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There are approximately 1,200 children living with juvenile idiopathic arthritis (JIA) in Ireland. JIA is not just one disease, but consists of various subtypes. These include the oligoarticular (where one to four joints are affected) and polyarticular (where five or more joints are affected) forms of the disease. It is not known what causes JIA, but treatment options, especially with the advent of biologics, are improving and leading to better outcomes for patients.
The primary referral centre for JIA patients in Ireland is Our Lady’s Children’s Hospital, Crumlin. Dr Emma MacDermott, Consultant Rheumatologist, who has been based in the hospital for the past four years, told the Medical Independent (MI) that demand for the service has grown in recent years. As with so many parts of the health system, the service is finding it difficult to meet the demand due to a lack of staff and resourcing. There are only two consultant rheumatologists in the hospital, even though international benchmarks suggest five to six consultants would be more appropriate given Ireland’s patient population. Dr MacDermott also argued there is the need for more multidisciplinary team (MDT) appointments, due to their centrality in managing this patient cohort.
“The care of a child with juvenile idiopathic arthritis has many aspects,” according to Dr MacDermott.
“This disease affects their ability to mobilise, it affects their muscles and joints and, therefore, they require the input of physiotherapists to help with that. Occupational therapists, obviously, are also important if the children have limitations in their mobility and in their ability to participate in school. One aspect that is under-recognised in these children is the huge psychological impact having a chronic disease often has on them, especially one that is not visible to their peers and is little understood in the community. So these children carry a burden of psychological impact as well. So psychological support, and the support of their families is critical. These are chronic illnesses. The majority of our patients will not grow out of their disease, the majority of our patients will carry their disease, in some form, through into adult life, and it is important they are supported in their childhood to maximise their potential, both medically as well as psychosocially.”
Treatment of JIA mirrors the treatment provided to adult patients with arthritis. Dr MacDermott said a “stepwise” approach to treatment is encouraged. Non-steroidal anti-inflammatory drugs (NSAIDs), such as ibuprofen and naproxen, are used to reduce pain and swelling. The disease-modifying anti-rheumatic drug (DMARD) methotrexate is also a gold standard of treatment, and can be used in combination with NSAIDs. Intra-articular steroid injections are also frequently used as a first-line treatment to treat joint inflammation in JIA. One recent advance in the treatment of the disease, according to Dr MacDermott, has been the advent of biologic therapy.
“The biologics have changed the face of paediatric rheumatology to some extent because these medications are very effective in controlling disease and there is strong evidence to support their use,” Dr MacDermott said.
“Before biologics, the drugs we had to choose from were limited. If patients were unresponsive or not completely responsive to those medications, we were in the position where they were maintained as best we could with the use of steroids and the use of non-steroidals, both of which have side effects. Steroids especially have a significant side-effect profile. The fact that we now have a next line of treatment that is extremely effective means we are bringing more and more children into remission status, albeit on medication remission.”
Dr MacDermott also noted it is important to take account of the differences in treating children compared to adults.
“The important difference between paediatric and adult rheumatology is we are dealing with a body that is growing both physically and the immune system is also changing and growing in these patients,” she explained.
“We are very conscious of having a limited number of years of growth and that growth can be significantly affected by arthritis. With that in mind we are anxious to get the disease under control as quickly as possible and so we are very aggressive in our approach to paediatric arthritis for that reason.”
When a patient is put on any treatment regime it is important to keep a close eye on their progress.
“Here in Crumlin we are very lucky to have clinical nurse specialists who are very active in the education and ongoing monitoring of patients,” Dr MacDermott said.
‘The fact that we now have a next line of treatment that is extremely effective means we are bringing more and more children into remission status, albeit on medication remission’
“We also are very dependent on our community paediatricians and GPs who provide a lot of the day to day monitoring of our patients with respect to their blood work. In terms of response to treatment, we try to get our patients back very promptly after a medication change to assess their response to treatment. Because we are aggressive in our treatment approach, it is important to make rapid treatment changes if we are not seeing a response to bring the disease as rapidly under control as we can. With our patients who are on biologic agents, we have introduced a biologic screening clinic. While our patients continue to come to our general rheumatology and teen clinic, we try to rotate them through that biologic clinic so the additional screening that we like to have for all our patients on biologics is performed on a semi-annual basis.”
