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One under-the-radar consequence of the opioid crisis is that patients with sickle cell disease are finding it more difficult to receive appropriate care when they present to the ED in excruciating disease-related pain. With no objective way to confirm that these patients are in pain, some emergency providers may decline to provide opioids, causing unnecessary suffering and a mistrust of the healthcare system. Experts note that management of sickle cell disease is complex and not well understood by most frontline providers. However, these experts note that opioids remain a first-line therapy for patients who present with pain crises.
At this point, the deadly ramifications of the opioid crisis are well known. The National Institute on Drug Abuse reports that more than 90 Americans die from opioid overdoses every day and that millions more suffer from substance use disorders. However, one consequence that is not discussed often is the undertreatment that many patients with sickle cell disease receive when they present to the ED in excruciating pain from their disease. “There are definitely a lot of new barriers being put into place,” explains Caroline Freiermuth, MD, an assistant professor in the division of emergency medicine at Duke University School of Medicine and a national expert on caring for patients with sickle cell disease. “Not only is the government now getting involved, but insurance companies are cracking down on what medications they will fill and for how long, so we are seeing some patients with sickle cell disease who come to the ED simply because they can’t get their outpatient medications filled.”
Sometimes, the problem is due to an insurance company’s unwillingness to pay, but other times clinicians are skeptical that these patients are really in pain. “It’s a frustrating thing for both the patients and the emergency physicians because there is no objective way to decide whether someone is having a pain crisis,” says Freiermuth, who educates emergency providers on how to manage sickle cell patients who present with pain crises. “There are no lab values that say the patients are not having a pain crisis, and there are no vital signs that rule out a pain crisis, so you really have to take people at face value.”
Contributing to the problem is the fact that while most sickle cell patients will never go to the ED in a given year, less than 5% will go the ED very frequently, Freiermuth shares. “That colors the perceptions of the providers in the ED. Then they think that all patients with sickle cell disease are coming to the ED frequently, and that they only want pain medications.”
Much of the blame for the opioid crisis has been directed at physicians, especially emergency physicians, who have become much stingier about who gets opioids in the ED and who gets a prescription to go home with, Freiermuth observes. But this reluctance can result in unnecessary suffering on the part of sickle cell patients who require opioids to relieve painful episodes that are caused by their disease. Beyond suffering, there may be other consequences. “The more acute pain a patient has, the higher the likelihood that the patient will develop chronic pain,” Freiermuth notes. “Distrust of the medical system is also a big problem, and then patients won’t seek care in the future when they are having pain because of the care they have had in the past.”
There is unsettled science on whether untreated acute pain from sickle cell disease can lead to adverse outcomes, but Freiermuth notes that many complications of the disease tend to occur in the setting of an acute pain crisis. For instance, she points out that acute chest syndrome is the number one cause of death for patients with sickle cell disease, and it frequently occurs in the setting of a pain crisis.
“Right now, the disease-modifying drugs that we have for sickle cell disease can reduce the amount of pain, but they by no means take it away, so opioids are still a first-line therapy for sickle cell disease,” explains Jeffrey Glassberg, MD, MA, an assistant professor of emergency medicine, hematology, and medical oncology, and associate director of the comprehensive sickle cell program at Mount Sinai School of Medicine in New York City. However, he notes that the medical community’s increasing awareness of the dangers associated with the dispensation of opioids is affecting the care of sickle cell patients for whom opioids still are indicated for the treatment of pain.
Glassberg maintains it is important to separate the two different ways that emergency physicians give opioids: by administering them in the ED and by writing prescriptions.
“In terms of prescription writing, doctors are being encouraged to be much more cautious and parsimonious about who they write prescriptions for and how much they write prescriptions for, and that is something I actually support,” Glassberg says. “In today’s climate, people who have legitimate indications to be receiving these medicines should still be receiving them from one doctor who really has the knowledge, the capability, and the training to prescribe these medicines, monitor for signs of trouble, and make sure that people are on the lowest dose.”
Consequently, Glassberg suggests that emergency providers shouldn’t be writing prescriptions for opioids just because someone says they have sickle cell disease, as this task can be managed much more effectively by a provider who regularly oversees the patient’s care. For instance, Glassberg’s sickle cell clinic, which now takes charge of more than 200 adult patients with sickle cell disease, has affected healthcare utilization.
