Hypertonic Saline in NCFB

Repeatability of Mucociliary Clearance (MCC) at 60 minutes

The primary outcome for Aim 1 will be the correlation between the average rate of MCC measured in the whole right lung compartment over 60 minutes (MCC60), calculated using point estimates collected every 10 minutes, assessed from two separate visits (Visit 2 and Visit 3)

Average change in MCC60 from baseline (Visit 2 and 3), after acute treatment with HS (Visit 4), and after two weeks of treatment with HS (Visit 5)

Mean baseline MCC60 from Visit 2 and optional Visit 3 (if participating in Aim 1) will be compared with MCC60 measured 30 minutes after first HS dose at Visit 4, and with MCC60 measured 12 hours after last dose of HS following 2 weeks of treatment with twice a day HS at Visit 5. MCC60 will be measured as the average rate of MCC measured in the whole right lung compartment over 60 minutes, calculated using point estimates collected every 10 minutes, at Visit 2 (mean Baseline from Visit 2 and Visit 3 if completing Aim 1), Visit 4, and Visit 5.

Change in Forced Expiratory Volume in 1 Second (FEV1) from Baseline (mean from visit 2 and visit 3, pre-HS treatment) to Visit 5 (post-HS treatment period)

FEV1 is measured by spirometry. On each occasion, the best of 3 trials, based on American Thoracic Society (ATS)/European Respiratory Society (ERS) criteria. The best forced vital capacity (FVC), FEV1 and Forced expiratory flow between 25% and 75% of vital capacity (FEF25-75) (from trial with highest FVC+FEV1 sum) will be recorded (absolute value and % of predicted). Baseline measurements will occur at Visit 2 (and Visit 3 if completing Aim 1). The change in FEV1 from Baseline (either Visit 2, or mean from Visit 2 and Visit 3 if completing Aim 2) to Visit 5 will then be assessed.

Change in Quality of life for Bronchiectasis (QOL-B) Domain Scores from Visit 3 (2nd baseline visit, pre-HS treatment) to Visit 5 (post-HS treatment period)

The QOL-B Version 3.1 questionnaire is a self-administered patient reported outcome for symptom assessment and health-related quality of life in people with NCFB. It is a validated tool containing 37 items in 8 domains translated into multiple languages, with minimal important difference determined per each domain. Domains include Physical Functioning, Role Functioning, Vitality, Emotional Functioning, Social Functioning, Treatment Burden, Health Perceptions, Respiratory Symptoms. Items are on 4-point Likert scales, except for the Respiratory Symptoms domain which includes an item about sputum color, which has 6 answer choices (see separate outcome measure for sputum color). Each domain is scored separately, scores are scaled up to be in the range of 0 to 100 (higher scores correspond to better health outcomes). Participants will complete the QOL-B at baseline (Visit 2), and again post-HS treatment period (Visit 5), and changes in the domain scores will be assessed.

Change in Quality of life for Bronchiectasis (QOL-B) Respiratory Symptoms Domain Sputum Score from Visit 3 (2nd baseline visit, pre-HS treatment) to Visit 5 (post-HS treatment period)

The Respiratory Symptoms domain includes one item that assesses sputum color with 6 answer choices (unlike other items on the QOL-B which are scored on 4-point Likert scales). Answer choices include "clear" (1 point, reverse coded for scoring as 4 points), "clear to yellow" (2 points, reverse coded as 3 points), "yellowish-green" (3 points, reverse coded as 2 points), "brownish-dark" (4 points, reverse coded as 1 point), "green with traces of blood" (4 points, reverse coded as 1 point), and "don't know" (6 points, reverse coded to be not scored). The sputum score is reverse coded so that higher scores correspond to better health outcomes. Reverse coded score minimum 1, maximum 4. Participants will complete the QOL-B at baseline (Visit 2), and again post-HS treatment period (Visit 5), and changes in the domain scores will be assessed.

Change in Lung Clearance Index (LCI) as measured by Multiple Breath Washout (MBW) from Baseline (mean from Visit 2 and visit 3, pre-HS treatment) to Visit 5 (post-HS treatment period)

The LCI is the number of lung turnovers required to reduce exhaled nitrogen (N2) containing tracer gas concentration to 1/40th of its starting values. It is calculated by dividing the sum of exhaled tidal breaths (cumulative exhaled volume) by simultaneously measured functional residual capacity (FRC). LCI is able to detect abnormalities in lung function earlier than more traditional modalities such as spirometry.