Initiation of home mechanical ventilation (HMV) around the age of 20 appears to slow respiratory deterioration in patients with Duchenne muscular dystrophy (DMD), according to a recently published study in ERS Publications.
DMD and respiratory insufficiency
When a breath is taken, muscles (such as the diaphragm) contract to expand the chest and create a pressure differential that pulls air into the lungs. In patients with DMD over the age of 20, these muscles have typically weakened, making difficulty breathing a common complication in young adults with the disease.
Respiratory insufficiency in DMD usually starts with slowed breathing (hypoventilation) during sleep. As those with DMD grow older, this progresses to include trouble breathing during the day, as well.
Non-invasive mechanical ventilation as a solution
Since the early 2000s, non-invasive mechanical ventilation (such as bilevel positive airway pressure, or BIPAP, machines) has been used to treat nocturnal hypoventilation in DMD and is associated with improved quality of life and increased survival.
This therapeutic technique involves a machine connected to the patient’s airway through a face mask. The machine pushes air into the lungs, replacing the pressure differential that would normally be generated by the respiratory muscles.
Slowing lung function decline through home ventilation
Several studies have supported the use of HMV over clinical ventilation, as it is associated with better quality of life and reduced caregiver burden.
The authors aimed to determine whether HMV was associated with a slower decline in ventilatory function. “In this retrospective longitudinal observational study in DMD patients, lung function and respiratory muscle strength at the time of starting HMV and their change under HMV were investigated,” the study’s authors wrote.
To this end, they analyzed data from 80 patients with DMD who began home ventilation, including both non-invasive ventilation and and invasive ventilation (ventilation using a tracheostomy tube). The median age of the study population was 20.
The results showed that lung function parameters, such as forced vital capacity (the maximum amount of air exhaled after a deep breath) and maximum inspiratory pressure (a measure of breathing muscle strength), were maintained throughout the study period.
“Initiation of HMV slows the decline in lung function,” the authors concluded.
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