‘Sometimes the whole room morphs into a different place’

Dr Lee Jones is Post-Doctoral Research Fellow at both Moorfields Eye Hospital and the UCL Institute of Ophthalmology, where he studies the impact of eye conditions through a health psychology lens. One of his areas of interest is Charles Bonnet Syndrome – a condition in which those with degenerative eye conditions experience visual hallucinations, including patterns, scenes, animals and figures. The prevalence of this syndrome is disputed, but may affect up to one in five people with vision impairments and sight loss. 

Jones spoke to Keith Valentine, Director of Development at RNIB, who has Retinitis Pigmentosa and experiences Charles Bonnet syndrome; and Judith Potts, the founder of Charles Bonnet Syndrome campaign group Esme's Umbrella. What are their experiences of the condition? 

LJ: Keith, could you tell us a little bit about yourself and how CBS affects you?

KV: In my professional life I'm the director of development at the RNIB… that involves planning strategy and the insight functions that help the organisation understand the lived experience of blind people. I was diagnosed with retinitis pigmentosa when I was 11-years-old. In the preceding 10 years many members of my family had also been diagnosed, and my mother had her diagnosis three or four weeks before me. 

I started to experience Charles Bonnet Syndrome without any concept of what it was. I was coming back from watching the Six Nations game in the pub. When I got back to my flat, I opened the door and there was a First World War soldier, with his face severely injured, stood in the middle of my front room. 

My experience of sight loss is it's like looking through a fog… it's been described as a smashed mirror with bits moved off and Vaseline rubbed all over it. My visual environment is very confused and very mobile – there's lots of flashing and things moving around – so it was odd to see something with such clarity. 

I also sometimes see woodgrain covering everything, which is quite common. I went through a phase of seeing staircases in pavements, but less so through lockdown as I'm not really in the environments where I see them. Generally it's the big London streets where I see that – it's actually particularly bad on Euston Road, where there actually are staircases in the pavements! 

It's something that's part of my life now, and I think I'm able to enjoy the colour of it, and get some satisfaction from it. I don't know if that sounds perverse… but in a world without visual clarity I can sometimes enjoy things that exist with visual clarity. 

LJ: How did you feel in those initial stages when the hallucinations first began? 

KV: There was a very small gap between me seeing the figure for the first time and raising it with my family, particularly my mother. She raised myself and my brother as a single parent in a tower block on Walthamstow Market, while experiencing sight loss herself. She was bullied at work as a consequence of sight loss. When I spoke to my mum she swiftly said 'oh yeah I see stuff'. She'd never mentioned that before, but she sees huge pythons on pavements quite a lot when she's walking around. I don't know whether it is triggered by certain patterns in the visual environment but she often sees them on zebra crossings. Recently she's been seeing the Coronavirus, those images that come up on the news, floating around the house. I was quite startled by that because I'd never thought of it as something that had any material relationship to what you were consuming.

I am very conscious that I'm losing my sight and experiencing things like Charles Bonnet Syndrome from a position of relative strength – I don't have to carry the concerns of the rest of my family around sight loss with me, because many of them are living with it. My first time seeing the images was scary… when I saw the soldier for the first time it was frightening because I thought it was the worst kind of burglar, the one that doesn't run away. I never thought that I was going mad, which is a worrying form of arrogance in the modern world… I should have at least considered it for a minute!

LJ: Are there any other occasions of experiencing these hallucinations that stand out to you?

KV: I chaired the Vision UK Conference at the Queen Elizabeth Conference Centre two or three years ago now. I was chairing the conference, chairing the panel discussion at the end, and doing a keynote speech, I was absolutely exhausted by the end of the day. When I walked across the stage, using a white cane, to stand at a lectern in front of 500 people in a great big hall, I started to talk and then saw my soldier stood there in the middle of the main aisle at the back. That was kind of nice, I'd rather all these things weren't happening to me, but when it turns up on a day like that and just stands there looking all odd I kind of took comfort from it. Not that it would have called out if I'd missed my line or anything like that but there's something about the routines of sight loss that can be perversely comforting. 

LJ: How would you say your Charles Bonnet Syndrome has been affected over the last year or so, with the pandemic and lockdowns? 

