BCV for Motor Neuron Disease (MND)

 Motor Neuron Disease (MND) is a fatal neurological disorder, which affects nerve cells in the spinal cord (lower motor neurons) and brain (upper motor neurons), progressively degenerating them further. When the motor neurons can no longer send signals to the muscles, the muscles begin to deteriorate (atrophy), progressing muscle weakness.

MND causes muscles to be weak and soft, or stiff, tight and spastic. Cramps and muscle twitches are common. Symptoms may be limited to a single body region, or mild symptoms may affect more areas of the body. When MND begins in the bulbar motor neurons, the muscles used for swallowing and speaking are affected first.

As the disease develops, symptoms become more widespread. Some muscles become paralyzed, while others are weakened or unaffected. In late-stage MND, most voluntary muscles are paralyzed.

With MND, a number of symptoms can occur directly related to respiratory failure, including overall weakness; shortness of breath, both on exertion and at rest; sleepiness and fatigue; and hypoventilation, particularly at night. Thus, one of the most widely used interventions for MND is noninvasive ventilation (NIV).

Biphasic Cuirass Ventilation (BCV)

Adopting Biphasic Cuirass Ventilation, or better known as BCV, improves the quality of life for those diagnosed with MND. BCV is non-invasive. BCV does NOT require a tracheostomy, or a mask. With BCV, capable patients can eat, drink, and talk while being fully ventilated. BCV can provide ventilation, secretion clearance, and a cough.

Early adoption is key! According to a recent case report, “BCV was initiated upon diagnosis of [MND] improving the patient’s tidal volume, decrease in respiratory rate, improved comfort and tolerance.”

Nearly all patients with MND will eventually lose most muscle function, including vocal communication abilities, cough and breathing. They will have to choose to stop eating or invite pneumonia as their swallowing muscles will lose the ability to direct food into the esophagus. As a result, food particles in whole or in part may go down the trachea with bulbar onset. Initially, the patient will cough and will clear the aspirate from their lungs with struggle. As time passes and these aspiration events reoccur, the reflexes that protect the airway from aspiration may elicit a weakened cough response if any; also known as silent aspiration. As the aspiration increases, cases of pneumonia are inevitable as oral aspirate is infectious in the lungs. It can occur in spite of the patient forgoing oral intake as saliva is aspirated. The rapidity of progression may be decelerated, stopped or reversed hopefully in time with pharmaceutical and gene therapeutic approaches, but for the exception of a rare few, the progression is inevitable. Pneumonia resulting in severe respiratory failure or sepsis is frequently the ultimate cause of mortality. BCV offers multiple clinical advantages over other non-invasive therapeutic interventions for symptomatic or prophylactic treatment of MND respiratory-related symptoms.

BCV can help manage the symptoms of Motor Neuron Disease (MND) by assisting in the following six key symptom areas, which are also common in other motor neuron illnesses: 

• • Respiratory Muscle Function
    • BCV use is often therapeutic. With the benefits of negative pressure lung inflation, BCV can fight the progression of respiratory symptoms related to pulmonary muscle fatigue with resulting loss of functional residual capacity and secretion retention
    • BCV can be started early in symptom progression, before the patient’s condition deteriorates and hospitalization is required

• • CO2 Retention/Hypoventilation
    • BCV’s ability to control both phases of the respiratory cycle allows for the generation of good tidal volumes. BCV can be adjusted to very accurately meet each patient’s individual carbon-dioxide clearance needs

• • Decreased Chest Wall Mobility
    • During BCV’s inhalation phase, air is pulled into the lungs in a natural way that causes descent of the diaphragm and expands the ribs naturally to maintains the normal range of motion of the chest wall
    • Assisted Cough modality provides a natural inspiration which is deeper than a patient can produce on his or her own strength. It also maximizes this effect while clearing retained secretions
    • Cycling the chest wall and the muscles of respiration through its full volume range is thought to have a possible strengthening effect on muscles of respiration

• • Decreased Cough Flow / Inability to Clear Pulmonary Secretions
    • The first phase of BCV’s 2-phase modality called “Secretion Clearance” uses the only true High Frequency Chest Wall Oscillation available to truly oscillate the chest wall and lungs, which produces a thinning effect on pulmonary secretions and facilitates mobilization of secretions, to beneficially clear them from the lungs
    • The second part of Secretion Clearance - cough assistance mode - ends each secretion clearance oscillation cycle by providing a deep breath and a strong cough flow bringing secretions up and out of the airways

• • General Sense of Fatigue
    • Many patients report a higher general level of energy and improved endurance for activities of daily living with the capacities they have after starting BCV
    • The active expiratory phase of BCV can assist patients whose vocal volume is waning to be able to speak a more normal volumes and with less fatigue.

• • Facial Injury Due to Prolonged Use of Mask Ventilation
    • BCV uses a non-facial interface and can be used in place or in conjunction with mask ventilation
    • BCV can be used alternating with mask for patients who want to continue with mask but are having problems with facial pressure injuries from the mask allowing injuries a chance to heal

Watch the videos below to learn how Biphasic Cuirass Ventilation (BCV)

benefitted these Motor Neuron Disease (MND)/Amyotrophic Lateral Sclerosis (ALS) patients!

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