Friday, June 3, 2016

PKU Essentials

I thought that I'd share a page from out PKU Protein book.  I look at this book on a daily basis.  It is so easy to read and gives me everything I need to know about "regular" and "low protein" food.  The book is divided into sections:  Baby Foods, Fruits& Veggies, Free Foods (this section is AWEsome- it lists a bunch of food that has NO phe in it!), Very High Protein, and several more.  This book is essential to managing PKU.  Without it, PKU is like a guessing game.  You would have no idea just how much phe/protein you were taking it.  It also helps to break down recipes.
     For those of you who follow this blog, but don't really know anyone with PKU, this book might not mean that much.  But I take this book with me to the grocery store, to restaurants, and just about everywhere.  It reminds me just how many McDonald's french fries that Lily can eat and still stay within her daily intake level.  Just in case you're wondering~  a small order of fries(68g) is 88 phe.  You still need to weigh the fries to get an accurate exchange.  So my digital scale goes with us everywhere also!

     When figuring out the phe, I locate the brand in the binder.  Like I said, everything is in tabbed sections, which makes locating the food item pretty easy.  Unfortunately, there are quite a few things that are not in the book.  I have had to call Gerber and ask to speak to a dietition regarding some baby food items.  Gerber is really good about locating the information and calling back with results.  Not all food companies are this helpful.  
     After I find the food item, I look for the category "Mg Phe/Gm Food."  This tells me the information that I really need.  It tells me how much phe is in 1 gram of food.  I then weigh my food and use my handy dandy calculator.  If Lily doesn't eat all that I measured out, then I need to measure what is left over and figure out just how much phe she took in.  This is when my math skills come to play.  I really didn't believe my math teachers when they said someday I really will use algebra!  Boy were they right!
                                             25 gm of food  =  18gm of food      
                                                  6 phe                       X                 

     I would multiply 18 times 6, then divide by 25 to figure out how much phe was consumed.  Did I tell you how much I love my calculator?

No. of
Mg Phe/
Gm Food
Baking IngredientsFlour & Baking MixesNamasteCookie Mix1/24 pkg.23412.731.780.880
Crackers & SnacksSnacksPirate's BootyBaked Rice & Corn Puffs, Aged White Cheddar1 oz.281208.004.292.0130
Crackers & SnacksSnacksRoberts AmericanPirate's Booty, Caramel1 oz.28604.002.141.0120
Crackers & SnacksSnacksRoberts AmericanTings1 oz.281187.874.212.0150
Crackers & SnacksSnacksRoberts AmericanTubes, Veggie & Original1 oz.28644.272.292.0130
Crackers & SnacksSnacksRoberts AmericanVeggie Booty1 oz.28664.402.361.0130
Crackers & SnacksSnacksRoberts AmericanVeggie Booty, Barbecue1 oz.28755.002.682.0130
Crackers & SnacksSnacksRoberts AmericanVeggie Booty, Spinach & Kale1 oz.28664.402.361.0130
Crackers & SnacksSnacksRoberts AmericanVeggie Chips1 oz.28664.402.361.0120
Crackers & SnacksSnacksRoberts AmericanSimply Booty Vegan1 oz.28392.601.390.8120
You might enjoy reading:

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Tuesday, May 31, 2016

The Economic Side of PKU Part 2

There are not that many people who have PKU in the United States.  Approximately 14,500 people in the United States have PKU. Of that 14,500 people, 5,500 of them were not diagnosed at birth.  Those 5,500 were not started on a proper diet immediately and are now living in dependent group homes or mental institutions!  There's also an unknown number of people who had PKU but it was never diagnosed, because they were born well before the newborn testing.  Most of these undiagnosed people were just labeled "Mentally Retarded".
       Knowing this information helps to put the next part into perspective.  The other day I mentioned that having PKU is expensive for families.  Having to buy low protein food and formula is tough.  But it would be even harder for families and society to have to take care of a person who has not had their PKU treated. 
The following information can be found on the National PKU Alliance website.

