Unanswered Prayers

It's hard to believe that this girl is 4! When we first found out Breeze had PKU, the dreams of the future became clouded with fear and uncertainty.This unknown 'scary' thing indeed rocked my world, some hard, but now, looking back thus far, I can see it has filled our life with more blessings than hardships.

I am so,so, thankful. 

I have an amazing support team at The Children's Hospital, that has given me amazing opportunities just in this year alone! I've  gone to an all women's camp, rode my bike over mountain passes and now to a low protein cooking class in Napa valley. As a Mom I've learned we all have our struggles, all we can do is our best with what God has given us, and support each other through it. 

Breeze and I have new friends!

 I have met amazing PKU moms who live minutes from me! (Which really is such a blessing) and Breeze has fellow PKU friends.

She can learn from this disease

I hope for Breeze to learn that PKU is not something that defines her or holds her back in life. But sees it as a a spring board to embrace all life has, because, it is in fact a gift. I hope she learns to use it as an opportunity to give empathy and compassion to those around her. We all have struggles in life, it is how we use those struggles that makes all the differences.

I can learn and teach(hopefully!)from this disease

 I am blessed beyond measures all the time by something that is a struggle. I hope as I Mom I can teach my children many things, but one thing for sure. Always thanking God for the many blessings in life, but probably mostly, for the unanswered prayers.

'Welcome to Holland' In light of National PKU day

Being a first time Mom is foreign enough. But to also find out your child has PKU, it was news no parents are ever prepared for. Today 3 years later PKU is part of life. Is it still hard, hell yes, but I like many other PKU Moms out there, are learning to 'love Holland'

WELCOME TO HOLLAND

by

Emily Perl Kingsley.

c1987 by Emily Perl Kingsley. All rights reserved

I am often asked to describe the experience of raising a child with a disability - to try to help people who have not shared that unique experience to understand it, to imagine how it would feel. It's like this......

When you're going to have a baby, it's like planning a fabulous vacation trip - to Italy. You buy a bunch of guide books and make your wonderful plans. The Coliseum. The Michelangelo David. The gondolas in Venice. You may learn some handy phrases in Italian. It's all very exciting.

After months of eager anticipation, the day finally arrives. You pack your bags and off you go. Several hours later, the plane lands. The stewardess comes in and says, "Welcome to Holland."

"Holland?!?" you say. "What do you mean Holland?? I signed up for Italy! I'm supposed to be in Italy. All my life I've dreamed of going to Italy."

But there's been a change in the flight plan. They've landed in Holland and there you must stay.

The important thing is that they haven't taken you to a horrible, disgusting, filthy place, full of pestilence, famine and disease. It's just a different place.

So you must go out and buy new guide books. And you must learn a whole new language. And you will meet a whole new group of people you would never have met.

It's just a different place. It's slower-paced than Italy, less flashy than Italy. But after you've been there for a while and you catch your breath, you look around.... and you begin to notice that Holland has windmills....and Holland has tulips. Holland even has Rembrandts.

But everyone you know is busy coming and going from Italy... and they're all bragging about what a wonderful time they had there. And for the rest of your life, you will say "Yes, that's where I was supposed to go. That's what I had planned."

And the pain of that will never, ever, ever, ever go away... because the loss of that dream is a very very significant loss.

But... if you spend your life mourning the fact that you didn't get to Italy, you may never be free to enjoy the very special, the very lovely things ... about Holland.

Being sick

So valentines week Breeze came down with a fever. I feel very lucky that we hadn't experienced a high fever before, but it's scary non the less. I had done my homework and read a lot about them and tried to keep reminding myself that fevers are good, they are helping the body heal, and that her body won't be able to heal as well if I brake it. But that advice is very hard to follow! I did give Motrin 3 times over the course of 4 days, after her tempature was 103+. I do feel confident that her immune system did a great job though. I do many things to help build her immune system so for that I am thankful( if your interested in what just ask!)

So as with any person who is sick, Breeze didn't feel like eating or drinking. To most this is not ok to do, but it is a lot worse for PKU folk. When her body had a fever it burned itself, burned protein. Making her levels go up. 

