Psychological aspects of patients with Niemann Pick disease, type B

• Main Author: Henderson, Shelly L
• Format: Dissertation
• Language: English
• Published: ProQuest Dissertations & Theses 01.01.2006
• Subjects: Clinical psychology; Mental health; Psychotherapy
• ISBN: 9780549025597; 0549025596

A relatively rare and unknown disease, scientists and health care providers have only begun to understand the medical aspects of Niemann Pick disease (NPD). Even less information is known about the psychological effects of living with NPD. Patients with NPD and their families face numerous psychological stressors including extensive medical testing, uncertainty of diagnosis, living and coping with a chronic illness, and grief and bereavement surrounding this progressively debilitating, and ultimately, fatal disease. The current study is the first investigation of the psychosocial consequences of NPD type B on patients and families. The study draws upon the developmental theory of Erikson (1968) and explores the relationship between psychological distress, psychosocial development, and the transition to adulthood in the context of the chronic illness NPD type B. A qualitative case study methodology was used to explore the human experiences of NPD type B patients and families. Information on the patients' behavior, quality of life, psychological distress, and psychosocial development was collected through standardized parent and self-report measures. Interviews with adolescents and their parents, as well as adult patients elicited information regarding life experiences, psychological development, school, relationships, and medical experiences in the context of NPD type B. Patients and families were also asked to make recommendations for improving care of patients and families. Four major findings emerged from the interviews: (a) all participants identified limited physical activity, social isolation, and peer rejection as significant stressors; (b) these stressors had a specific impact during the age span of 10 to16 years; (c) parents and adult patients expressed significant frustration regarding the lack of information and treatment available; and (d) adult patients identified early medical experiences as having a considerable psychological impact. The results of this investigation further awareness of the psychological impact faced by those living with a chronic illness. Although most of the participants showed relatively good adjustment, areas of psychosocial maladjustment were revealed. Accordingly, the psychological and social needs of NPD type B patients and families should be addressed. This study calls for a collaborative, multidisciplinary effort in the treatment of these unique patients and their families.