Health-related quality of life changes of children and adolescents with chronic disease after participation in therapeutic recreation camping program.

BACKGROUND: The principals of therapeutic recreation underpin a camping program for children and adolescents living with chronic disease. This study aimed to evaluate the campers' health-related quality of life (HRQoL) before and after the program. METHOD: We used the Hungarian version of Kidscreen-52 questionnaire to assess HRQoL. The study sample (n = 115) consisted of children and adolescents aged 10-18 (Mean Age: 13,34; SD: 2,20) collected two months before and two months after camp with the following illnesses: oncology patients (n = 32), diabetes (n = 55) and juvenile immune arthritis (JIA) (n = 28). Repeated measures of multivariate analysis of variance (MANOVA) evaluated pre and post camp changes. We used the Reliable Change Index (RCI) to calculate all the 10 subscales of clinically significant changes. RESULTS: The Self-perception subscale showed significant positive change from pre camp to post camp with small effect size. Autonomy scores showed time related decline as well as significant time and age group interaction: children under 14 years of age showed a significant moderate effect size decrease on the Autonomy subscale. 32 children (27.8%) showed clinically significant improvement (RCI > 1.96) at least on one subscale. All positive changes were independent of the type of disease, age, gender, and previous camp experience. CONCLUSION: The therapeutic recreation camping program had a positive impact on HRQoL of children and adolescents living with cancer, diabetes mellitus and JIA. The experience enhanced their self-perception in all age groups and reduced the autonomy of children under 14 years of age. This study is an innovative use of the KIDSCREEN-52 questionnaire to measure the outcome effectiveness of a psychosocial rehabilitation program and to assess and compare HRQoL of children living with different chronic diseases.

Artrodiastase para o tratamento da rigidez do quadril na artrite reumatoide juvenil (ARJ): resultados preliminares / Arthrodiastasis for the treatment of stiffness of the hip in juvenile rheumatoid arthritis (JRA): preliminary results

OBJETIVO: Apresentar os resultados preliminares da utilização da artrodiastase do quadril em pacientes portadores de artrite reumatoide juvenil e com comprometimento da articulação coxofemoral. MÉTODOS: Estudo prospectivo de 12 pacientes (seis meninos e seis meninas) com idades entre oito e 18 anos (média de 10,5 anos). Foi utilizado um fixador externo monolateral que permite os movimentos de flexão e de extensão no quadril. O fixador externo foi mantido por um período que variou de 78 a 90 dias, com média de 86 dias. O controle radiográfico foi realizado durante o ato operatório e, semanalmente, durante o período de tração e a cada quatro semanas, quando terminado este período. Na avaliação clínica dos resultados, incluímos a graduação da dor e o grau de movimentação articular, com medidas e avaliações pré e pós-operatórias. O período de acompanhamento variou de 12 a 15 anos, com média de 13 anos. RESULTADOS: O valor médio da escala de dor foi de nove (9) antes da operação e de quatro (4) no período pós-operatório. Em dois pacientes não ocorreu melhora da dor. O arco de movimento do quadril aumentou em todos os pacientes, com exceção de dois. Na avaliação radiográfica evidenciamos um aumento no espaço articular de 2mm, em média, e que se manteve no pós-operatório. Não foram observadas complicações com a utilização da técnica. Apenas verificamos soltura dos pinos de Schanz da região do osso ilíaco em dois pacientes. A técnica operatória não ocasionou resultado satisfatório. CONCLUSÃO: O procedimento de artrodiastase está bem indicado para a recuperação da mobilidade em uma articulação coxofemoral comprometida e rígida, como ocorre em pacientes com artrite reumatoide juvenil.

