scholarly journals Economic burden and health related quality of life of ultra-rare Gaucher disease in China

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Xinye Qi ◽  
Jiao Xu ◽  
Linghan Shan ◽  
Ye Li ◽  
Yu Cui ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Social Support ◽  
Health Care ◽  
Sleep Quality ◽  
Gaucher Disease ◽  
Health Related Quality ◽  
Sf 36 ◽  
Related Quality ◽  
Health Related

Abstract Background The diagnosis and health care of patients with rare diseases present a tremendous challenge worldwide. This study described the health care service utilization through participants’ perspective and estimated the cost of illness (COI), and patients with Gaucher disease (GD)’s/caregivers’ health-related quality of life in China. Method An online retrospective survey of patients with GD and their caregivers was conducted during May–June 2018. Socio-demographic, health service utilization, disease-related expenses, social support, sleep quality (Pittsburgh Sleep Quality Index [PSQI]), and the Short Form Health Survey (SF-36) were investigated. Using self-reported information, we estimated the annual COI, including direct healthcare, direct non-healthcare, and indirect costs. Results Forty patients and their 49 caregivers were surveyed. The patients’ onset age of GD was 9.3 ± 10.9; their disease course was 3.5 ± 3.1 years. 21 (42.9%) patients had ≥ 2 caregivers, but 35 (71.4%) caregivers reported have no experience as a caregiver. 79.6% caregivers have stopped working, and 87.8% changed weekly working schedule. Before final diagnosis, patients visited 3.9 ± 3.1 (max = 20) hospitals and took 1.2 ± 1.7 (max = 6.6) years for confirmed diagnosis. On average, 5.0 ± 9.6 misdiagnoses occurred, and the per-patient diagnoses cost was USD ($) 7576. After GD confirmation, 8 (16.3%) patients received no treatment, 40 (81.6%) received pharmacotherapy, 10 (20.4%) received surgery, 38 (77.6%) received outpatient service (8.8 ± 9.1 times/annually), and 37 (77.5%) received inpatient service (4.0 ± 3.5 times/annually). Annual per-patient COI was USD ($) 49,925 (95% confidence interval: 29,178, 70,672). Average direct healthcare cost was $41,816, including pharmaceutical ($29,908), inpatient ($7,451), and outpatient ($1,838). Productivity loss per-caregiver was $1,980, and their Zarit Burden Inventory score was moderate-severe (48.6 ± 19.6). Both patients/caregivers reported lower social support (32.4 ± 7.4, 34.9 ± 7.6), two times higher PSQI (7.9 ± 2.9, 8.7 ± 3.6), and half lower SF-36 (41.3 ± 18.6, 46.5 ± 19.3) than those reported for healthy Chinese individuals. Conclusions The high misdiagnosis rate, together with delayed diagnosis, substantial costs, and deteriorated health-related quality of life of GD patients as well as their heavy care burden, calls for extreme attention from policymakers in China. Further efforts of government and society are urgently demanded, including pharmaceutical reimbursement, screening newborns, developing precise diagnostic tools, and training doctors.

scholarly journals Analysis of the Situation of Unmet Health Care Need Among Extremely Rare Disease of Gaucher

2020 ◽  
Author(s):  
Xinye Qi ◽  
Jiao Xu ◽  
Linghan Shan ◽  
Ye Li ◽  
Yu Cui ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Social Support ◽  
Health Care ◽  
Health Service ◽  
Health Service Utilization ◽  
Health Related Quality ◽  
Sf 36 ◽  
Related Quality ◽  
Health Related

Abstract BackgroundThe diagnosis and health care of patients with rare diseases present a tremendous challenge worldwide. This study estimated the health service utilization, cost of illness, and patients with Gaucher disease (GD)’s/caregivers’ health-related quality of life in China.MethodAn online retrospective survey of patients with GD and their caregivers was conducted during May-June 2018. Socio-demographic, health service utilization, disease-related expenses, social support, sleep quality (Pittsburgh Sleep Quality Index [PSQI]), and the Short Form Health Survey (SF-36) were investigated. Using self-reported information, we estimated the annual cost of illness, including direct medical, direct non-medical, and indirect medical costs. ResultsForty patients and their 49 caregivers were surveyed. The patients’ onset age of GD was 9.3±10.9; their disease course was 3.5±3.1 years. 21 (42.9%) patients had ≥2 caregivers, but 35 (71.4%) caregivers reported have no experience as a caregiver. 79.6% caregivers have stopped working, and 87.8% changed weekly working schedule. Before final diagnosis, patients visited 3.9±3.1 (max=20) hospitals and took 1.2±1.7 (max=6.6) years for confirmed diagnosis. On average, 5.0±9.6 misdiagnoses occurred, and the per-patient diagnoses cost was USD ($) 7,576. After GD confirmation, 8 (16.3%) patients received no treatment, 40 (81.6%) received pharmacotherapy, 10 (20.4%) received surgery, 38 (77.6%) received outpatient service (8.8±9.1 times/annually), and 37 (77.5%) received inpatient service (4.0±3.5 times/annually). Annual per-patient cost was USD ($) 49,925 (95% confidence interval: 29,178, 70,672). Average direct medical cost was $41,816, including pharmaceutical ($29,908), inpatient ($7,451), and outpatient ($1,838). Productivity loss per-caregiver was $1,980, and their Zarit Burden Inventory score was moderate-severe (48.6±19.6). Both patients/caregivers reported lower social support (32.4±7.4, 34.9±7.6), two times higher PSQI (7.9±2.9, 8.7±3.6), and half lower SF-36 (41.3±18.6, 46.5±19.3) than those reported for healthy Chinese individuals.ConclusionThis study finds out that fill the unmet need for extremely rare diseases is very challenging. The high misdiagnosis rate, together with delayed diagnosis, substantial costs, and deteriorated health-related quality of life of GD patients as well as their heavy care burden, calls for extreme attention from policymakers in China. Further efforts of government and society are urgently demanded, including pharmaceutical reimbursement, screening newborns, developing precise diagnostic tools, and training doctors.

