Overview
This was a retrospective study, conducted at Mulago Hospital, the main National Referral and Teaching Hospital for Makerere University College of Health Sciences in Kampala, Uganda. A single reviewer extracted all the records of all children with SCA aged <18 years admitted to the Haematology Ward of Mulago Hospital during the study period from August 2012 to August 2014. In Uganda, there is no policy for routine sickle cell testing such as newborn screening. Children with symptoms and signs of sickle cell disease are tested. The confirmation of SCA was by Hb electrophoresis. Some of the children would have been tested during the infancy period when signs of SCD emerge and others are tested based on their clinical presentation to the hospital. In addition, some children were tested for SCD based on the presentation of a stroke. A standardized data collection sheet was used to collect information on demographic characteristics (name, age, sex, address) and clinical diagnosis, which included information on the diagnosis and other medical conditions in these children on admission. The data was cleaned to identify any duplication of patients. In Mulago Hospital, each patient is given a unique admission number and on subsequent admissions the same number is issued. In addition, the old file of the patient was retrieved were possible to include the new details of the admission. The first stroke admission was included in the analysis. Repeated admissions of the same patient with stroke were excluded. In cases where the admission number was not indicated these cases were also excluded.
Setting
In Uganda, Mulago Hospital is the National Referral hospital where patients with SCA are referred from other peripheral health facilities.. In the Acute care unit (ACU) of the paediatric department, we admit 7 to 10 children with sickle cell disease per day for an acute crisis. They are initially stabilized in the ACU and later transferred on a daily basis to the haematology ward of the hospital. In addition, the hospital has a SCA day care clinic which cares for the children with SCA. The clinic’s average attendance is 50 to 70 clients per day. They receive their routine medications and some interventions such as blood transfusions, intravenous antibiotics, and parenteral analgesics. Hydroxyurea is not registered for use in Uganda for people living with SCA.
Stroke management in Mulago Hospital: Children with SCA who are admitted with a clinical diagnosis of stroke are given a simple transfusion to raise the haemoglobin level up to 10 g/dl. Imaging such as CT scan and MRI/MRA are not accessible because of the cost associated with the tests. All radiological investigations are paid for at a cost to the patients in the hospital. The hospital only has a single CT scan machine and does not have an MRI machine. All cases of stroke are diagnosed using clinical symptoms and signs. As a result, the attending physician/paediatrician depends on their clinical skills to determine whether the patient has an ishaemic or haemorraghic stroke. In the event that the brain imaging is available, it usually is done well after 24 to 48 h of admission to the hospital. The children who suffer a stroke are offered a chronic transfusion program which involves a monthly visit to the sickle cell clinic for review and a simple blood transfusion. However, the biggest challenge is the lack of a reliable supply of blood to the clinic. This is because of the high demand for blood products to treat other conditions such as severe malaria anaemia, haematological malignancies, road traffic accidents and obstetric emergencies in the hospital and country at large.
Operational definitions
Primary diagnosis
This was considered as the first problem registered by the clinician on the admission record.
Secondary diagnosis
This was considered as the second problem registered by the clinician on the admission record.
Severe anaemia
This was defined using the World Health Organization classification as a haemoglobin concentration of less than 7 g/dl [8].
Stroke
This was defined using the World Health Organization definition of stroke: “rapidly developing clinical signs of focal (or global) disturbance of cerebral function, with symptoms lasting 24 h or longer or leading to death with no apparent cause other than of vascular origin [9].
Study variables
Data was extracted from the hospital medical record books using a standardized questionnaire. The information included socio-demographic characteristics such as: age, sex, tribe, address and clinical diagnosis such as primary and secondary diagnoses where applicable. The diagnosis extracted was based on the decision made by the attending team on discharge of the patient. In the event more than one diagnosis was made, we sub-divided them into a primary diagnosis and a secondary diagnosis.
Data analysis
All data was entered into Microsoft excel and analysis was done using the same software. The prevalence was calculated as the proportion of observation units that ever exhibited the state of interest, including those units that already exhibited the state during the period of study. Data was analyzed using descriptive statistics including proportions and percentages.