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Sickle cell disease is one of the most prevalent single gene disorders, affecting over 50,000 Americans. Sickle cell disease is a debilitating condition that is associated with chronic anemia, stroke, splenic and renal dysfunction, susceptibility to bacterial infections, particularly in children, acute chest syndrome, and pain crises. Each year in the US, an average of 75,000 hospitalizations are due to sickle cell disease, costing approximately $475 million. Athough tremendous resources have been invested in looking for a cure and palliative care, the management of acute and chronic pain remains the major intervention, focusing on improving quality of life and decreasing morbidity. In spite of some decrease in mortality in children with sickle cell disease over the last 25 years, median life expectancy for most sickle cell patients remains below age 50 years. Pain, the most frequent symptom experienced by the patients with sickle cell anemia, profoundly impairs patient function at home and work, their social interactions, and their careers. Despite the high prevalence of pain episodes, correlating with morbility and mortality, sickle cell anemia pain remains underestimated and undertreated. There are still multiple misconceptions regarding the use of pain medications, including opioids, tolerance, and addiction.

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This review discusses the importance of pain assessment and control in the treatment of patients with sickle cell anemia. The importance of a comprehensive interdisciplinary approach to therapy, including medications, psychological, spiritual and social support, education, as well as availability of the comprehensive support system, is emphasized.

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The following case illustrates complexity of the management of sickle cell anemia patients.

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JF is 21-year-old Hispanic man, admitted to the hospital with severe pain in the legs, shoulders, chest, and abdomen of 2 to 3 days’ duration.

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History of Present Illness

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The patient’s condition was diagnosed as hemoglobin SS sickle cell anemia shortly after birth. Severe pain, requiring hospitalizations, has been recurrent for years and most recently has occurred 4 to 5 times a month, lasting from 3 to 4 days to 2 weeks. The patient has developed hemachromatosis, autosplenectomy, hepatomegaly, ascites, left lower leg ulcer, chronic renal failure requiring hemodialysis, and seizures as complications of the disease. At home, pain crises were managed with fentanyl patch 75 mg transdermally every 48 hours, hydromorphone 4 mg orally every 4 to 6 hours as needed, amitriptyline 25 mg orally daily, and diphenhydramine 25 mg orally as needed. When an inpatient, JF was treated with meperidine up to 100 mg intravenously as needed, methadone 10 mg orally every 4 hours, fentanyl patch and hydromorphone intravenously via patient-controlled analgesia (PCA). Clinicians were concerned about his attention-seeking behavior, opioid dependence, preoccupation with his pain, and his withdrawal from social activities. Patient was seen in the Emergency room 3–4 times a month and was frequently admitted to the hospital with pain crises. Opioid analgesics were continued according to the standard protocol at the constant dose in spite of the patient’s report of poor ...

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