That's my PKU life

1 year milestone

Posted on by Amanda Horner

1 year ago today, Cole and I started fourth in a new chapter in our lives and into a new adventure. We relocated from my home town where I was born and Raised in Victoria BC to Kamloops BC.  We left behind many loved ones, my family and our wonderful friends. It was hard to leave but as I look back now a year later. I am so happy!

This move was really wonderful for us and just what we needed. A fresh start and a chance to live our lives they way they are ment to be.

I am so in love with my new life. I love our gorgeous home, I love coming home to it from my wonderful job where I have many wonderful work friends and some I have bonded with outside of work.  I love my residents and seeing them wherever I work. I feel great going to work and leaving work.

I love my 4 really great girl friends I have made up here. Sherry, Tiffany, Katie and Claudia.  I am so greatful to have them in my life. They are wonderful people who I enjoy spending time with. Each very kind and great people.

I love my neighbourhood, my community, my new town, all the things to do. I love how active I have become and how much fuller my life is. My health has come back to me and I am healing inside and out.

I love being closer to coles’ family , and spending more quality time with them.

I feel stronger, more confident, happier and I notice I am more relaxed, I find happiness in many more things and I laugh easier and harder.

But I am also content. I am content to be at home on my own, or to go out for a drive in my car and walk in one of our many parks. I am getting back into my own hobbies. I enjoy my photography, hiking, walking, swimming, yoga, going to the gym, meeting up with friends, camping, badminton and I have so many things I want to try! Like pottery !

My levels have gone from 11 to 1.5 in the last year. With an average of around 8. Though I have only lost 5 lbs since January I am working on my body and my fitness. I am learing about the balance between healthy clean organic eating , low protein foods, portion sizes, frequent small meals and activity.

I feel better about myself, though I still have a poor body image I can tell that my self confidence is higher.

I don’t feel weighed down and I have been living my life drama free. I stand taller and I feel freer.

I want to thank everyone who has been apart of our journey and our new lives. Our new friends , our families and our friends back home. Distance will never come between those who matter. Specially with facebook! LOL

Its been wonderful to have some of my good friends visit and I am looking forward to more visits over the summer and hopefully a trip back home to the island in September.

I am also very excited to finally have some of my family come and visit. To be part of this new life. To share with them our home, our lifes, to show them where we work and to share everything with them. In 4 days my sister arrives and I could not be happier. I am so excited to see her and have her here. It will be a short visit but one I have been waiting on all year and we have so many plans! I cant wait to show her everything!! and to spend time with her and hug her.

My family and my siblings are so important to me and it has been very hard not to be apart of their daily lives and see them whenever I want. It hurts me that my parents and my younger siblings have yet to come  to see us and our home but I am holding out hope that one day soon they will!

Its been a great journey and the adventure into new jobs and home ownership has been one of the best! Through the process relocating, the move its self, settling into a new routine, starting new jobs, making new friends,  of buying out first home, renovating, and all that it has entailed. Its been a heck of a ride!

So here we are a year later, blessed and happy. I wanted to take this time to celebrate and give recognition to our one year in kamloops.  A year ago I was apprehensive and worried if it would be stressful and hard. If it would draw cole and I closer or apart and if we where stong enough to make it work.  This past year has been so wonderful and busy, it has proven to me that we can make it through it all and it really has brought us closer together and I am so happy to be hear with cole.   The love of my life and my best friend! I am excited for the future and all the years ahead!

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PKU Book Project

PKU Book update

Posted on by Amanda Horner

Greetings everyone,

as many of you may know already about my latest project. A collection of personal stories about life with PKU. that I will be publishing into a book. I have been working with a number of people already and have already had many stories come in.

However, I still need way more! Also I am extending the deadline untill October 20th 2013. With hope of preparing the manual script and sending it to a publisher by winter and hoping for publication in the new year.

As I have stated in various posts before, there are 11 categories I am looking for .

Here are the 11 sections I have broken down to. I am looking for a minimum of 6  stories for each section, however I have no set limit for the time being.  :

Section 1 : New Diagnosis  

Examples are : a mothers perspective,
a fathers perspective
the new pku parents.
Section 2 : The Early years ( from a parents perspective) 

Examples being : “firsts” first foods, first clinics, first tests, first teeth ext ect
a growing baby
toddler adventures- raising a toddler with PKU
starting kindergarten 
the elementary years

Section 3 : The Teen years ( written by teenagers with  PKU )
Taking the reins – taking over the diet from your parents.
the diet
cheating
middle school/ high school
friends / relationships

 

Section 4 : The college years – (Written from pku patients)

post secondary education and dealing with diet, door life, classes, social outings ect ect.

 

Section 5 : Adult life – ( ADULTS WITH PKU THEMSELVES!)
 
careers, working, cheating, dating, relationships, marriage, families, traveling, inspirational stories.

 

Section 6 : Maternal PKU ( WOMEN WITH PKU WHO ARE PG OR HAVE BEEN PG )
Stories from PKU moms and their pregnancies

 

Section 7 : PKU Pioneer Adults

Older adults off diet, returning to diet and the struggles they face.

 

Section 8 : PKU Around the world

 

Stories about how treatment differs around the world, and clinics, tracking , foods , policies ect ect.

 

Section 9 : PKU FROM A FAMILY MEMBER’S PERSPECTIVE, SPOUSE, SIBLING OR CLOSE FRIEND) 

Section 10 : Stories from medical professionals who treat or work with PKU.

Section 11 : Treatments with KUVAN , Peg Pal or Large Neutral Amino Acids.

 

Many of these categories are still needing submissions and most categories have 1 or 2 submissions. I am in need or way more submissions for Sections 3 through to 11.  I have no limit to how many stories I will except but I would like to see a minimum of 50 over all.  The most popular sections are 1 and 2 and though id prefear to close those sections now and focus on the others, if you would really like to be apart of this project and submit a story please email me and we will work it out.

The idea behind this book, I have been reluctant to share do to wanting to protect my idea and not have more ideas stolen from me , is similar to something you would read in the “chicken noodle soup for the soul” series of books. If you are not familiar with these books, they are a collection of short personal stories with the purpose of something for everyone. Inspirational and educational.

My idea for this project was that anyone with PKU  , or even friends and family of PKU and maybe even the public will learn from it, find a story to relate to, for PKuers not to feel alone in their journeys and to create more awareness for PKU.

I am choosing to share this information with you all now publicly in hopes of generating more interest and hopefully that more people will come forward and share their stories.

I have high hopes for this project. I feel really great about it and I send a heart felt thank you to all those already participating and to those who have submitted their stories already.

I am hoping to read them all in the next couple days as I battle this stomach flu and get back to you all with feedback.

Please feel free to share this post with anyone that you think may be interested or have them email me for further information.

Also please if you have any questions, comments or would like to partake email me at my personal email adress!

Amandacosburn@gmail.com

 

thank you!

Your pku friend Amanda

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That's my PKU life

NewBorn Screening Saves lives! ( my 100th post!)

Posted on by Amanda Horner

In today’s age , with all our technology and tools at our disposal such as the internet and advances in health care I am surprised to be reminded that not everyone understands or knows enough about new born screening and some new parents would rather opt out if it. so as not to have their new infants “poked and prodded”

Being as my life was saved by newborn screening and I understand the importance this shocks and sickens me. Don’t get me wrong. its not the parents fault. It is society.  Even with our advances and our understanding of life and health care we as a hole are failing our future children. This is even more so a crisis for myself as a nurse. It is my job and all nurses job to educate everyone on the importance of new born screening.

I actually recently heard that a maternity nurse didn’t actually want to do the “PKU test” she didn’t think it was necessary unless you had a family history. This is not appropriate and very upsetting for me to hear.

