Applicable Conditions
Conditions with Breathing Muscle Weakness that can benefit from
Noninvasive Ventilatory Support and Mechanical Insufflation-Exsufflation
People with the following conditions are conventionally treated with low-span bi-level positive airway pressure (PAP) and uncomfortable airway suctioning, which are inadequate to fully support the respiratory muscles. As their condition/symptoms worsen, they require invasive airway tubes (e.g. tracheostomy tubes). However, if you can understand and cooperate with basic instruction, and you have breathing or coughing muscle weakness (i.e. if you are too weak to breathe or cough effectively), then you do not need a tracheostomy tube. In fact, even people intubated for acute respiratory failure fall under this criteria.
Diagnostic categories:
Outcomes for amyotrophic lateral sclerosis (ALS)
Outcomes for chronic lung disease and debilitated elderly in critical care
Outcomes for Duchenne muscular dystrophy (DMD)
Outcomes for type 1 neuromuscular disorders (e.g. SMA type 1)
Outcomes for other neuromuscular disorders (e.g. SMA types 2 and 3)
Outcomes for obesity hypoventilation syndrome
Outcomes for post-poliomyelitis syndrome
Outcomes for spinal cord injury/myelopathies
Myopathies (Muscle Disorders)
Muscular dystrophies:
Duchenne
Becker
Limb-girdle
Emery-Dreifuss
Facioscapulohumeral
Oculopharyngeal
Myotonic
Distal
Congenital
Fukuyama
Integrin-deficient
Merosin-deficient
Ullrich
Walker-Warburg Syndrome
Other myopathies:
Steroid myopathy
Carnitine-deficient
Myotubular
Arthrogryposis
Bethlem
Central core disease
Centronuclear/Titinopathy
Myotonia congenital
Paramyotonia congenita
Mucopolysaccharidoses
Acid maltase deficiency (Pompe disease)
Morquio
Cori disease (debrancher enzyme deficiency)
Finnish (tibial) distal myopathy
Inclusion-body myositis
ZASP-related myofibrillar
Nemaline rod
Andersen-Tawil syndrome
Mitochondrial myopathies
Pearson’s
Kearns-Sayre syndrome
Leigh syndrome
Progressive external ophthalmoplegia
MERRF
Fiber-type disproportion
Metabolic myopathies with respiratory dysfunction:
Lactate dehydrogenase deficiency
McArdle disease (phosphorylase mutase deficiency)
Myoadenylate deaminase deficiency
Periodic paralysis
Phosphofructokinase deficiency
Phosphoglycerate kinase deficiency
Acquired myopathies of systemic disease:
Carcinomatous myopathy
Cachexia/anorexia nervosa
Medication associated (e.g. glucocorticoid)
Inflammatory myopathies
Polymyositis
Hypothyroid
Hyperthyroid
Diseases of the myoneural junction:
Myasthenia gravis
Congenital myasthenic syndromes
Lambert-Eaton myasthenic syndrome
Motor Neuron, Spinal Cord, and Peripheral Nerve Disorders
Motor neuron diseases:
Amyotrophic lateral sclerosis (ALS)
Spinal muscular atrophy (SMA)
Kennedy disease
Poliomyelitis
Spinal cord injury (SCI)
Myelopathies of rheumatoid, infectious, spondylitic, vascular, traumatic, or idiopathic origin
Tetraplegia associated with toxic metals, pancuronium bromide, or botulism
Neuropathies:
Hereditary sensory motor neuropathies
Familial hypertrophic interstitial polyneuropathy
Charcot-Marie-Tooth disease
Chronic inflammatory demyelinating polyneuropathy
Guillain-Barré syndrome
Phrenic neuropathies associated with cardiac hypothermia, surgical, or other trauma (including radiation, phrenic electrostimulation, familial, paraneoplastic or infectious), associated with lupus erythematosus or other connective tissue diseases, or idiopathic
Central Nervous System Diseases
Multiple sclerosis (MS)
Parkinson’s disease
Disorders of supraspinal tone
Friedreich's ataxia
Cerebral palsy
Static encephalopathies including those associated with Arnold Chiari malformation, syringomyelia, myelomeningocele, and encephalitis
Other Medical Conditions
Intensive care deconditioning and neuromyopathy (i.e. healthy elderly people weakened by long stays in intensive care)
Chronic obstructive pulmonary disease (COPD) or other lung disease with severe respiratory muscle dysfunction
Obesity hypoventilation syndrome
Central alveolar hypoventilation including Ondine's curse
Kyphoscoliosis
Anyone with weak muscles at risk for respiratory complications of surgery
