PKU Families

PKU hotdogs

2011-07-23T08:28:00.000-04:00

Day in the Life of PKU

2010-01-30T10:26:00.000-05:00

-I posted this on my private family blog, and thought I'd share it with the PKU community)
For those who have asked (maybe you'll wish you hadn't after you see how long this is)...
Let me share a little bit (is a lotta bit a word?) about daily life with PKU. I wake up every morning (usually at the bottom-crack of dawn to hear Owen screaming for me to unlock his door,, the knob was purposefully turned inside out - an entirely different story) and fix Owen his "baba" (a.k.a. "special milk", "Phenex2", or as I fondly like to call it - "my retirement money in a sippy cup"). It's a strange feeling to be extremely bitter and profoundly grateful for something all at once. This formula, which Owen will take for life, provides Owen with over half of the protein he needs to grow and develop each day, it is just modified so his body can metabolize it.
To illustrate, the minimum protein requirements for most other toddlers, is 16 grams per day. Owen can have no more than 7 grams, and the rest has to come from his amino-acid modified milk. As Owen grows, he will still not be allowed more than 7-8 grams of protein per day, even though daily requirements for most people will increase to the 50+ grams into adulthood - a pregnant or nursing mother is recommended to take 71+ grams of protein per day. A Burger King whopper has 30 grams of protein. You get the idea. Anyway, as if my own life didn't already revolve around food (I can't seem to rid myself of my incessant love for all things edible), people with PKU literally plan their day around what's on the menu.

Breakfast...
This usually consists of low-protein bread (which I will detail later) with butter and cinnamon-sugar, some fresh fruit (favorites - cantaloupe, strawberries, blueberries, grapes) or applesauce, yogurt made from coconut milk (this is a miracle product for us), cereal with rice milk, low-protein pancakes/waffles, low-protein eggs ("feggs")/omelet or breakfast burrito in a corn tortilla, French toast (made w/ "feggs"), or sometimes a granola bar or cereal bar as a "high protein" option. I weigh and measure whatever I give him, and then I record it in a daily spreadsheet that I use to calculate his intake. I also get to count any masticated leftovers and subtract that since he didn't actually consume it. My goal is to offer him about 2 grams of protein per meal, and leave about 1-2 grams of protein for daily snacks.

Snack time (served mid-morning and late afternoon)...
Owen likes goldfish, but can't have the cheddar flavor, because there's too much protein. We give him the "original" flavor (the ones that taste like soda crackers) or the honey graham flavor. We count out 25 pieces and put them in a snack bag or bowl. I usually throw in some fresh or dried fruit or carrot/celery sticks, and if he's still hungry we add some low-protein pretzels, or certain brands of fruit snacks or fruit leather not made with gelatin (we read the label on EVERYTHING).
(Quick tangent here) Did you know that if a nutrition label says "1 g protein, it means that it can have anywhere from .51g-1.5g of protein? That may seem trivial to a regular eater, but to someone only allowed 7 g of protein - that is a HUGE variance that we have to take into account. We have a low-protein food list that we consult for EXACT amounts of protein and phenylanine.

Lunch time...
Lunch is usually a low-protein slice of cheese on low-protein bread for a grilled cheese sandwich (sadly, the cheese doesn't really melt), pasta, rice, french fries, and fruit and vegetables which are always a must for each meal - the staple of his diet. Don't forget weighing, counting, measuring (and I said I'd never use my tenth grade math skills!) and recording.

Dinner time...
Dinner around our house is a wild card, because by the end of the day - based on what Owen has or has not eaten - I modify the last meal to give him enough of the protein he needs, without exceeding his maximum. My menu has to be planned in advance for us regular eaters, so I can plan in advance what we'll fix for Owen. For example, if we're having spaghetti - I make a low-protein spaghetti, and set aside some pasta sauce that doesn't have cheese or meat in it. If we're having garlic bread, I make him a slice with his low-protein bread. It's a little more difficult to substitute, let's say...a roast? If we do a roast, I try to ask Owen what he'd like and make that for him. He likes pizza (low-protein crust, low-protein cheese shreds, tomato sauce and all kinds of vegetables), so that is common around here. We also make sweet potato fries, fruit salads, baked potatoes, veggie stews/soups, and salads - and of course, BREAD (see bread section below). Don't forget weighing, counting, measuring and recording.

Dessert...
Dessert is an easy option for PKU. Owen is allowed anything that is pure fat and/or pure sugar. He can eat any candy (with the exception of chocolate and things made with gelatin). We often serve rice krispie treats, sorbet or popsicles, Rice Dream frozen dessert, low-protein cookies and cupcakes, and low-protein chocolates.

