Purpose: People with intellectual disabilities often show challenging behaviour, which can manifest itself in self-harm or aggression towards others. Real-time monitoring of stress in clients with... Show morePurpose: People with intellectual disabilities often show challenging behaviour, which can manifest itself in self-harm or aggression towards others. Real-time monitoring of stress in clients with challenging behaviour can help caregivers to promptly deploy interventions to prevent escalations, ultimately to improve the quality of life of client and caregiver. This study aimed to assess the impact of real-time stress monitoring with HUME, and the subsequent interventions deployed by the care team, on stress levels and quality of life. Materials and methods: Real-time stress monitoring was used in 41 clients with intellectual disabilities in a long-term care setting over a period of six months. Stress levels were determined at the start and during the deployment of the stress monitoring system. The quality of life of the client and caregiver was measured with the Outcome Rating Scale at the start and at three months of use. Results: The results showed that the HUME-based interventions resulted in a stress reduction. The perceived quality of life was higher after three months for both the clients and caregivers. Furthermore, interventions to provide proximity were found to be most effective in reducing stress and increasing the client's quality of life. Conclusions: The study demonstrates that real-time stress monitoring with the HUME and the following interventions were effective. There was less stress in clients with an intellectual disability and an increase in the perceived quality of life. Future larger and randomized controlled studies are needed to confirm these findings. Show less
Purpose: To assess the longitudinal vision-related quality of life among patients with CRB1-associated inherited retinal dystrophies.Methods: A longitudinal questionnaire study included 22 patients... Show morePurpose: To assess the longitudinal vision-related quality of life among patients with CRB1-associated inherited retinal dystrophies.Methods: A longitudinal questionnaire study included 22 patients with pathogenic CRB1 variants. The National Eye Institute Visual Function Questionnaire (39 items, NEI VFQ-39) was applied at baseline, two-year follow-up, and 4-year follow-up. Classical test theory was performed to obtain subdomain scores and in particular 'near activities' and 'total composite' scores. The Rasch analysis based on previous calibrations of the NEI VFQ-25 was applied to create visual functioning and socio-emotional subscales.Results: In total, 22 patients with pathogenic CRB1 variants were included, with a median age of 25.0 years (IQR: 13-31 years) at baseline and mean followup of 4.0 +/- 0.3 years. A significant decline at 4 years was observed for 'near activities' (51.0 +/- 23.8 vs 35.4 +/- 14.7, p = 0.004) and 'total composite' (63.0 +/- 13.1 vs 52.0 +/- 12.1, p = 0.001) subdomain scores. For the Rasch-scaled scores, the 'visual functioning' scale significantly decreased after 4 years (- 0.89 logits; p = 0.012), but not at 4-year follow-up (+0.01 logits; p = 0.975). The 'socio- emotional' scale also showed a significant decline after 2 years (-0.78 logits, p = 0.033) and 4 years (- 0.83 logits, p = 0.021).Conclusion: In the absence of an intervention, a decline in vision-related quality of life is present in patients with pathogenic CRB1 variants at 4-year follow-up. Patient-reported outcome measures should be included in future clinical trials, as they can be a potential indicator of disease progression and treatment efficacy. Show less
Liem, S.I.E.; Bergstra, S.A.; Ciaffi, J.; Meulen, C. van der; Ueckert, D.A.; Schriemer, M.R.; ... ; Vries-Bouwstra, J.K. de 2023
Objective: The Health Assessment Questionnaire-Disability Index is an important outcome measure reflecting functional disability, but knowledge on its course over time in patients with systemic... Show moreObjective: The Health Assessment Questionnaire-Disability Index is an important outcome measure reflecting functional disability, but knowledge on its course over time in patients with systemic sclerosis is scarce. Therefore, we investigated the long-term course of the Health Assessment Questionnaire-Disability Index and its association with baseline characteristics in systemic sclerosis patients. Methods: Systemic sclerosis patients, fulfilling the European League Against Rheumatism and the American College of Rheumatology 2013 criteria, were included from the Leiden Combined Care in Systemic Sclerosis cohort with annual assessments including the Scleroderma Health Assessment Questionnaire-Disability Index (range = 0-3). The course of the Health Assessment Questionnaire-Disability Index was evaluated over the total follow-up (baseline to last available Health Assessment Questionnaire-Disability Index) and between yearly visits. Based on a minimal clinical important difference of 0.22, courses were categorized into worsening, stable or improvement. The course of the Health Assessment Questionnaire-Disability Index over time was evaluated with linear mixed models. Baseline characteristics were compared between patients with a worsening or improvement of