PKU Awareness Day 2014

This is my first PKU Awareness Day as a mother with 2 children with PKU.  Now it is doubly important for me to raise awareness, share, and remind the people in our lives and the people we will encounter on our paths in the future, what PKU is and how we live with it.  I want everyone who will be involved in the lives of my children to understand this condition, what it means, how it affects their lives, and how they can always rise above it.

PKU is not a disease, it is a condition, a rare genetic metabolic condition, which means it is inherited from parents, who usually don't even know they carry the gene, and metabolic means that it affects how the body breaks down foods.  People with PKU have a missing or malfunctioning enzyme in their livers that is supposed to digest phenylalanine (which we call "phe" for short), which is one of the 20 amino acids in protein.  If a PKU person eats high protein, like a hamburger, their digestive system breaks down all the protein except that one part, the phe.  Since the phe isn't digested, it gets into the blood, and too much of it builds up in the bloodstream.  When it gets into the brain, it is toxic and causes damage.  If this happens for a long period of time in a baby or a young child, whose brain is still growing and developing, it can cause serious mental disability and delays.  If it gets too high in an adult, whose brain has already grown, it can cause them to feel depressed, very sleepy, irritable, and have trouble concentrating, even have headaches.  The way to keep from having too many phes is to always eat a diet that is low protein.  Since nearly all foods have at least a little protein and phe, everything needs to be measured or weighed to count the phe.  The doctor and dietician look at blood tests and weight and figure out how much phe the body needs and give a phe prescription, or a limit to how much phe can be eaten each day.  People with PKU use a book or a website to find out how much phe is in foods.  They keep a journal of what and how much they eat to make sure they don't go over their phe limit each day.  Weekly or monthly they have to send in a blood sample, by sticking their finger, so the doctor can see how much phe is in their blood and that they are getting the right amount.  Because high protein foods, like meat, dairy, and soy, are too high for people with PKU to eat, they have to drink a special formula, or special milk, that is made with all the protein they need without the phe in it.  They have to drink this formula every day in order to grow and stay healthy.  People are diagnosed with PKU just a few days after they are born.  The hospital does a heel stick, called Newborn Screening, that checks babies' blood for all kinds of different diseases and conditions, one of which is PKU.  Since everyone in the U.S. is tested at birth, treatment is started right away, and as long as the diet and treatment are followed, PKU causes no problems at all.  The "only" thing a person with PKU has to worry about is what they eat and drink each day. 

It sounds easy, and probably is compared to some things, but in reality it does take a lot of work, planning and preparing.  And explaining.  And I am sure when the kids are older, it will be emotional for them to deal with at times in their lives.  As a parent of small children with PKU, I do a lot of work right now.  I read all the time, whether it is PKU information and research, cookbooks and recipes, or stories of other PKU families.  I do a lot of cooking and preparing.  I mix two different formulas every day, one for Carson, who is 5, and one for Allison, who is only 5 months old.  They use different formulas right now since they are so different in size and their needs are different.  I make a loaf of homemade bread every week or two for Carson to take sandwiches to school.  I sometimes make him a homemade snack to take to school, like chocolate chip cookies or brownies (low protein, of course).  I keep emergency foods made up in the freezer, like low protein cupcakes to take to parties.  I make Carson a separate supper every night, sometimes just one part of the meal, like I have to make him separate pasta noodles, but we can eat the same sauces (we like pesto or spaghetti sauce).  I like to keep little notebooks to calculate their intake, and it is about to get interesting because Allison is just now starting to eat baby food (eek!).  I do a lot of math, weighing and measuring food, and calculating phe intake.  I do a lot of planning, like deciding what Carson will eat when we go to a family dinner, or church, or a friend's house, or a restaurant.

It sounds like a lot, but I am not complaining at all.  I have been doing this for 5 years now so I am already used to it.  It is like second nature to me.  The only time I freak out is if I forget about something and Carson gets upset, but even then we have emergency plans, like go get some tater tots from Sonic, or a banana, or a Rice Krispie treat, or some kind of cereal he likes.  There are regular foods he can eat too, and he eats those quite a bit.  He eats Cap'n Crunch or Chocolate Cheerios for breakfast.  And yes my work is about to be doubled, but it will happen slowly and I will be able to ease into it, so it should be no big deal.  I only share all this so maybe those of you reading it will come to an understanding.  If I ever seem spacey or in another world, I may just be thinking about what needs to be done.  Maybe I am planning some recipe to look up or try for Carson because I saw something that gave me an idea.  Like the other day he wanted cheese dip, and he has never had cheese dip before, so I was thinking why he suddenly asked that and how I can make him some.  I may be thinking in my head what I need to buy for this recipe and how much phe will it have and I need to add to my grocery list and I need to look that up in the book and see how much phe is in that...


Now I am wondering what Allison will be like when she gets older.  Will she be as easy to please with the diet as Carson is?  He is partial to certain foods and I know what he will eat, but he is usually willing to try new things too.  He is pretty easy to plan for because he is routine-driven and pretty predictable, and he doesn't ever try to eat off anyone's plate.  But will she?  Will Allison be like Maggie and only want what is on my plate?  Will I have to put her food on my plate just so she will eat it?  Will she cry for something someone else has?  Will I have to make more of a variety of foods that match regular foods for her?  Or will Carson have an influence on her?  Will she be easy-going or dramatic?  While it seems stressful to wonder and worry, it is fun to think about too.  I look forward to trying new recipes and make it an adventure!

