"A baby is seen poking its tongue out in an amazing ultrasound scan. But while the image may appear to capture a cheeky pose, Ocea Varney was unable to close her mouth after being born with a congenital defect which meant her tongue was enlarged.
Medics diagnosed the potentially fatal condition after she was born and Ocea, now 18-months, has undergone surgery to shrink its size. But the condition was only picked up after the baby's mum Melanie Varney insisted to doctors that was something wrong with her daughter.
Melanie 28, said: "You could even see Ocea's tongue sticking out on the ultrasound. I remember the technician saying 'look how cute she is, she's sticking her tongue out at you'. "When she was born it was the first thing I noticed - I thought it was cute at first, but it caused some serious complications."
Melanie, from Vancouver, Canada said doctors, nurses and even strangers told her it looked cute, but she was bigger than her twin sister and her oversized tongue caused complications with feeding. After her birth, along with twin sister Indigo, nurses kept her in the NICU for a week to monitor her low glucose levels.
Melanie added: "The moment I gave birth to Ocea I thought she looked different to my other kids. Obviously she had her tongue sticking out, but she was bigger than her sister. When we were out at the store we would get comments about her tongue poking out - most of them said she was cute but others thought she was being rude.
"A couple of times people said it looked like she was thirsty and panting, as if she was some sort of dog. But she was miserable with it - I would try and breast feed her but she just couldn't latch on. It kept me up all night either trying or worrying."
Melanie claimed after three months of doctors telling her and husband Gabby Mason, 30, it was just a phase, the family resorted to their own research. From Googling her symptoms they came across another girl with a similar story who had been diagnosed with Beckwith Wiedemann Syndrome- a congenital overgrowth disorder.
Despite it being a common condition - affecting one in 14,000 babies worldwide - very few doctors knew about it. Thanks to some further research, Melanie found a specialist in Brisbane, Australia where she was living at the time and made the trip to see him. She said: "As soon as the doctor saw her, he told us it was BWS - it was heart-breaking to hear she had the condition. But after three months, at least we finally had a diagnosis and could start doing something about it. She instantly had blood tests to check whether she had any tumours, which she didn't."
The most common characteristics of BWS are:
- a large body size,
- enlarged organs,
- enlarged tongue,
- midline abdominal wall defects and
- neonatal hypoglycaemia.
Melanie and Gabby moved their twins and their two-year-old son Joey back to Canada so they could be closer to family. At just seven months old, Ocea had tongue corrective surgery in Missouri and continues to visit the hospital every six weeks to be screened for tumours.
Children with BWS are at an increased risk for developing various types of tumours, particularly liver and kidney tumours, and as many as 20 per cent of babies born with the disorder will develop cancer. Shortly after Ocea's diagnosis, Melanie created the website How Big BWS with the help of her friend Tricia Surles - whose two-year-old daughter Lane also has the condition - to inform and educate families and medical professionals.
They have already arranged a How Big Is Yours? day which encourages people to post selfies of themselves sticking their tongues out. Melanie added: Our goal is to raise awareness about this syndrome. Up to a quarter of sufferers develop cancer so it's important that an early diagnosis is made. We want doctors to recognise BWS and ensure families know there is a support network out there for them. If that means getting people to pull a silly face then that's worth it.""