the Health Assessment Questionnaire-Disability Index over the total follow-up period with logistic regression analyses. Results: A total of 517 systemic sclerosis patients were included, with a median follow-up of 7 years (interquartile range = 4-9; 2649 visits) and a baseline Health Assessment Questionnaire-Disability Index of 0.625 (interquartile range = 0.125-1.25). On group level, the Health Assessment Questionnaire-Disability Index is stable with an annual increase of 0.019 (95% confidence interval = 0.011 to 0.027). Looking at subgroups, patients >65 years or who died/were physically unable to come during follow-up had a worse mean Health Assessment Questionnaire-Disability Index. In individual courses from baseline to the last follow-up, the proportions of patients with a clinically meaningful worsening, stable or improved Health Assessment Questionnaire-Disability Index were 35%, 42% and 23%, respectively. Patients with immunosuppressants (odds ratio = 0.5, 95% confidence interval = 0.3 to 0.9) or gastrointestinal involvement (odds ratio = 0.6, 95% confidence interval = 0.4 to 0.9) at baseline showed a reduced chance of worsening of the Health Assessment Questionnaire-Disability Index over the total follow-up period. Conclusion: Over time, the average course of the Health Assessment Questionnaire-Disability Index was stable in systemic sclerosis patients. However, individual courses vary, with worsening occurring in one-third. Worsening occurred less often in individuals using immunosuppressants or with gastrointestinal involvement at baseline. Show less
BackgroundColorectal cancer is diagnosed in approximately 500,000 patients each year in Europe, leading to a high number of patients having to cope with the consequences of resection for colorectal... Show moreBackgroundColorectal cancer is diagnosed in approximately 500,000 patients each year in Europe, leading to a high number of patients having to cope with the consequences of resection for colorectal cancer. As treatment options tend to grow, more information on the effects of these treatments is needed to engage in shared decision-making. This study aims to explore the impact of resection for colorectal cancer on patients' daily life.MethodsPatients (≥18 years of age) who underwent an oncological colorectal resection between 2018 and 2021 were selected. Purposeful sampling was used to include patients who differed in age, comorbidity conditions, types of (neo)adjuvant therapy, postoperative complications and the presence/absence of a stoma. Semi-structured interviews were conducted, guided by a topic guide. Interviews were fully transcribed and subsequently thematically analysed using the framework approach. Analyses were carried out using the following predefined themes: (1) daily life and activities; (2) psychological functioning; (3) social functioning; (4) sexual functioning; and (5) healthcare experiences.ResultsSixteen patients with a follow-up period of between 0.6 and 4.4 years after surgery were included in this study. Participants reported several challenges experienced because of poor bowel function, a stoma, chemotherapy-induced neuropathy, fear of recurrence and sexual dysfunction. However, they reported these as not interfering much with daily life.ConclusionColorectal cancer treatment leads to several challenges and treatment-related health deficits. This is often not recognized by generic patient-reported outcome measures, but the findings on treatment-related health deficits presented in this study contain valuable insights which might contribute to improving colorectal cancer care, shared decision making and value-based health care. Show less
Allonsius, F.; Kloet, A.J. de; Markus-Doornbosch, F. van; Vlieland, T.P.M.V.; Holst, M. van der 2023
PurposeBrain injuries (traumatic-/nontraumatic, TBI/nTBI) in young patients may lead to problems e.g., decreased health-related quality of life (HRQoL), and causes family impact. Knowledge... Show morePurposeBrain injuries (traumatic-/nontraumatic, TBI/nTBI) in young patients may lead to problems e.g., decreased health-related quality of life (HRQoL), and causes family impact. Knowledge regarding the family impact and the relationship with patients' HRQoL over time is scarce. This follow-up study describes family impact/HRQoL and their mutual relationship in young patients (5-24 years) after TBI/nTBI.Materials and methodsParents of patients that were referred to outpatient rehabilitation completed the PedsQL (TM) Family-Impact-Module questionnaire to assess the family impact and the parent-reported PedsQL (TM) Generic-core-set-4.0 to assess patients' HRQoL (lower scores: more family impact/worse HRQoL). Questionnaires were completed at the time of referral to rehabilitation (baseline) and one/two years later (T1/T2). Linear-mixed models were used to examine family impact/HRQoL change scores, and repeated-measure correlations (r) to determine longitudinal relationships.ResultsTwo-hundred-forty-six parents participated at baseline, 72 (at T2), median patient's age at baseline was 14 years (IQR:11-16), and 181 (74%) had TBI. Mean (SD) PedsQL (TM) Family-Impact-Module score at baseline was 71.7 (SD:16.4) and PedsQL (TM) Generic-core-set-4.0: 61.4 (SD:17.0). Over time, PedsQL (TM) Family-Impact-Module scores remained stable, while PedsQL (TM) Generic-core-set-4.0 scores improved significantly(p < 0.05). A moderately strong longitudinal correlation was found between family impact&HRQoL (r = 0.51).ConclusionsFamily impact does not tend to decrease over time but remained a considerable problem, although patients' HRQoL improved. Next to focusing on patients' HRQoL, it remains important to consider family impact and offer family support throughout rehabilitation. Show less
