Juvenile Idiopathic Arthritis: Clinical Guideline for Diagnosis and Management

Juvenile Idiopathic Arthritis: Clinical Guideline for Diagnosis and Management


Hello, I’m Geraldine Moses. Welcome to this program
on juvenile idiopathic arthritis – clinical guideline for diagnosis
and management. The program is one of four in a new
musculoskeletal guideline series. These guidelines,
for general practitioners and other
primary health-care professionals, have been developed
by the Royal Australian College of General Practitioners and approved by the National Health
and Medical Research Council. Because of the relatively low prevalence
of JIA in the general population, GPs often have little experience
with this diagnosis. The guidelines have developed,
therefore, to fill that gap, and reflect the current evidence-based
approach to managing children with JIA. This program will cover
the recommendations for the early and accurate diagnosis
and multidisciplinary management of JIA in the primary-care setting. You’ll find a number of useful resources
available on the Rural Health Education
Foundation’s website. That’s: Now let’s meet our panel. Firstly, Dr Morton Rawlin.
He’s a general practitioner in Victoria and has worked in rural
and metropolitan practice. He is vice-president of the RACGP. He was a member of the JIA working group for the development of this clinical
guideline we’ll talk about tonight. – Welcome, Morton.
– Thank you. Dr Jeffrey Chaitow
is a paediatric rheumatologist and head
of the Department of Rheumatology at The Children’s Hospital, Westmead
in New South Wales. He practises both through the hospital
and in private practice on Sydney’s North Shore, and has recently commenced
a paediatric rheumatology clinic at the John Hunter Hospital
in Newcastle. He was also a member
of the JIA working group for the development
of the clinical guidelines. – Welcome Jeff.
– Thanks. Penny Barram
is an occupational therapist who’s been working in paediatrics
for nearly 30 years. She currently works with both
the Queensland Education Department and MontroseAccess
as part of the outreach team to the Darling Downs region
in south-western Queensland. She works for special-needs children,
including those with JIA. – Welcome, Penny.
– Thank you. Karen Filocamo is the chief executive
of Arthritis New South Wales. Karen has nearly 20 years’ experience
working in senior management within the health system,
in areas including health promotion, community participation
and palliative care. – Welcome, Karen.
– Thank you. And finally, Kerry West. She’s currently deputy head
physiotherapist of The Children’s Hospital at Westmead. She has extensive experience
working clinically as a paediatric physiotherapist,
especially with children with arthritis. Welcome to you all. – Thank you.
– Thank you. Let’s start the program
with our first case study, which involves two young people. The first is Priscilla. She’s aged 25.
The other is Peter. He’s aged 19. They both talk about their experiences
growing up with JIA. My name is Priscilla Richardson. I’m 25. I’ve had juvenile arthritis
since I was 18 months old. I’ve got arthritis in every bone
in my body. I have good days
where I can do nearly anything, then other days,
I’ll have really bad days where I’ll find it hard to walk around
or whatever. In the past, I’ve been really bad. I’ve had to get both my hips replaced
and both my knees. I just couldn’t take the pain anymore. I’m Peter Reid. I’m 19 years old. I first had problems with my hips
when I was about ten years old. The doctors didn’t know what it was. Each time I saw a different doctor,
they said something different – growing pains and other things. I went into hospital
because I wanted an answer. I was in there for about a month,
at Westmead Children’s. At first they thought it was
osteomyelitis. Then they found it was arthritis. It was first diagnosed in my left hip. A couple years later,
it was diagnosed in the right as well. I had a hard time with that. At school I got teased a lot ’cause my arthritis would change shapes
in my body. I developed scoliosis in my back,
and also developed a swayed back, which didn’t help at all. Doing PE for school, everyone got to
join in and do the activities, and I couldn’t do it. Therefore, I had to sit on the sideline,
and always felt left out. I found it very hard to make friends ’cause I was always getting teased
due to my arthritis. I was playing first-grade soccer
as a goalkeeper, then couldn’t keep up
with everyone else. So I was on the sidelines
for most of the games. It saddens you when you’re younger that you can’t keep up with everyone
or do what everyone else can. Some days, it’s hard just cutting
or holding cutlery or even cups or just doing the simplest things. Every day brings a new experience,
I guess. Young people with arthritis. Jeff, if we could go to you first,
how typical are these stories? They’re not so much typical in that
they are non-identical stories. Therefore,
they illustrate a spectrum or range of the severity of juvenile arthritis. As we’re discussing,
and as is listed in the guidelines, there are different types
of juvenile arthritis, some of which are very severe,
like Priscilla’s, in which they start as young children,
high fevers, systemically very unwell,
so-called Still’s disease, or the systemic-onset group. They may need intensive therapy,
fancy new drugs, immunosuppressive therapy
to maintain the disease. Then there is disease that might affect
one or two larger joints. They might start as an adolescent
like Peter did. They might have the HLA-B27 gene. Often the inflammation is
thought to be infection to start with. They’re thought to have had a septic hip
or sacroiliac joint. It becomes clear,
with persistence of their symptoms, with morning stiffness, that this is an inflammatory
and not an infective process. There’s a wide spectrum of the disease, which means we’ll be struggling
with definitions. For the benefit of our viewers,
can you define what is JIA? That’s been clearly defined,
so that part is easy. Juvenile, meaning that
you’re less than 16 years of age when you first get your disease. Idiopathic, that medicalised term
which means we don’t know the cause of it and it’s
not part of another disease process. And arthritis –
meaning you have inflamed joints. By definition,
a joint needs to be swollen, it needs to have limited range
of movement, and needs to have pain
when you move the joint. I notice you’re not saying,
juvenile rheumatoid arthritis, a term people often use. Perhaps we need to clear up
that terminology. Why is it not called juvenile
rheumatoid arthritis anymore? Historically, in 1977, when
a nomenclature group first got together, juvenile rheumatoid arthritis
was the term used, thinking that these were young children
who had rheumatoid arthritis. It was very clear then,
in fact it was clear 100 years before when Stills first described it, that these were not children
with an adult disease. These were juveniles who had arthritis. And only a small group of those
children, mostly adolescent girls, have positive-rheumatoid factor
and have an adult-type disease. The others are either
juvenile idiopathic arthritis, or JIA, which is the commonly used term, or there was a JCA,
which was juvenile chronic arthritis, which has been dispensed of as well. Still’s disease –
what’s the other terminology for that? George Frederick Still, who we think of as the grandfather
of paediatric rheumatology, 100 years previously had described
a group of children with high fevers, rash, very unwell, joint deformities. That’s the group we now call
systemic-onset juvenile arthritis. All this terminology,
is it outlined in the guidelines? It’s outlined in the guidelines. It’s available on many standard websites
and texts. GERALDINE: Terrific. JEFFREY: It’s more important
for targeting management and outcome measures than it is
for making the diagnosis. GERALDINE:
There’s a picture of the guidelines. It can be accessed
through many websites, particularly through the RACGP. More definitions – what’s the prevalence
of JIA in the community? The best data we’ve got is that somewhere between 1,000
and 4,000 children are affected. There’s been some local data
from Western Australia, but no registry to confirm that. Across the world,
it seems to be similar to the incidence of diabetes
in childhood. Is the prevalence in Australia
about the same as other countries? We think so,
the other Western countries. Is there predominance
with any ethnic groups? JEFFREY: There is.
