Management of sickle cell disease patients with COVID-19
As patients with sickle cell disease (SCD) are considered to be at high risk for COVID-19 infections, the Sickle Cell Disease Association of America made some recommendations and guidelines to educate both health care providers and SCD patients. During her presentation at the virtual EHA meeting, Prof. Minniti shared some patient data of the American SCD COVID-19 registry as well as guidelines for the management of SCD in the COVID-19 era.
Sickle cell disease (SCD) is an inherited disorder of haemoglobin that results in chronic anaemia, haemolysis, recurrent pain crises and progressive end organ damage. SCD patients are considered to be at high risk for severe COVID-19 infections because of their impaired immunity (resulting from hyposplenism and sterile inflammation), systemic vasculopathy that predisposes to end organ dysfunction (mainly in the brain, heart, kidney, lung, and liver) and a high risk of thrombosis. In addition, psychosocial factors that are associated with the COVID-19 pandemic can also heavily affect patient care in SCD. As such, SCD patients are often anxious to seek care in emergency departments owing to the fear of becoming infected or they receive care from an unfamiliar set of health care providers who may know little about their SCD history. As a result, patients with SCD often choose to delay or avoid care altogether, stay at home and suffer in pain.
SCD COVID-19 registry
In order to gather more information on COVID-19 infections in SCD patients, an SCD COVID-19 registry was set up and Prof. Minniti reported the results of the last update on June 5th. At that day, a total number of 210 SCD COVID-19 cases were reported. The average age of the patients was 28.95 years and by far most of them were of African American origin. Most patients (71.29%) had HgbSS disease, but patients with HgbSC (20.1%), HgbSb+ (4.31%) and HgbSb0 subtypes (4.31%) were infected too. More than half of the patients (55.71%) took hydroxyurea as SCD home medication. Cases mostly came from New York, but also from Michigan and other states with a high rate of African American citizens.
Pain (60.95%) and pneumonia (30.0%) were by far the most prevalent symptoms of COVID-19 infection and 31.9% of the patients even indicated that pain was the only symptom they experienced. Seven patients suffered from renal failure of which four required dialysis. In total, 14 patients (6.7%) died of which there was only one paediatric case (<19 years old). Interestingly, the SCD genotype does not seem to affect mortality as mortality was equally distributed between severe phenotypes (HbSS/HbSB0, 5.1% mortality rate) and milder phenotypes (HbSc/HbSB+, 9.8% mortality rate). Treatment during hospitalisation reflected the standard-of-care at the hospitals at the moment: 27.2% of the patients received heparin (mostly preventive, 82.35% of the cases), azithromycin (29.92%) and hydroxychloroquine (18.75%). In contrast to what was feared, only 37.62% of the patients required transfusion. Other frequent COVID-19 interventions included emergency room visits (84.76%), hospitalisation (67.62%), admission at the intensive care unit (10.95%) and ventilation (5.24%). The average length of hospital stay was 8.13 days.
Management of SCD in the COVID-19 era
In order to ensure adequate treatment of SCD patients during the COVID-19 pandemic, all outpatient medications (e.g. hydroxyurea, voxelotor, L-glutamine, folic acid) should be continued and tele-health visits should be encouraged. Furthermore, COVID-19 testing should be considered for all patients with vaso-occlusive crises. All patients with infiltrates on chest X-ray have acute chest syndrome (ACS) and should be treated accordingly with antibiotics, transfusion and close monitoring (especially for signs of thrombocytopenia). An early exchange transfusion is recommended for all patients that have hypoxia and deep vein thrombosis prophylaxis should be administered to all admitted patients unless there are contraindications to do so. Further evaluation for venous thromboembolism should be considered for those patients with elevated D-dimers and clinical symptoms. In addition, SCD patients with ACS can have transient elevations of pulmonary-arterial pressure, which can result in right-sided congestive heart failure so close monitoring, is necessary. Finally, as paediatric patients with SCD have an increased frequency of asthma, physicians need to consider asthma treatment in those patients with ACS.
Conclusions
As for now, our understanding of the interaction between COVID-19 and SCD remains limited. Patients with SCD and COVID-19 present across a spectrum of disease severity and severe disease is not limited to those with HbSS, but occurs equally in patients with milder subtypes of SCD. Similar to other populations, COVID-19 disease appears to be more severe in adults than in paediatric SCD patients. Unfortunately, a higher death rate in SCD patients has been observed this year. Although many of these patients were never tested for COVID-19, it seems that those patients with end-organ disease due to SCD are at the highest risk for death. Thus far, the role for experimental therapies in SCD patients remains unknown.
Reference
Minniti C. Handling patients – hematological patient point of view (SCD). Presented at EHA 2020; Oral presentation pq282-2.
