The well-meaning push to curb opioid prescribing could worsen healthcare for sickle cell patients. Clinicians tend to undertreat the substantial pain experienced by many sickle cell patients and treat them as drug addicts. However, research does not support increased risk of addiction in this patient population.
Challenging Pain, Few Options
A 2008 study in Annals of Internal Medicine revealed a vast undercurrent of chronic pain in sickle cell disease, with 29% of patients reporting pain—which is often severe—nearly every day. On top of that, vaso-occlusive episodes can trigger excruciating pain and life-threatening complications, such as organ damage. These crises prompt most ED visits for sickle cell disease.
Hydroxyurea, the one drug approved by the FDA to treat the disease, is used infrequently despite being effective. The drug lessens the frequency of pain crises but does not eliminate them. In any case, opioids remain essential for treating sickle cell pain. Clinical trials testing these agents in sickle cell populations are lacking, but even less is known about possible alternatives.
Inaccurate Assumptions of Pain
A recent study found that most sickle cell patients delay seeking treatment until their pain nears 9 on a 10-point intensity scale. When they finally go to the ED, they face longer waits for care than others in less pain. In some research, these patients report that healthcare providers sometimes treat them without respect. Indeed, clinicians often wrongly suspect them of exaggerating their pain or abusing drugs.
A Patient-Centered Approach to Treating Sickle Cell Pain
Treating sickle cell pain starts with assessment, but no laboratory test or physiological marker can measure this pain. Nor can clinicians gauge pain levels by simply looking at patients; they must rely on patients’ self-reports. Individualized pain treatment plans can speed relief to patients in the ED. They clarify what works for each individual, although primary care providers must keep them up to date. Getting ED staff to implement them is another challenge, underscoring the need for sickle cell treatment centers where feasible because they can provide prompt, knowledgeable pain management.
Unfortunately, subduing sickle cell pain often requires high doses of opioids, perhaps due to patients’ high morphine clearance rates or because they may simply need more medication than others to reach the same plasma level. None of this negates the need to monitor patients for drug addiction or diversion. Those who provide continuity of care to these patients should shoulder this burden.
Despite their risks, opioids remain indispensable for treating sickle cell pain. Efforts to rein in overprescribing must not ignore the needs of these undertreated patients.
Readings & Resources (click to view)
Elander J, Lusher J, Bevan D, Telfer P. Pain management and symptoms of substance dependence among patients with sickle cell disease. Soc Sci Med. 2003;57:1683-1696.
Smith WR, Penberthy LT, Bovbjerg VE, et al. Daily assessment of pain in adults with sickle cell disease. Ann Intern Med. 2008;148:94-101.
Minniti CP, Lu K, Groninger H. Pain in sickle cell disease #270. J Palliat Med. 2013;16:697-699.
Wilkie DJ, Molokie R, Boyd-Seal D, et al. Patient-reported outcomes: descriptors of nociceptive and neuropathic pain and barriers to effective pain management in adult outpatients with sickle cell disease. J Natl Med Assoc. 2010;102:18-27.
McGann PT, Ware RE. Hydroxyurea for sickle cell anemia: what have we learned and what questions still remain? Curr Opin Hematol. 2011;18:158-165.
Solomon LR. Treatment and prevention of pain due to vaso-occlusive crises in adults with sickle cell disease: an educational void. Blood. 2008;111:997-1003.
Jenerette CM, Brewer CA, Ataga KI. Care seeking for pain in young adults with sickle cell disease. Pain Manag Nurs. 2012 Jan 21 [Epub ahead of print].
Lazio MP, Costello HH, Courtney M, et al. A comparison of analgesic management for emergency department patients with sickle cell disease and renal colic. Clin J Pain. 2010;26:199-205.
Lattimer L, Haywood C Jr, Lanzkron S, Ratanawongsa N, Bediako SM, Beach MC. Problematic hospital experiences among adult patients with sickle cell disease. J Health Care Poor Underserved. 2010;21:1114-1123.
Ratanawongsa N, Haywood C Jr, Bediako SM, et al. Health care provider attitudes toward patients with acute vaso-occlusive crisis due to sickle cell disease: development of a scale. Patient Educ Couns. 2009;76:272-278.
Field JJ, Knight-Perry JE, DeBaun MR. Acute pain in children and adults with sickle cell disease: management in the absence of evidence-based guidelines. Curr Opin Hematol. 2009;16:173-178.
Darbari DS, Neely M, van den Anker J, Rana S. Increased clearance of morphine in sickle cell disease: implications for pain management. J Pain. 2011;12:531-538.