Sudden Death in Sickle Cell Disease: An Assessment of Risk Factors

• Published in: Blood 2018-11, Vol.132 (Supplement 1), p.4929-4929
• Main Authors: Nze, Chijioke, Fortin, Brooke M, Freedman, Revital, Puligandla, Maneka, Neuberg, Donna S, Mandell, Elyse, Achebe, Maureen
• Format: Article
• Language: English
• ISSN: 0006-4971; 1528-0020
• DOI: 10.1182/blood-2018-99-119860

Background Sickle cell disease (SCD) is the most common severe hereditary blood disorder in the United States. A single nucleotide mutation leads to a modified β-chain of hemoglobin (β6Glu-Val). The pathology of SCD includes debilitating pain crises, progressive organ damage, and finally premature death. A significant portion of premature death in adults with SCD is attributable to cardiopulmonary disease with a disproportionate amount of sudden death. Sudden death has been associated with as much as 23.4% of mortality in adults with SCD (Darbari et al. Am J Haematology, 2006). In this study we aimed to identify risk factors for sudden death. As in previous studies, sudden death was defined as an unexpected death occurring in a relatively healthy SCD patient who dies at home or within 24 hours of hospitalization with or without a vaso-occlusive crisis. Identification of risk factors for sudden death will aid in developing interventions targeted at specific SCD patients. Methods We conducted a retrospective study of SCD-related deaths using the Partners Research Patient Data Registry (RPDR), a centralized clinical data registry that gathers clinical information from various hospitals within the Partners hospital systems and patients' electronic health records. The study was approved by our institution's IRB. The RPDR was queried to identify all patients associated with SCD. Only patients with a vital status of ‘deceased’ were used for the patient population. Patients who died before 1998 were excluded. Sixty-one patients were included and categorized by ‘cause of death’ into 5 groups. The patients' charts were reviewed to identify cause of death and SCD-related data between two years and up to 2 weeks prior to death. The patient variables examined covered demographics, sickle genotype, hydroxyurea exposure, blood pressure, EKG abnormalities, medical histories of acute chest syndrome (ACS), stroke, pulmonary embolism, leg ulcers, priapism, and retinopathy as well as laboratory data. The data were analyzed in 2 categories; ‘sudden death’ and ‘other causes of death’. Results Of the 61 deceased patients, 33 (54%) were women and the average age at death was 39 years. Nineteen patients (31%) suffered sudden deaths: 7 (11%) sudden deaths at home (group I) and 12 (20%) deaths within 24 hours of hospitalization (group IV). Ten (16%) died of known causes related to SCD (group II), 22 (36%) died of known causes unrelated to SCD (group III) and 10 (16%) died of unknown