Gene therapy is now a reality for individuals with haemophilia, yet little is known regarding the quality-of-life impact of factor correction. As few data exist, and recognizing the analogy to liver transplantation (OLTX), we identified OLTX+ and OLTX- men in the ATHNdataset to compare post-OLTX factor VIII and IX on quality of life (QoL) by Haem-A-QoL and PROMIS-29.

OLTX- were matched to OLTX+ by age, race, and haemophilia type and severity. Deidentified demographic data, including post-transplant factor levels, genotype and target joint disease were analysed by descriptive statistics. Haem-A-Qol and PROMIS-29 were compared in OLTX+ and OLTX- by student's t-test and univariate regression models.

Of 86 people with haemophilia A (HA) or haemophilia B (HB) cared for at 10 haemophilia treatment centers (HTCs), 21 (24.4%) OLTX+ and 65 (75.6%) OLTX- were identified. OLTX+ and OLTX- had a similar frequency of target joint disease (p = .806), HA genotypes, null versus non-null (p = .696), and HIV infection (p = .316). At a median 9.2 years post-OLTX, median FVIII, .63 IU/mL [IQR 0.52-0.97] and FIX, .91 IU/mL [IQR .63-1.32], Haem-A-QoL, PROMIS-29, and HOT scores were comparable. Severe HA/HB had lower post-OLTX 'dealing with haemophilia' scores (p = .022) and higher 'sports and leisure' (p = .010) and 'view of yourself' scores (p = .024) than OLTX+ non-severe participants. Non-caucasian OLTX+ had significantly lower scores in sports and leisure (p = .042), future expectations (p = .021) and total score (p = .010).

Nine years after OLTX, QoL is comparable to OLTX-, but significantly better in OLTX+ with severe than non-severe disease and in caucasians than non-caucasians.

Given improvements in post-transplant patient and graft survival, there is a growing need to focus on patient experience and health-related quality of life (HRQOL). Though liver transplantation can be life-saving, it can also be associated with significant morbidity and complications. Patient HRQOL improves after transplantation, but it may not improve to that of age-matched cohorts. Understanding patient experience and the factors that contribute to it, including physical and psychological health, immunosuppression and medication adherence, return to employment or school, financial burden, and expectations, helps when thinking creatively about potential interventions to improve HRQOL.

The aim of this study was to assess employment rates and states and the factors influencing liver transplant recipients in China.

A cross-sectional study.

A total of 212 liver transplant recipients were included in this study. Questionnaires for general situation information and the Hospital Anxiety and Depression Scale, Perceived Social Support Scale and Chronic Disease Self-Efficacy Scale were used.

Among the liver transplantation, 212 recipients were included in the study, 125 (59%) were employed after liver transplantation compared with 148 (69.8%) who were employed before liver transplantation. Among the 125 recipients that returned to work, only 38.2% thought their employment states were good before liver transplantation, while 81.6% thought that their employment states were good after liver transplantation. Gender, age and employment status before transplantation, aetiology of liver disease, number of physical activities, per capita monthly income, comorbidity and depression were significantly associated with return to work. Self-efficacy, social support, liver function and nature of work were significantly associated with employment state.

Cognitive decline may prevent liver transplant (LT) recipients from staying healthy and independent. This study examined associations of objective and subjective, rated by LT recipients and caregivers, cognitive decline with patient-reported physical and psychological symptom distress, ability to perform household tasks, and workplace productivity among LT recipients. Sixty pairs of LT recipients and caregivers participated in this cross-sectional study. Subjective cognition was measured by the Everyday Cognition. Objective cognition was assessed with four cognitive tests, including the Repeatable Battery for the Assessment of Neuropsychological Status. Patient-reported outcomes were assessed with the Rotterdam Symptom Checklist-Modified, Profile of Mood States-Short Form, Creative Therapy Consultants Homemaking Assessment, and Work Limitations Questionnaire. Linear regression analyses related objective and subjective cognition to the patient-reported outcomes. While objective cognitive decline was not associated with any patient-reported outcomes, subjective cognitive decline was significantly associated with the outcomes. Higher LT recipient self-rated cognitive decline was associated with higher physical symptom distress ( β = 0.30, p = 0.006) and workplace productivity loss ( β = 14.85, p < 0.0001). Higher caregiver-rated cognitive decline was associated with lower household tasks performance ( β = -18.55, p = 0.015). Findings suggest to consider subjective cognition when developing an individualized post-transplant care plan.

Physical dysfunction, including exercise intolerance, is a major factor for delayed societal reintegration for patients who underwent living-donor liver transplantation (LDLT). However, what may contribute to early postoperative physical function is not well known. The purpose of this study is to elucidate the perioperative factors affecting early posttransplant exercise intolerance.

