About 100,000 people in the United States live with sickle cell disease—most of them African American. But every baby born here is tested for sickle cell disease at birth. This helps doctors start treatment as early as possible.
Children typically don’t show symptoms until they’re between six and 12 months old. Babies’ red blood cells have a different type of hemoglobin, called fetal hemoglobin. As they grow, the body switches to producing adult hemoglobin. Then, the sickling cycle begins.
A normal red blood cell lives for around three to four months. But in people with sickle cell disease, the cells usually live for just two to three weeks. This leads to anemia, a condition in which blood has low amounts of red blood cells or hemoglobin. Anemia lowers oxygen in the body, which can cause fatigue, dizziness and headaches.
Pain is another common symptom. It can be so severe that people end up in the hospital. These pain episodes are called a sickle cell crisis, and they can disrupt sufferer’s lives. For children, that means missing school.
Allison King, M.D., an NIH-funded sickle cell specialist at Washington University in St. Louis, discusses the repercussions of these challenges:
People with sickle cell disease—even children—have a higher than normal risk of stroke. What other challenges can sickle cell disease pose to the developing brain?
King: Both overt and silent strokes can harm the brain. But we’ve also realized there are some children who haven’t had a stroke who also have a lot of trouble in school. Now we know things like lower oxygen levels in the blood can also be associated with challenges. It’s also very stressful on the central nervous system to be chronically . Plus, from the disease can be a challenge to the brain as well.
How can these things potentially affect children’s education?
King: People with sickle cell disease can have more challenges in the way that they think, learn and remember. They tend to have weaknesses with fluid cognition. This is how you process and respond to a stimulus. So, how well can you remember things? How fast do you respond to tasks? Can you multitask? Can you remember multi-step commands? Kids with sickle cell disease may remember one step, then get really flustered about what to do next.
What can schools do to support children with sickle-cell disease?
King: One of the things we’ve been pushing for is to try to make sure kids have cognitive assessments completed while they’re still in the pediatric medicine setting. With that, there are several things that can happen in the school system. For example, if we find out kids have delayed processing speed, they can get accommodations in school. So, if they’re taking standardized tests, timed tests, they can get extra time. Or with other cognitive challenges, the way the material is presented can be broken down into simpler steps. Federal law supports that under the Individuals with Disabilities Education Act. And all kids who have sickle cell disease have a right to access those accommodations. Not all families know that. But accommodations can be a game changer. We’ve had a lot of really bright kids who’ve gone on to university, and even law school or medical school.
Is there still a misconception in schools that kids with sickle cell disease will die young?
King: That is so sad and true. I remember meeting with a high school counselor for one of my teenage patients who was trying to set up a learning plan. And that counselor was well intentioned, but she said, ‘your patient is so sweet, but we know she’s only going to live to 25, so we’ve just been passing her in her classes.’ And I had to say no, no, no, no. The average lifespan may have only been in the mid-20s back in the 1970s, but that’s not true anymore. We need to make this mind shift not only in the field of medicine, but out in the community.