National Audit of Criteria For Pre-Operative Sickle Cell Screening in Children.

Aim We set out to identify the current practice in the anaesthesiology departments of Ireland's public hospitals that deliver paediatric anaesthesia with regard to pre-operative screening for sickle cell disease (SCD) and Sickle cell trait (SCT). Methods The Departments of Anaesthesiology at 14 public HSE-funded hospitals that deliver paediatric anaesthesia were contacted over a three month period in 2020. Any existing policies regarding pre-operative screening of paediatric patients for Sickle cell disease or trait were sought. Comparisons were made between any screening policies in place. Results A response was received from 11 of the 14 hospitals. Three out of 11 of the Anaesthesiology Departments have formal policies in place. The ethnicities identified for pre-operative screening varied across these three hospitals. Conclusion Despite a significant increase in the number of people of African, middle Eastern & Indian descent living in Ireland in recent years, no neonatal screening programme for Sickle cell exists here, and no national policy exists with criteria to guide the practice of pre-operative screening of patients for SCD/SCT (trait). Our survey highlights a lack of standardisation in the approach to pre-operative sickle cell screening of children across Ireland's public hospital system. In view of the increasing multiculturalism in Ireland we recommend a national review of the merits of the introduction of developing a targeted national guideline for pre-operative screening for sickle cell in at-risk children.

The prevalence of sickle cell trait in Division I university athletics programs.

Objectives: To determine the prevalence of student-athletes with sickle cell trait (SCT) and describe their demographics, prior knowledge of status, and hemoglobin (Hb) profile. Methods: A retrospective chart review was conducted at two National Collegiate Athletic Association Division I universities. Participants were student-athletes during the 2010/11-2018/19 academic years. The independent variable was SCT status. Main outcome measures included actual prevalence of SCT positive student-athletes, sex, race, sport, prior knowledge of personal and family history SCT status, and Hb profile (HbA, HbA2, HbS, HbF, HbC) proportions. Results: Fifty-three SCT positive student-athletes (13.2 ± 2.0 per academic year) were identified, accounting for ~1% of the student-athlete population annually. The majority were Black/African-American (n = 49, 100.0%; 4 missing) and males (n = 44, 83.0%). Football had the majority (n = 28, 52.8%) of SCT student-athletes. Most student-athletes were unaware of their SCT status (n = 33, 62.3%). There was no difference between actual and expected prevalence of SCT student-athletes overall and by race in any academic year (p > 0.05). Results of Hb electrophoresis testing were available for 44 (83.0%) student-athletes. Average values for HbA, HbA2, HbS, HbF and HbC were 58.54 ± 4.26%, 3.42 ± 0.53%, 37.99 ± 4.60%, 0.17 ± 0.68% and 0.00 ± 0.00%, respectively. Conclusions: Student-athletes with SCT were a small proportion of the student-athlete population. The majority of SCT student-athletes had no prior knowledge of personal or family history; therefore, it is insufficient to rely on self-reported history. No difference was found between actual and expected prevalence of SCT student-athletes. Due to high proportion of student-athletes who are unaware of their SCT status, institutions should facilitate SCT screening with confirmatory testing for all student-athletes to prevent missed identification of those with SCT.

Malaria in patients with sickle cell anaemia: burden, risk factors and outcome at the Laquintinie hospital, Cameroon.

