NHD Issue 149 Ketogenic diet in an adult inpatient setting a case report

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KETOGENIC DIET IN AN ADULT INPATIENT SETTING: A CASE REPORT

This case report aims to describe the lessons learned from trialling a ketogenic diet in a complex adult inpatient with super refractory status epilepticus (SRSE), referred to as ‘patient A’ in the interest of confidentiality. The ketogenic diet is a high fat, low carbohydrate and adequateprotein diet recognised as an effective treatment for drug-resistant epilepsy in children.1 It mimics the starvation mode, thus inducing the production of ketones. Despite its established success in this area, the evidence base for the use of a ketogenic diet in the treatment of adults with SRSE is more limited. SRSE is where status epilepticus (SE)

continues for 24 hours or more after the initiation of anaesthetic therapy and includes cases that recur upon the weaning of anaesthetic agents. Several case reports and cohort studies have provided preliminary evidence for the feasibility, safety and effectiveness of treating SRSE with a ketogenic diet in adults.2-4 However, further randomised, placebo-controlled trials are warranted to fully establish its efficacy.

CASE REPORT

NUTRITION MANAGEMENT

Emma Jones RD Royal Stoke University Hospital

Emma has an interest in major trauma and specialised rehabilitation. Her current Band 6 role covers trauma, orthopaedic and neurosurgical wards, including the hyper-acute specialised rehabilitation unit within the Royal Stoke major trauma centre.

Patient A was admitted to the Royal Stoke University Hospital following a road traffic accident, in which she sustained a traumatic subarachnoid haemorrhage, acute subdural haematoma and multiple fractures. She remained nil by mouth and following a period of nasogastric (NG) feeding, a percutaneous endoscopic gastrostomy (PEG) was inserted to meet her full nutritional needs. Enteral feeding was tolerated well and her weight had been stable at approximately 60kg (BMI 23kg/m2) via a regime of 1260ml of Jevity Plus daily. Five months post-admission and whilst on an acute rehab ward, patient A went into SE; her seizures became refractory to medical treatment and she required reintubation. EEGs confirmed ongoing partial status and, after multiple unsuccessful attempts at extubation, a tracheostomy was reinserted. Patient A was subsequently transferred from Critical Care for specialised rehabilitation, where she remained clinically stable, although in partial status.

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Having exhausted anaesthetic and anti-epileptic drug therapies, her husband raised the possibility of a ketogenic diet as potential treatment option. Following multiple multidisciplinary team (MDT) discussions, the decision was made to commence a ketogenic diet trial, with the aim of resolving her SRSE. MDT WORKING The implementation of the ketogenic diet required extensive MDT working from the onset. Having no personal experience of ketogenic diets in practice, or guidelines within our NHS Trust, I initially made contact with a specialist paediatric dietetic team who provided invaluable information regarding blood monitoring and treatment protocols. This included the bloods to be checked prior to commencing the diet, target ketone range and frequency of monitoring, as well as the treatment of potential complications such as hyperketosis and hypoglycaemia. Blood glucose and ketone levels were monitored every four hours, with the aim of achieving and maintaining a therapeutic level of ketosis – 2-5mmol/l – as advised by the specialist dietetic team. A treatment protocol was devised in case of hyperketosis or hypoglycaemia, whereby the carbohydrate supplement Polycal would be administered.

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NUTRITION MANAGEMENT Medications can provide a significant source of carbohydrate, and thus regular liaison with medicines information and the ward pharmacist was required in order to keep this to a minimum. Information from Matthew’s Friends5 (UK registered charity specialising exclusively in medical ketogenic diet therapies) and the Great Ormand Street website6 were particularly useful with regards to this. On occasion, the manufacturers of certain drugs were also contacted directly. Where appropriate, carbohydrate content was reduced by changing liquid formulations – often high in sorbitol – to crushed tablets. The ketogenic feed was devised using individual components for protein and fat – Renapro powder and Calogen neutral respectively – with appropriate vitamin and mineral supplementation. As these are non-stock items in our Trust, this required regular liaison with both supplies and pharmacy. The administration of a modular feed, as well as ketogenic blood monitoring, was also new to the nursing staff on the ward. To ensure safe and consistent delivery, I spoke with nursing staff daily throughout the treatment period, as well as ensuring that all senior staff were kept up to date. Besides nursing staff, close liaison with the medical team was key to the safe implementation of the diet; this included regular updates regarding progress, agreeing goals and outcome measures and advising on vitamin and mineral supplementation. FEED TOLERANCE AND ADMINISTRATION A bolus regime was initially devised, consisting of both regular small boluses of Calogen neutral and dissolved Renapro powder (see Table 1). This was, however, poorly tolerated and ceased after day 3 due to recurrent vomiting. Following discussion with the MDT, it was agreed to recommence the diet trial with two new strategies: a gradual introduction of the ketogenic diet using a step-wise approach (weaning the standard feed whilst gradually increasing the fat content); and using a flexitainer and pump for a slower delivery (see Table 2). The introductory process lasted for six days and no further vomiting occurred. Although the continuous feed was well tolerated, technical issues were experienced with regards to pump delivery. Namely, the feeding pump regularly alarmed to say ‘feed empty’ shortly after commencing. Following discussion with our Abbott Nurse, this was felt to be caused by air in the mixed feed and was resolved by leaving the feed to ‘settle’ for approximately one hour prior to administration. Table 1: Initial bolus regime Time

