Jane Davison

Daisy Garland Ketogenic DietitianNottingham Children’s Hospital

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

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

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

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

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

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

Normal diet

Energy distribution from a normal diet:

Protein

Fat

Carbohydrate

Ketogenic diet

Energy distribution from a classical ketogenic diet:

Protein

Fat

Carbohydrate

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

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

Protein

lean meats

small amount of lentils

fish

eggsCheddar cheese

small amount of nuts

Fatcream cheese

double cream

mayonnaise

oils(e.g. olive oil, sunflower oil)

avocadobutter or margarine

Liquigen

Calogen

Carbohydrate

carrots

swede

tomatoes

mushrooms

raspberries

strawberries

peaches

pear

broccoliceleriac

Preferred sources of carbohydrate

CarbohydrateSources of carbohydrate to avoid

bread

peaches

sweets

cake

rice

pasta

chocolate

biscuits

sugar

Example of Foods

• F

Examples of foods

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

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

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

Short term problems

• Nausea• Vomiting• Hypoglycaemia• Acidosis• Sleepiness• Dehydration• Diet refusal

Long term problems

• Constipation • Renal stones• Abnormal lipid profile• Growth• Bone demineralization and fractures

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

Illness

• If IV fluids needed use 0.9% saline

• 4 hourly blood glucose

• Avoid sugar and carbohydrate containing drugs and IV

solutions

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

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

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

Websites

• www.thedaisygarland.org.uk• www.matthewsfriends.org.uk• www.charliefoundation.org

Keto santa’s!