A low-dose rituximab regimen for first-line treatment of acquired haemophilia A.

A low-dose rituximab regimen for first-line treatment of acquired haemophilia A. INTRODUCTION: Acquired haemophilia A (AHA) is a rare disease caused by the development of autoantibodies against FVIII. Diagnosis involves confirmation of FVIII deficiency and the presence of an inhibitor via the Bethesda assay. Severe bleeding is often managed with bypassing agents such as recombinant factor VII. This is then followed by eradication of the inhibitor with immunosuppression which typically includes a corticosteroid backbone. AIM: Review the current management and outcomes of AHA in Queensland, Australia. Determine the incidence, demographics and clinical characteristics of AHA patients. METHODS: Retrospective case series of AHA diagnosed between May 2014 and August 2018. Data were derived from the Australian Bleeding Disorders Registry and state-wide pathology database. Data collection proforma was completed by the treating haematologist and reviewed/compiled centrally. RESULTS: 24 patients were identified (incidence 1 in 1.27 million). The median age was 76.5 years. Median follow-up was 20 months. Index bleed was atraumatic and skin/soft tissue in the majority of patients. Recombinant FVIIa was the most commonly used haemostatic therapy and effective in 85% of patients. Immunosuppression and steroid usage were uniform. Upfront second agent was used in 75% of patients and was most commonly rituximab. 87.5% of patients achieved a complete remission in a median time of 48 days. Low-dose rituximab was frequently used and equally as efficacious as standard dose. CONCLUSION: Immunosuppression with combination therapy, notably rituximab, appears to be non-inferior and has a favourable side effect profile.

Psychological factors can cause false pain classification on painDETECT.

Background and aims The painDETECT questionnaire (PD-Q) has been widely used as a screening tool for the identification of neuropathic pain (NeP) as well as a tool for the characterization of patients' pain profile. In contrast to other NeP screening tools, the PD-Q is the only screening tool with weighted sensory descriptors. It is possible that responses to the PD-Q sensory descriptors are influenced by psychological factors, such as catastrophizing or anxiety, which potentially might contribute to an overall higher score of PD-Q and a false positive identification of NeP. This study aimed to explore (i) the relationship between psychological factors (catastrophizing, anxiety, depression and stress) and the total PD-Q score and (ii) if psychological factors are associated with false positive identifications of NeP on the PD-Q compared to clinically diagnosed NeP. Methods The study was a retrospective review of 1,101 patients attending an outpatient pain centre. Patients were asked to complete the PD-Q, the Pain Catastrophizing Scale (PCS), the Depression, Anxiety and Stress Scale (DASS) and the Brief Pain Inventory (BPI). For patients who were identified by PD-Q as having NeP, their medical records were reviewed to establish if they had a clinical diagnosis of NeP. Results Accounting for missing data, complete datasets of 652 patients (mean age 51 (SD14) years, range 18-88; 57% females) were available for analysis. Based on PD-Q scoring, NeP was likely present in 285 (44%) patients. Depression, anxiety, stress, catastrophizing, BPI pain and BPI interference were all significantly related to each other (p < 0.0001) and patients displaying these traits were significantly more likely to have a positive PD-Q score (p < 0.0001). For patients classified by PD-Q as having NeP, only 50% of patients had a clinical diagnosis of NeP. Anxiety was significantly associated with a false positive classification of NeP on PD-Q (p = 0.0036). Conclusions Our retrospective study showed that psychological factors including catastrophizing, depression, anxiety, and stress were all influential in producing a higher score on the PD-Q. We observed a high rate of false positive NeP classification which was associated with the presence of anxiety. Implications Clinicians and researchers should be aware that a patient's psychological state may influence the responses to PD-Q and consequently the final PD-Q score and its NeP classification.

Promoting adherence to nebulized therapy in cystic fibrosis: poster development and a qualitative exploration of adherence.

BACKGROUND: Cystic fibrosis (CF) health care professionals recognize the need to motivate people with CF to adhere to nebulizer treatments, yet little is known about how best to achieve this. We aimed to produce motivational posters to support nebulizer adherence by using social marketing involving people with CF in the development of those posters. METHODS: The Sheffield CF multidisciplinary team produced preliminary ideas that were elaborated upon with semi-structured interviews among people with CF to explore barriers and facilitators to the use of nebulized therapy. Initial themes and poster designs were refined using an online focus group to finalize the poster designs. RESULTS: People with CF preferred aspirational posters describing what could be achieved through adherence in contrast to posters that highlighted the adverse consequences of nonadherence. A total of 14 posters were produced through this process. CONCLUSION: People with CF can be engaged to develop promotional material to support adherence, providing a unique perspective differing from that of the CF multidisciplinary team. Further research is needed to evaluate the effectiveness of these posters to support nebulizer adherence.

Essential pain management: an educational program for health care workers.

