Physical and mental quality of life in adult patients with haemophilia in Belgium: the impact of financial issues

In Belgium, where haemophilia affects approximately 1:7000 people (2011), data on patients' quality of life (QoL) is scarce. This project aims to assess physical and mental QoL (P‐QoL and M‐QoL) simultaneously, and to analyse the influence of different variables on these two aspects of QoL. After Ethics Committee approval, we contacted 84 adult haemophilia A (HA) and haemophilia B (HB) patients, without current inhibitors, on replacement therapy (on‐demand or secondary prophylaxis), regularly followed up at our comprehensive treatment centre. Seventy‐one (n = 59 HA, n = 12 HB) replied to our questionnaire, which included the SF36v2 QoL assessment forms. We analysed two groups of variables: one including variables previously associated with decreased QoL, and another including variables with unclear impact on QoL (e.g. patients' understanding of haemophilia‐related issues, economical concerns). In our population (mean ± SD age: 45.2 ± 14.7 years old), P‐QoL appeared more reduced than M‐QoL. P‐QoL was strongly influenced by the number of arthropathies while M‐QoL was primarily affected by patients' concern of personal costs due to haemophilia. Among this latter group, having knowledge of insurance coverage had a positive impact on M‐QoL. Scores did not depend on haemophilia type. QoL was impaired in our haemophilia patients. A simultaneous assessment of P‐QoL and M‐QoL confirmed the benefit of primary prophylaxis in P‐QoL, while originally pointing out the major burden of patients' concerns and poor understanding of haemophilia‐related economical issues on their M‐QoL. This might become a particularly challenging issue in times of financial crisis.     

Using the Haemophilia Joint Health Score for assessment of teenagers and young adults: exploring reliability and validity

Outcome assessment in haemophilia is important to assess results of prophylactic treatment. Recently, the Haemophilia Joint Health Score (HJHS) was developed to assess early joint damage in children with haemophilia. Thus, the aim of this study was to assess reliability and explore validity of the HJHS in teenagers and young adults with haemophilia. Twenty‐two patients with haemophilia (mean age 20.4, range 14–30, including 15 severe) were assessed by the HJHS1.0, Haemophilia Activities List (HAL), SF36 and self‐evaluation was performed using a Visual Analogue Scale (VAS) scale. A subset of 12 patients were assessed by three physiotherapists to establish interobserver reliability (intraclass correlation coefficient: ICC). Total HJHS1.0 scores were calculated without overall global gait. Validity was explored by the assessment of Pearson's correlation with all outcome parameters and recent Pettersson scores. Overall outcome was good, with median HJHS score of 5.5 of a maximum 144 (range 0–34), median patients' VAS of 96.5 and maximum scores for HAL and SF36 physical functioning for the majority of patients. Pettersson scores were low (median 3.5 of 78, N = 18). Interobserver reliability was good (ICC 0.84), with limits of agreement of ±7.2 points. ICC was unaffected by different score calculation methods. Exploration of validity in 22 patients showed weak correlations of HJHS scores with patients' VAS (0.33) and HAL (?0.40) and strong correlations with SF36‐PF (?0.66) and Pettersson scores (0.86). These results suggest that interobserver reliability of the HJHS1.0 in teenagers and young adults with limited joint damage is excellent. Preliminary data on validity were similar or better than those in children.     

Point‐of‐care musculoskeletal ultrasound is critical for the diagnosis of hemarthroses,inflammation and soft tissue abnormalities in adult patients with painful haemophilic arthropathy

