LRI Children’s Hospital

Management of Children on Ketogenic Diet for Epilepsy

Staff relevant to: Health professionals caring for children with epilepsy
being managed on a ketogenic diet.

Team approval date: December 2022

Version: V4

Revision due: December 2025

Written by: Dr N Hussain, Dr A Khan, J Kearns

Reviewed & updated by: Dr R Samanta, Dr N Hussain, E Wilford, R Fox & G
Thorne

Trust Ref: C255/2016

Contents
1. Introduction and Who Guideline applies to ........................................................................ 2
Related Documents: .......................................................................................................... 2
2. Ketogenic diet is indicated for: .......................................................................................... 2
3. Referral letters should include the following information .................................................... 3
Table 1: Investigations & Monitoring .................................................................................. 5
4. Possible Side Effects of the Ketogenic Diet 2 .................................................................... 6
Table 2: Additional Monitoring for Adverse Effects:............................................................ 6
5. Principles of Management for Inpatient Admissions .......................................................... 6
6. Inpatient Management of Children on Ketogenic Diet ....................................................... 7
7. Formulation of Medication: ............................................................................................. 11
8. Education & Training ....................................................................................................... 12
9. Monitoring Compliance .................................................................................................. 12
10. Supporting References ................................................................................................. 12
11. Key Words .................................................................................................................... 13
Contact and review details............................................................................................... 14
Appendix A - Types of Ketogenic Diet: ............................................................................ 14
Appendix B: Adverse Effects of Ketogenic Diet 2 ............................................................. 15
Appendix: C – Paracetamol & Ibuprofen preparations ..................................................... 16
Appendix D: Ketogenic Diet Bloods ................................................................................. 17
Appendix E: Assessment Clinic for Ketogenic Diet (Proforma) ........................................ 18

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Title: Ketogenic diet for epilepsy
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NB: Paper copies of this document may not be most recent version. The definitive version is held on InSite in the Policies and Guidelines Library
 1. Introduction and Who Guideline applies to

The ketogenic diet (KD) is a high-fat, low carbohydrate and adequate protein diet used in
the management of childhood epilepsy. It is a therapeutic diet which has been shown to
improve seizure control in patients with drug resistant epilepsy. 1

Related Documents:
This guideline needs to be used in conjunction with relevant infection control and consent
policies to ensure the child receives safe care and children and families are able to
understand the reasons for care to facilitate co-operation.

The dietitian will work in line with the -

UHL C22/2017 - Clinical Guideline on the Dietary Management of Children with Intractable
Epilepsy Treated with Ketogenic Diet. Ketogenic Diet for Children with Epilepsy UHL Dietetic
Guideline

How does the ketogenic diet work?

Usually, the body uses glucose from carbohydrates found in foods like fruit, vegetables,
sugar, bread, rice and pasta for its energy source. In the KD, the body's energy source
comes from using fat instead of glucose. Ketones are made when the body uses fat as its
source of energy. This is called 'ketosis'.
For some people with epilepsy, seizures are greatly reduced or prevented when the body
makes ketones.

There are different forms of KD regimes: [Appendix A]

• The Classical KD
• The Medium Chain Triglyceride diet (MCT diet)
• The Modified Ketogenic Diet (MKD)

2. Ketogenic diet is indicated for:

See also (NICE 2022)

2.1 Children whose seizures fail to respond to at least 2 antiepileptic medications in

therapeutic doses

2.1a For certain epilepsy syndromes

Epilepsy syndromes and conditions where KDT is more beneficial (>70%
seizure reduction than average) 2
• Dravet syndrome
• Epilepsy with myoclonic–atonic seizures (Doose syndrome)
• Febrile infection–related epilepsy syndrome (FIRES)
• Infantile spasms
• Ohtahara syndrome
• Super-refractory status epilepticus
• Tuberous sclerosis complex
• Formula-fed (solely) children or infants
• Angelman syndrome

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2.1b Conditions where KDT moderately beneficial (>50% seizure reduction) –
(arranged alphabetically) 2
• Adenylosuccinate lyase deficiency
• CDKL5 encephalopathy
• Childhood absence epilepsy
• Cortical malformations
• Epilepsy of infancy with migrating focal seizures
• Epileptic encephalopathy with continuous spike-and-wave during sleep
• Glycogenosis type V
• Juvenile myoclonic epilepsy
• Lafora body disease
• Landau-Kleffner syndrome
• Lennox-Gastaut syndrome
• Phosphofructokinase deficiency
• Rett syndrome
• Subacute sclerosing panencephalitis (SSPE)

2.2 For children with metabolic disorders:

• Complex 1 mitochondrial disorders
• Glucose transporter protein 1 (Glut-1) deficiency syndrome (Glut1DS)
• Pyruvate dehydrogenase deficiency (PDHD)

2.3 For children who have intolerable and/or severe effects from antiepileptic
medication

2.4 Contraindications to the use of KDT 2

• Absolute
o Carnitine deficiency (primary)
o Carnitine palmitoyltransferase (CPT) I or II deficiency
o Carnitine translocase deficiency
o beta-oxidation defects
o Medium/Long / Short-chain acyl dehydrogenase deficiency (CAD)
o Long/Medium-chain 3-hydroxyacyl-CoA deficiency
o Pyruvate carboxylase deficiency
o Porphyria