So for the first week I was taking care  of a feverish, sick baby, trying to keep her hydrated and not think to much about the fact that her levels were going up, she was not drinking all her formula nor was she eating. It was rough. But she never did stop eating or drinking so that's good, and a lot of time she wanted water. Which, I know is great, but for Breeze her formula is better. But I am glad she drank it and she got it as much as she wanted.

Once her fever broke I was focused on getting her levels back in check. I think the second week was harder than the first! 

The second week of healing I experienced a  two year old dose of what high phe levels look like.  Yikes! I will say this, this week, Breeze is a little angel, yep, I said it. My 2 year old is an angel. 

It was hard to get her to drink her formula. ( I even gave her one of Raven's bottles,once just once) So our lovely dietician advised us to try putting tang in her formula. It's phe free. So as of four days ago, Breeze drinks all her orange milk, happily. She gets a choice, which I think helps. 'White or orange milk?' I'm also going to get her some mios which are also phe free to give her more of a selection.

What do you do to help your kids immune system?

Traveling, PKU style!

A while ago while packing for a trip I thought it might be of some interest to some newer PKU Moms, and also my family and friends  what all goes into traveling with medical food, feeding a PKUer on the go, and how to plan meals with out being in the comforts of one's own home/ hometown, local grocers, etc. When you just can't go to the store and buy all the food for your child, it makes it a challenge. But I have traveled at least ten times since Breeze has been born, in many different scenarios. So here is what I have learned.

Food usually in my suitcase to check is low protein pasta, rice, cereal, crackers, veggie broth cubes, Molly Mcbutter, cassava chips, fruit snacks, vegan jello, formula, pancake mix, frozen bread dough, sometimes its baked(cook for love recipe, see link below), glutino pretzels, lo pro cheese(sandwich mate, or follow your heart, vegan cheese) biscoft, fake egg mix and of course a scale!

Typically I have a soft cooler inside my suitcase so on the morning of the flight I put anything frozen into it with some frozen cooler packs.  I also have a soft cooler that I carry on. I have her formula made for the day as well as any food for her during our travels. I also carry on an extra can of formula, just in case.

Also inside my suitcase and my carry on is a medical letter from children's hospital if there needs to be any explanation. Some foods we have found in airports that are ok for her to eat are french fries,  at Starbucks they carry pouches, freeze dried fruit and fresh fruit. Many restaurants will serve steamed veggie sides.

Before I leave I Google where the nearest Whole Foods or a store similar is located and try to make that a must hit ASAP, or I ask a family member to do it for us. I have so many to thank for doing that! Many of our travels have been to a city so there was a Whole Foods nearby. But last October there was not one so I had to trust a family member to do the shopping, one who never hangs out with us, the results were doable. The main reason why I prefer Whole Foods is because of it predictability in what they carry, for example, gluten free pretzels, the milk I use in her formula, so delicious coconut yogurt, Annie's fruit snacks,(they have no gelatin) coconut/and or rice milk, butter, sauerkraut, pickles. Plus I like the selection of organic fruits and veggies. But King Soopers or affiliate stores are pretty good.(and cheaper!)

I have also had food mailed to the place we were visiting. Unless you are staying longer than a week I would not recommend this option. Most of our travels have been to friends or family residence but a few times we were not so I recommend staying at a place with a kitchen, which with kids is just crazy not to!

One other thing that I have always done is have a way to contact fellow PKU Moms. I am a member of a Facebook page with lots of Momma's and I also have a local Mom I have called(thanks Michelle!). The reason for this is sometimes I cant find a certain brand, of say fruit snacks, I need someone to look in our Low Protein food 'bible' and tell me what brand to buy and how much phe per gram so I can calculate it.

There has been one time while staying in Florida by John's Crossing that I had a package meal of lo pro mac n cheese and I asked the manager at a restaurant to make it for me and explained a little why, but I didnt go into detail. It was a total mess, she argued with me about not being able to boil water, so I told her to microwave it.When she brought it out she told me it ruined her microwave and she would never do it again. Then she tried to tell me she was a nurse and that there was chicken in the food I was about to feed my two year old and that I really should feed her fish. I was so stunned that I think my jaw hit the ground. I wish I would have walked out right then and there. The name of the restaurant is Walts. Just to be clear, there was chicken fat in the meal, fat does not have protein in it, but the smart nurse didnt know that.

