Podcast Review: Charlotte Raven on Life with Huntington's Disease

As a non-clinical research scientist, it can become commonplace to forget the practical reality of your work, so listening to 'Charlotte Raven on Life with Huntington's disease', an episode of the Bunker podcast, came as a stark reminder of the impact of neurodegenerative diseases. In the episode, Charlotte Raven, a British journalist who rose to prominence in the 1990s, discusses her life pre- and post- diagnosis and is joined by her clinician, Professor Ed Wild of University College London.

The story behind Charlotte discovering she has Huntington's disease is reminiscent of what I have heard several times through the academic grapevine, which is chronicled in her biography, 'Patient 1: Forgetting and finding myself'. At the age of 39, Charlotte was informed by her father that not only had her paternal grandmother died with the disease, but there was a 50 percent chance that he had passed on the disease-causing gene to her. As Charlotte quips, she did what any of us would likely do and 'stupidly went on Wikipedia and Googled 'Huntington's.' Immediately, she was confronted with the horrific nature of the condition, the symptoms of which Professor Wild recaps: inexorable progression of debilitating motor problems, drastic personality changes and cognitive difficulties such as impaired attention. Worst of all, the disease is currently incurable.

However, Nick Cohen, host of the podcast, notes that Charlotte's biography is not a 'misery memoir'; rather, it is an honest reflection of her life and her shifting perspectives – a description which reminds me of Paul Kalanithi's 'When Breath Becomes Air' but likely with more broadsheet wit. For instance, Charlotte indicates she has deep regret for past actions, such as her journalistic handling of the James Bulger incident, and describes her former self as destructive and narcissistic. However, she also insists her behaviour was reinforced by simply working in 'a culture that wanted her to be a controversialist' – a fact that none of us who consume modern media will be surprised by.

The podcast touches on the topic of genetic counselling – a process whereby those at risk of developing Huntington's disease are informed about the condition and given the option to take a conclusive genetic test. For Charlotte, she 'emotionally and intellectually felt that facing something head-on is going to minimise its impact later.' Although I concur with this perspective, we are in the minority as most individuals refuse to take the test. Professor Wild highlights that the decision is a balancing act between living in fear of developing Huntington's disease and the hope that you do not carry the disease-causing gene.

However, the road to Charlotte's diagnosis was full of diversions as she was frequently misled by holistic psychiatrists. This is indicative of the episode's overarching theme, the stigmatisation of Huntington's disease, which is reflected both in her father's suppression of their genetic history and the countless misdiagnoses from medical professionals. This stigma has historical roots in 19th century eugenics and the disease was even associated with witchcraft, the aftershocks of which we are still clearly seeing in modern-day medicine.

In the episode's most endearing moments, Charlotte focuses on how her family have been an emotional anchor throughout this process. Although both of her children are at risk of developing the disease, she doesn't regret having her son post-diagnosis. In her own words, 'I didn't want my daughter to be alone.' – a statement which, in my opinion, offers a contrast to the woman who described herself as narcissistic and destructive. Although Charlotte is aware of the difficulties ahead, particularly after watching her father succumb to the disease, her priority is clearly her family's wellbeing.

The literary account of Charlotte's life came to fruition as she wanted to create something which would outlive her, with the book's title hinting at her experience as patient number one in a clinical trial for Huntington's disease. Unfortunately, the clinical trial, which involved administration of the drug Tominersen by pharmaceutical company Roche, was recently halted as no benefits were observed in patients. However, the episode ends on an optimistic note: Charlotte states that, despite the disappointing outcome, she was glad to participate in something bigger than herself. Furthermore, Professor Wild acknowledges that we are not back to square one in terms of therapeutics – the data gathered will benefit future attempts at treating, what is often termed, the most curable 'incurable disease'.

This hopeful endpoint encapsulates the mood of the podcast – although featuring serious discussions, the topic is broached with anything but clinical detachment. Charlotte's anecdotes, and the humour shared between the speakers, kept the episode innately personal and accessible. Considering the biography is written in a similar tone, it has been fast-tracked to the top of my 'to read' pile.