BACKGROUND: It is believed that the current prevalence of malaria in endemic areas reflects selection for the carrier form of sickle cell trait through a survival advantage. Malaria has been incriminated as a great cause of mortality in people with sickle cell disease (SCD). However, people with SCD, a high-risk group, do not benefit from free or subsisized malaria prevention and treatment in Cameroon unlike other vulnerable groups which may be due to insufficient evidence to guide policy makers. This study aimed at describing clinical and socio-demographic characteristics of patients with malaria, determining the prevalence of malaria in hospitalized children and in those with SCD and without, compare frequency of presentation of malaria related complications (using clinical and laboratory elements that define severe malaria) between children admitted for malaria with SCD and those without and finally, determing the risk factors for death in children admitted for malaria. METHODS: This was a retrospective analysis of admission records of children age 1 to 18 years with a confirmed malaria diagnosis admitted at the Laquintinie Hospital during January 2015 through December 2018. Clinical features, laboratory characteristics and outcome of malarial infections, stratified by SCD status were studied. Patients with HIV infection, malnutrition, renal failure and discharged against medical advice were excluded from the study. Data were analysed using Epi-info 7 software and analysis done. Chi square test, Odds ratios, CI and student's t test were used to determine association between variables. Statistical significance was set at p-value ≤0.05. RESULTS: The prevalence of malaria was lower among children with SCD than it was among children without SCD (23.5% vs 44.9%). Similarly, among those with a positive microscopy, the mean parasite density was significantly lower among children with SCD than it was among children without SCD (22,875.6 vs 57,053.6 parasites/ µl with t-value - 3.2, p-value 0.002). The mean hemoglobin concentration was lower in SCD as compared to non SCD (5.7 g/l vs 7.4 g/l, t-value - 12.5, p-value < 0.001). Overall mortality in SCD was 3.4% and malaria was reponsible for 20.4% of these deaths as compared to the 35.4% in non SCD patients. Convulsion and impaired consciousness were significantly lower in SCD group (OR:0.1, CI: 0.1-0.3, p value < 0.01 and OR:0.1, CI:0.1-0.2, p-value < 0.001 respectively). Death was significantly higher in SCD patients with malaria as compared to SCD patients admitted for other pathologies (3.2% vs 1.5%., OR:2.2, CI:1-5, p-value 0.050). CONCLUSION: The SCD population has a lower mortality related to malaria compared to the non-SCD population. Meanwhile, within the SCD population, those admitted with malaria are twice more likely to die than those admitted for other pathologies. Jaundice, hepatomegaly and splenomegaly were common in SCD with malaria, however no risk factors for malaria severity or malaria related death was identified.

Perspectives and Practices of Athletic Trainers and Team Physicians Implementing the 2010 NCAA Sickle Cell Trait Screening Policy.

Sickle cell trait (SCT) is usually benign. However, there are some conditions that may lead to SCT-related problems and put athletes with the trait at particular risk. In 2010 the National Collegiate Athletic Association (NCAA) issued a policy that required all Division I (DI) student-athletes to confirm their SCT status or sign a liability waiver to opt out of testing. Athletic trainers and team physicians play key roles in the policy implementation and we examined their perceptions and practices. Between December 2013 and March 2014 we interviewed 13 head athletic trainers and team physicians at NCAA Division I colleges and universities in North Carolina. We used an interview guide with open-ended questions covering knowledge of SCT, historical screening and education practices, current implementation, and policy benefits and challenges. Participants were knowledgeable about SCT and thought the policy was beneficial in providing SCT health information to and for student-athletes. Schools varied in provision of genetic counseling, offering the waiver, SCT tests administered, and other aspects. Challenges included: insufficient guidance from the NCAA; financial considerations; and misunderstanding of the relationships of race and ancestry to SCT risk. Athletic staff found the policy valuable, but felt it needs clarity and standardization.

Medio diagnóstico rápido y de bajo costo en la anemia drepanocítica / Rapid and low cost diagnostic mean in sickle cell anemia

La anemia drepanocítica (AD) es la enfermedad hematológica y multisistémica de origen genético más frecuente en Cuba y el mundo 1-2, es consecuencia de una mutación puntual en la cadena β de la globina, tiene herencia recesiva y expresión homocigótica, que resulta en una hemoglobina (Hb) estructuralmente anormal (HbS), con un genotipo Hb/SS2,3. A diferencia de la Hb normal del adulto (HbA), la molécula de HbS se polimeriza y se convierte en insoluble en condiciones de hipoxia, lo que da lugar a anormalidades estructurales y funcionales de los eritrocitos. Los eritrocitos falciformes tienen una fragilidad aumentada y son propensos a romperse y ocluir los vasos sanguíneos donde se adhieren, lo que ocasiona una anemia hemolítica crónica, crisis dolorosas intensas, flujo sanguíneo anormal en órganos diana, lo que puede provocar sintomatología variada y la muerte temprana de los pacient