      Even though PKU is expensive, it would be even more expensive to take care of PKU patients who did not maintain a low protein diet or drink their formula.  Let's think about my state, Illinois.  In Illinois, the PKU formula is provided to my family for free.  Yes, I know that it's paid somehow, probably through the taxes that you and I pay. (Thank you)  I'm sure right now, you're thinking- great another state run program that's helping some other family but not mine.  But please read on....

The annual cost of medical formula for PKU averages $7,100 per year, per person.  However, the cost of diagnosing and treating the PKU is extremely less expensive that treating someone who was not properly diagnosed.  Here's the break down.

Cost per infant for newborn screening (varies by state)                                            $50
        *Covered by insurance companies
Number of newborn screenings each year                                                             4 million
Annual cost of newborn screening for all                                                            $200 million

Annual cost per  person for medical formula                                                           $7,100
Number of Americans needing formula                                                                   20,000
Annual cost of formula for all Americans with PKU                                             $142 million
     *Not all states pay for the formulas

Annual cost of Nursing Assistant care in residential care center                       $50,000-$100,000
Number potentially requiring care because of non-treatment                                    20,000
Annual cost of managing disable patients treated too late                                       $1-2 billion

     Can you see that even though PKU is expensive, it could be so much more?  Imagine if there was no newborn screening or no insurance or government help.  The ideal annual cost is only $7,100 compared to the non treated cost of $50,000-$100,000.

     When I first saw these numbers, it blew my mind.  I know that I would never not treat my children, but guess what, there are some families that just do, for a variety of reasons.  Plus, there's countries that don't provide newborn screening!  So, now do you understand why newborn screening is essential?  Please use this information to be more aware of PKU and to urge your state to screen for more diseases and disorders.

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Sunday, May 29, 2016

The Economic Side of PKU

Having PKU is expensive for many reasons.  In order to control the PKU and not develop permanent brain damage, a person with PKU must maintain a low protein diet for life and drink their metabolic formula for life.  Depending on the state that a person lives in, determines just how expensive this is.  Currently, most states don't mandate that insurance companies cover the metabolic foods and formula that PKU people need to survive.  Now if you needed to control your diet for diabetes, insurance companies cover the insulin medicine that you would need.  BUT if you needed to control your diet for PKU, insurance companies would not cover the medicine (medical grade food & formula) that you would need.  Does that seem fair?
      Just to put things into perspective, here is a list of typical foods that a PKU person would eat daily and the cost of the food items compared to "regular" food items....

                        PKU Foods/Cost                                              "Regular" Foods/Cost                                  
                    Low Protein Linguine- $10                                           Linguine- $3
                  Low Protein Bread Loaf- $12                                     Loaf of Bread- $4
                Low Protein Pierogoi- $17.50                                    Box of Pierogi- $4
                       Baking Mix- $29                                                       Flour- $3
                Low Protein Brownie Mix- $12                                    Brownie Mix- $3
                   8 Low Protein Bagels- $12                                       Bag of Bagels- $4
       25 pieces of Low Protein Chocolate- $22                  Big bag of mini chocolate bars- $6

     Now there's a lot of "regular" foods that PKU people can eat, but the foods still need to weighed and have the protein/phe levels known- veggies, fruits, coconut milk, coconut yogurts, margarine, certain salad dressings, hard candies, etc.  Some people with PKU have a high phe tolerance and can put in a small amount of white bread and cow's milk into their daily diet.

     Lily has a low phe tolerance of 295 mg per day.  Thomas is currently at 50 mg per day, but he's still a baby and his number is expected to rise.  Since Lily has a low tolerance, we rely heavily on the low protein foods and fruits and vegetables.  Currently our insurance does not cover Lily's food.  However Illinois hospitals have a division for families to provide food at a discounted cost for qualifying families.  Not all states have this.  So imagine if you live in a state where your medical food isn't covered, how do you get by?  It can be really tough.

