Guidelines for the management of emergencies and critical illness in pediatric and adult patients with sickle cell disease

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.

Fig. 1

Initial pain management of a vaso-occlusive crisis in adults with sickle cell disease. PCA patient controlled analgesia, VAS visual analog scale, VRS verbal rating scale, RR respiratory rate; SS sedation scale, 1 drowsy patient, easily awakened, 2 very drowsy, awakened by verbal stimulation, 3 very drowsy, awakened by tactile stimulation, SL sublingual, SC subcutaneous, PO per os, IV intravenous, IR immediate release, PR prolonged release, TTE time to effect, green smiley improvement, red smiley no improvement

Fig. 2

Initial pain management of a vaso-occlusive crisis in children with sickle cell disease. VAS visual analog scale, EMONO equimolar mixture of oxygen and nitrous oxide, NSAID non steroidal anti-inflammatory, EVENDOL évaluation enfant douleur, PCA patient controlled analgesia, IV intravenous, IN intranasal, PO per os,*every 30–60 min (personalized and based on age)

Fig. 3

Management of a priapism in adults with sickle cell disease