scholarly journals The impact of demographic and clinical characteristics on the trajectories of health-related quality of life among patients with Fabry disease

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Solrun Sigurdardottir ◽  
Birgitte Bjerkely ◽  
Trond G. Jenssen ◽  
Per Mathisen ◽  
Charlotte von der Lippe ◽  
...  

Abstract Background Fabry disease (FD) is an X-linked lysosomal storage disorder characterized by multiorgan dysfunction. Since individuals with FD usually experience progressive clinical disease manifestations, their health-related quality of life (HRQOL) is expected to change over time. However, there is limited longitudinal research examining HRQOL outcomes in individuals with FD. We aimed to: assess longitudinal outcomes in HRQOL in adults with FD; examine the physical- and mental HRQOL trajectories at the initial registration (baseline), 3–5 year, and 7–13 year follow-ups; and evaluate the possible associations of age, sex and medical complications with the physical- and mental HRQOL trajectories. Methods Forty-three individuals with FD (53% female) who were aged 18 to 81 years at baseline attended clinical follow-up visits between 2006 and 2020. Medical records were extracted retrospectively. Demographics and the 36-item Short-Form Health Survey (SF-36) were recorded at scheduled visits, except for the last data collection which was prospectively obtained in 2020. The physical (PCS) and mental (MCS) composite scores (SF-36) were chosen as outcome measures. Results The eight SF-36 domain scores were stable over a span of 13 years, and only physical- and social functioning domains worsened clinically over this follow-up period. Mean baseline SF-36 domain scores were all significantly lower (decreased HRQOL) in the FD sample compared with Norwegian population norms. Two hierarchical linear models were run to examine whether demographics and medical complications (measured at the last clinical visit) predicted physical and mental HRQOL trajectories. Age above 47 years (p < 0.001), male sex (p = 0.027), small fibre neuropathy (p < 0.001), renal dysfunction (p < 0.001), and cerebrovascular events (p = 0.003) were associated with lower HRQOL over time. No significant interactions were found between the time of follow up and the abovementioned predictors of HRQOL. Conclusions Overall HRQOL trajectories remained stable between baseline, 3–5 year, and 7–13 year follow-ups, with the majority of individuals reporting decreased physical and mental HRQOL. Medical complications in combination with older age and male sex are important predictors of lower HRQOL in FD. Awareness of this relationship is valuable both for health care providers and for patients. The findings provide indicators that can guide treatment decisions to improve physical and mental HRQOL outcomes.

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
José G. M. Hofhuis ◽  
Augustinus J. P. Schrijvers ◽  
Tjard Schermer ◽  
Peter E. Spronk

AbstractMany Intensive Care (ICU) survivors experience long lasting impairments in physical and psychological health as well as social functioning. The objective of our study was to evaluate these effects up to 10 years after ICU discharge. We performed a long-term prospective cohort study in patients admitted for longer than 48 h in a medical-surgical ICU. We evaluated health-related quality of life (HRQOL) before ICU admission using the Short-form-36 (SF-36), at ICU discharge, at hospital discharge and at 1, 2, 5 and 10 years follow up (all by patients). Changes in HRQOL were assessed based on linear mixed modeling. We included a total of 749 patients (from 2000 to 2008). During 10 years 475 (63.4%) patients had died, 125 (16.7%) patients were lost to follow up and 149 (19.9%) patients could be evaluated. The mean scores of four HRQOL dimensions (i.e., physical functioning (p < 0.001; mean 54, SD 32, effect size 0.77, 95% CI [0.54–1.0]), role-physical (p < 0.001; mean 44, SD 47, effect size 0.65, 95% CI [0.41–0.68] general health (p < 0.001; mean 52, SD 27, effect size 0.48; 95% CI 0.25–0.71) and social functioning (p < 0.001; mean 72, SD 32, effect size 0.41, 95% CI [0.19–0.64]) were still lower 10 years after ICU discharge compared with pre-admission levels (n = 149) and with an age reference population. Almost all SF-36 dimensions changed significantly over time from ICU discharge up to 10 years after ICU discharge. Over the 10 year follow up physical functioning of medical-surgical ICU survivors remains impaired compared with their pre-admission values and an age reference population. However, effect sizes showed no significant differences suggesting that surviving patients largely regained their age-specific HRQOL at 10 years.


