Why Food Is Like Air to My Son With Prader-Willi Syndrome

The air he breathes.

It’s his very own.

It’s not like yours and mine.

He thinks about it. All the time.

Our air gives us life and breath and oxygen and helps us grow.

His air could lead to death.

In Prader-Willi syndrome, at some point in childhood, hyperphagia sets in. Hyperphagia is experienced as a chronic hunger, or never feeling full. It’s an obsession with food, which leads to excessive overeating, and a host of medical issues, if not carefully supervised. Children and adults with PWS die every year from choking to death, and from ruptured stomachs due to eating binges.

It could look like this:

• A 5-year-old who sneaks into the pantry in the quiet of the night and opens all the cans. And eats. And eats.
• The kid at elementary school who takes a look into others’ lunchboxes and takes their food.
• The teenager who steals money from her parents to use the vending machine at school when no one is watching her for 20 seconds.

It could be my 11-year-old who was eyeing Luke and Kate’s food as they packed their school lunch bag, or prepared some breakfast. I’ve had to become the Kitchen Bodyguard, a role I never fathomed would be mine.

I want to describe a week in our life with Ryan, where his food drive went from zero to 60 overnight. We had this 7-day “episode” of intense hyperphagia, and then his food preoccupation returned to as it was. We have always kept a food schedule, watched his diet, and made adaptations along the way, as his food interest has increased. He has become more and more preoccupied with food, but it’s been manageable. It’s been a slow burn.

Yet, what I will describe is not uncommon in the daily lives of most persons with PWS.

One Sunday morning, all Ryan wanted was food. He was already a bit out of sorts (due to illness and the house remodel), so it was very difficult to use logic and the clock to explain when and what he would eat. I did my best to hold off and risked his aggression for not adhering to his demands and requests. I accelerated the schedule, but did not give in, no matter how much he told me he was hungry.

Nothing could distract him. Not the TV, the iPad, reading, his workbooks, music, the backyard swing, nothing. In his state of mind and with his fragility, I couldn’t leave home with him. If he wasn’t sleeping or crying, Ryan was asking for food.

Monday morning, he woke up in the same mindset. With coaxing and cajoling to do his before-breakfast-routine first, he did get to breakfast time. I gave him the usual choices and portions. Then he wanted to eat his snack and lunch. Right then. I had to talk him “off the ledge.” He got mad when Luke or Kate were eating their breakfast and he wanted more. He starting to become aggressive, so I had my husband watch him while I scooted out with Luke to get him to school.

When I returned, he kept asking me to give him his lunch. I had already taken it out of his backpack, so he couldn’t get at it. I relented and gave it to him because his agitation was rising and I really did not know what to do. This was new territory.

He ate his entire lunch box contents in less than five minutes.

It’s a wonder he didn’t choke he inhaled it so fast. We are used to Ryan inhaling his food, but this was an all-new pace. It felt frightening to watch Prader-Willi have such a hold on my son.

After he left for school with the help of school staff, I called my friend and mentor, Lisa Graziano, at the Prader-Willi California Foundation. I am sure she receives hundreds of these calls, yet she always makes you feel listened to, and doles out her expertise with such patience and a calm disposition.

Lisa reminded me, “Food is like air to them. You cannot take it away.” She let me know “the natural consequences that he ate his lunchbox before school, and therefore doesn’t get anything else till his afternoon snack, will not work.” (Consequences aren’t often effective in children with PWS anyway.) She told me I must bring to school the same lunch, nothing special, but exactly as packed before. And I was to lower the calories rest of day. Kids with PWS have low metabolism, so their diet must be regulated carefully. So, you are hungry all the time, and you get less than everyone else, that sounds like a winning combo double whammy. Vent over.

Lisa also reminded me to never give food outside of the schedule. Although it seemed like I was pacifying Ryan’s anxiety, it actually increased his anxiety. When you give extra food, you are showing them there is no schedule to rely upon, and to give them “food security” as we call it in PWS-land.

Kitchen Bodyguard was my role the rest of that week. I put spiral combination locks from Rite-Aid on the fridge and pantry. Ryan was not happy about this, but I kept telling him it because I cared for him and wanted to protect him. All of it was for him. If I was prepping food, I had another adult there to stand watch. I thought about all the PWS moms, dads and caregivers out there who have been dealing with this level of intensity for their child’s desire for food. Think about how many times a day you open the pantry and fridge, especially if you have other children to care for. Not to mention play-dates, parties, family get-togethers. I joked we would all lose weight because we just didn’t want to bother with the locks so many times a day.

I seriously wondered if this was going to stabilize, or if this was going to be the new normal with Ryan. By Monday night, Ryan’s cold and cough were completely resolved. And therefore, Ryan was returned to us. Just like that. It was over. I know it sounds difficult to believe, but it’s true. His food drive was just as it was before the crisis, manageable. I kept the locks positioned in the kitchen for the next few days, just to be sure. The intense obsession faded, and my job as Kitchen Bodyguard was shelved.

And what must it feel like for Ryan and all persons living with PWS and struggling with insatiable hunger? How does it feel to them to worry constantly whether they will get their daily meals and snacks without interruptions or changes?

We can easily imagine the anxiety and panic we’d feel if we were short of breath or suffocating.

So, if we can imagine that food equals air to those with PWS for just a moment, we can see the struggle they must face with every breath. 

Follow this journey on Jessica Patay