We don’t have data on that. Even within immune inflammatory disease,
we know for instance that lupus has much more prevalence
amongst the Asian population, either living in Australia,
and from Asia. It’s more prevalent amongst the Negroes
in America, it’s more prevalent amongst
the Black Indigenous in Australia. There is quite a disparity
across cultural groups. And do we have any idea… Because it’s called
idiopathic arthritis, we don’t really know the cause,
but what’s the possible pathology that lies behind this autoimmune
inflammatory disease? There have been lots of postulates
in terms of having a fertile field or receptive genetic predisposition,
then something may trigger that. There have been theories about
infectious triggers, ’cause we know you can get
a reactive arthritis. We know that you can have
gastroenteritis and swollen joints afterwards. That tends to be this group
that are HLA-B27-positive. But the reality is,
we don’t know the cause of arthritis. Therefore there is no obvious genetic
link for the juvenile form of arthritis? There are some risk genetic profiles,
but there’s no very strong link. What would you say is the difference
between juvenile idiopathic arthritis and the adult version of the disease? Are they the same disease? No, they’re not,
except for that small sub-group, which are the children, adolescent
girls, positive-rheumatoid factor – what we would call
polyarticular seropositive disease. They’ve got rheumatoid factor, and they are a juvenile
who has rheumatoid arthritis. But for the vast majority,
the other 90%, 95%, they’re children with swollen,
inflamed joints, but not with an adult-type
disease process. If I could ask Karen,
you’re from Arthritis New South Wales and your organisation speaks to
a lot of people dealing with arthritis. Do you get people saying that
children don’t get arthritis? Definitely.
That’s one of our greatest battles. Everyone thinks of arthritis
as an older person’s condition. We’ve made a lot of effort
in the last 12 months to get the message out there
that children get arthritis too. Parents can be quite bewildered
when they’re told that their child’s diagnosis
is arthritis. Parents think
that’s an older person’s condition. ‘How can my child have arthritis?’ It can be scary for parents. They don’t know what the future holds
for their child. Now to our first case study.
Second case study, sorry. Jack is a 15-month-old infant whose mother has brought him to her GP in a country town. She says he’s not been himself for weeks and thinks he may have a virus because he has a fever. He’s not eating well, has lost weight, he has skin rashes, is not walking like he used to and appears to be in pain. Morton, if I could turn to you. You’re the GP that brings this child in. What would be running through your mind
as a possible diagnosis? There could be quite a few things
going on here – the infective viruses,
some of the other traumatic things. It appears to be an acute inflammatory
process going on with this child. You do need to think about juvenile
idiopathic arthritis in this setting. It’s not common, but if you don’t think
about it, you’re not going to find it. – That’s a tricky thing.
– Absolutely. – You turn to the guidelines.
– You do, and they are there. We have a photo of the algorithm
for diagnosis for JIA to show you what it might look like. What would say are
some of the first things you should do? I suppose it would be
examining the patient. MORTON: The classic things that you do
with any patient. You take a good history. You make sure you’re covering the bases. Then you do a full examination
of the patient, particularly the joints that are
appearing not to work particularly well. Looking for signs that might be
in the differential diagnosis. Is this child possibly of an Indigenous
background that might be exposed to, for instance, rheumatic fever? Or perhaps looking at
another viral cause. It’s been going three weeks.
It’s a little bit long. In terms of examination, what are
the things you particularly focus on? From a general practice’s point of view,
you need to look for synovitis and swelling of the joint,
if you can possibly do that. Look at their fever,
their systemic symptoms and just actually watch them walk
or do what they do. In a young child like this, ask Mum. They’re so young,
they might not be able to say, ‘Goodness, my joint feels stiff.’ Take Mum’s word for it. Being in rural practice, you often
see children when they’re well, when you’re first looking after them. This child has obviously changed. Are there other things
in the differential diagnosis that we need to think about – other autoimmune inflammatory conditions
as well as infections? Sure. There certainly is. It depends a little on the age groups
you’re looking at. In the guidelines, there is a good
section on differential diagnoses. The key thing is just to bear in mind
that JIA is a possibility. Are there any blood tests that you’d do? Having thought about
whether this might be the case, yeah, there are blood tests – a full blood count,
looking for information in the blood with an ESR and a C-reactive protein. If you do believe
there may be a possibility of a JIA, you need to look for
their rheumatoid factor and look at HLA-B27 as a marker. You may even do a plain X-ray
of their joints. It depends on what is available
in your locality. Do you have access to all
those blood tests in a rural location? Most of those blood tests
are pretty ubiquitous. If your suspicions are mounting
that it might be JIA, at what point do you refer
to a rheumatologist? If you truly believe that it is JIA, I’d certainly be on the phone
to my friendly neighbourhood paediatric rheumatologist,
and ask an opinion. Look to see whether there were other
tests that could be done in your area to save the patient travel, then with the whole package,
they see the paediatric rheumatologist. Do you have comments about
getting to you in a timely fashion? Always available. A couple of things. Infection, inflammation, a young child, unexplained fever, weight
loss – malignancy always comes into it. That’s a differential. Even in bigger kids presenting
with bone pain rather than joint pain, bear that in mind. Sometimes an X-ray would be helpful
along that line. If geographically, the paediatric
rheumatology practice or hospital is not accessible, the phone always
is or emails always are, or leaving messages
and the doctor getting back to you if they’re not in the practice
at the time. A five-minute chat may save the child
a lot of unnecessary investigations and may target where to go more quickly. Do people send you photos of the child to demonstrate the swelling
of the joints? That’s happening increasingly, not so
much for swelling, but unusual rashes, vascular leak, inflammatory rashes,
easy to email pictures. Pictures of the child will often
jolt you into action more quickly, saying, ‘This kid needs to be seen
very soon,’ or, ‘Nothing about that looks worrying.
