The personal impact of living with a myeloproliferative neoplasm.

OBJECTIVE: The aim of this study is to gain insight into the physical, psychological and social impact of having a myeloproliferative neoplasm (MPN), a rare type of cancer with an often chronic course. METHODS: An online survey was conducted among 455 Dutch MPN patients (62.7% female, age M 63) to explore the impact of the disease by measuring the MPN symptom burden (MPN-SAF TSS) and quality of life (QoL) (EORTC QLQ-C30) and its subscales within a hierarchical QoL model. We examined differences in MPN symptom burden and QoL in relation to sociodemographic and disease-related factors. Hierarchical regression analysis was used to explain variances in QoL. RESULTS: Most patients (97%) experienced MPN-related health complaints, with a significantly higher MPN symptom burden in women (M 31.50) compared to men (M 24.10). Regarding to fatigue and cognitive functioning MPN patients suffered more compared to a reference group of other cancers. MPN subtype or type of treatment did not show significant differences in MPN symptom burden or QoL. However, experiencing side effects, complications or comorbidities significantly negatively affected MPN symptom burden and QoL. 48.8% of patients reported that MPN affected their ability to work. The explained variance in overall QoL was 58%, most importantly by disease progression, comorbidities, MPN symptom burden and role, emotional and social functioning. CONCLUSION: This study revealed that having an MPN has a negative impact on several domains of QoL. Symptom assessment and support should be included in the healthcare management of MPN patients.

The Use of the Voice Trainer App for Vocal Control in People with a Degenerative Ataxia: A Pilot Intervention Study.

Dysarthria is disabling in persons with degenerative ataxia. There is limited evidence for speech therapy interventions. In this pilot study, we used the Voice trainer app, which was originally developed for patients with Parkinson's disease, as a feedback tool for vocal control. We hypothesized that patients with ataxic dysarthria would benefit from the Voice trainer app to better control their loudness and pitch, resulting in a lower speaking rate and better intelligibility. This intervention study consisted of five therapy sessions of 30 min within 3 weeks using the principles of the Pitch Limiting Voice Treatment. Patients received real-time visual feedback on loudness and pitch during the exercises. Besides, they were encouraged to practice at home or to use the Voice trainer in daily life. We used observer-rated and patient-rated outcome measures. The primary outcome measure was intelligibility, as measured by the Dutch sentence intelligibility test. Twenty-one out of 25 included patients with degenerative ataxia completed the therapy. We found no statistically significant improvements in intelligibility (p = .56). However, after the intervention, patients were speaking slower (p = .03) and the pause durations were longer (p < .001). The patients were satisfied about using the app. At the group level, we found no evidence for an effect of the Voice trainer app on intelligibility in degenerative ataxia. Because of the heterogeneity of ataxic dysarthria, a more tailor-made rather than generic intervention seems warranted.

Detection of gluten in duplicate portions to determine gluten intake of coeliac disease patients on a gluten-free diet.

This study determined the gluten content of foods and meals consumed by coeliac disease (CD) patients who adhere to a gluten-free diet, and to estimate the total daily intake of gluten of these patients. CD patients fulfilling defined inclusion criteria were preselected and approached for participation in the study. Duplicate portions (DP) of foods and mixed dishes were collected from the CD patients for evaluating complete daily food intake during two individual days. Also, for these days, written food records were completed by the participants. From each DP, a laboratory sample was prepared and analysed for its gluten concentration and total daily gluten intake was calculated. Each individual's total daily intakes of energy and macronutrients were calculated using the Dutch food composition database. In total, twenty-seven CD patients participated, seven males and twenty females, aged between 21 and 64 years. In thirty-two (6 %) of 499 food samples collected in total, more than 3 mg/kg gluten was present. In four of these thirty-two samples, the gluten concentration was above the European legal limit of 20 mg/kg and three of the four samples had a gluten-free label. The maximal gluten intake was 3·3 mg gluten/d. The gluten tolerance for sensitive CD patients (>0·75 mg/d) was exceeded on at least six out of fifty-four study days. To also protect these sensitive CD patients, legal thresholds should be re-evaluated and the detection limit of analytical methods for gluten analysis lowered.

Multicentre study of multidisciplinary team assessment of pancreatic cancer resectability and treatment allocation.

