Sickle-Cell Patients Seek Respect

Bronx Beat Online

November 6, 1995

Sickle-Cell Patients Seek Respect

While many facilities disdain these "repeaters," one Bronx center is a safe haven.

by Loch Adamson

One night several years ago, Ivor Pannell went to the emergency room at Montefiore Hospital. He was suffering from an acute pain crisis, a common but potentially dangerous symptom of sickle-cell anemia. After being admitted, he lay in a hospital bed in agony, waiting for a nurse to bring him medication--a powerful narcotic prescribed by his doctor. The nurse arrived with the initial dose, but did not return at the time he was scheduled to receive another.

When the nurse finally reappeared, Pannell asked her why she was deliberately delaying his treatment. He recalls her having said, "'I'm going to continue to bring your medication late, and I'm going to do it on purpose because I know that you do not require this medication. You should just get out of that bed right now because you're not sick, you're just faking it. You just want this drug like all the rest of them.'"

Recounting the incident, Pannell shook his head, as if trying to free himself from the memory of her voice. "I hear myself saying it, and it's like, 'Damn, that couldn't have really happened,'" he said. "But I remember lying in that bed, and I remember hearing her."

His experience is not uncommon. Many individuals with sickle-cell disease tell stories of indifferent cruelty suffered under the care of emergency room and hospital staffs. Like patients with other chronic diseases, such as asthma and hemophilia, people with sickle-cell disease frequently require emergency care. The medical treatment of sickle-cell disease--including sickle-cell anemia--differs from other diseases, however, because it involves powerful narcotics, painkillers that can result in physical dependency. Because of the frequency of patients' crises and problems with physical dependency, hospital staff members have been known to stereotype individuals with sickle-cell disease. They are seen as difficult patients, or worse, as drug addicts, hooked on the painkillers prescribed for them.

"Of course there is prejudice in emergency rooms," said Dr. Ronald Nagel, professor of medicine and head of the division of hematology at Albert Einstein College of Medicine in the Bronx. "Individuals with sickle-cell anemia often have acute pain crises, many times in their lives, and sometimes many times in one year. And when they go to the emergency room and appear more than twice, they're not very welcome. They become known as repeaters, so they get really bad medical care. They're left to the last."

Dr. Lennette Benjamin, associate professor of medicine at Albert Einstein, attributes some of the behavior of emergency room staff to misplaced frustration--frustration taken out on patients who keep coming back. "You know, this is perceived as a kind of failure," she said. "We're taught that when patients come in, you make an assessment, you make a diagnosis, and you treat them, and you get results. Sickle-cell anemia doesn't always follow that course."

"Hemophiliacs can be just as difficult as patients with sickle-cell disease," added Nagel, "but somehow, the reaction to sickle-cell anemia is different in this city. There is a racial undertone to it here." A significant percentage of individuals with sickle-cell disease are African-American, and the racial attitudes of health care providers sometimes influence the care those patients receive in emergency rooms.

As part of an effort to improve patients' clinical care, Benjamin and Nagel established the Comprehensive Sickle Cell Center at Montefiore Hospital in 1988; the center's day hospital opened in 1989. The center is only one of 10 such sites created in the United States by the funding from the National Institutes of Health (NIH). Although Benjamin deals with patients' crises on a daily basis as the on-site director of clinical services, both she and Nagel administer the center's NIH grant. Since it opened, the Comprehensive Sickle Cell Center has become known as a refuge for compassionate care among people with sickle-cell disease in the Bronx.

As much as race complicates typical emergency room responses to patients with sickle-cell disease, it also complicates any discussion of sickle-cell anemia itself. The myth that sickle-cell anemia is a "black disease" persists in the public mind. Although it originated in sub-Saharan Africa and still affects a disproportionate number of blacks, it has since traveled across many continents and ethnicities. "In hematology, we call it 'gene flow,'" said Nagel. Mutations of sickle-cell disease can be found in populations in Sicily, Greece, Catalonia, Spain, and Northern Africa, he explained, and yet another variation exists in India and Saudi Arabia.

In the United States, approximately 2 million people carry at least one sickle-cell gene; it is estimated that some 65,000 have sickle-cell anemia. "In New York City, you can be of Italian descent and have sickle-cell anemia, although it is infrequent," said Nagel. "Most of our patients are of African-American descent, but many are also Caribbean. In effect, sickle-cell anemia is--how do you say it?--an equal-opportunity employer."

Sickle-cell anemia, named for the crescent shape of affected red blood cells, results from the inheritance of two recessive genes that determine hemoglobin production. In individuals with sickle-cell anemia, abnormal hemoglobin causes red blood cells to torque, or sickle. Although sickle-cell anemia is only one of a group of disorders characterized by abnormal hemoglobin production, it is the most common.

In some instances, people inherit only one sickle gene along with one normal gene; medical literature describes them simply as carriers of the sickle-cell trait. Such individuals' red blood cells function fairly normally, although they do contain a mixture of normal and abnormal hemoglobin types.

For people with sickle-cell anemia, the disease manifests itself in a variety of ways. The most common symptom is known as an acute pain crisis, or painful episode, when the sickled red blood cells clump in small blood vessels and prevent the flow of oxygenated blood from reaching body tissues. "The best way to describe it is like the worst toothache you can imagine," said Vielka White, curled on a vinyl couch in the waiting area of the Comprehensive Sickle Cell Center. In the midst of her own crisis, she spoke slowly, just above a whisper, "Now take that pain and put it in your back, your legs, your chest. It's constant."