“We have seen substantial reductions in the number of ED visits, the number of admissions, and the length[s] of stay of those admissions,” he says. “One of the things I attribute that to is that we are very aggressive about making sure that our patients always have the lowest possible dose of [opioids] as outpatients.”
Glassberg acknowledges that sometimes that means directing patients to arrive for weekly visits so that they can be evaluated constantly, even though the clinic providers know they will not be reimbursed by payers for weekly visits. However, such frequency enables providers to better titrate the medicines the patients are on and keep close tabs on their sickle cell disease.
“That is the kind of service that patients can get from a very knowledgeable sickle cell provider or someone who knows a lot about pain management,” he says. Therefore, Glassberg encourages emergency physicians to use caution when writing prescriptions for patients who say they can’t access their physician or who don’t visit a physician. “You really have to get them better access to a provider rather than just refilling prescriptions for opioids,” he says.
That said, Glassberg suggests emergency physicians should feel much more open to administering opioids to sickle cell patients while they are in the ED. “Because of the climate, people seem to be more reluctant to give opioids in the ED, but the truth is if you have a patient with sickle cell disease, I think the practice [of administering opioids in the ED] should continue, and in many centers where physicians may be withholding opioids, the practice should increase,” he says. “We should be very aggressive about treating pain in an ED, especially for someone with sickle cell disease.”
A dose of IV morphine that is administered to a patient in the ED is not a bottle of pills that can be diverted or sold on the street, so it is not one of the things that is contributing to the opioid crisis, Glassberg observes. “Sickle cell disease is a rare condition, and these patients unquestionably have many causes for acute and chronic pain,” he says. “And if you are giving them pain medicine in the ED, you are not enabling the opioid crisis.”
A sickle cell crisis is extremely painful, but patients don’t always react to this pain in the way emergency physicians expect, which can cause skepticism that the pain is real, Glassberg explains. “People with sickle cell disease are trained to distract themselves and to use techniques to minimize their experience of pain, so you might have a patient who says he has 10 out of 10 level pain, and yet he is playing on his cell phone,” he says. “That just doesn’t jive with what most physicians see as someone who is experiencing the worst pain of their life, so it creates a problem for the physician-patient relationship because the physician can’t verify if the patient is telling the truth.”
Compounding the problem is the reality that there is a small number of high-use sickle cell patients who tend to present with more psychiatric disorders. These patients may be coming to [the ED] every month, every week, or every day. Physiologically, it is not possible for a patient to experience a sickle cell crisis every day, Glassberg notes.
When such a high-use sickle cell patient presents to the ED, the physician’s approach may be that he or she is not going to treat the patient’s pain. However, while a decision to withhold opioids may be the correct decision in some cases, such a call never should be made on the fly by an emergency physician, Glassberg stresses. “The first thing you do when someone is utilizing in a way that you think is inappropriate is make a very careful assessment of why that might be, and if it is a reversible cause or a treatable cause, then you address that reason,” he says.
For example, Glassberg notes that patient might be visiting the ED every day because he has avascular necrosis in his hip, which is a condition associated with sickle cell disease that can cause constant pain. “The physician may conclude that the patient could not be having a sickle cell pain crisis, but the truth is the patient is having legitimate pain, which is being caused by a complication of sickle cell disease,” he says. “If it is severe enough, some of our patients get a hip replacement.”
Further, one of the most common reasons why sickle cell patients may frequent the ED is because they experience small infarcts in their brains, which can affect their executive functioning, Glassberg says. “It might be a patient who can’t follow a treatment plan. You tell them to take their medicine or to go to the doctor, but they don’t do anything,” he says. “They just come back to the ED the next day because they are feeling pain, and this is because they are basically having a traumatic brain injury from their sickle cell disease.”
As these cases suggest, Glassberg notes that providers must do their best to determine what a high-use patient’s unmet need is before concluding that the patient should not be given pain medication. He refers clinicians to research on caring for this specific patient group.1
“In some very rare cases, you may decide after careful consideration that for this individual, opioids are making things worse and that you need to stop using IV meds or switch to something else,” he says. “But what we are seeing occasionally is the emergency physician deciding in the moment that [he or she] doesn’t think someone is having sickle cell pain, and that is something that really should never be done.”
