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.

Informing carriers of beta-thalassemia: giving the good news.

This study explored the value of informing beta-thalassaemia carriers of the advantages, as well as the disadvantages of carrier status. Twenty-eight carriers of beta-thalassaemia were interviewed immediately after counselling, and again 2 weeks later. Both interviews included administration of a psychological scale (previously used for cystic fibrosis). Immediately after the first interview the intervention group (n = 18) were informed of the protective effect of the beta-thalassaemia trait against malaria and coronary heart disease. The control group (n = 10) was given the same information after the second interview. The effect of giving the positive information was assessed by comparing participants' scores at the first and second interview. Knowledge of carrier status aroused several negative feelings, including shock, sadness, and anger, but little feeling of stigmatization. Two weeks later, negative feelings were unchanged in the control group, but they were reduced in all members of the intervention group. All members of the intervention group considered it important to inform carriers of the positive aspects as well as the risks associated with carrier status. Carriers of recessive disorders with a known heterozygote advantage should be informed of the advantage. This information has now been incorporated into the comprehensive information system for hemoglobin disorders available at http://www.chime.ucl.ac.uk/ApoGI/.

A prospective diary study of stuttering priapism in adolescents and young men with sickle cell anemia: report of an international randomized control trial--the priapism in sickle cell study.

Priapism is defined as a prolonged, persistent, and purposeless penile erection. It is a common (35%) but frequently understated complication in young men and adults with sickle cell disease. We had previously demonstrated an association between stuttering attacks (<4 hours) and an acute catastrophic event with its consequent problems of erectile dysfunction and impotence. We describe a randomized, placebo-controlled, clinical study looking at medical prophylaxis with 2 oral α-adrenergic agonists, etilefrine and ephedrine, in preventing stuttering attacks of priapism. One hundred thirty-one patients were registered into a 2-phase (observational and intervention phase) study, and 86 patients (66%) completed Phase A diary charts. Forty-six patients (59%) completed a 6-month treatment phase (Phase B), and the remaining patients were lost to follow-up despite persistent efforts to contact them. Various reasons are postulated for the high attrition rates. The drugs were well tolerated, and no serious adverse events were reported. There was no significant difference among the 4 treatment groups in the weekly total number of attacks in Phase B (analysis of covariance P = .99) nor among the average pain score per attack after adjusting for attack rates and pain scores in Phase A (analysis of covariance P = .33). None of the patients who completed the study required penile aspiration at study sites while on medical prophylaxis. Young men with sickle cell disease are not comfortable engaging with health care providers about issues relating to their sexual health. The full impact of an improved awareness campaign and early presentation to hospital merits further standardized study. Priapism still contributes seriously to the comorbidity experienced by this previously inaccessible group of patients and medical prophylaxis with oral α-adrenergic agonists is feasible. Future international collaborative efforts using some of the lessons learnt in this study should be undertaken.