Excellent Health-related Quality Of Life Research Articles

Quality of Life and Exercise Capacity in Early Stage and Subclinical Hypertrophic Cardiomyopathy: A Secondary Analysis of the VANISH Trial.

The health-related quality of life (HRQOL) and cardiopulmonary exercise testing (CPET) performance of individuals with subclinical and early stage hypertrophic cardiomyopathy (HCM) have not been systematically studied. Improved understanding will inform the natural history of HCM and factors influencing well-being. VANISH trial (Valsartan for Attenuating Disease Evolution in Early Sarcomeric HCM) participants with early stage sarcomeric HCM (primary analysis cohort) and subclinical HCM (sarcomere variant without left ventricular hypertrophy comprising the exploratory cohort) who completed baseline and year 2 HRQOL assessment via the pediatric quality of life inventory and CPET were studied. Metrics correlating with baseline HRQOL and CPET performance were identified. The impact of valsartan treatment on these measures was analyzed in the early stage cohort. Two hundred participants were included: 166 with early stage HCM (mean age, 23±10 years; 40% female; 97% White; and 92% New York Heart Association class I) and 34 subclinical sarcomere variant carriers (mean age, 16±5 years; 50% female; and 100% White). Baseline HRQOL was good in both cohorts, although slightly better in subclinical HCM (composite pediatric quality of life score 84.6±10.6 versus 90.2±9.8; P=0.005). Both cohorts demonstrated mildly reduced functional status (mean percent predicted peak oxygen uptake 73±16 versus 78±12 mL/kg per minute; P=0.18). Percent predicted peak oxygen uptake and peak oxygen pulse correlated with HRQOL. Valsartan improved physical HRQOL in early stage HCM (adjusted mean change in pediatric quality of life score +4.1 versus placebo; P=0.01) but did not significantly impact CPET performance. Functional capacity can be impaired in young, healthy people with early stage HCM, despite New York Heart Association class I status and good HRQOL. Peak oxygen uptake was similarly decreased in subclinical HCM despite normal left ventricular wall thickness and excellent HRQOL. Valsartan improved physical pediatric quality of life scores but did not significantly impact CPET performance. Further studies are needed for validation and to understand how to improve patient experience. URL: https://www.clinicaltrials.gov; Unique identifier: NCT01912534.

QUALITY OF LIFE FOR NEW ZEALAND CHILDREN UP TO 13 YEARS FOLLOWING ACUTE HAEMATOGENOUS OSTEOMYELITIS

Acute Haematogenous Osteomyelitis (AHO) remains a cause of severe illness among children with the possibility of long-term consequences for growth and development. Previous research on sequelae from AHO rarely considers outcomes more than two years following treatment. This study aims to establish the quality of life of patients diagnosed with AHO in childhood up to 13 years after diagnosis, evaluating the impact on social, emotional, physical, and school function.Children treated for AHO between 2008-2018 at a tertiary referral centre in New Zealand were identified. PedsQL™ questionnaires were conducted via phone with either the child or primary caregiver and responses analysed.40 patients met inclusion criteria, were contactable by phone, and consented to participate. The mean age was 7 years (range 0-15) and most were female (60%). Health related quality of life (HRQOL) was scored as a percentage with most participants scoring >80% (n=27). Those who do experience reduced quality of life following treatment for AHO were likely to complain of pain, stiffness, or anxiety. The impact of significant childhood illness on mental health was not adequately captured by the PedsQL™ but was highlighted in qualitative feedback.We conclude that the majority of children treated for AHO reported excellent health-related quality of life up to 13 years following treatment although an negative impact on mental health was reported using qualitative analysis. A refined scoring system is needed to assess the long-term impact of musculoskeletal infection.

Quality of life in children up to 13 years following acute haematogenous osteomyelitis.

