Booklet  Corneal Grafts and Keratoconus

Introduction

In recent years a growing number of people with learning disabilities have been found to have keratoconus. Treatment for this condition may present individuals and their supporters with problems and procedures that this Look Up factsheet (one of a series on eye care) aims to address.

Look Up has two other factsheets concerning keratoconus: 

Keratoconus in people with learning disabilities - an introductory factsheet explains what keratoconus is, how vision is distorted and ways of helping people with 'low vision' 
Contact lenses for adults with learning disabilities and keratoconus - highlights some of the issues involved to be considered when deciding to support a person with learning disabilities wearing contact lenses.

Keratoconus and its treatment

Keratoconus literally means a cone shaped cornea - part of the front of the eye.

The cornea is the clear window through which you can see the pupil (the round black part) and the iris (the coloured part). Keratoconus occurs when the transparent, approximately dome-shaped cornea becomes more pointed due to the thinning of the layers near the centre.

Keratoconus does not normally cause total blindness. However, we have heard of people whose grafts have been continually rejected and who are now totally blind. This is because the eye atrophied (died).

Keratoconus may severely distort vision or cause double vision. This may make life tiring and stressful for people. The Look Up factsheet, 'Keratoconus in adults with learning disabilities' explains some of the effects of keratoconus and ways of helping people with 'low vision'.

Poor vision caused by keratoconus is initially resolved with glasses. Once glasses no longer help to improve vision when the condition progresses, contact lenses are the standard way of managing keratoconus. Most non-disabled people with keratoconus will wear contact lenses for years.

Contact lenses may not be practical for people with learning disabilities - but they should not be dismissed without careful consideration. Some people have been supported to wear them successfully. The Look Up factsheet, Contact lenses for adults with learning disabilities and keratoconus, highlights some of the issues.

A minority of people with severe keratoconus have an operation known as a corneal graft, or transplant. An even smaller minority will have an operation on their ‘second eyes’; this is being attempted more often than in the past.

Although this surgery has a high success rate, it is not the magic answer. There are substantial obstacles to be addressed in order for the process to be successful.

Parent: “A corneal graft is totally different from all other operations. The treatment has a start, a middle and a middle. It's a condition needing life-long treatment - never an end”.

Consultant: “A corneal graft ties someone to an eye department indefinitely”.

Key worker in a supported living setting: "We're used to supporting people through cataract surgery these days. But an operation for keratoconus is totally different and needs serious discussion because a whole staff team will be affected - for years".

Hospital treatment for people with learning disabilities who have keratoconus requires careful planning - before, during and after the operation.

There may be many follow-up visits to the hospital, and aftercare involves

• the frequent insertion of eye drops. Drops may be discontinued eventually.
• medication for the rest of their life, to prevent the graft being rejected. These drugs may have undesirable side effects, although major problems are rare. Allergies to these drugs are unusual.
• Some people may need to have further procedures under general anaesthetic if it is necessary to remove stitches from the cornea.

It may be necessary to convince ophthalmologists that surgery is in a person’s best interests, and that appropriate long-term support is available.

What is a corneal graft?

Corneal surgery involves removing a damaged disc of corneal tissue and replacing it with a clear, healthy cornea from a donor (who has died). The individual and their family have consented to their eyes being used for medical purposes after their death.

It may be necessary to discuss religious or cultural implications. Transplanting tissue from one individual to another may contravene deeply-held views.

All donors are screened for transmittable diseases such as HIV and Hepatitis viruses. The donated cornea is then treated with antibiotic solution before being used in an operation.

The cornea needs to be ordered from an eye bank in advance of surgery. A fixed date for surgery may then be dependent upon the availability of graft material.

It is a lengthy and complex operation, which is performed under general anaesthetic, with the aid of a microscope.

Unlike other organ transplants the risk of rejection is felt to be less as no blood supply is connected to a transplanted cornea.

Why might an operation be necessary?

