P KETOGENIC DIET X INTERCT ILLNESS X KRH

Background information about ketogenic diet:

Ketogenic Diet (KD)is now an established treatment for management of children with epilepsy. It is a high fat, low carbohydrate (usually less than 20 grams of carbohydrate per day), medically supervised diet. The diet aims to switch the brain’s fuel substrate from glucose to ketones, which are produced from the breakdown of fatty acids.

Children often have blood glucose levels of between 3 – 4mmol/L without symptoms.

The diet is generally well tolerated. Complications include vomiting, reflux, constipation, renal stones (3-6% long term), osteopenia (long term), pancreatitis (rarely)

Common reasons for hospital presentation in children on KD include febrile illness, acute gastroenteritis, increased seizures, excess ketosis, metabolic acidosis, hypoglycaemia

Recommended Investigations:

In addition to other bloods required as part of assessment of their illness, FBC, Renal function, Bicarbonate, Liver function, Lactate and blood gas would be recommended.

Hydration:

Oral and Nasogastric feeding

·         Avoid drinks containing carbohydrate (e.g. fruit juice, milk, soft drinks). Acceptable ketogenic fluids include water, sugar free squash, double cream mixed with water (patient specific recipe) and KetoCal. Each patient will have an emergency KetoCal or milkshake feeding plan.

·         If additional electrolytes are required due to  dehydration, oral rehydration solution may be given for each loose bowel motion or vomit. Up to 4 dioralyte sachets per day is acceptable.

Intravenous rehydration

·         Use normal saline (0.9% NaCl)

·         If blood glucose <2.9 mmol/L, use 5% dextrose with 0.9%saline solution

Medications:

·         Medication can contain a significant amount of carbohydrate which can disrupt ketosis. Work closely with the pharmacist to minimise carbohydrates from medications. Where possible avoid the use of syrups and elixirs which are often high in carbohydrates.

·         Antibiotics are frequently high in carbohydrate.

·         With the exception of cellulose, substances ending in “ose” or “ol” are usually converted to glucose in the body. This includes sorbitol. Medications using saccharin or those used in suppositories are suitable.

·         Monitor blood or urine ketones 4 hours post dose if starting any new medication

·         If  medication containing carbohydrates have to be given, please notify the dietitian, so that the diet can be altered to accommodate this.

Analgesia

·         Paracetamol or ­Ibuprofen (non-coated) in tablet form is recommended. The tablets can be crushed and mixed with water. Suppositories of Paracetamol or Diclofenac could be used.

·         Some children may tolerate small amounts of sugar free Calpol but this needs to be discussed with the KD dietician.

Anti-reflux medication

Gaviscon, both infant and liquid formulations are  not absorbed, and therefore suitable for use.

Ondansetron and Ranitidine in tablet form  are suitable for use. Lansoprazole is the lowest carbohydrate alternative (better for feeding tubes).

Laxatives

Movicol Paediatric plain is suitable for use on the diet.

Excess Ketosis:

Occasionally ketone levels may be very high and the child can become symptomatic. This usually occurs at initiation of diet, following changes in the diet  or during intercurrent illness. Symptoms of hyperketosis include: increased heart rate, facial flushing, irritability, vomiting and unexpected lethargy.

Management of hyperketosis is by addition of carefully measured amount of additional carbohydrate to bring the blood ketones into the acceptable range (2.5-5.5 mmol/L) as recommended below:

Under 1 year of age

·         3g of Maxijul (3 level small blue Nutricia scoop) in 30ml water

1 to 5 years of age

·         5g Maxijul (1 level yellow Nutricia scoop) in 50ml water, or

·         50mls of fruit juice

Over 5 years of age

·         10g Maxijul (2 level yellow Nutricia scoop) in 100ml water, or

·         100mls of fruit juice

Check patients’ symptoms, blood glucose and blood ketone levels 20 minutes after treatment and repeat above carbohydrate administration if necessary.

If second dose of carbohydrate does not result in decreasing in ketone levels to within acceptable range the child will require IV dextrose maintenance, either 2.5% (preferably) or 5%.

Notify the KD dietitian as it will be necessary to alter the diet ratio if ketone levels are persistently excessive.

Hypoglycaemia:

If blood glucose is <2.9 mmol/L additional carbohydrate is urgently required.

Give one of the following:

·         10g of Maxijul in 100ml of water

·         100mls pure fruit juice

·         1 x 25g tube Glucogel

For patients with reduced conscious level, give 2mls/kg 10% dextrose intravenously.

Metabolic Acidosis:

Metabolic acidosis can develop without excess ketosis.  Children who are on concomitant   Topiramate, Zonisamide or Acetozalomide are at risk of metabolic acidosis. Symptoms include increased seizures, clammy and pale skin, confusion and in severe forms “Kussmaul breathing” (increased rate and depth of breathing).

·         Check electrolytes, liver function, bicarbonate and blood gas

·         Give carbohydrate-free fluids if required (water, sugar free squash or 0.9% saline)

·         Consider bicarbonate replacement if severe acidosis

·         Follow local protocol to treat and liaise with Paediatric  Neurologist and KD Dietitian to prevent future occurrences.

Returning to the KD after illness

As soon as possible, introduce usual oral diet at ½ portions, given more frequently throughout the day. Children may use their emergency/sick day milkshake recipe instead of food.

If the child is enterally fed, the feeding regimen should be started at 50% concentration, increasing to 75% the next day and 100% thereafter according to tolerance.

Consider stopping glucose-containing intravenous infusion when safe to do so.