Acute haematogenous osteomyelitis (AHO) remains a cause of severe illness among children with the possibility of long-term consequences for growth and development. Previous research on sequelae from AHO rarely considers outcomes more than 2 years following treatment. This study aims to establish the quality of life of patients diagnosed with AHO in childhood up to 13 years after diagnosis, evaluating the impact on social, emotional, physical, and school function. Children treated for AHO between 2008 and 2018 at a tertiary referral centre in New Zealand were identified. Paediatric Quality of Life Inventory (PedsQL) questionnaires were conducted via phone with either the child or primary caregiver and responses analysed. Forty patients met inclusion criteria, were contactable by phone, and consented to participate. The mean age was 7 years (range 0-15) and most were female (60%). Health-related quality of life (HRQOL) was scored as a percentage with most participants scoring >80% ( n = 27). Those who do experience reduced quality of life following treatment for AHO were likely to complain of pain, stiffness, or anxiety. The impact of significant childhood illness on mental health was not adequately captured by the PedsQL but was highlighted in qualitative feedback. The majority of children treated for AHO reported excellent HRQOL up to 13 years following treatment although a negative impact on mental health was reported using qualitative analysis. A refined scoring system is needed to assess the long-term impact of musculoskeletal infection. Patient case series, Level IV.

Satisfaction and Health-Related Quality of Life Following Hip and Knee Arthroplasty Surgeries in Indian Patients: A Cross-Sectional Study.

Recently, the patient-reported outcome measures (PROMs) have been considered as the most important assessment tool for surgical outcome evaluation in arthroplasty. However, no study from the Indian subcontinent has evaluated the PROM in the total hip (THA) and knee (TKA) arthroplasties. This cross-sectional study evaluated the health-related quality of life (HRQoL) of 1244 North Indian patients following primary THA and TKA who had at least one-year follow-up. This study included 617 patients with 664 THA and 627 patients with 1152 TKA. The patients were asked to answer the EQ-5D-5L questionnaires and EQ-VAS in their own languages. The EQ-5D-5L values were used to derive level frequency scores (LFS) with validated Indian norms of EuroQoL. Ninety percent of THA and 82% of TKA patients rated excellent HRQoL using EQ-VAS. The regression analysis revealed age, gender, etiology and brand of prosthesis had a significant impact on EQ-5D-5L following THA. However, gender and simultaneous bilateral surgery were found to be important predictors of outcome in TKA. The mean value of LFS for THA was 0.95 ± 0.12 and TKA was 0.88 + 0.24 (p < 0.001). However, There was no difference in LFS between THA and TKA when only elderly patients (> 60years) were considered (p = 0.168). THA patients reported better HRQoL than TKA in the Indian subcontinent. One of the factors for a better outcome in hip arthroplasty was the relatively younger age of the patients. Patients above 60years of age reported similar levels of statisfactior in both THA and TKA. The online version contains supplementary material available at 10.1007/s43465-021-00589-x.

Quality of life in children living with PKU – a single-center, cross-sectional, observational study from Hungary

BackgroundPhenylketonuria (PKU) is an inherited error of metabolism, screened at 48–72 h of life since 1975 in Hungary. The patients have to keep a strict lifelong protein-restricted diet, resulting in PKU and its treatment can lead to social and financial burdens. The current study aimed to evaluate the health-related quality of life (HRQoL) of children living with PKU. Patients and methodsA single-centre, cross-sectional, observational study was conducted at the Center of Newborn Screening and Inherited Metabolic Disorders of Budapest, Hungary, using the PKU-quality of life (PKU-QoL) questionnaire. Responses of 59 parents and 11 teenagers were collected. Numerous aspects regarding HRQoL were analysed according to clinical compliance and severity. The patients were classified into groups with good or suboptimal adherence based on regular phenylalanine (Phe) values. The online officially translated versions of the adolescent or parental PKU-QoL questionnaire were used and analysed anonymously. Differences in HRQoL were compared - PKU vs. Hyperphenylalaninaemia (HPA) and good vs. suboptimal adherence. ResultsTwenty-five of 32 examined parameters had no or little impact on HRQoL. The most frequently reported symptom was irritability. Food enjoyment was the most impacted domain, with a major severity score in the adolescent group (median 62,5, IQR: 25–75). The emotional impact was scored at moderate severity by both the adolescents and parents. Classical PKU patients with good metabolic control were more frequently tired than HPA patients (0,0027). The group with poor metabolic adherence showed more frequent tiredness (p = 0,03), slow thinking (p = 0,018) and anxiety (p = 0,015). ConclusionOverall, our patients showed an excellent HRQoL; most domains (29/36) were reported as little/no impacted. Worse QoL was found in patients with suboptimal metabolic control. Particular attention should be paid to the emotional health of PKU patients.

