Cultural competence in the treatment of sickle cell disease

Sickle cell disease refers to a group of rare blood disorders that are inherited from a person’s biological parents. These disorders cause the body to produce abnormal red blood cells called sickle cells.

Sickle cells get their name from their shape. Instead of the round shape and flexible structure of healthy red blood cells, sickle cells are shaped like crescents (or sickles). These cells are ineffective at transporting oxygen, break apart and die easily, and can form blockages inside small blood vessels.

Signs, symptoms, and complications

The most common signs and symptoms of sickle cell disease are anemia and sickle cell crisis.

Anemia

Anemia is a shortage of functioning red blood cells. Anemia can cause fatigue, weakness, dizziness, pale skin, headaches, and cold hands and feet. It can also lead to more severe complications, including debilitating fatigue, problems during pregnancy, and irregular heart rhythms.

Sickle cell crisis

A sickle cell crisis is an episode of sudden and often severe pain. These episodes occur when sickle cells block the flow of blood, stopping blood from reaching organs and tissues. Pain is common in the limbs, chest, and abdomen, but it can occur almost anywhere in the body. Swelling in the hands and feet can also occur during a crisis.

Complications can include delayed growth and puberty, organ damage, vision problems, blood clots, leg ulcers, frequent infections, chronic pain, stroke, and the death of bone tissue. Sickle cell crises that affect the lungs can cause life-threatening complications.

Disparities in treatment

Although treatment for sickle cell disease continues to improve, significant disparities in care remain.

Many people with the condition do not receive regular treatment with medications that can prevent sickle cell crises. Few people are able to access treatments that can potentially cure sickle cell disease, such as bone marrow transplants and gene therapies. Many rely on emergency treatment and only receive treatment during sickle cell crises.

Factors like bias, stigma, discrimination, lack of access to healthcare, and healthcare expenses contribute to these disparities. One of the most commonly cited examples is the administration of pain medication during a sickle cell crisis. People with the condition may avoid emergency care for fear of being suspected of drug-seeking behavior. Sickle cell crises may also be undertreated in some healthcare settings, due to a provider’s previous experience with drug-seeking patients.

The need for culturally competent care

While sickle cell disease can affect people of many races and ethnicities, the disorder disproportionately affects people of African descent. In the United States, 90 percent of people with sickle cell disease are Black, while between 3 and 9 percent are Hispanic or Latino. These are populations that disproportionately face bias, stigma, and discrimination in healthcare settings.

There is a strong need for cultural competence in the treatment of sickle cell disease. Cultural competence is a healthcare provider’s ability to recognize how race, ethnicity, cultural background, and language affect a person’s healthcare needs.

Strategies for finding culturally competent care

One of the best strategies for seeking care for sickle cell disease is to seek treatment at a medical clinic that specializes in sickle cell disease. This is perhaps the surest option for finding healthcare providers who are experienced in treating these disorders and who understand what populations are impacted by sickle cell disease.

This may not be possible for everyone, and you may need to seek treatment outside of a specialty clinic. At minimum, a healthcare team should include a hematologist (a medical doctor who specializes in blood disorders) and a primary care provider (which would be a pediatrician for children and adolescents). These healthcare providers can oversee care, refer you to other specialists you may need, and guide you toward resources to help you or your family manage this condition.

What to ask when choosing a healthcare provider

Beyond working with a healthcare provider who has the necessary experience and training to treat sickle cell disease, here are some questions to consider:

• Can you be honest with this healthcare provider?
• Do you feel the healthcare provider and staff treat you with respect?
• Do you feel comfortable bringing up questions and concerns?
• Do you feel your questions and concerns are taken seriously?
• Do you feel like your healthcare provider is able to understand what you mean when you ask a question or explain something?
• Do you understand what your healthcare provider means when they give you an explanation?
• Does the provider have experience working with people who have backgrounds similar to mine?
• How does this healthcare provider compare to other healthcare providers you have worked with in the past?

Also consider asking a trusted friend or loved one to accompany you to an appointment to get their opinion. Social support can make an important difference when treating sickle cell disease.