Paediatric Rheumatology Fails to Meet Current Benchmarks, a Call for Health Equity for Children Living with Juvenile Idiopathic Arthritis, Using Digital Health Technologies.

PURPOSE OF REVIEW: This critical review begins by presenting the history of Juvenile Idiopathic Arthritis (JIA) management. To move the conversation forward in addressing the current shortcomings that exist in the clinical management of children living with JIA, we argue that to date, the advancement of successful treatments for JIA has been historically slow. Factors implicated in this situation include a lack of rigorous research, JIA being considered a rare disease, and JIA's idiopathic and complex pathophysiology. RECENT FINDINGS: Despite the well-intended legislative changes to increase paediatric research, and the major advancements seen in molecular medicine over the last 30 years, globally, paediatric rheumatology services are still failing to meet the current benchmarks of best practice. Provoking questions on how the longstanding health care disparities of poor access and delayed treatment for children living with JIA can be improved, to improve healthcare outcomes. Globally, paediatric rheumatology services are failing to meet the current benchmarks of best practice. Raising awareness of the barriers hindering JIA management is the first step in reducing the current health inequalities experienced by children living with JIA. Action must be taken now, to train and well-equip the paediatric rheumatology interdisciplinary workforce. We propose, a resource-efficient way to improve the quality of care provided could be achieved by embedding digital health into clinical practice, to create an integrative care model between the children, general practice and the paediatric rheumatology team. To improve fragmented service delivery and the coordination of interdisciplinary care, across the healthcare system.

Healthcare and Research Priorities of Adolescents and Young Adults with Systemic Lupus Erythematosus: A Mixed-methods Study.

OBJECTIVE: Managing juvenile-onset systemic lupus erythematosus (SLE) is particularly challenging. The disease may be severe, adolescent patients have complex medical and psychosocial needs, and patients must navigate the transition to adult services. To inform patient-centered care, we aimed to identify the healthcare and research priorities of young patients with SLE and describe the reasons underpinning their priorities. METHODS: Face-to-face, semistructured interviews and focus groups were conducted with patients with SLE, aged from 14 to 26 years, from 5 centers in Australia. For each of the 5 allocation exercises, participants allocated 10 votes to (1) research topics; research questions on (2) medical management, (3) prevention and diagnosis, (4) lifestyle and psychosocial; and (5) healthcare specialties, and discussed the reasons for their choices. Descriptive statistics were calculated for votes and qualitative data were analyzed thematically. RESULTS: The 26 participants prioritized research that alleviated the psychological burden of SLE. They allocated their votes toward medical and mental health specialties in the management of SLE, while fewer votes were given to physiotherapy/occupational therapy and dietetics. The following 7 themes underpinned the participants' priorities: improving service shortfalls, strengthening well-being, ensuring cost efficiency, minimizing family/community burden, severity of comorbidity or complications, reducing lifestyle disruption, and fulfilling future goals. CONCLUSION: Young patients with SLE value comprehensive care with greater coordination among specialties. They prioritized research focused on alleviating poor psychological outcomes. The healthcare and research agenda for patients with SLE should include everyone involved, to ensure that the agenda aligns with patient priorities, needs, and values.

Pediatric rheumatology consultant workforce in Australia and New Zealand: the current state of play and challenges for the future.

AIM: There have been no formal assessments of current levels of the pediatric rheumatology workforce in Australia and New Zealand. Despite this it is felt that we fall well behind international guidelines placing children and adolescents with rheumatic diseases at risk of suboptimal care. Overcoming this shortfall in specialist pediatric rheumatology care requires documentation and recognition of the shortfall and a commitment from the health system to support improvements to supplement the current specialist workforce. The purpose of this survey was to assess the current state of play of the pediatric rheumatology workforce in Australia and New Zealand. METHODS: The Australian Paediatric Rheumatology Group (APRG) conducted a survey, which examined the current pediatric rheumatology workforce in Australia and New Zealand. The survey was sent via email link to a survey hosted by Zoomerang™ to 49 physicians known to treat patients with pediatric rheumatic diseases and they were asked to forward the survey to any others who they knew saw children with rheumatic disease. RESULTS: Currently there is a shortfall in the pediatric rheumatology workforce of 68% based on minimum requirements and a shortfall of 225% based on an ideal scenario. CONCLUSION: Currently in Australia and New Zealand we fail to provide the level of care to children with pediatric rheumatic diseases comparable to other developed health economies worldwide. The current deficiency requires an increase in resource allocation to clinical service and speciality training to overcome this disparity and ensure children in Australia and New Zealand receive internationally recognized standards of care.

Children's Experiences of Epilepsy: A Systematic Review of Qualitative Studies.