We previously demonstrated in adult patients with haemophilia (PWH) that hemarthrosis is present in only ~1/3rd of acutely painful joints by using point‐of‐care‐musculoskeletal ultrasound (MSKUS). Therefore, other unrecognized tissue abnormalities must contribute to pain. Using high resolution MSKUS, employing grey scale and power Doppler, we sought to retrospectively (i) investigate soft tissue abnormalities in painful haemophilic joints and (ii) to determine to what extent MSKUS findings, functional or radiographic joint scores correlate with biomarkers of inflammation in PWH. Findings were correlated with Hemophilia Joint Health Scores (HJHS), Pettersson scores, high sensitivity C‐reactive protein and von Willebrand factor activity and antigen levels. A total of 65 MSKUS examinations for acute and chronic joint pains were performed for 34 adult haemophilia patients, mostly for chronic joint pains (72.3%). The most prominent findings (66.5%) pertained to inflammatory soft tissue changes including synovitis, tendinitis, enthesitis, bursitis and fat pad inflammation. Effusions were present in 55.5% and 46.8% of MSKUS performed for acute and chronic pain, respectively. Of those, 90.0% were bloody during acute and 47.6% during persistent pains. While inflammatory biomarkers correlated well with overall HJHS and total Pettersson scores (P < 0.05), they did not differ between those patients with synovitis and those without. MSKUS is emerging as an important modality to diagnose treatable musculoskeletal abnormalities contributing to pain in haemophilic arthropathy, and therefore seems critical for a personalized approach to haemophilia care. The role of biomarkers in this setting remains less clear and requires further investigation.     

Interpreting important health‐related quality of life change using the Haem‐A‐QoL

The Haemophilia Quality of Life Questionnaire for Adults (Haem‐A‐QoL) measures health‐related quality of life (HRQoL) in adults with haemophilia; however, change score thresholds for identifying individuals experiencing a HRQoL benefit have not been appropriately investigated. The objective of this analysis was to derive appropriate HRQoL responder definitions (RDs) for two Haem‐A‐QoL domains that reflect key impairments, ‘Physical Health’ and ‘Sports & Leisure,’ and the Haem‐A‐QoL ‘Total Score’ using anchor‐ and distribution‐based methods. In this analysis, data from adults in A‐LONG and B‐LONG, two Phase 3 clinical studies of rFVIIIFc in haemophilia A and rFIXFc in haemophilia B, respectively, were used. The anchor‐based approach identified Haem‐A‐QoL changes corresponding to EQ‐5D item improvements between baseline and 6 months; the distribution‐based methods examined the magnitude at baseline of one‐half standard deviation and the standard error of measurement. Through triangulation, the most appropriate RDs were derived. Of the 133 A‐LONG and 73 B‐LONG subjects with baseline Haem‐A‐QoL scores, 67 and 51 subjects, respectively, completed the Haem‐A‐QoL questionnaire at both baseline and 6 months follow‐up. Triangulation of anchor‐ and distribution‐based estimates with the observed Haem‐A‐QoL change scores identified a 10‐point reduction in the ‘Physical Health’ and ‘Sports & Leisure’ domains, and a 7‐point reduction in ‘Total Score’ as the RD thresholds most indicative of HRQoL benefit. These empirically derived RDs for two key Haem‐A‐QoL domains and ‘Total Score’ are reasonable and practical thresholds for identifying subjects with notable improvements in HRQoL, and provides HRQoL RDs that can be used for further analysis and interpretation of data from haemophilia clinical trials.     

Improvement in health‐related quality of life with recombinant factor IX prophylaxis in severe or moderately severe haemophilia B patients: results from the BAX326 Pivotal Study

