277 Transformational care at the All Wales Adult CF Centre (AWACFC) – is ivacaftor making us fat? The impact of ivacaftor (Kalydeco®) on body composition

Background A previous study (Ramsey 2011) has shown an improvement BMI and quality of life respiratory domain for patients on ivacaftor. Some local female patients were concerned with the weight they were gaining on ivacaftor. Gender differences previously reported suggest some female patients are more satisfied with their ‘thinness' than male patients (Quittner 2002). We aimed to retrospectively review body composition, handgrip strength and quality of life in patients at baseline and after a year of ivacaftor. Method The following data was collected from the 11 patients attending the AWACFC at initiation of and one year after starting ivacaftor: BMI, body Fat percentage, Fat Free mass (FFM) FFM index (FFMI), handgrip and CF quality of life scores (CFQ). Results All 11 patients (3 male) are delF508/G551D with a mean (SD) age of 28 (13) years. PrePostChangepBMI (kg/m 2 )20.9 (2.9)22.6 (3.1)1.70.009Fat percentage22.6 (8.7)25.0 (9.3)2.40.05FFM (kg)44.1 (13.1)47.8 (14.8)3.70.04FFMI (kg/m 2 )15.9 (2.8)16.6 (3.1)0.70.007Handgrip25.7 (10.9)29.7 (12.1)4.00.12QOL respiratory63.1 (26.7)89.4 (11.0)26.30.004QOL body image75.8 (39.1)84.8 (19.4)9.00.42QOL eating82.8 (27.4)98.0 (4.5)15.20.10QOL weight72.7 (41.7)87.9 (22.5)15.20.27 Conclusion There was a significant (deemed >4 point) improvement in all domains of the CFQ with only body image, eating and weight domains not reaching statistical significance. This may be due to small numbers. Reassuringly, although there is a gain in fat percentage much of the weight gained is actually due to a significant gain in FFM reflecting improved muscle mass which is also reflected in a trend of improved handgrip strength.