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

2.5 Adverse Effects of Ketogenic Diet 2 [Appendix B]

3. Referral letters should include the following information

• The patient’s present medical condition and medical history

• Seizure type, severity and frequency
• Previous medication tried
• Dosage of current medication
• Patients feeding ability and route for nutrition (oral or enteral tube)
• Information on recent investigations e.g. Electroencephalogram (EEG)

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Title: Ketogenic diet for epilepsy
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• If the patient recently had or is awaiting Vagus Nerve Stimulation (VNS)
• If the patient is a candidate for neurosurgery
• Meet basic standards of documentation as outlined by the Patient Health Records -
Documenting UHL Policy (in all media)’ Trust Ref B30/2006.

New patient referrals

New referrals are discussed in the monthly paediatric KD MDT meeting. If appropriate,
Children are invited to a pre-assessment clinic with the Consultant Paediatric Neurologist,
Senior Specialist Dietitian and Epilepsy Specialist Nurse.

The diagnosis is confirmed and if appropriate the ketogenic diet is offered as a treatment
option.

The efficacy of ketogenic diet, possible side effects, routine monitoring and the
practicalities of undertaking ketogenic diet are discussed in detail. Written information is
provided to support this.

The Dietitian assesses the child and takes into consideration age, weight, height, activity
level, other co-existing medical conditions, feeding issues, neurological deficits and
psychological issues before formulating the KD18.

The Dietitian regularly reviews the patient’s progress via telephone, fine tuning the KD to
maintain/improve ketosis and optimize the possibility for seizure reduction/cessation18.

Once the child is established on an effective and appropriate KD, weaning of the other AEDs
is considered.
For further information regarding this process and multidisciplinary roles, refer to the ‘Clinical
Guideline on the Dietary Management of Children with Intractable Epilepsy Treated with
Ketogenic Diet’.

Monitoring

Regular multidisciplinary follow up clinics are held and attended by the Consultant Paediatric
Neurologist, Specialist Registrar, Senior Specialist Dietitian and the Epilepsy Specialist
Nurse.

Children on the ketogenic diet are reviewed in this clinic three months after commencing
ketogenic diet and then every six months (or sooner if necessary) while on ketogenic diet.

Blood monitoring and urinalysis is carried out before the diet is initiated and then as
described below or more frequently as clinically indicated. 2, 3 (Please see following tables
below and section 6 for condition specific guidance on frequency of monitoring)

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Title: Ketogenic diet for epilepsy
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NB: Paper copies of this document may not be most recent version. The definitive version is held on InSite in the Policies and Guidelines Library
 Table 1: Investigations & Monitoring

Blood Investigation Frequency of monitoring

Essential:
• Full blood count
• Fasting cholesterol & triglyceride
• Ferritin, amino acid profile
• U&E, bicarbonate, lactate, albumin
• Urate
• Liver function tests
Baseline, 3 months, 6 months and then
• Calcium, phosphate
every 6 months.
• Magnesium
• Glucose Additionally at 6 weeks for infants under 2
• Free and total Carnitine years old
• Acylcarnitine profile
• Vitamin A, D & E
• Zinc, Selenium, Copper
• Anti-convulsant level

• Clotting screen Baseline only

Recommended:
• Blood ketones Baseline, 3 months, 6 months, and then
• Free fatty acids every 6 months.
Optional:
• Folate Baseline, 6 months, then every 6 months.
• Vitamin B12 Baseline, then every 12 months.
Urine Investigation Frequency of monitoring
Baseline, 6 months, and then every 6
• Calcium:Creatinine ratio months. Additionally at 6 weeks for infants
under 2 years old

• Amino Acids Baseline only

• Organic Acids

The Senior Specialist / Senior Paediatric Ketogenic Dietitian will aim to manage the
biochemistry where possible through the use of food and dietary changes; and if necessary
additional nutritional supplements or an increase in the vitamin and mineral preparation may
be necessary.

Where dietary manipulation is not likely to be sufficient, the Consultant Paediatric

Neurologist may need to arrange a prescription. This may include but is not limited to
• Iron and Vitamin D supplementation
• Potassium Citrate for raised urinary calcium:creatinine ratio, indicating an increased
risk of renal stones.
• Carnitine supplementation. Carnitine is essential for the transport of Long chain
triglyceride (LCT) fat to the cells to be broken down so Carnitine deficiency is
theoretically more likely to occur on a classical or modified KD than on an MCT
ketogenic diet.