Let’s get this other test done first.’ We had a question emailed from
a clinician in Far North Queensland, who was concerned about misdiagnosis
of acute rheumatic fever by doctors thinking it was JIA. These children with this infection were
being treated with drugs for arthritis, then getting worse. Are there any tests or examinations
that they can do to be more clear on the diagnosis,
to exclude acute rheumatic fever? It’s not so much tests,
it’s clinical pictures and patterns. In our population groups, the incidence
and prevalence of these diseases is really quite disparate. Australian Caucasian children getting acute rheumatic fever,
extremely uncommon. Indigenous population,
a much more common scenario. Age groups – rheumatic fever,
often a school-type age group, five to ten sort of age. The very young child – high fever and a rash at 15 months,
not going to be rheumatic fever. In rheumatic fever,
the joint swelling moves around, the so-called flitting arthritis. It may be a knee for a few days
and an elbow a few days later. Inflammatory arthritis
tends to be the same joint staying inflamed for some time
until they’re treated. Fever patterns, the duration of fever
and obviously cardiac involvement. If you go right back
to the Duckett Jones criteria, in which he described the criteria
for rheumatic fever, there are major and minor criteria. Cardiac involvement
as in valvular disease is not really a feature
of systemic-onset juvenile arthritis, although pericardial effusions
flit around the heart, and pleural effusions
may be a feature of that. Without necessarily getting antibodies
to streptococcus, the ASOT, you can tell from the clinical picture
in most cases. What would you say for GPs
watching this program is the most common presentation of JIA
in general practice? What sort of age, gender? Going through the seven subgroups,
which I don’t necessarily have to here, the group that stands out most commonly
numerically is the so-called oligoarthritis,
meaning a few joints, and by definition, four or fewer joints. They typically affect young girls,
meaning two-, three-, four-year-olds. I think of it as, the knee at three. It’s a two- or three-year-old girl
with a swollen knee, morning stiffness, limping, otherwise well in herself. That’s about 40% of the new
presentations of juvenile arthritis in most big series. These subtypes of JIA,
are they outlined in the guidelines? They are listed in the guidelines. In fact, because they differ
in their severity and presentation, there are management guidelines
particular to the subtypes as well. Are they relevant in general practice
for professionals or even for patients to know that
they’re a particular kind of JIA? They’re relevant to give prognosis
to the family when you first come. You can tell them, ‘This is severe. You’ll end up on much stronger
disease-modifying drugs.’ Or, ‘This is single-joint involvement. I’ll speak to my colleague,
but often I find a simple steroid joint injection
will settle it down very quickly, and the long-term is good.’ Right, now, I’d like to ask Karen
about the role of family in this. Say we’ve got a case
like this little infant. It’s going to drive the parents crazy. They’re going to be so worried. Hopefully they’ll know
to get onto an organisation like yours. What are things people ask about,
and what do parents need? Parents need an early diagnosis.
That’s the most important thing. I can’t emphasise that enough
because often that will mark the pattern for how the child actually progresses
with their condition. Once they have an early diagnosis
and they know it is a form of arthritis, then they can contact an arthritis
organisation in their State. There is one in every State
of Australia, including the Northern Territory. We can put them in touch with
other parents in a similar situation. We know that parents want to know
what the future is, what’s going to be the development
for their child. We can put them in touch with parents
and other children. And the other thing is
you mentioned families. It’s also important that the siblings
of the child with arthritis understand what’s going on
because it affects the whole family. We offer education, awareness raising. We offer a camp,
which we’ll talk about later. We can probably take the mystery away
from what the family are going through. I’d like to go to Penny now. Maybe you can tell us what is the role
of multidisciplinary care. The guidelines emphasise that that’s
something we’ve got to guide people to where it wasn’t so much done
in the past. GERALDINE: What’s the importance
of multidisciplinary care? From the OT’s point of view,
it’s about how they’re coping at home with everyday life and at school
and at problem-solving, so they can lead a more independent life
and participate in school and be generally able to participate
in everything that’s going on and not feel left out,
like they did in the movie. What would be some of the things
you’d help parents with? With this young child, would you
help families like this, as an OT? Yes, I’d come in and see
what the major problems were, the real ones and not the imagined ones. We’d try and help them
to be really independent – giving them bigger tools to work with, though obviously at 15 months,
we wouldn’t start with pens and pencils. We’d see that they can move around
and play with normal things and generally run around. Did you want to elaborate on
what you meant by imagined problems? Sometimes when people are told
they have arthritis, they feel like they stiffen up
or they can’t move. They tend to overprotect them instead of letting them join in
or just be normal and run around. Perhaps at that point,
we could ask Kerry, clearly there’s no role for bed rest in
the management of paediatric arthritis. You’re a physio. What would you say to the parents
of young children with JIA? Keeping moving
is the most important thing. If it’s too painful to move on land, we
might use water therapy, hydrotherapy, as a nice environment
where the child can be moving and not in pain
from the joint inflammation. As long as diagnosis is early and they’ve started quite aggressive
medical management, more and more with these young ones, we’re seeing there’s less of a role
for physio and OT because they’re well managed medically. More and more, I’m not seeing them at
young ages because they’re well managed. Jeff, if we could go back to you,
in the past with inflammatory arthritis, it was all about
getting people out of pain. What would you say we’re looking at now
with goals of therapy? The goal of therapy now
is disease remission. What we’re looking for
is not fewer active joints. We’re looking at no active joints. One needs to titrate that against potential side effects
of aggressive therapy. If the patient is not
tolerating their drugs or you’ve got to what you think
is the maximum level of drugs, you’re still using pain relief
to dampen the inflammation. But overall,
if you take the active joints and inject them
with a long-acting steroid, which we do under intravenous sedation, that settles inflammation and swelling
in that joint. That’s why Kerry said
she’s almost out of a job, because those joints
no longer have contractions. They now move freely
because the swelling has settled down. She can work to build up the muscles. At least she’s got a joint
that moves normally, and doesn’t need casting
and the things we used to do. Then we have the non-steroidal
anti-inflammatory drugs, which will have been initiated
by the general practitioner when they first see the patient,
to control pain and swelling. They do the best job they can, but that’s not good enough
at suppressing the disease. Depending on the disease,
we step up to disease-modifying drugs, of which methotrexate
is pretty much the standard in paediatric rheumatology
at the moment. Salazopyrin or sulfasalazine
are used as well. Then we have the new,
perhaps not wonder drugs but the biologic drugs,
which are specifically targeted against inflammatory molecules, and have been very effective,
although expensive, at switching off active disease, and not ending up with children
like Priscilla – stunted growth, joint deformities
and great disability. We’ll elaborate on the drugs
in a moment, but before we leave the discussion
about multidisciplinary care, we have an occupational therapist,
a physiotherapist and the support group. Who’s in charge of making sure that
patients with JIA and their families are referred
to these health professionals, Jeff? The original source of referral
is the general practitioner. The patients themselves
are taking an active role, contacting support groups, other parents and arthritis foundation-type
organisations. They may be driving and say,
‘Do I need to see a physiotherapist?’ Or, have his eyes been checked?
