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Ketogenic Dietetic Referrals

Our referral form can be found here. Alternatively referrals can be completed via consultant letter and/or referral details sent via secure NHS email to BRHCketodietitians@uhbw.nhs.uk. The referral must include a past medical history and list of current and previous epileptic treatment.

Please follow referral guidance below for new referrals for Ketogenic Dietary Therapy. Referrals will be triaged according to their priority and eligibility for ketogenic dietary therapy (KDT).

Referral Criteria

  • Referred by Paediatric Consultant/Neurologist/ Specialist Epilepsy Nurse with consent from managing Consultant
  • All patients must have a named Bristol Paediatric Consultant Neurologist/Metabolic Consultant if the referral is out of area
  • Contraindications excluded (see Table 1)

AND at least one of the below:

  • Intractable epilepsy and failed > 2 anti-epileptic medications*
  • Glucose Transporter Type 1 (Glut-1) Deficiency Syndrome and Pyruvate Dehydrogenase Deficiency (PDHD) (first line treatment option)**
  • Previously on KDT (past 12 months) and efficacy demonstrated.

*KDT could be offered earlier for Angelman syndrome, Dravet syndrome, Febrile infection-related epilepsy syndrome (FIRES), infantile spasms, epilepsy with myoclonic-atonic seizures and tuberous sclerosis complex.

**Other metabolic conditions where the ketogenic diet is considered to be a valid treatment option include Glycogen Storage Disease Type III and VII and certain mitochondrial disorders.

 

Table 1: Contraindications to KDT

Absolute

  • Carnitine deficiency (primary)
  • Carnitine palmitoyltransferase (CPT) I or II deficiency
  • Carnitine translocase deficiency
  • β-oxidation defects
  • Medium-chain acyl dehydrogenase deficiency (MCAD)
  • Long-chain acyl dehydrogenase deficiency (LCAD)
  • Short-chain acyl dehydrogenase deficiency (SCAD)
  • Long-chain 3-hydroxyacyl-CoA deficiency
  • Medium-chain 3-hydroxyacyl-CoA deficiency
  • Pyruvate carboxylase deficiency
  • Porphyria

Relative

  • Inability to maintain adequate nutrition
  • Surgical focus identified by neuroimaging and video-EEG monitoring
  • Parent or caregiver noncompliance
  • Propofol concurrent use (risk of propofol infusion syndrome may be higher)

 

For urgent referral of inpatients for Ketogenic Dietary Therapy, please additionally telephone the department with referral details (0117 34 21185).

For existing ketogenic patients requiring an inpatient review, please contact via Careflow Connect to 'BRHC Ketogenic Dietitians'.

References:

Klepper et al (2020). Glut1 Deficiency Syndrome (Glut1DS): State of the art in 2020 and recommendations of the International Glut1DS Study Group. Epilepsia Open, 5: 354-365

Kossoff et al (2018). Optimal clinical management of children receiving dietary therapies for epilepsy: Updated recommendations of the International Ketogenic Diet Study Group.Epilepsia Open, 3(2):175-192, doi: 10.1002/epi4.12225

Marusic et al (2020). Data highlighting effects of Ketogenic diet on cardiomyopathy and hepatopathy in Glycogen storage disease Type IIIA.Data Brief, 32:106205

Similä et al (2020). Case Report Article: Beneficial Effects of Ketogenic Diet on Phosphofructokinase Deficiency (Glycogen Storage Disease Type VII)Frontiers in Neurology. doi: 10.3389/fneur.2020.00057

Zweers et al (2021). Ketogenic diet for mitochondrial disease: a systematic review on efficacy and safety. Orphanet Journal of Rare Diseases, 16: 295