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Abstract

The management of crisis in sickle cell disease Okpala I. The management of crisis in sickle cell disease.Eur J Haematol 1998: 60: 1-6. 0 Munksgaard 1998. Abstract: The symptoms and signs of sickle cell disease are exacerbated in times of crisis, characterized by tissue infarction or worsening anaemia. Prompt medical intervention is required in these distressing situations to provide relief and comfort to the patient. Effective analgesia is crucial in treating the painful crisis of sickle cell disease. The haemoglobinopathy may cause hyposthenuria with reduced ability to excrete the sodium load in normal saline. A 5% dextrose solution or 5% dextrose in 25% normal saline is therefore recommended for intravenous hydration. As the leading cause of morbidity and mortality in sickle cell disease, infections call for vigorous antibiotic therapy. Oxygen administration should be reserved for hypoxic patients, and blood transfusion given only when really indicated. Acute chest syndrome and cerebrovascular accidents are life-threatening complications of sickle cell disease whereas priapism can cause important long-term sequelae; all deserve urgent attention. In the long term, comprehensive care is cost-effective in reducing the frequency and adverse effects of sickle cell I crisis.The first recorded description of the crisis now recognized as a feature of sickle cell disease was by Africanus Horton, who noted the bone pains and the association with fever and cold weather (1). The illness must have sustained the interest of medical scientists because by the beginning of this century Herrick had discovered the cellular basis of painful crisis (2) and, 75 years after Horton's account, sickle cell anaemia became the first human disease to be described at the molecular level (3). Haemoglobin S (HbS) is the result of a point mutation in the sixth codon of the P-globin gene (GAT+G?T), so that the 6th amino acid in P-globin is valine instead of glutamic acid, as found in normal haemoglobin (HbA). Sickle cell disease (SCD) is a generic term which includes sickle cell anaemia (HbSS), sickle cell haemoglobin C disease (HbSC), sickle cell thalassqemia disease (Shhal) and other compound heterozygous conditions which have in common the inheritance of the sickle P-globin gene and also cause clinical disease. It does not include sickle cell trait (HbAS). With the facility of modern travelling, human societies in various parts of the globe are becoming more lheanyi Okpala