ABSTRACT
Sickle cell disease (SCD) refers to a clinical syndrome resulting from homozygous or compound heterozygous inheritance of the sickle haemoglobin gene leading to characteristic haemoglobin polymerization and deformation of red cells into sickle or crescent shape. The resultant clinical effects from red cell deformation include vaso-occlusive events and decreased red cell survival (haemolysis). Vaso-occlusion occurs in different organ beds including the bones, leading to ischaemic injury. Major vaso-occlusive phenomenon causes acute disease manifestations such as bone pain crisis. Painful crisis resulting from vaso-occlusion in SCD is largely nociceptive in nature. Painful crisis is the main characteristic manifestation of SCD. Chronic pain in SCD could be primary (chronic pain syndrome) or secondary to other complications such as osteonecrosis.
The mainstay of treatment in acute pain episode is analgesics especially opioids. Drug administration and dosage is often titrated with pain severity in different protocols including fixed dose schedules, unscheduled doses including the option of patient-controlled analgesia. Chronic pain syndromes require long-term care. Non-pharmacologic pain control strategies such as physical therapy, meditation and relaxation techniques could be tried. Preventive management of pain will include avoidance of provocative stimuli, use of disease modifying agents such as hydroxyurea and transfusion therapy.
