BC Residents and news, CanPKU, facebook, Managing the diet, Social Media, That's my PKU life

PKU Strong.

Posted on November 9, 2018November 9, 2018 by Amanda Horner

Alone we are rare, together we are strong. PKU Strong.

What does it mean to be PKU Strong?

It means when faced with challenges we stand tall.

When a parent first receives a PKU diagnosis with their newborn, They stand up to the challenge and immerse themselves in our community. Learning everything they need to do to make our children strong, happy, healthy and normal. They mourn a little bit but then they learn that normalcy and a healthy life is still attainable and it pushes them through all the fears and dark days.

A parent raising a PKU child and just trying their best to give them the best start in life.

The PKU parent who spends hours and hours daily to log food records, take blood tests on their own child, makes formula, orders foods, weighs and portions their child’s food.

The PKU Parent who carries a scale in their purse.

The PKU parent who worries every day when their child is away from them that they are being fed properly or making the right choices for themselves.

The PKU parent who cries at night when their child is sick or cant get the last of their formula in.

A PKU child learning not to share their foods with friends or take food from others. Learning that what they eat can effect their brain development. Learning the yes foods and the No foods.

The first time they can explain what PKU is when asked. The First time they take their own blood dot or measure and weigh their food.

The brave face we put on every time we go to clinic and meet with our PKU teams.

The PKU teenager who is learning not to be peer pressured or not to be ashamed of their PKU. They learn to embrace it as a part of them and dont let it hold them back.

The PKU young adult when they transition away from their childhood clinic and team and start attending the adult clinic.

Every time a PKU’er tries a new PKU food or formula.

The PKU women who wants to become a mom’.

The PKU Women who experiences a Maternal PKU Pregnancy.

The PKU women who is raising children that do not have PKU.

The PKU adult who are learning how to feed their families a regular diet and cook or bake regular food.

The PKU’er who is learning to manage their diet through school, work , travel, and life.

Every time we take a level. Or go for our annual PKU blood draws.

When we learn to bake and cook and count the PHE in everything.

When we go out to eat and dont cheat or take the easy way out.

When we dont give in to temptations and cravings.

For those of us who have fallen off track and had to learn hard lessons.

For those of us who have fallen off and picked ourselves up again.

For those of us who constantly struggle to be compliant and maintain good PHE levels.

For those of us who just really hate this life and this diet but we do it anyways.

For those of us who want to give up or give in but try again tomorrow,

For those of us who dont let it break us. Don’t let it define us. Don’t let is consume us.

For those of us who have suffered high level effects and lost jobs, failed classes, failed relationships, lost hope, suffered physically and mentally.

For those of us who battle mental illness .

For those of us who each fight our own PKU battles daily.

For those of us who are trying, learning, pushing, accepting, and doing.

For those of us who walk this journey, whose life has been changed because of PKU.

For those of us have learnt and grown and developed skills because of our path and our journey,

For those of us who are a better person from learning and living with a rare genetic disease ,

that is PKU Strong.

I am not PKU Proud, I am not proud of a disease I was born with , that if their was a cure, I would take it. I am not proud I struggle , I am not proud that I fall down and get back up constantly. I am not Proud that I cheat and I eat things I am not suppoes to. I am not proud that I do not have the willpower to stick to my diet 100 % of the time. I am not proud that I make mistakes and I suffer from high levels a lot of the time.

I am Strong because I dont give up. I am strong because I dont let it hold me down. I am strong because I dont blame my life failures on having PKU. I am strong because I use my voice to advocate for others. I am strong because I want to help people avoid my struggles and challenges. I stand tall from the lessons I learnt and my past experiences with my diet and life. I am strong because I have seen both sides of the coin. I am strong because I have had bad high levels and suffered. I am strong because I have been very healthy with great levels. I am strong because I grew a life , safely and healthy .

I am strong because I keep going. I am strong because I dont give up. I am strong because I use my voice to improve the quality of life for others.

I am strong because I live this life. I have gone to school, I have changed careers, I have traveled, I have worked outside my home. I have a family, I have friends, I have a community. I have a team. I have a village. I am a mother. I am a wife. I am a friend. I am me.

I am PKU Strong.

BC Residents and news, CanPKU, facebook

Camp MagniPHEque

Posted on December 1, 2016 by Amanda Horner

Looking for Christmas/ Hanukkah present ideas? Just in time for the holidays, registration is now open for Canadian PKU and Allied Disorders’ first-ever PKU camp called “Camp MagniPHEque”- for those with, as well as those affected by, PKU. The camp is for children, teens, families and adults.

Registration is on a first come first serve basis and spaces are limited. Early Bird prices in effect until February 28.

The 2017 camp will run from Friday September 22 through Sunday, September 24.

Hope to see you all at the opening of Camp MagniPHEque on Friday, September 22, 2017.

http://canpku.org/canadian-pku-camp

BC Residents and news, CanPKU, Events

3rd Annual Kamloops Walk For PKU

Posted on May 28, 2016November 9, 2018 by Amanda Horner

Today, May 28th 2016 Brienna Wells and myself Co-Hosted the 3rd Annual Kamloops Walk for PKU at MacArthur Island Kamloops BC Canada. From 1:3o to 3:30 pm . We had around 60 attendees including the Honorable BC Health Minister and local MLA Terry Lake, Kamloops City Council member Arjun Singh . As well as The President of Canadian PKU and Allied Disorders John Adams himself. Who flew in from Toronto to join us on this rainy overcast day.

We spent the first few moments socializing and mingling. I got to meet my daughter Madelyn to Terry Lake. She had just woken up so she was shy on smiles for the first part of the day.