The best thing to do for my children right now is to have a positive attitude.  Not just myself, but everyone around us.  Carson and Allison are rare and pretty special, if I must say so.  I want them to always know that and to have a positive attitude about their diet.  We try to keep it low and not make a big fuss about it.  It is what it is.  They eat something different than we do.  Period.  We get our protein from high protein foods, they get it from their special milk.  I want to teach them all about it, how to make and measure and calculate and order all these foods for themselves, so when they grow up they will have all the knowledge they need to stay on their diet.  I want to motivate them and help them to remember how important it is to stay on their diet.  That is why I want to always stay positive, even saying they "don't" eat that instead of they "can't".  If I continue to do everything in my power, then I have done my job as a mother to equip them and just hope and pray they can handle it when the time comes for them to!

This year, I am thankful for so many things when it comes to PKU.  I am always thankful for the Newborn Screening diagnosing them early and for the nutritionists who are our lifeline at the clinic.  Even though it is devastating to find out that Allison has PKU too, I am thankful that I know how to handle it, and that Carson is not alone, nor is Allison.  They have each other for an inner support system now and hopefully for life.  I am so grateful for the wide and growing support system of other families who live with PKU, especially moms like myself.  I love to read and interact with others who understand and "get it".  We have a few facebook pages and websites where we can converse.  I am most thankful for the wonderful family and friends that I have who are so positive and supportive of us and the diet.  They help, they learn, they teach, they support, they ask questions, they make all the difference in the world.  I love them all!  We are so blessed!

Welcome to Our World

On Tuesday, June 17, 2014, our newest bundle of joy, Allison Lynne Rice, surprised us all by deciding to make her grand entrance into the world.  I dedicate the first section of this post to my labor story, so if you don't want to read about that, skip on down to the next section.

One of the best pictures of my pregnant belly,
on Mother's Day, about a month before
Allison was born.

Allison's Birthday

People will say to pregnant women all the time things like, "You don't look like you are going to make it another 3 weeks" or "You look like you are going to pop anytime", but I learned after having Maggie that it was just conversation, or wishful thinking.  Since Carson came 12 days early, I figured Maggie would too.  I was so miserable, swelling, tired, anxious, and so I decided to go ahead and take my maternity leave from work early.  Only Maggie had other plans.  Week after week, I went for checkups, dilated 3cm and having contractions, but never progressing.  At my final checkup, I made the difficult decision to be induced.  Maggie was due March 19, but we scheduled her to arrive March 16th, for many good reasons.  I found peace in my decision when God decided to let me go into labor that morning before we left for the hospital!  When I arrived, the nurse made the comment, "Looks like you started without us."  For that, I was thankful.

This time was a little different.  I did well, did not gain weight as quickly, and had a very similar pregnancy to that with Carson.  However, toward the end I got very miserable again.  No contractions, just swelling and tiredness.  I assumed we would schedule an induction like before.  Dr. Franklin was ready to oblige at my next checkup.  Allison was due June 29th, so we probably would have done it that week.  Everyone else in our lives wanted her to come on June 16th, so that all 3 of our children would have birthdates on the 16th.  Yes, it would have been cool.  But I was convinced she wasn't coming early, since I got burned with that last time.

June 16th passed and there were no contractions.  No signs of labor.  Nothing.  Since I had a checkup the next day, I wanted to go ahead and get the Pack n' Play ready to be used as a bed for the baby, just in case.  We spent that night cleaning it, putting it together, vacuuming, cleaning baseboards, etc.  June 17th was set to be an ordinary, run-of-the-mill day.  I had an appointment with Dr. Franklin in Cullman at 10:00.   I was so convinced this birth would be planned that I had not even finished packing bags for the kids, including food for Carson, but had most things in our hospital bag packed.  At 5:30am, the alarm went off for Marty to get up and ready for work.  I rolled over and tried to go back to sleep.  But I couldn't.  I was hurting, cramping, even.  Were these contractions?  I had not really had any contractions with this pregnancy like I did with Mags.  I grabbed my cell phone and starting timing these cramps.  I told Marty I was hurting and didn't feel well, and that I was timing them to see if they were contractions. 

It was now 6am and he was ready to leave for work.  I begged him, to the point of tears, not to leave me.  I was scared I would need to go to the hospital.  He works in Decatur and would be so far away from Birmingham!  I wanted him to go with me to my checkup.  These were contractions, but slightly irregular, and in a different area of the body than I remembered previously.  I was strangely unsure how long it would be if this was indeed labor.  You think you would know the third time around!  I tried getting up and moving around.  I decided to take a bath and start getting things ready just in case.  Marty chose to stay with me.  I was in so much pain in the bath I couldn't even shave my legs (horror!!!)!  Nothing relieved the pain.  The contractions got more regular and much stronger.  Realizing this was probably "it", we called my mom to come watch the kids.  I ran around the house like a crazy woman, throwing things into suitcases, stopping every 6 to 7 minutes to bend over and brace myself for the painful contractions.  This was happening fast!!!  At 8:00am I called the hospital, and they told me to make my way down and they would be expecting me.  I still wanted to check with Dr. Franklin first, so when I finally got hold of her office, she wanted me to come by.  Cullman is totally out of my way to Birmingham, but I was still in some denial and thought that this could be false labor.  Once we made it to Cullman, I was so miserable, and yet I still had to wait!  I finally told a nurse, "Were you not told I was here due to labor contractions?!" When I eventually made it back and Dr. Franklin checked me, I was dilated 3cm (up from 1cm), and she told us to head on down to the hospital.  It was bittersweet because I knew she would not be delivering this baby. 

