Best Practice & Research Clinical Rheumatology

Vol. 16, No. 3, pp. 333±345, 2002

doi:10.1053/berh.2002.0232, available online at http://www.idealibrary.com on

Reducing the impact of rheumatic diseases in

childhood

J. D. Akikusa MBBS, FRACP

Fellow in Rheumatology and General Paediatrics

R. C. Allen* MBBS, FRACP

Head, Department of Rheumatology

Department of Rheumatology, Royal Children's Hospital, Flemington Rd, Parkville, Melbourne 3052,
Australia

Reducing the impact of rheumatic diseases in childhood is the fundamental objective of every
member of the multi-disciplinary team involved in the care of a€ected children and families.
The means by which this objective may be achieved are broad and include the implementation
of a range of non-pharmacological therapies to address the e€ects of rheumatic diseases on the
physical development of the child. In addition, the treating team must be aware of the
psychosocial impact that these diseases may have and the ways in which this may be
minimized. This chapter is devoted to an examination of some of the non-pharmacological
issues that arise in the management of the commonest rheumatic disease found in children,
juvenile idiopathic arthritis (JIA). Aspects of physical rehabilitation, schooling, medication
compliance, pain management and family dynamics are discussed, as are interventions to
reduce the impact of this disease and its sequelae, utilizing, where possible, evidence-based
principles from the literature. Although speci®c issues applicable to children with arthritis will
be discussed, the broad principles of much of what follows applies to all of the rheumatic
diseases in childhood.

Key words: arthritis; impact; psychosocial; physical therapies.

The advent of disease-support groups and the Internet has meant that, compared with
20 years ago, paediatric rheumatologists today are caring for a better educated and
better informed group of parents of children with arthritis. These parents may have
di€erent expectations of therapy compared with previous times. The shortfall between
expected and actual treatment outcome may result in both parent and patient
dissatisfaction, which may reduce the child's sense of wellbeing and self-worth. This,
while signi®cant to the patient and family, may be too subtle to a€ect disease scores
derived by traditional methods. The dramatic rise in the use of complementary
medicines in the last decade in all age groups, including children, may re¯ect this

*Author for correspondence.

1521±6942/02/$ - see front matter *
c 2002 Published by Elsevier Science Ltd.
334 J. D. Akikusa and R. C. Allen

dissatisfaction; this is particularly ironic given the increasing emphasis placed on

evidence-based therapeutic decision making in current medical practice.
A useful model on which to base an approach to the management of chronic disease
is that of the `disablement process', originally formulated by Naji, and put into the
paediatric rheumatological perspective by van der Net et al.1 This model proposes
four distinct constructs ± acute pathology, impairment, functional limitation and
disability ± which behave as a continuum. Each component o€ers the potential for
intervention but other factors may in¯uence the outcome and therefore the patient's
overall quality of life. In the setting of paediatric rheumatology these `cofactors' may
include intrinsic qualities such as coping skills and behaviour, extrinsic elements such
as access to rehabilitation and medical services, and risk factors such as low family
resourcefulness. Newer therapeutic agents, by having a greater impact on the basic
pathology in JIA than previous ones, have the potential to diminish the subsequent
stages of disablement. In this setting the e€ect of interventions directed at the
cofactors in the disablement process may take on a key role in minimizing the impact
that these diseases have on the quality of life of a€ected children. The current
signi®cant challenge for paediatric rheumatologists is to develop means to measure and
address more consistently the issues that the patients themselves identify as
contributing to the burden of their disease.2

MEASURING AND PREDICTING DISEASE OUTCOME

A common misconception faced by paediatric rheumatologists is the anticipation among

patients' families, and probably some primary care physicians, that puberty heralds
some magical therapeutic milestone that will result in resolution of in¯ammatory joint
disease. However, to the contrary, long-term follow-up studies of tertiary-level clinic
populations such as that by Wallace and Levinson, and population-based studies such as
that by Gare and Fasth, report that between a third and a half of children/adolescents
with juvenile arthritis continue into adulthood with active joint disease.3,4
A ®rst step in reducing the impact of rheumatic diseases is identifying patients at risk
of poor outcome who may be suitable candidates for early aggressive therapy. The
poorer outcome in rheumatoid-factor-positive polyarticular disease has long been
recognized, particularly if early erosions are noted. Long-term follow-up studies,
however, indicate that a polyarticular course, irrespective of onset type, predicts
signi®cant sequelae in adulthood. Identifying patients likely to follow such a course is
therefore important. Guillaume et al, in a study of 207 patients with oligoarticular
disease onset, found that 50% had progressed to a polyarticular course after 6 years.5 The
strongest predictors for progression were involvement at onset of two to four joints,
relative risk (RR) 2.6, upper limb distribution (RR 2.6) and an elevated ESR 4 20 mm/
hour (RR 1.7), increasing to an RR of 4.4 if 4 100 mm/hour. Interestingly, presence of
anti-nuclear antibody was not found to be predictive of the development of chronic
uveitis whereas a family history of psoriasis was (RR 3). Disease features that identify
patients at risk of a poor outcome have also been examined in other forms of arthritis.
Spiegel et al evaluated 104 patients after a mean follow-up of 7.7 years in a multi-centre
study of systemic-onset patients. Poor functional outcome at follow-up could be
predicted at 6 months of disease by persistence of fever, the ongoing need for
corticosteroids to control systemic symptoms, and thrombocytosis of greater than
600 000.6 Predictors of disease course outcome in the psoriatic-onset and the enthesitis-
related forms of juvenile arthritis are less well de®ned. Of the latter group the presence
 Reducing the impact of rheumatic diseases 335

