The Thing About Jed

CityLife FEATURE STORY

The Thing About Jed
By Danae Jones

To the outside world, at first glance Jed Baker looks normal, but to notice the real Jed, you need to spend time with him and witness his behaviour. In the moments when he’s not seeking comfort from his Mum, just like a toddler would, when he really does start talking about things that matter to him, that’s when you see the depth of his disability. That is when you realise that this young man is something very special.

Jed was born perfect. He had all of his fingers and toes and showed all the signs of being a healthy baby boy. What his parents didn’t realise, was an underlying issue for Jed, something insidious, silent, and deadly. What they also didn’t know was that it would change the direction of their lives forever.

His mother Nicola Baker recalls the early days when Jed was a newborn.
“When I first had Jed as a little baby, he never really slept. So, we had a good period of nine months of this child who was sleeping for 20 minutes at a time. We knew something was wrong, but nobody could hear what we were saying until one morning, early in the morning he woke and was very difficult to settle. Settling Jed that particular morning was difficult because we could see that there was a twitching happening in his face. And it went down his face, into his hand, down into his leg, and I knew something was wrong. I called an ambulance, and my Mum came up and they took us off to the Cairns Base Hospital, where we found that our baby at 11 months old was having a stroke,” she said.

It was a number of years of hospital visits, testing, and troubleshooting, and still they couldn’t work out what was wrong. In fact, many doctors and nurses just dismissed Jed’s episodes. But this Mum knew that there was something far more significant occurring in the body of her son, and she continued to dig for answers.

“After the first stroke and after living for a year without any sign of any difficulty, we noticed that Jed was doing something rhythmic in his mouth again, and I thought, oh, this is something happening here. So we took him back to his paediatrician, Dr Tim Warnock, who said, everything looks fine, but I think I’ll just check your blood pressure. So he did and said, no, I think this is not okay. You need to go to hospital. We discovered that Jed had very high blood pressure and we were sent off to Brisbane again to see what was going on.
“At that point, we found out that Jed also had some issues in his renal arteries, and we didn’t know what had caused that. And again, we were sent home. With just one of those things, and one morning in particular, they took us into a private room that was full of doctors and they said to us, your son has Moyamoya disease.”

That diagnosis rocked the Baker family’s world in the worst possible way.

“Being the mother of someone with a diagnosis like Moyamoya disease is absolutely devastating. We understand that children are diagnosed with life-threatening diseases all of the time, but this one was so rare. It was one in a million people being diagnosed. It was thought to be mostly in Japanese children and here we were with our little blonde boy at risk of dying from this disease. So, there was no support for us and we just had to fumble through with our own contacts. Family and friends became vital to our existence because without support from people who love you, you just wouldn’t be able to get through this kind of a journey.”

Dr Tim Warnock who has been Jed’s paediatrician since birth and now a lifelong friend of the family’s, remembers the persistence of Nicola in trying to dig for answers for her child. He has one simple message for other parents and doctors after going through the harrowing journey with the Bakers over many years.

“Parents have all got their children’s best interest at heart. If they’re worried something is going on, listen to what they’re saying. Examine the child and do investigations if required. Mothers know their own children better than anyone,” said Dr Warnock.

Through investigations they discovered the amazing work of Professor Marcus Stoodley, a neurosurgeon that specialised in operating on people with Moyamoya Disease based in Sydney.

Jed went on to have numerous life-saving operations with Dr Stoodley and while once being diagnosed as palliative, he is still with us today. He has since won the hearts of a nation and many a Hollywood celebrity and his mother Nicola, has won the hearts of the entire North Queensland community in the process.

“The key message for Moyamoya Australia for our patients, and we call them warriors. That you are not alone. We are here to help you. We can give you a little bit of money, it’s not a lot, but it can pay for a taxi fare or a takeaway meal. We can fund the research and we are doing that. We can support you through friendship, companionship, and help you feel like you’ve got people that you can ask about your journey. And that’s where the passion comes from for me, knowing that when we did this alone, you don’t have to be alone anymore,” said Nicola Baker.

Nicola Baker has just released a book about their story titled “The Thing About Jed”, in the hope of raising awareness of Moyamoya Disease around the country. All proceeds from the book go towards fundraising for Moyamoya Australia, a charity she has founded to raise vital funds for research into the disease and to support families who are faced with a diagnosis in their time of need.

A documentary on the family and their inspirational journey is also about to be released.

“The documentary and the book are key to showing people what a family goes through when faced with a diagnosis of Moyamoya Disease. It is my hope that through sharing our story, more awareness is raised in the community about Moyamoya and the vital research is undertaken to save more lives in the process.”

To find out more about Moyamoya Australia or to buy a copy of the book go to: www.moyamoyaaustralia.org.au

What is Moyamoya Disease?
Moyamoya disease is a rare, progressive cerebrovascular disorder caused by blocked arteries at the base of the brain in an area called the basal ganglia.

The name “moyamoya” means “puff of smoke” in Japanese and describes the look of the tangle of tiny vessels formed to compensate for the blockage. Moyamoya disease was first described in Japan in the 1960’s and it has since been found in individuals in the United States, Europe, Australia, and Africa.

The disease primarily affects children, but it can also occur in adults. In children, the first symptom of Moyamoya disease is often stroke, or recurrent transient ischaemic attacks (TIA, commonly referred to as “mini-strokes”), frequently accompanied by muscular weakness or paralysis affecting one side of the body, or seizures.

Adults most often experience a hemorrhagic stroke due to recurring blood clots in the affected brain vessels.  Individuals with this disorder may have disturbed consciousness, speech deficits (usually aphasia), sensory and cognitive impairments, involuntary movements, and vision problems.

Because it tends to run in families, researchers think that Moyamoya disease is the result of inherited genetic abnormalities. Studies that look for the abnormal gene(s) may help reveal the bio mechanisms that cause the disorder.

Is there any treatment?
There are several types of revascularization surgery that can restore blood flow to the brain by opening narrowed blood vessels or by bypassing blocked arteries.  Children usually respond better to revascularization surgery than adults, but the majority of individuals have no further strokes or related problems after surgery.

What is the prognosis?
Without surgery, the majority of individuals with Moyamoya disease will experience mental decline and multiple strokes because of the progressive narrowing of arteries. Without treatment, Moyamoya disease can be fatal as the result of intracerebral haemorrhage.

What research is being done?
The NINDS conducts neurological research in laboratories at the NIH and also supports additional research through grants to major medical institutions across the country. These studies, which range from clinical trials to investigations of basic biological mechanisms, are aimed at discovering how and why diseases develop in the brain, and focus on finding ways to prevent, treat, or cure them.

“Family and friends became vital to our existence because, without support from people who love you, you just wouldn’t be able to get through this kind of a journey.”

“The key message for Moyamoya Australia for our patients, and we call them warriors. That you are not alone. We are here to help you.”