SICKLE CELL PAIN MAY BE FROM DAMAGED TISSUES OR NERVES

Researchers at the University of Illinois at Chicago have discovered
the pain caused by sickle cell disease may not occur solely from
damaged tissues, but also from injured nerves.

The research is published in the Journal of the National Medical
Association.

Sickle cell disease is a hereditary disorder where red blood cells
change from round to a sickle shape. It predominantly afflicts African
Americans, Hispanics and people from the Mediterranean region.

The sickle-shaped cells are stiff and sticky, and tend to get stuck in
blood vessels. When they become trapped, blood flow is blocked to the
limbs and organs, causing pain, serious infections and organ damage,
especially in the lungs, kidneys, spleen and brain. Some patients
survive into their 40s, while others do not live beyond infancy or
early childhood.

In the new study, 145 adult outpatients at UIC's Sickle Cell Center
used a pen-tablet computer to record the locations, severity and nature
of their pain -- the most prevalent symptom of the disease.

Patients gave their current, least and worst pain a number value from 0
(no pain) to 10 and compared it to their "worst toothache, headache and
stomach-ache," said Diana Wilkie, professor and Harriet H. Werley
Endowed Chair for Nursing Research and lead researcher on the study.

Along with the numerical values, patients also described their pain by
choosing from a list of 78 words. The most common descriptions of nerve
pain were aching and stabbing; the most common for tissue pain were
pounding and sharp.

"I can't imagine living with that pain year after year after year,"
Wilkie said. "The pain significantly reduces their quality of life."

Nearly half of the patients reported their pain was caused by the
disease. Other patients said their hurting was caused by the weather,
stress, and other or unknown causes, Wilkie said. They also reported
pain in multiple locations. The upper back and left arm were the most
common body areas noted. Other areas included the head, right arm,
chest, abdomen, lower back, left leg and right leg, she said.

Only 51 of the 145 patients said they were pain-free. Seventeen percent
of the sample reported mild pain, 27 percent said their pain was
moderate, and 19 percent reported severe pain.

Participants were also asked about their misconceptions and barriers of
pain management. Currently there is no cure for sickle cell disease;
treatment depends on the patient's symptoms.

Hydroxyurea is the only FDA-approved medication for the disease, but
other options include opioids and anti-inflammatory medications such as
aspirin and ibuprofen, said Dr. Robert Molokie, assistant professor and
medical director of the UIC Adult Sickle Cell Center program and a
co-researcher on the study.

The potential to become addicted to pain medications was the primary
concern of the participants. Opioids are considered safe, but many
patients were reluctant to use them because of the potential for
addiction or side-effects of nausea and constipation.

"The opioid drugs are helpful for many patients," Wilkie said. "The
obstacles for patients with sickle cell disease to use opioids are
greater than outpatients living with cancer. Their pain is severe and
continuous, persisting beyond the typical painful episodes commonly
associated with emergency room visits and hospitalizations."

Other authors on the report are Maria Suarez, Young Ok Kim and
Zhongsheng Zhao of UIC's department of biobehavioral health science; Z.
Jim Wang of UIC's department of biopharmaceutical sciences; and Dr.
Yogen Saunthararajah of the Cleveland Clinic.

The research was funded through a grant from the National Heart, Lung
and Blood Institute, one of the National Institutes of Health. For more
information about UIC, visit www.uic.edu

- UIC -