Mid-term clinical and health-related quality of life outcomes for the Trifecta bioprosthesis.

The Trifecta valve has been reported to have excellent hemodynamics. Controversy exists on occurrence of patient-prosthesis mismatch (PPM) and data on mid-term outcome is sparse. Health-related quality of life (HRQoL) assessment for the Trifecta valve has not been reported before. The aim of this study was to report the mid-term clinical and HRQoL outcomes in patients undergoing Trifecta valve implantation at our institution. In this prospective, observational study, patients undergoing an aortic valve replacement (AVR) using the Trifecta valve were included. Data collection was retrospective from prospectively collected institutional database. Clinical and echocardiographic data were collected prospectively during follow-up. Quality of life was assessed using the Short Form-36 (SF-36) questionnaire. Forty-seven patients were included in the study of which 9 (19%) were women. Isolated AVR was carried out in 33 (70%) patients. In-hospital mortality and 30-day mortality were 1 (2.1%) and 2 (4.2%), respectively. With a mean indexed effective orifice area (iEOA) 0.96 ± 0.1, none of the patients had severe PPM. Moderate PPM was seen in 19%. The mean follow-up was 3 ± 1.7years. The 5-year survival estimate was 83.2% in the overall cohort, 81.4% in the isolated and 87.5% in the concomitant procedure group. Freedom from re-operation and structural valve degeneration at 5years was 95.7% and 97.8%. The mean physical health composite was 69.24 ± 2 and the mean mental health composite was 69.7 ± 25, indicating excellent mental and physical well-being among patients. The Trifecta valve provides satisfactory hemodynamics, survival and freedom from re-operation and excellent HRQoL at mid-term follow-up.

Health Related Quality of Life in Osteoarthritis Patients with Total Knee Replacement: A Longitudinal Cohort Study

Introduction: End-stage knee results in pain, loss of physical function and a decrease in Quality Of Life (QOL). Total Knee Replacement (TKR) is one of the most successful surgical interventions, providing substantial relief from pain. Because patient’s perceptions of treatment outcomes may vary from clinician’s judgment; measuring patient’s perspectives using Health Related Quality of Life (HRQOL) instruments has become an important approach for outcome evaluation. Aim: To assess the HRQOL outcome after TKR in patients with Osteoarthritis (OA) Knee. Materials and Methods: A longitudinal cohort study was conducted in Orthopaedic Department of tertiary care teaching hospital from July 2018 to June 2020. Of 63 patients eligible for the study, 56 completed the follow-up data. Knee injury and Osteoarthritis Outcome Score (KOOS) was measured preoperatively. Patients were followed-up at three months and six months, and postoperative KOOS were measured to evaluate HRQOL after TKR. Student t-test was used to analyse differences between KOOS mean aggregate and subscale scores. Results: There were 34 (60.71%) females and 22 (39.29%) males in the study. Mean age of the study population was 58.93±9.57 years. Left knee was involved in 33 (58.93%) cases and right in 23 (41.07%) cases. There was significant difference in preoperative KOOS (prKOOS) and postoperative KOOS (psKOOS) in the patients (p-value &lt;0.001). It was observed that 29 (51.79%) patients were having excellent HRQOL outcome (psKOOS ≥90), 9 (16.07%) each good (psKOOS 80-89), 8 (14.29%) fair (psKOOS 70-79) HRQOL outcome and 10 (17.86%) poor HRQOL outcome (psKOOS &lt;70) at six 6 months follow-up. Conclusion: At six months follow-up there was significant improvement in KOOS as well as subscales of symptom, pain, Activities of Daily Living (ADL), sport/recreation and QOL score. Thus, it can be said that TKR leads to significant improvement in HRQOL.

