Thursday, May 2, 2013

Death through over-eating

This topic is a very serious one on many levels.  First, we all know that people with PWS have what is medically termed, hyperphagia.  (Greek words meaning "very much" and "eat".)  What it means in the PWS world is an inability to stop eating.  There are other disorders than can also cause this excessive appetite and lack of control:  diabetes, Kleine-Levin Syndrome (a malfunction in the hypothalamus) and Bardet Biedl Syndrome.  And there are many other issues that can cause hyperphagia as well - namely

  • Anxiety
  • Certain drugs
  • Hyperthyroidism
  • Hypoglycemia
  • Premenstrual syndrome
  • Bulimia
There are some links here, and I'm sure you can spot them: anxiety and diabetes.  Both these are often cited as causes for over-eating in the normal population (yes, I know, I always debate whether to use the word "normal" as well... but for the sake of this post, I'm choosing "normal", with reservations!) as well as in the PWS population.  

When we think about death through over-eating, we tend to think of people who have grown so huge  that their obesity eventually killed them (usually through vital organ failure). Interestingly, in Wikipedia there is a short list of six famous people who apparently died from over-eating:  
  1. Alexander the Great (loved his feasts, couldn't get enough)
  2. Zachary Taylor (12th President of the USA, overloaded on cold milk and cherries during a 4th July celebration; died from gastroenteritis)
  3. Mozart (apparently from eating too many undercooked pork chops)
  4. Adolf Frederick (King of Sweden, ate numerous servings of lobster, caviar, sauerkraut, kippers and champagne, topped off with 14 servings of his favourite dessert.  He died later that night.)
  5. Henry 1st  (not known to be an over-eater, but ate a "surfeit of lampreys" and died the same night)
  6. William Makepeace Thackery (a notorious glutton who regularly overate.  Addicted to spicy peppers.  After enjoying one last enormous meal, was found dead in his bed next morning, felled at the early age of 52) 
I imagine many of you have seen those "fattest person in the world" documentaries where they are usually so large they have to have some sort of wagon system to get them from A to B.  Personally, I hate to see these as they seem to suggest it's ok to be that large, it's ok to laugh at large people, and it's apparently ok to make media footage at their expense.

But I want to get back to my original point which is that people with PWS have a genetic and hypothalamic imbalance that causes the brain to cease signalling the stomach that it's full.  Many people with PWS do die at extreme weights and it is this obesity that has been a major cause of death.  However, many people with PWS are not obese, and yet can still die very suddenly from over-eating.  Just like our list of six above - although, quite obviously, none of these people had a genetic deletion on the 15th chromosome, and all would have had the capacity and cognitive ability, should they have wished, to eat sensibly.

It can be very difficult to understand that a normal-weight person with PWS can die suddenly from overeating.  They look normal.  They are not overweight.  They have not been seen to be overeating.  Yet, suddenly and inexplicably, they suffer serious stomach pain, vomiting, and are rushed to hospital.  What has happened to cause this sudden death?  Most often it is a stomach rupture and the seriousness of this cannot be over-stressed.  

The main symptoms of stomach rupture, or necrosis, are these:
  • Vomiting
  • Loss of appetite
  • Lethargy
  • Complaints of pain
  • Stomach bloating
  • Possible fever
  • Guaiac positive stools (blood in stools, not necessarily seen)
If you, as parents or caregivers, are aware of any of these symptoms, it is imperative you get medical help.  Just recently, I attended the funeral of a young man who complained of a 'tight tummy' and who had been vomiting.  He was rushed to hospital, but unhappily it was too late.

There are other causes of abdominal pain - of course there are - and the reasons for this could be far less drastic than gastric necrosis.  As our PWS population grows older, there are more health issues to consider. 

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