of HLA B27 positivity is predictive of the subsequent development of ankylosing

spondylitis7 and there may be ethnic variation in the rate of progression to sacroiliac
involvement radiologically.8
Many older outcome studies are limited in their usefulness as therapeutic planning
guides in that they have tended to concentrate on clinical and biological predictors
of joint outcome rather than overall measures of health status. The importance of
including the latter has been recognized; the core set of outcome variables now used
almost universally in paediatric clinical trials includes measures of health assessment
and overall wellbeing.9 They encompass estimates of disease activity derived from
physician global assessment, joint status (number swollen and/or limited in range) and
the erythrocyte sedimentation rate (ESR); measures of health status derived from
parent/patient global assessment of well-being; and a functional ability score which has
invariably been the Childhood Health Assessment Questionnaire (CHAQ). Trials
typically de®ne meaningful improvement as being at least a 30% improvement in three
of any of the six variables from baseline with no more than one of the remaining
variables worsening by greater than 30%.
An interesting result of the inclusion of measures of patient-reported functional
disability has been the ®nding that patients may perceive themselves as having a
disability where purely physical outcome measures would predict there to be none.
Measures such as the Health Assessment Questionnaire (HAQ) in adult follow-up
studies have shown reduced scores in components which may in¯uence the aspects of
overall quality of life (e.g. sense of wellbeing), independent of tangible measures such as
joint range and radiological joint changes.10 The CHAQ, developed as a childhood
equivalent of the HAQ, includes questions about daily function to give a disability
score and a visual analogue scale to provide an assessment of pain. Using modelling of
children with arthritis, Dempster et al have calculated that a minimum increase of 0.13
in the CHAQ score correlates with changes in health status of clinical signi®cance.11
Other functional assessment measures, for example, the Juvenile Arthritis Functional
Assessment Report (JAFAR)12 and the Juvenile Arthritis Self-Report Index (JASI)13,
may be useful particularly for physical and occupational therapists in monitoring the
physical function of the children. The important issue remains as to how to measure
the quality of life of children, which might be considered the summation of all the
in¯uences of the `disablement process'. Du€y et al in Montreal have developed the
Juvenile Arthritis Quality of Life Questionnaire (JAQQ)14 to address this issue. The
JAQQ aims to provide a quanti®able measure of an individual child's quality of life on
which the e€ect of therapeutic interventions can be assessed. Other measures in early
development include the Childhood Arthritis Health Pro®le (CAHP)15 and the Quality
of My Life scale (QOML).10 As these scales are further re®ned and validated some of
the less tangible aspects that a€ect children with arthritis can hopefully be measured
and prospectively followed.
Published long-term studies, by their very duration, have assessed children treated
largely before the therapeutic advances of the past 10 to 15 years. There has been a
documented improvement in short-term outcome with the early use of methotrexate
and intra-articular corticosteroid therapy, and the new biological agents o€er
potentially even greater promise. It is hoped that some of the poorer outcome
results do not re¯ect the prognosis to be expected for children undergoing therapy
with modern agents. Although these therapeutic modalities, as part of our standard
management, may signi®cantly reduce the potentially destructive nature of juvenile
arthritis, will this be enough to alter the perceived quality of life?
336 J. D. Akikusa and R. C. Allen

PSYCHOSOCIAL INFLUENCES IN DISEASE IMPACT

That the psychological milieu of a child/adolescent and their family is a determint of an