In the more advanced stages of keratoconus the corneal curve becomes too steep for a contact lens to be fitted satisfactorily. It may just fall out or become too painful to wear. A corneal graft may be considered if vision can no longer be improved satisfactorily by lenses.

It may not be possible to operate if the person develops kerotoglobus, when the curve of the cornea becomes too steep and/or too thin to accept a graft.

Between 10% and 20% only of people with keratoconus need a corneal transplant to improve their vision. Only about 5% will need operations on both eyes.

The importance of information

Keratoconus is a condition that may require complex life-long monitoring and treatment, even if people have a successful corneal graft. It is therefore important that the service user and their supporters understand what is involved.

Mother of service user: “Talking to him about the operation was hard. The whole decision was hard, but I’d say that if the surgeons recommend it, do it! He’s waiting on the other eye to be done now, and is quite relaxed about it”.

Service user: “It is helpful if there is a staff member who finds out all about the condition and what the treatment will mean for the person and for their staff team”.

Service user: “It was so hard to get information. I wanted to know, but I just couldn’t find anything. How can staff plan to support you if they can’t get the information they need? How can people advocate for you to have an operation if they don’t know what it entails?”

Mother of service user: "I felt apprehensive because I didn’t know anyone who had had the operation - but both the optician and the consultant explained things really well to my son and me".

Service user: “They should have talked to me more, not just to my support worker. The service user needs to be involved in decisions. I am glad I made the decision. It was a good thing to do”.

What to expect at the eye department

Waiting lists vary so people may wait several months for an outpatient appointment. There may also be long delays before operations can be performed. This may be due to the demand for eye surgery, but also may be the result of a shortage of available corneas.

Keratoconus is not a common condition in the “general population” but it is believed that up to 1 person in 10 with Down syndrome will develop the condition. There may be few professionals with experience of keratoconus in people with learning disabilities. Eye care staff may be unaware that some people have been supported to wear contact lenses and that an increasing number of people with learning disabilities have had successful corneal grafts.

The person will visit the ophthalmology department of a hospital several times before being considered for surgery. A number of tests (similar to those performed at the optometrist) will be necessary.

There are a number of factors to anticipate

Be prepared for long waiting times in clinics It is vital to inform the hospital if the individual is likely to become agitated or distressed if kept waiting too long. To avoid long waits it may be helpful to ask if the hospital can schedule all tests early in the clinic. However bear in mind that the individual will have to spend a lot of time in hospital, and it will help if they find it as comfortable and enjoyable a place to be as possible. (But make sure the appointment is at a time when they are at their best - not when the effects of sleeping pills have not worn off, or when people are tired after a long day.) Some hospitals have special clinics for people with learning disabilities, so it is worth asking if one is available.

Ensure that the person's regular supporter accompanies them - someone who knows the person and their medical history well. The supporter should know the person well enough to be able to predict how they will react to the various processes involved in the treatment. The supporter should also be able to explain how to gain the person's co-operation, have knowledge of their medication and any side effects. The person is more likely to have a successful outcome in outpatients and from surgery if there is consistency.

Take items to keep the person entertained - such as picture books or personal stereos.

Some people will need to be carefully prepared to cope with the tests:

Acuity test - The person will be expected to read an eye chart consisting of capital letters. It is important to advise the hospital in advance if the person cannot identify letters and would benefit from picture tests (such as the Kay picture test). The hospital may need to make sure the appropriate test is available in the clinic. The optometrist should know that the person would use this test, rather than presenting the letter chart and then having to find picture charts. ‘Failure’ to identify letters may make the person more anxious in the clinic. If someone in your Community Health Learning Disability team or Visual Impairment team has access to Kay picture cards some practice tests can be attempted at home.

Eye drops - Accepting drops is crucial to the success of the corneal graft. It is a key component in the healing and after-care process. (Further information in our factsheet, 'Eye drops for adults with learning disabilities'). Work with people before the operation may be necessary to get them to accept drops. Some people may find it less threatening if they can learn to hold their eye open.

