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Safety and caregiver satisfaction with gastrostomy in patients with Ataxia Telangiectasia.

Lefton-Greif MA, Crawford TO, McGrath-Morrow S, Carson KA, Lederman HM - Orphanet J Rare Dis (2011)

Bottom Line: 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.

View Article: PubMed Central - HTML - PubMed

Affiliation: The Ataxia Telangiectasia Clinical Center, Johns Hopkins Medical Institutions, Baltimore, Maryland, USA. mlefton@jhmi.edu

ABSTRACT

Background: 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.

Methods: 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.

Results: 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.

Conclusions: 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.

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A-T neurologic score and age of GT placement. Gray dots represent A-T neurologic scores for all 347 patients with A-T who were evaluated at the ATCC. Blue crosses represent the A-T patients who tolerated GT placement. Red circles represent the three patients with early mortality.
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Figure 2: A-T neurologic score and age of GT placement. Gray dots represent A-T neurologic scores for all 347 patients with A-T who were evaluated at the ATCC. Blue crosses represent the A-T patients who tolerated GT placement. Red circles represent the three patients with early mortality.

Mentions: Age of GT placement was marginally correlated with neurologic score (Pearson's r = -0.33; p = 0.08). (Figure 2) Age of GT placement was associated with tolerating GT placement (OR = 0.62; 95% confidence interval (CI) = 0.41-0.94); for each year increase in age there was a 38% decrease in the odds of tolerating GT placement. Factors examined that were not statistically significant for association with tolerating GT placement were male gender (OR = 4.81; 95% CI = 0.22-319.8), dysphagia (OR = 0.27; 95% CI = 0.00-2.69), neurologic score (OR = 1.02; 95% CI = 0.96-1.08 for a 1 unit increase) and BMI Z-score (OR = 1.11; 95% CI = 0.78-1.57 for a 1 unit increase). (Table 3) Pre-GT BMI Z-scores were obtained less than two years (median: 0.50 years, range: 0.08 to 1.40 years) before GT insertion for 16 patients tolerating tube placement. The median pre-GT BMI Z-score for these 16 patients was -3.82 (range: -11.89 to 0.90). The median post-GT BMI Z-score from anthropometric data obtained on 17 patients at a median of 2.57 years (range: 0.45 to 5.79 years) after tube placement was -1.17 (range: -11.15 to 1.54). Pre- and post-GT BMI Z-scores were available on 10 patients, and the median change was 0.87 (range: -3.58- 6.72; Wilcoxon signed rank p = 0.32). BMI Z-scores improved or remained stable for seven (70%) patients and decreased in 3 (30%) patients after GT placement. (Figure 3) GT's were not used in two patients as recommended. Caregivers did not administer GT feedings for the patient with a decreased BMI-Z score after GT placement and the second patient chose to not use the GT. Early mortality was not predicted by BMI Z-score alone. Gender, IgA, IgG, and presence of dysphagia were comparable for patients tolerating GT placement and those with early mortality. Minor post-GT complications included wound infection in 1 (4%) patient, tube dislodgement in 1 (4%) patient, and wound leakage in 9 (36%) patients.


Safety and caregiver satisfaction with gastrostomy in patients with Ataxia Telangiectasia.

Lefton-Greif MA, Crawford TO, McGrath-Morrow S, Carson KA, Lederman HM - Orphanet J Rare Dis (2011)

A-T neurologic score and age of GT placement. Gray dots represent A-T neurologic scores for all 347 patients with A-T who were evaluated at the ATCC. Blue crosses represent the A-T patients who tolerated GT placement. Red circles represent the three patients with early mortality.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3116459&req=5

Figure 2: A-T neurologic score and age of GT placement. Gray dots represent A-T neurologic scores for all 347 patients with A-T who were evaluated at the ATCC. Blue crosses represent the A-T patients who tolerated GT placement. Red circles represent the three patients with early mortality.
Mentions: Age of GT placement was marginally correlated with neurologic score (Pearson's r = -0.33; p = 0.08). (Figure 2) Age of GT placement was associated with tolerating GT placement (OR = 0.62; 95% confidence interval (CI) = 0.41-0.94); for each year increase in age there was a 38% decrease in the odds of tolerating GT placement. Factors examined that were not statistically significant for association with tolerating GT placement were male gender (OR = 4.81; 95% CI = 0.22-319.8), dysphagia (OR = 0.27; 95% CI = 0.00-2.69), neurologic score (OR = 1.02; 95% CI = 0.96-1.08 for a 1 unit increase) and BMI Z-score (OR = 1.11; 95% CI = 0.78-1.57 for a 1 unit increase). (Table 3) Pre-GT BMI Z-scores were obtained less than two years (median: 0.50 years, range: 0.08 to 1.40 years) before GT insertion for 16 patients tolerating tube placement. The median pre-GT BMI Z-score for these 16 patients was -3.82 (range: -11.89 to 0.90). The median post-GT BMI Z-score from anthropometric data obtained on 17 patients at a median of 2.57 years (range: 0.45 to 5.79 years) after tube placement was -1.17 (range: -11.15 to 1.54). Pre- and post-GT BMI Z-scores were available on 10 patients, and the median change was 0.87 (range: -3.58- 6.72; Wilcoxon signed rank p = 0.32). BMI Z-scores improved or remained stable for seven (70%) patients and decreased in 3 (30%) patients after GT placement. (Figure 3) GT's were not used in two patients as recommended. Caregivers did not administer GT feedings for the patient with a decreased BMI-Z score after GT placement and the second patient chose to not use the GT. Early mortality was not predicted by BMI Z-score alone. Gender, IgA, IgG, and presence of dysphagia were comparable for patients tolerating GT placement and those with early mortality. Minor post-GT complications included wound infection in 1 (4%) patient, tube dislodgement in 1 (4%) patient, and wound leakage in 9 (36%) patients.

Bottom Line: 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.

View Article: PubMed Central - HTML - PubMed

Affiliation: The Ataxia Telangiectasia Clinical Center, Johns Hopkins Medical Institutions, Baltimore, Maryland, USA. mlefton@jhmi.edu

ABSTRACT

Background: 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.

Methods: 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.

Results: 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.

Conclusions: 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.

Show MeSH
Related in: MedlinePlus