Background: COPD causes high morbidity and mortality, emphasizing the need for palliative care. Aim: To assess the effectiveness of palliative care in patients with COPD. Design: Cluster randomized... Show moreBackground: COPD causes high morbidity and mortality, emphasizing the need for palliative care. Aim: To assess the effectiveness of palliative care in patients with COPD. Design: Cluster randomized controlled trial (COMPASSION study; Netherlands Trial Register (NTR): NL7644, 07-04-2019). Healthcare providers within the intervention group were trained to implement palliative care components into routine COPD care. Patients completed questionnaires at baseline, after 3 and 6 months; medical records were assessed after 12 months. The primary outcome was quality of life (FACIT-Pal). Secondary outcomes were anxiety, depression, spiritual well-being, satisfaction with care, acute healthcare use, documentation of life-sustaining treatment preferences and place of death. Generalized linear mixed modelling was used for analyses. Setting: Eight hospital regions in the Netherlands. Participants: Patients hospitalized for an acute exacerbation of COPD and positive ProPal-COPD score. Results: Of 222 patients included, 106 responded to the questionnaire at 6 months. Thirty-six of 98 intervention patients (36.7%) received the intervention. Intention-to-treat-analysis showed no effect on the primary outcome (adjusted difference: 1.09; 95% confidence interval: -5.44 to 7.60). In the intervention group, fewer intensive care admissions for COPD took place (adjusted odds ratio: 0.21; 95% confidence interval: 0.03-0.81) and strong indications were found for fewer hospitalizations (adjusted incidence rate ratio: 0.69; 95% confidence interval: 0.46-1.03). Conclusions: We found no evidence that palliative care improves quality of life in patients with COPD. However, it can potentially reduce acute healthcare use. The consequences of the COVID-19 pandemic led to suboptimal implementation and insufficient power, and may have affected some of our findings. Show less
Background:COPD causes high morbidity and mortality, emphasizing the need for palliative care.Aim:To assess the effectiveness of palliative care in patients with COPD.Design:Cluster randomized... Show moreBackground:COPD causes high morbidity and mortality, emphasizing the need for palliative care.Aim:To assess the effectiveness of palliative care in patients with COPD.Design:Cluster randomized controlled trial (COMPASSION study; Netherlands Trial Register (NTR): NL7644, 07-04-2019). Healthcare providers within the intervention group were trained to implement palliative care components into routine COPD care. Patients completed questionnaires at baseline, after 3 and 6 months; medical records were assessed after 12 months. The primary outcome was quality of life (FACIT-Pal). Secondary outcomes were anxiety, depression, spiritual well-being, satisfaction with care, acute healthcare use, documentation of life-sustaining treatment preferences and place of death. Generalized linear mixed modelling was used for analyses.Setting:Eight hospital regions in the Netherlands.Participants:Patients hospitalized for an acute exacerbation of COPD and positive ProPal-COPD score.Results:Of 222 patients included, 106 responded to the questionnaire at 6 months. Thirty-six of 98 intervention patients (36.7%) received the intervention. Intention-to-treat-analysis showed no effect on the primary outcome (adjusted difference: 1.09; 95% confidence interval: −5.44 to 7.60). In the intervention group, fewer intensive care admissions for COPD took place (adjusted odds ratio: 0.21; 95% confidence interval: 0.03–0.81) and strong indications were found for fewer hospitalizations (adjusted incidence rate ratio: 0.69; 95% confidence interval: 0.46–1.03).Conclusions:We found no evidence that palliative care improves quality of life in patients with COPD. However, it can potentially reduce acute healthcare use. The consequences of the COVID-19 pandemic led to suboptimal implementation and insufficient power, and may have affected some of our findings. Show less
