Open-Label Phase III Long-Term Safety Trial of Liprotamase

Exclusion Criteria

• •Age \<7 years
• •Pregnancy, breastfeeding or of childbearing potential and not willing to use birth control
• •Participation in an investigational study of a drug, biologic, or device not currently approved for marketing within 30 days prior to Screening
• •History of fibrosing colonopathy
• •History of liver transplant, lung transplant or significant surgical resection of the bowel
• •Any chronic diarrheal illness unrelated to PI
• •Abnormal liver function (except for patients with Gilbert Syndrome)
• •Signs and/or symptoms of liver cirrhosis or portal hypertension (e.g., splenomegaly, ascites, esophageal varices), or documented liver disease unrelated to CF
• •Unable to discontinue enteral tube feedings during the study
• •Any condition that the Investigator believes would interfere with the intent of this study or would make participation not in the best interest of the patient
• •Baseline coefficient of fat absorption (CFA) ≥ 93% from the Phase III efficacy study
• •Patient is unlikely to complete the study, as determined by the Investigator