The problem is that the patient’s red blood cells are misshapen and are prone to clumping together, creating blood flow problems. These blood flow problems are referred to as a vaso-occlusive crisis. Patients present with moderate to severe pain, which has variable intensity and frequency. Young children can have severe pain and swelling of both hands and feet (dactylitis). Most patients with sickle cell crisis experience pain by the age of 6 years. Pain can begin from any part of the body but frequently affects the extremities and back and chest areas. Fever can accompany vaso-occlusive crisis in some patients. Although pain in patients with sickle cell crisis is likely to be due to vaso-occlusive crisis, it is prudent to perform a thorough evaluation for other life-threatening causes that can be misattributed to sickle cell pain. Sickle cells resulting in a narrowed blood vessels in the spleen result in left upper quadrant pain. The same problem occurs in the coronary arteries resulting in chest pain. There is no objective measure or lab test to determine the quality and severity of pain in sickle cell crisis.

Management

Initial management is guided by the primary survey. The SAMPLE history will reveal the patient’s history of sickle cell disease. The patient’s primary problem will be pain. The patient will all but beg for something to control the pain, leaving the medics with a disadvantageous ethical decision. The patient will probable request transport to a hospital that is either familiar to them or is a sickle cell center.