scholarly journals Health-Related Quality of Life in Older Coastal Residents After Multiple Disasters

2016 ◽  
Vol 11 (1) ◽  
pp. 90-96 ◽  
Author(s):  
Katie E. Cherry ◽  
Laura Sampson ◽  
Sandro Galea ◽  
Loren D. Marks ◽  
Kayla H. Baudoin ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Social Support ◽  
Older Adults ◽  
Social Engagement ◽  
Health Related Quality ◽  
Sf 36 ◽  
Related Quality ◽  
Health Related ◽  
Coastal Residents

AbstractObjectiveExposure to multiple disasters, both natural and technological, is associated with extreme stress and long-term consequences for older adults that are not well understood. In this article, we address age differences in health-related quality of life in older disaster survivors exposed to the 2005 Hurricanes Katrina and Rita and the 2010 BP Deepwater Horizon oil spill and the role played by social engagement in influencing these differences.MethodsParticipants were noncoastal residents, current coastal residents, and current coastal fishers who were economically affected by the BP oil spill. Social engagement was estimated on the basis of disruptions in charitable work and social support after the 2005 hurricanes relative to a typical year before the storms. Criterion measures were participants’ responses to the SF-36 Health Survey which includes composite indexes of physical (PCS) and mental (MCS) health.ResultsThe results of logistic regressions indicated that age was inversely associated with SF-36 PCS scores. A reduction in perceived social support after Hurricane Katrina was also inversely associated with SF-36 MCS scores.ConclusionsThese results illuminate risk factors that impact well-being among older adults after multiple disasters. Implications of these data for psychological adjustment after multiple disasters are considered. (Disaster Med Public Health Preparedness. 2017;11:90–96)

Social support and health-related quality of life among type 2 DM (T2DM) patients attending a public hospital in Lagos, Nigeria

2019 ◽  
Author(s):  
Olufolake Olabode ◽  
Timothy Omoluru ◽  
Olawunmi Olagundoye ◽  
Akinyele Akinlade ◽  
Henry Akujobi ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Social Support ◽  
Public Hospital ◽  
Health Related Quality ◽  
Type 2 Dm ◽  
Related Quality ◽  
Health Related

scholarly journals Impact of malnutrition on health-related quality of life in persons receiving dialysis: a prospective study

2021 ◽  
pp. 1-24
Author(s):  
Daniela Viramontes-Hörner ◽  
Zoe Pittman ◽  
Nicholas M Selby ◽  
Maarten W Taal
Keyword(s):  
Quality Of Life ◽  
Prospective Study ◽  
Health Related Quality ◽  
Poor Hrqol ◽  
Sf 36 ◽  
Related Quality ◽  
Health Related ◽  
The Impact ◽  
Over Time

Abstract Health-related quality of life (HRQoL) is severely impaired in persons receiving dialysis. Malnutrition has been associated with some measures of poor HRQoL in cross-sectional analyses in dialysis populations, but no studies have assessed the impact of malnutrition and dietary intake on change in multiple measures of HRQoL over time. We investigated the most important determinants of poor HRQoL and the predictors of change in HRQoL over time using several measures of HRQoL. We enrolled 119 haemodialysis and 31 peritoneal dialysis patients in this prospective study. Nutritional assessments (Subjective Global Assessment [SGA], anthropometry and 24-hour dietary recalls) and HRQoL questionnaires (Short Form-36 [SF-36] mental [MCS] and physical component scores [PCS] and European QoL-5 Dimensions [EQ5D] health state [HSS] and visual analogue scores [VAS]) were performed at baseline, 6 and 12 months. Mean age was 64(14) years. Malnutrition was present in 37% of the population. At baseline, malnutrition assessed by SGA was the only factor independently (and negatively) associated with all four measures of HRQoL. No single factor was independently associated with decrease in all measures of HRQoL over 1 year. However, prevalence/development of malnutrition over one year was an independent predictor of 1-year decrease in EQ5D HSS and 1-year decrease in fat intake independently predicted the 1-year decline in SF-36 MCS and PCS, and EQ5D VAS. These findings strengthen the importance of monitoring for malnutrition and providing nutritional advice to all persons on dialysis. Future studies are needed to evaluate the impact of nutritional interventions on HRQoL and other long-term outcomes.

