Airway Clearance Impairment, Cystic Fibrosis, Motor Neuron Disease
Conditions
Keywords
Airway Clearance, Cystic Fibrosis, Motor Neuron Disease, Home care
Brief summary
The study was a non-randomized open label pilot study. It was an observational design conducted at one (1) site in the US. All enrolled subjects received treatment with the MN4000. This pilot study evaluated subject satisfaction with the therapy and adherence to the therapy during the 90-day treatment period, and also collected clinical outcome data. Outcomes were assessed before, during and after the MN4000 treatment period.
Detailed description
The study was a non-randomized open label pilot study. It was an observational design conducted at one (1) site in the US. All enrolled subjects received treatment with the MN4000. This pilot study evaluated subject satisfaction with the therapy and adherence to the therapy during the 90-day treatment period, and also collected clinical outcome data. Outcomes were assessed before, during and after the MN4000 treatment period. The study did not include a control group. This pilot study was designed to provide initial information that could inform decisions for future larger-scale studies. Ten (10) patients total were enrolled from CF and NMD clinics. Eligible subjects were adult patients who were able to perform MN4000 therapy using a mouthpiece and who met all inclusion and none of the exclusion criteria. All patients received therapy with the MN4000 following the labeled instructions for the device. The MN4000 is an airway clearance and lung expansion therapy device that has been cleared to market by the FDA as The MetaNeb® System for Homecare environment, for clearance of pulmonary secretions and for treatment or prevention of pulmonary atelectasis. It is a Class II device, cleared to market on March 17, 2016 under premarket notification 510(k) K151689 as The MetaNeb® 4 System with application for homecare environment. It is commercially marketed as the MN4000. The device consists of a pneumatic compressor and an air pulse generator that delivers CHFO and CPEP to; 1. facilitate clearance of mucous from the lungs; 2. provide lung expansion therapy and; 3. enhance delivery of aerosol therapy. This triple mode device can provide aerosol therapy while alternating between CPEP for lung expansion and CHFO for airway clearance. Supplemental oxygen therapy may also be delivered when used with compressed oxygen. The MN4000 has three therapy modes: * CHFO (Continuous High Frequency Oscillation) - delivers aerosol therapy while providing oscillating pressure pulses to the airway * CPEP (Continuous Positive Expiratory Pressure) - delivers aerosol therapy while providing continuous positive pressure to help hold open and expand the airways * Aerosol - for delivery of aerosol only. In this mode, CHFO and CPEP are not available After assessing baseline status, therapy with the MN4000 was introduced and incorporated into the daily home respiratory care treatment regimen for all patients. Other airway clearance and/or lung expansion therapies were not to be performed during the three-month study period. The treatment regimen for other respiratory care modalities (e.g. aerosolized medications) was that which was prescribed by the patient's health care team in the routine standard care of each patient. During the three-month follow-up period, adherence to the daily prescribed therapy regimen was assessed. Subjects/caregivers were asked to provide adherence information for each day during the 90-day study period. Documentation of efficacy and safety Variables was completed by study staff at the time of occurrence, from review of the patient's medical records and from scores and rankings for questionnaires.
Interventions
DEVICEMN 4000
Patients who required regular home airway clearance therapy were enrolled in the study and were prescribed therapy with the MN4000. Adherence to the prescribed therapy regimen and patient/caregiver satisfaction with the therapy was assessed. Pulmonary function, was assessed for each subject at baseline, after 1 month and after 3 months of home therapy. Results from the therapy period was compared to the baseline period, during which the subject received their regular airway clearance regimen. Airway Clearance Satisfaction surveys were conducted at baseline, after 1 month, and after 3 months of therapy. Results from the MN4000 therapy period were compared to the baseline period, during which the subject received their regular regimen
Sponsors
Hill-Rom
Study design
Allocation
NA
Intervention model
SINGLE_GROUP
Primary purpose
TREATMENT
Masking
NONE
Eligibility
Sex/Gender
ALL
Age
18 Years to No maximum
Healthy volunteers
No
Inclusion criteria
* Documented diagnosis of CF or MND * Age ≥ 18 years * Signed informed consent
Exclusion criteria
* Requirement for continuous mechanical ventilation * Anticipated requirement for hospitalization within the next three months * History of pneumothorax within past 6 months * History of hemoptysis requiring embolization within past 12 months * Inability to perform MN4000 therapy using a mouthpiece (e.g. inability to create adequate mouth seal) * Inability to perform MN4000 therapy as directed * Inability or unwillingness to complete study visits or provide follow-up data as required by the study protocol
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Patient / Caregiver Satisfaction Scores | 90 days | The primary efficacy variable was patient and/or caregiver satisfaction with therapy, as evaluated using a Therapy Use Rating Questionnaire. The questionnaire was used to assess change in satisfaction or effectiveness on a variety of parameters. The satisfaction score was completed by the patient, if possible. Some of the patients in this study had Motor Neuron Disease. This disease can compromise the ability of the patient to interact and to respond to questions. In situations where patients could not respond, caregivers were asked to respond on their behalf. |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Maximal Inspiratory Pressure (MIP) | 90 days | Standard pulmonary function measures (FEV1, FVC, and FEV1/FVC) are commonly followed in the clinical care of patients with CF. Standard pulmonary function measures (SVC, SpO2, MIP, and Peak Cough Flow) are commonly followed in the clinical care of patients with MND. Maximal inspiratory pressure (MIP) is a measure of the strength of inspiratory muscles, primarily the diaphragm, and allows for the assessment of ventilatory failure, restrictive lung disease and respiratory muscle strength. Reduction in MIP is asociated with worsening neuromuscular status and poor ability to cough. |
| ALS-Functional Rating Scale (ALS-FRS) | 90 days | MND patients only. The Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS) is an instrument for evaluating the functional status of patients with Amyotrophic Lateral Sclerosis. It can be used to monitor functional change in a patient over time. It contains 10 distinct measures: * Speech * Salivation * Swallowing * Handwriting * Cutting food and handling utensils (with or without gastrostomy) * Dressing and hygiene * Turning in bed and adjusting bed clothes * Walking * Breathing * Climbing stairs These 10 parameters are scored on a scale of 0-4, based on the patients' ability to perform tasks. The minimum score is 0 and maximum 40.The higher the score the more function is retained. |
| Cystic Fibrosis Questionnaire - Revised (CFQ-R) | 90 days | CF patients only. The Cystic Fibrosis Questionnaire (CFQ) is a disease-specific instrument that measures health-related quality of life (HRQoL) for adolescents and adults with cystic fibrosis (CF) \> or = 14 years, consisting of 44 items on 12 generic and disease-specific scales. It offers 5 distinct 4-point Likert scales (e.g., always/often/ sometime/never). Scores for each HRQoL domain; after recoding, each item is summed to generate a domain score and standardized. Scores range from 0 to 100, with higher scores indicating better health. |
| Number of Participants With Exacerbation of Pulmonary Disease | 90 days | Hospitalization and/or antibiotics for respiratory infection or complication. Exacerbations of pulmonary disease were defined as respiratory infections that resulted in the requirement for hospitalization and/or antibiotics to treat the respiratory infection or complication. Hospitalizations that are part of routine care (e.g. hospital admissions for annual tune-up) or antibiotics that are part of the regular treatment regimen were not documented as exacerbations. Occurrence of exacerbation related hospitalization OR the necessity of a prescription for antibiotics qualify as worsening disease. |