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Title: Ketogenic diet for epilepsy
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NB: Paper copies of this document may not be most recent version. The definitive version is held on InSite in the Policies and Guidelines Library
 4. Possible Side Effects of the Ketogenic Diet 2

1. Vomiting, constipation, lack of energy, hunger and diarrhoea (these can generally be
resolved with dietary manipulation).
2. Elevated serum lipids.
3. Excess ketosis and acidosis.
4. Long term vascular outcomes of KD are unknown.
5. Renal stones.
6. Impairment / delay in growth.
7. Compromised bone health. 17

Table 2: Additional Monitoring for Adverse Effects:

Consider DEXA scan If on KD for >2y & 1 year later (if 1st scan is
abnormal)
Renal USS At 1 year on KD and annually thereafter
ECG (if family history of cardiovascular disease) Before starting

5. Principles of Management for Inpatient Admissions

Inform Neurology team (by switchboard) and Specialist Dietitian (ext 15400)

• Bloods FBC, U/E, bicarbonate, blood gas, lactate, LFT, blood and urinary ketones,
infection screen

• Standard target ketones 2-5mmol/l. Some children will have their upper or lower
ketone thresholds changed on review by the KD team due to individual response to
ketosis. If individual ketone thresholds are unknown then a range of 2 – 5mmol/l
should be used.

• Target blood glucose 2.5 mmol/l and above

• Choose appropriate IV fluids depending on ketone and blood glucose levels

• Do not start a non-ketogenic diet enteral feed, maintain on IV fluids until Dietitian
reviews and calculates a suitable ketogenic feed recipe – there is no standard
ketogenic feed for these patients.

• At evenings and weekends when a dietitian is not available parents/carers can

order food from the Ketogenic/Metabolic Diet Loose Foods Menu to achieve their
usual KD prescription. This is available via catering.

• Medications: Choose the lowest carbohydrate form possible (generally

dissolvable/crushable tablets or powder preparations). Contact Pharmacists via
dialling #6737

• Contact Medicines Information (LRI ext 16191/16491) to begin checking the

carbohydrate content of medications

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Title: Ketogenic diet for epilepsy
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6. Inpatient Management of Children on Ketogenic Diet 5
(Adapted from Ketogenic Diet in the management of Epilepsy; Clinical Guidelines. Great
Ormond Street Hospital)

6.1) Gastrointestinal or Intercurrent Illness

• Medications: Choose the lowest carbohydrate form possible (generally

dissolvable/crushable tablets or powder preparations) Contact Pharmacists via dialling
#6737

• Medicine Information (LRI ext 16191/16491) to begin checking the carbohydrate content of
medications

• If the child is unwell: medical assessment and urgent bloods to include FBC, U/E,
bicarbonate, blood gas, lactate, LFT, blood ketones, blood glucose, infection screen.

• On admission refer to the specialist dietitian electronically via the ICE system.

• If the patient is fed solely via NGT or PEG order 1 x 400g tin of Polycal powder from
pharmacy for treatment of hyperketosis and/or hypoglycaemia

• Check blood glucose and blood ketones 4 or 8 hourly (at discretion of clinician).
Maintain blood glucose above 2.5 mmol/l

• Maintain blood ketones less than 5 mmol/l (refer to sections 6.2- 6.3 below)
for treatment of hypoglycaemia and hyperketosis)

• Offer clear fluids as frequently as tolerated that are low in carbohydrate, e.g. water or
sugar free squash. Dioralyte can be used if necessary but discuss with the Dietitian
first as it contains glucose

• The Dietitian will arrange special meals or enteral feeds if required for the patient.

• If the child is usually enterally fed, Milk Kitchen will prepare the child’s usual feed recipe.

• When symptoms (diarrhoea, vomiting) subside, the diet can be reintroduced at a quarter
to half of all daily exchanges (parents will know their child's daily exchanges for fat,
protein and carbohydrate). If this is tolerated the exchanges can be increased gradually
as able. The medium chain triglyceride fat (MCT); Liquigen or MCT oil can be
reintroduced slowly. The Dietitian will provide an individual plan for the patient. If at any
stage the symptoms recur, there should be a return to the levels previously tolerated.
6.2) Hyperketosis (Ketones > 5mmol/l)

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Title: Ketogenic diet for epilepsy
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 6.2) Hyperketosis (Ketones >5mmol/l)

Target blood ketones 2-5mmol/l

Aim is to provide some carbohydrate to stop excess ketosis

Occasionally ketones can become too high. This may occur after starting the diet, if the diet has
recently been modified or during illness. Some children already established on the ketogenic diet
may have had their carbohydrate treatment dose changed on review by the KD team due to
individual response. If individual treatment doses are unknown then use doses suggested below.

Signs of excess ketosis:

- rapid, panting breath (Kaussmaul breathing)
- increased heart rate, facial flushing
- irritability
- vomiting

Oral treatment
A. For Children older than 1 year and over 9kg: Give 5g carbohydrate by giving 1 teaspoon of
sugar or 2 teaspoons jam or 50ml 10% polycal solution (see below).
B For infants under 1 year or under 9kg: Give 2g – 4g carbohydrate3 as 20 – 40ml 10% polycal
solution (see below).

• Recheck ketone levels in 20 minutes, if there is inadequate response the same treatment can
be repeated.
• Recheck ketone levels again in 20 minutes, if ketones are not back in the target range; IV
fluids will be necessary – 5% glucose with 0.9% sodium chloride solution. See section 6.4 for
further information.
• If oral fluids are not tolerated because of vomiting, intravenous fluids 5% glucose with 0.9%
sodium chloride, given as maintenance fluids, are required. See section 6.4 for further
information.
• If IV fluids are required to correct ketone levels then these children will require admission and
bloods as listed in table1.

