Published on 01 April 2011
Youngsters with arthritis are at risk of losing their eyesight. Jane Tadman talks to the mother of a little girl whose eyesight was saved by anti-TNF therapy, and reports on a new clinical trial which could make this drug more widely available to younger children.
For the parents of young children with severe arthritis, knowing that they face a lifetime of battling against pain and potential disability is hard to come to terms with.
Vicki Smith, the mother of a young girl diagnosed with arthritis at the age of just 18 months, had an even greater fear–that her daughter Clare would go blind.
If it’s little known among the general public that children and babies can develop arthritis, it’s even more of a surprise for many people to learn that these youngsters are also at real risk of losing their eyesight.
“It was heartbreaking...”
Uveitis, or inflammation of the eye, is a common symptom of certain types of juvenile idiopathic arthritis (JIA). Up to 30 per cent of children with JIA are at risk of uveitis, and in one third of these children the disease is of sufficient severity to cause visual loss, cataracts, increased pressure in the eye and blindness.
A few months after Clare, now nine, was born, her crying and screaming, high temperatures, rashes, and unwillingness and inability to stand up or walk made her mother suspicious that something was seriously wrong. By the age of 18 months doctors at the Bristol Royal Hospital for Children had diagnosed Clare with JIA and started her on medication that helped but never properly controlled her condition.
Three or four times a year the little girl would suffer terrible flare-ups that resulted in regular hospital stays. Four or five of her joints would be swollen and painful, and she needed numerous steroid injections to dampen down the inflammation ravaging her little body.
Through it all, her family – mum Vicki, dad Tony and 13-year-old sister Alex – tried to carry on with as normal a life as possible, with Clare developing into a high-achieving youngster, despite her regular absences from school because of her illness.
“If you could have seen Clare three or four years ago, it was heartbreaking. She would be walking to school because she didn’t like the buggy, dragging her leg, and I would be there trying not to cry,” remembers Vicki.
Worse was to follow. Clare developed uveitis, which required eye drops every two hours. Doctors gave her infusions of the anti-TNF therapy infliximab, but this didn’t help. As the inflammation attacked her eyes, pressure built up, leading to real fears she might go blind.
“When they told me that I was in a hell of a state,” says Vicki. “I was more worried about her losing her sight than anything else.”
“Things are so much better”
But following two successful operations to drain the eyes in order to relieve the pressure behind her eyes, doctors managed to get Clare onto another anti-TNF drug, adalimumab. This drug is not currently licensed for children of Clare’s age, and her rheumatologist Dr Athimalaipet Ramanan had to seek permission to use it off licence, on what is known as a named patient basis from the local primary care trust’s exceptional funding panel, on account of the severity of Clare’s condition. Happily, it worked.
“Since she has been on this drug and had the surgery everything has kicked in, and for the first time her joint pain – and the uveitis – is being controlled,” adds Vicki. “She only needs eye drops twice a day now, not every two hours and has hardly had any flare ups in the past 18 months. Things are so much better.”
Now a happy, tomboyish nine-year-old who loves football and supports Liverpool, Clare is doing well at school although her mum believes there has been real lack of understanding about how arthritis affects her daughter. “Because she looks well most of the time they don’t realise that she gets very tired in the afternoons, and can’t always do what other children do.”
Being off school so often also caused problems in that Clare often felt left out and excluded. When Vicki and Clare visited a child psychologist it was suggested that she might benefit from having a dog to keep her company.
“We’d talked about getting a dog for ages and hadn’t got round to it, and then I heard Clare talking to one of her friends and she said: ‘If I lose my sight at least I’d get a dog,’” says her mum. “That did it.”
Clare and Buster the Labrador are now inseparable.
How Arthritis Research UK is working to prevent blindness in children with arthritis
A major UK-wide clinical trial is about to start which researchers hope will prevent blindness in younger children with arthritis, like Clare.
With funding of £1.5m jointly awarded by Arthritis Research UK and the National Institute for Health Research Health Technology Assesment (NIHR HTA) programme, the trial aims to recruit more than 150 children with JIA, who are at risk of uveitis.
The study will be run by chief investigators Dr Athimalaipet Ramanan, lead consultant in paediatric rheumatology and honorary reader at Bristol Royal Hospital for Children and (and Clare’s doctor since the age of three) Dr Michael Beresford, senior lecturer in paediatric medicine and honorary consultant paediatric rheumatologist at Liverpool Children’s Hospital.
The trial will test the effectiveness of adalimumab, which is currently licensed to treat adults with rheumatoid arthritis and children between the ages of 13 and 16 with JIA.
However, doctors hope that if they can show the drug to be both safe and effective in reducing uveitis, it could also be approved for use in children with arthritis of all ages, in a range of doses.
JIA is a form of inflammatory joint disease that affects around 15,000 children and teenagers between the ages of six months and 17 years.
Fourteen paediatric rheumatology centres in the UK will start recruiting youngsters this summer. Youngsters will stay on the treatment for at least a year.
“If this trial shows that adalimumb is effective, it would be great news for children with JIA whose eyes are affected – children like Clare,” says Dr Ramanan. “Clare was given adalimumab off-licence on account of the severity of her uveitis. This is the whole reason for doing the trial – we need to get the evidence for what is becoming routine clinical practice.”
Current treatments are steroid eye drops, and for those with severe disease, methotrexate, which is a standard treatment for inflammatory forms of arthritis. Neither is a cure, but both relieve symptoms to some extent.
The trial will test the effectiveness of giving adalimumab by injection every two weeks, with methotrexate – which is known to be effective in treating the symptoms of inflammatory arthritis –compared to methotrexate on its own. Earlier, small studies have shown that children taking adalimumab have shown significant improvement in their uveitis.
Clare is pictured doing the Noddy Walk for the Portishead branch of Arthritis Research UK at the age of four. "More people should know about what it's like to have a child with arthritis and how it affects the whole family," says Vicki.
"Until Clare was diagnosed with it I had no idea that children could get arthritis, and a lot of people still think that. When people dismiss arthritis as not serious it makes me angry. People trivialise arthritis but it's not trivial. They haven't been in the house when Clare has been screaming in pain in the night. Arthritis emotionally cripples you."
• Arthritis Research UK’s funding for the uveitis trial has been made possible by the generosity of a major private donor.