Introduction

Children with drug resistant epilepsy often have severe intellectual disability(SID)¹. Existing quality of life (QOL) tools are unlikely to reflect changes in QOL of these children. We have previously published using a non-validated QOL questionnaire aimed at this population², which was  modified and aimed to validate with inter-rater agreement.

Method

The ‘Cambridge London QoL questionnaire’ was used to assess QOL in children with SID or less than 2 years age,  who attended  the Ketogenic diet (KD) clinics two of the tertiary paediatric neurology centres in the UK in 2015-2018. Parents/carers completed the questionnaire twice, over a stable period whilst other interventions remained unchanged- 6-12 weeks prior to KD initiation (M1,F1) and immediately prior to KD initiation (M2,F2). The questionnaire had 35 questions (23 for QoL and 12 for seizure severity).

Result

Completed questionnaires were collected from 20 eligible children’s parents/carers.Inter-rater agreement kappa statistic³between M1,M2 and F1,F2 showed good and moderate correlation respectively (values 0.66 and 0.50 respectively).   Using one sided Sign test, M1 was significantly higher than M2 (p=0.0037) whereas no significant difference was seen between F1 and F2.

Conclusion

F1 scoring was not significantly higher than M1 scoring (p=0.13), but M2  was slightly though not significantly higher than the F2 scoring (p=0.07) even with no interventions, implying a placebo effect of the baseline assessment and plan for KD.

We plan to revalidate the questionnaire on a larger cohort. We note the importance of controlling placebo effect and parental differences in QOL assessment.