Fig. 7.

Summary of spirometry and oximetry changes.

The figure above depicts the overall changes in spirometry and oximetry from baseline over the course of the study, grouped by each of the variables of interest. There was a general decline in spirometry variables over the course of the study, which appeared to be more significant in the ERT group. The decline in 6MWT was also more appreciable in the ERT group. The oximetry data showed that function remained more static with either small positive changes suggesting improvement.