The symptoms can appear anywhere in the body, and endanger virtually all organs. Pain becomes a harbinger of potential catastrophe; tissues die from lack of oxygen. Patients sometimes suffer strokes, but the most deadly manifestation of sickle-cell disease in adults is "acute chest syndrome," in which blood vessels in the lungs become clogged, dramatically increasing the danger of infection. Congestive heart failure can result.

In the most extreme pain crises, patients require regular blood transfusions. The most common way to treat uncomplicated crises, however, is with narcotics--such as Demerol, Dilaudid, and Percocet--in combination with intravenous fluids. The use of narcotic analgesics has proven a mixed blessing for patients. One of the most effective means for relieving their pain has also stigmatized them in the eyes of their health-care providers. Even medical journals openly acknowledge the problem. "Individuals with sickle-cell disease (SCD) enter the health care delivery system via the emergency room during pain episodes, and routinely experience long delays, extensive blood tests, accusations of drug-seeking behavior, staff ignorance regarding SCD and analgesics, and undertreatment of their pain," wrote Brian Shelly, Dr. Kathryn Kramer, and Dr. Kermit Nash in an article which appeared in the Journal of Health and Social Policy. "The fear of addiction persists among providers despite facts that addiction resulting solely from hospital medication is rare, and that SCD patients are deeply concerned over the side-effects of analgesics."

The subjectivity of pain also plays a role in its treatment. "Unfortunately, there is no objective method--at the bedside or in the laboratory--to confirm the existence of acute sickle vaso-occlusive pain," wrote Dr. Charles Pollack in an article for medical journal Emergency Medicine Clinics of North America. "Meanwhile, there have been studies that demonstrate that sicklers display no more of a propensity for narcotic dependence and drug seeking than any other patient with chronic pain."

The prejudice remains, however, perhaps because narcotic painkillers can, and still do result in physical dependency. "There are many of us who have problems with these narcotics, and we need to confront that truth," said Pannell, a former dancer, who, along with his wife, Deborah, recently founded a patient advocacy organization called S.C.A.R.E., or Sickle-Cell Advocates for Research and Empowerment. They suggest that many medical providers need to make a crucial distinction between physical dependency and addiction, a distinction that would begin to destigmatize accidental dependency.

Benjamin emphasizes that in addition to conflating dependency and addiction, many emergency room personnel fail to understand how they contribute to the problem. "Sometimes people are coming in [to emergency rooms] because they're physically dependent," she said. "But many times they're physically dependent because of how they've been managed. A lot of people who are on opioids--narcotic analgesics--don't need to be on them. But the treatment is not to penalize the person. The treatment is to try and get the person in, taper them [off the medication], prevent withdrawal; support them, and get them over it."

"Remember," Pannell said, "at the beginning of any physical dependency is a legitimate medical need for the drug and a doctor who legally prescribes it." Pannell himself articulates the difference between physical dependency and addiction as the difference between physiological need and psycho-compulsive behavior. "Of course, there are aspects of both in the other," he added. "There is a physical component to addiction, just as there are psychological components to physical dependency. But those psychological components are not the same. With physical dependency, those components are things like fear, anxiety, frustration and despair."

Pannell speaks from first-hand experience. In his early 20s, his health began to change, and he experienced several life-or-death crises just at the time he was transitioning from pediatric to adult care. "All of sudden," said Pannell, "I was being treated with much more protent narcotics, and I didn't have one doctor at the time who was in charge of all of my care. I was constantly coming under the care of different doctors who were all very young, and didn't really understand a whole lot about sickle-cell. This was the period of my life that was ripe for some kind of dependency problem." Pannell didn't even know at the time that he had developed a dependency problem, or that the narcotic analgesics being used to treat his pain were further complicating his symptoms.

Pannell credits Benjamin with saving his life by helping him break free of his dependency on analgesics. Benjamin slowly tapered Pannell's medication, helping him avoid both pain crises and withdrawal symptoms. "What was supposed to take five days took five and a half weeks," said Pannell.

During those weeks, Pannell not only recovered from his physical dependency; he also decided to survive. "You have to understand," Pannell said. "Before I started to work with Dr. Benjamin, I was counting on dying; I saw my demise as being in my early to mid-30s. And I was relieved of any responsibility for my health, or my disease, because it was clear to me that in a few years it wouldn't make any difference. That's the kind of twisted logic I was using."

Unfortunately, such a grim prognosis still has a factual basis. Until recently, many people with sickle-cell anemia--if they survived infancy--could only expect to live into their 20s or 30s. "Acute chest syndrome is the number one killer," said Nagel, "but mortality is now a moving target, because medical care has improved people's lifespans considerably."

Nagel and Benjamin hope to obtain additional funding to keep the day hospital at the Comprehensive Sickle Cell Center open around the clock, because there are many people in the Bronx and beyond who need responsive, consistent medical attention. The center currently serves between 300 and 400 people, although Nagel estimates that there are approximately 1,400 individuals with sickle-cell disease in the borough.

In the center's lobby, White said, "When you walk in here, you don't get judged. This place feels like a kind of home." White, who a member of S.C.A.R.E., talked about the way in which she has learned to manage the psychological aspects of sickle-cell anemia, as well as the pain. "One of my definite beliefs is that I have the disease, the disease doesn't have me," she said. "I'm in control--if I let the disease take control, I'd just be in pain all the time."

Pannell hopes to correct some of the harmful miconceptions about individuals with sickle cell disease, and encourage people who have the disease to take control of their lives and their treatment. Above all, he refuses to be defined by his illness, or by the stereotypes that surround it. "We refer to ourselves," he said, "as sickle-cell defiers."

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