OBJECTIVE: to estimate survival, mortality and cause of death among users or not of hydroxyurea with sickle cell disease.METHOD: cohort study with retrospective data collection, from 1980 to 2010 of patients receiving inpatient treatment in two Brazilian public hospitals. The survival probability was determined using the Kaplan-Meier estimator, survival calculations (SPSS version 10.0), comparison between survival curves, using the log rank method. The level of significance was p=0.05.RESULTS: of 63 patients, 87% had sickle cell anemia, with 39 using hydroxyurea, with a mean time of use of the drug of 20.0±10.0 years and a mean dose of 17.37±5.4 to 20.94±7.2 mg/kg/day, raising the fetal hemoglobin. In the comparison between those using hydroxyurea and those not, the survival curve was greater among the users (p=0.014). A total of 10 deaths occurred, with a mean age of 28.1 years old, and with Acute Respiratory Failure as the main cause.CONCLUSION: the survival curve is greater among the users of hydroxyurea. The results indicate the importance of the nurse incorporating therapeutic advances of hydroxyurea in her care actions.

OBJETIVO: estimar la sobrevida, la mortalidad y la causa de muerte de usuarios y no usuarios de hidroxiurea con enfermedad falciforme.MÉTODO: cohorte retrospectiva de 1980 a 2010 de pacientes internados en dos hospitales públicos brasileños. Se determinó la probabilidad de sobrevida con Kaplan-Meier, cálculos de sobrevida (SPSS versión 10.0), comparación entre curvas de sobrevida, método Log Rank. Nivel de significado p=0,05.RESULTADOS: de 63 pacientes, 87% estaban con anemia falciforme, siendo que 39 usaban hidroxiurea, promedio de edad en la institución del fármaco de 20,0±10,0 años y dosificación promedio de 17,37±5,4 a 20,94±7,2mg/kg/día, elevando la hemoglobina fetal. En la comparación de usuarios y no usuarios de hidroxiurea, la curva de sobrevida fue mayor en los usuarios (p=0,014). Ocurrieron 10 muertes, edad promedio de 28,1 años, siendo la Insuficiencia Respiratoria Aguda la causa principal.CONCLUSIÓN: la curva de sobrevida es mayor en los usuarios de hidroxiurea. Los resultados apuntan la importancia de que el enfermero incorpore los avances terapéuticos de la hidroxiurea en sus acciones asistenciales.

OBJETIVO: estimar a sobrevida, mortalidade e causa de morte em usuários ou não de hidroxiureia com doença falciforme.MÉTODO: coorte retrospectiva de 1980 a 2010, de pacientes internados em dois hospitais públicos brasileiros. Determinou-se a probabilidade de sobrevida com Kaplan-Meier, cálculos de sobrevida (SPSS versão 10.0), comparação entre curvas de sobrevida e método Log Rank. Nível de significância p=0,05.RESULTADOS: de 63 pacientes, 87% estavam com anemia falciforme, sendo 39 em uso de hidroxiureia, com média de idade na instituição do fármaco de 20,0±10,0 anos e dosagem média de 17,37±5,4 a 20,94±7,2mg/kg/dia, elevando a hemoglobina fetal. Na comparação de usuários e não usuários de hidroxiureia, a curva de sobrevida foi maior nos usuários (p=0,014). Ocorreram 10 óbitos, com idade média de 28,1 anos, tendo como causa principal a Insuficiência Respiratória Aguda.CONCLUSÃO: a curva de sobrevida é maior nos usuários de hidroxiureia. Os resultados apontam a importância do enfermeiro incorporar avanços terapêuticos da hidroxiureia em suas ações assistenciais.

