Ben Ayres-Evans, just on the cusp of adolescence, describes his life growing up in deepest rural Devon, with a heartfelt sigh. "Sometimes it can be very, very busy and sometimes very very boring." But unlike most 10 year-olds, Ben's family life for the past four years has revolved around his younger sister Stella's battle with acute lymphoblastic leukaemia or ALL.
For Ben, the busy times are when his sister is rushed to hospital with a life-threatening infection. He keeps a bag with pyjamas and toothbrush at school in case friends or neighbours have to take him home. Sometimes it's only days later that his parents can tell him what happened.
The boring times are the weeks that the family have spent living away from home in Bristol while their daughter undergoes treatment. And when Stella's immune system is vulnerable, cinemas, swimming pools and crowds are out of bounds. The worst for Ben is when his sister has to take steroids to stimulate her appetite after chemotherapy. "It gets annoying because it gives her a big appetite and makes her grumpy."
After Stella was initially diagnosed with ALL, the most common form of childhood leukaemia, she seemed to respond well to treatment, says her father Nic Evans. But last July, only half an hour after Evans and his partner Roberta Ayres had returned the outsized pushchair and the remains of her medicines to Exeter Wanford Hospital, the hospital rang. Leukaemic cells had been found in Stella's spinal fluid. They cancelled their summer holiday and she started another round of treatment. But the best hope for her recovery now was a bone marrow transplant.
In early March, Stella underwent her transplant at Bristol's Royal Hospital for Sick Children and her family are now cautiously celebrating the first signs that the procedure was successful. Ben was her donor.
Evans first broached the subject with his son last November. "Stella was in hospital with an infection and Roberta was with her. Ben and I had a little bonfire session together and I thought that was a good time to discuss it," says Evans. "He had a reasonable idea about what was involved, he knew there would be needles." But Stella had already softened the blow for her brother with hints about the "magic milk" you get before an operation. "The anaesthetic, that was nice," says Ben. Then dad threw a Lego computer game into the bargain.
But aside from talking to his parents, Ben also met with a paediatric oncologist who needed to be sure that he was aware of what a bone marrow transplant involved. "When they tested me and found I was a match, I was pretty happy about that," says Ben, who seems remarkably mature and articulate for his age. "I think the doctor said something like, 'Are you sure you want to have a bone marrow transplant?'" Having grown up with Stella's illness, he knew exactly what the procedure meant to her, to him and to his parents.
For Ben or any child who becomes a donor, this issue of consent is crucial. Reproductive technology has now moved ahead so rapidly that parents with a critically ill child wanting to conceive another can now apply for pre-implantation genetic diagnosis (PGD). An embryo that is an exact tissue match can be selected and implanted in the mother's womb. In 2002, Leeds couple Raj and Shahana Hashmi were given licence by the Human Fertilisation and Embryology Authority to conceive a child as a donor for their son Zain who has the potentially fatal genetic disorder thalassaemia.
In Northern Ireland, Joe and Julie Fletcher are anxiously awaiting the HFEA's decision to allow them to use PGD. Their two-year-old son Joshua suffers from Diamond Blackfan anaemia, a rare blood disorder that could be cured with a blood stem cell transplant from a tissue-matched donor. "In our case, we wanted another child anyway," Julie Fletcher has said in an interview. "Instead of having a 20% chance of a bone marrow match by conceiving naturally, we are just increasing our odds." There is no guarantee that their request will be accepted. In 2002, the HFEA turned down a request from a couple who had sought permission to use PGD for their son, who has the same disorder.
For parents with a sick or dying child, the issues are stark and most would do anything to prolong their child's life. But what is the emotional and psychological impact on what have become known as "saviour siblings"? American novelist Jodi Picoult has written a controversial novel exploring this emotional minefield from the perspective of a 13- year-old girl. Anna was conceived through IVF as a perfect tissue match for her sister Kate, who suffers from acute promyelocytic leukaemia. In My Sister's Keeper, Kate is now 16 years old and has had five years of remission before suffering a relapse. Her sister Anna has donated bone marrow twice and now that Kate needs a kidney transplant her mother Sara is applying subtle pressure on her younger daughter to give consent. But Anna refuses.
Picoult says that some parents who use PDG to create a saviour sibling in the US, where more than 6,000 procedures have been carried out, are demonised. "They are parents who start this process because they love their children and it's an odd Catch-22," she says. "We trust parents to make decisions in the best medical interests of the child but when there are two children's interests, that breaks down." Picoult discovered that hospital medical ethics committees in the US, which are designated to regulate such decisions, were run on an ad-hoc basis by medics who all had full-time jobs elsewhere.
The novel has some gut-wrenching descriptions of the pain that Kate undergoes and its terrible impact on her parents and siblings. Picoult has a particular empathy for such families after her own 10 year-old son Jake was diagnosed with a rare form of benign tumour in his ear four years ago. "As you watch your child undergoing a general anaesthetic for the umpteenth time, you think, 'Let me do it, let me have the surgery instead,'" she says. "We had to turn to our other two kids and say many times, 'Yes, you are going to have to change the date of your birthday party or holiday because your brother's having surgery.'"
Anna, who feels she grew up on a cancer ward, is finally given permission to voice her resentment at being designated her sister's saviour when she sues the state for the right to make decisions about her own medical care. "[In] addition to the piece of me that's always wanted Kate to live, there's another horrible piece of me that sometimes wished I was free," she confesses. Aside from the missed opportunities during childhood and the incredible sense of responsibility they feel towards their siblings, Picoult asks what happens if the donated kidney or bone marrow transplant doesn't actually work.
But Dr Simon Fischel, the doctor who treated Shahana Hashmi, is frustrated that medical bureaucracy and the law can stand in the way of helping his patients save their child's life. Fischel treated Hashmi, whose IVF treatment was suspended in late 2002, when a judicial review at the high court overturned the HFEA's licence to use PGD. Treatment was later resumed after the court of appeal overturned the high court's decision in 2003.
Fischel believes that concerns about the long-term implications for the donor child should not stop the conception of a saviour sibling. "There is no evidence that when an aura of goodwill and good health exists for that child, that it would be psychologically damaged even if the treatment doesn't work," says Fischel. He argues that there is a degree of "medical hypocrisy" at work where parents with a terminally ill child are often advised by their specialist to conceive another baby as a potential donor but may be refused this right if they opt for PGD.
"The parents are told their best option is to have another baby in the hope that it's a match," he says. "But in reality, they may have a match with another baby that has the same condition or they may have lots of children who are not matches. Or they may have a match who is born too late to save the sibling." The results of waiting for legal decisions may be devastating."
Shahana Hashmi has been unable to conceive another child. As Fischel points out, "The initial battle took so long that her age is now against her." Practically, there has to be an end point. While a successful stem cell transfer could cure Zain, Fischel says he is "OK for a child with severe thalassaemia, but his quality of life is miserable."
However, Dr Vivienne Nathanson, head of ethics for the British Medical Association says that a newborn infant giving cord blood is one thing but an older sibling donating bone marrow or an organ, is a much more complicated matter. "You may be subjecting a child to risk, to pain, to discomfort and, particularly if the child is very young, they may be frightened," says Nathanson. "We would say that parents wouldn't have the right to consent that for a child."
Specialists involved must determine whether a child understands and can therefore consent to such a procedure. In cases involving an application for PGD, Nathanson says parents are interviewed to assess whether they are conceiving a child they would love in and of itself.
Meanwhile, Ben is already thinking about the time when he will finally be able to tell Stella what it really meant for him to be his sister's keeper. "By then," he says, "she will be 12 and I'll be 15 and it will all be over."