| FEV1 (Actual) | 90 days | FEV1 is the actual volume measured in liters. Forced expiratory volume in one second (FEV1): The forced expiratory volume-one second is the total volume of air a patient exhales in the first second during maximal effort. Normal range is \>80%. The patient takes a full breath in and exhale as hard and fast as they can. FEV1 refers to the volume of air that an individual can exhale during a forced breath in the first 1 second. Decreasing percentage is associated with worsening / severe disease |
| FEV1 (% Predicted) | 90 days | FEV1 Percent Predicted (FEV1 % Predicted) is the actual volume exhaled in the first 1 second, divided by the Normal value for that actual value for a person of that age, gender, height and weight. A nomogram based on population norms provides a series of Normal Values. FEV1: The forced expiratory volume-one second is the total volume of air a patient exhales in the first second during maximal effort. Normal range is \>80%. Decreasing percentage is associated with worsening / severe disease |
| Mean Percentage Adherence to Prescribed Treatment Regimen | 90 days | Adherence to the treatment regimen was calculated for each patient (number of minutes of therapy performed / number of minutes of therapy prescribed). The adherence percentage calculated for each study subject was then used to calculate the of Mean adherence to prescribed treatment regimen for the group or groups. |
| FVC (% Predicted) | 90 days | FVC Percent Predicted (FVC % Predicted) is the actual volume exhaled in the first 1 second, divided by the Normal value for that actual value for a person of that age, gender, height and weight. A nomogram based on population norms provides a series of Normal Values. The functional vital capacity is the total volume of air a patient exhales for the total duration of the test during maximal effort. Normal range is \>80%. Decreasing percentage is associated with worsening / severe disease. FVC was only done in CF subjects because performance of the test requires intact respiratory muscle function. CF patients have intact respiratory muscle function and can therefore perform this maneuver. Patients with Motor Neuron Disease suffer from progressive weakness of all muscles including the respiratory muscles. The test results for FVC are affected muscle weakness and are therefore not a valid representation of their pulmonary function. |
| FEV1/FVC Ratio | 90 days | The proportion of the FVC expired in one second Results are given in both actual values (litres, litres per second) and %predicted-the test result as a percent of the predicted values for the patients of similar characteristics. Results over 80% are considered normal. Patients with Motor Neuron Disease suffer from progressive weakness of all muscles including the respiratory muscles. The test results for FVC are affected muscle weakness and are therefore not a valid representation of their pulmonary function. |
| SVC (Actual) | 90 days | Slow Vital Capacity (SVC) is the actual volume measured in liters. Slow Vital Capacity displays the volume of gas measured on a complete expiration after a maximal inspiration without forced or rapid effort. Useful measurement when FVC is reduced, and airway obstruction is present. Educed SVC is associated with worsening respiratory disease. Slow Vital Capacity provides the same measure as FVC - i.e. Vital Capacity. It does not, however, require a Forced expiratory maneuver (it requires a slow inspiration and slow expiration) and therefore is easier to achieve a valid result in patients with Motor Neuron Disease who suffer from progressive weakness of all muscles including the respiratory muscles. |
| SVC (% Predicted) | 90 days | SVC Percent Predicted (SVC % Predicted) is the actual volume exhaled in the first 1 second, divided by the Normal value for that actual value for a person of that age, gender, height and weight. A nomogram based on population norms provides a series of Normal Values. Slow Vital Capacity displays the volume of gas measured on a complete expiration after a maximal inspiration without forced or rapid effort. Useful measurement when FVC is reduced and airway obstruction is present. Educed SVC is associated with worsening respiratory disease. Slow Vital Capacity provides the same measure as FVC - i.e. Vital Capacity. It does not, however, require a Forced expiratory maneuver (it requires a slow inspiration and slow expiration) and therefore is easier to achieve a valid result in patients with Motor Neuron Disease who suffer from progressive weakness of all muscles including the respiratory muscles. |
| SpO2 | 90 days | Standard pulmonary function measures (FEV1, FVC, and FEV1/FVC) are commonly followed in the clinical care of patients with CF. Standard pulmonary function measures (SVC, SpO2, MIP, and Peak Cough Flow) are commonly followed in the clinical care of patients with MND. SpO2 stands for peripheral capillary oxygen saturation, an estimate of the amount of oxygen in the blood. Low SpO2 is associated with worsening respiratory function. |
| PCF | 90 days | Standard pulmonary function measures (FEV1, FVC, and FEV1/FVC) are commonly followed in the clinical care of patients with CF. Standard pulmonary function measures (SVC, SpO2, MIP, and PCF) are commonly followed in the clinical care of patients with MND. The Peak Cough Flow (PCF) is the maximum air flow generated during a cough. Decreasing air flow generated is associated with weakening neuromuscular status and associated poor cough impulse |
| FVC (Actual) | 90 days | FVC is the actual volume measured in liters. The functional vital capacity (FVC) is the total volume of air a patient exhales for the total duration of the test during maximal effort. Normal range is \>80%. Decreasing percentage is associated with worsening / severe disease. FVC was only done in CF subjects because performance of the test requires intact respiratory muscle function. CF patients have intact respiratory muscle function and can therefore perform this maneuver. Patients with Motor Neuron Disease suffer from progressive weakness of all muscles including the respiratory muscles. The test results for FVC are affected muscle weakness and are therefore not a valid representation of their pulmonary function. |
Countries
United States
Participant flow
Participants by arm
| Arm | Count |
|---|---|
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. Patients with CF who required regular home airway clearance therapy were enrolled to use the Metaneb device in the home setting. | 5 |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. Patients with MND who required regular home airway clearance therapy were enrolled to use the Metaneb device in the home setting. | 5 |
| Total | 10 |
Baseline characteristics
| Characteristic | Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Total | Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. |
|---|---|---|---|
| Age, Continuous | 30.4 Years STANDARD_DEVIATION 4.34 | 47.8 Years STANDARD_DEVIATION 10.44 | 65.2 Years STANDARD_DEVIATION 16.54 |
| Ethnicity (NIH/OMB) Hispanic or Latino | 0 Participants | 0 Participants | 0 Participants |
| Ethnicity (NIH/OMB) Not Hispanic or Latino | 5 Participants | 10 Participants | 5 Participants |
| Ethnicity (NIH/OMB) Unknown or Not Reported | 0 Participants | 0 Participants | 0 Participants |
| Race (NIH/OMB) American Indian or Alaska Native | 0 Participants | 0 Participants | 0 Participants |
| Race (NIH/OMB) Asian | 0 Participants | 0 Participants | 0 Participants |
| Race (NIH/OMB) Black or African American | 0 Participants | 1 Participants | 1 Participants |
| Race (NIH/OMB) More than one race | 0 Participants | 0 Participants | 0 Participants |
| Race (NIH/OMB) Native Hawaiian or Other Pacific Islander | 0 Participants | 0 Participants | 0 Participants |
| Race (NIH/OMB) Unknown or Not Reported | 0 Participants | 0 Participants | 0 Participants |
| Race (NIH/OMB) White | 5 Participants | 9 Participants | 4 Participants |
| Region of Enrollment United States | 5 participants | 10 participants | 5 participants |
| Sex: Female, Male Female | 5 Participants | 5 Participants | 0 Participants |
| Sex: Female, Male Male | 0 Participants | 5 Participants | 5 Participants |
Adverse events
| Event type | EG000 affected / at risk | EG001 affected / at risk |
|---|---|---|
| deaths Total, all-cause mortality | 0 / 5 | 0 / 5 |
| other Total, other adverse events | 0 / 5 | 0 / 5 |
| serious Total, serious adverse events | 0 / 5 | 0 / 5 |
Outcome results
Primary
Patient / Caregiver Satisfaction Scores
The primary efficacy variable was patient and/or caregiver satisfaction with therapy, as evaluated using a Therapy Use Rating Questionnaire. The questionnaire was used to assess change in satisfaction or effectiveness on a variety of parameters. The satisfaction score was completed by the patient, if possible. Some of the patients in this study had Motor Neuron Disease. This disease can compromise the ability of the patient to interact and to respond to questions. In situations where patients could not respond, caregivers were asked to respond on their behalf.