Nasogastric tube (NGT ) or Gastrostomy (PEG) treatment

Follow the steps above for oral treatment and use 10% Polycal solution as the source of carbohydrate,
given as a flush via the NGT or PEG. If using a jejunostomy feeding tube then give the 10% polycal
solution slowly and with caution.

10% Polycal Solution

• Prepare 10% polycal solution by making 1 level scoop of polycal (available via pharmacy) up to
50ml with water

If ketones are below 2mmol/l. No immediate action required – Dietitian will adjust the diet if
possible to optimise ketosis.

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Title: Ketogenic diet for epilepsy
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NB: Paper copies of this document may not be most recent version. The definitive version is held on InSite in the Policies and Guidelines Library
 6.3) Symptomatic Hypoglycaemia or Blood Glucose <2.5 mmol/l

Target blood glucose: Above 2.5 mmol/l

Aim is to provide some carbohydrate to increase blood glucose levels

Some children already established on the ketogenic diet may have had their carbohydrate
treatment dose changed on review by the KD team due to individual response. If individual
treatment doses are unknown then use doses suggested below.
Occasionally blood glucose levels drop below 2.5 mmol/l, usually as a result of illness.
Check Lab blood glucose and do a finger prick test every 2-4 hours.

Symptoms of hypoglycaemia;
- dizziness, confusion
- aggressive behaviour
- sweating, pallor
- cold and clammy

Oral treatment
A. For Children older than 1 year and over 9kg: Give 10g carbohydrate by giving one of the
following; 2 teaspoons of sugar or 4 teaspoons of jam or 100ml 10% Polycal solution (see below) or
B. 1 tube of 40% Glucogel or Dextrogel (10g glucose per 25g tube) can be squeezed into child’s
mouth if the child is uncooperative or not able to take oral liquids.For infants under 1 year or
under 9kg: Give 2g – 4g carbohydrate3 as 20 – 40ml 10% Polycal solution (see below).

• Recheck blood glucose again in 20 minutes, if it remains below 2.5 mmol/l; IV fluids will be
necessary – 5% glucose with 0.9% sodium chloride solution. See section 6.4 for further
information.
• If oral fluids are not tolerated because of vomiting, intravenous fluids 5% glucose with 0.9% sodium
chloride, given as maintenance fluids, are required. See section 6.4 for further information.
• If IV fluids are required to correct ketone levels then these children will require admission and bloods
as listed in table1.
• For patients with reduced level of consciousness or seizures. Give 5-10ml/kg of 10% glucose
intravenously according to UK Resuscitation Council guidelines.

Nasogastric tube (NGT) or Gastrostomy (PEG) treatment

•
Follow the steps above for oral treatment and use 10% Polycal solution as the source of carbohydrate,
given as a flush via the NGT or PEG. If using a jejunostomy feeding tube then give the 10% polycal
solution slowly and with caution.

10% Polycal Solution

• Prepare 10% polycal solution by making 2 level scoops of polycal (available via pharmacy) up to
100ml with water

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Title: Ketogenic diet for epilepsy
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 6.4) Choice of IV fluids if required

Target blood glucose: Above 2.5 mmol/l Target blood ketones 2-5mmol/l

If ketones and blood glucose are “within the target” range

• Use 0.9% sodium chloride
• An infant should not be nil by mouth and without IV glucose for more than 6 hours.3
• Check ketones 2-4 hourly, if ketones are rising always check glucose (in addition to ketones) if
on IV fluids (as likely ill, feed withheld etc.).
• If ketones are decreasing below the ideal range of 2-5 mmol/l – no immediate action required.
• Inform the Dietitian and check if any new medications introduced have high levels of
carbohydrate and switch preparation to reduce the carbohydrate level if possible.

If ketones are high (hyperketosis >5mmol/l) and/or blood glucose is low

(hypoglycaemia < 2.5mmol/l)
• Use 5% glucose with 0.9% sodium chloride solution
• Check ketones and blood glucose 2 hourly
• Once ketones have decreased to less than 4.0mmol/l and blood glucose is above 2.5mmol/l
change IV fluids to 0.9% sodium chloride if IV fluids are still required otherwise return to previous
KD plan. Otherwise ketosis may be lost and any positive effect of KD on seizure control will be
lost. An infant should not be nil by mouth and without IV glucose for more than 6 hours.3

6.5) Sedation for Procedures and General Anaesthesia 6,7

• Inform Paediatric Neurology team (via switchboard) and Specialist Dietitian (ext 15400)

• Medications: Choose the lowest carbohydrate form possible (generally dissolvable/crushable

tablets or powder preparations) Contact Pharmacists via dialling #6737

• Contact Medicines Information (LRI ext 16191/16491) to begin checking the carbohydrate
content of medications

• Ensure the patient is first on the morning surgical list to reduce the risk of hyperketosis and
hypoglycaemia

• Test blood glucose and ketones 4 or 8 hourly (at discretion of clinician)

• Take bloods: FBC, U/E, bicarbonate, LFT, urinalysis, blood gas, glucose, lactate.