KV: It's no more frequent, the incidental stuff is a bit more colourful at times. When the soldier appears – it's strange the way it happens now – where before it would be innocuously stood in spaces, it's more recently felt more intimidating. It doesn't actually move around or anything, it just stands there, but to have it stood right up against the window is very different than if I was just conscious that it was out in the garden. 

I often see people with different faces. I can't see anyone's face anymore, and a lot of the time people I've known in the past seem to crop up. When it first started I was routinely seeing facial disfigurement on people I was talking to. They often have their bottom jaw missing, and that is starting to occur more frequently. It's noticeably different but not dramatic, I would say the difference is it feels a little bit darker. But then my broader sense of self and what's going on, as with all of us, is a little bit disturbed by the presence of the disease in the community. 

LJ: Judith, could you tell us about yourself and how Esme's Umbrella came about? 

JP: My mum was the Esme of Esme's Umbrella, she was in her early 90s, very independent, very bright, intelligent, she did the Telegraph cryptic crossword every day, and she seemed fine. I knew she had glaucoma but I realise I didn't understand what glaucoma really was. One day she said 'I do wish these people would get off my sofa'. I paused and she said, 'well, they do go if I tap them on their shoulders'. And I thought what is this? And while I was thinking what on earth I should say, she said there's an Edwardian tear-stained street child who follows me everywhere and a hideous gargoyle-like creature that jumps from table to chair. She said sometimes the whole room, or garden in her case, morphs into a different place and she found herself in the middle of an Edwardian funeral procession with horses and the clergy in red cassocks. I thought this has got to be dementia, but it can't be that, because of this flipping crossword that she does! 

I was very fortunate and found a tiny paragraph in one of the health pages – there was an interview with a guy called Matt Harrison, who at that point worked for Beacon in Wolverhampton. He was talking about what happened to him, which was Charles Bonnet Syndrome. I thought great, I called mum told her not to worry, and called the ophthalmologist… That was where it all went wrong. He refused to discuss it, and even sent a junior doctor to her next eye appointment with strict instructions not to discuss it. Her GP had never heard of it and thought I was being fanciful. Our optometrist had never heard of it. So, onto the internet I went, and I found Dr, now Professor, Dominic Ffytche, at King's College London, and I went to see him. He agreed that that's exactly what my mum had. 

At the time I was writing a health column for The Telegraph and I said I'm going to start writing about this. And that's what I did. A couple of years went by and it was clear my mum was getting worse – this was happening to her 24 hours a day. I thought I've got to do something but, actually, it wasn't until she died that I really got my act together and launched Esme's Umbrella because of an email that arrived after a column that I'd written. It was from a professor at a university in New York and she told me the story of her mum who had been put into a dementia unit. The doctor said she was obviously demented: she was hallucinating worms and slugs in her food in her drink. The family thought she was perfectly sane, and this poor woman couldn't get past the worms and slugs. There was no one who'd heard of Charles Bonnet Syndrome. She stopped eating and drinking and died. That was so appalling that I thought, right, I have to do something. So in November 2015, at the House of Commons, I launched Esme's Umbrella in memory of mum, with three aims – one was obviously to raise awareness of this, because clearly no one had heard of it. Secondly to create ways of supporting people who do develop it and their families and thirdly to source funding for research, because I am a campaign not a charity, so I take my begging bowl out and ask for money.

LJ: How would you say that awareness of Charles Bonnet Syndrome has changed over the last few years? And what do you think that change has meant to people living with CBS?

JP: I think, blowing my own trumpet here, I have raised awareness, and more and more people know about it. Professor Ffytche has proved that if a warning is given to the patient before the first hallucination appears the outcome is much better for them. Because they see a hallucination and think 'oh, yes, I was told something like this might happen'. It might still be horrible, they might never come to terms with it, but that initial shock and horror is not there, and therefore it's easier to cope with. 

I think it's been proved by the patient awareness day that that we held online last November that awareness has been raised because of the number of people that attended. Also, the number of people that write to me from all over the world. I also worked with scriptwriters on Coronation Street and we know that that is watched by between seven and 10 million people. All those people will have now heard of Charles Bonnet Syndrome, so that will have made a big difference. I know from the Facebook page how many people are in desperate need. My helpline is answered for me by the eye health team at the RNIB because it just got too much for me, because Esme's Umbrella is only me. In the three days after the diagnosis was revealed in the episode of Coronation Street my helpline received just under 1900 calls. 