     Here in Illinois, our medical formula is provided for free.  Each month, I call the state office and order our formula.  It gets shipped from England, to New York and then to us!  I am extremely grateful that I live in a state that provides the formula.  Formula is not covered under insurance and is extremely expensive.  I can't go to a store and just pick something up.  PKU formula is medical grade and provides protein to help my children grow, but it doesn't have phe in it (the type of protein that my kids can't have).  If ordering the formula online, it costs anywhere from $67-$95 per can!  My kids go through 2 cans each week, each kid!  I'd be spending close to $300 each week on their formula.  Plus it's not like baby formula.  They need to drink their formula every day for life, in order to meet all of their dietary needs! 
     This is just one perspective of PKU economics.  We also take weekly blood draws and them sent to figure out the phe levels.  Plus our monthly drive to the clinic and cost of the appointment. 

     Please don't read this and think that I wish my kids didn't have PKU.  Honestly, sometimes I think about it and know that it would be easier that way.  But my kids are mine and are perfect just the way they are.  Their genetic disorder is part of them and has given us so much.  I'm beyond feeling the guilty part and hating the diagnosis.  I'm at the point where PKU is just part of our family, expensive or not!  Don't read this and think that I'm wanting your sympathy.  I'm just wanting to educate everyone about PKU, all aspects of it.  I know that having PKU children can be challenging, but there are so many other disorders and diseases out there that are much more difficult.  I'm grateful for the life that I've been given, and know that I'm right where I'm supposed to be!

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Thursday, May 26, 2016

Why Newborn Screening Is Important

 A month after Lily was born, we attended our first Illinois PKU Yearly Meeting.  It was a big deal.  The meeting was held at an extremely nice country club near Chicago.  I'd never been to a country club and I was impressed as we drove up the drive that was surrounded by perfectly manicured trees and shrubs.  It was almost like another world.  Ryan and I were introduced to quite a few people and I seemed to relax a little.  The other families were telling us how great their kids were doing on diet.  I also had the opportunity to meet a few adults living with PKU.  They had families, jobs, and were living a perfectly "normal" life. 
     Then the first speaker started his speech.  Within the first few minutes the speaker told us all of the terrible things that can happen to people with PKU- brain damage, mental impairment, ADHD symptoms, depression, etc.  My heart just sank.  I looked at Ryan and we both looked down at our precious Lily, only 3 weeks old.  That's when I just knew that those things were never going to happen to our little girl. 
     There are not that many people who have PKU in the United States.  Approximately 14,500 people in the United States have PKU. Of that 14,500 people, 5,500 of them were not diagnosed at birth.  Those 5,500 were not started on a proper diet immediately and are now living in dependent group homes or mental institutions!  There's also an unknown number of people who had PKU but it was never diagnosed, because they were born well before the newborn testing.  Most of these undiagnosed people were just labeled "Mentally Retarded". 

     It wasn't until 1958 that the first simple PKU test was created.  Robert Guthrie found a way to easily check the level of proteins through a simple blood drop.  It was much easier and less expensive than earlier methods.  Then in 1966, hospitals began testing all newborns with the Guthrie test at 24 hours after birth.  Finally there was a way to test for PKU and administer treatment immediately.  Remember, with an appropriate low protein diet, PKU can easily be managed and people can live a "normal" life.
     Newborn screening may have begun with PKU, but it's about so much more.  Eventually more disorders were added to the newborn screening.  Each year more than 4 million children in the United States are screened at birth for congenital diseases and disorders!  And of those babies, more than 5,000 are saved through early detection of their disorders and diseases. 
     So what exactly is tested in the newborn screening?  To be honest, most of them I had never heard of.  But I know that newborn screening saved the life of my Lily and Thomas.  Without the newborn screening, we would never have known that they had PKU and wouldn't have started them on a low protein diet.  They wouldn't have met their monthly development goals and would be labeled as having mental retardation.  Yes, mental retardation is the medical term that my children would have, if it wasn't for newborn screening.

     So, when your child comes back into your hospital room with a little band aid on their heal after 24 hours of birth.  Be thankful that newborn screening exists in our country.  Because there are still many countries around the world who do not offer newborn screening or have it as an option.  Newborn screening should never be an option.  It is essential in the livelihood of our future.  A simple heel prick and drop of blood on filter paper can change the future of every child.

