LRI Children s Hospital

Transcription

1 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: May 2016 Version: V 2 Revision due: May 2019 Written by: Dr R Samanta, Dr N Hussain, E Wilford & R Fox Trust Ref: C255/ Introduction/Scope 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 Related 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. Title: Management of Children on Ketogenic Diet for Epilepsy Page 1 of 14

2 3. 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. Ketogenic diet is indicated for: 1. Children whose seizures fail to respond to at least 2 antiepileptic medications in therapeutic doses 2. For certain epilepsy syndromes early in their course Lennox-Gastaut syndrome Myoclonic-astatic epilepsy Dravet syndrome (severe myoclonic epilepsy of infancy) Infantile spasms/west syndrome Symptomatic epilepsies of epilepsy surgery is not indicated 3. For children with metabolic disorders: Glucose Transporter Type 1 (GLUT 1) Deficiency Pyruvate Dehydrogenase (PDH) Deficiency For children who have intolerable and/or severe effects from antiepileptic medication 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 KD. 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/cessation. Title: Management of Children on Ketogenic Diet for Epilepsy Page 2 of 14

3 Once the child is established on an effective and appropriate KD, weaning of the other AEDs is considered There are different forms of KD regimes: The Classical KD The Medium Chain Triglyceride diet (MCT diet) The Modified Atkins Diet (MAD) 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 Blood Investigation Essential: Full blood count Fasting cholesterol & triglyceride Ferritin, amino acid profile U&E,bicarbonate, lactate, albumin Urate Liver function tests Calcium, phosphate Magnesium Glucose Free and total Carnitine Acylcarnitine profile Vitamin A, D & E Zinc, Selenium, Copper Anti-convulsant level Frequency of monitoring Baseline, 3 months, 6 months and then every 6 months. Clotting screen Baseline, 6 months, and then every 12 months. Title: Management of Children on Ketogenic Diet for Epilepsy Page 3 of 14

4 Recommended: Blood ketones Free fatty acids Optional: Vitamin C Vitamin B12, Folate Urine Investigation Baseline, 3 months, 6 months, and then every 6 months. Baseline, 6 months, and then every 12 months. Frequency of monitoring Calcium:Creatinine ratio Amino Acids Organic Acids Porphyrins Baseline, 3 months, 6 months, and then every 6 months. Baseline Side Effects of the Ketogenic Diet 4 - Vomiting, constipation, lack of energy, hunger and diarrhoea (these can generally be resolved with dietary manipulation). - Elevated serum lipids, excess ketosis and acidosis. - Renal stones, growth delay. - Selenium deficiency, poor vitamin D status which can compromise bone health. Title: Management of Children on Ketogenic Diet for Epilepsy Page 4 of 14

5 Principles of Management for Inpatient Admissions Inform Neurology team (by switchboard) and Specialist Dietician (ext 5400) Bloods FBC, U/E, bicarbonate, blood gas, lactate, LFT, blood and urinary ketones, infection screen Target ketones 2-5mmol/l 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 Dietician reviews and calculates a suitable ketogenic feed recipe there is no standard ketogenic feed for these patients. Medications: choose the lowest carbohydrate form possible (generally dissolvable/crushable tablets or powder preparations) Contact Pharmacists via dialling #6737 Medicine Information (LRI ext 6191/6491) to begin checking the carbohydrate content of medications Title: Management of Children on Ketogenic Diet for Epilepsy Page 5 of 14

6 Inpatient Management of Children on Ketogenic Diet 5 (Adapted from Ketogenic Diet in the management of Epilepsy; Clinical Guidelines. Great Ormond Street Hospital 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 6191/6491) 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 and urinary ketones, infection screen. On admission refer to the specialist dietician 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 2-4 hourly. Maintain blood glucose above 2.5 mmol/l Maintain blood ketones less than 5 mmol/l (refer to sections 2-4 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 Dietician first as it contains glucose The Dietician 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. Title: Management of Children on Ketogenic Diet for Epilepsy Page 6 of 14

