Benefits of Biphasic Cuirass Ventilation for SMA

Biphasic Cuirass Ventilation (BCV) can be used effectively to ventilate a patient with hypercapnia and acute or chronic ventilatory failure.  The patient’s work of breathing, the use of accessory muscles and tachypnea will decrease when BCV is implemented.   Alveolar recruitment will improve gas exchange and facilitate the management of secretions.   BCV has shown to have similar gas exchange benefits as active proning when used on infants and small children.  As noted in the January 2020 Respiratory Care Journal, “Negative pressure ventilation successfully supported 69% of pediatric subjects with acute respiratory failure with a complication rate of < 2%.”

 

Understanding Spinal Muscular Atrophy (SMA)

Spinal muscular atrophy (SMA) is a group of genetic diseases that cause weakness and wasting in the voluntary muscles of infants and children and, more rarely, in adults. It is a one of the most common genetic conditions affecting children. It is estimated that one in every 6,000 to 10,000 babies worldwide is born with SMA.  The types of spinal muscular atrophy (SMA) are:

 

• Type 1 (severe) SMA: This type is also called Werdnig-Hoffmann Disease. It is the most severe and the most common type of SMA. It is usually evident at birth, or in the first few months afterwards (0-6 months). Symptoms include floppy limbs and weak trunk movement. Children with this type usually have extremely limited ability to move. They will also have a hard time feeding and swallowing, holding their head up, and breathing. Type 1 SMA progresses rapidly, with the weakening of muscles leading to frequent respiratory infections and usually death by the age of 2. Infants with SMA type 1 can never sit.

 

• Type 2 (intermediate) SMA: Symptoms usually appear between the ages of 7 to 18 months. The rate of progression can vary greatly. The disease affects the child's legs more than his or her arms. Children with SMA type 2 can never stand. Respiratory infections are also common with this type of SMA. Life expectancy can range from early childhood to adulthood, depending on the severity of the patient's condition.

 

• Type 3 (mild) SMA: This type of SMA is also called Kugelberg-Welander or Juvenile Spinal Muscular Atrophy. Symptoms can first appear during a wide range of years, from 18 months to early adulthood. Patients with Type 3 SMA can stand and walk but may have trouble getting up from sitting position. They may also experience mild muscle weakness and are at greater risk for respiratory infections. Most patients with Type 3 SMA have a life expectancy close to normal.

 

• Type 4 (adult) SMA: Symptoms for this rare type of SMA do not usually emerge until the second or third decade of life. Patients with Type 4 SMA can walk during adulthood but will usually experience slowly progressive muscle weakness and other typical SMA symptoms.

 

Symptoms of Spinal muscular atrophy (SMA):

  • Limited mobility
  • Decreased muscle tone
  • Decreased mobility
  • Breathing problems
  • Problems eating and swallowing
  • Delayed gross motor skills
  • Spontaneous tongue movements
  • Scoliosis (curvature of the spine)

 

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. 

 

 

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