Understanding Amyotrophic Lateral Sclerosis (ALS) / Motor Neuron Disease (MND)

Amyotrophic lateral sclerosis (ALS), also known as motor neuron diease (MND) is a progressive nervous system disease that affects nerve cells in the brain and spinal cord, causing loss of muscle control.  ALS / MND is often called Lou Gehrig's disease, after the baseball player who was diagnosed with it.  ALS / MND often begins with muscle twitching and weakness in a limb, or slurred speech.  Eventually, ALS / MND affects control of the muscles needed to move, speak, eat, and breathe.  ALS / MND causes the death of neurons controlling voluntary muscles. ALS / MND often starts in the hands, feet, or limbs, and then spreads to other parts of your body. As the disease advances and nerve cells are destroyed, your muscles get weaker. This eventually affects chewing, swallowing, speaking, and breathing.  There is generally no pain in the early stages of ALS / MND, and pain is uncommon in the later stages.  ALS / MND does not usually affect your bladder control or your senses. 

Symptoms of Amyotrophic Lateral Sclerosis (ALS) / Motor Neuron Disease (MND):

  • Muscle twitches
  • Slurred speech
  • Muscle weakness
  • Difficulty walking or doing normal daily activities
  • Tripping and falling
  • Weakness in your leg, feet, or ankles
  • Hand weakness or clumsiness
  • Slurred speech or trouble swallowing
  • Muscle cramps and twitching in your arms, shoulders, and tongue
  • Inappropriate crying, laughing, or yawning
  • Cognitive and behavioral changes

 

ALS

Features of Biphasic Cuirass Ventilation: 

  1. Continuous Negative Extra Thoracic Pressure - By means of the negative pressure generated within the shell, the chest wall is held open, creating pathways for mucus to exit the lungs and recruitment of the alveoli to promote better gas exchange. 
  2. Control – Biphasic ventilation utilizes an active expiratory phase, which will then allow the ventilator to have full control of the patient’s breathing. This mode mimics our natural physiology and facilitates the use of our pulmonary musculature during inspiration and expiration to manage carbon dioxide retention without the cardiovascular repercussions associated with positive pressure ventilation. 
  3. Respiratory Synchronized – Synchronized utilizes biphasic ventilation as well, however, the patient can control their rate and inspiratory time. Once they initiate a breath, the ventilator is triggered and is then able to provide the pressure support needed to expel carbon dioxide from the lungs. 
  4. Secretion Clearance – Chest Physiotherapy that incorporates high frequency chest wall oscillation (HFCWO) to break up the thick secretions and cough assist to mobilize those secretions to the upper airways to be coughed out. Used as a scheduled therapy throughout the day to assist with keeping the lungs clear of secretions. 

 

Benefits of Biphasic Cuirass Ventilation

Biphasic Cuirass Ventilation (BCV) can be initiated early after the diagnosis of ALS/MND.  Studies have shown that early intervention may slow down the progression for the need of invasive mechanical ventilation and tracheostomy.  This will minimize the unnecessary side effects of prolonged use of positive pressure ventilation and decrease hospital readmissions, time spent in the ICU, and in the hospital.

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

benefitted these Amyotrophic Lateral Sclerosis (ALS) patients!

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