Importance of health guidance for family members of children with sickle cell disease.

Sarah Vieira Figueiredo,Macedônia Pinto Dos Santos,Letícia Alexandre Lima,Débora Pena Batista E Silva,Raquel De Maria Carvalho Oliveira,Ilvana Lima Verde Gomes

doi:10.1590/0034-7167-2017-0806

Sarah Vieira Figueiredo, Macedônia Pinto Dos Santos + Show 4 more

Open Access

https://doi.org/10.1590/0034-7167-2017-0806

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Abstract

To know the main health guidance needs of family members of children with sickle cell disease. Qualitative research, developed in a pediatric reference hospital of Ceará State, between April and May 2017, through the participation of 12 family members of children with sickle cell disease. The data were collected through semi-structured interviewees and analyzed according to the Bardin's Thematic Categorical Analysis. The relatives had divergent opinions about what this pathology would be and expressed the expectation of being broadly guided, from general information (signs and symptoms) to more complex ones about the disease, including major complications and ways of preventing them. The health fragility due to the punctual and/or meager guidance provided to family members reflects the importance of increasing knowledge and clarifying doubts of these relatives about the disease, which makes it urgent to develop health education strategies by multiprofessional teams.

Highlights

Hemoglobinopathies are a set of hereditary disorders related to genes responsible for the synthesis of globins
Sickle cell diseases are due to a mutation in the gene responsible for the production of HbA, which results in the formation of a different Hb, called S, which has recessive inheritance
Eleven children used folic acid, seven hydroxyurea, seven oral penicillins, two iron chelators, two analgesics and only one used ferrous sulfate

Summary

Introduction

Hemoglobinopathies are a set of hereditary disorders related to genes responsible for the synthesis of globins. It is estimated that approximately 7% of the world population has hemoglobin-related (Hb) disorders, with sickle cell disease being the most prevalent hereditary disease in the world[1]. It is estimated that in Brazil an average of 3,000 people are born with this disease per year[2]. Because it is a not notifiable disease, it is difficult to calculate the exact number of affected individuals. Sickle cell diseases are due to a mutation in the gene responsible for the production of HbA, which results in the formation of a different Hb, called S, which has recessive inheritance. The combinations of a mutant Hb with another of type S constitute sickle diseases, with the most frequent being: sickle cell anemia (Hb SS), S beta thalassemia and the double heterozygous HbSC and HbSD[2,6]

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