103 consecutive patients who underwent LDLT were enrolled, and 68 patients were retrospectively analyzed. We examined the relationship between postoperative exercise tolerance evaluated by a 6-minute walking distance (6MWD) at discharge after surgery and demographic data, surgical information, preoperative physical function, clinical course, and the postoperative decline in physical function with univariate and multivariate analyses.

Almost all patients were discharged within 3 months after surgery. The postoperative 6MWD was 408 ± 94 m (68 [61-84]% of the predicted value), and patients who had a low %6MWD at discharge had significantly lower preoperative physical function than patients who had a high %6MWD at discharge (grip strength: 29.8 ± 8.9 kgf vs. 23.0 ± 8.8 kgf, P < .01, knee extensor strength: 138.9 ± 59.4 Nm vs. 95.2 ± 42.1 Nm, P < .01). Multivariate analysis revealed that preoperative knee extensor strength (standardized β = 0.35, P < .01) and first postoperative walking day (standardized β = -0.22, P = .04) were independently associated with the postoperative %6MWD.

These results suggest that maintaining preoperative muscle strength and allowing for early postoperative mobilization might help to enhance the recovery of physical function and facilitate the patient's social reintegration after LDLT.

Background and Aims: This study serves to revisit the effects of liver transplantation (LT) on employment in an era of improving survival outcomes post-transplant, and to identify areas of improvement in the transplant process to better optimize post-LT employment and patient satisfaction. Methods: Prospectively, patients who had undergone LT at a single tertiary LT center were surveyed in person and by e-mail. Primary outcomes included employment rate pre- and post-LT, annual salary, weekly hours worked, barriers to re-employment, and patient satisfaction. Results: Responses were collected and analyzed from 121 patients who underwent LT. Pre-LT, 68 (56.1%) reported full-time employment, 13 (10.7%) part-time employment, and 40 (33.1%) unemployment. Post-LT, 26 (21.4%) reported continued full-time employment, 18 (14.9%) part-time employment, and 77 (63.6%) unemployment. Average weekly work hours decreased post-LT (16.1 h/week vs. 39.9 h/week). Mean annual salaries decreased post-LT (17 earning salary ≥$40,000 vs. 56 earning salary ≥$40,000). These outcomes differed from patient pre-LT expectations, with 81.0% of previously employed patients believing they would return to employment, resulting in decreased patient satisfaction. Patients working physically demanding jobs pre-LT were less likely to return to work. Reasons cited for lack of return to full employment included early fatigue and difficulty regaining physical strength. Conclusions: Re-employment rates remain low post-LT, which is particularly true for patients working physically active jobs. Fatigue is a significant barrier to re-employment and increased physical rehabilitation post-LT may prove to be beneficial. Patients should be given realistic expectations about return to employment prior to their LT.

Hip and knee replacements are regularly carried out for patients who work. There is little evidence about these patients' needs and the factors influencing their return to work. There is a paucity of guidance to help patients return to work after surgery and a need for structured occupational advice to enable them to return to work safely and effectively.

To develop an occupational advice intervention to support early recovery to usual activities including work that is tailored to the requirements of patients undergoing hip or knee replacements. To test the acceptability, practicality and feasibility of this intervention within current care frameworks.

An intervention mapping approach was used to develop the intervention. The research methods employed were rapid evidence synthesis, qualitative interviews with patients and stakeholders, a prospective cohort study, a survey of clinical practice and a modified Delphi consensus process. The developed intervention was implemented and assessed during the final feasibility stage of the intervention mapping process.

Orthopaedic departments in NHS secondary care.

Patients who were in work and intending to return to work following primary elective hip or knee replacement surgery, health-care professionals and employers.

Occupational advice intervention.

Development of an occupational advice intervention, fidelity of the developed intervention when delivered in a clinical setting, patient and clinician perspectives of the intervention and preliminary assessments of intervention effectiveness and cost.

A cohort study (154 patients), 110 stakeholder interviews, a survey of practice (152 respondents) and evidence synthesis provided the necessary information to develop the intervention. The intervention included information resources, a personalised return-to-work plan and co-ordination from the health-care team to support the delivery of 13 patient and 20 staff performance objectives. To support delivery, a range of tools (e.g. occupational checklists, patient workbooks and employer information), roles (e.g. return-to-work co-ordinator) and training resources were created. Feasibility was assessed for 21 of the 26 patients recruited from three NHS trusts. Adherence to the defined performance objectives was 75% for patient performance objectives and 74% for staff performance objectives. The intervention was generally well received, although the short time frame available for implementation and concurrent research evaluation led to some confusion among patients and those delivering the intervention regarding its purpose and the roles and responsibilities of key staff.