Traço falciforme como potencial determinante da progressão de doenças renais em Salvador/Bahia / sickle cell trait as a potential determinant of progression of kidney disease in Salvador / Bahia

INTRODUÇÃO: A doença renal crônica (DRC) é uma doença grave que atinge cerca de 10% da população mundial. Devido à perda irreversível da função dos rins, os pacientes precisam do tratamento dialítico e desde 2010, no Brasil, a taxa de pacientes em diálise cresce de 3% cada ano. Cerca 93% do tratamento está financiado pelo SUS o que corresponde a 10% do orçamento do Ministério da Saúde. As principais causas de DRC no Brasil e no mundo são diabetes mellitus (DM) e hipertensão arterial sistêmica (HAS), seguido de glomerulopatias. As alterações podem ser complicadas por condições de hipóxia tecidual, as quais podem ser intensificadas pela doença falciforme. Os indivíduos com traço falciforme podem apresentar esse quadro clínico em condições extremas como um esforço físico intenso e prolongado. OBJETIVO: O objetivo deste estudo foi investigar a associação entre o traço falciforme e a progressão de DRC em Salvador-BA. MATERIAL E MÉTODOS: Foi desenvolvido um estudo de corte transversal, no qual no período de maio de 2014 até novembro de 2015...

INTRODUCTION: Chronic Kidney Disease (CKD) is a serious disease that affects about 10% of world population. It is due to irreversible loss of kidney function, so necessitating the patient’s need of dialysis treatment and since 2010, in Brazil, the rate of patients on dialysis is growing by 3% each year. About 93% of the treatment is funded by SUS which corresponds to 10% of the Health Ministry´s budget. The main causes of CKD in Brazil and in the world are diabetes mellitus and arterial hypertension, followed by glomerulopathies. The alterations can be complicated by conditions of tissue hypoxia, which can be intensified by the sickle cell disease. Individuals with sickle cell trait, although asymptomatic may present these clinical features in extreme conditions such as intense and prolonged physical activities. AIM: The aim of this study was to investigate the association between sickle cell trait and progression of CKD in patients on hemodialysis (HD) in Salvador, Bahia. MATERIAL AND METHODS: A cross-sectional cohort study was conducted from May 2014 to November 2015...

Sickle Cell Trait Screening of Collegiate Athletes: Ethical Reasons for Program Reform.

The National Collegiate Athletic Association (NCAA) requires all student-athletes have their sickle cell trait (SCT) status confirmed prior to athletic participation. The NCAA approved the screening program in 2010 for institutions participating in Division I athletics and extended it in subsequent years to institutions at Division II and III levels. Ethical concerns about the controversial policy focus on its mandatory nature and potential impact on student-athletes, particularly through stigmatization of and discrimination against those with SCT. Organizations, such as the American Society of Hematology (ASH), oppose the imposition of SCT testing and instead recommend universal precautions that would protect the entire student-athlete population without revealing student-athletes' SCT statuses. This paper discusses these issues and offers recommendations, including genetic counseling, which would improve the current SCT screening program. It argues that implementation of universal precautions would ensure that the most ethically sound practices are afforded to every student-athlete.

