A Major Challenge to Improving Outcome in Juvenile Idiopathic Arthritis

Editorial Access to Pediatric Rheumatology Care — A Major Challenge to Improving Outcome in Juvenile Idiopathic Arthritis

Juvenile idiopathic arthritis (JIA) is a heterogeneous group care exists, with reports of children with incident JIA being of diseases1,2 and is one of the most common chronic rheu- subjected to multiple, often invasive and unnecessary inves- matic diseases in children. Improving the outcome for chil- tigations (such as arthroscopy or synovial biopsy) before a dren with JIA remains an important goal, with current man- pediatric rheumatology referral and this likely contributes agement of severe JIA involving increasingly aggressive to the reported delay9; further, anecdotally, children with immunosuppressive agents1. JIA are managed by doctors or other healthcare profession- Undoubtedly, improving outcome in JIA depends on the als who are not optimally trained or resourced to deliver availability of effective treatments, but if a child presents best practice. The British Society for Paediatric and late in their disease course or receives inappropriate treat- Adolescent Rheumatology (BSPAR) Standards of Care for ment or does not have access to treatment at all, then out- Children with JIA10 are a step in the right direction for equi- come is likely to be suboptimal. There is an increasing body table access to high quality care, with emphasis on early of evidence (Table 1)3 that many children with JIA have a recognition of JIA and prompt referral (target of within 10 prolonged interval from disease onset to pediatric rheuma- weeks of disease onset), and highlight the need for high tology care, and despite there being no published data from quality clinical trials to increase the evidence base to inform developing countries, anecdotal observations suggest that best practice; further the BSPAR Position Statement11 poor access to optimal care is likely to be a global issue. describes the training and experience required for members The longterm impact of delay is unknown, but a longer of the pediatric rheumatology specialist team. interval from disease onset to definitive treatment is likely Although Shiff, et al present limited data on the referral to adversely affect clinical outcomes4,5,6. In one study7, pathways, their findings are important as they corroborate many children with delay (defined as > 10 weeks from other studies that give some insight into the complexity of symptom onset to first pediatric rheumatology assessment) patient journeys to pediatric rheumatology care8. We pro- had prolonged untreated active disease, many presented pose that barriers and drivers to care can be divided into with multiple restricted joints, none had been referred for broad themes, as follows. eye screening (to detect chronic anterior uveitis), and the median interval from onset of symptoms to starting Physical Factors methotrexate was 10 months. Such delay is likely to have a Physical factors inherent in the disease influence the time to profound effect on the potential for functional disability and referral. For example, the ill child with severe JIA subtype psychosocial impact as a result of poorly controlled symp- who presents with raised inflammatory markers, often with toms and the family living with an uncertain diagnosis. fever or acute limp, is more likely to present quickly to The article by Shiff, et al8 in this issue of The Journal healthcare7,12,13; whereas more indolent presentations may describes a large incident cohort of children with JIA from be overlooked, such as the well child with normal inflam- across Canada, again reporting prolonged interval to pedi- matory markers and intermittent limp (a common finding in atric rheumatology care, but also describes factors that may oligoarticular JIA) or the child with chronic heel pain due to hinder or facilitate referral. Both this and many other stud- enthesitis, and it will often take longer for the diagnosis to ies refer to children presenting to pediatric rheumatology be made8. Differentiating early forms of JIA from reactive services, and there are no data on comparative outcomes or transient forms of inflammatory arthritis remains a diag- between children managed by specialist and nonspecialist nostic challenge. Screening questionnaires have been centers. However, inequity in access to most appropriate described but lack sensitivity and specificity to detect JIA

See Factors associated with longer time to specialist access for Canadian children with JIA, page 2415

Downloaded on September 29, 2021 from www.jrheum.org Table 1. Studies reporting interval from onset of symptoms to pediatric rheumatology care. Adapted from Foster, et al, Rheumatology 2010;49:401-33.

