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my Mason had toughed it out for hours one day this past July, trying warm soaks and heating pads and deep breathing to soothe pain that felt like her bones were being sawed with a rusty blade.

She knew this was a life-threatening emergency of sickle cell disease, in which her misshapen red blood cells were getting stuck in her blood vessels like tree limbs in a storm sewer. But she delayed going to the emergency room; previous visits hadn’t gone well.

Just before midnight, Mason, 34, finally had her boyfriend drive her to a Mobile, Ala., hospital. She told the triage nurse that she was having one of the worst sickle cell crises of her life and that she was off the far end of the 1-to-10 pain scale. She was told to wait.

As the hours passed, Mason begged her boyfriend to take her to another hospital, then passed out from the pain. She awoke, she told STAT, to her boyfriend’s shaking her and pleading with her to stay with him. I can’t do this anymore, Mason whispered.

Daylight broke. Around mid-morning, an ER nurse told her, you know, we’re not a pain clinic. She gave Mason a small dose of the narcotic Dilaudid.

“Every time, it’s a battle,” Mason said about the ER, where the staff suspected her of faking her condition in order to score opiates and viewed her as a non-emergency because she “only” had pain. “Nobody cares about people with sickle cell.”

The U.S. health care system is killing adults with sickle cell disease. Racism is a factor — most of the 100,000 U.S. patients with the genetic disorder are African-American — and so is inadequate training of doctors and nurses. And the care is getting worse, sickle cell patients and their doctors said, because the opioid addiction crisis has made ER doctors extremely reluctant to prescribe pain pills.

STAT interviewed 12 sickle cell patients who described the care they received and didn’t receive. They were old and young, men and women, scattered from coast to coast, some with jobs or attending school and some too sick to do either. Two who wanted to tell their stories were unexpectedly hospitalized and too weak to talk to a reporter.

In hospitals, sickle cell patients are typically treated by generalists who know little about the disease and patients’ desperate need for pain relief. At one hospital, researchers found that sickle cell patients waited 60 percent longer to get pain medication than other patients who reported less severe pain and were triaged into a less serious category.

Outside the hospital, fewer than one-quarter of adult patients who need it get the drug approved for preventing sickle cell crisis, according to the American Society of Hematology.

Only 20 percent of family physicians say they feel comfortable treating sickle cell disease, a 2015 survey of more than 3,000 such physicians found, leaving many patients without routine, preventive care. They are therefore more likely to seek care in ERs during an acute sickle cell episode, called a vaso-occlusive crisis, in which an inadequate blood supply triggers excruciating pain and damages vital organs.

The result of all these failures is “a breakdown in the system of care for these patients,” said Marsha Treadwell, of Children’s Hospital Oakland, who works on programs for adults with sickle cell. “People are needlessly dying.”

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And they are dying in droves, of vaso-occlusive crises and organ failure and chronic kidney disease and more. The ultimate cause is eponymous sickle-shaped blood cells. They don’t deliver oxygen to organs and tissues as healthy blood cells do, causing slow, inexorable damage over decades.

But an immediate cause of early death is a catastrophic failure to give sickle cell patients competent care. The mortality rate for adults with the disease cell has risen 1 percent every year since 1979, the hematology society reported in 2016. Half of adult sickle cell patients are dead by their early 40s. (Children fare better.)

Every doctor who cares for adult sickle cell patients “knows of one or more who were not treated properly and died,” said Dr. Keith Quirolo, a retired pediatric hematologist in California who saw adult patients because no other physicians would accept them.

Each and every one of the patients STAT reached, through doctors and social media, sounded exhausted, even defeated. Some spoke of hospital and other committees they had served on, only to see the treatment guidelines and educational materials they produced ignored. Most spoke about fellow patients who’d recently died — a man in his 30s, a woman in her 40s — and who might have been saved with competent care.

By the time Mason was given pain medication during her July episode, she said she was struggling to breathe. She was too weak to even boost herself onto a bedpan; her boyfriend lifted her. He had to buttonhole a doctor and insist he treat Mason.

“ You go into the emergency room and you don’t know if you’re going to leave alive.”

Amy Mason, sickle cell patient

The Mobile Infirmary hospital, where Mason was treated, said it couldn’t comment on the care of a specific patient. Registered nurse Kelley Hicks, director of emergency services, said, “We do our best” to give a bed to “each patient that arrives to the ED so that their care may begin as soon as possible. … Our goal is to always provide high quality, evidence-based care to all of our patients.”