• For general anaesthetic, keep NBM for normal recommended time period
(Food 6 hours and clear fluids for 1 hour)

• If IV fluids are required give 0.9% Sodium Chloride unless hyperketotic or hypoglycemic

• If anaesthetic is >3 hours monitor blood glucose and blood gas (PH and bicarbonate) 1-2
hourly.

• Consider Sodium bicarbonate if increase in acidosis.

• If fasting beyond 12 hours or blood glucose < 2.5 mmol/l use 5% glucose with 0.9% sodium
chloride solution to maintain blood glucose between 2.5 and 4 mmol/L.

• Reintroduce normal ketogenic diet as soon as possible.

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 6.6) Children on Ketogenic diet admitted to PICU

• Inform the Specialist Dietitian on extension 15400 and the Paediatric Neurology team via
switchboard.

• See section 6.4 for choice of IV fluids

• Monitor blood glucose levels and blood gas 1-2 hourly as appropriate

• Monitor ketone levels 4 hourly, excess ketosis and acidosis may require treatment with IV
sodium bicarbonate

• A base excess of -10 indicates significant metabolic acidosis and should be half corrected over
4 hours with IV sodium bicarbonate.

- If acidosis is explained by excess ketosis, glucose containing maintenance fluids

would be appropriate.

- If acidosis is not completely explained by excess ketosis i.e., high blood lactate this
requires further advice from the Metabolic Team.

• Medications: Choose the lowest carbohydrate form possible (generally dissolvable/crushable

tablets or powder preparations) Contact Pharmacists via dialling #6737

• Contact Medicines Information (LRI ext 16191/16491) to begin checking the carbohydrate
content of medications

• Enteral feeding: Inform the Specialist Dietitian on extension 15400. If the child is usually
gastrostomy or NGT fed the Milk Kitchen will provide the child’s usual ketogenic feeds for you. It
is essential that the parents or dietitian provide the latest dietary prescription to the milk kitchen
to avoid using a previous plan. If the patient is usually orally fed and requires NGT feeding the
Dietitian will calculate an appropriate ketogenic enteral feed recipe (note there is not a standard
ketogenic enteral feed that can be used as a starter plan for these patients).

7. Formulation of Medication:

7.1 Levetiracetam Granules (Desitrend) are a

low-Carbohydrate preparation for those children on Levetiracetam
liquid formulation.

7.2 In case of emergencies, kindly give anti-epileptics / antibiotics IV formulation so

Carbohydrate content of the medication does not become an issue at that time of
emergency.

7.3 Paracetamol & Ibuprofen formulations (while child on Ketogenic Diet)

(See Appendix C)

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Title: Ketogenic diet for epilepsy
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 8. Education & Training

None

9. Monitoring Compliance
None currently identified
What will be measured How will compliance Monitoring Frequency Reporting
to monitor compliance be monitored Lead arrangements

10. Supporting References

1. NICE 2022. NG217 Epilepsies in children, young people and adults (nice.org.uk)

2. Neal et al., 2008. The ketogenic diet for the treatment of childhood epilepsy: a
randomised controlled trial. Lancet Neurology; 7, 500-06 .

3. Kossof, E.H., 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) pp 175-192.

4. Van der Louw, E., et al. 2016. Ketogenic diet guidelines for infants with refractory
epilepsy. Eur J Paed Neurol. 20: 798-809.

5. Hartman, A.L. & Vining, E.P.G. (2007) Clinical aspects of ketogenic diet. Epilepsia 48
(1): p31-42.

6. The ketogenic diet in the management of epilepsy. Clinical Guidelines 2009.

Reviewed 2015 Great Ormond Street Hospital for Children

7. The Charlie Foundation .Professionals guide to the ketogenic diet. Protocols for
initiation and management. 2007.

8. Valencia I, Pfeifer H, Thiele EA. . (2002) General anesthesia and the ketogenic
diet: clinical experience in nine patients. Epilepsia. 43(5):525-9.

9. Ketogenic diet for the treatment of refractory epilepsy in children: A systematic

review of efficacy.

10. Lefevre F1, Aronson N. Pediatrics. 2000 Apr;105(4):E46.

11. A randomized trial of classical and medium-chain triglyceride ketogenic diets in the
treatment of childhood epilepsy. Neal EG1, Chaffe H, Schwartz RH, Lawson MS,
Edwards N, Fitzsimmons G, Whitney A, Cross JH. Epilepsia. 2009
May;50(5):1109-17. doi: 10.1111/j.1528-1167.2008.01870.x. Epub 2008 Nov 19

12. Optimal clinical management of children receiving the ketogenic diet:

recommendations of the International Ketogenic Diet Study Group; Epilepsia. 2009
Feb;50(2):304-17. doi: 10.1111/j.1528-1167.2008.01765.x. Epub 2008 Sep 23.