Time frame: 90 days
| Arm | Measure | Group | Category | Value (COUNT_OF_PARTICIPANTS) |
|---|---|---|---|---|
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Patient / Caregiver Satisfaction Scores | Missed Treatment (Day 30) | Missing | 0 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Patient / Caregiver Satisfaction Scores | Bring up sputum (Day 90) | Worsened | 2 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Patient / Caregiver Satisfaction Scores | Ability to perform tasks (Day 90) | Same | 2 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Patient / Caregiver Satisfaction Scores | Difficulty of Use (Day 90) | Missing | 0 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Patient / Caregiver Satisfaction Scores | Difficulty of Use (Day 30) | Improved | 0 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Patient / Caregiver Satisfaction Scores | Difficulty of Use (Day 30) | Same | 4 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Patient / Caregiver Satisfaction Scores | Missed Treatment (Day 90) | Improved | 3 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Patient / Caregiver Satisfaction Scores | Missed Treatment (Day 90) | Same | 1 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Patient / Caregiver Satisfaction Scores | Missed Treatment (Day 90) | Worsened | 1 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Patient / Caregiver Satisfaction Scores | Missed Treatment (Day 90) | Missing | 0 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Patient / Caregiver Satisfaction Scores | Bring up sputum (Day 90) | Improved | 1 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Patient / Caregiver Satisfaction Scores | Bring up sputum (Day 90) | Same | 2 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Patient / Caregiver Satisfaction Scores | Bring up sputum (Day 90) | Missing | 0 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Patient / Caregiver Satisfaction Scores | Shortness of breath (Day 90) | Improved | 1 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Patient / Caregiver Satisfaction Scores | Shortness of breath (Day 90) | Same | 1 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Patient / Caregiver Satisfaction Scores | Shortness of breath (Day 90) | Worsened | 1 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Patient / Caregiver Satisfaction Scores | Shortness of breath (Day 90) | Missing | 2 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Patient / Caregiver Satisfaction Scores | Lungs stay healthy (Day 90) | Improved | 0 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Patient / Caregiver Satisfaction Scores | Lungs stay healthy (Day 90) | Same | 3 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Patient / Caregiver Satisfaction Scores | Lungs stay healthy (Day 90) | Worsened | 2 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Patient / Caregiver Satisfaction Scores | Lungs stay healthy (Day 90) | Missing | 0 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Patient / Caregiver Satisfaction Scores | Ability to perform tasks (Day 90) | Improved | 0 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Patient / Caregiver Satisfaction Scores | Ability to perform tasks (Day 90) | Worsened | 3 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Patient / Caregiver Satisfaction Scores | Ability to perform tasks (Day 90) | Missing | 0 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Patient / Caregiver Satisfaction Scores | Difficulty of Use (Day 30) | Worsened | 1 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Patient / Caregiver Satisfaction Scores | Difficulty of Use (Day 30) | Missing | 0 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Patient / Caregiver Satisfaction Scores | Missed Treatment (Day 30) | Improved | 2 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Patient / Caregiver Satisfaction Scores | Missed Treatment (Day 30) | Same | 2 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Patient / Caregiver Satisfaction Scores | Missed Treatment (Day 30) | Worsened | 1 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Patient / Caregiver Satisfaction Scores | Bring up sputum (Day 30) | Improved | 1 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Patient / Caregiver Satisfaction Scores | Bring up sputum (Day 30) | Same | 3 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Patient / Caregiver Satisfaction Scores | Bring up sputum (Day 30) | Worsened | 1 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Patient / Caregiver Satisfaction Scores | Bring up sputum (Day 30) | Missing | 0 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Patient / Caregiver Satisfaction Scores | Shortness of breath (Day 30) | Improved | 0 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Patient / Caregiver Satisfaction Scores | Shortness of breath (Day 30) | Same | 2 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Patient / Caregiver Satisfaction Scores | Shortness of breath (Day 30) | Worsened | 1 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Patient / Caregiver Satisfaction Scores | Shortness of breath (Day 30) | Missing | 2 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Patient / Caregiver Satisfaction Scores | Lungs stay healthy (Day 30) | Improved | 0 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Patient / Caregiver Satisfaction Scores | Lungs stay healthy (Day 30) | Same | 4 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Patient / Caregiver Satisfaction Scores | Lungs stay healthy (Day 30) | Worsened | 1 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Patient / Caregiver Satisfaction Scores | Lungs stay healthy (Day 30) | Missing | 0 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Patient / Caregiver Satisfaction Scores | Ability to perform tasks (Day 30) | Improved | 1 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Patient / Caregiver Satisfaction Scores | Ability to perform tasks (Day 30) | Same | 2 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Patient / Caregiver Satisfaction Scores | Ability to perform tasks (Day 30) | Worsened | 2 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Patient / Caregiver Satisfaction Scores | Ability to perform tasks (Day 30) | Missing | 0 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Patient / Caregiver Satisfaction Scores | Difficulty of Use (Day 90) | Improved | 0 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Patient / Caregiver Satisfaction Scores | Difficulty of Use (Day 90) | Same | 3 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Patient / Caregiver Satisfaction Scores | Difficulty of Use (Day 90) | Worsened | 2 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | Patient / Caregiver Satisfaction Scores | Difficulty of Use (Day 90) | Same | 3 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | Patient / Caregiver Satisfaction Scores | Bring up sputum (Day 30) | Improved | 4 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | Patient / Caregiver Satisfaction Scores | Difficulty of Use (Day 30) | Worsened | 1 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | Patient / Caregiver Satisfaction Scores | Ability to perform tasks (Day 90) | Improved | 2 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | Patient / Caregiver Satisfaction Scores | Shortness of breath (Day 30) | Missing | 2 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | Patient / Caregiver Satisfaction Scores | Difficulty of Use (Day 30) | Missing | 0 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | Patient / Caregiver Satisfaction Scores | Ability to perform tasks (Day 30) | Same | 2 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | Patient / Caregiver Satisfaction Scores | Difficulty of Use (Day 30) | Improved | 0 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | Patient / Caregiver Satisfaction Scores | Missed Treatment (Day 30) | Improved | 2 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | Patient / Caregiver Satisfaction Scores | Difficulty of Use (Day 90) | Missing | 0 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | Patient / Caregiver Satisfaction Scores | Lungs stay healthy (Day 30) | Improved | 3 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | Patient / Caregiver Satisfaction Scores | Missed Treatment (Day 90) | Improved | 1 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | Patient / Caregiver Satisfaction Scores | Missed Treatment (Day 30) | Same | 2 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | Patient / Caregiver Satisfaction Scores | Missed Treatment (Day 90) | Same | 3 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | Patient / Caregiver Satisfaction Scores | Difficulty of Use (Day 90) | Improved | 1 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | Patient / Caregiver Satisfaction Scores | Missed Treatment (Day 90) | Worsened | 1 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | Patient / Caregiver Satisfaction Scores | Missed Treatment (Day 30) | Worsened | 1 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | Patient / Caregiver Satisfaction Scores | Missed Treatment (Day 90) | Missing | 0 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | Patient / Caregiver Satisfaction Scores | Missed Treatment (Day 30) | Missing | 0 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | Patient / Caregiver Satisfaction Scores | Bring up sputum (Day 90) | Improved | 4 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | Patient / Caregiver Satisfaction Scores | Lungs stay healthy (Day 30) | Same | 2 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | Patient / Caregiver Satisfaction Scores | Bring up sputum (Day 90) | Same | 0 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | Patient / Caregiver Satisfaction Scores | Bring up sputum (Day 90) | Worsened | 1 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | Patient / Caregiver Satisfaction Scores | Ability to perform tasks (Day 30) | Worsened | 2 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | Patient / Caregiver Satisfaction Scores | Bring up sputum (Day 90) | Missing | 0 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | Patient / Caregiver Satisfaction Scores | Bring up sputum (Day 30) | Same | 0 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | Patient / Caregiver Satisfaction Scores | Shortness of breath (Day 90) | Improved | 2 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | Patient / Caregiver Satisfaction Scores | Lungs stay healthy (Day 30) | Worsened | 0 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | Patient / Caregiver Satisfaction Scores | Shortness of breath (Day 90) | Same | 0 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | Patient / Caregiver Satisfaction Scores | Bring up sputum (Day 30) | Worsened | 1 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | Patient / Caregiver Satisfaction Scores | Shortness of breath (Day 90) | Worsened | 1 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | Patient / Caregiver Satisfaction Scores | Difficulty of Use (Day 90) | Worsened | 1 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | Patient / Caregiver Satisfaction Scores | Shortness of breath (Day 90) | Missing | 2 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | Patient / Caregiver Satisfaction Scores | Bring up sputum (Day 30) | Missing | 0 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | Patient / Caregiver Satisfaction Scores | Lungs stay healthy (Day 90) | Improved | 3 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | Patient / Caregiver Satisfaction Scores | Lungs stay healthy (Day 30) | Missing | 0 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | Patient / Caregiver Satisfaction Scores | Lungs stay healthy (Day 90) | Same | 1 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | Patient / Caregiver Satisfaction Scores | Shortness of breath (Day 30) | Improved | 2 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | Patient / Caregiver Satisfaction Scores | Lungs stay healthy (Day 90) | Worsened | 1 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | Patient / Caregiver Satisfaction Scores | Ability to perform tasks (Day 30) | Missing | 0 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | Patient / Caregiver Satisfaction Scores | Lungs stay healthy (Day 90) | Missing | 0 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | Patient / Caregiver Satisfaction Scores | Shortness of breath (Day 30) | Same | 0 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | Patient / Caregiver Satisfaction Scores | Ability to perform tasks (Day 90) | Same | 2 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | Patient / Caregiver Satisfaction Scores | Ability to perform tasks (Day 30) | Improved | 1 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | Patient / Caregiver Satisfaction Scores | Ability to perform tasks (Day 90) | Worsened | 1 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | Patient / Caregiver Satisfaction Scores | Shortness of breath (Day 30) | Worsened | 1 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | Patient / Caregiver Satisfaction Scores | Ability to perform tasks (Day 90) | Missing | 0 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | Patient / Caregiver Satisfaction Scores | Difficulty of Use (Day 30) | Same | 4 Participants |
Primary
Patient / Caregiver Satisfaction Scores
The Therapy Use Rating Questionnaire is an assessment of patient/caregiver satisfaction with the therapy and their subjective assessment of the benefit of the therapy. The questionnaire was used to assess change in satisfaction or effectiveness on a variety of parameters. The satisfaction score was completed by the patient, if possible. Some of the patients in this study had Motor Neuron Disease This disease can compromise the ability of the patient to interact and to respond to questions. In situations where patients could not respond, caregivers were asked to respond on their behalf.
Time frame: 90 days
| Arm | Measure | Group | Category | Value (COUNT_OF_PARTICIPANTS) |
|---|---|---|---|---|
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Patient / Caregiver Satisfaction Scores | Like to continue MN4000 (Day 30) | Much More | 3 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Patient / Caregiver Satisfaction Scores | Easy to perform (Day 30) | Less | 1 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Patient / Caregiver Satisfaction Scores | Effective (Day 90) | Much less | 0 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Patient / Caregiver Satisfaction Scores | Effective (Day 90) | More | 0 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Patient / Caregiver Satisfaction Scores | Effective (Day 90) | Less | 1 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Patient / Caregiver Satisfaction Scores | Easy to perform (Day 30) | Same | 1 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Patient / Caregiver Satisfaction Scores | Effective (Day 90) | Same | 3 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Patient / Caregiver Satisfaction Scores | Effective (Day 30) | More | 1 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Patient / Caregiver Satisfaction Scores | Effective (Day 90) | Much More | 1 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Patient / Caregiver Satisfaction Scores | Easy to perform (Day 30) | More | 1 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Patient / Caregiver Satisfaction Scores | Easy to perform (Day 90) | Much less | 2 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Patient / Caregiver Satisfaction Scores | Easy to perform (Day 30) | Much More | 1 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Patient / Caregiver Satisfaction Scores | Easy to perform (Day 90) | Less | 2 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Patient / Caregiver Satisfaction Scores | Effective (Day 30) | Less | 1 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Patient / Caregiver Satisfaction Scores | Easy to perform (Day 90) | Same | 0 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Patient / Caregiver Satisfaction Scores | Like to continue MN4000 (Day 30) | Much less | 1 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Patient / Caregiver Satisfaction Scores | Easy to perform (Day 90) | More | 1 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Patient / Caregiver Satisfaction Scores | Effective (Day 30) | Much More | 1 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Patient / Caregiver Satisfaction Scores | Easy to perform (Day 90) | Much More | 0 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Patient / Caregiver Satisfaction Scores | Like to continue MN4000 (Day 30) | Less | 0 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Patient / Caregiver Satisfaction Scores | Like to continue MN4000 (Day 90) | Much