Psychometric analysis of the adult sickle cell quality of life measurement information system (ACSQ-Me) in a UK population.

BACKGROUND: The Adult Sickle Cell Quality of Life Measurement Information System (ASCQ-Me) has been shown to be a reliable and valid questionnaire measuring health-related quality of life (HRQoL) in the US sickle cell disease (SCD) population. The study objective was to test the validity and reliability of the ASCQ-Me for use in the UK. METHODS: The US ASCQ-Me, Hospital Anxiety and Depression Scale (HADS), self-reported symptoms, and Medical Outcome Survey Short Form 36 (SF-36) were administered to 173 patients with SCD. Clinical severity was assessed by the number of painful episodes indicated by hospital admissions. RESULTS: The results showed that the item banks of the UK ASCQ-Me had good internal consistency. Anxiety and depression were strongly correlated with the emotional, and social item banks of the UK ASCQ-Me, with moderate correlations between the UK ASCQ-Me item banks and SF-36 components suggesting convergent validity. A confirmatory factor analysis confirmed the conceptual framework of the scale as being the same as the US ASCQ-Me, indicating construct validity. Known groups validity was found, with the ASCQ-Me being able to differentiate by SCD severity groups. CONCLUSION: The analysis of the sample shows evidence of both validity and reliability of the ASCQ-Me for use in the UK SCD population.

Targeting sickle cell disease.

Sickle cell disease is one of the most common global genetic disorders. It is a debilitating condition and children with sickle cell disease are more likely to have a stroke, and experience lower quality of life and shorter life expectancy.

Transcranial Doppler Screening in a Regional Care Network for Sickle Cell Disease in the United Kingdom.

The risk of stroke in children screened with transcranial Doppler ultrasound in the United Kingdom is not known. We evaluated a clinician-led program using a risk assessment modified from the STOP protocol. High-risk classification included abnormal velocities in the anterior cerebral artery, and single abnormal scan if initial velocity >220 cm/s (high abnormal) or if preceded by at least 2 conditional scans. In total, 1653 scans were performed in 542 children, followed for 2235 patient-years. Fifty-eight (10.7%) high-risk subjects were identified, including 18 (31%) with high abnormal, and 15 (26%) with previous conditional scans. In 2 (3%), abnormal velocity was restricted to the anterior cerebral artery. The estimated proportion of children at high risk, scanned before 6 years of age was >20%. There were 4 cases of acute ischemic stroke (AIS) and 2 of acute hemorrhagic stroke. The incidence of all stroke, AIS, and acute hemorrhagic stroke were 0.27, 0.18, and 0.09 per 100 patient-years, respectively. The proportion of children at high risk is higher than most previous estimates, partly as a result of our modified risk assessment. About 2 children per 1000 screened with transcranial Doppler ultrasound progress to AIS.

A skills framework for sickle cell disease and thalassaemia.

The standard care that people with sickle cell disease or thalassaemia experience varies widely. A specialist nurse was inspired to address this by developing a competency framework for these haemoglobin disorders, which has been accredited by the Royal College of Nursing. This article looks at the rationale for creating competencies for nurses caring for people with these conditions, and outlines the standards required to achieve optimum patient care.

Factors affecting hospital staff judgments about sickle cell disease pain.

Judgments about people with pain are influenced by contextual factors that can lead to stigmatization of patients who present in certain ways. Misplaced staff perceptions of addiction may contribute to this, because certain pain behaviors superficially resemble symptoms of analgesic addiction. We used a vignette study to examine hospital staff judgments about patients with genuine symptoms of analgesic addiction and those with pain behaviors that merely resemble those symptoms. Nurses and doctors at hospitals in London, UK, judged the level of pain, the likelihood of addiction, and the analgesic needs of fictitious sickle cell disease patients. The patient descriptions included systematic variations to test the effects of genuine addiction, pain behaviors resembling addiction, and disputes with staff, which all significantly increased estimates of addiction likelihood and significantly decreased estimates of analgesic needs. Participants differentiated genuine addiction from pain behaviors resembling addiction when making judgments about addiction likelihood but not when making judgments about analgesic needs. The treatment by staff of certain pain behaviors as symptoms of analgesic addiction is therefore a likely contributory cause of inadequate or problematic hospital pain management. The findings also show what a complex task it is for hospital staff to make sensitive judgments that incorporate multiple aspects of patients and their pain. There are implications for staff training, patient education, and further research.