Neuromuscular and rare diseases

The main causes of respiratory impairment are hypoventilation due to weak inspiratory muscles and a lack of ability to cough due to weak expiratory muscles. Ventilatory support via non-invasive mechanical ventilation or tracheotomy can prevent or reverse ventilatory failure in these patients.

The loss of expiratory strength means that patients are unable to expel bronchial secretions. If the bulbar muscles are also affected and patients run the risk of inhaling saliva, the contents of the mouth or food, this can induce multiple respiratory infections, pneumonia and atelectasis which results in obstruction of the airway and seriously endangers the patient's life.

The combination of non-invasive mechanical ventilation to assist coughing decreases morbidity and hospital admissions for these patients.

Symptoms

• Respiratory and ventilatory failure
• Unable to expel secretions and ineffective cough
• Dysphagia and dysarthria due to impairment of the bulbar muscles  
• Impairment of peripheral muscles limits function and basic daily activities

 There are currently around 60,000 people with the condition in Spain.

Diagnosis

In the Cardiorespiratory Rehabilitation Unit, we monitor maximal inspiratory and expiratory pressure (MIP and MEP) and peak expiratory flow (PEF), also known as peak cough flow (PCF) and carry out spirometry.

Treatment 

Treatment goals are focused on controlling the evolution of the ventilatory failure and avoiding or improving episodes of respiratory failure. To achieve these objectives, manual techniques or equipment have to be used. These are techniques to encourage pulmonary expansion, manually assist coughing, and others.

Prevention 

One very important objective is to train the main carer in physiotherapy techniques in order to avoid possible complications in the respiratory system.