ABSTRACT
In 2009, cystic brosis (CF) ocially became a disease of adults in the United Kingdom when the numbers of adults with CF exceeded that of children for the rst time. So, monitoring in childhood to aect what happens in adulthood assumes ever greater importance. Data collection is not an end in itself; its purpose is to enhance the clinicians’ understanding of the state of an individual patient. is chapter therefore deals with lung function testing at two different levels; the rst is how lung function should be monitored and interpreted on a day-to-day basis in the clinic and used for routine decision making, for example, whether a course of intravenous antibiotics is warranted. Second, how lung function declines as the CF patient gets older and the relationship between conventional lung function tests such as spirometry and newer measures such as lung clearance index (LCI) and their rates of change with time until death. e nal parts deal with other physiological tests and how to assess the safety of air travel in CF subjects. e use of lung function in databases as a quality improvement (QI) measure is discussed in Chapter 31.
