Effects of corticosteroids in patients with sickle cell disease and acute complications: a systematic review and meta-analysis

Whether corticosteroids improve outcome in patients with acute complications of sickle cell disease (SCD) is still debated. We performed a systematic review of the literature with the aim of estimating effects of corticosteroids on the clinical course of vaso-occlusive crisis (VOC) or acute chest syndrome (ACS) in patients with SCD. The primary outcome was transfusion requirement during hospitalization. Studies were identified by search of MEDLINE and CENTRAL database. Three randomized clinical trials (RCT) and three retrospective cohort studies (RCS) were included, involving 3,304 participants and 5,562 VOC or ACS episodes. There was no difference between corticosteroids and standard treatment regarding transfusion overall [OR=0.98 (95% CI 0.38 to 2.53)], but with a significant interaction of study type (P

The Impact of Sickle Cell Disease on Academic Performance among Affected Students

Background: Sickle cell disease (SCD) is a genetic disease that is highly prevalent in Jazan Province, Saudi Arabia, and is mostly characterized by many complications such as vaso-occlusive crises (VOC), acute chest syndrome (ACS) and well-documented neurological complications. These complications may affect patients’ academic performance. Methods: An observational, cross-sectional, retrospective study was conducted in Jazan Province. General and demographic data were collected and questions about academic performance of students with SCD were answered. Both t-tests and chi-square tests, along with multiple logistic regression, were used for analysis. Results: 982 participants were selected for this study with a mean age of 23 years (SD: 7). Most of the participants were female (64%). The number of participants with SCD was 339 (36%), of whom 42% were male. Students with SCD recorded lower grade point averages (GPA) and more absences compared to healthy participants. Further, about 60% of students with SCD thought they performed better than 40% of the participants without SCD during the COVID-19 pandemic when most of the educational activities were online. Conclusion: As has been previously reported, this study suggested that the academic performance of students with SCD is negatively affected compared to healthy individuals, and this is mostly due to complications associated with the disease. Further, students with SCD acknowledged better performance with online education, an option that should be considered to improve their academic performance. National studies on a larger population are required by health and education officials, and supportive online educational programs are warranted to enhance the academic performance of this population.

Crisis in Sickle Cell Disease: Review Article

Sickle cell disease is a very common inherited disorder of the hemoglobin. It is inherited in an autosomal recessive manner. Most affected are the people of African, Indian and Arabian origin. It occurs due to change in the single base pair gene wherein thymine replaces adenine in the 6th codon of the beta-globin gene. This result in the sickling shape of the red blood cells. Sickle cell disease includes a variety of phenotypes like the SS, AS, Sickle-thal, SC patterns, etc. Sickle cell- SS pattern also termed as sickle cell anemia is the most common of form of the disorder and is also responsible for the morbidity and mortality caused by the disorder. The sickling pattern of the red blood cells occludes the blood vessels and leads to a wide range of complication in the affected individuals. These complications can be seen in number of different systems of the body and also multiple systems at the same time. These complications are termed as crisis, which then include the vaso-occlusive crisis, acute chest syndrome, splenic sequestration crisis, etc. These crises can negatively affect the quality of life to a large effect, but are also largely controllable or rather delayed and effectively managed as far as possible with reduced effect in the normal well being. Hence the knowledge about these crisis and their treatment is an important aspect of medical practice, especially in the countries where this disorder is commonly seen. Here in this review article we aim to highlight the major crises seen in sickle cell disease and their treatment in brief.

Indications for transfusion in the management of sickle cell disease

The transfusion of red blood cells (RBCs) is a crucial treatment for sickle cell disease (SCD). While often beneficial, the frequent use of transfusions is associated with numerous complications. Transfusions should be offered with specific guidelines in mind. Here we present updates to the indications for transfusion of RBCs in SCD. We review recent publications and include expert perspectives from hematology and transfusion medicine. For some clinical indications, such as ischemic stroke, the role of transfusion has been well studied and can be applied almost universally. For many other clinical scenarios, the use of transfusion therapy has less conclusive data and therefore must be tailored to individual needs. We highlight the roles of RBC transfusions in preventing or mitigating neurological disease, in reducing perioperative complications, in managing acute chest syndrome, and in optimizing pregnancy outcomes in SCD. We further highlight various transfusion techniques and when each might be considered. Potential complications of transfusion are also briefly discussed.

Treatment dilemmas: strategies for priapism, chronic leg ulcer disease, and pulmonary hypertension in sickle cell disease

Sickle cell disease is a disorder characterized by chronic hemolytic anemia and multiorgan disease complications. Although vaso-occlusive episodes, acute chest syndrome, and neurovascular disease frequently result in complication and have well-documented guidelines for management, the management of chronic hemolytic and vascular-related complications, such as priapism, leg ulcers, and pulmonary hypertension, is not as well recognized despite their increasing reported prevalence and association with morbidity and mortality. This chapter therefore reviews the current updates on diagnosis and management of priapism, leg ulcers, and pulmonary hypertension.

Optimizing management of sickle cell disease in patients undergoing surgery

Individuals with sickle cell disease (SCD) are likely to be referred for surgery at some point in their lifetime due to a high incidence of musculoskeletal and intrabdominal complications such as avascular necrosis and gallbladder disease. Preoperative optimization is a multidisciplinary process that involves a hematologist with SCD expertise, an anesthesiologist, and the surgical team. The type and risk classification of the surgery, disease severity, medications, baseline hemoglobin, transfusion history, and history of prior surgical complications are often documented. Clinicians should consider perioperative risk assessment that includes determining the patient's functional status and cardiovascular risk and screening for obstructive sleep apnea. Many patients will require preoperative transfusion to reduce the risk of postoperative complications such as acute chest syndrome and vaso-occlusive pain crises. The hematologist should consider the patient's preoperative transfusion requirements and ensure that the surgical team has an appropriate plan for postoperative observation and management. This often includes follow-up laboratory studies, a postoperative pain management plan, and venous thromboembolism prophylaxis. The transfusion plan should be patient-specific and take into account the SCD genotype, baseline hemoglobin, disease severity, risk classification of the surgery, and history of prior surgical complications. In the intraoperative and postoperative period, dehydration, hypothermia, hypotension, hypoxia, and acidosis should be avoided, and incentive spirometry should be utilized to minimize complications such as acute chest syndrome. In this review we discuss preoperative, intraoperative, and postoperative strategies to optimize patients with SCD undergoing surgery.