Calories – Why you’re not just counting phe during maternal PKU

Like most women, I am no stranger to counting calories and limiting the amount I eat each day in order to lose or maintain weight goals. When returning to the low-protein PKU diet in January 2013, consuming a higher amount of calories was definitely a major concern of mine. In fact, I would say that calories – more so than taste – was a key decision factor for when selecting a formula. Let’s face it, when you’re drinking formula three and four times a day, those calories can rack-up fast!

But all that had to change after finding out I was pregnant.

Eating right during pregnancy

Before discussing calories and maternal PKU, here’s a quick look at what the National Institutes of Health recommends for progressively increasing calories throughout the course of a pregnancy:

• 1^st trimester: 1,800 calories/day
• 2^nd trimester: 2,200 calories/day
• 3^rd trimester: 2,400 calories/day

I suspect that because of the old adage, “when you’re pregnant, you’re eating for two,” most pregnant women do not have any trouble meeting those calorie goals. But if you have PKU, and are challenged with a low-phe tolerance, meeting those goals can seem next to impossible. Yet, doing so is just as critical as making sure you limit your protein intake.

Why you must count both phe and calories for maternal PKU

If you do not consume enough calories, your body can enter what’s known as a catabolic state. It’s a big concern for those in the bodybuilding industry because without adequate post-workout nutrition (a.k.a. a protein shake), bodybuilders are at risk for excessive breakdown of muscle mass and could thereby end up throwing all their years of hard training down the drain.

While counterproductive for fitness gurus, this catabolic phenomenon can be downright dangerous for someone with PKU. That is because when you’re not getting enough calories your body tries to compensate by breaking down muscle for energy. And since muscles are comprised of protein, blood phenylalanine levels will increase as a result – this despite the fact you may be strictly adhering to a low-protein diet. As you can imagine, this could also have grave implications for maternal PKU since phe levels are doubled when crossing the placenta.

Five tips for getting those calories

So what’s a gal to do? First and foremost, I had to switch my train of thought. While still self-conscious about pregnancy weight-gain, I realize that there’s more at stake than my ego. There’s a baby now that depends on me to set all that aside and make sure I tackle the daily balancing act of eating right. Here are a few other tips I learned along the way:

• Switch to a higher calorie formula – As soon as I found out I was pregnant, I made the decision to switch to a formula higher in calories. I’m currently taking four, 50 gm servings of Phenex-2 per day, which accounts for 820 of my daily caloric intake.
• Create a stash of low-phe/high-calorie snack options – My favorites are Welch’s Fruit Snacks (0 mg/80 calories per 0.9 oz. packet), Rice Krispies Treats (26 mg/90 calories per bar) and Little Debbie Zebra Cakes (38 mg/161 calories per cake). Adding Biscoff European Cookie Spread (25 mg/90 calories per tablespoon) to low-protein raisin bread or apple slices is another great way to increase those calories. Though not really a snack option, making pancakes using the Cambrooke Foods MixQuick product is another great way to front-load your day with a high-calorie breakfast. I usually half the serving size, which still comes out to 200 calories.
• Always pack snacks – Make sure to also carry some snack options in your purse, car or backpack. This will help prevent you from being hungry while stranded without food options.
• Take the time to figure out the calories/per serving of your favorite low-protein recipes – In order to have the most accurate picture of how many calories you still need, be sure to take the time to figure out the calorie equivalents of each recipe ingredient. I was particularly challenged with this when eating my favorite Cook for Love recipes. Thankfully, with the recent launch of the How Much Phe website, this process is not nearly as painful. If you haven’t subscribed to the site as of yet, I highly recommend it – especially if you’re pregnant with PKU.
• Replenish what your burn – Lastly, don’t forget that if you exercise, you’ll need to eat more than what your PKU dietician has recommended. Tracking calories burned during exercise is not an exact science, but I have found that mobile apps like My Fitness Pal and RunKeeper can be very helpful in providing an approximate calorie deficit count. My Fitness Pal can be used as an electronic food diary but unfortunately it isn’t all that convenient for those on the low-protein diet. However, I’m still able to use the app in a limited fashion by setting up a user profile that tracks my current weight, sedentary lifestyle, and estimates how many calories burned after completing a workout. RunKeeper is another calorie tracking app that uses GPS to track run/walk distances and then estimate the number of calories burned. I use both on a regular basis and have found that RunKeeper is great for cardio and My Fitness Pal is good for other exercise options like weight lifting and prenatal yoga.

I should also say that I haven’t taken this as a free pass to eat whatever I want. If I notice at the end of the night that I’ve already met my calorie goal, but still need some phe, I’ll opt for some yogurt or other low-calorie option to meet that goal without going overboard on the high-sugar, high-calorie options.

