Sickle Cell Disease (SCD) As The Umbrella Term Used To Describe An Inherited Disorders
Sickle cell disease (SCD) is the umbrella term used to describe a host of inherited disorders known as hemoglobinopathies, or blood diseases affecting hemoglobin, including sickle cell anemia. SCD refers to disorders characterized by a predominance of hemoglobin S (for sickle) protein in red blood cells. Because of the predominance of hemoglobin S, when red blood cells lose their oxygen on their way back to the lungs, they become sickle shaped, or c-shaped, and stiff. Sickle cells clump together and block blood flow, which often causes extreme pain for the patient. These pain crises often necessitate immediate hospitalization. Another problem associated with SCD is that vaso-occlusive crises, or the blocking of blood flow, can lead to bone underdevelopment and damage. Other health problems associated with SCD include organ damage, improper physical development (e.g., stunted growth, elongated limbs), silent or overt neurological damage (e.g., strokes), and cognitive impairment. Children with less severe subtypes of SCD (e.g., those who are at reduced risk of strokes or cognitive impairment) are still about three to four times more likely to incur neurological complications than non-SCD children. In addition to the above problems, SCD can lead to increased pneumococcal infections, exacerbation of anemia, delayed puberty, and changes in the spleen.
According to the CDC, People with SCD begin displaying signs of the disease during the first year of life, generally around 5 months of age. The symptoms and complications of SCD are unique to each person and range from moderate to serious in severity. The reason that infants don’t display symptoms at birth is due to baby or fetal hemoglobin which protects the red blood cells from sickling. When the infant is around 4 to 5 months of age, the baby or fetal hemoglobin is replaced by sickle hemoglobin and the cells begin the sickling process. Which leads to the symptoms mentioned above. It is here where the African American family begins to face their hardships.
In summation, African American families who have infants born with SCD face tremendous hardships economically, physically, and emotionally. They contend with the financial burdens from numerous days missed from work due to hospitalization during crises, as well as mounting hospital bills, doctor visits and medication cost. Physically they suffer from the increased hours of lack of sleep due to extra nurturing required both in the home and during hospital stays. Emotionally, dealing with regular household duties coupled with the anxiety that, at any moment, a relapse can happen starting a crisis is equally if not most taxing on the psyche. But recognize that there are support groups out there to ease the burden. Groups such as The Sickle Cell Disease Association of America strive to help parents with local resources, coping methods, and educational materials. If you or someone you know falls into this category, please take the time to share this information with them.