The five key things you need to know to manage adult patients with sickle cell disease

Abstract

The lack of a strong evidence base to guide the management of adults with sickle cell disease (SCD) makes it difficult for patients to receive high quality care outside of specialty centers. As there is a dearth of providers with sickle cell expertise, the purpose of this article is to identify some of the key things every provider who manages the care of adults with SCD should know. Managing adults with SCD requires excellent clinical skills, as it can affect every organ and cause life-threatening complications but it also requires a willingness to manage patients who often have psychosocial issues that are complex and impact care and care delivery in very significant ways. We have chosen topics for which there is a limited evidence base but which have significant clinical consequences if left unrecognized or poorly managed. The topics that will be addressed include chronic pain, neurocognitive dysfunction, renal disease, venous thromboembolism, and avoiding the inappropriate use of red cell transfusions.

Determinants of hematology-oncology trainees' postfellowship career pathways with a focus on nonmalignant hematology

“…The program directors' survey found that ,6% of graduates of adult training programs pursued careers in nonmalignant hematology, but there was little discussion of factors involved in this career choice. 5 Others have noted a decline in the number of physicians specializing in nonmalignant hematology careers such as care of patients with hemophilia and sickle cell disease, 7,8 and more recently, a group of hematologists published an overview calling for the need to expand the nonmalignant hematology workforce. 9 In this project, we surveyed recent graduates of a large academic hematology-oncology fellowship training program to determine postfellowship career pathways, determinants of those choices, and to specifically identify factors that may have influenced fellows' choices to pursue (or not to pursue) careers in nonmalignant hematology.…”

Docentes médicos e internos do curso de medicina estão preparados para uma boa prática médica no atendimento aos portadores de doença falciforme?

“…As manifestações são diversas e podem acometer diferentes sistemas. No gastrointestinal e abdominal pode haver crises de dor, cálculos biliares, icterícia obstrutiva, hepatopatia; no sistema nervoso central, acidente isquêmico transitório, infarto, hemorragia cerebral; no cardiopulmonar, cardiomegalia, insuficiência cardíaca, infarto pulmonar, pneumonia; no urogenital, priapismo, hipostenúria, insuficiência renal crônica; na superfície cutânea-mucosa, pode apresentar palidez, icterícia e úlceras; no osteoarticular, dores, necrose asséptica da cabeça do fêmur, gnatopatia; nos olhos, retinopatia proliferativa, glaucoma; sistema hematopoiético, temos anemia, asplenia, esplenomegalia crônica e nas manifestações gerais, retardo da maturação sexual e maior suscetibilidade a infecções 2,3,4,10 . A síndrome torácica aguda é um tipo particularmente perigoso de crise vaso-oclusiva envolvendo os pulmões, que tipicamente se apresenta com febre, tosse, dor torácica e infiltrados pulmonares.…”

Enhancing the care of patients with sickle cell disease

“…Accurate recording of previously prescribed doses of these analgesics could assist in informing subsequent hospital admissions, provide continuity of care and ensure that pain relief is individualised and appropriate. However, it remains necessary to monitor patients for opioid misuse or addiction (Lanzkron and Haywood 2015), and to review the effectiveness of any pain relief interventions used (Kotila et al 2015).…”