Prioritizing Hormone Therapy Over Vigabatrin as the First Treatment for Infantile Spasms

Abstract

Background and Objectives:Infantile spasms (IS) are early childhood seizures with potentially devastating consequences. Standard therapies (adrenocorticotropic hormone [ACTH], high-dose prednisolone, and vigabatrin) are strongly recommended as the first treatment for IS. While this recommendation comes without preference for one standard therapy over another, early remission rates are higher with hormone therapy (ACTH and high-dose prednisolone) when compared to vigabatrin. Using quality improvement (QI) methodology that included hormone therapy as the first treatment, we sought to increase the percentage of children with new onset non-tuberous sclerosis complex (TSC)-associated IS achieving 3-month electroclinical remission from a mean of 53.8% to >70%.Methods:Observational cohort study using a consecutive sample at a single academic tertiary care hospital comparing a prospective intervention cohort (5/2019 – 1/2022, N = 57) to a retrospective baseline cohort (11/2015 – 4/2019, N = 67). Our initiative addressed key drivers such as the routine use of vigabatrin over hormone therapy as first treatment and the common initiation of a second treatment after 14 days for initial non-responders. We included consecutive children without TSC presenting with new onset IS diagnosed and treated between 2 – 24 months of age. We displayed our primary outcome and process measures as control charts in which the centerline is the quarterly (previous three months) mean based on statistical process control methodology.Results:QI interventions that included the standardization of hormone therapy as the first treatment resulted in higher rates of 3-month remission, rising from 53.8% (baseline cohort) to 75.9% (intervention cohort). Process measure results included an increased rate of children receiving hormone therapy as first treatment (mean 44.6% to 100%) as well as a decreased number of days to both clinical follow-up after first treatment (mean of 16.3 to 12.6 days) and starting a second treatment within 14 days for initial non-responders (mean of 36.3 to 17.2 days).Discussion:For children with IS, improved rates of 3-month electroclinical remission can be achieved with QI methodology. Implementation of similar QI initiatives at other centers may likewise improve local remission rates.

Initial combination versus early sequential standard therapies for Infantile Epileptic Spasms Syndrome—Feedback from stakeholders

“…Another quality improvement study of sequential therapy (prioritizing initial hormonal therapy over vigabatrin followed by routine initiation of a second treatment after 14 days from the failed first treatment) ultimately resulted in a remission rate of 75.9% within 3 months. 7 In the future, early sequential therapy may become another reasonable treatment option for combination therapy for clinicians. A recent study evaluating the treatment response to initial standard therapy (ACTH, prednisolone, or vigabatrin) showed that remission can be identified as early as treatment day 7 in nearly 80% of responders.…”

“…Another quality improvement study of sequential therapy (prioritizing initial hormonal therapy over vigabatrin followed by routine initiation of a second treatment after 14 days from the failed first treatment) ultimately resulted in a remission rate of 75.9% within 3 months. 7 …”