less | 1 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Patient / Caregiver Satisfaction Scores | Effective (Day 30) | Much less | 0 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Patient / Caregiver Satisfaction Scores | Like to continue MN4000 (Day 90) | Less | 1 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Patient / Caregiver Satisfaction Scores | Like to continue MN4000 (Day 30) | Same | 0 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Patient / Caregiver Satisfaction Scores | Like to continue MN4000 (Day 90) | Same | 0 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Patient / Caregiver Satisfaction Scores | Easy to perform (Day 30) | Much less | 1 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Patient / Caregiver Satisfaction Scores | Like to continue MN4000 (Day 90) | More | 1 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Patient / Caregiver Satisfaction Scores | Like to continue MN4000 (Day 30) | More | 1 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Patient / Caregiver Satisfaction Scores | Like to continue MN4000 (Day 90) | Much More | 2 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Patient / Caregiver Satisfaction Scores | Effective (Day 30) | Same | 2 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | Patient / Caregiver Satisfaction Scores | Like to continue MN4000 (Day 90) | Much More | 4 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | Patient / Caregiver Satisfaction Scores | Effective (Day 90) | Same | 0 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | Patient / Caregiver Satisfaction Scores | Effective (Day 30) | Much less | 0 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | Patient / Caregiver Satisfaction Scores | Effective (Day 30) | Less | 0 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | Patient / Caregiver Satisfaction Scores | Effective (Day 30) | Same | 2 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | Patient / Caregiver Satisfaction Scores | Effective (Day 30) | More | 2 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | Patient / Caregiver Satisfaction Scores | Effective (Day 30) | Much More | 1 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | Patient / Caregiver Satisfaction Scores | Easy to perform (Day 30) | Much less | 0 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | Patient / Caregiver Satisfaction Scores | Easy to perform (Day 30) | Less | 2 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | Patient / Caregiver Satisfaction Scores | Easy to perform (Day 30) | Same | 1 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | Patient / Caregiver Satisfaction Scores | Easy to perform (Day 30) | Much More | 1 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | Patient / Caregiver Satisfaction Scores | Like to continue MN4000 (Day 30) | Much less | 0 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | Patient / Caregiver Satisfaction Scores | Like to continue MN4000 (Day 30) | Less | 0 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | Patient / Caregiver Satisfaction Scores | Like to continue MN4000 (Day 30) | Same | 0 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | Patient / Caregiver Satisfaction Scores | Like to continue MN4000 (Day 30) | More | 0 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | Patient / Caregiver Satisfaction Scores | Like to continue MN4000 (Day 30) | Much More | 5 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | Patient / Caregiver Satisfaction Scores | Effective (Day 90) | Much less | 0 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | Patient / Caregiver Satisfaction Scores | Effective (Day 90) | Less | 1 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | Patient / Caregiver Satisfaction Scores | Effective (Day 90) | More | 1 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | Patient / Caregiver Satisfaction Scores | Effective (Day 90) | Much More | 3 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | Patient / Caregiver Satisfaction Scores | Easy to perform (Day 90) | Much less | 1 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | Patient / Caregiver Satisfaction Scores | Easy to perform (Day 90) | Less | 0 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | Patient / Caregiver Satisfaction Scores | Easy to perform (Day 90) | Same | 1 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | Patient / Caregiver Satisfaction Scores | Easy to perform (Day 90) | More | 1 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | Patient / Caregiver Satisfaction Scores | Easy to perform (Day 90) | Much More | 2 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | Patient / Caregiver Satisfaction Scores | Like to continue MN4000 (Day 90) | Much less | 1 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | Patient / Caregiver Satisfaction Scores | Like to continue MN4000 (Day 90) | Less | 0 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | Patient / Caregiver Satisfaction Scores | Like to continue MN4000 (Day 90) | Same | 0 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | Patient / Caregiver Satisfaction Scores | Like to continue MN4000 (Day 90) | More | 0 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | Patient / Caregiver Satisfaction Scores | Easy to perform (Day 30) | More | 1 Participants |
Secondary
ALS-Functional Rating Scale (ALS-FRS)
MND patients only. The Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS) is an instrument for evaluating the functional status of patients with Amyotrophic Lateral Sclerosis. It can be used to monitor functional change in a patient over time. It contains 10 distinct measures: * Speech * Salivation * Swallowing * Handwriting * Cutting food and handling utensils (with or without gastrostomy) * Dressing and hygiene * Turning in bed and adjusting bed clothes * Walking * Breathing * Climbing stairs These 10 parameters are scored on a scale of 0-4, based on the patients' ability to perform tasks. The minimum score is 0 and maximum 40.The higher the score the more function is retained.
Time frame: 90 days
Population: MND subpopulation
| Arm | Measure | Group | Value (MEAN) | Dispersion |
|---|---|---|---|---|
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | ALS-Functional Rating Scale (ALS-FRS) | Bulbar Sub-Score at Baseline | 10.8 score on a scale | Standard Deviation 1.64 |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | ALS-Functional Rating Scale (ALS-FRS) | Bulbar Sub-Score after 30 days of therapy | 10.0 score on a scale | Standard Deviation 3.39 |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | ALS-Functional Rating Scale (ALS-FRS) | Bulbar Sub-Score after 90 days of therapy | 10.4 score on a scale | Standard Deviation 3.05 |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | ALS-Functional Rating Scale (ALS-FRS) | Respiratory Sub-score at baseline | 9.4 score on a scale | Standard Deviation 1.52 |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | ALS-Functional Rating Scale (ALS-FRS) | Respiratory Sub-score after 30 days of therapy | 10.2 score on a scale | Standard Deviation 1.1 |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | ALS-Functional Rating Scale (ALS-FRS) | Respiratory Sub-score after 90 days of therapy | 10.2 score on a scale | Standard Deviation 1.1 |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | ALS-Functional Rating Scale (ALS-FRS) | Total Score at Baseline | 33.6 score on a scale | Standard Deviation 6.84 |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | ALS-Functional Rating Scale (ALS-FRS) | Total Score after 30 days of therapy | 32.0 score on a scale | Standard Deviation 5.29 |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | ALS-Functional Rating Scale (ALS-FRS) | Total Score after 90 days of therapy | 32.2 score on a scale | Standard Deviation 5.07 |
Secondary
Cystic Fibrosis Questionnaire - Revised (CFQ-R)
CF patients only. The Cystic Fibrosis Questionnaire (CFQ) is a disease-specific instrument that measures health-related quality of life (HRQoL) for adolescents and adults with cystic fibrosis (CF) \> or = 14 years, consisting of 44 items on 12 generic and disease-specific scales. It offers 5 distinct 4-point Likert scales (e.g., always/often/ sometime/never). Scores for each HRQoL domain; after recoding, each item is summed to generate a domain score and standardized. Scores range from 0 to 100, with higher scores indicating better health.