But Mason felt they fell short. “You go into the emergency room and you don’t know if you’re going to leave alive,” said Mason, who lost two friends to the disease this summer. “We are dying way too young.”

Mobile Infirmary hospital
The emergency room at the Mobile Infirmary hospital in Mobile, Ala., where Mason was treated in July. Sharon Steinmann for STAT

‘I always dress professionally’

Physicians are taught that signs of drug seeking include asking for opioid medications by name, making multiple visits for the same complaint, having symptoms “out of proportion” to what an examination shows, and seeking treatment at a hospital. That means four strikes against patients seeking care for acute sickle cell crises in an ER.

The opioid crisis has made physicians even more vigilant and cautious, as everyone from the Centers for Disease Control and Prevention to state regulators to some hospitals have tightened guidelines for dispensing the addictive pain medications.

Racial bias makes matters worse. Numerous studies have found that black Americans receive less pain treatment than whites, often because physicians and nurses hold false beliefs such as “black skin is thicker.”

“If you are African-American and are in the ED for pain, you are almost automatically tagged as drug seeking,” Quirolo said. In one study, 63 percent of nurses surveyed said many patients with sickle cell are addicted to opioids. In fact, their rates of addiction are no higher than the general population’s, said Dr. Alexis Thompson, president of the hematology society. They may even be lower: Although blacks make up 13 percent of Americans, they accounted for only 8 percent of opioid overdose deaths in 2015, the most recent year studied.

Knowing the attitudes they’ll encounter, patients therefore prepare for the ER as for a job interview. “I always dress professionally,” said Wanda Williams, 67, a retired school administrator in the Bay Area: nice shoes, “interesting earrings,” every hair in place, and Vogue-worthy makeup. “It’s crazy that you, as an African-American, have to do this so you aren’t treated like a drug addict.”

Such efforts to increase the chance of receiving appropriate treatment often fall short. Several ER physicians told STAT that everything would go fine if sickle cell patients brought their “care plan” — a document from their doctor showing their diagnosis, and what drugs and intravenous hydration and possibly transfusions they should receive in a sickle cell crisis. “But what we find is that emergency physicians and hospitalists [who treat inpatients] don’t like to follow these plans,” said social worker Kim Major, who oversees the sickle cell clinic at Oakland Children’s.

The plans’ descriptions of pain medication instead raise suspicions. “We want to have informed, empowered patients, so we’ve taught them to be aware of what their medical needs are, what they take, and at what dose,” said Thompson. But knowing so much in such detail, she said, “is perceived as drug seeking.”

James Griffin, 36, knows that if he suffers a sickle cell crisis on a Saturday night, when ER doctors are extra suspicions of black men arriving in need of pain medications, he needs to hold on until morning. He also knows to be cooperative even at a sacrifice to his health.

“If the doctor suggests one pain medication, even when it’s one that I had a respiratory reaction to, I don’t suggest something else,” said Griffin, who lives in Phoenix and is training to become a medical assistant. “If I do, I might not get anything” because doctors are suspicious of patients who know “too much” about what pain medication they need, he said.

Emergency physician Dr. Jon Hirshon of the University of Maryland explains the wariness. “When a patient tells me that this dose of this pain medicine works for them, I need to have some type of objective information to support that,” he said. “If someone comes in off the street I don’t know what that’s based on.” There is no objective measure of pain, and because sickle cell patients build up tolerance to medication, their doses can shock physicians.

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Nevertheless, Hirshon said, “The vast majority of the individuals with sickle cell disease who go to the emergency department get excellent care.”

He and other emergency physicians feel they are in an impossible position, made worse by the opioid crisis and the resulting crackdown on irresponsible prescribing of the addictive drugs. “A lot of sickle patients are chronic pain patients, and state guidelines say you don’t treat chronic pain in the ED,” said Dr. Howard Mell, an emergency medicine physician in Ohio and spokesman for the American College of Emergency Physicians. “These patients are like most humans: Some of them are addicts. I have to verify the need for these powerful medications.”

If he makes the wrong call, Mell said, “I feel genuinely awful: 99 out of 100 [sickle cell] patients will have a legitimate need [for the pain medication they request], but the 1 in 100 could cost me my license.” While state regulators would be unlikely to discipline a doctor for one such mistake, many physicians voice this fear.

Mell tries to determine whether the pain is genuine, he said: “If I don’t know this patient, and she tells me her knees and ankles are killing her and this is her typical sickle cell crisis, if there’s no increase in blood pressure or pulse, I’ll question it. Emergency physicians are pretty good at sussing this out.”