The ophthalmologist will need to insert eye drops to dilate the pupils (make the pupils larger) in order to examine the eye properly. These eye drops sting initially when dropped into the eyes. If the person is likely to react badly to this, it may be best to suggest that the ophthalmologist leave this test till last.

The drops may blur the person’s vision, further reducing their ability to see clearly. Drops may make the person extra sensitive to light. The person is likely to require additional help until the blurring goes - such as being guided to the toilet.

Ophthalmic examination - The ophthalmologist may want to examine the eye on a slit lamp. A slit lamp is a table-mounted instrument with a chin rest. The person needs to be able to sit still during this examination. However slit lamps often dazzle the patient, and cause discomfort to people with keratoconus who are particularly sensitive to light. The ophthalmologist, wearing a head-mounted lamp called an indirect ophthalmoscope, may also look into the eyes. This may make the doctor look very strange! (Detailed information about eye examinations can be found in the Look Up factsheet, ‘Eye tests for adults with learning disabilities'. Look Up has produced an illustrated and taped booklet for service users, ‘Getting your eyes tested'. See further reading section at the end of this factsheet).

Who needs surgery?
There are numerous factors to be considered before corneal surgery is offered for keratoconus.

• The person's vision will need to have deteriorated significantly in both eyes before surgery is considered. Because of the risks involved with surgery, it is rarely considered for people with useful sight in one eye.
• How significantly does poor vision affect the person's lifestyle? There is a long-standing debate about whether people with learning disabilities should be offered surgery solely on the basis of how poor their vision is, and whether the operation can be safely performed. Often the case for surgery needs to be justified by evidence that:

An individual’s quality of life has deteriorated. For example, they may have lost confidence and/or skills. They may be depressed, anxious and afraid. This is sometimes difficult - if the eye condition has been undiagnosed since puberty, staff may not know him/her long enough to notice differences in behaviour.

The person's level of participation and activity is likely to improve should surgery be successful.

• The decision about which eye to operate on is also subject to a number of conditions:

The health of the person's tissue around the intended corneal graft. Sometimes their remaining cornea has become too thin to take a graft safely.

The shape of the cone (the ‘bulge’ in the cornea) caused by keratoconus may have become so pronounced that the cone has reached a stage known as keratoglobus. At this stage it is not possible to graft a cornea effectively, as the remaining tissue will have too steep a curve. The graft would not result in a regular shape after surgery and so would not improve vision.

• The presence of other eye diseases. The retina of an eye may be diseased, reducing the likelihood of successful surgery to improve vision. It may only be possible to assess this when under general anaesthetic. However, the presence of other eye problems (such as cataracts) does not automatically exclude people from having corneal surgery - but they may increase the risks involved.
• Other health problems, which increase the risks involved in surgery.

There should be a full discussion with individual, consultant, staff and/or family carers. It is important that everyone involved understands the implications of surgery and the long-term support that the person will require.

Early preparation

There is a risk of a corneal graft being rejected if people are prone to self-injury directed towards the face or head, eye poking or eye rubbing.

Before the operation it is important to establish the best way of discouraging the person from touching their eyes - perhaps with glasses with plain lenses or a clear eye shield.

When people are at risk of damaging their eyes they may need months or even years of preparation before surgery. Their eye-touching behaviour may need to be reduced with the help of a psychologist or behavioural support specialist.

Planning for the operation

When waiting for corneal surgery a time period will usually be given as opposed to a specific date. However, when discussing a corneal graft for a person with learning disabilities, the possibility of delay or postponement must be taken into consideration.

Service user: “They cancelled my operation without telling me. I had been building up to this operation for weeks and weeks. I cried and cried”.

It may be possible to make arrangements with the ophthalmologist for a provisional date so that support staff can be available.

Service user: “My care manager can't organise extra help for me until she's got the date. But I have to wait for a cornea. The hospital can't give me a fixed date for my graft yet, which causes her problems”. 

Support prior to the operation

 
It is important that the supporter is there when the person is prepared for their operation and goes to the operating theatre - and is available when the person comes to.

