Running For Research - PWSFact #3: PWS is recognized as the most common genetic cause of life-threatening childhood obesity. Research into PWS has implications far beyond those for our loved ones. Unlocking the key to PWS will also benefit the many people who suffer from related diseases such as childhood obesity and autism. Obesity is one of the most common diseases (yes, it’s now considered a disease, not a condition) in the US. According to the latest CDC figures, over 42% of the US adult population and over 19% of children are obese. Obesity drives the frequency of occurrence of the top 3 chronic diseases in our country. They are heart disease, cancer and diabetes. If you are someone who has PWS, the cards are truly stacked against you. PWS has many metabolic effects, all of which make it easier to gain weight and much harder to lose weight. For example, people with PWS generally experience lifelong hypotonia, which is low muscle mass and strength. Having hypotonia causes a person to fatigue easily and can make physical exercise difficult. It’s also the case that the less muscle mass you have, the fewer calories your body burns. People with PWS have a very skewed fat to muscle ratio. PWS also causes hyperphagia, which is an insatiable appetite. I will talk about this symptom in length in another post, but for now, just know that at some point in every affected individual’s life, they will begin to experience genuine and intense hunger signals that are unable to be satisfied. This can can lead to overeating and weight gain. The metabolism of someone with PWS is very poor. The daily recommended caloric intake of most PWS people is about 50 to 70% of the typical person’s caloric intake. PWS persons are also very sensitive to insulin. If diet isn’t carefully controlled and strictly nutritious, it can cause insulin levels to rise and appetite to ramp up even more. Think about what you have just read. People with PWS have a high fatigue level, a poor metabolism and an insatiable appetite. This is the description of a perfect storm. It’s a storm that most are unable to avoid. So far, we have managed to keep Clementine slim. It’s incredibly difficult and requires intense food management and a daily exercise regimen. Clem also has T1D, which adds another layer of complexity to her situation. I’ll talk more about that later too. Suffice it to say that every piece of food she puts in her mouth and every drop of liquid that finds her throat has been planned out in advance and accounted for. It’s all encompassing at times. This is why we fundraise for research. Life shouldn’t be so hard for my beautiful Clementine.
Please visit our fundraising page to find out how you can make a difference for Clem and for the whole PWS community.
Some interesting history. The paintings below are thought to depict people with PWS. The first is a painting by Juan Carreno de Miranda ( 1680) , which hangs in the Prado in Madrid. The second painting is titled” Bacchus” and is by Peter-Paul Rubens ( 1638-1640). It can be found at the State Hermitage Museum in St. Petersburg Russia.
