RNIB Multiple Disability Services offers information and advice to adults with learning difficulties and their supporters. We are contacted by a wide variety of people and nothing is too obscure for us to deal with.
In 1997 the services team launched the 'Access to Eye Care' campaign for adults with learning difficulties.
It had long been known that children and adults with learning difficulties are particularly prone to visual impairment, yet only a minority of adults had regular eye tests. The DoH states that 30 per cent have 'significant impairment of vision'.
With more people with learning difficulties having eye examinations, the number of enquiries concerning keratoconus has increased beyond all recognition.
'My world' is different from most people involved with eye care.
The RNIB Multiple Disability team seldom hears about the 'ordinary' causes of visual impairment. We are more used to enquiries about people with learning difficulties born with a visual impairment (often unidentified until adulthood) or refractive error that has been left uncorrected for years. However, when callers contact us about acquired sight problems, they are nearly always asking about keratoconus, cataracts or people who have damaged their eyes through self-injury.
Strategies to support people with keratoconus will only work if all members of the 'team' work together. This may mean a partnership between the optometrist and the individual, their relatives, an advocate, and support workers from the place where they live, day service and/or respite provision.
People with keratoconus and learning difficulties need sensitive help from different sources:
Eye care professionals
Staff and family carers.
If their vision deteriorates considerably they will also need help from low vision services and rehabilitation workers for visually impaired people.
Causes of keratoconus
The causes of keratoconus are unclear, but in the 'general population', it occurs in families with a history of allergies.
It is generally overlooked that people with learning difficulties suffer from the same health problems as the rest of the population, but their health needs are frequently greater Ð and unmet. In 20 years of working with people with learning difficulties I have seldom encountered people whose allergies have been treated.
Keratoconus has been more common in people with Down's syndrome, but also affects other people with learning difficulties. In recent months we have had a noticeable number of enquiries concerning people described as 'being on the autistic spectrum'.
Keratoconus is also associated with both 'eye poking' and 'eye rubbing', which are common mannerisms in people with learning difficulties. Eye rubbing as a direct cause of keratoconus has not, however, been established. People with learning difficulties do sometimes blind themselves by frequently rubbing their eyes.
There are many reasons why people frequently touch their eyes. They may be sore or itchy. People may also poke their eyes in order to get flashes of light in the brain. With my eyes shut I 'see' circles of white light above my left eye by pressing my right eye gently Ð more shapes and colours if I press harder! Eye poking may provide people with visual rewards, whose lives have been lacking in colour and interest
For some, it may be their way of communicating. Many people with low vision have not developed speech or sign language to express their needs. They often have limited mobility and self care skills. They may have little control over their environment and be bored or lonely. However, if someone rushes over every time the person rubs their eyes, this is a successful way of gaining the attention they desperately need.
People with learning difficulties can suffer from poor hygiene and they may be unaware of the risks involved. Staff and family carers may also be too busy to check regularly. The mother of a profoundly disabled young adult told me: 'The staff think I am a real pain, but I keep on nagging them to wash her hands and cut her fingernails properly and often. If they leave nasty square ÒcornersÓ on her nails then she can do a lot more damage when she rubs her eyes. The home manager has hinted that I am a fussy mother, but she has lots of eye infections.'
Keratoconus may be associated with recurrent eyelid infections and allergic skin conditions.
Water, shampoo, soap or 'foreign bodies' in the eye may encourage people to rub the irritation. Care should be taken when washing hair and it is important to ensure that sore, sticky or encrusted eyelids are gently cleaned. Lid hygiene advice should be given in such cases.
People with blurred vision Ð from a variety of causes, including sight-threatening conditions Ð may frequently touch their eyes. People with learning difficulties often need spectacles, others may have been given the wrong pair. The prescription may be incorrect or old, or they may be wearing someone else's spectacles.
It is obviously important to establish why people constantly touch their eyes, to reduce the risk of damage and it is especially important that their hands are kept scrupulously clean. However, it may be hard to maintain high standards of hygiene for some people with learning difficulties.
Assistance from a clinical psychologist may be needed to prevent the individual from habitual eye rubbing and self-blinding. A psychologist may be able to help develop a strategy with family carers and staff to reduce this behaviour. It may also be necessary to involve the local community learning disability team (CLDT) to carry out the strategy, and ensure that everyone is aware of the risk.
Identification of keratoconus
Keratoconus may be hard to identify, especially in people who find eye tests strange or frightening and have difficulty co-operating (slit-lamp assessment, for example).
Changes in the eye are unlikely to be noticed by family carers and supporters of people with learning difficulties. When the person has language skills, they may notice changes in their vision. In my experience, the most common initial signs of keratoconus are changes in behaviour. People suddenly have problems with steps or changes of surface. A constant refrain in the summer months is 'he/she hates going out in the sunshine'.
It is important that staff and family carers are aware of hydrops (stromal opacification due to endothelial compromise) and actively look for it in people with limited communication skills. Changes in behaviour may be their only way of telling staff and carers about changes in their vision. It is, therefore, crucial that:
Family carers and staff do not attribute hydrops to another eye condition, such as conjunctivitis
People suspected of having hydrops seek immediate treatment at the local eye hospital casualty department. A&E departments in general hospitals may know little about the condition.
