Bring Your Own Pancake Mix

We have started feeling bad that Laurel is never able to eat hot food when we go out to eat at a restaurant.  It's always fruit or cereal...boring.  Katie decided she would give the place we were headed to this morning a call to see if they would use our batter to make pancakes.  To my surprise (not Katie's) they said yes!

Ready to go

My grandparents were in town on their way back to Wisconsin so we met with them and my parents for breakfast at Larry's Diner in Plainfield.  We handed our little cups of milk and Cambrooke Mix Quick powder to the waitress and out came two golden brown silver dollar pancakes which Laurel quickly devoured!

Yum!

Gus, the owner, told my dad on the way out that we can bring anything we want in for Laurel and he would be happy to prepare it in their kitchen.  Needless to say we were really grateful that they would do this for us and hopefully every restaurant is just as accommodating in the future.  

Portobello and Zucchini Tacos

       


Five ingredients, easy, and delicious.  The recipe below is just for the filling.  You can improvise with your toppings, we went with colby jack cheese and black bean corn salsa.

Ingredients

• 5 portobellos - (480g, 365 phe)
• 2 teaspoons dried oregeno - (2g, 6 phe)
• 2 tablespoons olive oil (free)
• 2 zucchini, cut in 2 by 1/2 inch sticks (550g, 220 phe)
• 1 red onion (100g, 50 phe)

641 mg phe / 646g total weight = 0.99 mg/g

Instructions

• Preheat oven to 425 degrees
• Toss mushrooms with one teaspoon oregano, one tablespoon oil, and 1/4 cup water then place on a baking sheet
• Toss zucchini and onion with one teaspoon oregano and one tablespoon oil then place on separate baking sheet
• Place both sheets in oven and bake for 25 minutes tossing occasionally
• Serve with tortillas and toppings of your choice

You can use low protein tortilla shells for the PKU version, we went with 6 inch Mission flour tortillas (120 phe) since Laurel was a little low today.  Laurel only picked at the filling but devoured the tortilla or taco bun as she referred to it.

Creamy Avocado Pasta Sauce

This sauce could not be any easier, it is totally raw and heats itself a bit when you mix it in to the hot pasta.  We used Loprofin Animal Pasta for the PKU portion and a radiatore pasta for ours.

Ingredients

• 3 avocados - (364g, 339 phe)
• 2 garlic cloves - (11g, 18 phe)
• 1/4 cup cilantro - (8g, 8 phe)
• 2 tbsp lemon juice - (28g, 3 phe)
• 1 tbsp olive oil - (free)
• salt and pepper to taste

368 mg phe / 425 g total weight = 0.87mg / g

Instructions

• Mix all ingredients in food processor
• Blend until smooth
• Mix with pasta of your choice

Tips

• Reserve some pasta water to thin out the sauce if it seems to thick
• Roast the garlic if you want to take a bit of an edge off the finished product
• Garnish with lemon if you want to be fancy

Sweet Potato Chili

This was dinner tonight and it was delicious!

We made both a PKU and Non-PKU version of this one.  Since we were cooking for an 18 month old (and because I didn't want to weigh it all out) we went with the taco season rather than traditional chili spices.  Once the PKU version is separated out you can go ahead and add chili powder, cumin, etc if you so desire.

Ingredients

• 1/2 onion, diced - (48g, 16 phe)
• 1 bell pepper, diced - (197g, 49 phe)
• 1/2 bag frozen corn (227g, 282 phe)
• 2 sweet potatoes, diced (642g, 501 phe)
• 28 oz can diced tomatoes (794g, 397 phe)
• 1/2 packet McCormick taco seasoning (18g, 129 phe)
• 1 cup water (free)

1374 mg phe / 1982g total weight = 0.69 mg/g

Instructions

• Cook onions in oil until slightly browned
• Add pepper, corn, sweet potatoes, tomatoes, taco seasoning, and water
• Bring to boil, reduce heat to low and cover for 20 minutes
• Move PKU portion to to smaller pot and continue to cook on low for another 20 minutes

Additional Ingredients for Non-PKU Version

• 2 cans of chili beans
• additional seasoning if desired

Additional Instructions For Non-PKU Version

• Add chili beans and additional seasoning
• Cook on low for 20 minutes

We made the Cambrooke Corn Bread recipe to go along with the chili and it was awesome!  Laurel ate a ton of it (as you can see, quite literally).

Low Protein Coconut French Toast

I was very skeptical of this one when I saw a version of it on Pinterest.  It seemed to me that eggs were what made french toast well, french toast.  I stand corrected.  This turned out golden brown and delicious.  

