Nice to know 2: The impact of NICE guidelines on ketogenic diet services in the UK and Ireland - An update.

BACKGROUND: Ketogenic diet therapy (KDT) has been recommended as a treatment for drug-resistant epilepsy in children and young people since 2012 in the National Institute for Health and Care Excellence Clinical Guidelines for Epilepsies. The Ketogenic Dietitians Research Network completed a survey in 2017 to assess the impact of these guidelines. METHODS: An online survey was circulated to ketogenic dietitians across the UK and Ireland. The results were compared with those of the 2017 survey. RESULTS: The number of individuals following KDT was 854, comprising an increase of 13% since 2017. Service sizes ranged widely, with 1-74 (median 16) patients on the diet. Of 36 services, 30 had a waiting list, ranging from 2 to 67 (median 9) patients. The classical diet continued to be the most common KDT used (58% of patients). Ten services reported use of a new flexible medium chain triglyceride protocol. Some 48% of patients (n = 427) had been following the KDT for over 2 years, comprising an 18% increase since 2017. Of these, 68 (15.9%) had attempted to wean off KDT but had to re-start as a result of a deterioration in seizures. CONCLUSIONS: The number of individuals following medical KDT remains stable. Referral numbers and waiting lists remain high, highlighting that KDT is still a well-recognised treatment option for drug-resistant epilepsy. The types of KDT used are similar to previous years, although increasingly flexible protocols are being adopted. Longer-term use of KDT is increasing, with a proportion of patients requiring long-term use to maintain seizure control.

Maintenance of response to ketogenic diet therapy for drug-resistant epilepsy post diet discontinuation: A multi-centre case note review.

PURPOSE: There is limited research on the proportion of individuals with epilepsy who maintain response to ketogenic diet therapy (KDT) after discontinuing treatment. We aimed to determine the proportion of individuals who did / did not maintain response post KDT and explore factors that may influence the likelihood of maintaining response. METHODS: Retrospective data were collected from 97 individuals from 9 KDT centres. Individuals had achieved ≥50 % seizure reduction on KDT for at least 12 months, with seizure frequency data available at 3 months+ post diet. Outcome 1 was: recurrence of seizures or increase in seizure frequency post diet; outcome 2: recurrence of seizures, increase in seizure frequency or an additional anti-seizure treatment started post diet. RESULTS: 61/97 (62.9 %) individuals maintained response at latest follow-up (mean 2.5[2.0] years since stopping KDT). Approximately one third maintained response without further anti-seizure treatments. One quarter of individuals had an increase in frequency or recurrence of seizures within 6 months (95 %CI 4, 12) for outcome 1 and within 3 months (3, 6) for outcome 2. Individuals who did not achieve seizure freedom on diet were significantly more likely to have an increase in seizures or to require additional anti-seizure treatments post diet compared to those who were seizure-free on diet (hazard ratio 4.02, 95 %CI (1.46, 11.16) p < 0.01). CONCLUSION: Our findings should help guide clinical teams with the information they provide patients and their families regarding likelihood of long-term seizure response to KDT. Realistic costings for KDT services may need to be considered.

Impact of ketogenic diet therapy on growth in children with epilepsy.

RATIONALE: Ketogenic diet therapy (KDT) is an effective treatment for drug-resistant epilepsy in children. There is conflicting evidence regarding the impact of KDT on growth. We aimed to determine whether linear growth and weight were affected in children who followed KDT in the UK, and to explore potential associations with clinical or demographic factors with impacted growth. METHODS: A retrospective review of medical records of children with epilepsy following KDT at 3 UK centres was conducted. Height and weight measurements taken as part of routine clinical management were recorded at baseline, 1-8 years on diet, and 1-year post-diet. Measurements were converted into z-scores, and the differences from baseline analysed using Wilcoxon Signed Rank tests. Potential associations of impacted growth with feeding method, ambulatory status, diet type, age at diet onset and average daily protein intake were investigated using Mann-Whitney, Kruskal-Wallis tests or Spearman's Rank correlation. RESULTS: 265 individuals were included, of which 84 had post-diet data available. Median height z-score significantly decreased at 1- (n = 139, p = .018), 2- (n = 86, p < .0005) and 3 years (n = 27, p = .001) on diet. There was no significant change to height or weight z-score 1-year post-diet discontinuation. Median weight z-score significantly decreased from baseline at 4 years (n = 15, p = .020), and 6 years (n = 8, p = .025) on diet, but not at other time points. There was greater height z-score decrease in non-ambulatory children at 2 years (p = .009), in those following a classical diet compared with the modified ketogenic diet at 2 years (p = .006) and amongst younger children at 2 years (n = 86, p < .005) and 3 years (n = 27, p = .008) on diet. No significant differences were found in weight z-score change across any subgroup, following Bonferroni correction for multiple testing. CONCLUSIONS: Median linear growth was significantly adversely affected for the first 3 years on KDT but catch-up growth post diet discontinuation was observed. Non-ambulatory children, younger children, and individuals following a classical diet may be more vulnerable to impacted growth when on KDT, although this was not consistent across all time points. The potential short-term impact on linear growth should be discussed with individuals considering KDT, and monitored closely.

