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

Prader- Willi Syndrome Association awareness month in May!!

Luis Emilio and me! Look at that smile. Doesn't he make you happy just looking at him? :)

So far I’ve been blogging about finding happiness and treatments for depression.  My assignment this week is to blog about a study design.  The topic I chose will be a little different then what you are use to.  Never the less, it’s a topic very important to me: Prader -Willi Syndrome (PWS).  Infants and young children with PWS are typically happy and loving human beings, so I can sort of link the topics together.  Children and adults with PWS can definitely teach us to be grateful about simple things in life that many of us take for granted.  In my experience they have helped me recognize that it is the small, everyday miracles that make life worthwhile.

My nephew, Luis Emilio, was born with PWS.  He has brought a lot of happiness to our family.  Blogging about how he has brought our family together and filled our home with happiness is another way to spread the happiness to my readers and give hope to other families that struggle with family members with PWS themselves.

“Prader-Willis Syndrome on the Move is the new PWSA (USA) national awareness initiative, targeted to take place annually beginning this May, during PWS awareness month."  In honor of this event and in effort to increase awareness about PWS, I will dedicate my study design assignment to PWS.

The Study will try to answer the following question: 

Will blogging about PWS, increase awareness about the signs and symptoms of patients with PWS?

Can blogging make me happy?

I just read an article published on the ABC news website that says yes! But the studies they site as their source of information don't say that at all!!  

The only published study they site was a cross-sectional study which gives us a snap shot of New Myspace.com users intention to blog or not to blog and several psychological variables. "Intending bloggers scored higher on psychological distress, self-blame, and venting and scored lower on social integration and satisfaction with number of online and face-to-face friends."¹ The participants hadn't actually blogged though!! They just had the intention to blog.  So the exposure I'm interested in: "Blogging" and its effect on happiness weren’t really studied.

In a cross sectional study we can't determine temporal sequence.  What came first the intention to blog or the distress?  When I started blogging I would have reported increase distress and venting because I didn't want to blog!! I would have still reported I had the intention to blog.  Blogging was causing my distress not helping it though.... 

The second study mentioned had not been published.  According to the article the authors sent out questionnaires to the same group of MySpace users.  In the first study there where 134 participants.   Of those only 59 responded to the second questionnaires.  That means that we don't know what happened to 56% of the participants.  That is high number that could have had a negative effect from blogging or internet social networking. 

Based on these articles, I can’t start recommending my patients start to blog to help with their depression.  Intention to blog may be a good screening tool though.  Maybe we need to do a study about that.  

 

There are studies that journaling helps depression.  Blogging is not the same though.  Journaling is private.  When you blog your letting the whole world know what your feeling and they can comment on it. This feedback may be good or bad for you though. 

If you are interested you can download the UW-Madison patient hand out I use on Using Journaling to aid health by clicking on the link.  The website also has more information on non-drug approaches to help you feel happier.

What do you think?  Has blogging helped you? 

Bibliography: 

1. Baker JR, Moore SM. Distress, coping, and blogging: comparing new myspace users by their intention to blog. Cyberpsychol Behav. 2008 Feb;11(1):81-5. PubMed PMID: 18275317.