Sleep Disorders in Spinal Muscular Atrophy

Sleep Disorders in Spinal Muscular Atrophy

by | Jan 7, 2019 | Nursing Resource Page Two, Nursing Resource Pages, Nutritionist Resource Page Two, Nutritionist Resource Pages, Orthopedics Resource Page Two, Orthopedics Resource Pages, Pediatricians Resource Page Two, Pediatricians Resources

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 

by | Dec 20, 2018 | Nursing Resource Page Two, Nursing Resource Pages, Pediatricians Resource Page Two, Pediatricians Resources, Pulmonologist Resource Page Two, Pulmonologist Resource Pages

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 

by | Dec 19, 2018 | Nursing Resource Page One, Nursing Resource Pages, Pediatricians Resource Page Two, Pediatricians Resources, Physical Therapists Resource Page One, Physical Therapists Resource Pages, Pulmonologist Resource Page Three, Pulmonologist Resource Pages

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

by | Nov 28, 2018 | Neurologists Resource Page Three, Neurologists Resource Pages, Nursing Resource Page Three, Nursing Resource Pages, Pediatricians Resource Page Two, Pediatricians Resources, Radiology Resource Page Three, Radiology Resources

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

by | Nov 27, 2018 | Nursing Resource Page Two, Nursing Resource Pages, Pediatricians Resource Page Two, Pediatricians Resources, Pulmonologist Resource Page One, Pulmonologist Resource Pages

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

by | Oct 30, 2018 | Nursing Resource Page Two, Nursing Resource Pages, Orthopedics Resource Page Two, Orthopedics Resource Pages, Pediatricians Resource Page Two, Pediatricians Resources, Physical Therapists Resource Page Two, Physical Therapists Resource Pages

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...