Little is known about the health‐related quality of life (HRQoL) burden of haemophilia B. The aim of this study was to assess HRQoL burden of haemophilia B, the benefit of recombinant factor IX (rFIX) prophylaxis and the HRQoL benefit of achieving a zero annual bleed rate. Subjects receiving rFIX (BAX326) prophylaxis or on‐demand completed the SF‐36 survey. Baseline SF‐36 scores were compared to the general US population scores to understand the HRQoL burden. Changes in SF‐36 scores between baseline and follow‐up were tested using t‐tests. Subgroup analysis was conducted to examine SF‐36 change among subjects who switched to BAX326 prophylaxis. SF‐36 scores were also compared between those with zero bleeds and those who bled during the study. Compared to the US norms, subjects reported lower average scores in all physical and several mental HRQoL domains. At follow‐up, prophylaxis subjects reported statistically significant and clinically meaningful improvements in overall physical HRQoL, as measured by the Physical Component Score (PCS) (mean change 2.60, P = 0.019), Bodily Pain (BP) (3.45, P = 0.015) and Role Physical (RP) domains (3.47, P = 0.016). Subjects who switched to prophylaxis from intermittent prophylaxis or on‐demand experienced more pronounced improvements not only in the PCS (3.21, P = 0.014), BP (3.71, P = 0.026), RP (4.43, P = 0.008) but also in Vitality (3.71, P = 0.04), Social Functioning (5.06, P = 0.002) and General Health domains (3.40, P = 0.009). Subjects achieving zero bleeds reported lower BP (P = 0.038). Prophylaxis with BAX326 significantly improved HRQoL in patients with moderately severe or severe haemophilia B by reducing bleeds.     

Effects of haemophilic arthropathy on health-related quality of life and socio-economic parameters

Summary. Although prophylactic treatment is advised for all children with severe haemophilia, the optimal regimen is still under discussion. Should all joint bleeds be prevented, or can a limited amount of arthropathy be tolerated in adulthood without loss of quality of life? To answer this question, the effect of haemophilic arthropathy on health‐related quality of life (HRQoL) needs to be quantified. In a retrospective study, the effect of arthropathy on HRQoL and socio‐economic parameters was assessed in a single‐centre cohort of 96 patients with severe and moderate haemophilia with a minimum age of 13 years. Arthropathy was measured by the radiological Pettersson score of the elbows, knees and ankles (maximum: 78 points). HRQoL was assessed by the Short Form 36 (SF36), measuring eight domains of health. Labourforce participation and medical consumption were assessed using a separate questionnaire. Patients were studied at a mean age of 28.6 years (range: 13–54), the mean time between evaluation and the last Pettersson score was 0.4 years (SD: 1.1). The overall median Pettersson score was 13 (range: 0–78). There was a trend towards lower quality of life with increasing Pettersson scores and age, especially in the physical domains of the SF36. An age‐adjusted analysis showed that arthropathy had a small but significant effect on HRQoL in the domain of ‘physical function’ of the SF36, but not on its other domains, or on labourforce participation and medical consumption. Thus suggesting that the SF36 can be used to assess the effects of haemophilic arthropathy, especially in the domain of ‘physical function’.     

Assessment of pain, fatigue, sleep and quality of life (QoL) in elderly hospitalized cancer patients

As the proportion of older adults in the population continues to grow, the number of patients with cancer is expected to increase proportionally. In the previously conducted studies, data on elderly cancer patients were generally compared with the QoL scores of elderly patient group and with the data of non-cancer individuals. The purpose of this study was to examine differences in reported pain, fatigue, sleep problems and QoL between middle-aged and elderly hospitalized patients with cancer. We included 53 middle-aged (between 18 and 50 years) hospitalized cancer patients and 47 elderly (>60 years) hospitalized cancer patients in this study. Pain (visual analog scale = VAS, verbal pain rating), fatigue (brief fatigue inventory = BFI), sleep problems, QoL (Short Form 36 = SF36), and European Organization for Research and Treatment of Cancer (EORTC)-QoL-C30 data were gathered using standardized measures. In the elderly group, no significant difference was detected in terms of VAS, verbal pain rating, fatigue, fatigue type, sleep problems and QoL scores (p > 0.05). When the two age groups were compared, BFI scores were found to be significantly high among the elderly patients (p < 0.05). A significant relationship was observed in both age groups between the scores of pain, fatigue and sleep problems, and QoL (p < 0.05). Elderly hospitalized cancer patients did not demonstrate a distinctive difference in terms of pain, sleep and QoL compared to the younger group. The relationship between pain, fatigue, sleep and QoL should be definitely kept in mind in clinical practice.     