individual's quality of life, is fundamental to the concept that this can be enhanced by
encouraging or developing strengths such as resilience, coping skills and self-worth. As
with the diagnosis of any chronic disease, when given a diagnosis of juvenile arthritis,
patients and their families are likely to experience feelings of grief, anger and fear of an
unknown future. It is a time of extreme vulnerability and the manner in which families
cope and move on to the inevitable requirements of day-to-day management may have
long-term consequences.16 In a study of 72 school-aged children with JIA, Vandvik found
51% met the criteria of a DSM-III diagnosis, particularly dysthymic disorders and
separation anxiety, early in the course of their disease.17 It has been estimated one in ®ve
children diagnosed with a chronic medical condition will have additional emotional
problems as a result of their disease. Furthermore, almost a quarter of JIA patients
studied in adulthood had variable degrees of depression when assessed with a validated
depression questionnaire.18 Aasland et al did not ®nd clinical depression in their follow-
up study of older children with JIA, but using the Children's Global Assessment Scale
did ®nd anxiety and poor overall psychosocial functioning.19 Importantly, these ®ndings
were independent of disease activity and were particularly apparent for those
developing their arthritis in adolescence. As adolescence is a time of developing
independence, the onset of JIA during this period may add to the feeling of lack of
control and less positive adaptability.20
The way in which a family adjusts to the diagnosis of JIA in¯uences the psychosocial
wellbeing of not only the child but also the whole family unit. Parents, particularly
mothers, commonly experience feelings of depression when given the diagnosis of JIA
in their child. Those who develop a sense of mastery (i.e. learning how to deal with
features of the child's illness, such as pain) and active coping strategies (e.g. maintaining
social activities) develop more resilience and a sense of competence in dealing with
their child's illness.21 By contrast, parents who use coping strategies based on
avoidance (e.g. denial, increased alcohol intake to reduce tension) tend to report a
more depressed mood and personal strain. In comparing the adjustment of children
and their mothers to the diagnosis of JIA Ennett et al found that mothers perceive
more diculties for their children than do the children themselves, particularly with
respect to their athletic competence and peer acceptance.22 The children's report
of their disease experience was related to their adjustment to the arthritis but not
its severity. The children with more negative disease experiences felt less competent
in their athletic skills, and, of particular concern, less popular and less physically
attractive, with a lower sense of self-worth.22
Studies reporting psychosocial diculties experienced by, and adjustments required
of, children with arthritis do not necessarily always ®t with a clinician's `feel' for how
their own patients (and their families) are progressing. This gives rise to the question
of whether the methodology used in such studies is always appropriate for the arthritis
population. Some of the psychological tools used to assess, for example, social skills,
may be skewed by factors more to do with functional limitation of the arthritis rather
than an actual aberrant behaviour.20 The developmental variability through childhood,
adolescence and into young adulthood will also in¯uence studies of psychosocial
functioning so that, for example, parental over-protectiveness may be recognized as a
problem in adolescence yet be accepted as being appropriate in the younger child.23
The well established and widely used Arthritis Self-Management Program established
by Kate Lorig for adult rheumatoid arthritis has been shown to enhance self-ecacy
 Reducing the impact of rheumatic diseases 337

in areas such as pain control and psychological well-being. Recently, Barlow et al have
developed the Arthritis Self-Ecacy scales for parents (PASE), and children (CASE),
aiming to assess the extent of a parent's or child's, innate ability to cope with and adjust
to juvenile arthritis.24,25 The MEPS questionnaire (medical issues, exercise, pain and
social support) developed by Andre et al similarly aims to measure the perceived ability
to manage on the part of adolescents and their parents.26 Adequate education about the
disease and issues such as pain management, addressing any functional diculties
encountered and negotiating appropriate involvement in peer group physical activities
may empower the parents (and patient) and thus enhance their resilience and coping
skills.

EDUCATION ABOUT THE DISEASE AND COMPLIANCE WITH

MEDICATION

Non-adherence to medication is a feature of all chronic illnesses. It is probably naõÈ ve of

paediatric rheumatologists to think that parental supervision and/or the likelihood of
pain will ensure stringent compliance with medication and exercise programmes. The
sense that `knowing the patient' allows the physician to gauge compliance with therapy
has not been found to be correct. Studies have found that the physicians' assessment of
compliance is no better than that predicted by chance alone.27 Under the Health Belief
Model the likelihood of a patient/family following through with advice is related to
their perception of the severity of a condition, their susceptibility to its consequences
and an understanding of the long and short-term bene®ts of following through with
such recommendations.28 Placing emphasis on the education of patients and their
parents about the disease and its management, with adequate time allocation on the
physician's part for such a process, may therefore enhance compliance.29
Steps for improving education may include:
1. A separate session, away from the usual routine clinical follow-up assessments, with
the speci®c purpose of educating the family about juvenile arthritis and the
expectations of management.
2. Exploring patient/family beliefs about arthritis and attempting to correct any
misconceptions. In the age of the World Wide Web, information families may have
taken from the `Net' will invariably need to be addressed, both with respect to its
relevance, but perhaps more importantly its irrelevance, to their particular
situation.
3. Choosing the least complex treatment regimen with respect to both medications
and physical therapies. It is recognized that polypharmacy may be negatively related
to adherence. Unfortunately, avoiding polypharmacy is dicult in juvenile arthritis
when second-line agents, particularly in combination, may be necessary. In this
situation patients are expected to take not only the agents themselves but also
additional therapy such as folic acid and/or calcium supplements to o€set
medication-related adverse e€ects. Linking medication taking with some other
well-established daily activity may assist.
4. Careful discussion is needed about medications. This should not be merely a
discussion of potential side-e€ects but must include an explanation of what the use of
individual agents is expected to achieve. This is especially so in these days of a
fundamental mistrust of medication on the part of some parents, more so if
338 J. D. Akikusa and R. C. Allen

alternative (complementary) medicine has become part of their standard approach

to health care. Negotiating treatment goals for some families, and particularly
adolescents, may be necessary. The task of developing independence from the
parent±child relationship during adolescence may result in therapy needs being used
as a bargaining chip. The clinician needs to develop an approach to this situation that
satis®es the often-con¯icting needs of `disease' versus `patient'.

