September 14, 1999

Sickle Cell Guidelines

By WARREN E. LEARY

CLEVELAND -- Patients with the agonizing and intractable pain of sickle cell disease should get better, more consistent relief because of new treatment guidelines that call for comprehensive management of the pain, experts say.

The guidelines, intended for doctors, nurses and other health professionals who treat sickle cell disease, call for aggressive use of drugs along with behavioral, psychological and physical therapies to relieve patients' suffering.

The recommendations, issued here Friday at the annual meeting of the Sickle Cell Disease Association of America, reflect growing attention to the importance of relieving pain, not just in sickle cell disease but throughout medicine. Drafted by an expert panel sponsored by the American Pain Society, a national organization of medical specialists, the guidelines are the first in a series of research-based recommendations for treating pain in several diseases, including arthritis and cancer.

But pain is different in sickle cell patients, because it can start when they are a few months old and persist, off and on, for life. "A lifetime of unpredictable, recurrent, intense, and frequently persistent pain experiences and the accompanying recurring and necessary use of opioids make pain related to sickle cell disease unique among pain syndromes," the guidelines say. Opioid means narcotic. Dr. Lennette J. Benjamin, clinical director of the Comprehensive Sickle Cell Center at Montefiore Medical Center in the Bronx and a member of the drafting panel, said, "This is the first national guideline to treat acute and chronic sickle cell pain, and we will push to have it adopted by everyone treating sickle cell patients."

Pain is the primary reason sickle cell patients seek hospital care, Dr. Benjamin said, adding that these patients traditionally are not given enough medicine.

"I see patients every day who have suffered a lifetime of needless pain simply because their doctors and others treating them don't understand or practice the principles of good pain management," she said. "Unrelieved pain leads to longer hospitalizations, greater suffering and complications and more deaths."

Sickle cell disease encompasses a group of inherited blood disorders that afflict primarily people with ancestors from Africa, the Mediterranean and India. In this country, an estimated 80,000 people, mainly from African-American, Caribbean and African-Hispanic backgrounds, have the disease, and about 8 percent of blacks carry a genetic tendency for it.

With the disease, the body produces abnormal forms of hemoglobin, the oxygen-carrying protein of red blood cells. Sometimes this hemoglobin forms rigid crystalline structures that cause the normally pliable blood cells to stiffen and contort into jagged, sickle shapes. These cells then block small blood vessels and cause vascular inflammation that inhibits blood flow to organs, resulting in cell death or damage.

These episodes can result in bouts of acute pain, called crises, that often affect the chest, back, abdomen, joints and extremities, and are described by patients as being worse than the anguish of cancer or childbirth. In addition to crises that can last hours or days, some patients suffer chronic, but less severe pain that can last months or longer because of bone or organ damage.

The pain society's panel, which included experts in sickle cell disease, pain, medicine, nursing and psychology as well as a patient representative, took two years to draft the guidelines, consulting experts and examining published studies on sickle pain. A draft report underwent peer review by 61 experts, and a revision was evaluated at 10 hospitals or medical practices that did not have sickle cell programs to see if the recommendations were clear, useful and practical, even to nonexperts.

Experts say there are a number of barriers to effective treatment of sickle cell pain: inadequate knowledge of the condition and of pain management in general; the variability and unpredictability of episodes of the disease; skepticism among some doctors that patients are as miserable as they say they are, and an unfounded fear that pain medication will lead to addiction.

The panel said that pain assessment was a key part of treatment and that health workers should rely on patients' reports of discomfort as the primary source of this evaluation.

The recommendations also call for aggressive use of painkilling drugs for acute and chronic episodes, starting with aspirin or acetaminophen and moving to opioid medicine if needed. The guidelines include a detailed regimen of doses for many painkillers, and they call for detailed monitoring of any adverse side effects. They also include step-by-step procedures for looking for underlying causes of pain and moving patients through varying options for treatment.

Among those options are behavioral approaches, including relaxation therapy, meditation and biofeedback exercises; psychological methods like counseling, support groups and hypnosis, and physical treatments, including massage, acupuncture, heat and administering intravenous fluids to improve blood flow.

The American Pain Society plans to distribute the guidelines to its members and provide them to sickle cell treatment centers nationwide. Others may order copies for $15, not including shipping, by calling (847) 375-4715 or through the organization's Web site (www.ampainsoc.org/).

Jason Rasmussen
Communications Manager Resource Center of the American Alliance of Cancer Pain Initiatives
1300 University Avenue, Room 4720
Madison, Wisconsin 53706
608-265-9174 voice 608-265-4014 fax