Tuesday, August 9, 2011

Our new reality

We had only been home from the hospital with Avery for one whole day when three stupid letters changed our lives forever: PKU.

The pediatrician gave us very little information when he called to tell us we needed to go to Primary Children's Hospital for additional testing.  When Kyle asked him what PKU was, all he said was that it can cause brain damage.  You can maybe imagine how we felt.

We couldn't get in to the hospital until the next morning and I spent the whole night worried and scared.  We had no idea what PKU was but the thought that something could be wrong with my perfect, tiny newborn was absolutely devastating.  I kept praying that maybe it could be a mistake.

At the hospital we met with a geneticist and dietician who explained that because of her initial PKU test results, Avery was going to need treatment right away.  I thought we were just going in for additional tests, but that wasn't the case.  I totally lost it.  I cried the whole time we were at the hospital while trying to make sense of what they were telling us.  I don't think I've ever felt so heartbroken before.

She was only 5 days old and I felt cheated and angry and vulnerable.  I felt cheated out of enjoying every moment with our newborn without worrying about her health and her future.  She changes so much every day and instead of soaking in her 5 day old cuteness, I was emotionally shattered and cried pretty much nonstop.  I was so angry that this had to happen to her, and to us.  Angrier still that, because PKU is a genetic disorder, every child we have will have a 25% chance of having PKU.  Angry that everywhere I looked were people with "normal" children.  And I felt vulnerable as if now anything could happen.  As if something else could go wrong at any moment.  It was like we had a perfect shiny bubble of carefree happiness when we left the hospital and it was completely destroyed with the news about PKU.

Avery will have to deal with PKU every day of her life and that pissed me off.  I haven't been a parent long, but it's been long enough to understand that parents want to protect their children from everything possible.  There is enough out there already for her to worry about in life and the thought of her working every day to manage this disorder wasn't fair.  And it made me mad.

The pediatrician was right: PKU can cause brain damage.  But what we quickly learned is that it is also manageable through diet and that brain damage is totally preventable.  "Individuals with PKU cannot process the amino acid phenylalanine, which is present in most foods. Without treatment, phenylalanine builds up in the bloodstream and causes severe neurological complications, including mental retardation. The standard treatment consists of a strict diet very low in phenylalanine and daily consumption of special formula containing life sustaining nutrition. If the diet is consistently and strictly maintained, individuals with PKU will develop normally." from here

What this means for us is right now Avery alternates between a special (very expensive) formula and breastmilk every day.  When she starts regular food we will have to weigh everything she eats and record it.  She will never have foods high in protein like meats, fish, eggs and dairy.  We also have to prick her heel regularly (right now it's twice a week) and send blood samples to be analyzed to check her phenylalanine levels.

Avery is now 3 weeks old and I think I have come to terms with all this PKU stuff.  I expect that it will become difficult again when she starts baby food and again at other milestones and when she starts school and when she goes to a friend's birthday party and things like that.  But for now, I have accepted that this is our new reality and our lives will never be exactly the way I had expected they would be. 

Even if God had told me before I got pregnant that Avery would have PKU, we would not have changed anything.  We would have still had her.  We love her more than anything and wouldn't trade her for the world.  Some of my saddest moments in dealing with this are thinking that she will never have an ice cream cone or chocolate or a juicy steak or a hamburger.  It sounds so silly to even think that but food is such an important part of our culture (and especially my life!) and it is going to be very different for her.  How will it be when she gets invited to a pizza party?  Or to a friend's house for dinner?  Or gets asked on a date to a restaurant?  Or when she goes to college? 

In worrying about her future one night I had the impression that Avery was born with the strength and ability to deal with this and that we need her more than she needs us.  It scares me to think of the future and all the ways PKU is going to affect it, but the most important thing is that Avery will be fine.  She will be healthy and unlimited in anything she wants to do in life.  And as much as I wish I could take the PKU away, I am grateful she does not have anything more serious or life threatening and I am grateful it was detected before it could be damaging to her.  Most of all, I am grateful we have her.  She still is our perfect, tiny newborn and slowly our shiny bubble of carefree happiness is being put back together.  Even if PKU has to be a part of it.


More about PKU:

20 comments:

Crystal said...

You are such a strong woman, and I know that Avery is going to be, too. You sure are lucky to have each other. Love you.

Suzi Q said...

You guys amaze me. I love you and Avery.

Derek and Jen said...

Avery's lucky to have you both for parents! And you're right, despite the difficulties in the diet area, she'll be able to do whatever she wants to in life. Our thoughts and prayers are with you! Oh, and we still need to get together!! When life calms down a bit, let me know when we can stop by!

