Sickle Cell Sufferers Face Uncommon Pain

Intense pain, along with fatigue, is a common hallmark of the genetic condition.

October 7, 2008, 2:08 PM

Jan. 18, 2008 — -- It is depicted in the pages of nearly every biology textbook. Next to pictures of plump red blood cells, delivering oxygen and nutrients to other cells, are pictures of dull red blood cells with a characteristic sickle shape — clumped together, unable to flow to where they are needed.

Sickle cell disease looks unpleasant on the page and is even more unpleasant in real life.

While most people might associate having sickle cell disease with fatigue or anemia, many do not know that it can also bring about pain crises so violent, patients are rendered immobile.

"It feels like somebody has stabbed me with a knife and then twisted it," said La-Tasha Davis, 20. "Now imagine that pain in several places at the same time."

Such episodes can occur as often as several times a month for people with sickle cell disease, a genetic disorder that affects the hemoglobin, the crucial part of a red blood cell that allows it to carry oxygen.

Davis, a junior at North Carolina Central University, was diagnosed with sickle cell disease when she was 8 months old and has since been in the hospital almost 20 times for pain crises. Sickle cell disease kept Davis from enjoying sports and going on longer trips and play dates as a child.

"My best play buddy when I was a kid was Shirley Temple," Davis said. She was not allowed to overexert herself or be unsupervised for long in case she suffered another pain crisis and needed medication.

Sickle cell disease is a hereditary disorder. Each parent must carry a copy of the gene for the disease and pass it on to their child for the child to have the condition. People with only one copy of the gene are called carriers and do not have any of the physical traits of the disorder, such as pain or fatigue.

Pain crises are a hallmark of the disease and occur when the sickle-shaped blood cells clump and slow blood flow to tissues and organs. Besides pain, this clumping can lead to multiple organ failure.

"Society doesn't acknowledge how severe of a disease it is and how horrible these pain crises are," said Dr. Rebecca Kruse-Jarres, a professor of hematology at the Tulane University School of Medicine in New Orleans.

Davis has been in school when a pain crisis has hit and her teacher did not believe she was in pain.

In fact, the frequency with which sickle cell disease can land patients in the emergency room with crippling pain stigmatizes people with sickle cell disease as chronic complainers and drug seekers.

Care providers will often have a bias against people who seek treatment for pain brought on by sickle cell disease because there are no objective ways to measure the pain a patient is experiencing, said Dr. Lennette Benjamin, clinical director of the Comprehensive Sickle Cell Center at Montefiore Medical Center in New York.

Kruse-Jarres said race potentially plays a role in such biases. Sickle cell disease predominantly affects blacks. There are 70,000 affected people in the United States, and one in 12 African Americans are carriers for the trait.

But even after taking strong narcotics like morphine or Oxycontin and trying at-home remedies such as warm showers, there is often no way to override a painful episode.

"There is nothing anybody can do or give you to make you any better," said Arsenia Harrison, 37. "The pain gets so bad till you feel like you're going to die.

"I don't mind dying, just hurry up."

Harrison, a registered nurse at the New Orleans Sickle Cell Center, has struggled with sickle cell disease since she was a young girl. She was even placed on antidepressants as a teenager because she was so affected by being unable to participate in sports or school activities.

Though the struggle is far from over for sickle cell sufferers, there has been some medical headway. In the past, patients with sickle cell disease were not expected to live past their 20s. With the expanded research on the disease and improved clinical care, patients now may live past their 40s, according to the National Heart, Lung, and Blood Institute.

In treating sickle cell disease, Benjamin has found that aggressive pain treatment can shorten the length of the pain crisis, which can ease other health problems brought on by the decreased oxygen and stress related to the pain and, over time, improve the patient's quality of life.

"[Pain] is an extremely significant problem for these patients," Benjamin said. "When we address it, it impacts them immensely in a positive way."

Davis, a self-proclaimed clown, chooses not to dwell on the pain.

"Normally I downplay it," she said. "I think it's great when they don't know and when they find out say, 'Oh really?!'"

ABC News production associate Rony Camille contributed to this article.