*Validation of the ExSpiron© in patients with Amyotrophic Lateral Sclerosis*; a pilot study

Chronic non-invasive ventilation (NIV) started during the polio epidemic in
Scandinavia and the United States. Negative pressure ventilators such as the
Iron Lung, were the most commonly used mechanical ventilators throughout the
1950*s. After control of the Polio epidemic, invasive positive pressure
ventilation via endotracheal tube or tracheostomy was more predominate over
non-invasive ventilation. Nowadays this is the opposite, more than 80% of all
patients on home mechanical ventilation (HMV) in the Netherlands are treated
with NIV[1].
Types of diseases that have been successfully treated with chronic non-invasive
ventilation include, neuromuscular diseases, chest wall deformity, chronic
obstructive pulmonary disease and sleep related problems. Patients with these
types of diseases may develop chronic derangement of daytime gas exchange
yielding daytime hypoxia and hypercapnia and chronic respiratory insufficiency
is present. Patients with chronic respiratory insufficiency fail to achieve
adequate ventilation and gas exchange, especially during sleep. Correction of
such ventilatory and gas exchange abnormalities using non-invasive ventilation
(NIV) is an increasingly popular method for improving sleep quality of life and
overall survival in certain patient populations, including Amyotrophic lateral
sclerosis (ALS).
ALS is a progressive neuromuscular disease and most patients develop complaints
of dyspnea, fatigue, unrefreshing sleep and morning headache in the advanced
stage of their diseases. NIV is commonly regarded to be a treatment that is
effective in reducing these complaints. Several studies presented data
regarding the effects of NIV on quality of life in patients with ALS [2][3][4].
Some were positive, while others produced more reservations. A review done by
Piepers et al, concluded that studies on the use of NIV in patients with ALS
differ considerably in design and endpoint definitions[5]. In daily practice it
is difficult to determine what the best moment is to start NIV in ALS patients.
There are objective and subjective symptoms and by judging them a clinical
decision can be made. By using the ExSpiron© monitor an extra objective
measurement can be added to inform patients and caregivers about the
respiratory condition. This also helps to make the right decision on the right
time, to start NIV, in patients with the progressive disease ALS.

Primary Objective:
To validate the ExSpiron©, a device for non-invasive monitoring of respiratory
volume in patients with ALS during spontaneous breathing and during NIV.

Secondary Objective(s):
• to observe the difference between nocturnal respiration as compared to
respiration during daytime
• Borg score
• ALSAQ-40
• Gas exchange during the night with the transcutaneous monitor SenTec©.
• Respiratory muscle activity and patient-ventilator asynchrony during the
night and during NIV assessed with surface electromyography (EMG)

At the outpatient clinic the pulmonologist recruits participants and the
informed consent is signed a week after this visit if participants are willing
to take part in the study. The pilot starts at the first day of admission of
the participants on the Respiratory Care Unit (RCU) of the UMC Groningen. At
daytime, TV and MV will be measured during spontaneous breathing with the
ExSpiron© simultaneously with a pneumotachometer (gold-standard). After
installation of the chest pad of the ExSpiron© and the pneumotachometer, the
first measurements are in sitting position (bed or (wheel)chair). Three
complete cycles of ten breaths; normal breathing, slow-deep breathing and
rapid-shallow breathing, need to be completed. Between each cycle, a pause is
allowed. Patients may hold the pneumotachometer themselves, if unable to do so,
the researcher will hold it for them. After completing this breathing sequence,
it is repeated in supine position. If patients are not able to perform this in
supine position, an angle of 45 degree is sufficient. After completing these
cycles, a measurement of 30 minutes during spontaneous breathing will be
conducted in supine and prone position without the pneumotachometer. After this
measurement, the chest pad should be left in place and the EMG equipment should
be installed. During the first night of admission, a full night of measurement
of TV and MV will be conducted using the ExSpiron©. The SenTec© will be used
for measuring oxygen saturation and carbon dioxide. Respiratory muscle activity
will be recorded using the EMG equipment. Sleep quality is recorded by a
polysomnography. The nurse on the ward will be instructed to start all
measurements. NIV will be started as in regular care and terminated by the
nurse after 4 hours or earlier when the patients doesn*t or cannot proceed
further with NIV. The ExSpiron©, SenTec©, polysomnography and EMG stay in
position, measurements are continued during spontaneous breathing.

To validate the ExSpiron©, a device for non-invasive monitoring of respiratory
volume in patients with ALS during spontaneous breathing and during NIV.

not applicable.