Once we made it to the hospital, I was in so much pain I could barely even sign my name, so Marty had to finish signing us in.  Our delivery nurse, Diane, was awesome, such a hoot and very caring.  We were so blessed to have her.  She said Dr. Franklin had called down there about me and told them I was "a live one".  I probably got in that bed and got everything hooked up and started around 11:20am.  Sometime around the 1:00 hour, Diane decided she was going to grab her pb&j for a quick lunch while we waited.  I already had my epidural and I was just hanging out (still extremely uncomfortable).  Then suddenly it happened.  That gush of fluid I have always heard about.  My water broke on its own!  Never happened in any other pregnancy.  And since it was not planned, it went everywhere.  Gross.  I looked over at Marty, and possibly yelled, "My water just broke!  Go get somebody!!!"  So the expectant dad did his duty and opened the door, since we were right in front of the nurses' station, and told someone at the desk.  They went to interrupt poor Diane's 5 minute lunch.

When Diane entered the room and we began discussing the breaking of water, she looked down and said, "Umm...are these your flip flops?  I think they got caught in the crossfire."  Smiling, she picked them up and said, "I think we'll just put them over here."  And we all had a big laugh.  Yuck.

My favorite delivery picture EVER,
because I took it myself!

At 2:14pm, Allison made her very quick entrance into our world.  I had to push longer than with Maggie, but it still happened very quickly.  As Marty started to cut the cord, I realized there was no one left to take a picture, since my mom was keeping Maggie this time around, so I said, "Oh, just hand me the camera, I'll take it!"  The doctor and all the nurses started laughing about how I was trying to be "Supermom" because I am laying on this bed with a freshly delivered baby and I am taking my own pictures.  What else did I have to do?  It was not a big deal.  More laughs ensued.

Allison Lynne weighed 7 pounds and 7 ounces and was 20 inches long.  She weighed exactly one pound less than Maggie at birth, and half a pound more than Carson.  She was beautiful and a picture of health.  Everyone was all smiles.

The Call

The only thing that could dampen the cheerfulness of having a new baby was the looming question:  Did she or didn't she?  Have PKU, that is.  The odds are in favor of not having it, 75%, to be exact.  But 25% possible she could be like big brother.

The way babies are typically diagnosed with PKU is through the newborn screening program, when all baby's have their heel stuck the day after they are born, and which yields results after about a week.  Including the retest, it was 10 days with Carson.  Since we already know we carry that gene, we make a stop over at Children's Hospital after we are released from our maternity/post partum stay.  That way, we can get the results the next day or two, just a few days after birth.  It is helpful to know as soon as possible in case the treatment diet needs to be started.

With Maggie, Alicia from our clinic made the call, giving me the happy news that she was PKU-free.
This time, Alicia made the call again.  I was sitting on the couch, home alone with baby Allison.  Marty and Allen had taken my two big kids to Dodge City for haircuts.  Of all times, I had to be alone!  It was indeed "The Call"; Allison's blood phe was over 7, so she did indeed have PKU.

I tried not to cry, but of course ended up sobbing.  It wasn't like Alicia had never seen or heard me cry before.  But I still get embarrassed.  I debated whether I should wait until Marty got home or call him right away.  The decision I made was based on Allison's need for immediate treatment; the sooner, the better.  I needed Marty to go to UAB and pick up the special protein and non-protein formulas for Allison to start that day.  It was very difficult to handle.



That afternoon, after Marty had returned from UAB,
we stood out on the front porch talking, and this
appeared in the sky.  God was reminding me of His promises,
assuring me that everything was going to be just fine.

How to Deal

No matter how many times you say, "At least you know what to do", it doesn't make it better.  No one ever wants to hear there is something, anything at all, wrong with their child.  Allison and Carson are both beautiful and very healthy children, but if they want to stay that way and be at their best, they are sentenced to a lifetime of a very strict diet, and all that comes with it.  It might be a difficult road ahead for them, as they grow up and try to become independent and take care of the diet themselves, and battle peer pressure.  I hope to raise them both to be strong and disciplined and not feel like it is a burden.  Turn your burdens into blessings.  Be a blessing to someone else.

One thing is for sure:  I am so glad they have each other.  I am so thankful that Carson is not alone in the family anymore.  I am grateful he has a friend in Noah, who will be someone his own age and gender he can relate to.  But I am also glad that he and Allison can share a special bond at home.  They might even take care of each other when they get older, which would be a huge relief for me.  I sure hope it plays out that way.

I am also blessed to have a friend in Jackee, who I can talk to about raising two children with PKU, an older boy and a younger girl as well!  And I am especially thankful for Noah's mom, Julie, who has become such a dear friend and supporter whom I could not live without.  God could not have orchestrated these happenings any more perfectly than He did.  We are surrounded by friends and family, loved ones who care and show empathy and compassion, who are willing to help out as well as be an encouragement.  I have never been so happy.  I have a blessed life.

Allison, welcome to our world.  Our crazy circus, our wild ride, our PKU Life, our VERY BLESSED, HAPPY LIFE.  Praise God!

A Day in the Life with PKU: Blood Draws

A vital part of managing the PKU diet treatment is testing the blood to find out phenylalanine levels.  According to our clinic at UAB, the acceptable normal range for blood phe is between 2 and 6 (don't ask me to get technical, let's just look at the numbers! lol).  The first blood draw, through the Newborn Screening Program, done in the hospital after Carson was born, the level was 5.8.  For a newborn, that raises a red flag.  I believe it should have been closer to 1 (I think Maggie's was either 1 or lower when she was born, and she does not have PKU).  We got the call from our pediatrician about that level about a week after Carson was born.  The very next day, we were told to take him to Children's Hospital for more blood work.  The doctor even said it might have just been a fluke because it was so rare.  When the results came back from Children's, we were told to go to the genetics clinic at UAB.  Carson's blood phe level was 29!  He was indeed diagnosed with classic PKU at 10 days old.

