The use of K.Vita in a child with Angelman syndrome | Case study

The case study details the successful use of K.Vita in managing seizures for a 5-year-old boy with Angelman syndrome and drug-resistant epilepsy. Following the introduction of K.Vita, the patient showed significant improvements in mood, engagement, and physical abilities, along with a notable reduction in seizure frequency and hospital admissions.

Patient details and medical history

Age: 5 years (4 years at time of K.Vita initiation).

Gender: Male.

Relevant family and social history: Only child, parents separated, lives with Mum.

Diagnosis and relevant medical history

• Angelman syndrome.
• Epilepsy onset in the 1^st year of life.
• Constipation.

History before starting K.Vita

Absence, focal, drop and tonic-clonic seizures. When unwell would have hundreds of drop and absence seizures daily. Repeated seizure-related hospital admissions lasting between 1-43 days.

Medications

Lamotrigine, Levetiracetam liquid, Sodium valproate, Hydrocortisone.

Emergency Rescue medications - buccal midazolam, paraldehyde, chloral hydrate and lorazepam, movicol, melatonin, senna.

Dietetic Management

Feeding route

Oral - occasionally required thickened fluids.

Previous use of the ketogenic diet

He became seizure-free for 8 months on the Classical ketogenic diet despite low ketone levels and a low ratio (1.5:1). Remarkable developmental progress was noted.

The diet was then discontinued due to food refusal, but he remained seizure-free.

One year later, the ketogenic diet was restarted on recurrence of seizures but discontinued due to lack of efficacy.

Reasons for trying K.Vita

Due to a previous steroid-induced iatrogenic adrenal insufficiency, steroids were not a viable therapy during a prolonged 43-day hospital admission prompted by challenging seizure control.

His mother was eager to try K.Vita after the failure of the second attempt at the ketogenic diet.

Dietary management with K.Vita

Before the start of the introduction of K.Vita he had faecal dis-impaction to address his significant constipation (a known seizure trigger for him) and to help establish a regular bowel habit.  

K.Vita was introduced incrementally by following the Vitaflo 4-week introduction plan for children, aiming for a target daily amount of 120ml (4 x 30ml).

Dietary advice was to always give K.Vita with food, to continue his usual diet with minimal high-sugar items and to follow guidance on portion sizes and weight monitoring.

Acceptability and tolerability of K.Vita

Although he cannot express taste preferences verbally, he consistently accepts K.Vita, unlike other foods he chooses to refuse. No adverse side effects from introducing K.Vita in his diet have been reported.

He reached the full daily target amount 120mls of K.Vita at the end of 4-week introduction plan. He continues with 3 x 40ml daily, mixed into yogurt.

Anthropometry

There have been no growth concerns since K.Vita was started. Initial weight gain was deemed appropriate and proportional to his height and considered to be ‘catch-up’ due to an increase in dietary intake, possibly related to the reduction in seizure frequency.

Outcomes

Although adjustments to the amount of Lamotrigine coincided with the initiation of K.Vita, by the end of the introduction of 120ml of K.Vita daily he was demonstrating improved mood, engagement, playfulness, concentration, and crawling ability. A notable decrease in seizure frequency was observed. One week later he was seizure-free.

He remained seizure-free over the following three months before a chest infection triggered the reoccurrence of drop-seizures. However, he did not need to be admitted to hospital, and was cared for at home, a situation his mother believes would not have been possible before K.Vita.

Over the following year he was seizure-free except during periods of intercurrent illness, and apart from one hospital admission, was again successfully managed at home.

His mother considers the use of K.Vita has lessened the intensity and frequency of his seizures and now finds these episodes easier to manage. In addition, his use of emergency rescue medications has reduced. His bowel habit has improved, and his use of laxatives has reduced.

After nearly 18 months into his use of K.Vita for the dietary management of drug-resistant epilepsy, he continues to show positive progress.

Conclusions and key learnings

• K.Vita used in the dietary management of seizures for this 5-year-old boy with Angelman syndrome appears to have been beneficial, evident by fewer hospital admissions and decreased seizure frequency and duration over an 18-month period.
• His mother reported improvement in seizure frequency and severity shortly after reaching the target amount of K.Vita. Both the intake of K.Vita and increase in the dose of Lamotrigine coincided with the reported improvement in seizure frequency and severity.
• She also believes that the use of K.Vita has enabled her son to show progress in his developmental milestones with fewer hospital admissions and a reduced need for rescue medications.