Sickle Cell Disease: AJ Green’s Story
Sickle cell disease is a group of inherited red blood cell disorders. Healthy red blood cells are round, and they move through small blood vessels to carry oxygen to all parts of the body. In someone who has sickle cell disease, the red blood cells become hard and sticky and look like a C-shaped farm tool called a "sickle". The sickle cells die early, which causes a constant shortage of red blood cells. Also, when they travel through small blood vessels, they get stuck and clog the blood flow. This can cause pain and other serious problems such as infection, acute chest syndrome and stroke.
The Green Family Story
"When AJ was born in 1992, there was a real lack of information and education about newborn screening and sickle cell disease. They just told us he was having a PKU shot and then the doctor actually called on the telephone and gave us his results. It wasn't really something back then that they talked about. They just said that he has to have a PKU shot. We didn't know then that it was newborn screening. The doctor told us that the newborn screening showed that AJ has sickle cell disease. I have the sickle cell trait and my husband has the trait. I didn't really know anything about, or anybody with, the actual disease. But when the doctor said the word 'disease' I started crying because I knew that couldn't be good. At that time, of course, I was devastated. I went to the computer and looked it up and saw what could happen to him and what was down the line for him.
"AJ had his first sickle cell crisis at about 8 months. We didn't really know what to expect with a crisis, but the early newborn screening test was helpful because we knew he had sickle cell disease. When he had his first painful sickle cell crisis we would never have known to take him to the hospital. We probably would have just thought he was crying to be crying and put a bottle in his mouth. We knew something had to be wrong with him and we didn't take it lightly. We took him to the hospital and they said that he was having a crisis in the top of his foot. It was important to have the newborn screening done so that we knew to watch for these things. If we hadn't had the newborn screening done we wouldn't have known what was going on with him and sought appropriate care right away.
"It's important for parents to get newborn screening to know if there's anything wrong with the child. If it's sickle cell, it just makes you aware of what's going on with the child, things to look for (if they're dehydrated, not getting enough water, if they're crying a lot from pain) because you don't know that they're hurting, they can't tell you." – Pam Green
"For the most part I have been able to meet all my goals, but I always strive for bigger goals in life so everything's not complete just yet. My first aspiration was to be known as a singer and also as an advocate for sickle cell disease. I try to be that inspiration, you know. It's not as bad as people try to make it seem. I mean, it is, but it's not. People don't have to be afraid of it; they don't have to be held down by it. Try to find triumph in the situation."
AJ, at 19 years old, an accomplished singer and sickle cell disease advocate, has accomplished more than most teens his age and doesn't let his condition keep him from reaching his goals. In addition to performing for two U.S. presidents, Clinton and Obama, the Grammy-hopeful has shared the stage with Elton John, the Canadian Tenors, Jason Mraz, Rob Thomas, and sang a duet with Jennifer Hudson. He continues to be a strong advocate for the community in his efforts to "put sickle cell on the map" by raising awareness of the condition so that someday it will receive as much attention as other high profile disease states such as cancer. He recently spoke with the kids at the Sickle Cell Disease Association of Mobile, Alabama and joined up with Remington College's "3 Lives" blood drive campaign which raises awareness for minority blood donors and encourages people to donate blood.
CDC would like to thank AJ and Pam for sharing this personal story.
Importance of Newborn Screening
Soon after birth, all babies born in the United States are checked for certain medical conditions, including sickle cell disease. This is called newborn screening. Finding these conditions soon after birth can help prevent some serious problems, such as brain damage, organ damage, and even death.
Babies that are born in a hospital should be screened before they leave the hospital. Parents should take babies that are not born in a hospital or those that were not screened before leaving the hospital to a hospital or clinic to be checked within a few days of birth.
Early diagnosis and treatment of sickle cell is important. A baby with sickle cell disease is at risk for harmful infections. These babies can take a daily dose of penicillin, an antibiotic medicine, to help prevent infections. Although the penicillin will not change the fact that the baby has sickle cell disease, it can help prevent serious problems.
The sooner you know a child has sickle cell disease, the better. Parents can take steps to keep their child healthy and well. The following tips will help you, or someone you know with sickle cell disease, stay as healthy as possible.
Find Good Medical Care
Sickle cell disease is a complex disease. Good quality medical care from doctors and nurses who know a lot about the disease can help prevent some serious problems. Often the best choice is a hematologist (a doctor who specializes in blood diseases) working with a team of specialists.
Get Regular Checkups
Regular health checkups with a primary care doctor can help prevent some serious problems.
- Babies from birth to 1 year of age should see a doctor every 2 to 3 months.
- Children from 1 to 2 years of age should see a doctor at least every 3 months.
- Children and adults from 2 years of age or older should see a doctor at least once every year.
People with sickle cell disease, especially infants and children, are more at risk for harmful infections. Common illnesses, like the flu, can quickly become dangerous for a child with sickle cell disease. Pneumonia is a leading cause of death in infants and young children with sickle cell disease. The best defense is to take simple steps to help prevent infections.
Vaccines can protect against harmful infections. Babies and children with sickle cell disease should have all of the regular childhood vaccines, plus a few extra. The extra ones are:
- Flu vaccine (influenza vaccine) every year after 6 months of age.
- A special pneumococcal vaccine (called 23-valent pneumococcal vaccine) at 2 and 5 years of age.
- Meningococcal vaccine, if recommended by a doctor.
In addition, children with sickle cell disease should receive a daily dose of penicillin, an antibiotic medicine, to help prevent infections. This can begin at 2 months of age and continue until the child is at least 5 years of age.
Learn Healthy Habits
People with sickle cell disease should drink 8 to 10 glasses of water every day and eat healthy food. Try not to get too hot, too cold, or too tired.
Children can, and should, participate in physical activity to help stay healthy. However, it’s important that they don’t overdo it, rest when tired, and drink plenty of water.