From that day forward, we began this journey of diet plans and phe calculations and blood work.  I was still working full time when Carson was a baby, so I decided to take him to the doctor's office for the first months of his life and let them draw his blood for me.  It was free and they did all the work!  I am so glad I let them prick his heel every week; thankful I did not have to do it.  Whenever he was around 1 year old, really before, because he was so big and his heels got so thick, they said it was time to stick his finger.  After our genetics clinic visit, I got a prescription for lancets and got brave enough to start pricking Carson's finger at home.  He does great and I have no problem with him!  Unfortunately, I am not the world' greatest nurse, and sometimes I don't hit the right spot for good blood flow and have to stick him a few times.  That part is not fun.  But Carson is such a big boy and doesn't put up a fuss!

The process is different from that of a diabetic testing their sugar.  At this time we only have to do a finger stick every 2 weeks.  Unfortunately, an at-home test for phe levels for PKU has not yet been perfected, so we have to put the blood on a lab slip, the same lab slip used in the hospital for newborns (our clinic provides these to us), and then we mail it to the State Department of Health.  They process the blood and notify UAB genetics clinic with the results.  The dietician from the clinic in turn calls me to report the numbers.  Carson's last level was 4.9, I think, which is considered in normal range, but I feel his behavior is better when it is lower.  I just mailed another sample last week, but since today is a State holiday (yes, it is Jefferson Davis's Birthday) I probably will not hear the results until tomorrow, I hope.  If his level comes back over 6, then the nutritionist instructs us to do what is called a washout, where we feed Carson as little phe as we can for a day or two, then go back to normal diet for a few days and send another blood sample to see if it came down.  I can usually tell if Carson's blood phe level is high, because it makes him sleepier and crankier.  His behavior seems to be better when his phe is in the lower range.  Usually the only times it has been high was when he was younger and teething, or now especially when he is sick.  Fighting off infections or viruses causes a change in metabolism, which in turn affects the way his body processes and rids itself of phe.


This is Carson's blood drawing kit.  I keep cotton, Band-Aids, lancets,
and the lab slips all in one place to make it quick and easy.  I did not
get a picture of the actual act because I did this one while he was asleep!
If you notice the lab slip above, the blood has to drop in these
little circles on the special lab paper and then dry for several hours.
Then we mail it in the envelope provided.
Carson calls it "painting circles".  I am trying to teach him now that
we have to send it to the doctor to "count his phes".

We are so fortunate right now that Carson does so well with his diet.  He does sometimes ask for other foods and get curious, but he handles it very well.  I try to offer him a low protein alternative for everything.  If he asks for ice cream, for instance, I remind him he doesn't even like ice cream!  I have tried many different kinds of low protein ice cream, and he just won't eat them.  It is not the taste; I believe it is the cold he does not like due to his sensory perception issues (related to his autism).  I hope as he continues to grow he will learn all he needs to know about his diet and that he will handle it with strength and grace.  With his love for controlling situations and having routines, I think he is a great candidate for continuing a successful diet for life.

A Day in the Life with PKU: Phe Calculation: Following the Diet Prescription

For those of you who know little to nothing about PKU, let me explain a little bit here.  Phenylketonuria is a rare genetic (inherited) metabolic trait in which a gene mutation or deformity causes a specific liver enzyme not to be able to process phenylalanine ("phe" for short), which is one of the amino acids found in protein.  In other words, Carson's body cannot process part of protein, so it stays in his blood stream, which is dangerous for his brain if it is too much.  This is tested for in all newborn babies in most states through the Newborn Screening Program, which is why they stick babies' heels in the hospital after they are born.  It is just one of several disorders they test for.  The importance of finding it so early is that treatment is best started right away to avoid brain damage caused by high levels of phe in the brain.  The treatment is a low protein diet, preferably for life.  Phe levels are tested in the blood regularly to maintain levels within an acceptable range.

Once phe levels and phe tolerance are established, a nutritionist and physician specialist work together to determine a patient's phe prescription, or the amount of phe the body can handle and stay within the normal range.  As babies and through the toddler years, children grow so rapidly that this diet is monitored and changed frequently.  Once growth slows down, it is easier to keep a steady prescription.  Carson is currently allowed 350mg phe per day, and has been on this Rx for over a year, I think.

So how do we figure out how much phe he gets in his diet?  Protein is a listed ingredient on food nutrition labels, but phe is not!  Phe is just one part of protein!  Well, we have this wonderfully fabulous book I often refer to as the "Food Bible", or Carson's Phe Book, called the Low Protein Food List.  It was put together by a remarkable woman  named Virginia Schuett, who has done SO MUCH for the PKU community over the years.  She worked as a nutritionist at a PKU clinic in Wisconsin and founded the National PKU News Organization (www.pkunews.org).  Thanks to the tremendously hard work of her and her team, we have this awesome resource for keeping track of Carson's diet!

Inside, this awesome book contains the phenylalanine content of almost any food you can imagine.  I scour through it from time to time searching for new foods that Carson can try.  By now I have so many things memorized that Carson has been eating for a few years, I don't even open the book as much anymore.  I had to look up lettuce a few weeks ago when he started eating salad.  I plan on taking the book with me next time I go grocery shopping so I can check out some of the new gluten-free products to see if there are any he can try.  Below is a sample of a page from the book showing how the nutritional and phe content is listed for food items, plus a sample from Carson's notebook.  Each day (when I don't slack) I write a page in his "Food Diary" detailing what he ate, how much of it, and the phe content.  Then I add it up for the day and try, as we go, to make sure it is as close to 350mg as we can get.  Some food items have a specific measurement listed, like 1/2 cup, some I have to measure by the gram and weigh them on our digital kitchen gram scale.  Who ever said you would never use math in real life?  Trust me kids, math is my life now!