In healthy people, blood cells are round and flexible. But in people with sickle cell disease, blood cells are deformed and cause a range of health problems. Hyacinth Empinado/STAT

Fatal consequences

Providing competent treatment in the ER is about more than alleviating suffering. If patients in sickle cell crisis don’t get treated with appropriate quantities of fluids, medication, oxygen, and more, the consequences could be fatal.

Yet, ignorance of sickle cell among emergency nurses and physicians is well-documented. In one study led by emergency nurse Paula Tanabe of Duke University, the average score on a test of basic emergency care for sickle cell patients was 65.

Adrienne Shapiro, an advocate for patients with sickle cell disease in California, said she gets frequent calls like this from patients in the ER or their families: Come as quickly as you can. Last February, it was Natasha, sobbing.

Guidelines from the National Institutes of Health, in effect since 2014, call for patients to be given pain medication within an hour of registering in the emergency department, with additional doses until the pain is controlled. Natasha (whose family asked that she not be further identified) had not received any medication during the five hours and counting since she had arrived at an ER in southern California, nor had she been given fluids or oxygen.

Asked by Shapiro why Natasha, in her 40s, had not been treated, the ER physician said it was because Natasha had been there the day before, also complaining of pain.

“You do understand that she has sickle cell?” Shapiro asked. The doctor was adamant that she would treat Natasha’s pain only if she also agreed to a blood transfusion as an indication that she really had sickle cell disease.

Because Natasha had had so many transfusions, Shapiro explained to the doctor, any more could cause a fatal immune response. Natasha was eventually treated with fluids and pain medication and sent home.

Two days later she was admitted to another hospital, Shapiro said. Within 18 hours, she was dead of sepsis, a systemic inflammatory response to infection that is common in sickle cell. It’s impossible to know if she would have survived with faster ER care, but pain causes the blood vessels to constrict, exacerbating the oxygen deprivation to vital organs caused by the disease.

‘There’s no money in it’

There is no surefire way to avoid sickle cell crisis and therefore the ER, but receiving routine care helps. That is easier said than done.

“We don’t know what percentage of sickle cell patients have an adequate primary care provider,” said Thompson, the hematology society president. “But it’s safe to say a substantial number don’t.”

In part, that’s because sickle cell disease is complicated. Caring for patients requires knowledge of, and access to specialists in, cardiology, pulmonology, nephrology, and more. But another reason is that “most primary care doctors don’t know how to take care of these patients,” said Duke’s Tanabe. Medical schools devote an hour or two to sickle cell disease; unless they train in a city, interns and residents might never see a case.

“We don’t know what percentage of sickle cell patients have an adequate primary care provider. But it’s safe to say a substantial number don’t.”

Dr. Alexis Thompson, president of the American Society of Hematology

“That immediately puts into the mind of our future doctors and nurses, OK, this isn’t important,” said Marissa Cors, 40, a patient in southern California. “They don’t take it seriously. I once heard a doctor say, ‘Sickle cell is said to cause great pain’ — as if that were a rumor we started and not a medical fact.”

Cassandra Trimnell, 30, president of the education and patient-support site Sickle Cell 101, has had doctors ask her, “How long have you had sickle cell?” As an inherited disorder, it is present from birth.

Even the specialists who are supposed to treat sickle cell, hematologists, often don’t want these patients. “If you’re a hematologist, there’s no money

Gene Therapy Sickle Cell
A sickle cell (left) and normal red blood cells of a patient with sickle cell disease. Janice Haney Carr/CDC/Sickle Cell Foundation of Georgia via AP

in it,” Quirolo said. “The patients are complicated and hard to take care of, and it’s very time-consuming.” Only about five physicians in California, which has an estimated 7,000 sickle cell patients, specialize in treating adult sickle cell patients, he said, “and this is the way it is across the country.”

For hematologists, treating blood cancers such as leukemia is much more lucrative. “Let’s be honest, that’s where the money is,” said Dr. Jane Hankins, a sickle cell expert and hematologist at St. Jude Children’s Research Hospital in Memphis. “And hematology training isn’t focused on sickle cell disease.”

Wanda Williams tried to get treated at a center that specializes in sickle cell, but her insurer told her she had to instead choose an in-network hematologist.

“I called and described my situation, and they said we don’t know what to do with you,” she recalled. “I’ve had doctors say, ‘I don’t take care of sickle cell patients. They’re too much work, and they die.’ I have to sell myself in order to get someone to take me as a patient.” She now drives 30 miles each way to a hematologist.