PHYSICAL THERAPIES AND THE ROLE OF EXERCISE

Physical therapies commonly used in the treatment of childhood rheumatic diseases

include splinting, casting, positioning (e.g. prone lying) and therapeutic exercise of
a€ected limbs.30,31 While most commonly used in the treatment of JIA, these therapies
are also used in children with juvenile dermatomyositis, scleroderma and, less
commonly, systemic lupus erythematosus.31 They are designed to restore and maintain
range of motion, strengthen muscle groups and improve motor skills. Collectively,
time spent implementing these therapies constitutes the majority of time families
spend in the treatment of many of the rheumatic diseases. It is sobering to realize then
that there has been little comparative research regarding the ecacy of the treatment
approaches which have been devised ± a fact noted by previous authors.32±34
Di€erences in the approach to physical therapy in JIA between centres specializing
in its treatment are testimony to the current lack of good evidence of superior ecacy
of one treatment approach over another.33 Isolated attempts have been made to clarify
which patients are likely to bene®t from early intervention with splinting35 and report
outcomes using particular splinting techniques36; however, these have been small case
series with no comparator groups.
Joint contractures represent a common clinical problem in the management of
children with arthritis. Intensive physiotherapy, dynamic splints, serial casting, static
traction and intra-articular steroids if the involved joint is in¯amed are all suggested
means of dealing with these.30,31,37 Although experience suggests that all of these
methods are capable of bringing about desired outcomes there have been no formal
studies comparing their relative ecacy. Having a range of treatment options is
important in the management of a population of patients whose temperament and
developmental stages vary widely. Just as important is being able to make a decision
with regard to therapy based on sound ecacy data ± particularly if it is likely to make
signi®cant demands on family time and routines. Children with rheumatic diseases,
and their families, are a€ected not only as a result of the physical changes such diseases
bring about but also as a result of the therapeutic requirements clinicians place on
them. Ensuring that the physical therapies suggested are the most e€ective and time-
ecient means of achieving a desired outcome through well conducted, randomized
clinical trials should be a priority in the age of evidence-based medicine.

THE ROLE OF EXERCISE

Two universally accepted principles in the provision of physical therapy to children

with JIA are the need for treatment to commence early and for aerobic exercise to be
an integral part of the therapy programme.31,33,38 Children with JIA have been found to
have a reduced capacity for aerobic exercise compared with age- and sex-matched
healthy controls.39,40 The reasons for this physical deconditioning are complex and not
 Reducing the impact of rheumatic diseases 339

well understood. While arthritis-related pain, sti€ness and fatigue may be important
contributors to this phenomenon, other factors may play an equal role because the
degree of deconditioning has not been found to relate to the severity of articular
disease39 nor has activity of arthritis been found to relate to physical ®tness.41
That JIA does not necessarily entail physical deconditioning is suggested by Malleson
et al who found little di€erence in the aerobic and anaerobic ®tness of 31 children with
JIA when compared with 16 healthy controls.41 One possible explanation for this is that
their centre encourages patients to participate in physical activities `with almost no
limitations'. Key factors external to the child likely to in¯uence participation in aerobic
exercise are the expectations of parents and therapists. In the case of parents such
expectations may be based on pre-conceived ideas of what constitutes appropriate
activities for a child with `arthritis', which, in turn, may or may not be in¯uenced by the
attitudes of the therapist. Miller et al examined the relationship between parent-
reported disability, as measured by the CHAQ, and objective measures of joint disease
and arthritis sub-type in a group of 88 children with JIA.42 They found that parent-
reported disability in the patient group was greater than that in controls even in those
without synovitis or contractures at the time of assessment. Furthermore, in those
children with synovitis, contractures or both, the degree of reported disability was the
same irrespective of the extent of involvement. The authors speculate that psychosocial
adaptation to disease may in¯uence the physical functioning of children with arthritis
without active disease or joint contractures. We also speculate that, for many parents
whose child has been given the diagnosis of arthritis, there is a minimum degree of
disability that they will attribute to the child irrespective of his or her state of joint
disease. This has implications with regard to parental perceptions of their child's ability
to participate in weight-bearing exercise and therefore the extent to which they will
encourage these activities.
Another factor that may play a role in reducing the participation of children with JIA
in vigorous aerobic weight-bearing exercise has been the traditional view that such
activities may exacerbate joint symptoms. Recent evidence would suggest, however,
that the opposite might be true. Klepper examined the e€ects of an 8-week weight-
bearing physical conditioning programme on several measures of joint disease in a
group of 25 children with JIA.34 Not only were pain scores unchanged over the
duration of the programme but participants manifested signi®cant reductions in an
articular severity index and joint count and demonstrated improvement in aerobic
endurance as measured by a 9-minute walk test. Furthermore, of patients whose pain
score was signi®cantly di€erent following the programme, the majority demonstrated
an improvement over baseline. The bene®ts of other forms of exercise have also been
examined. Takken et al examined the e€ect of a 15-week hydrotherapy programme in
a group of 25 children with JIA,43 including in their evaluation the e€ects on the
CHAQ as a measure of disability and the JAQQ as a measure of health-related quality
of life. Although statistically signi®cant changes were not found in any of the measured
parameters overall, a trend towards improvement in the JAQQ over the programme
period was apparent. Furthermore, there was a statistically signi®cant improvement in
the `general symptoms' domain of the JAQQ, suggesting an improvement in the
subjects' perception of disease signs and symptoms during the programme.
Current evidence supports the approach of encouraging children with JIA to be as
active as possible in both weight-bearing and non-weight-bearing activities. Studies
involving greater numbers of patients and utilizing a randomized design will allow
clari®cation of many aspects of this area which remain unknown or incompletely
de®ned ± in particular, the role of patient and parent education on physical ®tness,
340 J. D. Akikusa and R. C. Allen

the relationship between physical ®tness and quality of life and the magnitude and
extent of change to be expected in terms of aerobic ®tness, disease activity and
disability, with di€erent interventions require clari®cation. While there is always likely
to be a requirement for traditional physical therapies, increasing emphasis on
understanding impediments to uptake of normal physical activity and persistent
perception of disability and reduced disease-related quality of life in the absence of
active joint disease42 is required if the goal of producing non-disabled adults is to be
achieved.