BACKGROUND: Multidisciplinary team (MDT) meetings have been adopted widely to ensure optimal treatment for patients with cancer. Agreements in tumour staging, resectability assessments and treatment allocation between different MDTs were assessed. METHODS: Of all patients referred to one hospital, 19 patients considered to have non-metastatic pancreatic cancer for evaluation were selected randomly for a multicentre study of MDT decisions in seven units across Northern Europe. Anonymized clinical information and radiological images were disseminated to the MDTs. All patients were reviewed by the MDTs for radiological T, N and M category, resectability assessment and treatment allocation. Each MDT was blinded to the decisions of other teams. Agreements were expressed as raw percentages and Krippendorff's α values, both with 95 per cent confidence intervals. RESULTS: A total of 132 evaluations in 19 patients were carried out by the seven MDTs (1 evaluation was excluded owing to technical problems). The level of agreement for T, N and M categories ranged from moderate to near perfect (46·8, 61·1 and 82·8 per cent respectively), but there was substantial variation in assessment of resectability; seven patients were considered to be resectable by one MDT but unresectable by another. The MDTs all agreed on either a curative or palliative strategy in less than half of the patients (9 of 19). Only fair agreement in treatment allocation was observed (Krippendorff's α 0·31, 95 per cent c.i. 0·16 to 0·45). There was a high level of agreement in treatment allocation where resectability assessments were concordant. CONCLUSION: Considerable disparities in MDT evaluations of patients with pancreatic cancer exist, including substantial variation in resectability assessments.

Quantitative risk assessment of the aggregate dermal exposure to the sensitizing fragrance geraniol in personal care products and household cleaning agents.

A quantitative risk assessment was performed to establish if consumers are at risk for being dermally sensitized by the fragrance geraniol. Aggregate dermal exposure to geraniol was estimated using the Probabilistic Aggregate Consumer Exposure Model, containing data on the use of personal care products and household cleaning agents. Consumer exposure to geraniol via personal care products appeared to be higher than via household cleaning agents. The hands were the body parts receiving the highest exposure to geraniol. Dermal sensitization studies were assessed to derive the point of departure needed for the estimation of the Acceptable Exposure Level (AEL). Two concentrations were derived, one based on human studies and the other from dose-response analysis of the available murine local lymph node assay data. The aggregate dermal exposure assessment resulted in body part specific median exposures up to 0.041 µg/cm(2) (highest exposure 102 µg/cm(2)) for hands. Comparing the exposure to the lowest AEL (55 µg/cm(2)), shows that a range of 0.02-0.86% of the population may have an aggregated exposure which exceeds the AEL. Furthermore, it is demonstrated that personal care products contribute more to the consumer's geraniol exposure compared to household cleaning agents.

Patients' intentions to inform relatives about Type 2 diabetes risk: the role of worry in the process of family risk disclosure.

AIMS: Patients with Type 2 diabetes may play a role as intermediary between medical professionals and at-risk relatives to promote diabetes prevention in their family. This study aimed to further our understanding of factors that influence the decisional process of familial risk disclosure in patients with diabetes. METHODS: In a cross-sectional study, patients with Type 2 diabetes (n = 546) filled in a questionnaire assessing family risk perception, worry, personal beliefs regarding diabetes prevention, diabetes-related family communication, intention and perceived ability to inform relatives about familial risk of diabetes. Data were analysed using hierarchical logistic regression and multiple mediation analyses. RESULTS: Sixty per cent of the patients were willing to inform their relatives about familial diabetes risk; 61% reported high family risk perception and 41% had positive control beliefs with regard to preventive options in relatives. A majority (69%) did not express serious concern about relatives developing diabetes. Worry about relatives, knowing what to tell, whom to notify, and communication about diabetes in general appeared to facilitate family risk disclosure. Unexpectedly, high family risk perception in itself did not significantly increase patients' intentions to inform relatives; rather, risk perception appeared to exert an indirect effect through worry and beliefs about diabetes prevention. CONCLUSIONS: Worry in patients with diabetes appears to be a key factor in the process of family risk disclosure. When professionals guide their patients in this process, they should not only provide risk information, but also address worries and emphasize opportunities for diabetes prevention.

Effects of tirapazamine on experimental colorectal liver metastases after radiofrequency ablation.

