Challenges in the Management of Sickle Cell Disease During SARS-CoV-2 Pandemic

Abstract

The management of sickle cell disease (SCD) and its complications in the COVID-19 era is very challenging. The recurrent sickling process in SCD causes tissue hypoxemia and micro-infarcts, resulting in end organ damage. Since the outbreak of SARS-CoV-2 pandemic, little data has been published about SCD concerning clinical presentation with COVID-19 and management. Hydroxyurea has been the cornerstone of management in children and adults with SCD, with evidence of its effect on controlling end organ damage. There are several anti-sickling drugs that have been approved recently that might have an additive value toward the management of SCD and its complications. The role of simple and exchange transfusions is well established and should always be considered in the management of various complications. The value of convalescent plasma has been demonstrated in small case series, but large randomized controlled studies are still awaited. Immunomodulatory agents may play a role in reducing the damaging effects of cytokines storm that contributes to the morbidity and mortality in advanced cases. Prophylactic anticoagulation should be considered in every management protocol because SCD and COVID-19 are thrombogenic conditions. Management proposals of different presentations of patients with SCD and COVID-19 are outlined.

Acute chest syndrome and COVID‐19 in hydroxyurea naïve sickle cell disease patient in a low resource setting

“…However, studies describing the impact of COVID‐19 in the clinical course of sickle cell disease crises are limited. Furthermore, the mechanistic interaction and effect of the COVID‐19 and ACS are poorly known, and one can only allude to a fatal synergistic effect of the two primarily respiratory diseases [ 7 ].…”

“…While pulmonary embolism due to thrombi can be one of the causes of acute respiratory distress, the preceding features of VOC and new radiodensities on the chest X‐ray in our patient made us rule it out, even in the presence of mild elevation of D‐dimers. The laboratory parameters in reported cases of SCD patients with COVID‐19 show a wide variation [ 7 ]. Nonetheless, most cases have elevated markers of acute inflammation and haemolysis.…”

“…Even with advanced healthcare systems and the best clinical settings, the management of ACS is challenging and requires prompt intervention to halt clinical deterioration [ 4 ]. The picture is even more complicated in low‐income regions such as Sub‐Saharan Africa, where over 75% of the 400,000 infants born annually with the disease globally originate [ 5 , 6 , 7 ]. With the outbreak of the COVID‐19 pandemic, which also primarily results in acute respiratory illness, the presentation and treatment outcome of ACS in SCD patients remain unknown.…”

Clinical presentations and outcomes of COVID‐19 infection in sickle cell disease patients: Case series from Komfo Anokye teaching hospital, Ghana

“…As a result of the prothrombotic state that SCD and COVID‐19 predispose patients to, some experts currently recommend treatment of asymptomatic SCD patients with mild COVID‐19 with prophylactic doses of low molecular weight heparin (such as enoxaparin 40 mg daily) while symptomatic patients with severe or critical COVID‐19 should be treated with therapeutic doses of 1 mg/kg 12 hourly barring any contraindications 13,26 . Evidence of the thrombogenic nature of COVID‐19 had not emerged at the time these patients were treated at HIIU and therefore patient 3 received only prophylactic doses of enoxaparin.…”

Internet‐based patient survey on the consequences of COVID‐19 lockdown on treatment and medical follow‐up of patients with aplastic anemia or paroxysmal nocturnal hemoglobinuria in Germany

“…We did not assess the psychological impact of COVID-19 on the AA/PNH patients in our survey. However, considering the high reported mortality of COVID-19 in patients with malignant hematological diseases, 5,6 and paucity of information in regards to patients with non-malignant hematological diseases 7 except for patients with hemoglobinopathies 8,9 improving the flow of information might have resulted in less distress for patients and relatives. This might be of particular relevance considering the expected start of a "second wave" of the COVID-19 pandemic in Europe during the fall of 2020 and the possibility of a further "lockdown".…”