Many new parents don’t even no what PKU is until their child is diagnosed. Let alone if they are a carrier or have a family history.

With out new born screening I would have never been diagnosed. I never would have grown and developed. In fact by the time I was 6 months old , I would be mentally retarded.  A lot of people find that term offending and don’t like to hear me use it. They would prefer I say something like “mentally challenged” however, I feel it needs to be said! That is the reality. This is what we face.

How would you feel if you denied your baby a future and quality of life, by denning a simple blood test they wont ever remember. Yes its hard to watch your baby’s heal be pricked and squeeze for blood and yes they may cry and scream.  But this test is so essential. It also screens for many more things other than PKU.

It was not even until the late 1960s that New born screening became mandatory and was initiated all through out Canada. Anyone born with PKU before this was mentally retarded and usually in a group home or institutionalized.

We are lacking in education and because these diseases are so rare and not enough is known about them in the general public they dont understand. If it was something more common like diabetes, CF, Cancer or anything else that is very much public knowledge than the education would be there already.

I feel like it is my job as an advocate to educate the public, my family and my friends to make the best possible decision and to be informed. To be educated and help provide them with the education and research they need.  I feel its all of our jobs.  Some people may think it is not our job or that is unfair that we always have to be the ones educating the public. We didnt ask for this, it was given to us.   But who better than us?

My parents had no idea what PKU was or new born screening and they didn’t put much thought into it at all until the diagnosis came back.   Maybe if they had of know about it , been educated and aware that PKU existed and other conditions it would have lessened the dreadful feeling they found themselves with, and the fear.

Here are some important facts about Newborn screening:

Newborn screening is a public health program designed to screen infants shortly after birth for a list of conditions that are treatable, but not clinically evident in the newborn period.

Some of the conditions included in newborn screening programs are only detectable after irreversible damage has been done, in some cases sudden death is the first manifestation of the disease.

Screening programs are often run by state or national governing bodies with the goal of screening all infants born in the jurisdiction. The number of diseases screened for is set by each jurisdiction, and can vary greatly.

Most newborn screening tests are done by measuring metabolites and enzyme activity in whole blood samples collected on specialized filter paper

Newborn screening debuted as a public health program in the United States in the early 1960s, and has expanded to countries around the world, with different testing menus in each country.

The first disorder detected by modern newborn screening programs was phenylketonuria, a metabolic condition in which the inability to degrade the essential amino acid phenylalanine can cause irreversible mental retardation unless detected early. With early detection, and dietary management, the negative effects of the disease can be largely eliminated.

Robert Guthrie developed a simple method using a bacterial inhibition assay that could detect high levels of phenylalanine in blood shortly after a baby was born. Guthrie also pioneered the collection of blood on filter paper which could be easily transported, recognizing the need for a simple system if the screening was going to be done on a large scale. Newborn screening around the world is still done using similar filter paper.

Newborn screening has expanded since the introduction of PKU testing in the 1960s, but can vary greatly between countries. In 2011, the United States screened for 54 conditions, Germany for 12, the United Kingdom for 2 (PKU and medium chain acyl-CoA dehydrogenase deficiency (MCADD)), while France and Hong Kong only screened for one condition (PKU and congenital hypothyroidism, respectively).[19]

The conditions included in newborn screening programs around the world vary greatly, based on the legal requirements for screening programs, prevalence of certain diseases within a population, political pressure, and the availability of resources for both testing and follow-up of identified patients.

Some of the diseases and disorders screened for in new born screening are as follows :

PKU and other amino acid disorders

Fatty Acid Oxidation disorders

Endocrinopathies

haemoglobins

organic academia

Cystic Fibrosis

Urea Cycle Disorders

Maple Syrup Urine Disease

Lysosmal Storage Disorders

Congenital Heart disorders

Sever combined immunodeficiency

Duchenne muscular dystrophy

adrenoleukodystrophy

 

and many others.

 

So Please I bag you, before you worry or want to denie the newborn screening heal poke test, please research it and understand it.   A simple blood test could save your infants life like it saved mine.

This is not like a vaccination, it is proven, it works, it saves lifes and it improves over all quality of life.

Thank you for reading this post.

Amanda

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That's my PKU life

8 years ago today, I gave my heart away.

Posted on by Amanda Horner

8 years ago today, there was a boy, and there was a  girl. This boy and this girl walked hand in hand across the cross walk on Kelly Road. After spending the day at the park and walking around town. The boy was talking about his family , his brother and his summer plans. The girl asked ” what did you tell your brother about me” The boy,  thought for a moment and said ” the girl that I am seeing, what do you say about me? ” The girl replied the same. The boy turned and asked.. ” So should we make it official then?” he seemed shy and unsure. The Girl smiled and said ” yes I think so”

The days turned in weeks, weeks into months , then months into years. The boy and the girl grew together, sharing their lives, their memories , their happiness and their sadness.  They where the best of friends and fell deeply in love. As they grew to be man and women,  life continued on sweeping them along. Through the ups and the downs of the roller coaster, and around and around on the Merry go round of life,   this women  knew, no matter what life through at them , together they could stand tall and face it all.  Together their hearts beat as one.

The Man  had long ago stolen her heart, with his gentle words and his gentle touch.  He taught her about love , and he taught her about life. With his patience and his support , they made it through many challenges and even more  celebrations.  Their love and their bond never wavering. Grew stronger with each moment and each memory.

This women had never felt anything so sure and so strong.  Never believed such a love could exist.  This Man opened her eyes and her heart.  This man gave this women strength when she was weak, he gave her compassion and he showed her a deep kindness.   He gently guided her from her shell and gave her wings to fly.

This man and this women,  2 souls drawn together, met as boy and girl and together became they so much more.

This Women is me, This Man is Cole.

I am so blessed and so grateful for the Love Cole has shown me this past 8 years, I look forward to our future  and many many more years , with a happiness and a confidence.  I thank god for bringing Cole into my life.  I am not sure what I did to deserve him but I treasure each moment .  Cole is my best friend, my soul mate, my life’s one greatest love.  Happy 8 years Cole.  Here is to many more! Thank you for opening your heart to me, for sharing your life, your family and your soul with me.  I love you more and more every day, to the moon and back , and a million times past heaven.

 

Always yours, your Mandy!

xoox

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That's my PKU life

Living with Pain

Posted on by Amanda Horner

This week marked 5 years since my life changed.

You see in 2008 I had a floating knee cap, It constantly dislocated. I injured it twice , once in middle school, I tripped over a cord at a school dance. I tore my muscles and dislocated my knee cap, it twisted all the way around to the back and tore my tendons and ligaments. Then In 2006 I injured it working as a cashier at Winners. I had been struggling with it since. In 2008 I had my first surgery for my knee. March 2008 , I was off work on medical leave for 15 weeks.  My recovery was slow , my knee got infected and I could not bend my knee. I had to go back and have my knee bent under sedation to help it move. As as a part of my physio I was suppose to go to physio and start riding my bike again. Though I could not afford physio and my knee was still not bending.

On the day my medical leave was up, I started a new job as a receptionist at a chiropractic office. My knee was stiff but started to bend so I decided to ride my bike to my first day of work.  I still remember my mom came over before my shift was suppose to start and asked me not to ride my bike and to take the bus. She didnt want to worry about me. She said she had a bad feeling. I didnt listen. It was july and it was beautiful sunny day.

I should have listened to my mom, something she still reminds me of every chance she gets.