One benefit of routine care from a physician who knows her way around sickle cell disease is receiving hydroxyurea, arguably the most significant breakthrough in sickle cell treatment ever. The oral drug increases healthy forms of oxygen-carrying hemoglobin, resulting in fewer pain crises, fewer transfusions, fewer emergencies, and less organ damage.

But many physicians are leery of hydroxyurea. It was originally, and still is, a cancer drug. It can reduce the number of white blood cells, so patients require blood tests every month or two. To work well, it must be given at the maximum tolerated dose, which patients build up to over about six months.

“It’s not a rocket science medication to manage,” said Treadwell, of Oakland Children’s. “But primary care physicians just don’t want to prescribe it.”

As a result, sickle cell patients who might have avoided vaso-occlusive crises and the ER wind up there and, often, as hospital inpatients.

There, they are typically treated by hospitalists, a specialty created about 20 years ago so physicians wouldn’t have to leave their offices to care for their hospitalized patients.

“Hospitalists know a little bit about everything, but with sickle cell, we’re talking about a multi-organ disease,” said Yvonne Carroll, director of patient services in the hematology department at St. Jude.

After her last vaso-occlusive crisis, Trimnell was admitted to a Florida hospital. Her mother told the hospitalist assigned to her that Trimnell needed to be transfused, but he refused, saying he didn’t understand how a transfusion would help. It’s a standard intervention.

“I was left for days with my hemoglobin dropping,” Trimnell said. Her doctor in California phoned the hospitalist at 3 a.m. explaining the need for a transfusion, which she finally received. “If he hadn’t insisted I would have died,” she said.

A number of efforts are underway to fix a system that is letting patient after sickle cell patient die. Goals include improving ER care, including by educating staff about the disease. But patients and doctors alike told STAT that the failings in clinical care won’t be solved without addressing racial prejudice.

“I just think,” said Duke’s Tanabe, “you can figure out a way to take care of sickle cell patients who come to the emergency department, once you get past the attitude of ‘they’re black and they’re addicts.’”

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  • Thank you for such an intellectually and empathetic portrayal of our challenges in living with a lifelong chronically painful existence. I live outside the USA and have a dedicated Hematologist who has provided flawless care and attention to my condition. It pains me to know that at least two or more people I know of pass away due to medical negligence and I believe there should be better training across the US and legislation that make it a criminal offense to deny people categorized with a chronic illness that proper pain medication and other procedures to save their lives. In addressing the racial aspect of this, specifically the statement that, “There is no money, too much work, and they all die anyway”, perspective of how we a re seen in American emergency rooms, you have hit the bulls-eye. If any health professional looks at our condition as a “medication addict” seeking person, they don’t deserve to be in that profession. More awareness is needed to prolong the life of those with Sickle Cell Anemia. I would hate to know that I am in the last decade of my life because the ignorance and intellectual abandonment of what I am suffering from is mostly based on biases.

  • This article is filled with truth; substandard healthcare/racial bias for patients with Sickle Cell runs rampant and people in positions of authority to make changes sit in silence.
    Adult patients with Sickle Cell Anemia are ignored,disrespected and intentionally made to suffer. The transition from pediatric care to adult care is NOT being done. I
    watched my son slip away right before my eyes…sent home from his Dr’s office on a Friday….rushed to hospital Monday..and dead within 36 hours. The horror I witnessed by staff in that 36 hours and on many other occasions is too much to express
    My condolences to all families who were forced to say goodbye to their loved ones much too soon.
    Thank you for the courage it took to write this article. God bless everyone

  • I could not agree more with your write-up and the inadequacies plaguing access to quality care for patients with SCD in the US. As a Nigerian who migrated to the US in 2009, I can state with confidence that care for SCD patients experiencing vaso-occlusive crisis is better in Nigeria, a third world country.
    I am glad to see that challenges faced by SCD patients are being focused on in recent times. I hope the attention recent advances in SCD treatment is bringing to the disease will help in reducing hurdles to adequate care.
    Another area where SCD patients are being sidelined is in the insurance industry. Patients with diabetes and heart failure are able to get life insurance albeit at slightly higher rates than the general population. However, I do not know of similar considerations being given to patients with SCD.
    That said, I just moved to Thousand Oaks area in California and would like to connect with the SCD advocate cited in this piece in order to determine best option for a PCP seeing/treating patients with SCD.
    Overall, more voice needs to be given to the plight of SCD patients.
    Thank you!