Baking...
Aside from making and/or buying low-protein items, one staple of our life is bread (who doesn't love great homemade bread?) - it is eaten at practically every meal, and sometimes in between. There's another PKU mom, Brenda (a.k.-in our house-a. Miss Amazing), who has a PKU daughter. She started a non-profit COOK FOR LOVE -because as she say, some people love to cook, and others have to cook for love. (I happen to do both). Anyway, she has developed recipes very similar to their high protein alternatives, most of which can be made with items you can buy at regular stores! This company and her bread recipe has changed our life. The bread is incredible (if you want to make a tax-deductable donation to her cause -which has become our cause- click HERE). Anway, the bread, along with all other PKU recipes is made by weighing every ingredient, and following directions exactly (unlike my regular cooking style). My dear mom and sisters decided to try out the Cook for Love bread recipe all the way across the country. It warms my heart and touches my soul that they did this. If you want to make my day - learn to make something for Owen.

Special Occasions...
Birthdays, dinner parties, holidays, school parties, nursery snacks, eating out - all require special thought and preparation. I usually call ahead to see what's on the menu so I can bring options for Owen. If I am bringing a dish to share, I try to make at least one thing that everyone, including Owen can have. I bought a deep freezer so I could make batches and batches of low-protein goodies and meals that I can pull out at the last minute.

That's It!
Owen has a pretty open mind and will try anything he is allowed to try. Ironically, baby sis, who is lucky enough to be able to eat anything, spits out anything green or orange, and will hardly open her mouth for anything besides yogurt and bread.
Two and a half years later, although the stress and strain of Owen's diagnosis has lessened, PKU continues to be something I think about every. single. day. Obviously. How could you not think about it when you get to raise such a handsome little angel? I would not have my PKU life any other way.

Food for Thought

2010-01-03T10:55:00.002-05:00

A good friend of mine, Beth over at Misadventures, Rants and Tidbits , recently posed this question to me:

"Out of curiosity, given the traditional high-pro foods normally served on T-day, what do you guys do or plan to do for your family meal now? That would be a stupendous, informative post!"

Well, ask a question and you shall receive an answer!

When we learned of Ryan's PKU diagnosis, we were righteously overwhelmed to say the least. I mean, food after all is what most of our daily lives are centered around; breakfast, lunch, dinner, snacks, parties, outings, vacations, family get togethers and I could go on and on. Wait, did I mention Holidays!?
I have never wanted to raise my children to focus on food. I never want to use food as a reward (ie: "If you let mommy shop, I'll give you a cookie"). I often tell my mother-in-law she doesn't always have to bring the twins a snack (even if it's grapes) or give them cookies every time they visit her house (which is now the reason my son runs to her snack cabinet or fridge everytime he walks in her door). Now, it's hard not to put a focus on something that is such a significant part of our lives. Every morning, every event, every outing we have to think about what Ry is going to eat for the day. If we go out, will there be something he can have? If we go to a party, will there be food for him to eat? What is he going to eat if we go to a carnival or a circus? These are the thoughts that cross my mind every day, throughout most of the day.

So here's is what a typical menu is like for Ryan. These are just the basics.
Breakfast: Most cereals, Low Protein (LP) waffles or homemade pancakes, LP breakfast bars, LP toast or a regular slice of toast, fruit

Lunch and Dinner: So Delicious Coconut Milk Yogurt, Grilled Cheese (LP cheese), Pasta (LP or regular depending in his protein intake for the day), LP Homemade Pizza, Homemade Veggie Nuggets or Burgers (veggie burgers from the supermarket are too high in protein), French Fries, most veggies and fruit, Walden Farms Peanut Butter, Veggie Stews & Soups.

Snacks: LP cookies, fruit, Gerber puffs, Glutino Pretzels, some chips, fruit snacks, ice pops, Coconut Milk Ice Cream, Hunts Lemon Pudding (only one dairy free), Veggie Chips, Crackers

We offer him a lot of products from Cambrooke Foods but at this age it is hit or miss. I make A LOT of homemade food. I like to experiment and try new things for him. Cook for Love is a great new resource for the PKU community. The recipes are OUTSTANDING! However, I can spend hours making something and all the boy will want to eat is tomatos and cucumbers. I know, we should all be so lucky that all our children want to eat is tomatoes and cucumbers! If you ever see me in the supermarket, I'm the crazy lady in the aisle getting excited over a product that has a LP nutritional value!

Keep in mind, just because it's low protein doesn't mean Ry can eat an unlimited amount. Currently, he is tolerating about 12-13 grams of protein a day(which was recently increased). 8 grams of that comes from an 8oz glass of milk he drinks once a day. Leaving only 5 grams for 3 meals and snacks. To put that into perspective, a small frozen pizza you and I eat can range from 20 grams of protein or more. There could be 2 grams of protein in 10 potato chips, 2 Oreo cookies have 1 gram of protein. I try to mimic a lot of food. For instance, when he goes to daycare and its pasta day, I send pasta, when its waffles, I send LP waffles, etc. It's nice to hear from his teachers "Wow, we can't wait to see what you come up with next!". For family dinners it is basically the same idea. We eat regular pizza, Ry eats LP pizza, we eat meatballs, Ry eats homemade veggie meatballs. It's not always that easy, but I do get creative!

