Non-disease specific patient-reported outcome measures of health-related quality of life in juvenile idiopathic arthritis: a systematic review of current research and practice.

Juvenile idiopathic arthritis (JIA), as a chronic condition, is associated with symptoms negatively impacting health-related quality of life (HRQL). Regarding growing interest in the implementation of the patient-reported outcome measures (PROMs), we aimed to review the non-disease specific PROMs addressing HRQL assessment, potentially useful in the clinical care of JIA and daily practice. A systematic literature search was conducted using MEDLINE/PubMed, Google Scholar, Scopus and Embase databases (1990 to 2021), with a focus on the recent 5-years period. Entry keywords included the terms: "children", "adolescents", "JIA", "chronic diseases", "HRQL", "PROMs" and wordings for the specific tools. Several available PROMs intended to measure HRQL, non-specific to JIA, were identified. The presented outcomes differed in psychometric properties, yet all were feasible in assessing HRQL in healthy children and those with chronic diseases. Both EQ-5D-Y and PedsQL have already been tested in JIA, showing relevant reliability, validity, and similar efficiency as disease-specific measurements. For PROMIS® PGH-7 and PGH-7 + 2, such validation and cross-cultural adaptation need to be performed. Considering the future directions in pediatric rheumatology, the large-scale implementation of PROMIS® PGH-7 and PGH-7 + 2 in JIA offers a particularly valuable opportunity. The PROMs reflect the patient perception of the chronic disease and allow to understand child's opinions. The PROMs may provide an important element of the holistic medical care of patients with JIA and a standardized tool for clinical outcomes, monitoring disease severity and response to treatment.

Development and validation of the CAREGIVERS questionnaire: multi-assessing the impact of juvenile idiopathic arthritis on caregivers.

BACKGROUND: The primary caregiver is an important person in the life of patients with JIA. Their reactions depend on social, emotional and economic factors that affect the therapeutic alliance. Some generic instruments have been used to evaluate burden, anxiety, or quality of life of caregivers. This study aims to develop a specific instrument to measure the psychosocial and economic impacts on primary caregivers of patients with JIA. METHODOLOGY: This is a mixed methods research, that includes qualitative and quantitative data, and was carried out in two phases. First phase: a pragmatic qualitative study (questionnaire construction) was conducted in two parts, a non-systematic literature review followed by interviews with primary caregivers. Second phase: a cross-sectional study (questionnaire validation) to complete validation and estimate Cronbach's alphas based on tetrachoric correlation coefficients, correlation matrix and Cohen's kappa coefficient test. RESULTS: There were 38 articles found related to the experience of caregivers. 15 primary caregivers were interviewed (female 93%, median age 45 years). Thematic analysis identified 9 important topics from the perspective of participants (economic impact, coping, family roles, impact of diagnosis, mental health, couple/mate relationships, impact at work, religion, and knowledge of the disease). These topics were combined to create the interview questionnaire (56 items). Later, it was modified to 62 items that were divided into five dimensions: impact of the disease (psychosocial, economic, family, and relationships), knowledge of the disease, alternative medicine, future, and religion. The interview questionnaire was applied to 32 primary caregivers (female 93%, median age 37 years), results identify depression on 29 (90%), 18 (56%) feel sadness at diagnosis, 20 (63%) mentioned that JIA has influenced in their financial situation, 23 (72%) feel anxiety about the future, and 11 (37%) considered that their family relationships have changed. Statistical analysis identified inconsistencies during convergent and divergent validity of the construct. Consequently, 11 items were eliminated, 3 relocated, 6 modified, and 39 compacted obtaining the "Impact of Pediatric Rheumatic Diseases on Caregivers Multi-assessment Questionnaire" (CAREGIVERS questionnaire). This final version resulted on an eight-dimension (28 items) instrument. CONCLUSIONS: The CAREGIVERS questionnaire captures perspectives of both the participants and clinicians. It will be helpful to measure the impact of the disease and thus, to improve the quality of care of children with JIA and their families.

[Mentalization Based Treatment of an Adolescent Girl with Conduct Disorder]. / Mentalisierungsbasierte Therapie einer Jugendlichen mit Störung des Sozialverhaltens.

