Category Archives: Formula

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.



Filed under Formula, Lo-pro Diet Management

PKU Letter Campaign to HHS Makes an Impact!

There’s some good news to report in the PKU community: the Health and Human Services letter writing campaign–initiated by the National PKU Alliance aimed at ensuring that every state’s essential health benefits plan provides coverage for medical foods–has made a difference. This article, originally published in Congressional Quarterly’s Healthbeat News on Dec. 21, 2012, provides more details about how those comments have been received.

 Patients Bombard HHS With Pleas on Essential Benefits Packages
By Jane Norman, CQ HealthBeat Associate Editor

Congressional Quarterly HealthBeatParents of children with rare diseases, advocates of acupuncture and other individuals are beginning to flood the Department of Health and Human Services with pleas and complaints about which specific services should be included in states’ essential health benefits packages.

Public comments on a proposed rule on essential health benefits aren’t due until Dec. 26, but more than 1,000 responses already have been received, according to the government website where the reactions are posted. Nearly all the comments so far are from individuals rather than organized groups or health business interests, though those groups also are starting to weigh in.

The letters from worried patients, doctors and family members across the country are an indication of the intense interest not just within the health industry but also among members of the general public – many with diseases or chronic conditions – in the outcome of decisions on benefit packages, which will vary from state to state and have a major impact on some people’s lives.

More than 11,000 comments had been filed in response to a bulletin issued on benefits before the rule was unveiled a month ago.

Some writing in are also asking for more time for comments to be gathered, saying that 30 days stretching over the holiday season isn’t enough time for people to digest a 119-page proposed rule and offer input on what should be changed. “Thirty days during the holiday is poor timing,” said an anonymous comment that suggested 90 days instead.

On Nov. 20, the Centers for Medicare and Medicaid Services released its proposed rule (See related story CQ HealthBeat, Nov. 20, 2012) establishing the essential health benefits required under the health care law (PL 111-148, PL 111-152). Non-grandfathered health plans offered inside and outside exchanges must, under the law, include benefits in 10 broad categories of services. State officials are supposed to pick a benchmark plan in their state as a model. HHS included some additional specific details on benefits in its proposed rule, but many commenters want more.

Don Hossler, who didn’t list an address, said in a comment that the federal government should have required that states get public input before naming their benchmark plans, including from groups that advocate for people with disabilities. “I realize there is a short time for a state to conduct meaningful public comment,” he said. “However, grass-roots organizations need to be allowed ‘at the table’ to enhance the decision based on consumer input.”

Dozens of people have written in already asking that coverage of medical foods be included for patients with PKU, a rare, inherited metabolic disorder in which the body can’t process the amino acid phenylalanine. Treatment must be started early in order to avoid damage to the body and involves a special diet, according to the National PKU Alliance.

Aleksandra Pisarek wrote that she is the mother of an 8-year-old boy with PKU who will require lifelong treatment to avoid brain damage. “Failing to ensure coverage for medical foods discriminates against people with PKU based on their disability,” she wrote.

The National PKU Alliance has urged members to write HHS, noting that the decision on medical foods may be left up to each state unless the government requires coverage of medical foods as part of the essential benefits category that covers chronic disease management. “Representatives from the NPKUA recently met with HHS on this issue, and HHS urged us to have as many people as possible submit comments on the new regulations,” the alliance’s website states.

Another group whose members sent many letters to HHS are those who receive or administer acupuncture treatment. Patricia Solomon, a Massachusetts pediatrician, said that “some of my most aware and careful parents seek acupuncture” and other alternative health treatments for their children. She said she was dismayed to see no alternative treatments included in the standards outlined for benefits. “Their value as adjuncts to traditional medicine is becoming clearer,” Solomon said.

Tabitha Silver of New York wrote, “Acupuncture has changed my life. It was the only thing that helped me with depression and pain.”

Stephen Scholle, the clinical director of a nonprofit that serves mental health consumers in Westchester and Rockland counties in New York, said his group provides acupuncture and should be included in essential health benefits, perhaps as part of wellness and preventive care. It helps with stress relief, pain relief and mental well-being, Scholle wrote.

Another commenter, Terri Bukacheski, said she is concerned that rehabilitative services and devices that are required to be provided in one essential benefits category are not well-defined. “Patients that require prosthetic limbs and orthopedic braces require these custom services for a lifetime, and the care must be provided by highly skilled experts,” she wrote. Devices should be clearly defined to include prosthetic and orthotic services, she added.

The time period for submitting comments in response to the “Patient Protection and Affordable Care Act: Standards Related to Essential Health Benefits, Actuarial Value, and Accreditation” rule has passed; however, visit the National PKU Alliance website for more information on how to support future advocacy efforts.



Filed under Advocacy, Formula

Billing PKU Formula According To Calories

Here’s something you may not know when it comes to the business of distributing and paying for PKU formula.

After trying the free sample of RESTORE LITE provided to me by Cambrooke Foods, I immediately connected with my clinic to have it added to my formula prescription. Apria Healthcare, which is the medical supply company that processes and ships my formula, called and told me, “We do not carry RESTORE LITE. We carry the regular RESTORE, but not the LITE.” After reporting this news back to my clinic, we decided to try Edgepark, a competitor of Apria Healthcare. What was Edgepark’s response? Not only does Edgepark not carry RESTORE LITE, they cannot carry the LITE because Apria, EdgePark and other “durable medical equipment” companies are reimbursed according to the number of calories in the formula.

PKU formula, Phenylketonuria formula, low-protein diet

The revelation that durable medical equipment companies are reimbursed according to the number of calories in PKU formula provides a whole new meaning to the phrase, “Spend your calories wisely!”