–NM

At-a-glance PKU Pantry Labels

One of my recent Pinterest-inspired projects was creating burlap labels for my pantry. It’s a fun DIY project that spoke to my inner organizing freak.

Grouping similar products in baskets or other containers make it easier to find products quickly and placing ingredients in clear jars let you know when you’re getting low. Among other labels, I decided to create hanging canister labels for brown sugar, wheat starch and baking mix—three ingredients I use frequently, especially when making tasty PKU-friendly cakes, cookies and pastries.

I use Cook for Love’s baking mix, which if you’re familiar with it, you know it is comprised of three key ingredients. To make life a little simpler, I decided to make an at-a-glance label on the backside of the baking mix canister that reminds me of the key ingredients without having to look it up online or find the recipe I printed eons ago. Also to save time, I like to make the baking mix in bulk. In fact, I have doubled the baking mix recipe in the canister shown here.

If you aren’t necessarily the crafty type, you can still take a similar approach by a using a large, sealable plastic container to make the baking mix and a permanent marker to jot down the ingredients on the outside of the container. Not as pretty, but definitely just as functional. Just remember to whisk before each use!

–NM

Five ways to recognize National PKU Awareness Day through Giving Tuesday!

Last year, in recognition of first-ever National PKU Awareness Day, I created a wish list of sorts for what a national platform could mean for a rare genetic disorder like Phenylketonuria. This year, I decided to make a different kind of list – one that capitalizes on the spirit of giving.

The inspiration for this list came to me after hearing a radio announcer observe that after just coming through Black Friday and yesterday’s Cyber Monday, today is known as Giving Tuesday. I immediately thought, what better way to celebrate National PKU Awareness Day (which also happens to be today) than to give-back to one of the many PKU non-profit causes!

So on that note, here are just a few ideas for how you can celebrate five worthy causes in honor of both National PKU Awareness Day and Giving Tuesday:

The National PKU Alliance – Created in 2008, the National PKU Alliance was established to serve as a voice for PKU patients and families by focusing on a number of mission goals such as improving insurance equality, funding for peer-reviewed research and supporting the ongoing effort to find a cure. You can read more about this non-profit’s accomplishments in its 2012 annual report and tax deductible donations can be made through the National PKU Alliance website.

Bring Fanni Home – Anna Parker, a PKU patient/mom, and her husband Brandon have been trying bring home Fanni, a little girl in China, also with PKU, who had been abandoned on the public transit system at the age of four. The $30,000 price tag for international adoption can seem insurmountable; however, with the support of generous PKU community, we can help bring Fanni home to the Parkers. This Giving Tuesday/National PKU Awareness Day, consider making a $10 donation to this very worthy cause. Visit the Bring Fanni Home website for more details.

National PKU News – Located in Seattle, Wash., National PKU News is pushing its 25^th year anniversary for providing current and accurate news related to PKU. Virginia Schuett, the organization’s founder is a former PKU nutritionist and is also responsible for several other fabulous PKU resources like the Low-Protein Food List, Low Protein Cookery for PKU and Apples to Zucchini: A Collection of Favorite Low Protein Recipes. According to the National PKU News website, “newsletter subscription fees and sales from books provide less than 50 percent of the financial needs of the organization.”  Your donations to this multi-faceted organization will help it reach its 25^th anniversary and many more to come!

Cook for Love – This invaluable resource has been one of my favorite go-to resources for PKU recipes. Cook for Love is a culinary website created by Brenda Winiarski, mother of two PKU children. The foods she has created come as close to traditional, high-protein foods as any other I’ve seen. My mother has often joked that some foods on the Cook for Love website feel more like science experiments than a recipes, but honestly Brenda and her partners have done an amazing job mimicking food properties like the rise of a loaf without flour and the binding of a cake without eggs. Completely dependent on donations, all of the recipes posted to Cook for Love are free to access with the creation of a user name and password. A $25 donation will help ensure that Cook for Love can continue to maintain the website as well as the genius behind these tasty foods.

Support your local PKU organization – Many groups like my local Tennessee PKU Foundation support efforts similar to the National PKU Alliance but on a more regional level. These groups also rely on donations to educate and raise awareness about PKU and other metabolic disorders. The Tennessee PKU Foundation accepts donations via mail or you may also contribute online. Be sure to check out your local PKU organization and contact them to see how you can support them.

–NM

Creating a kitchen tablet holder for PKU recipes

According to the Pew Internet & American Life Project, a third of American adults ages 18 and older own a tablet computer like an iPad, Samsung Galaxy Tab, Google Nexus or Kindle Fire. That’s almost twice as many from one year ago. Chances are, folks in the PKU community are no exception to this growing trend. In fact, I tend to use my own tablet for referring to recipes as opposed to the traditional printed cookbooks. That’s where the inspiration for this DIY project came from. Here’s how you can also make your own kitchen tablet holder for PKU recipes.