BACKGROUND: Radiofrequency ablation (RFA) is a common procedure for the management of colorectal liver metastases. RFA-generated lesions are surrounded by a rim of hypoxia that is associated with aggressive outgrowth of intrahepatic micrometastases. Hypoxia-activated prodrugs such as tirapazamine are designed selectively to induce apoptosis in tumour cells under hypoxic conditions. Therefore, it was hypothesized that tirapazamine may have therapeutic value in limiting hypoxia-associated tumour outgrowth following RFA. METHODS: Murine C26 and MC38 colorectal cancer cells were grown under hypoxia and normal oxygenation in vitro, and treated with different concentrations of tirapazamine. Apoptosis and cell cycle distribution were assessed by western blot and fluorescence-activated cell sorting analysis. Proliferative capacity was tested by means of colony-formation assays. Mice harbouring microscopic colorectal liver metastases were treated with RFA, followed by a single injection of tirapazamine (60 mg/kg) or saline. Tumour load was assessed morphometrically 7 days later. RESULTS: Tirapazamine induced apoptosis of colorectal tumour cells under hypoxia in vitro. Under normal oxygenation, tirapazamine caused a G2 cell cycle arrest from which cells recovered partly. This reduced, but did not abolish, colony-forming capacity. A single dose of tirapazamine largely prevented accelerated outgrowth of hypoxic micrometastases following RFA. Tirapazamine administration was associated with minimal toxicity. CONCLUSION: Tirapazamine induced apoptosis in colorectal cancer cells in a hypoxia-dependent manner and potently suppressed hypoxia-associated outgrowth of liver metastases with limited toxicity. This warrants further study to assess the potential value of tirapazamine, or other hypoxia-activated prodrugs, as adjuvant therapeutics following RFA treatment of colorectal liver metastases.

Prolonged portal triad clamping during liver surgery for colorectal liver metastases is associated with decreased time to hepatic tumour recurrence.

AIMS: The aim of this study was to evaluate the oncological outcome of portal triad clamping during hepatectomy in colorectal cancer patients. METHODS: 160 patients with colorectal liver metastases underwent a partial hepatectomy with curative intent. Data were collected in a prospective database and were retrospectively analyzed for time to liver recurrence (TTLiR) and time to overall recurrence (TTR). The prognostic significance of portal triad clamping of any type and severe ischemia due to prolonged portal triad clamping was determined by Cox regression models. RESULTS: TTLiR was reduced after clamping of any type, although not statistically significant (p=0.061). Severe ischemia due to prolonged portal triad clamping significantly decreased TTLiR (p=0.022), but not TTR. Furthermore, severe ischemia independently predicted TTLiR in a multivariable analysis (p=0.038). CONCLUSIONS: Severe ischemia due to prolonged portal triad clamping during hepatic resection for colorectal liver metastases appears to be associated with decreased TTLiR. Further research remains necessary to determine the causative effect of prolonged vascular clamping on liver tumour recurrence.

The impact of an organ donation registration information program for high school students in the Netherlands.

In 1998, a new organ donor registration system was implemented in the Netherlands to increase the number of potential donors. A high school education program was developed to prepare adolescents to make an informed decision about organ donation. A post-test only randomised controlled trial was conducted in 39 high schools including 2868 students. Students within schools were randomly allocated to either attend the organ donation education program or not. The impact of the program on students' intention to register their organ donation preference (and determinants thereof) were analysed using multivariate multileveling modelling (MlwiN). The results show that students who were exposed to the education program had more favourable registration intentions (B = .40), were more often willing to be donors (OR = 1.45), and had greater knowledge about (B = 3.84) and more positive social outcome expectations (B = .09) and self-efficacy regarding organ donation registration (B=.22). Lastly, they experienced significantly less negative outcome expectations related to organ donation registration (B = -.15). Students' evaluation of the school-based education program was favourable. The present organ donation registration program proved to be effective in changing determinants of organ donation registration, and a large-scale implementation in the Dutch high school curriculum is planned.

Determinants of surgery related anxiety in cataract patients.