OBJETIVO: estimar a sobrevida, mortalidade e causa de morte em usuários ou não de hidroxiureia com doença falciforme.MÉTODO: coorte retrospectiva de 1980 a 2010, de pacientes internados em dois hospitais públicos brasileiros. Determinou-se a probabilidade de sobrevida com Kaplan-Meier, cálculos de sobrevida (SPSS versão 10.0), comparação entre curvas de sobrevida e método Log Rank. Nível de significância p=0,05.RESULTADOS: de 63 pacientes, 87% estavam com anemia falciforme, sendo 39 em uso de hidroxiureia, com média de idade na instituição do fármaco de 20,0±10,0 anos e dosagem média de 17,37±5,4 a 20,94±7,2mg/kg/dia, elevando a hemoglobina fetal. Na comparação de usuários e não usuários de hidroxiureia, a curva de sobrevida foi maior nos usuários (p=0,014). Ocorreram 10 óbitos, com idade média de 28,1 anos, tendo como causa principal a Insuficiência Respiratória Aguda.CONCLUSÃO: a curva de sobrevida é maior nos usuários de hidroxiureia. Os resultados apontam a importância do enfermeiro incorporar avanços terapêuticos da hidroxiureia em suas ações assistenciais.

OBJECTIVE: to estimate survival, mortality and cause of death among users or not of hydroxyurea with sickle cell disease.METHOD: cohort study with retrospective data collection, from 1980 to 2010 of patients receiving inpatient treatment in two Brazilian public hospitals. The survival probability was determined using the Kaplan-Meier estimator, survival calculations (SPSS version 10.0), comparison between survival curves, using the log rank method. The level of significance was p=0.05.RESULTS: of 63 patients, 87% had sickle cell anemia, with 39 using hydroxyurea, with a mean time of use of the drug of 20.0±10.0 years and a mean dose of 17.37±5.4 to 20.94±7.2 mg/kg/day, raising the fetal hemoglobin. In the comparison between those using hydroxyurea and those not, the survival curve was greater among the users (p=0.014). A total of 10 deaths occurred, with a mean age of 28.1 years old, and with Acute Respiratory Failure as the main cause.CONCLUSION: the survival curve is greater among the users of hydroxyurea. The results indicate the importance of the nurse incorporating therapeutic advances of hydroxyurea in her care actions.

OBJETIVO: estimar la sobrevida, la mortalidad y la causa de muerte de usuarios y no usuarios de hidroxiurea con enfermedad falciforme.MÉTODO: cohorte retrospectiva de 1980 a 2010 de pacientes internados en dos hospitales públicos brasileños. Se determinó la probabilidad de sobrevida con Kaplan-Meier, cálculos de sobrevida (SPSS versión 10.0), comparación entre curvas de sobrevida, método Log Rank. Nivel de significado p=0,05.RESULTADOS: de 63 pacientes, 87% estaban con anemia falciforme, siendo que 39 usaban hidroxiurea, promedio de edad en la institución del fármaco de 20,0±10,0 años y dosificación promedio de 17,37±5,4 a 20,94±7,2mg/kg/día, elevando la hemoglobina fetal. En la comparación de usuarios y no usuarios de hidroxiurea, la curva de sobrevida fue mayor en los usuarios (p=0,014). Ocurrieron 10 muertes, edad promedio de 28,1 años, siendo la Insuficiencia Respiratoria Aguda la causa principal.CONCLUSIÓN: la curva de sobrevida es mayor en los usuarios de hidroxiurea. Los resultados apuntan la importancia de que el enfermero incorpore los avances terapéuticos de la hidroxiurea en sus acciones asistenciales.

Background: In 2001, the Brazilian Ministry of Health added hemoglobinopathies to the National Neonatal Screening Program to be implemented in three steps. In order to meet the proposed goals, it is crucial to establish periodic assessments of this program with the aim of monitoring its implementation. Objective: To assess the scope and the efficiency of the stages of the National Newborn Screening Program in identifying hemoglobin S. Methods: A cross-sectional study was developed with the results of the heel prick test using the high performance liquid chromatography method for babies born in Mato Grosso do Sul from 2006 to 2010. The following variables were investigated: year, number of live births, total screening, coverage ratio, prevalence, time between the child birth and the blood collection; age at diagnosis; age at the time of the first consultation; and time between the diagnosis and the first appointment. Results: Over the five years of the study, the mean coverage rate was 91.77%.The prevalences of hemoglobin FAS and hemoglobin FS were 1.65% and 0.011%, respectively. Blood samples from 43.48% of children were collected from the second to the seventh day. The age at diagnosis was within the first 28 days in 87.80% of the screened children. The lowest mean indices for the first consultation and the time between the diagnosis and the first appointment (58.8 and 46.4 days, respectively) occurred in 2010. Conclusions: The scope of the National Neonatal Screening Program for hemoglobinopathies is good, with a large number of individuals being tested. Efficiency is a suitable indicator to assess the program steps. Three points are recommended: the training of the individuals involved, studies to assess the assistance provided to the affected child, and genetic counseling to the mothers.

