Strategies to improve healthcare services for patients with sickle cell disease in Nigeria: The perspectives of stakeholders.

Sickle cell disease (SCD) continues to pose physical and psychosocial burdens to patients, caregivers and health workers. Stakeholder engagement in the processes of policy making and implementation is increasingly becoming the cornerstone of best practices in healthcare. To engage stakeholders with a view to assessing the knowledge of SCD; ascertain the challenges associated with accessibility and affordability of healthcare services; improve the quality of care, and thereby effect behavioral change through increasing attendance and follow-up of patients in the clinics.

Sickle cell disease clinical phenotypes in Nigeria: A preliminary analysis of the Sickle Pan Africa Research Consortium Nigeria database.

Background/objective: Sickle cell disease (SCD) is a monogenic disease with multiple phenotypic expressions. Previous studies describing SCD clinical phenotypes in Nigeria were localized, with limited data, hence the need to understand how SCD varies across Nigeria.

Method: The Sickle Pan African Research Consortium (SPARCO) with a hub in Tanzania and collaborative sites in Tanzania, Ghana and Nigeria, is establishing a single patient-consented electronic database with a target of 13,000 SCD patients.

Paediatric to adult transition care for patients with sickle cell disease: a global perspective.

Sickle cell disease is a life-threatening inherited condition designated as a public health priority by WHO. Increased longevity of patients with sickle cell disease in high-income, middle-income, and low-income countries present unprecedented challenges for all settings; however, a globally standardised solution for patient transition from paediatric to adult sickle cell disease health care is unlikely to address the challenges. We established a task force of experts from a multicountry (the USA, Europe, Middle East, and Africa) consortium.

Effectiveness and Safety of Hydroxyurea in the Treatment of Sickle Cell Anaemia Children in Jos, North Central Nigeria.

Background: Hydroxyurea has been shown to positively modify sickle cell disease pathogenesis, but its use is low among Nigerian sickle cell anaemia (SCA) patients because of effectiveness and safety concerns.

Methods: We conducted a quasi-experimental study to evaluate the effectiveness and safety of hydroxyurea in 54 SCA children aged 4-17 years. Clinical and haematological parameters were compared at baseline and 12 months after hydroxyurea therapy.

Unprepared and Misinformed Parents of Children with Sickle Cell Disease: Time to Rethink Awareness Campaigns.

Worldwide, sickle cell disease (SCD) poses a significant public health concern. It causes recurrent morbidity, and premature death is a distinct possibility, especially in Nigeria, which bears half the world's burden of SCD patients. Nigeria has yet to establish a newborn screening program; consequently, most affected children are diagnosed between one and three years of age when a health problem arises.

HemoTypeSC, a low-cost point-of-care testing device for sickle cell disease: Promises and challenges.

Background: Sickle cell disease (SCD) is a neglected burden of growing importance. >312,000 births are affected annually by sickle cell anaemia (SCA). Early interventions such as newborn screening, penicillin prophylaxis and hydroxyurea can substantially reduce the mortality and morbidity associated with SCD.

A Multi-centre Survey of Acceptability of Newborn Screening for Sickle Cell Disease in Nigeria.

Background Sickle cell disease (SCD) is a major genetic disease that manifests early in life and may lead to significant morbidities. One of the health care services that have been effective in reducing the burden of SCD in developed countries is newborn screening (NBS) followed by pneumococcal vaccines, penicillin prophylaxis, and hydroxyurea treatment. Yet, in sub-Saharan African countries, where about 75% of annual affected babies worldwide are born, NBS programmes are largely unavailable.

Pattern of Cerebral Blood Flow Velocity Using Transcranial Doppler Ultrasonography in Children with Sickle Cell Disorder in Lagos State, Nigeria.

Unlabelled: Cerebrovascular accident (CVA) is a common, devastating neurological complication of sickle cell disorder (SCD) with a high recurrent and mortality rate. The Stroke Prevention Trial in Sickle Cell Anaemia study (STOP) recommends routine screening with transcranial Doppler ultrasonography in children aged two to sixteen years with SCD. The present study assessed cerebral blood flow velocities of children with SCD in accordance with the recommendation of routine screening by the STOP study.

