September is Sickle Cell Disease Awareness Month

In 2023, September was declared as National Sickle Cell Awareness Month, a time to raise awareness of sickle cell disease and sickle cell trait. Adopting a healthy lifestyle is important, including learning how to prevent infections, which can be harder to fight off when you have Sickle Cell Disease.

According to the National Heart, Lung, and Blood Institute of the National Institute of Health (NIH), Sickle cell disease is a group of inherited red blood cell disorders that affect hemoglobin, the protein that carries oxygen through the body.

In sickle cell disease, the red blood cells become crescent or “sickle” shaped due to a genetic mutation. The sickle shaped cells do not move as easily and can get stuck and block blood flow to the rest of the body.

How common is sickle cell disease?

Sickle cell disease affects more than 100,000 people in the United States and 20 million people worldwide (CDC, 2024). In the United States, most people who have sickle cell disease are of African ancestry or identify themselves as Black.

  • Approximately 1 in 13 Black or African-American babies are born with sickle cell trait
  • About 1 in every 365 Black of African-American babies are born with sickle cell disease.
  • Many people from Hispanic, Southern European, Middle Eastern, or Asian Indian backgrounds also have sickle cell disease.

What is the difference between Sickle Cell Disease and Sickle Cell Trait?

  • People with sickle cell disease (SCD) inherit two sickle cell genes, one from each parent. People with sickle cell trait (SCT) inherit one sickle cell gene and one normal gene.
  • People with SCD SCT usually do not have any of the symptoms of sickle cell disease (SCD), but they can pass the trait on to their children.
  • Talk to your doctor about getting tested for SCT if you are at risk.

What are some of the complications of sickle cell disease?

SCD-associated complications include anemia, acute and chronic pain, infections, pneumonia and acute chest syndrome, stroke, and kidney, liver, and heart disease.

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New Treatments and Research

Up until recently, a bone marrow transplant was the only cure for sickle cell disease (CDC, 2024). Fortunately, in December 2023 the U.S. Food and Drug Administration approved two new therapies to treat the disease. Both of them have to do with genetic altering of some sort. Research is continuing. Treatments and symptom control methods are being actively researched for improvement in care delivery to patients with Sickle Cell Disease.

To read more about treatments to manage Sickle Cell Disease, please follow the link below:

Sickle Cell Disease – Treatment | NHLBI, NIH

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Vaccination for the Prevention of Infections

Vaccines can protect against harmful infections. It is important that children with SCD get all regular childhood vaccines. Similarly, it is important for children and adults to get the flu vaccine every year, as well as the pneumococcal vaccine and any other recommended vaccinations, including a flu vaccination.

People with SCD are considered “high risk” for certain infections and should follow a special vaccination schedule for the following vaccines:

Additionally, for children under 5 years of age, daily penicillin (or other antibiotic prescribed by a doctor) is recommended.

Penicillin greatly reduces the risk of infections in people with HbSS (the most common form of SCD in which people inherit two hemoglobin S genes) and has been shown to be even more effective when it is started earlier. 

The Immunization Schedules as listed by the CDC are contained in this link

https://www.cdc.gov/vaccines/schedules/parents/index.html

Curious about a specific vaccine? Vaccine specific recommendations, listed by Vaccine name, are contained in this link:

https://www.cdc.gov/vaccines/hcp/acip-recs/index.html

Please review the recommendations, and speak with your health care provider to make sure you are protected!

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Flu season is quickly approaching!

  • Did you know that Sickle cell disease increases the risk of serious flu complications?
  • Getting an annual flu vaccine is the best way to protect yourself and your loved ones from the flu.
  • Infants and children with Sickle cell disease, are more likely to get harmful infections, such as meningitis, influenza and pneumonia.
  • Pneumonia is the leading cause of death in infants and young children with sickle cell disease.
  • FEVER may be the first sign of infection, and can be LIFE-THREATENING for people with sickle cell disease.
  • If you have sickle cell disease, and suspect you have an infection, go to the Emergency department or hospital immediately for treatment.

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sickle-cell-worldwide-rect.png (1200×630) (cdc.gov)

Additional Sickle cell disease and related resources:

Steps to Better Health Toolkit: Caring for Common Complications (sickle cell disease)

https://www.cdc.gov/sickle-cell/toolkit/caring-for-common-complications.html

Data and Statistics on Sickle Cell Disease

https://www.cdc.gov/sickle-cell/data/index.html

Advisory Committee on Immunization Practices (ACIP) Vaccine Recommendations and Guidelines

https://www.cdc.gov/vaccines/hcp/acip-recs/index.html

Complications of SCD: Infection | Sickle Cell Disease (SCD) | CDC