OBJECTIVE: To assess the burden and quality of life of caregivers of patients with sickle cell anemia taking hydroxyurea versus those of patients not taking hydroxyurea. METHODS: A cross-sectional study was performed of caregivers of outpatients with sickle cell anemia in two public hospitals in Campo Grande, MS, from January through June 2010. The World Health Organization Quality of Life-BREF Scale and the Caregiver Burden Scale were used. RESULTS: Of the 37 caregivers in this study, 81.1% were women, 73.0% were mothers, 59.5% were married, 54.1%were mulattos, 48.6% were housewives, 54.1% had family incomes of up to one minimum wage and 75.7% had onlycompleted elementary education. The mean duration of care provided (time after diagnosis) was 16.08 ± 9.88 yearsand 89.2% reported that they provided 24-hour care. Regarding health, 27.0% of study participants reported having physical and 13.5% emotional problems. There were no significant relationships between these variables either with the different domains or the total score of the WHOQOL-BREF comparing caregivers of patients taking hydroxyurea versusthose of patients not taking hydroxyurea. There was a moderate negative linear correlation between the WHOQOL-BREF and the Caregiver Burden Scale scores (linear correlation test of Pearson: p-value = 0.003, r = -0.477). The burden of caregivers of patients who did not take hydroxyurea was significantly higher than those of patients who took the medication in terms of general tension, disappointment, environment and total score (student t-test: p-value < 0.05). CONCLUSION: In the perception of the caregiver, looking after sickle cell anemia patients represents a moderate negative burden.

OBJETIVO: Buscar na literatura aspectos epidemiológicos explorados sobre a hemoglobina S. MÉTODOS: Trata-se de um levantamento bibliográfico nas bases de dados Medline e Lilacs. Também foi feita uma busca não eletrônica, em publicações de 1976 a 2007. RESULTADOS: Os resultados mostraram que, dos 21 artigos selecionados, 7 (33,3%) foram publicados entre 1976 e 2000, 10 (47,7%) entre 2001 e 2004 e 4 (19%) entre 2005 e 2007. Com relação aos descritores, três referências (14%), são da área da enfermagem, descrevem o quadro clínico e a fisiopatologia, sendo que uma delas sistematiza a assistência à clientela com anemia falciforme à luz do referencial de adaptação de Roy; 11 (52%) destacaram estudos epidemiológicos e a distribuição mundial; e sete (34%) contemplaram diagnóstico médico, triagem neonatal e programas voltados à população falcêmica no Brasil. CONCLUSÃO: Os resultados apontam a necessidade de investigação nessa área pelos profissionais de saúde, principalmente os da área da enfermagem, em relação aos cuidados de prevenção, promoção e reabilitação dos pacientes falcêmicos.

PURPOSE: To search the literature for epidemiological aspects of hemoglobin S. METHODS: This study was a bibliographic investigation from 1976 to 2007 using Medline and Lilacs databases. A manual search was also conducted. RESULTS: Among the 21 articles selected, 7 of them (33.3%) were published between 1976 and 2000, 10 of them (47.7%) were published between 2001 and 2004, and 4 of them (19.0%) were published between 2005 and 2007. Three of those articles that described the pathophysiology and clinical presentation were published by nurses. One of the publications, using Roy's adaptation model, described the nursing process to clients with falciform anemia. Eleven publications (52.0%) were worldwide epidemiological studies. Seven publications (34.0%) described the diagnosis, neonatal screening, and programs for the management of falciform anemia in Brazil. CONCLUSION: There is a need for further research in the topic by health care professionals, especially by nurses regarding preventive measures, and the management and rehabilitation of patients with falciform anemia.

