Feedback We appreciate and encourage feedback. If you need advice or are concerned about any aspect of care or treatment please speak to a member of staff or contact the Patient Advice and Liaison Service (PALS): Freephone (City Hospital Campus): 0800 052 1195 Freephone (QMC Campus): 0800 183 0204 From a mobile or abroad: 0115 924 9924 ext 65412 or 62301 Minicom: 0800 183 0204 E-mail: [email protected] Letter: NUH NHS Trust, c/o PALS, Freepost NEA 14614, Nottingham NG7 1BR www.nuh.nhs.uk How to give eye drops directions adapted from Great Ormond Street Hospital (GOSH) (Updated 2008) How to give your child eye drops. [Online]. Available at: http:// www.gosh.nhs.uk/gosh_families/information_sheets/medicines_eye_drops/ medic ines_eye_drops_families.html.

If you require a full list of references for this leaflet please email [email protected] or phone 0115 924 9924 ext. 67754. The Trust endeavours to ensure that the information given here is accurate and impartial.

Chronic uveitis (eye inflammation) associated with juvenile idiopathic arthritis (JIA) Information for patients Children and Young People’s Rheumatology Service

This document can be provided in different languages and formats. For more information please contact: Children and Young People’s Rheumatology Service Nottingham Children’s Hospital Queen’s Medical Centre Campus Tel: 0115 924 9924 ext 61983

Dr Kishore Warrier © April 2015. All rights reserved. Nottingham University Hospitals NHS Trust. Reviewed April 2017. Ref: 0535/v2/0415/AM.

Public information

Aim of the leaflet

Other useful contacts

This leaflet is aimed at children with juvenile idiopathic arthritis (JIA), and/or JIA-associated uveitis and their parents/care givers. This leaflet aims to tell you about JIA-associated uveitis, how it is linked with JIA and how the condition is treated.

Arthritis Research UK Web: www.arthritisresearchuk.org

What is chronic uveitis?

Children’s Chronic Arthritis Association (CCAA) Web: www.ccaa.org.uk Tel: 01905 745595

Uveitis is a condition that causes swelling within the eye. It affects the iris and/or other parts of the eye. There are different types of uveitis:  Acute uveitis - this is when the inflammation occurs suddenly. Usually the eye is red and painful.  Chronic uveitis - this is when the disease occurs slowly often with no symptoms (signs).  Acute on chronic uveitis - this is an acute episode on top of chronic disease. In JIA the condition is often referred to as iridocyclitis or iritis because the main structure affected is the iris (the coloured part of the eye - see diagram below). The inflammation can have an effect on how the eye works and can cause vision to get worse. The disease can affect one or both eyes. There are different names for uveitis depending on the area of the eye affected:  Anterior uveitis (front of the eye)  Intermediate uveitis (middle section of the eye)  Posterior uveitis (back of the eye)

2

Paediatric Rheumatology International Trials Organisation (PRINTO) Juvenile Idiopathic Arthritis Web: www.printo.it Royal National Institute of Blind People (RNIB) Web: www.rnib.org.uk Tel: 0303 123 9999 Email: [email protected] Uveitis Information Group Web: www.uveitis.net Tel: 08456 045 660 Email: [email protected]

11

Where can I seek help If I am worried? If you suspect that your child has uveitis or if your child is being treated for uveitis and you feel the condition is getting worse you can get advice by contacting the following: Consultant’s secretary - if under the care of an ophthalmology consultant (eye doctor). Please call the hospital switchboard on 0115 924 9924 and ask to be put through to your consultant’s secretary. Normal working hours: Monday to Friday (9am-4pm) not public holidays. Nottingham Children’s Hospital Paediatric Rheumatology Nurse Specialists Tel: 0115 924 9924 ext 61983 (answer phone if nurses not available - calls will be returned within 48hrs Monday to Friday). Out of hours: Advice for children on immunosuppressant medication who are unwell. Please contact Ward E39 on 0115 924 9924 ext 69039. State that you are calling about a patient with inflammatory eye disease under the care of Dr Venning/Dr Rangaraj and also give the name of the ophthalmologist who is managing your child. Eye Casualty Tel: 0115 924 9924 ext 62882 (available 7am-10pm every day including weekends). If you are a patient with an existing eye condition please phone the department in advance. This allows the nursing staff to give you a time to be seen when a doctor is in the department Your GP practice NHS 111 Tel: 111

10

The body’s natural immune system normally helps to keep the body healthy by fighting off infections such as coughs, colds and stomach bugs. In conditions such as uveitis and JIA the immune system becomes overactive and it may start to attack parts of the body such as the linings of the joints and blood vessels for no apparent reason. This causes swelling. The diseases caused by this over activity of the immune system are called autoimmune diseases.

