New South Island service provides relief for kids with drug-resistant epilepsy

After beginning ketogenic dietary therapy (KDT) to control Zachary Dunn’s drug-resistant epileptic seizures two years ago, he went from having four hospital admissions and two helicopter transfers within three months, to a happy four-year-old with greatly reduced seizure frequency and no further admissions.

Zachary’s parents Danielle and Hamish Dunn had trialled five epilepsy medications that did very little to help him.

“We were in and out of hospital and the medication doses were increasing, but Zach was still having a lot of seizures at home,” Danielle says.

“He also had terrible side effects. It got to the point where he couldn’t move, sleep or feed himself and he was crying inconsolably. His quality of life was pretty poor and it was a really awful, stressful time for us as a family.”

The Dunn family live rurally in Alexandra, Central Otago.

They are one of the families being assisted by the South Island Paediatric Ketogenic Dietary Therapy Service, led by Charlene Tan-Smith, South Island Clinical Lead (Ketogenics) and Paediatric and Ketogenic Dietitian, Canterbury DHB.

The service, originally only available privately and in Canterbury DHB since 2016, has now expanded to cover all five South Island DHBs as a hub-and-spoke model (with Christchurch Hospital as an online hub).

Zachary’s seizures began when he turned two.

Danielle says they had noticed his development was delayed at around one year of age and after his seizures started it was thought that he had a rare, underlying genetic syndrome that was causing the epilepsy.

Danielle and Hamish found out about the use of KDT as therapy for refractory epilepsy through an internet search.

After researching what was available in New Zealand, they were informed about Charlene’s clinic through another parent in an online epilepsy support group.

Zachary is now solely on KDT to control his epilepsy, after being gradually weaned off the medication he was resistant to.

“We were lucky we found Charlene when we did, as Zach was so sick and all our DHB had to offer was more drugs. Many kids with refractory epilepsy spend their life on drug trials without being offered a ketogenic diet trial, while still having seizures and suffering the side effects.

“And once you’ve exceeded two to three ineffective drug trials, the chances of further trials being effective for controlling seizures falls exponentially, whereas the ketogenic diet – which has been used to treat epilepsy for a century – has a higher success rate.”

About a third of children with epilepsy will eventually develop refractory epilepsy – meaning medicines don’t work well, or at all, to control the seizures.

In the most severe cases, hundreds of seizures can occur each day, impacting on the quality of life of children, parents, siblings, grandparents, schools and the wider community.

There has been no improvement in the rate of refractory epilepsy, despite the addition of over 10 new antiepileptic medications to the market in recent years.

KDT is a treatment used internationally for children with refractory epilepsy who don’t respond to anti-epilepsy drugs.

It is not to be confused with the fad ‘keto diet’ and must be medically prescribed and monitored. KDT can significantly reduce seizures for some patients, with some even becoming totally seizure-free, says Charlene.

“KDT is an evidence-based mainstream treatment, using medical nutritional ketosis to simulate a starvation state and provide the brain with fuel from ketones, rather than glucose from carbohydrates.

“It’s a very specific, tightly controlled diet. All the components have to be carefully calculated, weighed and measured, which is why it’s registered dietitian-led.”

For children and their families in the South Island who live outside of Christchurch, the service is available remotely via Zoom sessions, email and phone calls.

Charlene provides families with the total amounts of fat, protein and carbohydrates their child needs each day and teaches them how to measure each meal and snack, depending on their specific KDT prescription.

The family’s lifestyle and food preferences are incorporated into the prescription, which is also carefully adapted as the child grows. Ketone levels are measured at home using a funded blood/glucose monitoring machine and frequent blood tests keep track of nutrition levels to ensure there are no deficiencies.

“It’s a whole new skill we had to learn as parents,” Danielle says. “There’s so much more involved than just dishing out medication a couple of times a day.

There’s a lot of effort involved in creating a diet that’s so precise and requires a huge amount of compliance from both the child and the parent.

Learning to use the keto calculator to make all the macronutrients in each recipe balanced down to 0.1g took a bit of practice and it has been great to have Charlene’s support to get me up to speed. It's a big change in lifestyle but parents will go to great lengths to help their sick child become well.

The diet is our life saver and it’s so much easier spending some time in the kitchen than living with poorly controlled epilepsy."

Danielle says while the diet itself is a lot of work, the convenience of not having to travel to Christchurch is a huge help.

“Once we had initiated the diet we were talking to Charlene every few days, as it takes a while for the ketones to rise and get to a steady state. Now that he is stable, we stay in touch every few weeks. It’s completely feasible to do it all from a distance.”

KDT is a highly specialised service. Charlene trained to become a ketogenic dietitian in Canada and at Matthew’s Friends UK Keto College; and has travelled to hospitals all over the world learning about how different countries use the therapy.

She is part of the Ketogenic Dietitian Group in the USA, UK and Canada. “I’m very blessed to have connections with the major keto centres around the world and I’m so excited about our service for the South Island – we want to help as many families as we can.”

The service is not only benefiting patients and their families but is also saving health resources by reducing emergency department admissions and hospital overnight stays.

The service was extended South Island-wide in August 2019, after Canterbury DHB put forward a case to the South Island Alliance’s Child Health Service Level Alliance, requesting improved access and equity for patients, no matter where they live in the South Island.

The average time a patient may need to be on the diet is between two and five years.

Danielle and Hamish were one of the families who had written letters to the Ministry of Health and Canterbury DHB asking for the service to be offered across the South Island. “Zach has done really well with the diet, which probably helped the case,” says Danielle.

“Within two months of starting, his tonic clonic and atonic seizures stopped. Now, he only has focal seizures, which are a lot smaller and usually triggered by illness or when he is really tired. Just to see the difference it’s made in his development is huge.

“He started walking earlier this year, at three and a half years of age. Obviously, that’s really delayed compared to a healthy child but I don't think he would have achieved walking with the kind of protocol he was on before, with frequent convulsive seizures and drug side effects.

“His communication is increasing and he is just so much happier within himself. Seeing him smiling and playing again and his character come back is amazing. It’s greatly improved his quality of life and for our family as well, it’s just been massive.

“Opening the service up across the South Island for other families to benefit from it as we have, is great and definitely a step in the right direction.”

Photo: Danielle and Hamish Dunn with son Zachary.

Photo by Emily Kerse Photography.