Background: Initiation to Non-Invasive Ventilation (NIV) in amyotrophic lateral sclerosis
(ALS) can be implemented in an inpatient or outpatient setting.

Aims: We aimed to evaluate the efficacy of adaptation (the number of needed sessions) to home-based NIV compared to an outpatient one in ALS in terms of arterial carbon dioxide (PaCO2) improvement. NIV acceptance (mean use of >=5 h NIV per night for three consecutive nights during the adaptation trial), adherence (night-time NIV usage for >=150 h/month), quality of life (QoL), and caregiver burden were secondary outcomes.

Methods: A total of 66 ALS patients with indications for NIV were involved in this randomized
controlled trial (RCT): 34 underwent NIV initiation at home (home adaptation, HA) and 32 at multiple outpatient visits (outpatient adaptation, OA). Respiratory function tests were performed at baseline (the time of starting the NIV, T0) together with blood gas analysis, which was repeated at the end of adaptation (T1) and 2 (T2) and 6 (T3) months after T1. NIV adherence was measured at T2 and T3. Overnight cardiorespiratory polygraphy, Short Form Health Survey (SF-36), Caregiver Burden Inventory (CBI), Caregiver Burden Scale (CBS), and Zarit Burden Interview (ZBI) were performed at T0, T2, and T3. Results: Fifty-eight participants completed the study. No differences were found between groups in PaCO2 at T1 (p = 0.46), T2 (p = 0.50), and T3 (p = 0.34) in acceptance (p = 0.55) and adherence to NIV at T2 and T3 (p = 0.60 and p = 0.75, respectively). At T2, the patients’ QoL, assessed with SF-36, was significantly better in HA than in OA (p = 0.01), but this improvement was not maintained until T3 (p = 0.17).

Conclusions: In ALS, adaptation to NIV in the patient’s home is as effective as that performed in an outpatient setting regarding PaCO2, acceptance, and adherence, which emphasizes the need for further studies to understand the role of the environment concerning NIV adherence.

This study assessed the feasibility of implementing a hybrid hospital–provider company
(PC) clinical pathway for patients with chronic respiratory failure (CRF) through the adaptation and follow-up of non-invasive ventilation (NIV). Over a 3-month period, a PC physiotherapist case manager oversaw the adaptation process, making adjustments as necessary, using remote monitoring and home visits. Outcome measures, including the number of patients enrolled, serious adverse events, hospitalizations, survival rates, professional time allocation, NIV adherence, nocturnal apnea–hypopnea, and oxygen saturation, Δ arterial carbon dioxide pressure (PaCO2), dyspnea, Short Physical Performance Battery (SPPB), exercise tolerance, quality of life, physical activity, and patient satisfaction, were collected. The recruitment rate was 74% (nineteen patients). Commonly reported
adverse events included leakage, discomfort and sleep disturbance. Predominant interventions were four home visits (3; 4) and two NIV adjustments (1; 5). The overall program time commitment averaged 43.97 h per patient (being hospital 40 ± 11% and PC 60 ± 11%). Improvements in PaCO2, dyspnea, SPPB and exercise tolerance were observed by the third month. Adherence to NIV was high, with good or very good satisfaction with its use. This study demonstrates that a hybrid hospital–PC service for NIV adaptation and follow-up is not only feasible but also shows validity, reliability, and acceptability.

Home mechanical ventilation (HMV) in the Netherlands is normally initiated in
hospital, but this is expensive and often a burden for the patient. In this randomised controlled study we investigated whether initiation of HMV at home in patients with chronic respiratory failure is non-inferior to an in hospital based setting.
Methods: Seventy-seven patients were included, of which 38 patients started HMV at home. All patients were diagnosed with chronic respiratory failure due to a neuromuscular or thoracic cage disease. Primary outcome was the arterial carbon dioxide (PaCO2) while quality of life and costs were secondary outcomes. Telemonitoring was used in the home group to provide therapeutic information, for example; transcutaneous carbon dioxide, oxygen saturation and ventilator information, to the caregivers. Follow-up was six months.
Results: PaCO2, improved by 0.72 kPa in the hospital group and by 0.91 in
the home group, both improvements being significant and the latter clearly not inferior.
There were also significant improvements in quality of life in both groups, again not being
inferior with home treatment.