Megan B said...

I used to work with a woman with PKU, and she has been able to live a very 'typical' lifestyle :) I remember her saying that nutrition became very routine. I'm sorry you and Avery have this struggle, but with a strong woman like you as her mother, she will be fine. Love you so much.

Rich and Brianne said...

Good luck figuring this new challenge--you'll do great. Love to you and Avery!!

James and Laura Neibaur said...

You seem to be handling everything so very well! You are good people and good parents, and Avery is obviously very special and very loved. We didn't get the "normal" baby experience either with our first, and it is hard "looking around" at those times, isn't it.

Tyandra Perez said...

I'm so sorry about little Avery. I wish you guys the best with all this and I know you will be able to care for your little angel in a way that will keep her healthy. The Lord sent her to you guys for a reason! Keep up the great attitude!

Jaclyn M. Howell said...

Well, i've spent the last 10 minutes bawling my eyes out at work. I love you guys and if anyone can handle this challenge, its the two of you. We send all of our positive thoughts your way and will keep you all in our prayers as you come to terms with this unwelcome surprise. Keep us updated! <3

Kim said...

Oh, Kourtney! I am so sorry that you were faced with such a demanding hardship so early in Avery's life. I love you and your family and our prayers are with you. I am amazed at your optimism and strength when we talked last week. You continue to be a wonderful example to me.

stacy said...

Sister! Did you know that Sister Robison from our mission had PKU? You should message her on facebook and ask her about it.

Jen said...

Kourtney, sorry to hear about the news but I'm sure everything will be ok and you guys will figure everything out. in time, the diet and other things will become routine and Avery will have a wonderful life. Prayers and love coming your way!

It Started With a Wink said...

I was going to say Sister Robison had it, she was my companion, every once in a while she could have stuff, I'm not sure how it all works!

Kellie said...

Sister Ashley Robison has PKU. I know she faces challenges because of it, but she is a strong, incredible woman. You should contact her. I'm sure it would be nice to talk to someone who 'gets you'. Your baby is so lucky to have a mom like you to love her SO much!!!

vickyj said...

This was so beautifully written, Kourtney. I've known of your heartache and pain from the very moment of that fateful phone call. I've prayed for understanding and acceptance for myself as well as for you and Kyle. We are all grateful for Avery, and for our faith that Heavenly Father will not give us more than we can do. I believe Avery had an opportunity to choose you as her mother knowing you could help her the best. May the love of your family and friends, and the hope and faith in your heart lift your spirits and carry you through each day. I love you so much. Mom

Nikki said...

Kourtney, you are so capable and resourceful you will have the diet and the Whole Foods market figured out in no time. You will be amazed how quick you adapt. Your beautiful Avery will be so thoroughly wonderful because she has a mom who will learn everything and be creative about making your lives work well

Chelsea said...

I'm so sorry to hear this! Sometimes life throws us curveballs when we least expect it, and it's hard, but we manage. Keep your chin up! I'm just so glad that the doctors were able to detect it before she suffered any permanent damage, and that she'll grow up just like every other kid - just without a few certain foods. You'll get past those pizza nights and birthday party hurdles just fine. :)

~Chelsea

Jen said...

I definitely don't feel like I have any sort of words that will give you comfort, but trust in our Heavenly Father. He will help you, Kyle, and Avery figure out a new way of life for her.

Tara said...

I just came on here to say my heart hurts for you. Not because she can't live a wonderful life (Sister Robison is a GREAT example of this) but because as you said, every parent wants to give everything to their child and protect them from everything. She has been blessed with a great mummy who will be able to give her the strength she needs along with her Father in Heaven.

Marla said...

Kourtney,

I agree with your mom, this IS so well written! You are amazing and will do a wonderful job with beautiful little Avery. Yes, it will be a challenge,and it does not seem fair, but-I know with your sense of humor and Kyle's, along with all your love for her, you will raise a wonderful child. So far, you have been a great role model for so many! We will always have a special place in our hearts for you!!

Jessica said...

I just found your blog. My husband and I have an almost two year old PERFECT little girl with PKU. I, too, remember that life-changing phone call... The moment our world broke. It stopped Turing around this newborn sweetness and started revolving around three stupid letters that no one had any information about. Positive information that is. Your post brought back so many memories... I thought the transisiron from baby food to solids would be the hardest for us, but our toddler is VERY picky... So her levels have stayed very low. Glad I found you!