Twenty-month-old Isabella Crosby acts like an average toddler; babbling constantly, moving around at lightning speed and keeping her mother busy with her demands. However, a large scar, which snakes across her distended abdomen and around her back, indicates her life has already been far from normal. A rare, life-threatening disease has ravaged her liver and a transplant may be her only chance for a future.
Isabella scoots across the timber floorboards on her bottom, pulls herself up by the edge of the coffee table and grins mischievously. Her 29 year old mother, Yani, watches intently. Her eyes are full of love but a sadness lurks in their depths.
Yani yearned to be a mother but thought falling pregnant would prove extremely difficult, if not impossible, due to polycystic ovary syndrome. She buried hopes of ever holding her own child, until she conceived “a very amazing mistake”.
She tells me how her dream pregnancy turned nightmarish. From 14 weeks she battled constant nausea; at 16 weeks her blood pressure began to climb; and by 18 weeks she weighed an extra 20kg. Diagnosed with pre-eclampsia, her blood pressure soared and dipped, causing her to faint several times. This resulted in regular hospital stays, increasing in length when her baby appeared to cease growing. Yani’s placenta also began to rupture so at 27 weeks, her baby girl was born via emergency caesarean. On 21st March 2015, 13 weeks premature, Isabella weighed just 750g.
13 weeks premature, Isabella weighed just 750g.
After her dramatic birth, a ‘very yellow’ Isabella spent 108 days in a Neo-natal Intensive Care Unit (NICU). Paediatric Gastroenterologist, Dr Madhur Ravikumara, who treated Isabella, confirms jaundice is common in newborns but should be investigated if it persists beyond two weeks. Isabella’s jaundice hadn’t dissipated and scans revealed issues with her liver. A needle biopsy showed Isabella’s bilirubin levels (pigments in her blood) were extremely elevated, revealing her liver was not functioning properly. Doctors confirmed she suffered from biliary artresia – a condition where malformed bile duct openings trap bile, forcing toxins to build up and damage the liver.
Yani says once Isabella’s weight reached one kilogram surgeons performed the Kasai Procedure (a portion of the intestine is used to surgically bypass blocked bile ducts to allow bile to drain to the gut). The surgery took around eight hours.
“I panicked the whole time,” Yani recalls. “We left the hospital because my anxiety got a bit too much. The nurses kept me in the loop about what was going on and then I came back and just sat in the waiting room … and waited … and waited, until she came out.”
Seeing her tiny baby in a crib surrounded by tubes and ‘beeps’ she says was, “probably the most confronting thing that I’ve ever been faced with …”
Dr Ravikumara states that the majority of babies born with biliary atresia require a Kasai procedure but achieving bile flow is not always attainable and the liver may continue to deteriorate. Yani believes Isabella’s liver was too damaged for the surgery to be successful, explaining her daughter already has cirrhosis and portal hypertension (partially blocked blood flow increasing pressure to the portal vein, which can cause gastrointestinal bleeding). This is evident by the venous pattern on her swollen belly. Bulging tummies, explains Dr Ravikumara, are due to enlarged livers and spleens and possibly fluid accumulation. In her daughter’s instance, Yani views the Kasai as “just a preventative, it’s kind of like a Band-Aid.”
Isabella undergoes regular blood tests to assess her bilirubin levels and ingests a “tonne of different medications” including vitamins (as her body struggles to absorb them) and a daily antibiotic to prevent infection.
Fortunately, her premature birth hasn’t manifested in other physical or developmental health issues and for that, Yani says, “We’re so blessed. She’s an absolute miracle.”
However, in August 2016, doctors advised Yani that “a transplant is inevitable” and will need to be done within the next 12 to 18 months. “I think we’ve got to the point now it’s very factual instead of being emotion-based … It’s too hard. I think if I cry every time I get upset about it or something, I wouldn’t get up in the morning …”
Doctors advised Yani that “a transplant is inevitable” and will need to be done within the next 12 to 18 months.
Apart from watching for yellowing eyes – the first sign Isabella’s liver is failing – Yani has purposely not researched the transplant operation. “It’s hard enough hearing all these words that no parent should ever have to hear in their life … it gets to that stage where you just don’t cope, and I didn’t for a long time. [For] a long, long time I didn’t cope at all.” Yani’s voice quietens and tears glisten in her eyes but she blinks them away and continues. “I think it benefits us this time not to know anything about it. I just sleep a little bit better at night. I’m sure it’ll come and I’m sure they’ll start telling me about it in the new year. But until then, no, I don’t want to know anything.”
Dr Ravikumara’s comment justifies her fears, “Liver transplantation is a major undertaking, associated with significant morbidity and even mortality. In general, the bigger the child, the better the outcome.”
To add to Yani’s distress, Isabella must fly to Sydney for the surgery as no paediatric liver unit in Perth exists to enable transplants to be performed on children. She may need to remain in Sydney for up to six months depending on the outcome. Although medical costs, flights and living arrangements are met by the Government, Yani says the emotional strain of being away from her support network, and the financial burden of losing one wage, will weigh heavily.
With support from her husband, Joe, and an au pair, Yani returned to full-time work in January 2016, looking to seek solace in keeping busy. She admits working and placing Isabella into day care has been the “best thing” for their family, and her daughter has thrived.
Interacting with others also gave Yani the impetus to become proactive in campaigning for a transplant facility in Perth, and to raise awareness of Isabella’s plight – and those of other children in her position – by fundraising for the liver foundation. “This is going to be our life. It’s going to be Izzy’s life. Why should another family have to go through what we, and families before us have gone through?”
She adds, “The last two and a bit years have been the greatest years of our life but they’ve also been the most horrific years.” She admits that if her pregnancy had run smoothly she would definitely have planned to have more children. Although biliary artresia is not hereditary there is no known cause and Yani is reluctant to try to become pregnant again as Isabella requires so much attention. Currently she must be taken to regular appointments, follow a diet, and Yani isn’t sure how much care she will need after the transplant. “I wouldn’t want another child to miss out or to feel like everything is dedicated to Izzy and not to them.”
Yani no longer relaxes with a glass of wine as she has chosen to give up alcohol, hoping that Isabella will notice and abstain as well. Before her daughter needs to worry about peer pressure, Yani hopes her toddler sails through the next 12 months “as unscathed as possible” and her memories of this time fade with her scar. “I don’t want her to grow up and remember being poked and prodded. I want her to grow up like any other kid. After everything she’s been through, [I hope she’s] a happy, healthy, compassionate, kind little girl.”
After everything she’s been through, [I hope she’s] a happy, healthy, compassionate, kind little girl.”
To help raise funds toward a liver transplant unit in WA, please contact The Liver Foundation of WA on +618 9336 3178 or firstname.lastname@example.org