Certainly, conducting careful assessments on high-use patients requires resources, Glassberg acknowledges. “You have to establish some kind of system to get the ultra-utilizing patients the help they need because it is too frustrating to providers to take care of patients with a high level of need without any help, and [providers] will then transfer their frustration to everyone they see with sickle cell disease,” he explains. “So the leadership of the institution, the hematologists, and the outpatient providers have to put in place things to mitigate the effects of extremely high-utilizing patients.”
While the management of sickle cell patients is complex and not well-understood by most frontline providers, there are steps that healthcare systems can take to facilitate the care of these patients when they present to the ED. For example, practitioners who take care of patients with sickle cell disease in the Duke Health System have developed individualized care plans for these patients that are embedded in the health system’s electronic medical record (EMR).
“When these patients come to the ED, we can just pull the care plans up, and they indicate what doses the patients should receive for an acute pain crisis,” Freiermuth explains. “That makes it very easy for our physicians in the ED to say they are just going to follow the plan.”
Duke hematologists also have started to make themselves available for consults to primary care providers who often need expert guidance in managing patients with sickle cell disease. The idea is to enlarge the group of providers willing to manage patients with this complex condition, thereby improving the care of this patient population while also reducing the need for ED visits.
“Our Duke Sickle Cell Center sees about 600 patients who drive from all over the state to be seen by our hematologists because it is a place where they can get care,” Freiermuth notes. “Many patients use hematologists as their primary care provider, which is not ideal, but it is the way things are because they don’t have access to a [traditional] primary care provider who is comfortable managing their disease.”
The dearth of providers with the knowledge and experience to manage patients with sickle cell disease is partly because the life expectancy for patients with the disease has more than doubled in recent years. “In the 1970s, the life expectancy for a person with sickle cell disease was 18, so there wasn’t a need for adult providers. The only physicians who would care for sickle cell patients would be pediatricians,” Glassberg explains. “Now, people live much longer, and it is purely because of comprehensive care and early identification.”
The most recent studies on the issue note that the average lifespan for people with sickle cell disease is 42 years for men and 48 years for women, but Glassberg notes that those figures were compiled before clinicians started using hydroxyurea, a drug that helps reduce the incidence of pain crises as well as the need for transfusions in sickle cell patients.
Glassberg strongly suspects the drug has extended the lifespan of sickle cell patients further, creating a greater need for experienced providers. “It is only recently — over the last 40 years — that sickle cell disease has become an adult disease, so we don’t have a lot of adult hematologists who have expertise in this disease, and we don’t have a lot of internal medicine physicians, and this is a problem,” he says.
Until that problem is solved, patients with sickle cell disease who are experiencing pain crises may have few alternatives other than to present to the ED. Glassberg strongly advises clinicians to believe these patients when they say they are in pain.
“You should take the patient’s words at face value,” he says. “If you are concerned about contributing to the opioid epidemic, then you can be cautious about the prescriptions you write; you can counsel the patient that you are happy to treat their pain [via IV] right there, but that you need them to follow up with somebody who is going to follow them long term ... so you won’t be providing prescriptions for more than a day or two.”
These steps, along with mechanisms in place to help emergency providers care for high-use patients, would go a long way toward improving the care of sickle cell patients, Glassberg explains.
Freiermuth advises emergency clinicians to educate themselves about sickle cell disease, and realize that it is a chronic disease process. “Patients are still dying early because of the high morbidity and mortality for their disease. These patients have strokes at early ages, and they have kidney failure by their 30s, so their sickle cell disease causes a lot of underlying issues,” she says.
Further, when it comes to pain, Freiermuth encourages clinicians to give patients the benefit of the doubt. “You may get burned by a few patients, but I would rather get burned by a few than undertreat the many.”
Financial Disclosure: Physician Editor Robert Bitterman, Author Dorothy Brooks, Editor Jonathan Springston, Executive Editor Shelly Morrow Mark, and Editorial Group Manager Terrey L. Hatcher report no consultant, stockholder, speaker’s bureau, research, or other financial relationships with companies having ties to this field of study.