Safety and caregiver satisfaction with gastrostomy in patients with Ataxia Telangiectasia
Open Access
- 1 January 2011
- journal article
- Published by Springer Science and Business Media LLC in Orphanet Journal of Rare Diseases
- Vol. 6 (1), 23
- https://doi.org/10.1186/1750-1172-6-23
Abstract
Ataxia Telangiectasia (A-T) is a rare monogenetic neurodegenerative disease with pulmonary, nutritional, and dysphagic complications. Gastrostomy tube (GT) feedings are commonly recommended to manage these co-morbidities. In general, outcomes of GT placement in patients with progressive diseases that develop during childhood are not well characterized. The primary purposes of this study were to determine whether GT placement in patients with A-T would be tolerated and associated with caregiver satisfaction. We completed a retrospective review of 175 patients who visited the A-T Children's Center at Johns Hopkins Hospital from 2001 through 2008, and identified 28 patients with A-T (19 males, 9 females) who underwent GT placement for non-palliative reasons. Information was obtained from medical records, interviews with primary health care providers, and 24 (83%) caregivers of patients with GT's who responded to survey requests. Twenty-five (89%) patients tolerated GT placement and were a median of 5.0 (0.4-12.6) years post GT placement at the time of this investigation. Three (11%) patients died within one month of GT placement. In comparison to patients who tolerated GT placement, patients with early mortality were older when GT's were placed (median 24.9 vs. 12.3 years, p = 0.006) and had developed a combination of dysphagia, nutritional, and respiratory problems. Caregivers of patients tolerating GT placement reported significant improvements in mealtime satisfaction and participation in daily activities. GT placement can be well tolerated and associated with easier mealtimes in patients with A-T when feeding tubes are placed at young ages. Patients with childhood onset of disorders with predictable progression of the disease process and impaired swallowing may benefit from early versus late placement of feeding tubes.Keywords
This publication has 43 references indexed in Scilit:
- Outcome After Percutaneous Endoscopic Gastrostomy in Children and Young AdultsJournal of Pediatric Gastroenterology and Nutrition, 2010
- Prévalence et conséquences de la dénutrition au cours de la bronchite chronique de l’enfantArchives de Pédiatrie, 2008
- Death after PEG: results of the National Confidential Enquiry into Patient Outcome and DeathGastrointestinal Endoscopy, 2008
- Surgery for Aspiration: Analysis of Laryngotracheal Separation in 23 ChildrenDysphagia, 2007
- Surgery in disabled children: General gastroenterological aspectsActa Paediatrica, 2006
- Risk Scoring in Perioperative and Surgical Intensive Care Patients: A ReviewCurrent Surgery, 2006
- Does gastrostomy tube feeding in children with cerebral palsy increase the risk of respiratory morbidity?Archives of Disease in Childhood, 2006
- Survival probability in ataxia telangiectasiaArchives of Disease in Childhood, 2005
- Impact of gastrostomy tube feeding on the quality of life of carers of children with cerebral palsyDevelopmental Medicine and Child Neurology, 2004
- THE GENETIC DEFECT IN ATAXIA-TELANGIECTASIAAnnual Review of Immunology, 1997