They may go through their own checklist. Overall, there is a team,
but someone needs to guide the team. I would say here that these type of patients
are really very good for a team-care arrangement plan, GP management plan-type activities
in general practice. Um, and… Certainly… I think the other thing to stress
from a general practice point of view, don’t forget good old paracetamol
on a regular basis in the acute phases of this. It can really make a difference. We’ll come back to the drugs. Would you say the treatment plan,
is that managed by you? Who devises it and who looks after it? I think that the GP has overall sight
of what’s happening with this patient. There are a lot of people
who are going to be involved. It does need some coordination. It depends a little on the severity
of the disease as to what diseases are invol…
what joints are involved. As to who’s working at what, if the GP is seeing the patient
and the family all the time for other things as well, they’re in a perfect position
to coordinate and make sure that things aren’t missed. Given that the infant in our case
had a rash and it’s suggestive
of perhaps more severe disease, is it the GP’s responsibility
to ensure regular monitoring of the systemic manifestations
of the arthritis? I think that… It’s a joint…
In these more severe cases, it’s joint responsibility
with the paediatric rheumatologist working very closely so that we both know
we’re on the same page. We have another case study now. A little girl, Imogen,
who’s five years old, she was diagnosed with
oligoarticular JIA when she was three. Her joint disease was well controlled
with local steroid injections to her left knee and right ankle
on two separate occasions. She has, however,
had troublesome uveitis detected
on a recent ophthalmological review. Jeff, the issue of uveitis. It’s such
a big deal with inflammatory arthritis. Can you tell me about
what needs to be done in this situation? Uveitis is something that is more
prevalent in certain of the subtypes. Essentially, any child newly diagnosed
with juvenile arthritis should go to an ophthalmologist and have
an initial slit-lamp eye examination. Some of them have
inflammatory eye disease at the time of their presentation. Then they’re stratified
according to risk as to how often they need
to see the ophthalmologist according to the risk
of the uveitis developing. Here, I’ll stress we’re talking about
low-grade, smouldering, asymptomatic uveitis. It’s not to say –
eyes normal, come back if they get red. This is not that disease. This is losing eyesight gradually
if you’re not right on top of things and watching for it. They may need to go three-monthly for
several years to the ophthalmologist, particularly this illustration –
the young girl, swollen knee easy-to-control joint disease. Her parents might think
nothing else is going to happen. They need to be reinforced –
regular visits to the eye doctor. This is outlined in the guidelines? Outlined in the guidelines
as to how often they should go, who they should see
and what the risk factors are. This little girl is very young. What about monitoring her bone density?
Is that important? It’s much more important
in children on steroids, which tend to be the more severe,
systemic-onset group. It’s important in anyone with
inflammatory disease who’s not mobile to think about bone density,
because they’re not moving around. The risk factors are –
high fevers and inflammation. That can make you osteoporotic. Being bedridden can do that. We’ve heard how
we want to get children up, mobile, in the pool and not bedridden. And cautiously titrating
their steroid dosage to make sure we’ve got more benefits
than side effects. Unless it’s severe disease, what sort of comorbidities
or complications would parents have to be monitoring
over the long term? The eye disease we’ve spoken about. If joint disease is not controlled, you
can sometimes get growth inequalities – one limb growing more than the other. Joint contractures, muscle wasting,
loss of appetite. Then not to forget
the social comorbidities that Karen mentioned –
they want to be part of the group. They want to be accepted by their peers. A lot of stress is placed on the family. Then, as you said, we’ll get into
the side effects of the drugs. With uveitis, if the five-year-old girl
is the kind of patient to have smouldering uveitis,
could it become acute? It’s not the worry
that it will become acute, it’s the worry that she will
progressively lose vision by the smouldering process if it’s not treated aggressively
with immunosuppression or steroid eye drops
to stop the inflammation. Acute uveitis that people may
be used to seeing in older people that may be B27-related
or so-called Reiter’s disease, acute eye inflammation, that’s OK, because they present
with a red, sore eye. They don’t have to get screened. They’ll come when they have symptoms.