Long-Term Financial, Psychosocial, and Overall Health-Related Quality of Life After Living Liver Donation

BackgroundTo assess the impact of living liver donation (LD) in a diverse and aging population up to 20 y after donation, particularly with regard to medical, financial, psychosocial, and overall health-related quality of life (HRQOL). MethodsPatients undergoing LD between 1999 and 2009 were recruited to respond to the Short-Form 36 and a novel Donor Quality of Life Survey at two time points (2010 and 2018). ResultsSixty-eight living liver donors (LLDs) completed validated surveys, with a mean follow-up of 11.5 ± 5.1 y. Per Donor Quality of Life Survey data, physical activity or strength was not impacted by LD in most patients. All respondents returned to school or employment, and 82.4% reported that LD had no impact on school or work performance. LD did not impact health insurability in 95.6% of donors, and only one patient experienced difficulty obtaining life insurance. Overall, 97.1% of respondents did not regret LD. Short-Form 36 survey–measured outcomes were similar between LLDs and the general U.S. population. LLDs who responded in both 2010 and 2018 were followed for an overall average of 15.4 ± 2.4 y and HRQOL outcomes in these donors also remained statistically equivalent to U.S. population norms. ConclusionsThis study represents the longest postdonation follow-up and offers unique insight related to HRQOL in a highly diverse patient population. Although LLDs continue to maintain excellent HRQOL outcomes up to 20 y after donation, continued lifetime follow-up is required to accurately provide young, healthy potential donors with an accurate description of the risks that they may incur on aging.

Health-related Quality of Life After Fractures of the Lateral Third of the Clavicle in Children and Adolescents.

The health-related quality of life after pediatric fractures of the lateral third of the clavicle is unknown. Given the trend in the literature toward surgical management of clavicular fractures in adults, we frequently receive referrals for surgical management of lateral clavicular fractures in children.Therefore, we examined (1) the health-related quality of life of patients who sustained a fracture of the lateral third of the clavicle in childhood and who were treated at a large regional pediatric trauma center, (2) if the health-related quality of life was associated with the age at the time of the injury, and (3) whether fracture and displacement patterns were associated with health-related qualify of life. This is a retrospective case series. We were able to identify 69 patients (21 girls and 48 boys) who sustained a fracture of the lateral third of the clavicle between the period spanning from January 2004 to June 2015. These patients were asked to fill in a questionnaire that included the Quick version of the Disability of Arm, Shoulder, and Hand (DASH) (primary outcome) and the Pediatric Quality of Life Inventory (Peds-QL). Until July 2017, we were able to get a response from 56 of these patients, resulting in a follow-up of 81%. Treatment was conservative in all cases but one. In these cases, after a mean follow-up of 6.5 years (range, 1 to 13.2 y), the mean Quick-DASH was 1.2 (SD, 4.4) at a scale of 0 to 100, with lower values representing better quality of life. The mean physical function score of the Peds-QL was 97.8 (SD, 4.4), and the mean psychosocial function score was 91.8 (SD, 10.8), both at a scale of 0 to 100, with higher values representing better quality of life.There was no statistically significant association of age at the time of injury on the primary outcome Quick-DASH.There were no statistically significant associations between fracture or displacement patterns with regard to health-related quality of life. Conservative treatment of lateral clavicular fractures in children and adolescents is associated with excellent health-related quality of life, as measured with the disease-specific Quick-DASH and the non-disease-specific Peds-QL at a mean follow-up of 6.5 years (range, 1 to 13.2 y). There was no effect of age at the time of injury, and we were unable to identify associations to commonly analyzed risk factors, such as displacement patterns.As the conservative treatment of this type of fracture in children and adolescents is associated with such a good health-related quality of life, it is unlikely that these results could be further improved by surgical interventions. Level IV-case series.

Treatment-Related Outcomes in Paget–Schroetter Syndrome—A Cross-Sectional Investigation

