Tiaré Johnston’s brave recovery with rare brain disease after having tonsils and adenoids removed

Most six year olds have no issue forming sentences but for Tiaré Johnston it’s a challenging task.

The Sydney girl was born a healthy, bright and clever girl.

Her parents, Tamara, 39, and father, Josh, 35, called her an overachiever.

The day before Tiaré had her tonsils and adenoids removed.

The day before Tiaré had her tonsils and adenoids removed.

Tiaré during ballet class.

Tiaré during ballet class.

But two years ago, when Tiaré was just three years old, she was diagnosed with a rare brain disease, which occurred after having her tonsils and adenoids removed.

Today, the longest, unprompted sentence Tiaré can string together is just six words.

She has started moving her left hand and walking with support.

The youngster relies on using her right hand to feel her way around as she suffers a vision impairment.

Tiaré receiving inpatient therapy with a tilt table in 2016.

Tiaré receiving inpatient therapy with a tilt table in 2016.

Tiaré being visited by Giggle and Hoot in the Sydney Children's Hospital in 2016.

Tiaré being visited by Giggle and Hoot in the Sydney Children's Hospital in 2016.

Her parents, from Willoughby on Sydney’s lower north shore, call these improvements.

“We are very proud of the accomplishments she has made during this time, but also miss the little girl she once was,” Mrs Johnston told 9news.com.au.

“There’s days when you compare her to before and that can be heartbreaking and then when you look at where she is now compared to in hospital, she didn’t have any language or movement in her hand.”

Tiaré suffered tonsillitis during early childhood.

Mrs Johnston took her to a number of doctors who all suggested tonsil and adenoid surgery was the way to go.

The family-of-four at a friend's birthday party in January 2016.

The family-of-four at a friend's birthday party in January 2016.

But a few days before surgery, Tiaré came home from preschool with a croaky voice. 

“I called the hospital and said ‘I think we will need to cancel surgery’,” Mrs Johnston said.

“They said it was quite common for ENT patients to have these symptoms but I felt uncomfortable.”

The surgery went ahead as planned on August 5, 2016.

When Tiaré woke up, Mrs Johnston said she was happy and even put on a little dance performance for her parents in hospital.

She also didn’t want to been wheeled out to the car the next day, but insisted on walking.

Tiaré was an active and bubbly little girl before surgery.

Tiaré was an active and bubbly little girl before surgery.

Tiaré is a huge Frozen fan.

Tiaré is a huge Frozen fan.

These were the last moments Mr and Mrs Johnston saw Tiaré as “the old Tiaré”.

That afternoon, Tiaré came down with a nasty fever.

“The hospital said it was completely normal after surgery and to keep an eye on her,” the mother-of-two said.

“They said if her temperature goes to 40 degrees or higher to call an ambulance.

“The next day I went to her room and she was in an awkward position in bed in a puddle of vomit and her pupils were dilated.

“I could see through her eyes that she was thinking what to say but couldn’t communicate it.

“I said say “mummy” and she sort of said it but her face was paralysed. I cut her jumper off to check her temperature and it was 40 degrees so I called an ambulance.”

Tiaré requested an Elsa cake for her "come as your favourite character" party for her 6th birthday.

Tiaré requested an Elsa cake for her "come as your favourite character" party for her 6th birthday.

By the time she arrived at hospital, Tiaré was unconscious.

She spent the next three weeks in ICU at the Sydney Children’s Hospital, Randwick.

In total, she was in hospital for a total of four-and-a-half months.

At first, it appeared she had sepsis.

Tiaré's most recent preschool photo.

Tiaré's most recent preschool photo.

Tiaré receives presents and flowers for her 6th birthday.

Tiaré receives presents and flowers for her 6th birthday.

It took about 27 hours for eight teams of medical staff to determine a diagnosis after doing an emergency MRI – it came back as acute necrotising encephalitis (ANE).

The aggressive and rare neurological brain disease is caused by a viral infection.

“There was a suspicion the anaesthetic from surgery had altered the blood-brain barriers,” Mrs Johnston said.

“When she went in for the MRI, I said to my husband “let it be anything but the brain, she’s such a clever kid”.

“They called Josh and I into a room and showed us the scans – in some areas there was more damage than viable tissue. They asked if we wanted them to treat her.”

Tiaré makes a new friend at NAPA.

Tiaré makes a new friend at NAPA.

It was day six in hospital when Tiaré nearly died after her brain swelled and herniated a nerve, resulting in her right pupil dilating.

It was suggested her parents fill out “do not resuscitate” paperwork and turn her ventilator off.

“The only responses she was giving was grinding her teeth. She couldn’t open her eyes and she was stiff in her left side,” her mother said.

The pair decided to pursue treatment to give Tiaré the best possible life.

She was placed on high-dose steroids, which she responded well to.

Now, she attends 10 therapry sessions a week, which includes speech, occupational therapy, physiotherapy, visual therapy and acupuncture.

Tiaré doing therapy at home.

Tiaré doing therapy at home.

Tiaré's legs are being supported while working on walking at the NAPA centre in Lane Cove West.

Tiaré's legs are being supported while working on walking at the NAPA centre in Lane Cove West.

On top of this, she does intensive therapy at NAPA Centre and four hours of therapy at home each day.

“We just want to give her the best chance at improving. We still have hope for her,” Mrs Johnston said.

“She’s nowhere near what she was previously but we have a lot of hope.”

Tiaré has a very supportive, close-knit family.

Tiaré has a very supportive, close-knit family.

The family have travelled to the US for treatment and next month are going to Russia for intensive brain stimulation and therapy treatments using the PoNS device, which sits on the tongue and assists in balance and motor skills.

Mrs Johnston said if her youngest daughter, Arianna, 3, ever needed her tonsils out “she would not be getting surgery”.

“When kids are little, they’re just a lot more vulnerable,” she said.

She said she believes the rare brain disease was brought on by the tonsil surgery.

Tiaré and her sister painting.

Tiaré and her sister painting.

“If we had the choice, we wouldn’t have put her in surgery,” she said.

“Her friends are now at school reading, writing and are learning to ride a bike. They’re milestones I was looking forward to. We decided to wait until next year to send her to school to enable more healing on her brain and focus on her therapies. 

“She’s a very happy child and a bit cheeky. She’s very social, she loves play-dates and birthday parties.”

Mater Hospital in North Sydney is where Tiaré had her tonsils and adenoids removed.

“The Mater wishes to express our sincerest sympathies to Tiaré and her family,” a Mater Hospital spokesman said.

“Owing to the hospital’s privacy obligations, the Mater is not able to disclose any confidential patient information relating to Tiaré’s care.” 

The Australian Medical Association declined to comment when contacted by 9news.com.au. 

© Nine Digital Pty Ltd 2018

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