Sleep Disorders in Spinal Muscular Atrophy

Sleep Disorders in Spinal Muscular Atrophy

Those with spinal muscular atrophy (SMA) often suffer from sleep disorders, but the specific reasons for sleep disturbances vary. For instance, some studies on SMA report sleep disturbances resulting from breathing disorders like sleep apneas, whereas others report...
Respiratory Care in Spinal Muscular Atrophy: Intubation and Extubation 

Respiratory Care in Spinal Muscular Atrophy: Intubation and Extubation 

Intubation in spinal muscular atrophy (SMA) can be difficult or impossible,1 but the need for endotracheal intubation in SMA patients is increasing.2 In SMA type 1, respiratory failure can require frequent intubation in children ages three and below.3 As life...
Assistive Devices and Equipment: Oxygenation and Breathing 

Assistive Devices and Equipment: Oxygenation and Breathing 

Spinal muscular atrophy (SMA) is associated with serious respiratory system complications,1 and pulmonary disease is the primary cause of morbidity and mortality in both SMA type 1 and SMA type 2 patients.2 Given the frequency and extent of oxygenation and breathing...
Alternate Nusinersen Administration Strategies

Alternate Nusinersen Administration Strategies

In the pivotal clinical trials that support FDA approval, nusinersen was administered via intrathecal injection. However, this approach requires that patients receive multiple intrathecal injections separated in time by weeks. During each infusion, the clinician must...
Respiratory Care in Spinal Muscular Atrophy: Airway Clearance

Respiratory Care in Spinal Muscular Atrophy: Airway Clearance

Weakness in the respiratory system is a common and serious complication of spinal muscular atrophy (SMA),1 and pulmonary disease is the primary cause of morbidity and mortality in SMA type 1 and SMA type 2 patients.2 Muscle atrophy and hypotonia are directly related...
Spinal Muscular Atrophy: Contractures

Spinal Muscular Atrophy: Contractures

Patients with progressive neuromuscular diseases like Spinal Muscular Atrophy (SMA) have extremity weakness that can predispose to contractures, defined as “a lack of full passive range of motion due to joint, muscle, or soft tissue limitations.”1 The etiology of...