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Patterns of Non-Invasive Ventilation in Amyotrophic Lateral Sclerosis

  • Nevena Markovic (a1), Marcus Povitz (a2), Joanne Smith (a3), David Leasa (a4), Christen Shoesmith (a1) and Teneille E. Gofton (a5)...

Abstract

Background: Non-invasive ventilation (NIV) improves quality of life and survival in patients with amyotrophic lateral sclerosis (ALS) and respiratory symptoms. Little is known about the patterns of NIV use over time and the impact of NIV on end-of-life decision-making in ALS. Objective: This study assessed the pattern of NIV use over the course of the disease and the timing of end-of-life discussions in people living with ALS. Method: A retrospective single-center cohort study was performed at London Health Sciences Centre. Daily NIV duration of use was evaluated at 3-month intervals. The timing of diagnosis, NIV initiation, discussions relating to do-not-attempt-resuscitation (DNAR) and death were examined. Results: In total, 48 patients were included in the analysis. Duration of NIV use increased over time, and tolerance to NIV was observed to be better than expected in patients with bulbar-onset ALS. There was a high degree of variability in the timing of end-of-life discussions in patients with ALS (356±451 days from diagnosis). In this cohort, there was a strong association between the timing of discussions regarding code status and establishment of a DNAR order (r2=0.93). Conclusion: This retrospective cohort study suggests that the use of NIV in ALS increases over time and that there remains a great deal of variability in the timing of end-of-life discussions in people living with ALS. Future prospective studies exploring the use NIV over the disease trajectory and how NIV affects end-of-life decision-making in people with ALS are needed.

Caractéristiques de l’utilisation de la ventilation non-invasive dans des cas de sclérose latérale amyotrophique. Contexte: La ventilation non-invasive (VNI) améliore la qualité de vie et la durée de vie de patients atteints de sclérose latérale amyotrophique (SLA) ainsi que les symptômes respiratoires qui lui sont associés. On connaît toutefois peu de choses quant aux caractéristiques de l’utilisation de la VNI au fil du temps et à son impact en ce qui regarde les décisions prises en fin de vie lorsqu’il est question de la SLA. Objectifs: Cette étude a cherché à évaluer les caractéristiques d’utilisation de la VNI au fur et à mesure de la progression de la SLA et le moment choisi pour aborder la question de la fin de vie dans les cas de patients atteints de cette maladie. Méthodes: Une étude de cohorte rétrospective a été menée dans un seul établissement de santé, à savoir le Centre des sciences de la santé de London. Le temps d’utilisation de la VNI a été évalué tous les trois mois. D’autres aspects ont également été examinés: le moment où un diagnostic de SLA a été posé ; où l’on a commencé à utiliser la VNI ; et où des discussions portant sur la non-réanimation et l’éventualité d’un décès ont été initiées. Résultats: Au total, 48 patients ont été inclus dans le cadre de notre analyse. Si le temps d’utilisation de la VNI a augmenté au fil du temps, on a aussi remarqué, chez des patients atteints dès le début de la forme bulbaire de la SLA, une tolérance meilleure que ce qui était escompté. Un fort degré de variabilité a été noté quant au moment choisi pour initier des discussions portant sur la fin de vie (356 +/- 451 jours à partir d’un diagnostic). Dans cette cohorte, on a pu établir une forte association entre le moment choisi pour initier des discussions portant sur les interventions en cas d’arrêt cardiaque ou respiratoire et l’inscription d’une ordonnance de non-réanimation (r2 = 0,93). Conclusions: Cette étude de cohorte rétrospective suggère donc que l’utilisation de la VNI dans le cas de la SLA augmente au fil du temps. Elle suggère également qu’il subsiste une variabilité considérable en ce qui a trait aux discussions portant sur la fin de vie de patients atteints de SLA. D’autres études prospectives demeurent néanmoins nécessaires. Elles devront en effet se pencher sur l’utilisation de la VNI au cours de la trajectoire de la maladie et sur ses impacts quant aux décisions prises en fin de vie.

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Copyright

Corresponding author

Correspondence to: Teneille E. Gofton, Department of Clinical Neurological Sciences and Critical Care Western, Western University, University Hospital, 339 Windermere Rd, London, ON, Canada N6A 5A5. Email: teneille.gofton@lhsc.on.ca

References

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