OBJECTIVE: To present the preliminary results of the use of hip arthrodiastasis in patients with juvenile rheumatoid arthritis and involvement of the hip joint. METHODS: A prospective study of 12 patients (six boys and six girls) aged between eight and 18 years (mean 10.5 years). We used a monolateral external fixator that allows flexion and extension at the hip. The external fixator was maintained for a period ranging from 78 to 90 days, with a mean of 86 days. Radiographic control was performed during surgery, weekly during the traction period, and every four weeks once this period was completed. The clinical evaluation of results included the degree of the pain and the degree of joint movement, measured and evaluatedpre-and post-operatively. The follow-up period ranged from 12 to 15 years, with a mean of 13 years. RESULTS: The average pain score was nine (9) before surgery and four (4) in the postoperative period. There was no improvement in pain in two patients. The range of motion of the hip increased in all patients except two. Radiographic evaluation evidenced a2 mm increase in joint space, on average,that has remained postoperatively. There were no complications with this technique. Only a loosening of the Schanz screws in the region of the iliac bone was observed in two patients. The surgical technique did not bring satisfactory results. CONCLUSION: The arthrodiastasis procedure is well suited for recovery of mobility in animpairedand rigid hip joint, as occurs in patients with juvenile rheumatoid arthritis.

Major improvements in health-related quality of life during the use of etanercept in patients with previously refractory juvenile idiopathic arthritis.

OBJECTIVE: To evaluate changes in health-related quality of life (HRQoL) in patients with refractory juvenile idiopathic arthritis (JIA) who are being treated with etanercept. METHODS: 53 patients with JIA from seven Dutch centres were included. HRQoL was measured by the Childhood Health Assessment Questionnaire (CHAQ), Child Health Questionnaire (CHQ) and Health Utilities Index mark 3 (HUI3) at the start and after 3, 15 and 27 months of treatment. At the same time points the following JIA disease activity variables were collected; physician's global assessment through the visual analogue scale (VAS), number of active and limited joints and erythrocyte sedimentation rate. A statistical method linear mixed models was used to assess outcomes over time. RESULTS: During etanercept treatment both disease-specific and generic HRQoL outcomes improved dramatically. Significant improvements were shown after 3 months and these improvements continued at least up to 27 months of treatment. The disease-specific CHAQ, including VAS pain and wellbeing, showed a significant improvement in all domains. The generic health-profile measure CHQ improved for all the health concepts except for "family cohesion", which was normal. The generic preference-based HUI3 showed impairment and, subsequently, significant improvement in the more specific domains ("pain", "ambulatory", "dexterity"). In accordance disease activity variables also improved significantly over time. CONCLUSION: This study shows that the HRQoL of patients with refractory JIA can be substantially improved by the use of etanercept for all aspects impaired by JIA. Information on HRQoL is crucial to understand the complete impact of etanercept treatment on patients with JIA and their families.

Reabilitação em artrite idiopática juvenil / Rehabilitation in juvenile idiopathic arthritis

INTRODUÇÃO E OBJETIVOS: a artrite idiopática juvenil (AIJ) pode acarretar em seu curso clínico incapacidades físicas permanentes em crianças e adolescentes. Este estudo teve como objetivos a des-crição das diversas modalidades de reabilitação, desde a avaliação até a prescrição de exercícios, bem como a elaboração de um guia prático de reabilitação para pacientes com AIJ. FONTE DE DADOS: a pesquisa foi realizada nas bases de dados do Medline e do Lilacs. Na discussão dos diversos tópicos, foi considerada a experiência dos especialistas em reumatologia pediátrica e reabilitação do Lar Escola São Francisco e da Universidade Federal de São Paulo. RESUMO: os pacientes com AIJ podem apresentar dor e limitação da amplitude de movimento articular e conseqüente diminuição da capacidade física, com comprometimento das capacidades aeróbia e anaeróbia. Não só o comprometimento articular, mas as disfunções cardíacas e autonômicas colaboram nesse processo, tendo como conseqüência uma baixa capacidade de executar atividades esportivas e atividades de vida diárias (AVDs). O American College of Rheumatology recomenda 30 minutos de atividade com intensidade moderada de duas a três vezes por semana. A hidroterapia está relacionada a uma maior aderência ao tratamento, além de auxiliar na diminuição da percepção dolorosa e dificuldade apresentada na realização das AVDs. As outras modalidades de reabilitação, tais como massagem, educação, proteção articular, conservação de ener-gia e órteses, também são discutidas nesta revisão. CONCLUSÃO: há poucos estudos na literatura sobre reabilitação em crianças com AIJ, especialmente no que se refere a temas como prescrição adequada de exercícios, cargas, número de séries e repetições, bem como qual a melhor opção a ser utilizada - solo ou piscina. Acreditamos que mais estudos científicos são necessários para que possamos prescrever adequadamente os diversos tipos de exercícios.