Time frame: 90 days
Population: Cystic Fibrosis Questionnaire - Revised (CFQ-R) administered on the CF patient subpopulation
| Arm | Measure | Group | Value (MEAN) | Dispersion |
|---|---|---|---|---|
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Cystic Fibrosis Questionnaire - Revised (CFQ-R) | Vitality domain after 30 days of therapy | 73.3 Score on a scale | Standard Deviation 6.97 |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Cystic Fibrosis Questionnaire - Revised (CFQ-R) | Social domain at baseline | 77.8 Score on a scale | Standard Deviation 14.7 |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Cystic Fibrosis Questionnaire - Revised (CFQ-R) | Physical domain at baseline | 83.3 Score on a scale | Standard Deviation 19.54 |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Cystic Fibrosis Questionnaire - Revised (CFQ-R) | Physical domain after 30 days of therapy | 78.3 Score on a scale | Standard Deviation 20.28 |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Cystic Fibrosis Questionnaire - Revised (CFQ-R) | Physical domain after 90 days of therapy | 80.0 Score on a scale | Standard Deviation 17.78 |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Cystic Fibrosis Questionnaire - Revised (CFQ-R) | Role domain at baseline | 80.0 Score on a scale | Standard Deviation 7.45 |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Cystic Fibrosis Questionnaire - Revised (CFQ-R) | Role domain after 30 days of therapy | 90.0 Score on a scale | Standard Deviation 6.97 |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Cystic Fibrosis Questionnaire - Revised (CFQ-R) | Role domain after 90 days of therapy | 86.7 Score on a scale | Standard Deviation 11.18 |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Cystic Fibrosis Questionnaire - Revised (CFQ-R) | Vitality domain at baseline | 75.0 Score on a scale | Standard Deviation 16.67 |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Cystic Fibrosis Questionnaire - Revised (CFQ-R) | Vitality domain after 90 days of therapy | 68.3 Score on a scale | Standard Deviation 13.69 |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Cystic Fibrosis Questionnaire - Revised (CFQ-R) | Emotion domain at baseline | 89.3 Score on a scale | Standard Deviation 7.6 |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Cystic Fibrosis Questionnaire - Revised (CFQ-R) | Emotion domain after 30 days of therapy | 81.3 Score on a scale | Standard Deviation 8.69 |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Cystic Fibrosis Questionnaire - Revised (CFQ-R) | Emotion domain after 90 days of therapy | 86.7 Score on a scale | Standard Deviation 10.54 |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Cystic Fibrosis Questionnaire - Revised (CFQ-R) | Social domain after 30 days of therapy | 77.8 Score on a scale | Standard Deviation 13.61 |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Cystic Fibrosis Questionnaire - Revised (CFQ-R) | Social domain after 90 days of therapy | 71.1 Score on a scale | Standard Deviation 13.83 |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Cystic Fibrosis Questionnaire - Revised (CFQ-R) | Body domain at baseline | 97.8 Score on a scale | Standard Deviation 4.97 |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Cystic Fibrosis Questionnaire - Revised (CFQ-R) | Body domain after 30 days of therapy | 91.1 Score on a scale | Standard Deviation 14.49 |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Cystic Fibrosis Questionnaire - Revised (CFQ-R) | Body domain after 90 days of therapy | 91.1 Score on a scale | Standard Deviation 9.3 |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Cystic Fibrosis Questionnaire - Revised (CFQ-R) | Eat domain at baseline | 97.8 Score on a scale | Standard Deviation 4.97 |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Cystic Fibrosis Questionnaire - Revised (CFQ-R) | Eat domain after 30 days of therapy | 100.0 Score on a scale | Standard Deviation 0 |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Cystic Fibrosis Questionnaire - Revised (CFQ-R) | Eat domain after 90 days of therapy | 100.0 Score on a scale | Standard Deviation 0 |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Cystic Fibrosis Questionnaire - Revised (CFQ-R) | Treat domain at baseline | 55.6 Score on a scale | Standard Deviation 11.11 |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Cystic Fibrosis Questionnaire - Revised (CFQ-R) | Treat domain after 30 days of therapy | 48.9 Score on a scale | Standard Deviation 9.94 |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Cystic Fibrosis Questionnaire - Revised (CFQ-R) | Treat domain after 90 days of therapy | 46.7 Score on a scale | Standard Deviation 9.3 |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Cystic Fibrosis Questionnaire - Revised (CFQ-R) | Health domain at baseline | 71.1 Score on a scale | Standard Deviation 6.09 |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Cystic Fibrosis Questionnaire - Revised (CFQ-R) | Health domain after 30 days of therapy | 71.1 Score on a scale | Standard Deviation 6.09 |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Cystic Fibrosis Questionnaire - Revised (CFQ-R) | Health domain after 90 days of therapy | 73.3 Score on a scale | Standard Deviation 14.91 |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Cystic Fibrosis Questionnaire - Revised (CFQ-R) | Weight domain at baseline | 73.3 Score on a scale | Standard Deviation 36.51 |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Cystic Fibrosis Questionnaire - Revised (CFQ-R) | Weight domain after 30 days of therapy | 100.0 Score on a scale | Standard Deviation 0 |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Cystic Fibrosis Questionnaire - Revised (CFQ-R) | Weight domain after 90 days of therapy | 100.0 Score on a scale | Standard Deviation 0 |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Cystic Fibrosis Questionnaire - Revised (CFQ-R) | Respiratory domain at baseline | 61.1 Score on a scale | Standard Deviation 23.9 |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Cystic Fibrosis Questionnaire - Revised (CFQ-R) | Respiratory domain after 30 days of therapy | 61.1 Score on a scale | Standard Deviation 19.64 |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Cystic Fibrosis Questionnaire - Revised (CFQ-R) | Respiratory domain after 90 days of therapy | 62.2 Score on a scale | Standard Deviation 10.69 |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Cystic Fibrosis Questionnaire - Revised (CFQ-R) | Digestion domain at baseline | 84.4 Score on a scale | Standard Deviation 23.04 |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Cystic Fibrosis Questionnaire - Revised (CFQ-R) | Digestion domain after 30 days of therapy | 93.3 Score on a scale | Standard Deviation 9.94 |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Cystic Fibrosis Questionnaire - Revised (CFQ-R) | Digestion domain after 90 days of therapy | 91.1 Score on a scale | Standard Deviation 4.97 |
Secondary
FEV1 (Actual)
FEV1 is the actual volume measured in liters. Forced expiratory volume in one second (FEV1): The forced expiratory volume-one second is the total volume of air a patient exhales in the first second during maximal effort. Normal range is \>80%. The patient takes a full breath in and exhale as hard and fast as they can. FEV1 refers to the volume of air that an individual can exhale during a forced breath in the first 1 second. Decreasing percentage is associated with worsening / severe disease
Time frame: 90 days
| Arm | Measure | Group | Value (MEAN) | Dispersion |
|---|---|---|---|---|
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | FEV1 (Actual) | FEV1 measure at baseline (Actual) | 1.8 Liters | Standard Deviation 0.94 |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | FEV1 (Actual) | FEV1 measure after 30 days of therapy (Actual) | 1.9 Liters | Standard Deviation 1.05 |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | FEV1 (Actual) | FEV1 measure after 90 days of therapy (Actual) | 1.9 Liters | Standard Deviation 0.93 |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | FEV1 (Actual) | FEV1 measure at baseline (Actual) | 1.7 Liters | Standard Deviation 0.54 |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | FEV1 (Actual) | FEV1 measure after 30 days of therapy (Actual) | 1.6 Liters | Standard Deviation 0.71 |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | FEV1 (Actual) | FEV1 measure after 90 days of therapy (Actual) | 1.7 Liters | Standard Deviation 0.73 |
Secondary
FEV1/FVC Ratio
The proportion of the FVC expired in one second Results are given in both actual values (litres, litres per second) and %predicted-the test result as a percent of the predicted values for the patients of similar characteristics. Results over 80% are considered normal. Patients with Motor Neuron Disease suffer from progressive weakness of all muscles including the respiratory muscles. The test results for FVC are affected muscle weakness and are therefore not a valid representation of their pulmonary function.