  • Need to get a hard copy of this article. I am the mother of a sickle cell adult child. Battling this same situation at his hospital he attends for treatment. They are assuming he is seeking drugs when they have no clue of the pain sickle cell adults experience. Need to get the word out to the facilities about the history of a siclevxell individual.

  • It’s the very thing that killed my daughter. The hospital wouldn’t believe her and there were no other hospitals nearby. She would be here today if they had admitted her, and not sent her home! These ER doctors NEED TO BE EDUCATED!!

  • I love this article and it’s absolutely true. My mother passed away from the diease at 42. I have the trait of sickle cell and thalsemia. I call my crises “pain attacks” becausr that’s exactly what it feels like. When I go to the E.D. I am treated like i’m an addict who is pretending, even though it’s in my file. Even my own doctor who is a generalists will question me. I am also black and when I’m in pain asking for pain meds, I am always made to feel like I’m a criminal and addict looking for a score. I’ve even told my doctor this. He admits it’s because of the opiods issue and he knows my history. It toom 3 years to diagnos me and that eas because I did the research not him. This has to stop. I even asked my doctor to read this, not sure if he will or not.

    • First, let me say I am sorry for the loss of your mom at such a young age. Mine was 46, and although I am nearly 72, I still feel that pain. Also, as a medical lab scientist, let me say the reason you were likely not diagnosed sooner, is because today’s healthcare system is too corporate. Unless your labwork was done in a small venue where the lab staff would have had time to review your blood smear, your diagnosis would be missed. In addition, training is not as good as it used to be ( everywhere and in every profession) so it may also be missed for that reason. Yes, an attitude adjustment is needed big time! I am sorry you have had to endure that dehumanizing treatment. Best wishes

  • I’ve had similar situations in the E R as a Caucasian male. Suffering from kidney stones and the inability to urinate which causes dramatic pain.
    You still have to wait in line unless you tell them you’re having a heart attack then you get immediate care -otherwise you suffer .
    No matter the issue if they can tell you are in pain they should relieve your pain and determined from then what to do .

  • While this article is very well written, I would have liked to have seen more emphasis on the psychological trauma surrounding the transition from a child to an adult SC patient. There was very little guidance nor preparation when I made my transition. It felt as though I had been unceremoniously dumped into the deepest, darkest and coldest region of the Bering Sea, to fend for myself.
    You also spoke of the racial component, yet did not mention what a diagnosis of Sickle Cell Disease does to the insurability of that patient, versus that of the of Sickle Beta Thallicemia patient. Those patients are often white and, even though their treatment includes the same hydroxyurea, transfusions, and strong pain medications, their disease is not considered a “pre-existing” condition. Because of that, they aren’t denied coverage by private insurers. This allows the Sickle Beta Thalicemia patients to receive regular check-ups and screenings that are not available to people with the “pre-existing” condition of Sickle Celll Disease, and many others, who must rely on public insurance. The message that our treatment is too costly, and that mortality rates are prohibitively disproportionate compared to illnesses like cancer is constantly conveyed to us. The angst this causes, along with the assumption, by the very health care provider who is there to provide our treatment, that, because we are African-American, we therefore must be drug seeking, both worsens the pain and severity of the Vaso-occlusive crisis, and
    impedes us from initially seeking treatment, further elevating the chances of a less than ideal outcome to that treatment, once and if it is obtained. To realize that you are being relegated to die from your illness primarily because of the choices that private insurers make regarding people of color is devastating to the individual patient, family members and friends. It is also a blight on both the history and future of this country.

    • I have nothing but heartbreaking sympathy and compassion reading all of your stories. It’s hard enough as a pain patient to receive effective treatment. I can’t imagine the betrayal and outrage you must feel when you have a clinically-proven condition that requires an unquestionable need for effective, immediate pain relief (as well other crucial, life-saving therapies). How unfair to be judged by whether or not you will accept a blood transfusion! My heart breaks for each and everyone of you in this situation.

      We are advocating for you – I am with an organization called ATIP, the Alliance for the Treatment of Intractable Pain. We are a dedicated advocacy group composed of knowledgeable physicians, scientists, researchers, journalists, and patient advocates. As a group, we publish articles; lobby legislators, policy makers, and medical professionals, and attend hearings and seminars to help ensure that effective treatment of intractable pain syndromes, including sickle cell, becomes standard instead of the criminal mindsets we all face that views pain patients as drug seekers.

      Please know we are advocating for you. For more information, send an email to me at teaandabook1992@yahoo.com.

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