Family management ofchildren who experience sickle cell disease: a qualitative study.

Elaine Cristina Rodrigues Gesteira,Regina Szylit,Edilene Aparecida Araújo Silveira,Carolliny Rossi De Fariaichikawa,Maiara Rodrigues Dos Santos,Patricia Peres De Oliveira

doi:10.1590/0034-7167-2019-0521

Elaine Cristina Rodrigues Gesteira, Regina Szylit + Show 4 more

Open Access

https://doi.org/10.1590/0034-7167-2019-0521

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Abstract

to know the family management experience of children with sickle cell disease in the light of the Family Management Style Framework. a qualitative case study carried out between September/2015 and July/2016 with 12 members of eight families registered in a blood center in Minas Gerais. The semi-structured interviews were recorded, and the data were analyzed and interpreted by the hybrid model thematic analysis. three management styles were identified: five families in the accommodating style; two families in the struggling style; and only one family in the enduring style.It was noted that empowerment was paramount in the acquisition of skills and abilities to care for these children. family management knowledge of children with sickle cell disease provided a reflection on nurses' role in supporting, orienting and encouraging the empowerment of these families aiming at the search for comprehensive care.

Highlights

Sickle cell disease (SCD) is an autosomal recessive hemoglobinopathy of genetic origin, in which sickle hemoglobin arises from a variation of the hemoglobin A’s (HbA) beta globin (HBB) gene
The main results in the family management experience of children with SCD were evidenced with the research, according to the conceptual components and dimensions of the FMSF model
The deductive analytical process guided by the theoretical model of the FMSF allowed to know the functioning of the family when dealing with children with SCD

Summary

Introduction

Sickle cell disease (SCD) is an autosomal recessive hemoglobinopathy of genetic origin, in which sickle hemoglobin arises from a variation of the hemoglobin A’s (HbA) beta globin (HBB) gene. Parents transmit the altered gene to their children, and children receive the modified gene in double dose ofHbSS, the most serious form of SCD, that is, sickle cell anemia Other hemoglobinopathies, such as HbC, HbD, HbE and beta-thalassemiamay appear in combination with HbS, which present similar clinical symptoms and that form SCD[2]. Sickle cells are less flexible when compared to normal cells In this way, they join the vascular endothelium causing an inflammatory process that, with the local increase of erythrocytes, granulocytes, monocytes and platelets, originates the vasoocclusive phenomena (hemolysis, venous stasis, tissue hypoxia). They join the vascular endothelium causing an inflammatory process that, with the local increase of erythrocytes, granulocytes, monocytes and platelets, originates the vasoocclusive phenomena (hemolysis, venous stasis, tissue hypoxia) As a consequence, they affect different organs, causing the following clinical manifestations: painful crises, hemolytic anemia and lesions in different systems[1]

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