This is a long-term extension (LTE) follow up study where recruitment of subjects will continue as the parent study (AV-101-002) progresses from Phase 2b to Phase 3. Subjects who were on placebo in Phase 2b and the Intermediate Part of the study who enroll in the LTE study will be re-randomized to one of the 3 active AV-101 doses until such time as the optimal dose has been selected. Once the optimal dose of AV-101 has been selected, all subjects will be transitioned to the optimal dose while they continue in the LTE, and subjects completing the Intermediate Part or parent study Phase 3 will enroll into the LTE study at the optimal dose.
1) Subjects who have successfully completed the placebo-controlled 24-week study AV-101-002 without a treatment-limiting toxicity resulting in discontinuation of study drug.
2) Subjects who have consented to participate.
1) Subjects who were not compliant with study medication in AV-101-002 as assessed by the Investigator
2) Subjects for whom the investigator believes that it would not be in the best interest of the subject to be included in the LTE e.g., for clinical or social reasons.
3) Clinically relevant history or current psychological abnormality (including alcohol abuse), psychiatric or neurological illness or autonomic neuropathy, which in the opinion of the Investigator could jeopardize or would compromise the subject’s ability to participate in the trial.
Total four clinic visits in first six months and then onwards one visit every three months until the medication is FDA approved or study is terminated by sponsor. Each clinic visit requires blood draw for safety laboratory tests and Lung function assessed.