Roasted Garlic Vegetables and Penne

Roasted Garlic Vegetables and Penne, low-protein pasta dish, PKU-friendly pasta recipeThis 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:

Roasted Garlic Vegetables and Penne, low-protein pasta dish, PKU-friendly pasta recipePreheat 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

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On the hunt for aspartame-free gum

aspartame gum, sugar-free gum

Growing up on the low-protein PKU diet, I was well aware to stay clear of chewing gum that contained aspartame (also marketed under brand names AminoSweet ®, Equal®, NutraSweet® and NatraTaste®). I had a ‘safe list,’ if you will, of all the brands I could choose from, but after searching through all my familiar fav’s on a recent visit to the grocery store, it appears that nowadays you cannot find a single pack of gum that doesn’t contain aspartame.

Discovered in 1965 and later approved as a food additive by the FDA in 1981, aspartame is used in chewing gum to provide a long-lasting sweet sensation since other ingredients such as sugar, dextrose, and corn syrup dissolve soon after chewing begins. Chemically, aspartame is comprised of a small amount of methanol and the two amino acids aspartic acid and phenylalanine – the last of which we know is very harmful to PKU patients.

According to www.aspartame.org, aspartame is currently found in more than 6,000 products worldwide including soft drinks, confections, gelatins, dessert mixes, puddings and fillings, frozen desserts, yogurt, tabletop sweeteners, and some pharmaceuticals such as vitamins and sugar-free cough drops. That’s a lot of products for PKU patients to be wary of!

Thankfully, with the growing popularity of all-natural products, there’s a new selection of chewing gums hitting the market featuring xylitol, an all-natural sugar alcohol. Also, since xylitol is an anti-microbial, it helps prevent tooth decay and provide a quick breath-freshener, especially after just chugging a serving of PKU formula.

Here’s a look at some of the aspartame-free gum currently on the market. Since they’re still rather new, finding these in common grocery and drug stores is difficult, but I’ve been able to buy some online, specifically through Amazon. Depending on where you live, you may also be able to find these products at specialty grocery stores like Whole Foods Market, Trader Joe’s, Jungle Jim’s or Earth Fare.

aspartame-free gum, PKU-friendly gum

Click to enlarge image.

  • Pür Gum
  • XyloBurst Xylitol Gum
  • Epic Xylitol Gum
  • Peppersmith Chewing Gum
  • Sugar-Free Glee Gum

If you’ve tried any of these aspartame-free gum products – or have others to add to the list – just let me know in the comments section below.

–NM

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Grab your walking shoes–spring has arrived!

Walk for PKU, PKU Research, Donate to PKU

The trees are turning green, flowers are in bloom and the birds have started singing again. Here in the South spring has sprung and that also means that lots of planning is underway for the Tennessee PKU Foundation’s two spring fundraisers–a walk for PKU in Nashville on May 3 and another taking place in Knoxville on May 17.

As they have done in years past, these two walks—as well as a third organized for Memphis, Tenn., later in the fall—are organized to raise money in support of PKU research. Since its inception, the Tennessee PKU Foundation has raised more than $63,400 for individual researchers and those supported through the National PKU Alliance. Other funds raised by the foundation stay within the state to support camp and conference scholarships as well as newborn welcome packets.

On a national level, the National PKU Alliance’s Scientific Advisory Committee—comprised of physicians, researchers and clinicians—reviews proposals submitted by researchers who are working in the inherited metabolic disease field and awards grants to those committed to advancing PKU treatments and ultimately developing a cure. Those researchers receiving grants from the National PKU Alliance in 2014 were just recently announced. You can read the full list of recipients on the nonprofit’s website, but two examples include Dr. Shawn Christ, associate professor of psychological sciences and associate director of the University of Missouri’s Brain Imaging Center, and Dr. Kristen Skvorak from the University of Pittsburgh Medical Center. Christ is examining the effects of PKU on gray matter structures in the brain, and Skvorak has been pursing ground-breaking experiments in liver cell transplants for PKU in a mouse model.

Several other U.S. states are also member organizations of the National PKU Alliance and if there’s one nearby your home, I encourage you to support them in their own individual fundraising efforts. However, if you do not have an affiliation with any of these groups but still feel so moved to also support PKU research, you can contribute online via the Tennessee PKU Foundation’s walk registration website. Even if you cannot attend one of these events in person, you can still play a role in supporting PKU research.

