Redefining Pain
New guidelines challenge misconceptions about sickle cell disease

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What Is Sickle Cell Disease?

As many as 100,000 people in the United States have some form of sickle cell disease (SCD), a group of inherited blood disorders that can cause both acute and chronic pain.

In the United States, the disease is most common among African Americans, who represent about 80 percent of the sickle cell patient population. The National Institutes of Health estimates that 10 percent of African Americans—about 2.5 million people—carry the sickle cell trait and .3 percent have some form of the disease. But the disease also strikes people with Middle Eastern, Mediterranean, and Indian ancestry, and affects South American and Latin-Caribbean people as well.

People with sickle cell disease carry an abnormal type of hemoglobin called hemoglobin S, the blood pigment in red blood cells responsible for carrying oxygen through the body. Normal red blood cells contain hemoglobin and are disc-shaped and smooth, and live about 120 days in the body. Cells affected by hemoglobin S live just 16 days and are prone to lose oxygen.

Though both unaffected and affected cells can carry about the same amount of oxygen, the affected cells tend to crystallize when they become de-oxygenated, then distort into a sickle shape. No one knows what percentage of the body’s cells are sickled at any one time, though it is believed that at moments of acute pain, the number of sickled cells in the blood has already begun to decrease.

Not all patients with sickle cell disease, however, have sickle cell anemia. Many people carry only the sickle cell trait, meaning they received the hemoglobin S gene from one parent only. When the sickle cell trait is passed down by one parent and other hemoglobin abnormalities, such as hemoglobin C disease, are passed down by the other, different sickle cell conditions arise. Sickle hemoglobin C disease, SO, S-thalassemia, and SE are all forms of SCD, and each tends to affect people within a specific ancestry. Children must inherit the hemoglobin S gene from both parents in order to get SS, the only form correctly identified as sickle cell anemia.

A small number of patients have received bone marrow transplants to cure the disease, but the procedure is risky and matches are difficult to find. Researchers know more about sickle cell disease today than ever before, but a cure for the disease is as far off as it was two decades ago.

Paul Swerdlow, MD, a sickle cell specialist in Detroit, says a number of clinical drug trials that may provide relief for sickle cell patients are under way. For now, however, the most effective treatment is still compassionate treatment with appropriate pain medication.

~ Chris Schreiber
 

By Chris Schreiber
January 20, 2000
Illustration: Hertz Nazaire

After years of being ignored, disbelieved, or dismissed as drug addicts, many patients with sickle cell disease (SCD) may finally get the relief they have long sought. New clinical guidelines written by a panel of sickle cell experts and sponsored by the American Pain Society (APS) advocate the aggressive use of drug therapy, behavioral counseling, and a greater trust in the self-reporting of pain by SCD patients.

According to Guidelines for the Management of Acute and Chronic Pain in Sickle Cell Disease, “pain is the leading cause of emergency department visits and hospitalizations and is the major focus of home management for people with sickle cell disease.” The guidelines, written for physicians, nurses, and other healthcare providers, are the first of their kind for SCD.

Misunderstood and mistreated

“[SCD] is a very poorly understood pain problem,” said Ada Jacox, PhD, RN, professor of nursing and associate dean for research at Wayne State University in Detroit and chair of the APS clinical practice guidelines committee. “There are a lot of hospitals around the country that don’t take a consistent assessment of pain. There’s a lot of unmanaged pain—and that’s across all settings.”

Sickle cell patients typically experience a host of pain problems, many of which can be excruciating and even fatal. Painful episodes can result from a variety of conditions, including vaso-occlusion in any part of the body, swelling of the extremities and joints, splenic and hepatic sequestration (blood trapped in the spleen and liver), and acute chest syndrome, a condition that resembles a heart attack.

SCD patients seeking care for painful episodes, especially acute episodes, have long reported being mistreated and having their claims ignored, said Paul Swerdlow, MD, a sickle cell specialist. Swerdlow is director of the Red Cell Disorder Program at the Barbara Ann Karmanos Cancer Institute at the Detroit Medical Center, where about 280 SCD patients receive treatment.

“Your average person with sickle cell has a very difficult time getting adequate, appropriate care,” Swerdlow said. “Their condition is terribly misunderstood. What happens is that if you have a lot of episodes of pain, you learn ways to cope with pain. When they go in [for treatment], they don’t look like they’re in a lot of pain. People don’t think they’re in as much pain as they say.”

The opioid obstacle

Swerdlow said mistreating pain can be attributed, in part, to a well-documented reluctance by physicians to prescribe opioids such as morphine for the treatment of pain. The APS guidelines indicate proper treatment for mild to moderate pain should include a regimen of prescription and over-the-counter drugs like aspirin, acetaminophen, ibuprofen, and naproxen. But the guidelines also advocate the use of opioids to treat acute and chronic pain. Swerdlow said the use of opioids is clouded with misperceptions.

“There’s a fear about using even low doses of opioids,” he said. “The first myth with opiates is that people who use them for pain get addicted. That’s not the case. If you have pain and you use morphine, you don’t get addicted. Opioids don’t have side effects and they don’t require higher and higher doses.”

The stigma of morphine use has led to a number of problems for SCD patients. Jacox said larger prescriptions of morphine have incited investigations from the Drug Enforcement Agency. And patients requesting morphine in an emergency room are often perceived as seeking the drugs to get high, not to relieve pain. The problem is compounded by racial stereotypes since the vast majority of SCD patients are African American.

“It’s not easy to communicate how it’s best to be treated at the time of a crisis,” said Hertz Nazaire, an artist with SCD who was on the APS panel. “There have been a lot of negative feelings toward the way we’re treated in an emergency room. I’ve gone in and they believe I’m addicted to drugs. It’s devastating for a person to be in pain but not be believed. It makes you feel less than human. The trust is broken when the person you come to for help reacts negatively.”

Nazaire hopes the guidelines will help the next generation of SCD patients “bypass the stigma” that the pain can cause. Making patients comfortable with the disease is a multifaceted undertaking, said Lennette Benjamin, MD, an associate professor of medicine at the Albert Einstein College of Medicine and clinical director of the Comprehensive Sickle Cell Center at the Montefiore Medical Center in the Bronx, N.Y., which treats about 400 adults and 150 children.

“For anybody who has a chronic disease, there are psychological issues that factor into treatment,” Benjamin said. “We work toward preventing as much as we can so that we’re just not addressing things after the fact. We try to deal with the total person in context, looking at pain as a function of mood.”

Treating the whole person

Benjamin said that because pain and mood can be so intertwined, it is important to help treat painful episodes by teaching patients how to reduce stress through self-hypnosis, breathing, and physical therapy. The earlier patients learn these techniques, the more manageable their pain will be as adults, said Wanda Shurney, MD, assistant clinical professor of pediatrics at Wayne State, where about 500 children with SCD come for care.

Shurney said the psychological component of SCD treatment is essential. “In a lot of ways, it’s more important than the physical.” Shurney said most children with SCD develop more slowly and can display physical signs of the disease, including painful episodes that can begin as early as 6 months, which make them vulnerable to teasing. They are also more susceptible to infection and even to occlusive stroke, she said.

“The severe recurrent pain is very disruptive to their schoolwork and their lives in general,” she said. “Even getting stuck [with needles]—adults get used to it, but when you’re a child, it’s just another bad thing about sickle cell disease.”