Young Haven has one hundred seizures a day
Haven Holley’s right arm is covered with around a hundred neatly placed bracelets. It’s the latest habit she’s developed over lockdown, mum Brenda Jones says. Coincidentally, a hundred is also the number of seizures Holley experiences daily.
‘‘She’s developed a few OCD habits during lockdown,’’ Jones said.
‘‘When you’ve got very little control over everything else, sometimes those small things you can control mean everything to you.’’
Control is something the Morrinsville mum and daughter have been grasping for since Haven was first diagnosed with Doose Syndrome, a form of epilepsy, at the age of two.
Now five-years-old, Holley has become so conditioned to her seizures that she will grab hold of someone while her head drops into her lap.
But as the seizure numbers rise and ‘‘script after script’’ becomes ineffective — a ketogenic diet has become Haven’s last shot at a seizure-free life.
‘‘She had a really bad prolonged seizure just before her birthday,’’ Jones said.
‘‘It didn’t go to plan like her normal seizures have done in the past and it lasted about 45 minutes — the longest one ever.’’
It’s also impacted her ability to speak.
This has been the driver to push for Haven to get back on the ketogenic programme permanently, after she received funding to trial the diet in 2019.
‘‘I’m hoping that she will be seizure free on the diet.
‘‘I don’t expect it to happen quickly or instantly, but... it really is our last hope. We are on our tenth medication so what else is there.’’
Jones said within a week of Haven being on the trial she noticed a reduction in episodes and cognitive improvement.
‘‘I was disappointed when she got taken off it. I was the one caring for her. I saw her start doing really well and even her immune system improved, for the first time ever she didn’t get sick.’’
Waikato DHB neurologist Matthew Phillips has studied the effects of the ketogenic diet on neurological disorders for over four years.
He said Holley should see significant results on the diet and could even become completely seizure free.
Phillips completed a world-first randomised study of a keto diet in Parkinson’s two years ago, just completed the first randomised study of a keto diet in Alzheimer’s and has plans to do the same for Huntington’s Disease.
‘‘I can tell you right now for her Doose Syndrome, keto is going to be the most amazing thing. It’s going to really hammer it.’’
The keto diet mimics fasting, and as a result can shift the body into cell maintenance and repair processes, Phillips said.
He said medication only has ‘‘one target’’ and generally ‘‘masks symptoms’’ whereas diet therapy changes everything making the body resistant.
‘‘What it’s thought to do is reduce neuron excitability, so for epilepsy the neurons get too excited too easily and they start going haywire. The keto diet somehow dampens that excitability.’’
Currently, the dietary programme is only offered in Auckland and Christchurch, meaning Haven will only have access to a modified version.
Expected to commence in August, the programme will be accessed through skype appointments with a private dietitian in Auckland.
Jones said the limited options available for Haven’s situation is ‘‘far from ideal’’ and would like to see the programme introduced in the Waikato — something the Waikato DHB are trying to source funding for.
‘‘Ideally a dietitian would come to your house and meet with you and go through products with you and maybe help you with your shopping if it was needed, but a skype appointment is all they offer.’’
Phillips said the programme is not widely spread because it is ‘‘difficult for a drug company or any company to market fasting’’ when there is no financial benefit.
‘‘I’m hoping that she will be seizure free on the diet. I don’t expect it to happen quickly or instantly, but... it really is our last hope’’ Mum Brenda Jones