When vision loss leads to hallucinations

Living with Charles Bonnet Syndrome

By Nicola O’Connell

It was by pure chance that Judith Potts, founder of campaign Esme’s Umbrella, happened to notice a very small newspaper article on Charles Bonnet Syndrome (CBS) soon after visiting her 92-year-old mother. Although this was the first time that Judith had heard of CBS, she was immediately convinced that her mother had the condition.

“My mother could have written that tiny piece about CBS – it described her symptoms so precisely,” recalls Judith. “It was incredibly lucky that I happened to see it. Earlier that day my mother had described seeing faceless people, an Edwardian street child and a horrible gargoyle face, telling me how she wished they’d ‘get off her sofa’.

“I didn’t think she had dementia – she did a cryptic crossword every day and showed no other signs – but I left her not knowing what to do. She had glaucoma but as she could still read I didn’t think her sight was too bad. But obviously it was much worse than we had realised.”

Judith was to then discover the lack of support and information available for CBS, ultimately leading to her launching Esme’s Umbrella (named after her mother) to provide help and information for anyone who feels they might be affected by CBS.

“I didn’t get anywhere with my mother’s ophthalmologist – apparently he felt CBS was unimportant and wouldn’t talk about it – or her GP. Eventually I contacted CBS expert Dr Dominic ffytche, who confirmed my mother most likely did have CBS. She was plagued by hallucinations for the rest of her life, but at least I was able to pass on some coping strategies to help her,” says Judith.

About CBS

Charles Bonnet Syndrome is named after the Eighteenth Century Swiss philosopher, lawyer and writer, who wrote about his grandfather experiencing visual hallucinations of people, birds, carriages, buildings and tapestries. His grandfather had visual loss due to cataracts, he wrote, but did not show any signs of dementia or mental illness.

Despite this early and clear description of CBS, the condition was largely ignored until the late 1990s, when finally some new research was undertaken. Dr ffytche, Reader in Visual Psychiatry at King's College, London pioneered the research. Dr ffytche is now globally acknowledged as the sole expert in CBS, having published hundreds of articles on the subject.

CBS can develop when over 60 per cent of sight is lost, through any diseases or injury affecting eyesight. These conditions might include, for example, age-related macular degeneration (AMD), cataracts, glaucoma, inherited retinal disorders, and any diseases affecting the eye and other parts of the body including diabetes, stroke and cancer. In the UK an estimated two million people currently live with sight loss, RNIB figures reveal, and this is projected to increase by a further 500,000 by 2020. It is predicted that by 2050, over four million people in the UK will be affected by sight loss.

Visual loss is more likely in older people so they are the largest group affected by CBS, but the condition also occurs in younger adults and children. Says Dr Mariya Moosajee, Consultant Ophthalmologist and Senior Clinical Lecturer, Moorfields Eye Hospital and UCL Institute of Ophthalmology, London: “The most common causes of blindness in the western world are AMD, glaucoma and diabetic retinopathy. And as these diseases tend to affect the elderly, there is a higher proportion of elderly people with CBS by default. In some situations CBS could be overlapping with conditions like dementia so careful investigating may need to be done to determine the cause.”

Not everyone with visual loss will develop CBS, but research suggests that approximately one in five people who experience sight loss are afflicted by the condition. Those with severe visual loss are far more likely to develop CBS – up to 50 per cent, compared with 10 to 15 per cent of people with moderate visual loss. It is not known why the condition affects some people and not others. The hallucinations may be short term – sometimes they stop or lessen after approximately 18 months – but they can continue indefinitely.

“There are probably many patients at Moorfields Eye Hospital affected by CBS, but we don’t know the numbers,” says Dr Moosajee. “It is believed that about 10 to 20 per cent of patients with AMD are affected by CBS whereas other studies say that around 60 per cent of severe cases are affected.”

So why does CBS develop?

It is unknown why or how CBS develops, but it is believed that the condition develops due to the relationship between the eyes and the brain. Explains Dr Moosajee: “CBS is highly variable. Some of my patients have reported visual hallucinations as they were losing their sight, which ceased when they lost all vision, but in others the symptoms persisted.

“This goes with the theory that as you are losing your vision, your brain is filling in the gaps and may be hyperexcitable. If the visual cortex isn’t being used for vision anymore, it can be reallocated for touch, sound and other sensory input.

“If you are born blind or lose your vision in the first few years of life, then your visual cortex is repurposed for touch and sound. This also occurs if you lose your vision once it has been established – from late childhood – but to a lesser degree. However, this is only what we hypothesise is happening from studies using functional magnetic resonance imaging (MRI) brain scans. No one knows the definite answer yet.”

The types of hallucinations people have vary from one person to another. They generally fall into two categories: simple repeating pattern hallucinations (grids, shapes, mosaics, for example) or more complex, involving people, animals or detailed images of events. The hallucinations are always only visual, and therefore not accompanied by sound or smell.