Implementation and uptake of the intervention was not standardised and was limited by the study time frame. Evaluation of the intervention involved a small number of patients, which limited the ability to assess it.

The developed occupational advice intervention supports best practice. Evaluation demonstrated good rates of adherence against defined performance objectives. However, a number of operational and implementation issues require further attention.

The intervention warrants a randomised controlled trial to assess its clinical effectiveness and cost-effectiveness to improve rates and timing of sustained return to work after surgery. This research should include the development of a robust implementation strategy to ensure that adoption is sustained.

Current Controlled Trials ISRCTN27426982 and PROSPERO CRD42016045235.

This project was funded by the National Institute for Health Research (NIHR) Health Technology Assessment programme and will be published in full in Health Technology Assessment; Vol. 24, No. 45. See the NIHR Journals Library website for further project information.

Young people (YP) born with biliary atresia (BA) are an emerging population for adult hepatologists with 40% to 45% of children entering adolescence with their native liver intact. For those requiring liver transplantation (LT) during adolescence, disparity on the waiting list and post-LT outcome for young adults compared with younger and older age groups has stimulated discussion about the optimal timing of listing. In this study, we review our experience of YP with BA requiring LT during adolescence and young adulthood.

Retrospective, single-center review of patients with BA requiring LT > 11 years.

Thirty-six YP (16 male) underwent LT between 1991 and 2014 at a median age of 16.6 (interquartile range [IQR], 14.2 to 19.5) years. The commonest indications for listing were refractory cholangitis (31%), synthetic failure (25%), and variceal bleeding (14%). Patients listed by the adult team (n = 14) waited longer than those listed by the pediatric team (10 [IQR, 7.7 to 24.6] vs 5.8 [IQR, 4.0 to 15.1] months; P < 0.05) and were more likely to require intensive care support at time of listing (29% vs 5%; P < 0.05). Admission to intensive care unit at listing was associated with poorer patient and graft survival and support from a multidisciplinary liver transition service improved survival. Liver disease severity scores did not correlate with time on waiting list or outcome.

YP with BA requires close monitoring by specialists familiar with their condition and timing for LT needs to be fine-tuned to avoid clinical decompensation and improve long-term outcomes.

The aim of the present study was to assess the employment rate among kidney and liver transplant recipients in Poland and identify the predictors of employment.

The study was based on a self-prepared questionnaire containing five parts: demographic data and professional, medical, physical and psychological factors. This is a multicentre study involving four hospitals in Poland.

The investigated group consisted of 285 individuals (92 women and 185 men; 8 no sex given) aged between 18 and 45 years who underwent kidney or liver transplantation 1-5 years prior. Because of missing data, 279 questionnaires were considered regarding employment status after transplantation and 277 when sex differences were considered.

A total of 120 patients (43%) worked full-time, 42 (15%) part-time and 117 (42%) remained unemployed, with a higher employment rate post-transplantation among men than women (60% vs 55%, p=0.04). More women performed white collar work (74% vs 41%, p=0.0003) and had to modify their working position than men (22% vs 7%, p=0.0118). Factors determining employment after transplantation included higher level of education (OR=27.08; p=0.0096), employment 1 year prior to transplantation (OR=11.05; p<0.001) and good psychological adaptation (OR=4.23; p<0.001). Men working the year before transplantation had higher education (OR=9.66; p=0.0356). Among men, more kidney recipients worked compared with liver recipients (OR=3.567; p=0.0417). Among women, age determined employment status (OR=0.89; p=0.0234).

Higher education is the most important predictor of employment after transplantation. Therefore, rehabilitation programmes including vocational activity should be directed to increase the level of education, both pretransplantation and post-transplantation if possible.

Background Return to work with or after a chronic disease is not a very well understood process, influenced by a variety of personal, professional, societal and medical factors. The aim of this study is to identify predictors for return to work 12 months after a solid organ transplant applying a bio-psycho-social model. Methods This study is based on patients included in the Swiss Transplant Cohort Study, a national prospective multicentre cohort, who underwent a first solid organ transplant (kidney, liver, heart, lung). Bio-psycho-social factors were tested and predictors of return to work identified using logistic regression models. Results Among the 636 patients included in the study, 49.8% (317) were employed 12 months post-transplant. The major predictor for returning to work 12 months posttransplant was pre-transplant employment status (OR 10.8). Accordingly, the population was stratified in employed and not employed pre-transplant groups. Age, self-perceived health (6 months post-transplant) and the transplanted organ were significantly associated with post-transplant employment status in both groups. Return to work was influenced by education, depression (6 month post-transplant) and waiting time in the employed pre-transplant group and by invalidity pension in the not employed pre-transplant group. Conclusion Employment status pre-transplant being highly associated with employment status post-transplant, the process promoting return to work should be started well before surgery. Biomedical, psychological and social factors must be taken into account to promote return to work in transplanted patients.

