T.S. Akingbola1, B. Kolude2, E.C. Aneni3, A.A. Raji1, K.U. Iwara1, Y.A. Aken’Ova1, and O.A. Soyannwo4
- Department of Haematology, University College Hospital, Ibadan, Nigeria.
- Department of Oral Pathology and Dentistry, University College Hospital, Ibadan, Nigeria.
- Department of Medicine, University College Hospital, Ibadan, Nigeria.
- Department of Anaesthesia, University College Hospital, Ibadan, Nigeria.
Background: Abdominal pain is a relatively frequent occurrence in sickle cell disease. The aetiology of abdominal pain in sickle cell disease is often difficult to diagnose clinically. Despite the frequent occurrence, diagnostic dilemma, and the need for an accurate, early diagnosis, abdominal pain in sickle cell disease has not been rigorously studied.
Objective: We therefore sought to describe the different presentations and patterns of abdominal pain in persons with sickle cell disease.
Methods: A prospective case series of 20 patients was done in which data was collected on demographic characteristics, hemoglobin electrophoresis patterns, a description of the abdominal pain including sites, severity, and type of pain, packed cell volume and the provisional and final diagnosis.
Results: Haemoglobin S patients were 17 in number constituting eightyfive percent (85%) of our study population whilst the rest 3 were Hb S+C. Most patients (70%) had one site of abdominal pain. The pain was mainly colicky or tightening, moderate to severe in nature and, in some cases, associated with vomiting. We did not find any significant difference between the steady state PCV and the PCV during the acute abdominal pain episodes. The final diagnosis showed that only 38.8% of the patients had vasoocclusive crises and the reliability index between the provisional diagnosis and the final diagnosis was 67%.
Conclusion: Abdominal pain in sickle cell disease may present in different ways and it is important to recognize that the possible diagnoses are numerous. Not all cases are due to vasoocclusive crises. Early diagnosis and prompt treatment can be life saving.
Keywords : Abdominal Pain, Sickle Cell Disease, Anaemia
Dr. Titilola S. Akingbola
University College Hospital,
Sickle Cell Disease (SCD) is defined as the inheritance of a sickle cell gene (Haemoglobin S) and another abnormal haemoglobin gene1, 2. It is the commonest genetic condition in Africa with up to 2% of children being born with the condition in some sub-Saharan countries3. Sickle cell anaemia, the homozygous form of sickle cell disease, is the commonest SCD variant in Africa with most of the rest being haemoglobin S+C disease1. In Nigeria, where our study population is drawn from, about 150,000 children are born with sickle cell anaemia yearly3 and it appears that an increasing number of children with SCD are surviving into adulthood.
SCD has clinical manifestations in many organs which are thought to be as a result of structural changes in the haemoglobin molecule1. Pain, the hallmark of sickle cell disease, is usually the major concern of patients and their care givers. Abdominal pain is a commonly encountered form of sickle cell pain2.
Abdominal vasoocclusive crisis is thought to be secondary to vasoocclusion of mesenteric vessels, and may be a sole complaint or may be associated with pain in other parts of the body usually musculoskeletal2,4. Abdominal vasoocclusive crises may be clinically indistinguishable from acute abdominal pain from other causes and can pose a diagnostic dilemma2, 4. Despite abdominal pain being a relatively frequent complaint in persons with sickle cell disease, and difficulty with the diagnosis, acute abdominal pain crisis has not been extensively evaluated in adult Africans with sickle cell disease.
Since the initial diagnosis is largely clinical, and decides the early line of management in most sub-Saharan settings including ours, we sought to describe the pattern of presentation and associated clinical features, the initial and the final diagnosis of abdominal pain in patients attending the haematology day care unit (HDCU) of the University College Hospital in Ibadan, South Western Nigeria.
Location The study was conducted in the Haematology day care unit (HDCU) of the University College Hospital, Ibadan.
This study is a pioneer prospective case series of 20 consecutive SCD patients presenting with abdominal pain at the HDCU, University College Hospital, Ibadan, Nigeria over an 18-month period spanning May 2005 – Nov 2006. All participants were previously diagnosed cases of sickle cell haemoglobinopathy with prior haemoglobin electrophoresis by cellulose acetate at an alkaline medium (pH 8.4). Structured questionnaires were administered to the SCD patients presenting with abdominal pain after due consent by calibrated inter viewers (Resident doctors). Questionnaire items included sociodemographic data, clinical features, laboratory findings, treatment modalities and pain assessment (location, frequency and intensity). Pain quality assessment was based on both the numerical and descriptive scales. The numerical or nominal scale spanning 1 to 10 was employed5. Data was analyzed using version 11 of window based statistical soft ware package (SPSS). Qualitative variables were expressed as percentages and compared using Chi square statistics while quantitative variables were summarized as means, SD, and confidence intervals and further compared using either student’s t-test for two variables or analysis of variance (ANOVA) test for more than two variables.