You have heard me say Ryan has mild hyperphe/mild PKU. He can tolerate more protein than a person with Classic PKU. Every case is different. Some may only tolerate 5 grams of protein a day while others who have hyperphe are not on a restricted diet at all. The majority of PKU'ers have to weigh and measure everything they eat to calculate their intake. We don't have to do that with Ryan's food. In one sense it makes it just a tad bit easier.

So, there you have it. Any more questions B?!

Cross posted at A Double Serving of Love

Alphabet

2010-01-03T10:45:00.002-05:00

Cross posted at A Double Serving of Love

Ever since Ryan has been eating table food, we have tried to introduce "PKU language". We use words like "Low- protein" and "Phe". We don't like to use the words "can't eat" instead we say "doesn't eat".
Today Ryan and I had a little conversation during lunch.

Mommy: "Does Ryan eat chicken?"

Ryan: "Nooooo."

Mommy: "Does Ryan eat meat?"

Ryan: "Nooooo."

Mommy: "Ryan eats low protein foods."

Ryan: "Low protein!"

Mommy: "Ryan eats low protein because Ryan has PKU. Can you say PKU?"

Ryan: "No mommy, P Q..."

My son teaching me the alpahabet!

Nursing a Child With PKU

2009-12-12T09:38:00.004-05:00

I thought I would quickly share my thoughts on this topic since I hear all sorts of different ideas on the subject.

First of all, This ---->
is Audrey! "Hi, Audrey!" She is now 3 months old and is successfully nursing and taking her medical formula (Phenex-1). She is staying right in the middle of the range and growing and developing right on schedule.

I made the decision to nurse her, and it is very important to me. I was lucky that her tolerance allows me to do this (she is not hyper phe) and my clinic is supportive. I nursed my son Owen for 10 1/2 months till his clinic asked me to stop. I did it hoping we would figure out why he wasn't growing on a standard curve... I regret that decision but made the best one I could with the information I had at that time. His growth had nothing to do with nursing.

My clinic has told me that most Moms don't nurse as long as I did with their PKUers. I was a bit surprised by this statement and think some clinics do not want the added hassle of a breastfeeding variable. Unlike other foods, breast milk can't be measured and accounted for as easily. Every week is a little game... how much did Audrey nurse? And was her medical formula enough for that day? As the numbers fluctuate up and down (nothing drastic, though) our clinic has to adjust and readjust her therapy plan. Luckily we have found our groove.

I nurse as an investment in my daughter. There are some times I wish I could have nursed Owen longer... we had a hard time breaking that habbit and just a few short months later he became really sick. I wonder if nursing through his illness would have helped shorten his 3+ month ordeal. I stick with it with the help of my trusty Medela pump, to keep me producing, and good milk storage containers. I use glass and Medela BPA free plastics.

My best advice for new parents would be to have a discussion with your clinic right away. I started off the first visit by stating (not asking!) that I would be trying to nurse Audrey longer. We were up front about our wishes and hopefully things will work out for us. I know it will be more difficult to juggle once she is eating solids, but I know it can be done. Please work with your clinic if you want to nurse and don't be afraid to advocate for yourself and your new baby.

Vlog- our trip to the genetecist

2009-10-11T22:37:00.000-04:00

Welcome to the community!

2009-09-28T17:41:00.002-04:00

I just wanted to post an update for our family. We welcomed into the world our daughter, Audrey, on August 31st. Audrey now joins her older brother Owen in the PKU community.

We received the diagnosis 2 weeks ago and are happy to report she has taken off on her medical formula and is still gaining weight steadily! I am working hard to keep nursing her...not an easy job with 2 kids, formula, logging of feedings and a pump. But, I am determined (i.e. stubborn?) to do it all.

So, please welcome a new girl to the block... our little Audrey girl.

Have you seen this?

2009-09-21T22:40:00.002-04:00

This movie made me so much more grateful for the newborn screen. PKU may not be easy, but at least it can be treated.

If I may toot my own horn...

2009-05-27T07:31:00.002-04:00

A while back, I made a video explaining PKU. Well, there was a European organization for rare diseases that was running a video competition. I thought I would go ahead and submit my video (even though I was a bit embarrassed to show them my goofiness). If anything, my video was surely different- and PKU would get some attention.

Well, guess what?

I WON!

Yep. The article is HERE.

