Prospective evaluation of chronic organ damage in adult sickle cell patients: A seven‐year follow‐up study

Abstract

Organ damage in sickle cell disease (SCD) is a crucial determinant for disease severity and prognosis. In a previous study, we analyzed the prevalence of SCD-related organ damage and complications in adult sickle cell patients. We now describe a seven-year follow-up of this cohort.All patients from the primary analysis in 2006 (n = 104), were included for follow-up. Patients were screened for SCD-related organ damage and complications (microalbuminuria, renal failure, elevated tricuspid regurgitation flow velocity (TRV) (≥2.5 m/seconds), retinopathy, iron overload, cholelithiasis, avascular osteonecrosis, leg ulcers, acute chest syndrome (ACS), stroke, priapism and admissions for vaso-occlusive crises (VOC) biannually. Upon 7 years of follow-up, progression in the prevalence of avascular osteonecrosis (from 12.5% to 20.4%), renal failure (from 6.7% to 23.4%), retinopathy (from 39.7% to 53.8%) was observed in the whole group. In HbSS/HbSβ -thal patients also progression in microalbuminuria (from 34% to 45%) and elevated TRV (from 40% to 48%) was observed while hardly any progression in the prevalence of cholelithiasis, priapism, stroke or chronic ulcers was seen. The proportion of patients with at least one episode of ACS increased in the group of HbSS/HbSβ -thal patients from 32% to 49.1%. In conclusion, 62% of the sickle cell patients in this prospective cohort study developed a new SCD-related complication in a comprehensive care setting within 7 years of follow-up. Although the hospital admission rate for VOC remained stable, multiple forms of organ damage increased substantially. These observations underline the need for continued screening for organ damage in all adult patients with SCD.

Elevated tricuspid regurgitation velocity in congenital hemolytic anemias: Prevalence and laboratory correlates

“…LDH was associated with TRV elevation in children with SCD if genotype or treatment exposure was not accounted. TRV elevation increases over time in adults with SCD; therefore, prospective serial evaluations are necessary in children with SCD to better understand the longitudinal changes in hemoglobin that may occur with TRV and how it relates to disease severity.…”

Rapid decline in estimated glomerular filtration rate is common in adults with sickle cell disease and associated with increased mortality

“…Glomerular filtration rate progressively declines with age, with an annual rate of decline of kidney function of 1·27 ± 1·97 ml/min/1·73 m 2 reported in African‐American adults (Young et al , ). Multiple previous studies suggest that kidney function progressively declines in SCD (Gosmanova et al , ; Asnani et al , ; van Tuijn et al , ; Xu et al , ; Derebail et al , ). In a 25‐year prospective longitudinal demographic and clinical cohort study, 31 out of 725 patients with HbSS (4·2%) and 5 out of 209 patients with HbSC disease (2·4%) developed chronic renal failure (Powars et al , ).…”

“…A retrospective, single centre study of 98 SCD patients found that the prevalence of CKD, defined according to the 2012 KDIGO recommendations, increased from 28·6% to 41·8% after a mean follow‐up of 5 years (Gosmanova et al , ). In a more recent single‐centre, prospective study of 104 patients in the Netherlands, the prevalence of renal failure increased from 6·7% to 23·4% after 7 years of follow‐up (van Tuijn et al , ). Although these studies assessed the progression of CKD, they did not evaluate rapid decline in kidney function and its association with mortality.…”

Sickle Cell Clinical Research and Intervention Program (SCCRIP): A lifespan cohort study for sickle cell disease progression from the pediatric stage into adulthood

“…This survival increase is primarily due to mandatory newborn screening, infection prevention with penicillin prophylaxis and pneumococcal vaccination, improved supportive care, and increased use of disease‐modifying therapies, such as hydroxyurea and chronic erythrocyte transfusions . However, accumulation of end‐organ damage to the heart, lungs, brain, kidneys, and bones continues to occur . This organ damage manifests in young adults with higher rates of acute health utilization, emergency room reliance, and hospitalization .…”