Sickle cell disease (SCD) is a group of genetic disorders that affect red blood cells, causing them to become shaped like crescents, or sickles. The sickle-shaped cells travel through the blood stream where they can get stuck and cut off blood flow, causing intense pain and organ damage. According to the National Institutes of Health (NIH), SCD affects about 1 in 365 African Americans, with approximately 100,000 having the disease in the U.S.
Unfortunately, people in communities, healthcare facilities, and even family units sometimes develop an unfair perception of people who have SCD, which can lead to bias and stigma. And though there are tips an individual with SCD can follow to minimize stigma, healthcare providers, families, communities and the public at large may also play a role in neutralizing stigma. Getting educated, having compassion, and learning to listen are important first steps everyone can take to overcome SCD-related stigma.
What is Health-Related Stigma?
Stigma occurs when a negative stereotype about a group is attributed to an individual. Health-related stigma refers to the rejection of people with certain health conditions, based on a negative stereotype. In the case of SCD, health-related stigma can be compounded by racism (actual or perceived) and can pose significant barriers to getting appropriate care. For example, the seeking of a specific medication for chronic or acute pain may be perceived as the behavior of a person affected by substance abuse. Or, people who are experiencing SCD-related pain may be falsely thought of as malingers (those who pretend to be sick to get out of their responsibilities).
Unfortunately, in some instances, even healthcare providers may think that SCD patients are exaggerating their pain or seeking painkillers to satisfy an addiction. In fact, a study in 1999 interviewed 57 people with SCD and found that nearly all of those interviewed felt that they had been subjected to stigma and viewed as though they were individuals who were affected by substance abuse.
Why Stigma is a Problem in SCD
There are many factors that can contribute to stigma in SCD. The most common are:
- Physical factors that make people with SCD stand out. In SCD, people may have a yellow discoloration of the eyes (called jaundice) that may cause people to judge them. Adults and adolescents with SCD may have joint damage, which makes them unable to participate in activities involving physical effort resulting in them feeling different than their peers.
- Some people with SCD may seem stoic or unemotional. A reduced expression of emotion may be a way of dealing with the severe and chronic pain that accompanies SCD. This may lead others to perceive that a person with SCD is not experiencing pain, when he or she really is.
- People with SCD often know what pain medication works for them and even what doses are needed. People with SCD experience chronic pain and have experience managing frequent, acute pain crises which may give providers in the emergency room or other settings the false impression that they are affected by substance abuse, rather than simply being knowledgeable about their health needs.
- People with SCD often suffer from extreme pain and fatigue. The frequent pain episodes and extreme fatigue can lead to absenteeism from both school and work. This may create a false impression that the person with SCD is “lazy.”
As a result, SCD-related stigma may leave some sufferers feeling mistrustful of the healthcare system.
What You Can Do About It
Strategies that may help reduce the impact of stigma include counseling, therapy, support groups, empowerment and self-help, and advocacy. There are also several things you, as a person with SCD, can do to discourage SCD-related stigma when you are seeking medical care. Here are some tips:
- Stay up-to-date with your medical care and keep yourself informed about your condition.
- Work together with your hematologist and your regular healthcare team.
- Create a ‘pain plan’ (which is a list of pain medications that works for you) with your healthcare team, and either carry a validated copy with you at all times or have easy access to an electronic version.
- Carry a document with you at all times that summarizes previous hospitalizations or emergency room visits. The individualized pain plan from your doctor can also be included.
- If you have acute pain, document the symptoms and location of the pain before and during attempts to manage pain with therapy.
- Find ways that help to reduce and/or manage your pain before it becomes an emergency. The National Heart Lung Blood Institute has some very good ideas to get you started.
These tips may help you avoid bias and stigma on an individual level. Involvement and education of family members and the extended community surrounding you is essential as well.
Dr. Sivamurthy is a pediatric hematologist-oncologist and a Medical Director at Pfizer.
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