Sickle cell disease is a major health problem, worldwide. Timely and appropriate medical care can reduce the burden of disease and prevent serious health complications for sickle cell patients.
Sickle cell disease is the most common globin gene disorder globally and is associated with extremely painful episodes in which sickle-shaped blood cells cause vaso-occlusion resulting in a lack of oxygen delivery to tissues and tissue infarction.
For patients with sickle cell disease, pain is a constant issue. Pain from a sickle cell crisis can last from hours to days and is difficult to alleviate. A pain management strategy can aid in the management of chronic pain as well as pain resulting from a sickle cell emergency.
Sickle cell pain can vary in severity and can be acute or chronic. Chronic pain generally involves avascular necrosis of a hip, shoulder, ankle or vertebra. Many sickle cell patients experience sickle cell emergencies several times a year. Some, however, do not.
Pain-relieving treatments for sickle-cell disease
Choice of pain treatment depends on whether the pain is acute, chronic, or a combination, and on the patient’s opioid history. For mild to moderate acute pain dihydrocodeine is recommended. Pain that is not alleviated by 2 dihydrocodeine every 4 hours is considered moderate to severe and is treated with diamorphine. Diclofenac or ibuprofen can be added to help with bone pain. Side effects of opiates are treated symptomatically with sodium docusate or senna for constipation, metoclopramide for nausea, oral hydroxyzine for pruritus, and naloxone for respiratory depression.
Chronic pain is treated with multimodal therapy, as needed including analgesics, nerve blocks, orthopedic care for chronic back pain or other orthopedic issues, and surgical intervention for avascular necrosis. Additionally, physical therapy can help decrease joint stiffness and pain and prevent muscle contracture, and cognitive behavioral therapy can assist the patient in developing coping strategies for dealing with pain and stress.
Reference
Okpala, I., & Tawil, A. (2002). Management of pain in sickle-cell disease. Journal of the Royal Society of Medicine, 95(9), 456-458. https://doi.org/10.1258/jrsm.95.9.456
