20 January 2008, 14:24--Tiny Leone van Jaarsveld has to double her weight before she can undergo a life-saving liver transplant.
Cherub-faced Leone of Vanderbijlpark in Gauteng is only 11 months old and likes fluffy toys, giggles uncontrollably when amused and throws tantrums when she is unhappy. The difference between Leone and other babies of her age is Leone's size - she looks like a two-month-old and weighs just 5,5kg. That's because she suffers from an inflammatory liver disease called Biliary Atresia, and is fighting for her life. Her enlarged liver is rejecting her blood flow. Without a transplant she will die.

When she was just over five months old, and after repeated tests and incorrect diagnoses, it was established that Leone had the liver disease. Her parents, Anton and Kiewiet van Jaarsveld, were then told of their four options. They could opt to put her on the government's waiting list to have the operation at the Red Cross Children's Hospital in Rondebosch, where all costs would be covered by the State - but there were no guarantees on how long the wait would be. They could approach a medical aid scheme, but none has been willing to assist because of the operation's high cost. Alternatively, they could raise the money on their own, up to R500 000, and go to a private facility - the Donald Gordon Medical Institute in Johannesburg. Lastly, Leone could be taken off all medication, taken home and made comfortable for her last months.

They chose options one and three: to put her on the State waiting list and to start raising the money to go private. But before doctors will even consider the operation, Leone has to gain 5kg to reach the minimum weight of 10kg.

To do this, nutritionists have designed a special diet for her which includes albumin, the protein responsible for lean body mass. Her special milk formula supplement contains a large dose of kilojoules. Her mother said she also has weekly hospital visits to drain her body of the fluid it retains. As young as she is, Leone is aware of her visits to Donald Gordon and the Cormet Clinic in Johannesburg. "She will never get used to the hospital. She never sleeps deeply there. When she sees the doctor, who puts the needle in her tummy to drain the fluid, she starts crying."

Her illness is also taking a toll on the family, and the Van Jaarsvelds admit their lives are structured around Leone. They have largely stopped socialising with family and friends as they are never sure if Leone will be up to it. The family kitchen cupboard-fitting business is also taking strain, and Anton has had to cancel many jobs to be with his daughter. "People will tell us everything is going to be fine and we must keep believing that. But no one else can really understand that. It's so hard for us to see her going through this."

But the baby, who is not able to sit on her own, is curious and intrigued by her surroundings. "Her legs and arms are never still, she is always busy with something or watching something and trying to figure things out." She also draws stares from onlookers when the family is out, not because of her diminutive size or enlarged abdomen, but because of "how cute and adorable she is".

Liver biopsies done last August and then again earlier this month, show that the condition of the liver is not deteriorating. This means there is no immediate deadline for her transplant. The family have no doubt their little girl will survive. "Leone was born to stand out; she is destined for great things. As parents we will be here to love and support her. She is a beautiful girl and her future is bright," said Kiewiet.

Faiza Steyn, of the Western Cape health department, said young children with "irreversible liver disease" were candidates for a transplant.
But Steyn said a range of factors were considered in liver transplants. These included finding a suitable match and the health of the recipient when a liver became available. The transplant costs R300 000 to R500 000, and, in Leone's case, can only be performed at the Red Cross Children's Hospital or the Donald Gordon Medical Institute.