On my way home from work, I was riding on the galloping goose. A old railroad track trail.  Gravol.  As I approached the end of the trail , it broke over the Sooke Road Highway. I dissmounted my bike and began to walk it down to the cross walk.  Just before the cross walk  there is a store called ” Willow Wind Feed Store” its a tack and farm animal shop. There was a car in the drive way sitting there. I could not see a turn singal on or any sign she was going to move. The driver did not look at me.  After that the details are hazzy. The next thing I remember is I was going down under her car. She had hit me just above my knee at my thigh. The same leg that I had just had the surgery.  She paniced as I fell. I landed half on my bike, and half on the ground with my legs twisted and under her car. She want to back up, I got my legs out just in time. Then I was being pulled out from under the car.

I could not wait bare on my legs. I was shaking and I learnt after I kept repeating “I am fine , I just want to go home.”

I remember calling cole, In a calm voice I told him ” I have just been hit by a car” and then I kinda lost it.  He was at the mall and was coming as soon as he could. He got there just as the ambulance came. I believe at this point I was in shock.

I don’t remember what happened at the hospital. I know that their diagnosis was soft tissue damage. I remember standing up when they told me I could go home and a shot of pain ripped through me and I could barely walk but they still sent me home.

some time later, days or maybe weeks later, I was sitting at my desk and work when all of a sudden I went into spasms and convolutions of pain. It ripped through my back, I could not breathe., I could barely walk or stand. I made it to the back room to be away from customers and crumbled. I remember my boss came, they put me on a table and tried some acupuncture needles, nothing was working and my legs where numb. They called Cole and I left.

I had a ruptured disk. I learnt later, When I landed I shifted my pelvic floor and ruptured my L5 S1 disk. I had static nerve pain and damage.

I was told the pain would never go away, and it has not.  The pain took over my life, it effected every aspect of my life. It held be back from living my life and it effected my relationship with cole, my family and friends.

It effected my work and my ability to hold a job, it effected my moods and my confidence. It caused me to fall in to depression and anxiety. Chronic major depression, anxiety and panic disorder , so I am told.

I wouldn’t leave my house, I couldn’t work, I was dependent on Cole.  The pain was so intense, every day for the moment I woke up and stepped out of bed, to the moment I went to bed and even while I slept.  It kept me awake and made for restless nights.  So to say it changed my life is putting it lightly.

I lived my life day to day in pain, with numbing from the waste down. I let it control my life and lived my life in fear of hurting it worse. I didn’t do anything. I was not active, I did not swim or go for walks or bike rides. I was left out of activities and held myself back from trying anything . I had “fear of movement”

I would try here and there, only making it worse. Id go to the gym for a few months or do something active and then id hurt more and id stop. Starting and stopping over and over again.

Physio sometimes 4 times a week. I wore a back brace all through my LPN course.  I have taken many many many types of pills even narcotics like hydromorphone. Nothing came close to managing the pain. Now I take gabapentin, 2 300 mg tablets twice a day and use tramadol for break through pain.

Tylenol and Advil just don’t cut it.

Then in 2011 I had another accident at work , A medication cart fell over and I caught it. IT felt like some one stabbed me in the back instantly.  a year off work , 10 weeks of physio and massage every day, a graduated return to work program that I could barely get through 4 hours on  , with out driving myself to emergency with spasms ripping through my body.  I was told I should consider giving up nursing. That Id never have a pain free day again. That I should consider doing something else or going on disability.

We then decided to move to kamloops. I went back to work as a nurse. I just could not give up.  I pushed through. I still remeber the first week up here, at my new job. I called cole in tears. He was still in Victoria finalizing things down there. before joining me 2 weeks later.  Cole feared I had done something else, another injury or accident. He said ” what did you do now?” but they where happy tears. I made it through a week of shifts, back to back and was in very little pain. I even had a pain free day! I cried and cried.

over the last year , I have gotten stronger, and I have been improving. Every day is a new day, a fresh start and a chance to start over. I am happier, I am healthier and I am more active. Since those first few pain free days turned into pain free weeks I began to test my new found freedom, I joined the gym, I started swimming regularly again. I went to aqua fit, I made new friends. I went for long walks with my camera and my new friends.  I joined drop in badminton and I tried Zumba. I joined yoga and was going twice a week.

The pain is not all gone, I still wake up and have painful days or painful moments. But compared to a year ago when I could barely put on my own socks, I take each day as a blessing.  But I keep going, and I keep trying. I keep working and I am making up for lost time.

I have a pain managment routine that finally works for me and being active has helped to. The yoga and the swimming loosen up my muscles . I get so stiff and sometimes it feels like my back muscles are trying to suffocate my spin. I get all twisted up and my muscles feel like there ripping and tearing. Or my legs are more numb and contracted.  So I have learnt by swimming, walking, hiking, biking and yoga I can keep them loosen up and I can do more things. I feel like I have so much catching up to do! So many things I missed out on!

I love sports! I love hiking, I love being out doors, I feel great after a good swim or walk. I am just starting to cycle again , i am up to 30 mins. I love yoga to. Its been great for my body and moving slowly, stretching each and every muscle. Its intense but it sets my day and my mood.

Its been a bit tough since starting my night line in march to continue to be active. There was the hole adjustment period and changing my sleep schedule and daily life. So I have not been as active since April and man do  I feel it!!

I can tell now. I can hear my body and its telling me to get back at it! Sometimes its hard to be active, I lack motivation, i hate going alone and sometimes ‘im lazy, or to tired. Then my body pays the price.

This week I went for a short Hike with cole and have gone swimming 3 times. It has really made a differnace. My legs where so sore , they felt like my thighs and calves where ripping apart. They where so tight and hard as rock. My massage therapist said they felt like hers does after 4 back to back hockey games.

The nerves where firing alot and lots of numbing. stiff neck , headaches and very tight and sore low back. Poor range of motion. Already I can feel it getting better.

I have learnt alot about myself, my body and my health. Its a journey and its never ending. I am more positive now and I have hope   that one day I will realize that I have not felt pain a very long time. I want to forget what its like to have pain every single day!

I am 27 years old and my body feels 80 some days!

I decided to share this story with you today , to recognize my milestone, to recognize my journey and to mark the 5 years since my life changed. I decided to take my life back and I work at it every day.  This is something I live with , its a part of me just like my PKU, it impacts my life but it dosent have to control my life!

 

 

 

 

 

 

 

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News

Bringing home Charlie

Posted on by Amanda Horner

Many years ago I met a inspirational Young Lady on the Message Forum PKUboard.info , Her Name was Breanna Hardy. We kept in touch over the years and have become online friends. When I joined Facebook in 2005 I was able to add all my friends from pkuboard and my network and my friends grew from there. Since meeting Breanna I have had the pleasure of following her family and eventually meeting her mom Michelle. I feel like over the years I have really gotten to know the Hardy family and have been privileged to be apart of their online family. Michelle and her Husband Bradly are very active in the PKU community and  have 9 children. 3 of their girls have PKU.  Breanna, Erica and Genavieve.  The Hardy family have some exciting news , once again, the hardy family are opening their hearts, and their home and  they are in the process of adopting a international baby boy with PKU. Who they have named “Charlie”

The Hardy family had not expected to find a baby so soon with PKU. In fact the agency told them that it would be nearly impossible , and it could take years.  The Hardy family had a financial plan in place to raise the money to adopt a pku child in the next year or so. However God works in mysterious ways, and their prayers have been answered. The agency has found them a child. 21 month old “Charlie” with PKU. So now the time frame has been drastically moved up and the family is stuggeling to raise the money to bring charlie home. They have started a fundraising page to raise the money to bring home Charlie. I was so touched by their story and wanted to do something to help bring attention to their story. . So I arranged an interview with Michelle in hopes by sharing their story we can generate some awareness and help make their dream of bringing charlie home a reality.

This is the interview with Michelle.