2021 ◽  
Author(s):  
Rose K Sia ◽  
Denise S Ryan ◽  
Daniel I Brooks ◽  
Janice M Kagemann ◽  
Kraig S Bower ◽  
...  

ABSTRACT Purpose The purpose of this study was to assess visual and health-related quality of life (QOL) among U.S. military service members who sustained combat ocular trauma (COT) with or without associated traumatic brain injury (TBI). Methods This was a single-center, prospective observational study of U.S. service members (n = 88) with COT who were treated at Walter Reed National Military Medical Center. Participants completed the National Eye Institute Visual Function Questionnaire (VFQ-25) at enrollment and at follow-up (&gt;1 year) and supplemental surveys: Neurobehavioral Symptom Inventory, the Medical Outcomes 36-item Short Form Survey (SF-36), and Mayo-Portland Adaptability Inventory. Results Initial and follow-up VFQ-25 showed a statistically significant increase in median scores for near activities (initial: 75.0, follow-up 83.3; P = .004) and peripheral vision (initial: 50.0, follow-up: 75.0; P = .009) and in composite scores (initial: 79.5, follow-up: 79.8; P = .022). Comparing those who did (n = 78) and did not (n = 8) have a TBI history, there were no significant differences in median change in VFQ-25 composite scores (with TBI: 2.3 vs. no TBI: 10.7; P = .179). Participants with a TBI history had a significantly lower median SF-36 General Health score (with TBI: 67.5 vs. no TBI: 92.5; P = .009) Conclusions Vision-related QOL of COT patients is generally good in the long term. However, those with both COT and a history of TBI conditions showed significantly worse functioning in several domains than those without TBI. As TBI is a common finding in COT, this association is an important factor impacting this population’s overall clinical presentation and daily functions.


PLoS ONE ◽  
2021 ◽  
Vol 16 (10) ◽  
pp. e0257981
Author(s):  
Jung-Hwa Ryu ◽  
Tai Yeon Koo ◽  
Han Ro ◽  
Jang-Hee Cho ◽  
Myung-Gyu Kim ◽  
...  

Renal functional deterioration is associated with physical and mental burdens for kidney transplant (KT) and chronic kidney disease (CKD) patients. However, the change in health-related quality of life (HRQOL) over time in KT patients compared to that of native CKD patients has not been evaluated. We addressed this issue using KT patients registered in the KNOW-KT cohort study and patients at CKD stage 1–3 registered in the KNOW-CKD cohort study. HRQOL scores were assessed using the Kidney Disease Quality of Life Short Form at baseline, 2-, and 4-years follow-up in 842 KT patients and at baseline and 5-year follow-up in 1,355 CKD patients. SF-36 scores declined at the 4-year follow-up, whereas CKD-targeted scores showed no change in the KT group. In contrast, CKD-targeted scores as well as SF-36 scores were decreased at the 5-year follow-up in CKD patients. When prognostic factors were analyzed for longitudinal HRQOL data over time, renal functions, diabetes, cardiovascular and cerebrovascular diseases, hemoglobin level, marital status, income, employment, and health care were significant prognostic factors. Furthermore, KT was an independent prognostic factor for better HRQOL. These results highlight that KT can offer a better HRQOL than that of CKD patients, even when renal function is similar.


