Objective  To define the incidence, risk factors and complications of priapism in a large population of patients with sickle‐cell anaemia in five centres in the UK and Nigeria, as priapism is common among these patients, but the precise characteristics of the condition in this population are poorly documented.Patients and methods  A questionnaire was developed and administered to patients with sickle‐cell disease. Questions were designed to define the incidence, nature, precipitants, duration, treatment and complications of priapism. A distinction was made between acute (severe) priapism and the recurrent, ‘stuttering’ type.Results  The questionnaire was completed by 130 patients (mean age 25 years, sd 11, range 4–66) from the five centres; 102 (78%) were homozygous Hb SS genotype, 19 (15%) were Hb SC genotype and two (1.5%) were Hb Sα−thalassaemia. Of the patients, 46 (35%) reported a history of priapism, and of these, 33 (72%) had a history of stuttering priapism, while 24 (52%) had had an acute episode of priapism. The mean age of onset of priapism was 15 years, with 75% of patients having the first episode before their 20th birthday. Sexual activity was the most frequent precipitating factor, with fever and/or dehydration being the next most common. Of the 46 patients, 10 (21%) with a history of priapism reported having erectile dysfunction. A similar proportion reported dissatisfaction with sexual intercourse, including a fear of engaging in sexual activity.Conclusion  The incidence of priapism among patients with sickle‐cell anaemia is high (35%). The implications of priapism for erectile and sexual function are significant and documented in this large series. The treatment of this condition in these patients remains unstandardised. This study highlights the need for an increased awareness of the problems associated with priapism among patients, families and medical professionals.

Nigeria has a population of 112 million with an annual growth rate of 3.2%. About 25% of adults throughout the country have the sickle cell trait, AS, while the Hb C trait is largely confined to the Yoruba people of southwestern Nigeria in whom it occurs in about 6%. Other variant hemoglobins including beta thalassemia are rare, but alpha thalassemia occurs in 39% (32% with 3 alpha-globin genes; 7% with 2 alpha-globin genes). Of a total of 5.4 million expected live births in 1988, about 90,000 will have SCD and 1.1 million the trait, AS. The clinical phenotype of sickle cell anemia is severe with manifestations occurring very early in childhood and mean Hb level 7.6 g/dl with HbF 5.9%. A very high infant mortality due to infections occurs especially in rural areas. Gallstones, leg ulcerations, and stroke appear less common than in American sicklers, and aplastic crises have not been described. Poor availability of resources to the public health and welfare sectors and economic inflation are severely curtailing access to appropriate medical and social services. This situation is frustrating to the families of a growing number of surviving patients in urban or middle to upper income groups. Efforts to create more awareness of SCD are paradoxically increasing frustration and stigmatization in the absence of a commensurate improvement of services. Any measures aimed at enhancing the sensitization of health professionals, policy makers, and resource allocators to the pertinent issues in the control of SCD would seem to be at this stage an important step in the right direction.

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.

Summary:In order to assess the role of the gonads and anterior pituitary gland in the production of poor quality semen of males with homozygous sickle cell disease (SCD) serum gonadotrophins, namely follicle stimulating hormone (FSH), luteinizing hormone (LH), prolactin and serum testosterone, were assayed radioimmunologically in 33 men with sickle cell disease and in 29 age-matched normal control subjects.Our results show a significantly lower mean serum testosterone, a higher mean FSH and prolactin in SCD subjects than in normal controls. No single SCD subject had significantly low serum testosterone associated with low FSH, LH or prolactin.The tendency for higher gonadotrophins associated with lower testosterone in subjects with SCD suggests that the hypothalamic/pituitary function in these patients is intact and that the primary fault leading to poor sperm production lies in the testes.

The pattern of illness in 60 consecutive children with homozygous sickle cell disease who attended the Paediatric Emergency Room of a busy Lagos hospital with acute illness was studied prospectively. Their ages ranged from 3 months to 13 years with a peak in the 2nd year. There were twice as many boys as girls. The commonest symptoms were fever, limb or abdominal pain and cough, and the commonest signs were pallor and hepatomegaly. Painful crises occurred in 27, anaemic crises in 11, and a combination of these in 12 children. Infection was detected in 76% of subjects in crises. Infection was found in 82% of all the children and was mainly bacterial. The commonest infections were pneumonia (35%), bacteraemia (32%), tonsillitis/pharyngitis (17%) and osteomyelitis (8%). The predominant bacteria isolated were Klebsiella spp (38%), E. coli (23%), Staph. aureus (23%), Staph. albus (23%) and Pseudomonas spp (23%). Some children had multiple isolates. Bacterial infection was a major cause of morbidity in very young children and merits appropriate control and preventive measures in this age group. The spectrum of bacteria isolated makes it unlikely that the specific anti-pneumococcal measures widely advocated in Europe and America for young children with SCA would be appropriate in Nigeria.