By Malerie Hartsell, MPH, CHES
Children with Special Health Care Needs
Did you know September is National Sickle Cell Awareness Month?
Efforts to recognize Sickle Cell Month began in 1983 when the Congressional Black Caucus introduced the resolution to Congress. President Reagan signed the resolution in August of 1983 making September National Sickle Cell Awareness Month. Since then, organizations across the globe have increased public awareness surrounding this crippling illness.
By recognizing National Sickle Cell Awareness Month, more individuals are educated about the struggles that come along with daily management of this debilitating genetic disease. Increasing awareness is one way to debunk stereotypes and stigmas associated with persons who have sickle cell disease (SCD) and further highlights risk factors related to SCD, such as having the sickle cell trait (SCT).
What is SCD? 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.
Who is affected? The actual number of persons living with SCD is unknown in the United States, but it is estimated that SCD affects approximately 100,000 people annually. Sickle cell disease also affects millions of people worldwide and it is more common among African-Americans.
What is SCT? SCT is where 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 common is SCT? Sickle cell trait is an inherited blood disorder that affects 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. (Source: http://www.hematology.org/Patients/Anemia/Sickle-Cell-Trait.aspx)
SCD and SCT are inherited conditions, which means a person may be born with the illness or trait. SCD or SCT cannot develop overtime nor is it contagious. 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 that child may have SCD, and 25 percent chance the child will 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 Centers for Disease Control and Prevention 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 from 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.
Currently, only hematopoietic stem cell transplantation (HSCT) can cure SCD; however, this is very intensive treatment and not everyone qualifies or can afford this treatment. To help prevent complications and medicate the acute or chronic pain episodes as they occur, hematologist will start children on daily doses of penicillin at birth up until at least 5 years of age. Blood transfusions are utilized to help reduce the risk of stroke. Additionally, hydroxyurea, a drug that increase the levels of fetal hemoglobin has helped reduce pain, hospitalizations, and lung damage.