72 HOUR KITS

2009-02-20T17:36:00.003-05:00

I AM MAKING MY FAMILIES 72 HOUR KITS BECAUSE I HAVE BEEN A HUGE SLACKER. I THOUGHT THAT SOMEONE MAY HAVE POSTED ON THIS BLOG ABOUT A PKU VERSION BUT I CANT FIND IT. ANYWAY I THOUGHT I'D TRY DOING IT MYSELF BEFORE ORDERING FROM PKU PESPECTIVES. ANY ADVICE WOOD BE GREAT. WHEN I GET IT ALL PREPARED I WILL POST THE WHOLE KIT. THIS IS SO IMPORTANT FOR ALL OF US BUT ESPECIALLY BEING PREPARED WHEN OUR CHILDREN HAVE SUCH A RESTRICTIVE DIET.

ANY ADVICE FOR FOOD STORAGE WOULD BE A HUGE HELP FOR ME AND EVERYONE ELSE.

THANKS A BUNCH

CRISP FAM

Preaching the the Choir

2009-02-02T14:16:00.001-05:00

I know, I know. We all know what PKU is. Still, I made a video explaining it a bit. If you follow me at all, you know Im a bit of a goof ball- but If you want to share the link its here:

http://www.youtube.com/watch?v=rHh0yhBYBbI

PKU Kid's Blogs

2009-01-20T20:38:00.000-05:00

I want to make a sidebar of other PKUers family blogs...if you'd like yours to be listed, please leave a comment. Thanks.

Frustrated with Formula

2009-01-20T20:36:00.001-05:00

Our son, Owen, 18months, just transitioned from a bottle to a sippy cup. Lately, he's been refusing to drink his "milk" from either. It is so frustrating. Sometimes I get to the end of the day, and he has only had 2/3 of his required formula intake, and he'll fall asleep fighting me not to drink the rest. HELP! Has anyone else had this problem? What did you do?

I asked people on the Listserv, and got a bunch of different ideas about distributing it throughout the day. What if he won't drink it at any time? Argh! Thanks for letting me vent.

Late thoughts on gratefulness

2008-12-02T22:08:00.006-05:00

Painful thoughts can enter your mind at any moment. For me, it was today, as I was getting Katy buckled into her car seat. Having just finished her supper, I was doing the usual mental math to recap the amount of phe's she consumed for that meal. I calculated that she had about a gram and a half of protein. I was proud of how much I was able to make her meal stretch. That's when the thought that has crossed every PKU parent's mind ran through mine-

"How Unfair"

I sulked inwardly, thinking of all the great stuff I wish I could feed my little girl. I imagined cheese cubes, and yoghurt cups. I thought of cookie jars and sandwiches. Is that too much to wish for? To hope that my daughter can one day eat good, nutritious food and not have to worry about it damaging her brain?

How unfair!

But then I thought about the Norwegian doctor- Folling, who insisted on finding the cause for mental retardation in two siblings. I thought about his pioneer research in the 1930's that led to the discovery of Phenylketunria. It was his enlightenment that led others to take up the torch and walk the next mile. From him came those who advocated and made blood screening at birth mandatory in the united states. Then, there were others- like Virginia Shuett- who spent their entire lives finding ways to feed people like Katy. PKU is one of the few genetic diseases that can be controlled by a diet.

There are so many other metabolic conditions that have no cure. Many are even fatal. That is not the case with Katy. We have research, support, information, and many types of foods fabricated to make PKUers live a more normal life. We have a formula that supplements her nutrition, a geneticist who checks up on her development (and always gives us good news), a well informed nutritionist, a whole team of therapists, and best of all- we have what so many other families wish for-

HOPE

So, even though this thanksgiving my daughter could not eat turkey, I am grateful that she could sit at the table and much on her special Cambrooke's food as we shared smiles, love and laughter.

PKU- what a cinch!

With all my pride, I post a video

2008-11-10T01:24:00.002-05:00

Many of you know that my daughter, Katy, was diagnosed late with PKU. She was 13 months old when I put her on the diet. By the time she had her diagnosis, Katy was suffering from serious symptoms. She had four to six seizures per day. Each episode lasted between ten and fifteen minutes. Her muscle tone was extremely weak. She had no sense of balance. She lost all her milestones, and we felt like we were losing her. We spoke with the doctors about feeding tubes, walking aides, wheel chairs and even coma inducing medications.

That's when I decided that I was not going to sit back and let this over take my daughter. I have wholeheartedly devoted myself to her recovery, rejecting any notion of brain damage. I refuse to go down without a fight. And I refuse to fight and not win. 24/7 I am her nutricionist, therapist, cheerleader and mom. From this blog, you will see awesome things take place...even if they delay a bit.

For now, I leave you with a video of her healing progress. Katy has just turned three years old.

I truly believe in the healing power of laughter. We all deserve a chuckle. After all, we're all "normal"

Toodles, PKU community. I love you all. Thanks for the friendship.

Apples for a sweet New Year

2008-09-29T12:17:00.003-04:00

I try to give Ryan food as similiar as to what others are eating as much as possible. It is especially important for me to do that because he has a twin sister. Tonight marks the first night of the Jewish New Year, Rosh Hashanah. In honor of the New Year, we often eat apples and honey to symbolize a sweet New Year. I made an Apple Crisp for our family dinner and tried a recipe for Lo-Pro Apple Crisp for Ryan.

