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By Stephanie Stephens
We hear so frequently about opioid overuse in the United States and its devastating consequences, but there is one chapter of this story that doesn't always make news headlines: not everyone who comes to the emergency department (ED) seeking help for pain is there because they have a substance abuse addiction. People with sickle cell disease (SCD) are an example of individuals who go to the ED seeking relief from excruciating pain.
When Blood Cells Get “Stuck”
Nearly 100,000 Americans have SCD,1 a lifelong disorder present at birth. It occurs most often in blacks or African Americans, but also can occur among many different ethnicities, such as Indian, Middle Eastern, Hispanic, and Mediterranean.2
So, what is sickle cell? For those with the disease, some red blood cells develop in the shape of a sickle. This makes the cells prone to “getting stuck” and blocking blood flow in the body, which also cuts off the oxygen supply in certain areas.2
The cut off oxygen supply can cause sudden, intense, and debilitating pain during what's called a vaso-occlusive crisis. These crises occur throughout a person’s life, and some people with SCD may have six or more episodes per year.3 There's no predicting these episodes, and the pain is so intolerable that patients understandably demand immediate relief. Doctors may utilize opioid analgesics such as codeine, morphine, oxycodone, and fentanyl, since opioids are the recommended treatment for these crises.
“They Don't Believe Me”
Medical literature shows health care providers may doubt patients' descriptions of their pain during a vaso-occlusive crisis. Doctors may also question when a patient shares which pain medications have worked best in the past.4 In fact, research shows that people with SCD misuse opioids less than people with other chronic pain conditions and less than the general population.5
Equally troubling, a study in The Clinical Journal of Pain found that people with SCD wait an average of 30 minutes longer in an ED to receive pain medication, when compared to other patients who have similar levels of severe pain due to conditions such as kidney stones.6
The Optimal Choice for Now
It's important to be strategic about use of the words “tolerance and dependence” in the same sentence with “sickle cell disease” and “opioid use.” Repeated use of opioids may encourage tolerance when patients don't respond to the drug the way they did initially and then need higher doses. This is not the same as dependence, which is when a person stops taking a drug and experiences withdrawal. It's also not the same as addiction, when someone uses a drug and can't stop.
Until a new treatment option is made available, ED physicians will likely prescribe opioids when nothing else is as effective for the pain of a vaso-occlusive crisis. The drugs do their job in stemming intense discomfort and pain, but as we already know, they have consequences, especially in the long term.
More Work to Be Done
Researchers aren't resting in their pursuit of a cure for SCD or in trying to better understand the relationship between the disease and the use of opioids. For example, a 2016 study7 in the American Journal of Preventive Medicine shared that adult patients with SCD who were treated long term with opioids often fared worse in measures of pain, fatigue, and curtailed daily activities than those not on long-term opioids.
A late 2017 study8 in The Journal of Pain, although small, found a link between negative emotions, like sadness and anxiety, and higher opioid use in people who reported low levels of pain. The study's lead author noted in a prepared statement that “the way we think about pain is associated with opioid use even if our pain levels are low.” Researchers wanted to demonstrate ways to track and define risk for prescription opioid misuse when patients experience daily pain.
Teamwork and an open line of communication between a patient and their primary care provider can help keep acute events at bay, starting with a pain management plan. Patients should get to the ED at the first sign of a pain crisis, and bring their validated pain plan with them on paper or digitally. It's also smart to summarize previous ED visits on a separate and accessible document. All of these measures help those working in an ED understand and validate that yes, even though I can't see “it,” this patient has SCD and no, they aren't just here to get opioids.
1 Sickle Cell Disease. Pfizer Rare Disease. https://www.pfizer.com/health-wellness/disease-conditions/rare-diseases/.... Accessed September 5, 2018.
2 About Sickle Cell Disease. Sickle Cell Disease Coalition. http://www.scdcoalition.org/sickle-cell-disease.html. Accessed September 5, 2018.
3 Yale SH, Nagib N, Guthrie T. Approach to the vaso-occlusive crisis in adults with sickle cell disease. Am Fam Phys. 2000;61(5):1349–1356, 1363–1364.
4 Ballas SK. The sickle cell painful crisis in adults: phases and objective signs. Hemoglobin. 1995;19(6):323-333. doi:10.3109/03630269509005824.
5 DeBaun MR, Vichinsky EP. Vaso-occlusive pain management in sickle cell disease. UpToDate. https://www.uptodate.com/contents/vaso-occlusive-pain-management-in-sick.... Accessed September 5, 2018.
6 Lazio MP, Costello HH, Courtney DM, et al. A comparison of analgesic management for emergency department patients with sickle cell disease and renal colic. Clin J Pain. 2010;26(3):199-205. doi: 10.1097/AJP.0b013e3181bed10c.
7 Sickle Cell and Opioids. Hopkins Medicine. https://www.hopkinsmedicine.org/news/publications/hopkins_medicine_magaz.... Accessed September 5, 2018.
8 Finan PH, Carroll CP, Moscou-Jackson G , et al. Daily opioid use fluctuates as a function of pain, catastrophizing, and affect in patients with sickle cell disease: an electronic daily diary analysis. J Pain. 2018;19:46-56.