INTRODUCTION AND AIMS: juvenile idiopathic arthritis (JIA) may cause permanent physical disabilities in children and adolescents. This study aimed to describe the several kinds of rehabilitation procedures, ranging from evaluation to prescription of exercises, as well as the elaboration of a practical rehabilitation guide for JIA patients. SOURCES OF DATA: the research was based on data from Medline and Lilacs. The opinion of experts working on the Pediatric Rheumatology service from Lar Escola São Francisco and Universidade Federal de São Paulo was considered on the debate of several topics. SUMMARY: JIA patients may present pain and limitation of joint movement thereby leading to decrease in physical capacity, affecting both aerobic and anaerobic activities. In addition to the joint compromise, cardiac and autonomic dysfunctions collaborate on this process, impairing sport and everyday activities. The American College of Rheumatology recommends 30-minute activity with moderate intensity, two to three times weekly. Hydrotherapy is associated to treatment adherence, besides helping in decreasing pain perception and adding to cope with daily activities. Other rehabilitation modalities, such as massage, education, joint protection, energy conservation, and splints are also considered in the present review. CONCLUSION: there are few studies in the literature focusing on rehabilitation in children with JIA. Particularly, there is a lack of studies concerning aspects of adequate prescription of exercises, weight-bearing, number of series and repetitions, as well as the best choice regarding ground or water activity. We believe that additional information is needed in order to improve the physical care to these patients.

Total hip arthroplasty in children with juvenile chronic arthritis: long-term results.

From 1984 to 2002, 20 total hip arthroplasties in children with juvenile chronic arthritis were performed in the authors' department. All patients had polyarticular disease. Average age of patients was 15.8 (range 13-24) years. Early onset of the disease occurred at an average age of 7.3 (4-10) years. In six patients the physes were open at the time of surgery. All patients had complete loss of joint space and various combinations of subchondral sclerosis, flattening of the femoral head, and anterior inclination of the neck. All patients used crutches for walking and joint function was very restricted. All patients were operated on under general anesthesia. One of them was supported additionally with laryngeal mask because of cervical spine involvement. No intra- or postoperative complications occurred. Mobilization of the patients started immediately after surgery, followed by a special rehabilitation program. Follow-up examination was based on the Merle d'Aubigne et Postel scale as modified by Charnley. Patients were followed at 6 weeks and 3 months after surgery and thereafter every 6 months with clinical and radiologic examination. Average follow-up was 9.2 (2-20) years. All patients had no pain and full functional ability. Although total hip arthroplasty is technically difficult in this age, it should be performed in specialized centers because pain relief, decreased deformity, and improved quality of life can be achieved in most patients. The only disadvantage is wear of the prosthesis.

A difference in perception of quality of life in chronically ill children was found between parents and pediatricians.

BACKGROUND AND OBJECTIVES: Quality of life measurements can help to estimate the well-being of chronically ill patients, and disclose discrepancies in perception between physicians and patients that might otherwise interfere with the effectiveness of treatment. The objective was to investigate the differences in perception of quality of life between parents of chronically ill children and pediatricians. METHODS: A cross-sectional study was conducted in four tertiary pediatric care centers in The Netherlands. The Health Utilities Index mark 3 (HUI3) was used by 37 pediatricians and 279 parents of patients (children aged 1 to 17 years) with cystic fibrosis admitted either in daycare or for a pneumonia, or patients with newly diagnosed acute lymphoblastic leukemia, juvenile idiopathic arthritis, or asthma. RESULTS: Differences in perception of quality of life between parents and pediatricians appeared to be dependent of the disease. In patients with acute lymphoblastic leukemia (OR 7.4; [95% CI 2.88-18.97], juvenile idiopathic arthritis (4.7; [95% CI 2.00-11.22]), and asthma (2.3; [95% CI 1.13-4.69]) a difference in perception was more likely to occur than in patients with cystic fibrosis admitted in daycare. CONCLUSION: At the onset of a chronic disease, the parents of pediatric patients may be misunderstood by health care professionals, especially in subjective attributes. Assessment of quality of life may contribute to better understanding between pediatricians and parents, and thus may even enhance compliance and treatment effects.

