Category Archives: Lo-pro Diet Management

How to conduct an at-home blood test for PKU

Regularly monitoring blood phenylalanine (phe) levels is a critical element to the treatment of PKU. The frequency of collecting samples will vary depending on age, clinic preference and the PKU patient’s current health status. For example, maternal PKU patients may collect samples as many as twice a week. It is important to discuss with your clinic how often you should send in blood tests, but should you ever need a quick-reference guide for conducting an at-home blood test, here are a few tips.

Organizing PKU Blood Level Supplies

General Tips:

  • In order to reduce the amount of time it takes to perform a blood level, organize supplies in clear containers. This also lets you know when supplies are running low.
  • Fast for at least two hours, no more than six, before taking a blood level.
  • Chose a different puncture site each time in order to avoid scar tissue or oversensitivity to the puncture-area.
  • Fill at least three circles within the filter paper. I will usually fill all five, just to provide my clinic with several specimen options.

Blood Test Prep:

  • Use a heating pad or hand warmer for about 10 minutes to increase blood flow. Running your hand under hot water may also do the trick.
  • Fill out filter paper with your personal information.
  • Gather supplies needed for single-time use.

10 Easy Steps:

  1. Clean fingertip (or heel if performing the test on an infant) with alcohol wipe. If you do not have an alcohol wipe, wash hands with soap and warm water.
  2. Open Band-Aid from its packaging for easy access after test is complete.
  3. Position the lancet on the side of your finger.
  4. Press the lancet firmly on your skin at the puncture site.
  5. Turn the hand over and let a drop of blood form on your fingertip.
  6. Fill filter paper circles with blood by allowing the drop to freely fall onto the paper (without touching) and while being careful not to overlap drops.
  7. Wrap Band-Aid over the puncture-area.
  8. Discard used lancet in a biohazard sharps container. If you do not have a biohazard sharps container, you can also collect used lancets in a Ziploc plastic bag and bring those to your clinic for safe disposal.
  9. Allow the filter paper to dry overnight, although if you are under a deadline, 2-3 hours will suffice.
  10. Seal the filter paper in an envelope and mail to your clinic (don’t forget the stamp).

For other references and tips for performing at-home blood tests, the National PKU Alliance’s PKU Binder has an entire section focused on Monitoring Blood Phenylalanine Levels and BioMarin has 12-page, electronic brochure describing How to Take Your Blood Phe Levels at Home.

–NM

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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.

How to Save Money with a PKU Herb GardenThe 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

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Tervis Tumbler + Olive Garden = Awesome PKU Dining Experience!

Earlier this week, I ventured out for my first restaurant meal since returning to the PKU low-protein diet. I was meeting my mother for lunch and we selected Olive Garden because we knew the unlimited salad (minus croutons and cheese) would be very PKU friendly. I also had the idea to bring some of my Aproten low-protein noodles with me. I had a batch already cooked and sitting in a Tupperware container in my refrigerator so all I had to do was re-heat them and find a way to transport the noodles while still keeping them warm. I immediately thought about a casserole dish/thermal carrying tote combo that I have, but I didn’t like the idea of walking through the restaurant carrying a large dish.

Tervis Tumbler, Low-Protein Noodles, Eating out on a PKU Diet

An insulated Tervis tumbler is a great way to transport low-protein noodles when eating out on a PKU diet.

What did I do next? Well, this may sound a little silly, but I transferred my noodles into a Tervis tumbler. If you aren’t familiar with the brand, Tervis tumblers have a double-walled design that allows the air in between to keep hot drinks hot and cold drinks cold. Knowing about the tumbler’s insulated properties, I thought it might also be feasible that the tumbler would keep my noodles warm. Even better was that after sealing the tumbler with its tight-fitting travel lid, I was able to discretely carry the noodles in my purse (For the record, I do carry a very large purse!).

When I ordered my meal, my mother and I explained to the waitress that I had special, dietary needs and asked if it would be possible to order just sauce and an extra plate. Specifically, I wanted to order the sauce that came with the Penne Rigate Pomodoro, a mixture of roma tomatoes, garlic, fresh basil, extra-virgin olive oil and marinara sauce. Rather than charging me full-price for the dish, the Olive Garden staff was kind enough to provide me the Pomodoro sauce at the cost of two dipping sauce orders. When our entrees arrived, the waitress brought an extra plate and two dipping saucers full of Pomodoro sauce. I grabbed the Tervis tumbler from my purse, dumped the noodles on the empty plate and covered the noodles with the sauce. Over all, I had a completely awesome experience and I give the staff at the Knoxville/Turkey Creek Olive Garden mad props for helping to make my dining experience as pleasant as it was!

Finally, in closing here’s an update on how my return to diet is going: Yesterday marked three weeks since returning to the low-protein diet. My first on-diet blood level, taken on Jan. 27, came back at 4.3 mg/dl. That’s right in the ‘sweet spot’ of my clinic’s desired treatment range between 2 and 6 mg/dl. I was definitely impressed at how quickly my phe levels dropped, especially considering the last level I had prior to returning to diet was 20 mg/dl. I’ll be taking another level later today since my goal is to send a level to my clinic every two weeks.