scholarly journals Effect of multimorbidity patterns on the decline in health-related quality of life: a nationwide prospective cohort study in Japan

BMJ Open ◽  
2021 ◽  
Vol 11 (6) ◽  
pp. e047812
Author(s):  
Takuya Aoki ◽  
Shunichi Fukuhara ◽  
Yasuki Fujinuma ◽  
Yosuke Yamamoto
Keyword(s):  
Quality Of Life ◽  
Cohort Study ◽  
Clinical Outcomes ◽  
Prospective Cohort ◽  
Summary Score ◽  
Health Related Quality ◽  
Sf 36 ◽  
Related Quality ◽  
Health Related

ObjectivesLongitudinal studies, which consider multimorbidity patterns, are useful for better clarifying the effect of multimorbidity on health-related quality of life (HRQoL) and for identifying the target population with poorer clinical outcomes among patients with multimorbidity. This study aimed to examine the effects of different multimorbidity patterns on the decline in HRQoL.DesignNationwide prospective cohort study.SettingJapanese adult residents.ParticipantsResidents aged ≥50 years selected by the quota sampling method.Primary outcome measureClinically relevant decline in HRQoL was defined as a 0.50 SD (5-point) decrease in the 36-Item Short Form Health Survey (SF-36) component summary scores for 1 year.ResultsIn total, 1211 participants completed the follow-up survey. Among the multimorbidity patterns identified using confirmatory factor analysis, multivariable logistic regression analyses revealed that high cardiovascular/renal/metabolic and malignant/digestive/urologic pattern scores were significantly associated with the clinically relevant decline in SF-36 physical component summary score (adjusted OR (aOR)=1.25, 95% CI: 1.08 to 1.44 and aOR=1.28, 95% CI: 1.04 to 1.58, respectively). High cardiovascular/renal/metabolic pattern score was also significantly associated with the clinically relevant decline in SF-36 role/social component summary score (aOR=1.23, 95% CI: 1.06 to 1.42).ConclusionsOur study revealed that multimorbidity patterns have different effects on the clinically relevant decline in HRQoL for 1 year. These findings can be useful in identifying populations at high risk and with poor clinical outcomes among patients with chronic diseases and multimorbidity for efficient resource allocation.

Factors Affecting Health-Related Quality of Life After the Arterial Switch Operation

2021 ◽  
Vol 12 (3) ◽  
pp. 344-351
Author(s):  
Julie Cleuziou ◽  
Anna-Katharina Huber ◽  
Martina Strbad ◽  
Masamichi Ono ◽  
Alfred Hager ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Short Form ◽  
Arterial Switch Operation ◽  
Health Related Quality ◽  
Switch Operation ◽  
Sf 36 ◽  
Related Quality ◽  
Health Related ◽  
Cardiac Medication

Background: Long-term morbidity and mortality outcomes of the arterial switch operation (ASO) in patients with transposition of the great arteries and Taussig-Bing anomaly are excellent. With an increasing number of patients reaching adolescence and adulthood, more attention is directed toward quality of life. Our study aimed to determine the health-related quality of life (hrQoL) outcomes in patients after the ASO and identify factors influencing their hrQoL. Methods: In this cross-sectional study, hrQoL of patients after ASO was assessed with the German version of the Short Form-36 (SF-36) and the potential association of specified clinical factors was analyzed. Patients of at least 14 years of age who underwent ASO in our institution from 1983 were considered eligible. Results: Of the 355 questionnaires sent to eligible patients, 261 (73%) were available for analysis. Compared to the reference population, patients who had undergone ASO had a significantly higher score in all subscales of the SF-36 except for vitality ( P < .01). Patients with an implanted pacemaker ( P = .002), patients who required at least one reoperation ( P < .001), and patients currently taking cardiac medication ( P < .004) or oral anticoagulation ( P = .036) had lower physical component scores compared to patients without these factors. Conclusions: Patients’ self-assessed and self-reported hrQoL after ASO (using German version of the Short Form 36) is very good. In this population, hrQoL is influenced by reoperation, the need for a pacemaker, and current cardiac medication or anticoagulant use. The development of strategies designed to mitigate or minimize the requirements for, and/or impact of these factors may lead to better hrQoL in this patient population.

Health-related qualıty of lıfe, sleep qualıty, and depressıon in peritoneal dialysis and hemodıalysıs patıents

2011 ◽  
Vol 16 (2) ◽  
pp. 198-206 ◽  
Author(s):  
Kultigin Turkmen ◽  
Raziye Yazici ◽  
Yalcin Solak ◽  
Ibrahim Guney ◽  
Lutfullah Altintepe ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Peritoneal Dialysis ◽  
Sleep Quality ◽  
Hemodialysis Patients ◽  
Health Related Quality ◽  
Related Quality ◽  
Health Related
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