Study of Dose Escalation of Liposomal Amikacin for Inhalation (ARIKAYCE™) - Extension Phase

Inclusion Criteria:

1. Written informed consent obtained from the patient or designated legal guardian priorto the performance of any study related procedures.

2. Male or female study subjects ≥ 6 years of age or older.

3. Confirmed diagnosis of CF defined as a positive sweat chloride > 60 milliequivalents (mEq)/liter (by pilocarpine iontophoresis) and/or a genotype with 2 identifiablemutations consistent with CF accompanied by one or more clinical features of the CFphenotype.

4. History of chronic infection with P. aeruginosa (defined as 3 documented positivecultures in the prior 2 years of which at least one was obtained in the 3 months priorto randomization). The cultures could be obtained from the following respiratorysecretions: sputum, throat swabs, nasopharyngeal swabs, or broncho-alveolar lavagefluid specimens.

5. Study subjects must produce a screening specimen (expectorated or induced sputum,throat swabs, nasopharyngeal swabs, or broncho-alveolar lavage fluid) that is positivefor growth of P. aeruginosa.

6. FEV1 ≥ 40% of predicted at Screening.

7. SaO2 ≥ 90% at Screening while breathing room air.

8. Ability to comply with study medication use, study visits, and study procedures asjudged by the investigator.

9. Ability to produce 0.5 grams sputum or be willing to undergo an induction to producesputum for clinical evaluation.

10. Clinically stable with no evidence of acute upper or lower respiratory tract infectionor history of pulmonary exacerbation within the 4 weeks prior to Screening. Main criteria for inclusion of patients participating in the 18-month extension period:

11. Written informed consent obtained from the patient or designated legal guardian priorto the performance of any study-related procedures in the extension period.

12. Patient meets all of the above listed inclusion criteria (1-10) of the main protocol.

Exclusion Criteria:

1. Administration of any investigational drug within 8 weeks prior to Screening.

2. Emergency room visit or hospitalization for CF or respiratory-related illness withinthe 4 weeks prior to Screening.

3. History of alcohol, medication, or illicit drug abuse within the 1 year prior toScreening.

4. History of lung transplantation.

5. Female of childbearing potential who is lactating or is not practicing an acceptablemethod of birth control (e.g., abstinence, hormonal or barrier methods, partnersterilization, or IUD).

6. Positive pregnancy test. All women of childbearing potential will be tested.

7. Use of any anti-pseudomonal antibiotics (IV antibiotics, all inhalation antibiotics,oral fluoroquinolones) within the 28 days prior to Screening.

8. Initiation of chronic therapy (i.e. TOBI®, high-dose ibuprofen, rhDNase, macrolideantibiotics) within the 28 days prior to Screening.

9. History of sputum or throat swab culture yielding Burkholderia cepacia within 2 yearsof Screening.

10. History of mycobacterial and/or Aspergillus infection requiring treatment within 2years prior to Screening, and/or history of allergic bronchopulmonary aspergillosis (ABPA).

11. History of biliary cirrhosis with portal hypertension, or splenomegaly (refer to studymanual).

12. GGT, AST, or ALT ≥ 3 times the upper limit of normal at Screening visit.

13. ANC ≤ 1000 performed at Screening visit.

14. Serum creatinine > 1.5 times normal performed at Screening visit.

15. History of daily, continuous oxygen supplementation or requirement for more than 2L/min at night.

16. Change in chest x-ray at Screening (or within the 3 months prior to Screening) withnew onset infiltrates or that which compromise the safety of the study patient or thequality of the study data. Main criteria for exclusion of patients participating in the 18 months extension period:

17. Patient meets any criteria for exclusion as listed above in the main protocol.

18. Patient who met any criteria for study drug discontinuation in the main protocol (TR02-105).