Safety and Efficacy Study of ULTRASE® MT20 in Participants With Cystic Fibrosis (CF) and Exocrine Pancreatic Insufficiency (PI)

Inclusion Criteria:

• Participants or their legally authorized representative must understand the nature ofthe study and sign an informed consent or assent form along with a parental form

• Participants must have a confirmed diagnosis of CF based on 1 or more clinicalfeatures consistent with the CF phenotype, and one of the following:

• A genotype with 2 identifiable mutations consistent with CF

• A sweat chloride test greater than 60 millimole per liter (mmol/L) byquantitative pilocarpine iontophoresis

• Participants must have PI as demonstrated by a fecal elastase-1 (FE-1) concentrationless than 100 microgram per gram (mcg/g) of stools (ScheBo test) and must requirepancreatic enzyme supplementation

• Participants must be clinically stable as evidenced by medical and medication history,baseline physical examination including vital signs and laboratory analyses

• Participants must be 7 years and older

• Participants must have an adequate nutritional status based on the following body massindex (BMI):

• Participants 7 to 20 years old must have a BMI greater than or equal to fifthpercentile

• Female participants greater than 20 years old must have a BMI greater than orequal to 16

• Male participants greater 20 years old must have a BMI greater than or equal to 16.5

• Participants must be on an optimal clinical dose of pancreatic enzymes (Ultrase® MT18or MT20 or other pancreatic enzymes preparations including Ultrase® MT12) prior toentry in the study, and must tolerate this medication in the opinion of theinvestigator

• Participants must be able to swallow capsules and must be able to eat a high fat dietcalculated as 2 gram (± 15%) fat per kilogram body weight per day

• Participants must be, in the opinion of the investigator, able and willing to completethis study

• Female participants must be premenarcheal, surgically sterile or postmenopausal for atleast 12 consecutive months. Otherwise, the women of childbearing potential (WOCBP)must not be pregnant and must have practiced an acceptable method of contraception forat least one month prior to the study entry

Exclusion Criteria:

• Participants with a known contraindication, sensitivity or hypersensitivity to Ultraseor any porcine protein

• Participants with a known allergy to the food drug and cosmetic (FD&C) Blue No. 2 dyeindicator (stool marker)

• Participants not willing to stop the prohibited medications or products at study entryand throughout the study

• Participants who are using narcotics

• Participants who are using bowel stimulants and/or laxatives on a regular basis

• Participants with acute pancreatitis or acute exacerbation of chronic pancreaticdisease

• Participants with an acute pulmonary infection

• Participants with a history of bowel resection

• Participants suffering from any dysmotility disorders

• Participants with chronic or severe abdominal pain

• Participants receiving enteral tube feeding and not willing to stop during the courseof the study

• Participants known to have a significant medical disease that would compromise theirwelfare or confound the study results

• Participants with a history of or a current diagnosis of clinically significant portalhypertension

• Participants who have a condition known to increase fecal fat loss including celiac'sdisease, biliary cancer, biliary stricture, cholelithiasis, Crohn's disease, pancreascancer, radiation enteritis, tropical sprue, Whipple's disease, lactose intolerance,pseudomembranous colitis

• Participants with a current diagnosis or a history of complete distal intestinalobstruction syndrome (DIOS) in the past 6 months; or, participants who had 2 or moreepisodes of DIOS in the past year

• Participants with poorly controlled diabetes to the investigator's opinion

• Female participants who are pregnant or lactating

• Participants who received an investigational drug within 30 days prior to entry intothe study