Treatment and prevention of pain due to vaso-occlusive crises in adults with sickle cell disease: an educational void

Abstract

Pain due to vaso-occlusive crisis is the major cause of hospital use in sickle cell disease. Although available guidelines provide recommendations for opioid administration in this setting, only 4 (21%) of 19 medical textbooks present treatment regimens that are consistent with them. Moreover, only 7 texts (37%) note that addiction is infrequent in this population, while 11 (92%) of 12 texts provide such reassurance for cancer-related pain (P < .005). Finally, hydroxyurea use to decrease the frequency of vasoocclusive crises is completely defined only in 2 textbooks. Thus, most medical texts provide neither adequate information for the treatment or prevention of pain due to vaso-occlusive crisis in sickle cell disease nor reassurance of the unlikelihood of addiction in this population. In contrast, treatment recommendations for less common hematologic disorders are consistent with current standards in 53% to 84% of appropriate texts (P < .05). Limited knowledge regarding the principles and appropriateness of opioid therapy; a lack of evidence-based research on pain control; and misconceptions and prejudices about drug abuse and addiction contribute to this educational void. Thus, research and training on pain control in sickle cell disease are needed to parallel studies of environmental and genetic factors contributing to the known clinical heterogeneity of this disorder. (Blood. 2008;111:997-1003)

How I use hydroxyurea to treat young patients with sickle cell anemia

“…Often occurring without a specific trigger, pain typically presents in the chest, back, or extremities and requires hydration, analgesia, and frequent treatment, including hospitalization. Pain is common in SCA 8 and often inadequately recognized and treated, 9 but pain is not the whole picture and should not be considered the only clinical manifestation of SCA worth treatment or prevention. Other acute vaso-occlusive events include splenic sequestration, acute chest syndrome (ACS), and stroke, whereas others derive more from hemolytic anemia, such as pigmented bilirubin gallstones and jaundice.…”

Current Management of Sickle Cell Anemia

“…This association usually follows sternal or truncal pain, which leads to splinting and poor inspiratory effort, and lack of active and complete inspiration following opioid-induced sedation. The frequency and severity of pain in SCA is more than just episodic and acute, however; pain in SCA is often chronic, underrecognized and underreported, and therefore undertreated (Solomon 2008). Pain diaries of 232 adult patients showed that SCA pain is common and often chronic; pain was present on 54.5% of days and 29.3% of patients reported pain on .95% of days (Smith et al 2008).…”

Timing of Opioid Administration as a Quality Indicator for Pain Crises in Sickle Cell Disease

“…Unfortunately, the management of VOC, consisting primarily of opioids, anti-inflammatory medication, and intravenous hydration, has not changed in decades. [5][6][7][8] Recent studies of novel agents for VOC treatment, including inhaled nitric oxide and purified poloxamer-188, demonstrated little or no clinical benefit. 9,10 Although some centers in larger cities have established "day hospitals" for the specialized care of SCD patients with VOC, 11 the vast majority of initial medical care occurs in hospital emergency departments (EDs).…”