Our Journey Raising Two Children with Special Needs

This blog chronicles our life raising two children, Nicholas 14, diagnosed with Prader-Willi Syndrome and Weston 17, diagnosed with Autism/Asperger's/ADHD. It's the ups, the downs, the joys, the sorrows and most importantly, the beauty of living a life less perfect, a life more meaningful.




Monday, March 14, 2011

And So It Begins

My son Nicholas is 9-years-old.

He is diagnosed with Prader Willi Syndrome.

As most of you know, individuals diagnosed with PWS are born with an insatiable appetite. They never feel full. They will do whatever it takes to get food. They will lie. They will sneak. They will steal to get it.

Due to a dysfunctioning hypothalamus in the brain, they are designed to eat 24-hours-a-day. Their bodies tell them they're hungry all of the time. There is nothing they can do to stop it. No pills, no medications, no therapy is currently available to help stop this strong drive for food. Left unsupervised, they will eat until their stomachs rupture. They will eat until they die.

As parents of children diagnosed with Prader Willi Syndrome, it is our job to protect our children from themselves. It is our job to protect them from eating until they die. It sounds harsh, I know, but this is the cold reality of living day-to-day with this syndrome. These are the facts and they are horrific.

Usually, this uncontrollable drive to eat, will begin when children are about 2-years-old. Parents must secure all food access by locking refrigerators, food cabinets, even the garbage. Some children, acquire the food-seeking issues sooner and some, like Nicholas, are very, very fortunate, and begin the process later.

Nicholas is one of the lucky ones. Our family is very fortunate.

I have thanked every power-source in the universe every single day, for nine years. I have thanked God and the Universe that our need to secure all food access has been delayed for so long. I have thanked the Lord for the luxury of living a life with no locks.

Nicholas's drive to eat has been a slow increase in interest, building little by little over the years. Since he was diagnosed as acquiring the syndrome via UPD (uniparental disomy), we noticed more behavioral problems and less hunger issues his first few years of life. I will discuss what UPD is, in another post since it will require a long discussion of genetics.

As a family, our philosophy has always been...to expect the best but prepare for the worst.

We have now noticed a slow increase in Nicholas's weight, despite an appropriate dosage of his growth hormone. We increased his thyroid medication but still noticed an increase in weight.

Lately, Nicholas has been getting up early with Dad in the morning. He likes to eat breakfast with his Daddy before he goes to work. Typically, Nicholas stays in the living room playing until Weston and I awake. Last week, however, I was awoken by a frightening sound. It was our food pantry door and  it was closing. As  I came down to investigate, Nicholas had a small plastic bag of Doritos in his hand, he was eating it on the couch as he watched Tuff Puppy. It was a sandwich bag Weston had left in the pantry from a school snack he hadn't eaten.

My heart sank as I realized...............it has begun.

Now, this is behavior Weston exhibits every single day. Taking snacks without asking is what boys do. But because it is Nicholas, and because he suffers from Prader Willi Syndrome, this behavior is SO much more.

This behavior is dangerous.

This behavior has changed all of our lives forever.

This weekend my husband installed a lock on our pantry door. Nicholas's interest in food does not (at this point) include the refrigerator, the garbage, or the bread and apples I keep on the counters. But once again, as a family, we are ready. My husband has purchased locks for them all.

Surprisingly, Nicholas thinks the pantry lock is the coolest thing ever invented. He loves keys and locks and pretending he's going to work. To Nicholas, this is just another way to play "make-believe" and he is delighted. He presses the numbers on the lock pad and enjoys watching the light change color.

But I knew it was just a question of time, before he would ask the dreaded question.

"Mom how do I open the door?"

I knew he was going to ask it, but I refused to prepare myself for it. I was in denial, my mind shut down. It was unwilling or unable to process anything. The sight of the lock had created fear in my mind and it was this fear that now paralyzed my brain.

"We have a new rule now in the house." I replied. "Only Mum can open the pantry door. I need to keep out the mice."

The mice, I thought to myself, how stupid is that! How can I lie to my son? I need to tell him the truth.

"Am I a little mouse?" Nicholas asked and smiled at me.

Once again I am amazed at my son's ability to diffuse my fear with his unconditional love. It was then that I realized it was this unconditional love would help us to overcome. This love would make the locked cabinets and the chained refrigerators a little easier to accept. This love would help us find our way.

"Yes, sweetheart, you are my special little mouse." I answered.