So that is a few things I have learned along the way, anyone have any tips to add? I have not done international travel so when that happens I'll let you know
                                           

No time for a 'Bottle'

Alright well it's been about a week since we have thrown away the bottle, literally Breeze threw it in the trash,and I think we have finally made it over the hump. 

    Last bottle ever!!!

This past week has been a struggle, I'm not gonna lie. I am glad I threw the remainder of the bottles in the trash as well because I am not quite sure I would have made it.

But I think that we have crossed over and I am so happy. I know it's so important for her to drink her formula, but on days that she was really refusing  to drink and eat I got so upset. Upset, that she has PKU, upset that I had to give her this liquid that is more important than food. There where moments I just got so frustrated I didn't know what to do. I realized on day two that with holding food until she drank was not the way to go. 

Breeze 1, Mom, 0. 

So then I started to feed her, meaning she ate food then I gave her her 'big girl cup' and slowly she started to drink, the less I stressed the more she wouldn't fight it. Have I ever said being a Mom is the hardest thing I've ever done? Well it is. I thank God everyday for my girls and for the patience to be the best Mom I can be. It's frickin hard.

This was a particular bad night. 

This is what was left at the end of the night

Party Time

Today we went to a birthday party for one of Breeze's best friends. Parties can be difficult for pkuers so I was hoping to bring along some lo protein food that was similar to what was being served. I asked the Momma and my challenge was crescent roll wrapped baked hotdogs and Mickey Mouse cupcakes. Food available that she could have was fruit, veggie straws and veggies! 

I decided to try a vegan recipe for the hot dog. It was a marinated carrot wrapped and baked in some lo protein bread dough. My bread dough recipe is from Cook for Love and it is delicious! I always have some bread dough in the fridge for easy access. For the cup cakes I used a bought mix (maddies) from the low protein food store at the hospital( a large storage closet with food on some shelves) for the ears, k- too gluten free Oreo type cookie from whole foods and a store bought frosting that is phe free.

She ate some of the 'hotdog' as well as the other things available. The cupcake looked super cute and I think she ate part of an ear and some frosting. ( Hers is the vanilla one) Regardless of what she ate I feel this party was a success!

There's a New Kid in Town!

It happened! We had another beautiful baby girl. Her name is Raven Star Porter. She was born May 29th and 3:54am (on her due date!) She weighed in at 6lbs 11oz and 19 1/4" long.  To answer the burning question, no she does not have PKU. We found out when she was 6 days old that her levels came back normal. I think that I was shocked, I was expecting her to have PKU. But what a relief it is. Raven has nursed like a champ since she came out and I really did not want to have to take that away, if even temporarily. I am so grateful and feel so blessed. Having  a non-PKU little baby and being able to do things "normal" has been like I am a first time Mom. It is not something to be taken for granted. I am relishing in every cry. every late night feeding in every poopy diaper.

Breeze is adorable with her and calls her 'Baby Sister awww' The aww part came in because if she would kiss Raven or hug her people around her would go awwww, and it stuck. Its pretty cute and I hope it stays around for a while. She is adjusting to sharing Mom as well. I just make sure to get lots of cuddle time with Breeze when baby sister is sleeping.

With Raven not having PKU its hard not to think about the issues that may arise with having one sibling with and one without. But that's for another blog in another year far from now. So at the moment  I'm just going to enjoy the many blessing that have come my way.

Time Flies

Holy cow where does the time go? I am at the moment, Mom to a 19 month old toddler with,as you all know, CPKU. I am also expecting another babe any day now, I am 38 weeks pregnant. In case you were wondering the chance of us having another PKU child is about 25%. I am fine saying out loud that I hope that this next baby does not have PKU. Some have asked, wont it be easier for Breeeze? My answer is this. Maybe, but why would I wish something on my child that will make their life hard just so that Breeze will have that in common with her sibling? There are plenty of other things they can have in common that can help create that bond that siblings have. I have tried to not think to much about it and will nurse in complete bliss until we find out the results of the newborn screen. I've been there done, done that and will just role with what ever happens. I'm sure I will cry either outcome.