Quality of life in chronic illness: perceptions of parents and paediatricians.

AIMS: To investigate the differences in perception of quality of life between parents of chronically ill children and paediatricians at diagnosis and follow up. Quality of life was assessed using the (HUI3). METHODS: Longitudinal study (July 1999-January 2002) of 37 paediatricians and 181 parents of patients (children aged 1-17 years) with cystic fibrosis admitted for a pneumonia or patients with newly diagnosed acute lymphatic leukaemia, juvenile idiopathic arthritis, or asthma. Main outcome measure was percentage agreement on the attributes of the HUI3 between parents and paediatricians. RESULTS: Differences in perception of health and wellbeing between paediatricians and parents of children with a chronic disease were found, not only at diagnosis but also after a period of follow up. Differences were particularly clear in the subjective attributes emotion (range of agreement 28-68%) and pain/discomfort (range of agreement 11-33%). In all patient groups, at baseline and follow up, the paediatrician assessed the patient to have less pain/discomfort in comparison to the parents. Despite a prolonged patient- paediatrician relationship, differences at follow up did not decrease compared to baseline. CONCLUSION: At the onset of a chronic disease, but also after a period of follow up, quality of life of paediatric patients may be misunderstood by healthcare professionals, especially in the subjective attributes. Systematic assessment of quality of life may contribute to better understanding between physicians and parents.

Pediatric rheumatology: what does the future hold?

Effectiveness of the traditional rehabilitation approaches used in pediatric rheumatology has been difficult to prove and, in times of cost containment, this lack of evidence may lead to undertreatment with physical and occupational therapies. Quantitative methods such as those described in this issue by Broström and colleagues can be used to validate those approaches and to reinforce the need for careful attention to the effects of even minor loss of range and strength in children with juvenile arthritis. Historically, up to half of the children affected by polyarticular juvenile arthritis became disabled. Some factors that have led to improved outcomes for childhood rheumatic diseases are discussed, including medications (use of weekly low-dose methotrexate, intra-articular steroid injections, new biologic agents that specifically block mediators of inflammation, for example, tumor necrosis factor and interleukin-1), surgery (joint replacements), and psychosocial interventions (with schools and families). The importance of maintaining range of movement, strength, weight bearing, and ambulation, in an effort to prevent sequelae such as osteoporosis and wheelchair dependence, is emphasized. Early identification of children with rheumatic diseases and aggressive intervention, with a combined medical, rehabilitation, psychosocial, and, rarely, surgical approach, should now allow most affected children to reach adulthood with little or no disability.

Is it juvenile rheumatoid arthritis or fibromyalgia?

For the clinician evaluating adolescents with chronic musculoskeletal pain and fatigue, the distinctions between JRA and FS are clear based on physical examination findings. The two conditions can coexist. For the patient with an initial diagnosis of either JRA or FS whose clinical response to therapy is not in keeping with expectations or physical examination findings or whose clinical course worsens without explanation, reevaluation to determine if FS in the JRA patient has developed or JRA in the FS patient has emerged is warranted. Until clinicians have a better understanding of the intricacies of the neurohormonal and immunologic systems and how they affect somatic symptoms, they can continue to provide patients with a treatment plan based on current knowledge that should minimize patients' discomfort and allow them to have appropriately functional lives.

Juvenile rheumatoid arthritis and the spondyloarthropathies.

Juvenile rheumatoid arthritis (JRA) and the spondyloarthropathies affect approximately one in a thousand children under the age of 17 years and are more common than diabetes, cystic fibrosis, and many other chronic conditions. The author explores differential diagnoses for musculoskeletal pain, classifies types of JRA and the spondyloarthropathies, and describes how such conditions may be effectively managed with drug and physical rehabilitation therapy. The article also discusses behavioral, school, and vocational issues.

[The chronically ill child with rheumatoid arthritis]. / Das chronisch rheumakranke Kind.