That’s a different subtype. They might not even know
they’ve got inflammatory arthritis? They may present with uveitis, exactly. I’m not sure what sort of medications
Imogen would be on for her JIA. Initially,
when she saw her general practitioner, she would have gone onto a non-steroidal
anti-inflammatory drug like naproxen to try and control the inflammation. If that wasn’t working
after a few weeks, she’d be best treated with intra-articular long-acting steroid
into that joint to settle it. It’ll do that for six months or longer
after a single injection targeted properly
and given the right steroid. Then for the uveitis, she’d be on
specific anti-inflammatory eye drops. She wouldn’t need any other
disease-modifying drugs such as methotrexate or the biologicals. With respect to
these analgesic medications, would a general practitioner be
administering intra-articular steroids? I think it depends on the GP and their relationship
with the paediatric rheumatologist. There are GPs who do. I would say that in these sort of cases, most GPs would be doing it
in conjunction with the rheumatologist. Particularly in young children,
it’s a painful procedure. Often,
it needs to be done in an atmosphere where they can have some analgesia or perhaps even a neuroleptanaesthesia
occasionally in children to reduce the stress of the procedures. I’m sure it hurts. We’re talking a baseline paracetamol. The guidelines would emphasise that
young children under 10 or 12 should have intravenous sedation,
anaesthesia, especially since it’s likely to be
a repetitive process and you want to stay friends. How frequently in a year would a paediatric patient be allowed
to have steroid injections? The guidelines are not
entirely specific about that because it’s left up
to the treating physician. In general,
we would say any single joint could be treated two or three times
in a year. If there were multiple joints flaring
at different times, that doesn’t matter. You can do the other joints
at other times. Because you’ve had your knee injected doesn’t mean you can’t have your wrist
done a month later. Imogen is five
in the original case scenario. What would be the likely scenario
when she’s 12? Imogen is in
a relatively good prognostic group from the joint point of view. I like to say it’s a disease that
licks the joints but bites the eyes. The joints, one can control easily. She’ll be running around
and going to Camp Footloose and playing netball and climbing rocks
and doing 12-year-old things. The eye disease may be the driver
of her morbidity because sometimes that needs
immunosuppressive therapy if that’s not controlled. She may flare
during her later childhood. I’ve been in this long enough
to see the 3- and 4- and 5-year-olds turn 14 and 15 and present again
with a swollen knee or wrist. They might ring, or their parents, and
say it’s come back several years later. But it still has a good outcome. You treat it again,
and it becomes controlled again with intra-articular steroids. Would you say to the family that this is going to be a waxing
and waning condition over the years? But a very treatable one, yes. In terms of treatment, we’ve talked
about baseline paracetamol. The drug we usually move to next – and it’s in the guidelines
and the algorithm for drug treatment – is non-steroidal
anti-inflammatory drugs. What are the important clinical points
our viewers need to know about NSAIDs? Morton,
would you like to talk about that? NSAIDs are often used
in general practice. They’re now available over the counter
at the pharmacy. I think you have to guide the patient. It depends on their pain
and the inflammation of their joints. In the guidelines, it appears that naproxen
has the most appropriate evidence at this point in time,
in the licence for it. In terms of NSAIDs, we need to be aware
that it can give stomach problems, it can give gastrointestinal
side effects. Be aware. Generally speaking,
they’re relatively safe drugs. Are COX-2 antagonists used in children? At the moment, there is no evidence,
and they’re certainly not licensed, as far as I’m aware, for use in JIA, but that’s not to say that into
the future, that evidence may come. Jeff, I believe you use other NSAIDs
besides naproxen. Is that correct? A lot of it
comes down to compliance issues. For very young kids, ibuprofen and
naproxen are the only two available as suspensions,
although compound pharmacists will make anything into a suspension. For ease of use,
there’s a soluble piroxicam, which can be dissolved in water
and given on a once-daily basis. That often helps compliance. Anything given 12-hourly is more likely
to be given three times a day, in which the parents have to administer
a school dosage. Diclofenac, or Voltaren, would also be
used with some frequency. What about topical NSAIDs? – Very little data.
– Not helpful. Not helpful? Helpful because you’re massaging
the sore limb, but you may as well be using
sorbolene cream to massage a sore limb. You don’t get the penetration into
the joint that people believe you do. It doesn’t have an anti-inflammatory
effect on the joint. Do we worry about the risks
that were associated with Vioxx and the reason
it was taken off the market? No, because
that wasn’t a paediatric risk. It was adults with hypertension. Children suffered by withdrawal
of a good drug. We miss it very much. Marisa Pilla from Queensland asks, ‘Is there any decrease
in life expectancy in children started early on therapy?’
Jeff? I presume she means, by controlling
the disease, do they live a normal life rather than,
is the therapy having an adverse effect? GERALDINE: I hope so.
– I’ll answer both. If her question historically related
to children with severe and untreated disease
in the time of George Frederick Still, there was a significant mortality
from sepsis, from being bedridden, from having bedsores,
from all the things we can now prevent, even relating to osteoporosis
and crush fractures. If she’s talking about, does the therapy
have long-term effects, the belief in that is no, although these new biologic drugs have
only been around ten years in children. we’re still following them. When I talk to parents, I expect
the children to live a normal life, get married, have children,
if that’s still normal. A normal adult life expectancy. We also have a question from a nurse
in New South Wales – ‘Is there any connection
with a female infant being treated for a clicky hip
to later risk of developing JIA?’ – No.
– Good. I guess it’s more orthopaedic. I think what she’s talking about is if you have a misdiagnosis
of a dislocated hip, you end up with osteoarthritis in a hip that’s not symmetric
and not properly formed. Different condition
from inflammatory arthritis. We also have a health professional
in North Queensland asking, ‘Is there any connection
between Ross River fever and JIA?’ Ross River fever virus will cause
a viral-induced arthritis. Historically, when we look at serum
of children who have juvenile arthritis, there are some viruses that come out as
more common in the general population, included parvovirus B19
is more common in the oligo group. In the main,
if you have Ross River virus, you have a much more transient arthritis
that should go. Given that JIA is potentially
a lifelong condition, a chronic disease
you have to think about forever, isn’t there an absence
of any known infection in JIA? I think they’re talking about
an infectious trigger, then the disease persisting.
That may happen. It’s important to say that Ross River
is in the differential diagnosis of an acute child with painful joints. It’s something that
you may have to rule out to come to the diagnosis of JIA. – Like acute rheumatic fever?
