Recent studies suggest that infection, fat embolization, and primary infarction may all lead to this clinical problem. Episodes may be severe, life-threatening events and recurrent episodes may result in chronic lung disease and pulmonary hypertension. Recurrent pain episodes occur at any age but appear to be particularly frequent during late adolescence and early adult life. These unpredictable events are characterized by severe pain in the back, chest, abdomen, extremities and head that are very unpleasant and disruptive to life. Pain episodes are the most common reasons for individuals to seek health care.
Bone disease is common in sickle cell disease.
Early risk is primarily from osteomyelitis. Bone infarctions become more common as patients age. Avascular necrosis of the femur and humerus are major causes of chronic pain and disability in older patients. Leg ulcers are seen in patients older than 10 years of age. They are resistant to therapy and cause significant morbidity.