Sickle cell anemia is a reality for millions of people with roots in Africa, the Mediterranean basin, the Middle East and parts of India. It's a genetic disease of the blood. Red blood cells, which usually look like donuts, deform into the shape of a crescent, or sickle blade shape, thus the name. This severely impedes their ability to carry oxygen throughout the body, and can result in debilitating pain for patients. As Rose Hoban reports, doctors are just now learning how painful the disease can be.

Dr. Wally Smith from the Virginia Commonwealth University says most doctors believe that the 'pain crises' associated with sickle cell disease are occasional events. But in his practice, he was seeing patients who reported pain more frequently.

"Patients felt like they were getting a lot of pain at home," Smith says. "Their quality of life was poor, and they were not able to work, go to school, have children, get married and do the things that you would expect a patient who's mostly healthy to do."

So, Smith decided to study how often these patients actually experienced pain. He gave several hundred people diaries to record daily how much pain they were having. He says he was surprised by what the subjects reported.

"More than a quarter, almost of third of the patients experienced sickle cell pain nearly daily, 95 out of every 100 days," he says. "And that was completely different than what the textbooks had said, or what researchers have generally perceived in the past."

"We also found that in general, this pain was much more frequent and severe than what was previously reported. About a good half of the patients said they had pain more than half of the time."

Often, Smith says, sickle cell patients are perceived as manipulative or as complainers when they come to a hospital or doctor's office looking for opiate medication to treat sickle cell pain. But his study shows that the patients were only asking for drugs when the pain became unbearable. "They managed their pain at home, that was the other big surprise," Smith reports. "So no matter how bad the pain was, most of the time they stayed at home, even if they thought they were in a crisis."

Smith says his findings are particularly disturbing in light of the fact that many countries in Africa and Asia, where there are many sickle cell sufferers, lack ready access to pain medicine. Smith recounts a story of a visiting government official from an African country who asked him to write a prescription for his son, because he was unable to obtain opiate medications in his own country.

His study is published in Annals of Internal Medicine.