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Sickle Cell Anemia/Hemoglobin SS Disease
Published in Charles Theisler, Adjuvant Medical Care, 2023
Early signs and symptoms of sickle cell disease include swelling of the hands and feet; symptoms of anemia, including fatigue, or extreme tiredness; and jaundice. Over time, sickle cell disease can lead to complications such as infections, delayed growth, and episodes of pain, called pain crises.1 Neurologic complications are also common in sickle cell disease and include ischemic and hemorrhagic stroke, seizures, CNS infection, hearing loss, cognitive impairment, and, rarely, spinal cord infarction.2 SCD can cause lifelong disabilities and reduces average life expectancy to about 40 years in the U.S.
Sickle Cell Disease
Published in Vincenzo Berghella, Maternal-Fetal Evidence Based Guidelines, 2022
Sickle cell disease is an autosomal recessive disease resulting from an alteration in the structure of hemoglobin producing hemoglobin S (HbS). It is characterized by chronic hemolytic anemia and vaso-occlusive events.
Sickle cell disease
Published in Hung N. Winn, Frank A. Chervenak, Roberto Romero, Clinical Maternal-Fetal Medicine Online, 2021
Marc R. Parrish, John C. Morrison
Despite advances during the past 20 years and the appreciation of the molecular basis of sickle cell crisis, there is no specific therapy available. However, an understanding of the pathophysiology involved in vaso-occlusive crisis allows the provider to address those aspects of this event that can be treated. Such therapeutic regimens generally include (i) adding fluids to reduce intracellular hemoglobin concentration, (ii) correcting acidosis when present, (iii) treating infection, and (iv) increasing oxygen concentration.
Emergency department visits and hospitalizations among patients with sickle cell disease in illinois, 2016–2020
Published in Pediatric Hematology and Oncology, 2023
Christina M. Barriteau, Joe Feinglass, Mariam Kayle, Paige VonAchen, Robert I. Liem, Sherif M. Badawy, Kristin Kan
Sickle cell disease (SCD) affects 100,000 individuals in the USA, predominantly African Americans.1 SCD is associated with high acute healthcare utilization and an estimated annual cost of $2 billion.2 Despite high healthcare utilization and expenditures, gaps in SCD research funding and surveillance limit informed program development and resource allocation at the state level.3 Current SCD surveillance programs by the Centers for Disease Control and Prevention (CDC) were expanded to eleven states in 2021, which does not include Illinois (IL).4,5 The most recent published data on SCD healthcare utilization in IL demonstrated 8,400 admissions and $59 million in hospital charges from 1992 to 1993; however, data on emergency department (ED) utilization were limited.6 There is a need for further understanding of SCD acute healthcare utilization in IL to inform state-level programming and resource allocation for SCD in this state.
Attitudes and practices of unmarried adults towards sickle cell disease: emergent factors from a cross sectional study in Nigeria’s capital
Published in Hematology, 2022
Obi Peter Adigwe, Godspower Onavbavba, Solomon Oloche Onoja
Globally, the prevalence of the disease is highest in sub-Saharan Africa, with Nigeria saddled with the highest burden of carrier prevalence ranging from 25% to 30% [5,6]. Furthermore, in the country, sickle cell anaemia occurs in approximately 3% of all births [7–9]. Some of the common characteristics of sickle cell disease include chronic haemolytic anaemia and recurrent vaso-occlusion, with the latter being responsible for painful crises associated with the disease. Another common feature of the condition is chronic vasculopathy triggered by free heme resulting in nitric oxide scavenging and upregulation of adhesion molecules in reticulocytes neutrophils and endothelial cells [10,11]. One of the major causes of mortality among children is overwhelming bacterial infections occurring due to encapsulated organisms, particularly pneumococcus [1213]. Other common causes of death include splenic sequestration [14], acute chest syndrome [15], stroke [16,17] and multiple organ failure [18]. There is therefore substantial evidence that the disease constitutes a high mortality risk for under-five sickle cell disease patients who live in areas without adequate access to healthcare [19]. This is further complicated by the fact that empirical evidence now correlates malaria episodes with increased risk of crises, alongside the possible morbidity and mortality associated with these events [20,21].
Audit of clinical and laboratory parameters of hemoglobin SS patients in a Nigerian teaching hospital
Published in Annals of Medicine, 2022
Titilola Stella Akingbola, Oladapo Wale Aworanti, Sunday Peter Ogundeji
As the survival improves, sickle cell disease patients are predisposed to different complications of the disease such as chronic leg ulcer, priapism, chronic osteomyelitis, acute chest syndrome, stroke, pentazocine addiction. In these participants, the commonest complication reported was chronic leg ulcer, this is seen in about 12% of the total participants. The cause of leg ulcer is the vaso-occlusion of skin microvasculature, worsened by trauma, infection, warm climate, and iron overload. This is consistent with the findings of Bazuaye et al. where the incidence of chronic leg ulcer was about 10% [26]. Next to the chronic leg ulcer is priapism, seen in about a quarter of male respondents. Previous findings showed prevalence of priapism to be about 45% in SCD male patients [27], our finding in this study is relatively lower compared to the findings by Nwogoh et al. [28]. The least complications seen were pentazocine addiction, sickle cell nephropathy and infertility.