 

 

Amanda:

Good morning Michelle, Thank you for meeting with me.  So I thought we would start with some back ground information. For instance, can you tell me about your husband and yourself ,  how you met? when you got married how long have you been married and some details about your family. Like did you always want a big family.

 

Michelle :

Brad and I met in the 7th grade. We were in the same homeroom and our lockers were close together. He was very quiet and very reserved, and other than being friends I couldn’t imagine him ever being my boyfriend. In the 9th grade he got up the courage to ask me to dance at a school dance and I will never forget how sweaty his neck was (it is funny now to think about what I remember). After that I thought he didn’t like me and life continued. In the 11th grade he finally started to walk me home and the rest is history. We were married nine months after we graduated from high school and have been married for 25 yrs. As far as family…..we always said we wanted lots of kids, but to us five was a lot. After we had Breanna I was so devastated by her diagnosis of PKU I couldn’t imagine EVER having another child. I cried for her entire first year of life, but then as with anything PKU became easier and easier and that all changed. After our fifth we just kept the possibility open to having more children join our family. After our 7th child, Faith I really never wanted to be pregnant again (pregnancies were not kind to me and were filled with being sick from morning until night from the beginning of the pregnancy until I gave birth). We changed our minds when Faith was 5 yrs old and then it took us 1 1/2 yrs to get pregnant with Genavieve and then Brennan was our bonus baby because I conceived him when Genavieve was 9 months old.

 

 

Amanda :

when did you first consider adoption and why?

 

Michelle:

We had always talked about adding to our family through adoption, but I didn’t want to pursue it while there was still a possibility of having biological children. I was terrified of coordinating travel, a new child and being so sick. So we always talked about it, but it was put on the back burner. Each time a child would come up on the PKU Listserve the conversation would come up, but we thought we were too old and couldn’t afford it.

Then a few weeks ago the conversation came up again and we seriously sat down and discussed it and decided this was the path we were meant to travel. As far as why I want to adopt….. first of all I love my children and I would have had more had my pregnancies weren’t so awful. Age also played a factor in my decision to not give birth anymore. While I know women who give birth much older than I am I just couldn’t do it anymore.

 

Amanda:

While you where talking about adoption did you think about if you wanted a boy or girl or did it not matter? did you think about race and culture? did you have a country in mind you wanted to aopt from? or did it matter where? also how did you decide on these answers?

 

Michelle:

We didn’t care where the child came from or what his/her race was. We originally talked about a girl because in our minds there would be more females out there to adopt, but after talking to the agency we realized that there are actually more boys waiting. So we thought we were going to have a girl and then when the agency contacted us and asked about a boy instead I knew it didn’t matter especially because I viewed this like I did my pregnancies….it really didn’t matter. One thing that ddid happen that I never expected was how my husband cried when I asked him if he wanted a boy instead. It was tears of happiness because as you know we have seven daughters and two sons. The only thing that really mattered to us was PKU. We knew we wanted a child with PKU.

The adoption agencies tried to match us with other disorders, but I knew that wasn’t right for us. We knew we wanted a child younger than your youngest. We were very open to whatever country and race. We wanted a child with PKU because we knew they would be harder to place and we have life experience with PKU. We know we can provide the care a child with PKU needs because we have done it before and are doing it.

 

Amanda :

when did you decide to involve your other children in your decision? what did you tell them?

 

Michelle:

They always new it was a possibility but when we seriously started talking about it we involved them immediately because this child will be their sibling. I was never good at holding off when I was pregnant either. They knew immediately after I told my husband. As far as what we told them they were there when we were discussing it so they heard their dad and I talking about it.

 

Amanda :

when did you first apply to adopt? what was the application process like?   how did you research agencies and when you found one how did you decide it was the right one for you and your family? did you go through the ministry ? or government?

 

Michelle :

I actually started out looking online for agencies. It was all confusing and overwhelming. I started to call them and was highly disappointed because many told me they didn’t think they would ever find a child that met my requirements. On Thursday of last week I cried and cried (I was at my office) and before I left finally just told God that it was in his hands and if it was meant to be he had to make it happen. I went home, told my husband the disappointing news and cried some more. The next day I had to pick up some stuff and do some copying at my office. I took the kids with me and my husband went along too because he had some work to do there for me. It was while there that I got the call and the agent told me about this little boy, texted me a photo because I didn’t have a computer to look at his profile. I cried and ran to my husband with the photo and he said definitely yes. So the agency found us really.

 

Amanda :

what has the process for adoption been like? both legally and emotionally? how much does it cost? what do the fees provide? and what is the next steps in your process?

 

Michelle:

Emotionally I am a wreck because I am so nervous, but since it has only been since last Thursday very scary too. It is moving way faster than I thought. It will cost us between $30,000-40,000. This will provide us with everything we need to bring our child home including our travel to get him. Right now we are going over the contract and are drawing up a letter of intent and doing the paperwork process. We have to do a home study as well. There is a lot to do and right now it feels overwhelming. I just got a list of 7 things I need to do over the next few days.

 

Amanda :

Sounds very overwhelming and exciting! Thank you Michelle, My next group of questions are specific to Charlie.

 

Amanda :

what where your first thoughts when you saw charlie? and how did you decide on a name? was it already his name? I know you have your kids names following the alphabet? is that something you decided on or more just happened?

 

Michelle:

I immediately thought he looked a little bit like my youngest son Brennan and I cried. The name was something my husband decided on. He has a different name, but we can’t disclose that.  I don’t want to do anything to chance the agency dropping us. Also Charles was next in alphabetical order.  Yes we opted to do the names in alphabetical order. It didn’t start out that way but when we saw the pattern we continued it.

 

Amanda :

Just to expalin, Michelle’s  Girls names range from the Olderst Girl with a an “A” name , and her youngest Girl is a “G” name. Michelle Currently has 2 boys, The Oldest boy is an “A” name and her youngest Son Is a “B” name, then there will be Charlie!

 

Amanda:

Charlie is an international adoption, Are you and your family planning on learning about his culture and bringing it into your family and every day life?

 

Michelle :

We are hoping to learn about his culture and incorporating it into his life, but I don’t know what that will look like right now. When we pick him up we will spend two weeks in the country.

 

Amanda :

Charlie also has PKU, Do you know if he has been treated since birth or , if he is a late diagnosis do you know if he suffers from any side effects?

 

Michelle :

He has been treated since 10 months, but I am not sure what the treatment consists of and how good it is because he only had one level taken and that was at ten months. I am sure there will be side effects.

 

Amanda:

You and your family are so brave! I really admire you for what you are doing.  My next group of questions are more specific to Charlie and his PKU.

 

Amanda :

Have you talked to your PKU clinic staff about your pending adoption? have they been supportive?  how long after charlie comes home will be be going to clinic and begin treatment?

 

Michelle:

No I haven’t talked with them, We plan on contacting them as we get closer and the plan is to make sure we have formula on hand for Charlie when we bring him home, will do his blood test when he gets home and hopefully will be seen in the clinic right after his return. My goal is to do the test, get the results and be seen in the clinic immediately.

 

Amanda :

What testing will charlie have to undergo to determine course of treatment and his tolerance? Will you be able to  pick his formula or will the clinic?  I assume since he is 1 he is eating some solid foods?

 

Michelle:

The clinic will have to determine the testing but I am guessing it won’t be any different than what the girls went through (finger sticks, estimating and changing the diet as needed). Since the girls are on Kuvan my husband and I will have to discuss this option for Charlie as well.  As far as formula we have to see what he is on now and if it compares to anything we have here. We want to make the transition easy for him, so we will see what formula he ends up on. We will probably do like we did with the girls at that age … taste tests.

 

Amanda :

How does having 3 daughters with PKU prepare you for caring for Charlie? Do you know that as a little boy with PKU If Charlie will  face different challenges then your girls?