Breeze is THRIVING. There is no better word to describe her. She brings so much joy into our lives. She has become quite the little talker putting three-four words together already. Her vocabulary is huge, and I am not just saying that cause I 'm her Mom ;) She took off walking two days after her birthday and has been running ever since. She loves to swing, look for airplanes and birds,watch her signing times videos, go to the park, play in the water, love on her babies and make people laugh. She looks at my belly and says" big ball" or as of recent, 'baby loud" After hearing the babies heart beat the other day at our appointment.

Breeze is not quite as good of an eater as she use to be but I think this is a common frustration with any Mom of a toddler. Its just a little more stressful on a PKU Mom. But her phe levels have still been good, if even slightly low. I stopped nursing around 14 months but had a freezer full of frozen milk so it wasn't until around 16 months that she was completely done. She now gets phe from food and whole milk that I add to her formula. To some whole milk may come as a surprise, but she cant taste it, so she is not acquiring a taste for milk and its better than giving her say 'real' bread that she would acquire a taste for. She eats a mixture of fruit, veggies, some gluten free products, lo protein pastas, rice, cheese, bread, crackers. Im always trying to come up with new ideas and new products to give her. I do feel like the more products she can buy from a grocery store the easier it will be for her to stay on diet when she is older. I am lucky and blessed to be able to spend a little more on food!

I will keep you posted about baby #2!

This girl can eat!

Adjusting to what Breeze can and cant eat has been slightly difficult for me at times. Processed food, what? Cheese like food, gross. I find myself thinking, "well that looks too healthy, she probably cant have that."Everything touts protein, protein, protein! Me get negative? Never!

So I have come to the conclusion(and a more positive one) everything I have control over, the food she can have, will be the best(to me that means organic, fresh, power foods). At around 10 months old it was getting difficult to get this girl full on just veggies and fruit, it was time to add lo-protein food(the filler) to her diet. It was time for carbs. So I bought some lo-pro pasta, crackers, cereal, made some bread, tortillas, pancakes, oh and she can have gluten free pretzels. I am blessed that Breeze loves to eat, but her food taste good, I make sure of that. I always try it first, if its not good to me, I am not giving it to her. I am not a picky eater and I like most things, but there have been a few things that she wont eat and I understand why!

Another thing is even if I have never had it, or know that I am not really a big fan, but its low in phe I offer it to her. One being sauerkraut,  Breeze would drink the juice if I let her! I also bought her some sea weed chips and she likes those as well! One thing I have found that it is easier to find sweet food for Breeze than savory/salty. So when I do find something in that area she tends to gobble it up. "Solids" was daunting to start with Breeze and took some work but I feel we are adjusting. 

Our dietitian has been great and supportive in allowing me to continue breastfeeding, and I have a great group that I am part of on FB and also a non profit organization called' Cook for Love' that has a ton of great PKU friendly recipes. I feel very blessed to be so connected to so many supportive Moms in the same situation even if miles are between us. I have connected with Three other PKU Moms in the Denver area with girls all around Breezes age that has been great as well.

Veggie Nuggets

Cassava Potato Latkes

Lo-Protein Bread

One Happy Eater!

One of those day...

Today has been one of those days where I wish I had another PKU mom friend to just talk with. It all started with my dream I had last night. I dont remember the details but the gist of it was basically the world as we know was ending and that included how I got Breeze's periflex formula. What would I do with out it? How would Breeze become if she didn't have it? These are very scary questions for me and this dream made me face one of my biggest fears. What if we could not get her formula? The "healthy" way I eat is not healthy for her, and today, that makes me sad and frustruated. I dont want to feel this way and I considered not sharing this post,but reality is, this is not always easy for me. Reality is, I get upset still, I get sad and it is hard. I have always wanted this to be a positive blog about PKU, but also I want it to be real. What is real? My daughter has PKU. There are lots and lots of foods for her to eat. She is really healthy and really happy, and,I have a very large basement to hold lots of periflex formula stores.


Side note

After reading this post, if you feel any sort of sadness towards me please feel free to read the post'Please dont tell me your sorry.';) I,just like any new Mom, have had one of those days. And I feel better after telling you all about it!