Juvenile chronic arthritis (JCA) and other pediatric chronic rheumatic diseases such as the more rare juvenile collagenoses and the systemic vasculitides represent illnesses with a substantial potential of long-term problems and are additionally associated with a significant degree of mortality. Seen among the long-term problems associated with JCA are functional loss, deformities and the destruction of afflicted joints, a visual loss which may extend as far as blindness as a consequence of rheumatic iridocyclitis, irreversible organ damage caused by AA-amyloidosis as well as growth failure. Apart from cutaneous manifestations, on the other hand, the collagenoses and systemic vasculitides also demonstrate an involvement of specific organ systems, particularly of the kidneys, the lungs, the brain or the gastrointestinal tract. There is no causal therapy available. However, the application of a multidimensional therapeutic regimen primarily involving antirheumatic drugs, physiotherapeutic and ergotherapeutic methods as well as the application of a psychosocial treatment and the aid of governmental support, has resulted in good long-term results for most children suffering from JCA. The collagenoses and systemic vasculitides, however, have proven to be more problematic although the full utilization of the possibilities which are available today has provided encouraging results for most of these afflicted children. The complex therapeutic scheme, however, requires interdisciplinary cooperation. The pediatrician and/or family physician must fulfill crucial responsibilities which are relevant for the continuing prognosis of the illness. The pediatrician/general practitioner must thereby maintain a situation of close cooperation with the patient and their family as well as with the various colleagues active in the therapeutic team, especially with the pediatric rheumatologists. Moreover, these physicians also play a central role in establishing an early diagnosis in monitoring the disease progress and the therapy as well as in providing the mandatory assistance in the event of acute problems.

[The EHF therapy of children with juvenile rheumatoid arthritis]. / KVCh-terapiia detei s iuvenil'nym revmatoidnym artritom.

138 children with juvenile rheumatoid arthritis were exposed to EHF treatment. Three regimens of the microwave therapy were tested. The best results were achieved at the exposure of the thymic acupuncture points and most affected joint. Combination of microwave therapy with radon or effervescent sulfurated hydrogen baths is also recommended.

Involvement of the hip in juvenile rheumatoid arthritis. A longitudinal study.

Thirty-five children who had rheumatoid arthritis were followed for three to twenty-two years. Four categories of involvement of the hip were seen. In the first group, thirteen patients had mild disability and slight radiographic changes. In six of those patients the disease was in remission at the time of writing, and the patients were asymptomatic. In the second group, two patients had episodic disability that correlated with the activity of the disease. In the third group, fourteen patients had progressive disability and radiographic changes. Fifty-five of the fifty-nine procedures that were performed on the hip and knee in this series were done on those fourteen patients. In the fourth group, six patients had dramatic clinical and radiographic findings but, at the time of the latest follow-up, had little functional disability. In all six of these patients, the disease was in remission. In the children in this study, protrusio acetabuli was more cephalad than has been found in adults who have rheumatoid arthritis. All of the children had psychosocial problems, but they responded well to counseling. These problems influenced the timing of the surgical treatment.

Comparison of synovectomy and no synovectomy in patients with juvenile rheumatoid arthritis. A 24-month controlled study.

Thirty patients with pauciarticular or polyarticular juvenile rheumatoid arthritis entered a randomized, 24-month, parallel trial of synovectomy (n = 15) versus no synovectomy (n = 15). The joints studied were 18 wrists, 8 ankles and 4 knees. The synovectomies were performed as radically as possible. Joint motion was slightly decreased in the operated joints, especially for passive movements during the first 6 months following surgery. Swelling and disease activity of the joints studied as well as subjective evaluation of joint pain improved in the synovectomy group compared with the non-synovectomy group. This improvement seemed to continue for at least 2 years.

Physical and occupational therapy for children with rheumatic diseases.

Total management of rheumatic disorders of children includes antiinflammatory drugs, active therapy, maintenance of ADLs, and attention to the psychosocial development of the child. This article focuses on the role that physical and occupational therapists play in the management of children with arthritis. The complexity of the problems of these children necessitates a multidisciplinary team approach, with professionals who are committed to helping the child lead as normal a life as possible. This objective can be accomplished only by teaching families and school personnel how to manage the child's daily therapeutic needs.