Time frame: 90 days
Population: FVC and FEV1/FVC ratio are pulmonary function tests appropriate to assess lung function in patients with CF. These tests could not be physically performed by the patients in the MND population and thus these patients were not included in these analysis. For the MND population, SVC, SpO2, MIP, and PCF were performed instead of FVC and FEV1/FVC ratio. Hence, no data were collected from MND patients for this outcome measure.
| Arm | Measure | Group | Value (MEAN) | Dispersion |
|---|---|---|---|---|
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | FEV1/FVC Ratio | FEV1/FVC at baseline | 78.4 FEV1/FVC ratio | Standard Deviation 19.78 |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | FEV1/FVC Ratio | FEV1/FVC after 30 days of therapy | 81.4 FEV1/FVC ratio | Standard Deviation 18.42 |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | FEV1/FVC Ratio | FEV1/FVC after 90 days of therapy | 82.4 FEV1/FVC ratio | Standard Deviation 14.54 |
Secondary
FEV1 (% Predicted)
FEV1 Percent Predicted (FEV1 % Predicted) is the actual volume exhaled in the first 1 second, divided by the Normal value for that actual value for a person of that age, gender, height and weight. A nomogram based on population norms provides a series of Normal Values. FEV1: The forced expiratory volume-one second is the total volume of air a patient exhales in the first second during maximal effort. Normal range is \>80%. Decreasing percentage is associated with worsening / severe disease
Time frame: 90 days
| Arm | Measure | Group | Value (MEAN) | Dispersion |
|---|---|---|---|---|
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | FEV1 (% Predicted) | FEV1 measure after 90 days of therapy (% Predicted) | 60.4 percentage of predicted normal | Standard Deviation 24.76 |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | FEV1 (% Predicted) | FEV1 measure at baseline (% Predicted) | 55.2 percentage of predicted normal | Standard Deviation 25.49 |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | FEV1 (% Predicted) | FEV1 measure after 30 days of therapy (% Predicted) | 58.8 percentage of predicted normal | Standard Deviation 27.81 |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | FEV1 (% Predicted) | FEV1 measure after 90 days of therapy (% Predicted) | 59.4 percentage of predicted normal | Standard Deviation 27.67 |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | FEV1 (% Predicted) | FEV1 measure at baseline (% Predicted) | 58.6 percentage of predicted normal | Standard Deviation 19.49 |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | FEV1 (% Predicted) | FEV1 measure after 30 days of therapy (% Predicted) | 57.8 percentage of predicted normal | Standard Deviation 28.42 |
Secondary
FVC (Actual)
FVC is the actual volume measured in liters. The functional vital capacity (FVC) is the total volume of air a patient exhales for the total duration of the test during maximal effort. Normal range is \>80%. Decreasing percentage is associated with worsening / severe disease. FVC was only done in CF subjects because performance of the test requires intact respiratory muscle function. CF patients have intact respiratory muscle function and can therefore perform this maneuver. Patients with Motor Neuron Disease suffer from progressive weakness of all muscles including the respiratory muscles. The test results for FVC are affected muscle weakness and are therefore not a valid representation of their pulmonary function.
Time frame: 90 days
Population: FVC and FEV1/FVC ratio are pulmonary function tests appropriate to assess lung function in patients with CF. These tests could not be physically performed by the patients in the MND population and thus these patients were not included in these analysis. For the MND population, SVC, SpO2, MIP, and PCF were performed instead of FVC and FEV1/FVC ratio. Hence, no data were collected from MND patients for this outcome measure.
| Arm | Measure | Group | Value (MEAN) | Dispersion |
|---|---|---|---|---|
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | FVC (Actual) | FVC at baseline (Actual) | 2.6 Liters | Standard Deviation 0.98 |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | FVC (Actual) | FVC after 30 days of therapy (Actual) | 2.6 Liters | Standard Deviation 1.08 |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | FVC (Actual) | FVC after 90 days of therapy (Actual) | 2.7 Liters | Standard Deviation 0.99 |
Secondary
FVC (% Predicted)
FVC Percent Predicted (FVC % Predicted) is the actual volume exhaled in the first 1 second, divided by the Normal value for that actual value for a person of that age, gender, height and weight. A nomogram based on population norms provides a series of Normal Values. The functional vital capacity is the total volume of air a patient exhales for the total duration of the test during maximal effort. Normal range is \>80%. Decreasing percentage is associated with worsening / severe disease. FVC was only done in CF subjects because performance of the test requires intact respiratory muscle function. CF patients have intact respiratory muscle function and can therefore perform this maneuver. Patients with Motor Neuron Disease suffer from progressive weakness of all muscles including the respiratory muscles. The test results for FVC are affected muscle weakness and are therefore not a valid representation of their pulmonary function.
Time frame: 90 days
Population: FVC and FEV1/FVC ratio are pulmonary function tests appropriate to assess lung function in patients with CF. These tests could not be physically performed by the patients in the MND population and thus these patients were not included in these analysis. For the MND population, SVC, SpO2, MIP, and PCF were performed instead of FVC and FEV1/FVC ratio. Hence, no data were collected from MND patients for this outcome measure.
| Arm | Measure | Group | Value (MEAN) | Dispersion |
|---|---|---|---|---|
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | FVC (% Predicted) | FVC at baseline (% Predicted) | 68.6 percentage of predicted normal | Standard Deviation 22.09 |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | FVC (% Predicted) | FVC after 30 days of therapy (% Predicted) | 69.8 percentage of predicted normal | Standard Deviation 23.19 |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | FVC (% Predicted) | FVC after 90 days of therapy (% Predicted) | 71.0 percentage of predicted normal | Standard Deviation 21.3 |
Secondary
Maximal Inspiratory Pressure (MIP)
Standard pulmonary function measures (FEV1, FVC, and FEV1/FVC) are commonly followed in the clinical care of patients with CF. Standard pulmonary function measures (SVC, SpO2, MIP, and Peak Cough Flow) are commonly followed in the clinical care of patients with MND. Maximal inspiratory pressure (MIP) is a measure of the strength of inspiratory muscles, primarily the diaphragm, and allows for the assessment of ventilatory failure, restrictive lung disease and respiratory muscle strength. Reduction in MIP is asociated with worsening neuromuscular status and poor ability to cough.
Time frame: 90 days
Population: FVC and FEV1/FVC ratio are pulmonary function tests appropriate to assess lung function in patients with CF. These tests could not be physically performed by the patients in the MND population and thus these patients were not included in these analysis. For the MND population, SVC, SpO2, MIP, and PCF were performed instead of FVC and FEV1/FVC ratio. Hence, no data were collected from CF patients for this outcome measure.
| Arm | Measure | Group | Value (MEAN) | Dispersion |
|---|---|---|---|---|
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Maximal Inspiratory Pressure (MIP) | MIP at baseline | -41.0 cmH2O | Standard Deviation 21.24 |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Maximal Inspiratory Pressure (MIP) | MIP after 30 days of therapy | -35.6 cmH2O | Standard Deviation 19.65 |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Maximal Inspiratory Pressure (MIP) | MIP at 90 days of therapy | -34.8 cmH2O | Standard Deviation 15.32 |
Secondary
Mean Percentage Adherence to Prescribed Treatment Regimen
Adherence to the treatment regimen was calculated for each patient (number of minutes of therapy performed / number of minutes of therapy prescribed). The adherence percentage calculated for each study subject was then used to calculate the of Mean adherence to prescribed treatment regimen for the group or groups.
