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 ID: 928999
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Damien Stevens MD Associate Professor of MedicineDivision of Pulmonary Critical Care and SleepMedical Director KU Sleep Laboratory
KUMC Myotonic Dystrophy Day
Pulmonary Treatments
July 20, 2019
Slide2Respiratory 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
Slide3Slide4Respiratory 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
Slide5Slide6Slide7Respiratory 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.
Slide8Sleep Issues in Myotonic Dystrophy
Assess with Epworth Sleepiness Scale or a similar instrumentSleep study if sleep disturbance is suspectedNocturnal oximetry possible usefulPossible obstructive sleep apnea or central sleep apneaSleep study often indicated depending upon symptoms
Excessive daytime sleepiness not explained by other process
Slide9Sleep Issues in Myotonic Dystrophy
Nocturnal or daytime hypoventilation also can be seen Noninvasive positive pressure ventilation may be beneficialPressure cycled versus volume cycled assistanceConsider referral to a pulmonologist
Slide10Sleep Issues in Myotonic Dystrophy
Excessive daytime sleepiness can be difficult to treatTypically corelates with severity of other organ disease
Wake promoting agents typically first line option
Stimulants can be effective