Treatment of Pain Due to Vaso-Occlusive Crises (VOC) in Adults with Sickle Cell Disease (SCD): Limited Awareness of Available Guidelines.
Authors: Solomon Lawrence R. 
Published: October 2019

SCD is characterized by frequent VOC marked by severe pain often requiring emergency department visits and inpatient admissions for treatment with parenteral opioids. Guidelines for managing pain due to VOC, published in the US (in 1999 and 2002) and England (in 2003) suggest: 1) rapid initiation of parenteral opioids (within 15–30 min of arrival); 2) use of an adequate opioid starting dose (usually 5–10 mg or 0.1 mg/kg of morphine intravenously); 3) use of frequent repeat opioid doses (every 15–30 min) until pain is significantly improved; and 4) selection of an alternative treatment regimens based on an individual’s prior opioid-response history. However, attending physicians on the adult hematology service were generally unaware of the existence of these guidelines. Thus, all standard textbooks in internal medicine (N=7), hematology (N=3) and emergency medicine (N=3) available in hard-copy or on-line at the Yale University School of Medicine library and published after 2002 were reviewed for recommendations regarding: a) treatment of pain due to VOC; b) treatment of pain due to acute myocardial infarction; and c) treatment of bleeding due to Hemophilia A. For treatment of pain due to VOC, none of the texts indicated a target time for initiating opioid therapy and only 2 texts commented on the need to individualize therapy based on patient history. Suggested starting doses of opioids were not given in 5 of 13 references and were lower than guideline recommendations in one additional text. The frequency of opioid administration was not noted in 6 reference books; only 2 texts suggested repeat doses within a 20–30 minute interval; one text suggested an interval of 1 to 3 hours; and the other 4 texts suggested intervals of 2 to 4 hours. Overall, appropriate initial opioid doses were provided in 7 texts (54%); recommendations for dosing frequency were consistent with guidelines in 2 texts (15%); the need to individualize therapy based on patient history was noted in 2 texts (15%); and no text indicated a target time for the initiation of analgesic therapy. In 1996, guidelines for opioid use in the treatment of pain due to acute myocardial infarction suggested doses of 2–4 mg of intravenous morphine administered as often as every 5 minutes. Of the 10 relevant internal medicine and emergency medicine texts reviewed, recommendations were not given in one text but both the morphine dose and frequency of administration were consistent with these guidelines in the remaining 9 (90%). Finally, for hemophilia A, all 13 reference works provided specific and consistent directions for the calculation of Factor VIII treatment requirements while 10 texts (77%) described available Factor VIII preparations. In conclusion, standard texts do not present adequate infomation to permit effective treatment of acute pain in SCD. Since guidelines for frequent opioid use to treat pain due to acute myocardial infarction are given in most texts, this cannot be explained by constraints on recommending opioid therapy per se. Nor can it be explained by the rarity of SCD since more detailed information is provided for the treatment of hemophilia, an even less common disorder. It is of note then that only 5 of 13 texts (38%) reassure readers that narcotic addiction is infrequent in SCD patients while 9 of 10 texts (90%) offer this reassurance when discussing cancer pain treatment. Thus, greater educational efforts are needed to facilitate pain treatment in the VOC of SCD.