Coping with the Pressure of Adrenoleukodystrophy（ALD）
Date Issued
2006
Date
2006
Author(s)
Huang, Ai-Chu
DOI
zh-TW
Abstract
This study aimed at understanding the expectation, planning for future and copying mechanisms of the adrenoleukodystrophy patients.
We hope through the study of those individual patients, we can find the best way to help those ALD patients, not only to fulfill their medical need, but also to relieve their immense "hopeless" feeling.
By using case study method, 8 ALD patients, both affected but asymptomatic yet and symtomatic but still able to express freely, were interviewed and observed. Informed consents were obtained from each subject.
We collected all the medical records and underwent interview on each office visit for information including daily life function, perception and cognition about ALD and coping mechanisms within family and school.
Our study revealed:
（1）AMN( adrenomyeloneuropathy )type demonstrated clear "hopelessness" for the future while juvenile type, infantile type , adrenal type and asymptomatic cases showed more hope and expectation for the future. The extent of family intimacy seems to contribute to the degree of hopefulness.
（2）Patient age is an important determinant of the behaviors and coping mechanisms in ALD patients. The younger they are, the less fear they show. Parental attitudes were found critical in this dimension.
This investigator has come up a protocol for the examination of suspected ALD patients. If high enough scores were present from this checklist, ALD should be highly suspected and proper clinical workup is instituted in order not to delay timely clinical intervention for the confirmed ALD patients.
Since bone marrow transplantation is a treatment option often considered in the disease course, great efforts should be taken to communicate with ALD patients and their family for their full understanding of the procedures, risks and prognosis of bone marrow transplantation?
In conclusion, the clinical course of ALD is highly variable and unrespectable and the psychological impact is immense. Proper psychological care should be offered in each office visit and hospitalization.
Subjects
遺傳諮詢
腎上腺腦白質退化症
骨髓移植
疾病因應
個案研究
genetic counseling
adrenoleukodystrophy
bone marrow transplantation
coping
case study
Type
other