When Alex Simpson was born, she seemed to be a typical baby in many respects—but she would cry for 20 hours a day. For two months, her parents didn’t know why. Then, an answer, reports KETV in Omaha, Neb. Alex was diagnosed with a rare congenital disorder called hydranencephaly, meaning she was born missing a good chunk of her brain.
Most babies with the condition die before their first birthday; the longest any known person has lived with hydranencephaly was 33 years, reports the Pediatric Brain Foundation. (A July 2014 study published in Acta Paediatrica puts the former figure at 35 years.) The foundation notes that some children display no obvious symptoms of the condition at birth, but “increased irritability, increased muscle tone, and the lack of purposeful movement generally initiate suspicion of an underlying cause.”
Alex is now 10. She has part of her cerebellum, allowing her to maintain some awareness of her surroundings. “She knows her mom and dad, her little brother,” her father Shawn Simpson says. “She knows when bad things are going on; she will cry or have a sad face.” That experience is backed up by the Acta Paediatrica study. It observed that “drastic loss of cortical tissue … has encouraged the default assumption that these children are not conscious.”
To get a better window into whether that was the case, researchers surveyed 108 primary caregivers of children with hydranencephaly, asking questions like “Will your child echo or imitate you?” (29% said yes) and “Does your child reach for objects?” (26% yes). Their conclusion, in part: “The indications that they are not only physiologically awake but conscious during waking makes it appropriate to apply the concept of quality of life to these children.” (This baby was born without a nose.)
By Elizabeth Armstrong Moore