Non-invasive ventilation and hypercapnia-associated symptoms in amyotrophic lateral sclerosis

Abstract

Objectives During the course of amyotrophic lateral sclerosis (ALS), progressive weakness of respiratory muscles leads to chronic hypercapnia which causes various symptoms like sleep disturbances, daytime fatigue, and depression. Non‐invasive ventilation (NIV) improves survival and quality of life, but little is known about its effect on these specific symptoms, in particular during the later course of disease. Our aim was to evaluate the short‐ and long‐term effects of NIV on hypercapnia‐associated symptoms in ALS. Material and Methods We prospectively analyzed sleep disturbance, daytime fatigue, and depression using standardized scales (Pittsburgh sleep quality index [PSQI], stanford sleepiness scale [SSS], Beck depression inventory [BDI], and Clinical hypoventilation score [CHS]) in 58 patients with NIV. Follow‐up was done every 3 months up to a maximum of 24 months. Results We found significant improvements of all outcome parameters except BDI within the first three months after NIV initiation. The median PSQI improved from 6.5 (95% CI: 5.0‐8.5) to 6.0 (95% CI: 4.5‐7.0; P = 0.042), the SSS from 3.0 (95% CI: 2.0‐4.0) to 2.0 (95% CI: 2.0‐3.0; P = 0.004), and the CHS from 22.0 (95% CI: 19.5‐25.0) to 18.0 points (95% CI: 12.0‐23.5; P = 0.013). Patients with bulbar and spinal onset were not significantly different, and positive effects were long‐lasting. Conclusions Our data show that NIV improves hypercapnia‐associated symptoms within the first 3 months after initiation in spinal as well as bulbar patients, and that beneficial effects are long‐lasting.

Sleep in chronic respiratory disease: COPD and hypoventilation disorders

“…Although the optimal criteria for initiation of NIV for SRH in NMDs remain uncertain, NIV improves nocturnal oxygen saturation, diurnal and nocturnal P aCO 2 , subjective sleep quality, quality of life and mortality in various NMDs [61,[116][117][118][119][120]. There are still conflicting data regarding the effect of NIV on objective sleep quality [121][122][123].…”

Predictive parameters of early respiratory decline in amyotrophic lateral sclerosis

“…During the course of the disease, patients develop progressive weakness of respiratory muscles causing a decrease of oxygen and a rise of carbon dioxide levels in the blood. Early clinical symptoms of respiratory insufficiency are variable, including sleep disturbances (difficulty falling asleep and/or staying asleep), fatigue, apathy, cognitive impairment, poor appetite, depression and morning headaches [2,3]. Rapid shallow breathing, shortness of breath on exertion, during speech or at rest, orthopnea, and the use of accessory respiratory muscles are frequently observed [4].…”

Fisioterapia Respiratoria en Pacientes Con Esclerosis Lateral Amiotrófica. Una Revisión Sistemática

“…En todos los estudios seleccionados 6,[8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23][24][25] se llevan a cabo diferentes intervenciones de fisioterapia respiratoria con el objetivo de tratar la ELA. A continuación, se exponen los resultados de los artículos en distintas tablas para facilitar su lectura y comprensión.…”

“…El tipo de estudio es muy variable: la mayor parte de los estudios son observacionales 9,[13][14][15][17][18][19][20][21]23 y, en menor cantidad, experimentales 6,8,[10][11][12]16,22,24,25 , de los cuales solos cuatro son ensayos clínicos 6,8,11,25 . Además, nos encontramos con cinco estudios retrospectivos 9,15,17,18,22 .…”

“…El tamaño muestral oscila entre 13 participantes en los artículos realizados por Vandoorne, et al 20 y Vrijsen, et al 24 , y 194, en el realizado por Vitacca, et al 17 . Las investigaciones restantes 6,[8][9][10][11][12][14][15][16]18,19,[21][22][23]25 se encuentran entre los 18 y los 80 participantes, y sobresale Elamin et al 13 , que cuenta con 137. Estos datos indican que la muestra no es exageradamente variada, por lo que los resultados son perfectamente comparables.…”