Guidelines for the management of emergencies and critical illness in pediatric and adult patients with sickle cell disease
Armand Mekontso Dessap, Stéphane Dauger, Mehdi Khellaf, Maïté Agbakou, Sophie Agut, François Angoulvant, Jean‐Benoît Arlet, Cécile Aubron, Florent Baudin, Florence Boissier, Nicolas Bounaud, P. Catoire, Jérôme Cecchini, Djamila Chaiba, Anthony Chauvin, Richard Chocron, Bénédicte Douay, Delphine Douillet, Narcisse Elenga, Olivier Flechelle, Ségolène Gendreau, Sybille Goddet, Jérémy Guénézan, Anoosha Habibi, Claire Heilbronner, Bérengère Koehl, Pierrick Le Borgne, Philippe Le Conte, Annick Legras, Michaël Levy, Bernard Maître, M. Oberlin, Mehdi Oualha, Nicolas Peschanski, France Pirenne, Corinne Pondarré, Jérôme Rambaud, Keyvan Razazi, Geoffroy Rousseau, Aurelie Schirmann, Isabelle Thuret, Ruddy Valentino, Guillaume Voiriot, Barbara Villoing, Marion Grimaud, Sandrine Jean
Abstract
Forty-two questions were evaluated concerning management of emergencies and critical illnesses in paediatric and adult patients with sickle cell disease. The assessment covered the following areas: patient referral, vaso-occlusive crisis, acute chest syndrome, transfusion therapy, and priapism. The patient referral category included guidelines for admission to intensive care unit and management at specialized reference centers. The vaso-occlusive crisis topic encompassed pain management, hydration, incentive spirometry, and target oxygen saturation levels. For acute chest syndrome, the focus areas included imaging techniques such as lung ultrasound, computed tomography scans, and echocardiography; treatment with systemic corticosteroids; non-invasive ventilation; prophylactic and therapeutic anticoagulation; and procalcitonin and antibiotic therapy. The section on transfusion therapy addressed indications and methods of transfusion, as well as the diagnosis and prediction of delayed hemolytic transfusion reactions. A total of 45 recommendations were proposed, including 14 specific to adults, 13 specific to pediatrics, and 18 applicable to both adults and children, along with three therapeutic algorithms. The Grade of Recommendation Assessment, Development, and Evaluation (GRADE) methodology was adhered to throughout the process. Sixteen recommendations were based on a low level of evidence (GRADE 2+ or 2-), while 26 were based on evidence that could not be classified under the GRADE system and were therefore considered expert opinions. Finally, for three aspects of sickle cell disease management, the experts concluded that no reliable recommendations could be made based on the current state of knowledge. The recommendations and therapeutic algorithms received strong agreement from the experts.