News this week that a 16-year-old girl in England died from a gastrointestinal infection caused by a hair ball has put a rare condition known as Rapunzel syndrome in the spotlight.
Named after the Brothers Grimm fairy tale about a princess held hostage in a tower where the only way to enter or exit was through a window you could climb by crawling up her extremely long hair, Rapunzel syndrome refers to complications when a hairball blocks the digestive tract.
These hairballs are a type of bezoar — a hard, solid mass of human or vegetable fibers that accumulate in the gastrointestinal tract. The first reference of a human death caused by a bezoar occurred in 1779, when one was found during an autopsy. The masses that are made of hair are called trichobezoars, and unlike other types (like ones made up of accumulated pills or capsules), are common in patients with psychiatric disorders like trichotillomania (which causes people to pull out their own hair) and trichophagia (compulsion to eat hair). Most bezoars in children fall under this category and result from swallowing their own hair, as well as hair from dolls or brushes.
The Tech Times reported that Jasmine Beever, the girl who recently died in England from Rapunzel syndrome, likely had trichophagia, and according to a family friend had been chewing and sucking on her own hair for years. Over time, the accumulated hair formed a mass, which infected the lining of her stomach, eventually bursting a stomach ulcer and causing her organs to shut down.
Most cases of Rapunzel syndrome are reported in women and girls between the ages of 13 and 20 — especially in areas where long hair is common in the culture. There is at least one documented case in a male, who reportedly ate his sisters’ hair. An article in the Journal of Translational Internal Medicine published in 2015 said that to date, only 64 cases of Rapunzel syndrome has been described in the medical literature.
But if you’ve put your hair in your mouth from time to time (or have kids who do), there’s no need to panic. Even in cases where someone actually has trichophagia — and compulsively eats their own hair — it’s estimated that only around 1 percent even develop a trichobezoar, let alone experience the blockage of the intestinal tract characteristic of Rapunzel syndrome.
Alongside the infections, people with Rapunzel syndrome can experience abdominal pain, nausea, vomiting and/or unexplained weight loss. Because it can take years for a hair mass to accumulate, a person can live for extended periods without experiencing any symptoms. While some smaller hair balls can be removed via endoscopy, stomach pumping or a prescribed course of enzymes to aid the digestive process, larger masses must be surgically removed before a blockage occurs.
The good news is that once a trichobezoar is removed, they typically do not form again. Although there is no cure or conclusive data on treatment for trichophagia, common methods for managing the condition include cognitive behavior therapy, possibly in conjunction with medications used to treat other obsessive-compulsive disorders or mental illness.
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