APPLE CRISP

from Family Friendly Recipes

4 Apples, peeled and cored, sliced

1 1/2 C CBF Baking Mix

1/4 C Sugar

1/2 C Brown Sugar

1 TSP Cinnamon

1/2 C Margarine

Heat oven to 350 degrees. Mix together apples and sugar and spread in greased baking dish. Blend CBF Mix, brown sugar, cin., and margarine until crumbly using a fork or processor. Spread evenly over apple mixture, pressing down to cover all apples. Bake for 30-35 minutes or until top is golden brown. Serve with non dairy topping such as Cool Whip. For a variation, add strawberries to apple mixture.

6 Servings/ 1 slice each

4 PHE per serving (.2 exchanges)

Our Story

2008-09-16T10:29:00.004-04:00

(As told on our blog...)

Ryan has a metabolic condition called Phenylketonuria, or PKU for short. PKU is an inherited, genetic condition in which the body can’t process phenylalanine (Phe), an amino acid found in many foods. Matt and I were both silent carriers for the gene. When babies are born, a heel stick blood test is done to test for various disorders - PKU is one of the disorders that is tested for. In the US an average of 1 in 15,000 babies is born with the disorder. People with PKU have a deficiency of an enzyme which is necessary for the proper metabolism of an amino acid called Phenylalanine(phe). Phe is an essential amino acid and it is found in nearly all foods which contain protein: meat, eggs, dairy, nuts and many other foods that are not generally thought of as containing protein (eg, most wheat products, such as pasta and bread, and some fruit, such as oranges and cherries). Additionally, Phe is found in aspartame, the sweetener found in most diet soft drinks,sugar-free jello and sugar-free candies/gum (look on the can of soda, you'll see the warning). If you have PKU you must follow a strict "low protein" diet to avoid all food sources that have high Phe. If the diet is not followed, phenylalanine is not adequately digested in our bodies and it builds up in our blood. Too much Phe is toxic to the brain. High Phe levels over an extended period of time can lead to vomiting, irritability, eczema, the loss of pigmentation in the skin/hair/eyes (people with untreated PKU often have light skin, blond hair and blue eyes) seizures, psychological and behavioral issues, and severe mental retardation. The goal of PKU management is to keep the amount of Phe in the blood at a safe level in order to protect the brain. The National Institutes of Health (NIH) recommends that blood Phe concentrations remain between 2 and 6 mg/dL for newborns to age 12. It is recommended that Phe levels be maintained between 2–15 mg/dL after 12 years of age. Currently, management of PKU consists of limiting Phe intake. To get adequate amounts of other amino acids found in protein (without the damaging phenylalanine), people with PKU have to drink a special formula. They have to drink this for their lifetime. However, there have been such amazing advances in the PKU foods, there are now other form he can get the amino acids he needs...special chocolate bars, flavored drinks. There are several companies that produce numerous kinds of "lo-pro" foods such as their version of mac-n-cheese, brownies, cake mix, pastas, cookies, breads, burgers, etc. Majority of the food will be veggie based. There is a special book which lists most foods on the market and their PHE content. PKU is certainly a detrimental disorder if not treated properly. If babies are put on the low protein diet at a young age, and they follow the diet throughout life, there is no evidence that their PKU will be a major hindrance in their accomplishments.

So here is Ryan's story. A few days after we came home form the hospital, we received a phone call telling us Ryan was a "red flag" for PKU and needed to be brought back in to be tested immediately. We had heard of PKU but really had no idea what it was. We were so worried and upset. When we started doing research online, the only words that stuck out were "mental retardation" and neurological disorders". After a few blood tests, meeting with a specialist and a nutritionist we learned Ryan had a milder version called mild hyperphe. Basically what this means is Ryan will be on a restricted diet, he may be allowed some things that a classic PKU person may not. We will know this as time goes on. We may have to "count" his Phe for the day and he may only be allowed a certain anount. He is goes for blood work once a month and sees his specialist and nutritionist. His levels remain within normal range. He drinks 2 bottle a day of his special formula and one bottle of milk.

I'm not going to sugar coat it...I had a really hard time accepting that Ryan has PKU. I was devastated. Here I was at home with my 2 beautiful babies and then one phone changed a huge part of our life. I constantly thought about it, it overtook me some days. I became extremely anxious. I started worrying about what he is going to eat in school or hard it is going to be to feed Katie things that Ryan can't eat. What's going to happen at birthday parties or family outings? As I started researching online and reading other families stories, I learned everyone felt this way and it will get easier. When we have children, all we want is for them to be happy and healthy. Well, Ryan is healthy and he certainly is one if the most happiest babies we know!

