Sickle cell disease (SCD) patients are at increased risk for a whole slew of life-threatening problems. One of the many reasons they are vulnerable is because people with SCD are functionally asplenic, so they’re more likely to suffer from serious bacterial infections like meningitis, osteomyelitis, and septic arthritis. For a variety of reasons, they’re also more likely than the general population to suffer from cholecystitis, priapism, leg ulcers, avascular necrosis of the hip, acute coronary syndromes, pulmonary embolism, and even sudden exertional death. Many of these diagnoses present with pain similar to a sickle cell crisis and can be challenging because the presentations of some are less typical than usual.

Sickle Cell Pain Crisis Is a Diagnosis of Exclusion

Patients with SCD can sometimes present a challenge when it comes to pain management because it’s often difficult to discern whether they’re malingering. The majority of SCD patients suffer real pain but may not look uncomfortable because they have learned to adapt to a lifetime of chronic pain. In the emergency department, they may appear calm, be preoccupied with their handheld device, or be casually chatting. The rates of true opioid addiction in sickle cell patients are low (less than 5 percent), and the literature suggests that emergency physicians undertreat pain in sickle cell patients. I recommend that unless there is clear evidence the patient does not have SCD, take the patient’s complaint seriously and use analgesics aggressively in the emergency department.

As in all of emergency medicine, physicians need to think of the worst diagnoses first. Therefore, it is imperative not to assume that a patient with SCD who is in pain is suffering from a sickle cell crisis. If the pain is different from previous pain crises, broaden your differential diagnosis to include not only all the painful conditions that should be considered in all emergency patients but also sickle cell–specific conditions such as acute chest syndrome. The diagnosis of a pain crisis is a clinical one; no laboratory test will reliably determine whether the patient is suffering from a pain crisis, and SCD has been described in all races. Do not assume that a patient does not have SCD just because they have a light skin color. Sickle cell patients presenting with an uncomplicated pain crisis will often have normal vital signs. Any abnormal vital signs (especially fever) should raise the suspicion of alternative diagnoses. Examine joints and soft tissues looking for evidence of cellulitis, septic joints, osteomyelitis, and joint avascular necrosis. Perform a careful respiratory exam looking for evidence of an acute chest syndrome. Look for hepatosplenomegaly if you have a concern for splenic sequestration.