As Dr Moosajee explains: “Some studies report 70 per cent of patients seeing in colour and 60 per cent see motion. Some reports show that most people have the visions daily, lasting seconds rather than minutes. But other studies have shown the opposite – that more people experience complex visions lasting minutes rather than seconds. It is a very varied mix of hallucinations and length of time.”

The hallucinations can be pleasant in some cases. People have reported seeing beautiful countryside scenes, landscapes with waterfalls or colourful flowers. For some people however, the hallucinations can be highly unpleasant or frightening.

“Some people see writhing worms on their food or dirt floating in their water,” says Judith. “They can actually stop eating because of this, clearly a concern and particularly for the elderly living on their own. Some people fall over with the sudden appearance of a wall or steps going down. Other people have described the sudden appearance of a lion right in front of them.

“In some cases the complete environment changes. One person with CBS told me that her entire room changed into a cathedral, while my mother found herself at a funeral procession.

“Fire can be one of the more terrifying hallucinations. One woman woke thinking her bed was on fire. She moved and the hallucination disappeared. Another woman called the fire brigade when she saw fire on the other side of her glass panel. When it happens now she touches the door to check that it is cold because she finds it hard to tell the difference between a hallucination and a real-life situation. Clearly, this is no way to live.”

Frequency of hallucinations is also always variable. For some, the hallucinations can be once or twice a week, but, as Judith stresses: “Some people have the hallucinations all day every day and all night. Occasionally they can last hours. They truly are different for every person.

Diagnosing CBS

There is no specific test for CBS, so doctors diagnose the condition by talking to patients about their symptoms and taking a detailed medical history. However, people may be reluctant to discuss their symptoms with a clinician, potentially due to embarrassment, concern that it signifies the start of dementia or fear that they will not be taken seriously. This does not only apply to adults either; some children experiencing CBS hallucinations are also very reluctant to discuss their visions and may suffer in complete silence. This is why it is estimated that there are likely to be large numbers of unknown individuals secretly living with CBS.

NHS figures suggest that there are more than 100,000 cases of CBS in the UK, but this only takes into account the confirmed diagnoses. “We don’t know how many people in the UK are really living CBS, but we believe it is around one million,” says Judith.

Certainly, there are other conditions that could also produce visual and dramatic hallucinations, including dementia, drowsiness/sleep disturbance, falls, Parkinson’s disease, epilepsy, schizophrenia and stroke. In some cases tests may need to be taken to rule out other illnesses before CBS is diagnosed.

However, as Judith has discovered, a consultation with a GP does not always lead to a diagnosis and support. She recalls: “One patient calling my helpline had seen her GP, already believing she had CBS and looking for relevant help. However, her GP told her to just forget about it.”

Ophthalmologists should all be aware of CBS though, Dr Moosajee stresses. “We are all taught about CBS and there is no myth about it,” she says. “We definitely know it affects patients and if people are misdiagnosed then that is probably coming from non-ophthalmologists who are not aware of the condition.

“The problem is that doctors don’t ask enough about it when they see patients. For instance, if we see someone with AMD, then we are more likely to ask about further vision deterioration, monitoring for changes, eye vitamins or maybe smoking. But asking about visual hallucinations may be overlooked. We need to train doctors to raise CBS with patients because it’s common and can be easily managed in many cases.”

Treating and living with CBS

There is no cure for CBS and no specific treatment for it, but sometimes medications designed for other conditions – including epilepsy, Parkinson’s disease and dementia – can be helpful in preventing hallucinations. Antidepressants can be prescribed in cases where the hallucinations cause significant anxiety.

However, to avoid medication side effects, most people do not take drug treatment and are encouraged to try a number of self-help measures when a hallucination begins. For example, changing lighting levels sometimes helps – moving somewhere brighter or somewhere dimmer. Other people find that moving their eyes helps. One study showed that looking left to right about once every second for up to 30 seconds, without moving the head, may stop the hallucination. Some people find that sweeping the vision away with their hand or reaching out to touch the vision can also make a difference.

“Everyone has their own ways of coping and some of methods can be quite unusual,” says Judith. “One woman sees a family living at the bottom of her garden and takes trays of tea down there, even though she realises they are not actually there. She would never mention this to her doctor for fear she would considered mad! But this is how she copes and everyone has to find his or her own strategies.

“Some people find that standing up and waving arms gets rid of a hallucination. For others, just getting up and performing a simple task can be the answer.”

Campaigning for awareness and support

Judith set up Esme’s Umbrella in memory of her mother (with the word umbrella to signify sheltering doctors, scientists and people with the condition under one umbrella). When Judith came across such a lack of awareness and interest in her mother’s condition, she felt there had to be a way to reach out to the thousands of people suffering from CBS, particularly those who have been too afraid to seek help, or who have sought help and not been given any support.