UPDATE:

Ryan is 15 months old and doing great. His Phe levels are between 2-5 and he is maintaining. His diet consists of lots of fruits and veggies, pasta and lo-pro veggie meatballs and ravioli. He loves cheese sandwiches and on occasion he is even allowed a yogurt. Because Ryan is hyperphe we are able to try higher protein foods like regular bread and pasta. He loves waffles and Trader Joes sell wheat free/dairy free waffles which happen to be lower in pro then regular waffles. Some days are still hard for me. I find that if I start to think too much about it, I tend to get overwhelmed. I do know however that PKU is a manageable condition and we are all doing great.

If you would like to learn more about Ryan and his twin sister Katie, check out our blog at Double Serving of Love.

Hello from Oregon!

2008-09-15T15:31:00.002-04:00

We have been following this blog for a while, but haven't posted to share our story. We originally found all of this because my husband started a blog for our son Owen. You can find it at itsowen.blogspot.com

After our son was diagnosed with PKU we started talking about it on our blog and somehow we stumbled acrossed a blog about another little boy with PKU also named Owen.

The last 10 months have been a wild ride, though not all due to PKU. My son has had amazingly steady blood levels, but he has taken after my husband and is proving to be a very busy, thin little boy. We have had all sorts of trouble getting him to gain weight at the "normal" rate. He IS gaining, but because it is not the norm his diet keeps getting switched around. At first we were told since he hasn't gone over 360 (on a 120-360 range) as often as a growing child should without a formula increase, he is probably hyperphe. We even had a clinic visit where we were told to test his phe tolerance with all the food he could eat! (All logged and calculated of course!)This was quickly changed to 150 mg. of phe a day... The latest change has been due to his slow weight gain and we are now on 50 mg. of phe but with a rocket fuel concoction of milk. It includes Phenex-1, Similac and Duo Cal for the added beef-cake effect. Owen though continues to gain slowly. As a family we think the more calories we shove into him, the more he needs to burn it off.

We still hope to test that phe tolerance. After a fabulous week at PKU Family Camp I am feeling more sure of myself as a mother of a PKU toddler. I have no idea what new bump will come our way, but I am sure we will tackle it head on and do wonderfully. Owen is an amazing little boy, growing up faster than his mother would like and we adore him.

How to Calculate Phe/Protein

2008-08-31T22:23:00.001-04:00

To derive phe from protein: multiply protein by about 30 (for fruit), 40 (for vegetables) or 50 (for grains like flour, etc.). The label gives only a rounded protein amount, so to be more safe in your estimates, you should add 0.5 to the value on the label. For example, if you have a food label from a baked product where the first ingredient is flour and the protein is 1 gm per serving, you would multiply 1.5 gm protein by 50 (=75 mg phe) to get the maximum amount of phe this product probably contains. If you have a food label from a fruit-based product that says 1 gm per serving, you would multiply 1.5 by 30 (=45 mg phe) to get maximum amount of phe. If you are using an exchange system, you would need to convert phe to exchanges.

This is a gross estimate of the phe content of a food. To be more accurate, you would need to have protein content of the item to one decimal place rather than the rounded figure.

(this came from the Listserv - from Virginia Schuett - thought it was a good thing to have on file)

Not just milestones- Historical Markers

2008-06-09T14:22:00.006-04:00

If you've read my previous blog, you will know that Katy was diagnosed with PKU at the age of thirteen months. By then, she was having six seizures a day. The hope for her future was very grim. But I am a firm believer in miracles, and I understand that God made me her mom for a very good reason. Katy was given a 95% chance of severe brain damage. She was diagnosed (among many things) with West Syndrome. Most children who suffer from this condition learn to hold their bottles at five years of age, and some dont even learn to walk or talk.

I decided that one way or another, I would get my daughter back- the way she was handed to me- healthy and whole. Wether it would be through an unexplainable, supernatural miracle, or through hard work to aide the neuroplastic process, I would have my miracle. I would fight.

But, like anything that is worth sweating, crying and bleeding over- a result has started to yield. Katy is making a comeback, and deep down, I knew she would.

I've just returned from Carls Jr.- a fast food restaurant. Inside, they have a little play center for children. Time after time, I have taken Katy there with the hope that she would catch on and play. At first, she would just stand there in a fright. She didn't get it. Children zipped past her, shouting and screaming in glee. She didnt even bother to look at them. All she ever did was stand still- waiting to be rescued from this strange place. After much coaching and insisting, she finally sat on the little floor and banged on it. I would get inside the toy and try to teach her to go up and down the slide. I guided her arms and legs through the toy, and she almost enjoyed it. As long as I moved her body through it. This went on for months and moths.

Today we revisited the place.

Katy took to the game almost immediately. She fluttered up the little steps and scooted all the way down the slide. Once, twice, three times- who knows- she kept going and going for half an hour. The children were no longer zipping by, she was keeping right up with them. Every now and then, she would stop a child and turn him to her face. It was almost as if she was saying

"wait a second... how come you're short like me?"