Time frame: 90 days
Population: CF and MND population
| Arm | Measure | Value (MEAN) |
|---|---|---|
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Mean Percentage Adherence to Prescribed Treatment Regimen | 97 Percentage of adherence to therapy |
| Home Based Airway Clearance With Metaneb in Patients With Motor Neuron Disease. | Mean Percentage Adherence to Prescribed Treatment Regimen | 95 Percentage of adherence to therapy |
Secondary
Number of Participants With Exacerbation of Pulmonary Disease
Hospitalization and/or antibiotics for respiratory infection or complication. Exacerbations of pulmonary disease were defined as respiratory infections that resulted in the requirement for hospitalization and/or antibiotics to treat the respiratory infection or complication. Hospitalizations that are part of routine care (e.g. hospital admissions for annual tune-up) or antibiotics that are part of the regular treatment regimen were not documented as exacerbations. Occurrence of exacerbation related hospitalization OR the necessity of a prescription for antibiotics qualify as worsening disease.
Time frame: 90 days
| Arm | Measure | Group | Value (COUNT_OF_PARTICIPANTS) |
|---|---|---|---|
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Number of Participants With Exacerbation of Pulmonary Disease | Exacerbation of pulmonary disease at baseline | 2 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Number of Participants With Exacerbation of Pulmonary Disease | Exacerbation that required antibiotics but not hospitalization at Day 30 | 1 Participants |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | Number of Participants With Exacerbation of Pulmonary Disease | Exacerbations that required antibiotics at Day 90 | 3 Participants |
Secondary
PCF
Standard pulmonary function measures (FEV1, FVC, and FEV1/FVC) are commonly followed in the clinical care of patients with CF. Standard pulmonary function measures (SVC, SpO2, MIP, and PCF) are commonly followed in the clinical care of patients with MND. The Peak Cough Flow (PCF) is the maximum air flow generated during a cough. Decreasing air flow generated is associated with weakening neuromuscular status and associated poor cough impulse
Time frame: 90 days
Population: FVC and FEV1/FVC ratio are pulmonary function tests appropriate to assess lung function in patients with CF. These tests could not be physically performed by the patients in the MND population and thus these patients were not included in these analysis. For the MND population, SVC, SpO2, MIP, and PCF were performed instead of FVC and FEV1/FVC ratio. Hence, no data were collected from CF patients for this outcome measure.
| Arm | Measure | Group | Value (MEAN) | Dispersion |
|---|---|---|---|---|
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | PCF | PCF after 30 days of therapy | 320.0 liters per minute | Standard Deviation 152.81 |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | PCF | PCF at baseline | 344.0 liters per minute | Standard Deviation 105.74 |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | PCF | PCF after 90 days of therapy | 280.0 liters per minute | Standard Deviation 144.91 |
Secondary
SpO2
Standard pulmonary function measures (FEV1, FVC, and FEV1/FVC) are commonly followed in the clinical care of patients with CF. Standard pulmonary function measures (SVC, SpO2, MIP, and Peak Cough Flow) are commonly followed in the clinical care of patients with MND. SpO2 stands for peripheral capillary oxygen saturation, an estimate of the amount of oxygen in the blood. Low SpO2 is associated with worsening respiratory function.
Time frame: 90 days
Population: FVC and FEV1/FVC ratio are pulmonary function tests appropriate to assess lung function in patients with CF. These tests could not be physically performed by the patients in the MND population and thus these patients were not included in these analysis. For the MND population, SVC, SpO2, MIP, and PCF were performed instead of FVC and FEV1/FVC ratio. Hence, no data were collected from CF patients for this outcome measure.
| Arm | Measure | Group | Value (MEAN) | Dispersion |
|---|---|---|---|---|
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | SpO2 | SpO2 at baseline | 97.4 Percent of oxygen saturation | Standard Deviation 0.89 |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | SpO2 | SpO2 after 30 days of therapy | 96.8 Percent of oxygen saturation | Standard Deviation 1.3 |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | SpO2 | SpO2 after 90 days of therapy | 95.8 Percent of oxygen saturation | Standard Deviation 1.64 |
Secondary
SVC (Actual)
Slow Vital Capacity (SVC) is the actual volume measured in liters. Slow Vital Capacity displays the volume of gas measured on a complete expiration after a maximal inspiration without forced or rapid effort. Useful measurement when FVC is reduced, and airway obstruction is present. Educed SVC is associated with worsening respiratory disease. Slow Vital Capacity provides the same measure as FVC - i.e. Vital Capacity. It does not, however, require a Forced expiratory maneuver (it requires a slow inspiration and slow expiration) and therefore is easier to achieve a valid result in patients with Motor Neuron Disease who suffer from progressive weakness of all muscles including the respiratory muscles.
Time frame: 90 days
Population: FVC and FEV1/FVC ratio are pulmonary function tests appropriate to assess lung function in patients with CF. These tests could not be physically performed by the patients in the MND population and thus these patients were not included in these analysis. For the MND population, SVC, SpO2, MIP, and PCF were performed instead of FVC and FEV1/FVC ratio. Hence, no data were collected from CF patients for this outcome measure.
| Arm | Measure | Group | Value (MEAN) | Dispersion |
|---|---|---|---|---|
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | SVC (Actual) | SVC at baseline (Actual) | 2.1 Liters | Standard Deviation 0.5 |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | SVC (Actual) | SVC after 30 days of therapy (Actual) | 2.2 Liters | Standard Deviation 0.66 |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | SVC (Actual) | SVC after 90 days of therapy (Actual) | 2.1 Liters | Standard Deviation 0.89 |
Secondary
SVC (% Predicted)
SVC Percent Predicted (SVC % Predicted) is the actual volume exhaled in the first 1 second, divided by the Normal value for that actual value for a person of that age, gender, height and weight. A nomogram based on population norms provides a series of Normal Values. Slow Vital Capacity displays the volume of gas measured on a complete expiration after a maximal inspiration without forced or rapid effort. Useful measurement when FVC is reduced and airway obstruction is present. Educed SVC is associated with worsening respiratory disease. Slow Vital Capacity provides the same measure as FVC - i.e. Vital Capacity. It does not, however, require a Forced expiratory maneuver (it requires a slow inspiration and slow expiration) and therefore is easier to achieve a valid result in patients with Motor Neuron Disease who suffer from progressive weakness of all muscles including the respiratory muscles.
Time frame: 90 days
Population: FVC and FEV1/FVC ratio are pulmonary function tests appropriate to assess lung function in patients with CF. These tests could not be physically performed by the patients in the MND population and thus these patients were not included in these analysis. For the MND population, SVC, SpO2, MIP, and PCF were performed instead of FVC and FEV1/FVC ratio. Hence, no data were collected from CF patients for this outcome measure.
| Arm | Measure | Group | Value (MEAN) | Dispersion |
|---|---|---|---|---|
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | SVC (% Predicted) | SVC at baseline (% Predicted) | 55.2 percentage of predicted normal | Standard Deviation 13.59 |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | SVC (% Predicted) | SVC after 30 days of therapy (% Predicted) | 58.2 percentage of predicted normal | Standard Deviation 21.1 |
| Home Based Airway Clearance With Metaneb in Patients With Cystic Fibrosis. | SVC (% Predicted) | SVC after 90 days of therapy (% Predicted) | 54.6 percentage of predicted normal | Standard Deviation 27.09 |