ABSTRACT
Sickle cell disease (SCD) induces a chronic prothrombotic state. Central venous access devices (CVADs) are commonly used for chronic transfusions and iron chelation in this population. CVADs are an additional venous thromboembolism (VTE) risk factor. The role of thromboprophylaxis in this setting is uncertain. The objectives are: (1) to determine whether thromboprophylaxis reduces VTE risk in SCD patients with CVAD and (2) to explore characteristics associated with VTE risk. We identified adults with SCD and CVAD intended for chronic use (≥3 months) at two comprehensive SCD centers. Thromboprophylaxis presence; type; intensity; and patient-, catheter-, and treatment-related VTE risk factors were recorded. Among 949 patients, 49 had a CVAD (25 without and 24 with VTE prophylaxis). Thromboprophylaxis type and intensity varied widely. Patients without thromboprophylaxis had higher VTE rates (rate ratio (RR) = 4.0 (95% confidence interval: 1.2−12.6), p = 0.02). Hydroxyurea was associated with lower VTE rates (RR = 20.5 (6.4−65.3), p < 0.001). PICC lines and Vortex and Xcela Power implantable devices were associated with higher rates compared with Port-a-Cath (RR = 5.8 (1.3−25.9), p = 0.02, and RR = 58.2 (15.0−225.0), p < 0.001, respectively). Thromboprophylaxis, hydroxyurea, and CVAD subtype were independently associated with VTE. The potentially protective role of thromboprophylaxis and hydroxyurea for VTE prevention in patients with SCD and CVAD merits further exploration.
ABSTRACT
Importance: Adults with sickle cell disease (SCD) disproportionally experience early cognitive decline; however, guidance on the optimal screening strategy for cognitive dysfunction is lacking, and several available tools are biased by language, educational level, socioeconomic status, and race/ethnicity. The Rowland Universal Dementia Assessment Scale (RUDAS) was specifically designed for cognitive screening in multicultural populations. Objective: To ascertain the prevalence of suspected dementia in adults with SCD using the RUDAS, and to identify whether age, sex, educational level, several biological variables, and SCD complications were associated with RUDAS scores. Design, Setting, and Participants: This multicenter, bilingual, cross-sectional study was conducted in 2 SCD comprehensive care centers in Canada (Centre Hospitalier de l'Université Montréal in Montréal and University Health Network in Toronto). Participants were adults aged 18 years or older and were enrolled in the study between July 1, 2018, and July 30, 2019. All outpatients were eligible and offered study participation, unless they had an acute medical condition that required inpatient care or they were unable to follow study instructions. Interventions: The RUDAS was administered by trained personnel in either French or English, according to the patient's language preference. A questionnaire on social determinants of health was also administered, and participants underwent screening for anxiety and depression. Main Outcomes and Measures: Proportion of participants with RUDAS scores that were suggestive of dementia and the RUDAS score. Any score lower than 23 points was suggestive of dementia, a score between 23 and 27 points indicated a possible association with mild neurocognitive disorder, and a score higher than 27 points was normal. Results: A total of 252 adult patients with SCD were included (136 women [54.0%]; mean [range] age, 34.8 [18-75] years). Overall, 29 patients (11.5%) had RUDAS scores that were suggestive of dementia, and this proportion increased with age (15 [8.7%] in the 18-39 years age group, 10 [14.5%] in the 40-59 years age group, and 4 [36.4%] in the ≥60 years age group). The RUDAS scores were not associated with sex, language, SCD genotype, and SCD complications. The highest level of education was significantly associated with the RUDAS score; however, the association was small (η2 = 0.02; 95% CI, 0.00-0.07; P = .02). In a multivariable analysis, lower glomerular filtration rate (r = 0.40; 95% CI, 0.29-0.50; P < .001) and increasing age (r = -0.37; 95% CI, -0.47 to -0.26; P < .001), but not SCD genotype or disease severity, were associated with lower RUDAS scores. Conclusions and Relevance: This study found that using the RUDAS revealed a high prevalence of suspected dementia in adult patients with SCD that was associated with worsening kidney function and age. Cognition should be screened in all adult patients with SCD, regardless of age, disease severity, and SCD genotype; further validation of the RUDAS is ongoing.