BACKGROUND: Education for health care workers on pain-related topics is not always readily available, and this is especially so in low and middle income countries (LMICs). The Essential Pain Management program (EPM) has been developed to offer a simple interactive educational opportunity for health care workers in LMICs. METHODS: Following a needs analysis in Papua New Guinea, an 8 h educational program with the aims of improving pain knowledge and providing a simple pain management framework was developed. An evaluation of the program using the Kirkpatrick model is being used. The program has a "teach the teachers" component to encourage sustainability. RESULTS: The program has been run in 30 countries, delivered to 1,600 participants, and 340 instructors have been trained. Feedback has been positive, pre post testing in 27 sites showed a mean pre score of 65.89% rising to 75.23% (n = 581 respondents). A subanalysis demonstrates doctors and nurses improving by similar degrees. When local instructors have delivered the program after attending the trainer's session the participant test results were comparable to the results seen when the overseas instructors taught the course. DISCUSSION: The widespread adoption of the EPM program suggests there is a need for pain education in LMICs. The teach the teachers component of the program and the comparable results from their teaching should contribute to sustainability. Further support and mentoring using electronic systems such as Facebook, text messaging, and a website may also contribute to sustainability.

Perceptions of a clinical psychology support group for spinal injury.

BACKGROUND: A service evaluation was performed exploring nurses' perceptions of a clinical psychology facilitated peer support group in a spinal injury rehabilitation setting. AIMS: To determine whether staff found the meetings useful while, more broadly, to highlight the need to support and supervise nursing staff in psychological care appropriately. METHOD: A questionnaire was developed and distributed to the 30 members of staff who worked on the ward. Seventeen questionnaires were returned (57%). Data was analysed using thematic analysis. RESULTS: The meetings were viewed as a place to discuss issues, and a safe protected space to share stresses. Staff felt the meetings aided team cohesion and helped them share ideas and draw up clinical strategies. Meetings aided stress management and confidence building. Staff considered the meetings to increase their psychological awareness and understanding. CONCLUSION: Staff involved in the acute care and rehabilitation of spinal injured patients are consistently exposed to highly demanding and stressful clinical environments. Support meetings where staff can discuss patient and ward issues are invaluable. Other clinical nursing areas would benefit from similar support systems.

Needle anxiety in the clinic: a needs assessment.

BACKGROUND: A needs assessment for needle anxiety was carried out across people with either spinal cord injury (SCI) or spina bifida (SB). AIMS: to identify the numbers of people reporting anxiety when having blood taken and being given injections; to explore individual beliefs; and to identify appropriate action. METHOD: A self-report questionnaire was developed for clinical use, piloted and administered across a 6-month period. Two-hundred and thirty-one questionnaires were returned (188 SCI group and 43 SB group). Data were analysed using SPSS. RESULTS: There was a significant report of anxiety for both areas in both groups: 10-12% for SCI and 16-19% for SB. Eleven per cent of the SCI group and 19% of the SB group believed it was a problem for them; however, only 4% of each group identified they would want help. CONCLUSION: There is a clinical responsibility to address the level of need identified by this project. Six proposals are made to highlight awareness of this issue and provide guidance on strategies and interventions for patients and staff.

The Everyday Memory Questionnaire-revised: development of a 13-item scale.

PURPOSE: The Everyday Memory Questionnaire (EMQ) was developed as a subjective measure of memory failure in everyday life. Previous studies have investigated the factor structure of the EMQ in both healthy participants and people with multiple sclerosis (MS). The aim of the present study was to confirm the factor structure of the EMQ, to determine the internal consistency and criterion validity of the scale and to develop a shortened version. METHOD: A retrospective design, including participants from a study on MS patients and their carers and a study on stroke patients. Psychometric properties of the EMQ-28 were explored, and the measure was further revised from comparative analyses between the clinical and non-clinical groups. RESULTS: Reliability and factor analysis of the EMQ-28 identified two main factors, general memory and attentional function, showing some concordance with previous research. Further analysis reduced the questionnaire to a 13-item measure (EMQ-R), with two main factors (Retrieval and Attentional tracking), strong internal reliability, and good discriminatory properties between clinical and control groups. CONCLUSIONS: The 28-item questionnaire consistently differentiated between two broad systems of memory and attention, with some differentiation of visual and verbal, or language systems. Results showed some consistency with previous findings. The revised, 13-item questionnaire is a valid and reliable tool that has good face validity for use with neurological patients. Further exploration of the revised EMQ is recommended to provide information regarding its psychometric and clinical properties.

Consumer involvement in the health technology assessment program.

OBJECTIVES: This study aims to describe a cycle of development leading to sustainable methods for involving consumers in the management of a program commissioning health technology assessment. METHODS: Staff time was dedicated to developing procedures for recruiting and briefing consumers to participate in prioritizing, commissioning, and reporting research. Resources and support were developed in light of early feedback from consumers and those working with them. These were piloted and amended before being used routinely. RESULTS: Over 4 years, procedures and resources have been developed to support six consumers attending seven to eight prioritization meetings a year; thirty to forty-five consumers each year commenting on research need for particular topics; thirty consumers a year commenting on research proposals, and twenty a year commenting on research reports. The procedures include clear job descriptions, induction and development days, clear briefing materials, payment for substantial tasks, and regularly seeking feedback to improve procedures. CONCLUSIONS: Explicit, inclusive, and reproducible methods for supporting consumer involvement that satisfy National Health Service policy recommendations for involving consumers in research require dedicated staff time to support a cycle of organizational development.