PAIN MANAGEMENT

Dealing with day-to-day pain can be one of the most dicult aspects of disease
management. Pain reporting has not been found to correlate consistently with disease
activity, and interaction with the psychosocial environment may be substantial. Children
with arthritis have been found to have lower pain thresholds than normal controls using
quanti®able provocative measures of pain such as the cold-pressor test.44 This is perhaps
surprising given the apparent tolerance many children appear to demonstrate during
routine clinical review, but it supports the `central sensitization' model of chronic pain
whereby recurrent pain sensitizes an individual to future pain stimulation. Observa-
tional studies looking for physical mannerisms that might indicate a pain behaviour, such
as rubbing or withdrawing of a limb and bracing postures, have been found to correlate
with reported pain and disability levels.45 Interpreting body behaviour as a potential
indicator of pain may be particularly useful in younger children. It may assist not only
family and therapists but also teachers and caregivers to modify activities and implement
appropriate pain-relieving therapies in a timely manner.
Ross et al used a battery of psychological tests to examine the correlation between
reported pain and speci®c psychological variables. Using a visual analogue scale,
children completed a symptom diary three times daily over a 28-day period. Higher
reported pain levels correlated with the child's self-reported anxiety and maternal
distress. Surprisingly, increased pain was also found in children of families scoring
higher on measures of family harmony using the Family Environmental Scale. This may
indicate an element of enmeshment between the child and parent which may impair
pain coping, as is seen commonly in other non-in¯ammatory chronic pain syndromes.46
Pain-management strategies using cognitive-behavioural techniques have been shown
to have a positive e€ect47 and the development of validated speci®c tools such as the
Pain Coping Questionnaire (PCQ)48 may o€er further opportunities to enhance clinical
assessment. Utilizing the PCQ, Thastum and colleagues found that children with lower
reported pain made greater use of distraction strategies whereas, by contrast, higher
levels of pain and reduced pain thresholds were reported in children who
catastrophize.44

ARTHRITIS IN THE SCHOOL SETTING

For the majority of waking hours on the majority of days through the year children are
required to attend school. Here they learn not only what is set out in the formal
curriculum but also about interacting with people other than family and about
themselves and their position among their peers. The social hierarchy among children
and its e€ect on the individual should not be underestimated. Attributes such
 Reducing the impact of rheumatic diseases 341

as personality, physical appearance, athletic ability and scholastic achievement in

addition to a myriad of less-well de®ned issues determine where a child is placed in
this hierarchy.
For children with arthritis, morning sti€ness, pain with prolonged ambulation and
limitation in joint range of movement may all require modi®cation of school routine. It
is easy, however, to adopt an `adult' perspective when considering these issues and
focus on interventions which, while appropriate in a `medical' sense, may serve to
increase the child's sense of `being di€erent'.
It is instructive to examine the di€erence in perspective that parents and teachers of
children with arthritis have compared with the children themselves, on issues of
schooling diculties. In a study of 24 children, Taylor et al found that parents and
teachers related the diculties faced by the children at school largely to activities of
daily living whereas the children related most of their problems to self-concept and peer
relations.49 The role of perspective in the perception of school diculties is
demonstrated in the same study by teachers rating medication scheduling problems
as being more frequent in less severely a€ected rather than more severely a€ected
children. Similar were the ®ndings of a study of 113 children with poly- and
pauciarticular arthritis by Sturge et al in which children with polyarticular arthritis
were rated by teachers as having fewer school problems compared with population
norms.50 An explanation o€ered for both of these observations is that the degree to
which teachers make allowance for a child's condition depends not only on their
knowledge of the condition but also the extent to which its e€ects are evident
externally.
An idea of the extent to which teachers may lack experience in dealing with
children with arthritis may be gained from Taylor's study where only three of the
13 teachers had taught more than one pupil with juvenile arthritis in the preceding
5 years.50 The lack of exposure of teachers to children with arthritis may be even more
profound than this. In a study of 135 children with juvenile arthritis and their parents,
Whitehouse et al found that 7% of parents had not informed the school about the
child's illness.51 Furthermore, 30% had not met the teacher to discuss the child's
condition. As the main caregiver of the child through the day the teacher should be
well informed about the child's condition and particular needs. Even if those needs are
deemed to be nothing above those of other children it is important that the teacher be
aware of this in order that their preconceptions of `arthritis' do not inappropriately
in¯uence expectations of the child.
Any approach to minimizing school diculties in children with juvenile idiopathic
arthritis and rheumatic diseases more generally must include considerations related to
the physical e€ects of the disease. It must also include education about the disease for
the child's teachers and, if appropriate in the child's opinion, their peers. The approach
cannot be formulaic but must be individualized to the child and their particular
circumstances. Involving the child in decisions about strategies to be put in place to
improve their school experience is vital.

