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.

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 …