Background: Many patients with neuromuscular diseases suffer from a weak cough. Due to infection or aspiration a life-threatening situation may occur. There are different options for the therapists to improve secretion clearance from the airways in the patient with a weak cough. Furthermore, there are indications that consequent practice of techniques to ameliorate bronchial clearance may also improve the prognosis of patients with neuromuscular diseases.
Management: The management of secretions in neuromuscular disorders does not consist of single actions but is rather a complex programme. Diagnostics and several therapeutic measures have to be performed intensely and regularly. The diagnosis of a weak cough flow is based on anamnestic data, e. g., increase in secretions or dysphagia, physical examination, e. g., paradoxical breathing, and easily measured lung function parameters like vital capacity and peak cough flow (PCF). The diagnosis of an accumulation of secretions in the airways can be made easily by means of a pulse oximetry: while breathing room air the oxygen saturation in the case of a healthy lung and clean airways will be better than 95 %. A decline can, among others, be induced by amounts of secretions in the airways. The consequence should be measures to improve secretion expectoration (so-called oximetry-feedback protocol). To assist in secretion elimination from the airways several means are available like air stacking, manually assisted cough and mechanical assisted coughing--e. g., mechanical insufflator-exsufflator. Which of these techniques should be used depends on the extent of the disease: with preserved facial and bulbar muscles, air stacking alone or in combination with manually assisted coughing may be adequate and effective in the home care of the patient. In case of failure of these means, e. g., in bulbar paralysis, there is the possibility to apply mechanically assisted coughing by means of the mechanical insufflator-exsufflator. In case of tracheostomy, air stacking or mechanical assisted coughing has to be combined with tracheal suctioning. Acute infections of the lower airways are a special challenge: personnel intensive application of a combination of different secretion eliminating techniques, e. g., bronchoscopy in the hospital, manually assisted coughing and mechanically assisted coughing have to be performed in high intensity to avoid intubation.
Conclusion: The early diagnosis of a weak cough in NMD patients is important for the timely start of existing and effective measures for improving the capacity of elimination of secretions--air stacking, manually assisted cough and mechanically assisted cough. Although there is no high degree of evidence, we believe that morbidity and possibly mortality can be affected in a positive manner.