2015 ◽  
Vol 16 (6) ◽  
pp. 633-641 ◽  
Author(s):  
Anne Henriette Paulsen ◽  
Tryggve Lundar ◽  
Karl-Fredrik Lindegaard

OBJECT Treatment for hydrocephalus has not advanced appreciably since the advent of CSF shunts more than 50 years ago. The outcome for pediatric patients with hydrocephalus has been the object for several studies; however, much uncertainty remains regarding the very long term outcome for these patients. Shunting became the standard treatment for hydrocephalus in Norway during the 1960s, and the first cohorts from this era have now reached middle age. Therefore, the objective of this study was to review surgical outcome, mortality, social outcome, and health-related quality of life in middle-aged patients treated for hydrocephalus during childhood. METHODS Data were collected in all patients, age 14 years or less, who required a CSF shunt during the years 1967–1970. Descriptive statistics were assessed regarding patient characteristics, surgical features, social functioning, and work participation. The time and cause of death, if applicable, were also determined. Kaplan-Meier survival estimates were used to determine the overall survival of patients. Information regarding self-perceived health and functional status was assessed using the 36-Item Short Form Health Survey (SF-36) and the Barthel Index score. RESULTS A total of 128 patients were included in the study, with no patient lost to follow-up. Of the 128 patients in the study, 61 (47.6%) patients died during the 42–45 years of observation. The patients who died belonged to the tumor group (22 patients) and the myelomeningocele group (13 patients). The mortality rate was lowered to 39% if the patients with tumors were excluded. The overall mortality rates at 1, 2, 10, 20, and 40 years from time of initial shunt insertion were 16%, 24%, 31%, 40%, and 48% respectively. The incidence of shunt-related mortality was 8%. The majority of children graduated from a normal school (67%) or from a school specializing in education for physically handicapped children (20%). Self-perceived health was significantly poorer in 6 out of 8 domains assessed by SF-36 as compared with the background population. Functional status among the survivors varied greatly during the follow-up period, but the majority of patients were self-dependent. A total of 56% of the patients were socially independent, and 42% of the patients were employed. CONCLUSIONS Approximately half of the patients are still alive. During the 42–45 year follow-up period, the mortality rate was 48%. Two deaths were due to acute shunt failure, and at least 8% of the deaths were shunt related (probable or late onset). The morbidity in middle-aged individuals treated for pediatric hydrocephalus is considerable. The late mortality rate was low, but not negligible. Twelve patients died during the last 2 decades, 1 of whom died because of acute shunt failure. Although the shunt revision rate was decreasing during the study period, many patients required shunt surgery during adulthood. Forty-one revisions in 21 patients were performed during the last decade. Thus, there is an obvious need for life-long follow-up in these patients.


2020 ◽  
Vol 22 (4) ◽  
pp. 187-192
Author(s):  
Joseph M. Gasper ◽  
Megan Lewis ◽  
Anne Kroeger ◽  
Ben Muz ◽  
Nicholas LaRocca ◽  
...  

Abstract Background: Multiple sclerosis adult day programs (MSADPs) offer life-enhancing services for individuals and informal caregivers affected by multiple sclerosis (MS), including medical care, rehabilitation therapies, nutrition therapy, cognitive training, tailored education, exercise programs, and social interaction. The purpose of this study was to examine the effects of MSADPs on health-related quality of life (HRQOL) and health care utilization of persons with MS and HRQOL and well-being of informal caregivers. Methods: Using a quasi-experimental design, outcomes between baseline and 1-year follow-up in persons with MS and informal caregivers who used MSADP services and a comparison group of similar persons with MS and caregivers who did not use MSADP services were compared. For persons with MS, outcomes included standardized measures of physical and mental HRQOL and health care utilization. For caregivers, outcomes included physical and mental HRQOL and well-being. Changes in outcomes between baseline and follow-up were examined using propensity score–weighted difference-in-differences regression analysis. Results: For persons with MS, MSADP use had a significant positive effect on 12-Item Short Form Health Survey physical component scores, although the difference was not clinically meaningful. Use of MSADPs did not have effects on any other outcomes for persons with MS or caregivers. Conclusions: Use of MSADPs did not show a clinically meaningful effect on HRQOL for persons with MS or informal caregivers. The MSADPs do not seem to offer sustained benefits to persons with MS or caregivers, but the possibility of initial short-term benefits cannot be ruled out.