How common is chronic uveitis in children with JIA? One in 1,000 children in the UK develop JIA. Around 30-40% of children with JIA are at risk of developing uveitis. Uveitis may not occur at the same time that the arthritis is active. Uveitis most commonly develops within two years of the child or young person being diagnosed with JIA. Eight years after diagnosis of JIA the risk of developing uveitis is reduced.

Why do some children with JIA get chronic uveitis? The tissues (linings) in the joints are similar to the tissues found within the eye. It is thought that the trigger which causes arthritis in the joints also triggers an inflammation in the eye. The trigger that causes the arthritis to develop is not yet known. Chronic uveitis is more common in girls with JIA than in boys with JIA.

Is chronic uveitis painful? In most cases the condition is not painful. Unlike in other types of eye inflammation where the eye may become red and look sore, in uveitis there may not be any signs or symptoms and the eye may look normal. The lack of signs and symptoms can make the inflammation in uveitis difficult to detect. Sometimes the eye may become red and painful if other complications occur.

3

How will the condition be detected? Chronic uveitis is hard to detect because it can be painless and doesn’t cause the eye to become red. Uveitis may be suspected because the shape of the pupil changes and becomes irregular instead of round, or the child or young person’s eyesight becomes worse. This is why children with JIA are given regular eye examinations or screening, so that any inflammation present in the eye can be detected. Children who are at risk of developing inflammation in the eyes will be seen on a regular basis by orthoptists who will perform regular eye examinations to look for signs of uveitis. High risk is associated with:  Age. Uveitis is more common in younger children  Girls with JIA are more commonly affected  Previous episodes of uveitis  Anti nuclear antibody– a specific antibody detected by a blood test.  Oligo articular JIA - where arthritis affects fewer joints. It is important to remember that uveitis may occur after the arthritis has settled down.

Remember   

Store eye drops correctly - information for how to do this is on the bottle label. Make a note of the date the bottle was opened and do not use after the stated ‘in use’ expiry date. Store medicines out of the reach and sight of children.

Can chronic uveitis affect my child’s sight? For many children the inflammation is mild and will settle down without causing any permanent sight loss. Early detection of the inflammation and making sure treatment is given as prescribed are very important, even when it seems that the eye is fine. This can prevent complications from developing. However, there is a risk that the inflammation can affect eyesight, especially if treatment is not given as prescribed or if treatment is delayed. Some children who develop chronic uveitis will experience some degree of permanent sight loss due to complications such as the development of a cataract (cloudy lens) or glaucoma (raised pressure within the eye). Keeping hospital appointments is very important so that the condition can be monitored and the effect of treatment checked.

It is important to seek help if you notice or suspect any of the following:  Worsening (deterioration) in your child’s eyesight - this may be noticed in school  An irregular (odd) shaped pupil or change in pupil size - the pupil is normally round and the pupils are normally the same size  A red eye - if the white part of the eye becomes red or pink in colour  An uncomfortable or painful eye  Sensitivity to light

4

9

5. Gently pull down the skin at the top of your child's’ cheek or lower eyelid with the index finger of your free hand so that you create a small pocket in which to put the medication. You should be able to see the pink inner eyelid. 6. Hold the eye drop bottle above your child’s eye and squeeze one drop into the pocket you have created. Do not touch the end of the bottle against your child’s eye, eyelashes or any other surface as this could cause contamination of the contents. 7. Release the lower eyelid and let your child blink a few times to make sure the drop is spread around the eye. 8. If your child is distressed by point 5, ask your child to stand or lie down with their head tipped back and to the side with their eyes closed. Place an eye drop in the inner corner of their eyelid (the point close to the nose). Encourage them to open their eye slowly, allowing the eye drop to fall into the eye, or gently rub the closed lid to let the drop bathe the eye. 9. Wipe away any excess medication with a clean tissue. 10. Press gently against the inner corner of the eyelid close to the nose. This helps block the tear drainage system and slows the drainage of the medication from the eye, which helps allow the eye to absorb the medicine. 11. Encourage your child to sit with their eyes closed for a few minutes as this will help the eye absorb the drops. Try to discourage your child from blinking too much as this may cause the eye drop to be flushed away by tears. 12. When giving more than one type of eye drop at a time you should wait a few minutes after giving the first drop before giving the next medication to allow the first medication to be absorbed. Wash your hands when you have finished. 8

