With the COVID-19 pandemic causing nusinersen treatments to be postponed, a study published in Frontiers in Neurology sought to understand the impact of delayed infusions and other supporting interventions for patients with spinal muscular atrophy (SMA) during the initial lockdown period in Italy in 2020.
Researchers from the pediatric palliative care Centre of Padua (Veneto) evaluated patients with SMA through medical record review and questionnaires to see the change in quality of life for patients and their families as a result of delays in treatment.
The study examined 25 patients at specific times in their diagnosis and treatment during the COVID-19 lockdown, including whether the nusinersen infusions during lockdown were on time or delayed. Data were also gathered from a cross-sectional survey administered to patients age 6 or older and their parents at the first nusinersen treatment after lockdown was lifted. The survey inquired about physical functions and emotions during the lockdown period.
Overall, the study results shed light on the impact on patients with SMA and their parents when there is delayed treatment and modified or diminished interventions. While the researchers found patients got worse in their functional score in short- and long-term evaluations, they accredited that finding to the difficulty of family situations for patients, such as single-parent families or social issues, rather than to the delay in infusions.
The researchers also noted that parents had access to nurses 24/7 via telephone, which was utilized by participants throughout the study, as well as video calls for respiratory physiotherapy.
The initial lockdown in Italy due to COVID-19 in early 2020 had a notable effect on patients with SMA and their families. The example presented in this study underscores the importance of home physiotherapy, as well as the impact delayed nusinersen infusions can have on muscle strength when also examined in the context of lacking typical supports.
A call to action is made to continue studying this population for longer periods of time to better understand changes in swallowing function attributed to SMA [1].
Source:
[1] Agosto, C., Salamon, E., Giacomelli, L., Papa, S., Benedetti, F., & Benini, F. (2021). Effect of the COVID-19 pandemic on children With SMA receiving nusinersen: What is missed and what is gained? Frontiers in Neurology, 12. https://doi.org/10.3389/fneur.2021.704928