First it lists the brand, if there is one; then the name of the food, next the measurement of a given portion size (like 1/2 cup), then the weight in grams of that portion, and the last thing I look at in the first gray column is the mg phe per that portion size.

See how I keep a running tab of mg of phe
to the side?  This helps me know what
Carson can eat next the rest of the day. 
For example, 1/2 cup of rice is 90mg phe, so he would not have been able to fit that in without going way over 350mg.

I do most of it in my head now, or refer back to previous pages and copy the figures.  I also have learned how to "eyeball" a lot of measurements, like today, when we ate at O'Charley's, Carson ate broccoli, and it was a much larger portion than we usually get.  I estimate, after weighing several portions at different restaurants, we usually get 50 to 60 grams of broccoli; today looked more like 80g!  Then I would calculate, for example, (I still use the amount from the previous book version), since there is 0.83mg phe per gram of broccoli, that 80g x 0.83mg=66mg phe.  It is amazing how quickly you learn to adapt to things like this.  It has just become like second nature to me, and for that I am so thankful.  I am trying to start teaching Carson to count certain things that he eats, like potato chips, for example.  I have figured, since in the book it says 95mg for  1oz of most brands of chips, and say Pringles are about 14 chips per 1oz serving, that 1 Pringle chip has 6mg phe.  So I just count the number of chips he eats instead of weighing them on a scale (this is why I like chips that are more uniform in size and shape! lol).

 I hope I am able to teach Carson all of the tricks I am learning in managing his diet, so maybe one day he will be independent enough to do it himself. 

A Day in the Life with PKU: Ordering and Purchasing Low Protein Foods

In my previous post, I discussed all the preparations we make, that I could think of, for Carson's low protein food.  This food is so important to his diet because he is so limited in what regular foods he can safely consume, and the low protein food supplements the calories and things he needs, or in other words, it fills up his tummy so he does not get so hungry.  It also gives us the opportunity to cook foods that are similar to the kinds of foods we eat, like pastas and pizzas and desserts, so he hopefully does not feel too alienated eating around others.

In order to have these special foods to prepare, we have to begin, of course, by obtaining them.  In the State of Alabama, there is no government assistance for the purchase of low protein foods.  There are some states that do provide this, even some insurance companies, but not where we live.  There is a bill that has been introduced and is reintroduced nearly every year known as the Medical Foods Equity Act, which is attempting to change this.  I provided a link to it in a past blog post, maybe last year's PKU Awareness Month, but you can Google it just as well.  :)  I am not sure how I feel about this issue, since we are living it I know how expensive the food is, but I am also not very fond of expanding government assistance.  For now, we survive.  Jehovah Jireh, God is my Provider!  People who need help can try for grants or samples or other assistance that is currently available. 

I began my journey of ordering low protein foods for Carson by visiting www.pkunews.org.  There is a box there labeled Diet-Related Information, and if you click on it you will find a link, near the bottom of the page I think, for a list of low protein food companies.  It includes website links and phone numbers of each company.  I do all of my shopping and most ordering online and it is shipped right to our door, which makes it so easy!  I love technology!  Some companies charge shipping, some actually do not.  Some companies offer coupons and specials from time to time, so it pays to check your email regularly for deals, and like them on facebook, if they have a facebook page.  Also, some of them may sell sample packs so you can try their products before spending a bundle of money, which is really important to me!

Just to give you a sample of what we order, I will share with you the invoices from the last orders I placed for some of Carson's main staples.

From Cambrooke:

Product		SKU		Qty		Unit Price		Ext Price
Shake 'N' Cheese		10309		2		$11.49		$22.98
MixQuick		10302		3		$13.33		$39.99
GO! Pockets - Cheesy Broccoli		10930		3		$8.99		$26.97
Pierogi		10412		1		$18.49		$18.49
Toasted Pierogi		10429		1		$33.99		$33.99
				Subtotal				$142.42
				Sales Tax				$0.00
				Shipping & Handling (with coupon				$0.00
				Order Total				$142.42

From PKU Perspectives:

Product ID	Product Name	Price	Quantity	Total
KMAA0322	Country Sunrise EGG/OMELET (UNIVERSAL Egg) Mix/ 1- #10 Can (3.75 lbs)	$45.00 (Not Taxable)	1	$45.00
Subtotal Amount :				$45.00
Discount :				$0.00
Promo Code Discount :				- $2.25
Shipping (Shipping is free) :				$0.00
Tax Amount :				$0.00
Current Total :				$42.75

From Nutricia North America:

I ordered 4 boxes of Loprofin Macaroni elbows ($11 each) for a total of $44 (no tax and no shipping for orders over $30).  Each box is 250g (about the size of a box of Pasta Roni).

I also ordered from Applied Nutrition (they are now merged with Nutricia) a case of Maddy's Fudge Brownie Mix (4 cans) for a subtotal of $29.50.  The killer here is that I can't just order one can, so I can't get it to a $30 minimum, therefore I had to pay $15 shipping!  That irks me!  The grand total on that one was $44.50.

This entire order totaled around $275.  Some of those items I might only order once a year, some I order every 3 months.  I am usually ordering something at least every 3 months.  Things that I did not have to order at this time were wheat starch and other baking mix for his bread, pizza pockets, and other types of pasta we use.  I think last year I estimated we spent somewhere over $800 for Carson's low protein food items.  We do count this as a deduction on taxes, which is helpful.