Mother of service user: “It was stressful watching him go into the operating theatre. I was worrying about if he would come back”.

Service user: “My keyworker held my hand as I went to sleep, and was holding it when I woke up”.

When surgery is refused

Non-disabled people may request surgery if they find it difficult to wear contact lenses. But consultants will refuse to operate if a graft is unlikely to resolve the person's problems.

Staff and family carers contacting Look Up sometimes assume that ophthalmologists are prejudiced against people with learning disabilities. But several ophthalmologists have pointed out that only a minority of people with keratoconus will benefit from a corneal graft.

Parent: "I had assumed the consultant was refusing to operate on my son because of his severe learning disabilities. But the consultant carefully explained that Simon would not be suitable for a graft - even if he wasn't so disabled".

Optometrist: "The consultant was happy to examine him, but felt that Joe's eye was in such a poor state that it would not be safe to operate. It was better to leave him with some vision, albeit blurred. The risk of blindness from surgery was far too great”.

Staff member: "They said it was too early to discuss a graft. They wanted us to support him in contact lenses instead, saying that the condition might stabilise, or even improve”.

It is important to establish why a graft has been refused - and to try to keep options for treatment open at a later date if at all possible.

Hospital optometrist: “There is no evidence that delaying surgery reduces the success rate - but some surgeons believe it can”.

Hospital social worker: “Don’t allow the person to be discharged their situation may change. There may be a shortage of donated corneas and surgeons may consider that other patients will have better visual outcomes. But things do change”.

For example:

• The person may not have adequate support after the operation now, but additional support may be available in due course.
• The person constantly pokes or rubs their eyes and makes their eyes sore, but treatment for an allergy and/or a psychologist/behavioural support worker working with supporters might be able to reduce the eye touching over a period of time.
• The person is prone to eye infections from rubbing their eyes. A programme of 'hand hygiene' might be initiated. Supporters need to ensure that people's fingernails are cut carefully with rounded sides. 'Square corners' to fingernails can cause considerable damage to the eye. It is important to prevent hair from falling into people's eyes and irritating them.

When an operation has been refused, it is important that the individual is closely monitored by the staff, to see if vision is deteriorating. It may be possible to obtain photocopies of eye tests (letter charts or Kay picture charts) from optometrists. Having access to the charts would enable staff to check that the person's vision has not deteriorated.

If keratoconus progresses it is important to refer the person to a rehabilitation worker for visually impaired people. They are usually employed by the local social services/social work department or voluntary society for blind and partially sighted people.

Risks

All operations entail some risk and transplant surgery carries a risk of rejection. However, at present 95% of all corneal grafts are successful. The reason for this being the most successful of operations using donated tissue is that the cornea does not have a blood supply.

But any injury to the grafted eye, at any time after the graft, may seriously damage it.

Rejection of the donated cornea is most likely to occur in the first year after the graft, but it can happen at any time.

When a graft is rejected, it is mostly only the front of the eye which is affected, so it may be possible for another graft to be attempted at a later date.

Supporting people with learning disabilities

It is vital that people undergoing grafts should have sufficient support to enable family carers or staff to take the person to a casualty department immediately if eyes look red.

Grafts are only possible for people with learning disabilities when there are consistent supporters who have learned to insert drops safely and regularly. The person must consent to these drops being administered. Surgery cannot be considered if the person does not agree to and accept eye drops.

A successful corneal graft requires the patient to be able to co-operate with all the treatment. This requires supporters to prepare the person for the different stages - such as the many outpatient appointments - and continuity of support is vital. The supporter will need to:

• Understand about keratoconus and the treatment proposed
• Obtain information about the hospital and its approach to grafts
• Explain to the person that the operation is to improve their vision - but that improved vision is not guaranteed
• Ensure the person understands that it may take time before their sight does get better
• Outline the procedures involved in the graft and follow-up to the person
• Gain their consent to the different procedures

 
In addition, it is important that:

• there is sufficient support for the person at all appointments, including transport being available
• the person's family carers and/or supporting staff members fully understand the operation and follow-up treatment
• supporters are able to monitor the health of the person's eye and insert drops regularly
• hospital staff appreciate that the person may need more time and a special approach in order to gain their co-operation.
• It is crucial that all members of staff involved with the person understand the plan to operate and what support they will need after the operation and in the long term.