Background:COPD causes high morbidity and mortality, emphasizing the need for palliative care.Aim:To assess the effectiveness of palliative care in patients with COPD.Design:Cluster randomized... Show moreBackground:COPD causes high morbidity and mortality, emphasizing the need for palliative care.Aim:To assess the effectiveness of palliative care in patients with COPD.Design:Cluster randomized controlled trial (COMPASSION study; Netherlands Trial Register (NTR): NL7644, 07-04-2019). Healthcare providers within the intervention group were trained to implement palliative care components into routine COPD care. Patients completed questionnaires at baseline, after 3 and 6 months; medical records were assessed after 12 months. The primary outcome was quality of life (FACIT-Pal). Secondary outcomes were anxiety, depression, spiritual well-being, satisfaction with care, acute healthcare use, documentation of life-sustaining treatment preferences and place of death. Generalized linear mixed modelling was used for analyses.Setting:Eight hospital regions in the Netherlands.Participants:Patients hospitalized for an acute exacerbation of COPD and positive ProPal-COPD score.Results:Of 222 patients included, 106 responded to the questionnaire at 6 months. Thirty-six of 98 intervention patients (36.7%) received the intervention. Intention-to-treat-analysis showed no effect on the primary outcome (adjusted difference: 1.09; 95% confidence interval: −5.44 to 7.60). In the intervention group, fewer intensive care admissions for COPD took place (adjusted odds ratio: 0.21; 95% confidence interval: 0.03–0.81) and strong indications were found for fewer hospitalizations (adjusted incidence rate ratio: 0.69; 95% confidence interval: 0.46–1.03).Conclusions:We found no evidence that palliative care improves quality of life in patients with COPD. However, it can potentially reduce acute healthcare use. The consequences of the COVID-19 pandemic led to suboptimal implementation and insufficient power, and may have affected some of our findings. Show less
Background:COPD causes high morbidity and mortality, emphasizing the need for palliative care.Aim:To assess the effectiveness of palliative care in patients with COPD.Design:Cluster randomized... Show moreBackground:COPD causes high morbidity and mortality, emphasizing the need for palliative care.Aim:To assess the effectiveness of palliative care in patients with COPD.Design:Cluster randomized controlled trial (COMPASSION study; Netherlands Trial Register (NTR): NL7644, 07-04-2019). Healthcare providers within the intervention group were trained to implement palliative care components into routine COPD care. Patients completed questionnaires at baseline, after 3 and 6 months; medical records were assessed after 12 months. The primary outcome was quality of life (FACIT-Pal). Secondary outcomes were anxiety, depression, spiritual well-being, satisfaction with care, acute healthcare use, documentation of life-sustaining treatment preferences and place of death. Generalized linear mixed modelling was used for analyses.Setting:Eight hospital regions in the Netherlands.Participants:Patients hospitalized for an acute exacerbation of COPD and positive ProPal-COPD score.Results:Of 222 patients included, 106 responded to the questionnaire at 6 months. Thirty-six of 98 intervention patients (36.7%) received the intervention. Intention-to-treat-analysis showed no effect on the primary outcome (adjusted difference: 1.09; 95% confidence interval: −5.44 to 7.60). In the intervention group, fewer intensive care admissions for COPD took place (adjusted odds ratio: 0.21; 95% confidence interval: 0.03–0.81) and strong indications were found for fewer hospitalizations (adjusted incidence rate ratio: 0.69; 95% confidence interval: 0.46–1.03).Conclusions:We found no evidence that palliative care improves quality of life in patients with COPD. However, it can potentially reduce acute healthcare use. The consequences of the COVID-19 pandemic led to suboptimal implementation and insufficient power, and may have affected some of our findings. Show less
Background:COPD causes high morbidity and mortality, emphasizing the need for palliative care.Aim:To assess the effectiveness of palliative care in patients with COPD.Design:Cluster randomized... Show moreBackground:COPD causes high morbidity and mortality, emphasizing the need for palliative care.Aim:To assess the effectiveness of palliative care in patients with COPD.Design:Cluster randomized controlled trial (COMPASSION study; Netherlands Trial Register (NTR): NL7644, 07-04-2019). Healthcare providers within the intervention group were trained to implement palliative care components into routine COPD care. Patients completed questionnaires at baseline, after 3 and 6 months; medical records were assessed after 12 months. The primary outcome was quality of life (FACIT-Pal). Secondary outcomes were anxiety, depression, spiritual well-being, satisfaction with care, acute healthcare use, documentation of life-sustaining treatment preferences and place of death. Generalized linear mixed modelling was used for analyses.Setting:Eight hospital regions in the Netherlands.Participants:Patients hospitalized for an acute exacerbation of COPD and positive ProPal-COPD score.Results:Of 222 patients included, 106 responded to the questionnaire at 6 months. Thirty-six of 98 intervention patients (36.7%) received the intervention. Intention-to-treat-analysis showed no effect on the primary outcome (adjusted difference: 1.09; 95% confidence interval: −5.44 to 7.60). In the intervention group, fewer intensive care admissions for COPD took place (adjusted odds ratio: 0.21; 95% confidence interval: 0.03–0.81) and strong indications were found for fewer hospitalizations (adjusted incidence rate ratio: 0.69; 95% confidence interval: 0.46–1.03).Conclusions:We found no evidence that palliative care improves quality of life in patients with COPD. However, it can potentially reduce acute healthcare use. The consequences of the COVID-19 pandemic led to suboptimal implementation and insufficient power, and may have affected some of our findings. Show less