One last great way to save money is through a great resource, the National PKU Alliance.  If you donate to this organization, maybe no more than $30 for a year, you get all kinds of freebies and/or discounts from most of these low protein food companies.  This year I got an awesome variety of goodies!  It is the second year I have donated.  It more than pays for itself.   The biggest savings is Cambrooke, who gives $100 gift certificate the first time you donate, then $50 each year you renew.  I saved a lot of money by doing that again this year!  Look at all the great stuff we received from Nutricia (about $100 worth as well!)!

A Day in the Life with PKU: Food Preparation

For this post series, I am going to discuss in each post a different task or activity involved in PKU management/treatment in daily life.

Saturdays are usually good days for baking.  We typically stay at home, and since Daddy is nearby, Mommy has more of an opportunity to cook.  Our most intensive baking projects at this time include loaf bread, rolls, and chocolate chip cookies.  This past weekend, I made a new loaf of bread.  The recipe I use is from the cookbook Apples to Zucchini, which is Virginia Schuett's newer low protein cookbook you can purchase directly through www.pkunews.org, or I am sure you can find it on Amazon or maybe other places as well.  The recipe is called Trish's Best White Bread, and it is an updated version of the recipe from the older cookbook (the red checkered one).  This recipe adds a few variations from the former, such as a combination of Welplan Baking Mix and wheat starch, Coffeemate creamer powder, and molasses for color.  The "magic" ingredient in any lopro bread recipe is Metamucil, which gives the bread fiber and shape.  And no, you cannot tell it is there!  This bread is delicious!

A few of the bread ingredients





The dough mixing in the bread machine

Yummy! Carson loves eating the dough while
we are baking.

Finished product! Soft, fluffy, and yummy on the inside.

I also use this same recipe to make rolls.  I put the same ingredients in the bread machine on the dough cycle.  When it is finished, I follow the instructions in the "older" red checkered cookbook to make and bake cloverleaf rolls (we like these best).  I make a batch and keep them in the freezer.  Whenever we plan or think there is a possibility we may be eating at a "sit-down" restaurant, I take a couple of Carson's rolls along so that he can have rolls (and butter!) while we do. 








For lunch that same day I made the last bread, I also made Carson a low protein hot dog.  It is made from a powdered mix, and they are the most amazing things I have seen!  We use the Country Sunrise Hot Dog Mix, which you can purchase from www.pkuperspectives.com.  Preparation time is nothing, just measure the ingredients, dump them in a bowl, mix well.  I learned the hard way that handling it bare handed is not a great idea.  I put my hand in that pink stuff the first time and it stuck all over me!  I could not get it off!  Plastic gloves might work well, but since I don't keep those in my house, I just rake the mixture onto a sheet of plastic wrap, wrap it up, and roll and shape it into a hot dog shape.  Then I unroll it onto a plate and microwave for 30 seconds...voila!  Instant hot dog!  Carson must also eat with ketchup (we are still not to bun stage yet, but then again, I never eat my hot dogs on a bun either! lol).

The powder

Add vegetable oil and water...it turns pink!

After mixing, roll into hot dog shape



Microwave just 30 seconds and this is the finished product!

 

Besides the occasional hot dog, lunch at the Rice household usually consists of some kind of sandwich.  For Carson, there are only two choices that he enjoys so far:  it is either a sandwich with Biscoff spread (which we buy at Walmart on the peanut butter and jelly aisle) or a grilled cheese (made with imitation cheese slices which we buy at our local grocery stores, which are great since they include calcium, unlike Cambrooke's very expensive cheese slices!).  We of course use the loaf bread we made (above).  These sandwiches are great options to take out when we are not sure if there is anything Carson can eat in a restaurant (especially if we go fast food), or if we are having a picnic lunch.

 

 

 

 



For breakfast, Carson typically eats Cap'n Crunch.  2/3 cup has about 50mg phe, which is not too bad for a meal by itself.  He drinks his formula, which I now mix with a bit of rice milk for added calcium (separate post later).  Some days he might eat a banana, or if we are at home he might ask for pancakes and eggs (or for brunch or even lunch!).  I use the Country Sunrise Scrambled Egg Mix, which is a powder to which you add oil and water just like the hot dog mix.  Scramble in a skillet and you have low protein scrambled eggs!  They are delicious!

Here he had toast, but now he usually prefers pancakes,
which I make using Cambrooke's MixQuick.

At suppertime, I find preparing things a bit more difficult.  We do have plenty of options and things that Carson likes to eat, but I generally prepare his food fresh instead of fixing up large batches of things ahead of time.  If I am cooking a big meal for the rest of the family, then I have lots of cooking to do to keep me busy!  I usually try to make things similar to what the rest of us are eating, but if I do not have anything, I will just ask Carson what he wants and he usually has a preference!  I almost always fix him some type of low protein pasta (we use Loprofin Fusilli) with a variety of sauces and some vegetables.  Right now I think Carson's favorite is pesto, so I use the Knorr packet of Pesto Mix and prepare a whole supply.  I can use this for him for several days, and Maggie and I will eat it as well (picky Marty doesn't like it!).  I cook a pot of noodles for us, a small pot for Carson, then the sauce.  We almost always have garlic seasoned cauliflower or broccoli in microwave steamable bags.  Makes for an easy and delicious meal!  I do the same with spaghetti and stroganoff (without meat in either one).

One of Carson's go-to meals right now is low protein macaroni and cheese.  This I have to prepare completely separate from our meal.  I use Loprofin Macaroni Elbows, boil 1/2 cup (dry measured), drain and set aside, then prepare the cheese sauce using the same pot.  I use Cambrooke's Shake n' Cheese powder and follow the directions on the container, but I only make 1/3 the recipe.  It calls for butter, which I melt in the pot first, then I use coconut milk creamer, because it is virtually phe-free, then add 1 Tbsp Shake n' Cheese and mix it with a whisk, heating it until it bubbles and thickens.  Stir in the noodles and we are done!  The phe for this portion Carson eats is estimated at only 15mg!