Mentalization Based Treatment of an Adolescent Girl with Conduct Disorder This paper will give a short overview on the theoretical concept of mentalization and its specific characteristics in adolescence. A previous study on Mentalization based treatment for adolescents (MBT-A) demonstrated the effectiveness of MBT-A for the treatment of adolescents with symptoms of deliberate self-harm (Rossouw u. Fonagy, 2012). Based on the results of this study Taubner, Gablonski, Sevecke, and Volkert (in preparation) developed a manual for mentalization based treatment for adolescents with conduct disorders (MBT-CD). This manual represents the foundation for a future study on the efficacy of the MBT-A for this specific disorder in young people. The present case report demonstrates the application of specific MBT interventions, as well as the therapeutic course over one year in a 16-year old girl who fulfilled all criteria of a conduct disorder. During the course of treatment, the de-escalating relationship-oriented therapeutic approach can be considered as a great strength of MBT-A, especially for patients with conduct disorders. The clinical picture, as well as the psychological assessment, showed a positive progress over the course of treatment. Despite frequent escalations, forced placements due to acute endangerment of self and others, and a precarious situation with the patient's place of residence towards the end of therapy, MBT-A treatment enabled the patient to continually use the evolved mentalizing capabilities as a resource.

Psychological therapies (remotely delivered) for the management of chronic and recurrent pain in children and adolescents.

BACKGROUND: Chronic pain is common during childhood and adolescence and is associated with negative outcomes such as increased severity of pain, reduced function (e.g. missing school), and low mood (e.g. high levels of depression and anxiety). Psychological therapies, traditionally delivered face-to-face with a therapist, are efficacious at reducing pain intensity and disability. However, new and innovative technology is being used to deliver these psychological therapies remotely, meaning barriers to access to treatment such as distance and cost can be removed or reduced. Therapies delivered with technological devices, such as the Internet, computer-based programmes, smartphone applications, or via the telephone, can be used to deliver treatment to children and adolescents with chronic pain. OBJECTIVES: To determine the efficacy of psychological therapies delivered remotely compared to waiting-list, treatment-as-usual, or active control treatments, for the management of chronic pain in children and adolescents. SEARCH METHODS: We searched four databases (CENTRAL, MEDLINE, EMBASE, and PsycINFO) from inception to June 2014 for randomised controlled trials of remotely delivered psychological interventions for children and adolescents (0 to 18 years of age) with chronic pain. We searched for chronic pain conditions including, but not exclusive to, headache, recurrent abdominal pain, musculoskeletal pain, and neuropathic pain. We also searched online trial registries for potential trials. A citation and reference search for all included studies was conducted. SELECTION CRITERIA: All included studies were randomised controlled trials that investigated the efficacy of a psychological therapy delivered remotely via the Internet, smartphone device, computer-based programme, audiotapes, or over the phone in comparison to an active, treatment-as-usual, or waiting-list control. We considered blended treatments, which used a combination of technology and face-to-face interaction. We excluded interventions solely delivered face-to-face between therapist and patient from this review. Children and adolescents (0 to 18 years of age) with a primary chronic pain condition were the target of the interventions. Each comparator arm, at each extraction point had to include 10 or more participants. DATA COLLECTION AND ANALYSIS: For the analyses, we combined all psychological therapies. We split pain conditions into headache and mixed (non-headache) pain and analysed them separately. Pain, disability, depression, anxiety, and adverse events were extracted as primary outcomes. We also extracted satisfaction with treatment as a secondary outcome. We considered outcomes at two time points: first immediately following the end of treatment (known as 'post-treatment'), and second, any follow-up time point post-treatment between 3 and 12 months (known as 'follow-up'). We assessed all included studies for risk of bias. MAIN RESULTS: Eight studies (N = 371) that delivered treatment remotely were identified from our search; five studies investigated children with headache conditions, one study was with children with juvenile idiopathic arthritis, and two studies included mixed samples of children with headache and mixed (i.e. recurrent abdominal pain, musculoskeletal pain) chronic pain conditions. The average age of children receiving treatment was 12.57 years.For headache pain conditions, we found one beneficial effect of remotely delivered psychological therapy. Headache severity was reduced post-treatment (risk ratio (RR) = 2.65, 95% confidence interval (CI) 1.56 to 4.50, z = 3.62,p < 0.01, number needed to treat to benefit (NNTB) = 2.88). For mixed pain conditions, we found only one beneficial effect: psychological therapies reduced pain intensity post-treatment (standardised mean difference (SMD) = -0.61, 95% CI -0.96 to -0.25, z = 3.38, p < 0.01). No effects were found for reducing pain at follow-up in either analysis. For headache and mixed conditions, there were no beneficial effects of psychological therapies delivered remotely for disability post-treatment and a lack of data at follow-up meant no analyses could be run. Only one analysis could be conducted for depression outcomes. We found no beneficial effect of psychological therapies in reducing depression post-treatment for headache conditions. Only one study presented data in children with mixed pain conditions for depressive outcomes and no data were available for either condition at follow-up. Only one study presented anxiety data post-treatment and no studies reported follow-up data, therefore no analyses could be run. Further, there were no data available for adverse events, meaning that we are unsure whether psychological therapies are harmful to children who receive them. Satisfaction with treatment is described qualitatively.'Risk of bias' assessments were low or unclear. We judged selection, detection, and reporting biases to be mostly low risk for included studies. However, judgements made on performance and attrition biases were mostly unclear. AUTHORS' CONCLUSIONS: Psychological therapies delivered remotely, primarily via the Internet, confer benefit in reducing the intensity or severity of pain after treatment across conditions. There is considerable uncertainty around these estimates of effect and only eight studies with 371 children contribute to the conclusions. Future studies are likely to change the conclusions reported here. All included trials used either behavioural or cognitive behavioural therapies for children with chronic pain, therefore we cannot generalise our findings to other therapies. However, satisfaction with these treatments was generally positive. Larger trials are needed to increase our confidence in all conclusions regarding the efficacy of remotely delivered psychological therapies. Implications for practice and research are discussed.

