Rationale:
The ketogenic diet (KD) is recommended as a second line treatment in Dravet syndrome but reported success in the literature is variable, often with high drop out as prolonged seizure freedom is not expected. The KD has also been reported to reduce status epilepticus (SE) and hospital admissions, therefore has an important role as a longterm “maintenance” treatment for children with Dravet Syndrome.
Our goal was to evaluate the success of the KD in the largest cohort of patients with Dravet syndrome from our resource-rich center and compare our success to published literature. Our center is a well-established program built on a family-centered care model with resources such as dedicated physicians, nurses, and dieticians; KD chef educator; a KD teaching kitchen; parent coaches; newsletters, websites, and videos; and other resources.
Method:
Retrospective review of 36 children (21 female) with Dravet syndrome treated at Children’s Hospital of Philadelphia between 1997 and 2020. Data abstracted included demographics, age at seizure onset, medications failed, seizure frequency at KD onset, duration of KD, change in seizure frequency, and reason for discontinuation. Effectiveness and KD retention rate within the first year were compared to other published cohorts. KD retention rate was examined up to 3 years. Responders were defined as ≥50% reduction in seizure frequency of all seizure types.
Results:
Responder rate to KD was 60% at 3 months, 68% at 6 months, 53% at 1 year. Seizure-freedom occurred in 17% at 3 months, 32% at 6 months, and 25% at 1 year. Sixteen patients achieved any period of seizure-freedom on the KD ranging from 2 weeks to 60 months, which occurred in 7 of 16 without concurrent medication change. There were 4 episodes of SE in patients on the KD over the first year, compared to 80 episodes among all patients prior to KD initiation. Patients continued the KD for a median of 25 months (range 0.1 to 93 months). Twenty-four (69%) patients were on the KD at 1 year, 18 (53%) at 2 years, and 14 (44%) at 3 years. Discontinuation was due most often to difficulty with patient adherence or ineffectiveness. Compared to other published studies, we have improved long-term retention rate (see Figure).
Conclusion:
The KD is a feasible long-term treatment for patients with Dravet syndrome. Patients benefit both in regards to seizures control and reduced number of status epilepticus episodes. Higher retention rates are achieved by providing multiple family support services including frequent KD team evaluations, access to a chef and cooking classes, keto coaches and a yearly family party, that all help build a KD community and improve long-term retention in patients with Dravet syndrome.
Funding:
:None
FIGURES
Figure 1