–NM

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Planning a PKU Menu

I’ve said this before on PKU Parlor, but the ability to plan and organize is going to be instrumental for me adhering to the low-protein diet. And if you know me, then you know I have affinity for being obsessed with organization.

Allow me to introduce my new chalkboard wall…or as mother referred to it, my command center. 🙂

Menu-Board

After 10 coats of magnetic primer and two quarts of chalkboard paint, I have successfully turned one wall of my laundry room into a customizable reminder/menu board.

You may recall that in my last post, I briefly mentioned that I intended to plan-out my menu a week at a time. As you can see here, after I completed my grocery shopping, I jotted down the menu items on this chalkboard wall as a way to remind myself of any items left for eating as I get further into the workweek. At the end of the week, I erase the items and start all over again. The left-hand side of the board has a special section reserved for reminders, both PKU-related and non-PKU related.

Now it’s worth noting that planning a low-protein menu a week at a time can be challenging. Among other items, it demands a ton of fresh produce and an arsenal of spices for livening up what otherwise might be considered bland, low-protein foods. Since I don’t want to lose track of all the hard work put towards menu planning, I also plan on creating an electronic database of meal plans. I haven’t created the database yet, but when I do, I’ll likely create it using Microsoft Excel and include columns noting phe amounts and whether the recipe calls for low-protein products. The last of these discriminators will prove valuable for those moments when I find myself running low on low-protein foods and need to get through the coming week eating whatever is available in local grocery stores.

As I conclude this post, allow me to reflect on what today marks one week since first cutting out all high-protein food items in my diet. I agree that I feel better. That’s hard for me to admit since I prided myself for so long as someone who could eat whatever and still function at a high level. One of the harder things for me to do mentally is to stop counting calories and start to worry more about protein. I realize that eating low-protein won’t always align with low-calorie so I’m having a bit of a struggle letting go of that.

Until next time!

–NM

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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.

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.

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

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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?

Maternal PKU, Pregnancy and PKU, PKU, PhenylketonuriaFor 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

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Caring for Maternal PKU: Part II

Maternal PKU, Phenylketonuria, Maternal PKU Knowledge Test

For the second installment in the Caring for Maternal PKU series, let’s take a closer look at the knowledge factors incorporated into the participant interviewee questions for Kimberly R. Regis’ Doctor of Nursing Practice final project, Childbearing age women with PKU: Assessment of care needs and preferences. In Part I of the series, questions four and five asked:

4. What is the knowledge level of the participants regarding PKU and maternal PKU?

5. How does knowledge level regarding PKU impact decisions regarding birth-control and family planning?

Why would Regis focus on the knowledge level of the adult women with PKU? For the last two decades, PKU research has focused in large part on emphasizing the need to educate adult women with PKU so they could better self-manage their condition and prevent the occurrence of maternal PKU syndrome. Regis wanted to “gain a better understanding of the ways in which a children’s hospital-based metabolic specialty clinic may best serve them [adult PKU women of childbearing age].” Was the answer that clinics should continue to serve maternal PKU patients by following conventional research, which emphasized the importance of ongoing education? Or was there a need for something more?

To explore these options, Regis started with the knowledge variable and had the six participants take the Maternal PKU Knowledge Test, a 10-question quiz that was developed in 1990 by Shiloh, St. James and Waisbren. The three authors recommended the test as a way for clinics to screen and identify adult women with PKU who might benefit from more education.

The study’s participants answered a majority of the questions correctly, with scores ranging from seven to 10 correct. The question that elicited the most trouble when trying to answer was number seven (you can see a complete list of questions and take the test for yourself by visiting the Maternal PKU Knowledge Test page on PKU Parlor). A common theme that emerged from the open-ended questions was that participants felt they received vague and oversimplified information, which lacked details for explaining the science behind why a developing fetus could be harmed by poor dietary control. One participant stated she had never been taught about maternal PKU saying, “I don’t even remember having the conversation.”

With regard to how the participants’ knowledge level may have impacted their family planning decisions, all of the participants reported that PKU strongly influenced their decision about whether or not to have children. Regis noted that one participant commented that she was afraid to have children because of the possible outcomes, while another participant who originally wanted a large family said that concerns over the PKU diet changed the number of children they would ultimately plan for.

“I never thought growing up that I could never had kids. I always had the big family in my head—five kids, four kids.”

Observations within the study also revealed that even though there were 12 pregnancies among the six participants, only four resulted in live births. So one begs to ask the question: If these six women were relatively well-informed about the risks associated with maternal PKU…AND if knowledge of those risks, as well as ways to minimize those risks, impacted the decisions these women made with regard to family planning, then what else could be the reason behind such a low pregnancy success rate?

Stay tuned for the concluding post in this three-part series on Caring for Maternal PKU. Assuming that education alone may not be enough to improve pregnancy success rates among adult PKU women, Part III will take a look at the support systems available to the participants in Regis’ study.

–NM

Caring for Maternal PKU: Part I

Caring for Maternal PKU: Part III

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