The sickle cell painful crisis in adults: phases and objective signs

The severe sickle cell painful crisis that requires hospitalization in adults typically seems to evolve along four distinct phases: Prodromal, initial, established, and resolving. Each phase may to be associated with certain clinical and laboratory findings. Objective clinical and laboratory signs may not be evident in the initial phase of the crisis especially in the absence of reliable base line steady state data. Several clinical, hematological, biochemical, and rheological changes emerge as the crisis progresses. This sequence of events creates several logistic issues in the care of these patients. The absence of objective findings early in the crisis usually discourages health care providers from repeating such tests and makes some of them suspicious of the authenticity of the painful episode in question. Consequently, an atmosphere of mistrust may develop between the patients and their care providers. This review puts the sickle cell painful crisis in perspective and, based on available data in the literature, shows that it is a dynamic process characterized by several changes during its evolution. It is hoped that this review will dispel some misconceptions about the painful crisis, elucidate its clinical course, and encourage further research to answer the questions it raises.