A broad spectrum of long-term sequelae may be present in venous thromboembolism (VTE) survivors, affecting their quality of life and functioning. To monitor recovery and improve the prognosis of... Show moreA broad spectrum of long-term sequelae may be present in venous thromboembolism (VTE) survivors, affecting their quality of life and functioning. To monitor recovery and improve the prognosis of patients with persistent functional limitations, the development of a new outcome measure that could better capture the consequences of VTE was an unmet need. Starting as a call to action, the Post-VTE Functional Status (PVFS) scale was developed to meet this need. The PVFS scale is an easy-to-use clinical tool to measure and quantify functional outcomes after VTE by focusing on key aspects of daily life. As the scale was considered useful in coronavirus disease 2019 (COVID-19) patients as well, the Post-COVID-19 Functional Status (PCFS) scale was introduced early in the pandemic after slight adaptation. The scale has been well incorporated into both the VTE and COVID-19 research communities, contributing to the shift of focus toward patient-relevant functional outcomes. Psychometric properties have been evaluated, mainly for the PCFS scale but recently also for the PVFS scale, including validation studies of translations, showing adequate validity and reliability. In addition to serving as outcome measure in studies, guidelines and position papers recommend using the PVFS and PCFS scale in clinical practice. As broad use of the PVFS and PCFS scale in clinical practice is valuable to capture what matters most to patients, widespread implementation is a crucial next step. In this review, we discuss the development of the PVFS scale and introduction in VTE and COVID-19 care, the incorporation of the scale in research, and its application in clinical practice. Show less
Hoepelman, R.J.; Minervini, F.; Beeres, F.J.P.; Wageningen, B. van; IJpma, F.F.; Veelen, N.M. van; ... ; NEXT study grp 2023
IntroductionMost studies about rib fractures focus on mortality and morbidity. Literature is scarce on long term and quality of life (QoL) outcomes. Therefore, we report QoL and long-term outcomes... Show moreIntroductionMost studies about rib fractures focus on mortality and morbidity. Literature is scarce on long term and quality of life (QoL) outcomes. Therefore, we report QoL and long-term outcomes after rib fixation in flail chest patients. MethodsA prospective cohort study of clinical flail chest patients admitted to six level 1 trauma centres in the Netherlands and Switzerland between January 2018 and March 2021. Outcomes included in-hospital outcomes and long-term outcomes, such as QoL measurements 12 months after hospitalization using the EuroQoL five dimensions (EQ-5D) questionnaire. ResultsSixty-one operatively treated flail chest patients were included. Median hospital length of stay was 15 days and intensive care length of stay was 8 days. Sixteen (26%) patients developed pneumonia and two (3%) died. One year after hospitalization the mean EQ5D score was 0.78. Complication rates were low and included hemothorax (6%) pleural effusion (5%) and two revisions of the implant (3%). Implant related irritation was commonly reported by patients (n = 15, 25%). ConclusionsRib fixation for flail chest injuries can be considered a safe procedure and with low mortality rates. Future studies should focus on quality of life rather than solely short-term outcomes. Show less
BackgroundThe Pulmonary Embolism Quality of Life (PEmb-QoL) questionnaire is the first disease-specific scale for assessing the quality of life in patients with a history of pulmonary embolism (PE)... Show moreBackgroundThe Pulmonary Embolism Quality of Life (PEmb-QoL) questionnaire is the first disease-specific scale for assessing the quality of life in patients with a history of pulmonary embolism (PE).ObjectivesTo assess the cross-cultural validity and reliability of the disease-specific PEmb-QoL questionnaire.MethodsThe Persian version was prepared through the forward and backward translation of the English questionnaire. Six months after the diagnosis of acute PE, consecutive Persian-speaking patients were asked to complete the PEmb-QoL, the generic 36-item Short Form (SF-36) questionnaires and undertake a 6-minute walk test (6MWT). Acceptability was assessed via item missing rate, reproducibility by the test-retest method, and internal consistency reliability by Cronbach’s α and McDonald’s ω coefficients. Convergence validity was assessed using the Spearman rank correlation between scores of PEmb-QoL, SF-36, and 6MWT. The questionnaire structure was evaluated through exploratory factor analysis.ResultsNinety-six patients with a confirmed diagnosis of PE completed the questionnaires. The Persian version of PEmb-QoL had good internal consistency (α = 0.95, 3-factor ω = 0.96), inter-item correlation (0.3–0.62), item-total correlation (0.38–0.71), reproducibility (test-retest ICC with 25 participants = 0.92–0.99), and good discriminant validity. Convergence validity was confirmed by the moderate-to-high correlations between PEmb-QoL and SF-36 scores, and a good correlation between the “limitation in daily activities” dimension of the PEmb-QoL questionnaire and 6MWT results. Exploratory factor analysis suggested a 3-component structure with functional (items 1h, 4b-5d, 6, 8, 9i, and 9j), symptoms (1b-h, 7, and 8), and emotional (5a, 6, and 9a-h) components.ConclusionThe Persian version of the PEmb-QoL questionnaire is valid and reliable for measuring the disease-specific quality of life in patients with PE. Show less