People with hydrops often are prescribed saline eye drops. Staff may assume it is impossible to insert drops into the eyes of people with learning difficulties but there are successful ways to do this. (See RNIB Focus Factsheet entitled Eye drops for adults with learning difficulties.)
A minority prefer to insert their own drops. A woman with learning difficulties told me: 'I'd rather do them myself. I get fed up with people poking around my eyes. It took a bit of time to get the hang of it, but it was worth the effort'.
Effect on vision
Keratoconus sufferers have explained their visual symptoms as:
Problems with glare
Double vision (which is often confusing or embarrassing)
Multiple images which people find very distressing
Haloes and stars around lights
Floaters Ð black dots floating across one's vision
Difficulty adapting to changes in light levels.
It is vitally important that optometrists explain how keratoconus affects people's vision. It may cause 'ordinary people' considerable stress and anxiety and present additional problems for people with learning difficulties, especially if they have limited communication skills.
A keratoconus group member told me: 'Living with double vision is hell. But at least I can discuss it with people. And I know I'm not going mad.'
A community nurse commented on the case: 'He clearly is seeing things. I discussed with the psychiatrist whether it was his keratoconus causing hallucinations Ð or if he has mental health needs, or both.'
People with keratoconus may find that distortion of vision causes them:
Difficulties in judging depth and distance
Problems on stairs or coping with changes of level underfoot
Blurring of straight lines. One person may look at the letter H and see the verticals clearly but find the horizontal stroke blurred. Another person may see the reverse, and yet others may see what looks like a letter N. Corneal irregularity leads to variable astigmatism.
The effects of keratoconus can vary throughout the day, and may be influenced by a variety of factors Ð such as tiredness, ill health, lighting and other environmental factors.
One mother of an adult with learning difficulties said of her daughter: 'She copes well during the day, but by 5pm she seems to see less well, regardless of the lighting around her.'
Most of us find it hard to describe exactly what we are seeing to another person. Supporters of people with learning difficulties should listen to what they are saying about their vision, and question them carefully.
A young woman with learning difficulties recounted that she kept on trying to explain how she saw things: 'But it took time to get the staff to understand. I then got the book from the Keratoconus Group and it was brilliant.'
A small loss of vision may have a profound effect on the behaviour of people with learning difficulties. They may not understand what is happening to them, nor why their familiar environment seems so different. They may not be able to devise their own 'coping strategies'.
People may become anxious and/or depressed, or develop 'challenging behaviour'. They may lose skills and self-confidence, needing more help than before. People with learning difficulties need to be offered help to re-learn lost skills.
Treatment for keratoconus
The usual methods of managing and treating keratoconus may need to be adapted for people with learning difficulties. This does not mean that spectacles, contact lenses and surgery should automatically be ruled out, and indeed may be very successful.
Prompt treatment is especially important for people with learning difficulties because the standard management of keratoconus (contact lenses) may not be practical for them, even with the most committed of supporters. Surgery is often hard to obtain since there are increased risks in people with learning difficulties.
Preventative healthcare must aim to reduce the likelihood of potential problems such as secondary infection.
One staff member recalled: ' I rang the RNIB and was told that keeping Jenny's hands clean and helping her stop touching her eyes might be a major consideration in the management of her keratoconus. We've started a hand-hygiene programme with her.'
However, many people (including people with severe learning difficulties) have had successful outcomes when sensitive support has been available.
People with learning difficulties often need preparation to get the maximum benefit from a visit to an optometrist. Because people with keratoconus are sensitive to bright lights, they may need to learn how to sit still to have their eyes examined, and to cope with the equipment used.
Feedback from optometrist
It is important that the individual, staff and carers are informed about a person's vision loss. In a perfect world, optometrists would write a report as supporters seldom know what questions to ask. It is also important for the optometrist to say how often the person needs re-testing and if any new equipment is to be used so they can be prepared.
There are many ways to help learning impaired people get used to wearing spectacles, which reduce the distortion of vision that causes problems with stairs, or finding their way in a strange environment. A person may take time to adapt to their new spectacles if they have not worn spectacles before, or a much stronger prescription has been prescribed than previously.
Many people with learning difficulties need help to adjust to wearing spectacles. They may have become accustomed to their distorted vision and the new spectacles may seem strange and confusing. Giving people 'better vision' with spectacles may present them with an unfamiliar 'perfect' world where they see things in a different way. Things may appear larger or smaller, making it hard to judge distance.
As the condition progresses and the cornea becomes more irregular, spectacles become less likely to correct vision adequately. People may refuse to wear them because they do not help them to see better. It is helpful if optometrists explain this, as staff or family carers need this information. Further information can be gathered from the RNIB Focus Factsheet, Glasses for adults with severe learning difficulties.
Tinted prescription glasses, sunglasses and hats
It is worth optometrists enquiring if photophobia is a problem Ð people may not recognise this as a symptom of keratoconus. Tinted lenses and changes in lighting may be required.
