1. Jane Davison Daisy Garland Ketogenic Dietitian Nottingham Children’s Hospital

2. What is the ketogenic diet? It is a high fat, low carbohydrate and adequate protein diet used in the treatment of intractable epilepsy It aims to decrease the number and severity of the patients’ seizures Therefore improving the quality of life and hopefully reducing medication use

3. Indications for use Intractable childhood epilepsy* *incomplete seizure control despite > 2 AED GLUT 1 deficiency syndrome PDH deficiency Consider as early treatment for myoclonic astatic epilepsy Age not a barrier but works well in younger children, e.g. under 10years

4. Efficacy Randomised controlled trial at GOS 2008 150 patients After 3 months 38% had >50% seizure reduction compared with 6% in the control phase 7% had >90% reduction in seizures

5. Efficacy Study also compared the Classical and MCT (Medium Chain Triglyceride) diets Results showed no difference in efficacy or tolerability between the 2 diets after 3,6, and 12 months Those on the MCT version had a little more nausea

6. Once selected Family agree to a 3 month commitment period on the diet as there is a huge investment of professional time 3 months is the best time scale to determine outcomes Diet usually advised for up to 2 years Expectations from medical team and families need to be discussed to assist future monitoring

7. The dietetic aims To achieve ketosis by providing fat for metabolism To ensure that growth is adequate To ensure nutritional well being is not compromised To implement a dietary regimen that is palatable and possible to comply with

8. Normal diet Energy distribution from a normal diet:

9. Ketogenic diet Energy distribution from a classical ketogenic diet:

10. Types of Ketogenic Diet Classical 4:1 and 3:1 ratio i.e. Calorie controlled with 4 or 3 parts fat to 1 part protein and carbohydrate combined MCT diet (medium chain triglycerides) Calorie controlled 40-60% energy from MCT allows a little more flexibility but high MCT intake can lead to nausea

11. Types of Ketogenic Diet Modified Ketogenic diet 60% fat 30% protein 10% CHO The carbohydrate intake is limited to approximately 20g per day Less strict still needs good knowledge of foods to choose Energy intake monitored for weight control Low Glycaemic Index (GI) diet Energy distribution as above but choice of foods with GI less than 50 typically high fibre foods Total carbohydrate load 40-60g per day

12. Protein lean meats small amount of lentils fish eggs Cheddar cheese small amount of nuts

13. Fat cream cheese double cream mayonnaise oils (e.g. olive oil, sunflower oil) avocado butter or margarine Liquigen Calogen

14. Carbohydrate carrots swede tomatoes mushrooms raspberries strawberries peaches pear broccoli celeriac Preferred sources of carbohydrate

15. Carbohydrate Sources of carbohydrate to avoid bread peaches sweets cake rice pasta chocolate biscuits sugar

16. Example of Foods F

17. Examples of foods

18. Products for the Ketogenic diet KetoCal 4:1Multi fibre For enteral feeding 200ml carton 300kcal 6.2gP 1.2gCarb 29.6gFat KetoCal 3:1 powder For enteral feeding Suitable from birth

19. Supplementation Products Calogen 50% LCT fat CarbZero 20% LCT fat Liquigen 50% MCT fat Betaquik 20% MCT fat Vitamin and Minerals e.g. Fruitivits Phlexy Vits

20. Monitoring Growth: weight, height, and well being Bloods, urine other tests e.g. ECG, renal USS Record of seizures Record of ketosis -Blood use Abbott meter Freestyle Optium Neo (need lancets & ketone strips) aim 2-5mmol/l -Urine use Ketostix aim for (+2 to +4) or 8-16mmol/l

21. Short term problems Nausea Vomiting Hypoglycaemia Acidosis Sleepiness Dehydration Diet refusal

22. Long term problems Constipation Renal stones Abnormal lipid profile Growth Bone demineralization and fractures

23. Excess ketosis Rapid panting or breathing Facial flushing, vomiting Irritability and unexpected tiredness Acidosis Blood ketone reading 6mmol/l or “Hi” with symptoms Treat with 1-2 tablespoons of sweet drink If no improvement after 15 minutes repeat and contact Dr

24. Illness If IV fluids needed use 0.9% saline 4 hourly blood glucose Avoid sugar and carbohydrate containing drugs and IV solutions

25. Other considerations Carnitine It is a protein which is necessary to carry fat into the mitochondria to be broken down to energy (ATP) KD high in fat therefore ↑need KD low in lysine + methionine needed to synthesize carnitine ↑Excretion of acylcarnitine with ketosis

26. Nottingham experience Daisy Garland funding since 2009 initially 0.5WTE now service is 0.3WTE with 0.2WTE funded from Daisy Garland Over 30 patients have been put on the diet in 5 years November 2014 Current patients 13 paediatric patients plus 1 adult gastrostomy fed patient

27. Nottingham experience 3 metabolic patients 6 gastrostomy fed patients experiencing >75% seizure reduction and 1 with complete seizure freedom weaned off medication and is now weaning off diet gradually 4 on oral K.D. diet having >75% seizure reduction 2 waiting to start

28. Websites www.thedaisygarland.org.uk www.matthewsfriends.org.uk www.charliefoundation.org

29. Keto santa’s!