Medical clowns facilitate nitrous oxide sedation during intra-articular corticosteroid injection for juvenile idiopathic arthritis.

BACKGROUND: Intra-articular corticosteroid injection (IACI), a common procedure in juvenile idiopathic arthritis, is usually associated with anxiety and pain. In a previous study, we concluded that nitrous oxide (NO2) provides effective and safe sedation for such procedures. Following the introduction of medical clowns in our hospital, we added them as an integral part of the team performing IACI. OBJECTIVES: To prospectively evaluate the effect of a medical clown on pain perception during intra-articular corticosteroid injection for juvenile idiopathic arthritis using NO2 conscious sedation. METHODS: Patients scheduled for IACI first met and interacted with the medical clown. During the procedure, the rheumatologist and the medical clown worked in parallel to create distraction. NO2 was administered. The patient, parent, physician, medical clown and nurse completed a visual analog scale (0-10) for pain. Change in heart rate ≥ 15% was recorded to evaluate physiologic response to pain and stress. RESULTS: A total of 46 procedures were performed in 32 children: 23 girls, 9 boys, with a mean age of 10.9 ± 3.6 years. The median visual analog scale pain score for the patients, parents, physicians, medical clown and nurses was 2, 2, 1, 1 and 1, respectively. Five patients had increased heart rate and experienced increased pain. CONCLUSIONS: Active participation of a medical clown during IACI with nitrous oxide for juvenile idiopathic arthritis further decreases pain and stress and results in a positive patient experience.

[Medical clowns--dream doctors as important team members in the treatment of young children with juvenile idiopathic arthritis].

Juvenile idiopathic arthritis (JIA) is the most common chronic rheumatic disease in children. An intra-articular corticosteroid injection (IAS), one of the cornerstones of treatment for this disease, is usuaLLy associated with anxiety and pain. A major part of the success in reducing the pain is associated with the level of the child's anxiety even before starting the procedure. This is a case study of a 5 year old girl with JIA, who has been treated with an intra-articular corticosteroid injection to her knee joint. The case study is presented from the point of view of the medical clown, who is an important integral part of the team of the IAS procedure. In this article we will discuss the participation of medical clowns in the treatment of children in general, and in the IAS procedure in particular. The importance of the subject stems from the fact that it has been proven that the presence of medical clowns significantly alleviates the children's anxiety and pain. This study, as well as others on this subject, shows that we should encourage medical clowns as an integraL part of the treatment of children.

[Stress and rheumatoid arthritis]. / Stress und Rheuma.