Call me crazy, but billing insurance carriers based on caloric value seems ridiculous, so while my dietician continues to search for an alternative medical supply company from which I can order my preferred formula, I decided to investigate this a little further.

A basic keyword Internet search turned up a 56-page document created by Abbott Nutrition and titled “Medicare Part B Enteral Nutrition Reimbursement Manual, 9th Edition.” Abbott Nutrition, which creates powdered PKU formulas such as Phenex-1 and Phenex-2, created this manual as a way to guide health care suppliers through the process of billing the U.S. federal health insurance program called Medicare. Here’s how the process is explained on page 9 of the manual:

Enteral nutrition formula is billed in “units.” A unit is defined as 100 Calories.

  • Calculation of units: Cal per day ÷ 100 = units per day
  • e.g., 6 cans/day  x 250 Cal/can = 1500 Cal/day ÷ 100 = 15 units/day
  • 15 units per day x 30 days per month = 450 units per month

Sound all Greek to you? Well, it does to me too. It appears that billing formula based on calories is a government-mandated method, but why (if I am not on Medicare) does it apply to me? Do durable medical equipment supply companies decide to apply this method across the board since they are already required to do so for Medicare patients? And what does that mean on a personal level? Do PKU patients now have to decide whether eating healthier is worth what is likely a significant increase in cost?

This way of thinking–whether it originates with the U.S. Government or with the medical supply companies–sounds all too familiar. For years, PKU patients have struggled with medical foods coverage based in large part on the incorrect assumption that a low-protein diet is cosmetic and only for achieving weight-loss.

Despite calorie count, medical formula is medical formula. Period.

If anyone from Cambrooke and/or Abbott Nutrition are reading this blog, I’d love to hear another perspective on this formula conundrum in the comment field below. Hopefully someone can explain why the 100-calorie difference you see between regular RESTORE and RESTORE LITE ultimately determines whether a patient in need receives their medical formula.



Filed under Advocacy, Formula

Remember to Take Kuvan, PKU Formula with RxmindMe

RxmindMe IconIt is probably safe to assume that most, if not all, PKU patients do not want their genetic disorder to define who they are as a person. Instead, we attend school, hold down jobs, and engage in hobbies just like people without PKU. There is no denying, however, that PKU patients must juggle these activities while also managing their low-protein diets. Well, as the popular saying goes, “there’s an app for that!”

The app, which is called RxmindMe, is available for free on iTunes and includes several different types of reminders, including:

  • Every “x” number of days
  • Hourly
  • Daily
  • Weekly
  • On specific days (like say the 15th of every month)
  • Spontaneous (recording medication that was taken as needed as opposed to scheduled)
  • Prescription quantity (to remind you when it is time to order a refill)

Users can also export their prescription data into Excel or email their prescription history directly from their phone. This seems like an awesome feature for when you find yourself in a doctor’s office and have to list all the current medications you are taking. It is also nice to know that all data within RxmindMe is connected to the U.S. Food and Drug Administration’s database so that you can search for your drug(s) and perhaps investigate possible side effects or hazardous drug interactions.

RxmindMe, Kuvan Reminder, PKU ReminderPKU patients can use RxmindMe to enter reminders for taking Kuvan, formula or even non-PKU meds like allergy pills, antacids and contraception. There’s no limit to how many reminders you can enter. For those parents with PKU kids, getting them involved with creating the reminders on the iPhone or iPad might even encourage them to be more independent in their treatment.

Finally, for those of you into social media, follow RXmindMe on Twitter—their Twitter handle, or name, is @RxmindMe—so you can get updates and tips for using the application.



Filed under Formula, Kuvan, Lo-pro Diet Management, Technology

Cambrooke’s RESTORE Brings Convenience to the PKU Diet

Cambrooke Foods, RESTORE, PKU, PKU formulaTo the untrained eye, this photo may seem like a magazine advertisement for Gatorade, but look a little closer and you’ll see that the innovative food scientists at Cambrooke Foods have out-done themselves again!

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.



Filed under Formula, Product Reviews

BetterMilk Challenge Recipe 4: BetterMilk Island Smoothie

For my final recipe on Cambrooke Foods’ Three-Day BetterMilk Challenge, I tried the BetterMilk Island Smoothie. While all of the BetterMilk recipes were relatively easy to make, this one was by far the easiest of all. Part of the reason why the BetterMilk Island Smoothie is so simplistic in nature is that the ingredients list is really not that long. Here, have a look:

Cambrooke Foods BetterMilk Island Smoothie

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!!

Cambrooke Foods BetterMilk Island SmoothieFinal 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

Measurement Per Serving Per Recipe
PHE: 34 mg 34 mg
LEU: 3118 mg 3118 mg
Pro: 0.5 g 0.5 g
P.E.: 15 g 15 g
Calories 364 364


Filed under Formula, Recipes

BetterMilk Challenge Recipe 3: BetterMilk Mochalatte

Before I even tried my third BetterMilk recipe on the Three-Day BetterMilk Challenge, I already knew I would love it!  That’s because the first ingredient—coffee—is one of my favorite pick-me-ups when I get up and go to work every morning. The recipe is called the BetterMilk Mochalatte and here are the ingredients you’ll need to get started:

Cambrooke Foods BetterMilk Mochalatte

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.

Cambrooke Foods BetterMilk MochalatteFinal 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

Measurement Per Serving Per Recipe
PHE: 51 mg 51 mg
LEU: 3140 mg 3140 mg
Pro: 0.5 g 0.5 g
P.E.: 15 g 15 g
Calories 400 400

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Filed under Formula, Recipes