Check out our Sickle Cell 101: A School’s Guide video on YouTube!

September is Sickle Cell Awareness month, an opportune time for school nurses to provide education and advocacy to their school communities on the rare and often-invisible disease that over 2,000 babies are born with every year in the United States.  Affecting over 100,000 Americans, and significantly more prevalent in African Americans or individuals of African descent, Sickle Cell Disease (SCD) is an inherited group of blood disorders in which red blood cells contain an abnormal form of hemoglobin, where, in the presence of infection, dehydration, or any other circumstance that reduces oxygen levels in the blood, the cells can harden and become sickle-shaped.  Their contorted shape and stiff texture make it difficult to travel efficiently through the blood vessels, leading to reduced oxygen throughout the body and can result in several different types of acute crises.  Supporting students with SCD in school occurs through close collaboration with families and healthcare providers, as well as with specific training, education, and partnership with appropriate school staff.  For students with SCD, their daily life can be severely impacted in a multitude of ways, and keeping them safe at school while promoting a positive academic outcome is a team effort, led by the school nurse.

Symptoms, Complications, and Treatments

The blocked blood flow that results from SCD can lead to pain, stroke, damage to organs, eye problems, priapism, frequent infections, and anemia.  Pain is the most common symptom and can range from acute to chronic and mild to severe.  For children with SCD, about 10% will develop stroke and another 20% will develop scarring to the brain without stroke.

Treatment for SCD is centered around pain management, infection prevention, and control of complications.  Stem cell, or bone marrow, transplants are a potential cure for SCD but carry significant associated risks and limitations.  Pain is most often treated by acetaminophen, NSAIDs, or opioids, and every individual should have a pain treatment plan from their healthcare provider.  Vaccinations are the best defense against certain bacteria and viruses that are especially dangerous to individuals with SCD.  Finally, blood transfusions can be used to treat both symptoms and complications of SCD, with some individuals needing up to 100 units of blood each year.  September is a great time to consider hosting a blood drive at your school or providing materials to your school community on the importance of blood donations.

Common Triggers

SCD pain and complications can be unpredictable but certain factors are known to trigger an acute crisis.  These include dehydration, intense physical activity, exposure to extreme temperatures, infection, stress, and caffeine.

School Support

In addition to schools being prepared with individualized health plans, specific medical orders, and acute emergency response, students with SCD should be provided appropriate accommodations to help manage their symptoms, prevent crises from occurring, and best support their physical and emotional well-being, in addition to their academic success.  Typical accommodations can include:

• Pacing exercise and allowing modifications or rest during PE and/or recess
• Unlimited access to water throughout the school day to remain hydrated
• Allowing frequent trips to the bathroom
• Careful attention to temperatures, preferential seating in the classroom to avoid drafts or cool air; avoidance of exercise in extreme heat or cold; no cold packs if injured at school
• Copies of notes, extra time for homework/projects/tests as needed due to frequent absences
• Close observance of stroke symptoms including physical symptoms as well as seemingly minor changes in attentiveness, organization, or academic performance
• Rapid recognition of emergency conditions in a student with SCD including high fever, seizure, swollen abdomen, severe and persistent pain, decreased mental status, shortness of breath, or concerning signs of cardiopulmonary collapse
• Emotional support from appropriate school staff

Like any other chronic illness, living with sickle cell disease is challenging, inconvenient, and uniquely painful.  Schools can provide safe environments where children with SCD are cared for and supported, where their individual health needs are met, and where their learning and growth are wholly nourished.