical services (Schechter & Shelton, 2001).

Survival beyond the fourth decade presents new questions and potential problems which are still being formulated. The effects of CF on the aging process, and vice versa, are not known, apart from a preliminary international study of malignancy. Other diseases with a prevalence closely linked to age, such as arterial disease, type 2 diabetes mellitus, osteoarthritis and Alzheimer disease, could in theory be more or less common in age-matched CF patients — who usually have, for example, low cholesterol levels but an increased risk of diabetes. Understanding the nature of such interaction between diseases could shed important light on the mechanisms and control of both CF and other conditions (Picci & Cameran et al., 2007).

As a starting point it was therefore decided to look at individuals over 40years of age in more detail, to try to identify the factors which had contributed to their better survival, whether genetic, socioeconomic or therapeutic, and to discover whether later complications of CF could be anticipated, and whether they would experience a relatively greater or lesser risk of common age-associated disorders. The experience of four large CF centres, two in Europe (London and Verona) and two in North America (Minneapolis and Toronto), has now been analysed. Data were obtained from existing medical records and are presented in this report, which provides baseline data for planned prospective and more detailed enquiries.

Thesis Statement

The International Study of Aging in Cystic Fibrosis was set up to address these and other issues, and to share the limited experience of caring for relatively elderly CF patients with other professionals. A preliminary search among interested CF centres in 2003 identified more than 1800 patients who were still alive beyond the age of 40. It was further evident that the numbers of such patients would escalate rapidly over the next decade. The current actuarial mean survival in countries with well developed CF services being about 35-40 years for both sexes combined and increasing year on year (Drumm & Konstan, 2005). Male and female patients in the United Kingdom, who were alive in 2003 at the age of 30years, had an estimated mean expectation of dying by 52.7 and 49.4years respectively. In the Canadian Patient Data Registry, the percentage of patients over age 40 has steadily doubled every 5years, from less than 0.2% in 1977 to 6.7% in 2002. At this rate, 20% of Canadian CF patients will be over 40years of age by ...