To investigate treatment-related outcomes, namely radiological clot resolution, post-thrombotic syndrome (PTS), and health related quality-of-life (HRQoL) scores, in children with Paget-Schroetter syndrome (PSS) undergoing multidisciplinary management, including anticoagulation and decompressive rib-resection surgery, with or without thrombolytic therapy. We identified all patients treated for PSS at our institution between the years 2010 and 2017. Baseline clinical and radiologic data were abstracted from medical records. Two validated survey instruments to quantify PTS and HRQoL were mailed to eligible patients. Standard statistical methods were used to summarize these measures. In total, 22 eligible patients were identified; 10 were treated with thrombolysis followed by anticoagulation and rib resection, and 12 were treated with anticoagulation and rib resection alone. Nineteen patients responded to the survey instruments. Median age at deep vein thrombosis diagnosis and survey completion were 16.3 and 20.4 years, respectively. Nineteen of 22 patients had thrombus resolution on radiologic follow-up. Fourteen of 19 survey respondents reported signs/symptoms of PTS of which the majority (12/14) reported mild PTS. Aggregate total, physical, and psychosocial HRQoL scores reported were 90.6, 96.7, and 93.3, respectively. Thrombolytic therapy was not associated with a significant improvement in radiologic, clinical or HRQoL outcomes. Most patients with PSS had complete thrombus resolution on imaging. Only 11% of survey respondents reported moderate PTS. The entire cohort reported excellent HRQoL scores. The role for thrombolytic therapy in the management of childhood PSS remains incompletely elucidated.

Swedish Version of the Distress Thermometer: Validity Evidence in Patients With Colorectal Cancer.

Background: The objective of this study was to validate the NCCN Distress Thermometer (DT), including the accompanying Problem List (PL), in a Swedish population of patients diagnosed with colorectal cancer (CRC). Methods: A total of 488 patients diagnosed with CRC completed the DT/PL and EORTC core quality-of-life questionnaire (QLQ-C30) before surgery. Construct validity of the PL was analyzed using a confirmatory factor analysis. Internal consistency reliability (ICR) was tested using Cronbach's alpha coefficient. Correlations between the reported PL areas and QLQ-C30 function scales were used to explore convergent validity. Discriminant validity was examined by evaluating associations between the DT and QLQ-C30 measures of overall health-related quality of life (HRQoL). Results: Findings showed that the Swedish translation of the DT/PL is consistent with the original English version. The DT has good ICR, with the total number of reported problems significantly correlating with DT scores (r=0.67; P<.001). Analysis of convergent validity indicated that the PL areas significantly correlated with QLQ-C30 function scales, with emotional problems showing the highest correlation (r=0.76; P<.001), and item-level correlation analyses showed significant correlations between symptoms. There was also good discriminant validity between the DT and the QLQ-C30 in terms of HRQoL, including overall health status (r=-0.49; P<.001) and overall quality of life (r=-0.57; P<.001). Furthermore, there was good discriminant validity between the DT and QLQ-C30 regarding poor, moderate, and excellent HRQoL. Conclusions: These findings provide validity evidence regarding the DT, including the PL. Findings also show that the DT has good potential for screening distress-related practical, family, emotional, and physical problems during the cancer trajectory in Swedish-speaking patients. Additionally, the DT seems to be an effective screening tool to detect patients with poor, moderate, and excellent HRQoL.

Living-Donor Liver Transplant: An Analysis of Postoperative Outcome and Health-Related Quality of Life in Liver Donors.

Living-donor liver transplant represents an established alternative to deceased-donor liver transplant. The procedure is considered safe for donors; however, concerns about the donors' health-related quality of life and health status have not been fully addressed. Here, we aimed to assess the health-related quality of life and postoperative and 1-year clinical outcomes in living liver transplant donors. All patients undergoing liver resection for adult-to-adult living-donor liver transplant at our center between December 1999 and March 2013 were evaluated retrospectively. Health-related quality of life was evaluated in a second assessment through written health-related quality of life questionnaires (the Short Form 36 assessment tool) sent to all patients who underwent liver resection for living-donor liver transplant between 1989 and 2012. We identified 104 patients who underwent liver resection for living-donor liver donation between December 1999 and March 2013. Postoperative morbidity was 35.9%, with 56.8% of patients having minor complications. No postoperative, 30-day, or 90-day mortality was evident. At year 1 after transplant, 30 patients (28.8%) had (ongoing) complications, of which 80% were considered minor according to Clavien-Dindo classification. Regarding health-related quality of life, liver donors were characterized as having significantly higher scores in the general health perception component in the Short Form 36 assessment tool (P < .001). We found no significant results in other assessment components (all P > .05). Liver donors are characterized by an excellent health-related quality of life that is comparable to the general population. Because some donors tend to have concerns regarding their employment status after the procedure, a comprehensive and critical evaluation of potential donors is needed.

