Abstract
To analyze the profile of pain, its triggering factors, and the relationship with the use of hydroxyurea in children and adolescents with sickle cell disease. This was an analytical cross-sectional study of 80 patients with sickle cell disease, both male and female, aged 6 to 18 years, seen at the Center for Hematology and Hemotherapy of Pernambuco, Brazil. To assess the pain profile, forms with the adapted visual scale from the “Fear of Dental Pain Questionnaire – Short Form” were used. Fisher's exact test was used to evaluate the association between pain manifestations and the use of hydroxyurea. Pain was reported by 68.7% of the patients and 52.7% of them reported severe pain, which eventually affected their daily routines. Physical triggers presented the highest rate (78.2%). The most frequent sites of pain were the trunk (80%) and lower extremities (54.5%), and constricting (40%) and deep (40%) pain were the most frequent types. Thirty percent of the patients reported being treated with hydroxyurea for prevention of painful events. It is concluded that the patients with sickle cell disease showed a high rate of painful events and physical factors, such as cold temperature, trauma and physical effort, were considered to be the most prevalent. There was no association between the use of hydroxyurea and pain improvement.
Highlights
Sickle cell disease (SCD) is a single-gene genetic and inherited disorder
Pain was an apparent symptom among the children and adolescents who participated in the present study and it is an important clinical manifestation of SCD
The analysis of the perception of painful events associated with Sickle cell anemia (SCA) among children and their caregivers demonstrated that assessing pain in terms of its characteristics, impact, and coping and management strategies is a protective measure, as such assessment allows adopting approaches that could minimize the potential detrimental effect of pain on child development (Dias et al, 2013)
Summary
Sickle cell disease (SCD) is a single-gene genetic and inherited disorder. It is caused by a mutation in the gene that produces hemoglobin A, converting it to sickle hemoglobin (HbS). Sickle cell anemia (SCA) is considered the most common type of SCD (Campelo et al, 2018). SCD is recognized by the World Health Organization (WHO) as a serious public health problem, having a large impact on the morbidity and mortality of affected individuals if it is left undiagnosed or is not treated properly (McGann, 2014). The distribution of SCD in Brazil is quite heterogeneous, but the northern and northeastern regions have the highest prevalence rates (6 to 10%) owing to the predominance of the African American ethnic group, a risk factor that is well established in the literature (Jesus, 2010)
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