Physiotherapy and occupational therapy for children and young people with juvenile idiopathic arthritis

Chapter 22 Physiotherapy and occupational therapy for children and young people with juvenile idiopathic arthritis

Janine Hackett, MSc BA(Hons), Dip.COT Department of Occupational Therapy, University of Derby, Derby, UK

Bernadette Johnson, MCSP, Children’s Physiotherapy Service, South Staffordshire PCT, Samuel Johnson Hospital, Lichfield, Staffordshire, UK

CHAPTER CONTENTS

Adherence and concordance 331

Prognosis 331

Conclusion 331

Acknowledgements 331

INTRODUCTION

Although musculoskeletal pains are common in childhood, Juvenile Idiopathic Arthritis (JIA), or Juvenile Chronic Arthritis (JCA) as it was formerly known, is a fairly uncommon condition with a prevalence of one in a 1000. This is similar to that of childhood diabetes but more common than cystic fibrosis. American literature will refer to Juvenile Rheumatoid Arthritis (JRA). JIA is a chronic auto-immune disease characterized by persistent joint inflammation in children and young people with onset before their 16^th birthday.

AETIOLOGY

The aetiology of JIA is still unclear although a genetic pre-disposition has been suggested (Thomas et al 2000).

CLASSIFICATION AND FEATURES OF JIA

There are several different sub-groups of JIA and, although primarily designed for research purposes, the International League against Rheumatism Classification (Petty et al 2004) is now in general usage globally, with the exception of North America (see Table 22.1).

Table 22.1 The subgroups and features of JIA

SUB GROUP	SEX DIFFERENCES	FEATURES (ILAR CLASSIFICATION)
Systemic arthritis	Usually occurs in younger children M = F	Arthritis with once to twice daily spikes of fever and one or more of the following: rash, lymph node enlargement, hepatomegaly, splenomegaly, serositis
Oligoarthritis: Persistent	Most common in 1-3 year old white girls	Affects one to four joints during first 6 months
	Girls:boys 4:1	Most common type of JIA
		Knee most commonly affected joint
		Associated with chronic anterior uveitis particularly those who are ANA positive
Extended		If > four joints after first 6 months then defined as extended oligoarthritis
Polyarthritis (rheumatoid factor negative)	Girls:boys 3:1	Affects five or more joints in first 6 months
		Usually symmetrical
		Often involves small joints
Polyarthritis (rheumatoid factor positive)	Most common in adolescent girls	Affects five or more joints in first 6 months
		Rheumatoid factor positive
		Usually symmetrical
		Often involves small joints of hands
Psoriatic arthritis	Girls slightly more affected than boys Onset between 7 and 10 years of age	Arthritis and psoriasis or arthritis plus two of: dactylitis, nail abnormalities, family history of psoriasis in 1^st degree relative
Enthesitis related arthritis	More common in boys over the age of 8	Arthritis and/or enthesitis and at least two from:
		Sacro-iliac joint tenderness, HLA-B27 positive, 1^st degree relative with HLA-B27 disease, anterior uveitis or onset of arthritis in a boy > 8 years
Other arthritis Unclassified		Arthritis persisting > 6 weeks that does not meet criteria for other categories or fulfils criteria for more than one of other categories

Key: ILAR – International League Against Rneumatism (Petty et al 2004)

DIFFERENTIAL DIAGNOSIS

JIA’s relative rarity can make it difficult to diagnose. Some children and young people may have seen many doctors and therapists before receiving their diagnosis as there are many conditions which present with joint pain and/or swelling (Allen 1993). The diagnosis is often one of clinical presentation and exclusion. There are no specific blood tests which will confirm the diagnosis although some may be helpful in establishing the presence of inflammation.

MULTI-DISCIPLINARY TEAM APPROACH

Although there is no cure for JIA many of the disease’s consequences are preventable with good medical management. Referral to a paediatric rheumatologist is therefore essential. Since JIA is an unpredictable chronic disease a team of professionals attending to the global needs of patients can help mediate many of the disease’s effects. A multi-disciplinary team (MDT) approach is therefore viewed as the most beneficial for the child/young person and their family (Lady Hoare Trust 1997, Southwood & Malleson 1993).

THE CHANGING ROLE OF PHYSIOTHERAPY AND OCCUPATIONAL THERAPY

Traditionally, splinting and exercise played a dominant role in physiotherapy and occupational therapy management of JIA (Ansell & Swann 1983, Hackett et al 1996, Jarvis & Lawton 1985). However, the role of these two professions has changed dramatically over the years with the advent and earlier use of second line disease modifying drugs, such as methotrexate and the subsequent reduction in morbidity. The introduction of biologics, such as etanercept and infliximab, has also offered hope to those who have not responded to methotrexate. This has been an exciting time for therapists who have been forced to evaluate their practice and develop new roles for themselves, as well as design treatment interventions which meet the emerging needs of patients. The focus is now firmly on equipping the child/young person with the skills to manage their own condition and to lead to a healthy and meaningful life.

ASSESSMENT

Delays in diagnosis of JIA often result in anxiety and frustration for the family and may lead to mistrust of health care professionals. Therapists are therefore encouraged to spend time at the start of their initial assessment listening to the patient’s and family’s ‘journey to diagnosis’, acknowledging any distress or unhelpful delays in diagnosis, as a way of building trust and positive relationships (Britton & Moore 2002a) which are likely to be long term due to the chronic nature of the disease.

In paediatrics it is often tempting to talk solely to the parent who, we might assume, will be able to give a quick and accurate account of the symptoms of JIA and the impact this has on their child’s life. However, children and young people spend a considerable amount of time away from their parents from an early age and experience their JIA on a daily basis. Parents may also not be the most reliable reporters and indeed may be unaware of some of the issues affecting their child.

OUTLINE OF ASSESSMENT

Introduction

After eliciting information on a range of topics including: history of the condition, the child/young person’s past medical/developmental history, as well as drug history, it is important to gain insight into the family/social history. This will allow therapists to identify potential support systems as well as any social/housing issues. The British Society of Paediatric and Adolescent Rheumatology (BSPAR) Allied Health Professional Guidelines for Assessment of Children and Young People can be obtained from the Society’s website (BSPAR 2002).

SUBJECTIVE ASSESSMENT

The Childhood Health Assessment Questionnaire (CHAQ) (Nugent et al 2001, Singh et al 1994) is a standardised assessment which quantifies levels of functional ability in a number of domains including dressing, walking and reach, and is commonly used in rheumatology clinics in the UK. Although this may be a useful tool for screening for functional deficits, it should not replace a comprehensive therapy assessment. It is however a valuable tool for audit/research and is one of the core outcome variables used by medics to determine drug efficacy.