Evaluation of the Safety and Tolerability of Inhaled Nitric Oxide to Subjects With Cystic Fibrosis

Inclusion criteria

• Subjects (Male or female) ≥10 years old
• Confirmed diagnosis of CF
• Resting awake oxygen saturation of at least 92% in room air
• Approved and signed informed consent:

• Subject aged over 10 years old (10 included) -

• signed an informed consent by the subject
• Parents/ legal guardian signed informed consent.
• Subject aged over 18 years old (18 included) - • signed an informed consent by the subject
• 80% ≥FEV1≥ 30%
• Confirmed to be colonized with Pseudomonas aeruginosa

Exclusion criteria

• Subjects younger than 10 years old
• FEV1< 30% or FEV1> 80%
• Pulmonary exacerbation resulting in antibiotic treatment (except prophylactic antibiotics) within1 month before enrollment
• Subject is pregnant (when applicable, a negative pregnancy test result must be verified prior to enrollment and during treatment)
• Subjects diagnosed with methemoglobinemia, immunodeficiency and/ or heart disease.
• Use of an investigational drug within 30 days prior enrolment and/ or the subject is expected to participate in a new study within three months from enrollment to this study.
• History of frequent epistaxis (>1 episode/month)
• Significant hemoptysis within 30 days (≥ 5 mL of blood in one coughing episode or > 30 mL of blood in a 24 hour period)
• Methemoglobin level>3% at screening
• Patients on systemic steroids (1mg/kg or > 20mg of prednisone per day) within 30 days of screening;
• Smokers;
• History of illicit drug or medication abuse within 1 year of screening ;
• history of lung transplantation;
• Patients treated for high blood pressure
• Subjects cannot comply with the study design
• Presence of a condition or abnormality that in the opinion of the Investigator would compromise the safety of the subject or the quality of the data.
• The subject is identified by the investigator as being unable or unwilling to perform study procedures.