Traço falciforme como potencial determinante da progressão de doenças renais em Salvador/Bahia / sickle cell trait as a potential determinant of progression of kidney disease in Salvador / Bahia

INTRODUÇÃO: A doença renal crônica (DRC) é uma doença grave que atinge cercade 10% da população mundial. Devido à perda irreversível da função dos rins, os pacientes precisam do tratamento dialítico e desde 2010, no Brasil, a taxa de pacientes em diálise cresce de 3% cada ano. Cerca 93% do tratamento está financiado pelo SUS o que corresponde a 10% do orçamento do Ministério da Saúde. As principais causas de DRC no Brasil e no mundo são diabetes mellitus (DM) e hipertensão arterial sistêmica (HAS), seguido de glomerulopatias. As alterações podem ser complicadas por condições de hipóxia tecidual, as quais podem ser intensificadas pela doença falciforme. Os indivíduos com traço falciforme podem apresentar esse quadro clínico em condições extremas como um esforço físico intenso e prolongado. OBJETIVO: O objetivo deste estudo foi investigar a associação entre o traço falciforme e a progressão de DRC em Salvador-BA. MATERIAL E MÉTODOS: Foi desenvolvido um estudo de corte transversal, no qual no período de maio de 2014 até novembro de 2015; foram incluídos 306 indivíduos portadores de DRC em programa de hemodiálise nos hospitais e clínicas de referência tais como, Instituto de Nefrologia e Diálise (INED), Hospital Ana Nery (HAN) e Hospital Geral Roberto Santos (HGRS) há no máximo três anos. cinco mililitros (mL) de sangue total foram coletados em cada paciente para a caracterização do perfil de hemoglobinas variantes pela técnica de cromatografia líquida de alta eficiência (HPLC). Como grupo controle, foram utilizados os resultados dos testes de triagem neonatal do APAE realizados em recém-nascidos em Salvador de 2012-2014. RESULTADOS: A frequência de HbAS foi significamente maior nos pacientes em hemodiálise (10,2%) em comparação ao grupo controle (5,05%) OR: 2,04 IC 95% (1,35–2,99). Quando comparamos os pacientes com DRC com e sem traço falciforme, não houve diferença em relação à distribuição do sexo (homens 57,6% vs 50%, respectivamente, p = 0,43). A média de idade não foi diferente entre os dois grupos (52 ± 1 anos vs 56 ± 2, p = 0,21).CONCLUSÕES: A frequência do traço falciforme é maior em pacientes portadores de DRC em programa de hemodiálise em comparação à população geral. Estudos que avaliam o impacto e fisiopatologia da doença renal em indivíduos portadores de traço falciforme podem fornecer informações importantes para desenvolvimento de estratégias de prevenção da progressão para estágio final da doença renal.

INTRODUCTION: Chronic Kidney Disease (CKD) is a serious disease that affects about 10% of world population. It is due to irreversible loss of kidney function, so necessitating the patient’s need of dialysis treatment and since 2010, in Brazil, the rate of patients on dialysis is growing by 3% each year. About 93% of the treatment is funded by SUS which corresponds to 10% of the Health Ministry´s budget. The main causes of CKD in Brazil and in the world are diabetes mellitus and arterial hypertension, followed by glomerulopathies. The alterations can be complicated by conditions of tissue hypoxia, which can be intensified by the sickle cell disease. Individuals with sickle cell trait, although asymptomatic may present these clinical features in extreme conditions such as intense and prolonged physical activities. AIM: The aim of this study was to investigate the association between sickle cell trait and progression of CKD in patients on hemodialysis (HD) in Salvador, Bahia. MATERIAL AND METHODS: A cross-sectional cohort study was conducted from May 2014 to November 2015. The subjects consisted of 394 of both sexes with chronic renal failure on hemodialysis sessions for up to three years and treated in hospitals and clinics of reference such as the Institute of Nephrology and Dialysis (INED), Ana Nery’s Hospital (HAN) and Roberto Santos General Hospital (HGRS). 5mls of whole blood was collected from each patient to characterize the hemoglobin variants profile by High Performance Liquid Chromatography (HPLC). As a control group, the results of neonatal screening tests of APAE performed on newborns in Salvador 2012-2014 were used. RESULTS: The frequency of HbAS was significantly higher in hemodialysis patients (10.2%) compared to the control group (5.05%) OR: 2.04 95% CI (1.35 to 2.99). When comparing patients with CKD with and without sickle cell trait, there was no difference in relation to the distribution of sex (men 57.6% vs 50%, respectively, p = 0.43). The mean age was not different between the two groups (52 ± 1 years vs 56 ± 2, p = 0.21)...