BACKGROUND/AIMS: Not much is known about the relative importance of different determinants of anxiety in cataract patients. This study analysed the predictive value of factors related to surgery induced anxiety. METHODS: In 128 cataract patients, recruited from two hospitals (Medical Centre Maastricht Annadal (MCMA) and Rotterdam Eye Hospital (REH)), state anxiety was assessed at four different time points using the State-Trait Anxiety Inventory (STAI). The following predictive factors of anxiety were measured: trait anxiety, outcome expectancies, doctor-patient relationship, coping strategy, social support, information supply, sociodemographic variables, and previous cataract surgery. Repeated measures ANOVA, t tests, multiple regression analysis, and correlations were used to analyse data. RESULTS: In general patients reported little anxiety. The level of anxiety (scale 1-4) was the highest before surgery, decreased immediately after surgery, and increased again after the postoperative visit. Patients with higher trait anxiety levels (r = 0.41; p<0.01), and women (r = 0.30; p<0.01) reported more anxiety. The REH patients showed lower anxiety scores than the MCMA patients. CONCLUSION: Women and patients with higher trait anxiety were more likely to experience higher levels of state anxiety. Positive outcome expectancies and social support may decrease anxiety.

Cost effectiveness of foldable multifocal intraocular lenses compared to foldable monofocal intraocular lenses for cataract surgery.

AIM: To analyse the cost effectiveness of foldable monofocal intraocular lenses (IOLs) compared to foldable multifocal IOLs in cataract surgery alongside a prospective, multicentre randomised clinical trial (RCT). METHODS: Patients underwent cataract surgery with bilateral monofocal (n = 97) or multifocal (n = 93) IOL implantation. Cost data and patient preferences, using the visual analogue scale (VAS), the time trade-off (TTO), and the standard gamble (SG) technique were obtained preoperatively and postoperatively by structured interviews. The incremental costs (multifocal minus monofocal), mean costs per patient, and differences in preferences were computed. RESULTS: Mean costs for glasses per patient in the monofocal group were 41.67 and in the multifocal group 149.58. The difference in costs between the multifocal and monofocal group was -92.09 and was statistically significant (p = 0.008). No significant differences were found in total costs or in effectiveness between the monofocal and multifocal IOL group. CONCLUSION: The cost effectiveness of multifocal IOLs is reduced to a cost minimisation analysis, because of the inability to demonstrate significant differences in effects. The use of multifocal IOLs in cataract surgery resulted in a significant reduction in costs for patient's postoperative spectacles.

Quality of care from the perspective of the cataract patient: the reliability and validity of the QUOTE-cataract.

BACKGROUND/AIMS: To assess reliability and validity of the QUOTE-cataract, a questionnaire that measures the quality of care from the perspective of cataract patients. METHODS: The QUOTE-cataract was tested in a multicentre study among 540 cataract patients in three different hospitals. Reliability was represented by internal consistency (Cronbach's alpha), and repeatability (intraclass correlation coefficient (ICC)). Validity was evaluated qualitatively and by factor analyses. RESULTS: A strong internal consistency coefficient (0.89), and high repeatability (ICC = 0.76) demonstrated good reliability. Content validity was assured by involvement of patients in the development of the questionnaire. Factor analysis confirmed an underlying taxonomy of generic and disease specific items. CONCLUSION: The QUOTE-cataract has good reliability and provides a valid assessment of quality of care in cataract surgery.

Determinants of patient satisfaction after cataract surgery in 3 settings.

PURPOSE: To analyze the determinants of satisfaction and postoperative visual function after cataract surgery in 3 settings in The Netherlands. SETTING: University Hospital Maastricht (outpatient care), Atrium Medical Center Heerlen (inpatient care), and Medical Center Maastricht Annadal (outpatient care), Maastricht, The Netherlands. METHODS: This cross-sectional study consisted of 150 patients of 50 years and older who had first-eye phacoemulsification with intraocular lens implantation. Data were collected by a written questionnaire. The following parameters were measured: medical outcome, postoperative function, patient satisfaction with medical outcome and hospital care, and overall patient satisfaction. RESULTS: In general, patients were very satisfied (mean score 8.43 on a 10-point scale ranging from 1 = very bad to 10 = excellent). The 3 centers did not differ regarding the patient satisfaction (P =.092). However, postoperative visual function (P =.012), counseling (P =.010), and waiting time (P <.001) were different among the settings. Patient satisfaction with hospital care had a stronger correlation with overall satisfaction than patient satisfaction with the medical outcome (r = 0.669 versus r = 0.543, respectively). CONCLUSIONS: A causal model of patient satisfaction was tested, indicating that satisfaction was related to the patient's preoperative expectations and the quality of care given during the hospital stay and follow-up at the outpatient clinic. This emphasizes the relevance of patient education (to set realistic expectations) and counseling (need for care) by hospital staff in a cataract surgery setting.