COVID-19 in individuals with sickle cell disease: an integrative review of what we know so far

What is it about?

Sickle cell disease is one of the most common genetic conditions in the world and is known to be associated with an increased risk of infections and coagulation disorders. In light of this, scientists and health professionals grew increasingly concerned about this population when the SARS-CoV-2 pandemic began at the end of 2019. Many questions have emerged about the impact of COVID-19 in the context of sickle cell disease, but there was scant systematized information about the clinical course and outcomes of this new infection in these patients specifically. Thus, our review aimed to map and organize the scientific production on the subject to provide a detailed and extensive overview of the evolution pattern of COVID-19 in combination with sickle cell disease. The searches were carried out in three databases—Medline, PubMed, and Virtual Health Library—according to descriptors extracted from Medical Subject Headings and considered studies published between 2020 and October 2022, resulting in the inclusion of 90 articles organized into six categories. The analysis of these publications revealed that there is no consensus on how different aspects related directly or indirectly to sickle cell disease, such as the state of chronic inflammation, hemolytic anemia, hypercoagulability, the use of medications like hydroxyurea, and access to medical care, can interfere with the clinical course of COVID-19. Additional research must be conducted in order to obtain a broader understanding of the pathophysiology of the infection in this scenario. However, we can affirm that SARS-CoV-2 infection can manifest in an atypical way in these individuals, as well as acting as a trigger for the development of common and potentially serious complications of sickle cell disease, such as acute chest syndrome and vaso-occlusive crises. Hence, we recommend healthcare professionals be trained to recognize the different ways in which COVID-19 may present among these patients.

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Photo by Elena Mozhvilo on Unsplash

Photo by Elena Mozhvilo on Unsplash

Why is it important?

Sickle cell disease is highly prevalent around the world. At least 300,000 children are born with the condition every year. Thus, understanding the particularities of the clinical course of COVID-19 in individuals with sickle cell disease is crucial to providing proper medical care for these patients in the face of this complex infectious context. Ultimately, the relevance of our review lies in offering subsidies to assist institutions and health authorities in formulating therapeutic protocols and public policies directed at this population.

Perspectives