And now, for the best part, Carson likes to start off supper almost every night (when possible) with....

SALAD!!!!!!!!!!

I cannot believe he actually likes salad now!  I am one happy momma!  It makes for yet another great option, especially when we are out, to get that tummy full with something healthy.  I just weigh out salad pieces on the gram scale and pour a bit of Italian dressing (which is phe-free) and he likes to pick it up with his fingers and dip it.  Nothing fancy, but it is awesome!  Got me eating more salad too.

We do also have several convenience foods we can use on days when cooking is just too much, and we can just heat these up in the oven or microwave.  We keep Cambrooke's Mini Pizza Pockets, Go Pockets Broccoli and Cheese, and Toasted Pierogi (which is kind of like breaded/fried ravioli filled with lopro cheese and potatoes).

For dessert, there are only a few things I have to bake that Carson will eat.  He loves chocolate (like his momma!), so his favorites are chocolate chip cookies and brownies.  For the chocolate chip cookies, I use the Butterscotch Chip Cookie recipe off Cambrooke's website (which uses MixQuick), but I substitute butterscotch chips for 1/2 cup Chocolate Dream dairy-free chocolate chips (awesome!).  For brownies I use Maddy's Fudge Brownie Mix (from a can).  It is super-duper easy, much like our regular boxed brownie mixes.  We just add oil and water, mix, pour in the pan, and bake.  I also like to add 1/4 cup of the dairy-free chocolate chips to these, which I started just because the chocolate in the brownie mix tasted too "fake" to me.  Carson loves it!

Carson's famous chocolate chip cookies!  Yummy!

These were made fresh today by Carson's request!

On the occasion we attend a party of some sort, including birthday parties, or anywhere there might be cake or cupcakes, I try to always come prepared with a cupcake for Carson.  I make batches of cupcakes and keep them in the freezer, so that when we are going I can get one out, put it in a plastic individual cupcake holder I bought for this purpose, and decorate it with icing and sprinkles.  I like to use Pilsbury's (since it's phe-free) icing in a spray can because it is super easy...just squirt it and go!  I have two different kinds of cupcakes so far:  both Maddy's Yellow Cake Mix, one made chocolate from the recipe on their website by adding a bit of cocoa powder and instant coffee!  He likes these okay, but usually only eats a few bites and mostly just eats the icing.  I am going to try some different homemade recipes in the near future.

For his own birthday with our family, on his actual birthday, I usually make him his own low protein cake and decorate it myself.  I am not very good at it, and someday will probably get someone more professional to decorate a really good-looking one (I will have to bake the cake part and take it to them), but I finally found an easy cake recipe that everyone liked.  I used a recipe from a free cookbook that uses Maddy's Yellow Cake Mix and Brownie Mix to make a marbled cake.  Carson ate every bite.  I hope this continues to be successful!  I plan to get a little more adventurous this year and make a character-shaped cake, maybe even a tractor!

I think that sums up most of the foods I actually prepare, cook, and bake for Carson that are separate from our own.  Other things that make it onto his menu are things we can get anywhere, like applesauce, bananas, potato chips, and dry cereal.  Eating out is a whole different story!

I also think that writing this very long post felt like more work than actually making all this food!  It seems like a large amount of work, making separate meals all the time, but we are used to it.  I have been doing it for almost five years now.  That does not mean that it is not tiring sometimes, because it is.  But I am so thankful to be able to be a low-protein chef for my son, and learn new things every day.

If you are reading this and live in Alabama, or know someone, who might need some support with PKU-related things, I want you to know that there has been an Alabama PKU Support Group started and growing on facebook.  It is a private group, but if you want to join the network I highly recommend checking it out!  I am loving it!  There are also great national support groups, like PKU Worldwide Support Group, and Cook for Love on facebook.

PKU Awareness Month 2014

Here we are once again...already!  May is National PKU Awareness Month.  Carson must be really special since he gets two whole months of blog posts dedicated to him!  I will definitely make it a point to focus on Maggie and new baby girl this summer!  I just want everyone to know one reason why I focus so much during the awareness months is because that is when people search the internet the most for these topics.  I began receiving hits on my blog about PKU awareness before the month even started.  I watch the traffic flow and try to take the opportunity to write about things I feel are important for everyone to know.

This year, raising awareness and educating people about PKU and the diet are especially important to me because Carson is beginning kindergarten in regular public school this fall.  I have mentioned it in many posts already, and that is because it is a hot topic issue I am working very diligently on right now.  I want to make sure that Carson is cared for at school by people who are truly interested and willing to learn to understand how important his diet is.  Since Carson has autism, I am especially strict with his diet because I do not want to take any risks:  1.  I don't want to cause any damage to Carson's unique brain.  2.  I don't want Carson to even have a taste of foods he should not have on a regular basis, since at this point he still cannot comprehend the reason why he needs different foods.  3.  I want Carson to stay in his routine and learn to understand how important what he eats really is.

You can read all about PKU (phenylketonuria) in several places on my blog:  by clicking the PKU tab at the top of the page, or by clicking on any of the Topics related to PKU to link to previous posts.  Also, I have a few informative links under Helpful Links, which will take you to some of the websites I use for resources.  My blog is simply my own personal opinions, experiences, and feelings, as well as some shared information I have read and learned over the years.  Please keep that in mind when reading my posts.  I am not a professional expert; but I am a mom expert who is currently living it!  I would love to share stories with anyone out there and I try to be an encouragement to anybody who struggles.  Sometimes in life we just need someone else to say they understand how we feel because they have been there.  I know I do. 

