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Transcript
Jane Davison
Daisy Garland Ketogenic Dietitian
Nottingham 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
small amount of
nuts
fish
lean meats
eggs
Cheddar
cheese
small amount
of lentils
Fat
cream cheese
double cream
oils
(e.g. olive oil, sunflower oil)
Calogen
butter
or margarine
avocado
Liquigen
mayonnaise
Carbohydrate
Preferred sources of carbohydrate
raspberries
strawberries
carrots
celeriac
broccoli
peaches
mushrooms
swede
tomatoes
pear
peaches
Carbohydrate
Sources of carbohydrate to avoid
bread
sweets
sugar
rice
biscuits
cake
pasta
chocolate
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!