Residential worker: “The whole staff team learned to do his drops. We practised on one another before we went near him!” 

Protecting the eye after surgery

 
Preparing the person for surgery is the key to successful treatment.

Guidelines must be prepared to ensure that the individual will not touch the eye after surgery. It is important that all potential risk factors to the eye are anticipated and strategies put in place to prevent further damage occurring.

After surgery the individual will need to wear an eye patch and a protective shell. The time that these have to be worn will vary according to the anticipated risks to the eye. The patch and shell protect the eye safely - but they require the person to co-operate and not remove them.

Staff and family carers have adopted familiarisation programmes with patches. Some of these programmes are more akin to games.

Wearing a patch can irritate and itch. Some people will not accept a patch being taped to their face, and may try to rip it off, possibly causing themselves harm. Some people who reject patches are allergic to them, so alternative methods of protection must be considered.

Wearing a patch reduces people’s vision - or may even make them totally blind. Whilst supporters may believe this is a small price to pay when considering a lifetime of restored vision, the person may find that coping with a patch is frustrating, frightening and unpleasant. It may be a major problem if the person’s ‘good’ eye has received the graft. A clear ‘see through’ shell can often be provided to allow some light to enter the eye.

An alternative to this is to encourage the person to wear wrap-around anti-glare glasses. The person can be introduced to wearing these months before surgery, with a gentle ‘de-sensitisation’ programme. These special glasses have the added advantage that they eliminate glare as they reduce light sensitivity. The recommended colour filters for front of eye conditions such as keratoconus are dark greys or greens. Although these glasses may not reduce eye touching, they do have a rim, which makes it difficult for the person to rub underneath them. In addition, these glasses may act as a barrier, reminding the person not to touch their eye.

Thorough risk assessment needs to be carried out on all aspects of the person’s lifestyle. Some of the risk factors that need to be considered are:

• Safe activities need to be found to keep the individual stimulated for the two months after surgery.
• All sports that involve contact or bending need to be avoided. Swimming may cause irritation or infect the graft.
• Boredom or under-stimulation may increase the likelihood of self-injurious behaviour.
• It may be appropriate for the person to withdraw from settings (such as day centres, college and so on) during the risk period in case another service user causes injury to the individual. During the risk period, it is safer to provide a more tailored 1:1 service away from busy environments.
• Hygiene - hair washing, bathing and face washing all need to be done with care to prevent the risk of injury. Non-perfumed baby products may be safer to use than the usual soap or shampoo - but advice should be obtained.
• Wearing eye make-up should be avoided. There is a possibility that some products could aggravate the graft or cause problems for contact lens wearers.

Pre-operative procedures

The patient’s understanding of the procedure may be limited so it is vitally important to have the same member of staff or family carers supporting them at appointments to explain and give confidence. There will be frequent and lengthy hospital visits, and the prognosis of visual outcome is important before decisions are made.

Before the operation there must be careful preparation. The eye pad and the shield to be worn after the operation must be shown, there will be a general anaesthetic and frequent instillation of eye drops. It is important that someone familiar and trusted by the patient is there continuously. 

The individual will be required to attend hospital two or three weeks before their operation to have a pre-operative assessment. This involves health tests to assess if it is safe for them to have a general anaesthetic. Some people with learning disabilities will be wary of some or all of these tests, so staff or family carers need to be aware of them and prepare the individual appropriately.

Tests usually include:

A urine sample - the person will be asked to urinate into a jar or small bottle. If the person needs help to do this, having a supporter of the same sex may make it less embarrassing!

Blood tests often hurt and involve needles that frighten many people. If the individual is likely to get very distressed by a blood test, it may be best to ask for this test to be done at the end of the pre-operative visit.

