Background:- Airway clearance using an airway clearance technique (ACT) is one of the core treatments for children with chronic lung diseases such as cystic fibrosis, periciliary dyskinesia, and non-CF bronchiectasis who are unable to clear their secretions effectively. One of the most frequently used ACTs is called positive expiratory pressure (PEP) which uses a mask to produce a back pressure in the lungs getting air behind the mucus to mobilize it up the airways. Unfortunately adherence to performing an ACT is low with a reported rate between 40 - 70%. Up to the present, there has been no way to objectively measure adherence to an ACT. However, with new technology, it is now feasible to connect an electronic device to the PEP mask to objectively measure how often the child is actually performing their ACT. This proposed study would be the first study to objectively measure adherence against reported adherence.~Purpose:~To objectively measure adherence, using an electronic manometer attached to a PEP mask, against reported adherence.~Secondly, to objectively measure adherence after connecting the electronic manometer to an age appropriate video game designed to operate only if the PEP mask is used properly.~The hypothesis is that actual adherence is lower than reported adherence, and that by using a video game as a feedback mechanism while performing PEP, adherence will be improved.~Methodology:-20 subjects with either a diagnosis of cystic fibrosis or non CF bronchiectasis, between the ages of 6 - 12 years old will be recruited into this study. Subjects will be their own control, in that their reported adherence will be measured against their actual adherence. After enrollment, subjects will be provided with an electronic device which connects to the pressure port of their PEP mask. They will be told that the device attached to the PEP Mask pressure port will measure pressure. Subjects will be asked to continue using their PEP Mask as prescribed for 4 months referred to as period one. During this time the electronic device will collect data on pressure and how often and when the PEP mask is used. In addition the subject or parent will be asked to keep a log book of when and how often they did their PEP Mask.~At the end of 4 months, during a second 4 month period, the electronic manometer will be connected to software which will allow the subject to play video games operated by correct breathing through the PEP Mask. During this period actual adherence captured through the electronic device will be measured against reported adherence for this period and also to actual adherence measured during the first 4 month period.~Primary outcome is rate of adherence to prescribed therapy, measured between reported and objectively measured adherence during the first 4 month period. During the second 4 month period, rate of adherence will be compared to rate of adherence during the first 4 month period. Secondary outcome is change in FEV1 from period one to period two. It is hoped that by using fun video games while performing airway clearance, adherence will be improved.
An airway clearance technique (ACT) is one of the core treatments for children with chronic lung diseases who are unable to clear their secretions effectively. Unfortunately adherence to performing an ACT is low with a reported rate between 40 - 70%. Up to the present, there has been no way to objectively measure adherence to an ACT. With new technology, it is now feasible to connect an electronic manometer to an airway clearance device to objectively measure how often the child is actually performing their ACT. The first part of this proposed study is to objectively measure adherence against reported adherence over a 4 month period. During the second 4 months a video game will be added to the digital manometer which only operates if participants are performing their ACT properly. Adherence will again be measured.