The primary objective of this program is to provide expanded access to aztreonam lysine for
inhalation (AZLI) 75 mg prior to its commercial availability to patients with cystic
fibrosis (CF) and chronic P. aeruginosa airway infection who have limited treatment options
and are at risk for disease progression.
Inclusion Criteria:
Subjects must meet all of the following inclusion criteria to be eligible for
participation in this study:
- ≥ 6 years of age
- Subject has CF as diagnosed by one of the following:
- Documented sweat chloride ≥ 60 mEq/L by quantitative pilocarpine iontophoresis
test
- Two well characterized genetic mutations in the CFTR gene
- Abnormal nasal potential difference with accompanying symptoms characteristic of
CF
- At high risk for disease progression as defined by one of the following subject
populations:
- First Cohort Criteria:
- Those who are wait listed or eligible for lung transplant based on FEV1
criteria. Patients who have a level of lung function impairment consistent
with lung transplantation criteria, but who are ineligible for
transplantation for other reasons, can enroll in this program; or
- Completed participation in CP-AI-006 (through Visit 20). Subjects who
withdraw from CP-AI-006 prior to completing all courses of AZLI and all
study visits will not be eligible for this protocol.
- Second Cohort Criteria:
- Meets first cohort criteria
- FEV1 ≤ 40% predicted at the time of consent
- Third Cohort Criteria:
- Meets first or second cohort criteria
- FEV1 ≤ 50% predicted at the time of consent
Exclusion Criteria:
Subjects who meet any of the following exclusion criteria are not to be enrolled in this
study:
- Subjects with any serious or active medical or psychiatric illness that, in the
opinion of the investigator, would interfere with subject treatment, assessment or
compliance with the protocol or dosing requirements
- Subjects with hypersensitivity to any of the components of the drug product
- Currently enrolled in another clinical trial
- Pregnant or lactating females
San Juan 366528, Puerto Rico
Birmingham, Alabama 35233, United States
Anchorage, Alaska 99508, United States
Phoenix, Arizona 85016, United States
Little Rock, Arkansas 72205, United States
Los Angeles, California 90027, United States
San Francisco, California 94115, United States
Sacramento, California 95819, United States
San Francisco, California 94143, United States
Denver, Colorado 80045, United States
Denver, Colorado 80206, United States
Hartford, Connecticut 06106, United States
Hartford, Connecticut 06102, United States
Orlando, Florida 32803, United States
Tampa, Florida 33606, United States
St. Petersburg, Florida 33701, United States
Gainesville, Florida 32610, United States
Ft, Meyers, Florida 33908, United States
Boise, Idaho 83712, United States
Chicago, Illinois 60614, United States
Maywood, Illinois 60153, United States
Chicago, Illinois 60302, United States
Des Moines, Iowa 50309, United States
Wichita, Kansas 67214, United States
New Orleans, Louisiana 70112, United States
Boston, Massachusetts 02111, United States
Boston, Massachusetts 02111, United States
Boston, Massachusetts 02114, United States
Detroit, Michigan 48201, United States
Minneapolis, Minnesota 55455, United States
Minneapolis, Minnesota 54404, United States
Columbia, Missouri 65212, United States
Kansas City, Missouri 64108, United States
Great Falls, Montana 59405, United States
Las Vegas, Nevada 89107, United States
Long Branch, New Jersey 07740, United States
New Brunswick, New Jersey 08901, United States
Albuquerque, New Mexico 87131, United States
Buffalo, New York 14222, United States
New Hyde Park, New York 11040, United States
New York, New York 10032, United States
Valhalla, New York 10595, United States
Albany, New York 12208-3479, United States
Syracuse, New York 13210, United States
New York, New York 10011, United States
Chapel Hill, North Carolina 27599-7220, United States
Columbus, Ohio 43205, United States
Cincinnati, Ohio 45229, United States
Akron, Ohio 44308, United States
Cleveland, Ohio 44106, United States
Toledo, Ohio 43605, United States
Oklahoma City, Oklahoma 73104, United States
Philadelphia, Pennsylvania 19104, United States
Pittsburgh, Pennsylvania 15213, United States
Philadelphia, Pennsylvania 19134, United States
Hershey, Pennsylvania 17033, United States
Sioux Falls, South Dakota 57117, United States
Memphis, Tennessee 38103, United States
San Antonio, Texas 78212, United States
Dallas, Texas 75230, United States
Ft. Worth, Texas 76104, United States
Salt Lake City, Utah 84132-4701, United States
Richmond, Virginia 23219, United States
Portsmouth, Virginia 23708, United States
Norfolk, Virginia 23507, United States
Seattle, Washington 98195, United States
