This subproject is one of many research subprojects utilizing theresources provided by a Center grant funded by NIH/NCRR. The subproject andinvestigator (PI) may have received primary funding from another NIH source,and thus could be represented in other CRISP entries. The institution listed isfor the Center, which is not necessarily the institution for the investigator.The trial will be randomized, double-blind, placebo-controlled. Up to 120 study subjects (60 per arm) will be treated with placebo or sirolimus (2 mg) orally for a period of 12 months, then studied off therapy for 12 months. Pulmonary function, exercise tolerance and quality of life will be measured. There will be an interim analysis when 50 patients reach the one year point. The total duration of the study will be three years. If, at the time of the interim analysis, it is determined by the Data Safety Monitoring Board (DSMB) that sirolimus has a beneficial effect on lung function, then all subjects will receive sirolimus and the study will continue 'open label' through the two-year point. The DSMB may interrupt or stop the study at any time if the treatment causes unacceptable side effects or worsens lung function.
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