Cross‐cultural development and psychometric evaluation of a patient‐reported health‐related quality of life questionnaire for adults with haemophilia

Summary. Co‐morbidities of haemophilia, such as arthropathy and blood‐borne infections, can adversely affect the quality of life of adult patients with haemophilia. The purpose of this study was to develop and validate a haemophilia‐specific health‐related quality of life questionnaire for adults (HAEMO‐QoL‐A). Subjects with varying severities of haemophilia completed the HAEMO‐QoL‐A at baseline and 4 weeks. Other assessments included the SF‐36 and Health Assessment Questionnaire – Functional Disability Index (HAQ‐FDI). Two‐hundred and twenty‐one participants completed the 41‐item HAEMO‐QoL‐A covering six domains (Physical Functioning, Role Functioning, Worry, Consequences of Bleeding, Emotional Impact and Treatment Concerns) and four independent items. Internal consistency was good‐to‐excellent (Cronbach’s α‐range: 0.75–0.95). Test–retest reproducibility was good, with intraclass correlation coefficients >0.80 except for the Emotional Impact domain (0.79). Concurrent validity between the HAEMO‐QoL‐A total and subscale scores and all SF‐36 subscale scores were generally good (correlations range: 0.13–0.87). Significant correlations between the HAEMO‐QoL‐A and the HAQ‐FDI ranged from ?0.14 to ?0.69. There were non‐significant correlations with the Treatment Concerns subscale and with the Worry subscale. The HAEMO‐QoL‐A discriminated significantly between adults with haemophilia by severity and HIV status. The Physical Functioning subscale discriminated between patients receiving prophylactic or on‐demand therapy. The HAEMO‐QoL‐A is a valid and reliable instrument for assessing quality of life in haemophilia patients.     

Social determinants and health‐related dimensions of quality of life in adult patients with haemophilia

The availability of safe and effective factor replacement therapies, in persons with haemophilia (PWH), has in some countries answered the basic need for treatment of these patients. The findings suggest that adult patients who have always been on prophylaxis reported significantly better physical functioning, and thus better quality of life. This study is designed to evaluate the QoL in adult PWH, by focusing on social determinants of QoL and their relationship with health‐related dimensions, in Tabriz, Iran. The survey instrument was a self‐report 36 items questionnaire, ‘A36 Hemofilia – QoL’, which is a disease‐specific questionnaire for the assessment of the health‐related QoL in adults living with haemophilia. A total of 100 haemophilia A and B patients, aged over 17 years participated in this study within 1 year. QoL total score was 71.88 (±26.89 SD). Patients who treat in our Hemophilia Treatment Center, had better QoL score (P = 0.000), and education has a significant impact on the social aspects of QoL (P = 0.18). The QoL was very poor in urban area in contrast to patients who lived in the city (54.45 vs. 74.21 respectively). Single patients have a better QoL than married patients (76.56 vs. 68.50 respectively). Our results showed that low education and lack of awareness of the diseases among PWH lead to reduce of QoL and more disease complications. More and wider treatment and psychological care for improving quality of life of these patients are seriously recommended.     

