Beatmung und Sekretmanagement bei amyotropher Lateralsklerose^[1]

Zusammenfassung

Die Amyotrophe Lateralsklerose (ALS) ist eine neurodegenerative Systemerkrankung mit variablem, aber meist raschem Krankheitsverlauf und einer immer noch infausten Prognose, die wesentlich von der bei nahezu allen Patienten eintretenden Parese der Atemmuskulatur und ihren Folgen bestimmt wird. Angesichts begrenzter kausaler Therapiemöglichkeiten kommt einer umfassenden und qualifizierten symptomorientierten Behandlung eine zentrale Rolle zu. Symptome einer Zwerchfellschwäche, einer schlafbezogenen Hypoventilation und im Verlauf einer chronischen hyperkapnischen respiratorischen Insuffizienz haben erheblichen Einfluss auf die gesundheitsbezogene Lebensqualität und die Teilhabefähigkeit der betroffenen Patienten. Im vorliegenden Übersichtsartikel wird systematisch auf die klinische Präsentation der Atemmuskelschwäche, Methoden zur (Früh-)Diagnose sowie praktische Gesichtspunkte und bewiesene Effekte der Beatmungstherapie und Hustenassistenz eingegangen. Der diagnostische Einsatz technischer und elektrophysiologischer Methoden hat die Feststellung der Atempumpenschwäche und der schlafbezogenen Hypoventilation zum Ziel. Hierbei finden spiromanometrische Methoden zur direkten oder indirekten Messung der Atemmuskelkraft, die kardiorespiratorische Polygraphie und Polysomnografie, die transkutane Kapnometrie und die Blutgasanalyse Anwendung. Therapeutisch haben die nicht-invasive Beatmung, bei entsprechendem Patientenwunsch die invasive Beatmung über eine Trachealkanüle und das Sekretmanagement einen im Krankheitsverlauf zunehmenden Stellenwert.

Abstract

The term amyotrophic lateral sclerosis (ALS) comprises a group of motor neuron diseases which are characterized by rapid disease progression and poor prognosis which is mostly due to severe respiratory muscle weakness and its sequelae. Since causative treatment options are limited it is crucial to offer comprehensive symptomatic therapies to affect patients. Symptoms of respiratory muscle weakness, sleep-disordered breathing and, subsequently, chronic hypercapnic respiratory failure are known to severely affect health-related quality of life and social functioning of patients with ALS. This review article delineates the clinical presentation of respiratory muscle weakness, diagnostic procedures to assess diaphragmatic function, and practical aspects of both mechanical ventilation and cough assistance, respectively. Various technical and electrophysiological methods allow for detection of diaphragmatic weakness and nocturnal hypoventilation. These include spiro-manometric tests of respiratory muscle strength, cardiorespiratory polygraphy and polysomnography, transcutaneous capnography, and blood gas analysis. Once the diagnosis of respiratory muscle weakness is established, non-invasive ventilation, tracheostomy-invasive ventilation (if the patient agrees to it), and management of secretions all become increasingly important in the course of the disease.

¹ Erstveröffentlichung in Klin Neurophys 2015; 46: 163 – 172.

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