Fact five: Hunger=Overeating=Obesity= Chronic Disease in PWS

The increased appetite in children with PWS leads them to overeat and become obese. Like in all of us, obesity increases our risk of chronic diseases like hypertension and diabetes.

To make the problem worse,   they have reduced muscle tone as mentioned in earlier posts. This leads to low muscle mass, which means they need less calories then most of us do.

It's important to control their diet. Even if this means locking up food in the house.  Like all of us, they should be very active to maintain a healthy weight!

Luis Emilio started suffering from central obesity from a very young age.

Fact four: Hunger

He is hungry in that picture and he is still smiling! What's your excuse?

The free on line dictionary defines hunger as "the discomfort, weakness, or pain caused by a prolonged lack of food". Children with PWS feel this all day, everyday. The part of our brain that tells us we are full does not work.

The hunger is physiological and uncontrollable. So when Anonymous commented on one of my post: "you all ready have everything to be happy, you have that power to control and know when you are full and you know and you can stop eating, tell me is not that enough to be happy." Click on the link to read the complete comment.  I think he or she is right.

I'm going to bed tonight with out the feeling of hunger.  I am one of the lucky people that had the means to buy food today. The part of my brain that controls hunger was able to register that I ate and made that "discomfort" I had when I came home from work go away. Millions of children and adults are not that lucky. They will spend all day feeling hungry, either because they live in poverty or have PWS.  Click on the link for PWSA-USA below and learn how you can get involved and help us in finding a cure.

Fact Three: Hypotonia, why does it matter?

Hypotonia in children with Prader-Willi Syndrome (PWS) will begin to improve between the ages of 8 and 11 months. Their motor-skills will be one-two years behind compared to other children.

If hypotonia improves over time, why is it such a big problem? During infancy they have significant problems feedings.  It takes muscle strength to be able to breast feed or suck on a bottle.  Many children will need to have a feeding tube placed to get enough calories. Like I mentioned yesterday, they have a weak cry if they are able to cry at all. Crying is how babies communicate they are hungry, in pain, uncomfortable, and/ or need their diaper changed for example. Their lack of muscle tone will also cause them to have delayed speech.

It's important for these children to start physical therapy as early as possible.  Luis Emilio was the youngest child to receive physical therapy in Nicaragua at the time.  Although it took several years for us to find out his diagnosis.  He started therapy as soon as the motor skill deficit was noted. 

 

 

Luis Emilio in physical therapy/swimming lessons

Luis Emilio with his Mother, Perla, at physical therapy.

Fact two: Hypotonia in PWS

Luis Emilio with significant hypotonia.

 
Fact Two:   When babies with Prader-Willi Syndrome (PWS) are born,  88% of them have neonatal hypotonia.  Hypotonia means they have decreased muscle tone. In one word they are floppy babies.  Luis Emilio wasn't able to cry for most of his first year of life.  It takes a lot of muscles to cry and he just didn't have the muscle strength.

Luis Emilio (My Nephew) at 7 months of age.

First Fact about PWS

I have already not followed the study protocol! The plan was to start posting facts 3 days ago.  If this was a real study, we would need to report that Exposure to facts about PWS was not started as established by the study protocol.  These things happen in real research though.  Life happens.  We just have to do our best to follow our protocol and report any variations that occur in the process. That way the reader is aware of the possible circumstances that could effect our results.

I wish I had a great story to tell you of why I didn't blog.....Something exciting or at least relevant to my blog.  But I don't. It's just been a busy week and remember my first couple post? My main goal in life is happiness.  I kinda feel like the guy in this article published in the onion: Grown Adult actually expects to be happy.  So the last three days when I came home exhausted: sleep won!! I'm sorry.  Better late then never.

Fact number one:

Prader Willi Syndrome is a genetic disorder. We won't get too technical about it. Bottom line: part of chromosome 15 from the father of patients with PWS isn't expressed.

Portrait of Doña Eugenia Martínez Vallejo at age 6.   Painted by a Spanish baroque court painter: Juan Carreno de Miranda.  This painting done in 1680 displays classic characteristics of PWS