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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 ...