In upcoming posts, I plan to write a series called A Day in the Life with PKU.  I am going to try to just document our daily routine, including formula making, meal planning and preparation, food ordering, and blood drawing.  I want to show everything we do (in pictures), even though some of it is not done very often.  My goal is for everyone to see how we manage Carson's diet every day, and how routine it is with very little change, which is very important to Carson.  Check in soon for my first post!

Carson the Awesome

The sky is the limit for this boy!

On this last day of Autism Awareness Month 2014, I wanted to reflect over this past year and the amazing progress Carson has made.  One year ago he first received a diagnosis of Autism Spectrum Disorder.  One year ago he started occupational therapy and private speech therapy, and also received both therapies through the school system.  In August of last year, Carson started preschool 3 half-days per week at the Cullman County Child Development Center, a special school for children with all kinds of developmental delays.

All of these things, along with general maturity, I am sure, have helped tremendously with his development.  This child is so different from a year ago.  Last year, Carson did not usually speak in full sentences, and most words he spoke were hard to understand.  There were times when I never knew what to expect from him:  what he wanted, how he felt, or what he understood that was going on or being said around him.  He was nowhere near being potty-trained.  Today, this child can argue with me.  He tells me how to drive.  He answers me when I ask him what he wants to eat.  He tells me if his tummy hurts.  He tattles on his little sister.  He is brutally honest.  And he talks about the baby in my belly (he has decided she should stay there; he doesn't want her to come out and play with him and Maggie!).  And he is potty-trained.  All by himself.  He tells me when he needs to go.  None of these things did I dream possibly would happen within a year.  At least not all of them collectively. 

Yesterday, we went for his semi-annual PKU checkup at the UAB Department of Genetics.  The doctor he has seen there the last few times, Dr. Maria Descartes, was so amazed with his progress over the last 8 months since she had seen him, and she just went on and on about it.  She remembered how he cried and fussed and did not want to be there, even seemed afraid (or annoyed).  He did not want to cooperate with anyone and would scream and cry if anyone tried to get near him or get him to do anything.  Yesterday, my big boy climbed up on the exam table, pulled up his shirt, and said, "Listen to the baby in my tummy, doctor!"  He cracks me up.  He loves going to the doctor.  Dr. Descartes declared that Carson was "awesome", and taught him to say so.

Tomorrow, we register for kindergarten at our local public school.  A typical school with all different kinds of students.  Tomorrow we take a giant leap of faith.  This mommy is scared to death!  I know this is just the beginning, just the legal/formality part.  We have not yet had his IEP meeting to discuss all his needs.  That will be coming soon, I am sure.  Everyone at preschool says he is ready.  I worry about how much help he will need.  No one is even sure if he needs an aide!  There is so much to consider, so much to be done, so much paperwork, so many decisions, so much planning...but it is going to happen.  Starting tomorrow.  I cannot believe it.  I never imagined it would be here this soon...or that he would be ready.  But I think he is.  I hope I am.

Everyone who knows Carson and is around him at any time is amazed.  I am constantly amazed by the leaps and bounds he has made.  It is so much easier to communicate with him now.  It helps that he can relay that he understands what we are telling him.  That makes meltdowns easier to diffuse before they happen.  We can explain plans to Carson before they happen, and when he knows what to expect, he handles it so well (most of the time).  Of course, because he has autism, he still struggles with things.  He has times when he cannot transition from one thing to another without getting upset, or times when he is focused on something and doesn't seem to hear or listen.  There are moments when he does not seem to understand what is going on.  There is a need for routine and sameness, repetitive things.  He wants to do everything himself and to always be first.  If these things don't happen, some days there may be a meltdown.  But for the most part, compared to a year ago, it is diminished.  He is easier to rationalize with, although sometimes so stubborn that he would argue whether or not the sky is blue!

It feels like a fog has been lifted from around Carson's head.  He seemed so far away, so hard to reach, but now he is with us.  He sees us, he hears us, he talks to us.  I am beyond thankful for all the reasons that he is where he is today.  I am thankful to Dr. Lane Rutledge, his other genetics doctor at UAB, for setting us on this path to have him evaluated and diagnosed.  I am thankful for Early Intervention beginning speech therapy with him right away, over two years ago.  I am thankful for all the therapists that work with him now, privately and at school.  I am thankful for his teacher and aides and nurse and everyone at the preschool who has any contact with him.  I am thankful for all my closest friends and their children, who help me by lending a listening ear or a shoulder to cry on, and for allowing our children to spend time together and learn from each other.  I am thankful for our parents and grandparents and all our close family members who care about Carson, take an interest in all things to do with him, who have patience with him, and have taught him so much of what he knows already.  And for helping me by being willing to babysit so I can be with Carson at therapy and learn how to help him at home.  Most of all, I am thankful to God for sending me this precious gift, the most precious little boy who is such an inspiration and such a treasure to my life.  I am thankful for being entrusted with his care, and for being equipped with all the tools I need to make sure he gets everything he needs to flourish and grow and learn.  We are definitely beyond blessed.

And Carson is definitely AWESOME!

I want to stress again to all parents out there who have been given any reason to believe their child is developing differently in any way, to have them tested.  Take them to a professional, whether they are behind in speech, or motor skills, or social communication...there are therapies for all of these things!  There is NO shame in admitting your child needs help!  The best thing you can do for your child is to find the help he or she needs, and you need.  Early intervention is the key, and it is the best thing that can happen for you, your child, and your family.  Don't delay!