One family's struggle with sickle cell disease: in the home of Dorian and Lavender Simpson, both of their children are victims of the painful blood disorder

EVERYTHING about Tiffany Simpson appears to be perfectly fine. But pain and drugs have been her unwelcome, constant companion for much of her life. The 15-year-old, who lives in the San Fernando Valley section of Los Angeles with her parents and brother, has sickle cell disease. And so does her 5-year-old brother, Dorian Simpson Jr.

The bright, attractive and, when she can be, very sociable youngster has been in and out of hospitals most of her life. She hasn't been to school since early January and desperately misses her friends and the daily routine that many students take for granted. "People have heard of sickle cell disease, but they don't pay attention to it because they're not dealing with it," says Tiffany's mother, Lavender Simpson. "We're dealing with it every day."

Dorian Jr. has, so far, fared better than his sister. However, several months ago, while Tiffany was in the midst of her crisis, he, too, was hospitalized for similar, excruciating pain. "Tiffany was in the hospital in Northridge [Calif.] and he was in Tarzana," says Dorian Sr., who, like his wife, is 36 and a native of Detroit. "My wife and I had to tag-team. We would both be at a different hospital. In the morning, I'd be at the hospital visiting him, then switch with Lavender and go to see my daughter in the afternoon."

Sickle cell disease is an inherited blood disorder passed from parents to their children. It causes red blood cells to change their shape to sharp sickle or crescent shapes and can clog blood vessels. "When blood vessels become blocked, body tissue can become damaged as the result of not receiving an adequate supply of blood," says Dr. Willarda Edwards, president of the Sickle Cell Disease Association of America. "This blockage, depending on where it occurs in the body, can cause tissue damage, pain episodes and stroke. Common symptoms of sickle cell disease [SCD] include fatigue, paleness, jaundice, shortness of breath and pain in the body organs and joints."

If both parents have the trait, there is a 25 percent chance of them having a child with the disease. Sickle cell affects between 80,000 and 100,000 victims, primarily Black individuals. Dr. Edwards points out, however, that it affects those of Mediterranean descent and Hispanics in smaller numbers.

And, sadly, despite ongoing research, there still is no known cure. Parents can only try to make their children as comfortable as possible and, according to Lavender Simpson, "keep them hydrated, ]make sure they] exercise and eat a balanced diet. And pray."

"The pain is really hard to deal with. I experience pain pretty much through my entire body--my arms, my back, my legs," says Tiffany, in a voice of quiet resignation. "It's sharp pain everywhere. I have to stay hydrated at all times. That can help, but I've been in continuous pain since November."

Because of her ongoing crisis, Tiffany completes her school assignments at home, and she hopes to join her classmates soon. In the meantime, she is at home, battling the excruciating, almost unbearable pain.

When Tiffany is in crisis, doctors give her extremely potent drugs in an effort to alleviate the pain, but unfortunately many of them no longer give her relief.

According to Dr. Edwards, proper hydration and blood transfusions can help, especially when patients are young. However, the downside of transfusions is the fact that iron accumulates in the body and can adversely affect various organs. Fortunately, research has produced a new product called Exjade, an oral medication that is designed to help reduce the iron buildup in the body, she says.

Sickle cell disease (also known as sickle cell anemia), in the opinion of many African-Americans, has not gotten the attention it deserves from the medical community, thereby directly impacting the possibility of finding a cure. The Simpsons say too few doctors deal with the disease or even have knowledge about it. "Many times, when a crisis comes up, we know more [about the situation] than the doctors do," Lavender Simpson points out.

In the 1970s, Dr. Edwards says the disease garnered significant attention, but it has almost fallen off the radar, with less public information being disseminated about it. She says an estimated 2.5 million to 3.5 million people are carriers or have the trait. But the fund-raising, advertising and educational efforts are lacking, she says.

The Simpsons, due to their family situation, are probably as knowledgeable about sickle cell disease as anyone whose lives have been affected by it. After Tiffany was born, the Simpsons tried to find as much information as they could about the disease. Armed with the growing knowledge they had gained over the years, they eventually had thoughts about having another child, despite the risks. But they took the chance.

"It was devastating when we found out that DJ also had it," says Dorian Sr., who works with the California Highway Patrol. "We had weighed our options. We knew that both of us had the trait, but there was a 75 percent chance that our child would be born with just a trait or nothing at all. Normal. We wanted to have a larger family. The odds were in our favor."