PAIN CRISIS INTERVENTION FOR CHILDREN WITH FABRY DISEASE

Fabry disease is a lysosomal storage disorder inherited by X-sex chromosomal recessive that cause various symptoms to various organs in the body due to deficiency of αgalactosidase A. Signs and symptoms of Fabry disease begins at a young age, where the average age of onset of symptoms begin at age of 7 for boys and 8 for girls. Symptoms often occur during early childhood, and these symptoms include chronic neuropathic pain, acute pain crisis, thermal hypersensitivity, cold sensitivity and fatigue. In particular, the main characteristic symptoms of children with Fabry disease are the severe pain in the tip of the fingers and toes as a peripheral neuropathy. The pain starts with acroparesthesia, where due to intermittent Fabry crises or chronically, tingling paresthesias occurs which brings burning sensation at the fingertips. Since the child pain from Fabry disease which is not properly mediated affects the safety, proper management of pain is important. Uncontrolled pain of children with Fabry disease is accompanied by growth disorders and delay in puberty. Pain assessment is essential for pain management and continuous nursing of children with Fabry disease, where inappropriate pain assessment may be a major cause of pain management hindrance. The purpose of this study was to examine the main symptom of pain in children with Fabry disease and to utilize it as basic data for preparing the intervention plan.