Cross-cultural validation of the CHO-KLAT and HAEMO-QoL-A in Canadian French

Summary. Multi‐site studies are necessary in the field of haemophilia to ensure adequate sample sizes. Quality of life (QoL) instruments need to be harmonized across languages and cultures to facilitate their inclusion. The purpose of this study was to adapt the Canadian Haemophilia Outcomes – Kids Life Assessment Tool (CHO‐KLAT^©) and HAEMO‐QoL‐A^© to French for Canada. The CHO‐KLAT and the HAEMO‐QoL‐A are haemophilia‐specific measures of QoL for boys and men respectively. Both measures originated in English, were translated into Canadian French by clinicians with expertise in haemophilia care, back‐translated by expert translators and harmonized by a multi‐disciplinary team. The harmonized versions were evaluated through a cognitive debriefing process with 6 boys with haemophilia, their parents and 10 men with haemophilia. The final versions were validated in a sample of 19 boys with haemophilia, 19 parents, and 22 men with haemophilia along with a generic QoL scale: the PedsQL for children; and the SF‐36 for adults. The translation and cognitive debriefing processes resulted in a preliminary version that maintained the intent of the original questions. The validation study estimated the mean score for the child‐reported CHO‐KLAT at 71.9 (SD 10.4), and the adult‐reported HAEMO‐QoL‐A at 79.1 (SD 21.3). The CHO‐KLAT correlated 0.64 with the PedsQL and the HAEMO‐QoL‐A correlated 0.78 with the SF‐36 physical component summary score. The French‐Canadian version of the CHO‐KLAT and HAEMO‐QoL‐A are valid. These measures are available for use in multi‐site haemophilia trials and clinical practices to capture QoL data from French Canadians.     

Comparison of ultrasound and magnetic resonance imaging for diagnosis and follow‐up of joint lesions in patients with haemophilia

Haematomas and recurrent haemarthroses are a common problem in haemophilia patients from early age. Early diagnosis is critical in preventing haemophilic arthritis, and recent years have seen excellent advances in musculoskeletal ultrasound as a diagnostic tool in soft tissue lesions. In this study, we compared the results of ultrasound imaging for the diagnosis of musculoskeletal injuries in haemophilia patients with scores obtained using magnetic resonance (MRI) scans. A total of 61 haemophilia patients aged 4–82 years were included in this study. Both knees and ankles of each patient were assessed using the Gilbert (clinical assessment) and Pettersson scores (X‐ray assessment). Patients with severe haemophilia (n = 30) were examined using ultrasound and MRI (Denver scoring system). Results obtained with ultrasound and MRI in severe patients were correlated using the Pearson test. In patients with severe haemophilia, normal joints were similarly assessed with MRI and ultrasound (κ = 1.000). By component of joint assessment, haemarthrosis was similarly diagnosed with both techniques in all joints (κ = 1.000). A good positive correlation was found between these techniques in detecting and locating synovial hyperplasia (κ = 0.839–1.000, knees and ankles respectively), and erosion of margins (κ = 0.850–1.000). The presence of bone cysts or cartilage loss was better detected with MRI (κ = 0.643–0.552 for knees and ankles, and κ = 0.643–0.462 respectively). Ultrasound is useful in detecting joint bleeds, synovial hyperplasia and joint erosions, with results comparable to those of MRI. A quick and affordable technique, ultrasound imaging may be useful for monitoring joint bleeds and structure normalization and maintenance in routine practice.     

Clinical Impact of Solifenacin on Generic and Symptom‐Specific Quality of Life for Females with Overactive Bladder: Using the Overactive Bladder Symptom Score and Rand Medical Outcomes Study 36‐Item Health Survey

Objectives: We evaluated the effectiveness of antimuscarinic treatment on disease‐specific and generic quality of life (QoL) in females with clinically diagnosed overactive bladder (OAB) by prospectively analyzing improvements in the overactive bladder symptom score (OABSS) and the Rand Medical Outcomes Study 36‐Item Short Form Health Survey (SF‐36). Methods: We prospectively recruited newly diagnosed female patients with OAB. Pretreatment disease‐specific symptoms were documented, and generic QoL questionnaires were administered. All subjects received solifenacin 5 mg/day for >8 weeks. Symptoms and general health‐related QoL (HRQoL) were assessed using the OABSS and SF‐36, respectively. Other objective variables, such as maximum urinary flow rate and postvoid residual urine volume, were also evaluated. Results: Seventy‐eight subjects met all inclusion criteria and no exclusion criteria. After 8 weeks, the mean OABSS decreased by approximately 50% compared with baseline (from 9.1 ± 2.8 to 4.5 ± 3.6). All individual scores in OABSS improved after administration of solifenacin. Before treatment, the scores of the study subjects in all SF‐36 domains were significantly worse than the age‐ and gender‐adjusted Japanese national norms (P < 0.01), except the vitality (VT) scale. Intra‐group comparisons between age groups showed worse mental health (MH) scores in all age groups. In the OAB group, three mean SF‐36 scales (physical function [PF], VT, and MH) significantly improved after treatment. Conclusion: Treatment of OAB with solifenacin is associated with significant improvement in generic HRQoL and disease‐specific symptoms at 8 weeks after drug administration. Particularly for generic HRQoL as measured by the SF‐36, solifenacin treatment effectively improves three SF‐36 scores: PF, VT, and MH.     

