To evaluate functional work capacity, a symptom limited 6 minute walking test will be performed on enrolment, to gain a baseline reading, as well as at the 6 and 12 month follow-up appointments. This test will be used for the evaluation of the primary endpoint after the 6 month follow-up.
The test will be performed according to the American Thoracic Society guidelines. The subject will be asked to walk along a flat surface and to attempt to walk as far as possible. The subject may stop to rest and resume walking or choose to terminate the test when they can walk no further. A modified Borg Scale will be used to assess perceived difficult or laboured breathing and fatigue before and after the walk with a subjective numerical scale ranging from 0 to 10. Heart rate, blood pressure and oxygen saturation using pulse oximetry will be measured before, during, and after the test. The test score will be reported and the distance walked rounded to the nearest meter. This test is according to EMA guidelines and scientific advice from FIMEA.