Severe daily pain common in sickle cell disease

NEW YORK (Reuters Health) - The pain that adults with sickle cell disease experience has been greatly underestimated, primarily because these patients usually manage their symptoms alone without seeking health care, study findings indicate.

"Our findings suggest a vast, mostly submerged iceberg of sickle cell pain that is not seen by most professionals, but rather is managed outside of medical facilities," Dr. Wally R. Smith and associates report in the Annals of Internal Medicine.

Sickle cell disease is an inherited blood disorder in which red blood cells contain an abnormal type of hemoglobin and frequently take on a sickle- or crescent-shape. These defective red blood cells can block small blood vessels, which can lead to tissue damage or even stroke. Other complications are anemia, jaundice, gallstones, severe leg and arm pain, and spleen, liver and kidney damage.

To examine the relationship between health care use and pain episodes in patients with sickle cell disease, Smith, at Virginia Commonwealth University in Richmond, and associates recruited 232 patients who were age 16 years or older. They completed daily diaries for up to 6 months recording maximum pain, whether they were in a crisis, and whether they had sought care in a hospital, emergency department, or doctor's office.

The patients reported pain 56 percent of the time, sickle cell crises 13 percent, and health care utilization 4 percent," Smith's team reports. Nearly one third of the patients had pain on more than 95 percent of days.

Evidence that patients usually managed even severe pain at home suggests that "they succumbed to pressing obstacles to care, were afraid their pain would not be managed better by a professional, or were forced to manage competing life priorities instead," the authors state.

As a result, they add, pain due to sickle cell disease is probably undertreated, highlighting "the importance of attending to sickle cell disease pain not only when patients present acutely for treatment, but also in the ambulatory setting, where home pain management regimens are prescribed."

SOURCE: Annals of Internal Medicine, January 15, 2008.