CONTEXT: Epilepsy is a common and severe neurologic disease associated with increased mortality, seizure-related injury, and adverse psychological and quality-of-life outcomes. OBJECTIVE: To describe the perspectives of children and adolescents with epilepsy. DATA SOURCES: Medline, Embase, PsycINFO, and CINAHL from inception to August 2015. STUDY SELECTION: Qualitative studies on children's experiences of epilepsy. DATA EXTRACTION: Results from primary studies. We used thematic synthesis to analyze the findings. RESULTS: Forty-three articles involving 951 participants aged 3 to 21 years across 21 countries were included. We identified 6 themes: loss of bodily control (being overtaken, susceptibility to physical harm, fragility of the brain, alertness to mortality, incapacitating fatigue), loss of privacy (declarative disease, humiliating involuntary function, unwanted special attention, social embarrassment of medicine-taking), inescapable inferiority and discrimination (vulnerability to prejudice, inability to achieve academically, consciousness of abnormality, parental shame, limiting social freedom), therapeutic burden and futility (unattainable closure, financial burden, overwhelming life disruption, exhaustion from trialing therapies, insurmountable side effects, awaiting a fabled remission), navigating health care (empowerment through information, valuing empathetic and responsive care, unexpected necessity of transition, fragmented and inconsistent care), and recontextualizing to regain normality (distinguishing disease from identity, taking ownership, gaining perspective and maturity, social and spiritual connectedness). LIMITATIONS: Non-English articles were excluded. CONCLUSIONS: Children with epilepsy experience vulnerability, disempowerment, and discrimination. Repeated treatment failure can raise doubt about the attainment of remission. Addressing stigma, future independence, and fear of death may improve the overall well-being of children with epilepsy.

Patients' Perspectives and Experiences Living with Systemic Sclerosis: A Systematic Review and Thematic Synthesis of Qualitative Studies.

OBJECTIVE: Systemic sclerosis (SSc) is a chronic, progressive autoimmune disease with major end-organ involvement. Much attention has been focused on the management of physical and clinical manifestations; however, the effect of the disease and treatment on the patient's identity, relationships, functioning, and mental well-being are less known. We aimed to describe the patients' perspectives and experiences of living with SSc. METHODS: Electronic databases were searched to October 2014. Thematic synthesis was used to analyze the findings. RESULTS: We included 26 studies involving 463 patients. Six key themes were identified: distressing appearance transformation (disturbing facial changes, stigmatizing sickness, unrecognizable self), palpable physical limitations (bodily restrictions, frustrating mind-body disconnect, pervasive fatigue, disabling pain), social impairment (breaking intimacy, struggling to fulfill family responsibilities, maintaining work, losing independence), navigating uncertainty (diagnostic ambiguity, medically fending for oneself, unpredictable course of illness), alone and misunderstood (fearful avoidance of fellow patients, invisible suffering), and gradual acceptance and relative optimism (adapting to change and accepting limitations, taking a positive spin, cautious hoping, empowering relationships, valuing medical support). CONCLUSION: SSc is a rare and unpredictable illness that undermines patients' sense of certainty and control and impairs their self-image, identity, and daily functioning. Patient-centered care that encompasses strategies to promote self-esteem, resilience, and self-efficacy may help to improve treatment satisfaction and health and quality of life outcomes for patients with SSc.

Maternal stress associated with juvenile idiopathic arthritis.

OBJECTIVE: To assess parental stress levels of mothers of children with juvenile idiopathic arthritis (JIA) aged between 2-12 years and compare with those reported for other chronic childhood illnesses. METHODOLOGY: Mothers of children aged between 2-12 years with JIA were recruited from hospital-based outpatient clinics. Maternal stress was measured by using the Parenting Stress Index Long Form (PSI). The physician assessing the child completed an active joint count, a physician's global assessment and recorded the C-reactive protein and/or erythrocyte sedimentation rate if one was clinically indicated. RESULTS: The mothers recruited had children with a mean age of 6 years. The mean total stress score of mothers of children with JIA measured by the PSI was 235.4 (95% CI 218.5-252.3) was greater than the mean total stress scores for mothers of normal children at 222.8 (95% CI 221.4-224.2). It was also greater than children with other chronic disorders such as insulin-dependent diabetes mellitus (IDDM), 218.1 (95% CI 204.7-231.6) and profound deafness, 221.7 (95% CI 206.4-237.0). One third of mothers had total PSI scores that were in the clinical range (Total PSI > 260), indicating a need for intervention. CONCLUSION: JIA should be regarded as a significant illness in which maternal stress is at least equivalent to that associated with the care of children with other chronic diseases of childhood.

