The Complexities of Ageing in Cystic Fibrosis

Abstract

Survival in cystic fibrosis (CF) is increasing exponentially and we are now seeing an ageing population. At the Manchester Adult Cystic Fibrosis Centre (MACFC), 20% of the total patient group are aged 40 years and above. Survival is likely to increase further in this exciting era of cystic fibrosis transmembrane conductance regulator protein (CFTR) modulation. Although there has been a paradigm shift in CF management and disease perspectives in recent years, there remains a paucity of research in this important and fascinating older CF patient population, particularly over the last decade. The CF population aged 40 years and above at MACFC is heterogeneous. There was a high proportion of patients with class I-III CFTR mutations and associated severe phenotypic disease. However, the prevalence of residual function CFTR mutations increased with age and were associated with lower sweat chloride values and later age of CF diagnosis. Cardiovascular risk exists in this older CF cohort, with 34.9% of patients showing elevated QRisk®3 scores of 10% or greater. The prevalence of cardiovascular disease may increase with an ageing CF population. The recognition and management of cardiovascular risk is essential to reduce further comorbidity in this already complex and challenging disease. At 3.6%, the prevalence of chronic kidney disease (CKD) was not as high in this older adult CF cohort as perhaps initially predicted. However, the measurement of estimated glomerular filtration rate (GFR) can be inaccurate in CF patients. There may be some justification for the wider use of measured GFR methods, such as iohexol, to improve the reliability of renal monitoring and in the assessment of GFR decline in select phenotypic groups of older CF patients. Cardiac magnetic resonance imaging (CMR) is feasible and well tolerated in this older adult CF group with a range of pulmonary disease severity. The use of CMR may provide an opportunity to analyse cardiac morphology and function in greater detail in CF than can be done with echocardiography alone, and cardiac imaging may become increasingly relevant in an ageing CF population.

Date of Award	1 Aug 2021
Original language	English
Awarding Institution	The University of Manchester
Supervisor	Andrew Jones (Supervisor) & Rowland Bright-Thomas (Supervisor)