Bone density and health‐related quality of life in adult patients with severe haemophilia

Summary. Severe haemophilia and reduced bone density can negatively influence perception of patient’s health‐related quality of life (HRQoL), especially considering future aspects, the risk of losing independence or pain suffering. The aim of this study was to assess levels of HRQoL in severe haemophilia patients and to compare HRQoL to those of the general population as well as to determine whether reduced bone density is correlated to the perceived HRQoL. Patients were divided into two groups based on timing of being treated with prophylaxis: Group A (started prophylaxis at age of ≤3 years; n = 22); Group B (at age of >3 years; n = 15). The bone mineral density (BMD g cm^?2) of different measured sites was measured by dual energy X‐ray absorptiometry (DXA). HRQoL was assessed using SF‐36 questionnaire. Group A have mean BMD T‐score >?1.0 (i.e. normal score) at all measured sites, and have almost similar scores in the SF‐36 domains compared with the reference population. Group B have mean BMD T‐score ?1.0 at lumbar spine and total body, and their scores in the SF‐36 domains were lower compared with the reference population. Moreover, significant correlations were found between BMD at femoral neck and total body with physical domains. With adequate long‐term prophylaxis since early childhood, adult patients with haemophilia report a comparable BMD and HRQoL to the Swedish reference population. Reduced BMD in group B correlated with impaired physical health, which underscores the importance of early onset of adequate prophylactic treatment.     

The association between depression and health‐related quality of life in people with type 2 diabetes: a systematic literature review

The relationships between co‐morbid depression in people with diabetes and adverse outcomes including poor HbA_1c control, adherence to medication and mortality have been examined and confirmed. However, as the awareness of the decrement to health‐related quality of life (HRQOL) in people with diabetes and its clinical consequences grows, investigators have become increasingly interested in measuring HRQOL in clinical trials. Given that the psychological factors such as depression may contribute to diminished HRQOL, the present review sought to summarize the association between these variables in people with type 2 diabetes. Articles for a systematic review were obtained via a search performed using MEDLINE, EMBASE and PsycINFO (1980–2007). Fourteen articles fulfilled the inclusion criteria. Studies indicated that self‐reported depressive symptoms markedly impaired HRQOL on several domains. However, depression was not related to all sub‐domains of HRQOL in all studies, suggesting that the effects of depression on certain aspects of HRQOL may vary between clinical and demographic subgroups. Although a number of shortcomings identified in the current literature should be taken into account for future research, the importance of this review lies in the possibility it raises that the improvements in HRQOL and clinical practice may potentially be achieved by placing greater attention on the identification and management of depression. Copyright © 2010 John Wiley & Sons, Ltd.     

A longitudinal evaluation of health‐related quality of life in patients with AL amyloidosis: associations with health outcomes over time

Light chain (AL) amyloidosis is a rare disease associated with significant, irreversible organ dysfunction and high case fatality. An observational study was conducted to assess health‐related quality of life (HRQoL) in patients treated for AL amyloidosis between 1994 and 2014 with both high dose melphalan and stem cell transplantation (HDM/SCT) or non‐SCT chemotherapy regimens. The SF‐36v1^® Health Survey (SF‐36) was administered to assess HRQoL during clinic visits. Analysis of variance was used to compare pre‐ and post‐treatment HRQoL within each treatment group to an age‐ and gender‐adjusted general population (GP) normative sample. Cox proportional hazard models were fit to examine associations between pre‐treatment levels of HRQoL and mortality within 1 and 5 years after initiating specific treatment regimens (HDM/SCT: n = 402; non‐SCT chemotherapy regimens: n = 172). Among patients who received HDM/SCT, there were significant improvements following treatment in vitality, social functioning, role‐emotional and mental health. Worse pre‐treatment SF‐36 physical component scores were associated with a greater risk of mortality in both treatment groups and follow‐up periods (P ≤ 0·005 for both). [Correction added on 20 October 2017, after first online publication: This P value has been corrected]. Using HRQoL assessments in every physician visit or treatment may provide valuable insights for treating rare conditions like AL amyloidosis.     

Assessment of factors influencing health‐related quality of life in HIV‐infected patients

Objectives

Health‐related quality of life (HRQL) is used in the assessment of chronic illness. Regarding HIV infection, HRQL assessment is an objective for physicians and institutions since antiretroviral treatment delays HIV clinical progression. The aim of this study was to determine the factors with the most influence on HRQL in HIV‐infected people and to create a predictive model.

Methods

We conducted a cross‐sectional study in 150 patients in a tertiary hospital. HRQL data were collected using the Medical Outcomes Study HIV Health Survey (MOS‐HIV) questionnaire. The research team created a specific template with which to gather clinical and sociodemographic data. Adherence was assessed using the Simplified Medication Adherence Questionnaire (SMAQ) and depression data were obtained using the Beck Depression Inventory, Second Edition (BDI‐II) inventory. Logistic regression models were used to identify determinants of HRQL.

