A model for active and healthy ageing with a rare genetic disease: cystic fibrosis

Abstract

@ERSpublications Cystic fibrosis management today: an example of active and healthy ageing in patients suffering from a rare genetic disease http://ow.ly/V3JXtCystic fibrosis (CF), the most common life-limiting genetic condition in Caucasians, affects many organ systems. Specialised medical care, aggressive treatments [1] and proper nutrition both lengthen and improve quality of life for CF patients. Advances achieved over the last four decades have transformed CF from a fatal childhood disease to a long-term chronic condition. CF patients can now reach adulthood [2][3][4][5], although most of them still have a reduced life expectancy, and the adult population has passed from 44.55% in 2005 to 49.3% in 2010 (figure 1), the oldest patient being aged >80 years, from the 2010 European CF registry [6]. In the near future, even though CF paediatric care will remain crucial to assure the best growth for patients and transition to adulthood, CF centres may largely be considered as adult oriented [7], and we should, therefore, aim for the new concept of active and healthy ageing (AHA) in CF patients. Important controversies exist concerning the early-and long-term management of CF, which should be carefully considered when proposing a strategy for ageing well with CF [8].Health is a multidimensional concept, capturing how people feel and function. It includes at least three dynamic components: first, survival to old age; second, delay in chronic diseases onset and disability; and third, optimal functioning for the maximal period of time. AHA includes functioning, activities and participation, diseases, and resilience (the ability to adapt).

The changing demography of the cystic fibrosis population: forecasting future numbers of adults in the UK

“…Improvements in management of cystic fibrosis (CF) delivered in specialist centres in the United Kingdom (UK) have been associated with an overall step-change in CF, from a fatal disease of childhood in the 1960s and 1970s to a disease requiring long-term management through adulthood in the twenty-first century 1 . The UK CF Registry is a key resource for monitoring of patient numbers and outcomes, and according to current registrybased statistics, half of babies born today with CF in the UK are expected to survive beyond the age of 47 [2][3][4] .…”

Senescence in the lung: is this getting old?

“…Older patients are also at significantly higher risk of developing acute respiratory distress syndrome (ARDS) compared with younger patients (28) while pulmonary hypertension (PH), which is increasingly recognized in the elderly, is associated with greater morbidity and mortality (45). Interestingly, with improved care, longer survival of patients with cystic fibrosis has necessitated an understanding of the links between aging and CF (16,51). However, across these chronic lung diseases, diagnosis and management are complicated by a paucity of information regarding normal aging-related structural and functional changes, the influence of genetics, and the likely myriad of mechanisms that contribute to aging-related changes in the lung (36,50,52,62).…”

Positive Effect of Liposomal Amikacin for Inhalation on Mycobacterium abcessus in Cystic Fibrosis Patients

“…Cystic fibrosis (CF) is the most common, potentially lethal, autosomal recessive disease in whites [ 1 ]. It is mainly characterized by recurrent cycles of airway infections and chronic inflammation, eventually leading to permanent lung damage [ 1 , 2 ].…”

“…Cystic fibrosis (CF) is the most common, potentially lethal, autosomal recessive disease in whites [ 1 ]. It is mainly characterized by recurrent cycles of airway infections and chronic inflammation, eventually leading to permanent lung damage [ 1 , 2 ]. Lung infections with nontuberculous mycobacteria (NTM) are emerging as a global threat to individuals with chronic lung diseases [ 3 , 4 ].…”