Amyotrophic lateral sclerosis (ALS) 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.
ALS 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 ALS 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 ALS, most voluntary muscles are paralyzed.
With ALS, 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 ALS is noninvasive ventilation (NIV).
Adopting Biphasic Cuirass Ventilation, or better known as BCV, improves the quality of life for those diagnosed with ALS. 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 ALS improving the patient’s tidal volume, decrease in respiratory rate, improved comfort and tolerance.”
Nearly all patients with ALS 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 ALS respiratory-related symptoms.