ORIGINAL ARTICLE   

Minerva Pneumologica 2018 September;57(3):57-63

DOI: 10.23736/S0026-4954.18.01820-5

Copyright © 2018 EDIZIONI MINERVA MEDICA

language: English

Respiratory management and non-invasive ventilation in patients with amyotrophic lateral sclerosis

Tina BREGANT ¹ ✉, Lea LEONARDIS ²

¹ University Rehabilitation Institute of Republic Slovenia - URI Soca, Ljubljana, Slovenia; ² Division of Neurology, Institute of Clinical Neurophysiology, University Clinical Center Ljubljana, Ljubljana, Slovenia

BACKGROUND: Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease which affects motor neurons in the brain and the spinal cord. Respiratory system complications and abnormalities are common; non-invasive ventilation (NIV) has proven to be helpful to cope with the increasing hypoventilation due to weakness of ventilatory muscles.
METHODS: We analyzed the data of our patients with ALS that used non-invasive bilevel positive airway pressure (BiPAP) ventilation; ALSFRS-R was calculated for each visit.
RESULTS: Data from 27 patients with ALS, 11 females and 16 males, 7 with predominant bulbar and 20 with predominant spinal form, mean age at diagnosis 64.3±9.0 years and at death 67.0±8.8 years, who used NIV for the median of 9 months (median EPAP 4, IQR 0 and median IPAP 14, IQR 6 cm H20) was analyzed. The median survival was 25.0 months, IQR 22.5, with the trend of spinal form patients to live longer (P=0.13), by approx. 10 months. Mean and SD of the functional outcome ALSFRS-R at diagnosis was 33.3±8.6; at NIV introduction 25.0±8.5 and at the last visit before death 18.6±8.6. ALSFRS-R showed statistically significant/borderline significant differences between the spinal and the bulbar form groups at diagnosis (P=0.02) and NIV introduction (P=0.08). Blood gas analysis (BGA) remained stable.
CONCLUSIONS: Non-invasive ventilation (NIV) is well accepted by some patients with ALS shown by stable BGA despite ALSFRS-R Score deterioration. Ventilatory support can be optimized by reading of the ventilator data from the SD card.

KEY WORDS: Noninvasive ventilation - Amyotrophic lateral sclerosis - Blood gas analysis