– Correct. Let’s talk about some non-
pharmacological interventions for JIA which includes going to camp. Arthritis New South Wales runs
a terrific camp called Camp Footloose. It’s an annual camp run for children
between the ages of 8 and 18 with all forms of arthritis. This case study highlights
many guideline recommendations in terms of support, education
and physical activity. The kids here understand what it’s like
to have arthritis. It’s really good because if you say
you’re sore, they won’t argue with you. Talking to kids about it,
they know what’s happening. It’s just a great place. Camp Footloose is a camp for children
aged between 8 and 18 with juvenile idiopathic arthritis. It’s an opportunity for them
to come to many of the same spots they come to for school camps, but
with a much more supported atmosphere. Instead of having 2 teachers
to 40 students, we’ve got one leader
to three campers here. They’ve got more opportunity
to do those activities because they’ve got that support. The student leaders
are students at university studying physiotherapy
or occupational therapy. We also have peer leaders –
kids that have come to camp previously and got so much out of it
that they want to keep coming back and give something back because it’s
been a huge difference in their lives. Because I was a child here myself,
I know what the kids are going through. I’ve been through the experience.
I’m still going through it. If they have a problem, sometimes they don’t feel comfortable
going up to the leaders, who might not have arthritis. They find it easier to talk to someone
who’s going through the same situation than someone who’s not. The aims of Camp Footloose
are to promote independence, to increase the child’s awareness
of arthritis and look at it in a more positive light. The aim is to also reduce isolation, as well an opportunity for them
to push their limits. The first camp I went to was pretty much
a brand-new experience for me. Going on school camps,
like, a rock-climbing wall, I was like, ‘No way I can do that.’ Here, they’re supportive.
It doesn’t matter if you get to the top. Everyone’s behind you, and it doesn’t
matter what everyone else does. It’s what you can do yourself. The camp made an effect on me,
achieving my personal best. That’s the best part about it. How do you reckon you’re going to go? – Bad.
– No, you’re going to go good! As long as you try,
you’re doing good already. Pull yourself up! Come on, Amy! The yellow one above it! Ready? 1, 2, 3. You’re not going anywhere yet. You’re going too fast. You’re right.
We’ll take it at your pace. We’ve got you, we’ve got you. It’s really good. You’ve got a lot of assistance
even though you’re afraid to do it. You’ve just got to get in there
and jump in. I feel pretty proud. Come on, Amy, you can do it! Physical activity is very important, but more important is the balance
between rest and activity. With juvenile arthritis,
about 70% to 80% will grow out of it when they reach adulthood, but what they won’t grow out of
is any disability they’ve come across. If they’re not moving
and keeping the joints active, then that can reduce their ability, and in the future,
that’s where the disability comes from. Today, I was helping the kids do craft. The reason we do the craft activity is so that the kids
who are having a bad day, they have the opportunity to come up
and get a part of something else that may not necessarily be so strenuous
on their joints. You have the pain, the stiffness. On top of that is fatigue, which, to my mind, is really a disease
in and of itself. It’s not like,
‘I haven’t slept, I’m tired.’ It’s all-encompassing. You can’t lift your arm,
you’re so fatigued. That makes it difficult,
and the fact that it is unpredictable – that one day they’ll be fine,
the next day they won’t, which means people don’t believe them. For those in regional areas
that don’t get as much access, schools, health-care professionals
aren’t as aware of it. They’re not getting the services that
those in city areas are getting. Are you able to pull back? Most people,
when you talk about juvenile arthritis, they say,
‘No, children don’t get arthritis.’ The biggest thing is the ignorance, and having to explain themselves,
not once, not twice, but every single time that it comes up. I thought I was the only one,
at a young age, who had it. I came to camp,
and I met probably 60 people my own age or even younger who have it. When I went back to school after camp, I had more self-esteem
and I could talk to people more. I found it a bit easier
to actually try and make friends. Yeah, I think it was just
the whole communication. I wasn’t judging myself so much
after that. I got a Christmas card from a parent whose child came for the first time
last year. She was the child that –
‘I can’t do that because it will hurt. I can’t do this. It’ll hurt.’ Now she’s the one that says,
‘OK, this is going to hurt. Mum, can you put an ice pack
in the freezer? I’m giving it a go.’ The mother was saying I had delivered
home to her a completely different child who had gone from being reserved,
didn’t want to talk about her disability to being out there and doing things
and giving everything a go. When I first came, I was very down,
very sad, having to give up soccer, which was one
of my favourite things at the time. Coming here builds you up,
makes you feel on top of the world after achieving all the activities. The leaders and kids
getting behind you and supporting you makes you feel a lot happier
and better as a person. Come on, Amy! (Children cheer) What a wonderful camp to go to. Karen, tell us, how do we get to go
to Camp Footloose? I get to go! It’s wonderful. It’s one of the more enjoyable parts
of my job. You have to be a child to go,
unfortunately, with diagnosed arthritis,
and to be aged between 8 and 18. Basically the activities are suited
for that age group. The benefit you have seen
in that wonderful footage. Priscilla summed it up when she said,
a child with juvenile arthritis can feel like the odd one out at school, but at Camp Footloose,
they all have some form of arthritis. The activities are paced
to suit the child. No-one’s rushed. The kids are quite happy, when
they’re climbing up the rope, to wait. Everyone knows
they’ve got to go at their own pace. There’s also craft activities,
as we saw. If people watching do have children
with juvenile arthritis, they just need to contact our office and
we can pass them on to the other States. – I can give you a 1800 number.
GERALDINE: That’d be great. 1800 011 041. If they contact us in New South Wales,
we’ll pass on the details. Children come from all over Australia
to that particular camp. GERALDINE: How long does the camp last?
– Just under a week. We have between 40 and 50 children. GERALDINE: Where do you hold it?
– We change the location every year because some of the children are
repeat children and it can get boring if you go back to the same place. That one was Dubbo, which was exciting.
The facilities were great. This year we’re going to a place called
Milson Island on the Hawkesbury River. Do parents go? Unfortunately for other parents, it is only for New South Wales parents,
the sessions that we run. If parents want to come
from inter-State, they could attend. We run an education session for parents,
usually at the beginning of camp, often with a rheumatologist
and other allied health staff, who do an education
and awareness session for the parents while the children are
loaded into the buses, and off they go. ‘Cause it’s a nice diversion for them,
too, rather than than saying goodbye
to Mum and Dad if it’s the first time
they’ve been at camp. They get a quick goodbye,
then whisked onto the bus, then Mum and Dad participate
in an education session. Do you have any therapists attending
like Kerry and Penny, OTs, physios, at the camp? It varies each year.
