Approximately 100,000 individuals in the United States are affected by sickle cell disease, with the majority being of African American descent. However, all newborns in the country undergo sickle cell disease testing at birth, a crucial step that enables early intervention by medical professionals.
Symptoms of sickle cell disease typically manifest between six and 12 months of age in children. Initially, infants possess fetal hemoglobin within their red blood cells. As they develop, this transitions to adult hemoglobin, marking the commencement of the sickling cycle.
In individuals without the disease, a red blood cell’s lifespan is around three to four months. In those with sickle cell disease, this duration shortens to only two to three weeks, resulting in anemia—a condition characterized by reduced levels of red blood cells or hemoglobin. Anemia leads to diminished oxygen in the body, culminating in fatigue, dizziness, and headaches.
Pain represents another prevalent symptom, occasionally severe enough to necessitate hospitalization. These episodes, termed sickle cell crises, can significantly disrupt the lives of those affected, including children who might miss school due to the severity of the pain.
Dr. Allison King, a sickle cell specialist supported by NIH funding at Washington University in St. Louis, discusses the ramifications of these challenges:
- Sickle cell disease, even in children, elevates the risk of stroke beyond the norm. What additional obstacles can this ailment pose to developing brains?
Dr. King: The brain is susceptible to both overt and silent strokes due to sickle cell disease. However, we’ve also recognized that some children without a history of stroke struggle considerably in school. Lower oxygen levels in the blood can lead to cognitive difficulties. Moreover, chronic anemia places significant stress on the central nervous system, and inflammation arising from the disease compounds the challenges faced by the brain.
- How might these factors impact the education of children?
Dr. King: Individuals with sickle cell disease often encounter difficulties in cognitive processes like memory, learning, and thinking. Their fluid cognition—how they process and respond to stimuli—tends to be impaired. This encompasses memory retention, task response speed, multitasking, and recall of multi-step instructions. A child with sickle cell disease might recollect the first step but become flustered regarding subsequent actions.
- What measures can schools adopt to support children with sickle cell disease?
Dr. King: Our efforts encompass conducting cognitive assessments within the pediatric medical framework to facilitate early intervention. Subsequently, schools can implement various strategies. For instance, if a child demonstrates delayed processing speed, they can receive accommodations, such as extended time for standardized or timed tests. Moreover, instructional methods can be tailored to the child’s cognitive needs, breaking down complex tasks into simpler steps. The Individuals with Disabilities Education Act enforces these accommodations under federal law. Every child with sickle cell disease has the right to access these provisions, though this information may not be universally known. Accommodations can yield transformative outcomes, enabling bright students to pursue higher education, including law or medical school.
- Is there still a prevalent misconception in schools that children with sickle cell disease will not live long?
Dr. King: Unfortunately, this misconception persists. I recall a conversation with a high school counselor regarding a teenage patient, where the counselor, though well-intentioned, presumed the patient’s life expectancy to be just 25 years and subsequently bypassed the student in classes. I had to correct this misunderstanding. While average life expectancy was indeed limited to the mid-20s decades ago, this is no longer the case. It’s crucial to rectify this misconception not only within the medical community but also in society at large.