“I thought someone has to do something and thankfully I received funding from the Help and Information Service to set up my campaign. We officially launched Esme’s Umbrella at the House of Commons in November 2015.

“The Esme’s Umbrella website provides information on CBS and calls to the helpline are answered by the Royal National Institute of Blind People (RNIB) Eye Health Team, specifically on behalf of Esme’s Umbrella. Should callers want to speak to someone who lives with CBS, they are referred to Retina UK, whose helpline is answered by people with CBS, or who I have trained to give help and support.

“I often advise people to see their GP armed with as much information as possible printed from the website, and I also give whatever tips I can on coping with the condition. Sometimes people just need lots of reassurance.”

Judith continues to look for new ways to support people with CBS, including organising spaces (‘Esme Room Support Groups’) where people with CBS can gather together with their families and friends in a relaxed environment to discuss coping strategies, exchange ideas or simply share their experiences.

Reaching out to people with CBS and providing support is, as Judith explains, just one part of the equation. There is still the need for much greater awareness and research into CBS and Judith aims to achieve this by working closely with medical experts and other relevant organisations.

The world’s first-ever CBS Patient Day was held last November at Moorfields Eye Hospital. A number of medical experts spoke at the event, including Dr Moosajee and Dr ffytche, who is also Esme’s Umbrella’s medical adviser. Part of the day was dedicated to discussion, sharing patients’ stories and allowing people with CBS and their families or friends to ask questions and seek advice.

Says Judith: “We have funding for research at Newcastle University, which is a wonderful help. This is enabling us to compare the brains of people with sight loss and CBS against those with sight loss who do not develop CBS, ultimately leading to greater understanding for treatment. But we are now raising money to fund another new researcher, which will allow us to continue our good work and eventually be able to make a big difference for those with CBS.”

Dr Moosajee also believes that ongoing research is very important. She says: “I think that the best approach for patients is if we can combine ophthalmology with psychiatry to try to explore why some people get hallucinations and others don’t, even though they have similar levels of vision. We need to consider how the brain and eye are linked to cause this manifestation.

“At Moorfields Eye Hospital I would like to set up patient support group sessions, facilitated by someone with experience or knowledge of CBS. These will provide people with regular opportunities to talk about what is happening to them and discuss coping strategies. For the majority of people with CBS, this will be the best therapy. People who talk about it will get the affirmation that they are not going mad.

“If someone has a severe problem and is constantly plagued by hallucinations, then a referral can be made for further diagnosis and treatment. Severe cases are not common – they are actually quite rare.

“First, however, we need to raise awareness so that we can reach out to those suffering from CBS. We need to remember that it is not just a condition of the elderly. Eye Clinic Liaison Officers often address CBS, but not all clinics or hospitals have ECLOs. Ophthalmologists need to ask about it more, but so do others in the community – from GPs and nurses to opticians, rehabilitation officers for the visually impaired and carers. There is no need to live with CBS in isolation when there are charities and campaigns like Esme’s Umbrella that can offer support and advice.”

Esme’s Umbrella:

www.charlesbonnetsyndrome.uk

Helpline: 020 7391 3299

Esme’s Umbrella restricted fund page (raising funding for more research):

www.justgiving.com/fundraising/EsmesUmbrella

Case study:

Amit’s story

Amit Patel was caught out so suddenly by a disturbing Charles Bonnet Syndrome (CBS) hallucination that he nearly threw his newborn son into the air.

Two years ago Amit, a 38-year-old former A&E trauma doctor, was changing three-week old Abhi’s nappy when he suddenly saw a girl who resembled ‘scary girl Samara’ in the horror film ‘The Ring’.

“The girl was about half a metre to my right, floating and looking directly at me. She had long black hair and a white dirty cloth over her. It was a such a shock that I nearly threw Abhi,” Amit recalls. “At that point I hadn’t had any hallucinations for many months and I’d never had one like that.”

Amit – who has been blind for five years due to suffering a haemorrhage behind his eyes – can vividly remember the first time he ever experienced a major hallucination due to CBS.

He recalls: “It was approximately three months after I lost my sight and I was walking down the stairs in my house when I suddenly saw a red BMW Estate driving towards me. It took me by such surprise that I actually fell down the stairs – I really believed there was a car in the house. Fortunately I wasn’t hurt.”

Although Amit still sees the car sometimes, his main ongoing hallucination now is seeing horror girl ‘Samara’. A horror film fan, Amit sees the girl precisely as he remembers her from watching ‘The Ring’.

“It’s like she’s literally there,” he says. “There is no connection to anything I’m thinking or doing. It’s completely random. I have the hallucination anywhere – sitting on the couch, lying in bed or eating dinner. I can be on a packed train and the girl is just inches from me. It is so real that I feel I can reach out and touch her. I can feel her breath on me because she’s so close.

