Effects of Emotional Symptoms on Pain and Social Functioning in Youth with Sickle Cell Disease

Abstract

Sickle cell disease (SCD) is a group of genetic blood disorders primarily affecting people of African, Latino, and Mediterranean descent and has currently been diagnosed in over 100,000 Americans. This chronic disease causes red blood cells (RBC) to take on a sickled or C-shaped form, which obstructs blood flow, consequentially leading to a multitude of complications. The most prevalent complication is acute pain (Steinberg, 1999). SCD pain, also known as vaso-occlusive pain, results in about 70,000-90,000 yearly hospitalizations, and over 200,000 emergency room visits each year (National Heart, Lung, and Blood Institute [NHLBI], 2015). Patients most frequently report pain in the chest, back, and abdomen, while forty percent of all patients will develop a fatal complication known as acute chest syndrome (Steinberg, 1999). Pain episodes that lead to patients being hospitalized are known as pain crises, and they are commonly treated with blood transfusions, hydration, and opiates. To date, there is only one proven cure for SCD, which is a bone marrow transplant. Bone marrow transplants are very rare and associated with host of additional complications. As patients with SCD grow older, their pain tends to worsen. This is particularly problematic in children and adolescents, because it affects their school attendance and limits their ability to participate in social activities (Shapiro et al, 1995). This makes it difficult to establish and maintain friendships, and may cause some kids to retreat into themselves. Children and adolescents with SCD report more feelings of shyness and loneliness, and are more likely to be victims of bullying (Forgeron et al., 2010). Studies have suggested that poor emotional functioning may also lead to reduced social interactions among youth with SCD; thus, leading to poorer social functioning in this population. Also, poor emotional functioning may worsen the impact of pain on social functioning. However research exploring the impact of emotional functioning on social outcomes and the impact of pain on social outcomes in the pediatric SCD population is limited. The purpose of the current study is address this lack of research by exploring the influence of emotional functioning on social outcomes and the impact of pain on social outcomes in youth with SCD. The following literature review provides an in depth description of SCD, which is followed by descriptions of social and emotional functioning in youth with SCD.