Feed

6am

50ml Calogen neutral; 1 x Renapro sachet

10am

50ml Calogen neutral

Noon

50ml Calogen neutral; 1 x Renapro sachet

3pm

50ml Calogen neutral

6pm

50ml Calogen neutral

9pm

50ml Calogen neutral; 1 x Renapro sachet

Table 2: Example of continuous regime Time

Feed

6am10am

Flexitainer: 150ml Calogen neutral 1 x Renapro sachet (1 x 20g) mixed in 150ml water (use shaker to mix) Run at 75ml/hour x 4 hours

5pm9pm

Flexitainer: 150ml Calogen neutral 1 x Renapro sachet (1 x 20g) mixed in 150ml water (use shaker to mix) Run at 75ml/hour x 4 hours

MEETING NUTRITIONAL REQUIREMENTS The use of a modular feed required careful consideration of vitamin and mineral supplementation. Whilst Forceval soluble met the RNI for most vitamins and minerals, it fell significantly short of calcium, potassium, magnesium and phosphorus. Liaison with the medical team enabled appropriate prophylactic supplementation to be prescribed and bloods were regularly monitored (see Table 3).

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NUTRITION MANAGEMENT Table 3: Prophylactic mineral supplementation Mineral

LRNI

RNI

Requirement/ kg (enteral)

Provision

Calcium

10mmol

17.5mmol

N/A

Forceval: 3mmol AdCal: 15mg

Potassium

50mmol

90mmol

1.0mmol/kg (=60mmol)

Forceval: 7.7mmol Renapro: 1.8mmol Sando K: 48 mmol

N/A

Forceval: 2.3mmol Renapro: 0.3mmol Magnesium aspartate: 10mmol

N/A

Forceval: 105mg Renapro: 44mg Phosphate Sandoz: 500mg

Magnesium

6.2mmol

Phosphorus

N/A

10.9mmol

550mg

OPTIMISING KETONE PRODUCTION Maintaining optimal blood ketone levels proved challenging, resulting in further liaison with the specialist dietetic team and several alterations to the treatment plan. Ketone levels were rarely within the desired therapeutic range (2-5mmol/l), with a ketogenic ratio of just over 2:1, and all options for reducing the carbohydrate content of medications had been exhausted. To achieve a higher ketogenic ratio and upon advice from the specialist dietetic team, protein intake was temporarily reduced to 36g per day (equivalent to approximately 0.6g/kg of body weight). Optimal ketone levels were largely achieved, but began to reduce approximately three weeks into the diet and no cause could be identified. We had exhausted minimising the carbohydrate content of medications and, thus, the decision was made to trial adding medium-chain triglycerides (MCT) in the form of Liquigen. MCT yields higher levels of ketones per kilocalorie than long-chain triglycerides (LCT) and is, therefore, commonly used in ketogenic diet therapy. Unfortunately, this did not have the desired effect of optimising ketone production and optimal ketone levels became very hard to maintain. OUTCOME Although some subtle changes were noted by the therapy team – for example, a higher WHIM (Wessex Head Injury Matrix) score – these were not consistent, and repeat EEGs confirmed that the patient remained in partial SE. It was therefore agreed with the MDT and patient’s family that there was no clinical indication to remain on the ketogenic diet trial. A standard feed was gradually reintroduced, and several months later the patient was discharged to a care facility. CONCLUSION The safe implementation of a ketogenic diet requires extensive MDT working and robust treatment protocols. As its use in adults with SRSE is very sparse, the adaption of paediatric guidelines proved necessary and invaluable. Although the ketogenic diet proved unsuccessful at resolving seizure activity in this case, there are multiple case reports which have shown its efficacy and, thus, further research is warranted.

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