Please select the eligibility criteria that correspond to the clinical trial below.
Title: Infant Study of Inhaled Saline in Cystic Fibrosis
Brief Summary: The purpose of this study is to assess whether 7% hypertonic saline (HS) is an effective and safe therapy in infants and young children with CF.


Options:
A: Inclusion Criteria:

* Diagnosis of CF by newborn screening or at least one clinical feature of CF, AND either: (a) A documented sweat chloride ≥ 60 mEq/L by quantitative pilocarpine iontophoresis or (b) A genotype with two identifiable CF-causing mutations
* Informed consent by parent or legal guardian
* Age 4 months to \< 60 months at Enrollment visit. If participating in Infant Pulmonary Function testing (selected sites), age 4 months to \< 16 months at Enrollment visit.
* Ability to comply with medication use, study visits, and study procedures as judged by the site investigator

Exclusion Criteria:

* Acute intercurrent respiratory infection, defined as an increase in cough, wheezing, or respiratory rate with onset in 1 week preceding Enrollment visit
* Acute wheezing at Enrollment visit (prior to HS test dose), or at Infant PFT visit (prior to infant pulmonary function testing), as applicable
* Oxygen saturation \< 95% (\< 90% in centers located above 4000 feet elevation) at Enrollment visit (prior to HS test dose) or at Infant PFT visit (prior to infant pulmonary function testing), as applicable
* Other major organ dysfunction, excluding pancreatic dysfunction
* Physical findings that would compromise the safety of the subject or the quality of the study data as determined by the site investigator
* Investigational drug use within 30 days prior to Enrollment visit, or within 30 days prior to Infant PFT visit as applicable
* Treatment with inhaled hypertonic saline at any concentration within 30 days of Enrollment visit, or within 30 days prior to Infant PFT visit as applicable
* Chronic lung disease not related to CF
* Intolerance of test dose of HS at Enrollment visit
* A sibling that has been randomized and is still enrolled in ISIS002

Additional Exclusion Criteria for Participation in Infant Pulmonary Function Testing:

* History of adverse reaction to sedation
* Clinically significant upper airway obstruction as determined by the Site Investigator (e.g. severe laryngomalacia, markedly enlarged tonsils, significant snoring, diagnosed obstructive sleep apnea)
* Severe gastroesophageal reflux, defined as persistent frequent emesis despite anti-reflux therapy
* Acute intercurrent respiratory infection, defined as an increase in cough, wheezing, or respiratory rate with onset in 2 weeks preceding visit
B: Inclusion Criteria:

* Diagnosis of CF as evidenced by one or more clinical feature consistent with the CF phenotype or positive CF newborn screen AND one or more of the following criteria:
* A documented sweat chloride ≥ 60 milliequivalents of solute per litre (mEq/L) by quantitative pilocarpine iontophoresis (QPIT)
* A documented genotype with two disease-causing mutations in the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene
* Informed consent by parent or legal guardian
* Age ≥ 36 months and ≤72 months at Screening visit
* Ability to comply with medication use, study visits and study procedures as judged by the site investigator
* Ability to perform technically acceptable MBW measurements at the screening and enrollment visits

Exclusion Criteria:

* Acute intercurrent respiratory infection, defined as an increase in cough, wheezing, or respiratory rate with onset within 3 weeks preceding Screening or Enrollment visit
* Acute wheezing at Screening or Enrollment visit
* Oxygen saturation \< 95% (\<90% in centers located above 4000 feet elevation) at Screening or Enrollment visit
* Physical findings that would compromise the safety of the participant or the quality of the study data as determined by site investigator
* Investigational drug use within 30 days prior to Screening or Enrollment visit
* Treatment with inhaled hypertonic saline at any concentration within 30 days prior to Screening or Enrollment visit
* Chronic lung disease not related to CF
* Inability to tolerate first dose of study treatment at the Enrollment visit
C: Inclusion Criteria:

1. Diagnosis of CF as evidenced by one or more clinical features consistent with the CF phenotype or positive CF newborn screen AND one or more of the following criteria:

   1. A documented sweat chloride ≥ 60 mEq/L by quantitative pilocarpine iontophoresis (QPIT)
   2. A documented genotype with two disease-causing mutations in the CFTR gene
2. Informed consent by parent or legal guardian
3. Age ≥ 36 months and ≤72 months at screening visit
4. Ability to comply with medication use, study visits and study procedures as judged by the site investigator
5. Ability to cooperate with chest CT at the enrollment visit as determined by the lung function technician

Exclusion Criteria:

1. Chest CT within 8 months prior to the Screening visit
2. Acute intercurrent respiratory infection, defined as an increase in cough, wheezing, or respiratory rate with onset within 3 weeks preceding screening or enrollment visit
3. Acute wheezing at screening or enrollment visit
4. Oxygen saturation \< 95% (\<90% in centers located above 4000 feet elevation) at screening or enrollment visit
5. Other major organ dysfunction, excluding pancreatic dysfunction
6. Physical findings that would compromise the safety of the participant or the quality of the study data as determined by site investigator
7. Investigational drug use within 30 days prior to screening or enrollment visit
8. Treatment with inhaled HS at any concentration within 30 days prior to screening or enrollment visit
9. Initiation (i.e. new prescription) of any inhaled hydrating agent such as mannitol or mucolytic agents such as dornase alpha within 30 days prior to the screening or enrollment visit
10. Chronic lung disease not related to CF
11. Inability to tolerate first dose of study treatment at the enrollment visit
D: Inclusion Criteria:

Patient Participant:

1. Participant was randomized into the SIMPLIFY study
2. Person with CF is ≥ 14 years of age at the time of randomization into SIMPLIFY
3. Is English speaking
4. Participant has provided consent (or assent if applicable) in the SIMPLIFY study to provide contact information and share data from the SIMPLIFY study with the QUEST study team
5. Participant has provided contact information via the QUEST portal
6. Is willing and able to adhere to the study requirements
7. Provides consent (or assent if applicable) for the QUEST study
8. Completed participation in the SIMPLIFY study (i.e. completed through Visit 3 or early withdrawal, if applicable)

Caregiver Participant:

1. Lives in the same household as a child with CF who is ≥ 14 years of age but \<18 years of age who meets the eligibility requirements for the QUEST study
2. Is English speaking,
3. Is willing and able to adhere to the study requirements
4. Provides consent for the QUEST study

Exclusion Criteria:

An individual who meets any of the following criteria will be excluded from participation in this study:

1. Any situation that, in the opinion of the Investigator, would compromise the safety of the patient or the quality of the data

Answer: A