With improvements in the survival of liver transplantation (LT) recipients, the focus is shifting to patient quality of life (QOL), and employment is an important factor in aiding the social reintegration of LT patients. This study aims to evaluate the current employment status of liver graft recipients and various factors that may hinder reemployment.

Fifty patients above age 18 who underwent either living or deceased donor LT at a single center from March 2009 to July 2016 were interviewed during their visit to the outpatient clinic. The internally developed questionnaire consisted of 10 items. The Karnofsky Performance Scale and EQ-5D were used to evaluate patient function and QOL.

A total of 25 (50%) patients returned to work after transplantation (the working group), and 21 (84%) patients in the working group returned to work within the first year after transplantation. In the non-working group (n=25), 17 (68%) answered that their health was the reason for unemployment. Fatigue and weakness were the most frequent symptoms.

The data shows that as many as 50% of total patients returned to work after receiving LT. Fatigue and weakness were the most common complaints of the unemployed group, and resolving the causes of these symptoms may help to increase the employment rate.

The majority of liver transplant recipients survive long term after the procedure.

To assess if this positive outcome is associated with improved employment post-transplant.

A systematic review of publications between 2001 and 2016 was performed. A standard procedure was used to search for suitable publications from two databases (PubMed and EMBASE). Duplicates were removed and abstracts screened by both authors for possible inclusion. Possible suitable publications were obtained and examined for the presence of pre- and post-employment information. Full articles that had this information were reviewed by standard methodology for assessment of bias.

A total of 162 individual abstracts were screened. Thirty-five full papers were reviewed and 13 papers included in the detailed review. Risk of bias was considered high due to low response rates, poor assessment of prognostic and confounding factors and varying definitions of employment. Heterogeneous data precluded meta-analysis. Eight studies focused on return to work as a primary outcome and five on quality of life with employment as a secondary outcome. Follow-up varied between 2 and 13 years. Rates of employment fell in all studies assessed. Employment rates ranged from 26 to 80% pre-transplant and 18 to 44% post-transplant. The proportion of those categorized as ill-health retired was 24% greater after orthotopic liver transplantation.

Improved survival after liver transplantation was not reflected in a return to employment and retirement was common. Areas for further study include interventions to minimize physical deconditioning, depression associated with lower employment rates and type of work available after transplant.

Many liver transplant recipients return to work, but their patterns of employment are unclear. We examine patterns of employment 5 years after liver transplantation.

First-time liver transplant recipients ages 18-60 years transplanted from 2002 to 2009 and surviving at least 5 years were identified in the United Network for Organ Sharing registry. Recipients' post-transplant employment status was classified as follows: (i) never employed; (ii) returned to work within 2 years and remained employed (continuous employment); (iii) returned to work within 2 years, but was subsequently unemployed (intermittent employment); or (iv) returned to work ≥3 years post-transplant (delayed employment).

Of 28 306 liver recipients identified during the study period, 12 998 survived at least 5 years and contributed at least 1 follow-up of employment status. A minority of patients (4654; 36%) were never employed, while 3780 (29%) were continuously employed, 3027 (23%) were intermittently employed, and 1537 (12%) had delayed employment. In multivariable logistic regression analysis, predictors of intermittent and delayed employment included lower socioeconomic status, higher local unemployment rates, and post-transplant comorbidities or complications.

Never, intermittent, and delayed employment are common after liver transplantation. Socioeconomic and labor market characteristics may add to clinical factors that limit liver transplant recipients' continuous employment.

To investigate the impact of hepatic encephalopathy before orthotopic liver transplantation (OLT) and neurological complications after OLT on employment after OLT.

One hundred and fourteen patients with chronic liver disease aged 18-60 years underwent neurological examination to identify neurological complications, neuropsychological tests comprising the PSE-Syndrome-Test yielding the psychometric hepatic encephalopathy score, the critical flicker frequency and the Repeatable Battery for the Assessment of Neuropsychological Status (RBANS), completed a questionnaire concerning their occupation and filled in the short form 36 (SF-36) to assess health-related quality of life before OLT and 12 mo after OLT, if possible. Sixty-eight (59.6%) patients were recruited before OLT, while on the waiting list for OLT at Hannover Medical School [age: 48.7 ± 10.2 years, 45 (66.2%) male], and 46 (40.4%) patients were included directly after OLT.

