Symptom Patterns and Progression of Complications in Sickle Cell Anemia and Sickle Cell Trait: Implications for Enhanced Medical Care and Education

Abstract

Background. Sickle cell anemia and sickle cell trait are two conditions characterized by the presence of hemoglobin S. However, sickle cell anemia is a disorder with wide clinical variability, and the sickle cell trait is considered an asymptomatic condition. Careful medical attention and educational programs about the disease have been successful in decreasing the mortality rate in patients with sickle cell anemia. A lack of these measures could increase the severity in patients with sickle cell disease and even in individuals with sickle cell trait who without adequate information may not take appropriate precautions. This study was conducted to establish the distribution of symptoms in patients with sickle cell anemia in Colombia and to determine whether there is an increase in the frequency of symptoms in sickle cell trait individuals in the context of Colombian medical care. Methods. We examined 109 patients with sickle cell anemia and 99 individuals with sickle cell trait. Symptom information was collected from the patients for both conditions. Pearson’s chi-square and Student’s t-test were used to identify differences between sickle cell anemia and sickle cell trait in clinical variables. To identify whether any clinical variable was associated with any condition, a correspondence analysis was performed. Results. The most frequent symptom was pain crisis, followed by dactylitis and pneumonia. Blood transfusion was required in 46% of this population. The frequency of pain crises and pneumonia was higher in patients with sickle cell anemia than in patients with sickle cell trait. However, 68.6% of patients with sickle cell trait suffered pain crises. Patients with sickle cell anemia also showed a 31-fold higher probability of receiving a blood transfusion than patients with sickle cell trait. Splenomegaly, pneumonia, and cardiac problems appeared to be interrelated symptoms in this population, suggesting an axis of progressive complications in patients with sickle cell anemia. Conclusions. These results show that the population with sickle cell anemia and sickle cell trait present clinical conditions that increase the severity of both conditions. Therefore, it is necessary to improve medical care and design educational campaigns to reduce the risk of complications.