And to think that not even a month ago I was told by a psychologist that Katy could not engage in social play. This day marks a new era in Katy's life. It says to the world:

YES I CAN.

And now she is saying her first word. Here is the video:

Going Lo-Pro

2008-06-04T12:24:00.002-04:00

We introduced the Aproten ditalini to our almost 11-month-old son, and he LOVED it! We made it with the Cambrooke Shake N' Cheese, and it turned out great. Tasted just like the "real" mac n cheese. He also got to try out the Cambrooke butterscotch cookies at the Walk for PKU Research in Cincinnati last weekend, and those were a hit! He's also liking broccoli and bananas.

Phe-Counter and Some Gooey Cupcakes

2008-05-18T23:02:00.003-04:00

My first stab at PKU friendly cupcakes! I baked them this weekend, I'm practicing for Owen's 1st birthday!
They were a little gooey on the outside, but tasted just like Devil's Food! Just like the recipe said....I'll post the recipe on PKU recipes later. I just made it from the Cambrooke website using their all purpose baking mix. I gave Owen a sample taste, and he liked them!
Just bought this cool wipe-board at Wal-Mart, so I can keep track of Owen's daily phe-intake. Does anyone else have a good idea on how to keep track?

Katy and her late PKU diagnosis

2008-05-08T22:55:00.006-04:00

My name is Michelle and I have a daughter who was diagnosed with PKU at thirteen months of age. We are missionaries in Mexico which is where my daughter, Katy, was born. PKU screenings were not standardized in the country, and she went undiagnosed as we left the hospital with our bundle of joy.

Things seemed to be wrong since the very begining, Katy was in constant pain and cried most of the time. I was breast feeding, and thinking that perhaps she was intolerant to spices and dairy, I kept my diet to rice and dry chicken breasts. Katy developed a umbilical hernia, and a few months later dislocated her elbow (due to low muscle tone). Her hair began to lighten and she slowly drifted away from us.

It was at six moths of age when things started to get real bad. Not only was she missing her milestones, but she was losing the ones she had accomplished. By the time Katy was eight months old she could not roll over, reach for a toy or even hold her head up. Then, her seizures started.

It took a while to identify her seizures, because they weren't the "typical" grand mal seizures one would expect. They were infantile spasms, or west syndrome which manifest in the form of short spasms, very much like the kind newborns do in the middle of their sleep. After a few months, they became worst and we started to worry. Having seen a whole team of doctors, we wound up in a neurologists office- where Katy was diagnosed with epilepsy. Not just any epilepsy, but West Sydrome and a 95% chance of brain damage. The search for its cause began.

It was five in the morning when it all sunk in for me. My husband and I harldy slept as we cried together for the lost future of our only little baby. We had planned and hoped for so long! We had waited through a horrible infertility crisis. I nearly lost my life giving birth (emergency stroke operation was done one me). Katy was our world, our life, our herriatage! All we had strived for with sweat, tears and blood- was now being snatched right from under us and there was nothing we could do to stop it. All we could do was stand over her crib and watch as she convulsed yet again.

Then my husband made a stand. We would not bow our heads in defeat. Our daughter needed us to believe in her. Her future depended on it. She was not just another number or another statistic. Katy was as unique as her figerprint. And we would carry her through and out of this mess.

Many tests were done and much blood was drawn. My little trooper turned into what seemed like a science experiment. After many months, we were down to our last two options: an aplified amino acid test and a mithochondria biopsy. We did the first of the two- which proved a positive for PKU. It was relieving not to do a muscle biopsy on our little angel. We set up an appointment with a genetisist in the US. In the mean time, we starved our daughter for a week, until we could get our hands on some PKU formula.

Katy's phe levels were at 19. Much too low for epilepsy, according to the genetecist. He assumed her condition was much worse than a classic case of PKU. Co-factor deficiency tests were run. There had to be something else provoking the epilepsy.

"Your daughter may not even walk by the age of 5," the geneticist informed

The world spun right under my feet. I had to hold on to a desk for balance. Even then, in that aweful moment, I clung to my faith. No matter how hard, I would not lose hope. I had promised that to my husband. To Katy. To God.

The formula finally arrived. It tasted aweful to my daughter and she hardly drank any of it. I was crushed. It was all the food I could offer her. It was precisely then that her blood tests were taken. The genetisist was confused. There was no co-factor defficiency. For a one year old with an unrestricted diet (chicken, rice, beans, milk, oatmeal and more), Katy's levels were much too low for CPKU. What was even stranger was that her tyrosine levels were twice as much as she needed them to be.

"I dont understant why," her genetecist said "but there seems to be some kind of battle going on in her mind. High levels of tyrosine are protecting her from the high levels of Phenyl alanine."

