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Families of children with sickle cell disease: an integrative review

Elaine Cristina Rodrigues Gesteira, Regina Szylit Bousso, Maira Deguer Misko, Carolliny Rossi de Faria Ichikawa, Patrícia Peres de Oliveira


Aim:  To  identify  scientific  evidence  about  families  of  children  with sickle  cell  disease.  Method:  An  integrative  review  of  the  search  used the  following descriptors:  anemia  sickle  cell;  hemoglobin  SC  disease;  hemoglobin sickle;  family; family  relations;  and,  child.  The  search  was  carried  out  for  literature  in  Portuguese, English,  and  Spanish.  The  search  used  databases  such  as  MEDLINE,  LILACS,  CINAHL, and PubMed, and looked for articles published between January 2005 and January 2015. Results:   There   were   16   articles   selected  that   originated  the   following  thematic categories: the impact of sickle cell disease on the family, psychosocial effects on family dynamics,  child's  quality  of  life,  and  family  and  social  support/support  networks. Discussion:  The  studies  report  that  the  family  remains  the  main  provider  of  care  for children  with  sickle  cell  disease,  and  that  families  faces  challenges  in  achieving comprehensive care and fighting for their children’s quality of life. C onclusion: This evidence  will  provide  support  for  multi-professional  teams  in the  construction  of continuous care for the families of children who are sickle cell patients.


Sickle Cell Desease; Family; Nursing.


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Gold JI, Treadwell M, Weissman L, Vichinsky E. The mediating effects of family functioning on psychosocial outcomes in healthy siblings of children with sickle cell disease. Pediatr Blood Cancer. 2011; 57(1):1055-61.[included in the review].

Guimaraes TMR, Miranda WL, Tavares MMF. The day-to-day life of families with children and adolescents with sickle cell anemia. Rev Bras Hematol Hemoter.2009;31(1):9-14. [included in the review].

Hildenbrand AK, Barakat LP, Alderfer MA, Marsac ML. Coping and coping assistance among children with sickle cell disease and their parents. J Pediatr Hematol Oncol. 2015; 37(1):25-34. [included in the review].

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Kam J,Panepinto JA,Brandow AM,Brousseau DC. Parental perception of quality of hospital care for children with sickle cell disease. Wis Med J. 2008;107(3):131-35. [included in the review].

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Raphael JL, Butler AM, Rattler TL, Kowalkowski MA, Mueller BU, Giordano TP. Parental information, motivation, and adherence behaviors among children with sickle cell disease. Pediatr Blood Cancer. 2013; 60(7):1204-10. [included in the review].

Marsh VM, Kamuya DM, Molyneux SS. ”All her children are born that way”: gendered experiences of stigma in families affected by sickle cell disorder in rural Kenya. Ethn Health. 2011; 16(4-5):343-59. [included in the review].

Tunde-Ayinmode MF. Psychosocial impact of sickle cell disease on mothers of affected children seen at University of Ilorin Teaching Hospital, Ilorin, Nigeria. East Afr Med J. 2007; 84(9):410-19. [included in the review].

Mitchell MJ, Lemanek K, Palermo TM, Crosby LE, Nichols A, Powers SW. Parent perspectives on pain management, coping, and family functioning in pediatric sickle cell disease. Clin Pediatr (Phila). 2007;46(4):311-19. [included in the review].

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