     Here's the current listing of disorders that can be checked during newborn screening in the United States.  Please note that not all states check for all disorders!  Currently Illinois (my state) tests for 31 disorders during the newborn screening.  Most states only check for 10 of the top disorders.  The main reason- money!  Isn't money always the problem?  The machine that administers the tests costs around $400,000.  For a list of what your state tests for, please check out the March of Dimes website.  For a list of international newborn screening, please check the International Newborn Screening and Global Resource Center's website.

          *Amino Acid Metabolism Disorders:
               1.  Arginosuccinic Acidemia
               2.  Citrullinemia
               3.  Homocystinuria
               4.  Maple Syrup Urine Disease
               5.  Phenylketonuria (PKU)
               6.  Tyrosinemia Type I
          *Biotinidase Deficiency
          *Congenital Adrenal Hyperplasia
          *Cystic Fibrosis
          *Fatty Acid Metabolism Disorders
               1.  Carnitine Uptake Deficiency
               2.  Long-Chain L-3-Hydroxyacyl-CoA Dehydrogenase Deficiency
               3.  Medium-Chain Acyl-CoA Dehydrogenase (MCAD) Deficiency
               4.  Trifunctional Protein Deficiency
               5.  Very Long-Chain Acyl-CoA Dehydrogenase Deficiency (VLCAD)
          *Glucose-6-Phosphate Dehyrogenase Deficiency (G6PD)
          *Human Immunodefiiciency Disease (HIV)
          *Organic Acid Metabolism Disorders:
               1.  3-Hydroxy-3-Methylglutaric Aciduria (HMG)
               2.  3-Methylcrotonyl-CoA- Carboxylase Deficiency (3MCC)
               3.  Beta Ketothiolase Deficiency
               4.  Glutaric Acidemia Type I
               5.  Isovaleric Acidemia
               6.  Methylmalonic Acidemia
               7.  Multiple Carboxylase Deficiency (MCD)
               8.  Propionic Acidemia
          *Sicle Cell Disease and other hemoglobinopathy disorders and traits

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Wednesday, May 25, 2016

National PKU Month

     May is PKU awareness month and I've been wanting to write an entry about this for a couple of weeks now.  The problem is I just don't know what to write.  Yeah, I've got two kids with PKU and I don't know what to say.  Sounds pretty silly right?
     I mean, I obviously pay attention to PKU.  I have to weigh out their foods each day and prepare special meals.  But that's about it.  I don't research PKU and know what's the latest in treatments.  I don't participate in clinical trials.  I don't do public speaking  about PKU.  We currently just live with it.
     My dad asks me a lot about what's going on with PKU and research.  And I don't have a great answer.  I know that many people are using Kuvan and are having some success.  I know that the local trials of PegPal have been stopped.  But other than that, I know nothing else!  I do feel really guilty about my lack of understanding or maybe desire to research PKU.

     Maybe I don't pay close attention to it because I don't have to.  My kids have always had wonderful phe levels.  They've always taken their formula.  They love low protein foods.  They don't try to sneak other foods.  And our 3 year old knows how to advocate for herself.  I'm lucky and maybe that's part of the problem.
     I want to make better decisions for my children and that means that I need to become more of aware of what's happening in the PKU world.  I need to become more involved.  I need to be more than just a mother of PKU children, I need to be knowledgeable.
     I've starting taking some steps in this direction.  I recently started a meatless meal link up, hoping to find more PKU/low protein recipes.  The problem, only 1 person with PKU has added a recipe!  I have 3 cookbooks full of recipes, but I want to hear from others who are actually in the trenches.  I've also started a Northern Illinois PKU group and we're going to have a get together in a couple of weeks.  My fear is that me and the kids will be the only ones who show.
     I'm not loud.  I'm not an extrovert.  I'm not aggressive.  I'm just a simple, passive mother who wants to show her children (all 4 of them) that PKU is just something extra.  That there are many successful adults with PKU and that if on diet, anyone can be successful.  I'm hoping that by taking my small steps on the PKU path, that I may one day be able to look back and know that I did what I could for all of my children.
     I don't want to look back and wish that I'd been a better advocate for my children's special needs.  With that being said, I will do my best to learn more about PKU, the current research, potential treatments, etc and share them with my readers.  By being better educated myself, I am spreading PKU awareness this month and always.

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