20 March 2018

Everyone agreed that Shanade Jooste was a ray of sunshine, with big brown eyes, shiny blonde locks and a smile that touched the hearts of everyone who met her.

“My little princess,” says proud Pam Jooste, who has become a marathon mum in her golden girl’s memory.

Born in November 1995, Shanade was a happy baby with a gurgling laugh. A younger sister for two-year old Tyrone, their little angel was perfect, Pam and husband Trevor agreed.

The family was blissfully unaware, though, that Shanade had been born with a progressive disorder, called Batten Disease. The first signs unexpectedly appeared when their little girl was nine months old and began having mild seizures.

Perhaps, they thought, it was epilepsy?

Pam, Trevor and Shanade on her Christening day
Pam, her husband Trevor and Shanade on her Christening day

Batten Disease is a rare, life-limiting disease of the brain which can cause blindness, hallucinations, loss of speech and seizures. The symptoms can be subtle at first, but grow progressively worse.

Shanade was becoming poorly at an age when other children begin reaching early-years milestones, but it wouldn’t be until she was aged four that doctors would finally identify why.

The illness can affect children’s motor skills, yet Shanade would try and try again with a doughty perseverance.

“She especially loved to play with her stacking cups,” says Pam.

“Although she couldn’t stack them correctly, she was so patient and determined.”

Tyrone's precious little sister
Tyrone's precious little sister

Although it took a little longer for her to achieve them, Shanade also reached those special childhood milestones.

Pam and Trevor were overjoyed when aged 15 months old, she took her very first wobbly steps.

While her seizures were a worry, Shanade brought the family so much joy. Whether she was playing chase with Mum, squealing with laughter, or holding-hands while watching cartoons with big brother Tyrone on her favourite beanbag, Shanade seized upon the gladness of every moment.

Tyrone and Shanade
Quality time with her older brother Tyrone, was one of Shanade’s favourite things

Gradually, though, her symptoms became more serious. Her fits became more frequent. On one terrifying occasion after a seizure, she was rushed, unresponsive, to hospital.

“Thankfully we didn’t have another episode like that but hallucinations and smaller seizures were still common,” says Pam.

Doctors prescribed medicines and treatments, but Shanade’s symptoms were still a puzzle. Then, on February 1, 2000, Pam received a fax from their doctor.

We finally had a solution to make our little girl better

“I remember hurrying over to the fax machine in anticipation,” she says.

“We finally had a solution to make our little girl better.”

But it wasn’t the good news that the family had been hoping for.

“My eyes skimmed through the symptoms, all the way to the bottom of the page,” remembers Pam.

“It said: ‘Batten Disease. A brain disease with no cure and that is always fatal’.”

Fatal.

Shocked, Pam and Trevor hurried to the doctors to discuss a way forward.

“All I wanted to know was how much time we had left,” she says.

Tyrone and Shanade
Family is forever

Three years, the doctor told them.

Three years was all they could hope for.

It made the family determined to make the most of the time they had left and to make unforgettable memories together.

Gradually, Shanade became blind and could no longer walk or even sit up. Fun and messy meal times were replaced with a pouch that provided Shanade’s tummy the nutrition it needed, through a gastrostomy tube.

Even though she couldn’t see me, she knew I was always near.

“Most of Shanade’s days were spent on her beanbag, close to me, listening to ‘Major for Minors’ — a favourite compilation of classical, nursery rhymes,” says Pam.

“When she lay on her bed, I would walk in and greet her, and her face would always light up. Even though she couldn’t see me, she knew I was always near.”

Time seemed to be passing too quickly, and in the early hours of April 26, 2003, Pam and Trevor’s angel died. She was just seven years old.

“The best way to sum up Batten Disease,” says Pam, “is that it takes away the childhood, then it takes away the child.”


A painting of Mum’s little princess

For the few years after Shanade passed away, on her angelversary, Pam and Trevor would wake up early, walk to the beach and sprinkle flower petals into the sea where her ashes were spread.

Shanade's memory garden
A special memory garden in honour of Shanade, at the Jooste's home in Durban, South Africa

“Our home is surrounded with Shanade’s photographs,” adds Pam “and at times when I missed her, I would go out and spend time in the memory garden we made for her.”

A family photo in the African bush
Pam (far right), her husband, Trevor (left), and their son, Tyrone

Feeling empty without her darling daughter by her side, Pam began thinking about how she could honour Shanade’s life and help other families enduring a similar experience with Batten’s.

Without her daughter to care for, she now had time to herself that she was unfamiliar what to do with, so took up running to fill the void.

Gradually, Pam built up the stamina and confidence to take on challenges and in 2007, signed up to a mega-race, the Comrades Marathon.

It was an emotionally wonderful day.

“It was an ultra-marathon,” she smiles, “89 kilometers, which I wanted to run, in memory of Shanade.”

Friends and family all over the world sponsored every tough kilometer that Pam ran.

“I made it within 10 hours and 55 minutes.

“It was an emotionally wonderful day and I felt Shanade was running every step with me. That run was dedicated to all the Batten children who would never walk or run again.”

Pam ready for the Cape Argus
Pam all set and ready for her cycling challenge

Pam’s ultra-marathon success motivated her to cycle South Africa’s Cape Argus, next— the world’s most popular cycle race at 109 kilometers long.

In South Africa, where the Jooste family live, medical aid doesn’t cover things like wheelchairs, nappies, caregiver fees and suction machines to help children with Batten disease.

Pam made it her mission to help fund them for children just like Shanade and enable their families to make the most of the precious time they have together.

Pam’s still running and cycling, and has also hosted dozens of more relaxing fundraisers.

There have been golf days, hamper raffles and on Shanade’s 21st birthday, Pam and her closest gal pals celebrated her life with champagne and a high tea.

Pam and close friends
Pam and some of her closest friends celebrating what would’ve been Shanade’s 21st birthday

“I always buy Shanade a big bunch of flowers for her birthday and on her angelversary every year,” says Pam.

“Celebrating and talking about my little princess soothes the hole in my heart and keeps her memory alive.”

Shanade in wheelchair
Still smiling even though she could no longer walk

Pam is a spiritual person and takes great comfort from the thought that Shanade is never far from her family.

“I believe Shanade is constantly with us,” she smiles.

“When she was ill she could never see or experience things with us, but now she goes wherever we go.”

##Keeping memories alive - Pam suggests

Don’t keep your thoughts to yourself. Express your feelings, laugh if you want to, cry if you want to.

Do things you know they loved doing and visit places they loved visiting.

Let go of the anger and the ‘why’. Although we can’t bring our children back, we can keep their memories alive.