Time from Onset to First Pediatric Rheumatology Assessment Study Country n median, mo (range, yrs) Type of Study

Tzaribachev, 200921 Germany 132 7.2 (0–1.67) Retrospective cohort, regional service Shiff, 200929 Canada 35 4.2 (0–13.6) Prospective cohort, regional service Adib, 200812 UK 507 4.6 (0–2.75) Prospective cohort, multicenter Foster, 20077 UK 152 5 (0–7.9) Retrospective cohort, regional Shapiro, 200730 Canada 836 2.57 (0–5) Retrospective cohort, regional Arguedas, 200231 Costa Rica 47 3.7 (0–2.75) Prospective cohort, population based Guillaume, 200032 France 207 3 (0–7.98) Retrospective, longitudinal, regional; oligoarticular-onset JIA only Minden, 200033 Germany 171 10 (0–7.7) Retrospective, longitudinal, regional Manners, 199913 Australia 42 10 (0–4) Retrospective cohort, regional Andersson Gare, 199534 Sweden 124 3.5 (0–3.4) Prospective cohort, population based early14; in the absence of a diagnostic test, careful clinical Canada, long distance to the nearest pediatric rheumatology assessment, knowledge, and exclusion of other diagnoses clinic was not a significant factor to explain the delay, remain key to early recognition of JIA. although this finding conflicts with data from Germany21 and from the US, where the greater the distance from pedi- Recognition of JIA atric rheumatology services, the more likely services are Pathways of care for children with suspected JIA are not delivered by adult rheumatologists24. The paucity of appro- reported in detail by Shiff, et al, but undoubtedly they are priately trained pediatric rheumatologists and multidiscipli- complex and vary across healthcare systems. Children with nary teams24 is likely to contribute to inequity in delivery of JIA often present to healthcare professionals who are not appropriate care, and increased recruitment to the specialty expert in pediatric rheumatology; many doctors in family is needed. Access to appropriate care may be limited by practice and hospital care (such as general pediatrics and healthcare costs such as in the USA, where children with orthopedics) are not confident in their musculoskeletal JIA covered by basic healthcare insurance are less likely to assessment of children15,16 — perhaps not surprising, given have eye screening25. Biological agents are not available in that pediatric musculoskeletal medicine is not core teaching many parts of the world, and may be subject to “rationing” in medical schools17,18, which may contribute to the report- based on cost-effectiveness (www.nice.org.uk), and avail- ed delay in JIA and other conditions presenting with muscu- ability may be geographically dependent on local health loskeletal features19,20. Notably, children with JIA present- budgets. ing via orthopedics are reported to have the longest interval to pediatric rheumatology care7,13,21. Clearly, improved Sociocultural Factors education and training to improve awareness and skills to Sociocultural factors may influence health-seeking behav- recognize JIA are important22; these should be acquired by ior. Shiff, et al report that children of South Asian ethnicity all doctors who are likely to encounter children (especially and parents with higher levels of education are likely to doctors working within orthopedics, emergency medicine, present more quickly and suggest that this may reflect general pediatrics, and family medicine); and such training greater parent empowerment or may be due to ethnic varia- needs to start at medical school. tion in the epidemiology of severe JIA subtypes. Such obser- The development of a simple validated musculoskeletal vations are important and need to be explored further; we screening examination (pGALS23) increasingly taught to can learn much from parent and child experiences. There is medical students and in the UK, the introduction of compe- increasing need, given the great advances in management, to tency frameworks for postgraduate training for all general understand the factors that drive or hinder the referral to pediatricians with musculoskeletal themes incorporated in pediatric rheumatology. Irrespective of the ultimate diagno- the mandatory assessments (www.rcpch.ac.uk/training) will sis, the visibility, persistence, and perceived severity of hopefully facilitate early recognition of JIA. symptoms are central to a parent’s actions around help-seeking behavior26. Parents will notice a problem, but may not Healthcare Provision be able to judge the actual severity and need to seek expert Conditions of healthcare provision, such as the geographical advice27. In consultations with health professionals, parents distance to pediatric rheumatology specialist services and often act as advocates for their sick child26, and have to bal- the availability of medicines, are major influences on access ance being seen as responsible and vigilant against being to high quality care. In the study by Shiff, et al across labeled as overly neurotic, protective, or even exploitative.

