Wednesday, May 8, 2013

PWS Awareness Day #8: The Hunger

"It," "the H word," "the monster" ... all ways that you will hear people with PWS and their families describe the unrelenting hunger that is the hallmark of PWS. Even though babies with PWS have difficulties feeding and lack of appetite, there is then a period of somewhat normal appetite and eating.

Then sometime in the early years, often between ages 2 and 8 (although sometimes much later), the hyperphagia begins. As with all things related to PWS, the severity and nature of this varies, but hyperphagia is characterized by an obsessive interest in and anxiety about food. And you know that awful feeling of being really hungry? People with PWS feel that all the time, even after consuming a large meal. ALL. THE. TIME.

Left unchecked, people with PWS experiencing hyperphagia can and will eat themselves to the point of their stomach rupturing, and therefore, death. Families lock up pantries, refrigerators, and sometimes even the trash to protect people with PWS from hurting themselves. Even items you might think are safe, like a bottle of olive oil or a bag of flour, are fair game. As you might imagine, this presents enormous difficulties when out and about or at school, never mind at home.

Food schedules and routines become vitally important to let people with PWS know when to expect food and alleviate some degree of anxiety about when they will next get to eat. Deviations from this routine can often result in meltdowns because of how that unpredictability increases anxiety.

If that weren't enough, people with PWS have resting metabolisms that are far slower than those of their peers. So a low-calorie, low-carb diet is often what is called for to maintain a healthy weight. Sometimes "low-calorie" means as few as 600 calories a day.

Researchers don't know why this happens. We need a cure....

We are fortunate that thus far, Dean does not have hyperphagia. Every day that we don't have to deal with this issue, I am thankful. Dean occasionally has meltdowns related to food and expectations of what is to come to eat. He has had a few meltdowns when a snack or meal is over, but redirection often works to help him calm down. Sometimes we even have to convince him to stay at the table to eat. I know this is not typical of what the rest of his life will be, but we'll take it as long as we can. We do still stick to a pretty tight schedule and Dean has certain foods that we do or don't allow in his diet. So far, this is manageable. Hoping that a cure is found before it becomes less so...

1 comment:

Anonymous said...

I and my husband is thinking about doing foster care for a lady with PWS . She is also mildly IDD. What advice do u give me. Email Deb