Keto-on-the-Clock: A Survey of Dietetic Care Contact Time Taken to Provide Ketogenic Diets for Drug-Resistant Epilepsy in the UK.

Medical ketogenic diets (KDs) are effective yet resource-intensive treatment options for drug-resistant epilepsy (DRE). We investigated dietetic care contact time, as no recent data exist. An online survey was circulated to ketogenic dietitians in the UK and Ireland. Data were collected considering feeding route, KD variant and type of ketogenic enteral feed (KEF), and the estimated number of hours spent on patient-related activities during the patient journey. Fifteen dietitians representing nine KD centres responded. Of 335 patients, 267 (80%) were 18 years old or under. Dietitians spent a median of 162 h (IQR 54) of care contact time per patient of which a median of 48% (IQR 6) was direct contact. Most time was required for the classical KD taken orally (median 193 h; IQR 213) as a combined tube and oral intake (median 211 h; IQR 172) or a blended food KEF (median 189 h; IQR 148). Care contact time per month was higher for all KDs during the three-month initial trial compared to the two-year follow-up stage. Patients and caregivers with characteristics such as learning or language difficulties were identified as taking longer. Twelve out of fifteen (80%) respondents managed patients following the KD for more than two years, requiring an estimated median contact care time of 2 h (IQR 2) per patient per month. Ten out of fifteen (67%) reported insufficient official hours for dietetic activities. Our small survey gives insight into estimated dietetic care contact time, with potential application for KD provision and service delivery.

Biochemical assessment of patients following ketogenic diets for epilepsy: Current practice in the UK and Ireland.

OBJECTIVE: Biochemical assessment is recommended for patients prior to initiating and following a ketogenic diet (KD). There is no published literature regarding current practice in the UK and Ireland. We aimed to explore practice in comparison with international guidelines, determine approximate costs of biochemical testing in KD patients across the UK and Ireland, and promote greater consistency in KD services nationally. METHODS: A survey was designed to determine the biochemical tests requested for patients at baseline, 3, 6, 12, 18, and 24 months + on KD. The survey was circulated to 39 centers across the UK and Ireland. RESULTS: Sixteen centers completed the survey. Full blood count, electrolytes, calcium, liver function tests (LFTs), lipid profile, and vitamin D were requested at all centers at baseline, in keeping with international guidelines. Bicarbonate, total protein, and urinalysis were less consistently requested. Magnesium and zinc were requested by all centers, despite not being specifically recommended for pre-diet evaluation in guidelines. Urea and electrolyte profiles and some LFTs were consistently requested at follow-up, in accordance with guidelines. Other LFTs and renal tests, full blood count, lipid profile, acylcarnitine profile, selenium, vitamin D, and urinalysis were less consistently requested at follow-up. The mean costs of the lowest and highest number of tests requested at baseline in our participating centers were £167.54 and £501.93; the mean costs of the lowest and highest number of tests requested at 3-month follow-up were £19.17 and £450.06. SIGNIFICANCE: Biochemical monitoring of KD patients varies widely across the UK and Ireland and does not fully correspond to international best practice guidelines. With an ongoing drive for cost-effectiveness within health care, further work is needed to streamline practice while ensuring patient safety.