Patient Power

   I found  a great website called Patient Power. It has a lot of PKU (and other diseases) information. There motto is "Knowledge can be the best medicine of all."  Not only is it educational but also it has a wealth of stories about people with PKU. Another thing that is really great is that you can watch the story, read the story or listen to the story. So for my husband who refuses to read, this is especially great! I just read an interview between a young adult with PKU and a doctor who specializes in PKU. Its called

 PKU: 4 Keys to the Foundation for a Healthy Life 

 It was a great interview that gave two perspectives on raising a child with PKU.

" ...this is the way we check the phe, check the phe, check the phe..."

      Today is Wednesday. Blood draw day/ errand day.  The draw is almost always on Wednesday at anytime, she just can't have any phe, no breast milk, two hours prior. The tests are run on Tuesdays and Thursdays and take a couple hours to do and only like three people in the whole hospital are qualified to run the machine that test the blood for phe and tyrosine.Yes, my health insurance covers these weekly test, thank goodness. I have seen the bills and it adds up quick!Casey, the nutritionist calls me on Friday mornings with the results and tells me if there are any changes to how much formula she is getting.
     On Tuesday evening I begin thinking about the draw and trying to time her feedings based on what time I am going to be going to the hospital vs when to pump or nurse. So there needs to be enough formula to last her through  the draw but then I have to consider my timing of pumping to ensure if she gets hungry before 1pm I can nurse her. It's a juggling act, sometimes I drop all the balls. But most of the time it works out .
      So today i,t worked out.  At around 7:30am she nursed on one side and then around 8:30 she ate about 2 oz of formula. Then she went down for a nap. While she slept I did dishes, pumped and ate breakfast. Then when she got up around 9:30 we played, and got dressed she was not hungry and we still had about an 1 oz of formula to eat before 1. She went back down around 10:30 since her morning nap was so short. I finished getting ready and around 11:30 I put her in her car seat and we left for the hospital. The drive to Childrens hospital is about 30 mintues. When we get there I always park in the garage because it is free for patients. I pop her and her car seat into her stroller and we head into the outpatient lab, getting our parking validated along the way. At the front desk I fill out a sticker that has Breeze's name, birthdate and time, plus I write the   phlebotomists that I want. Kelly, Marissa or Justine. It is not Children's policy to let parents hold their children while they are drawing blood. To me this is ridiculous and I only go to these three because the let me hold Breeze. Gravity, hello? While waiting she finished up that last ounce of formula, about 30 minutes before one, just in the nik or time!When her name is called we head into the room and pick the opposite heel from last week. We place a special warming gel pack on her foot to warm it up, makes the blood run better. The phlebotomists runs the paper work and gets out her tools. I hold Breeze and she pricks her heel with this razor blade and then squeezes her heel until the tiny tube is full. Breeze no longer cries unless she is hungry or tired. She mostly just squirms making it difficult for all involved. Today she tried to look over her shoulder, as if to say "what the..." We may start drawing from a finger, but I am not to sure how I feel about that. She is always putting her hands in her mouth and people touch them and then she would have a open little cut..., we'll cross that bridge when we get there I guess. Then once the band-aid is on we head upstairs to get weighed. We either see Marevel or Amy. Both love Breeze and make her smile lots. I take off her clothes put her on the scale and then get her dressed again. Sometimes if I am curious we do a length measurement as well. After that If Breeze is hungry I either nurse her or give her a bottle, depending on what time it is. Today it was past one so she started on her new batch of formula, and another 24 hour shift begins

The front of Children's Hospital

This is Marissa, Breeze's eyes are glued to the TV!

The tools of the trade

Putting on the warming pack, eyes still in the TV

 

Drip by Drip

 

The tiny tube of blood

This is Marevel, she weighs Breeze

  

A boo boo

      

This part is a piece of cake!

