Sickle Cell Disease: The Importance of Raising Awareness and Screening

Sickle cell disease can be devastating and painful. Those with it are prone to severe infections, strokes, and other complications. Sickle cell disease affects as many as 100,000 people in the U.S. and is the most common inherited blood disorder. It’s most common in the Black community — affecting 1 in 365 Black people. 

But more awareness is needed to help those at risk of this condition. Sickle cell disease can be silent early in infancy, which is why screening is important. Early detection gives parents and caregivers the opportunity to learn how best to care for children and get set up with a supportive healthcare team. 

What is sickle cell anemia?

Sickle cell anemia, or sickle cell disease, is caused by a genetic difference in how red blood cells are shaped. Red blood cells carry oxygen to the organs. And they usually have a smooth wall so that they can glide through blood vessels. 

But in sickle cell disease, the cells can form a jagged, sickle-like shape and get stuck in tiny blood vessels, which cuts off the blood supply. When this happens, it causes a sickle cell crisis.

This process can also be triggered by:

• Cold temperatures

• Dehydration

• Exercise

• Infections

• Stress

What causes sickle cell disease?

Sickle cell disease occurs when a person gets a copy of the sickle cell gene from each of their biological parents. A person with only one copy of the gene has sickle cell trait (SCT), which does not usually have any major health risks. But when two people with SCT come together to have a child, there’s a 1 in 4 chance the baby will be born with sickle cell disease. 

In the U.S., the life expectancy for people with sickle cell disease is at least 22 years shorter than for those who don’t have sickle cell. The healthcare costs are also significantly higher. And, for many, quality of life is lower.

What is ‘Sickle Cell Awareness’?

Sickle Cell Awareness is an intentional effort to draw attention to the need to address the impact of sickle cell disease. Initiatives like World Sickle Cell Awareness Day on June 19, and National Sickle Cell Awareness Month in September help to increase awareness about what people living with sickle cell disease are facing. And it creates an opportunity for people to learn from each other and support those living with sickle cell disease.

These initiatives are also an opportunity to draw government attention to the need for more research. There are huge disparities in research funding for sickle cell disease and other serious conditions. 

For example, cystic fibrosis affects about ⅓ as many people in this country as sickle cell disease. But most of the people affected are white, and it's more heavily funded. One study found that, between 2008 and 2018, U.S. funding for research into sickle cell disease was about $800 per person affected. This is compared to about $2,800 per person affected by cystic fibrosis. 

The disparity is even greater when you look at federal funding by foundations. Funding totals are about $100 per person for those with sickle cell disease versus around $7,700 per person for those with cystic fibrosis.

Looking for a cure

The Cure Sickle Cell Initiative was launched in 2018 in recognition of the need for more attention to finding a cure for this disease. Bone marrow and stem cell transplantation has been used successfully in some cases. But this treatment comes with a high risk of complications, and it’s not an option for most people with sickle cell disease. 

Fortunately, there has been more research into potential cures. And there have been some recent advancements in treatments. Gene therapy is one of these new potential cures.

Casgevy (exagamglogene autotemcel) and Lyfgenia are two FDA-approved genetic treatments for sickle cell disease. They create new stem cells so people with sickle cell disease are no longer affected by an abnormal hemoglobin gene. They can make normal red blood cells instead. There’s ongoing research on the long-term risks and benefits of gene therapy, but it could be an effective cure.

The importance of screening your children for sickle cell anemia

Universal screening for sickle cell disease was launched in New York state in 1975. By 2006, it was in place across the country. Newborn screening lets healthcare teams be more attentive to the need for preventive treatments. This helps to reduce the risk of some severe infections from sickle cell disease.

Screening also gives families the opportunity to get set up with a skilled healthcare team. They can manage the progress of the disease and help families get started with a preventive program. It’s very important to have a stable, up-to-date team healthcare to lower the risk of serious complications. 

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Written by:

Khama Ennis, MD, MPH, FACEP

Dr. Ennis is a board-certified emergency physician based in western Massachusetts. She is the medical staff president and associate chief of emergency medicine at Cooley Dickinson Hospital.

Edited by:

Karla Robinson, MD

Karla Robinson, MD, is a medical editor for GoodRx. She is a licensed, board-certified family physician with almost 20 years of experience in health through varied clinical, administrative, and educational roles.

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