 

Michelle:

I honestly don’t know if challenges will be different for him, but I believe living with PKU for 22 yrs gives us a lot of knowledge to work through whatever challenges come our way. To me this is no different than when I had our biological children…there are no guarantees in life and we figure it out as we go along with the knowledge that we have.

 

Amanda :

Great answer! How involved will your children be in charlies care and what can they do to best support him?

 

Michelle:

They will be as involved as they are right now with their other siblings based upon where they are at in their lives. He will be their sibling just like they all are to each other.  With kids ranging in age from 25 yrs down to 3 yrs there is a huge difference in what they contribute.

 

Amanda:

Yes for sure! Do  you plan on telling Charlie he is adopted? and when and how do you plan to do that?

 

Michelle:

He will definitely know he is adopted and something that will be incorporated from the start. Kids like to hear the story of how they came to be adopted or biological. His story will be his own and he will know it just like the others know their stories.

 

Amanda :

Aww, so true! what are your hopes and dreams for charlie are they the same or different for your hopes and dreams for all your children?

 

Michelle:

They are the same as all of my other kids. I hope and pray they grow up to be happy and productive individuals.

 

Amanda :

I can tell that you are a wonderful mother, and you have a wonderful family. Charlie is very lucky to have you. All of your children are lucky to have you and Bradly for parents!

 

Michelle:
 Thanks. I just hope we can get him here. It will definitely be a journey!!! My husband keeps telling me that now I know how an expectant dad feels. *Laughs*
Amanda:
Is there anything you would like to add about your fundraising efforts?
Michelle:
As far as fundraising we are doing it because when we went into this we thought that it would take a long time to be matched with a child. That is what you hear all the time. We had a plan to raise the money through working, etc and it happened way too quickly. Again my husband laughs and says just like everything else in our lives because when we would start to talk about getting pregnant and had a plan in place to start in a year or months later I always turned up pregnant that month.
Amanda :
aww well, I  wish you the best of luck and will be following your journey closely. would you be interested in doing a follow up interview after charlie is home?
Michelle:
 Definitely. Then I can share photos too! Ohh, and  I forgot to add that Charlie isn’t the only person with PKU needing to be adopted!! I know of three more in the country he is in.
Amanda :
Thank you for taking the time to do this with me. I hope it will help create awareness for your situation and help raise donations for you . May god bless you and your family.
Michelle :
Thanks for doing it!
If you would like more information or to to make a donation to help bring Charlie home to his Family, please visit the fundraising website : http://www.youcaring.com/adoption-fundraiser/help-us-bring-charlie-home/72801
Thank for reading! My thoughts and prayers are being sent out to the Hardy family and I cannot wait for Charlie to come home! I am so blessed to call the Hardy family my friends. I really admire them and all that they do for their  children and the PKU Community.
Amanda Cosburn
1
Managing the diet

Phenyl Ade PheBloc Tablets.

Posted on by Amanda Horner

A few days ago while I was on night shift, I was working with a friend of mine who is working as a care aid but is taking her Registered Nursing course. She is in her second year and we have been discussing PKU together.  On this particular shift we where discussing different forms of treatments available like Peg Pal, Kuvan and LNAA’s. We where also talking about different formulas. This was also the night I was working on my formula Journey and Formula Summery blog posts. To look up some examples to show her, I pulled up the website for the Phenyl ade product line. Together we read about their diffrent options and then I came across the PheBloc tablets. My clinic had told me about them before but admittedly where I was at that time in my life I was not that interested.

I was surprised to see, that the website said that by taking the Phe Bloc Tablets, You could change your diet from 20 % regular foods and 80 % low protein to the opposite 20% low protein and 80 % regular foods. There was a list that had what products you could eat more of , like regular rice, and breads. Which is what sparked my interest. I love Rice and bread and avacado sushi or yam sushi.  From what I understood, is that you take multiple tablets every day and it floods your blood with other amino acids so much so that it blocks the phe in your blood from getting into your brain . I was very keen to learn more, specially since it is actually a covered treatment unlike KUVAN which helps in some people to activate the enzyme that breaks down phenylalaine into tyrosine.  KUVAN is said to only work with certin types of PKU and does not have a great sucess rate in Classical PKU , which is what I have.

So yesterday I called my clinic and this is what they told me.

My dietitian Taryn , does not recommend it for women hoping to get pregnant  soon or of child bearing years , as it does not lower your PHE levels. The PHE in your blood is still high, but it is suppose to lower how much PHE gets into the brain. However there is no way to test how much PHE is getting into the brain to be sure. So there is still risk. What the PHEbloc tablets are suppose to do is push through all the other amino acids through to the brain and that is suppose to block the PHE from being entered into the brain. So you are suppose to take the tablets every day and pretty much flood your blood so there is more amino acids rather than PHE. Where I was really excited about Phebloc, this does not seem like something I would want to take the risk of. They do recommend it for others who have very high levels and are having a hard time bringing them down, or for adults returning to diet. Some adults have reported that they do feel better over all. That their side effects feel more manageable. However there blood levels still come back high. We do have a few patients on Phebloc at our clinic but,  they are adults who regularly have levels of like 20 to 30. Or adults returning to diet who eat more regular food.

I think that my Bettermilk and low protein foods are working fine for me and I am happy with my levels so this is not something I would be interested in. Just thought id pass the information on to those who are interested.

 

The Reason why My Dietitan does not recommend Phe Bloc for women of child bearing years and women who are wanting to become pregnant is  that even though it blocks PHE from entering the brain, it does not lower the PHE in your blood , and so that blood can still go to the uterus. High levels are toxic to a uterus and can cause damage to the baby.  Whatever your levels are in your blood are double in the uterus and toxic to a unborn fetus. So even though PheBloc can limit the amount of phe that gets into your brain, you still have high phe levels in your blood.

Even though they can test to see the levels of PHE in your brain with an MRI or brain scans, they are costly and not worth it to bring every patient in regularly to use these invasive testings. It is not as a simple as a blood test.

It is for these reasons, I have decided PHE bloc and LNAA therapy is not something I am interested in at this time. Though i do not doubt it may work for others.

 

 

0
Managing the diet

A summery of PKU Formulas.

Posted on by Amanda Horner

Here is a summery of as many formulas I can think of. ( wording is not my own, information taken from the websites provided) Please be sure to check with your dietitians and medical staff before requesting a sample or switching formula.

Applied Nutrition: 

http://www.medicalfood.com 

Phenyl Ade Essentials : 

The only PKU formula with flax & soluble fiber to support heart health

American Heart Association recommend 4:1 ratio of Omega-6 to Omega-3

Complete with essential vitamins and minerals to help meet daily requirements

5 great choices: Chocolate, Vanilla, Orange Creme, Strawberry and Unflavored

Packaged in cans and convenient ‘go anywhere’ single serve pouches

Suitable for individuals over 1 year of age with proven PKU

PhenylAde Essential Drink Mix is the foundation for building a healthy PKU diet. This great tasting PKU product provides an ideal blend of vitamins and minerals with a superior amino acid profile to meet the needs of children, teens and adults following a PKU diet. PhenylAde Essential contains less saturated fat than other leading PKU formulas with added fiber and flax to support heart health

Phenyl ade RTD 10 :

  • Only 60 calories per bottle
  • Complete with essential vitamins and minerals
  • 10g protein equivalents in each 2.5 fl oz bottle
  • No preservatives
  • No artificial colors or flavors
  • Suitable for individuals over 4 year of age with proven PKU

PhenylAde RTD is a ready to drink PKU formula. This great tasting PKU product helps you meet your full daily prescription in less total volume by supplying 10 grams of protein equivalent in each 2.5 fluid ounce bottle. PhenylAde RTD is complete with vitamins and minerals including a superior amino acid profile to meet the needs of children, teens and adults following a PKU diet.

