From the Guidelines
Patients with muscular dystrophy should undergo baseline swallowing assessments when diagnosed and then periodic evaluations based on symptoms and disease progression, typically every 1-2 years or more frequently if dysphagia symptoms develop, as recommended by the American Journal of Respiratory and Critical Care Medicine 1. The rationale for monitoring swallowing function is that muscular dystrophy often affects the oropharyngeal muscles, leading to progressive dysphagia, which increases the risk of aspiration pneumonia, malnutrition, and dehydration. Early detection of swallowing difficulties allows for timely interventions such as dietary modifications, swallowing techniques, and in advanced cases, consideration of alternative feeding methods. These assessments may include videofluoroscopic swallow studies (VFSS) or fiberoptic endoscopic evaluation of swallowing (FEES), as suggested by the International Journal of Stroke 1 and Stroke 1. The frequency of swallow studies should be individualized based on the specific type of muscular dystrophy, rate of disease progression, and presence of symptoms such as coughing during meals, frequent respiratory infections, or unexplained weight loss. It is essential to note that while there is limited evidence specifically supporting regular swallow studies in patients with muscular dystrophy, the available guidelines and recommendations from reputable sources emphasize the importance of swallowing assessments in patients with conditions that affect the oropharyngeal muscles, such as stroke 1. Therefore, it is reasonable to apply these principles to patients with muscular dystrophy, prioritizing their morbidity, mortality, and quality of life. Key considerations include:
- Baseline swallowing assessments at diagnosis
- Periodic evaluations based on symptoms and disease progression
- Individualized frequency of swallow studies
- Use of VFSS or FEES for assessments
- Timely interventions for swallowing difficulties, such as dietary modifications and alternative feeding methods.
From the Research
Swallowing Dysfunction in Muscular Dystrophy
- Swallowing dysfunction is a common symptom in patients with muscular dystrophy, with studies indicating that it can lead to serious complications such as aspiration pneumonia 2, 3.
- Research has shown that dysphagia can worsen the condition of ageing patients with muscular dystrophy, and that it is often a consequence of impairment in the pharyngeal phase of swallowing 3.
- A study on Duchenne muscular dystrophy found that combining the modified water swallowing test (MWST) and flexible endoscopic evaluation of swallowing (FEES) is useful for evaluating bedside swallowing function and predicting the onset of pneumonia 2.
Diagnostic Tools and Techniques
- Videofluoroscopy has been used to investigate swallowing physiology and function in individuals with oculopharyngeal muscular dystrophy (OPMD), revealing significant differences in pharyngeal constriction, hyoid movement, and airway closure 4.
- A study on myotonic muscular dystrophy used videofluoroscopy to compare swallow function in patients with that of healthy controls, finding that patients with myotonic dystrophy had longer bolus transit times and delayed onsets of swallow gestures 5.
- Radiographic findings in patients with myotonic dystrophy have shown reduced pharyngeal peristalsis, hypopharyngeal stasis, and fragmented swallowing, with aspiration seen in half of the patients 6.
Management and Recommendations
- A clinical algorithm has been proposed to facilitate the diagnosis of dysphagia, identify symptoms, and provide practical recommendations for treating dysphagia in adult patients with Duchenne muscular dystrophy 3.
- Recommendations for managing dysphagia in patients with muscular dystrophy include prohibiting solid food and promoting intake of fluids, as well as considering tracheostomy if cough augmentation techniques are ineffective 3.
- Strategies to facilitate pharyngeal clearing, such as swallow facilitation strategies, have been found to be worthwhile in patients with myotonic muscular dystrophy 5.