BBC1, Tuesday 24 January 2012 |
In between the epidemic of 'will they, won't they?' relationships spreading throughout Holby City, the show dealt with the genetic disorder epidermolysis bullosa (EB).
EB is recessive inherited, and causes the skin to be fragile and blister easily in responses to minor injuries, friction and scratches. It affects around 1 in 17,000 live births, with around 5,000 people thought to have the condition in the UK.
Cindy, 16, has a type of EB where the blistering occurs in the basement membrane of the skin, known as dystrophic EB. She is in hospital to undergo a bone marrow transplant. The donor, Kay, is her 15-year-old sister, who is a genetic match.
Kay appears distant from her mother, Pam, shown to be over protective of Cindy, and her jealousy highlights the issues siblings go through in such situations. Kay then withdraws her consent for the operation, leading Pam to be openly hostile to her in front of Cindy.
However, Cindy responds to the conflict by ripping at her skin, causing herself severe pain and bleeding. The situation is worsened when Dr Malick attempts to stop Cindy, causing her even more pain, which made me realise just what a difficult disorder EB is to treat.
Kay eventually agrees to the transplant. While she is still a minor, legally her consent is needed as she is deemed ‘Gillick' competent, a term used when deciding if children are mature enough to make their own choices. This was an area the show could have dealt with more thoroughly. Overall the storyline was compelling, if a little clichéd in parts, and I think it's important that genetic disorders take a more prominent space in the public consciousness.
The outcome of the bone marrow transplant was left unknown. The treatment is still in its early stages, with two US studies showing mixed and inconclusive results. At this point there are no planned studies in the UK.
Sources and References
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Holby City, Series 14, Episode 15
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Holby City episode features EB story
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