What came later was even more astounding. Katy's epilepy simply vanished. It got wiped off the map. The neurologist couldnt explain it. He said that this form of epilepsy, even if controlled, would still manifest on an EEG until childhood. Katy's brain showed no evidence of having ever suffered from epileptic seizures!

After that, Katy begun to catch up. Each milestone led to another- and another, Today, she is two and a half years old and we are in the process of looking for a school for her. She is still a bit delayed, but watching her grow. Seeing her strength and determination, there is no doubt in my mind that Katy will come out of this with flying colors.

As of today, she walks, runs and understands and follows instructions. We're still waitng on the speech, but don't worry. That will come too. She is happy and engaged and has a mind all of her own. I used to feel sad about her genetic mutation, but then I realized: if chromosome 12 been composed of a healthy PAH gene, she would have been a different child. It took this precise genetic combination to make up who she is.

Unique.

Miraculous

Strong.

I'll keep you posted.

www.michellehays.blogspot.com

www.katyhays.blogspot.com

www.youtube.com/michellehays

her conception story----> http://www.cci.org.mx/index.php?option=com_content&task=view&id=123&Itemid=51

Jiselle Elizabeth's Baby Food

2008-05-08T15:53:00.003-04:00

Jiseul Elizabeth is now 6 months old. She was diagnosed with PKU and my whole world seemed to cave in that day. But we took it one day at a time, and here we are. She's crawling now, and even cruising a little. We started solids a couple of weeks ago, and her PHE allowance for this week is 30mg.

I called a couple baby food companies and here is what I found.

Gerber
They're kind, understanding, and if you ask them what the PHE content is of certain foods they will tell you. BUT so far they don't have a booklet or list that they are willing to send...

Beech-Nut
They are awesome!! Kind and understanding, and they even have a booklet that they said they would send to me in the mail with a list of the PHE content in their foods. They also said they would send the booklet to my sister in law because that's where we'll be spending the summer~! VERY VERY COOL!!!

Earth's Best
They were the worst. They were not very kind, and proceeded to tell me that aspartame is not in their foods so not to worry, and they didn't have a booklet and were not willing to find what the PHE content was in any of their foods...

Parent's Choice
Was kind ad said they would call me back...

As soon as the book arrives I will update once again~

Owen - food update

2008-05-07T18:07:00.000-04:00

Owen has been introduced to the low-pro pancakes (cambrooke mixquick variety) - he loves them. We've also tried some baked fries - just sweet potatoes and regular potates, sprayed with pam, and sprinkled with season salt. Any other ideas?

Food! For Starters....

2008-01-26T21:20:00.000-05:00

I know I'm one of the only ones posting here (if you want authorship rights, just send me an email to heidisrecipes@hotmail.com ) but I'm trying to document our experiences as they happen. Feel free to do the same. Owen began on rice cereal, and has moved up to Gerber 1st Foods. He's had applesauce, bananas, pears, green beans, carrots, squash and sweet potatoes. The fruit is not his favorite, but he really likes the green beans, especially mixed with rice cereal. He's still breastfeeding about 4 times a day, and has 2 bottles of Phenex per day. He's growing and progressing just like a normal baby, and we could not be more relieved and happy. He is such a joy!

Just an update

2008-01-11T18:57:00.000-05:00

Our son, Owen, is doing great. His phe levels have been in the "normal" range, between 2-6, every weekly test we've done, with the exception of 1 test at the beginning, while we were adjusting his diet, and one time, when he was in the hospital with a nasty stomach bug. 6 months after his birth, we are feeling much more confident as parents, and have comed to realize that PKU is NOT that bad. There are so many worse things, and we are happy to be able to have a child with something that is treatable. We are especially thankful for the listserv, and our medical/diet caregivers. Owen has started rice cereal and will begin fruit and veggies soon. Then I'm sure we'll have more challenges, but we wouldn't trade him for the world!

Good Lo-Pro Recipe Sites

2007-11-11T08:18:00.000-05:00

http://home1.gte.net/magol/page23.htm

http://depts.washington.edu/pku/recipe.html

http://www.lowprotein.com/

http://www.pkuil.org/RecipieIndex.htm

A PKU Blog for PKU Families

2007-07-25T23:19:00.000-04:00

Our baby Owen was diagnosed with PKU July 20, 2007. It was a very stressful time, but we have been helped by so many wonderful people. We are starting this blog to have a place for PKU families to share your stories, research and recipes. If you would like to become a contributing author and post your story or information about current research, leave your email address in the comments, and you will receive an email invitation.

If you would like to contribute any recipes you have tried, I am going to begin organizing them in the link you can see on the right "RECIPES". Click on the link, and once I receive recipes, they will be organized by category. Please send recipes to heidisrecipes@hotmail.com, and include any comments about the recipe, your first name and how you have been affected by PKU, and the nutritional information of your recipe (if available). Eventually, I am hoping that this site will be a great resource for all of us. Thank you for your contributions.