We brought this along with us to Uncle Dan's birthday breakfast, Laurel loved it!  We made the dip the night before and threw it in a Tupperware.  It was made along with the traditional french toast on an electric griddle.

The best part is the dip itself is only 0.17mg/g so really the amount of phe depends on the bread you choose to use!

Ingredients

• 1 banana (78g, 38 phe)
• 1 1/2 cup canned coconut milk (340g, 27 phe)
• 1/2 tsp vanilla (free)
• 1/4 tsp ground cinnamon (1g, 1 phe)
• 1/4 tsp ground nutmeg (1g, 2 phe)

68 mg phe / 391g total weight = 0.17 mg/g (for the dip)

Now choose your bread!

67 phe

34 phe

101 phe

Instructions

• Puree banana, coconut milk, vanilla, cinnamon, and nutmeg in food processor or blender.
• Pour in to shallow dish for dipping.
• Heat griddle or skillet to medium heat and spray with non-stick cooking spray.
• Dip the slice of bread you choose in the liquid.
• Cook until golden brown on both sides, turning once.

Low Protein Irish Stew

Happy St. Patrick's Day!

Recipe from Cook For Love and it came out great!

This Irish Stew is Laurel's special St. Patrick's Day dinner!  The recipe came from the website cookforlove.org.  

You should definitely go there, sign up, and check out the amazing recipes if you have not already!  We also made the Irish soda bread...can't wait to try it!

Laurel...St. Patrick's Day 2012

Hope everyone has a wonderful St. Patty's Day!

Newborn Screening and PKU

Obviously all of those numbers are estimates but you get the point

Newborn screening was introduced as a public health program in the 1960’s and as you can see, made a huge difference in the lives of those born with PKU since then (info from NPKUA.org).  PKU was the first disorder to be tested for using modern newborn screening.  Robert Guthrie developed a simple method to detect high levels of phenylalanine in the blood shortly after a baby was born. 

Robert Guthrie looks exactly like Steve Higgens, 

the announcer from the Late Night with Jimmy Fallon

Guthrie realized at the time that a simple, inexpensive (some things never change) method would be needed if testing were to be done on a large scale.  Through the years, using mass spectrometry, we can now detect a wide variety of conditions from just a single drop of dried blood on a piece of filter paper based on the original methods used by Guthrie.

Newborn Screening is essential for PKU because any damage done is irreversible.  We are forever grateful for the advances made in health science and the work of Robert Guthrie.

Cute Video of Laurel (Finches and Doves)

Low Protein Sweet Corn and Coconut Soup

This recipe contains .61 mg/g of Phenylalanine.

I felt like the one thing missing from most PKU recipes on Pinterest is the amount of Phe in them so the recipes on here will have the Phe content listed.  When we are deciding what to make for Laurel, we are so much more likely to make something out of one of our low protein cookbooks because the Phe is listed.

Let’s face it, everyone is super busy and the last thing you want to be doing with the few minutes or hours you have with your kid is a ton of weighing and math.

Going forward I will list both the measurements and weights on the ingredients (we threw away the post-it...oops!).

Ingredients

• 1 Tbs coconut oil
• 1 onion
• 2 cloves garlic
• 2 medium sweet potatoes
• 1 chopped red pepper
• 1 cup frozen corn
• 2 cups unsweetened coconut milk
• Chopped basil or cilantro (if desired, not included in listed phe)

Cooking with coconut oil is amazing because of the smell alone.

Instructions

• Melt coconut oil in large pot over medium heat.  Add onion and garlic.  Cook for 5 minutes.
• Add red pepper and sweet potatoes and cook for 15 minutes.
• Add corn and cook for 5 minutes.
• Add coconut milk, place lid on pot, turn heat to low, and simmer 10-15 minutes.

What Is PKU?

This Really Happened At Laurel's 18 Month Checkup Today

I decided to dedicate this post about what PKU is to our pediatrician in hopes that he will some day stumble upon our blog and be able to have an actual conversation that pertains specifically to our daughter when we visit him.

I had no idea what Phenylketonuria (PKU) was either when we were told Laurel might have it (but then again I am not a doctor), neither did either of our families. Katie did because of a genetics class she had taken in school.  Not too many people know what it is but almost everyone has seen the word Phenylalanine, the warning “Phenylketonurics – contains phenylalanine” is on gum (that is sugar free), diet soda, and packets of artificial sweetener.

