Safety and caregiver satisfaction with gastrostomy in patients with Ataxia Telangiectasia

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.Ataxia Telangiectasia (A-T) is a rare neurodegenerative disease characterized by ataxia, immunodeficiency, sinopulmonary infections, premature aging, nutritional compromise and oropharyngeal dysphagia[1-6]. The prevalence of A-T is estimated to be between 1 in 40,000 and 1 in 300,000 live births[7-9]. Life expectancy has increased from 19 to 25 years, with respiratory failure, complications of chemotherapy or cancer, and neurologic deterioration remaining the leading causes of death[10]. Increased morbidity and mortality is associated with impairments in deglutition, nutritional status, immune function, and neurologic status in other chronic progressive co