2021 ◽  
Author(s):  
MeiXuan Lin ◽  
Liqun Huang ◽  
Danwen Zheng ◽  
Linjie Zhang ◽  
Bing Feng ◽  
...  

Abstract Background: COVID-19 is a multi-systemic disease that is highly contagious and pathogenic. The long-term consequences of it are not yet clear, as is whether society and life can return to a healthy state. Long-term assessment of their health-related quality of life (HRQoL) is essential. This study aimed to investigate HRQoL and its risk factors in COVID-19 survivors at a follow-up of 6-month. Methods: A multicenter cross-sectional survey was conducted among 192 COVID-19 patients with confirmed age ≥ 18 years who were discharged from various hospitals in Wuhan from January to April 2020. The demographic characteristics, clinical characteristics, and laboratory results of the study subjects were obtained from the hospital's medical records. Survivors' HRQoL was assessed using the Short Form 36 (SF-36), cognition was assessed using the ascertain dementia eight-item informant questionnaire (AD8), and survivors' pulmonary function were examined. All participants in this study completed the survey and testing at Hubei Provincial Hospital of Chinese and Western Medicine. SF-36 scores were compared with the Chinese norm, and logistic regression and multivariate analysis were used to investigate the factors affecting HRQoL in COVID-19 survivors. Results: SF-36 showed significant differences in HRQoL between COVID-19 survivors and the general Chinese population ( P< 0.05).Multiple linear regression demonstrated that age was negatively correlated with physical functioning (PF), role-physical limitation (RP) and social functioning (SF) ( P <0.05). Bodily pain (BP), vitality (VT), SF and role-emotional limitation (RE) were negatively correlated with females ( P <0.05). Length from discharge to follow‐up was positively correlated with PF and RP ( P <0.05). Abnormal cognitive function was negatively correlated with PF, RP, general health (GH), VT, SF, RE and mental health (MH) ( P <0.05). Abnormal Carbon Monoxide Diffusing Capacity (DLCO%<80%) was significantly negatively correlated with PF and SF ( P <0.05).In addition, there was a significant negative correlation between Coronary heart disease and RP, GH, VT and RE ( P <0.05).Logistic regression analysis demonstrated that age(OR 1.032) and AD8 scores (OR 1.203)were risk factors associated with a low physical component summary (PCS) score. Length from discharge to follow‐up (OR 0.971) was the protective factor for PCS score. Abnormal cognitive function (OR 1.543) was a significant determinant associated with a mental component summary (MCS)<50 in COVID-19 patients. Conclusions: The HRQoL of COVID-19 survivors remains to be improved at six-month follow-up. Future studies should track HRQoL in older adults, women, patients with abnormal DLCO, and abnormal cognitive function for a long time and provide them with rehabilitation advice and guidance.


2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Xuxi Zhang ◽  
Siok Swan Tan ◽  
Carmen Betsy Franse ◽  
Tamara Alhambra-Borrás ◽  
Arpana Verma ◽  
...  

