Journal of pediatric oncology nursing : official journal of the Association of Pediatric Oncology Nurses
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J Pediatr Oncol Nurs · Mar 2007
Review Case ReportsJuvenile metachromatic leukodystrophy: understanding the disease and implications for nursing care.
Hematopoietic stem cell transplants are increasingly being performed in attempt to halt the progression of juvenile metachromatic leukodystrophy, which is a rare neurodegenerative disease. Children who are diagnosed with metachromatic leukodystrophy are not commonly cared for by nurses who specialize in pediatric stem cell transplants. ⋯ The article provides information about the pathophysiology of metachromatic leukodystrophy, the natural progression of symptoms, and how hematopoietic stem cell transplant may work to halt the progression of juvenile metachromatic leukodystrophy. It also focuses on the implications of nursing care, including a review of systems, the need for increased patient and family education, and the complexities of caring for a family with multiple affected children.
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J Pediatr Oncol Nurs · Mar 2007
Health-related quality of life following hematopoietic stem cell transplant during childhood.
The purpose of this study is to examine the self-reported and parent-reported health-related quality of life (HRQoL) of children (age<19 years) who received a hematopoietic (bone marrow, cord blood, or peripheral blood) stem cell transplant (SCT) at the University of Nebraska Medical Center (UNMC). A total of 35 families participated: 31 SCT recipients, 35 mothers, and 28 fathers. Mean scores were 73.6 child, 71.2 mothers, and 76.2 fathers (range of 0-100, with high scores indicating better HRQoL). ⋯ Older age at time of transplant was associated with higher procedural anxiety according to child (r=.498, P=.005) and mothers (r=.466, P=.008). Older age at time of transplant was associated with less worry according to fathers (r=-.589, P=.002). Overall, SCT recipients and their parents report moderately high HRQoL.