OBJETIVO: Buscar en la literatura aspectos epidemiológicos explorados sobre la hemoglobina S. MÉTODOS: Se trata de un levantamiento bibliográfico en las bases de datos Medline y Lilacs. También se hizo una búsqueda no electrónica, en publicaciones de 1976 a 2007. RESULTADOS: Los resultados mostraron que, de los 21 artículos seleccionados, 7 (33,3%) fueron publicados entre 1976 y 2000, 10 (47,7%) entre 2001 y 2004 y 4 (19%) entre 2005 y 2007. Con relación a los descriptores, tres referencias (14%) son del área de enfermería, describen el cuadro clínico y la fisiopatología, siendo que una de ellas sistematiza la asistencia a la clientela con anemia falciforme bajo el marco teórico de adaptación de Roy; 11 (52%) destacaron estudios epidemiológicos y la distribución mundial; y siete (34%) contemplaron el diagnóstico médico, la clasificación neonatal y los programas dirigidos a la población con anemia falciforme, en Brasil. CONCLUSIÓN: Los resultados apuntan la necesidad de realizar investigaciones en esa área por profesionales de la salud, principalmente los del área de enfermería, en relación a los cuidados de prevención, promoción y rehabilitación de los pacientes con anemia falciforme.

PURPOSE: To search the literature for epidemiological aspects of hemoglobin S. METHODS: This study was a bibliographic investigation from 1976 to 2007 using Medline and Lilacs databases. A manual search was also conducted. RESULTS: Among the 21 articles selected, 7 of them (33.3%) were published between 1976 and 2000, 10 of them (47.7%) were published between 2001 and 2004, and 4 of them (19.0%) were published between 2005 and 2007. Three of those articles that described the pathophysiology and clinical presentation were published by nurses. One of the publications, using Roy's adaptation model, described the nursing process to clients with falciform anemia. Eleven publications (52.0%) were worldwide epidemiological studies. Seven publications (34.0%) described the diagnosis, neonatal screening, and programs for the management of falciform anemia in Brazil. CONCLUSION: There is a need for further research in the topic by health care professionals, especially by nurses regarding preventive measures, and the management and rehabilitation of patients with falciform anemia.

OBJETIVO: Buscar en la literatura aspectos epidemiológicos explorados sobre la hemoglobina S. MÉTODOS: Se trata de un levantamiento bibliográfico en las bases de datos Medline y Lilacs. También se hizo una búsqueda no electrónica, en publicaciones de 1976 a 2007. RESULTADOS: Los resultados mostraron que, de los 21 artículos seleccionados, 7 (33,3%) fueron publicados entre 1976 y 2000, 10 (47,7%) entre 2001 y 2004 y 4 (19%) entre 2005 y 2007. Con relación a los descriptores, tres referencias (14%) son del área de enfermería, describen el cuadro clínico y la fisiopatología, siendo que una de ellas sistematiza la asistencia a la clientela con anemia falciforme bajo el marco teórico de adaptación de Roy; 11 (52%) destacaron estudios epidemiológicos y la distribución mundial; y siete (34%) contemplaron el diagnóstico médico, la clasificación neonatal y los programas dirigidos a la población con anemia falciforme, en Brasil. CONCLUSIÓN: Los resultados apuntan la necesidad de realizar investigaciones en esa área por profesionales de la salud, principalmente los del área de enfermería, en relación a los cuidados de prevención, promoción y rehabilitación de los pacientes con anemia falciforme.

OBJETIVO: Buscar na literatura aspectos epidemiológicos explorados sobre a hemoglobina S. MÉTODOS: Trata-se de um levantamento bibliográfico nas bases de dados Medline e Lilacs. Também foi feita uma busca não eletrônica, em publicações de 1976 a 2007. RESULTADOS: Os resultados mostraram que, dos 21 artigos selecionados, 7 (33,3%) foram publicados entre 1976 e 2000, 10 (47,7%) entre 2001 e 2004 e 4 (19%) entre 2005 e 2007. Com relação aos descritores, três referências (14%), são da área da enfermagem, descrevem o quadro clínico e a fisiopatologia, sendo que uma delas sistematiza a assistência à clientela com anemia falciforme à luz do referencial de adaptação de Roy; 11 (52%) destacaram estudos epidemiológicos e a distribuição mundial; e sete (34%) contemplaram diagnóstico médico, triagem neonatal e programas voltados à população falcêmica no Brasil. CONCLUSÃO: Os resultados apontam a necessidade de investigação nessa área pelos profissionais de saúde, principalmente os da área da enfermagem, em relação aos cuidados de prevenção, promoção e reabilitação dos pacientes falcêmicos.