Abstract Background Physical activity (PA) may play a key role in healthy aging and thus in promoting health-related quality of life (HRQoL). However, longitudinal studies on the association between PA and HRQoL are still scarce and have shown inconsistent results. In this study, we aimed to examine the longitudinal association between frequency of moderate PA and physical and mental HRQoL. Secondly, to assess the association between a 12-month change in frequency of moderate PA and HRQoL. Methods A 12-month longitudinal study was conducted in Spain, Greece, Croatia, the Netherlands, and the United Kingdom with 1614 participants (61.0% female; mean age = 79.8; SD = 5.2) included in the analyses. Two categories of the self-reported frequency of moderate PA including 1) ‘regular frequency’ and 2) ‘low frequency’ were classified, and four categories of the change in frequency of moderate PA between baseline and follow-up including 1) ‘continued regular frequency’, 2) ‘decreased frequency’, 3) ‘continued low frequency’ and 4) ‘increased frequency’ were identified. Physical and mental HRQoL were assessed by the 12-Item Short-Form Health Survey (SF-12). Results The frequency of moderate PA at baseline was positively associated with HRQoL at follow-up. Participants with a continued regular frequency had the highest HRQoL at baseline and follow-up. Participants who increased the frequency of moderate PA from low to regular had better physical and mental HRQoL at follow-up than themselves at baseline. After controlling for baseline HRQoL and covariates, compared with participants who continued a regular frequency, participants who decreased their frequency had significantly lower physical (B = -4.42; P < .001) and mental (B = -3.95; P < .001) HRQoL at follow-up; participants who continued a low frequency also had significantly lower physical (B = -5.45; P < .001) and mental (B = -4.10; P < .001) HRQoL at follow-up. The follow-up HRQoL of participants who increased their frequency was similar to those who continued a regular frequency. Conclusions Maintaining or increasing to a regular frequency of PA are associated with maintaining or improving physical and mental HRQoL. Our findings support the development of health promotion and long-term care strategies to encourage older adults to maintain a regular frequency of PA to promote their HRQoL.


1996 ◽  
Vol 7 (5) ◽  
pp. 763-773 ◽  
Author(s):  
K M Beusterien ◽  
A R Nissenson ◽  
F K Port ◽  
M Kelly ◽  
B Steinwald ◽  
...  

As a component of the open-label, multicenter National Cooperative Recombinant Human Erythropoietin (Epo) Study, the health-related quality-of-life effects of Epo therapy were assessed in 484 dialysis patients who had not previously been treated with Epo therapy (New-to-Epo) and 520 dialysis patients who were already receiving Epo therapy at the time of study enrollment (Old-to-Epo). Using scales from the Medical Outcomes Study 36-item Short Form Health Survey (SF-36), health-related quality of life was assessed on study enrollment (baseline) and at an average of 99 days follow-up. At baseline, SF-36 scores for Old- and New-to-Epo patients were well below those observed in the general population, reflecting substantial impairments in functional status and well-being among patients with chronic renal failure. Significant improvements from baseline to follow-up were observed among New-to-Epo patients in vitality, physical functioning, social functioning, mental health, looking after the home, social life, hobbies, and satisfaction with sexual activity (P < 0.05 for each). The mean improvements in hematocrit values among New-to-Epo and Old-to Epo patients were 4.6 and 0.3, respectively. At the time of follow-up, SF-36 scores for New-to-Epo patients were comparable with those observed among Old-to-Epo patients, whose scores did not change significantly from baseline to follow-up. Analysis of the relationship between Epo therapy, hematocrit values, and health-related quality of life suggest that some of the beneficial quality-of-life effects of Epo are mediated through a change in hematocrit level.


2021 ◽  
Vol 36 (Supplement_1) ◽  
Author(s):  
Vasileios Koutlas ◽  
Eirini Tzalavra ◽  
Vasileios Tatsis ◽  
Anila Duni ◽  
Haralambos Pappas ◽  
...  

