Early to bed, Early to rise,
Makes a man,
healthy, wealthy and wise.
Many children diagnosed with Prader Willi Syndrome seem to instinctively follow Ben Franklin's sound advice, or at least this is true for my son, Nicholas.
No matter what I do to try to engage my young son, by 7 pm he is tired and ready for bed. Of course, with this early to bed time comes the opposing early to rise time.
While the rest of the house is sound asleep in their beds, Nicholas is wide awake and enjoying his morning quiet time. He watches TV, plays with his toys and explores our dark, quiet home. Often, I have awoken to find all of the contents of our front closet scattered across the hallway floor or my favorite antique lamp sitting disassembled on the dining room table.
More seriously however, while Nicholas does get 10 hours of ample sleep time, the early rising creates a problem for a child who is designed to eat.
Parents of children diagnosed with PWS must be vigilant in their efforts to ensure that all food is secure before bedtime thus preventing the life threatening food seeking that can occur during this early wakening period.
It is not unusual for children diagnosed with PWS to raid basement freezers, eating things like frozen meats, pies, pastries etc. Household baking items like powdered sugars and syrups stored in pantries are other potential food sources for our always hungry children.
In severe cases, some children diagnosed with PWS will elope in an effort to find more available sources of food. Many PWS parents resort to installing home alarm systems that will warn them if their child tries to escape the house in search of food.
There is hope, however.
Researchers have uncovered a gene called Magel2 which is highly expressed in the hypothalamus of the brain. Recent studies of this gene and its expression, seem to suggest that it plays a role in circadian rhythms (early rising), growth, metabolism, endocrine function and fertility.
It is the hope of all parents of children diagnosed with PWS that this research will help us to better understand and more importantly "treat" these unusual and life threatening features common to so many of our children.
If and when Nick's food seeking escalates, we are ready to install an alarm or redesign our kitchen to accommodate the unusual needs of our hungry child.
As Ben Franklin also said....
By failing to prepare, you are preparing to fail.
Our family has adopted its own PWS motto...
We hope for the best but like our favorite founding father, we are aways prepared to find solutions for the worst!