Background: While patients with diffuse low-grade glioma (LGG) often survive for years, there is a risk of tumor progression which may impact patients' long-term health-related quality of life ... Show moreBackground: While patients with diffuse low-grade glioma (LGG) often survive for years, there is a risk of tumor progression which may impact patients' long-term health-related quality of life (HRQOL) and neurocognitive functioning (NCF). We present a follow-up of LGG patients and their informal caregivers (T3) who took part in our previous HRQOL investigations (T1, M = 7 and T2 M = 13 years after diagnosis). Methods: Participants completed HRQOL (short form-36 health survey [SF-36]; EORTC-BN20), fatigue (Checklist Individual Strength [CIS]), and depression (Center for Epidemiological Studies-Depression [CES-D]) questionnaires and underwent NCF assessments. T3 scores were compared with matched controls. Changes over time (T1-T2-T3) on group and participant level were assessed. Where available, histology of the initial tumor was revised and immunohistochemical staining for IDH1 R132H mutant protein was performed. Results: Thirty patients and nineteen caregivers participated. Of N = 11 with tissue available, 3 patients had confirmed diffuse LGG. At T3, patients (M = 26 years after diagnosis) had HRQOL and NCF similar to, or better than controls, yet 23.3% and 53.3% scored above the cut-off for depression (>= 16 CES-D) and fatigue (>= 35 CIS), respectively. Caregivers' HRQOL was similar to controls but reported high rates of fatigue (63.2%). Over time, patients' mental health improved (P < .05). Minimal detectable change in HRQOL over time was observed in individual patients (30% improvement; 23.3% decline; 20% both improvement and decline) with 23.3% remaining stable. NCF remained stable or improved in 82.8% of patients. Conclusions: While HRQOL and NCF do not appear greatly impacted during long-term survivorship in LGG, depressive symptoms and fatigue are persistent. Show less
BackgroundWhile patients with diffuse low-grade glioma (LGG) often survive for years, there is a risk of tumor progression which may impact patients’ long-term health-related quality of life (HRQOL... Show moreBackgroundWhile patients with diffuse low-grade glioma (LGG) often survive for years, there is a risk of tumor progression which may impact patients’ long-term health-related quality of life (HRQOL) and neurocognitive functioning (NCF). We present a follow-up of LGG patients and their informal caregivers (T3) who took part in our previous HRQOL investigations (T1, M = 7 and T2 M = 13 years after diagnosis).MethodsParticipants completed HRQOL (short form-36 health survey [SF-36]; EORTC-BN20), fatigue (Checklist Individual Strength [CIS]), and depression (Center for Epidemiological Studies-Depression [CES-D]) questionnaires and underwent NCF assessments. T3 scores were compared with matched controls. Changes over time (T1–T2–T3) on group and participant level were assessed. Where available, histology of the initial tumor was revised and immunohistochemical staining for IDH1 R132H mutant protein was performed.ResultsThirty patients and nineteen caregivers participated. Of N = 11 with tissue available, 3 patients had confirmed diffuse LGG. At T3, patients (M = 26 years after diagnosis) had HRQOL and NCF similar to, or better than controls, yet 23.3% and 53.3% scored above the cut-off for depression (≥16 CES-D) and fatigue (≥35 CIS), respectively. Caregivers’ HRQOL was similar to controls but reported high rates of fatigue (63.2%). Over time, patients’ mental health improved (P < .05). Minimal detectable change in HRQOL over time was observed in individual patients (30% improvement; 23.3% decline; 20% both improvement and decline) with 23.3% remaining stable. NCF remained stable or improved in 82.8% of patients.ConclusionsWhile HRQOL and NCF do not appear greatly impacted during long-term survivorship in LGG, depressive symptoms and fatigue are persistent. Show less
