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The name of the disease comes from the name of the heroine of a German folk tale written by the Brothers Grimm. A girl named Rapunzel had long and strong hair. It was them that led to the tower, where she was imprisoned by the evil witch, that both the witch and the prince – the girl’s savior, could climb.
In medicine, this term describes a rare type of intestinal obstruction caused by trichophagia, the habit of eating your hair. Trichophagia is sometimes associated with a hair-pulling mental disorder called trichotillomania. To understand the essence of the disease, let’s explain both concepts.
Trichotilomania (TTM) is a mental illness that manifests itself in the compulsive tearing of hair from the head, nose, chest, eyebrows or eyelashes. Usually, the patient starts pulling his hair out under the pressure of stress. The rising tension intensifies the action. Sometimes, however, a sick person tears their hair out also when relaxing, e.g. while watching a movie. Patients say that this behavior brings relief, but soon self-regret and dislike develops. There is no precise data on the number of people suffering from this condition. Estimates show that about 0,6-3 percent suffer from it. population. It is known that this problem affects more girls than boys. It usually appears between the ages of 6 and 13.
Trichophagia is the habit of eating your hair. People with long hair often bite the ends and then swallow the bitten off pieces.
Rapunzel syndrome – causes of the disorder
There is no single and proven theory about the causes of this ailment. Scientists dealing with this problem claim that there may be many causes of the disorder. They mention depression, sexual violence, traumatic separation from the mother, anxiety states, Tourette’s syndrome. Others talk about the causes in the body of a sick person, i.e. mutations in the SLITRK1 gene, which plays a role in creating connections between neurons. When these connections malfunction, trichotillomania can develop.
A person with this disorder is not difficult to recognize because they usually have irregular bald patches on their head, often no eyebrows or eyelashes, or they are very thinning.
Mental aftermath of Rapunzel’s syndrome
People with Rapunzel syndrome often isolate themselves from the world. They avoid contact with other people because they feel they are not understood and rejected. However, it is often the case that, fearing rejection, they do not seek specialist help. It also happens that they are unable or unwilling to admit the disorder they suffer from.
Rapunzel’s syndrome – physical aftermath
The habit of eating your hair can lead to serious problems with your digestive tract. Patients often use laxatives to get rid of hair from the digestive tract. This is usually an effective therapy, but this is not always the case. Sometimes, surgical intervention is necessary to remove tangled hair, called bezoars, from the stomach or other parts of the digestive tract.
What are bezoars?
Bezoars are undigested fragments of plant fibers, medicines, and sometimes also hair that is swallowed accidentally or intentionally by people with mental disorders. Trichobezoars are formed in people who swallow their hair. They are found not only in people who are forced to swallow their hair, but also in men with a beard or mustache and in women who are their partners, i.e. people who accidentally swallow their hair.
Moving away from trichophagy for a moment, it should be said that the cause of the formation of hard, tightly sticky formations in the stomach are usually disturbances in the movement of undigested debris to the distal part of the gastrointestinal tract. This usually happens when the pylorus is narrowed. The stomach walls then do not constrict enough to push the food through. Another cause of bezoar formation is gastric surgery, which has impaired normal stomach function. Another reason may be dental problems, such as missing teeth, poorly fitting dentures, or diseases of the jaw, which make it impossible to refine the food well.
It also happens to people who have low levels of acid in the stomach, making it difficult to digest properly. The tendency to form bezoars also occurs in some people with diabetes, hypothyroidism or suffering from connective tissue diseases.
If the stomach is less fit, the body produces more mucus. This, in turn, does not speed up digestion, but only clumps undigested debris into bumps. They can be of different sizes. They can be the size of a pea, walnut, and sometimes even the size of an orange.
Small bezoars often do not cause any discomfort. When they get larger, the person may lose their appetite, feel full even after a small meal, feel nauseous or experience pain in the upper abdomen (epigastric region). Any such ailment should be discussed with the primary care physician, as similar symptoms may occur in gastrointestinal malignancies.
If undigested debris clumps into a ball, it is difficult to detect without specialized testing. Only very large bezoars can be felt by the doctor by examining the patient’s abdomen with his fingers. Usually, however, these changes are detected accidentally, e.g. during an X-ray of the stomach or an ultrasound of the abdominal cavity. However, the best method of diagnosing such lesions is endoscopic examination, which allows to distinguish bezoar from another lesion, e.g. cancerous. In addition, if the undigested debris is quite soft, it can be divided into smaller pieces and removed from the stomach using an endoscope.
Treatment of Rapunzel syndrome
There is no fully effective treatment for the swallowing habit. Trichotillomania is treated with psychotherapy and by treating the root cause of the abnormal behavior. Antidepressants and anti-anxiety medications are often given.
People who are exposed to the formation of bezoars for other reasons should avoid swallowing large, carelessly chewed pieces of meat, as well as vegetables and fruit that contain a lot of hard-to-digest fibers, e.g. raw pineapple, kohlrabi. You can eat anything, but you should pay attention to the way you prepare your meals. For example – if you want to eat a pineapple slice for dessert, it is better to blend it. The same should be done with kohlrabi. Other vegetables and fruits are better diced than swallowed in large pieces. It is also important to drink at least 2 liters of fluid every day so that the food in the stomach is thinned. Thanks to this, it will be digested faster and it will be easier to move to further parts of the digestive tract.