Results

HIV‐related symptoms and presence of depression were found to be negatively associated with all the MOS‐HIV domains, the Physical Health summary score and the Mental Health summary score. Patients receiving protease inhibitor (PI)‐based treatment had lower scores in four of the 11 domains of the MOS‐HIV questionnaire. Gender, hospitalization in the year before enrolment, depression and parenthood were independently related to the Physical Health Score; depression and hepatitis C virus coinfection were related to the Mental Health Score.

Conclusions

Optimization of HRQL is particularly important now that HIV infection can be considered a chronic disease with the prospect of long‐term survival. Quality of life should be monitored in follow‐up of HIV‐infected patients. The assessment of HRQL in this population can help us to detect problems that may influence the progression of the disease. This investigation highlights the importance of a multidisciplinary approach to HIV infection.     

Relationship between health‐related quality of life and blood pressure control in patients with uncontrolled hypertension

We sought to investigate the psychosocial characteristics of patients with uncontrolled hypertension and examine factors that influence blood pressure (BP) control. A total of 1011 patients with uncontrolled hypertension were enrolled in 13 tertiary hospitals. Uncontrolled hypertension was defined as systolic BP ≥140 mm Hg or diastolic BP ≥90 mm Hg despite on antihypertensive therapy. Socio‐demographics, anthropometrics, behavioral risk factors, medication pattern, adherence, and measures of health‐related quality of life (HRQoL; EuroQol 5D visual analog scale [EQ‐5D VAS]) were assessed at baseline and during follow‐up visits (3 and 6 months). Patients were divided into 2 groups based on BP control status at 6 months (controlled group [n = 532] vs uncontrolled group [n = 367]). There were no differences in clinical characteristics except the proportion of smokers and baseline BP between patients with controlled BP and uncontrolled BP. At 6 months, the adherence of antihypertensive medication did not differ between the groups but the proportion of combination therapy with ≥3 antihypertensives was significantly higher in patients with uncontrolled BP. EQ‐5D VAS at follow‐up was significantly lower in patients with uncontrolled BP despite similar baseline values. Multivariate logistic regression analysis revealed that EQ‐5D VAS at follow‐up significantly correlated with BP control. Patients with worse HRQoL had higher Charlson Comorbidity Index and higher proportion of taking ≥3 antihypertensives, but medication adherence was similar to those with better HRQoL. These findings suggest that along with pharmacologic intervention of hypertension, management of comorbid conditions or psychological support might be helpful for optimizing BP control in patients with uncontrolled hypertension.     

Real‐world comparative analysis of bleeding complications and health‐related quality of life in patients with haemophilia A and haemophilia B

Introduction

Clinical severity and impact of haemophilia on quality of life have been generally considered to be lower for haemophilia B (HB) compared with haemophilia A (HA) patients.

Aims

To compare annual bleeding rate (ABR), target joint development and health‐related quality of life (HRQoL) between adult (≥18 years) severe HA and HB patients using recent data from the Cost of Haemophilia in Europe: a Socioeconomic Survey (CHESS) study.

Methods

Multivariate generalized linear models (GLM) were constructed to assess the relationship between haemophilia type, ABR, HRQoL (derived from EQ‐5D index scores) and the presence of target joints while controlling for covariates.

Results

Of the 1225 patients included, 77% (n = 949) had HA and 23% (n = 278) had HB. Of the 514 patients who completed the EQ‐5D, 78% (n = 405) had HA, and 22% (n = 110) had HB. Unadjusted mean ABR was 3.79 in HA and 4.60 in HB. The presence of ≥1 target joint was reported in 59% and 54% of patients with HA and HB, respectively. Unadjusted mean EQ‐5D index score was 0.78 in HA and 0.76 in HB. Haemophilia type was not a significant predictor of ABR, target joints or HRQoL when adjusted for confounding factors such as BMI, age and replacement therapy regimen.

Conclusion

Data suggest comparable ABR, incidence of target joints and HRQoL between patients with HB and HA indicating comparable clinical severity and disease impact on patient quality of life.     

Effect of long‐term late‐evening snack on health‐related quality of life in cirrhotic patients

Aim: In patients with liver cirrhosis, abnormal energy metabolism induces low health‐related quality of life (HRQOL) scores. However, late‐evening snack (LES) prevents morning starvation in cirrhotic patients. Our aim is to assess the effect of long‐term LES on HRQOL in cirrhotic patients, using the 36‐item Short Form (SF‐36) health survey. Methods: Thirty‐nine cirrhotic patients classified as Child–Pugh grade A were recruited. The patients were randomly divided into two groups: 24 were assigned to the non‐LES group and 15 to the LES group. SF‐36 scores, anthropometric data and serum biochemical parameters were examined in the non‐LES and LES groups at 0, 6 and 12 months. Results: Neither anthropometric data nor laboratory data showed significant differences between the non‐LES and the LES groups at 0, 6 and 12 months. The role–emotional (RE) HRQOL scores at 6 months and mental health (MH) scores at 6 and 12 months were significantly reduced from the baseline level in the non‐LES group. In contrast, these scores remained unchanged in the LES group. General health perception (GH) scores at 12 months, RE at 6 months and MH at 6 and 12 months in the LES group were significantly higher than those of the non‐LES group. Conclusion: Long‐term LES administration may be helpful in maintaining higher HRQOL in patients with cirrhosis.