Bitter Inheritance is a series of five programmes looking at genetic diseases. In this first episode we follow the story of the Gorry family as they discover the reason behind a long history of devastating sudden deaths in the family. The lucky diagnosis of a distant relative with the rare genetic heart defect ‘Long QT Syndrome’, also known as Sudden Death Syndrome, starts a race against time to track down and test all relatives within the family, in the hope of saving as many lives as possible. This disorder results involves an abnormally long delay between the electrical excitation and relaxation of the ventricles in the heart, which can lead to syncope (fainting) or sudden death due to ventricular fibrillation. Once diagnosed, however, the patient can take a daily beta-blocker which essentially allows them to lead a normal life.
The story illustrates both the jubilation when a relative is found in time and the distress when the family’s efforts were in vain, including one time when they were literally two weeks too late (00:10:40). The programme raises an interesting ethical question, one that crops up in later episodes too, concerning who has a right to know genetic risk factors – the individual tested or the broader family? (This issue, incidentally, was the subject of a recent debate in the British Medical Journal. The full article is now password protected but a summary derived from a press release can be found here).
In this particular case, the discovery of an inherited condition prompts an aunt to reveal that she had secretly given up a son for adoption when she was sixteen (00:13:40 to 00:15:52, 00:31:12 to 00:32:15 and 00:36:30 to 00:37:42). The ethical issues here are therefore even broader – does her child know he was adopted? Does he want to know his biological mother? Does he want to know he may have a life-threatening inherited condition? What about sharing the information with any children he may subsequently have fathered? As it transpires, the questions prove academic as the boy had sadly died a number of years previously.
Bitter Inheritance: The Gorry Family, provides a unique insight in to the families desperate struggle to find all living relatives. The advancement in knowledge of the syndrome means that although they “can’t change the past, they can influence the future”. The programme demonstrates how improved understanding of the genetics of disease can change and save peoples lives. In this case, the family no longer has to live in fear of “the Gorry curse”.
This 40 min programme appears to have only been broadcast twice, 10th January 2002 (BBC2, 21:50) and 19th December 2002 (BBC 2, 21:50), but copies can be obtained from the BUFVC (TRILT identifier 00161D52). An article about the family can be found on the BBC website.
Follow this link for an account of The Allen Family, the second episode in the Bitter Inheritance series.