Comprehensive assessment, pain and ventilatory management during acute complications of adult sickle cell disease: A clinical practice survey in French intensive care units

Abstract

Painful vaso-occlusive crisis (VOC) and acute chest syndrome (ACS) 1 are the main complications of sickle cell disease (SCD) leading to ICU. The French Internal Medicine Society published guidelines 2 in 2015 without any intensivists' participation. Despite the frequent use of ICU resources, there may be heterogeneity in treatment and global management, likely because SCD is complex and often requires a multidisciplinary care. Moreover, the geographical distribution of patients with SCD is not homogeneous across the country, as the prevalence of this disease is higher in specific areas. Finally, there is a lack of ICU-specific guidelines for SCD. The aim of this study was to assess, nationwide, medical practices and opinions concerning the evaluation and ventilatory management of patients with SCD admitted to the ICU. This clinical practice survey was an observational study collecting medical opinions and practices. It was approved by the Ethics and Research Committee of the Paris-Saclay University. Clinicians were provided an information letter. We developed the questions with the CARMAS Research Group and focused on the early evaluation and management of patients with SCD in the ICU. We carried out this survey online and sent it to the medical teams of the 55 reference/ competence centres in France (and overseas departments), identified on the Orphanet database (https:// www. orpha. net). 3 The survey (available in Data S1) included questions relating to three typical clinical case scenarios. The first was a hyperalgesic VOC, without other symptoms. Because chest pain is seen as a sign of early ACS, 4 in the second case, we added chest pain to the first clinical situation. The third clinical case was an ACS with chest pain, dyspnoea and fever. We grouped centres according to their annual case volume (more or less than 10 SCD patients per year). Continuous variables are reported as mean ± SD or median (interquartile range [IQR]), and categorical variables as n (%). The statistical analysis protocol is available in Data S1.Thirty-four centres completed the survey, including 22 (64.7%) university hospitals, 11 (32.4%) general hospitals and 1 (2.9%) private hospital. The median [IQR] number of ICU beds in these centres was 24 [18][19][20][21][22][23][24][25][26][27][28]. Half the ICUs admitted less than 10 patients with SCD annually. We therefore grouped centres according to the annual number of