Confronting Adversity: Pain, Anxiety, and Sickle Cell Anemia in Adolescence
Authors: Vuppala Neil K 
Published: June 2024

Abstract

Presentation Date: 6/8/2024
Presentation Start Time: 6:00:00 PM

Background
Sickle cell anemia represents a genetic hemoglobin disorder marked by the distortion of red blood cells into a sickle shape, rendering individuals more susceptible to episodes of vaso-occlusive crises. These painful crises are known for their severity with their frequency and intensity influenced by various factors, including emotional stress and anxiety. While the physiological mechanisms driving these crises are well-established, the impact of psychological factors remains a subject of significant clinical interest.
Anxiety, frequently observed in conjunction with sickle cell anemia, can initiate a chain reaction of physiological responses. The release of stress-related hormones like adrenaline and cortisol may induce vasoconstriction, further obstructing the normal blood flow and exacerbating the sensation of pain. Additionally, anxiety can lower an individual’s pain threshold, intensifying the perception of discomfort. This heightened pain perception may lead to “pain catastrophizing,” wherein individuals interpret their pain as more severe than objective measures suggest. Furthermore, anxiety can profoundly affect an individual’s overall quality of life, potentially leading to withdrawal from social interactions, heightened levels of worry, and intrusive obsessive thoughts.

Methods
Case Presentation: We present the complex case of a 17-year-old African-American female referred to the child psychiatry clinic by St. Jude’s Hospital. Her referral stemmed from recurrent, debilitating chronic pain episodes, leading to frequent visits to the emergency department and hospitalizations. Her primary medical condition is sickle cell anemia, with her struggle against pain crises commencing at the age of 10 only intensifying throughout her adolescence, resulting in substantial school absences. Concurrently, she was diagnosed with anxiety at 13, forming a complex interplay where one condition exacerbates the other. Additionally, her medical history includes a prior diagnosis of prolonged QT syndrome, which reached a critical point when she experienced a cardiac arrest with torsade de pointes. This medical history necessitated a careful approach to medication selection due to the expected adverse effect of QT prolongation associated with many 1st-line medications to treat her anxiety. In her family history, her younger sister was diagnosed with sickle cell anemia, while her brother possesses the sickle cell trait. The patient’s surgical history includes a cholecystectomy and wisdom teeth extraction, and her medication regimen initially included folic acid, hydroxyurea, Asmanex, and Lortab.

Results
N/A

Conclusions
This case showcases the intricate relationship between chronic pain crises in sickle cell anemia and the amplifying effects of anxiety. The vital need for comprehensive patient care is underscored by the physiological responses, heightened pain perception, and emotional distress seen in this patient. This case report delves into the complexities of addressing these challenges, emphasizing the importance of a multidisciplinary approach to optimize the patient’s overall well-being and enhance her quality of life.