Palliative care in 9 children with neurodegeneration with brain iron accumulation

Title

Palliative care in 9 children with neurodegeneration with brain iron accumulation

Creator

Dangel T; Kmiec T; Januszaniec A; Wazny B

Publisher

Neurological Sciences

Date

2020

Subject

article; body mass; bone density; bone strength; botulinum toxin; child; clinical article; contracture; controlled study; densitometry; dystonia; facilitation; female; femur; home care; Hospice and Palliative Care Nursing; human; hypersalivation; iron; ketogenic diet; male; multidisciplinary team; neurodegeneration with brain iron accumulation; pain; palliative therapy; pathologic fracture; prevention; questionnaire; school child; skeleton; spasticity; spine; thorax

Description

Aim: Evaluation of pediatric palliative home care of families with children suffering from neurodegeneration with brain iron accumulation (NBIA) and their parents. Material and methods: The children were treated at home by a multidisciplinary team. Densitometry was used to evaluate the condition of the skeletal system. Botulinum toxin was injected into the muscles in doses between 22 and 50 units/kg. The quality of palliative care was assessed on the basis of a specially designed questionnaire for parents. Results: The observations were performed on a group of 9 patients with NBIA. On admission, the median age of patients was 9 years (7-14). The average time of palliative home care was 1569 days (34 days-17 years). The median age at death (6 patients) was 11 years (7-15). The botulinum toxin injections gave the following results: reduction of spasticity and dystonia, reduction of spine and chest deformation, relief of pain and suffering, facilitation of rehabilitation and nursing, prevention of permanent contractures, and reduction of excessive salivation. Bone mineral density and bone strength index were reduced. Two patients experienced pathological fracture of the femur. The body mass index at admission varied between 9.8 and 14.9. In 7 cases, introduction of a ketogenic diet resulted in the increase of body mass and height. The ketogenic diet did not worsen the neurological symptoms. The parents positively evaluated the quality of care. Conclusion: Palliative home care is the optimal form of treatment for children with NBIA.

Rights

Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).

Citation List Month

April 2020 List

Collection

Citation

Dangel T; Kmiec T; Januszaniec A; Wazny B, “Palliative care in 9 children with neurodegeneration with brain iron accumulation,” Pediatric Palliative Care Library, accessed August 1, 2021, https://pedpalascnetlibrary.omeka.net/items/show/17020.

Social Bookmarking