Treatment of Sickle Cell Pain

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

Disclosures

Pharmacotherapy. 2002;22(4) 

In This Article

Assessment

Acute sickle cell pain has been described as more severe than postoperative pain and as intense as cancer pain.[13] It is unpredictable and may be provoked by temperature extremes, changes in altitude, physical and emotional stress, dehydration, menstruation, fatigue, or infections. It may have no apparent precipitating factor. Fewer than 50% of patients can identify a precipitant.[14] The most common sites for pain are lower back, thigh, hip, knee, abdomen, and chest. Pain tends to recur in the same areas for individual patients and typically occurs in two or more sites at a time.[3] Bone pain is usually symmetric and bilateral. Abdominal pain mimics pancreatitis, cholecystitis, or appendicitis.[6] Episodes often begin at night[14]; they typically last 3-14 days and follow a pattern. Most patients are pain free between exacerbations; however, some experience a chronic pain syndrome. Many have prodromal signs, such as numbness, tingling, fatigue, or scleral icterus. Pain accelerates over several days, reaches a peak, and then declines to resolution or to baseline levels.[15,16]

A thorough history, physical examination, and review of systems should be conducted with each sickle cell pain crisis. Patients should be evaluated for possible precipitating factors and educated on avoiding triggers. Pain should be assessed as to quantity, quality, location, time course, and aggravating and alleviating factors. Various scales are available to help rate pain intensity; most common is the visual analog scale, which consists of a horizontal line labeled from 0 (absence of pain) to 10 (worst pain ever experienced). Patients should rate their current level of pain and compare it with their pain rating for an average day. A pediatric version of this scale incorporates photographs of African-American children displaying emotions ranging from happiness to neutrality to distress. Children are asked to point to the face that illustrates how they feel. Also, children who cannot describe the location of their pain may be able to mark on a drawing of a person the place that hurts on their own body.[13]

Few patients with sickle cell disease experience constant pain. However, one report describes sickle cell disease as a chronic pain syndrome with acute exacerbations.[17] Several sources of chronic sickle cell pain are aseptic necrosis, leg ulcerations, and bony infarctions. The goal of therapy for patients with chronic pain is to improve functional capability while decreasing the amount of pain experienced.[3] Chronic pain can be assessed by means of diaries in which patients record daily their pain rating, location of pain, and steps they took to control their pain.

Patients with sickle cell disease should be assessed at least once/year during pain-free periods to record characteristics of their acute and chronic pain (e.g., quality, quantity, aggravating and alleviating factors, effects on school or work activities). This is also an appro-priate time to discuss their response to various treatment methods and determine a pain manage-ment plan for their next crisis. Involving patients in planning encourages them to be more self-reliant and take an active role in their care.[3,13]

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