Limb Girdle Muscular Dystrophy
- Cardiomyopathy and/or dysrhythmias are common in LGMD.
- Symptoms of nocturnal hypoventilation may signal the development of significant respiratory muscle weakness and the need for intervention. Noninvasive ventilation (NIV) may be required. If supplemental oxygen is required during a respiratory crisis, this must be carefully controlled and carbon dioxide levels monitored, especially in the context of chronic respiratory failure.
- Assisted coughing with chest physiotherapy and breath-stacking techniques with an AMBU bag help to clear lower airways secretions. This can also be facilitated by a cough assist device. These interventions should be performed only by trained and experienced persons.
- Immunisations should be kept up-to-date, including the flu and pneumococcal vaccines.
Medication and anaesthetic precautions
- It is essential that the anaesthetist is aware of the diagnosis of LGMD to allow appropriate pre-operative assessment and post-operative monitoring.
- LGMD patients may experience increased sensitivity to sedatives, inhaled anaesthetics and neuromuscular blockade.
- Local anaesthetics and nitrous oxide are safe (e.g. for minor dental procedures).
Fractures and falls
- If the patient is ambulant before fracture, internal fixation is preferable to casting as it helps to preserve muscle and speeds a return to walking.
- It is advised to check vitamin D levels and bone mineral density on a regular basis, especially following a fall or fracture.
Recommendations and precautions
- Swallowing difficulties are rarely reported in LGMD patients, however if present, they should be assessed by a speech and language therapist (SALT).
- Liver enzymes (AST/ALT/alkaline phosphatase) may be mildly raised on blood tests in up to 50 percent of patients. The clinical setting dictates whether further investigation is indicated.