Health-related quality of life among Indigenous Australians diagnosed with cancer.

Health-related quality of life (HRQoL) and associated factors were assessed among 155 Indigenous Australian adult cancer patients 6months post-diagnosis. The Assessment of Quality of Life-4D Questionnaire was used to assess HRQoL. Differences in the median utility score among subgroups of interest were examined using nonparametric tests. Factors associated with excellent HRQoL were assessed through logistic regression. Participants' mean age was 52years (range 20-78), and the majority were female (60%), unemployed (72%), and recruited from outpatients clinics (64%). Breast cancer (27%) was the most common diagnosis. The median HRQoL score was 0.62; 14% of participants reported excellent HRQoL (>0.90). After adjusting for age, admission status, and treatment, excellent HRQoL was more likely among participants of Torres Strait Islander origin [adjusted odds ratio (AOR) 3.68; 95% CI 1.23-11.01], those living in regional areas (AOR 5.59; 95% CI 1.42-22.06), and those whose main language spoken at home was not English (AOR 3.60; 95% CI 1.08-11.99) and less likely among those reporting less contact with Indigenous people (AOR 0.23; 95% CI 0.68-0.81). Assessing HRQoL is important to identifying and improving the length and quality of cancer survivorship, especially in groups that have significantly poorer cancer outcomes, such as Indigenous Australians. Acknowledging the study's observational nature, we found HRQoL was lower than reported for other Australians, and we identified some socio-demographic factors that were associated with excellent HRQoL. Such assessments are an important component of identifying and evaluating appropriate interventions to improve the health and well-being of Indigenous cancer patients.

Physical activity and health-related quality of life among physiotherapists: a cross sectional survey in an Australian hospital and health service

BackgroundPhysiotherapists are a professional group with a high rate of attrition and at high risk of musculoskeletal disorders. The purpose of this investigation was to examine the physical activity levels and health-related quality of life of physiotherapists working in metropolitan clinical settings in an Australian hospital and health service. It was hypothesized that practicing physiotherapists would report excellent health-related quality of life and would already be physically active. Such a finding would add weight to a claim that general physical activity conditioning strategies may not be useful for preventing musculoskeletal disorders among active healthy physiotherapists, but rather, future investigations should focus on the development and evaluation of role specific conditioning strategies.MethodsA questionnaire was completed by 44 physiotherapists from three inpatient units and three ambulatory clinics (63.7% response rate). Physical activity levels were reported using the Active Australia Survey. Health-related quality of life was examined using the EQ-5D instrument. Physical activity and EQ-5D data were examined using conventional descriptive statistics; with domain responses for the EQ-5D presented in a frequency histogram.ResultsThe majority of physiotherapists in this sample were younger than 30 years of age (n = 25, 56.8%) consistent with the presence of a high attrition rate. Almost all respondents exceeded minimum recommended physical activity guidelines (n = 40, 90.9%). Overall the respondents engaged in more vigorous physical activity (median = 180 minutes) and walking (median = 135 minutes) than moderate exercise (median = 35 minutes) each week. Thirty-seven (84.1%) participants reported no pain or discomfort impacting their health-related quality of life, with most (n = 35,79.5%) being in full health.ConclusionsPhysical-conditioning based interventions for the prevention of musculoskeletal disorders among practicing physiotherapists may be better targeted to role or task specific conditioning rather than general physical conditioning among this physically active population. It is plausible that an inherent attrition of physiotherapists may occur among those not as active or healthy as therapists who cope with the physical demands of clinical practice. Extrapolation of findings from this study may be limited due to the sample characteristics. However, this investigation addressed the study objectives and has provided a foundation for larger scale longitudinal investigations in this field.