13. National Institute for Health & Care Excellence. February 2016. Epilepsies:
Diagnosis and management. CG137/1.12.1

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 14. Updates from Northern Meeting for Ketogenic Dietitians June 2015

15. Lord K & Magrath G (2010). Use of the ketogenic diet and dietary practices in the
UK. The Journal of Human Nutrition and Dietetics, volume 23, p 126-132.

https://onlinelibrary.wiley.com/doi/pdf/10.1111/j.1365-277X.2010.01040.x

16. Neal EG & Cross JH (2010). Efficacy of dietary treatments for epilepsy. The
Journal of Human Nutrition and Dietetics, volume 23 (2), p 113-119.
https://onlinelibrary.wiley.com/doi/full/10.1111/j.1365-277X.2010.01043.x

17. Joo HS, Young ML, Joon SL, Hoon CK, Heung DK (2007). Efficacy and Tolerability
of the Ketogenic Diet According to Lipid:Nonlipid Ratios—Comparison of 3:1 with
4:1 Diet. Epilepsia, 48(4), p 801-805.
https://onlinelibrary.wiley.com/doi/full/10.1111/j.1528-1167.2007.01025.x

18. Progressive bone mineral content loss in children with intractable epilepsy treated
with the ketogenic diet; AG Christina Bergqvist Joan I Schall Virginia A Stallings
Babette S Zemel; The American Journal of Clinical Nutrition, Volume 88, Issue 6,
December 2008, Pages 1678–1684, https://doi.org/10.3945/ajcn.2008.26099

19. Clinical Guideline on the Dietary Management of Children with Intractable Epilepsy
Treated with Ketogenic Diet (KD) V1 Approved by CSI Quality & Safety meeting on
10/05/2017 Trust Ref: C22/2017

UHL Intravenous policy B25/2010

UHL Hand hygiene policy B32/2003

UHL Personal protective equipment guideline B9/2004

UHL Sharps policy B8/2013

UHL Infection prevention policy B4/2005

11. Key Words

Blood Glucose, Dietitian, Hyperketosis, Hypoglycaemia, Ketogenic diet, Ketones, Epilepsy

The Trust recognises the diversity of the local community it serves. Our aim therefore
is to provide a safe environment free from discrimination and treat all individuals
fairly with dignity and appropriately according to their needs.
As part of its development, this policy and its impact on equality have been reviewed
and no detriment was identified.

Page 13 of 20
Title: Ketogenic diet for epilepsy
V:4 Approved by Children’s Clinical Practice Group on: December 2022 Trust Ref: C255/2016 Next Review: December 2025
NB: Paper copies of this document may not be most recent version. The definitive version is held on InSite in the Policies and Guidelines Library
 Contact and review details
Guideline Lead: Executive Lead
Dr Rajib Samanta FRCPCH(UK), MRCP(UK), DCH(UK), CCT (UK) Chief Nurse
Consultant Paediatric Neurologist

Changes made during review;

Page 4 – Urine investigations, amino and organic acids monitored once as baseline only rather than
every 6 months.
Page 9 6.4 IV fluids – if ketones are rising, blood glucose testing changed from ‘begin to check BG’ to
always check glucose (in addition to ketones) if on IV fluids. If hyperketosis and/or hypoglycaemia,
monitoring/treatment changed to - Once ketones have decreased to less than 4.0mmol/l (previously 3-
4mmol/l) and blood glucose is above 2.5mmol/l change IV fluids to normal saline – added, ‘if IV fluids
are still required otherwise return to previous KD plan.’
Updated references
Converted all references to dextrose to glucose, and normal saline now 0.9% sodium chloride in line
with Trust recommendations.
Added preceding 1 to all UHL extension numbers
Removed appendix B – indications and contraindications for Ketogenic diet as in the main body of the
guideline section 2
Added appendix D – Ketogenic diet bloods
Added appendix E – Clinic assessment proforma

Appendix A - Types of Ketogenic Diet:

CLASSICAL KETOGENIC DIET (KD)

The classical KD is calculated in a ratio of grams of fat to grams of protein plus
carbohydrate. It is the strictest of all of the KDs, in that it allows the least amount of
carbohydrates alongside prescribed amounts of fat and protein. The fat source is usually
long chain triglycerides (LCT). Children who are exclusively enterally fed will have their diets
calculated as a classical KD and therefore expressed as a ratio. It is also the recommended
choice in infants because of the greater level of control over constituents of the diet.

MEDIUM CHAIN TRIGLYCERIDE (MCT) DIET

This diet has up to 60% of energy derived from MCT fat. MCT fats are thought to yield more
ketones per kcal of energy than LCT. This increased ketone potential allows less total fat to
be used, and slightly more protein and carbohydrate than the classical KD. However, MCT
fat can cause gastrointestinal discomfort in some children. This diet is also more complex for
families in its calculations than other KDs.

MODIFIED KETOGENIC DIET

Carbohydrate is restricted to 10-20g daily so generally a lower allowance than on the MCT
KD. Uniquely, protein foods which are not sources of carbohydrate are not restricted and do
not have to be weighed or measured making this the most liberal KD. A recommended
number of fat choices are prescribed at UHL, although not all centres in the UK give specific
advice on the quantity of fat to consume. Some centres simply encourage high fat foods at
every meal. Quantified advice on fat is given at UHL to support parents to understand the
very large amounts of fat required and to facilitate effective manipulation of the diet when
required. MCT fats can be incorporated into the modified KD to increase ketones.