Dosis moderadas de hidroxiurea en niños con drepanocitosis y accidente vascular encefálico. Resultados preliminares / Moderate dose of hydroxyurea in children with sickle cell disease and stroke. Preliminary results

Se trataron 20 niños con anemia drepanocítica, dos ellos con hemoglobinopatía SC y uno con S/â0 talasemia con accidente vascular encefálico o Doppler transcraneal con velocidades del flujo sanguíneo mayor de 170 cm/s. La media de seguimiento fue de 41 ± 19 meses. En los pacientes con accidente vascular encefálico se administraron 25 mg/kg/día de hidroxiurea y se realizó régimen de transfusión crónica por un año. En los niños con Doppler transcraneal patológico se administró la hidroxiurea sola en igual dosis. Hubo una disminución significativa del número de accidentes vasculares encefálicos (p <0.02) y de la velocidad del flujo sanguíneo en la arteria cerebral media derecha (p <0.003). En tres niños con velocidades de flujo muy aumentadas en el Doppler transcraneal sin accidente vascular encefálico fue necesario asociar régimen de hipertransfusión por no respuesta al tratamiento. La asociación de hidroxiurea y transfusiones de glóbulos rojos durante un año pueden ser útiles en el tratamiento y prevención del accidente vascular encefálico

Twenty children with sickle cell anemia, two with SC hemoglobinopathy and one with S/â0 thalassemia were treated, with a previous stroke or abnormal ultrasound transcranial Doppler (TCD) flow velocities more than 170 cm/s. The mean follow-up was of 41 ± 19 months. Hydroxyurea (HU) at a dose of 25 mg/kg/day associated with chronic transfusion therapy, was administrated during one year to patients with stroke. Patients with abnormal TCD received only HU at the same dose. There was a significant decrease of stroke (p <0.02) and TCD flow velocities in the right middle cerebral artery (p <0.003). It was necessary to associate chronic transfusion therapy in three children with high velocities in TCD without stroke, due to the lack of response to the treatment with HU. The combination of HU and transfusions during one year can be useful for stroke therapy and prevention

Dosis moderadas de hidroxiurea en niños con drepanocitosis y accidente vascular encefálico. Resultados preliminares / Moderate dose of hydroxyurea in children with sickle cell disease and stroke. Preliminary results

Se trataron 20 niños con anemia drepanocítica, dos ellos con hemoglobinopatía SC y uno con S/â0 talasemia con accidente vascular encefálico o Doppler transcraneal con velocidades del flujo sanguíneo mayor de 170 cm/s. La media de seguimiento fue de 41 ± 19 meses. En los pacientes con accidente vascular encefálico se administraron 25 mg/kg/día de hidroxiurea y se realizó régimen de transfusión crónica por un año. En los niños con Doppler transcraneal patológico se administró la hidroxiurea sola en igual dosis. Hubo una disminución significativa del número de accidentes vasculares encefálicos (p <0.02) y de la velocidad del flujo sanguíneo en la arteria cerebral media derecha (p <0.003). En tres niños con velocidades de flujo muy aumentadas en el Doppler transcraneal sin accidente vascular encefálico fue necesario asociar régimen de hipertransfusión por no respuesta al tratamiento. La asociación de hidroxiurea y transfusiones de glóbulos rojos durante un año pueden ser útiles en el tratamiento y prevención del accidente vascular encefálico(AU)

Twenty children with sickle cell anemia, two with SC hemoglobinopathy and one with S/â0 thalassemia were treated, with a previous stroke or abnormal ultrasound transcranial Doppler (TCD) flow velocities more than 170 cm/s. The mean follow-up was of 41 ± 19 months. Hydroxyurea (HU) at a dose of 25 mg/kg/day associated with chronic transfusion therapy, was administrated during one year to patients with stroke. Patients with abnormal TCD received only HU at the same dose. There was a significant decrease of stroke (p <0.02) and TCD flow velocities in the right middle cerebral artery (p <0.003). It was necessary to associate chronic transfusion therapy in three children with high velocities in TCD without stroke, due to the lack of response to the treatment with HU. The combination of HU and transfusions during one year can be useful for stroke therapy and prevention(AU)