There are doctors from around the world who are calling and contacting me. I was contacted recently by an ophthalmologist in Paris who wrote to me because he was very ashamed – he said he thought this was incredibly rare until he looked at my website and realised that it is very common, and we have to do something about it. And I wrote back and said, 'yes, you do, actually, So would you get on please and do something!' There are people all over the world with this, Professor Ffytche reckons that there are probably a million in this country… so how many are there in America, Australia, South Africa, India?

When people know what it is, it does help, but as Keith says it can get in the way of everyday life even if what you see isn't frightening. One lady said to me the other day that she was making a cake and suddenly leaves and twigs were falling into the bowl that she's mixing the cake in – you just have to stop until that goes away. 

There are two quotes from lockdown I remember in particular – one was from a lady who said that she'd lost her sight six years before, so for six years she'd seen no colour. Suddenly Charles Bonnet Syndrome developed and she said it's stunningly beautiful – like watching a gardening documentary, she sees the colours of the flowers and the plants and the trees, a drop of rain on a leaf. She said it's wonderful. The only problem is she cannot choose when she sees it so it can interrupt her life at any time of the day. On the other end of the spectrum is the man who's told me it's like watching a horror film that he would never have chosen to watch and which he can't switch off. 

LJ: Would you say that over lockdown that you noticed a change in the number of phone calls you were receiving, or the nature of the types of complaints and problems people were experiencing?

JP: Yes definitely. A week into lockdown, a guy called Mike Bailey, who works in Liverpool for a big charity called Christopher Grange, contacted me and said that he'd been running a group. He said we had to do something because his members were contacting him and were having a really difficult time. So we launched something called Esme's Friends which are telephone chat groups, and more and more people are joining those. And I knew we were onto something when someone at the RNIB called and said they were overwhelmed with calls. She said the regular sight loss questions had disappeared and the calls we were getting to the helpline were all about Charles Bonnet Syndrome and how much worse the episodes are, how much more frequent the episodes are, and also that people were seeing things which were much more frightening. 

LJ: This reflects what we've found in our work with people experiencing CBS and the changes in their hallucinations during lockdown. Some studies have found a link between social isolation and visual hallucinations, so we carried out a survey online and by telephone of 45 people with CBS about their experiences. We found that 56 per cent of those people were experiencing more frequent hallucinations. For example, people described how they were now experiencing hallucinations several times per day, whereas prior to the lockdown it was once or twice per week. Around half of the people we spoke to said the nature of their hallucinations had changed, and they were finding them increasingly difficult to cope with. For example, some told us that ghostly figures had started to appear in their homes, often with grotesque facial features, such as jagged teeth, and bloodied or melting faces. Some also described experiences similar to prosopometamorphopsia – where faces become strangely disfigured.

We found that there was a statistically significant association between self-reported feelings of loneliness and changes in the phenomenology of CBS. Our findings also suggested that people who felt lonelier during the pandemic were, on average, older than those who did not report feeling lonelier. We think this finding might point to a difference in the needs of visually impaired older adults compared to older adults without vision impairment and it raises important questions around the manifestation of loneliness among the visually impaired.

Is there anything you'd like to see in the future for people experiencing Charles Bonnet Syndrome?

KV: If there's anything that I could do right now it would be to get the money to further the research that yourself and other colleagues are doing. It's something we have to persist with. I firmly believe that if people are aware of it, and they can understand it for themselves and their family, then the experience for the patient will be that much better than it would be but also the world's sensitivity to it would be improved as well. 

JP: I think it would be great if psychologists over here volunteered. There is a guy in America, a clinical psychologist, who works exclusively with the visually impaired – we don't have that role here. If they could volunteer to be on hand to talk to people and even to run the kind of groups that my friend in America does, which are the same as Esme's Friends. With the best will in the world, the local charities who host groups over here are lovely, but people want to know that there is a medical person that they're talking to. Any psychologist volunteers would be fantastic. I am also working with the nurses at the Manchester University School of Nursing and hopefully they're going to go back to their NHS trusts as CBS champions. There's so much to be done to help this community and their children as well. 

- Dr Lee Jones is a previous winner of the British Psychological Society's William Inman Prize.

Editor's note: This piece originally appeared online 22/3/21