Determinants of Change in Quality of Life in the Cardiac Insufficiency Bisoprolol Study in Elderly (CIBIS-ELD)

ObjectiveLittle is known about parameters that lead to improvement in QoL in individual patients. We analysed the data of the Cardiac Insufficiency Bisoprolol Study in Elderly (CIBIS-ELD) in order to answer the question of how and to what extent change in health-related QoL during up-titration with bisoprolol vs. carvedilol is influenced by clinical and psychosocial factors in elderly patients with heart failure.MethodsThis is a QoL analysis of CIBIS-ELD, an investigator-initiated multi-center randomised phase III trial in elderly patients (65 years or older) with moderate to severe heart failure. Clinical parameters such as New York Heart Association functional class, heart rate, left ventricular ejection fraction (LVEF), 6-min walk distance, as well as the physical and psychosocial component scores on the short-form QoL health survey (SF36) and depression were recorded at baseline and at the final study visit.ResultsFull baseline and follow-up QoL data were available for 589 patients (292 in the bisoprolol and 297 in the carvedilol group). Mean physical and psychosocial QoL improved significantly during treatment. In regression analyses, changes in both SF36 component scores from baseline to follow-up were mainly predicted by baseline QoL and depression as well as change in depression over time. Changes in cardiac severity markers were significantly weaker predictors.ConclusionMean QoL increased during up-titration of bisoprolol and carvedilol. Both baseline depression and improvement in depression over time are associated with greater improvement in QoL more strongly than changes in cardiac severity measures.     

Quality of Life and Emotional Distress in Peritoneal Dialysis and Hemodialysis Patients

Cardiovascular disease‐associated morbidity and mortality are reportedly higher in hemodialysis (HD) patients compared with peritoneal dialysis (PD) patients. However, few studies have estimated changes in state of depression and cognitive impairment in patients undergoing HD and PD. The present study evaluated the impact of HD or PD on patients' quality of life (QoL), cognitive impairment, and depression status over 2 years. This 24‐month observational, prospective study included 45 HD and 30 PD patients. Patients were assessed before and every 12 months after starting dialysis for 24 months. Measurements included QoL (36‐Item Short‐Form Health Survey [SF‐36]), cognitive impairment (Mini‐Mental State Examination [MMSE]), depressive state (Center for Epidemiologic Studies Depression Scale [CES‐D]), grip strength, and 24‐h urine volume (UV). Physical and social component scores of the SF‐36 significantly improved in PD patients at 24 months compared with those observed at baseline (42.8 vs. 39.4; P < 0.05 and 46.4 vs. 37.3; P < 0.05, respectively); however, scores remained unchanged in HD patients. MMSE scores were significantly decreased at 12 and 24 months in HD patients (29.0 vs. 26.0, 25.0; P < 0.05), but remained unchanged in PD patients. Moreover, CES‐D scores significantly worsened at 24 months in HD patients (12.8 vs. 16.5), but remained unchanged in PD. Preservation of UV and grip strength was associated with SF‐36, CES‐D, and MMSE scores. Our findings indicate that PD is associated with higher QoL and recovery from cognitive failure compared with HD.     

