Literature DB >> 33735176

Descriptive analysis of sickle cell patients living in France: The PHEDRE cross-sectional study.

Marie Gerardin1, Morgane Rousselet1,2,3, Marie-Laure Couec4, Agathe Masseau5, Marylène Guerlais1, Nicolas Authier6, Sylvie Deheul7, Anne Roussin8, Joelle Micallef9, Samira Djezzar10, Fanny Feuillet3,11, Pascale Jolliet1,3, Caroline Victorri-Vigneau1,3.   

Abstract

BACKGROUND: Sickle cell disease (SCD) induces chronic haemolytic anaemia and intermittent vaso-occlusion that results in tissue ischaemia causing acute, severe pain episodes that can lead to frequent hospitalizations. These consequences can have repercussions on family, social, school and/or professional life. Here, we present some of the results of the PHEDRE study (Pharmacodépendance Et DREpanocytose-drug dependence and sickle-cell disease), which is the largest study of patients with SCD in France. This paper intends to describe characteristics of the French SCD population. We also aimed to assess the impact of the disease on the lives of patients using objective and subjective variables.
METHODS: The PHEDRE study was a national multicentric observational study. Adults, adolescents and children with a confirmed SCD diagnosis were included in the study by their referring doctor. Then, they were interviewed by phone about their socioeconomic status, about the impact of the disease on their lives and about their analgesic and psychoactive drug use.
RESULTS: The study population consisted of 872 patients (28% were minors). Seventy-two percent of adults were active, and all minors were in school. Many patients presented criteria of severe SCD. Seventy-five percent were homozygous SS, 15% were double heterozygotes SC and 8% were heterozygotes Sβthal, 87% received specific treatment, 58% were hospitalized at least once for vaso-occlusive crisis in the past 12 months, and the number of analgesic drugs taken averaged 3.8. Seventy-five percent of patients reported academic or professional consequences related to their SCD, and 52% reported social consequences.
CONCLUSIONS: The impact of SCD on patients' lives can be significant, nevertheless their social integration seems to be maintained. We highlighted respect of recommendations regarding analgesic treatments and only a few patients used tobacco, alcohol or cannabis. TRIAL REGISTRATION: Clinical Trials, NCT02580565; https://clinicaltrials.gov/ Registered 16 October 2015.

Entities:  

Year:  2021        PMID: 33735176      PMCID: PMC7971579          DOI: 10.1371/journal.pone.0248649

Source DB:  PubMed          Journal:  PLoS One        ISSN: 1932-6203            Impact factor:   3.240


  42 in total

Review 1.  The natural history of sickle cell disease.

Authors:  Graham R Serjeant
Journal:  Cold Spring Harb Perspect Med       Date:  2013-10-01       Impact factor: 6.915

2.  Parent pain catastrophizing predicts child depressive symptoms in youth with sickle cell disease.

Authors:  Alana Goldstein-Leever; Lindsey L Cohen; Carlton Dampier; Soumitri Sil
Journal:  Pediatr Blood Cancer       Date:  2018-03-07       Impact factor: 3.167

3.  Cannabis use in sickle cell disease: a questionnaire study.

Authors:  Jo Howard; Kofi A Anie; Anita Holdcroft; Simon Korn; Sally C Davies
Journal:  Br J Haematol       Date:  2005-10       Impact factor: 6.998

Review 4.  Psychological complications in sickle cell disease.

Authors:  Kofi A Anie
Journal:  Br J Haematol       Date:  2005-06       Impact factor: 6.998

Review 5.  Quality of life in adults with sickle cell disease: an integrative review of the literature.

Authors:  Sandra Luzinete Felix de Freitas; Maria Lucia Ivo; Maria Stella Figueiredo; Maria Auxiliadora de Souza Gerk; Cristina Brandt Nunes; Fernando de Freitas Monteiro
Journal:  Rev Bras Enferm       Date:  2018 Jan-Feb

Review 6.  Can parents rate their child's health-related quality of life? Results of a systematic review.

Authors:  C Eiser; R Morse
Journal:  Qual Life Res       Date:  2001       Impact factor: 4.147

Review 7.  Differences in the clinical and genotypic presentation of sickle cell disease around the world.

Authors:  Santosh L Saraf; Robert E Molokie; Mehdi Nouraie; Craig A Sable; Lori Luchtman-Jones; Gregory J Ensing; Andrew D Campbell; Sohail R Rana; Xiao M Niu; Roberto F Machado; Mark T Gladwin; Victor R Gordeuk
Journal:  Paediatr Respir Rev       Date:  2013-11-15       Impact factor: 2.726

8.  The relationship between genotype, psychiatric symptoms and quality of life in adult patients with sickle cell disease in São Paulo, Brazil: a cross-sectional study.

Authors:  Érika Bergamini Mastandréa; Fátima Lucchesi; Marcela Mayumi Gomes Kitayama; Maria Stella Figueiredo; Vanessa de Albuquerque Citero
Journal:  Sao Paulo Med J       Date:  2015-08-21       Impact factor: 1.044

9.  Parent proxy-report of their children's health-related quality of life: an analysis of 13,878 parents' reliability and validity across age subgroups using the PedsQL 4.0 Generic Core Scales.

Authors:  James W Varni; Christine A Limbers; Tasha M Burwinkle
Journal:  Health Qual Life Outcomes       Date:  2007-01-03       Impact factor: 3.186

10.  Sickle Cell Anaemia Prevalence Among Newborns in the Brazilian Amazon-Savanna Transition Region.

Authors:  Rayane Cristina Souza; Pedro Agnel Dias Miranda Neto; Jessflan Rafael Nascimento Santos; Sílvio Gomes Monteiro; Maria Cláudia Gonçalves; Fabrício Brito Silva; Rodrigo Assuncao Holanda; Julliana Ribeiro Alves Santos
Journal:  Int J Environ Res Public Health       Date:  2019-05-10       Impact factor: 3.390

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