Exploring the Illness Experience of Patients with Central Nervous System Hemangioblastomas in Von Hippel–Lindau Disease: A Qualitative Study

TL;DR

This study explores the emotional and psychological experiences of patients with Von Hippel–Lindau disease and brain tumors, highlighting their struggles and coping strategies.

Contribution

The study provides novel qualitative insights into the illness experience and psychological responses of VHL disease patients with CNS hemangioblastomas.

Findings

Patients reported feelings of powerlessness due to unpredictable disease progression.

Negative emotions like guilt, depression, and low self-esteem were commonly experienced.

Participants expressed persistent worry about family, finances, and future uncertainty.

Abstract

Background/Objectives: Von Hippel–Lindau (VHL) disease is a rare autosomal dominant hereditary disorder. Central nervous system hemangioblastomas are one of the most common tumor types associated with VHL disease. Although these tumors are histologically benign, delayed diagnosis and treatment may result in severe neurological dysfunction, permanent disability, and even death. However, little is known about the experiences of patients with VHL disease. The aim of this study was to gain a better understanding of the illness experiences and psychological responses of patients with VHL disease accompanied by central nervous system hemangioblastomas. Methods: A qualitative study based on a semi-structured guide was conducted. Twelve participants were recruited. Data were collected through face-to-face interviews and analyzed using the constant comparative method. Results: Four themes and their subthemes were identified: 1. powerlessness—unpredictable disease progression and uncontrollable continuity; 2. negative emotional experiences—guilt and self-blame, depression, and low self-esteem; 3. compromise—acceptance of fate, positive outlook, and sense of hope; and 4. persistent worry—worries about family members, anxiety regarding finances and employment, and uncertainty regarding the future. Conclusions: This study identified four major themes in the illness experiences of patients with VHL disease accompanied by central nervous system hemangioblastomas, which provided deep insights into the care needs of individuals with VHL disease. Healthcare providers should develop effective measures to enhance patients’ ability to maintain a good quality of life and confront the future with resilience.