Children's experiences of cystic fibrosis: a systematic review of qualitative studies.

BACKGROUND AND OBJECTIVE: Cystic fibrosis (CF) is a common life-shortening genetic disease and is associated with poor psychosocial and quality of life outcomes. The objective of this study was to describe the experiences and perspectives of children and adolescents with CF to direct care toward areas that patients regard as important. METHODS: MEDLINE, Embase, PsycINFO, and Cumulative Index to Nursing and Allied Health Literature were searched from inception to April 2013. We used thematic synthesis to analyze the findings. RESULTS: Forty-three articles involving 729 participants aged from 4 to 21 years across 10 countries were included. We identified 6 themes: gaining resilience (accelerated maturity and taking responsibility, acceptance of prognosis, regaining control, redefining normality, social support), lifestyle restriction (limited independence, social isolation, falling behind, physical incapacity), resentment of chronic treatment (disempowerment in health management, unrelenting and exhausting therapy, inescapable illness), temporal limitations (taking risks, setting achievable goals, valuing time), emotional vulnerability (being a burden, heightened self-consciousness, financial strain, losing ground, overwhelmed by transition), and transplant expectations and uncertainty (confirmation of disease severity, consequential timeliness, hope and optimism). CONCLUSIONS: Adolescents and children with CF report a sense of vulnerability, loss of independence and opportunities, isolation, and disempowerment. This reinforces the importance of the current model of multidisciplinary patient-centered care that promotes shared decision-making, control and self-efficacy in treatment management, educational and vocational opportunities, and physical and social functioning, which can lead to optimal treatment, health, and quality of life outcomes.

Consumer perspectives on pediatric rheumatology care and service delivery: a qualitative study.

OBJECTIVES: This study aimed to elicit parental and adolescent perspectives on pediatric rheumatology care and service delivery and to describe the impact of this process on a proposed model of care addressing pediatric rheumatology service delivery. METHODS: Adolescents with juvenile idiopathic arthritis (JIA) and caregivers in New South Wales, Australia, participated in focus groups or semistructured interviews conducted from August to November 2011. Transcripts were coded and thematically analyzed. Changes to the model of care as a result of this process were identified. RESULTS: Thirty-seven parents and 13 adolescents participated. Five main themes were identified including (1) optimize service efficiency (promoting awareness about JIA, minimizing delays in diagnosis and treatment, continuity of care, holistic and integrated care, access to health care providers with JIA expertise, extending pharmacy services, regional outreach programs); (2) transitional care (trust and rapport with the adult rheumatologist, comprehensive information transfer of patient history); (3) psychosocial support (social and financial aid, mental health services, vocational guidance, peer support); (4) informational needs (medicine adverse effects, nutritional guidance, pain management, administering medications, obtaining medical equipment); and (5) school-based support and advocacy (to promote acceptance, provide academic assistance, facilitate environmental modifications, offer alternative activities). The model of care was extended to include consumer-focused concerns. CONCLUSIONS: A well-coordinated network of services, timely and accurate information about the illness, treatment and support services, adequate pharmacy support, and school-based advocacy are proposed to be needed to ensure pediatric rheumatology services that are accessible and responsive to the needs of patients and their families.

Children's experiences of living with juvenile idiopathic arthritis: a thematic synthesis of qualitative studies.

OBJECTIVE: To describe the experiences and perspectives of children and adolescents living with juvenile idiopathic arthritis (JIA). METHODS: We conducted a systematic review of qualitative studies that explored the experiences of children living with JIA. We searched electronic databases (to week 2 of July 2011) and reference lists of relevant articles. RESULTS: Twenty-seven studies that reported the experiences of more than 542 participants were included. Six major themes were identified: aversion to being different (unrelenting and unpredictable pain, disablement, internal disfigurement, differential treatment, and forced dependency on others); striving for normality (preserving social identity, resourcefulness, sense of community, focus on remission, and mastery over body and pain); stigma and misunderstanding (trivialization of disease, invisible pain, and discrimination); suspension in uncertainty (control versus powerlessness, hope versus disappointment); managing treatment (benefits of taking medicines, respect and involvement in health care, and motivation for physical therapy); and desire for knowledge (medical treatment and advances, lifestyle management). CONCLUSION: JIA disrupts a child's sense of normality and impairs his or her capacity for social participation. Children with JIA have a sense of being misunderstood and stigmatized, and they feel perpetually caught between having hope and control over their bodies and overwhelming pain and despair. To increase their confidence, the ability to manage pain, and their resourcefulness for self-management, children need ongoing information about treatments and lifestyle management, strong social support, community advocacy, and active involvement in their own health decision making.