Monday, September 29, 2008

Apples for a sweet New Year

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.


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)

Tuesday, September 16, 2008

Our Story

(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!
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.

Monday, September 15, 2008

Hello from Oregon!

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

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.