Sickle Cell and Pain

Seven's On Call with Dr. Jay Adlersberg
January 14, 2008 9:00:00 PM PST
There is new information about just how much pain some patients with sickle cell anemia are in. It's a genetic disease that affects African-Americans. Seven's On Call with Dr. Jay Adlersberg. Sickle cell anemia causes low blood counts, fatigue and blocked blood vessels. Less than one percent of blacks have it. This week's report in the "Annals of Internal Medicine" says of all its symptoms, pain is the most constant one, and has the greatest effect on quality of life.

Thirty eight-year-old Nicole Bryant and her grandmother know about sickle cell anemia. It's made Nicole's lungs so weak that she needs oxygen all the time just to breathe easier. The other constant in her life is pain. It was most severe in her teens.

"It was bad. I just stayed in bed with pain," says Nicole. "I don't know how to describe it. It was always pain. I didn't have much of a life, a social life."

Patients show up in the emergency room for severe attacks. But the study found that, as with Nicole, patients have pain every single day.

"It's an improtant finding," says Doctor Babette Weksler of New York Presbyterian Weill Cornell Hospital, "because sickle cell patients are often not belived when they turn up at the emergency room needing medication for pain."

Patients have two doses of the sickle gene, one from each parent. It causes red blood cells to change from round to pointy, like a curved sickle. The abnornal cells clump together and block blood vessels, causing excruciating pain in the bones. About eight percent of African-Americans have one dose of the gene, which causes fewer symtoms.

There's one situation in which the sickle cell gene is protective: In africa, people with one dose of the gene are protected against malaria. Maybe thats why its hung around so long, long enough to cause this illness which can lead to strokes from blood clots.

Nicole checks her blood routinely, as she takes blood thinners for that reason. The chronic pain issue is now out in the open.

"Hopefully docctors will believe a patient more," Doctor Weksler says, "when they come with pain and need medication to take care of it."

There are medications that prevent patients' red blood cells from sickling, and of course, plenty of good pain-relief drugs. Perhaps this article will change how doctors perceive what their patients have to deal with day in and day out.