Clinical and care profiles of children and adolescents with Sickle Cell Disease in the Brazilian Northeast region

Abstract

Objectives: to describe the clinical epidemiological and care profiles of pediatric patients with sickle cell disease. Methods: a descriptive study of all (48) children and adolescents with sickle cell disease, assisted at a public referral hospital in Paraíba State. The information were obtained from the patients’ medical records and interviews were analyzed by using the Epi-Info program 7.2 version, frequency tables were built for the categorical variables and the central measurements and dispersion tendencies were calculated for the variables related to age and hospitalizations. Results: the patients’ age ranged from 15 months to 19 years old (median 8.6 years old); 91.7% considered their skin color mixed/black; 81.3% belong to D and E social class; 48% of the responsible guardians reported to have less than nine years of schooling; 70.9% lived in other cities; 93.8% received late diagnosis and 87.5% had irregular outpatient follow-up, 62,5% had an incomplete or outdated vaccination record. There were 226 hospitalizations; painful crises were the most common causes (55.7%). Each patient was hospitalized, about 5.2 times in the period; the median of total days for being hospitalized was 28. There were no deaths. Cardiac (56.2%) and hepatobiliary (54.3%) were the most common chronic compli-cations. Conclusions: sickle cell disease is a neglected clinical condition in the Brazilian Northeast region, where the appropriate political support for the patients is not fulfilled.

Clinical-Epidemiological Characteristics and Mortality in Patients with Sickle Cell Anemia: A Retrospective Cohort Study of 1980 at 2018

“…Furthermore, the patients' follow-up period was less than 20 years in a little less than half of the cases. Other Brazilian states such as Amazonas 25 -North region -and Paraíba 26 -Northeast region -also present similar data concerning gender and age group, follow-up period, and age at diagnosis.…”

“…34 These figures can be associated both with the number of comorbidities and to the complications developed by the patients during the follow-up period, especially pain crisis. 26 Despite the expressive number of hospitalizations observed, this data can be underestimated since dozens of patients lived in the state's inland and may have sought medical care in their municipalities of residence. This is due to the territorial distance separating them from the specialized centers for treatment and the logistical difficulties the patients face.…”

Ser adolescente apesar das restrições e da discriminação impostas pela doença falciforme

“…Esses fatores poderiam conduzir a complicações e agravos maiores decorrentes da doença. (17) A categoria "Vivenciando situações ruins", constituída pelas subcategorias "Vivenciando a dor intensa", "Submetendo-se a consultas e exames laboratoriais" e "Sendo hospitalizado frequentemente", foi demarcada como a condição interveniente, na qual a diferença entre adolescente com DF e outros sem esta condição crônica de saúde teve como destaque as crises dolorosas.…”

“…(18) Estudos relatam que os adolescentes com DF necessitam frequentar regulamente os serviços de saúde, (15,19) são submetidos a internações hospitalares, uso contínuo de medicamentos, além da realização de procedimentos diagnósticos e terapêuticos à medida que a doença progride. (17) As hospitalizações frequentes, em decorrência das doenças crônicas, interferem nos laços afetivos e, por vezes, causam rupturas nos relacionamentos, devido aos longos períodos de afastamento. Esse tipo de evento também pode aumentar a vulnerabilidade do adolescente com doença crônica, dar início a um processo de depressão e evidenciar enfrentamento ineficaz, alterações do autoconceito e diminuição da autoestima.…”

Effect of reading an educational booklet about sickle cell disease on family members' knowledge

“…Pesquisa desenvolvida com 48 crianças e adolescentes com doença falciforme identificou que 87,5% possuíam seguimento ambulatorial de consultas irregulares, o que também foi encontrado no Figueiredo SV, Moreira TMM, Campos DB, Chaves EMC, Gomes ILV presente estudo (16) . Porém, destaca-se o tamanho reduzido da amostra (20 participantes) e as diferenças que possam existir, a depender do tipo de doença falciforme e de suas complicações, uma vez que quanto mais grave, maior pode ser o número de consultas necessárias.…”