One of the subject areas I left unresolved during my five-month hiatus was the results of my Kuvan responder trial. To be fair though, I had anticipated hearing at the end of the trial a clear yes or no as to whether or not I was considered a responder to the first PKU prescription drug. Instead, my phe levels were all over the place and a number of variables were suspected to have led to the erratic results. Needless to say, I needed to work through some of this confusion before reporting back to my blog followers. To sum it all up in one word, the results were inconclusive. Here’s a closer look at my blood test results:

For any readers who might need some additional context, I have been told that a person who does not have PKU will normally have a phe level of 0. If that same person just finished eating a meal that is high in protein, their phe level might register at a 1 or 2. However, once the food has been fully digested, the phe level will return to 0. Clearly, from these results, my phe level hasn’t dropped anywhere near safe levels.

In addition to blood tests, I was also asked to track mood or disposition changes I experienced throughout the trial. I will admit that after being on Kuvan for several days, I felt as though I might be slightly more relaxed and getting a better night’s sleep. However, assuming that because I knew this medicine might improve the way I feel, there’s a possibility that these mood changes could also be attributed to the “placebo effect”—the idea that a treatment appears to exhibit results simply because a patient believes it will work.

Ultimately, my clinic recommended I consider a second Kuvan responder trial; this time with more emphasis on controlling some of the variables that might be responsible for the inconclusive results. I haven’t rejected the idea of giving it another shot, but obviously with grad school still in progress, it will probably be several months before I do. During my next post, I’ll take a look at what some of those variables are and also discuss what changes might occur in a second Kuvan responder trial.

–NM

Their new product is called RESTORE and according to the medical foods company, it is the “first ever thirst-quenching metabolic formula specifically developed for patients with PKU.”

Sports drinks like Gatorade are not restricted for PKU patients (unless they contain nutrasweet or aspartame), but what makes this new product unique is its dual functionality: part super-charged formula, packed with 10 grams of PKU-friendly protein, and part hydration beverage that contains critical electrolytes commonly lost during prolonged exercise.

I requested a free sample from Cambrooke and tried the new product for myself. The lemon-lime flavor wasn’t too tart or harsh and the serving size was relatively large enough (16.9 fl oz) to quench your thirst after some pretty intense cardio.

Contrary to what has been said on the PKU ListServ, I did not experience any grittiness in my sample. Since I knew that the product’s instructions required you to shake before drinking, I continued to shake the beverage while I was drinking it, especially if I put the lid back on and walked away from it for a moment.

Despite any negative reviews the product has received, I believe PKU patients will really enjoy Cambrooke’s RESTORE and the convenience it brings to the low-protein diet. The sports drink does not require pre-measured water, blender bottles or any post-consumption dish washing activities. Just grab, shake, drink and toss—perfect for those individuals at the office, heading home from the gym, or even great for kids who don’t like to pack formula in their school lunch.

In the future, Cambrooke plans to release additional flavors of RESTORE. If you’d like more information about the product or would like to know how to request your own sample, visit Cambrooke’s website.

I give the product an A+ and will certainly be talking with my clinic about how to go about getting a supply.

–NM

Initially, I found it tricky to figure out a good method for capturing my daily protein intake. I explored the possibility of using Cambrooke’s DietWell app—a fabulous tool for the PKU community—but the more I experimented with it, the more I realized that the items that are truly high in protein are absent from its database.

So now that I’m nearly a week into my Kuvan responder trial, just how have I managed to keep track of my protein consumption? Well, here are the three “secrets” to my success:

1. Take advantage of publicly available information—Obviously, checking a food product’s nutrition label is a great place to start when keeping a food diary. Be sure that the first thing you look at is the serving size because many products you buy in the grocery store will contain multiple servings per container. Use a weighted food scale, measuring cups or measuring spoons to gauge you portions. For times when not eating at home, try to plan ahead. Many restaurants list nutritional information on their Websites, have apps you can download to your mobile device or sometimes you can even ask your server for a printed brochure of nutritional information.
2. Men’s Health—Whether it’s the New York Times Bestselling “Abs Diet” or the popular “Eat this, Not that” series, Men’s Health does an excellent job of providing all the information you ever possibly want on a food item. I especially like their recipes where we can cook healthy, in the comfort of our own home, and know exactly how much protein there is in a serving size. I’m sure other recipes, whether in a magazine or posted on a popular cooking website, will provide the same type of useful information. The Men’s Health product line has worked very well for me though.
3. iPhone Notepad App—Lastly, because I am getting my protein equivalent information from a variety of sources, I decided to use my iPhone’s notepad application to track my meals. Even though this notepad app came standard on my iPhone, it has the mobile convenience of the DietWell app, but the flexibility of a blank page. I’m able to enter whatever food product I ate, total the daily protein amount, and when I’m done, forward the finished document directly to my email.

