Sickle Cell and Acute Chest Syndrome Clinical Pathway — Inpatient
Long-term Outcomes
Patients with recurrent ACS are more likely to require additional therapies such as hydroxyurea or chronic blood transfusions. Patients with recurrent acute pain who are developing acute chest syndrome while inpatient should be considered for crizanlizumab, particularly if clinically unresponsive to hydroxyurea. Crizanlizumab is a p-selectin protein inhibitor and has been shown to significantly decrease the incidence of VOC episodes in patients with SCD.
ACS episodes are often preceded by an episode of VOC; early recognition and management may avoid escalation of symptoms. Recurrent episodes of ACS have been shown to be associated with the development of abnormal lung function tests (restrictive lung disease in adults). Although this has not been consistently shown in pediatric age group, pediatric pulmonologists remain concerned about the effects of recurrent ACS on lung function in children with SCD.