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Evaluation of Respiratory Muscle Activation Using Respiratory Motor Control Assessment (RMCA) in Individuals with Chronic Spinal Cord Injury

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[Amyotrophic lateral sclerosis: mechanical ventilation--or not right?].

M J Kampelmacher¹, E J Westermann, L H van den Berg

¹Centrum voor Thuisbeademing, Universitair Medisch Centrum Utrecht, Postbus 85.500, 3508 GA Utrecht. m.j.kampelmacher@digd.azu.nl

Nederlands Tijdschrift Voor Geneeskunde

|April 2, 2004

Summary

Early discussion of home mechanical ventilation for amyotrophic lateral sclerosis (ALS) patients is crucial. Advance directives ensure patient wishes are respected regarding ventilation, feeding tubes, and supportive care.

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Area of Science:

Neurology
Pulmonology
Critical Care Medicine

Background:

Amyotrophic lateral sclerosis (ALS) progressively impacts respiratory function, necessitating advanced respiratory support.
Management of respiratory failure in ALS requires careful consideration of patient preferences and available interventions.

Observation:

Tracheostomy ventilation was initiated in two ALS patients due to acute respiratory failure, one lacking advance directives.
A third ALS patient with chronic respiratory failure faced challenges with percutaneous endoscopic gastrostomy (PEG) placement, impacting non-invasive positive pressure ventilation (NIPPV) effectiveness.
A fourth ALS patient successfully initiated elective NIPPV after timely referral and PEG placement.

Findings:

The timing of discussing domiciliary ventilation significantly influences patient management and outcomes in ALS.
Lack of advance directives can lead to interventions not aligned with patient wishes.
PEG placement is critical for effective NIPPV in ALS patients with swallowing difficulties.

Implications:

Early and comprehensive discussions about domiciliary ventilation are essential for ALS patients.
Advance directives are vital for guiding decisions on invasive vs. non-invasive ventilation, PEG placement, and palliative care options (oxygen, morphine).
Proactive planning ensures patient autonomy and optimizes quality of life during advanced ALS progression.