It has taken four surgeries, one liver transplant, and more than 114 days in hospital to help 16-month-old Stella McGear battle a life-threatening disease.

When Cape Town parents Adrian and Judine McGear took baby Stella to their paediatrician for her six-week check, they were concerned about her jaundiced appearance. “Her skin was yellow from her chest upwards, even though we had heeded the doctor’s advice to let her lie in the sun,” Adrian explains.

Blood tests ordered to gauge Stella’s liver function were just the start of what would become a year-long ordeal. The results showed there was something wrong with the organ, and she was booked into hospital for a full week of further tests and scans. “The hardest part for me was knowing how hungry and unhappy she was,” says Judine, who was advised not to visit her breastfed baby. Stella was on a nil-per-mouth diet and the scent of Judine’s milk would make her feel even more frustrated, doctors explained.

After more blood tests, plus an MRI and CT scan, doctors couldn’t confirm whether Stella did, indeed, have biliary atresia (a condition where a child is born without the ducts that allow bile to drain from the liver), as they suspected.

“Biliary atresia is an extremely rare condition affecting only one in 5 000 live births.”

Dr Francisca van der Schyff, transplant surgeon at Wits Donald Gordon Medical Centre.

Proud dad Adrian McGear with Stella, who’s now a “friendly, people-loving little person”, according to mom Judine.

The first surgery

Eventually, they decided that Stella would undergo a Kasai procedure, an operation in which sections of the small intestine are attached to the liver, so bile can drain through the intestine. Although not a long-term cure, it would buy her some time until the medical team could settle on a more permanent solution. “The procedure was the longest four hours of my life,” Adrian says. “Afterwards, she slept for three days, waking up on Father’s Day – the best present I could have hoped for.”

Stella’s Kasai procedure had been rescheduled twice because she had tested positive for COVID-19 each time she was supposed to check into hospital – lost days which proved crucial, as her health deteriorated rapidly. By this time, it was clear the little girl was in dire need of a transplant, a procedure that had to take place in Johannesburg at Wits Donald Gordon Hospital, where the only team qualified to operate on young children is located.

“Knowing there is a part of me keeping her alive makes our bond incredibly special.”

Adrian McGear

By the time the family arrived in Johannesburg from Cape Town to consult the transplant team, Stella was days away from celebrating her first birthday: a skinny little girl whose distended belly and darkened skin revealed the seriousness of her condition. She was admitted to hospital, where she received nutritional support – a necessary step to reduce the risks associated with the upcoming surgery. Meanwhile, both parents were tested to see who the more compatible donor was.

Here, too, they hit a stumbling block: Adrian’s blood type was a match, but he was found to have a condition called haemochromatosis, which causes the body to store iron at dangerously high levels. Doctors were initially reluctant to allow him to go ahead with the donation until they discovered Stella had inherited the condition, and therefore wouldn’t be exposed to any further risk from the donated liver.

Transplant day

Finally, in May, two months after the family’s arrival in Johannesburg, the day of the transplant arrived. Adrian’s procedure was successful, but Stella’s weakened lungs meant she battled to breathe when she was taken off her ventilator the next day. She was resuscitated within seconds, but another attempt to let her breathe independently proved similarly unsuccessful. This time, the team battled for three minutes to get her breathing again and decided to put her on an oscillator. “That was terrifying, because the machine made her entire body shake,” Judine recalls. After a third attempt, the team performed a tracheostomy, because they were concerned Stella’s throat would sustain damage from the repeated removal and reinsertion of the ventilator.

Finally, after 63 days in ICU, the McGears were able to visit their child in a medical ward. After a few more days they were discharged. Adrian’s own healing proceeded without incident.

Starting over

Judine says returning home to Cape Town was strange. “It was like we had taken home a new baby. We witnessed her skin and eye colour change and we’ve seen her become a friendly little person who is always smiling and loves other people.” She says in some ways Stella’s journey seems unreal – but, despite all the adversity the family has faced, she wouldn’t change it for anything. “God gave us this little girl for a reason, and I am so happy to be her mother.” Adrian shares her pride. “Knowing there is a part of me keeping her alive makes our bond incredibly special. It was an honour to be able to do this for her.”

Challenges still lie ahead for Stella and her parents, but Adrian believes “it’s a small price to pay”.

Although Stella’s biliary atresia has officially been cured, the family must remain extremely cautious to prevent infections. She will remain on immunosuppressant medication to prevent rejection of the donated liver for the rest of her life. “We know we have some challenges ahead of us – at some point, we’ll have to explain why she can’t share her friends’ food or why she looks different in a bikini to other little girls, for example,” Adrian says. “But that’s a very small price to pay.”

The surgeon’s perspective

Dr Francisca van der Schyff, who headed Stella’s transplant team, says biliary atresia is an extremely rare congenital condition affecting only one in 5 000 live births. It is very difficult to diagnose because, unlike syndromes that cause damage to several organs, only the liver is affected.

Because bile acids in the liver are unable to drain away, they accumulate within the organ, she explains. Initially, this causes a jaundiced appearance that doesn’t diminish over time. Within six months, if untreated, the child’s jaundiced colour will deepen, her belly will become distended as it fills with fluids, and she will become confused. Without any intervention, biliary atresia results in liver failure. Although transplant offers a permanent cure, Dr van der Schyff notes that it’s difficult to find deceased or living donors in South Africa.

Dr van der Schyff says although Stella can now look forward to enjoying good health, managing her immunosuppression medication will be a long-term balancing act. “That said, she can look forward to attending school, playing sport, going to university, getting married and eventually having her own children,” she says.

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