We thought it’d be helpful to do some “day in the life” posts to understand the implications of Kennedy’s genetic disorder. If any of you are like us (Nick and I), then you knew nothing of Urea Cycle Disorders (which is what Kennedy has, hers specifically being OTC Deficiency) before hearing about our story. So, today we are going to share more about Kennedy’s diet and special metabolic formula. Please read below if you’d like to learn more about what this looks like for her!

 

One main way we (try to) control Kennedy’s genetic disorder is through her diet. She specifically is on a low protein diet. However, Kennedy’s metabolic genetic disorder is very sensitive. She can’t just do any amount of “low” protein and stay stable – it has to be a specific amount determined by her team based on her blood work, medication, and what her body is showing us she can handle. We also cannot eliminate protein entirely – her body is growing lots at this age and needs some protein to continue properly growing. 

 

There are two ways Kennedy gets her necessary protein. First, she has a special metabolic formula – we call this her “milk” – that she drinks each day. Her milk is made from three ingredients, shown in the picture below. Cyclinex provides protein in the form of amino acids, so her body doesn’t have to break the protein down (which means it doesn’t have to go through her urea cycle…which is good because hers doesn’t work correctly). PFD essentially provides nutrients and calories to her diet. It’s not uncommon for kids with this disorder to have little appetite – her team isn’t exactly sure why but suspects that their bodies almost know that they can only handle so much. So, the extra calories are important. We have to make this formula everyday, as it is only good for 24 hours. It also has to be refrigerated once it is made for the day. Her current goal for her metabolic formula is 15 ounces per day. 

 

Second, Kennedy needs to eat 9 grams of protein total per day (her team calls this her “intact” protein). Yes, you read that right….9 grams. To put that in perspective, consider the protein amounts in some everyday foods listed below:

 

Colby Jack String Cheese: 5 grams/stick

Sausage Link: 2 grams/link

Egg: 5 grams/egg

Hummus: 1 gram/tablespoon

1% Milk: 8 grams/cup

Chicken: 31 grams/3.5 ounces

 

So, it can be quite challenging to provide options to Kennedy throughout the day. Things get even trickier when we are out and about during the day. We choose to pack food options for Kennedy wherever we go – eating out or picking something up for her on the go is hard to do. We could, for example, try to order her something at a fast food restaurant. To do so safely we need to be able to find or be given accurate nutritional information. We also need our scale with us to measure out how much of that food that Kennedy can have. So…packing our own pre-measured food options for Kennedy is much easier and safer for her. Fruit and vegetables are “free” options and we do not have to track the protein in them, thankfully. 

 

With that all being said, while Kennedy’s diet can be challenging, we continue to be thankful that she is able to be home (most of the time) and that she is overall a happy, wild toddler. 

 

As always, thank you for being here! Please feel free to subscribe to this page to receive more updates about Kennedy as they are posted!