Anemia drepanocítica y embarazo. Experiencia en el Instituto de Hematología e Inmunología, Cuba / Sickle cell disease and pregnancy. Experience at the Instituto de Hematologia e Inmunología, Cuba

El embarazo en la anemia drepanocítica (AD) es considerado una situación de alto riesgo por la alta incidencia de la morbimortalidad materno-fetal. En Cuba, el programa de atención integral a las embarazadas se incluye desde el nivel primario de salud y la captación se realiza antes de las 12 sem de gestación y los partos son institucionales. Todas las embarazadas con AD en La Habana son atendidas en el Instituto de Hematología e Inmunología (IHI) por un equipo multidisciplinario y los partos se realizan en el Servicio de Obstetricia del Hospital General Docente Enrique Cabrera . Desde enero del año 2000 hasta diciembre del 2009, 68 embarazadas con AD fueron atendidas por un equipo multidisciplinario. La frecuencia de las consultas fue quincenal hasta las 32 sem de la gestación y posteriormente semanal hasta la sem 36 en que fueron ingresadas; el embarazo se interrumpió en la semana 38. Las pacientes que presentaron algún evento fueron hospitalizadas y en ellas la interrupción se realizó en la semana 36 si el feto era viable. El bienestar fetal fue evaluado desde la semana 28 cada 2 semana hasta el nacimiento. No se realizaron transfusiones ni exanguinotransfusiones profilácticas y solo fueron indicadas según los criterios del equipo médico tratante; 16 pacientes recibieron transfusiones de glóbulos y la exanguinotransfusión se realizo en 4, todas en el tercer trimestre del embarazo. En 47 pacientes se realizó cesárea y siempre por indicación obstétrica; 17 recién nacidos tuvieron bajo peso pero solo uno tuvo un conteo de Apgar bajo. Ocurrieron 2 muertes fetales y una neonatal; se reportó una muerte materna

Pregnancy in women with sickle cell disease (SCD) is a high-risk situation associated with increased incidence of maternal and fetal morbidity and mortality. In Cuba, the maternal care program includes the primary level and the gestational age at booking is before the 12 week of gestation and all deliveries are institutional. All pregnant women with SCD in La Habana are attended at the Institute of Hematology and Immunology (IHI) by a multidisciplinary team and labor takes place at the obstetrics service of the General Hospital next to the IHI. From January 2000 to December 2009, 68 pregnant women with SCD were attended in labor; the frequency of the visits is every two weeks from gestational age at booking until week 32 of pregnancy and weekly until week 36 when they are hospitalized, in week 38 induction of labor is made. Patients were hospitalized upon the appearance of any event and in such cases induction of labor was made in week 36, if fetus was mature. The fetal well-being was evaluated starting from week 28 and every two weeks until childbirth. Non prophylactic blood transfusion or prophylactic exchange transfusions were indicated as this depends on the criteria of attending team; only 16 patients presented alert signs of requiring blood transfusion, 4 requiring blood exchange transfusions. All these procedures were carried out in the third trimester of pregnancy; 47 patients required caesarea indicated by the obstetrician; 17 newborns were underweight but only one with low apgar score. Two fetal deaths occurred and one new born had early neonatal death. Only one maternal death was reported

Anemia drepanocítica y embarazo. Experiencia en el Instituto de Hematología e Inmunología, Cuba / Sickle cell disease and pregnancy. Experience at the Instituto de Hematologia e Inmunología, Cuba