TRANSITION TO ADULT CARE

Long-term follow-up studies indicate that signi®cant numbers of adult patients

continue to have issues related to their juvenile arthritis, be it active disease or
sequelae of past in¯ammation. An essential component of care for adolescents with
chronic arthritis is to prepare for appropriate transition to adult-orientated care. It
342 J. D. Akikusa and R. C. Allen

isimportant to di€erentiate the concept of `transition' from simply the act of

transferring care to an adult physician. Transition implies a purposeful, planned
approach, for which the preparation begins earlier.52 Adolescents and their families,
particularly if managed from early childhood, will typically have developed a strong
relationship with their paediatric rheumatology team. Anxiety about the continuation
of care, establishing new relationships with adult services and the changed emphasis to
a more individual management process can be assisted by introducing the concept of
transition well before it occurs. Patients need to be con®dent that the adult service
understands the paediatric issues that have been relevant to that adolescent even
though the nature of their subsequent consultations may change. Experience has
shown that, to the patient's detriment, poorly arranged transition will lead to the
potential `dropping out' from adequate medical review.
A task of adolescence, and part of the move towards independent adulthood, is
developing employment goals or at least some vocational direction. Despite a higher
secondary school completion rate the number of adults with past juvenile arthritis in
regular employment is reported to be lower than una€ected controls.53 The impact of
juvenile arthritis may produce some limitation on the types of employment available,
but assisting an adolescent to develop, and hopefully achieve, appropriate career
expectations should be seen as part of the management of adolescents as the time of
transition approaches.
When the time comes, paediatric rheumatologists themselves need to recognize the
appropriateness of an adolescent patient wishing to move on to an adult service. This
should be seen as an indication of the maturity and sense of autonomy that the patient
has developed within the context of the patient±doctor relationship. Transfer, forced
out of an acute medical event when, for example, hospitalization in a paediatric centre
is not possible, is neither fair to the patient nor to one's adult rheumatology
colleagues. As such it is preferable to introduce the patient into the adult service at a
time when the patient is clinically stable, although the exact age may vary, depending
on patient choice, geographical variables or hospital policy.

CONCLUSION

The results of follow-up studies of juvenile arthritis underscore the signi®cant physical
and emotional sequelae that a€ected children may carry into their adult lives. The
opportunity to minimize the impact of such sequelae constitutes the ongoing challenge
for all members of the paediatric rheumatology team. There is likely to be little
argument about the issues identi®ed in this chapter as being important problems to be
faced. The challenge is being able to incorporate appropriate interventions into the
busy clinic setting to address them adequately, particularly if resources are limited.
Carefully designed and conducted paediatric clinical drug trials have improved the
evidence base on which decisions regarding therapy are now being made. They have
advanced considerably the pharmacological opportunities available to patients with
rheumatic diseases, and JIA in particular. Aspects of management raised in this chapter,
however, currently lag behind drug trials as a focus for clinical research despite the
emphasis they are given as integral components of the day-to-day care of the child with
arthritis. The imperative now is to generate high-quality studies addressing a broader
spread of clinical questions which may dispense with dogma based on clinical assumption
and move all areas of paediatric rheumatological practice into the era of evidenced-based
medicine.
 Reducing the impact of rheumatic diseases 343

Practice points
. follow-up studies of children with arthritis indicate signi®cant numbers, especially
among those having a polyarticular course, continue into adulthood with active
disease and/or sequelae
. current quantitative/qualitative measures of functional status may underestimate
how patients perceive their own sense of wellbeing and the e€ect of the disease
on their quality of life
. clinically signi®cant psychological dysfunction may occur in children and their
parents, particularly around the time of diagnosis, and adolescents are especially
vulnerable
. measures to empower families and to enhance their coping skills include
appropriate disease education, addressing medication compliance and pain self-
management
. regular involvement in age-appropriate physical activities is encouraged and this
has both disease-related and probably psychological bene®ts
. ensuring that school teachers or other non-parental caregivers have an accurate
understanding of an individual child's disease will hopefully optimize the school
experience for a child
. transition of care to adult services is an important phase of care which warrants
planning just as any other aspect of management

Research agenda
. the opportunity to investigate means to reduce the impact of a disease such as JIA
should be an ideal focus for research
. the development and validation of measures of functional assessment, quality of
life and disease impact, as well as evaluating disease outcome measures, continues
. the opportunities coming with the newer therapeutics also demand the need for
detailed long-term on-going evaluation
. multicentre registries and clinical trials akin to those which paediatric oncologists
have long pursued may be a useful model for studying many such questions, not
only involving therapeutics, but also approaches to physical therapies
. traditional approaches to therapy, particularly physical therapy, warrant
appropriate evaluation, hopefully to give some evidence base to many of our
clinical recommendations

REFERENCES

* 1. van der Net J, Prakken AB, Helders PJ et al. Correlates of disablement in polyarticular juvenile chronic
arthritis ± a cross-sectional study. British Journal of Rheumatology 1996; 35: 91±100.
2. Tucker LB. Whose life is it anyway? Understanding quality of life in children with rheumatic diseases.
Journal of Rheumatology 2000; 27: 8±11.
3. Gare BA & Fasth A. The natural history of juvenile chronic arthritis: a population based cohort study. II.
Outcome. Journal of Rheumatology 1995; 22: 308±319.
 344 J. D. Akikusa and R. C. Allen