Phenylketonurics – contains phenylalanine

It scares people; I have actually spoke with someone who thought it was something they could get from drinking diet soda. The truth is, the only reason that warning is on there is to inform people like Laurel (a phenylketonuric) that the product contains Aspartame. Aspartame breaks down into a ridiculously high amount of phenylalanine and must be avoided by people with PKU.

PKU is an inherited metabolic disorder which makes the body unable utilize the essential amino acid, phenylalanine (phe). A deficiency in phenylalanine hydroxylase or PAH, which is produced by the liver, causes PKU.

PAH should convert Phe to tyrosine, in PKU patients it only does just slightly or not at all which causes Phe to accumulates in the blood and body tissues. Phe is toxic to the central nervous system in excess and causes the problems normally associated with PKU. If left untreated, people with PKU who consume too much Phe are at risk for IQ loss, memory loss, concentration problems, mood disorders, and in some cases, severe mental retardation especially if diet is not followed early in life.

Laurel, I am happy to say, has not experienced any of the side effects of PKU since we have followed the diet strictly.  

Another problem that children with PKU experience is that they can potentially not get enough protein due to all the diet restrictions.  Laurels Phe levels have come back between 2 - 6 (the perfect range), except for once when it was slightly below 2.  

This morning, at her doctors appointment, she was in the 98% for height, 92% for head size, and 70% for weight plus she is one smart cookie.

So in closing, despite our pediatrician refusing to learn the first thing about Laurel's PKU she is doing absolutely awesome!

Happy Johnny Appleseed Day

In honor of Johnny Appleseed Day and the importance of apples in a low-protein PKU diet, here are some fun apple facts.

•Apples are fat, sodium, cholesterol free (and nearly protein free).

•The crabapple is the only apple native to North America.

•2,500 varieties of apples are grown in the United States.

•7,500 varieties of apples are grown throughout the world.

•Apples are grown in all 50 states.

•The pilgrims planted the first United States apple trees.

•The apple tree originated in an area between the Caspian and the Black Sea.

•Apples are a member of the rose family.

•Apples harvested from an average tree can fill 20 boxes that weigh 42 pounds each.

•It takes the energy from 50 leaves to produce one apple.

•Apples are the second most valuable fruit grown in the United States. Oranges are first.

•The largest U. S. apple crop was 277.3 million cartons in 1998.

•Apples ripen six to ten times faster at room temperature than if they were refrigerated.

•A peck of apples weight 10.5 pounds.

•A bushel of apples weights about 42 pounds and will yield 20-24 quarts of applesauce.

•It takes about 36 apples to create one gallon of apple cider.

•Don't peel your apple. Two-thirds of the fiber and lots of antioxidants are found in the peel. 

•The apple variety ‘Red Delicious' is the most widely grown in the United States with 62 million bushels harvested in 2005.

Source: http://urbanext.illinois.edu/apples/facts.cfm

Legend tells of John Chapman, better known as Johnny Appleseed randomly spreading seeds throughout the Midwest.  The truth is he actually stopped and built entire nurseries that would be left in the care of local settlers.

Mr. Appleseed would set out every year, for over forty years, following streams headed west.  He'd fence in a large area of suitable ground and begin planting his apple seeds.  Some of these orchards still remain today well over 150 years later throughout Ohio, Indiana, and Illinois.

Happy Johnny Appleseed Day!

Keeping Track of Phe

We have to determine exactly how much phenylalanine is in everything Laurel eats.  To do this we weigh what she eats and then multiply the weight by the number of milligrams of Phe per gram of the food, which is published in the Low Protein Food List, published by Virginia E. Schuett.

Virginia E. Schuett has been publishing and updating the Low Protein Food list since 1995.  Ms. Schuett is a nutritionist and founder of National PKU News, a non-profit devoted to providing news and info and people living with PKU.  Over the years she tracked down the phenylalanine content of thousands of foods by contacting companies a variety of ways. Protein is listed on the nutritional facts of all food items but not the specific breakdown of which proteins.

The book also includes baking ingredients, herbs, and low protein products which helps when trying to create low protein versions of recipes we find on the internet (Pinterest, blogs, etc). Sometimes with just a few omitted ingredients or replacements we could have a recipe that is suitable for Laurel.

A Few Substitutes We Use Frequently

We are forever grateful for this index of nutritional facts in the Low Protein Food List but with such a wide variety of foods listed it is not always quick to navigate.  I have been trying to figure out the best method of keeping just the numbers for the food Laurel eat, as easy to access as possible for all of the people that need to use it.

We started with “The Foot”. 

