5 Things to Know During Sickle Cell Awareness Month

September is Sickle Cell Awareness Month, a time to increase understanding of a disease that can affect nearly every part of a person's life, often in ways people around them cannot see.

Sickle cell disease is a group of inherited blood disorders in which red blood cells can become hard and sticky. Those cells can block blood flow, causing severe pain and potentially serious complications throughout the body. Pain is the most common complication and the leading reason people with sickle cell disease visit an emergency department or hospital.

But understanding sickle cell means understanding more than the medical definition. Here are five things people may not realize about what patients and their families experience.

 Patients can face stigma when seeking pain treatment

A sickle cell pain crisis can be excruciating and may require opioid medication. Yet patients have reported being viewed as “drug seeking” when requesting treatment that has worked for them before. Research has documented both disease-related stigma and racial bias in the care of sickle cell patients, particularly because the disease disproportionately affects Black Americans. A recent study found that race-based bias experienced by Black sickle cell patients can be compounded by stigma involving chronic pain and opioid treatment.

CDC educational materials for emergency providers specifically note that requests for pain medicine are most commonly based on patients’ previous treatment experience, rather than drug-seeking behavior.

 “Normal” activities may require much more planning

Swimming, playing sports, exercising or simply spending a long time outside in extreme temperatures may not be as simple for someone with sickle cell disease. Dehydration, becoming too hot or cold, low oxygen levels and extremely strenuous exercise can increase the risk of complications or pain episodes.

That does not mean people with sickle cell disease cannot exercise, swim or participate in sports. Rather, activities may require individualized precautions, hydration, breaks and attention to temperature and exertion based on a person's medical needs.

Someone can look fine and still be experiencing serious pain

Sickle cell is often an invisible disease. A person experiencing a pain crisis may not necessarily cry, scream or display outward signs that match another person's idea of severe pain. There is also no single vital sign or laboratory test that can confirm or rule out a sickle cell pain crisis; CDC materials emphasize the importance of believing patients' reports of their pain.

That can make understanding from employers, teachers, coaches, friends and relatives especially important.

Families live with more than pain crises

Sickle cell disease can cause serious complications including stroke, severe anemia, infections, vision problems and organ damage. Children at increased risk for stroke may require regular screening, while some patients need frequent blood transfusions or ongoing medications to reduce complications.

For families, that can mean specialist appointments, emergency room visits, medication schedules, missed school or work and constantly monitoring symptoms that could indicate something more serious.

Sickle cell disease and sickle cell trait are not the same thing

Someone with sickle cell trait carries one sickle cell gene and typically does not experience the symptoms of sickle cell disease. However, the trait can be passed to children. If both parents have sickle cell trait, each pregnancy has a 25% chance of resulting in a child with sickle cell disease and a 50% chance of resulting in a child with sickle cell trait.

Sickle Cell Awareness Month provides an opportunity to learn about those realities and listen to the experiences of patients and families living with the disease.

Awareness can start with understanding that a person missing an activity, requesting accommodations or seeking strong pain relief may be managing complications that aren't visible to everyone else.