Clinical management of acute and chronic pain in patients with sickle cell disease

Sickle cell disease (SCD) is a rare hereditary red cell disorder. One of the main clinical manifestations of SCD are the acute painful vaso-occlusive crisis, which are responsible for frequent accesses of SCD patients to the emergency departments (EDs). Multiple pathophysiological mechanisms cause the onset and the maintenance of pain. In SCD patients, three different pains might be recognized: the acute pain related to VOCs, the inter-VOC pain and the chronic pain. A significant number of patients develop chronic pain with characteristic of centralized or neuropathic pain. Pharmacologic treatment is a valid option in SCD pain, even if, in chronic pain it is often unsatisfactory, particularly in patients where pain is sustained by a neuropathic mechanism. Opioids don’t represent the only therapeutic choice, and their use don’t seem to satisfy all the patients or physicians. A multimodal analgesia is the correct approach to SCD pain, and the choice of drug must always take into consideration the site and mechanism of action of it. The non-pharmacological treatments may be an alternative and they might represent very efficacious approaches in several patients, particularly considering cognitive-behavior therapy. In this narrative review will be analyzed the mechanisms of pain involved in the SCD and the current management of acute and chronic pain.