Frightening and disturbing

It is the incredible realness and lucidity that Amit finds overwhelming and alarming. “That is what is so scary, causing my body to start sweating and my heart to start beating really quickly,” he explains. My whole body feels numb and I feel like I’m going to fall. I feel like a scared small child. Most hallucinations are disturbing and scary. To the person, it is very real.”

Amit tries some of the suggested techniques in attempt to block out the hallucinations. He says: “I close my eyes and look up or down, like I’m getting away from the girl. If I’m at home I sometimes close the door or go into another room. I try to grip on to something but if I’m on the street I can’t do that so I just stop.

“I tend to often get the hallucinations when I relax so at night I go to sleep listening to music with headphones. I need my brain to be doing something.”

Unlike many other people who start to experience CBS-related hallucinations, the condition wasn’t a complete surprise for Amit. He knew about the condition before his first hallucination – but he says he “still wasn’t ready for it”.

“When it first happened with the BMW my first thought was ‘Oh my, I can see!’ Then of course I realised that I was on the stairs and there was no window in front of me. I put it down to the fact that I really wanted to see something. But then it kept happening and the car would be driving towards me. That’s when it clicked that I had CBS.”

Sudden sight loss

Amit had good vision until the age of 21, only ever requiring reading glasses. But while at medical school, he began getting frequent headaches, which affected his ability to concentrate. Eventually he was diagnosed with keratoconous, an non-inflammatory condition in which the eye’s cornea progressively thins, causing difficulty focusing and potentially leading to impaired vision.

Initially Amit’s eyesight was treated with different lenses, but when it deteriorated further he had his first cornea transplant at the age of 26. Says Amit: “The lenses had worked well but there came a point when I couldn’t get the right fitting.”

Over the next few years, Amit had two further transplants, but each followed the same pattern – no problems for the first nine months, but then his body began to slowly reject the transplants, leaving him with no option but to start the process all over again. “I received excellent care from Moorfields Eye Hospital during this time, but the rejections kept recurring,” he says.

This was despite the fact that Amit was taking immunosuppressant medication to help his body adjust to the new transplant (standard treatment for any type of transplant to help the body’s immune system to stop rejecting what it perceives as a ‘foreign’ addition). So when Amit became aware of a promising immunosuppressant regimen of medication only available in the USA, he was keen to try it.

“I packed my bags and went to New York,” he recalls. “For a while I was back and forth between New York and the UK. Moorfields Eye Hospital still looked after me all of this time too, which was amazing. The new immunosuppressant treatment worked well and for a while everything was perfect. I was working full-time, I had been married for a year and life was good.”

But upon waking one morning, Amit’s life took a drastic change. He had some unexplained pain in his eyes and 36 hours later Amit had complete sight loss. It turned out that Amit had suffered a haemorrhage behind his eyes.

“I had no health issues and I had eye checks every couple of months, so there was nothing to warn me about this and there was no way to see it coming. After the hemorrhage I had to wait a few weeks for an examination – the backs of my eyes were so cloudy until then that no one could see them properly – and at that point I was told nothing could be done to save my sight. Life more or less stopped – and suddenly.”

Over the past five years a number of consultants worldwide have told Amit that there might be a way for him to regain vision – but there is always a risk involved, one that Amit is not prepared to take. “I’m actually used to living in the dark. It took me a long time to get back on my feet and but I’m now really happy where I am, with an amazing wife and son. I’m used to living in the dark now.

“I don’t have any useful vision. I can’t see anyone standing in front of me. I just see what I call ‘little hot air balloons’ floating around. It’s like if you’re in a very deep pool, standing up and looking up on a misty day. You see ripples but that is it.”

Active campaigner

Thanks to Amit’s determination, positive attitude and close bond with his guide dog Kika, he remains very busy campaigning for people with disabilities and giving motivational speeches. Once a month, he and Kika feature on the well-known American TV Show ‘Good Morning America’. Amit frequently talks to people about how best to assist blind and other disabled people on public transport.

“Kika and I do a lot of radio work too. We also go into schools and Scouts centres to talk about guide dogs. We go into hospitals to talk to staff members about how to guide visually impaired people, and sometimes we go into hospices for young adults.”

Amit feels that there should be much more awareness of CBS among people with visual impairment. He says: “I think that consultants need to highlight CBS much earlier. People who are losing their vision need to be informed about CBS right from the beginning.

“If the subject of CBS is raised during clinics, then people might take the opportunity to talk about their hallucinations. Lots of people having hallucinations think they are going crazy and some have lived with them for 15 or 20 years – sadly never saying a word. The time has come for everyone at risk to be informed about CBS and encouraged to talk about any hallucinations that they are experiencing.

“Losing your sight in the first place is hard enough – people shouldn’t also have to suffer CBS without knowing what is going on.”