Blood pressure test usually involves a strap being wrapped around the arm, with air being pumped into the strap. This does not normally hurt, but it can feel strange.

A cardiogram requires sticky pads being attached to the person’s bare chest, which are wired to a machine that measures their heart rate. If female service users need support, female staff or relatives should accompany them. At the end of the test the pads are pulled off. This can hurt - especially when people have hairy chests.

All these tests are important to monitor the individual’s safety while under anaesthetic, and should be done if possible. However, it may be that the person will refuse to co-operate with certain tests. Surgery is still possible, but there are greater risks without this information being available. However, some of the information may be obtained when the person is under general anaesthetic.

It should be possible for the individual to visit the ward and meet the staff prior to their admission. This will be an opportunity to discuss the person’s special needs and to anticipate any problems. 

Day of surgery

 
Most people worry before their operation - so it is important to prepare people with learning disabilities to reduce their level of anxiety. The day of surgery presents a number of stressful and risky situations. Many of the problems people encounter are caused by their lack of familiarity with hospital routines and procedures.

Individuals having surgery must be consistently supported by familiar staff or family carers. They must trust their supporter and feel able to confide in them.

Whilst medical staff must explain about the operation to the individual, this may be done in a hurried way. The hospital staff member may not have experience of people with learning disabilities so supporters or relatives should also be able to explain procedures to the individual if necessary.

Service user: “The nurse explained about the graft to me, but she was in a hurry and I wanted more time to ask about the operation and the aftercare”.

Nurse: “We always have to explain the procedure several times, and people need accessible written information too. Medical words need explaining. We usually go over the information lots of times. Even the most able people still find it hard to understand. That’s keratoconus for you!”

Supporters will also need to outline to nursing and medical staff how the person is likely to react to unknown situations.

People with learning disabilities may feel uncomfortable in hospitals. Some common reasons for this are:

• Many people with learning disabilities had negative experiences when living in long-stay hospitals. They may fear that they will never leave hospital after their operation. It is therefore important to stress the difference between a hospital which plans to discharge people (a ‘go home hospital’) and a long-stay institution.
• They may feel intimidated by so many unfamiliar faces and people in white coats.
• Hospitals are generally unfriendly places for people with severe sight problems. They may find it hard to cope with flickering fluorescent light, echoes from rooms with high ceilings, glare from shiny floors. Buildings may be poorly labelled, making people rely on help from others.
• The individual will not be allowed food or drink before they have surgery under anaesthetic. They may not understand this change of routine, and be upset by it.
• However, some people may positively enjoy new situations and all the extra attention they receive in hospital.

Immediately after the operation

After-effects of surgery will vary dramatically from patient to patient. Some people can be quite sick after a general anaesthetic. Time in hospital varies - and many people will just stay in overnight.

Mother of service user: “He was in hospital about 24 hours. It was no bother. He was very calm about stopping in overnight”.

The operated eye will be covered with an eye pad and plastic eye shied to protect the eye. As the anaesthetic wears off the person may feel some discomfort or pain - and so should ask for medicine for pain relief. Eyes can feel sore for about two weeks.

Vision may not return instantly. It can take up to a year for some people to regain clear vision.

Keratoconus group member: “The vision in my first eye took a while to improve, but with my second eye the improvement was immediately obvious”.

There is a risk that people may become depressed if the operation does not immediately improve vision. People may have been upset by sight loss and spent months building up to the operation, hoping to see clearly again.

Service user: “They told me that they'd given me a new eye, but I couldn't use it yet! Then they explained my sight would improve over time. It did - but not immediately. Lots of people had warned me about this, so I was not surprised or upset”.

People may expect perfect vision and so are disappointed to discover that their vision only improves when they are prescribed glasses or contact lenses. People who had no obvious benefit from glasses before the operation may find that glasses help them considerably after the graft.

Eye rubbing must be avoided after surgery, as it can cause severe damage.

Service user: “I was worried about rubbing my eye after the operation. But all I wanted to do for the first two weeks was sleep - in hospital and at home. Nothing would keep me awake!”

