COVID‐19 infection and sickle cell disease: a UK centre experience

Abstract

During the COVID-19 pandemic, patients with sickle cell disease (SCD) have been included in the ‘high-risk’ category of the population. This is due to their impaired immunity resulting from functional hyposplenism, systemic vasculopathy, which predisposes them to end organ dysfunction, and a high risk of thrombosis.1 We report here the clinicopathological features, management and outcomes of the first 10 patients (eight men and two women) with SCD and COVID-19 infection treated in our institution from 10th March to 23rd April 2020. This includes six patients (Patients 1–6 on Table I and Table II) with COVID-19 confirmed by reverse transcription polymerase chain reaction (RT-PCR) of nasopharyngeal swabs, and four patients (7–10) with very suggestive clinical and radiological/laboratory features, but a negative swab. These patients have been included as it is recognised that the currently utilised RT-PCR technique has a low sensitivity of around 70%.2 In fact, the first patient we treated tested negative, despite having typical clinical and radiological features. COVID-19 was only confirmed after he was tested for a second time. Nine patients had HbSS or HbSβ0 and one had Hb SC disease. The mean age was 36 years (range, 23–57). One patient had several pre-existing co-morbidities, including severe neurological impairment as a result of a previous stroke, and was bedbound and living in a nursing home. One patient had severe sickle cell nephropathy and resulting stage III chronic kidney disease (CKD). For the remaining eight, there was no evidence of end organ damage, but six suffered with recurrent vaso-occlusive crises (VOC). Five patients were on a regular transfusion programme, with two having evidence of moderate/severe iron overload. At presentation, eight patients had varying degrees of pain, indicating that the infection had triggered a VOC. Other presenting features included fever, dry cough and hypoxaemia. We noted variability in the combination of clinical symptoms between patients at presentation. Equally, for individual patients, presenting symptoms may settle during admission and symptoms not present initially developed during the course of the illness. Details of clinical features at presentation and during admission are summarised in Table I. An interesting observation was the lack of a subjective sense of breathlessness in two of the four patients with significant hypoxaemia (Patients 3 and 4). There was also great variability between patients regarding different haematological and biochemical parameters (summarised in Table II). Of note, no patient developed coagulopathy or thrombocytopenia. All patients received oxygen therapy as required to maintain SaO2 ≥ 95%, analgesia for VOC, broad spectrum antibiotics, as per local policy, and thromboprophylaxis. The latter was standard dose (enoxaparin 40 mg OD) for the first eight patients we treated, but was subsequently increased to 1·5 mg/kg OD given the prothrombotic state of SCD and the emerging evidence of the high thrombotic risk of COVID-19.3 Three patients whose haemoglobin had dropped from baseline received simple transfusions for optimisation. One patient (Patient 2) died: the 57-year-old patient with previous stroke and multiple co-morbidities presented extremely unwell and a decision was made to offer only palliation. A patient with stage III CKD (Patient 3) developed significant deterioration of his renal function and required temporary peritoneal dialysis, but otherwise made a full recovery. The remaining patients also made a complete recovery. The mean length of stay was 7·2 days (range, 3–17). No patient required admission to the intensive care unit, mechanical ventilation nor non-invasive ventilation. There was no case of thrombosis or bleeding, superimposed bacterial infection or macrophage activation syndrome. Overall, the outcome of COVID-19 infection was favourable for this cohort of SCD patients, unless there were significant pre-existing comorbidities. We strongly advocate social distancing, as per local public health guidance, and enhanced thromboprophylaxis for hospitalised patients. The authors declared no conflicts of interest. KM designed the project and wrote the paper. JM and RH collected data and co-authored the paper. DAT co-designed the project and critically reviewed the manuscript. All authors read and approved the final manuscript.

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