Abstracts

Rationale: Epileptic spasms (ES) are the most common form of infantile epilepsy, and is associated with significant morbidity in children, often with devastating consequences on developmental and intellectual function. Early initiation of treatment is more effective in controlling ES and improving outcomes.  Despite its frequent presentation at our large Children’s Hospial, there have historically been difficulties in standardizing care and follow up of these patients, in part due to controversies in the history of its treatment, but also in the need for frequent and timely follow up visits, EEG evaluations, modifications to treatment regimen to ensure optimal response. To address this concern, we established a comprehensive treatment program for the identification, management, follow up, and coordination of care for patients with ES.

Methods: Standardized procedures for accurate diagnosis and treatment of ES were developed and disseminated, with education regarding the urgency of identification and prompt treatment for best outcomes. Standardized workup included EMU admission with overnight EEG monitoring, imaging, and laboratory workup for treatable metabolic/genetic conditions. A vital program component was the establishment of two nurse coordinators: an inpatient EMU coordinator, and an outpatient clinic coordinator. These serve as consistent contacts for inpatient teams, assisting in the process of obtaining specialty medications (ACTH, vigabatrin) and coordination of prompt follow-up visits/procedures. Patients have access to social workers, epilepsy nursing staff, and prompt referral to therapies including ketogenic diet, neurosurgical procedures, neurogenetics and genetic counseling services.

Results: Implementation of the ES program has resulted in a reduction in the time for EMU electrodiagnostic evaluation of potential spasms from a timeframe of 2 weeks, now to 24-72 hours. Additionally, by sequestering 2-6 new onset spasms clinic visit slots per week, patients are promptly established with an outpatient provider (one of two epileptologists or an epilepsy nurse practitioner) within 1 week of diagnosis in order to monitor clinical response, treatment side effects, discussion of prognoses, next steps. A practice improvement committee was formed to identify barriers to this process, and in doing so achieved a goal over 80% of newly diagnosed spasms patients undergoing follow up overnight EEG within 20 days of diagnosis and treatment. Furthermore, this program has allowed for multi-site collaborations and initiate translational research aimed at developing novel therapeutics.

Conclusions: The optimal success of urgent identification and management of ES relies on accurate and timely diagnosis, as well as initiation of appropriately dosed and closely managed therapies. When feasible, care for ES patients can be best accomplished by the formation of an ES program with a dedicated team of providers, nurse coordinators, and implementation of evidence-based protocols for diagnosis and management, in order to standardize of therapy, ensure close monitoring/follow up, and inclusion in clinical trials for novel therapeutics.

Funding: None