Preliminary Evaluation of a Point-of-Care Testing Device (SickleSCAN™) in Screening for Sickle Cell Disease.

Sickle cell disease affects about 150,000 births annually in Nigeria. Early diagnosis is hampered by factors such as centralized and urban localization of laboratories, high cost of diagnostic equipment and inadequate skilled manpower to operate them. The need for a low-cost, portable, easy-to-use diagnostic test for sickle cell disease is critical, especially in resource-poor countries.

Head circumference of children with sickle cell disease in Lagos, Nigeria.

Introduction: Growth retardation and under-nutrition are common in children with sickle cell disease (SCD). The aim of this study was to compare the head circumference (HC) of SCD children and non-SCD children and to determine the effect of malnutrition on head circumference of children with SCD.

Methods: This was a prospective case-control study conducted at the Lagos State University Teaching Hospital, Nigeria, involving SCD children (subject, n = 118) and non-SCD children (control, n = 118) matched for age, sex, and socioeconomic status.

Blood transfusion services for patients with sickle cell disease in Nigeria.

Background: Safe, timely red blood cell transfusion saves lives and chronic transfusion therapy (CTT) prevents or limits morbidities such as stroke, therefore improving quality of life of patients with sickle cell disease (SCD).

Methods: This questionnaire-based study assessed the ability of sickle cell centers in Nigeria to provide safe blood to patients with SCD between March and August 2014.

Results: Out of the 73 hospitals contacted, responses were obtained from 31.

Health related quality of life and perception of stigmatisation in adolescents living with sickle cell disease in Nigeria: A cross sectional study.

Background: Sickle cell disease impacts the physical, emotional and psychological aspects of life of the affected persons, often times exposing them to disease associated stigma from the society and alters the health related quality of life (HRQoL). This study compared the HRQoL of adolescents with sickle cell disease with their healthy peers, identified socio-demographic and clinical factors impacting HRQoL, and determined the extent and effects of SCD related stigma on quality of life.

Procedure: We conducted a cross-sectional survey among 160 adolescents, 80 with SCD and 80 adolescents without SCD.

Acute soft head syndrome in children with sickle cell anaemia in lagos, Nigeria.

Acute soft head syndrome is rare complications seen in children with sickle cell anaemia. A case report of a child with sickle cell anaemia who developed acute soft head syndrome. A 12-year old known sickle cell anaemia patient presented with acute, rapidly progressive skull pain and swelling, manifestations indicative of the rare complication of SCD which is called acute soft head syndrome.

Evaluation of arm span and sitting height as proxy for height in children with sickle cell anemia in Lagos, Nigeria.

Background: Sickle cell anemia may affect linear growth, and complications like avascular necrosis of femoral head may make direct measurement of height difficult.

Objective: To determine the relationship between height and arm span as well as between height and sitting height among children with sickle cell anemia in Lagos, Nigeria.

Methodology: A random sample of 200 children aged 8 months to 15 years were studied-100 with hemoglobin genotype SS and 100 with hemoglobin genotype AA, matched for age and sex.

Iron Deficiency Anaemia among Pre-School Children with Sickle Cell Anaemia: Still a Rare Diagnosis?

Background: The frequent need for blood transfusion in children with SCA creates the impression that IDA is rare in this class of children.

Objectives: The objective of the study is to determine the prevalence of IDA in a population of under-five children with SCA in Lagos, Nigeria.

Methodology: Serum iron, total iron binding capacity, transferrin saturation and serum ferritin were assayed in 97 under-five children with SCAand 97 age/sex matched controls.

Obesity--does it occur in Nigerian children with sickle cell anemia.

Children with sickle cell anemia are vulnerable to growth deficits; thus, it would be thought that obesity would be rare among them. The objective of the study is to examine the prevalence of obesity in a sickle cell anemia population in Lagos. A random sample of children with sickle cell anemia aged 2-15 years was interviewed and anthropometric measurements including weight and height were taken.

Helicobacter pylori infection among a pediatric population with sickle cell disease.

Objectives: Recurrent abdominal pain (RAP) is a common presenting symptom in children with sickle cell disease (SCD). This may be as a result of complications of the disease, surgical problems, or Helicobacter pylori gastritis. The prevalence of H pylori infection in SCD children is not known.