Favorable Outcomes in Patients Surviving 5 or More Years after Allogeneic Hematopoietic Stem Cell Transplantation for Hematologic Malignancies

Allogeneic hematopoietic stem cell transplantation (HSCT) is a curative treatment for some hematologic malignancies. As the overall number of survivors continues to increase, studies systematically examining outcomes in long-term survivors are needed. We studied the clinical and quality-of-life outcomes in HSCT recipients surviving 5 or more years from HSCT. Since 1993, 262 patients with hematologic malignancies received a T cell-depleted myeloablative HSCT from an HLA-identical sibling at a single center. Ninety-two survived beyond 5 years from HSCT (median follow-up 9.4 years, range: 5.1-15.3). Median age at transplantation was 35 years (range: 10-56). Twenty-two (24%) received a bone marrow transplant, and 70 (76%) received a peripheral blood HSCT. Of the 92 survivors, 60 completed quality-of-life measures. The main outcomes examined were chronic graft-versus-host-disease, disease relapse, survival, health-related quality-of-life (HRQL) (Functional Assessment of Cancer Therapy-General), physical and mental health (SF-36), and symptom experience (Rotterdam Symptom Checklist). Seventy-five (82%) of 92 survivors no longer required systemic immunosuppressive treatment. Four (4.3%) relapsed with leukemia at a median of 8.5 years (range: 6.2-14.0) after HSCT. Four (4.3%) died between 7.4 and 13.4 years post-HSCT (1 relapse, 1 lung cancer, 1 pneumonia, 1 brain hemorrhage). Most survivors beyond 5 years had an excellent performance status with no difference in physical and mental health and higher HRQL scores (P = .02) compared with population norms. Although physical and psychologic symptom distress was low, those with higher symptom distress experienced inferior HRQL. These results show that 5 or more years after T cell-depleted HSCT for hematologic malignancy most individuals survive disease free with an excellent performance status, preserved physical and psychological health, and excellent HRQL.

Patient-Reported Outcomes for Open versus Laparoscopic Living Donor Nephrectomy

Rates of living kidney donation have increased dramatically in recent years, in large part because of improved surgical techniques such as laparoscopic nephrectomy. To compare patient-reported outcomes of laparoscopic nephrectomy versus open donor nephrectomy in 84 adult live kidney donors. Outcomes included perceptions of pain and surgical scarring, number of surgical/medical complications, hospital length of stay, physical health problems related to donation, return to work, financial impact, health-related quality of life, and satisfaction with the donation experience. The 2 groups did not differ significantly in pain perceptions, number of surgical/medical complications, physical health problems, financial impact, health-related quality of life, or overall satisfaction. However, laparoscopic nephrectomy donors had significantly fewer hospital days and faster return to work time than open donor nephrectomy donors. The majority of donors report excellent health-related quality of life and no complications in the months following surgery. In addition, it appears that laparoscopic nephrectomy, in comparison to open donor nephrectomy, may reduce barriers to living kidney donation by reducing hospital length of stay and time away from work. Being able to return to work much sooner after surgery may significantly reduce the indirect costs (ie, lost wages) associated with living donation.

Advances in care of children with hemophilia.

Care for children with severe hemophilia has moved from pediatric hospital wards and rehabilitation services to the home, school, and community. Advances in hemophilia are due largely to the development of specialized hemophilia treatment centers, which created a system of comprehensive care and focused healthcare efforts on prevention and education. Parallel advances in coagulation resulted in identification of clotting factors VIII and IX, elucidation of the protein molecular and biochemical structures and functions, sequencing of their respective genes and transfer of the human genes for production of proteins by recombinant technology, and development of gene therapy. The tragedy of the human immunodeficiency virus and hepatitis C raised awareness in patients as well as healthcare providers of the vulnerability of blood products to viral contamination and spurred progress in science leading to viral inactivation of purified proteins. Concomitantly, physicians treating bleeding episodes in the clinic investigated pharmacokinetics and pharmacoeconomics of various strategies of clotting factor replacement. The observation that trough factor levels as low as 1 to 2% were adequate to prevent most bleeding episodes led to current prophylactic regimens that allow boys to participate fully in school and community activities while factor concentrate is infused at home on a regular schedule. Currently, children with hemophilia look forward to a normal life expectancy and excellent health-related quality of life. Physician and community partnerships through research and advocacy societies have accelerated clinical advancements as well as extension of treatment to developing countries. The future of hemophilia promises a cure with gene therapy. Given the past accomplishments in hemophilia, a long-term solution to replacement of the genetically deficient protein lies on the horizon.