Silent Multiorgan Injury in Sickle Cell Disease: Redefining Mortality Beyond Pain Crises
Daniel Obinna Eke
Department of Nursing, Myrtle E. and Earl E. Walker College of Health Professions, Maryville University of St. Louis, St. Louis, Missouri USA.
Obiageri Ihuarulam Okeoma
Department of Medical Laboratory Science, Trinity University Yaba, Lagos, Nigeria.
Leo Tata
College of Health and Human Sciences, Iowa State University, Ames, Lowa, USA.
Salamah Abimbola Junaid
Emergency Medicine Department, Northern General Hospital, Sheffield, England.
Williams Temidayo Solomon
Department of Medicine, Howard University College of Medicine, Washington DC, USA.
Esther Uyoyooghene Olokede *
Department of Medical Laboratory Science, Faculty of Basic Medical Sciences, University of Benin, Benin City, Edo State, Nigeria.
*Author to whom correspondence should be addressed.
Abstract
Sickle cell disease (SCD) has typically been seen through the prism of acute vaso-occlusive crises and other clinically noticeable issues that call for medical attention. The mortality rate among people with sickle cell disease (SCD) is still much higher than that of the general population, despite improvements in survival and a decrease in the frequency of severe acute episodes brought about by advancements in disease-modifying treatments and supportive care. A significant amount of this increased mortality may be caused by silent, progressive multiorgan harm that occurs long before clinical symptoms manifest, according to emerging data. The objective of this review was to evaluate the contribution of silent multiorgan injury to sickle cell disease morbidity and mortality, as well as its underlying pathophysiological mechanisms, patterns of organ-specific damage, diagnostic limitations, and potential for early detection and prevention. A narrative review of the literature was conducted using published studies addressing the mechanisms, manifestations, detection, and management of subclinical organ injury in SCD. The review found that the main causes of silent tissue damage were endothelial dysfunction, oxidative stress, persistent haemolysis, chronic inflammation, hypercoagulability, and repeated microvascular occlusion. The brain, kidneys, liver, spleen, cardiovascular system, and skeletal system are all gradually affected by these processes, frequently years before obvious clinical symptoms show up. Avascular necrosis, higher tricuspid regurgitant velocity, diastolic dysfunction, silent cerebral infarcts, microalbuminuria, and functional asplenia were found to be significant markers of subclinical disease development and unfavourable long-term outcomes. Current therapies have a limited ability to prevent the increasing organ deterioration, and current clinical techniques are still primarily focused on acute problems and often miss early organ damage. The narrative structure of this review and the lack of quantitative synthesis are its main limitations. All things considered, a significant yet underappreciated factor in sickle cell disease morbidity and early death is silent multiorgan damage. To enhance long-term results and address the hidden burden of disease, there must be a greater focus on proactive surveillance, early detection, and organ-protective therapies.
Keywords: Sickle cell disease, silent organ injury, multiorgan dysfunction, endothelial dysfunction, microvascular occlusion, silent cerebral infarcts, sickle nephropathy, mortality