First, I found an old cutting board from a thrift store for $1.50. Then I purchased a Scrabble tile holder from a local antiques and collectables store for $5. For a little embellishment, I found unfinished wooden letters from JoAnn Fabrics (although you could also find something similar at other hobby stores such as Michaels and Hobby Lobby). Lastly, you need a wooden wedge of sorts. My brother-in-law, who is handy with a power saw, made mine, but a child’s wooden block would also work. Use wood glue to assemble the pieces, allow to dry over night, and cover with your favorite paint color. I decided to use a slightly darker shade for the “Create” letters so that it would pop a little.

Enjoy!

–NM

Returning to the PKU Diet—Easy Does It!

Going from a completely liberated diet to one that consists of limited protein intake can be rather intimidating when you look at it from a 25,000-foot view. However, breaking-up the task in small chunks and tackling the goal with baby steps can make all the difference between success and failure.

That’s the approach I’m taking after recently deciding to give the low-protein diet a try. I first started this effort last week by committing to taking my formula four times a day. I didn’t worry about altering my diet any; just made sure that I had 60 grams of protein via formula and see how that goes.

Use the RxmindMe app to set reminders on your phone to take your formula.

For me, getting all the formula in was not a problem. I would take one with breakfast, the second at 10 a.m., a third near the end of the workday before heading to the gym, and the final before heading to bed. I even set reminders on my phone using RxmindMe and on my Outlook Calendar just in case I got caught-up with work and forgot to take a break for drinking my formula.

After about the third day of doing this, I started to get really strong headaches, especially right after waking up in the morning. At first, I attributed them to stress at work, but when they persisted even on into the weekend, I figured there might be something else at play. Eventually, I concluded that by not altering my diet, but still consuming 60 grams of protein through formula, I was in essence consuming twice as much protein as a non-PKU person would normally eat.

First time making Cook for Love’s low-protein bread! The 2nd loaf (on the right) definitely came out better.

The next step in my goal of returning to the low-protein diet was to cut-out all meat. Given the persistent headaches though, I decided to ramp-up my progress. I decided to try my hand at making Cook for Love’s sandwich bread—in fact, I made two loaves and have frozen the second for eating later. I also started incorporating a few low-protein modified foods such as Aproten pasta and Cambrooke’s rice. That being said, I went from eating 60-90 grams of protein to just 15 grams as of yesterday. And the best part? No headaches when I woke this morning!

I realize the real trick will be to continue this progress in the long term, but again, to keep from getting overwhelmed, you have to take it one day at a time. Today, I am going to work on building out my menu for the entire week. I’ll pick out some new recipes, search some free foods through Virginia Schuett’s Low Protein Food List and go from there. As I try new things or find tips to share, I’ll regularly post those here. Of course, if you have any to share, please feel free to do so in the comments section below.

–NM

Cook for Love’s Banana Bread

This weekend I got an itch to try a new low-protein recipe. I’ve had my eyes on Cook for Love’s banana bread for about a year now, but because of work and grad school only now have I had the chance to give it a shot. Like most of the baking recipes on Cook for Love’s website, this banana bread started out with 2 cups of the website’s low-protein baking mix. I made a double batch of my own and stored it in a leftover Mix Quick bucket I had from Cambrooke Foods. Then using a Sharpie permanent marker, I labeled the bucket with its new contents, including all the individual ingredients and their amounts. This way, whenever I want to try another baking recipe from Cook for Love, I already have the baking mix assembled!

I was able to gather all of the ingredients from my local Kroger Marketplace with the exception of two items. I had to special order wheat starch from the Internet (either through Amazon.com or direct through Cambrooke Foods) and I had to make a separate trip to Wal-Mart in order to find cake flour (specifically Swans Down). Once I had everything in-hand, the preparation time was practically nothing!

Final review: After letting the loaf pan cool, I sliced a couple pieces and shared them with my husband. At first-bite, the bottom of my banana bread seemed chewy, like it had been over cooked, but as I cut slices closer to the center of the loaf, the chewiness disappeared. The banana bread was extremely moist, almost “spongy” like a poppy seed cake might be. My husband was surprised at how much the batter actually rose in the oven, even without eggs or traditional flour. Overall, this is a very delicious recipe and pretty darn hard to tell it’s not the real thing!

Hands on Time: 10 minutes
Cooking Time: 55-65 minutes
Yield: 1 loaf, 12 slices
Phe per Serving: 27 mg per slice

Out of respect for the hard work, sweat and tears that the folks at Cook for Love have invested in developing PKU-friendly foods, please visit their website to see details on the ingredients and specific cooking instructions for this recipe and many others.

–NM