Heather Avant always dresses up when she goes to the emergency room.<\/p>\n

\u201cI\u2019ve been conditioned to act and behave in a very specific way,\u201d said Avant. \u201cI try to do my hair. I make sure I shower, have nice clothes. Sometimes I put on my University of Michigan shirt.\u201d<\/p>\n

It\u2019s a strategy to combat discrimination the 42-year-old photographer in Mesquite, Texas, has developed over a lifetime of managing her sickle cell disease, a rare blood disorder that affects an estimated 100,000 Americans. 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.<\/p>\n

For people living with the disease, a sickle cell crisis can happen at any time. When it does, their rigid, sickle-shaped red blood cells become stuck in their 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.<\/p>\n

When a pain crisis can\u2019t be managed at home, patients head to the ER to get the high dosage of opioids they need, in addition to IVs to help with dehydration or even blood transfusions. Yet staffers in emergency departments \u2014 already overextended and grappling with nursing shortages<\/a> \u2014 don\u2019t always have experience in treating the rare disease. Doctors, amid a still-raging opioid crisis, remain resistant<\/a> to prescribing the painkillers necessary to treat sickle cell crises. So, patients say, they face long delays before receiving essential care, plus discrimination and suspicion that they are seeking drugs to get high.<\/p>\n

\u201cI have to look like I\u2019m not coming in here off the street looking for medication,\u201d said Avant. \u201cI have to put on an entire show to get you to believe that I need care.\u201d<\/p>\n

Years of research have documented the delays. A study published in 2013<\/a> found that patients seeking care from 2003 through 2008 at an ER for their sickle cell crises waited 50% longer than patients who arrived with broken legs or arms. A study published in 2021 found that 50% of sickle cell patients reported having to wait at least two hours<\/a> before their pain was treated, despite medical guidelines recommending such patients in crisis receive their first dose of pain medication no more than 60 minutes after arriving at the ER.<\/p>\n

Medical associations such as the American Society of Hematology<\/a>, the National Heart, Lung, and Blood Institute<\/a>, 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> a point-of-care tool<\/a> to help medical professionals manage the disease in the ER.<\/p>\n

But patients and sickle cell experts said those best practices haven\u2019t been widely adopted. A 2020 survey<\/a> of nearly 250 emergency medicine providers found that 75% of them were unaware of the NHLBI\u2019s recommendations, first published in 2014, yet 98% felt confident in their ability to treat patients with sickle cell disease.<\/p>\n

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Still, ER horror stories abound among adults with sickle cell disease. 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.<\/p>\n
\u201cAnd when they finally got to me, they told me they could help with \u2018my addiction,\u2019 but they decided that there was nothing that they could do for me,\u201d Chavez said. \u201cThey just flat-out said no and sent me home while I was in crisis.\u201d<\/p>\n
Chavez said she has since avoided that hospital even though it\u2019s 10 minutes from her home. Now she drives to an ER 30 minutes away.<\/p>\n
Chavez, who is Hispanic, said she confronts \u201cdoubt everywhere I go\u201d because sickle cell disease primarily affects Black Americans. (Those who are Hispanic can be of any race.)<\/p>\n
Paula Tanabe<\/a>, a professor of nursing at Duke University who has spent decades researching ways to improve care for sickle cell patients, said a confluence of factors adds to the racial bias patients may face.<\/p>\n
\u201cEmergency rooms are incredibly overcrowded, at rates that we have never seen before, and that\u2019s for everyone,\u201d said Tanabe.<\/p>\n
Legislators are trying to help. A federal bill introduced in June<\/a> would allocate $8.2 million annually for five years to a program that trains doctors on best practices for caring for sickle cell patients. Another, introduced this spring<\/a>, would provide funding for community organizations working to spread awareness about the condition and give student loan relief to medical providers who commit to working on the disease. Some state legislatures have established sickle cell task forces to improve physician education and care coordination.<\/p>\n
Advocates for sickle cell patients said investment in data collection to track the disease is also important. Although the Centers for Disease Control and Prevention estimates that some 100,000 Americans have it, the true number is unknown. That\u2019s because no national system exists to collect data on sickle cell, unlike other conditions<\/a> such as diabetes, cancer, and Alzheimer\u2019s.<\/p>\n
\u201cI\u2019m 32 and we\u2019ve been saying it\u2019s 100,000 my entire life,\u201d said Quannecia McCruse, who co-founded the Sickle Cell Association of Houston<\/a>. \u201cI know there\u2019s more. I know people are going uncounted.\u201d<\/p>\n
Quannecia McCruse (center), who has sickle cell disease, co-founded the Sickle Cell Association of Houston. For people living with the disease, a sickle cell crisis can happen anytime and be excruciatingly painful, even life-threatening. Still, ER wait times are long and often frustrating. Patients like McCruse must balance advocating for themselves during bouts of pain against the need not to irritate or alienate hospital staffers. Here, she stands between friends Faith Adjei-Sarpong (left) and Precious Uwaezuoke at the Sickle Cell Houston Walk in September.(Alex Arvie)<\/span><\/figcaption><\/figure>\n
Eleven state-led data collection programs currently exist<\/a> and, in February, the CDC opened a new grant application for additional states. Improved data would allow funding to be allocated toward the areas with the greatest need, sickle cell patient advocates said.<\/p>\n
Texas had an opportunity to join those efforts. This spring, the state legislature passed a bill<\/a> with broad bipartisan support to create a sickle cell patient registry, but Republican Gov. Greg Abbott vetoed it, saying it would compromise patient privacy.<\/p>\n
\u201cThat was a bad excuse,\u201d said McCruse. \u201cWe have a cancer registry already, and everyone\u2019s information is safe. That registry would have gone a long way to help.\u201d<\/p>\n
While progress grinds slowly, patients like McCruse say they\u2019re forced to balance advocating for themselves during bouts of excruciating pain against the need not to irritate or alienate hospital staffers.<\/p>\n
\u201cIt feels like someone is taking a Taser and shocking the crap out of me. Or when it\u2019s really bad, and it feels like shards of glass are just moving through my veins,\u201d said the mother of two. \u201cIt\u2019s very, very painful. And you\u2019re telling somebody whose body is torturing them that it\u2019s not that bad?\u201d<\/p>\n
Alexis Thompson<\/a>, a hematologist who treats sickle cell patients at the Children\u2019s Hospital of Philadelphia, said she works with her pediatric patients to develop self-advocacy skills. But sometimes that backfires.<\/p>\n
\u201cThe great irony is patients who are well informed and capable of self-advocating are being accused of being manipulative, because they are capable of articulating very clearly what\u2019s effective for them down to the name of the medication or the absolute dose,\u201d Thompson said.<\/p>\n
Sickle cell experts recommend that doctors adhere to a patient\u2019s individual pain plan, if available. Thompson said those plans, which document patients\u2019 diagnoses alongside a recommended medication and dosage, can be uploaded to online portals that patients can pull up on their cellphones when visiting an ER to verify what they need.<\/p>\n
Patients such as Avant hope such steps can help decrease their ER waits while easing their anxiety about seeking emergency care.<\/p>\n
\u201cI don\u2019t fear dying,\u201d said Avant, \u201cbut I do fear dying in the hospital.\u201d<\/p>\n
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