BCV for SMA

BCV for SMA

Children with SMA type 1 have a weakness of the intercostal muscles (the muscles between the ribs) that help expand the chest, and the chest is often smaller than usual.

The strongest breathing muscle in an SMA patient is the diaphragm. As a result, the patient appears to breath with their stomach muscles. The chest may appear concave (sunken in) due to the diaphragmatic (tummy) breathing. Also, due to this type of breathing, the lungs may not fully develop, the cough is very weak, and it may be difficult to take deep enough breaths while sleeping to maintain normal oxygen and carbon dioxide levels.

Children with Type II also have weak intercostals muscles and are diaphragmatic breathers. They have difficulty coughing and may have difficulty taking deep enough breaths while they sleep to maintain normal oxygen levels and carbon dioxide levels. Scoliosis is almost uniformly present as these children grow, resulting in need for spinal surgery or bracing at some point in their clinical course.

The goal of BCV in SMA patients is to offload the work of breathing full time, nocturnally and/or at naps to prevent hypoventilation that subsequently leads to more pathological changes such as hypercapnia, hypoxemia, atelectasis and V/Q mismatch to name just a few.

Secretion Clearance is often indicated for secretion management and assisted coughing in some patients. The use of BCV will improve quality of life and prevent reoccurring admissions to health care facilities.

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