Page 14 of 20
Title: Ketogenic diet for epilepsy
V:4 Approved by Children’s Clinical Practice Group on: December 2022 Trust Ref: C255/2016 Next Review: December 2025
NB: Paper copies of this document may not be most recent version. The definitive version is held on InSite in the Policies and Guidelines Library
 Appendix B: Adverse Effects of Ketogenic Diet 2

• Gastrointestinal system and are often seen during the initial few weeks of dietary
therapy. Constipation, emesis, and abdominal pain may occur in up to 50% of children.
These symptoms are usually mild and easy to correct with minimal interventions. When
adequately managed and prevented, gastrointestinal side effects are rarely a reason to
discontinue KDT.

• Hyperlipidemia is a well-known side effect of almost all KDT. Increased serum

triglycerides and total and low-density lipoprotein (LDL) cholesterol levels have been
reported in 14–59% of children on the classic KD. Hyperlipidemia can be seen as early as
the first month of therapy. An early increase in serum lipids during the first months of KDT,
this increase is usually temporary. In one study, 60% of those on the classic KD had
hypercholesterolemia (>200 mg/dl). By 12 months, the serum lipid values often normalize
and remain within normal limits. Strategies to prevent KD-induced hyperlipidemia include
increasing consumption of MCT and olive oil, supplementing with omega-3 fatty acid or
carnitine while decreasing the intake of trans fat, saturated fat, and cholesterol; decreasing
the KD ratio; and excluding all fatty meats, egg yolk, cream, butter, animal fat, palm oil and
coconut oil; and using a solely formula-based KD.

• Risk for coronary artery disease may increase with long-term elevations of
cholesterol levels, previous paediatric studies showed no change in the carotid intima-media
thickness compared to baseline at 6 and 12 months of therapy. Nevertheless, long-term
vascular outcomes of this high fat diet are not known.

• Renal calculi occurs in approx. 3–7% of children on KDT. They typically do not
require KDT discontinuation and lithotripsy is necessary only rarely. As previously stated,
oral citrates appear to help prevent stone formation.

• Growth - There is mixed data on the effect of KDT on growth in children. However, all
six studies with longer than 6 months duration indicate that the classic KD has negative
effects on growth, and over time may cause a height deceleration. One retrospective review
described 86% with slowed growth, and this effect was not related to age, KD duration,
protein, or calorie intake. A prospective study of 237 children found that the while older
children grew “almost normally,” younger children had more difficulties. A small change in
protein, offered by the MCT diet, does not seem to result in better growth.

• Cardiac abnormalities have been reported in children on the KD, including

cardiomyopathy and prolonged QT interval. The mechanism of these complications is not
fully understood; one case was associated with selenium deficiency, but others were not.
Routine ECG is not recommended at this time as a screening test.

• Pancreatitis has also been reported.

• Hepatic dysfunction may be more likely to occur in children who are on both valproic
acid and KDT, with intercurrent viral illness furthering increasing the risk of elevated
transaminases.

• Long-term complications in children maintained on KDT for >2 years;

• Higher risk of bone fractures
• Kidney stones
• Decreased growth
• Dyslipidemia is theoretically possible, but was not identified.

Page 15 of 20
Title: Ketogenic diet for epilepsy
V:4 Approved by Children’s Clinical Practice Group on: December 2022 Trust Ref: C255/2016 Next Review: December 2025
NB: Paper copies of this document may not be most recent version. The definitive version is held on InSite in the Policies and Guidelines Library
Conclusions:
• Like all medical therapies KDTs have potential adverse effects.
• Overall, the risk of serious adverse events is low;
• KDTs do not need to be discontinued for most adverse effects.
• Gastrointestinal complaints are often the most common but can be mostly remedied.

Appendix: C – Paracetamol & Ibuprofen preparations

PARACETAMOL:

CALPOL SIX-PLUS (FASTMELTS) TABLET (250 mg)

Or Crushable Paracetamol tablet

………………. Tablets to be dissolved in ………… mls water.

…….. mls of that solution ( mgs) to be taken 4 (FOUR) times daily or as required.

IBUPROFEN:

NUROFEN MELTLET TABLETS (200mg)

……………… Tablets to be dissolved in ………. mls water.

……….. mls of that solution ( ………mgs) to be taken 3 (THREE) times daily or as required.

The doses above will need to be reviewed as your child’s age changes.
Parents might have these medications at home.
Hence need to remind the parents so they bring those special preparations from home.

These preparations are the preferable choice of Paracetamol and Ibuprofen to minimise the
carbohydrate content from these medicines.
Additionally, these will provide a formulation which is dispersible and easy to administer to a
child orally or via an enteral feeding tube.

