Cognitive functioning in children from Nigeria with sickle cell anemia.

BACKGROUND: Cognitive impairment is a major neurological complication of sickle cell anemia (SCA) in the United States, but there are limited studies of cognitive impairment in Nigeria, the country with the highest SCA burden. We hypothesized that children from Nigeria with SCA have worse cognitive functioning than comparison children and explored the association between lower cognitive functioning and key laboratory demographic and socioeconomic variables among children with SCA. PROCEDURE: We conducted a cross-sectional survey, supplemented by anthropomorphic and laboratory data, among a convenience sample of children from Nigeria with and without SCA. We administered the Wechsler Intelligence Scale for Children, Version IV. Our primary outcome measures included (1) estimated IQ (Est. IQ), (2) working memory (WM), and (3) processing speed (PS). RESULTS: The sample included 56 children with SCA (mean age 9.20 [SD 2.75], 46.43% girls) and 44 comparison children (mean age 9.41 [SD 2.49], 40.91% girls). Children with SCA performed worse on Est. IQ (84.58 vs. 96.10, P = 0.006) and PS (86.69 vs 96.91, P = 0.009) than comparison children. There was no significant difference in WM between both groups. Factors associated with lower Est. IQ and PS among children with SCA included age, maternal education, weight-for-age Z scores, and height-for age Z scores. CONCLUSION: In this small sample of children from Nigeria, we found worse cognitive functioning in children with SCA than in comparison children, and that sociodemographic and anthropomorphic factors were correlated with cognitive functioning.

Capacity building and stroke risk assessment in Nigerian children with sickle cell anaemia.

BACKGROUND: Transcranial Doppler ultrasonography (TCD) measures blood flow velocities in the large cerebral vessels and thus, detects the risk of stroke. This report describes a capacity building program which enabled the use of TCD for detecting stroke risk and also describes the pattern of non-imaging TCD examinations seen in Nigerian children with sickle cell anaemia (SCA). PROCEDURE: Ten university graduates were trained on the use of TCD in a 5-day capacity building workshop after which, the three best candidates were employed to provide a 5-day a week TCD screening service in Lagos. Data from TCD examination collected between March 2011 and September 2013 were analysed and reported. RESULT: Between March 2011 and September 2013, 2,331 children with SCA aged 2-16 years had TCD studies. TCD's findings were classified as normal (standard risk) in 70.4%, conditional in 19% and abnormal (high risk) in 9.3%. The majority of children (76.9%) in the high risk category were aged 2-8 years. TCD study was inadequate for risk categorisation in 1.3% of the patients. CONCLUSION: Effective capacity building of middle level manpower is feasible and can provide a credible TCD screening service to communities with a high demand and a shortage of trained professionals. The pattern of TCD abnormalities seen in Africa are comparable to those obtained in several previous worldwide reports.

Psychosocial impact of sickle cell disorder: perspectives from a Nigerian setting.

Sickle Cell Disorder is a global health problem with psychosocial implications. Nigeria has the largest population of people with sickle cell disorder, with about 150,000 births annually. This study explored the psychosocial impact of sickle cell disorder in 408 adolescents and adults attending three hospitals in Lagos, Nigeria. A questionnaire was designed for the study, with some of commonly described areas of psychosocial impact including general public perceptions and attitudes, education, employment, and healthcare issues, and emotional responses.The majority of participants thought that society in general had a negative image of SCD, and reported negative perceptions and attitudes. Some issues in education, employment, and healthcare were expressed, however these were in the minority of cases. The results also showed that depressive feelings were experienced in almost half the study population, even though feelings of anxiety or self-hate were uncommon. Clinical implications of these findings are considered.

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.