Tuesday, February 26, 2013

PKU ~ A Day in Our Life

Last week my youngest daughter crossed a threshold from toddler into preschooler and I spent most of her birthday remembering not the day of her birth but the defining moment when she was 7 days old. Perhaps you've read that story HERE so I won't repeat it but it was a terrifying time for us as a family. We didn't know what PKU would mean for us and for our lives and most of all what that would mean for our precious Carolyn.

Here we are 3 years later. Carolyn wakes up in the morning and comes to snuggle and promptly announces she wants her "milky" but before I'm even up she's off again playing with her older sisters- somedays she's a horse named Cookie and some days she's a kitten named Saraphina. Every day is full of laughter and fun. And most days a bit of mischief too. So after her morning "milky" (her PKUspecial formula that she will be on for the rest of her life) we all sit down for breakfast- she enjoys vanilla flavored cultured coconut milk and raspberries. She also enjoys a specialized PKU porridge that took me forever to figure out how to make (I boil water and let it sit for about 5 mins then add about one cup of water to 62 grams of PKU porridge and stir for 4 min). Some days we special pancakes that come from a special pPKU pre made mix. And some days she has her special PKU toast with Biscoff spread. I was so worried when she was born that she would never be able to sit and eat with us- because she would long for what we were having and not have anything at all. Instead the opposite is true- most days her sisters wish they had PKU because they think that her food looks more interesting than theirs (although when I've ever let them taste something they say it is "yucky" so we now have a stricter no sharing policy and a severe no saying "yuck" policy!). She doesn't cry because she is hungry- she cries because i make her sit at the table to finish her food! After i re-weigh what she didn't eat and log her food and plan what food she will need for the rest of the day to reach exactly her phe requirements and her calorie requirements then our day really begins.

I homeschool our children and the reason I started was actually out of fear. You're not supposed to admit that but I was afraid that Carolyn would not be able to have PKU and thrive in the school system. I was worried she would get a cookie from a parent helper or be bullied because her "milky " smells weird. So I pulled our oldest out of school and have never looked back. We love homeschooling and we're all thriving in so many ways. Today though I don't have that same fear- at barely 3 years old whenever someone gives her anything she asks if it is "low phe" or "high phe" and if they do not know the answer she asks if they have weighed it and if not then she will not eat it. In fact one day I was feeding her and absentmindedly put my spoon from my plate in her mouth and she wouldn't close her mouth- I quickly realized what I had done and took the offending food out of her mouth and she promptly said " I don't want to get SICK mom!".

So our day begins with a bit of reading and puzzles (she can do a 24 piece puzzle at this point) and then either play doh or beading or coloring while I help her older sisters. She does a computer reading program a couple of days a week. She is amazing. She works on writing her letters and colors better and better every week. She is a bit of a handful sometimes- she has lots of spunk and always thinks she is right and doesn't always listen to rules and is sometimes openly defiant. There are times when I cannot find a consequence or a bribe for her to change her behavior and I wonder if that is PKU related. She's sometimes shey- even with us- and I wonder if that is PKU related. I suppose I will always wonder. She is gentle with her dolls and loves them very very much - but she also is in love with tractors and big trucks and likes to wrestle and play farme (which is interesting as we have a houseful of girls who are all very girly and so she's had to find this path on her own). She is the most empathetic child I've ever met and is always there to kiss it better and make people get along.

We have lunch and she's started to eat soup (although she doesn't like it) or sandwhiches with her special PKU bread and lots of veggies or sometimes for a special treat - french fries! My daughter has a tolerance of 250mg of PHE per day. It is one of the lowest tolerances in our area. I was terrified there wouldn't be anything I could feed her- but there really is. Her favorite is PKU pasta and Daiya Cheese Alternative. And of course more of her "milky"!

After lunch she still has a nap- and I generally can gauge her level of PHE in her blood by how well she sleeps both at naptime and during the night. When her levels are high she simply doens't sleep- she'll fall asleep for a few moments and then wake up again- she won't nap and the combination of her feeling unwell from her high levels and her lack of sleep makes her difficult. She's uncharacteristically mean, will destroy things, have tantrums and generally be angry and defiant. Thankfully these times are rare. Most of the time we have a change in levels it is becaue she's been ill. Another PKU mother once suggested that i manipulate the calories and PHE on days she is ill and since then it has been much better. They need more calories when they are ill in order to function.

After naptime we might head out to swimming lessons or go to for a playdate or go shopping. She is very social and while she is sometimes very shy as well she thoroughly enjoys other children - and plays well with them. She 's starting to play real games like Candyland and she somehow always wins.

For supper she eats only vegetables and has her  "milky" so we are always careful to make sure that we all share the same vegetables as often as possible. There are many families who take the stance that their PKU child must learn to be different from an early age so they do not provide the same kinds of food whenever possible. I take the opposite stance. I believe that PKU is a big deal and we talk about it a lot- we talk about how many calories and how much PHE she's eaten and if she wants something that is higher PHE we negotiate on what she'll need to eat that is lower phe for the rest of the day. But her plate looks similar to her sisters whenever possible!

By the end of the day it is time for bed and she pretty much gets herself ready, we read a story and thank God for our blessings and for the fact we live in such an awesome country that provides access to special food and formula to help her thrive.


  1. Carrie, Carolyn has truly been blessed with the best parents that she could have been born with. For anyone who doesn't know this family they are very close knit, yet always part with you. You have taken the time to educate yourself and with it us too. Thanks Carrie for sharing, and by doing so strengthening other parents who will be, or are faced with PKU children. You have given a voice to children, reminding us how awesome they are.

  2. I just found your blog, which is great! My husband and I recently adopted our 2 year old daughter from Beijing and she has PKU. Your "day in the life" looks similar to ours. We adopted her in December 2013 and are still doing weekly blood draws to ensure she has the correct prescription. I look forward to keeping up with your posts.