Phenyl Ade 40: 

  • Only 1/2 gram of fat per serving
  • Reduced volume for easier dietary compliance
  • Complete with essential vitamins and minerals to help meet daily requirements
  • Available in convenient ‘go anywhere’ single-serve pouches
  • Mild, neutral flavors allows for easy drinking
  • Suitable for individuals over 1 year of age with proven PKU

PhenylAde40 Drink Mix is a reduced calorie, fat and volume PKU formula. This great tasting PKU product helps you meet your full daily prescription in less total volume. It provides an ideal blend of vitamins and minerals with a superior amino acid profile to meet the needs of children, teens and adults following a PKU diet.

 

Phenyl Ade 60 :

  • Only 50 calories per serving
  • Excellent for weight management
  • Low volume for easier dietary compliance
  • Complete with essential vitamins and minerals to help meet daily requirements
  • Available in cans and convenient ‘slimline’ single-serve pouches
  • Suitable for individuals over 1 year of age with proven PKU

PhenylAde60 Drink Mix is a high protein, low calorie, low volume PKU formula. This great tasting PKU product mixes easily with only 3 fluid ounces of cold water allowing you to meet your full daily prescription with less to drink. The superior amino acid profile and full range of vitamins and minerals help meet the needs of older children, teens and adults following a PKU diet.

Phenyl ade Amino Acid Blends::

  • Create your own PKU formula by adding to low protein foods or drinks
  • Fat free, sugar free & low calorie
  • Tasteless and Odorless
  • Boost amino acid protein intake by adding to current PKU formula
  • Available in cans and convenient ‘slimline’ single serve pouches
  • Suitable for individuals over 1 year of age with proven PKU

PhenylAde Amino Acid Blend and MTE Amino Acid Blend are a precise mixture of essential and nonessential amino acids for the dietary management of Phenylketonuria (PKU). These products allow you to “create your own PKU formula” to improve taste, variety and flexibility to reduce formula fatigue and improve diet compliance.

 

Phenyl Ade Amino Acid Bars: 

  • An alternative to liquid PKU formula
  • Packaged in convenient “go anywhere” travel bars
  • Available in a great tasting Chocolate, Chocolate Crispy and White Chocolate flavors
  • Suitable for individuals over 1 year of age with proven PKU

PhenylAde Amino Acid Bars are an innovative medical food intended to provide an alternative source of protein, fat and carbohydrate for the dietary management of PKU. PhenylAde Amino Acid Bars can supplement all or part of the protein prescription when traditional liquid formula is insufficient or not tolerated. Available in 3 delicious flavors, PhenylAde Amino Acid Bars look, taste and smell like real chocolate.

 

Phenyl Ade PheBlock : 

  • Simplifies PKU diet management
  • An alternative to PKU drug therapy
  • Allows for a relaxed PKU diet
  • Eliminates need for expensive low protein foods
  • Available in Tablets or Powder
  • Suitable for individuals over 12 years of age with proven PKU

PheBLOC LNAA allows for a relaxed PKU diet which includes more choices of natural protein from a wide variety of foods. The relaxed PheBLOC LNAA diet helps to reduce the burden of high priced, low protein specialty products. You will have more choices in restaurants and social settings making the diet less complicated.

SHS Nutricia 

http://nutrition.nutricia.com/ as well as  https://www.myspecialdiet.com/ and http://www.nutricia-na.com/index.htm

Add-ins:

Add-ins is a flavourless phenylalanine-free powder containing balanced amounts of the other essential and non-essential amino acids, fat, vitamins, trace elements, calcium, phosphorus and magnesium.

Easiphen:( 8 years and up) 

Easiphen is a ‘ready to drink’ phenylalanine free liquid product containing a balanced mixture of essential and non-essential amino acids, carbohydrate, fat, vitamins, minerals and trace elements. Available in Forest Berries flavour. A food for special medical purposes.

Liquid Duocal : 

Liquid Duocal is an emulsion consisting of a blend of carbohydrate and fat. A food for special medical purposes.

Lophelx LQ 10 : ( 4 years and up) 

A flavoured phenylalanine-free liquid containing a balanced mixture of the other essential and non-essential amino acids, vitamins, trace elements and some minerals. A food for special medical purposes. Comes in Berry, Juicy Orange, and Tropical

Minaphelx: ( ages 1 to 10 ) 

Minaphlex is a palatable phenylalanine free drink mix containing essential and non-essential amino acids, carbohydrate, fat, vitamins, minerals and trace elements. Available in Unflavoured, Pineapple & Vanilla or Chocolate flavour (contains colour, sugar and sweeteners). A food for special medical purposes.

 

Phelxy 10 bars : ( 8 years and up) 

The Phlexy-10 Bars is a citrus fruit flavoured bar free from phenylalanine, but containing a balanced mixture of essential and non-essential amino acids, fat, carbohydrate and colouring. A food for special medical purposes.

Phlexy 10 Capsules: ( 8 years and up) 

Phlexy-10 Capsules are cellulose capsules, free from phenylalanine but containing a balanced mixture of essential and non-essential amino acids. A food for special medical purposes.

Phlexy 10 – Drink Mix ( 8 years and up) 

Phlexy-10 Drink Mix is a flavoured powder, free from phenylalanine but containing a balanced mixture of essential and non-essential amino acids, carbohydrate, colouring, sweetener and flavouring. Available in Citrus Burst, Apple and Blackcurrant and Tropical Surprise Flavours. A food for special medical purposes.

Phlexy 10 – tablets ( 11 years and up )  

PHLEXY-10 Tablets are free from phenylalanine but containing a balanced mixture of essential and non-essential amino acids. A food for special medical purposes.

Phlexy Vits: (ages 11 and up )

Phlexy-Vits is a concentrated powdered vitamin, mineral and trace element preparation. A food for special medical purposes.

Phlexy Vits Tablets : ( 11 years and up ) 

Phlexy-Vits are tablets containing vitamins, minerals and trace elements.

PK-Aid 4 ( 6 months and up) 

PK Aid-4 is a phenylalanine free unflavoured blend of essential and non-essential amino acids. A food for special medical purposes.

Anamix  Junior LQ : (1 to 10 years ) 

A phenylalanine-free drink containing a balanced mix of the other essential and non-essential amino acids, carbohydrates, fat (including docosahexanoic acid (DHA)), vitamins, minerals and trace elements. Available as unflavoured, orange or berry flavoured.

Lophlex LQ 20 ( 4 years and up) 

Lophlex LQ is a “ready to drink,” phenylalanine-free liquid containing a balanced mixture of essential and non-essential amino acids, carbohydrates, vitamins, trace elements and some minerals. Available in Berry, Orange and Citrus flavours. A food for special medical purposes.

XP Analog :( 0 to 1 years ) 

XP Analog is a phenylalanine free powdered infant feed, containing essential and non-essential amino acids, carbohydrate, fat, vitamins, minerals, and trace elements. A food for special medical purposes.

XP Analog LCP ( 0 to 1 year) 

XP Analog LCP is a phenylalanine free powdered infant feed supplemented with long chain polyunsaturated fatty acids (LCPUFA’s), particularly Docosahexaenoic Acid (DHA) and Arachidonic Acid (AA), containing essential and non-essential amino acids, carbohydrate, fat, vitamins, minerals, and trace elements. A food for special medical purposes.

XP Maxamaid : ( 1 to 8 years)

XP Maxamaid is a phenylalanine free drink mix containing essential and non-essential amino acids, carbohydrate, vitamins, minerals and trace elements. Available in Orange flavour (contains colours, sugar and sweeteners) or Unflavoured. A food for special medical purposes.