Rheumatoid arthritis (RA) is a chronic rheumatic disease of unknown aetiology and variable severity. It is now well known that several risk factors are involved in its pathogenesis, including genetic factors and sex hormones as well as environmental factors, i.e. infections and stress. In particular stress is now recognised as an important risk factor for the onset and even more for the modulation of disease activity in RA. Many studies have clearly shown that chronic mild stress (family or professional stress) may lead to proinflammatory effects, increasing disease activity. Furthermore, a positive correlation between the stress level at the onset of RA and radiological progression could be demonstrated. The onset of RA was associated with moderate stress at work, underlining the possible interactions between the various stress systems and the immune system. In this respect it could be demonstrated that coping strategies reduce stress episodes and change stress management with a positive impact on disease activity in RA. However, more studies are warranted to further explore the pathophysiological implications of stress on onset and activity of chronic autoimmune diseases.

A qualitative study of fitness instructors' experiences leading an exercise program for children with juvenile idiopathic arthritis.

Children with arthritis face challenges when they try to increase their physical activity. The study's objective was to identify elements of a successful community-based exercise program for children with arthritis by investigating the perspectives of fitness instructors who led the program. This qualitative study used a phenomenological approach. Four fitness instructors participated in individual interviews. Themes were developed through inductive analytic methods. Three main themes were identified: (a) children with arthritis require encouragement and guidance throughout the program from fitness instructors who understand their arthritis, and support from parents and peers; (b) children need help to overcome their negative perceptions about exercise; and (c) exercise program participation can launch the adoption of a more active lifestyle. Pediatric physiotherapists can encourage the establishment of successful exercise programs for children with arthritis in nonmedical or community environments through the formation of supportive, education-based partnerships with community-based fitness instructors.

Late consequences of chronic pediatric illness.

There are many challenges in coping with and adapting to life with a chronic disease, and increased survival cannot be assumed to be associated with increased quality of life. A recent systematic review shows there is wide variation in outcomes depending on the definitions and measurements used to estimate the prevalence of chronic health conditions, making the impact of disability on children's health and social functioning difficult to assess; various authors have called for an international consensus about the conceptual definition of chronic health conditions in childhood. It frequently is difficult to determine if problems in psychosocial functioning are caused by the underlying illness, by treatment, or by the resultant effects of either illness or treatment on physical growth or cognitive development. Assessment and treatment of mental health should be an integral component of the comprehensive care of chronically ill children and adolescents. Transition of care is an important process that addresses significant changes from child-oriented to adult-oriented care. Adults who have chronic health conditions should continue to be evaluated periodically for late consequences of the childhood illness and early medical care, and attention should be paid to their ongoing psychosocial, psychiatric, educational, and vocational needs.

Brief report: use of complementary and alternative medicine and psychological functioning in Latino children with juvenile idiopathic arthritis or arthralgia.

OBJECTIVE: To describe the use of complementary and alternative medicine (CAM) and its relationship to symptoms of anxiety, depression, and dysthymia in Latino children with juvenile idiopathic arthritis (JIA) or arthralgia. METHODS: Parents of 36 children between the ages of 6 and 16 years with either JIA (n = 17) or arthralgia (n = 19) completed questionnaires during routine pediatric rheumatology clinic visits assessing use of CAM and psychological functioning. RESULTS: CAM was used by the majority of children primarily to treat pain episodes. The most common modalities were prayer and massage therapy. CAM use was associated with decreased symptoms of anxiety and dysthymia in children with arthralgia, but not in children with JIA. CONCLUSION: Preliminary findings suggest that CAM use is associated with improved psychological functioning in children with arthralgia. Healthcare providers are encouraged to routinely screen for CAM usage and to educate families about the potential benefits and limitations of CAM.

The impact of a coordinated transitional care programme on adolescents with juvenile idiopathic arthritis.

OBJECTIVE: There is an extensive evidence base for the need of transitional care, but a paucity of robust outcome data. The aim of the study was to determine whether the quality of life of adolescents with juvenile idiopathic arthritis (JIA) could be improved by a co-ordinated, evidence-based programme of transitional care. METHODS: Adolescents with JIA aged 11, 14 and 17 yrs and their parents were recruited from 10 rheumatology centres in the UK. Data were collected at baseline, 6 and 12 months including core outcome variables. The primary outcome measure was health-related quality of life (HRQL): Juvenile Arthritis Quality of Life Questionnaire (JAQQ). Secondary outcome measures included: knowledge, satisfaction, independent health behaviours and pre-vocational experience. RESULTS: Of the 359 families invited to participate, 308 (86%) adolescents and 303 (84%) parents accepted. A fifth of them had persistent oligoarthritis. Median disease duration was 5.7 (0-16) yrs. Compared with baseline values, significant improvements in JAQQ scores were reported for adolescent and parent ratings at 6 and 12 months and for most secondary outcome measures with no significant deteriorations between 6 and 12 months. Continuous improvement was observed for both adolescent and parent knowledge with significantly greater improvement in the younger age groups at 12 months (P = 0.002). CONCLUSIONS: This study represents the first objective evaluation of an evidence-based transitional care programme and demonstrates that such care can potentially improve adolescents' HRQL.

