Growing Up With a Deadline
The handful of children born with the devastating disorder known as Batten disease have a life expectancy of only eight to twelve years. Sammie just turned eight.
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Photos by Maria Edible
Sammie stares out the window, where the sun is just starting to replace the rain. Around her neck: a raspberry, pig-shaped pillow. She groans faintly as her mother places a tissue between her teeth to prevent a bitten lip, then covers her nose and mouth with a clear mask that has jagged pink projections — dinosaur spikes, simultaneously innocent and stern. Her feet are pointed, toes curled. A symptom of immobility, her mother calls them “ballerina feet.” Sammie’s arms twitch slightly as a small compressor starts up, emitting a sound like a muffled lawnmower.
The dino mask administers Pulmicort, a steroid that helps loosen mucus, which accumulates due to Sammie’s lack of motion. The twice-a-day treatment raises her oxygen levels in order to facilitate breathing and minimize the chance of pneumonia.
Sammie’s eight-year-old body is regressing due to a condition known as Spielmeyer-Vogt-Sjögren-Batten disease, commonly referred to as Batten disease, which occurs in an …
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