key: cord-0705673-lrgmj36v authors: Heilbronner, C; Berteloot, L; Tremolieres, P; Dupic, L; De Saint Blanquat, L; Lesage, F; Odièvre, M H; de Marcellus, C; Fourgeaud, J; de Montalembert, M; Grimaud, M; Moulin, F; Renolleau, S; Allali, S; Oualha, M title: Patients with Sickle cell disease and suspected COVID‐19 in a pediatric ICU date: 2020-05-18 journal: Br J Haematol DOI: 10.1111/bjh.16802 sha: 4d47eb30a8862fa193cfa9c0c22caa764e911220 doc_id: 705673 cord_uid: lrgmj36v Concern has been raised on sickle cell disease (SCD) patients and the new viral infection with SARS‐COV‐2 since SCD patients are particularly prone to infectious diseases and acute chest syndrome (ACS). Although case reports have been published to describe COVID‐19 related ACS in adult patients (1–4), pediatric data are lacking. We conducted a single center retrospective observational study between March 1, and April 15, 2020 in the PICU of Necker Hospital for Sick Children in Paris (tertiary care, SCD reference center, regional reference center for emerging infectious diseases). All SCD patients with suspected COVID-19 admitted in the PICU were eligible for the study. Confirmed COVID-19 was defined as a positive SARS-COV2 RT-PCR on nasal swab. ACS was defined accordingly to Vichinsky's criteria (5) Our local protocol for ACS was as below -intravenous fluid -prophylactic enoxaparin for all -antibiotics: cefotaxim and azithromycin. Antiviral therapy's left to physicians' assessment -analgesics (excepted non -steroidal anti-inflammatory drugs) -hydroxyurea or deferasirox continued except in case of drug toxicity. -early NIV for respiratory distress, oxygen for hypoxemic patients to obtain a SpO2 > 95%. This article is protected by copyright. All rights reserved -red blood cell (RBC) transfusion or automated exchange transfusion according to physicians evaluation (6) . Automated erythrapheresis were performed with a Spectra Optia® machine, on a central venous catheter. CT-scans were not mandatory. Nasal swab was collected in the first 12 hours of the patients' arrival and SARS-COV2 RT-PCR was performed. The local ethical board (Necker Enfants-Malades) waived the need for approval. All patients were informed about the study. From March 1 to April 15, 2020, 12 SCD children were included, aged 5 to 17.5 years old. RT-PCR for SARS-COV2 could be performed on 11/12 (91%) and was positive in 4/12 (33%) cases. All 4 patients were SS patients, with no G6PD deficiency. Patients 2 and 4 were on hydroxyurea (HU), Patient 2 had also been on a transfusion program for 3 years, before he was switched to HU and received desferasirox. Patient 2 had also undergone splenectomy and had experienced previous ACS episodes. Baseline hemoglobin level and HbF level when available are displayed in table I. Patients presented COVID-19 symptoms from 2 to 12days before hospital admission (fig 1) . All four patients experienced chest pain, patient 4 also experienced shoulder and back pain, all requiring intravenous morphine. The maximum daily dose of morphine received ranged from 0.6 to 1.5mg/kg/d. All four patients presented with ACS. Oxygen requirement before non-invasive ventilation (NIV) was between 1 and 6 L/min, with respiratory rate from 32 to 50/min. Maximum venous PCO2 was 50mmHg for all four patients (only one patient had an arterial blood gas). All This article is protected by copyright. All rights reserved patients received early NIV on arrival in PICU, with worst FiO2 from 30 to 46%, PEP between 5 and 7 cmH2O, and inspiratory pressure between 10 to 15 cmH2O. NIV was administered continuously at first and then sequentially. Patients received from 58 to 128 hours of NIV during their PICU stay. All patients had favorable respiratory outcome with no apparent respiratory distress remaining after PICU. This article is protected by copyright. All rights reserved Automated RBC exchanges have been realized in all 4 proven Covid-19 patients, as early as possible after PICU admission for each of the 4 patients, well tolerated. No patient developed any other organ dysfunctions. Due to expanding knowledge on different forms of COVID-19, it is now common practice to perform CT-scans for adults to diagnose patients with no viral excretion. It is not certain if CT-scans are as reliable for pediatric patients and for SCD patients, because of a probable overlap of some radiological findings with sickle cell images (7-9). As to thrombotic risk, ACS in adult patients had already been associated with high risk of thrombosis in pulmonary arteries but pulmonary thrombosis is also a major concern in COVID-19, both conditions combined might generate an even higher risk for patients. In our usual protocol for ACS, we usually limit RBC transfusion or exchange to most severe patients with NIV failure or with other sickle cell condition requiring exchange transfusion like stroke (6) . In COVID19 patients we chose aggressive treatments because of the high lethality of COVID-19 related ARDS, yet it is possible that our patients would have also had a favorable outcome without this aggressive treatment. Importantly, specific attention must be taken in care-givers' protection while performing long procedures like RBC exchange for COVID-19 patients. This is the first case series of ACS related to COVID-19 in children. All COVID-19 patients with ACS received erythrapheresis for their ACS with NIV and usual supportive treatment. This article is protected by copyright. All rights reserved One patient received tociluzimab. All patients had favorable outcomes. Screening for pulmonary thrombosis might be useful. Future studies are mandatory to determine the best therapeutic options for these patients. COVID-19 Infection in Patients with Sickle Cell Disease Rapid and Severe Covid-19 Pneumonia with Severe Acute Chest Syndrome in a Sickle Cell Patient Successfully Treated with Tocilizumab Vaso-occlusive crisis and acute chest syndrome in sickle cell disease due to 2019 novel coronavirus disease (COVID-19) COVID-19 pneumonia as a cause of acute chest syndrome in an adult sickle cell patient Acute chest syndrome in sickle cell disease: clinical presentation and course. Cooperative Study of Sickle Cell Disease Early Noninvasive Ventilation and Nonroutine Transfusion for Acute Chest Syndrome in Sickle Cell Disease in Children: A Descriptive Study Radiological Society of North America Expert Consensus Statement on Reporting Chest CT Findings Related to COVID-19. Endorsed by the Society of Thoracic Radiology, the American College of Radiology, and RSNA. Radiol Cardiothorac Imaging English editing the article This article is protected by copyright. All This article is protected by copyright. All rights reserved This article is protected by copyright. All rights reserved