Hand hygiene is important, and support should be offered to minimize risks.

Drops

The individual will need to have eye drops inserted regularly - usually four times a day for six months or more after surgery.

Staff and family carers will be shown how to insert drops - but some people prefer to do them it themselves.

People should wash their hands before and after inserting drops, to reduce the risk of infection.

Service user: “I quickly learned to do the drops myself. It was better than having people poking around your eyes all the time”.

Mother of service user: “The main problem was the drops after the operation. He did his own drops at college because there was no nurse on site. I later learned that the community nurse could have done them”.

People may need to be woken up at night for drops, which may result in people being constantly tired and having problems getting back to sleep. However others may want to sleep most of the time in the days after surgery.

Allergies to drops

It is important to look out for allergic reactions to the drops. People with learning disabilities may not have been tested for allergies in the past.

Keratoconus group member: “In the first week after my operation, which was successful, I suffered from chronic eye irritation. It turns out that I am allergic to eye drops with preservative in them. As soon as I had preservative-free eye drops, everything was fine”.

Keratoconus group member: “The operation went well, but then my eye became very swollen and irritated almost immediately. I was prescribed preservative-free drops. However, a few days later a mistake occurred, and I inadvertently received drops with preservative again. Immediately there was a flare-up. Nursing staff and carers must be aware of how drops with preservatives in them can have such an unpleasant effect”.

Recovering at home

It is recommended that people have a gentle routine for the first two weeks after the operation, avoiding bending and lifting anything heavy.

Keratoconus group member: “The graft was successful although the healing process was slow. I did not return to work for nearly 9 months”.

Service user: “I felt OK almost immediately, and it didn't take me long to get back to normal - except I couldn't bend to put the rubbish out!”

People should obtain advice from their ophthalmologist about activities that might put the operated eye at risk, such as sports or swimming.

Mother of service user: “The main problem was keeping him occupied. He loves swimming, and just had to watch”. 

Medication

 
Anti-rejection drops can increase the pressure inside the eye, causing problems to the back of the eye. People need careful monitoring - at home and in clinic.

The regime is complex, with frequent eye drops and other medication, such as immunosuppressant drugs to reduce the possibility of the graft being rejected. These drugs often have side effects that need careful monitoring.

Consultant: “I've tried everything to prevent rejection in this patient who has complex medical problems. But to increase the medication would probably cause him a stroke, which is more dangerous. But at least his eyes are good enough for another graft”. 

Aftercare and follow up

 
There will be many hospital outpatients visits and tests, after the operation, initially every fortnight.

Vision may be improved, and then may deteriorate - or it may stabilise.

People who have a corneal graft need life-long medication and monitoring to prevent the graft being rejected. Over half the people who have had a graft still need glasses.

The eye is very vulnerable and will always need extra care and protection.

Before a positive improvement in vision is obvious, people may still have some visual problems, such as light sensitivity and double vision. Tinted lenses may be necessary for a time - because of problems with glare.

Keratoconus group member: “It's meant taking particular care with my grafted eye, and coping with the light sensitivity that goes with keratoconus - such as finding the right sunglasses”.

It is recommended that the individual is examined by an optometrist three or four months after their operation to check vision and see if glasses or contact lenses are needed.

People may need long-term steroid treatment. This can cause new health problems and people need close monitoring. Anyone taking steroids should carry a card saying that they are on steroids in case of an emergency.

People may worry about a lack of continuity at the eye clinic.

Mother of service user: “We didn’t see the consultant ophthalmologist every time after the operation, but he made a point of checking with the junior doctors to ensure some continuity”.

Service user: “I didn’t see the same doctor each time after the operation, so I always worried that my consultant did not know what was going on”.

Contact lenses

After corneal surgery many people need contact lenses, but these are not always practical for people with learning disabilities. However, contact lenses should not be dismissed outright - people with learning disabilities have been successfully supported with contact lenses. 