We added to The Foot by hand as Laurels food repertoire expanded.  It consisted of mostly fruits and vegetables but we would still have to refer back to the foot list book for a lot of items.

My first attempt at using technology to keep track was by using a Google Document that everyone who watched Laurel had access to. 

It didn’t catch on.

I have checked out Android and iOS apps but none really stuck my fancy.

Current List

We currently keep a printable word document that is updated periodically with new foods.  It is kept stuck in The Book for everyone to use.

My other idea is to create a Pinterest board with photos of all the items Laurel eats, in alphabetical order, with the Phe listed on each of them. Visually appealing...easy to access…maybe slightly too ambitious.

We shall see what the future holds for keeping track of Laurel’s Phe but in the mean time feel free to share any stories you have about tracking Phe.

The Diet

Laurel cannot metabolize the amino acid Phenylalanine.  Phenylalanine is an essential amino acid that is found in everything that contains protein.  So this means no meat, dairy, nuts, beans, tofu, bread, and on and on. 

So what does she eat?!  Laurel eats measured amounts of fruits, vegetables, low protein bread, vegan cheese, noodles (in moderation), coconut milk products (yogurt, ice cream), and low protein snacks (crackers, cookies).  Also, something that we are learning to enjoy is creating alternatives for existing recipes using low protein products.  I hope in the near future that this blog can be an outlet for sharing some of these recipes as well as the outcome of following others’ recipes in the PKU community.

Doesn’t seem so bad…

The problem is, all of those foods have phenylalanine too.  So that leads us to our most utilized kitchen accessory…the scale (and his friend, travel scale).

The scales

We use the Low Protein Food List to determine the milligrams of phenylalanine per gram of the food that Laurel eats.  Once we locate the mg/g we are able to weigh the food, Laurel eats, we reweigh, do some math, and record in The Book.  I will get to the The Book in just a second but first I’d like to expand on who “we” is.

Laurel does not have to attend daycare because our amazing families were able to more or less rearrange their lives to help us.  Katie and I are extremely blessed to not have to worry the entire time we are at work because we know Laurel is the hands of people who are just as concerned about getting the math right as we are!  You will all never know how grateful we are for what you do.

How much pineapple did Laurel eat on August 27th, 2012?  She ate 105 grams if you really want to know.

The Book in all its glory

We have recorded every bite of food that Laurel has eaten since she was three weeks old.  This is necessary because Laurel, as I mentioned in another post, has to have an exact amount of phenylalanine each day.  The Book keeps a running total throughout the day to be sure that her dinner gets her to exactly the number her doctor is looking for.

Making sure your child eats all of their vegetables is one thing; watching, weighing, and measuring every morsel, of every piece of food they eat is something else altogether.

The Treatment (Formula)

Kisha shipped a container of metabolic formula, Phenex, to us overnight.  She explained that we should substitute Phenex for Enfamil immediately. We were told that we'd need to bring Laurel in to the hospital again in 3 days to have her Phe levels tested.  The mix of formulas need to be adjusted depending on the results so she would receive just the right amount of phenylalanine.  This was important because too much and she would see the ill effects of PKU, too little and she would not develop properly due to lack of protein.  This is the thin line we walk daily.

Laurel rocking her shades

For the first few weeks we had to bring Laurel in for blood tests about twice a week.  This was necessary because the amount of protein she consumes, sickness, and growth rates all affect her blood phe levels.  The results of the blood tests are anything but easy to get, the blood is tested out of state so we get results based on levels from a week prior (which is aggravating sometimes).  The mix of Phenex to Enfamil jumped around quite a bit the first few weeks but eventually stabilized to a 50/50 mix.

  

Phenex

The formula is ridiculously expensive ($282.49 for 6 Cans ($3.21 / oz) on Amazon) so we are very lucky to live in a state that provides it to us.  Unfortunately, not everyone is as lucky.  Some states still do not have the necessary financial help for children with inborn errors of metabolism.  The NPKUA is fighting to get the Medical Foods Equity Act passed through congress and you should help!  It seems like a no-brainer due to the fact that the annual cost for medical formula averages $7,100 compared to the millions it would cost over time for specialized nursing care or residential care facilities to those left untreated. 

While everyone is patiently waiting for the Medical Food Equity Act to pass you should visit theFacebook page for the National PKU Food Bank (scary this is even necessary) and if you can donate medical food for those who cannot afford the formula they require, which their state/insurance will not provide.

After the initial shock of finding out Laurel had PKU we quickly fell in to a nice routine of weekly blood tests and delivering just the right amount of formula to Laurel but we knew that the challenges of “real food” were just on the horizon.