Please select the eligibility criteria that correspond to the clinical trial below.
Title: The Effect of Inhaled Hypertonic Saline (7%) Versus Normal Saline (0.9%) on the Lung Clearance Index in Patients With Cystic Fibrosis
Brief Summary: This study is examining the effect of hypertonic saline compared to placebo on the Lung Clearance Index in Cystic Fibrosis patients.


Options:
A: Inclusion Criteria:

* Gender: Females or Males. If the subject is female and of childbearing potential (first menses has occurred), she must have a documented negative pregnancy test at screening and prior to each mucociliary clearance study. Those of childbearing potential must be abstinent or using an acceptable method of birth control (i.e. an Intrauterine Contraceptive Device with a failure rate of \<1%, hormonal contraceptives or a barrier method).
* Age: 5-12 years, inclusive
* Diagnosis: Cystic fibrosis documented by a compatible clinical presentation and sweat chloride \> 60 mEq/l or 2 disease causing CFTR mutations.
* Severity of the Disease: Suitable patients will have mild lung disease, as defined by:

  * Pulmonary Function: Each patient must have an FEV1 of greater than or equal to 60% of predicted at the screening visit.
  * Hemoglobin saturation: Patients must have an oxygen saturation of \>92% on room air as determined by pulse oximetry at the screening visit.
* Informed consent - The patient and a parent or legally authorized guardian must agree to the subject's participation in the study by signing and dating the informed consent/assent forms after the nature of the study has been fully explained and all questions have been satisfactorily answered.

Exclusion Criteria:

* Unstable or asthmatic lung disease: As defined by a change in medical regimen during the preceding 2 weeks; an FEV1 15% below recent (within 6 months) clinical measurements. Patients with a history of co-existent asthma, as manifested by wheezing and significant bronchoreactivity (\>15% increase in FEV1 with bronchodilator), will also be excluded.
* Other medication usage: Patients unable or unwilling to be withdrawn from hypertonic saline therapy for two weeks prior to Visit 1 (baseline MCC visit). Patients using Pulmozyme will be permitted to participate in this trial. Patients on chronic, cycling antibiotics will be required to have completed at least 2 full cycles of the prescribed antibiotic prior to enrollment and should not cycle on or off this therapy during the treatment period of the study.
* Spirometry Performance: Those subjects who are unable to perform acceptable, reproducible spirometry will be excluded from this study.
* Drug allergy: A history of allergy or intolerance to any of the study medications, including albuterol or hypertonic saline.
* Have received an investigational drug or therapy during the preceding 30 days.
* Have had radiation exposure within the past year that would cause them to exceed Federal Regulations by participating in this study.
B: 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
C: Inclusion Criteria:

* Clinical and laboratory diagnosis of cystic fibrosis
* Age greater than or equal to 5 years
* Forced expiratory volume in 1 second (FEV1) greater than or equal to 40% predicted
* Sinus surgery within one week of enrollment

Exclusion Criteria:

* Pregnancy
* Intolerance of orally inhaled Pulmozyme (dornase alfa)
D: Inclusion Criteria:

* Diagnosis of CF as defined by two or more clinical features of CF and a documented sweat chloride \> 60 mEq/L by quantitative pilocarpine iontophoresis test or a genotype showing two well characterized disease causing mutations
* Informed consent and verbal assent (as appropriate) provided by the subject's parent or legal guardian and the subject
* 6-18 years of age at enrolment and able to perform reproducible spirometry
* Clinically stable at enrolment
* FEV1 % predicted ≥ 80 % as calculated by the Wang reference equations
* Ability to comply with medication use, study visits and study procedures

Exclusion Criteria:

* Respiratory culture positive for NTM or B. cepacia complex within past year or screening
* Use of intravenous antibiotics or oral quinolones within 14 days of screening
* Investigational drug use within 30 days of screening
* Physical findings at screening that would compromise the safety of the participant or the quality of the study data

Answer: D