Sickle Cell Disease Crisis (Vaso-Occlusive Crisis)

Sickle cell disease (SCD) is an autosomal recessive hemoglobinopathy caused by a missense mutation in the beta-globin gene resulting in hemoglobin S. Under deoxygenation, HbS polymerizes, distorting erythrocytes into the characteristic sickle shape. These rigid cells obstruct the microcirculation, triggering ischemia and the cardinal vaso-occlusive crisis (VOC) that drives lifelong morbidity.

Crises range from typical bone-pain VOC to life-threatening acute chest syndrome, splenic sequestration, aplastic crisis (parvovirus B19), priapism, and cerebrovascular accidents. Triggers include infection, dehydration, hypoxia, cold exposure, acidosis, and stress. Recurrent vaso-occlusion damages multiple organs (kidney, lungs, brain, liver, eyes, hips), shortening life expectancy and impairing quality of life.

Acute crisis management focuses on prompt analgesia (opioids per pain protocol), aggressive hydration, supplemental oxygen if hypoxic, and treatment of triggers. Acute chest syndrome requires antibiotics, transfusion, and sometimes exchange transfusion. Long-term care includes hydroxyurea, L-glutamine, voxelotor, crizanlizumab, transfusion programs, hydroxyurea education, and curative options such as hematopoietic stem cell transplantation or gene therapy. Comprehensive multidisciplinary follow-up reduces mortality.