4. Wallace CA & Levinson JE. Juvenile rheumatoid arthritis: outcome and treatment for the 1990s.
Rheumatic Disease Clinics of North America 1991; 17: 891±905.
5. Guillaume S, Prieur AM, Coste J & Job-Deslandre C. Long-term outcome and prognosis in
oligoarticular-onset juvenile idiopathic arthritis. Arthritis & Rheumatism 2000; 43: 1858±1865.
6. Spiegel LR, Schneider R, Lang BA et al. Early predictors of poor functional outcome in systemic-onset
juvenile rheumatoid arthritis: a multicenter cohort study. Arthritis & Rheumatism 2000; 43: 2402±2409.
7. Cabral DA, Oen KG & Petty RE. SEA syndrome revisited: a longterm followup of children with a
syndrome of seronegative enthesopathy and arthropathy. Journal of Rheumatology 1992; 19: 1282±1285.
8. Burgos-Vargas R, Vazquez-Mellado J, Cassis N et al. Genuine ankylosing spondylitis in children: a case
controlled study of patients with early de®nite disease according to adult onset criteria. Journal of
Rheumatology 1996; 23: 2140±2147.
* 9. Giannini EH, Ruperto N, Ravelli A et al. Preliminary de®nition of improvement in juvenile arthritis.
Arthritis & Rheumatism 1997; 40: 1202±1209.
*10. Feldman BM, Grundland B, McCullough L & Wright V. Distinction of quality of life, health related
quality of life, and health status in children referred for rheumatologic care. Journal of Rheumatology 2000;
27: 226±233.
*11. Dempster H, Porepa M, Young N & Feldman BM. The clinical meaning of functional outcome scores in
children with juvenile arthritis. Arthritis & Rheumatism 2001; 44: 1768±1774.
12. Howe S, Levinson J, Shear E et al. Development of a disability measurement tool for juvenile rheumatoid
arthritis. The Juvenile Arthritis Functional Assessment Report for Children and their Parents. Arthritis &
Rheumatism 1991; 34: 873±880.
13. Wright FV, Law M, Crombie V et al. Development of a self-report functional status index for juvenile
rheumatoid arthritis. Journal of Rheumatology 1994; 21: 536±544.
14. Du€y CM, Arsenault L, Du€y KN et al. The Juvenile Arthritis Quality of Life Questionnaire ±
development of a new responsive index for juvenile rheumatoid arthritis and juvenile spondyloarthri-
tides. Journal of Rheumatology 1997; 24: 738±746.
15. Tucker LB, De Nardo BA, Abetz LN et al. The childhood arthritis health pro®le: validity and reliability
of condition speci®c scales. Arthritis & Rheumatism 1995; 38 (supplement): 183.
16. Vandvik IH & Eckblad G. Mothers of children with recent onset of rheumatic disease: associations
between maternal distress, psychosocial variables, and the disease of the children. Journal of
Developmental and Behavioural Pediatrics 1991; 12: 84±91.
17. Vandvik IH. Mental health and psychosocial functioning in children with recent onset of rheumatic
disease. Journal of Child Psychology and Psychiatry 1990; 31: 961±971.
18. David J, Cooper C, Hickey L et al. The functional and psychological outcomes of juvenile chronic arthritis
in young adulthood. British Journal of Rheumatology 1994; 33: 876±881.
*19. Aasland A, Flato B & Vandvik IH. Psychosocial outcome in juvenile chronic arthritis: a nine-year follow-
up. Clinical and Experimental Rheumatology 1997; 15: 561±568.
20. Daltroy LH, Larson MG, Eaton HM et al. Psychosocial adjustment in juvenile arthritis. Journal of Pediatric
Psychology 1992; 17: 277±289.
21. Timko C, Stovel KW & Moos RH. Functioning among mothers and fathers of children with juvenile
rheumatic disease: a longitudinal study. Journal of Pediatric Psychology 1992; 17: 705±724.
22. Ennett ST, DeVellis BM, Earp JA et al. Disease experience and psychosocial adjustment in children with
juvenile rheumatoid arthritis: children's versus mothers' reports. Journal of Pediatric Psychology 1991; 16:
557±568.
23. Ungerer JA, Horgan B, Chaitow J & Champion GD. Psychosocial functioning in children and young
adults with juvenile arthritis. Pediatrics 1988; 81: 195±202.
24. Barlow JH, Shaw KL & Wright CC. Development and preliminary validation of a self-ecacy measure for
use among parents of children with juvenile idiopathic arthritis. Arthritis Care Research 2000; 13: 227±235.
25. Barlow JH, Shaw KL & Wright CC. Development and preliminary validation of a children's arthritis self-
ecacy scale. Arthritis & Rheumatism 2001; 45: 159±166.
26. Andre M, Hedengren E, Hagelberg S & Stenstrom CH. Perceived ability to manage juvenile chronic
arthritis among adolescents and parents: development of a questionnaire to assess medical issues,
exercise, pain, and social support. Arthritis Care Research 1999; 12: 229±237.
27. Goldberg AI, Cohen G & Rubin AH. Physician assessments of patient compliance with medical
treatment. Social Science Medicine 1998; 47: 1873±1876.
28. Rosenstock IM, Strecher VJ & Becker MH. Social learning theory and the Health Belief Model. Health
Education Quarterly 1988; 15: 175±183.
29. Rapo€ MA, Purviance MR & Lindsley CB. Educational and behavioral strategies for improving medication
compliance in juvenile rheumatoid arthritis. Archives of Physical Medicine and Rehabilitation 1988; 69:
439±441.
 Reducing the impact of rheumatic diseases 345

