trait | Live Healthy S.C.

September is National Sickle Cell Awareness Month, let’s raise the awareness bar to highlight sickle cell trait and sickle cell disease.

What is Sickle Cell Disease?

SCD is a blood disorder that causes sickling of the red blood cells, which diminishes the amount of oxygen the red blood cell can carry throughout the body. Persons who have SCD suffer from crises — episodes of intense and excruciating pain that may be in one or multiple parts of the body when sickle-shaped red blood cells become stuck in a blood vessel and cause a disruption of blood flow in that particular area. While people are most familiar with sickle cell anemia, other variations of sickle cell, or mutations, include sickle cell thalassemia, sickle beta thalassemia, and others.

What is Sickle Cell Trait?

SCT results when a person inherits one sickle cell gene and one normal gene from either of their parents. Persons with sickle cell trait usually do not have any of the symptoms of SCD, but they can pass the trait on to their children.

How are SCT and SCD related?

An individual who has SCD has a family history of SCT – meaning the person’s parent(s) have sickle cell trait or sickle cell disease. SCD is inherited when a child receives two sickle cell genes from each parent. For someone who has SCT, the likelihood of having a child that has SCD or SCT is different. If both parents have SCT, there is a 50 percent chance the child will have SCT, a 25 percent chance the child may have SCD, and a 25 percent chance the child will not have SCD or SCT.

The urge to increase awareness on sickle cell trait and disease is apparent across several organizations. The Centers for Disease Control and Prevention released new informational materials and videos of individuals’ personal experiences living with sickle cell disease or sickle cell trait. To view these videos, visit cdc.gov/ncbddd/sicklecell/materials/video.html.

What is South Carolina’s response?

The South Carolina Sickle Cell Disease Advocacy Team (SCSCDAT) was established in 2017 with one common goal – to improve the treatment and care received by individuals and their families who have sickle cell disease. A multidisciplinary team comprised of physicians, hematologists, government agencies, non-profit organizations, healthcare management organizations, and individuals living with SCD and their family members, has been working on a statewide sickle cell disease plan to address the lack of resources to proficiently care and treat individuals of all ages living with sickle cell disease. The plan will help coordinate and improve collaboration in the areas of education, outreach, treatment, and funding.

Community-based organizations at work

Currently, DHEC maintains partnerships with four sickle cell community-based organizations — the James R. Clark Memorial Sickle Cell Foundation, the Louvenia D. Barksdale Sickle Cell Anemia Foundation, the Orangeburg Area Sickle Cell Foundation and the COBRA Human Services Agency Sickle Cell Program. Through these partnerships, more people with sickle cell are able to obtain services and support. These organizations work to provide education, counseling, testing for sickle cell trait, and family support.

Although September is National Sickle Cell Awareness Month, increasing public and community knowledge about sickle cell is a 365-day initiative. Raise the awareness bar on sickle cell trait and sickle cell disease.

If you have questions about testing for you or your family, you can visit one of the four sickle cell community-based organizations. For more general information about sickle cell, visit cdc.gov/sicklecell or www.nhlbi.nih.gov/health/health-topics/topics/sca.

By Malerie Hartsell, MPH, CHES
Program Coordinator
Children with Special Health Care Needs

September is National Sickle Cell Awareness Month. The purpose of the month is to bring attention to a crippling genetic disease that many people around the world struggle with and manage daily.

Sickle Cell Awareness Month helps to debunk stereotypes and stigmas associated with persons who have sickle cell disease. Not only does this month shine light on sickle cell disease (SCD) but also sickle cell trait (SCT).

Here are Key Points to know about SCD & SCT:

SCD is a blood disorder that causes sickling of the red blood cells.
SCD is the most common hereditary blood disorder.
Red blood cells become hard and sticky and look like a C-shaped farm tool called a “sickle”.
Persons who have SCD may suffer from crises — acute episodes of intense and excruciating pain.
Different types of sickle cell disease are caused by different genetic mutations.
SCT is where a person inherits one sickle cell gene and one normal gene from either of their parents.
Persons with SCT can pass the trait on to their children.

Who is affected?

While sickle cell disease affects millions of people throughout the world, it is more common among African-Americans. It is estimated that SCD affects 100,000 people in the United States.

(Source: cdc.gov/ncbddd/sicklecell/traits.html)

Sickle cell trait is estimated to affect 1 million to 3 million Americans and 8 to 10 percent of African-Americans. Sickle cell trait can also affect Hispanics, South Asians, Caucasians from southern Europe, and people from Middle Eastern countries. More than 100 million people worldwide have sickle cell trait.

While SCD is inherited when a child receives two sickle cell genes from both parents, for someone that has SCT, the likelihood of having a child that has SCD or SCT is different. If both parents have SCT, there is a 50 percent chance the child will have SCT, a 25 percent chance the child may have SCD, and a 25 percent chance the child will have not have SCD or SCT.

Care and Treatment

Early diagnosis and monitoring can make a difference in the quality of life and number of years lived for someone with SCD. The Center for Disease and Control recommends:

Babies from birth to 1 year of age should see a doctor every two to three months.
Children from 1 to 2 years of age should see a doctor at least every three months.
Children and adults 2 years of age or older should see a doctor at least once every year.

Persons with SCD should be referred to a hematologist or an experienced general pediatrician, internist or family practitioner.

There are treatments that can help to prevent complications and medicate the acute or chronic pain episodes as they occur. Treatment options for persons with SCD differ by patient. Children with SCD can begin taking daily doses of penicillin starting at birth up to 5 years to help prevent bacterial infections. Blood transfusions are utilized to help reduce the risk of stroke. Hydroxyurea, a drug that increases levels of fetal hemoglobin that is less likely to become sickle-shaped, has helped reduce pain, hospitalizations and lung damage.

Currently hematopoietic stem cell transplantation (HSCT) is the only cure for SCD.

For more information about sickle cell, visit http://www.cdc.gov/ncbddd/sicklecell/index.html or http://www.nhlbi.nih.gov/health/health-topics/topics/sca.