[Stress, hormones, and neuronal signals in the pathophysiology of rheumatoid arthritis. The negative impact on chronic inflammation]. / Stress, Hormone und neuronale Signale bei rheumatoider Arthritis Der negative Einfluss auf das chronische Entzündungsgeschehen.

This review demonstrates that hormonal and neuronal factors, which are released during stressful situations, can unfavorably influence the two chronic diseases of rheumatoid arthritis and juvenile idiopathic arthritis. Noradrenaline and cortisol are in the focus of this review. In the said two chronic inflammatory diseases, it is obvious that apart from the immune system also the endocrine and nervous system play an essential role in the stress-induced initiation and aggravation of these diseases.

Factors associated with the use of complementary and alternative medicine in juvenile idiopathic arthritis.

OBJECTIVE: To describe the frequency of complementary and alternative medicine (CAM) use in patients with juvenile idiopathic arthritis (JIA) and to explore whether CAM was associated with patient-specific characteristics, parent-specific characteristics, and medical management factors. METHODS: Parents of children with JIA completed questionnaires that addressed the use of CAM, adherence and perceived problems, parental distress, and coping with childhood illness. Clinical variables were abstracted from the patients' medical files. RESULTS: One hundred-eighteen children with JIA, mean +/- SD age of 10.4 +/- 3.9 years and mean +/- SD disease duration of 4.5 +/- 3.5 years, participated in the study. Ever use of CAM was 33.9% and was higher in patients whose parents used CAM (odds ratio [OR] 5.1, 95% confidence interval [95% CI] 1.4-19.5) and among those who considered themselves as "Canadian" as opposed to belonging to a specific ethnic group (OR 10. 7, 95% CI 1.2-99.8). Adherence to conventional treatment was high for both users and nonusers of CAM. CONCLUSION: Use of CAM is common among patients with JIA. CAM use is not related to any decrease in adherence to conventional medical treatment.

[Rheumatic diseases in children as an important specialty. Bone marrow transplantation is the hope for the most severely ill patients]. / Reumatiska sjukdomar hos barn viktigt specialområde. Benmärgstransplantation ger ökat hopp för de svårast sjuka.

Chronic inflammatory joint disorders can be viewed as a spectrum of disease presenting one set of characteristics during childhood and another in adulthood. Few of the disorders are specific for children of a specific age, even if some conditions might be very rare in certain age groups. Juvenile idiopathic arthritis is a new name suggested for pediatric chronic inflammatory joint disorders. Drug therapy is based on the same principles as for adults: an aggressive approach with NSAID-drugs and low dose methotrexate in combination with local steroid injections. New drugs like TNF-blockade and COX-2 inhibitors are almost untested in children, with the exception of etanercept which has been studied in children with polyarticular disease and proven to be very effective. For rare cases with very severe joint disease hematopoietic stem cell transplantation is under evaluation.

Juvenile rheumatoid arthritis: benefits from massage therapy.

Studied children with mild to moderate juvenile rheumatoid arthritis who were massaged by their parents 15 minutes a day for 30 days (and a control group engaged in relaxation therapy). The children's anxiety and stress hormone (cortisol) levels were immediately decreased by the massage, and over the 30-day period their pain decreased on self-reports, parent reports, and their physician's assessment of pain (both the incidence and severity) and pain-limiting activities.

Pain associated with juvenile rheumatoid arthritis.

Despite a comprehensive approach to the treatment of JRA patients, pain remains a largely underdeveloped area of research and an undertreated clinical problem. Pain is a highly prevalent condition in JRA patients, one that in adults has been shown to be highly predictive of current medication usage and future disability. This recurrent or chronic pain syndrome is currently treated primarily with nonsteroidal anti-inflammatory drugs, but inclusion of pain management specialists on the interdisciplinary team will allow for greater use of nonpharmacologic means of pain management.