Sometimes there will be therapists. We always have an on-site nurse. As we’ve heard,
there’s a lot of medications involved with juvenile arthritis. Medications are always administered
by a registered nurse. In terms of other staff,
it varies from year to year. Kerry, if we could go back to you. We haven’t heard a lot about the role
of exercise, but clearly it’s an important part of the multidisciplinary care
of the JIA patient. Can you tell us a bit about what you do
for paediatric patients? As far as exercise, wherever possible, we want children to join in with
regular physical activity and sport. Sometimes there’s pain and swelling
in the joints, and it’s difficult. My role often will be helping children
find a sport they can do, modifying sports sometimes. Lighter bats and balls, for example,
can be used if they’ve got sore wrists. It’s looking at how they can be active,
how they can join in at school, be part of everyday activity, modifying
where necessary to allow that to happen. Do you find the children understand
the role of fatigue and how it might impact
on their ability to exercise? That is hard. We work with schools
because often at school, it’s a problem. It’s a big day to get through at school. So working with schools
so they understand that sometimes the children
will have to rest. They may only be able
to play half a game of sport, or even during class time,
they might need rest periods. Mentioning schools, I might take that
to ask Penny a question about teachers and educating them. The people you work with,
MontroseAccess, put out this terrific guideline booklet
for therapists, but I believe there’s also education
for teachers. People can access the services
through schools. Is that right? Yes. In the country areas
or even in the cities in Queensland, they have therapists
working in the schools. They can ask for therapists
to go and see children. They don’t do home visiting,
they only see them in the schools. It has to be school-relative. If there aren’t any therapists, there are sometimes therapists
in hospitals in rural areas. Often they’ll contact Montrose Access
if we can go up and consult with them if they don’t feel happy that
they’ve got enough information or if they don’t know enough about it. We can go out and visit country areas. What sort of benefit would you provide
to a school-aged JIA patient, things like the pens you have. Would you like to show us? We suggest fatter tools
for some of the students so it’s easier for them to hold,
to use when they’re at school. There’s triangular biros
that push on and off. These are just pencils.
I forget what their name is. These are other ones
with the rubber at the end. These are just fatter and softer. These are biros with the same sort
of adapting things. Are there any other problems you would
see frequently in school-aged children? You mentioned not being able
to get clothes on. Many children need these sort of things. When children go swimming,
some children with arthritis have difficulty changing their clothes
independently. They’ll often need help on ideas
on how to do it more independently. GERALDINE: What are those ideas? Sitting down to get dressed
and undressed, wearing clothes
that are a bit more loose. If they’ve got sore shoulders
and opening-down-the-front clothes, they can just drop off. They might need help to put them on
when their shoulders are sore. Not using T-shirts necessarily, ’cause that’s hard for some children
to put their arms up and through. Some children, if the uniform
is a T-shirt, if the hips are all right, they have tricks where they can go down and put their hands through
and pull it up that way. There are tricks you can do. Some of it is not knowing names,
or ways to access. The major children’s teaching hospitals
in all the States have at least the name of a paediatric rheumatologist
either on-site or available to be contacted
via the hospital. The various arthritis associations, including the Australian
Rheumatology Association, has a find-a-rheumatologist website
in which you can go ‘paediatric rheumatologist’, and it will find someone
with paediatric expertise. Rheumatologist is a big word. Perhaps people don’t know that’s
the kind of doctor they need to see. I wonder whether
the arthritis associations in each State would play a role there too.
Karen, do you? We couldn’t recommend
a particular specialist, but we’ll steer people
in the right direction about where they can find out
that advice. Parents can be groping in the dark
trying to find information. We’ll either speak to them
or we’ll send them material, whatever meets their needs, but we
won’t talk about specific doctors. Let’s talk about long-term management
and support of a JIA patient. For that, we have a case study of Amy, who is a 17-year-old girl living on a rural property 30km out of a town with her parents, two brothers and a sister. She was diagnosed with polyarticular, RhF-positive JIA at the age of 11. Her movement and physical ability are very restricted. She attends school, but spends a lot of time avoiding physical activity and school excursions. Amy needs assistance with most activities, and is due to have a knee replacement. Penny, do you see a role
in long-term management for an OT like yourself? At home? GERALDINE:
Yes, looking after a patient like Amy. Yes, we’d go in and problem-solve
about what her issues are at home and how she can get around them and
to keep her as independent as possible. GERALDINE: Probably forever, isn’t it?
– Yeah. Kerry, as a physio, what sort of things
would you do for Amy? Try to maximise her mobility
as much as possible. Even though a child with severe
deformity may need a wheelchair, trying to make sure that she is still
able to to get out of the wheelchair and walk as much as possible. If she’s due for the knee replacement,
finding a good local physio, because she’s going to need to do a lot
of work after the knee replacement. There will be times
when there will be more need for therapy and then times when she’s going to be
getting on with her everyday life. Karen, in terms of family
and family support, what do you see are the issues with a
17-year-old with a condition like this? What do they ask at this stage? All sorts of things, because they’re also going through
many other issues at that age. They’ll want to know about their
prognosis, how they’re going to develop. They will have lots of questions
that any normal adolescent will have. Sometimes these questions come up
at Camp Footloose. We will have trained people there
to try and answer these. They’re not always arthritis-specific. They might be – ‘Will I be able
to to do this, this and this even though I have juvenile arthritis?’ We’re there to offer support
and information to parents, who might ask the same questions. They might know
it’s bothering their child. They might ask on the child’s behalf. We’re there to reassure,
put them in touch with other parents. Whatever it is they need,
we’ll try and assist. Another issue with her is, she’s on that transition
between being a child and an adult. We have a question from
an allied health professional and mum, who’s asked whether steroid medication
would have an impact on the reproductive health
of teenage females. Jeff? A question that’s frequently asked
of adolescent females, particularly. The drugs that are used in juvenile
arthritis, including methotrexate as an immunosuppressive
and steroids, the long-term effects, the data is very reassuring,
particularly methotrexate. The advice is to stop the methotrexate
before you’re considering conceiving for at least three cycles beforehand, and also for male partners
to not be taking methotrexate at the time of conception. Listening to those cautionary words has resulted in
completely normal pregnancies not very different
from the population incidence in young women
with juvenile-onset arthritis who have taken methotrexate for years,
they conceive healthy babies. You can reassure that mother that the prime aim
is to get good disease control, get a healthy young adult,
then her chance of conceiving is better. Methotrexate is much better tolerated
than it looks on paper. – Absolutely.
