Understanding Sickle Cell Disease
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Understanding Sickle Cell Disease

Disease Targets Mostly Blacks

Sherithia Boyd's mother had come in to wake the little girl to get her ready to go to the babysitter's house so she could go to work. Boyd, then 3 years old, fell out of bed and began crying out that her legs were hurting her. Boyd’s mother called the girl's aunt, who suffered from sickle cell disease, and she said it sounded like Boyd might have the disease too. The little girl was rushed to the hospital where doctors confirmed her aunt's suspicions.

"I can be talking to someone. Go to get some water, come back and within five minutes be in pain. And people don't understand why," Boyd said. "It's like being hit by a truck or repeatedly hit by a pole. You're unable to catch your breath and feel paralyzed."

Boyd has passed the disease on to her 11-month-old daughter, Janiya Boyd, while her son, Jybrell Boyd, 3, has tested positive for the trait, meaning he does not have the disease, but could pass it on to his future children. Boyd's husband is also a carrier of the disease.

According to the Virginia Department of Health, it is estimated that more than 3,000 Virginians suffer from sickle cell disease, while another 118,000 carry the trait. The disease affects mostly blacks, but is also found in people from South and Central America, Saudi Arabia, East India, Egypt, Iran, Italy, Greece and Turkey.

SICKLE CELL DISEASE is an inherited blood disease that results in the production of sickle cells along with red blood cells. The sickle cells block the blood vessels, which deprives the body of needed oxygen and blood and causes pain to various body parts, called a crisis, and eventually organ damage. The degree and duration of the crisis can vary from a slight pain that can be managed with over-the-counter medication such as Tylenol to severe, debilitating pain that requires hospitalization. There is no warning before a crisis strikes, however, some known factors are stress, cold weather, dehydration, trauma or even exercise. There are at least five varieties of sickle cell disease: sickle cell anemia, sickle hemoglobin C disease, sickle beta zero thalassemia, sickle beta plus thalassemia, and hemoglobin C disease.

Besides intense pain that can occur anywhere in the body, sufferers are also subject to infections because of a weakened immune system; a suddenly enlarged spleen, which must be removed; pneumonia; stroke; gallstones; vision problems such as retinopathy; jaundice and abnormal growth and development causing the sufferer to have a lower average height and weight than most in adolescence.

Boyd said as a parent she pays close attention to her daughter to determine if she is in crisis or is just suffering the everyday bumps and bruises of being an infant. Typically, when a child is in crisis, he or she can have swollen hands and feet, cries without stopping, is fussier than normal, can be pale and have a temperature.

SINCE 1989 in Virginia, according to the state health department, all infants are automatically screened for sickle cell, as well as seven other inherited diseases, and more than 750 have been diagnosed with the disease. Even so, say advocates, sickle cell is often misunderstood by the public.

"We got together last year and said we wanted to promote awareness of sickle cell," said Jeannette Magruder, a sickle cell carrier and teacher at Hutchison Elementary in Herndon. "We starting going to churches ... to talk about sickle cell in general and share personal experiences. We talk about the hardships and how it affects the body and the complications so the community has a better understanding."

Magruder, along with a group of volunteers is in the midst of creating a organization that will focus on providing support to families ranging from transportation to screenings to advocacy and even babysitting services, and educating the public at large.

"In today's society, dealing with any disease is hard. Coming together to share testimonies is key to education," said Orlando Magruder, Jeannette's husband and a certified personal trainer. "It doesn't hit a person what sickle cell really is until they deal with it."

BECAUSE OF THE LACK of information, sufferers of sickle cell can be subject to hard times at school and later in the work force, said Boyd.

People with sickle cell can often be sick without warning and there are very few outward signs of a problem, which can lead to criticisms of laziness or even drug addiction, said Jeanette Magruder.

"Sufferers have difficulty remembering things. It's difficult to stay focused. Without oxygen and blood, a person can't function," Jeannette Magruder said. "A lot of school teachers don't know that. They could think the child is just being lazy or think the child has a learning disability and that may not necessarily be the case. School officials think the child is just trying to use it to their advantage."

Jeannette Magruder has started an awareness campaign at her school, so teachers can recognize when a child is truly in crisis. Boyd said the stress brought on by the criticisms and teasing by other children often cause sufferers to drop out of school and causes issues with low self-esteem.

"I remember as a kid being told by the other kids they were having a party and I could not come because I had a disease," Boyd said. "And there were times that even my mom had a hard time believing me because the pain jumps around your body."

In the work place, people with sickle cell can find it hard to keep a job because of the sudden need for time off and frequent crisis periods. Boyd said often times sickle cell sufferers become depressed and withdrawn.

"I don't condone suicide, but the pain can get so severe I would think about suicide," Boyd said. "I would get back pain that hurts so bad, I'd be laying on the floor hoping someone would blow my brains out."

She said when someone is in crisis, often they do not like to be touched, they don't want to move and can "knot up in weird positions" until the pain passes.

"Sometimes I feel like the doctors don't understand how intense the pain is," Boyd said. "One doctor told me it's like the last stages of cancer. I don't know, I don't know what cancer feels like."

Currently, there is no cure for sickle cell disease.

The Magruders, along with other volunteers, will hold a sickle cell awareness workshop Saturday, Sept. 20, from noon-2 p.m., at the Herndon Neighborhood Resource Center, 1086 Elden St. in the Dulles Park Shopping Center.