There is a genetic defect that is estimated to afflict one in 5,000 obese people.

It’s believed that currently, about 12,000 U.S. people have been diagnosed with this problem: Prader-Willi syndrome.

It’s also assumed that a number of people are walking around with this affliction and have not been diagnosed at all.

The hypothalamus in the brain regulates hunger. It tells you when your stomach is beginning to get full with food.

You begin feeling this sensation of fullness—or at least, satiation—as you continue eating. At some point, eating becomes undesirable and you leave the table.

Compulsive Overeating Disorder
In compulsive overeating, or a person who is receiving the wrong environmental cues to eat (such as being constantly offered food; boredom; socializing; seeing other people eat), the person may feel satiated or even full, but continue eating anyways.

 

It may even feel quite uncomfortable, but they continue eating because of some emotional or social need that is over-riding the physical need to eat.

Starvation
Imagine, however, the hunger that a starving child in a developing country feels day in and day out, or how hungry you’d feel if you were stranded in the wilderness and hadn’t eaten for days.

This kind of hunger makes such individuals rummage through garbage cans for food. They’ll eat anything, including rotting fish washed ashore from the banks of a filthy river.

People stranded in the wilderness have been known to eat hand lotion, insects, tree twigs and rodents.

This is the kind of hunger that a victim of Prader-Willi syndrome feels, because the mechanism in the hypothalamus that controls hunger doesn’t work.

So no matter how much food they consume, they always feel starved and famished, as though they haven’t eaten for days.

Maybe you’ve read that this disorder prevents patients from “feeling full.” This is a very erroneous descriptor.

The absence of “fullness” is NOT the same as the presence of feeling that you haven’t eaten a crumb for days.

I’m not full as I write this. I’m mildly hungry. But it’s hardly distracting, and certainly not something that would make me continuously eat if it were a permanent sensation.

Someone with Prader-Willi syndrome will eat continuously unless they are supervised and barred from food.

The sensation of not having eaten in days is nonstop no matter how much they eat, which is why they are capable of eating themselves to death. They live in a state of never-ending starvation mode.

PWS results from a deletion of part of chromosome 15, when this chromosome is inherited from the father.

When a deletion occurs on maternally-derived chromosome 15, interestingly, a totally different condition results called Angelman syndrome, in which hunger is not affected.

There is no cure for PWS. If there were, there’d be a way for chronic dieters to stick to a diet without any problem.

How About Just Ignoring the Hunger?
You may be wondering why someone with Prader-Willi syndrome just doesn’t learn to put up with the hunger.

After all, “normal” people can put up with hunger without any problem (long plane rides, being tied up in business meetings all day, other jobs that are demanding, and of course, hunger from dieting).

But apparently, the hunger in PWS is unlike the hunger that normal people experience.

If you ask a person with PWS what their hunger feels like, their description won’t help us understand too much, because they have never experienced satiation—except when they were too young to remember, as the raging hunger usually kicks in between ages two and five years.

• People with PWS have been known to break into cars and steal food on the car seats.

• They have been known to eat out of garbage cans, eat dog food, cardboard, paper – anything to fill up the stomach.

• Some will even consume whole bottles of vegetable oil, sacks of flour, raw meat and other foods in unprepared form.

You would eat this stuff, too — if you were in a plane wreck in the mountains and without food for five days and saw no rescue team in sight.

But why can’t a person with PWS control themselves, knowing that they are NOT starving, knowing that they are dangerously obese because of it? Can’t they just ignore the hunger?

Well, this is difficult if the person has mental retardation, and about 70 percent of people with PWS have mental retardation.

A very small number of people with PWS, who function on the high end of intellectual normalcy, live portions of their lives unsupervised.

But there are those who have normal intelligence but need continuous supervision lest they be at risk of eating themselves to the point of stomach rupture.

Other characteristic traits include short stature, distinct facial characteristics (though they can be subtle) poor muscle tone, underdeveloped sex organs, a stubborn and sometimes belligerent nature and aggression.

Food in their homes must be under lock and key.
Even those with normal intelligence must have food locked away. There are group homes for adults with PWS in which food is locked up, and doors and windows are secured so that they cannot escape in the middle of the night to seek food.

Or, doors and windows are equipped with an alarm so that caretakers know when someone is sneaking out. With 24-hour supervision, a person with PWS can maintain a healthy body weight.

Some people with PWS are completely out of control with the food seeking, while others truly try to work with their caretakers and work hard to resist the temptation to eat more than they should.

Lorra Garrick has been covering medical, fitness and cybersecurity topics for many years, having written thousands of articles for print magazines and websites, including as a ghostwriter. She’s also a former ACE-certified personal trainer.  
Source:
mayoclinic.org/diseases-conditions/prader-willi-syndrome/basics/definition/con-20028982 eat to death, obesity