Green Pepper Soup

Here’s a super easy, one-pot dish that’s become a favorite of mine for making in bulk and taking it for lunch during the work week.

Ingredients:

• 28 oz. can tomato sauce
• 28 oz. can diced tomatoes, undrained
• 275 gm. chopped green pepper
• 170 gm. chopped onion
• 2 pks. G. Washington brown seasoning

Directions:

• Combine all ingredients in a large stock pot
• Bring to a boil
• Cover and simmer until vegetables are tender (approximately 1 hour)

Yield: 8 (1 cup) servings
Phe: 114 mg
Calories: 73

Add low-protein pasta or low-protein rice for more volume. Just adjust phe accordingly.

-NM

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

Roasted Garlic Vegetables and Penne

This is an adaptation of a recipe that was recently served to my entire family over the Memorial Day weekend. It originally included smoked sausage, but that was obviously omitted for my portion. I liked its flavor so much I made it again last night (and probably will again this evening).

Ingredients:

• 116 gm zucchini, cubed
• 74 gm bell pepper, cubed (used approximately ½ red pepper and ½ yellow pepper for color)
• 28 gm red onion, coarsely chopped
• 58 gm cherry tomatoes, halved
• 23 gm garlic cloves (approximately 2 cloves)
• 2 tbs. olive oil
• 93 gm Aproten Penne
• 2 tbs. butter
• 2 tbs. white wine
• Salt and pepper to taste

Directions:

Preheat your oven to 375ºF. Mix all of the vegetables, garlic, olive oil, salt and pepper well in a large mixing bowl. Spread the vegetable mixture evenly on a large sheet pan.

Roast the vegetables on the middle oven rack for 10 minutes. Stir vegetables and place back on the middle oven rack for another 10 minutes. Meanwhile, cook the pasta according to the package directions, reserving ½ cup of the cooking liquid.

* Since the pasta will be cooked again briefly with the roasted vegetables and other ingredients added, you may want to cut off a few minutes of the recommended cooking time in order to avoid mushy noodles.

Remove the vegetables and set the pan aside. Remove the roasted garlic cloves from the roasting pan, smash to a paste with a fork and add to the reserved pasta cooking liquid. Return the pasta to the pot it was cooked in over medium-high heat. Add the butter, wine, reserved cooking water and vegetables to the pasta. Stir the pasta gently and cook until the liquid is about ¾ absorbed. Serve immediately, adding more salt and pepper if needed.

I’m listing this as one serving; however, if you’re making this for kids with limited appetites, you could probably stretch this a bit further. I’ve also listed separate phe and calorie counts in case you’d like to use a different kind of low-protein pasta or even experiment with imitation rice.

Yield: 1 serving (adult size)
Phe: 181 mg (155.5 mg without Penne)
Calories: 884 (or 554 without Penne)

–NM

Garlic Roasted Baby Bella Mushrooms

Here’s a flavorful and easy side dish I came across on Pinterest. I adapted the recipe slightly by using baby portabella mushrooms and also did the math to figure out phenylalanine and calorie numbers.

Here’s what you’ll need to get started.

Ingredients:

• 1 lb. pre-sliced baby portabella mushrooms (approx. 452 gm)
• 2-3 tbsp. olive oil
• 1 tbsp. balsamic vinegar
• 3-5 cloves garlic, minced or pressed
• 1/4 tsp. dried thyme
• 1-2 pinches cayenne pepper
• 1/4 tsp. salt, or to taste
• 1/4 tsp. freshly cracked pepper, or to taste
• 2 tbsp. chopped fresh parsley

Directions:

Preheat oven to 400°F. Rinse the mushrooms, if necessary, and set aside. Combine the olive oil, balsamic vinegar, garlic, thyme, cayenne, salt and pepper in a bowl and whisk until evenly blended. Toss the mushrooms in this mixture until they are evenly coated. Place them into a 7×11″ baking dish and make sure the mushrooms are spread out in a single layer. Roast the mushrooms for about 25 minutes. Remove from oven, sprinkle with the parsley and serve.

Yield: 3 servings
Phe: 124 mg per serving; 372 mg entire recipe
Protein: 3.5 gm per serving
Exchanges: 8.3 per serving
Calories: 144 per serving; 432 entire recipe *
Fat: 11.8 gm per serving

* Based on 2.5 tbs. olive oil and 4 cloves of garlic

After making this a couple times, I’ve served it along with Cambrooke Foods Pierogi or if I still have a good amount a phe left in the day, I’ll use it as a side dish to a baked potato.

–NM

Hair Loss experienced by PKU patients returning to the low-protein diet is temporary

There is some good news to report back to my blog followers: hair loss related to a return to the low-protein diet is temporary.

Earlier this month, I published a post titled Losing it: Both literally and figuratively over PKU and hair loss and during that post, I mentioned that PKU patients experiencing significant hair loss after returning to the PKU diet may want to see a specialist for insight behind the cause. I decided to take my own advice and scheduled an appointment with a dermatologist (I was referred to a dermatologist because of the profession’s specialization in matters related to the epidermis, or skin).

The dermatologist diagnosed my hair loss as telogen effluvium, or excessive hair shedding. She proceeded to describe to me a common cause of telogen effluvium where a major life stressor—say a car accident, pregnancy or even a crash diet—causes the hair follicles to stop growing. It is very similar to what was described to my clinic by another dietician. The dermatologist then sketched out a rough timeline on the paper covering the examination table and here below, I have tried to recreate it.

