My mother, Esme, was in her early nineties and – despite glaucoma slowly diminishing her sight – lived a totally independent life. She was a great improviser and had adapted remarkably well to her sight-skewed world. However, we had no idea that something else was lurking - a condition which would remove her confidence, bring her great distress and add to the feelings of isolation, fear and loneliness which go hand in hand with sight loss.
One day, as I was leaving her flat, she suddenly confided in me about the months in which she had lived with faceless people sitting on her sofa, an Edwardian tear-stained street-child following her everywhere and a grotesque gargoyle-like creature hopping from table to chair. Sometimes the whole room or garden morphed into somewhere else entirely. I had absolutely no idea what could be causing her ‘visions’, as she called them, but the word ‘dementia’ hung, unspoken, between us. Yet, surely, I thought, her ability to complete the Telegraph cryptic crossword every day proved her sanity.
Before I had a chance to contact the GP, I read a small paragraph in a newspaper which described an experience that mirrored Esme’s. The condition was called Charles Bonnet Syndrome (CBS)and it was caused by lack of sight. With the name to hand, I called her ophthalmologist. Unfortunately, he refused to discuss CBS with me, nor explain why he had never issued a warning. Neither her GP nor her optometrist were aware of the condition and it was not until I consulted the internet and found Dr Dominic ffytche at King’s College London, that I understood why the hallucinations appeared and was relieved to discover that they did not mark any sort of mental health issue.
Dr ffytche is the sole, globally-acknowledged expert in CBS. He explained that when sight diminishes, the messages from the retina to the visual cortex slow or stop, but the brain does not. It fires up and creates its own images, which appear as everything from patterns, words, musical notes and maps to animals, birds, snakes, rodents, buildings, vehicles, trees, plants, grass, water, fire or people (real or Lilliputian-sized) alone or in groups and often in costume. Sometimes, the whole room changes into an alien place. Esme found herself in the middle of an Edwardian funeral procession and I have heard of medieval, candle-lit cathedrals, fast-flowing rivers and walls with countless doors. What is seen depends on which part of the brain is firing at that moment.
It is estimated that there are, at least, 1 million people of all ages in the UK who have lost more than 60% of sight and developed these vivid, silent, visual hallucinations. No other sense is involved and the loss of sight can be from any of the eye diseases, stroke, accident, cancer, diabetes or another condition that affects the optic nerve. There are no medical consultants in CBS and no specific medication to alleviate the hallucinations.
Too few ophthalmologists warn their patients that CBS might develop. It might not, but if frogs flow from the tap, a weary second-world war pilot appears in the room or flowering plants grow from the shelves, it helps a little to have been made aware of the condition. Having spoken at the Royal College of Ophthalmology’s Congress last year, I hope the figure of 17.2% of patients who receive a warning, will begin to increase.
Fearing that the silent, visual hallucinations herald some form of mental health issue, means that people who develop CBS tend not to confide in anyone. Falls can happen while trying to avoid the sudden appearance of a dog, rushing water or fire. When asked by the GP or hospital doctor to explain what happened, a choice has to be made. Very few GPs and hospital doctors are aware of CBS, so if the hallucination is described, it is entirely possible for mis-diagnosis to occur and patients can find themselves ushered down the mental health route – sometimes, particularly for the elderly, on a one-way ticket. This is an appalling waste of medication and NHS time, not to mention the distress caused to the patient.
I launched Esme’s Umbrella in 2015 at the House of Commons, in memory of my mother, to raise awareness of the condition and with Dr ffytche as my medical adviser. His research was the first piece of CBS work since 1760, when Charles Bonnet first documented the experiences of his grandfather. However, thanks to Fight for Sight, The Thomas Pocklington Trust and National Eye Research Centre, there is an Esme’s Umbrella researcher at Newcastle University, who is working in conjunction with Dr ffytche at King’s London. Identifying the difference in the brain of a person with sight loss and CBS and that of someone with sight loss who does not develop the condition, could result in a non-medication, non-invasive treatment which is currently being researched for all types of visual hallucinations at the same university. Nowhere else in the world is CBS research happening.
Not everyone develops unpleasant hallucinations but even if the images are benign, it is the irritating frequency that affects every-day life. With a little sight left, watching television can be a pleasure – but not if there is always an image of someone standing in front of the set, or the screen is covered in patterns. Reading can be disturbed by a tarantula climbing out of every page.
Some elderly people find they are unable to tolerate the perceived writhing worms and slugs on food and in drink, so they stop eating and drinking, with tragic results. Others, with multiple hallucinations, become house-bound because they can no longer distinguish between what is real and what is not. A few, who are distressed or embarrassed by the nature of their hallucinations, contemplate suicide. Children and young people find their school-days or careers constantly interrupted.
I hosted the world’s first CBS Patient Day at Moorfields’ Eye Hospital in central London last year to coincide with CBS Awareness Day (16th November). The event was a great success and it was decided to focus on two aims. The first is to lobby for a tick-box to be added to the CVI form, which has to be completed by the ophthalmologist to register the patient as blind. The tick box will confirm that the doctor has informed the patient about CBS. The second is to persuade the NHS of the need for specialist CBS Nurses, to whom doctors of all specialities can refer their patients.
In April, there will be a CBS Information Day for patients and professionals in York and more planned in Scotland and Lancashire – with another London event in November. Esme Room Support Groups are springing up in local low vision charities and eye clinics in the UK, at which people with CBS, their families and friends can meet over a cup of tea to find that all-important reassurance.
The condition has been too easily dismissed as a ‘just a side effect of sight loss’, but it is so much more than that. CBS affects not only the quality of life of someone who lives in a world of hallucinations, but that of their family and friends too. I have been lobbying for CBS to be included in ICD-11 - the World Health Organisation’s new taxonomy of diseases and conditions – as a condition in its own right. If this happens, it will inspire more vital funding for research. We need to begin now because, by 2050, the number of people with sight loss will have risen to 4 million and most of those will develop CBS too.
Until last year, I responded to every call made to my Helpline, but the escalation of calls and GDPR made it impossible for me to continue. The RNIB stepped in and my Helpline is now answered by the Eye Health Team at the charity. The number is 020 7391 3299.
CBS stayed with Esme for the rest of her life, causing great distress to her and the family. She would be amazed that her name has become synonymous with CBS in the UK, but I know that she would encourage everyone in the sight loss community to add their voices to the call for the NHS to spearhead a campaign for medical, social and prevalence research, so that the 1 million people in the UK – plus the hundreds of millions all over the world – have access to the support that was lacking for her and, eventually, a cure for this disturbing condition.
JUDITH POTTS – Founder of Esme’s Umbrella
To donate for research: www.justgiving.com/fundraising/esmesumbrella