Abstracts

Rationale: Children with epilepsy, particularly those with severe seizures and neurologic disability, are at significant risk for bone mineral density (BMD) loss which can lead to fractures, pain, and challenges for caregivers. Those treated with the ketogenic diet (KD) are at even greater risk despite preventative measures with supplementation of calcium, phosphorous, and vitamin D.  Our center established a relationship with the Metabolic Bone Health Clinic, a multidisciplinary clinic with Endocrinology and Orthopedics, who not only assist with monitoring, but also with pamidronate infusions. The goal of this study was to report our experience with the management and treatment of KD patients in Metabolic Bone Health Clinic. Methods: All children on one of the KDs who were seen in the Metabolic Bone Health Clinic from 5/1/2015-5/1/2017 were reviewed in this study.  Children were referred to this clinic if a provider had a specific concern about bone health, the child had been on the KD for 2 years, if a dual-energy X-ray absorptiometry (DEXA) scan demonstrated evidence of BMD loss, or if the child had a fracture.  15 children were evaluated in the clinic.  Their records were then reviewed for history of fractures, DEXA scan results, and use of pamidronate. Results: Of the 15 children seen in this clinic, all were on the classic KD with ratios ranging from 2:1 to 5:1.  13 children received DEXA scans, with 2 families choosing not to pursue the study.  Nine of the children had z scores more than 3 standard deviations below the mean and 8 children had a history of fractures.  Pamidronate infusions were offered to all children with a low z score and a history of fractures with the first treatment performed in an inpatient setting due to concerns of complications.  Otherwise, follow-up DEXA scans were recommended on a yearly basis.  Of the 5 who received pamidronate, 1 developed profound hypocalcemia with treatment and one had increased seizures in the weeks following treatment.  The other 3 children tolerated the infusion without difficulty.  One had normalization of BMD, one had improvement, and one showed no further progression of BMD loss. Conclusions: The Metabolic Bone Health Clinic assisted in providing diagnosis, management, and the treatment option of pamidronate to children on the KD who had BMD loss and a history of fractures. Given the risk of hypocalcemia or increased seizures associated with pamidronate, it must be used with caution in the KD population. The review of these cases suggests that with guidance from endocrinology and orthopedics, the use of pamidronate may be considered as a possible therapeutic option for osteoporosis in children on the KD and moving forward we plan to work to establish a standardized protocol. Funding: None.