Aspiration events tied to higher risk of respiratory complications
Rett patients should be monitored for risk, researchers say
An aspiration event— when something other than air enters the lungs — increases the risk of lower respiratory tract infection and respiratory failure in people with Rett syndrome, a study found.
Patients with known or suspected aspiration events more commonly had risk factors like epilepsy and difficulty swallowing, and were more likely to be hospitalized.
“Providers should monitor aspiration and institute preventative measures among individuals with aspiration risk factors even in the absence of aspiration symptoms,” the researchers wrote.
The study, “Aspiration, respiratory complications, and associated healthcare resource utilization among individuals with Rett syndrome,” was published in the Orphanet Journal of Rare Diseases.
In most cases, Rett is caused by mutations in the MECP2 gene, which disrupts the function of a protein that plays a role in maintaining nerve cell connections important for brain development and function.
Aspiration event data limited
Lower respiratory tract infections and respiratory failure are among the main causes of death in people with Rett. These patients have a high risk of aspiration events due to the combination of breathing issues, trouble swallowing, and risk factors that include needing a tube for nutrition, epilepsy and sideways curvature of the spine, or scoliosis.
But there is “limited published real-world data describing the rates of aspiration, respiratory complications, and healthcare resource use” in Rett patients, the researchers noted.
To learn more, they retrospectively analyzed electronic health data from the Vanderbilt University Medical Center, a Rett Center of Excellence. A total of 89 Rett patients were included, most of whom were female (92.1%) and white (66.3%). Twenty-five (28.1%) had had at least one suspected or known aspiration event.
Participants had a high prevalence of risk factors for aspiration, including epilepsy (73%), difficulty swallowing (51.7%), acid reflux (44.9%), scoliosis (sideways curvature of the spine, 40.4%), vomiting (40.4%), and gastrostomy, or a feeding tube inserted into the stomach (30.3%).
Almost one in five participants were admitted to the hospital due to respiratory failure (18%). Other respiratory causes for hospital admission included lower respiratory tract infection (14.6%) and aspiration (9%).
During the six months preceding the first aspiration event, patients with aspiration were significantly more likely to have epilepsy (54.5% vs. 4.5%), difficulty swallowing (40.9% vs. 0%), acid reflux (31.8% vs. 0%), scoliosis (31.8% vs. 4.5%), gastrostomy (27.3% vs. 4.5%) or vomiting (18.2% vs. 0%) compared with those who did not experience aspiration events.
Patients with known or suspected aspiration events were more likely to have respiratory failure (60% vs. 6.3%) and lower respiratory infections (50% vs. 5%). They also were about twice as likely to have been hospitalized at least once (75% vs. 35%). Almost one-third had another aspiration event in the 12 months following the first episode.
The rates of inpatient admissions tended to be higher in the aspiration group, although not significantly different from controls, while all-cause outpatient and emergency department visits were similar in both groups.
“While documenting aspiration events may be difficult, identifying individuals at high-risk for aspiration and providing anticipatory management would seem to be prudent,” the researchers wrote wrote. “The high rate of healthcare utilization highlights the morbidity of this subpopulation and the need for more effective diagnostic and treatment to avoid aspiration and its consequences.”
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