Sickle Cell in the ED: Old Therapies, New Strategies

— With treatments unchanged in decades, docs seek to improve delivery

MedpageToday

SAN DIEGO -- Sickle cell disease stands out in the emergency room, and not just because patients with the condition often seek care again and again. It's also one of the few conditions whose treatment has remained the same for 5 decades. Still, there's plenty of room for doctors to update their strategies, an emergency physician told colleagues this week.

While hydration, pain medication, and oxygen are as crucial as ever, physicians should consider new treatment protocols and modern pain drugs, said Jon Mark Hirshon, MD, MPH, PhD, of the University of Maryland School of Medicine in Baltimore.

"We can make a difference in their lives by changing practice, and treating them better," he said during a presentation at the annual scientific meeting of the American College of Emergency Physicians.

Emergency department visits by patients with sickle cell disease are common. A 2016 California study found that among 4,636 patients with sickle cell disease, 88% visited the ED over a 9-year period from 2005-2014, and they averaged two visits a year. In one year, 2005, 9% had 4-10 visits and 3% had at least 11 visits.

The main acute complication is vaso-occlusive crisis. According to Hirshon, patients with sickle cell disease also come to the ED with complications such as fever, chest pain (acute chest syndrome is a major risk in these patients), gallstones (which affect an estimated 70%-75% of adult patients), priapism, and more.

Hirshon recommends that emergency departments triage patients with sickle cell disease at level 2. "They need to be assessed quickly," he said. "Before you put them in a room and ignore them, make sure they don't have something life-threatening."

It's important to teach staff to prioritize sickle cell disease cases, he said, and they should be flagged within the electronic medical record system if possible. "You have to know there's a patient with sickle cell disease," he said. "Look for signs of complications. Make sure they don't have acute chest syndrome and treat pain aggressively."

He recommended that physicians begin analgesic management within 30 minutes of triage or 60 minutes of registration. "Administer IV opioids every 15-30 minutes until pain relief is obtained," he said. "One of the things I've learned in this process is that when they come in with pain, it can make a difference if we can get them taken care of quickly, get them started."

Intranasal fentanyl is also an option, he said. Another study in 2016 found that it appeared to produce lower pain scores in children with vaso-occlusive crisis.

What about drug seekers? "The number of opioid abusers within this patient population is roughly the same as the general population," Hirshon said. "But we see [these patients] all the time, so our sample is a bit biased."

Ultimately, he said, boosting the priority of sickle cell disease cases and treating pain effectively will lead to better care and fewer inpatient admissions. "You get to almost half of them going home," he said, instead of needing further hospital care.

  • author['full_name']

    Randy Dotinga is a freelance medical and science journalist based in San Diego.

Disclosures

Hirshon discloses consulting for Pfizer and accepting travel reimbursement from Global Blood Therapeutics.