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Gastrostomy buttons for nutritional support in children with cystinosis
1Children and Young People's Kidney Unit, Nottingham City Hospital NHS Trust, UK. watpaed@aol.com
Insights
Gastrostomy buttons (GBs) improved nutritional intake and weight in children with cystinosis. These devices effectively delivered nutrition and medications, showing positive growth outcomes.
Area of Science:
- Pediatric Nephrology
- Clinical Nutrition
- Gastroenterology
Background:
- Cystinosis often leads to poor growth due to nutritional deficits and gastrointestinal issues.
- Managing complex medical needs in children with cystinosis requires effective nutritional and medication delivery methods.
Purpose of the Study:
- To evaluate the efficacy and safety of gastrostomy buttons (GBs) in improving nutritional status and growth in pediatric cystinosis patients.
- To assess the feasibility of using GBs for both nutritional support and medication administration in this population.
Main Methods:
- A retrospective case series of five children with cystinosis receiving overnight gastrostomy button feeding.
- Data collected on feeding duration, button lifespan, medication administration, energy intake, and anthropometric measurements (height and weight SDS).
Main Results:
- Gastrostomy buttons were used for a mean of 27 months, with a mean button life of 5.7 months.
- Mean energy intake reached 118% of requirements, with GBs delivering 58% of energy and 55% of protein.
- Significant improvement observed in weight standard deviation score (SDS) (from -3.79 to -2.17, P=0.042) and a trend towards improvement in height SDS.
Conclusions:
- Gastrostomy buttons are a valuable tool for ensuring adequate nutritional intake and medication delivery in children with cystinosis.
- GBs are well-tolerated by families and contribute to improved weight gain and overall growth in this challenging condition.
Abstract:
Poor growth in children with cystinosis may be a combination of sub-optimal nutritional intake, gastrointestinal dysfunction and polypharmacy. We report our experience with gastrostomy buttons (GBs) in five children (4 male) with cystinosis treated at a median age of 3 years (range 1.4-8.8 years). All children received overnight GB feeding for a mean duration of 27 months (range 7-42 months). During 135 patient-months of observation, the mean life of the GB was 5.7 months (range 3-7.7 months), with a mean of 3.4 GB changes (range 1-6) per patient. An average of five (range 3-7) medications were administered daily via the button. The mean energy intake achieved was 118% of the estimated average requirement (range 86%-183%), with 58% of total energy and 55% of protein intake being delivered via the GB during the study period. Mean height standard deviation score (SDS) was -3.79 at the start of GB feeding and -3.08 at the end of the study period, with mean weight SDS of -3.79 and -2.17 (P=0.042), respectively. The buttons have been welcomed by the families and should be considered as a treatment option for providing the nutritional prescription and administering medications.