El embarazo en la anemia drepanocítica (AD) es considerado una situación de alto riesgo por la alta incidencia de la morbimortalidad materno-fetal. En Cuba, el programa de atención integral a las embarazadas se incluye desde el nivel primario de salud y la captación se realiza antes de las 12 sem de gestación y los partos son institucionales. Todas las embarazadas con AD en La Habana son atendidas en el Instituto de Hematología e Inmunología (IHI) por un equipo multidisciplinario y los partos se realizan en el Servicio de Obstetricia del Hospital General Docente Enrique Cabrera . Desde enero del año 2000 hasta diciembre del 2009, 68 embarazadas con AD fueron atendidas por un equipo multidisciplinario. La frecuencia de las consultas fue quincenal hasta las 32 sem de la gestación y posteriormente semanal hasta la sem 36 en que fueron ingresadas; el embarazo se interrumpió en la semana 38. Las pacientes que presentaron algún evento fueron hospitalizadas y en ellas la interrupción se realizó en la semana 36 si el feto era viable. El bienestar fetal fue evaluado desde la semana 28 cada 2 semana hasta el nacimiento. No se realizaron transfusiones ni exanguinotransfusiones profilácticas y solo fueron indicadas según los criterios del equipo médico tratante; 16 pacientes recibieron transfusiones de glóbulos y la exanguinotransfusión se realizo en 4, todas en el tercer trimestre del embarazo. En 47 pacientes se realizó cesárea y siempre por indicación obstétrica; 17 recién nacidos tuvieron bajo peso pero solo uno tuvo un conteo de Apgar bajo. Ocurrieron 2 muertes fetales y una neonatal; se reportó una muerte materna(AU)

Pregnancy in women with sickle cell disease (SCD) is a high-risk situation associated with increased incidence of maternal and fetal morbidity and mortality. In Cuba, the maternal care program includes the primary level and the gestational age at booking is before the 12 week of gestation and all deliveries are institutional. All pregnant women with SCD in La Habana are attended at the Institute of Hematology and Immunology (IHI) by a multidisciplinary team and labor takes place at the obstetrics service of the General Hospital next to the IHI. From January 2000 to December 2009, 68 pregnant women with SCD were attended in labor; the frequency of the visits is every two weeks from gestational age at booking until week 32 of pregnancy and weekly until week 36 when they are hospitalized, in week 38 induction of labor is made. Patients were hospitalized upon the appearance of any event and in such cases induction of labor was made in week 36, if fetus was mature. The fetal well-being was evaluated starting from week 28 and every two weeks until childbirth. Non prophylactic blood transfusion or prophylactic exchange transfusions were indicated as this depends on the criteria of attending team; only 16 patients presented alert signs of requiring blood transfusion, 4 requiring blood exchange transfusions. All these procedures were carried out in the third trimester of pregnancy; 47 patients required caesarea indicated by the obstetrician; 17 newborns were underweight but only one with low apgar score. Two fetal deaths occurred and one new born had early neonatal death. Only one maternal death was reported(AU)

Reproductive attitudes and behaviors in people with sickle cell disease or sickle cell trait: a qualitative interpretive meta-synthesis.

Sickle cell disease (SCD) is a serious and life threatening disorder. The literature on informed reproductive decisions among people with SCD is limited, suggesting that there is a lack of awareness about SCD and Sickle cell trait status (SCT) among high-risk populations. The purpose of this qualitative interpretive meta-synthesis (QIMS) is to examine the reproductive attitudes and behaviors in people with SCD or SCT to create effective genetic counseling programs to inform reproductive decision making. Three studies were included in the QIMS for a total sample of 79 participants. The majority of the sample was African American, ages 16 to 63. Other demographic characteristics of the study populations and data collection methods used in these studies varied. Seven themes on reproductive attitudes and behaviors among people with SCD or SCT emerged: lack of awareness or education about SCD and SCT; men who deny having SCT; attitudes toward learning one's SCT status; rationales for learning a partner's SCT status prior to commencing a relationship; valuing relationships over SCD risk; learning one's own and partner's SCT status; and the consequences of not asking about partners' SCT status. Implications for prevention programming involving improved education and awareness about the disease and reproduction are discussed.