7 2) Hyperketosis (Ketones > 5mmol/l) Target urine ketones: 8-16mmol/ 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. Signs of excess ketosis: - rapid, panting breath (Kaussmaul breathing) - increased heart rate, facial flushing - irritability - vomiting Oral treatment Give 50ml of pure fruit juice or NON DIET cola or lemonade or 25ml Lucozade Energy Original 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% dextrose with 0.45% saline solution. See section 4 for further information. If oral fluids are not tolerated because of vomiting, intravenous fluids 5% dextrose with 0.45% saline, given as maintenance fluids, are required. See section 4 for further information. These children will require admission and bloods as listed in section 1. Nasogastric tube (NGT ) or Gastrostomy (PEG) treatment Dissolve 1 level scoop of Polycal powder in 50ml of water and administer via the NGT/PEG 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% dextrose with 0.45% saline solution. See section 4 for further information. These children will require admission and bloods as listed in section 1. Title: Management of Children on Ketogenic Diet for Epilepsy Page 7 of 14

8 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 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 One of these rapidly absorbed carbohydrate sources should be used; ml of NON DIET cola or lemonade ml fruit juice - 55 ml of Lucozade Energy Original - 2 tsp of sugar or jam - 1 tube of 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 Recheck blood glucose again in 20 minutes, if it remains below 2.5 mmol/l; repeat the initial treatment. Recheck blood glucose again in 20 minutes, If it still remains below 2.5 mmol/l; IV fluids will be necessary 5% dextrose with 0.45% saline solution. See section 4 for further information. If oral fluids are not tolerated because of vomiting, intravenous fluids 5% dextrose with 0.45% saline, given as maintenance fluids, are required. See section 4 for further information. These children will require admission and bloods as listed in section 1. For patients with reduced level of consciousness or seizures. Give 5-10ml/kg of 10% Dextrose intravenously according to UK Resuscitation Council guidelines. Nasogastric tube (NGT) or Gastrostomy (PEG) treatment Dissolve 2 level scoops of Polycal powder in 50ml water and administer via the NGT/gastrostomy. NB Polycal is available from pharmacy. Recheck blood glucose again in 20 minutes, if it remains below 2.5 mmol/l; repeat the initial treatment. Recheck blood glucose again in 20 minutes, if blood glucose levels are not normalising with the above measures, IV fluids will be necessary 5% dextrose with 0.45% saline solution. See section 4 for further information. Title: Management of Children on Ketogenic Diet for Epilepsy Page 8 of 14

9 4) Choice of IV fluids if required Target urine ketones: 8-16mmol/l 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 normal saline (0.9% sodium chloride) Check ketones 2-4 hourly, if ketones are rising begin to check blood glucose also If ketones are decreasing below the ideal range of 2-5mmol/l no immediate action required. Inform the Dietician 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% dextrose with 0.45% saline solution Check ketones and blood glucose 2 hourly Once ketones have decreased to 3-4mmol/l and/or blood glucose is above 2.5mmol/l change IV fluids to normal saline; otherwise ketosis may be lost and any positive effect of KD on seizure control will be lost. Title: Management of Children on Ketogenic Diet for Epilepsy Page 9 of 14

10 5) Sedation for Procedures and General Anaesthesia 6,7 Inform Paediatric Neurology team (via switchboard) and Specialist Dietician (ext 5400) Medications: choose the lowest carbohydrate form possible (generally dissolvable/crushable tablets or powder preparations) Contact Pharmacists via dialling #6737 Medicine Information (LRI ext 6191/6491) 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 hourly 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 2 hours) If IV fluids are required give normal saline (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% dextrose with 0.45% saline solution to maintain blood glucose between 2.5 and 4 mmol/l. Reintroduce normal ketogenic diet as soon as possible. Title: Management of Children on Ketogenic Diet for Epilepsy Page 10 of 14