30. Scull SA, Dow MB & Athreya BH. Physical and occupational therapy for children with rheumatic
diseases. Pediatric Clinics of North America 1986; 33: 1053±1077.
*31. Emery HM, Bowyer SL & Sisung CE. Rehabilitation of the child with a rheumatic disease. Pediatric Clinics
of North America 1995; 42: 1263±1283.
32. Emery HM & Bowyer SL. Physical modalities of therapy in pediatric rheumatic diseases. Rheumatic
Disease Clinics of North America 1991; 17: 1001±1014.
*33. Hackett J, Johnson B, Parkin A & Southwood T. Physiotherapy and occupational therapy for juvenile
chronic arthritis: custom and practice in ®ve centres in the UK, USA and Canada. British Journal of
Rheumatology 1996; 35: 695±699.
34. Klepper SE. E€ects of an eight-week physical conditioning program on disease signs and symptoms in
children with chronic arthritis. Arthritis Care Research 1999; 12: 52±60.
35. Helders PJ, Nieuwenhuis MK, van der Net J et al. Displacement response of juvenile arthritic wrists
during grasp. Arthritis Care Research 2000; 13: 375±381.
36. Barden W, Brooks D & Ayling-Campos A. Physical therapy management of the subluxated wrist in
children with arthritis. Physical Therapy 1995; 75: 879±885.
37. Fredriksen B & Mengshoel AM. The e€ect of static traction and orthoses in the treatment of knee
contractures in preschool children with juvenile chronic arthritis: a single subject design. Arthritis Care
Research 2000; 13: 352±359.
38. Rhodes VJ. Physical therapy management of patients with juvenile rheumatoid arthritis. Physical Therapy
1991; 71: 910±919.
*39. Giannini MJ & Protas EJ. Aerobic capacity in juvenile rheumatoid arthritis patients and healthy children.
Arthritis Care Research 1991; 4: 131±135.
40. Klepper SE, Darbee J, E€gen SK & Singsen BH. Physical ®tness levels in children with polyarticular
juvenile rheumatoid arthritis. Arthritis Care Research 1992; 5: 93±100.
*41. Malleson PN, Bennett SM, MacKinnon M et al. Physical ®tness and its relationship to other indices of
health status in children with chronic arthritis. Journal of Rheumatology 1996; 23: 1059±1065.
*42. Miller ML, Kress AM & Berry CA. Decreased physical function in juvenile rheumatoid arthritis. Arthritis
Care Research 1999; 12: 309±313.
43. Takken T, van der Net J & Helders PJ. Do juvenile idiopathic arthritis patients bene®t from an exercise
program? A pilot study. Arthritis & Rheumatism 2001; 45: 81±85.
44. Thastum M, Zachariae R & Herlin T. Pain experience and pain coping strategies in children with juvenile
idiopathic arthritis. Journal of Rheumatology 2001; 28: 1091±1098.
45. Jaworski TM, Bradley LA, Heck LW et al. Development of an observation method for assessing pain
behaviors in children with juvenile rheumatoid arthritis. Arthritis & Rheumatism 1995; 38: 1142±1151.
46. Ross CK, Lavigne JV, Hayford JR et al. Psychological factors a€ecting reported pain in juvenile
rheumatoid arthritis. Journal of Pediatric Psychology 1993; 18: 561±573.
47. Varni JW. Evaluation and management of pain in children with juvenile rheumatoid arthritis. Journal of
Rheumatology 1992; 33 (supplement): 32±35.
48. Reid GJ, Gilbert CA & McGrath PJ. The Pain Coping Questionnaire: preliminary validation. Pain 1998;
76: 83±96.
*49. Taylor J, Passo MH & Champion VL. School problems and teacher responsibilities in juvenile rheumatoid
arthritis. Journal of School Health 1987; 57: 186±190.
50. Sturge C, Garralda ME, Boissin M et al. School attendance and juvenile chronic arthritis. Brithis Journal of
Rheumatology 1997; 36: 1218±1223.
51. Whitehouse R, Shope JT, Sullivan DB & Kulik C-L. Children with juvenile rheumatoid arthritis at school.
Functional problems, participation in physical education. Clinical Pediatrics 1989; 28: 509±514.
52. Blum RW, Garell D, Hodgman CH et al. Transition from child-centered to adult health-care systems for
adolescents with chronic conditions. A position paper of the Society for Adolescent Medicine. Journal of
Adolescent Health 1993; 14: 570±576.
53. Peterson LS, Mason T, Nelson AM et al. Psychosocial outcomes and health status of adults who have had
juvenile rheumatoid arthritis: a controlled, population-based study. Arthritis & Rheumatism 1997; 40:
2235±2240.