Treatment of Sickle Cell Pain

Karen F. Marlowe, PharmD., Michael F. Chicella, PharmD.


Pharmacotherapy. 2002;22(4) 

In This Article

Abstract and Introduction


Sickle cell disease affects 70,000 Americans who experience an average of 0.8 painful episodes each year. The pathophysiology of sickle cell pain is not completely understood. The disease is characterized by both acute and chronic pain syndromes. Patients with sickle cell pain often encounter barriers to receiving appropriate care, including lack of continuity of care and perceived opiate addiction. Studies describing pharmacotherapy for sickle cell pain have been primarily retrospective and uncontrolled. In analyzing the available literature regarding pathophysiology, assessment, and treatment of sickle cell pain, we found a need for increased practitioner education and inter-vention to improve the level of care provided to patients with this disease.


Approximately 70,000 Americans have sickle cell disease, and 1000 babies are born with the disease each year.[1] Although sickle cell disease can be present in any ethnic group, it is most common in people of African ancestry.[2] The disease is characterized by episodes of pain. Patients experience an average of 0.8 pain episodes each year.[3] In fact, pain crises are the most common cause for hospitalization of patients with sickle cell disease, representing 75,000 hospitalizations and $475 million in health care expenditure annually.[2,4]

Pain is debilitating for many patients with sickle cell disease; however, not all patients experience pain episodes with the same frequency or intensity. In one survey, 38% of patients did not report a pain crisis during a 5-year evaluation period.[2] In fact, only a few patients experienced most episodes. Of the respondents, only 5% of patients experienced three or more painful episodes a year, but these represented approximately one-third of all episodes.[2] A correlation was reported between the number of pain crises and early mortality from sickle cell disease.[2] Patients with risk factors for poor pain control are those who have poor coping strategies or are poorly adjusted, those with adverse social situations, and those whose lives are more profoundly affected by their pain episodes.[5]

Many health professionals are reluctant to prescribe adequate dosages of opiates due to concerns regarding addiction and side effects.[6] An evaluation of adequacy of pain control in 21 children with sickle cell disease found that 71% had not achieved adequate pain control during the study period.[7] Three possible explanations were proposed: pain was inadequately assessed, choice or dosage of analgesic was inappropriate, or providers did not understand the nature of sickle cell pain.[7]

In March 1999, the Joint Committee on Accreditation of Healthcare Organizations (JCAHO) approved standards for assessment and management of pain for inpatients and outpatients. The standards stress that patients have a right to appropriate assessment and management of their pain. In 2000, JCAHO began to determine institu-tional compliance with pain management through interviews with patients, families, and clinical staff and through review of pain-related policies and procedures.[8]

Inadequate treatment of pain, new treatment standards, and increasing patient awareness of treatment alternatives all emphasize the need to improve the management of sickle cell pain crises. We reviewed the current literature on acute and chronic pain in patients with sickle cell disease by identifying clinical trials, case reports, and reviews in the Medline database from 1980-2001. Several other articles were identified through reference citations.


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