–NM

It was just six or seven weeks ago that I announced plans to delay the Kuvan responder trial due to hectic personal and professional activities.  So what’s changed? Not a whole lot. Life is still hectic. I’m still working and going to grad school. But I think I’ve gained perspective. The trial won’t last forever and if I can just make it through the next two and half weeks, then we’re golden!

During the Kuvan responder trial, I am required to keep 6 daily food diaries, take 7 blood samples and consume 13 tablets of Kuvan each morning.

To help juggle all the pills I have to take, I went out a purchased pill box. This one—with its a.m. and p.m. compartments—is pretty convenient since I will take Kuvan in the morning and my allergy meds in the evening. Might seem kind of silly, but its organization and routine that is going to get me through this process. If you’re taking Kuvan and want to get one for yourself, I purchased this one from Walgreens for $9.99.

I’ll check back in frequently to let you know how it’s going. At the end of the whole experience, I’ll report any symptoms, note whether I feel any improvements in concentration or mood, and of course, let you know if I am a responder.

Wish me luck – ‘cause it’s on like Donkey Kong!!

–NM

When my husband and I recently attended the Network PKU Conference in Denver, Colo., there was a very interesting presentation given by Dr. Shawn Christ, director of the Clinical Neuropsychology Laboratory at the University of Missouri’s Brain Imaging Center. His research focuses on the brain and why conditions like PKU impact its structural and functional integrity.

After reminding attendees that hi-phe levels can build up and cause damage, Christ explained that he and his team have started using magnetic resonance imaging, or MRIs, to study impact to the brain. The equipment located at the Brain Imaging Center not only uses magnetic fields to generate images of the brain, but is also outfitted with hi-definition projectors and surround sound speakers for conducting visual and auditory memory experiments.

When used on PKU patients, MRI scans can capture any damage that might have been caused to the white brain matter, thus providing a glimpse into the structural integrity of the brain. What’s more though, Dr. Christ has begun looking at the functional integrity of the brain by asking research subjects to solve math problems while undergoing an MRI. Another test the team might use examines memory function by asking participants to watch a series of letters and then push a button when the letter they see is the same as what they saw two letters before.

Dr. Christ performed these tests with six PKU patients (who had elevated protein levels) and six non-PKU patients. He then looked to see what parts of the brain people with PKU are using differently than those without PKU. What Dr. Christ found was that PKU patients were activating specific areas of the brain more than the non-PKU participants. In other words, they were compensating.

Fascinating, huh? Well, it gets better. The research, as it turns out, can be applied beyond PKU. That’s because people with learning disabilities, autism and even schizophrenia will all benefit from research focused on brain structure and function. Here’s a video created by the MU News Bureau that further explains the possibilities:

Research from University of Missouri Brain Imaging Center May Lead to Treatment of a Variety of Mental Disorders from MU News Bureau on Vimeo.

In the future, Dr. Christ hopes to explore why it is that some PKU patients are more affected by hi-phe levels than others. He is also planning to team up with BioMarin to see what happens in the brain when phe levels are dropped using Kuvan treatments.

I’m certainly looking forward to hearing more about the ongoing research Dr. Christ and the other researchers are pursuing at the University of Missouri’s Brain Imaging Center!

–NM

What I’m trying to get at here is that last week, when I was supposed to begin my Kuvan responder trial, I started to feel a little overwhelmed. The thought of having to record every single thing I’m going to consume for the next three weeks does not appeal to me in the least. In addition to recording all foods, I also need to make sure that the amount of protein I consume one day does not vary drastically from the next. This takes some thought, some foresight and a whole lot of preparation. What’s more, because I’m currently off-diet, a lot of the foods I regularly eat, which are high in protein, are not included in the wonderful food lists, cook books, and software applications that are specially created for a low-protein diet.

To the rest of the PKU community, all this recording, measuring and weighing doesn’t sound that abnormal; however, with my life unfolding at break-neck speeds, I venture to guess that my non-PKU friends and family are wondering how I’ll even find the time to add this to my plate. The simple answer is you have to make it work, and, eventually, I will. Yet, for now I think there is some value in acknowledging the frustrations and hesitancy I have in getting started on the responder trial. Perhaps there are other off-diet adults considering Kuvan who can benefit from my ramblings, or maybe writing about the experience will turn out to be cathartic. Nonetheless, I intend to work through it and find some ways to make it more manageable.