In terms of prognosis, Dr MacDermott said the aim is for remission, be that drug-free remission, or, as often is the case, remission achieved through medication continuation. In both cases, the consultant and MDT are looking for a situation where these patients are functioning in the community as independently as possible.
“The aim of the treatment is to get them performing academically and also psychosocially the same as their peers. That means they are participating in sporting events, that they are going out and socialising with their friends and they are getting through school with the same ups and downs as their peers who do not have arthritis. I think it is one of the nicest things we hear in our rheumatology clinics when patients come in and tell us they have been named ‘player of the year’ or been elected captain of one of their sporting teams, or are back in dance class. It is a testament to the MDT treatments the children are receiving, and the hard work of the children themselves, which allows them to get back out there and participate.”
Children with JIA can also have eye problems, as a result of inflammation. Dr MacDermott said it is important that consultants are aware of the potential of ocular complications arising from the disease.
“Obviously we work closely with all of our sub-specialist colleagues but the co-existence of inflammatory uveitis in patients with JIA is something we highlight,” she said.
“These patients should be followed regularly by ophthalmologists. Unfortunately, in much the same as there is a very limited paediatric rheumatology resource, there is also a very limited access to ophthalmology. Obviously we try to work within the system and to provide the best service for these patients.”
Transition to adult services
Planning the transition of adolescent patients into adult services is an often under-appreciated part of paediatric care. It is a stage of care, however, that the rheumatology department in Crumlin takes very seriously.
“We in Crumlin are very proud of our transition service and we have actually won an award for our transition process,” Dr MacDermott stated.
“I think it is increasingly recognised that a smooth, co-ordinated and conscious transition of patients from the paediatric to the adult world really impacts their long term health, their compliance and outcomes in general. We run a transition clinic so our teen patients begin in their very early teens, where we begin to treat them more independently of their patients. They come in independently and then the parents come and join in the consultation a little bit later. Obviously all decisions are made in a group setting, but it is just to empower the children a little bit more before they move onto adult services. We have a direct link with St Vincent’s Hospital, so there is a direct transition process there, and we are establishing similar pathways with some of the other Dublin hospitals and identifying adult rheumatologists with an interest in transition. But more broadly across the country, the transition process is less cohesive and that would be something we would love to improve upon with our adult colleagues.”
Dr MacDermott believes that the key in transitioning patients from paediatric to adult services is recognising the importance of the transition. “It should be realised that it is not a straightforward change,” she added.
“In paediatrics, there is a little bit more time [with the patient], and more support with respect to compliance issues, with respect to encouragement of children to take responsibility for their health. I think when they move directly to an adult setting they can be quite unprepared for the speed, the short duration of patient encounters, the need to be very able to discuss their disease and to have a good knowledge of their medications. In situations where a patient has had their disease for a very long time and left everything up their parents, it can be quite shocking to appear in the adult setting where the physicians are expecting a fair amount of input from the patient. We pride ourselves on our transition process and having the patients ready to deal with the slightly different approach in the adult setting.”
Dr MacDermott pointed to exciting research taking place in Crumlin regarding JIA patients with Down Syndrome. The research seeks to examine the increased risk and incidence of arthritis occurring among children with Down Syndrome. This is the largest study to date regarding Down’s arthropathy, and promises to yield new and informative results that will help to improve the provision of care and quality-of-life of children with Down’s arthropathy.
“We have developed the largest cohort of Down Syndrome patients in the world,” Dr MacDermott explained.
“We have helped define the kind of disease, the pattern of arthropathy seen in Down Syndrome and potential biomarkers for Down’s arthropathy will also be examined. This is very exciting work and it has actually changed our approach to patients with Down’s arthropathy. We have discovered that our patients with Down’s arthropathy are very sensitive to methotrexate intolerance and so this would be a group that we would move very quickly to using biologics on.”
In conclusion, Dr MacDermott stressed the importance of the juvenile arthritis service being supported with sufficient resources so it can continue its vital work in treating and researching this disease.
“More funding would allow us to hire more consultants, develop our transition services and regional links and continue ground-breaking research into the disease,” she said.