Abstracts

Rationale: Ketogenic therapy (KT) is well-established for children with medically refractory epilepsy. However, it is not without adverse effects (AE), especially during initiation. AE may include hypoglycemia, lethargy, metabolic acidosis, or vomiting. According to the International Ketogenic Diet Study Group (Kossoff 2018), 80% of centers initiate KT in the hospital to allow close observation and medical intervention as needed. Historically, infants < 12 months required hospitalization for initiation to monitor for AEs (Van Der Louw 2016).  However, at Phoenix Children's Hospital, all patients are initiated outpatient. To enhance safety and optimize  outpatient management of KT, a home monitoring app (HMA) was developed, focusing on AE in the first 30 days after initiation of KT.

Methods: A multidisciplinary group (information technology (IT), epileptologists, ketogenic dietitians) designed a HMA to provide a way to monitor patients during initiation of KT. Caregivers voluntarily consented to enroll in the app. Once enrolled, they received a text message daily to log weight, ketones, blood glucose, alert symptoms (lethargy, sleepiness, flushed cheeks, fast breathing, nausea, vomiting), feeding intake (as usual, less or more than usual) and seizures (baseline, less or more than baseline). The on-call dietitian received data by email /text for review. If alert symptoms noted, the dietitian contacted the family. A retrospective chart review of the first 32 patients enrolled in the HMA was performed for the first 30 days of submissions since app was launched in April 2021 to May 2022.

Results: Of the 50 patients initiated on KT since April 2021, 32 (64%) enrolled in the HMA, median age of 2.4 yrs (2 mo.-18 yrs, mean=3.5 yrs),  All 7 infants enrolled. Of the 32 patients enrolled, 22 (69%) submitted ≥ 10 days of data in first 30 days (median=13 days). Data were submitted for 6 of 7 (86%) infants ≥ 15 days in first 30 days (median=20 days). Analysis of AE excluded 11 patients (5 inpatient initiations, 4 enrolled > 30 days after initiation, 2 never on full KT) for n=21. Of 329 submissions by 21 patients, 65 (20%) noted one or more alert symptom. Vomiting was the most common symptom reported by 7 patients (33%), followed by 5 (24%) sleepiness, 4 (19%) lethargy, 2 (10%) nausea, and 1 (5%) flushed cheeks. Only 11 (12%) of 90 submissions for 3 of the 7 infants reported alert symptoms.  Only 1 emergency department (ED) visit occurred resulting in hospital admission. No infants required ED visit or hospitalization.

Conclusions: The HMA serves as an effective way to monitor children, especially infants, during outpatient initiation of KT by identification of AE with notification of the ketogenic team and subsequent intervention.  The app was well-received by caregivers, with all infants initiated on the diet enrolled in the app. Only mild AE noted and treated primarily outpatient. Limitations are the small sample size, reliance on self-reporting of caregivers and retrospective review. Future opportunities for research include validating caregiver education and compliance with KT, and may not be limited to just the first 30 days, but for patients established on KT requiring long-term monitoring.

Funding: None