Spinal Muscular Atrophy: Scoliosis

Spinal Muscular Atrophy: Scoliosis

Scoliosis is an early orthopedic complication of Spinal Muscular Atrophy (SMA).1 The estimated incidence of scoliosis in SMA types 1 and 2 ranges from 60-90%2 and is approximately 50% in SMA type 3.3 The etiology of scoliosis in SMA is weakness throughout the muscles...
Spinal Muscular Atrophy: Maximizing Quality of Life

Spinal Muscular Atrophy: Maximizing Quality of Life

Children with Spinal Muscular Atrophy (SMA) have complex medical needs which can lead to diminished quality of life.1 Historically, patients diagnosed with SMA and their families report significant stress and a low quality of life.2-4 It is incorrect to assume that...
Signs and Symptoms

Signs and Symptoms

One of the pathological hallmarks of spinal muscular atrophy (SMA) is the degeneration of anterior horn cells in the spinal cord and motor nuclei in the lower brainstem.1 Alpha motor neurons are preferentially affected, so the disease is limited to the motor system...
Prognosis

Prognosis

The disease course and prognosis of spinal muscular atrophy (SMA) depends heavily on the clinical classification in a particular patient. For example, the prognosis of children with SMA type 0 is very poor while the prognosis in SMA type 4 patients is relatively good....
Feeding, Swallowing, and GI Issues – Sitters

Feeding, Swallowing, and GI Issues – Sitters

Children with spinal muscular atrophy (SMA) who are able to sit (i.e., “sitters”) often encounter substantial difficulties with chewing, choking, coughing, and food aspiration. While these issues are less severe in sitters than they are in non-sitters,...