Prevalence of depression in adults with haemophilia

Multiple factors place adults with haemophilia at risk for depression. Health outcomes can be compromised in depressed patients secondary to increased risk taking behaviour and poor compliance with treatment recommendations. To assess the prevalence and risk factors associated with depression in adult patients with haemophilia treated at a haemophilia treatment centre. Adults with haemophilia were screened for depression during their annual clinic visit using the Patient Health Questionnaire 9 (PHQ‐9), a validated tool for depression screening in adults. Depression was defined as a PHQ‐9 score ≥ 5. Risk factors associated with depression were collected by chart review and correlated with depression scores. A total of 41 adult patients consented to the study and 37% met criteria for depression. Fifty‐three per cent of patients with depression reported moderate to severe symptoms of depression (PHQ‐9 score >10). Seventy‐six per cent of patients with depression reported suffering functional impairment due to their depressive symptoms. Lack of social support and unemployment were significantly associated with higher PHQ‐9 scores (P = 0.04 and P = 0.01 respectively). Adult patients with haemophilia have a high prevalence of depression. The addition of depression screening to the comprehensive care of adults with haemophilia may result in improved overall health outcomes and treatment adherence.     

Health-related quality of life in ankylosing spondylitis,fibromyalgia syndrome,and rheumatoid arthritis: a comparison with a selected sample of healthy ındividuals

In this study, we compared health-related quality of life (HRQoL) scores of patients with rheumatoid arthritis (RA), fibromyalgia syndrome (FS), or ankylosing spondylitis (AS), with those of a selected sample of healthy individuals. The study group comprised 530 patients (264 with RA, 149 with FS, and 117 with AS). Three hundred fifteen healthy controls were used for comparison. HRQoL scores were obtained using the Short-Form (SF)-36 Health Survey. Frequency, mean, correlation, and multiple regression analyses were performed; in addition to the Student's t test, one-way ANOVA test, Kruskal–Wallis test, and chi-square test. The average physical component summary (PCS) and mental component summary (MCS) scores of patients in the RA, FS, and AS groups were much lower than those in the control group (p < 0.05). In all three patient groups, the lowest PCS score was found in the RA group, and the lowest MCS score was found in the FS group. The PCS scores of RA patients with disease-related changes in daily activities and the MCS scores of FS patients were lower (p < 0.05). In the three patient groups, the quality of life (QoL) of university graduates and those with a high level of income was good, whereas the QoL of patients who lacked information about the disease was poor. This study shows that AS, FS, and RA have a negative impact on HRQoL. Evaluating the HRQoL of patients with these conditions may provide guidance on their treatment and care.     

National, multicentre, prospective study of quality of life in patients with osteoarthritis of the knee treated with hylane G-F 20

The efficacy of viscosupplementation for the relief of pain and disability caused by knee osteoarthritis has been demonstrated, but its effects on Quality of Life (QoL) are less well known. The aim of this study was to describe the changes in QoL in patients receiving hylane G-F 20 in routine practice for the treatment of knee osteoarthritis and to determine the factors associated with changes in QoL. An observational, prospective, multicentre study was carried out in private ambulatory care patients. The study population consisted of patients receiving hylane G-F 20 injections for symptomatic, radiological Kellgren stage II or III knee osteoarthritis. The clinical and demographic data were recorded at inclusion and the QoL, SF36 and OsteoArthritis Knee and Hip Quality Of Life (OAKHQOL) questionnaires administered before the first injection and then during the 3- and 6-month visits. Three hundred patients were enrolled in the study (61% female, mean age 67?±?11 years, 45% Kellgren stage II). After 3 and 6 months, all the SF36 scores and three OAKHQOL scores had significantly improved. In the multivariate analysis, low QoL scores at inclusion were correlated with a more marked improvement (p?相似文献   

Adherence to prophylaxis is associated with better outcomes in moderate and severe haemophilia: results of a patient survey