Optometrists may prescribe spectacles with tinted lenses to be worn indoors and outdoors. Indoor lighting, such as unshaded tube lighting, may also be a problem.
Supporters need to be advised that tinted lenses will not damage people's eyes. It may be obvious to the optometrist that tinted lenses change the way people see different colours, but people with learning difficulties and their supporters need to be told how vision will be affected.
Ordinary sunglasses may be acceptable to the minority of people with learning difficulties who refuse to wear prescription lenses. Wraparound shields preventing light from entering at the sides are also useful.
Hats, caps or eyeshades may help, especially if seen as 'cool' or 'trendy', and have been chosen by the person themselves. However, some people (especially those with autism) may resist wearing hats or spectacles Ð even if the benefits seem obvious to everyone else. They may need a 'de-sensitisation programme' so they gradually become comfortable wearing hats or spectacles.
Advice on position
While 'ordinary' people may discover that a change of location can improve their vision considerably, this may not be obvious to people with learning difficulties.
Some people (such as wheelchair users) may need practical help to change location. Facing a window may cause major glare problems, but sitting with one side to the window may improve vision as their 'activity area' is illuminated. Care needs to be taken that the dominant arm does not cause a shadow on the activity.
Optometrists have proved to be good allies in getting GPs to refer people with learning difficulties to eye clinics.
Some optometrists have also convinced ophthalmologists that treatment is worth attempting. The comparative rarity of keratoconus in the 'non-disabled population' means that GPs may not feel knowledgeable about the condition and not know that severely disabled people can be helped to wear spectacles, contact lenses, and be supported through surgery.
CLs may improve vision when spectacles no longer help as the condition progresses.
One residential worker said: 'Staff were squeamish initially; they couldn't believe they would ever learn to insert them, but they did.' A key worker or parent with experience in CL handling or a willingness to learn is essential if children and adults with learning difficulties are to wear them safely. It is easier if they are also lens wearers.
An optometrist said, 'CLs must be seen as a possibility for people with learning difficulties and not be dismissed outright.'
In its more advanced stages the corneal irregularity becomes too pronounced for a contact lens to be adequate. Lenses no longer improve the vision. A corneal graft may be considered for a small percentage of people to improve their vision. Aftercare may be lifelong and requires careful consideration and planning.
One mother of a service user said: 'My older daughter has had both eyes done and is fine, now. The whole business was traumatic for the family, lots of stress and hospital visits. We agonised about our other daughter, who has severe learning difficulties. We decided that it would be cruel to put her through all that Ð without the certainty of a permanent cure.'
Although corneal grafts for keratoconus have a high success rate, continued care is necessary. The regime is complex, with frequent eye drops and other medication, such as immuno-supressant drugs to reduce the possibility of the graft being rejected. These drugs often have side effects that need careful monitoring. There will be many hospital visits. People may still need to wear spectacles or CLs after a successful graft.
A residential social worker said: 'It was worth all the battles and the hard work. The eye drops every two hours to start with, the patch at night, then the glasses after all that. But he's a different person now he can see again Ð happy and loving life. The staff team made the commitment. We just believed it was his right.'
Help for low vision sufferers
As someone's vision deteriorates they may find it increasingly difficult to cope with activities that they previously managed successfully. However, poor vision should not be allowed to spoil people's lives.
Low vision services and support from rehabilitation workers for visually impaired people are aimed at helping people to make the best possible use of their existing sight and to retain or re-learn skills.
Most people with learning difficulties and keratoconus are never referred to these types of services because much of the support for people with low vision is only directed towards helping people who can read. As people with learning difficulties may not be able to read, it is wrongly assumed that they will not benefit from low vision aids or training.
Social services and social work departments normally employ rehabilitation workers for visually impaired people. However, some work for a local voluntary society for blind and partially sighted people.
They may offer assessment of how someone uses his or her vision ('functional vision'). They make recommendations to help the individual use their sight more effectively, such as help to improve a person's environment with better lighting, use of contrasting colours, and other techniques.
They may also provide training on moving about safely ('mobility skills'), and help people learn or re-learn skills such as cooking, cleaning, care of clothes and so on. In addition, they can recommend and provide equipment and information on local resources, benefits and concessions.
Such support should be available to everyone. The Visual Impairment Standards specifically mention help for people with learning difficulties.
Treatment for keratoconus may be complicated and last a lifetime. Many people will always have low vision even if the treatment they receive is 'successful'. However, with the right help, people with learning difficulties and keratoconus can continue to live full lives. People with learning difficulties have an equal right to high quality healthcare and support so they can make the best possible use of their existing vision. This help can radically improve all aspects of their life and lives may be spoiled if this help is not provided.
For a list of RNIBsupportive material and details on ordering it, contact william.harveyrbi.co.uk
I would like to acknowledge considerable help in our work from optometrist Valerie Scarr, Leigh Harris, a specialist worker for adults with learning disabilities in Lewisham, London, secretary Anne Klepacz and members of the Keratoconus Group and Will Sellar a consultant ophthalmologist, and eye care professionals too numerous to name.
Gill Levy is information and practice development officer, multiple disability services at the RN