When six-year-old Aviana (Avi) was born, her parents Bethan and Jonny were so excited to welcome their first child into the world!
She passed her all of her post-birth tests with flying colours and the new parents headed home with their bundle of joy to start their lives together as a family.
But when Avi was 6 weeks old, Bethan noticed she seemed quite "floppy". She didn’t move her arms and upper body or shift position in her sleep. Bethan took her baby to the paediatrician who ran some blood tests.
It would take over a month to receive the results, and when they did arrive, they were devastating.
Avi was diagnosed with Type 1 Spinal Muscular Atrophy (SMA), a severe form of juvenile Motor Neurone Disease. "When Avi was first diagnosed, it felt like the end of the world," recalls Bethan. "At the time, Spinal Muscular Atrophy was the biggest genetic killer of kids under two years old. It was so scary to face that unknown."
Bethan and Jonny were told to prepare for the worst, and Avi was placed into palliative care. Her family moved from their home in the Northern Territory to Sydney, where Avi was involved in a clinical trial for SMA at just five months old.
But to everybody's amazement and delight, Avi's treatment began to work!
Emerging from her paralysed state, as well as being tube-fed, Avi was able to breastfeed without being totally exhausted, she began to move her arms, and sit up. Over time she continued to improve.
While doctors still don’t know what her future will hold, Avi was discharged from palliative care after five years of successfully responding to treatment.
"We know we are living on borrowed time, but the treatment seems to be pausing her disease progression," says Bethan. "There's no evidence, science, or data on how long she has to live, but we try just to focus on the here and now. Avi sets goals so she's not being held back by her illness. It's important to us that SMA doesn't define who she is."
Today, Avi is a determinedly independent girl with a fantastic sense of humour and a love of storytelling. She goes to gymnastics and does "rollie pollies" with her friends and joins in Ballet classes from her wheelchair.
Despite being described as "chalk and cheese", Avi and her little brother Hugo are best friends. They bond over their shared fascination with cruise ships - which their mum used to work on as a professional dancer - and love to roleplay a high seas adventure together!
For Jonny, Avi's condition has put everything into perspective for the family.
"We just don't care about the things we used to anymore. When we have had close calls with Avi, it makes everything else seem so insignificant. People say how laid back and patient we are as parents, but it's because we understand what's really important in life because of Avi's prognosis."
For Avi, the hardest part of her condition is having to be in hospital so often. She splits her time between Royal Darwin Hospital, and a children's hospital in Sydney where she has her specialist treatment.
She is traumatised by her hospitalisation experience, and is terrified of the needles that are a constant part of her care.
One shining light for Avi throughout her hospital journey has been the fun and positive energy of Captain Starlight and the Starlight Express Rooms.
"Avi loves the Captains so much," shares Bethan. "She measures her days around when she can go to the Starlight Express Room, or when the Captains can visit her on the wards if she’s in isolation."
When Avi is well enough to visit the Starlight Express Room, she loves that Hugo can join her, and that they can have some family time together, away from all the medical interventions she faces.
Bethan also appreciates the happy vibes the Captains bring. As a parent in hospital, she is thankful for some light-hearted human interaction to distract her from sadder moments.
It's on the special occasions that Avi has missed out on though, that the Captains have made the greatest impression. The events run by Captain Starlight for the New Years, Easter and Halloween Avi has spent in hospital have helped her experience the joy of being a kid, when she would otherwise have missed out. To those who support Starlight, Bethan and Jonny have a special message of thanks.
"Thank you so much for making such a huge difference to kids who really need it. Starlight has shone a bright light on the darkest times for our family. The Captains have taken our daughters' mind off the hard medical procedures that she’s gone through over the last six years and they never fail to put a smile on her face. When things are tough, the Starlight Express Room has helped our whole family to be together to have some much-needed fun and laughter, which truly is the best medicine!"
