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Contrary to international trends, the mortality rate of sickle cell disease increased in Brazil after the implementation of the neonatal screening program, probably due to improving access to diagnosis. This study aimed to assess differences in the temporal trend of the mortality rate and median age at death from sickle cell disease in Brazil, considering implemented measures to expand diagnosis, and improve health care access in-country and in the international scenario. Time series were extracted from the Brazilian Mortality Information System from 1996 to 2019. Changes in the mortality rate and median age at death were verified via segmented regression models, which were stratified by sex, region of residence, and age. Most deaths occurred in non-white people, young adults, and the Southeast and Northeast population. Sickle cell disease mortality rate increased until 2010 (13.31%; 95%CI: 6.37; 20.70), particularly in individuals aged 30 years or more (12.78%; 95%CI: 2.98; 23.53) and in the Northeast (12.27%; 95%CI: 8.92; 15.72). Most deaths occurred in the second decade of life (3.01 deaths/million), with a 59% increase in the median age of death in Brazil, from 27.6 to 30.3 years, more pronounced in females and the North Region. The observed gain in the survival of sickle cell disease in Brazil is still much lower than in developed countries and presents regional disparities, probably due to the lack of access to health care and recent treatments, such as hydroxyurea, still restricted to hematological referral centers in Brazilian capitals.

Background and Aim. Hematological disorders (HD) affect millions of people annually worldwide. Nutritional care is essential in acute or chronic HD, especially in controlling metabolic and gastrointestinal disorders that affect the nutritional status of individuals. Methods. A bibliographic survey was carried out between 2020 and 2022 using two databases: PubMed/MEDLINE and Scientific Electronic Library Online (SciELO) and the information source Academic Google, irrespective of language or geography. Results. In the first nutrition consultation (FNC), there should be an investigative direction focused on nutritional interventions in the short, medium, and long term. The record in the patient’s medical record is relevant for carrying out the consultation, according to the recommendations of the normative councils of medicine and nutrition. The main steps to be followed are the investigation of the presence of food allergies and intolerances; the drugs/nutritional supplements in use; changes in the digestive tract; the presence or absence of picamalacia; and socioeconomic and lifestyle data. In addition, it is necessary to carry out laboratory evaluations, semiological assessment, anthropometric assessment, and assessment of food consumption. In the end, the nutritional approach should be composed of calculation of energy and macronutrient and micronutrient needs, intervention in nutritional status deviations, nutritional guidelines, and nutritional therapeutic planning of return, focusing on adherence to treatment. Conclusion. The first nutrition consultation may represent investigative steps that help the clinical nutritionist in the management, allowing a longitudinal and specific nutritional therapeutic planning for patients assisted in large reference centers for hematological disease.

Sickle Cell Disease (SCD) is a chronic inherited disorder, characterized by the presence of abnormal erythrocytes containing S hemoglobin (HbS) that leads to diverse clinical complications and special nutritional needs. This article aimed to describe food and nutrition education actions for people with SCD. This is a report of the experience with educational practices developed by a Reference Center and performed at a hematology ambulatory in the university hospital from 2010 to 2012 that aimed to promote adequate and healthy eating habits. Data were obtained by consulting the educational practice notes; 171 people participated in 15 educational practices. The subjects were grouped into four main themes: General recommendations for people with sickle cell disease; Increased nutrient requirements; Strategies to reduce salt and sodium intake; and Iron content and availability in foods. Educational practices allowed for the reflection and guidance on appropriate feeding practices and for the findingof doubts on dietary recommendationsfor SCD through people's own reports. The space in the waiting room promoted the exchange of knowledge between the professionals who conducted the practices and participants. However, it was noted as weaknesses, the focus centered on the nutrients, the difficulty of adopting methodological strategies more targeted for age and the lack of inclusion of a multidisciplinary team. The experience presented has important reflections on the need for food, and food and nutrition education is common tool to healthcare professionals throughout the network of assistance to people with sickle cell disease. DOI: http://dx.doi.org/10.12957/demetra.2014.10539