What is Neuromuscular Disease?

Although rare, neuromuscular diseases can arise at any stage of life, acquired or hereditary.

Neuromuscular diseases lead to the use of mechanical ventilation at home

There is no specific neuromuscular disease to receive this type of treatment. In general, they are diseases that progress with total or partial inability to perform breathing movement.

Neuromuscular diseases encompass a group of more than 200 pathologies that lead to generalized muscle weakness involving upper and/or lower limbs, oropharyngeal and breathing muscles, causing difficulties in swallowing, speaking and breathing. They have different names according to the involvement of the motor centre of your brain unit.

Amyotrophic Lateral Sclerosis – ALS

ALS is one of the most well-known neuromuscular diseases also known as Motor Neurone Disease. It's a progressive neurological condition in which the nerves that control voluntary muscles stop working. 

Spinal muscular atrophy - SMA

SMA is a rare neuromuscular disorder that results in the loss of motor neurons and progressive muscle wasting. It is usually diagnosed in infancy or early childhood and if left untreated it is the most common genetic cause of infant death.

  • Treatment Phases

    In the initial phase, the patient may present nocturnal respiratory symptoms, with desaturation during sleep, inability to reach the REM phase, nocturnal awakenings, fatigue and daytime sleepiness. At this stage, it may be necessary to use non-invasive ventilatory support (CPAP or BiPAP)  during the night, with a face or nasal mask. 

Secretion management (cough assist)

The loss of respiratory muscle strength in addition to hypoventilation also leads to the inability to remove secretions from the airways. In this case, bronchial hygiene can be performed using a cough assist machine which is a device that helps simulate a cough in order to clear secretions from the lungs. Indicated from mild to advanced stages of the disease.

Home Ventilators

With the progression of the disease and respiratory muscle weakness, there will be an increase in dependence on ventilatory support (>16 hours/day) and it is at this point that the use of life support equipment is suggested: mechanical home ventilators. These are capable of correcting changes in compliance and pulmonary resistance and are designed to work 24 hours a day.
Current models are light, portable, have an internal battery, can also be used for travel, school and other daily activities as they have a carry bag and/or can be attached to a wheelchair.