11 6) Children on Ketogenic diet admitted to PICU Inform the Specialist Dietician on extension 5400 and the Paediatric Neurology team via switchboard. See section 4 for choice of IV fluids Monitor blood glucose levels and blood gas 1-2 hourly as appropriate Monitor ketone levels 2-4 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, dextrose containing maintenance fluids would be appropriate. - If acidosis is not completely explained by excess ketosis i.e., high blood lactate this requires further advise from the Metabolic Team. Medications: choose the lowest carbohydrate form possible (generally dissolvable/crushable tablets or powder preparations) Contact Pharmacists via dialling #6737 Medicine Information (LRI ext 6191/6491) to begin checking the carbohydrate content of medications Enteral feeding: Inform the Specialist Dietician on extension If the child is usually gastrostomy or NGT fed the Milk Kitchen will provide the child s usual ketogenic feeds for you. If the patient is not 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). Title: Management of Children on Ketogenic Diet for Epilepsy Page 11 of 14

12 6. Legal Liability Guideline Statement Guidelines issued and approved by the Trust are considered to represent best practice. Staff may only exceptionally depart from any relevant Trust guidelines providing always that such departure is confined to the specific needs of individual circumstances. In healthcare delivery such departure shall only be undertaken where, in the judgement of the responsible healthcare professional it is fully appropriate and justifiable - such decision to be fully recorded in the patient s notes. 7. Equality Statement 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/guideline and its impact on equality have been reviewed and no detriment was identified. 8. Supporting and Key References 1) Neal et al., The ketogenic diet for the treatment of childhood epilepsy: a randomised controlled trial. Lancet Neurology; 7, ) Kossof, E.H. et al., Optimal clinical management of children receiving the ketogenic diet: Recommendations of the International Ketogenic Diet Study Group. Epilepsia, 50 (2) pp ) Matthews Friends 4) Hartman, A.L. & Vining, E.P.G. (2007) Clinical aspects of ketogenic diet. Epilepsia 48 (1): p ) The ketogenic diet in the management of epilepsy. Clinical Guidelines Reviewed 2015 Great Ormond Street Hospital for Children 6) The Charlie Foundation.Professionals guide to the ketogenic diet. Protocols for initiation and management ) Valencia I, Pfeifer H, Thiele EA.. (2002) General anesthesia and the ketogenic diet: clinical experience in nine patients. Epilepsia. 43(5): Title: Management of Children on Ketogenic Diet for Epilepsy Page 12 of 14

13 8) Ketogenic diet for the treatment of refractory epilepsy in children: A systematic review of efficacy. Lefevre F1, Aronson N. Pediatrics Apr;105(4):E46. 9) 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 May;50(5): doi: /j x. Epub 2008 Nov 19 10) Optimal clinical management of children receiving the ketogenic diet: recommendations of the International Ketogenic Diet Study Group; Epilepsia Feb;50(2): doi: /j x. Epub 2008 Sep ) National Institute for Health & Care Excellence. February Epilepsies: Diagnosis and management. CG137/ ) Updates from Northern Meeting for Ketogenic Dieticians June ) UHL Intravenous policy B25/ ) UHL Hand hygiene policy B32/ ) UHL Personal protective equipment guideline B9/ ) UHL Sharps policy B8/ ) UHL Infection prevention policy B4/ Key Words Ketogenic diet, Epilepsy Title: Management of Children on Ketogenic Diet for Epilepsy Page 13 of 14

14 Author / Lead Officer: Reviewed by: Approved by: DEVELOPMENT AND APPROVAL RECORD FOR THIS DOCUMENT Dr N Hussain, Dr A Khan, J Kearns Dr R Samanta, Dr N Hussain, E Wilford & R Fox Acknowledgement: D Harris principal paediatric pharmacist Children s Clinical Governance Group REVIEW RECORD Date Issue Reviewed By Description Of Changes (If Any) Number Aug May May Dr R Samanta Changed normal ketones to target ketones, normal blood glucose level to target blood glucose level. Target blood glucose level reduced from 3mmol/l and above to 2.5mmol/l and above Recommendation to select lowest carbohydrate medications and seek pharmacy advice Inclusion of medicine information contact numbers Addition of dietician ICE referral Signpost to use switchboard to contact Paediatric neurology team Inclusion of related UHL policy documents Updated reference list DISTRIBUTION RECORD: Date Name Dept Received Title: Management of Children on Ketogenic Diet for Epilepsy Page 14 of 14