The Use of K.Vita in a child with Dravet syndrome | Case study

This case study discusses a 5-year-old male with Dravet syndrome who experienced improved seizure control using K.Vita as part of a multitherapy approach. The patient successfully discontinued the ketogenic diet, and the multitherapy approach significantly enhanced his quality of life. 

Patient details and medical history 

Age: 5 years.

Gender: Male.

Family and social history: Lives with parents, child of two healthy parents. 

Diagnosis and relevant medical history

Dravet syndrome, diagnosed at 9 months of age.  

History before starting K.Vita

Focal seizures occurred at any time of day.  

Myoclonic seizures occurred in clusters during the morning and individually throughout the day. Convulsive seizures mainly occured during naps and sleep. 

Facial convulsions occurred that involved the patient dropping to the floor and did not follow a specific pattern. 

Medications 

Levetiracetam, Sodium Valproate, Stiripentol, Clobazam, Clonazepam, Chloral Hydrate, Topiramate, Ethosuximide, Fenfluramine, Phenytoin and rescue medications of midazolam, diazepam and paraldehyde.

Parents had also administered CBD bought online before this was available via NHS. VNS implant. 

The patient was participating in an investigational drug trial looking at a new treatment for Dravet syndrome. 

Dietetic Management 

Feeding route

Oral, no feeding difficulties, occasional loss of or lower appetite during periods of high seizure burden and hospitalisation. 

Food allergy or intolerance

No formal allergy diagnosis; the family identified specific foods that they believed caused negative reactions and avoided gluten, dairy, various fruits, additives, and sweeteners. 

Previous use of the ketogenic diet

The ketogenic diet was initially started at the age of one and successfully improved seizure control. Discontinued after two years due to difficulties in maintaining the diet during an illness, leading to poorly controlled seizures and hospitalisations. 

The diet was reintroduced at the age of four and had a positive impact on seizure control. It was later discontinued when the patient experienced significant benefits from an investigational drug trial. 

After completing the first phase of the trial, seizures worsened, with the patient experiencing a focal seizure every other day and 10-12 convulsive seizures during the night. The ketogenic diet was restarted, resulting in a reduction of the seizure burden to 1 focal seizure every five days and 2-3 convulsive seizures each night. 

Reason for implementing K.Vita

Parents wanted to try K.Vita as the child had been on and off ketogenic diet over the last 4 years, and they were keen to try K.Vita as an alternative. The child was on the ketogenic diet at the time of starting K.Vita. 

Dietary Management with K.Vita 

Parents were advised to:  

Introduce K.Vita incrementally over an 8-week period* 

Aim for a K.Vita target amount: 120 ml pack per day¹  (1 pack contains 371 kcal, providing ~25 % of his daily energy requirement - daily estimated energy requirements = 1482 kcal/d²). 

Maintain the avoidance of high-sugar foods, as the patient is already following a ketogenic diet. 

Slowly reduce the fat with each meal as the K.Vita amount increased.  

* To address concerns about potential gastrointestinal issues, the parents decided to extend the weaning plan for introducing K.Vita over eight weeks with regular phone reviews to monitor any symptoms. The extended plan was based on Vitaflo's 4-week introduction guide for children taking K.Vita. However, each day of the plan was repeated once, doubling the time it took to reach the target amount. 

Acceptability and tolerability of using K.Vita 

The patient suffered from diarrhoea throughout the first 3 months of introducing K.Vita to the diet. Weaning on to the target amount was slowed to prevent unmanageable symptoms. The parents said they preferred not to give a bedtime snack, so they opted for three amounts of K.Vita per day.  

After 8 weeks, the patient was on 60ml/day (3 x 20ml), and by 4 months, the amount of K.Vita increased to 90ml/day (3 x 30ml with breakfast, lunch, and evening meal) achieving 75% of the target amount providing 276 kcal = ~19% of the total daily energy requirement. The diarrhoea had settled by this point. 

After 8 months, the patient stopped taking K.Vita due to a dislike of the flavour, resulting in the return of seizures. K.Vita was reintroduced, and the parents attempted various methods to mask the flavour, such as adding it to yoghurt, porridge, fruit, and muesli, mixing it with cow's milk, and serving it with ice cream. These strategies initially succeeded in disguising the flavour for a short period, possibly due to the child's limited preference for sweet foods resulting from the ketogenic diet and avoiding sweeteners. 

The most successful strategy has been to give K.Vita orally via syringe, aiming to reduce the time the patient could taste the K.Vita. 

Anthropometry

The patient tracked the 50th centile for weight and height whilst taking K.Vita. 

Weaning off the ketogenic diet

Four months after starting K.Vita, the ketogenic diet was weaned off successfully over 6 weeks.  An attempt was made to increase the amount of K.Vita to 120 ml/day (3 x 40 ml) to see if this further improved seizure control. However, the patient did not tolerate the increase due to his dislike of the flavour. The parents plan to try this again in the future.  

After discontinuing the ketogenic diet, the family maintained their avoidance of high-sugar foods and opted to avoid ultra-processed foods and sweeteners. While using K.Vita, they further reduced the carbohydrate content of their child's diet by avoiding foods with added sugars and sweeteners. They decreased the portion size of starchy carbohydrates during meals. They included a generous amount of fats, estimating their diet to consist of approximately 40% energy from carbohydrates, 40% energy from fat (including K.Vita), and 20% energy from protein. 

Outcomes 

The patient currently has 1-2 seizures per month, managed by a multitherapy approach including K.Vita, Fenfluramine, Levetiracetam, Clonazepam, VNS and the investigational drug trial. Clonazepam is very slowly being reduced. The parents estimate that the inclusion of K.Vita within a multitherapy approach offered a 25-30% seizure reduction overall.

Conclusions and Key Learnings

A multitherapy approach using K.Vita has been effective in managing difficult-to-manage epilepsy in this patient, who had previously responded well to a ketogenic diet but experienced reduced seizure control upon discontinuation.

The patient successfully weaned off the ketogenic diet by adopting a multitherapy approach that included the use of K.Vita. This approach resulted in a 25-30% reduction in seizures for a child with a high seizure burden, significantly improving their quality of life.   

Slower introduction of K.Vita helped minimise and manage gastrointestinal symptoms, resolving once established after 4 months. Patients with some pre-existing gastrointestinal symptoms may benefit from a slower introduction of K.Vita.