PT  - JOURNAL ARTICLE
AU  - Teresa Salerno
AU  - Martino Pavone
AU  - Maria Beatrice Chiarini Testa
AU  - Francesco Paolo Rossi
AU  - Elisabetta Verrillo
AU  - Serena Soldini
AU  - Renato Cutrera
TI  - Adaptation of children with spinal muscolar atrophy type 1 and 2 to non invasive ventilatory support
DP  - 2011 Sep 01
TA  - European Respiratory Journal
PG  - p2091
VI  - 38
IP  - Suppl 55
4099  - http://erj.ersjournals.com/content/38/Suppl_55/p2091.short
4100  - http://erj.ersjournals.com/content/38/Suppl_55/p2091.full
SO  - Eur Respir J2011 Sep 01; 38
AB  - Spinal Muscolar Atrophy (SMA), the most common fatal inherited disease in infants. The impaired respiratory function is the main cause of the high mortality. Non Invasive Ventilation (NIV) has reduced morbidity and mortality due to respiratory insufficiency and has a favourable impact on respiratory infectious complications.Aim: Evaluation of the necessary time and mode to achieve adaptation to this tool in SMA patients (pts) free from acute respiratory insufficiency.We prospectively studied 28 consecutive SMA type 1 pts (11 pts) and 2 (17 pts), who were enrolled in a standardized clinical protocol of adaptation to NIV. Mean age was 19±23 months, M/F 10/18. NIV was delivered via a nasal mask in 23 pts, nasal progs in 4 pts, full face mask in one, with Pressure Support Ventilation (PSV) mode (25 pts) and Assisted Controlled Pressure Ventilation (ACPV) mode (3 pts). Mean IPAP was 12±2.7 cm H2O, mean EPAP 3.9±0.74 cm H2O.Success was defined as the necessary time to accustom the SMA pts free from acute respiratory insufficiency to NIV and the mode to achieve acceptability of this tool.According to this definition, all pts (100%) were considered as successfully ventilated, as all of them tolerated NIV with a mean adaptation time of 8±2 days. No major complications were observed.In conclusion, our study demonstrates that NIV is well tolerated in SMA children and, probably, the high rate of success was obtained thanks to a standardized protocol of NIV initiation/administration, the proper parents education, a strict follow-up, the lack of major complications. With these assumptions, success can be expected in a high rate of pts before the onset of respiratory insufficiency.