MN4000 for Treatment of CF and MND Patients in the Home Setting

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.

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