Quality of life and pain management in sickle-cell disease

Abstract

Sickle-cell disease is the most common genetic disorder worldwide and is characterized by intermittent severe painful episodes and other complications such as stroke, priapism, cholecystitis and acute chest syndrome. This review outlines the causes and characteristics of pain in children with sickle cell disease, how pain can be assessed and how painful episodes can be prevented. Sickle cell disease can have a significant impact on the patient’s quality of life, and the ways in which this can manifest, how it can be measured and how interventions such as cognitive behavioral therapy can be used to manage it are outlined. Medical management of acute painful episodes both in the community and in the hosptial setting are discussed, including the role of different analgesic and adjuvant agents. Prevention of painful episodes with hydroxycarbamide is discussed.