Severe haemophilia is associated with bleeding into joints and development of arthropathy. Prophylactic treatment with infusion of replacement clotting factor is known to prevent bleeding, preserve joint functioning and result in higher health‐related quality of life (HRQoL) than episodic treatment; however, adhering to standard prophylaxis schedules can be difficult, and little is known about the relationship between adherence to prophylactic treatment and outcomes. The aim of this study was to assess the relationship between self‐reported adherence to prophylaxis and health outcomes, including HRQoL and bleeding episodes. Adults with haemophilia (n = 55) and caregivers of children with haemophilia (n = 55) in Australia, Canada, and the United States completed an online questionnaire which included measures of HRQoL (SF‐12v2 for adults and SF‐10 for caregivers of children), self‐reported bleeding episodes, and the VERITAS‐Pro measure of adherence to prophylaxis in haemophilia. Regression analysis was used to test the association between VERITAS‐Pro total score and outcomes. Poorer adherence (higher VERITAS‐Pro scores) was associated with a greater number of self‐reported bleeding episodes in the past year among adults (p < 0.01), more days of work/school missed among paediatric patients (p < 0.01), and lower physical health status scores among paediatric patients (p < 0.05). This study highlights the benefits of adherence to prophylaxis among those with severe haemophilia and provides evidence for the utility of the VERITAS‐Pro by demonstrating a relationship between adherence and outcomes.     

Impact of Uveitis on Quality of Life in Adult Patients With Juvenile Idiopathic Arthritis

Objective

To establish the impact of uveitis on the quality of life (QoL) in adult patients with juvenile idiopathic arthritis (JIA ).

Methods

Adult patients with a history of JIA , both with (n = 31) or without (n = 51) chronic anterior uveitis, were included. Their scores on 3 validated QoL questionnaires (National Eye Institute Visual Functioning Questionnaire [NEI VFQ ‐25], Medical Outcomes Study 36‐Item Short Form health survey [SF ‐36], and EuroQol 5‐domain questionnaire [EQ ‐5D]) were analyzed to find factors that could influence QoL.

Results

The median overall composite score (OCS ) of the NEI VFQ ‐25 was significantly worse in the uveitis group compared to the non‐uveitis group (respectively, 83.4 [range 15.2–94.7] and 94.9 [range 46.3–100]; P < 0.001). Nearly all subscale scores were lower in patients with uveitis than in patients without uveitis (P < 0.001 for all). After adjusting for duration of arthritis, JIA subtype, arthritis onset before or after 1990, and the use of systemic immunomodulatory medication, the QoL was still worse in patients with uveitis (NEI VFQ ‐25 OCS regression coefficient = ?11.7; P = 0.002). No significant differences were found between the groups for the SF ‐36 and the EQ ‐5D. In the total JIA group, the use of systemic medication appeared to negatively influence some general QoL scores.

Conclusion

Having a history of uveitis has a substantial negative effect on the vision‐related QoL in JIA in adulthood, despite good visual acuity. General QoL scores did not differ between uveitis and non‐uveitis patients, but the use of systemic immunomodulatory treatment, independent of uveitis, did negatively influence general QoL scores in adult JIA patients.

     

Quality of life assessments with SF 36 in different musculoskeletal diseases

The aim of this study is to evaluate comparatively the life quality of patients with knee osteoarthritis (KO), shoulder impingement syndrome(SIS), fibromialgia(FM), or osteoporosis(OP) using SF 36 and establish the impact of these diseases on quality of life (QoL). A total of 193 patients with one of the above-mentioned different diagnoses completed SF 36 scale. The diseases were compared to each other with SF 36 subgroups scores. There were significant differences among patients with KO and SIS,SIS and FM with respect to all SF 36 subgroups scores. According to these assessments, QoL of KO and FM patients was worst than that of SIS. The QoL scores of KO patients were worse than those of FM patients considering the physical function, while QoL scores of FM patients were lower than those of KO patients with respect to their general well-being. Scores of physical function and pain in KO patients were lower than those of OP patients. In domains of social functioning, emotional role, energy, pain, and general health condition QoL of FM patients was worse than that of OP patients. Quality of life of SIS patients was less affected than the patients of the other disease groups. In spite of their young age, FM patients appear to be the group with the worst quality of life scores.