ABSTRACT
Adrenoleukodystrophy has only recently been recognized as a disorder unique to children. Its debilitating sequelae have a profound impact on the physical, psychosocial, and cognitive development of children. Superimposed on coping with the crisis of illness is the stressor of hospitalization, a common experience for children with adrenoleukodystrophy. This article focuses on nursing's contribution to facilitating children's adaptation to the stressors of a progressive disorder, adrenoleukodystrophy, and hospitalization. Utilizing a developmental approach, the psychosocial needs of these children are addressed in a format based on the nursing process.
Subject(s)
Adrenoleukodystrophy/nursing , Diffuse Cerebral Sclerosis of Schilder/nursing , Nursing Assessment , Patient Admission , Adrenoleukodystrophy/psychology , Child , Child, Preschool , Chronic Disease , Humans , Male , Nursing Diagnosis , Social AdjustmentABSTRACT
Adrenoleukodystrophy is an X-linked disorder characterized by adrenal insufficiency and progressive demyelination of the cerebral white matter. Young boys usually become symptomatic during pre- or primary school years and follow a time-variable, downhill, terminal course. Diagnostic and carrier testing and prenatal diagnosis are available. Several treatments have been used but presently treatment is symptomatic. Nursing in adrenoleukodystrophy involves management of issues including but not limited to diagnosis, genetics, counseling, physical care, emotional care, family care and research.
