Written by: Kairav Shah, MD
Sickle cell disease is an inherited group of disorders that affects hemoglobin, the molecule in red blood cells that transports oxygen to cells throughout the body. It is an autosomal recessive genetic disease, caused by a mutation in the hemoglobin-Beta gene found on chromosome 11. In simple terms, people with this condition have abnormal hemoglobin that converts red blood cells into sickle or crescent shapes.
The signs and symptoms of sickle cell disease include anemia, jaundice, pulmonary hypertension, retinopathy, stroke and renal failure. A classic symptom of the condition is severe pain. Painful episodes, or “pain crises,” can occur when sickled red blood cells — which are stiff and inflexible — get stuck in small blood vessels, preventing the flow of blood and oxygen to the organs.
Signs and symptoms usually begin in early childhood. The severity of symptoms varies from person to person. Some people have mild symptoms, while others are often hospitalized for more serious complications. These frequent and disruptive pain crises can greatly impact the growth and development of a child, potentially leading to difficulties with social isolation, forming and maintaining peer relationships, academics, and developing a cohesive identity.
Patients often suffer from significant psychological distress due to the complications of sickle cell disease. Their pain can represent a non-specific sign of a mental health disorder. Some of the most common psychiatric comorbidities are depression, anxiety disorders, sleep disorders, and bipolar disorder. Another major concern is opioid use disorder, as many patients are on long-term opioid therapy to reduce chronic pain. Poor mental health can also worsen symptoms of sickle cell disease, making it more difficult to cope. Stress, in particular, is a common trigger of painful episodes.
Although these psychological characteristics are palpable, they are rarely assessed. All patients with sickle cell disease should be routinely screened for mental health conditions. Early identification and treatment is crucial for effectively managing pain, reducing emergency room visits and hospitalizations, and improving quality of life. Treatment options include pharmacotherapy, cognitive behavioral therapy, and psychoeducation. Certain antidepressant medications can also help with pain management.
The UT Physicians Comprehensive Sickle Cell Center is an outpatient clinic dedicated to treating patients with sickle cell disease. It offers primary care services, an infusion center, behavioral health care, and various specialties such as cardiology, pulmonary, and dental services. This unique facility is the only family comprehensive sickle cell center in Houston.
Plaza Medical Center
1200 Binz , Suite 850
Houston, TX 77004
713-486-8876 (24-Hour Nurse Help Line)
Kairav Shah, MD, is a board-certified psychiatrist who treats adult patients 18 years and older at UT Physicians Comprehensive Sickle Cell Clinic and UT Physicians-Rosenberg locations. His special interests are ADHD, anxiety, depression, sickle cell disease, pain management, substance use disorders, and OCD.