Downloaded on September 29, 2021 from www.jrheum.org When parents of children with cancer first reported that their arthritis. J Rheumatol 2010;37:2415-22. child was unwell, their concerns were often disregarded and 9. McMahon A, Friswell M, Myers A, Foster HE. Do interventions delay access to paediatric rheumatology care for children with they struggled to obtain further investigations and a diagno- suspected juvenile idiopathic arthritis? [abstract]. Arthritis Rheum 28 sis of cancer . Anecdotally, parents of children with JIA are 2007;56 Suppl:S379. sometimes spurred on to seek healthcare by observations 10. Davies K, Cleary G, Foster H, Hutchinson E, Baildam E; British from teachers or nursery workers who may have noticed Society of Paediatric and Adolescent Rheumatology. BSPAR altered function in the child during school or play activities. standards of care for children and young people with juvenile idiopathic arthritis. Rheumatology 2010;49:1406-8. Centrally, the inequalities of referrals to appropriate care 11. Baildam E, Davidson J. BSPAR position statement on professionals often stem from inequalities of knowledge and experience of working in paediatric rheumatology. Rheumatology 2008;47:743-4. the health professionals to recognize JIA; experience with a 12. Adib N, Hyrich K, Thornton J, Lunt M, Davidson J, prior case or contact with a specialist service, or both, are like- Gardner-Medwin J, et al. Association between duration of ly key drivers in triggering concern and a prompt referral. symptoms and severity of disease at first presentation to paediatric rheumatology: results from the Childhood Arthritis Prospective In summary, while the reasons for delay, inequity, and Study. Rheumatology 2008;47:991-5. inappropriate care are likely to be multifactorial, they are 13. Manners PJ. Delay in diagnosing juvenile arthritis. Med J Aust increasingly important to address as a major challenge to 1999;171:367-9. improve outcome for children with JIA. More work is 14. Len CA, Terreri MT, Puccini RF, Wechsler R, Silva EK, Oliveira required to identify and understand the barriers and drivers LM, et al. Development of a tool for early referral of children and adolescents with signs and symptoms suggestive of chronic in the referral process, identify and address the training arthropathy to pediatric rheumatology centers. Arthritis Rheum needs of doctors likely to encounter children presenting with 2006;55:373-7. suspected JIA, and identify strategies to empower parents 15. Jandial S, Myers A, Wise E, Foster HE. Doctors likely to encounter and caregivers to seek appropriate healthcare attention. children with musculoskeletal complaints have low confidence in their clinical skills. J Pediatr 2009;154:267-71. 16. Bonds DE, Mychaleckyj JC, Watkins R, Palla S, Extrom P. MD, MBBS(Hons), FRCPCH, FRCP, DCH, Cert Med Ed, HELEN FOSTER, Ambulatory care skills: Do residents feel prepared? Med Educ Musculoskeletal Research Group, Online 2002;7:7. Institute of Cellular Medicine; 17. Jandial S, Rapley T, Foster H. Current teaching of paediatric TIM RAPLEY, PhD, MA, BA(Hons), musculoskeletal medicine within UK medical schools — a need for Institute of Health and Society, change. Rheumatology 2009;48:587-90. Newcastle University, 18. Pinney SJ, Regan WD. Educating medical students about Newcastle upon Tyne, UK NE2 4HH musculoskeletal problems: are community needs reflected in the curricula of Canadian medical schools? J Bone Joint Surg Am Address correspondence to Prof. Foster. E-mail: [email protected] 2001;83:1317-20. 19. Dang-Tan T, Franco EL. Diagnosis delays in childhood cancer: a REFERENCES review. Cancer 2007;110:703-13. 1. Ravelli A, Martini A. Juvenile idiopathic arthritis. Lancet 20. Mohamed K, Appleton R, Nicolaides P. Delayed diagnosis of 2007;369:767-78. Duchenne muscular dystrophy. Eur J Paediatr Neurol 2000; 2. Petty RE, Southwood TR, Manners P, Baum J, Glass DN, 4:219-23. Goldenberg J, et al. International League of Associations for 21. Tzaribachev N, Benseler SM, Tyrrell PN, Meyer A, Rheumatology classification of juvenile idiopathic arthritis: second Kuemmerle-Deschner JB. Predictors of delayed referral to a revision, Edmonton, 2001. J Rheumatol 2004;31:390-2. pediatric rheumatology center. Arthritis Rheum 2009;61:1367-72. 3. Foster H, Rapley T, May C. Juvenile idiopathic arthritis: Improved 22. Foster H, Kimura Y. Ensuring that all paediatricians and outcome requires improved access to care. Rheumatology rheumatologists recognise significant rheumatic diseases. Best Pract 2010;49:401-3. Res Clin Rheumatol 2009;23:625-42. 4. Albers HM, Wessels JA, van der Straaten RJ, Brinkman DM, 23. Foster HE, Kay LJ, Friswell M, Coady D, Myers A. Suijlekom-Smit LW, Kamphuis SS, et al. Time to treatment as an Musculoskeletal screening examination (pGALS) for school-age important factor for the response to methotrexate in juvenile children based on the adult GALS screen. Arthritis Rheum idiopathic arthritis. Arthritis Rheum 2009;61:46-51. 2006;55:709-16. 5. Sherry DD, Stein LD, Reed AM, Schanberg LE, Kredich DW. 24. Mayer ML, Mellins ED, Sandborg CI. Access to pediatric Prevention of leg length discrepancy in young children with rheumatology care in the United States. Arthritis Rheum pauciarticular juvenile rheumatoid arthritis by treatment with 2003;49:759-65. intraarticular steroids. Arthritis Rheum 1999;42:2330-4. 25. Brunner HI, Taylor J, Britto MT, Corcoran MS, Kramer SL, Melson 6. Chia A, Lee V, Graham EM, Edelsten C. Factors related to severe PG, et al. Differences in disease outcomes between Medicaid and uveitis at diagnosis in children with juvenile idiopathic arthritis in a privately insured children: possible health disparities in juvenile screening program. Am J Ophthalmol 2003;135:757-62. rheumatoid arthritis. Arthritis Rheum 2006;55:378-84. 7. Foster HE, Eltringham MS, Kay LJ, Friswell M, Abinun M, Myers 26. Dixon-Woods M, Kirk D, Agarwal S, Arthur T, Harvey J, Hsu R, et A. Delay in access to appropriate care for children presenting with al. Vulnerable groups and access to health care: a critical musculoskeletal symptoms and ultimately diagnosed with juvenile interpretive review. London: NHS SDO R&D Programme: 2005. idiopathic arthritis. Arthritis Rheum 2007;57:921-7. 27. Kai J. Parents’ difficulties and information needs in coping with 8. Shiff NJ, Tucker LB, Guzman J, Oen K, Yeung RSM, Duffy CM. acute illness in preschool children: a qualitative study. BMJ Factors associated with a longer time to access pediatric 1996;313:987-90. rheumatologists in Canadian children with juvenile idiopathic 28. Dixon-Woods, M, Findlay M, Young B, Cox H, Heney D. Parents’

