This retrospective study demonstrates that safe GT placement and caregiver satisfaction can be achieved when patients with A-T have feeding tubes placed at younger ages and before the accumulation of severe complications associated with nutritional compromise or dysphagia with concomitant aspiration. Therefore, we recommend GT's when patients with A-T are young and begin to present with nutrition, respiratory, and dysphagic compromises that are unresponsive to common conservative measures (e.g., dietary modifications and medical therapies) or when feeding disrupts activities of daily living. Although A-T is a rare and complex disease, these findings may have clinical relevance to other children and young adults with neurodegenerative conditions being considered for GT placement.
Survival for patients with A-T has increased with good quality care[10
]. Our study was not designed to evaluate the impact of GT's on life expectancy. Longitudinal studies are needed to determine whether early GT placement improves survival. Nonetheless, GT placement is likely to be beneficial for some patients with A-T and we believe that this investigation shows that safety of GT placement can be improved.
At the time of this investigation, our patients who tolerated GT placement had feeding tubes for a median of 5.04 years and approximately two-thirds of them were alive. Those who expired lived to an average age of 20 years, comparable to previously reported survival data[10
]. Causes of death were cancer or complications of cancer (55%), congestive heart failure (22%), and respiratory complications including aspiration (22%). Therefore more than half of the deaths among those who tolerated GT insertion were related to conditions or processes unlikely to be influenced by placement of a feeding tube. We continue to follow surviving patients to ascertain the long term impact of GT placement. Given that many factors influence survival we will focus on measures, such as weight gain and stability, frequency of respiratory tract infections, and the onset of dysphagic presentations. Additionally, we will track changes in the ability to participate in daily routines that are most directly related to GT placement. The latter outcome is a key factor in the quality of life of those increased survival years.
Three (11%) patients died within 30 days of GT placement. (Table ) None of these patients had GT's placed at a tertiary care center. Although our 30-day mortality rate was comparable to the 0 - 27% mortality rates reported in other populations undergoing GT insertion [2
], the differences between those who tolerated GT placement and those with early mortality are substantial. The three with early mortality had GT's placed at older ages and all demonstrated co-morbidities associated with poor outcomes with GT placement that have been identified in other patient populations with progressive conditions including advanced lung disease, malnutrition, aspiration, and immune deficiency[18
]. Nonetheless, their immunoglobulin levels, BMI, and neurologic scores did not significantly differ from those who tolerated GT placement. Postponing GT placement as long as possible in patients with A-T may not be in a patient's best interest. Minor complication rates were comparable to the wide range of 6 - 95% previously reported[2
Despite efforts to place GT's at younger ages and before the development of significant co-morbid conditions, some of our patients present to clinic when they are older and have developed risk factors associated with poor outcomes in other patient populations[11
]. For these patients, we recommend evaluations and interventions that may help minimize complications including nutritional rehabilitation when needed [38
] and initiation of necessary pulmonary interventions before GT placement[39
]. Due to the increased risk of complications in all patients with A-T, we recommend placement of GT's at a tertiary medical care center. Additionally, the risk and benefits of GT placement should be discussed with the patient and guardian. Those with more significant lung pathology, including an abnormal chest x-ray, compromised pulmonary function, the need for bronchodilators, older age and concurrent other medical problems may be at higher risk for pulmonary complications during anesthesia[42
]. Post-operatively, a slow re-introduction of feeds to minimize feeding intolerance, and prompt weaning from mechanical ventilation and early extubation may minimize complications associated with underlying respiratory disease in patients with A-T[44
Caregiver's were very satisfied with GT's and reported that patients had more energy to participate in daily activities. Our results are comparable to other reports of meals being easier and more enjoyable for caregivers and patients alike[2
]. Nonetheless, one of the limitations of this study was that all respondents were caregivers of patients who tolerated GT placement. Additionally, it is possible that satisfied caregivers were more likely than displeased caregivers to respond to our inquiries and that recollection bias may have played a role in their responses. Another potential limitation is that the survey was comprised of descriptive terms that were not defined precisely and may have been open to variable interpretation.
Many of the limitations of this investigation can be attributed to the retrospective study design and small sample size. For example we were unable to determine the impact of GT feedings on nutritional status because our data did not allow us to standardize the timing of anthropometric measures before and after GT placement, verify whether all patients used GT's as recommended, and obtain complete sets of comparable data for all patients. Additionally the natural course of anthropometric changes associated with disease progression has not been characterized. The small sample size (e.g., three patients in the early mortality group) may contribute to possible discrepancies between findings of clinical and statistical significance. Available records did not permit identification of a control group of patients who did not undergo the procedure despite our recommendations for GT placement. It is notable that GT placement had been recommended for several years prior to their eventual placement in the three patients with early mortality. During the interval between the initial recommendation for GT placement and tube insertion, nutrition and respiratory compromises increased for these three patients. Prospective investigations that track markers of respiratory and nutrition status pre- and post-GT placement are needed to facilitate decision making for determining when GT's should be placed.