Background and objectives In older people with kidney failure, improving health-related quality of life is often more important than solely prolonging life. However, little is known about the... Show moreBackground and objectives In older people with kidney failure, improving health-related quality of life is often more important than solely prolonging life. However, little is known about the effect of dialysis initiation on health-related quality of life in older patients. Therefore, we investigated the evolution of health-related quality of life before and after starting dialysis in older patients with kidney failure. Design, setting, participants, & measurements The European Quality study is an ongoing prospective, multicenter study in patients aged >_65 years with an incident eGFR <_20 ml/min per 1.73 m2. Between April 2012 and December 2021, health-related quality of life was assessed every 3-6 months using the 36-item Short-Form Health Survey (SF-36), providing a mental component summary (MCS) and a physical component summary (PCS). Scores range from zero to 100, with higher scores indicating better health-related quality of life. With linear mixed models, we explored the course of health-related quality of life during the year preceding and following dialysis initiation. Results In total, 457 patients starting dialysis were included who filled out at least one SF-36 during follow-up. At dialysis initiation, mean +/- SD age was 76 +/- 6 years, eGFR was 8 +/- 3 ml/min per 1.73 m2, 75% were men, 9% smoked, 45% had diabetes, and 46% had cardiovascular disease. Median (interquartile range) MCS was 53 (38-73), and median PCS was 39 (27-58). During the year preceding dialysis, estimated mean change in MCS was -13 (95% confidence interval, -17 to -9), and in PCS, it was -11 (95% confidence interval, -15 to -7). In the year following dialysis, estimated mean change in MCS was +2 (95% confidence interval, -7 to +11), and in PCS, it was -2 (95% confidence interval, -11 to +7). Health-related quality-of-life patterns were similar for most mental (mental health, role emotional, social functioning, vitality) and physical domains (physical functioning, bodily pain, role physical). Conclusions Patients experienced a clinically relevant decline of both mental and physical health-related quality of life before dialysis initiation, which stabilized thereafter. These results may help inform older patients with kidney failure who decided to start dialysis. Show less
Background As a first step to reach consensus on the key constructs and outcomes in neuro-oncology caregiver research, we performed a systematic review to evaluate the constructs that are being... Show moreBackground As a first step to reach consensus on the key constructs and outcomes in neuro-oncology caregiver research, we performed a systematic review to evaluate the constructs that are being evaluated in research studies and how these have been assessed. Methods All peer-reviewed publications with primary data reporting on outcomes of family caregivers of adult primary brain tumor patients were eligible. Electronic databases PubMed/Medline, Embase, Web of Science, Emcare, Cochrane Library, and PsycINFO were searched up to September 2021. Using Covidence, title and abstract screening, full-text review, and data extraction were done by two researchers independently, with a third guiding consensus. Constructs as reported in each study, and how these were assessed were the primary result. Results Searches yielded 1090 unique records, with 213 remaining after title/abstract screening. Of these, 157 publications met inclusion criteria, comprising 120 unique studies. These originated from 18 countries and were published between 1996 and 2022. Most were observational (75%) cross-sectional (61%) studies, reporting on quantitative methods (62%). Twenty-seven different constructs were assessed and mapped along the Caregiver Health Model (CGHM) categories, namely, caregiver health, needs, tasks, beliefs and attitudes, and environment. Seventeen questionnaires were used >2 times to measure the same construct, with the vast majority of questionnaires only used across one or two studies. Conclusions Neuro-oncology caregiving research is a field gaining traction, but lags behind in clear definition of key constructs, and consistency in assessment of these constructs. Developing consensus or guidance will improve comparability of studies, meta-analyses, and advance the science more quickly. Show less
Linden, M. van der; Hees, C.L. van; Beurden, M. van; Bulten, J.; Dorst, E.B. van; Esajas, M.D.; ... ; Hullu, J.A. de 2022
BACKGROUND: Vulvar Paget disease is an extremely rare skin disorder, which is most common in postmenopausal women. Most vulvar Paget disease cases are noninvasive; however, it may be invasive or... Show moreBACKGROUND: Vulvar Paget disease is an extremely rare skin disorder, which is most common in postmenopausal women. Most vulvar Paget disease cases are noninvasive; however, it may be invasive or associated with an underlying vulvar or distant adenocarcinoma. The current treatment of choice for noninvasive vulvar Paget disease is wide local excision, which is challenging because of extensive intraepithelial spread and may cause severe morbidity. Recurrence rates are high, ranging from 15% to 70%, which emphasizes the need for new treatment options. Imiquimod, a topical immune response modifier, has been shown to be effective in a few studies and case reports, and is a promising new treatment modality.OBJECTIVE: To prospectively investigate the efficacy, safety, and effect on quality of life of a standardized treatment schedule with 5% imiquimod cream in patients with noninvasive vulvar Paget disease.STUDY DESIGN: The Paget Trial is a multicenter prospective observational clinical study including 7 tertiary referral hospitals in the Netherlands. A total of 24 patients with noninvasive vulvar Paget disease were treated with topical 5% imiquimod cream 3 times a week for 16 weeks. The primary efficacy outcome was the reduction in lesion size at 12 weeks