Gastrostomy buttons for nutritional support in children with cystinosis.

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.