Pulmonary and Sleep Management in Myotonic Dystrophy
Damien Stevens MD
Associate Professor of Medicine
Division of Pulmonary Critical Care and Sleep
Medical Director KU Sleep Laboratory
KUMC Myotonic Dystrophy Day
Pulmonary Treatments
July 20, 2019
Respiratory and Sleep Issues in Myotonic
Dystrophy
Pulmonary complications leading cause of death in DM1
◦
Ineffective cough
◦
Pneumonia
◦
Chronic respiratory failure
◦
Acute respiratory failure
◦
Sleep disordered breathing
◦
Excessive daytime sleepiness
Respiratory Issues in Myotonic Dystrophy
Ineffective cough
Normal peak expiratory flowrate is >270 liters/minute
Maximal inspiratory pressure is <60 cm H2O
FVC values of 50% less than predicted
Vaccinate for pneumonia and influenza
Treat respiratory infections quickly
Use cough assistance as needed
Noninvasive ventilatory assistance as needed
Respiratory Issues in Myotonic Dystrophy
Some patients will eventually require nighttime ventilator support or continuous
ventilation.
Most patients with chronic respiratory insufficiency respond to noninvasive
ventilatory support (NIV).
Use supplemental oxygen with caution and in conjunction with NIV
Patients experiencing acute respiratory failure require endotracheal intubation
Preoperative evaluation and clearance if need for anesthesia.
Sleep Issues in Myotonic Dystrophy
Assess with Epworth Sleepiness Scale or a similar instrument
Sleep study if sleep disturbance is suspected
Nocturnal oximetry possible useful
Possible obstructive sleep apnea or central sleep apnea
Sleep study often indicated depending upon symptoms
Excessive daytime sleepiness not explained by other process
Sleep Issues in Myotonic Dystrophy
Nocturnal or daytime hypoventilation also can be seen
Noninvasive positive pressure ventilation may be beneficial
Pressure cycled versus volume cycled assistance
Consider referral to a pulmonologist
Sleep Issues in Myotonic Dystrophy
Excessive daytime sleepiness can be difficult to treat
Typically corelates with severity of other organ disease
Wake promoting agents typically first line option
Stimulants can be effective
Myotonic dystrophy presents challenges in respiratory and sleep function. The condition can lead to pulmonary complications, such as ineffective cough, pneumonia, and respiratory failure, making proactive management crucial. Treatment strategies include vaccinating against infections, utilizing ventilatory support, and addressing sleep disturbances. Regular assessment and intervention are essential to improve quality of life and prevent complications in individuals with Myotonic Dystrophy.
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Presentation Transcript
KUMC Myotonic Dystrophy Day Pulmonary Treatments July 20, 2019 Damien Stevens MD Associate Professor of Medicine Division of Pulmonary Critical Care and Sleep Medical Director KU Sleep Laboratory
Respiratory and Sleep Issues in Myotonic Dystrophy Pulmonary complications leading cause of death in DM1 Ineffective cough Pneumonia Chronic respiratory failure Acute respiratory failure Sleep disordered breathing Excessive daytime sleepiness
Respiratory Issues in Myotonic Dystrophy Ineffective cough Normal peak expiratory flowrate is >270 liters/minute Maximal inspiratory pressure is <60 cm H2O FVC values of 50% less than predicted Vaccinate for pneumonia and influenza Treat respiratory infections quickly Use cough assistance as needed Noninvasive ventilatory assistance as needed
Respiratory Issues in Myotonic Dystrophy Some patients will eventually require nighttime ventilator support or continuous ventilation. Most patients with chronic respiratory insufficiency respond to noninvasive ventilatory support (NIV). Use supplemental oxygen with caution and in conjunction with NIV Patients experiencing acute respiratory failure require endotracheal intubation Preoperative evaluation and clearance if need for anesthesia.
Sleep Issues in Myotonic Dystrophy Assess with Epworth Sleepiness Scale or a similar instrument Sleep study if sleep disturbance is suspected Nocturnal oximetry possible useful Possible obstructive sleep apnea or central sleep apnea Sleep study often indicated depending upon symptoms Excessive daytime sleepiness not explained by other process
Sleep Issues in Myotonic Dystrophy Nocturnal or daytime hypoventilation also can be seen Noninvasive positive pressure ventilation may be beneficial Pressure cycled versus volume cycled assistance Consider referral to a pulmonologist
Sleep Issues in Myotonic Dystrophy Excessive daytime sleepiness can be difficult to treat Typically corelates with severity of other organ disease Wake promoting agents typically first line option Stimulants can be effective
