He’s bright, curious and charismatic with a wide grin that endears the almost two-year-old Devonport boy to all who meet him.

He adores animals and spaceships, he loves dancing and playing with toy cars, and he also enjoys sharing cups of tea with his teddies.

But delve beyond his cheeky smile and the heartbreaking reality is that George has a life-threatening medical condition and he urgently needs a liver transplant to save his life.

George suffers from idiopathic extrahepatic portal vein thrombosis (EHPVT), a rare condition that is often difficult to diagnose and treat.

The condition is characterised by the blockage of the portal vein (which drains blood from the gastrointestinal tract, gallbladder, pancreas and spleen to the liver), without any known underlying cause.

George’s portal vein is irreversibly blocked, leading to severe portal hypertension. And portal vein thrombosis (a blood clot in the major vein of the liver) puts him at risk of life-threatening internal bleeding.

Despite his young age, George has already undergone nine general anaesthetics and has endured multiple hospitalisations and interstate trips for medical procedures, having spent a lot of time seeing specialists at the Royal Children’s Hospital in Melbourne.

George’s parents, Joanna Benjamin and Nic van Essen, now face the daunting and heartbreaking reality of their beautiful boy needing to have liver transplant surgery.

They are currently in Melbourne, where doctors are working through the final checklist of blood tests and other procedures needed to get George onto the liver transplant list.

Joanna, 27, says in some ways it’s a relief to have a diagnosis and have hope for the future as they get closer to George receiving a new liver.

But the transplant procedure, which will hopefully save George’s life, also comes with immense risks and a lengthy recovery period.

“We’re still processing a lot, a liver transplant is a huge surgery and George is still so small,’’ Joanna says.

“Outwardly, he seems like a healthy child, so it’s difficult to come to terms with what we have been told. But we are ready to do whatever it takes to care for him in every way throughout this journey.’’

George was born in June 2022, and for the first few months of his life everything seemed perfectly normal.

Then, when George was five months old, his poo was suddenly black.

The first-time parents were concerned – they turned to Google and discovered it could be related to internal bleeding or other serious health issues, so they took George straight to a GP.

But they didn’t get the support they had hoped for.

“They laughed me off,’’ Joanna recalls.

“They said it was not blood and to stop being so paranoid and stop overthinking everything.’’

Despite their concerns, Joanna and Nic took the doctor’s advice. But they soon realised there was something seriously wrong with their son.

“One-and-a-half months later George woke up in the morning, vomiting blood, at which point we realised this is obviously very serious and called an ambulance,’’ Joanna says.

George ended up having a blood transfusion in Burnie and was sent via air ambulance, with Joanna by his side, to the Royal Children’s Hospital in Melbourne for specialist care.

Nic quickly flew over on a commercial flight to join them.

George endured two weeks of tests, until doctors were finally able to diagnose EHPVT, followed by another week of learning what that condition meant for George going forward.

“It was so confronting to hear,’’ Joanna recalls.

“That it would be back and forth (from Tasmania to the Royal Children’s Hospital) until he was 18 and beyond, that perhaps surgical options might be something to look at, and to hear he would have to have gastroscopies every few months for the foreseeable future, and all these medications.’’

Also confronting was the idea of how their single income family would cope with the stress and expense of having a sick child who needed extra support and regular trips interstate.

Nic works in IT, and Joanna was studying prior to having George, completing her TAE qualification with a plan to teach early childhood education.

But George’s condition and the need for constant and careful monitoring means returning to the workforce is not an option for Joanna, and Nic has had to take extended periods of leave from his job to provide emotional support to his partner and son during their frequent trips to Melbourne.

They’re often required to fly to Melbourne at a moment’s notice, and the timeline is usually sketchy.

“The nature of these trips is unpredictable,’’ Joanna says.

“Emergency situations present frequently and delays happen, as you would expect in a large hospital. We can’t book a return flight until we are discharged on the day, as we can never rule out unexpected delays for George’s imaging, meetings or appointments.’’

George’s extended family members established two GoFundMe campaigns to lighten the financial burden, in a bid to help cover medication costs, and the cost of extended stays away from home, and ultimately allow Joanna, Nic and George to stay together through George’s liver transplant surgery and during his recovery.

The first campaign, when George was first diagnosed, raised $12,000, to assist the family with the initial expense and upheaval of being away from home. A subsequent campaign, established especially to support the family during George’s upcoming liver transplant journey, has raised more than $18,000, and Joanna and Nic say they are fortunate to have the support of so many wonderful family and friends during such a challenging time.

“We know that outside of our routine trips for imaging and monitoring George’s condition, there is likely to be a number of trips to Melbourne with little to no notice,’’ Joanna says.

“Each of which may, or may not, result in a transplant going ahead. We have been told to expect two or three false starts, (which would mean) we get home again in a day or two. Or it may mean we will be away from home for months. It’s impossible to plan around that, (so the GoFundMe) is about having the financial security to know that we can drop everything to focus on George’s recovery as well as taking care of each other’s wellbeing through everything that we are about to face this year.’’

Having a child with a serious health condition has meant that everyday life has had to change in many ways.

“We do feel like we’re living on edge a lot of the time,’’ Nic, 31, says.

“And it’s honestly just incredibly mentally taxing on all of us just not really knowing if today is the day we have to call another ambulance. It’s hard to plan things knowing he may need care urgently, but he is our top priority, and we are ready to make the changes that we need to make for him, we have learned to make our plans, accepting that they may change at the last minute.

“We’ve very much accepted that we’ll be a single income family for the foreseeable future as we just can’t risk daycare or anything that would ever delay emergency treatment should George ever need it.’’

