Fix My Genes (Great Ormond Street)

The programme gives a fascinating insight into the different dilemmas facing children with severe immunological problems

The programme gives a fascinating insight into the different dilemmas facing children with severe immunological problems

Broadcaster: BBC 2

Year: 2015

Genre: Reality TV, Documentary, Fly on the wall


Review by: Prof John Bryant (University of Exeter)

“In this area of medicine, nothing is risk-free

If I were to say what mistakes we had made over the years, it would always be that we have gone too far. We should have stopped earlier. We have added to the suffering of this child and family. But …”

Great Ormond Street is the flagship London hospital for paediatrics. It has been the focus for a long-term “fly on the wall” series. In this moving and sometimes upsetting episode Fix My Genes (Season 3, episode 1, 1 hour duration) we are introduced to three children who were born with inherited conditions that affect their immune systems. In all three, the conditions are, to different extents, life-limiting.

Sibling donation: Herb is a lively five-year-old who needs a bone marrow transplant due to a rare genetic disorder, NEMO. Without it, he is ‘unlikely to reach his second decade.’ His six-year-old brother Rufus is a perfect match and their parents give consent for Rufus to donate bone marrow to save his brother. They are warned that with Herb’s condition there is a 10% chance of mortality. In this instance everything goes well and we see the happy outcome of the procedure, albeit that Herb was in hospital for several weeks before being allowed to go home. I note that we also meet Herb’s sister Lily, who, rather strangely, has started to show symptoms at the age of ten. She may at some time in the future need a transplant but certainly not for the present. Finding a matched donor would be an issue here.

Mismatched donation: Keano is a nine-year-old boy whose condition has recently worsened. His lung function is compromised and he now has indications of a ‘pre-leukaemia’ state that is likely to develop into full-blown leukaemia. Without a bone marrow transplant he is very likely to die within the year. However, because of his very mixed ethnic origins, finding a matched donor is very difficult (he has no siblings – or at least, we were not told of any siblings). The transplant team suggest that his mother, a 50% match, is the best donor available. Transplants with partly matched donors have been to some extent successful but it is ‘a massive gamble’, the ‘level of risk is very high’ but on the other hand, Keano’s condition is severely ‘life-limiting’. The risk of mortality during or after the procedure is put at 30%. In the event, the transplant was not successful but in the meantime, a matched donor had been found in the USA and the necessary formalities for sending the cells to London are completed ‘in record time’. Keano thus has a second transplant soon after the first has failed. There is then a period of several weeks during which he develops a rare and very dangerous fungal infection but eventually the transplanted bone marrow begins to work and Keano survives.

Gene therapy (starts at 24:15): The third child is a chirpy ten-year-old Scottish girl, Teigan who has to make the 400-mile trip from Glasgow for her treatment. She has SCID (severe combined immune deficiency) which is a condition for which gene therapy has been successful (at the time of the programme, for 35 children). Indeed Teigan had started on a gene therapy treatment  when much younger but it had to be abandoned because of her becoming too ill during the ‘chemotherapy’ phase of the process. In the meantime, she is kept alive by drugs and by monthly injections. Is it now time to try gene therapy again? The conclusion reached is Yes, and we see attempts to extract enough of Teigan’s stem cells on which to perform the genetic manipulation. In the sequences filmed for the programme, these attempts were unsuccessful. It is apparently harder to mobilise enough stem cells in older children than in small children and babies; Teigan is the oldest child for whom Great Ormond Street have attempted this procedure. However, after the majority of the TV programme had been made, enough cells were obtained (by freezing batches collected on different occasions) and she was able to undergo gene therapy. We do not know whether it was successful.

I note in passing that the strain of having a seriously ill child in the family can put enormous pressure on relationships. Two of the couples had separated (although in Teigan’s case both were still involved in the decision-making).

This is a programme that raises deep issues in medical ethics, including issues around risk-benefit analysis, informed consent, autonomy, medical responsibility, transparency, commitment of resources …

If I were using this in teaching, I would first divide the group into three, with each group to focus on one child. Take notice of what the doctors say about the child’s condition and the need for treatment. How do the parents react – what are the main issues for them? How do they react to the statistics on mortality. Listen in carefully to the medical case conferences and to other clinical staff who become involved. Watch and listen to the children themselves. Each group can then do a presentation to the other two groups. To help with this, it might be good to get each group to nominate a few members to perform a role play – with ‘actors’ to represent the children (not forgetting Rufus, Herb’s slightly older brother), the doctors, the parents (or in Keano’s case, the mother and her father, Keano’s grandfather). See whether you can come up with a brief role-play that presents the main ethical issues.


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