The hall way to the weigh in we go to hall F
or where I sit afterwardsand feed her

When life gives you lemons, make lemonade

       I am the happy, fortunate mother of Breeze. She is the most wonderful thing that has ever happened to me.  Well, besides my husband Adam,without him there would be no Breeze(no pun intended!)
        Nothing about our parenthood journey has been traditional. We were unable to conceive the old fashion way. So with a little help from modern medicine we got pregnant on January 15th 2010(yes I know the actual date!). Breeze was born on October 9th 2010 after an amazing birth experience at Mountain Midwifery. We went in for the normal two day check up and Breeze had a little heel prick to take her blood for what is generally called the' newborn screening test'. Six blissful days later, the 'Happy New Mommy Bubble' was popped by a single phone call.
        A midwife nurse called to inform me that Breeze's screening for PKU had come back high and I needed to go to Children's Hospital that day to get a more specific test. I was holding it together pretty good until she told me this could interfere with breastfeeding. Until that day I had not heard Breeze really cry,the pain she felt broke my heart into a thousand pieces. One good thing about a blood draw is when they are over, so is the pain, I think that Breeze recovered quicker than I did though.  I'm not sure I will ever fully recover from learning that my child has PKU,  although she is and will be a perfectly normal child.

 I'm not sure I will  recover from the fact that I have a child who has a disease.

 I'm not sure I will ever recover from how blessed and lucky I feel that she has a disease that is treatable.
   
        I will never fully recover from the fact that God chose me to be this little girls Mother. Chose Me, the one who hates to do dishes, but will wash bottles and nipples all day long. Chose Me, the one who hates hospitals, but will take her there to get her blood drawn once a week. Chose Me, who is all about organic and non processed food, will make formula and processed lo-protein food for her and tell her"this is a "Yes Food" So, so far nothing about this journey has been what I would call the traditional experience, and I can honestly say now, I would not have it any other way.

With this blog I am hoping to be a source of information to my family and friends and possible other PKU Mom's about a day in the life of Breeze, our precious child who is made even more special and rare because she has PKU. With a little extra info thrown in as well. Enjoy!

Boy or Girl? We did not know...

What is PKU? Good question

PKU stands for phenylketonuria. Don't ask me to pronounce it cause I can't. PKU,for short, is a rare genetic condition that babies are born with and will have their entire life. Adam and I both had a recessive gene and had a 25% chance of having a PKU baby. No this is not something we knew nor did any of our family know anyone who had PKU in the past. So yes we have that same chance the next go round.

Breeze has what is called the classic case pku. She has an enzyme that is deficient and does not break down the amino acid phenylalanine (phe, for short) to another amino acid, tyrosine. Failure of this conversion results in a toxic build up of phe in her system. The deficiency in every child is different and so treatment is individualized. We have a nutritionist that I work closely with on a weekly basis. Once a week I take Breeze to the children's hospital and get her blood drawn from her heel, which by the way does not even phase her anymore.Then we go upstairs and get weighed. Two days later Casey, the nutritionist will call and give me Breeze's levels. One for her phe and another for the tyrosine. The goal is to have her phe levels be between a 2-6. A normal persons levels are at zero. Her phe levels at the time of her newborn screening they were an eight, a eight days later they were 34! The goal was to get her levels back into range as quickly as possible, within the first month of life. If levels are in check in that time then there is no brain damage that will occur. She was temporarily taken off breast feeding and put strictly on a phe free formula called periflex. This was so difficult for me. Adam gave Breeze her first bottle that day and I cried and prayed she would come back to my breast when she was allowed. She took to the bottle with out any problems and I pumped every time she ate to keep my milk supply up. Her levels dropped beautifully and with in a week and a half I was able to start nursing again. She came back to me with gusto! It still makes me sappy just thinking about it. So from then on we give her a prescribed amount of formula and then I breast feed after that with in a 24 hour time segment. We go from 1pm -1pm. PKU is not something she will grow out of. The amount of protein she is allowed to have is minimal, but there are lots of yummy options and alternative foods out there. Plus Mom and Memaw love to cook. She will have to drink a special formula her whole life in order for her to get all the other proteins her body needs as well as nutrients.  The risk for brain damage subsides as she gets older and more developed. But if her levels do get high when she is older, high being a 9 or something, it will be noticeable in her behavior. She could exhibit a number of behavior problems and be fuzzy headed. She won't feel good unless she sticks to her diet. Sounds kinda crazy but we have adjusted well. We will see how things go once we start introducing solids...I will keep you posted.
Any questions?