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Kristian Adey is a lively 13-year-old with a mischievous sense of humour, who loves animals and playacting. But these days he is often in a wheelchair because he tires easily, goes blue and becomes breathless. The brutal truth is he is dying. His mother Maralyn has watched his decline.

Kristian Adey spends much of his time in a wheelchair

"The frustrating part is his mind is telling him to do things that everybody else is doing, all his friends are doing, go to the disco, to dance, to play rugby, to play football, but his body won't let him," she says.

Within three weeks of his birth, it was clear that Kristian was seriously ill. Like 40% of children with Down's, he had a congenital heart defect. Doctors know the condition as atrio ventricular septal defect - or AVSD for short. Most of us call it a hole in the heart.

Even 13 years ago, it was well known that any attempt to fix it should be done sooner rather than later. Back in 1985, Kristian was referred to Killingbeck Hospital, a specialist heart centre in Leeds. There, the Adeys had a series of consultations with a senior cardiac paediatrician, Dr David Dickinson.

"That was when he told us it was inoperable and to take Kristian home and enjoy him whilst we've got him," Maralyn Adey says.

Risky procedure

Although the procedure was regarded as risky at that time - there was a one in four chance of dying on the operating table - it was the only chance the five-month-old Kristian had of living a full life.

From his medical notes, it is clear that Dr Dickinson focused on the "difficulties" of surgery and emphasised the risks, which he rated as "extremely high". The notes concluded that the Adeys would be "coming back to see me in 3 months". But with every month that passed, time was running out for Kristian.

The BBC took the medical notes to a leading surgeon at Great Ormond Street Hospital for Sick Children and asked him what they would have done.

Dr Martin J Elliott, Consultant Cardiothoracic Surgeon, said: "[For] a child like this at 5 months of age, nowadays we would do a complete repair straight away. In 1985, we might have done it in two stages, but certainly we would have intervened."

It was not just the Adeys who got the idea that Killingbeck would not operate. The parents of Maria Hinds, who was also born with a hole in the heart, received a very similar impression back in 1988. Mother Celeste Hinds says: "Dr Dickinson said 'well, she's got a major heart defect. There's nothing that can be done. Its inoperable'.

Canadian hospital

The Hinds were determined to leave no stone unturned and discovered that a hospital in Canada did the operation as a matter of routine. Then they went back to Dr Dickinson, but could get no satisfaction:

Maria Hinds made newspaper headlines

"He just looked at me very quietly and said 'you don't want to be asking where it should be done, but whether it should be done...anyway we do that operation in this hospital"_and I said to him 'do you do this operation on all of the babies with this heart defect, both the babies with Down Syndrome and the regular babies?' and he said 'oh no, only on the normal children in the 6 years that I have been here', Mrs Hind recalls.

Eventually, the Hinds family raised money through a public appeal and took themselves off to the Toronto Hospital for Sick Children. When the Canadian doctors did their own tests they assessed the chance of a successful outcome at 99%.

Dr Robert Freedom, Head of Cardiology at the Toronto Hospital, recalls the case: "The risk to Maria at the time of surgery was extremely low and clearly the benefit was very high. She would have certainly become chronically ill in her teens or 20s, with a much shortened life span, if she had not been operated on here.

"In my 24 years at Sick Childrens the issue of whether a child with Down Syndrome should or should not be operated upon has not been an issue for us. We do not discriminate against children with Down syndrome."

Media attention

When Maria flew back home in September 1988 she was famous - her story having caught the imagination of the media in both Canada and here. Maria is 10 now and doing fine. Although children with Down Syndrome have a shorter life expectancy than other children, she is expected to live to her full potential now.

Toronto

Dr Dickinson declined to be interviewed by the BBC, but he did issue a statement. In it he says: "Given the high mortality for the operation, I would not have been pushing families into agreeing to surgery". He also does not think he was "out of step with practice in the UK at that time..."

The hospital in London that Zena Rose was taken to eight years ago was in no hurry to operate either.

Her father Brian Rose remembers the conversation with the doctor: "He told us that she had an AVSD he obviously wasn't inclined to operate on - he said that we should go away and return in 6 months to the outpatient clinic.

Lost records

When the BBC asked the Brompton Hospital in London to comment on the case, the Brompton said they could not find Zena's medical records - they did not even know which specialist had seen her.

What the Rose family did not know was that the medical team at the hospital had caused a stir in 1985 by writing a paper which promoted the idea that "children with Down Syndrome should be treated differently to normal children with AVSD" - not because the operation was any less successful or more risky in their case, but because children with Down have shorter lives anyway and would benefit less.

Many would regard this as a straight case of discrimination. However, Dr Elliott Shinebourne, Consultant Paediatric Cardiologist at the Royal Brompton Hospital disagrees.

"It's not a case of straight discrimination, life expectancy is different for a person with Down Syndrome than a chromosomally normal person and the life goals and expectations are not the same," he says.

Life expectancy

Zena Rose was eventually operated on at the Alder Hey hospital in Liverpool by Dr Roxane McKay. The surgeon takes issue with Dr Shinebourne.

Zena is now a very active eight-year-old

"You can say OK, Down's have a shortened life expectancy, so we won't bother to treat them. But then you might say, well, people who have blue eyes have a different life expectancy than people who have grey eyes - so shall we stop treating people with grey eyes. I mean this is the whole discrimination issue."

The date of Zena's operation was July 1990. The date is significant, because by 1990 the success rate for the operation on very young babies had improved dramatically - and the doctors at the Brompton were fully aware of this development.

Dr Elliot Shinebourne accepts this: "I think the awareness came in about 1990 or a year or two before when a paper was published from Melbourne Children's Hospital and they had a mortality for the operation of about 6-7%. We had also changed our policy around that time and certainly subsequently to that paper we have tried to operate on any child with that combination of abnormalities within the first 6 months.

Changing policy

Whatever their policy towards other children, in 1990 the Brompton Hospital saw Zena when she was 5 months old - and told her to go away and come back a few months later. Brian Rose is glad he ignored their advice.

Dr Elliot Shinebourne: "expectations are not the same"

"If we had paid attention to what the Brompton people told us, then Zena would not be here now. If we'd waited those six months she would just have deteriorated I'm sure, and died as Miss McKay outlined to us."

Zena is now eight and a picture of health. She swims like a fish, rides horses and does ballet. Unlike Zena who had the operation, Kristian Adey is now severely restricted in what he can do physically, but he is shining in other ways. He has won a prize for academic excellence in his hometown of Bradford, and 2 years ago the Prime Minister presented him with a Child of Achievement Award.

"I often feel like picking him up and putting him in front of these doctors and saying 'this is the child you said wouldn't do anything', his mother says. "But I can't do that, much as I would love to."

The Royal Brompton gave the BBC a statement in which they said they were proud of their record and never had a policy of discrimination based on the presence of Down Syndrome. They said their results compared favourably with the best in the world and were superior to many other hospitals. But they did not explain why they had not operate on Zena Rose