Daily Press (Sunday)


- By Sara Hutchinson

Heather Avant always dresses up when she goes to the emergency room.

“I’ve been conditione­d to act and behave in a very specific way,” said Avant, 42. “I try to do my hair. I make sure I shower, have nice clothes. Sometimes I put on my University of Michigan shirt.”

It’s a strategy to combat discrimina­tion that the photograph­er from Mesquite, Texas, has developed over a lifetime of managing sickle cell disease, a rare blood disorder that affects an estimated 100,000 Americans, according to the Centers for Disease Control and Prevention. The hereditary condition can affect a person of any race or ethnicity, but Black patients, like Avant, make up the majority of those afflicted in the U.S.

For people living with the disease, a crisis can happen at any time. When it does, their rigid, sickleshap­ed red blood cells become stuck in the blood vessels, blocking flow and causing extreme pain or breathing difficulti­es. A crisis can escalate into life-threatenin­g complicati­ons, such as strokes, seizures and sepsis.

When a pain crisis can’t be managed at home, patients head to the emergency room to get the high dosage of opioids they need, in addition to IVs to help with dehydratio­n or blood transfusio­ns. Yet staffers in emergency department­s — already overextend­ed and grappling with nursing shortages — don’t always have experience in treating the disease. Doctors, amid a raging opioid crisis, remain resistant to prescribin­g the painkiller­s necessary to treat sickle cell crises. So, patients say, they face long delays before receiving care, plus discrimina­tion

and suspicion that they are seeking drugs to get high.

“I have to look like I’m not coming in here off the street looking for medication,” Avant said. “I have to put on an entire show to get you to believe that I need care.”

Years of research have documented the delays. A study published in 2013 found that patients seeking care from 2003 through 2008 at an emergency room for their sickle cell crises waited 50% longer than patients who arrived with broken legs or arms. A 2021 study found that half of sickle cell patients reported having to wait at least two hours before their pain was treated, despite medical guidelines recommendi­ng such patients receive their first dose of pain medication no more than an hour after arriving

at the emergency room.

Medical associatio­ns such as the American Society of Hematology; the National Heart, Lung, and Blood Institute; and the Emergency Nurses Associatio­n have establishe­d guidelines for emergency department-based care of sickle cell pain. And, in 2021, the Emergency Department Sickle Cell Care Coalition, a national collaborat­ion of hematologi­sts, pharmacist­s and nurses, helped launch a point-of-care tool to help providers manage the disease in the emergency room.

But patients and sickle cell experts said those best practices haven’t been widely adopted. A 2020 survey of nearly

250 emergency medicine providers found that 75% of them were unaware of

the NHLBI’s recommenda­tions, first published in 2014, yet 98% felt confident in their ability to treat patients with sickle cell disease.

Emergency room horror stories abound among patients. For Lesly Chavez, 29, a Houston hairstylis­t, her worst experience occurred a few years ago. She said she spent four hours in a waiting room before getting seen.

“And when they finally got to me, they told me they could help with ‘my addiction,’ but they decided that there was nothing that they could do for me,” Chavez said. “They just flat-out said no and sent me home while I was in crisis.”

Chavez said she has since avoided that hospital even though it’s 10 minutes from her home. Now she drives to one 30 minutes away.

 ?? HEATHER AVANT/KFF HEALTH NEWS ?? Heather Avant is careful about what she wears when she has to go to the emergency room for extremely painful sickle cell crises.
HEATHER AVANT/KFF HEALTH NEWS Heather Avant is careful about what she wears when she has to go to the emergency room for extremely painful sickle cell crises.

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