3.2.2 Nutritional evaluation

efficacy on seizure control. Potential complicating comorbidities include gastrointestinal issues (such as gastroesophageal reflux and constipation), hypercholester-

Order investigations

Provide supplements Educate caregiver

cardiomyopathy [2]. Once identified, most comorbidities can be preventatively

Second, it is critical to identify psychological, socioeconomic, cultural, and religious factors that may affect the child's diet [2]. Challenging behavior traits in the child or caregiver should be addressed early. Since many patients with epilepsy are of lower socioeconomic status, an appraisal of the family environment, including finances, is essential before deciding to proceed; even if deemed adequate, the caregiver must be made aware of the impact of a ketogenic diet on time and resources, including separate meal preparation from the rest of the family and increased costs [26]. It is also necessary to consider the family's cultural and religious background, which may result in some recipes being more suitable than

Third, the child's medications should be reviewed. Generally, blood levels of common anti-epileptic drugs are not significantly altered by a ketogenic diet, therefore dose adjustments are not required. However, it may be worth considering dose reductions in the case of valproate, zonisamide, and topiramate; although these drugs are generally safe alongside a ketogenic diet, there have been rare instances of hepatotoxicity and secondary carnitine deficiency with valproate, as well as chronic metabolic acidosis and a slight increase in kidney stones with zonisamide and topiramate [2]. All medications should be reviewed for carbohydrate content, which may neces-

Fourth, the child and caregiver should be provided with a means of selfmonitoring the diet, which critically provides feedback as to how effectively the child is achieving physiological ketosis [2]. Traditionally, this has been done using urine ketone dipstick testing, but it is now possible to prescribe a blood glucose and ketone monitor in many countries. The former is less expensive and avoids finger pricks, but the latter is easier, more specific, and more accurate [38]. Unless there is a compelling reason to measure urinary ketones, a blood glucose and ketone moni-

Finally, investigations should be ordered before commencing a ketogenic diet in

a child, primarily to rule out contraindications (a ketogenic diet is absolutely contraindicated in disorders of fat metabolism, including carnitine deficiency, carnitine palmitoyltransferase I and II deficiency, any of the short, medium, or longchain acyl dehydrogenase deficiencies, and porphyria) [2]. Basic laboratory

sitate a switch to lower carbohydrate preparations [26].

tor should be prescribed and the caregiver instructed on its use.

olemia, low weight gain, kidney stones, chronic metabolic acidosis, and

Medical Assess baseline epilepsy state and comorbidities

Nutritional Assess baseline physical parameters

Identify psycho-socioeconomic, cultural, and religious factors

Provide blood glucose and ketone monitor; show how to use

Select most appropriate conventional ketogenic diet

Review anti-epileptic drugs and medications

Evaluation Steps

Epilepsy - Advances in Diagnosis and Therapy

Preparing a child (and caregiver) for a ketogenic diet.

managed.

Table 4.

others [2].

154

A nutritional evaluation should be performed by a dietitian experienced in managing ketogenic diets in children, and include an assessment of baseline physical parameters, selection of the most suitable ketogenic diet, and education of the caregiver on what to expect with the diet.

Baseline weight, body-mass index, and height are routinely measured (in infants, head circumference is also measured) [2]. The child's recommended calorie and fluid intake should be calculated, as well as the desired fat to protein to carbohydrate ratio. Food aversions and allergies must be clearly documented.

When selecting a conventional ketogenic diet for a child, the most important factor to consider is the family environment, rather than perceived diet efficacy [2]. The CKD is highly effective for seizure control, but restrictive and time-consuming; depending on the family environment, it may be more feasible to implement the MCT diet, MAD, or LGIT diet. Regarding diet efficacy, the CKD appears to be superior to the MAD in infants under 2 years of age, whereas both are equally effective in older children [27]. For the transition to adolescence, the MAD and LGIT diet are less restrictive and more appropriate; the LGIT diet may not provide an adequate level of ketosis to treat GLUT1 DS and PDHD, although it is highly effective in Angelman syndrome [2, 39]. For infants and children on enteral feeds, ketogenic diets can be administered in liquid form, which may be more convenient and efficacious [2].

Given the limited fruit, vegetable, and calcium content in conventional ketogenic diets, supplementation with a carbohydrate-free multivitamin containing minerals, as well as supplementation with calcium and vitamin D, is considered mandatory in children [26]. No particular recommendations exist for supplementing a ketogenic diet with magnesium, selenium, carnitine, laxatives, probiotics, or exogenous ketones [2, 26].

Caregiver education is essential; the caregiver needs to understand exactly what is required of them to implement the diet. A classroom-based format, with several different caregivers present, can be advantageous [26]. The dietitian should demonstrate how to identify sources of fat, protein, and carbohydrate, how to count net carbohydrate (total carbohydrate minus fiber) for those on a MAD, how to identify foods with a low glycemic index for those on an LGIT diet, and how to navigate potential pitfalls [26]. Helpful additional resources should be provided [40, 41] and any expectations addressed.
