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Pediatr Pulmonol ; 57(4): 1096-1099, 2022 04.
Article in English | MEDLINE | ID: covidwho-1650861


Children with sickle cell disease (SCD) are at increased risk for severe illness due to severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). We describe the successful native lung recovery of a child with SCD referred for lung transplant (LTx) evaluation who was on prolonged veno-venous extracorporeal membrane oxygenation (VV-ECMO). He initially presented with acute chest syndrome complicated by SARS-CoV-2 infection that ultimately required dual-lumen, single bicaval VV-ECMO cannulation for respiratory support. Despite the increased risk of hemolysis and thrombosis from SCD and SARS-CoV-2 infection, he was successfully supported on VV-ECMO for 71 days without complications leading to native lung recovery with meticulous management of his SCD therapy. This report provides new insight on our approach to VV-ECMO support in a child with SCD and SARS-CoV-2 infection. With a successful outcome, the patient has returned home but still on mechanical ventilation with LTx still an option if he is not eventually liberated from invasive respiratory support.

Acute Chest Syndrome , Anemia, Sickle Cell , COVID-19 , Extracorporeal Membrane Oxygenation , Acute Chest Syndrome/complications , Acute Chest Syndrome/therapy , Anemia, Sickle Cell/complications , Anemia, Sickle Cell/therapy , COVID-19/complications , COVID-19/therapy , Child , Extracorporeal Membrane Oxygenation/adverse effects , Humans , Male , SARS-CoV-2
Blood Adv ; 5(1): 207-215, 2021 01 12.
Article in English | MEDLINE | ID: covidwho-1035171


We aimed to identify predictors of outcomes and survival in patients living in 4 major metropolitan areas who had sickle cell disease (SCD) and COVID-19 to inform best approaches to prevention and care. Data were collected at baseline and during the clinical course in SCD patients diagnosed with COVID-19 in four COVID-19 epicenters. Patients were followed up posthospital discharge for up to 3 months. Of sixty-six SCD patients with COVID-19, fifty patients (75%) required hospitalization, and seven died (10.6%). Patients with preexisting kidney disease (chronic kidney disease) were more likely to be hospitalized. The most common presenting symptom was vaso-occlusive pain. Acute chest syndrome occurred in 30 (60%) of the 50 hospitalized patients and in all who died. Older age and histories of pulmonary hypertension, congestive heart failure, chronic kidney disease, and stroke were more prevalent in patients who died, as were higher creatinine, lactate dehydrogenase, and D-dimer levels. Anticoagulation use while inpatient was twice less common in patients who died. All deaths occurred in individuals not taking hydroxyurea or any other SCD-modifying therapy. Patients with SCD and COVID-19 exhibited a broad range of disease severity. We cannot definitively state that the overall mortality is higher in patients with SCD, although our case fatality rate was ∼10% compared with ∼3% in the general population, despite a median age of 34 years. Individuals with SCD aged >50 years, with preexisting cardiopulmonary, renal disease, and/or stroke not receiving hydroxyurea, who present with high serum creatinine, lactate dehydrogenase, and D-dimer levels, are at higher risk of death, irrespective of genotype or sex.

Anemia, Sickle Cell/complications , COVID-19/complications , Acute Chest Syndrome/blood , Acute Chest Syndrome/complications , Acute Chest Syndrome/mortality , Acute Chest Syndrome/therapy , Adult , Anemia, Sickle Cell/blood , Anemia, Sickle Cell/mortality , Anemia, Sickle Cell/therapy , Antisickling Agents/therapeutic use , COVID-19/blood , COVID-19/mortality , COVID-19/therapy , Disease Progression , Female , Fibrin Fibrinogen Degradation Products/analysis , Humans , Hydroxyurea/therapeutic use , Male , Risk Factors , SARS-CoV-2/isolation & purification , Young Adult
Pediatr Blood Cancer ; 67(11): e28579, 2020 11.
Article in English | MEDLINE | ID: covidwho-746153


New York City has emerged as one of the epicenters of the SARS-COV-2 pandemic, with the Bronx being disproportionately affected. This novel coronavirus has caused significant respiratory manifestations raising the concern for development of acute chest syndrome (ACS) in patients with sickle cell disease (SCD). We report a series of pediatric SCD SARS-COV-2-positive patients admitted with ACS. SARS-COV-2-positive SCD patients, who did not develop ACS, were the comparison group. Hydroxyurea use (P-value = .02) and lower absolute monocyte counts (P-value = .04) were noted in patients who did not develop ACS. These preliminary findings need to be further evaluated in larger cohorts.

Acute Chest Syndrome/complications , Anemia, Sickle Cell/complications , COVID-19/complications , Acute Chest Syndrome/diagnosis , Acute Chest Syndrome/drug therapy , Adolescent , Anemia, Sickle Cell/drug therapy , Anti-Bacterial Agents/therapeutic use , Antisickling Agents/therapeutic use , COVID-19/diagnosis , COVID-19/drug therapy , COVID-19 Testing , Child , Doxycycline/therapeutic use , Female , Hospitals, Urban , Humans , Hydroxyurea/therapeutic use , Male , New York City , Polymerase Chain Reaction , SARS-CoV-2 , Tomography, X-Ray Computed , Treatment Outcome , Young Adult