after the end of treatment. Secondary outcomes were safety, clinical response after 1 year, and quality of life. Safety was assessed by evaluation of adverse events and tolerability of treatment. Quality of life was investigated with 3 questionnaires taken before, during, and after treatment.RESULTS: Data were available for 23 patients, 82.6% of whom responded to therapy. A complete response was reported in 12 patients (52.2%), and 7 patients (30.4%) had a partial response. A histologic complete response was observed in 10 of the 12 patients with a complete response. Patients experienced side effects such as fatigue (66.7%-70.9%) and headaches (16.7%-45.8%), and almost 80% needed painkillers during treatment. Eight patients (34.8%) adjusted the treatment protocol to 2 applications a week, and 3 patients (13.0%) stopped treatment because of side effects after 4 to 11 weeks. Treatment improved quality of life, whereas a slight, temporary negative impact was observed during treatment. Two patients with a complete response developed a recurrence within 1 year after treatment. Follow-up showed 6 patients with a noninvasive recurrence after a median of 31 months (14-46 months) after the end of treatment.CONCLUSION: Topical 5% imiquimod cream can be an effective and safe treatment alternative for noninvasive vulvar Paget disease, particularly when compared with treatment with surgical excision. Show less
Background Migraine-attributed burden, impact, disability and migraine-impacted quality of life are important concepts in clinical management, clinical and epidemiological research, and health... Show moreBackground Migraine-attributed burden, impact, disability and migraine-impacted quality of life are important concepts in clinical management, clinical and epidemiological research, and health policy, requiring clear and agreed definitions. We aimed to formulate concise and precise definitions of these concepts by expert consensus. Methods We searched the terms migraine-attributed burden, impact, disability and migraine-impacted quality of life in Embase and Medline from 1974 and 1946 respectively. We followed a Delphi process to reach consensus on definitions. Results We found widespread conflation of concepts and inconsistent terminology within publications. Following three Delphi rounds, we defined migraine-attributed burden as "the summation of all negative consequences of the disease or its diagnosis"; migraine-attributed impact as "the effect of the disease, or its diagnosis, on a specified aspect of life, health or wellbeing"; migraine-attributed disability as "physical, cognitive and mental incapacities imposed by the disease"; and migraine-impacted quality of life as "the subjective assessment by a person with the disease of their general wellbeing, position and prospects in life". We complemented each definition with a detailed description. Conclusion These definitions and descriptions should foster consistency and encourage more appropriate use of currently available quantifying instruments and aid the future development of others. Show less
Valassi, E.; Chiodini, I.; Feelders, R.A.; Andela, C.D.; Abou-Hanna, M.; Idres, S.; Tabarin, A. 2022
Background: Cushing's syndrome (CS) is a rare condition of chronically elevated cortisol levels resulting in diverse comorbidities, many of which endure beyond successful treatment affecting the... Show moreBackground: Cushing's syndrome (CS) is a rare condition of chronically elevated cortisol levels resulting in diverse comorbidities, many of which endure beyond successful treatment affecting the quality of life. Few data are available concerning patients' experiences of diagnosis, care and persistent comorbidities.Objective: To assess CS patients' perspectives on the diagnostic and care journey to identify unmet therapeutic needs.Methods: A 12-item questionnaire was circulated in 2019 by the World Association for Pituitary Organisations. A parallel, 13-item questionnaire assessing physician perceptions on CS patient experiences was performed.Results: Three hundred twenty CS patients from 30 countries completed the questionnaire; 54% were aged 35-54 and 88% were female; 41% were in disease remission. The most burdensome symptom was obesity/weight gain (75%). For 49% of patients, time to diagnosis was over 2 years. Following treatment, 88.4% of patients reported ongoing symptoms including, fatigue (66.3%), muscle weakness (48.8%) and obesity/weight gain (41.9%). Comparisons with delay in diagnosis were significant for weight gain (P = 0.008) and decreased libido (P = 0.03). Forty physicians completed the parallel questionnaire which showed that generally, physicians poorly estimated the prevalence of comorbidities, particularly initial and persistent cognitive impairment. Only a minority of persistent comorbidities (occurrence in 1.3-66.3%; specialist treatment in 1.3-29.4%) were managed by specialists other than endocrinologists. 63% of patients were satisfied with treatment.Conclusion: This study confirms the delay in diagnosing CS. The high prevalence of persistent comorbidities following remission and differences in perceptions of health between patients and physicians highlight a probable deficiency in effective multidisciplinary management for CS comorbidities. Show less
