Sickle Cell Disease

Sickle cell disease (SCD) is a life-threatening inherited red blood cell disorder affecting millions worldwide but primarily affecting the African American/Black population in the United States. In SCD, the production of abnormal hemoglobin S limits oxygen and blood flow throughout the body. Despite advances in treatment (e.g., blood transfusions and hydroxyurea therapy), patients with SCD have high levels of healthcare utilization because of acute and chronic complications that include pain and damage to the organs, bones, and vascular system. They are also at significant risk for neurologic impact, including stroke and cognitive deficits that occur primarily in the domains of executive function and processing speed. Patients with SCD experience functional impairment related to acute and chronic pain, poor quality of life, anxiety, and depression. Pain and adherence are the most common referral concerns, followed by referrals related to mood, development (e.g., academic concerns), and transition to adult care. Acceptance of psychological interventions (e.g., psychoeducation, cognitive behavioral therapy, motivational interviewing) are complicated by patient experiences with racial bias within the healthcare system, health-related stigma, misunderstanding of the role of psychological services, and perceived lack of cultural competency by providers. The consultation-liaison psychologist has an opportunity when working with patients with SCD to decrease potential stigma around mental health, contextualize the role of the pediatric psychologist in their work with patients with a chronic condition, support the medical team in their approach to pain management, and interact regularly with the entire psychosocial team to support consistent care.