Four-year-old Sienna Gordan lives with Schizencephaly, a rare condition where her brain did not develop properly resulting in large holes being formed.
Camera IconFour-year-old Sienna Gordan lives with Schizencephaly, a rare condition where her brain did not develop properly resulting in large holes being formed. Credit: Supplied

WA Rare Disease Care Centre: Sienna’s ongoing battle

Pip WallerPerthNow - Western Suburbs

Cassie Gordon said her second pregnancy with her daughter Sienna was “pretty ordinary”.

After the healthy delivery of her now 17-year-old son Mekhi Paterson, both Cassie and husband Jay thought the birth of their four-year-old Sienna would be just the same.

“In my first scan there was a bit of excess fluid, meaning there was a high risk of my baby having Down syndrome, but the anatomy scan said she was a normal baby, so that’s what we thought,” Cassie said.

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“But during labour, we noticed something was wrong.

“Her heart rate wasn’t coping with the contractions so I ended up having an emergency C-section and as soon as I held her, she turned blue in my arms.”

Sienna was taken to a special care nursery before being flown from the Bunbury Regional Hospital where she was born to Perth Children’s Hospital.

By just two months old, Sienna had already had multiple brain surgeries to relieve the fluid build-up in her brain.
Camera IconBy just two months old, Sienna had already had multiple brain surgeries to relieve the fluid build-up in her brain. Credit: Supplied

From there she underwent multiple tests as well as x-rays on her head and chest, which resulted in a diagnosis of hydrocephalus, or water on the brain.

The Gordons had to decide quickly if they were going to proceed with surgery to ease the pressure from the fluid in Sienna’s brain, which may not have had a good outcome, or to take her home.

“It was December, so we decided to take her home for Christmas so we could have some happy memories of her,” Cassie said.

“But her head had grown two centimetres every week by the time we had her next check-up, so we decided to do the surgery when Sienna was just one month old.

“After her surgery was successful, she had another one at two months old, and I remember in the ICU, the nurses kept saying ‘schizencephaly’, so I asked the doctor what that was.

“He told us it was a rare brain malformation and that they didn’t think Sienna had very long to live.”

Sienna with her older brother, Mekhi.
Camera IconSienna with her older brother, Mekhi. Credit: Supplied

At not even a year old, Sienna was diagnosed with schizencephaly or split brain, meaning her brain had not developed properly, resulting in large holes on both sides of her brain.

It affects 1.5 of every 100,000 live births.

Being diagnosed at such a young age, Sienna was scheduled for additional brain surgeries so she could get a ventriculoperitoneal, or VP, shunt put in her brain. Cassie said the shunt was a small device implanted in the ventricles of the brain to drain excess cerebral spinal fluid into the stomach area.

“But if it drains too fast, like it did in the start, Sienna could get headaches,” she said.

“So she had to have a programmable one put in that uses a magnetic device to change its settings from low to high pressure.

“It took a while to get the pressure right; she was so irritable and would cry all the time during the first year of her life.

“But there still isn’t much research on her disease, which is why a rare disease care centre in Perth would be so beneficial.”

Cassie said her daughter Sienna had proved the doctors wrong every step of the way with how much she has thrived despite living with her disease.
Camera IconCassie said her daughter Sienna had proved the doctors wrong every step of the way with how much she has thrived despite living with her disease. Credit: Supplied

Living in Bunbury, the Gordons travel to Perth at least once a week for some of Sienna’s therapies.

Cassie said having a rare disease care centre in Perth would ensure the family, who was originally required to do “a lot of research” into Sienna’s condition, would have support.

“I haven’t found anyone here in WA to connect with who has a child with schizencephaly, so if we had a centre here, we’d be able to connect with people in similar situations instead of me having to go on Facebook and find them myself.

“It would also be beneficial just for research to help find out more about her condition.

“We’ve got a really good place at the PCH but the centre would be somewhere to go and get support and understanding.

“And it’ll further help Sienna who today is doing really well. She’s so sassy with so much attitude, using her eyes and facial expressions to communicate with us.

“She’s just amazing and continues to prove her doctors wrong every step of the way.”

To help children just like Sienna, and to make the Perth Children’s Hospital Rare Care Centre a reality, you can donate at pchf.org.au/appeal.