My wife has PF due to systematic sclerosis (diffused sclerodrma) since April 2018. She takes OFEV 150mg x 2 and Cellcept 500mg x 4 daily. She is annually checked with RHC (right heart cathetirization). At the last test (05/2022) results show that PAH has initiated (25mmHg and resistance >3.2) . She started treatment using volibris initially and macitentan later- due to side effects- and tadalafil. Recently she proceeded to heart ultrasound and the results did not show any improvement. Due to some side effects and the “not good results” of ultrasound, reumatologist and pulmonogist agreed (concerning the PAH) to change to Tyvaso. Tyvaso has not gain an approval in EU yet. However they will provide it under specific conditions as it approved from FDA. I will come back after a few months.
All data provided from members of the forum are very useful and interesting!
In Greece the medical social system provides orphan and costly drugs free of charge under specific prescription.
Best regards to all