How is chronic uveitis treated? The aim of treatment is to control the inflammation causing chronic uveitis. The first line of treatment for uveitis is in the form of eyedrops. The types of eyedrops used are steroid eye drops or dilating eye drops. Steroid eye drops (such as dexamethasone Maxidex ®, prednisolone Predforte®, fluoromethalone FML®, rimexolone Vexol ® and loteprednol Lotemax ®) Steroid eye drops are used to control the inflammation that causes uveitis. These eye drops may be needed many times throughout the day, sometimes up to every hour for a number of days. If your child is under the care of the rheumatology team please let the rheumatology nurses know if a frequent eye drop schedule has been prescribed as they can help with arrangements for medication to be given during school hours. Contact details are at the end of this leaflet. Parents of children not under the care of the children's rheumatology team should contact their school nurse. Dilating (mydriatic) eye drops (such as cyclopentolate) These are used to relax the muscle which control the iris. This helps to control pain and inflammation and helps stop the iris from sticking to the lens of the eye, which causes an irregular shaped pupil (not the usual round shape). Dilating drops can have an effect on the muscles in the eye which control the size of the pupil, making it bigger and less reactive to bright light. This might make your child uncomfortable in bright light. Wearing sunglasses or a hat may help in these conditions. Vision may also be blurred as the muscles affected by dilating drops help the eye to focus for near vision. This effect is only temporary. 5

Dilating drops are used until the inflammation settles. Dilating drops can sting when they are put into the eye but they must be used as prescribed. Being aware that these drops sting can help you/your child prepare in advance. If eye drops are not effective on their own other forms of treatment may be needed such as:  Steroid injections around the outside of the eye. This may be done under a general anaesthetic.  Steroid infusions. A cannula (needle) is placed into a vein so that an infusion (drip) of the steroid medication can be given. An infusion is used because a high dose of steroids is needed to treat the inflammation. This is a higher dose than can be given as a medicine by mouth. The nurses will explain the procedure to you and your child and some local anaesthetic cream or numbing spray can be used to make the cannula insertion more comfortable. Any blood tests that are needed can be done at the same time. A steroid infusion is usually given daily for three days running. For this you/ your child will need to come into hospital as a day case each day and go home after the steroids have been given. This course of treatment may be repeated as needed.  Eye pressure relieving drops such as latanoprost Xalatan® , brimonidine tartrate Alphagan® and timolol maleate Timolol® . These help to reduce pressure within the eye which can cause a condition called glaucoma. If left untreated glaucoma can cause damage to the optic nerve and affect vision.  Immune suppressant medication to slow down the activity of the immune system may be needed to control the overactive immune system and reduce the inflammation present in diseases like uveitis and JIA. Methotrexate is one of the medicines used to treat uveitis and JIA. Methotrexate is given once a week usually as a subcutaneous injection (an injection under the skin) or occasionally by mouth.

6



Biologic therapies may be used to control the immune system by blocking some of the proteins (chemicals) which cause inflammation. These medicines include infliximab (given as an infusion or drip) and adalimumab (given as an injection). Biologic therapies are a newer type of drug and are normally used if the drugs previously mentioned have not been effective enough at controlling the disease.

How to give eye drops to your child

1. Wash your hands. 2. Shake the eye drop bottle. 3. Remove the top from the bottle and throw away the plastic seal. 4. Place your child into one of the positions below:  Tilt your child’s head back.  Lay your child flat on his or her back.  Ask someone to hold your child in a safe position.  Wrap your baby or young child in a light blanket or sheet to keep his or her arms and legs still.

7