Circumstances of death in adult sickle cell disease patients

Abstract

The purpose of the study was to analyze clinical and/or autopsy findings at the time of death among adults with sickle cell disease (SCD) at Howard University in Washington, DC over a 25-year period. A single physician recorded circumstances of death among 141 adult SCD patients he treated and knew well from 1976 to 2001. These findings were determined by autopsy report and/or clinical assessment. In a subset of 31 patients, autopsy records were reviewed for reports of iron deposition in liver and heart and of organ pathology. One hundred and fourteen (80.9%) of the patients had SS phenotype and 66 (46.8%) were female. The mean ± SD age at death was 36 ± 11 years. Leading circumstances of death included pulmonary hypertension (PHT) (26.2%), sudden death (23.4%), renal failure (22.6%), infection (18.4%), thromboembolism (14.9%), cardiac diagnoses (12.0%), cirrhosis (11.3%), pneumonia or acute chest syndrome (9.9%), bleeding (7.8%), and iron overload (7.0%). When circumstances of deaths that occurred after 1991 (n = 69) were compared to those that occurred in 1991 or earlier (n = 72), PHT (36.2% vs. 16.6%; P < 0.01) was significantly more common in 1992 or later. Significant associations were found between PHT and thromboembolism and between cirrhosis and iron overload. In this proportional mortality study of adults with SCD, PHT was the leading finding at the time of death. Thromboembolism was associated with PHT, and iron overload was associated with cirrhosis. Am. J. Hematol. 81:858-863, 2006. V V C 2006 Wiley-Liss, Inc.

Peripheral Vasoconstriction and Abnormal Parasympathetic Response to Sighs and Transient Hypoxia in Sickle Cell Disease

“…Loss of HRV is also a significant risk factor for cardiovascular adverse events, including sudden death, in the general population (45). Interestingly, up to 23% of deaths in adults with SCD are so-called "sudden death" events with no detectable cause found at autopsy (4,46,47). The present study demonstrates significant withdrawal of parasympathetic activity in response to transient hypoxia in subjects with SCD that was not present in non-SCD control subjects.…”

Evolution of sickle cell disease from a life‐threatening disease of children to a chronic disease of adults: The last 40 years

“…In adults with Hb SS and Sb(0) followed at a tertiary care medical care center, the median age of death was 58 years of age [22], still significantly lower than that contemporary life-expectancy for African-American adults without SCD. Cardiopulmonary disease remains the major, but not exclusive, cause of death in adults with SCD [23][24][25][26]. More specifically, a significant proportion die from causes related to chronic organ dysfunction including pulmonary hypertension, renal failure, thromboembolism, infections and excessive iron stores.…”

“…More specifically, a significant proportion die from causes related to chronic organ dysfunction including pulmonary hypertension, renal failure, thromboembolism, infections and excessive iron stores. [24]. Treatments targeting vascular dysfunction to prevent major organ damage are under evaluation and may have the greatest impact in adults with SCD [27].…”

Uric Acid as a Potential Biomarker of Pulmonary Arterial Hypertension in Patients with Sickle Cell Disease

“…The median age at death is 42 years for men and 48 years for women [2]. Pulmonary complications, acute chest syndrome (ACS) and PAH account for a large proportion of deaths in adults with SCD [3,4]. One retrospective study reported a median survival of 25.6 months for SCD patients with PAH confirmed by right heart catheterization [5].…”