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In the early stages, shortly after the diagnosis of ALS is made, it may seem to the physician and other caregivers that there is no need for discussion of the effects of respiratory muscle weakness. However, even in this early stage, it is helpful to encourage the patient and family to think about details and learn about neuromuscular function. Respiratory function is a good place for the educational process to start, because everybody has some knowledge about this area and some concerns about the dangers of aspiration, particularly if some swallowing and breathing problems have already been experienced.
Respiratory care requires more than neurologists, and it is extremely helpful if an interested physician/pulmonologist can be identified to help coordinate respiratory care for a patient and family. This care usually involves an educational period about general strategies, aspiration precaution, and information about respiratory assistive devices, especially bimodal passive airway pressure (BIPAP).
Mechanisms of respiratory muscle failure in neuromuscular disease.
There are multiple causes of respiratory muscle failure in ALS that act in synergy to create a slowly progressive decline in pulmonary function. Respiratory muscle weakness and diaphragmatic fatigue cause atelectasis and reduced lung compliance. Aspiration due to bulbar dysfunction also contributes to reduced lung compliance and can lead to pneumonia. Increased lung compliance increases the work of breathing and produces shortness of breath. Malnutrition contributes to respiratory muscle weakness and diaphragmatic fatigue. [1-3]
Clinical signs of respiratory problems.
There is general appreciation of the fact that frank complaints of dyspnea, shorter spoken phrases, increased respiratory rate, and use of accessory muscles of respiration, along with weak cough and sniff, are all important signs of declining respiratory muscle function. Symptoms of carbon dioxide retention can be insidious in onset and consist of early morning headache, excessive daytime somnolence, and …
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