Downloaded on September 29, 2021 from www.jrheum.org accounts of obtaining a diagnosis of childhood cancer. Lancet 32. Guillaume S, Prieur AM, Coste J, Job-Deslandre C. Long-term 2001:357:670-4. outcome and prognosis in oligoarticular-onset juvenile idiopathic 29. Shiff NJ, Abdwani R, Cabral DA, Houghton KM, Malleson PN, arthritis. Arthritis Rheum 2000;43:1858-65. Petty RE, et al. Access to pediatric rheumatology subspecialty care 33. Minden K, Kiessling U, Listing J, Niewerth M, Döring E, Meincke in British Columbia, Canada. J Rheumatol,2009;36:410-5. J, et al. Prognosis of patients with juvenile chronic arthritis and 30. Shapiro C, Maenz L, Hossain A, Pahwa P, Rosenberg A. Onset to juvenile spondyloarthropathy. J Rheumatol 2000;27:2256-63. first visit intervals in childhood rheumatic diseases. J Rheumatol 34. Gare BA, Fasth A, The natural history of juvenile chronic arthritis: 2007;34:1913-7. a population based cohort study. I. Onset and disease process. 31. Arguedas O, Fasth A, Andersson-Gare B. A prospective population J Rheumatol 1995;22:295-307. based study on outcome of juvenile chronic arthritis in Costa Rica. J Rheumatol 2002;29:174-83. J Rheumatol 2010;37:2199–202; doi:3899/jrheum.100910

Downloaded on September 29, 2021 from www.jrheum.org