Click image to enlarge.

To summarize this graphic, the progression of my own hair loss coincided with what is commonly seen in cases of telogen effluvium. The initial shock to the system was my sudden return to the low-protein diet, and as a result, my hair follicles entered a resting phase. Generally speaking, the hair loss becomes noticeable about one to two months after the initial stressor when the new hair growth pushes out the resting hair.

Thankfully, this means that PKU patients who experience hair loss after returning to the PKU diet will eventually grow back their hair. In my case, I can already see “baby hairs” peeking through my hairline. Unfortunately, my dermatologist estimates it will take approximately two years before those baby hairs are long enough to tie back into a ponytail. In the meantime, I’ll embrace the heck out of some hairspray! 🙂

–NM

How to save money with a PKU herb garden

An article published in the March 2013 edition of Genetics in Medicine found that 50 percent of states provide either no coverage of medical formula and modified special low-protein products or only partial coverage of these required PKU therapies.

Those of us living with PKU do not need a reminder about just how expensive it is to remain on-diet; however, there is certainly a bit of vindication whenever a peer-reviewed, academic journal reiterates this point. Beyond specially formulated low-protein products, families dealing with PKU are often advised to experiment with herbs and spices. The idea being that some creativity in the kitchen can lead to flavorful meals that people with PKU will actually want to eat!

Yet, I have found that even buying fresh herbs on a regular basis can be costly. Plus, you generally have to purchase them in large quantities and then you are left tossing the left over herbs when they have eventually spoiled.

The solution? I recently started my own PKU herb garden and it features a handful of herbs that I have used most over the past three months: cilantro, basil and rosemary (and for good measure, I’ve also included two cherry tomato plants).

Here is a basic cost comparison (tax not included) for purchasing herbs at a local grocery store versus growing your own:

Herbs at Grocery Store	Herbs at Home Improvement Store
Cilantro, $1.99/0.66 oz	Cilantro, $2/plant
Basil, $1.99/0.66 oz	Basil, $2/plant
Rosemary, $1.99/0.66 oz	Rosemary, $2/plant
TOTAL: $5.97	TOTAL: $6

There you have it; only a $0.03 difference for the initial upfront cost. What’s significant is you’ll have to pay approximately $6 each time you buy these three herbs at the grocery store whereas you’ll pay $6 one time for plants that will last you all growing season!

Of course, there is the added cost of potting soil and a planter, but you could also experiment by planting your own garden directly in the ground.

Have you also started a PKU herb garden? Perhaps you’ve planted more than just herbs. Either way, let me know how green your thumb is!

–NM

Caring for Maternal PKU: Part III

Welcome to the third and final post in the Caring for Maternal PKU series. In Part I, readers were introduced to the research Kimberly R. Regis RN, MS, CPNP, conducted in support of her Doctorate of Nursing Practice final project at The Ohio State University. The objective of Regis’ research was to “describe the care needs and preferences of adult women of childbearing-age with PKU” by going directly to the women and asking about their experiences. In Part II of the series, we learned that despite being well-informed about the risks associated with maternal PKU, the group of women Regis interviewed had a relatively low combined pregnancy success rate. In an effort to see what factors—in addition to knowledge level—might be at play, Regis explored maternal PKU support systems by asking her participants the following questions:

• What support systems do participants have that either help or hinder adherence to dietary management?
• From the participants’ perspectives, what can be done to improve support for ongoing dietary management within the clinic service?

For all of the women who participated in Regis’ study, family members were identified as being the greatest source of support. Mothers in particular were mentioned not only as a supporter but also as an individual who truly understood and empathized with what it was like to struggle with the low-protein diet. Other examples of how family members provided support included finding new recipes to cook or share with the PKU patient, or even in some cases, helping to pay for costly medical foods.

With regard to the support participants received from clinics, most felt that clinicians were friendly and supportive, but some concerns still existed. For example, some participants felt like information about new foods and treatment was only provided if the patient asked about it. Clinic staff did not always provide individualized advice and overall communication from the clinic occurred less frequently overtime.

“Right now I’m still just in the monthly stages of taking my level every month, and that’s pretty much all that I hear from my nurse. And I never really hear from my dietician other than when I call her to order formula.”

The reality is that most PKU clinics are spread-thin and overworked because of having to respond to more than just PKU patients. But developing specialized care for maternal PKU is significantly more complicated than just refreshing clinical best practices. That’s because as Regis acknowledged, treatment for PKU has evolved over the years, allowing for more PKU patients to live longer and engage in “traditional” lifestyle activities such as pursuing advanced college degrees and starting families. Unfortunately, despite the need for individualized care, many PKU adults are still seeking treatment at a pediatric-focused facility such as Children’s Hospital. To be fair, participants within Regis’ study did not explicitly express concerns about receiving care from a pediatric institution; however, there was strong agreement on the need for more support beyond education.

What would this additional support look like? Regis suggests that social work, mental health and gynecological services may all be applicable for advancing the care of maternal PKU. No matter what the medical treatment of PKU will look like in the years to come, it must evolve to include geneticists, dieticians and nursing practitioners who understand and specialize in maternal PKU. Perhaps Regis herself said it best when she recognized women of childbearing age with PKU as being a “distinct population with the shared risk of having offspring with disabilities as a result of their genetic disorder.”

A distinct population – even one that is a subset of a group defined by a rare genetic disorder – should translate into individual, specialized care.

–NM

Caring for Maternal PKU: Part I

Caring for Maternal PKU: Part II