Most patients achieved resolution of status epilepticus on KD therapy, suggesting it could be an effective therapy that can be utilized early in the treatment of children with super refractory status epilepticus.

Ketogenic metabolic therapy (KMT) has demonstrated effectiveness in seizure reduction. However, patient compliance and adverse effects limit its use. Ready-to-feed (RTF) ketogenic formulas improve compliance and include components that mitigate adverse effects. This study is the first to evaluate the efficacy and tolerability of an RTF, whey-based, medium-chain triglyceride-enhanced (WBME) ketogenic formula. Methods: Retrospective data from patients who received KMT between January 1, 2015, and February 28, 2018, were analyzed. Patients who received ≥75% of their total calories from the WBME formula and who were monitored for 3 months were included. Outcome measures were gastrointestinal issues, acidosis, serum blood glucose and beta-hydroxybutyrate levels, unintentional weight changes, diet response (≥50% reduction in seizures), seizure freedom, and change in formula or discontinuation of therapy. Patients with incomplete outcome data or who received <75% of total calories from the formula were excluded. Results: Twenty-six patients (13 males; mean [SD] age, 6.1 [5.8] years) met the inclusion criteria. Thirteen patients were established patients who received a standard ketogenic formula before changing to the WBME formula; 13 were patients new to KMT whose therapy was initiated using the WBME formula. This formula was well tolerated; no patient in either group discontinued therapy or required a change in formula. The combined diet response rate (95% CI) for established and new patients was 96% (80-100%). Seizure-freedom (95% CI) for both groups at 3 months posttreatment was 20% (7-41%). The most prevalent adverse effect was constipation (69% [95% CI, 48-86%]). Conclusion:The WBME ketogenic formula appears to be effective and well tolerated by pediatric patients with refractory epilepsy.