Beatmung und Sekretmanagement bei amyotropher Lateralsklerose

Zusammenfassung

Die Symptome und Folgen der muskulären Ateminsuffizienz tragen bei der amyotrophen Lateralsklerose maßgeblich zur Morbidität und Mortalität der Erkrankung bei und beeinträchtigen nachhaltig die Lebensqualität. Während initial Symptome und Zeichen der schlafbezogenen Atmungsstörung im Vordergrund stehen, kommt es im Verlauf zu chronischem hyperkapnischem Atemversagen und zu einer klinisch hoch relevanten Hustenschwäche mit Sekretverhalt, fehlendem Schutz vor Aspiration und erhöhtem Infektrisiko. Die frühzeitige Erkennung und adäquate Therapie atmungsassoziierter Symptome ist für die Lebensqualität und die Prognose der betroffenen Patienten entscheidend. Dieser Übersichtsartikel fasst den gegenwärtigen Stand der diagnostischen und therapeutischen Möglichkeiten zusammen und gibt praxisrelevante Empfehlungen zum Management der in- und exspiratorischen Atemmuskelschwäche.

Abstract

In most patients with ALS, progressive respiratory muscle weakness is a major aspect of morbidity and mortality. It inevitably evolves and substantially reduces quality of life. Initially, respiratory muscle weakness presents as sleep-disordered breathing but progresses to chronic hypercapnic respiratory failure. Diaphragmatic dysfunction also causes significant weakness of cough resulting in mucus retention, increased risk of aspiration, and recurrent pulmonary infections. Early detection and adequate treatment of respiratory muscle weakness is of utmost importance for both quality of life and overall prognosis in ALS patients. This review article summarizes current diagnostic and therapeutic options and gives practical recommendations for the management of inspiratory and expiratory muscle weakness in ALS.

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