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Am Fam Physician. 2000;61(5):1544-1550

The American Pain Society (APS) has released a comprehensive new guideline to aid physicians and other health care professionals in the management of patients with acute and chronic pain associated with sickle cell disease. Published in August 1999, the “Guideline for the Management of Acute and Chronic Pain in Sickle Cell Disease” is the nation's first evidence-based guideline on this disease. The APS guideline development process combined a systematic review of scientific evidence with the judgment of experts.

According to the APS, pain is the hallmark clinical manifestation of sickle cell disease, which predominantly affects African-American, African/Hispanic-Caribbean and South American persons in the United States. Severe pain can occur in patients as young as six months of age, and may continue throughout a person's life. The pain can be managed effectively, as long as barriers to adequate pain assessment and management are overcome.

The guideline covers the following information: an overview of the disease, the types and characteristics of pain associated with the disease, recommendations for pain assessment, methods for treatment of pain and a discussion of pain in the various developmental stages. It also includes discussions of pain assessment instruments.

For more information about the guideline, write to the APS at 4700 W. Lake Ave., Glenview, IL 60025-1485, or call 847-375-4715. The cost is $15 for each guideline with a discount on 10 or more.

Pain Assessment

According to the APS guideline, the goals of pain assessment are to characterize patients' pain status and related experiences over time, to provide a basis on which treatment decisions can be made and to document the effectiveness of pain management strategies. Because pain management is a subjective experience, pain assessment relies heavily on self-reports of patients and physicians' use of valid and reliable clinical measurement instruments. The two main categories of pain assessment are rapid and comprehensive. The goal of rapid assessment is to provide immediate treatment of a brief painful episode. A comprehensive assessment is multidimensional, geared toward treatment planning. It includes assessment of physiologic, sensory, affective, cognitive, behavioral and sociocultural components. The accompanying figure is a flow chart for pain assessment by physicians.

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The APS makes the following recommendations on pain assessment of patients with sickle cell disease:

  • Physicians should ask patients about their pain. Patients' self-reports should be the main source of pain assessment, with the exception of infants, for whom behavioral observations are the primary source of assessment.

  • For rapid assessment of pain during an acute painful event, physicians should select a simple measurement of pain intensity, reassess the patient frequently and record the measurement for treatment evaluation.

  • Patients should have a comprehensive clinical assessment of their pain every year, and more often if they have frequent pain.

  • Patients should be reassessed frequently. Patients should be asked how much their pain has been relieved after the first treatment and then after subsequent treatment adjustments.

  • When physicians consistently observe a disparity between patients' verbal self-report of their pain and their ability to function, further assessment should be performed to ascertain the reason for the disparity.

  • Physicians should be able to understand and describe the pain in sufficient detail so that treatment can be tailored to the individual needs of each patient.

  • When selecting assessment methods and tools, developmental stage, chronologic age, functional status, cognitive abilities and emotional status should be considered.

Treatment of Pain in Sickle Cell Disease

Pain in sickle cell disease ranges from acute to chronic. Some patients only have occasional episodes of acute pain; if it is managed adequately, they can cope well. Other patients have more frequent episodes of pain; if it is not adequately managed, it is considered to be chronic.

The APS makes the following recommendations for the treatment of pain in patients with sickle cell disease:

  • Pain management should be aggressive to ease pain and enable patients to attain maximal functional ability.

  • Analgesics are the foundation for management of sickle cell pain; their use should be tailored to each patient.

  • Nonsteroidal anti-inflammatory drugs or acetaminophen should be used to manage mild to moderate pain, unless contraindicated. If this type of pain persists, an opioid should be added.

  • The type of opioid used should be based on the type and expected duration of the pain.

  • Because meperidine is toxic to the central nervous system, it should not be used if frequent large doses or long treatment durations are anticipated.

  • Opioid tolerance and physical dependence are expected with long-term opioid treatment and should not be confused with psychologic dependence.

  • Sedatives and anxiolytics should not be used alone to manage pain because they can mask the behavioral response to pain without providing analgesia.

  • Severe pain should be considered a medical emergency, and timely and aggressive management should be provided until the pain becomes tolerable.

  • Equianalgesic doses of oral opioids should be prescribed for home use when necessary.

  • Appropriate tapering of opioids in patients at risk for withdrawal syndromes is essential.

Psychologic, Behavioral and Physical Interventions

The use of psychologic, behavioral and physical interventions may enhance the positive impact of pain medications or help reduce the use of medications. The integration of these modalities into treatment should be culturally sensitive, as well as tailored to the patient's individual needs.

The APS makes the following recommendations on the integration of psychologic, behavioral and physical intervention:

  • Patients and their families should receive information about the pain as part of the ongoing treatment.

  • Analgesic medications should be used in combination with psychologic, behavioral and physical modalities in the management of sickle cell disease.

  • Pain assessment and treatment should be conducted early to provide a foundation on which to build further constructive pain management interventions throughout a patient's life.

  • Cognitive therapies should be used to enhance coping strategies and reduce negative thoughts.

Coverage of guidelines from other organizations does not imply endorsement by AFP or the AAFP.

This series is coordinated by Michael J. Arnold, MD, Assistant Medical Editor.

A collection of Practice Guidelines published in AFP is available at

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