–NM

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At-a-glance PKU Pantry Labels

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.

At-a-glance PKU Pantry LabelsI 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

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Cabbage Burgers

This recipe from The Wooden Spoon sounds absolutely delicious! Can’t wait to give it a try.

The Wooden Spoon

photo 4(2)

 

This is a great simple recipe. Cabbage is a great source of fiber, vitamins C, K and folate. Try these burgers as a school or work lunch. They re-heat and freeze well.

 

3 cups cabbage

1 cup Loprofin baking mix (or any other baking mix)

2 tbsp Kingsmill egg replacer

1 small onion

2 cloves garlic

2 tsp ground black pepper

1 tsp salt

Vegetable oil for frying

In a food processor mince onion, garlic and raw cabbage until you get smooth fine texture. Transfer minced vegetables into a mixing bowl. Stir in baking mix, egg replacer and salt and pepper. Mix well until you achieve thick dough like consistency. If your cabbage did not give enough juice and mixture seems too dry you may add about one tablespoon of water. Let the mixture rest for about 10min. Spoon the mixture into a frying pan with some hot…

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Garlic Roasted Baby Bella Mushrooms

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

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What’s in a name? A closer look at changing the name of PKU

Changing the name of PKU, PKU name change, from PKU to PAH deficiency

Recently, the American College of Medical Genetics and Genomics released new guidelines in support of lifelong treatment for PKU. Among other recommendations, the organization also suggested the name of the disease be changed from PKU, short for phenylketonuria, to phenylalanine hydroxylase deficiency, or PAH deficiency.

The ACMG acknowledges that there are several ways to describe PKU severity and specifically calls-out at least two naming conventions that are commonly used:

  1. PKU, the more severe diagnosis where untreated patients have blood phe levels greater than 1,200 μmol/l or 20 mg/dl and,
  2. hyperphenylalaninemia, which represents a milder diagnosis where untreated patients are still above what’s considered normal but less than 1,200 μmol/l or 20 mg/dl.

After the National Institutes of Health introduced the term hyperphenylalaninemia in 2000, we started seeing an increasing number of clinicians, patients and families of patients refer to the first, more severe category as classical PKU. The new name PAH deficiency, the ACMG proposes, would eliminate the practice of using the blood phe level as the standard of classification by representing all of these variations.

The communications and marketing professional in me knows that over the life of a product or business, there may be many reasons the decision is made to change a brand name including mergers/acquisitions, changing markets, new leadership, and to even eliminate a negative reputation.

In the case of PKU and the ACMG’s recommended name change, the decision was made because PAH deficiency more accurately describes what the authors refer to as the “spectrum of severity.” But, it’s worth noting that descriptive names can also have their own subset of issues. Brand names that are too literal can become forgetful or inaccurate over time. Names that are too complex or long can impact a person’s ability to pronounce or remember it.

What’s more, we also know that the use of medical jargon can make it difficult for individuals to absorb and process health-related information. So what will the impact be when folks within the PKU community try to make the transition to phenylalanine hydroxylase deficiency? When phenylketonuria was introduced into the vernacular in 1934, it likely created its own source of confusion in the patient community. Yet, there is something to be said for brand recognition. Overtime, the brand familiarity of PKU has become stronger within the community and it’s likely that many PKU patients have a strong association with the term PKU, and to some extent, identify themselves with the now-outdated term.

It will take time for PAH deficiency to be wholly embraced. Even I have started to wonder, “What does this name change do to the title of my blog?” I’m not quite ready to makeover PKU Parlor, but I can acknowledge that with the name change there could be some benefits. For example, wrapping-up the varying levels of PKU severity under one umbrella may go a long way for securing universal medical food and formula coverage. Lawmakers and other decision makers will be less inclined to offer coverage to a severe group of PKU patients while simultaneously denying or restricting access to those with milder cases.

So as a member of the PKU PAH deficiency community, how do you feel about the name change? Are you reluctant to make the transition? Or do you embrace the new name and find yourself excited for the fresh start? I’d like to hear from you and what your reaction was to the suggested name change. Also, if you ever thought of alternative names for PKU, be sure to share them in the comments field below.

–NM

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