Page 16 of 20
Title: Ketogenic diet for epilepsy
V:4 Approved by Children’s Clinical Practice Group on: December 2022 Trust Ref: C255/2016 Next Review: December 2025
NB: Paper copies of this document may not be most recent version. The definitive version is held on InSite in the Policies and Guidelines Library
 Appendix D: Ketogenic Diet Bloods
Form 1 : TEST Venous Blood Sample Required

3-OH BUTYRATE 1 single YELLOW paediatric bottle

Form 2 : TEST Venous Blood Sample Required

Lactate Both samples can be done from 1 single YELLOW
paediatric bottle on ICE straight to lab
Glucose

Form 3 : TEST Venous Blood Sample Required

Acylcarnitines 1 single ORANGE paediatric lith hep bottle
(Nb free and total carnitine removed on advice
from Elaine Maddox)

Form 4 : TEST Venous Blood Sample Required

Vit A ALL SAMPLES can be done from 3 BROWN
paediatric bottles
SE White-topped plain gel-free serum tube (S-
Monovette Serum, 4.9 mL, cap white)
Drug LEVEL eg sodium valproate, phenytoin if
requested by Dr Samanta
Amino Acids *AT BASELINE ONLY* 1 paediatric orange lith hep bottle

Form 5 : TEST Venous Blood Sample Required

INR *AT BASELINE ONLY* 1 paediatric green bottle (1.4mls)
FBC 1 paediatric red EDTA bottle
U & E, LFT, Bone Profile,
Fasting Chol/trig/HDL Take bloods 2 hours fasted

Bicarbonate These could all be done out of 2 FULL brown

Urate bottles
Folate*AT BASELINE ONLY*
Ferritin If you have the possibility of getting an extra
Vit D brown bottle here it would be good as VIT B12
Vit B12 *AT BASELINE ONLY* and D require a good sample.

Form 6 : TEST urine Sample Required

ca:crea (request as Ca and Creatinine seperately) Urine
Organic acids *AT BASELINE ONLY* *To be at lab within 1 hour of sample being taken
Amino Acids *AT BASELINE ONLY*
State on Form 6 (under clinical details) which AEDs patient is on e.g. valproic acid, as these
can affect levels in urine

Page 17 of 20
Title: Ketogenic diet for epilepsy
V:4 Approved by Children’s Clinical Practice Group on: December 2022 Trust Ref: C255/2016 Next Review: December 2025
NB: Paper copies of this document may not be most recent version. The definitive version is held on InSite in the Policies and Guidelines Library
Ketogenic Diet Bloods

• The bottles MUST be FULL.

• The nature of the specimen should always be marked V.B or Venous blood not
SERUM as Sheffield lab will send back samples where forms are incorrectly labelled
as SERUM.
• ALL on Combined CHEM PATH Request forms.

Drug Levels:

If patients are on the following anti-epileptic drugs add the drug name to Form 4 under
other.

Drug name (Brand Names) Drug name (Brand Names)

Sodium valproate Epilim carbamazepine Tegretol
Levetiracetam Keppra oxcarbazepine Trileptal
Topiramate Topamax phenobarbitone N/A
Lamotirigine Lamictal phenytoin Epanutin

Page 18 of 20
Title: Ketogenic diet for epilepsy
V:4 Approved by Children’s Clinical Practice Group on: December 2022 Trust Ref: C255/2016 Next Review: December 2025
NB: Paper copies of this document may not be most recent version. The definitive version is held on InSite in the Policies and Guidelines Library
 Appendix E: Assessment Clinic for Ketogenic Diet (Proforma)

Assessment Clinic for Ketogenic Diet (Proforma)

Keto Diet Started;…………………; Date of review;……………………..

Name: Weight:

DOB: Height:

Hospital No: OFC:

Tel: School:

Diagnosis / Epileptic syndrome /specific Clinical Past Medical History:

conditions

Complications: Anti Epileptic Medications + Others

Drowsy / Lethargy
Irritable
Hyperactivity
Poor attention
Diarrhoea
Constipation
Vomiting

Compliance: Last changes in Medication:

Sleep Hygiene

Hyperketosis: Medications used / stopped in the past

Hypoglycaemia

Page 19 of 20
Title: Ketogenic diet for epilepsy
V:4 Approved by Children’s Clinical Practice Group on: December 2022 Trust Ref: C255/2016 Next Review: December 2025
NB: Paper copies of this document may not be most recent version. The definitive version is held on InSite in the Policies and Guidelines Library
Current Seizure Descriptions Semiology of each type of seizure:
Seizure Type Per Per Per Per
Day week month year
Tonic
TC
Myoclonic
Spasms
Atonic/Drop
attacks
Head drops
Absences
(Typical)
Absences
(Atypical)
Complex
partial Sz
Auras

MRI KD Therapy Annual Surveillance Tests:

RENAL USS date

EEG

Calcium : Creatinine Ratio:

Others Tests:

Outcome of KD treatment: Plan / Referral to other services

Seizure reduction:

Medication reduction

Cognition Behaviour change / improvement

Length of KD treatment at the assessment:

Keto Diet - Weaned / Stopped / Continued Follow Up - 3 month / 6 month,

Page 20 of 20
Title: Ketogenic diet for epilepsy
V:4 Approved by Children’s Clinical Practice Group on: December 2022 Trust Ref: C255/2016 Next Review: December 2025
NB: Paper copies of this document may not be most recent version. The definitive version is held on InSite in the Policies and Guidelines Library