Adults and children with neuromuscular disorders — including those with spinal muscular atrophy (SMA) — are living longer, according to a 12-year population-based study in Ontario, Canada.
The recent drop in mortality is likely due to the latest advances in the management of respiratory complications, newer technologies for lung hygiene and night ventilation along with more prompt access to specialized services.
In addition, even though the prevalence of neuromuscular disorders (NMDs) as a whole is rising, in adults and children with SMA the proportion of subjects with the disease tends to remain stable.
These findings come from the study “Trends in incidence, prevalence, and mortality of neuromuscular disease in Ontario, Canada: A population-based retrospective cohort study (2003-2014),” published recently in the journal PLOS ONE.
Understanding the incidence and impact of NMDs in the population is important to evaluate the burden those disorders represent to patients, their families and the healthcare system. It is useful to learn more about the natural history of these diseases, which are highly variable, as well as the effects of new management approaches. It also can be a valuable to source to inform healthcare policy.
Canadian researchers conducted a population-based estimate of NMDs’ trends regarding incidence, prevalence and mortality over time. To calculate those trends, they used the public health administrative database of the Canadian province of Ontario, spanning the period from 2003 to 2014.
A study of this type provides a comprehensive picture of the natural history of the disease at a population level, exceeding other study designs more prone to bias and variability (e.g., chart reviews, surveys, family histories).
The database used covered all residents of Ontario, a population of approximately 13 million during the study period.
The study primarily used data from individuals who were identified as having one NMD during a hospital stay, or a visit to the emergency department.
NMDs reported included amyotrophic lateral sclerosis (ALS)/motor neurone disease, cerebral palsy, Guillain-Barré syndrome, metabolic disorders, multiple sclerosis (MS), muscular dystrophy (MD), myasthenia gravis (MG), neuromuscular disorders (non-specific diagnoses), neuropathy, post-polio syndrome, spina bifida and spinal muscular atrophy (SMA).
The team also noted that even though a reasonable proportion (61.8%) of adults and children received palliative care before death, further improvements in delivery and uptake of palliative care should be made.
In Canada, most palliative care services are provided at the hospital, which results in unequal access to these care units across the country. Other barriers to palliative care include poor family awareness, cost, age-appropriateness of services, and people’s perceptions about this type of care.