Abstract Background and Aims Kidney transplantation is recognized as the treatment of choice for patients with End Stage Kidney Disease (ESKD). Improvement in short term patient and graft survival of kidney transplant recipients (KTRs), has shifted interest to the long-term outcomes and Health-Related Quality of Life (HRQoL) estimation is closely related. Thus, reliable evaluation of HRQoL with disease specific questionnaires is of great importance. The aim of this study was to assess the HRQoL in adult KTRs of our Transplant Unit with Kidney Transplant Questionnaire 25 (KTQ-25) and the 36-item Short Form Health Survey (SF-36). Method The Greek version of the recently translated and adapted by our team disease specific instrument KTQ-25 and the Greek SF-36 were administered in KTRs during their routine visit in the transplant outpatient clinic. Patients who met the eligibility criteria (time since transplant≥1 year, aged≥18 year old, functioning transplant) were included after providing written informed consent. Results A total of 84 KTRs (59 males; mean age 53.45±10.72; mean e-GFR 47.69±15.07; average time since transplantation 55.68±48.26 months) completed the two questionnaires. The scores in each dimension of KTQ-25 were: Physical Symptoms 3.98±1.60, Fatigue 5.30±1.36, Uncertainty/Fear 5.16±1.33, Appearance 6.31±0.94, Emotions 5.03±1.07 and Total Score 5.20±0.87. The mean SF-36 Physical Component Score (PCS) and Mental Component Score (MCS) scores were 47.98±8.70 and 46.94±9.70 respectively. Fatigue (p=0.009), Uncertainty/Fear (p=0.008) and Total Score (p&lt;0.001) were better in male sex. Physical Symptoms (p=0.013) was better in KTRs with children than those with no children. There was positive correlation of e-GFR with Fatigue (p 0.002), Uncertainty/Fear (p=0.034) and Total Score (p=0.017). Hgb was positive correlated with Fatigue (p&lt;0.001), Uncertainty/Fear (p&lt;0.001), Emotions (p=0.031) and Total Score (p&lt;0.001). KTRs who were receiving EPO for anemia had worse scores in all dimension of KTQ-25 (Physical Symptoms p=0.001; Fatigue p=0.001; Uncertainty/Fear p=0.021; Emotions p=0.010; Total Score p=0.002) except Appearance. Appearance was negative correlated with Weight (p=0.001) and BMI (p=0.001). Total number of daily taken pills was also significantly negative correlated with Fatigue (p=0.006) and Total Score (p=0.007). No differences found between different immunosuppressive therapies. Conclusion In our cohort of Greek adults KTRs maximum KTQ-25 score was obtained in Appearance dimension and the minimum in Physical Symptoms dimension. Fatigue, Uncertainty/Fear dimensions and Total Score were better for male sex and for KTRs with higher e-GFR and Hgb levels. Physical Symptoms score was better only for KTRs having children. Overweight and high BMI negatively affected A dimension. This is the first study to use a specific-disease tool for evaluating HRQoL in Greek KTRs and among our significant results is that better graft function is significantly correlated with better HRQoL.


2019 ◽  
Vol 29 (1) ◽  
pp. 99-107
Author(s):  
Gudrun Rohde ◽  
Kari Hansen Berg ◽  
Are Hugo Pripp ◽  
Anne Prøven ◽  
Glenn Haugeberg

Abstract Background Axial spondyloarthritis (ax-SpA) causes pain, fatigue, stiffness, loss of physical function and impaired health-related quality of life (HRQOL). Aims The study aimed to explore the changes in HRQOL over 5 years in patients with ax-SpA and to identify baseline predictors associated with changes in HRQOL assessed using three HRQOL measures. Methods Demographic, disease, medication and HRQOL data were collected at baseline and at 5-year follow-up. HRQOL was assessed using SF-6D, 15D and SF-36. Analyses involved McNemar, independent paired t tests and multiple regression. Results In the 240 (women 31%, men 69%) ax-SpA patients assessed (mean age 46 years), measures reflecting disease activity decreased and co-morbidities increased, and more patients were treated with biologic drugs during follow-up. No deterioration in HRQOL was observed between baseline and 5-year follow-up; indeed, there was a significant increase in SF-6D and SF-36 PCS scores. Improvement in HRQOL measured by SF-6D was associated with younger age, higher education, low Bath Ankylosing Spondylitis (BAS) Activity Index (BASDAI), high BAS Patient Global Score and high C-reactive protein; improvement in SF-36 PCS was associated with younger age, higher education, low BASDAI and no use of biological treatment at baseline. Conclusion Our ax-SpA outpatient clinic patients, with more patients treated with biologic drugs during the 5-year follow-up, did not deteriorate in HRQOL. In fact, the physical dimension in HRQOL improved over the years, as did measures reflecting disease activity. Our study adds evidence to the importance of suppressing inflammation to maintain and improve HRQOL in ax-SpA patients.


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