Protecting vulnerable patients with inherited anaemias from unnecessary death during the COVID‐19 pandemic

Abstract

Summary With the developing COVID‐19 pandemic, patients with inherited anaemias require specific advice regarding isolation and changes to usual treatment schedules. The National Haemoglobinopathy Panel (NHP) has issued guidance on the care of patients with sickle cell disease, thalassaemia, Diamond Blackfan anaemia (DBA), congenital dyserythropoietic anaemia (CDA), sideroblastic anaemia, pyruvate kinase deficiency and other red cell enzyme and membrane disorders. Cascading of accurate information for clinicians and patients is paramount to preventing adverse outcomes, such as patients who are at increased risk of fulminant bacterial infection due to their condition or its treatment erroneously self‐isolating if their fever is mistakenly attributed to a viral cause, delaying potentially life‐saving antibiotic therapy. Outpatient visits should be minimised for most patients, however some, such as first transcranial dopplers for children with sickle cell anaemia should not be delayed as known risk of stroke will outweigh the unknown risk from COVID‐19 infection. Blood transfusion programmes should be continued, but specific changes to usual clinical pathways can be instituted to reduce risk of patient exposure to COVID‐19, as well as contingency planning for possible reductions in blood available for transfusions. Bone marrow transplants for these disorders should be postponed until further notice. With the current lack of evidence on the risk and complications of COVID‐19 infection in these patients, national data collection is ongoing to record outcomes and eventually to identify predictors of disease severity, particularly important if further waves of infection travel through the population.

Care of patients with hemoglobin disorders during the COVID‐19 pandemic: An overview of recommendations

“…However, it is advisable to avoid the routine use or increasing doses of hydroxyurea to reduce the need for repeated phlebotomy and hospital visits. 2,6 In areas where severe blood shortages are expected due to the pandemic, a low dose of hydroxyurea is recommended in all pediatric patients with sickle cell anemia, who receive regular blood transfusion therapy for primary or secondary stroke prevention. 7 In the absence of regular blood transfusion therapy, hydroxyurea treatment will also decrease the incidence rates of acute vaso-occlusive pain and ACS events.…”

“…However, should the patient become symptomatic, it is advisable to interrupt iron chelation therapy. 6,13 Unless conditioning has already been initiated, stem-cell transplants and gene therapy should be deferred for these patients until the pandemic resolves. 13 Should a patient be on the novel erythropoiesis maturation agent luspatercept, therapy should be maintained even if the patient is diagnosed with COVID-19, as there is no evidence that it should be discontinued in this case scenario.…”

Impact of COVID-19 pandemic in the activity of a Therapeutic Apheresis unit in Italy

“…Frequent hospital access, needed for cross-matching and RBCEx procedures, have been discouraged during the outbreak and regular treatments have been resumed at the end of the lockdown. This policy has been in line with the UK National Haemoglobinopathy Panel recent report [16].…”

A review on how to do hematology consults during COVID-19 pandemic

“…Unlike SCD, patients with thalassemia have comparatively a lower risk of lung infections but, an overall compromised cardiopulmonary system, diabetes, and hemochromatosis secondary to severe iron overload increase the vulnerability to complications of the virus. Another concern of hypoadrenalism in thalassemia secondary to iron overload may merit using rescue steroids as a stress dose [ 115 ]. Concerns over chelation related side effects also merit discussion on case to case basis.…”