Joanna says it is “astonishing” to be at the receiving end of such generosity from the wider community, with so many people sharing messages of support.

“I know that while it is a gift of money, it’s really a gift of people enabling us to stay together as a family,’’ she says of the GoFundMe account.

“It’s the most difficult thing we’ve ever been through up until this point, so that (support) is something we will never forget.’’

She says having the three of them together as a family, to support George as well as supporting each other, was hugely important.

Since George received his diagnosis, Nic and Joanna have had to sit through many important meetings with medical specialists, with a lot of detailed information to absorb about his diagnosis, treatment options, medications and impending transplant. Together, the couple has had to make many important decisions about George’s future and his treatment, and Joanna says she is so grateful to have had Nic by her side.

“Nic and I have appreciated having two sets of ears to take in all the information in our conversations with the specialists over the past 14 months,’’ Joanna says.

Nic says they’re also grateful to the “incredible” teams at the Royal Children’s Hospital and the North West Regional Hospital for their “patience and help for our lovely boy and his two very stressed parents”.

Portal vein thrombosis itself is not rare, and it is often caused by liver disease, making it more common in adults. But George’s idiopathic extrahepatic portal vein thrombosis, which is idiopathic because it has no known cause, is extremely rare in children.

Other aspects of George’s case make it even more unusual – such as the anatomy of his upper gastrointestinal veins which have spread out like spider webs under the pressure from portal hypertension, some of which pose the greatest risk to him through spontaneous bleeding. Plus the position of George’s extensive clot makes the usual surgical options impossible for him.

George also has an enlarged spleen, abdominal fluid retention and high blood pressure. His enlarged spleen pushes on his stomach, which means he can only eat a few bites of food before feeling full.

George has to be closely monitored to ensure medical attention can be quickly sought if needed.

But despite the challenges, his parents say he’s a “happy” “bright” and “active” boy who “brings a smile to the face of everyone he meets”.

“He’s not really aware that his life is different to anyone else’s,’’ Joanna says.

“He definitely knows about it when he’s in the hospital though.’’

Joanna is urging other parents, especially first-time parents, to trust their instincts when it comes to their children, and seek a second medical opinion if they feel their concerns are not being heard.

She still finds it hard to believe that life changed so quickly for their family when George suddenly became unwell. Especially when most people who look at the energetic toddler have no idea about his uncertain future and the serious challenges he faces.

“He’s at such a beautiful age, everything is so interesting,’’ Joanna says of George, who turns two in June.

“He’s really bright, he’s counting to four at the moment ... he loves farm animals and books – he loves sitting to read books with us – and he’s very social. If we take him out to tea he’s making friends with people at five different tables across the room ... by the end of the night he’s made friends everywhere. It’s really lovely for us, because while he’s got this very serious condition going on inside, he’s not aware of it very much. He can go about day-to-day life, it’s so invisible. Outwardly he looks like a healthy, happy child – looking at him you wouldn’t have a clue.’’

As they get closer to having George’s name on the transplant list, Joanna and Nic are preparing themselves for the upheaval of spending a long stint away from home.

They need to be prepared to drop everything to make the trip to Melbourne for a transplant if a liver becomes available, and they have their fingers crossed that a successful surgery can happen soon and be free of complications, to give their precious George a new lease on life.

Even if George is lucky enough to receive a transplant, the road to recovery is a daunting one, with an estimated three-month recovery period, which includes time in ICU, followed by a ward stay and some time staying in Melbourne to be seen as an outpatient, as well as his recovery time at home once George can return to Tasmania.

A liver transplant comes with lifelong immunosuppressant and anti-rejection medication, as well as frequent procedures for monitoring.

Medical experts say that getting George’s name on the transplant list, to receive a liver from a deceased donor, is his best chance of survival. Donate Life figures show that in the past year, 1396 Australians received organ donations from 513 deceased donors, including 57 Tasmanian transplant recipients. There were 289 Australian recipients who received a liver last year.

Joanna and Nic have also discussed the idea of becoming living donors if required.

“As his parents, our first thought was that one of us would be a living donor,’’ Joanna explains.

“However we have been assured that by following the usual transplant list pathway, it should not become necessary to take that risk. However, if George’s condition was to rapidly deteriorate and a transplant had not become an option, it would be our intention to both undergo a work up to determine whether either of us could be living donors for him.’’

Joanna and Nic know the risks of major transplant surgery for George and the possible complications, but Joanna says they have had many “reassuring conversations” with surgeons and nurses at the Royal Children’s Hospital and remain hopeful for the future.

“A lot young children who receive a liver transplant go on to live a very normal life which is wonderful,’’ she says.

“A successful liver transplant would save his life – there’s no other conversation I could begin to have. We have put our hearts in the hands of skilled surgeons, experienced specialists, and in many ways, all of the incredible people who register as organ donors willing to give a part of themselves to save a life and live on in a special way.’’

Like any parents, they just want to see their son lead a long, healthy and happy life.

“We see a bright future ahead for George,’’ Joanna says.

“He is intelligent, empathetic, has a wonderful sense of humour and such a strong ability to learn how things work. I think if you were to ask him what he’d like to be he’d tell you ‘an astronaut, a farmer, a doctor, a builder, and a rockstar’. Of course we want to give him the best opportunities that we can to achieve his dreams. But for now we see a sweet little boy, who will grow into a resilient man with a hell of a story to tell!’’ •

To follow George’s transplant journey or to make a financial donation visit gofundme.com/f/little-george-needs-a-lifesaving-liver-transplant