Before OLT 43.0% of the patients were employed. The patients not employed before OLT were more often non-academics (employed: Academic/non-academic 16 (34.0%)/31 vs not employed 10 (17.6%)/52, P = 0.04), had more frequently a history of hepatic encephalopathy (HE) (yes/no; employed 15 (30.6%)/34 vs not employed 32 (49.2%)/33, P = 0.05) and achieved worse results in psychometric tests (RBANS sum score mean ± SD employed 472.1 ± 44.5 vs not employed 443.1 ± 56.7, P = 0.04) than those employed. Ten patients (18.2%), who were not employed before OLT, resumed work afterwards. The patients employed after OLT were younger [age median (range, min-max) employed 47 (42, 18-60) vs not employed 50 (31, 29-60), P = 0.01], achieved better results in the psychometric tests (RBANS sum score mean ± SD employed 490.7 ± 48.2 vs not employed 461.0 ± 54.5, P = 0.02) and had a higher health-related quality of life (SF 36 sum score mean ± SD employed 627.0 ± 138.1 vs not employed 433.7 ± 160.8; P < 0.001) compared to patients not employed after OLT. Employment before OLT (P < 0.001), age (P < 0.01) and SF-36 sum score 12 mo after OLT (P < 0.01) but not HE before OLT or neurological complications after OLT were independent predictors of the employment status after OLT.

HE before and neurological complications after OLT have no impact on the employment status 12 mo after OLT. Instead younger age and employment before OLT predict employment one year after OLT.

Purpose There is growing research evidence that workplace factors influence disability outcomes, but these variables reflect a variety of stakeholder perspectives, measurement tools, and methodologies. The goal of this article is to summarize existing research of workplace factors in relation to disability, compare this with employer discourse in the grey literature, and recommend future research priorities. Methods The authors participated in a year-long collaboration that ultimately led to an invited 3-day conference, "Improving Research of Employer Practices to Prevent Disability, held October 14-16, 2015, in Hopkinton, Massachusetts, USA. The collaboration included a topical review of the literature, group conference calls to identify key areas and challenges, drafting of initial documents, review of industry publications, and a conference presentation that included feedback from peer researchers and a question/answer session with a special panel of knowledge experts with direct employer experience. Results Predominant factors in the scientific literature were categorized as physical or psychosocial job demands, work organization and support, and workplace beliefs and attitudes. Employees experiencing musculoskeletal disorders in large organizations were the most frequently studied population. Research varied with respect to the basic unit of assessment (e.g., worker, supervisor, policy level) and whether assessments should be based on worker perceptions, written policies, or observable practices. The grey literature suggested that employers focus primarily on defining roles and responsibilities, standardizing management tools and procedures, being prompt and proactive, and attending to the individualized needs of workers. Industry publications reflected a high reliance of employers on a strict biomedical model in contrast to the more psychosocial framework that appears to guide research designs. Conclusion Assessing workplace factors at multiple levels, within small and medium-sized organizations, and at a more granular level may help to clarify generalizable concepts of organizational support that can be translated to specific employer strategies involving personnel, tools, and practices.

Liver transplantation, although now a routine procedure, with defined indications and usually excellent outcomes, still has challenges. Donor shortage remains a key issue. Transplanted organs are not free of risk and may transmit cancer, infection, metabolic or autoimmune disease. Approaches to the donor shortage include use of organs from donors after circulatory death, from living donors and from those previously infected with Hepatitis B and C and even HIV for selected recipients. Normothermic regional and/or machine perfusion, whether static or pulsatile, normo- or hypothermic, are being explored and will be likely to have a major place in improving donation rates and outcomes. The main indications for liver replacement are alcoholic liver disease, HCV, non-alcoholic liver disease and liver cancer. Recent studies have shown that selected patients with severe alcoholic hepatitis may also benefit from liver transplant. The advent of new and highly effective treatments for HCV, whether given before or after transplant will have a major impact on outcomes. The role of transplantation for those with liver cell cancer continues to evolve as other interventions become more effective. Immunosuppression is usually required life-long and adherence remains a challenge, especially in adolescents. Immunosuppression with calcineurin inhibitors (primarily tacrolimus), antimetabolites (azathioprine or mycophenolate) and corticosteroids remains standard. Outcomes after transplantation are good but not normal in quality or quantity. Premature death may be due to increased risk of cardiovascular disease, de novo cancer, recurrent disease or late technical problems.