One such way is that I believe it would be helpful to have access to equivalency data for foods that are high in protein. What I mean by that is this: where can I find information that tells me that the protein found in 4 ounces of chicken is equal to X-ounces of ground beef? I spoke with my clinic about my questions and I was directed to the U.S. Department of Agriculture’s (USDA) National Nutrient Database. Unfortunately, I found the site cumbersome to use, so I continued to conduct some online research. I eventually came upon a meat and protein chart maintained by Dr. J. D. Decuypere, a chiropractic physician with a nutrition background. According to her site, the doctor uses the same data provided by the USDA, but in my opinion, displays it in a more user-friendly format. My clinic also said that in general, 1 oz of meat is equal to 7 g of protein.

Hopefully, this information will help me feel more confident in tackling a responder trial. I’ll certainly continue to keep you posted. For those other off-diet PKU patients out there who took the Kuvan trial, how did you manage the process?  Did you figure out any tricks for making it easier? Share your thoughts in the comment field below. I’d love to hear from you!

–NM

Unfortunately, Kuvan doesn’t work for everyone, so before you can receive a prescription, you are required to go through trial to see if you actually respond to the medication. I will be starting my Kuvan trial this week and here’s how it breaks down:

• I should be receiving my package of Kuvan on Monday (that’s tomorrow)
• Then on Thursday, I’ll start keeping a very specific food diary (notepad icon). I’m not yet on the low-protein diet, but my clinic advised that as long as I consume close to the same amount of protein each day then we should be in good shape. Otherwise, it would be impossible to tell if Kuvan was working or if I just ate less protein.
• In addition to the food diary, I also have to take 7 blood samples (red circles). The blood work is pretty intensive in the beginning, but that’s to make sure we get a good baseline reading.
• Then about three days into the trial, I’ll start taking my Kuvan (yellow circles). The number of pills you take depends on your body weight. I was prescribed to take 13 pills. I need to take Kuvan at about the same time every day and with food since it is acidic and can cause stomach problems.

At the end of the trial, we should know whether I have responded to Kuvan or not. I have some concerns about possible side effects, some of which can be very serious, and I haven’t made any final decisions whether I will take Kuvan even if I am a responder. I am confident that I will not take Kuvan during any pregnancies. Nonetheless, the more information I have, then the better my decisions will be.

Stay tuned and I’ll let you know how it goes!

–NM

1/2 cup pineapple chuncks or juice; 1/3 cup ginger ale; 1/2 cup of ice cubes (not pictured; 1 packed of Camino pro GMP BetterMilk

After measuring out your ingredients, combine the first three in the blender, cover and process until smooth. Then add the packet of BetterMilk, pulse until it appears to be evenly mixed and voila, you’re done!!

Final review: Has the consistency of a tall glass of milk, but tastes closer to a virgin piña colada. Enjoy!!

Serving size: 364 g

Serving per recipe: 1

1/2 cup of strong coffee or espresso, cooled; 4 Tbs sugar or Splenda; 2 Tbs Nestle Nesquick chocolate syrup; 1/2 tsp vanilla extract; 1/4 tsp almond extract; 1 packet of Camino pro GMP BetterMilk; 2 cups of ice (not pictured)

The instructions said to “brew strong coffee by using twice the amount of grounds required by your coffee maker OR use twice the amount of instant coffee with boiling water.” In order to keep things simple, I decided to simply brew a little extra coffee in the morning, set a 1/2 cup aside in the fridge and then when I got home from work, it was already chilled.

Once you get your cold coffee in hand, combine the coffee, sugar, chocolate syrup, vanilla extract and almond extract in a blender. Process the ingredients on “low” for about one minute to dissolve the sugar. Then add the BetterMilk and ice. Blend on high until the drink has a smooth texture.

Final review: O.M.G It’s like Tiramisu in a glass! However, when the instructions said to “pour into a tall glass,” they weren’t kidding. I actually tried making this after already eating dinner and could not finish the entire beverage. This recipe might actually be a great option for a meal replacement – huge kudos to Cambrooke Foods’ food scientists!!

Serving size: 555 g

Serving per recipe: 1

1/3 banana; 1/4 medium kiwi, peeled; 1/4 cup fresh or frozen blueberries (I went with resealable frozen blueberries); 1/3 cup ice cubes (not pictured); 1 packet of Camino pro GMP BetterMilk; blender

Place the first four ingredients into the blender, cover and process until smooth. Add the packet of BetterMilk and use the “pulse” setting to blend the mixture evenly.

Final review: A very pleasantly sweet and fruity taste! I tried this recipe out at my parent’s house when we had gotten together for dinner and everyone had to try a sip. My dad was even inspired to try his hand at a non-low protein shake, but his didn’t have the same smoothie-like consistency. Definitely hit the spot on a hot summer afternoon!

Serving size: 273 g

Serving per recipe: 1