XP Maxamum : ( 8 years and over) ( powder) 

XP Maxamum is a phenylalanine free drink mix containing essential and non-essential amino acids, carbohydrate, vitamins, minerals and trace elements. Available in Orange flavour (contains colours, sugar and sweeteners), Strawberry flavour (contains colours, sugar and sweeteners) or Unflavoured. A food for special medical purposes.

XP Maxamum Liquid TETRA Packs : ( 5 years and over) 

A flavored phenylalanine-free amino acid-based drink containing a balanced mix of the other essential and non-essential amino acids, carbohydrate, fat, vitamins, minerals and trace elements for the dietary management of phenylketonuria (PKU). Suitable for ages 5 through adult, including pregnant women. Come in Forest Berry and Orange.

Periflex Infant

phenylalanine-free powdered infant formula with iron, containing DHA and ARA, for the dietary management of phenylketonuria (PKU).

 

 Periflex Junior

  • Phenylalanine-free
  • Easy transition from Periflex Infant
  • Contains a balanced mixture of all other essential and non-essential amino acids, carbohydrate, fat, vitamins, minerals and trace elements
  • Excellent balance of linoleic acid and α-linolenic acid, providing a preferable 4:1 ratio of n-6 to n-3
  • Age-appropriate DRI formulation
  • Periflex Junior provides 25 g of protein equivalent and 14 g of fat per 100 g of powder
  • Meets protein requirements in lower volume
  • Pleasant taste, smell and texture
  • Available in unflavored and orange
  • Optimal nutrition of all ages
  • Osmolality (mOsm/kg)

Preiflex Advance : 

A phenylalanine-free, powdered medical food for the dietary management of phenylketonuria (PKU) in older children and adults, including pregnant women and women of child-bearing age.

Milupa PKU 2: 

A phenylalanine-free powdered medical food for the dietary management of phenylketonuria (PKU) in children over 1 years of age.

Milupa PKU 3: 

A phenylalanine-free powdered medical food for the dietary management of phenylketonuria (PKU) for adolescents, adults, and pregnant women

Lanaflex : 

amino acids (large neutral amino acids and lysine) for the dietary management of phenylketonuria (PKU) in adults (over 18 years of age) who are no longer following a phenylalanine-restricted diet.

 

Vitaflo

http://www.vitafloweb.com/

PKU Start : ( birth to 1 years ) 

PKU start is a ready-to-feed phenylalanine free infant formula, containing essential and non essential amino acids, carbohydrate, fat, vitamins, minerals, trace elements and long chain polyunsaturated fatty acids (LCPs); Arachidonic acid (AA) and Docosahexaenoic acid (DHA)

PKU Squeezie ( 6 months to 10 years) 

PKU squeezie is an apple & banana flavoured ready-to-feed phenylalanine free protein substitute containing essential and non essential amino acids, carbohydrate, sugar, vitamins, minerals, trace elements and the long chain polyunsaturated fatty acids (LCPs); Arachidonic acid (AA) and Docosahexaenoic acid (DHA).

PKU Gel – ( 6 months to 10 years)

PKU gel is a powdered phenylalanine free protein substitute, available in unflavoured, orange and raspberry flavoured options, containing essential and non essential amino acids, carbohydrate, sugar, vitamins, minerals and trace elements. Flavoured options contain sweeteners.

PKU Cooler System: ( 3 years to adult) 

The PKU cooler® system is a range of ready-to-drink phenylalanine free protein substitutes, available in neutral-tasting (white), and flavoured (orange, purple and red) options, containing essential and non essential amino acids, carbohydrate, vitamins, minerals, trace elements and the Omega-3 long chain polyunsaturated fatty acids (LCPs); Docosahexaenoic acid (DHA) and Eicosapentaenoic acid (EPA). Contains sugar and sweeteners.

PKU Express System: ( 3 years to adult) 

PKU express is a range of powdered phenylalanine free protein substitute, available in unflavoured, orange, lemon and tropical flavoured options, containing essential and non essential amino acids, carbohydrate, sugar, vitamins, minerals and trace elements. Flavoured options contain sweeteners.

Cambrooke Foods

http://www.cambrookefoods.com/

Camino Pro Amino Acid : 

  • For PKU and MSUD patients age 5 and older
  • Ready-to-drink pouches
  • Available in Fruit Punch and Pina Colada flavors
  • Great flavor and no aftertaste
  • Reduced sugar and low volume
  • Complete with macronutrients, vitamins, minerals, and trace elements.

 

Bettermilk : 

A mix-with-liquid powdered formula for PKU that makes a creamy, milk-like beverage with a hint of sweetness.

BetterMilk™ 3-12 is designed with 10 grams of Glytactin and optimal age specific vitamins and minerals for those ages 3-12. It is available in two delicious flavors: orange creme and strawberry creme.

BetterMilk™ 12+ is designed with 15 grams of Glytactin and optimal age specific vitamins and minerals for those ages 12 and older. It is available in two delicious flavors: orange creme and strawberry creme.

BetterMilk™ original is designed with 15 grams of Glytactin and vitamins and minerals for those ages 1 and older. It is available in unflavored.

  • Made with Glytactin™ protein, our advanced proprietary blend of glycomacropeptide and essential amino acids, that is better utilized by your body.
  • Helps to better maintain blood Phe levels between meals.
  • Low volume – mix with 4 fluid ounces of water.
  • 32 mg of DHA Omega-3 Fatty Acids per serving (DHA Omega-3 for a healthy brain and heart!)
  • Probiotic cultures to support digestive health.
  • Great non-medicinal taste.
  • Combines well with your favorite foods.

Camino Pro Complete Bars: 

A complete and balanced meal for the PKU patient. It has age specific vitamins and minerals. Each bar includes, 15 grams of Glytacin, Vitamins, minerals, fiber, fat and carbohydrates. Comes in peanut butter and Fruit Frenzy flavors. Ready to eat. Each bar has 3 grams of fiber.

Restore: 

Restore has 10 grams of hole protein per 16.9 oz ( 500 ml) , is made with Glytacin. It includes amino acids, carbohydrates, electrolytes , B vitamins and is a source of calcium.  Restore is an isotonic beverage, that re-hydrates while you exercise and replaces electrolytes lost through perspiration. Comes in 2 flavors. Lemon Lime and Tangerine.

Camino Pro Swirl :

Camino Pro Swirl is the newest formula available.  It is a powdered formula that can be mixed with 3 to 4 ounces of cold water ( thick) to make a creamy pudding , or mix with 6- 8 ounces to make a shake or smoothie!

Swirl is made with 15 grams of Glytacin Protein. It is fortified with vitamins and minerals. Swirl is naturally high in the Large Neautral Amino Acids (LNAA’s) Threonine, isoleucine, and Valine. Each serving contains 32 mg of DHA Omega 3’s. Can be used as a meal replacement or a snack.

 

To the best of my knowledge, this may not be a complete list of all available formulas. If you know of any others please feel free to message me with information. As you can see, there is a such a variety. We are so blessed with the advancements of PKU through out the years in PKU formulas. I am forever grateful to all the low protein food companies and manufacturers of our formulas.   It is really incredible and inspiring to see such a variety today. Not all products are suitable for everyone with PKU and no 2 products are the same. If you wish to try any of these formulas please check out the websites listed for more information and consult your PKU clinic before requesting a sample.

 

 

 

 

0
Managing the diet

My Formula Journey

Posted on by Amanda Horner

For the past little while I have been having writers block and was unsure on what I could write about here in my PKU blog that would be interesting and valuable. I have been looking for suggestions and I got a reply from Jane McDonald this evening on writing a blog post about great vs nasty formulas and daily formula compliance. This gave me an idea to record my personal journey with formula.

