BACKGROUND Caring for a child with a chronic condition, such as sickle cell disease, can have a significant impact on parents and families. In order to provide comprehensive care and support to these families, psychometrically sound instruments are needed as an initial step in measuring the impact of chronic diseases on parents and families. We sought to evaluate the psychometric properties of ...

Introduction Sickle cell anaemia (HbSS) and sickle cell haemoglobin-C disease (HbSC) are the varieties of sickle cell disease usually encountered during pregnancy in Nigeria (Akinla, 1972). The incidence, though low, is certainly increasing. This is because young girls with a chronic haemolytic anaemia who in the past readily succumbed to intercurrent disease and malnutrition are now, with impr...

The sickle cell disease constitutes a major problem of public health. We find 5% to 20% of carriers of this disease in West Africa and 40% among some populations in central Africa (Congo, Zaire) and Nigeria (Beguè). In Mali prevalence is estimated to 12% with 3% for the homozygote form. It is a known disease and well documented on the scientific plan and its management is better and better codi...

There are 14 modules, in multimedia format and in English and French languages, addressing major issues in sickle cell disease, interspersed with multiple choice questions which allow self assessment of the individual’s knowledge and proficiency. These modules have been prepared and recorded by 11 different experts from Belgium, the Democratic Republic of Congo, France, India, Jamaica and Monac...

OBJECTIVE To analyze the perception of primary care physicians and nurses about access to services and routine health care provided to sickle cell disease patients. METHODS This descriptive exploratory study took a qualitative approach by surveying thirteen primary care health professionals who participated in a focus group to discuss access to services and assistance provided to sickle cell ...

The high prevalence of sickle cell disease (SCD) and trait in Sub-Saharan Africa coincides with the distribution of Plasmodiumfalciparum malaria. Due to prolonged heavy use of chloroquine (CQ) as an antimalarial, drug resistance has developed. Many countries including Tanzania abandoned the use of CQ for uncomplicated malaria, except its use as prophylaxis in patients with sickle cell disease. ...

OBJECTIVE Sickle cell disease comprises chronic, genetically determined disorders, presenting significant morbidity and high prevalence in Brazil. The goal of this study was to evaluate the quality of life of sickle cell disease patients (hemoglobin SS and SC) and their sociodemographic and clinical characteristics. METHODS Data was collected from clinical records and semi-structured intervie...

Recently, ithas been demonstrated thatthe offspring of one parent with the sickle cell trait and one w’ith thalassemia may develop a severe hemolytic anemia with some of the characteristics of both thalassemia and sickle cell disease, ineluding microcytosis and numerous sickled erythrocytes in the blood film. This syndrome has been called mi(’rodrepanocytic,9 or sickle cell-thalassemia, disease...

High altitude seemed to be responsible for seven recent cases of sickling crisis. People with sickle-cell trait are at risk if they fly in unpressurized aircraft, which are used for many local air services. Those with sickle-cell haemoglobin C disease should avoid air travel even in pressurized aircraft. Possibly as a result of "autosplenectomy," patients with sickle-cell anaemia seem to be abl...

While cerebral vasculopathy is a well-known early complication of sickle cell disease that has warranted transcranial doppler surveillance, little has been documented regarding the role of cervical circulation in the development of cerebrovascular events and transient ischemic attacks amongst sickle cell patients. We present the unique case of a symptomatic 18-year-old male with sickle cell dis...