Fitting lenses after surgery may be difficult as the eye may be an odd shape. In addition, there may be problems in the donated cornea - such as astigmatism which may need correcting with contact lenses or glasses.

Sensation may take up to ten years to return to cornea. Fitting contact lenses in some people may be hazardous if their cornea is not sensitive. This means that the person might be unaware of any potential damage to the eye.

Glasses

Keratoconus is a complex condition. People may not remember warnings not to expect perfect vision after surgery. They may be disappointed to discover that their vision only improves when they are prescribed glasses or contact lenses.

People who received no obvious benefit from glasses before the operation, may find that glasses help them considerably after the graft.

Stitches to the eye (sutures)

Consultants use different ways to stitch the cornea. It may be appropriate to discuss the merits of the different techniques with the surgeon.

Stitching a corneal graft has been compared to sewing a patchwork quilt. The stitches must be even and of the same tension around the graft in order for the eye to heal at an equal rate and to regain its football-shaped curve. These stitches are thinner than a human hair and unlikely to interfere with vision. However, stitches can irritate.

Depending upon the technique used, stitches may need to be removed. This may take place from three to eighteen months after surgery. While some people have their stitches removed in clinic, people with learning disabilities usually have day surgery or hospital admission for a general anaesthetic.

Consultant: “Loose stitches cause irritation and need to be removed because they can do more harm than good”.

Consultant: “Just ask about stitches being removed. There are many variables”.

An eye patch needs to be worn for a few weeks after stitches have been removed.

Stitches in the eye may be visible.

Preventing the graft being rejected

Although corneal grafts for keratoconus have a high success rate, treatment continues to be necessary.

Rejection can occur at any time in a person’s life after surgery but is most likely during the first year. Rejection is more likely to occur in people with allergies - including hay fever.

The grafted eye is very vulnerable and will always need extra care and protection.

Rejection of donated corneas is less likely than with any other donated organ, but it can occur and the person will need careful monitoring. It may be possible to give the person another corneal graft.

Specialist worker for adults with learning disabilities and visual impairment: “Sad to say, her cornea was constantly rupturing, so a second graft was attempted. That did not work and now she is blind”.

Keratoconus group member: “I’ve had grafts in both eyes, and not had any signs of rejection. I have found ways of managing my lifestyle - you have to be positive and find your own solutions”.

Symptoms of rejection

Initial symptoms of rejection are:

• redness of the eye
• cloudiness of the eye
• eye infection
• sensitivity to light
• deterioration of vision
• watery eye

If rejection is suspected, it is vital that people go to an Accident and Emergency Department of an eye hospital immediately.

A&E departments in general hospitals may not know about keratoconus.

Prompt treatment can usually control the reaction. Some people with learning disabilities have been put at considerable risk because staff have confused the symptoms of rejection with eye infections which are not related to keratoconus.

Hospital optometrist: “GPs are unlikely to know much about keratoconus, and optometrists vary. The knowledge of keratoconus is not in the community. In an emergency, people must go to eye hospitals, not ordinary hospital accident and emergency”.

Conclusion

An increasing number of adults with learning disabilities have had successful corneal grafts for keratoconus. This has radically improved their quality of life. However, treatment for keratoconus is life-long and careful planning is necessary to ensure a successful outcome.

Mother of service user: “He can see out of his ‘good eye’ much better now, although the vision still isn’t perfect because he had some scarring”.

Residential social worker: “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 - happy and loving life. The staff team made the commitment. We just believed it was his right”.

Service user: "The operation has changed my life. So much better. But it wasn't easy from start to finish, but everyone was so kind".

Specialist worker for visually impaired people with learning disabilities: "The staff were wonderful, supportive before, during and after the graft. I think there should be awards for people like them".

Further Reading

'Keratoconus: some questions answered' produced by

Moorfields Eye Hospital, with support from the Keratoconus Group. Available from:

Keratoconus Self Help and SuportGroup

P O Box 26251, London, W3 9WQ

Telephone 020 8993 4759

Email: chair@keratoconus-group.org.uk  

Website: www.keratoconus-group.org.uk