My Journey with my formula has been a lengthy one and not always easy. I myself have been on many different formulas.  I began my formula journey on Phenyl Free.  I am assuming based on its name that it was a phenylade product. It was made to taste like potatoe starch and was unflavored. My mom mixed it up every night in our blender and flavored with Nestle Strawberry quick. I was on this formula until I was about 12 I believe. As it was discontinued. When I was 11 and 12 they first came out with the phenyl ade amino acid  bar, the crispy and the plain. They where AWFUL! It was the first non formula meal replacement bar . The crispy had rice krispies in them. Though I preferred the Crispy I much rather would not have either. It is still an on going joke between adults my age.

When I was admitted to BC Children’s hospital I trailed the new phenyl ade essential formulas. At that time there was only the 3 , the Vanilla, strawberry and Orange Cream. I was not a fan at all of the Vanilla and the strawberry. I am not sure how to describe the taste other than fake and chalky. I had trouble staying compliant with the formula as I got older , I found it separated to easily, I could only drink it cold and with a straw. Also I was chalky and had an after taste. I ended up pouring it down the drain alot. I stayed on this formula until I was 19. At 19 they finally had liquid formulas.  I tried the 2 flavors of the SHS XPHE Maxamum Forest Berry and Orange. I loved them instantly, and was thrilled they came in “juice boxes” I switched to both formulas in the beginning. However, I am very sensitive to formulas and they really upset my stomach. So even though I loved how they tasted , I started to notice I was re acting to the forest berry and had to switch to just the orange XPHE Maxamum. I stayed on the Xphe Orange Maxamum until I was 23 , when I noticed the taste had changed. I am very sensitive to changes in my formula and have had many arguments with my clinic. They don’t seem to believe me , They said they tasted them and noticed no change. but I am the one drinking it every day and it tasted awful and i began reacting to it too.  By this time there was many many new formulas. So I got samples of every formula available in Canada.  For a while I drank the Vitaflo coolers, but they smelt to strongly of fish oil and the omegas where to hard on my stomach. Then I tried the Camino Pro amino acid blend by Cambrooke. They came in tropical and berry blast.  They did not taste bad but I didn’t think they tasted great either. They where very gritty and had a strong after taste. I just was not satisfied with the formulas I had been trying.

On my next clinic appointment my dietitians had me trial almost every form of formula including ones i had tried previously just in case I could tolerate them or my taste buds had changed.  So I tried samples of the Phenyl ade essentials, 40s and 60s. I tried the powder forms of the xphe maxamums and those where so terrible i voted them the worst formula I had tried. Way to bitter, gritty and such a strong after taste. A strong second for the worst spot was the Vitaflo Express. Even though it was a smaller volume I couldn’t stand it. Very sweet and bitter, very watery or chalky depending on how much water you used. They came with a little shaker. Much to strong for me and I ended up pouring them down the drain at work a few times. I was to old to try the gels and squeezie as they are only for children but they seem like a very good and fun idea.

I eventually tried the bettermilk by cambrooke. It was so different it was an adjustment for me. It is creamy and does not have an after taste.  It took a while for me to get use to it. I did not like the unflavored flavor so I decided to go back to my roots and flavor it with the strawberry quick. I feel in love! Probably the best formula I have ever had,  I drank it for 7 months, But then they changed it too! I was heart broken. It was doing wonders for my sensitive stomach and my IBS. However, this time I was not alone! A lot of people noticed the change and we all wrote or called in to Cambrooke.  I was to upset. They did promise they where changing it back but I couldn’t wait that long and go with out a formula so it was back to the drawing board.

I then tried the Cambrooke Restore. It tastes just like Gatorade or Powerade. Its liquid and tastes just like juice. Its thin and super easy to drink. However not enough protein equivalent for me. Id have to drink 6 of them and that was way to many calories and way to much liquid for me. There was no way I could get them all in in a day. I also tried the Cambrooke Complete bars. They seemed like a great idea and I had been hearing a lot about them. They are essentially a meal replacement bar you eat instead of drinking formula. However I did not like them either. They where super thick and chewy. I could barely get through one. I did not like the taste either or the flavors. I could not see myself eating them regularly.

In the end I went back on the Phenyl ade Orange cream with much reluctance. I was pleasantly surprised though. This time around i loved it! And I found it much easier to drink and to travel with. I use to mix it with water in a cup , stir it with a fork and drink it with a straw. This time around I shook it in a sports bottle and drank it with a straw. I had no problem drinking my full amount. It was more volume than I was use to and I stayed full longer. it really filled me up and tasted great. Only problem was that it was high in calories. I drank it again for over a year. I had decided not to go back to the bettermilk because I really enjoyed my formula again.  As the months went on though and my struggle with my weight loss persisted I started to get really concerned with my calorie intake. The clinic suggested I try the Phenyl ade amino acid MTE blend. You can add it to formula, water and juice. It cut my intake in half so I was having 1 scoop of my formula and 1 scoop of the MTE 3 times a day, of course that created another problem.. now I was hungry!!  I just could not seam to win!

Then one day a few months ago I was at a PKU friends house for lunch and I forgot my formula, She was on the bettermilk and I tried it again. Was sooo very happy that it tasted great again! So good I called my dietitian and made the switch back. They sent me samples of the new flavored bettermilk in the orange and strawberry.  I was not a fan of the strawberry, tasted like the phenyl ade essentials, fake and to sweet. The orange was not bad, it tastes like orange sherbert. I still preferred my unflavored. Since I am trying to loose weight though I was looking for something to add for flavour that did not have as much calories as the strawberry quick. That is when Cole suggested I use his MIO. When we moved to kamloops, Cole did not like the taste of the water and he began using MIO in his water. I prefer my water plain and ice cold. So I had not been using it. SO Cole and I went to the grocery store and we bought one of each flavor for my formula. I love it! Its been working out so great. I have so many different flavors now! Even more since I found crystal light and Disaini, and nestle flavors that do not have aspartame. AS I have mentioned in my previous blog posts. They do not use aspartame, they use surculouse. Which is unable to be broken down in the body and is excreted through urine. SO there fore there is no calories or protein. I am not adding anything on to my calorie intake and I am not taking away from what I can eat in a day! Finally , a win win situation. I am satisfied and happy once again , its easier to be compliant with formula when you enjoy it! I enjoy my bettermilk so much I drink it after a meal so I can savor it! another bonus is that I don’t need to use a straw either! I have drank my formula with a straw for as long as I can remember and was very dependent , id have to take straws with me every where and If I forgot I couldn’t drink my formula. I have stacks of straws every where, my house, my purse, my back pack, at work , at my in laws, and even at my friends houses!

I drink my bettermilk in a sports bottle with water and shake it up. I am suppose to have 1 package 4 times a day, but because the volume is smaller with bettermilk I have been doubling up and drinking 2 in the morning with breakfast and 2 with dinner.  With no formula at lunch.  Specially on night shifts since I am asleep through the day and miss lunch anyways this works out really well with me. As long as they don’t change the taste again , I am quiet happy to continue to stick with bettermilk for as long as possible.

My Journey has been long, but I am happy with where I had ended up. It is so important to have a formula you enjoy and that you can drink. I could not imagine not being able to choose. We are so blessed to have so much variety now. I am very thankful that my clinic has such great aces to formula. They are willing to bring in any formula that is available in Canada for me to try and I thank them for that.

My journey would not be complete with out  big shout out and thank you to Cambrooke foods for inventing formula . It really does help my digestive system health. It helps manage my IBS and my sensitive stomach is very happy :)

 

 

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