Abstracts

Rationale: The ketogenic diet is typically initiated following a multidisciplinary clinic evaluation and preliminary parent education. This allows for parental consideration of the role and potential risks vs benefits of the diet, as well as psychological preparation for the commitment required. At our institution, an urgent initiation occurs when the patient is already an inpatient and cannot be discharged because of the severity of his/her seizure disorder. We describe 11 urgent ketogenic diet initiations. Methods: We identified from the clinic database 11 instances of urgent diet initiation in 10 pediatric patients, ranging in age from 2 months-13 years at diet initiation. Epilepsy diagnoses included symptomatic epilepsy (6), Migrating Partial Epilepsy of Infancy (2), Lennox-Gastaut Syndrome (1), and Doose syndrome (1). None of the patients was in status epilepticus. Uncontrolled seizures (mean 15/day, range 5-70) was the primary indication in 9 patients. A tenth patient had PDH deficiency with seizure onset during the hospitalization. Results: The patients were taking multiple antiepileptic drugs (AEDs) (mean 4.7, range 3-6). In all cases ketogenic formula or eggnog (in orally fed patients) was used to initiate the diet. Advancement to meals of solids occurred in patients that were medically able to take oral foods. At least one parent of each child received comprehensive ketogenic diet teaching. Three initiated patients remained on the diet for ? 1 month. The reasons for diet discontinuation included financial constraint, hematuria, and visualization of a large gallstone. Eight patients were discharged on the diet (one patient on 2 separate occasions.) Duration of the diet in these patients ranged from 6 weeks to >3 years. Reasons for discontinuation were family concern for kidney stone risk, lack of efficacy, and food refusal. With concurrent correction of metabolic acidosis all patients tolerated the diet, and all patients demonstrated ketosis with ketonuria /- betahydroxybutyrate of at least 4.5 mmol/L. Two patients discharged on the diet subsequently died from their underlying disorders (mitochondrial disease, and Down syndrome with aspiration pneumonia.) One month following discharge, 5/8 patients had achieved a ?50% reduction in seizures. Two patients were seizure free. AEDs had been reduced in 6 patients. Average length of stay in patients that were discharged home on the diet was 62 days. In these patients, the average number of days to discharge was 15 days from the first day of diet initiation (range 4-27 days). Conclusions: Urgent ketogenic diet initiation is feasible and may provide an effective additional therapeutic approach in acutely refractory epilepsy. However, urgent initiation may not allow adequate time for full evaluation of family/patient financial resources, attitude towards the diet, expectations, acceptance of medical risks, education, and psychological adjustment.