Quality of life and compliance to long term autotitrating non-invasive ventilation in Latvian children

Background: The use of non-invasive ventilation (NIV) increases exponentially worldwide with varying degrees of success due to compliance. Aim: To evaluate adherence to long term NIV (optimally >4h for 70% nights), using pediatric sleep questionnaire (PSQ) and EQ-5D-Y as tools in assessing quality of life (QoL) in our centre. Methods: Data of PSQ, EQ-5D-Y and adherence (Dec 2017 – Oct 2018; NIV use for > 3 months) downloaded from ventilators were used. Results: Currently, 11 children (6 males; 2 months - 15 years 2 months (median 7 years 6 months)) use domiciliary autotitrating NIV (8 CPAP, 3 BiPAP). Sleep study was performed in 8 cases (7 polygraphies, 1 polysomnography). The diagnostic categories include infants with laryngomalacia (n=2), unspecified interstitial lung disease (n=1), mixed sleep apnea (n=1), genetic diseases (n=7) (Duchenne muscular dystrophy, Crouzon disease, Beckwith-Wiedemann syndrome, ROHHAD syndrome, Down syndrome, Prader-Willi syndrome (n=2)). The median percentage of daily use was 88,8 (65.6-98.1)%, with median usage 8,1 (4-9,6) hours/night. Percentage of daily use was related to teenage (used less) and decreased due to complications (skin lesion, n=2). According to PSQ, EQ-5D-Y patients felt improvement in QoL and in weight loss. Conclusions: The data showed fairly high adherence in terms of percentage of daily use. Domiciliary NIV service in Latvia is quite recent (since Nov 2017) and limitation, undoubtedly, is the small patient number.