Neuromuscular disease and the use of home mechanical ventilation

What is Neuromuscular Disease?

Although rare, neuromuscular diseases can arise at any stage of life, acquired or hereditary. Because it is often progressive, it affects other points in the peripheral nervous system. As a result, diseases such as: Amyotrophic Lateral Sclerosis, myopathies, radiculopathy, peripheral neuropathies, among others, can arise. It is a disease that significantly affects the quality of life of patients.

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 depending on the involvement of the motor unit, and may progress to the inability to breathe spontaneously, being necessary to receive treatment with mechanical ventilation at home.

Amyotrophic Lateral Sclerosis – ALS

ALS  is one of the  most well-known neuromuscular diseases, see below the reason and the main numbers:

  • 200,000 people worldwide are diagnosed with ALS;
  • Between 12 and 15 thousand Brazilians are diagnosed with ALS;
  • Most patients are men, between 40 and 60 years old;
  • In Brazil, the average time to receive the correct diagnosis is 10 to 12 months;
  • The median survival of these patients is 36 months. 

  • How is the treatment done?

    In the initial phase of neuromuscular disease, the patient may have nocturnal respiratory symptoms, with desaturation during sleep, inability to reach the REM phase, nocturnal awakenings, fatigue and daytime sleepiness. At this stage, it will be necessary to use non-invasive ventilatory support (CPAP or BIPAP) during the night, associated with face or nasal masks according to the patient's comfort.

Cough Assistant

Loss of respiratory muscle strength and hypoventilation lead to an inability to remove secretions from the airways. In this case, bronchial hygiene can be performed using equipment for assisted coughing, capable of efficiently and safely reproducing the mechanics of coughing. Indicated from mild to advanced stages of the disease.

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High-tech equipment that guarantees more quality of life.

Home Mechanical Fans

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 time that the use of life support equipment capable of correcting changes in compliance and resistance is suggested. pulmonary function such as mechanical home ventilators.

Indicated to work 24 hours a day, the current models are light and portable, with an internal battery, and can also be used for travel and tours, adapting to a wheelchair and carrying bag.

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Safety in treatment

In case of doubts about the use or installation of the products, call 01000033373.

  • Keep the home mechanical ventilator on a flat and stable base, if its pedestal is not used, to prevent the risk of falling.
  • Do not leave any ventilator accessories, such as the exhalation circuit and valve, on the seat/bed or other absorbent materials when not in use.
  • Do not smoke in the environment where the lung ventilator and/or medical gas cylinder is installed.
  • Do not use oils, fats or any substance to lubricate valves, regulators and connections.
  • The home mechanical ventilator must not be exposed to direct sunlight.
  • The fan cannot be covered by any material so as not to have its cooling inlet obstructed.
  • Do not keep liquids close to the fan and its accessories, in order to avoid the risk of electric shock.
  • Handling of equipment should only be performed by a qualified healthcare professional. Ventilatory parameters cannot be changed without the formal agreement of the person responsible for the prescription.
  • Alarms cannot be changed or turned off without prior knowledge of the physician responsible for the patient. If you have any questions regarding alarms, contact VitalAire's Home Care and Call Center on 01000033373.
  • Air Liquide prohibits the customer/user from performing maintenance and repairs on the equipment.
  • The home mechanical ventilator must be used exclusively under the responsibility of a physician and parameterized according to the prescription .
  • The electrical installation must support the electrical power indicated on the equipment (see user manual).
  • Use only external batteries or UPS supplied by Air Liquide. Only fan and heated base must be connected to the UPS. It is prohibited to connect any other electronic equipment.
  • It is recommended for safety reasons to avoid connecting the fan and your UPS to an electrical outlet.
  • The fan, even when not in use, must always be connected to the electrical network to prevent the internal battery from being discharged. If using a UPS, the fan must remain connected to it, and likewise, the UPS must be connected to the mains.
  • UPS – Check daily that the UPS is charged and plugged into an electrical power point (other than a power strip/extension strip) that is working properly.