neuromuscular disorders
IMPACT ON RESPIRATORY HEALTH
Respiratory health is crucial for children and adults with neuromuscular disorders. These disorders progressively weaken muscles, often including the respiratory muscles like the diaphragm and abdominal muscles. Severely weakened respiratory muscles most commonly occurs in patients with amyotrophic lateral sclerosis (ALS), spinal muscle atrophy (SMA) type 1, muscular dystrophy (MD) and other neuromuscular disorders.
Neuromuscular conditions can cause breathing problems in several ways, directly and indirectly, because of related complications. Reduced lung function and hypoventilation can develop with muscle weakness problems. Weakness of the diaphragm and other breathing muscles leads to restricted lung function. If these muscles don’t work well, individuals cannot take as deep a breath as usual. Muscle weakness and a weak cough can make it difficult to get mucus out of the lungs leading to airway mucus plugging and chest congestion.
The human body uses a variety of reflexes and voluntary actions to keep the airway clear, primarily through coughing. When the muscles get too weak to perform effective coughing, mucus can accumulate in the airways, partially blocking it and impairing breathing. The reduced ability to cough leads to a buildup of mucus, recurring infections and potential respiratory issues.
TREATMENT & MANAGEMENT
Secretion mobilization techniques are intended to mobilize the mucus in the peripheral airways so they can be removed more easily. An advantage of secretion mobilization methods is that they don’t require the patient’s cooperation and thus can be used on infants, young children, and patients who are incapable of participating in the therapy. One secretion mobilization technique is chest physical therapy (CPT). CPT utilizes postural drainage and percussion (clapping on the chest and/or back) to loosen thick, sticky mucus in the lungs so it can be removed by coughing or suctioning. CPT is generally thought of as the gold standard, but it can be difficult to perform consistently over the course of individual treatment.
High-frequency chest wall oscillation (HFCWO) is an airway clearance technique in which external chest wall oscillations are applied to the chest using a vest that wraps around the chest. HFCWO vests produce vibrations at variable frequencies and intensities, helping to loosen and thin mucus and separate it from airway walls. The frequency and intensity for this type of airway clearance needs to be customized to each patient and will need to be re-evaluated regularly throughout the patient’s disease course. HFCWO airway clearance therapy is a cornerstone technique in managing respiratory disorders associated with neuromuscular diseases such as MD, multiple sclerosis (MS), ALS, and quadriplegia.
Respiratory health is crucial for children and adults with neuromuscular disorders. These disorders progressively weaken muscles, often including the respiratory muscles like the diaphragm and abdominal muscles. Severely weakened respiratory muscles most commonly occurs in patients with amyotrophic lateral sclerosis (ALS), spinal muscle atrophy (SMA) type 1, muscular dystrophy (MD) and other neuromuscular disorders.
Neuromuscular conditions can cause breathing problems in several ways, directly and indirectly, because of related complications. Reduced lung function and hypoventilation can develop with muscle weakness problems. Weakness of the diaphragm and other breathing muscles leads to restricted lung function. If these muscles don’t work well, individuals cannot take as deep a breath as usual. Muscle weakness and a weak cough can make it difficult to get mucus out of the lungs leading to airway mucus plugging and chest congestion.
The human body uses a variety of reflexes and voluntary actions to keep the airway clear, primarily through coughing. When the muscles get too weak to perform effective coughing, mucus can accumulate in the airways, partially blocking it and impairing breathing. The reduced ability to cough leads to a buildup of mucus, recurring infections and potential respiratory issues.
BENEFITS OF AIRWAY CLEARANCe
Cough is an effective method of clearing secretions from the larger airways in healthy individuals. However, respiratory muscle impairment leads to difficulty with coughing and clearing secretions, subsequent mucous plugging and pulmonary infection.5 With narrow airways, coughing can have detrimental effects if used inappropriately over an extended period as the primary method of clearing secretions.5
Effective mucus clearance is essential for lung health, and airway disease is a consistent consequence of poor clearance.6 Airway clearance techniques (ACTs) to assist with secretion clearance are widely recommended and can include both mucus-mobilizing techniques and assisted cough techniques.6 Studies recommend that ACTs be used as the primary method of mobilizing secretions from the middle and small airways to the larger airways. Then, effective coughing can be used to clear secretions from the larger airways, thereby preserving the integrity of the larger airways.6