– It’s not a very pretty drug on paper. We’re tarred with the oncology brush, where it’s used in high doses
for chemotherapy. That’s very different
from rheumatologic doses, which are very spared of side effects
other than mouth ulcers and nausea, and those can be eliminated, almost,
by taking supplementary folic acid. That’s another question
from a pharmacist in Queensland – what is the dose of folic acid
in a child on methotrexate? The most commonly used dose
would be 5mg once a week, not given on the day
they take their methotrexate, but people would sometimes use
0.5mg or 1mg every day
other than the methotrexate day. Those regimens haven’t been head-to-head
studied, but both would be effective. We also have two questions
about complimentary medicines. This comes up
with long-term management. With chronic disease, people want
to explore alternative therapies. One question from Elizabeth in Victoria, asking about the use
of copper bracelets, and a question from a physio
in New South Wales about acupuncture. Perhaps I can ask Morton to comment
on the copper bracelets, given that the guidelines looked at
complementary medicines in general. We have to be clear that the guidelines
are looking at whether they’re going to modify
the disease process, and there’s absolutely no evidence
at this point in time that complementary therapies actually
alter the disease process. And I think copper is one of those. There isn’t a lot of scientific evidence
that can be reproduced and looked at in a systemic way. That’s not to say that into the future
there may be, but at the moment there isn’t. Do we have any information
on acupuncture for pain relief in JIA? I think the answer there is, again, for modification of the disease,
there isn’t, but acupuncture certainly does
have a role in pain management. There are good studies these days
that show it can be effective. But it doesn’t work for everybody. We need to talk about
how the face of JIA is changing now that we use modern medicines. These questions allude to that – that it’s not just about pain relief
anymore. We want to stop the disease process. That’s what these fancy
biological agents do. Jeff, could you talk about which
monoclonal anti-TNF drugs are available for use in children,
and how they’re working? The only TNF-alpha inhibitor approved
for juvenile arthritis – cytokine-targeted biologic molecules
that stop the inflammatory process – is etanercept,
and that has proven very effective. Prescribing is restricted
to paediatric rheumatologists because of the expense of the drug, and once children have failed
methotrexate or another disease-modifying drug
or if they can’t tolerate it. The other TNF-alpha inhibitors
like infliximab, or Remicade, is approved for Crohn’s disease,
where it controls the bowel disease and also helps arthritis, but it’s not primarily approved
for juvenile arthritis, in other words, it’s not funded
by the PBS for that condition. If they go on etanercept,
do they still stay on the methotrexate? Most of the data would say that
methotrexate and etanercept together are better than etanercept alone. Some patients having methotrexate
side effects may choose to stop it because of the nausea,
and be in remission on etanercept alone. But ideally,
both are used together at the start. Would you like to comment
on the long-term issues of side effects with these medicines,
methotrexate in particular? And monoclonal antibodies, what
side effects should people look for? Methotrexate – nausea, mouth ulcers. Monitoring liver function tests maybe
three or four times a year is adequate, and looking for anaemia
and low white-cell count, none of which have proved to be
a clinical problem, and being cautious with varicella. Children will get very serious varicella
if they’re on methotrexate, and it needs to be stopped. Ideally, they should be immunised
against it before starting it. TNF-alpha inhibitors – there has been
a cautionary warning from the FDA about an increased incidence
in Crohn’s children on infliximab getting a rare type of lymphoma
called hepatic lymphoma. That’s currently being looked at. At the moment it’s a black-box warning without strong statistical data
to support it. Do you think there is risk of malignancy
with the TNF inhibitors? There’s now a biologic registry
worldwide, and that data is being looked at
prospectively. If the risk is increased,
it’s not substantial. What about tuberculosis? Adults – mandatory because of occult
or hidden or quiescent TB – have TB testing. In fact, all children should be tested
for tuberculosis before starting TNF-alpha inhibitors. Although the incidence of occult TB
in childhood is very low in our population group. With your JIA patients, what’s your
approach to multidisciplinary care? – I follow the guidelines implicitly.
– (All laugh) Do you prescribe a physio
and prescribe an OT? There are some children
who have a quite mild disease where it’s easy to inject them,
and they run around normally and don’t need ancillary support. For children with more severe disease,
they need the physio, the OT. They may need a social worker,
they need support groups. They certainly need an ophthalmologist
to check them regularly, and they need their general practitioner
to be involved in all the interim care. We’ve covered a lot of ground tonight, so I wonder if I could ask each of you
for a take-home message. – Kerry?
– I get to go first? Keeping physically active, and being
able to participate in physical activity is my main goal. GERALDINE: No role for bed rest?
– Definitely not. Karen, your point? Early diagnosis
is the most important thing. Parents will be worried and anxious, so refer them to someone like us
who can calm them down. – Penny?
– Always aim for independence. Keep them as independent as possible
and to believe that they can be. GERALDINE: Jeff?
– Kids get arthritis too. Don’t attribute the swollen joint to
some minor trauma that no-one saw or some minuscule tear which doesn’t
really occur in two- or three-year-olds. Think of inflammatory disease
when the joints are stiff and swollen. GERALDINE: Morton? I’d say, make sure that you remember
that JIA does exist, and it does present in general practice. It’s a disease that you can markedly
change the outcome if you pick it up. Fantastic. I’d like to thank
the Department of Health and Ageing for making the program possible. Thanks also to you for taking the time
to attend and contribute. If you’re interested in obtaining
more information about issues raised in the program, there are a number of resources
available on the Rural Health Education Foundation
website. That’s: Don’t forget to register for CPD points. Please complete and send in
your evaluation forms. I’m Geraldine Moses. Goodnight. Captions by
Captioning & Subtitling International Funded by the Australian Government
Department of Families, Housing, Community Services
and Indigenous Affairs�

Leave a Reply

Your email address will not be published. Required fields are marked *