We opened the event with a few works from John Adams , who presented Terry Lake with a friend of PKU award. Terry said a few words and talked about his part in our PKU community as well as how we met and what we have done so far , with mention of Kuvan in the future. Councillor Arjun said a few words, He even mentioned he reads my blog!!!! What an honour! After I thanked everyone for coming and how great it was to see so many supports. I thanked Bri for her hard work and dedication. This event would not have been possible with out her! John then announced the walk and everyone headed out in small groups around the park. Cole, myself, Bri and Amanda J stayed behind. I did walk out to the end of the trail to meet everyone on their way back to take photos, and I had the pleasure of walking back with Councillor Arjun. We where able to connect briefly and discuss some of my history with diet . I do hope to keep in contact and further our conversation in the future.

After we enjoyed refreshments of cake donated by Costco and low protein cupcakes and donuts baked by Bri. Coffee and regular donuts donated from Tim Hortons. We had another chance to bid on the Silent Auction and then we wrapped up the event and announced our grand total.

Insert Drum Roll Here:

We raised 1500 Dollars for Canadian PKU and Allied Disorders INC !

The Event was a huge success. Our biggest yet. It was truly amazing to see so many families out showing their support of our community. It made me quiet emotional. I had trouble speaking and looking back now I wish I had of said more. OR at least taken Madelyn out of her Ergo carrier so she could see Terry Lake , or we could have had a photo with her actually looking out.

Madelyn was awake until Terry Left then she passed out on my chest in her carrier till the end.

For those of you who are wondering, Madelyn is doing very well. She is the light of our lives and such a wonderful baby. The changes and advancements I see in her daily astound me.

If you had of told me years ago that one day I would be standing in front of a crowd of PKU families , friends and supporters next to the BC Health Minister holding my baby girl on my chest I could not have imagined. Madelyn is so healthy and she is because of my amazing control of my diet, which I could not have done with out the Low Protein Food Subsidy that Terry Lake made possible.

So in away, with out Terry Lake, Madelyn would not be with us today. He has changed out lives, and the lives of all the PKU patients in BC. We are forever grateful.

So wraps up another annual PKU event. Chalked up as a great success and already has my wheels turning for next year. Always striving for Bigger and better. Ways to improve, things to change or add . We learn each time.

If you would like to host a PKU Walk-A-thon please feel free to contact me for advice or visit the new CanPKU website.

BC Residents and news, CanPKU, Events, News

Kamloops Walk For PKU

Posted on May 25, 2016 by Amanda Horner

PKU or Phenylketonuria is a rare metabolic and genetic disease where people who have PKU are born with out the enzyme to break down an amino acid in protein called Phenylalanine into tyrosine. The more protein they consume the more phenylalanine is consumed and it accumulates in the blood and causes neurological impairments leading to mental disabilities and brain damage. PKU is a serious disease that affects the brain but can be controlled with a restricted low protein medical diet. A person with PKU must drink a special medical formula, eat medical low protein imitation and chemically altered foods and test their blood regularly to keep phenylalanine levels in a safe and non toxic range.

The PKU diet is for life. The is one drug currently approved in the treatment of PKU but it is not covered in most canadian provinces.

when a women with pku wants to become pregnant it is recommend that they be on a pre conception diet for 3 to 6 months prior to conceiving. As levels in the uterus is 1.5 times higher in the uterus and can cause damage to a fetus. If a women with pku is pregnant and suffers from high levels it can cause brain damage and physical defects to the unborn baby. Blood levels must be maintained between 2 and 6 mg dl to maintain a non toxic environment and optimal growth development.

weather you are a women with PKU or or not, the low protein diet is very time consuming and challenging. Many adults who have PKU struggle with depression and anxiety as well as complying with diet.

All food even low protein medical foods all need to be weighed and measured , they need to be tracked by amount of phenylalanine per milligram .

Every day a person with PKU must track everything they eat, weigh out all foods, and

meet a daily intake of phenylalanine . They cannot be under or over their daily amount.

Intake can be tracked by phenylalanine, grams of protein and calories. Every gram of food, including oils, spreads, condiments, spices, fruits , vegetables , snacks, desserts and everything has to be counted. As every thing has protein in it.

If someone is not on diet or has control of there levels, they can suffer from a wide rage of side effects that can impact activities of daily living, maintaining relationships, and quality of life.

However if diet and control are maintained people with PKU can live a very normal life.

Despite having PKU , I have gone to college, became a nurse and worked in long term care for 6 years and now have an 8 week old healthy baby girl!!!!

I also spend most of my free time volunteering for CanPku , advocating for PKU , hosting events, and writing a blog about my life with PKU.

There is approx 200 people in BC living with PKU. There are approx 8 families in kamloops that has a loved one living with PKU.

On May 28th 2016 we will be hosting our “kamloops walk for PKU” At MacArthur Island from 130 pm to 330 pm and we hope to raise $1000.00 .

100 % of Donations will donated directly to Canadian PKU and Allied disorders INC to help fund education seminars, socials, patient suppots, research, and lobbying campaigns for coverage of medical supports in each province.

Please consider making a donation to support PKU and CanPKU.

CanPKU is not a charity so therefore we cannot issue tax recpits.

Please feel free visit the following links for our fb event page and our go fund me page :

Go Fund me page :

https://www.gofundme.com/kamwalk4pku

Fb Event page :

https://www.facebook.com/events/492861974252263/

BC Residents and news, Levels, Managing the diet, News

Interview with Nathan

Posted on April 19, 2016April 19, 2016 by Amanda Horner

Back in february before Madelyn was born , i was corresponding with Nathan Kuehne the young man from Victoria who is working on the home phe testing device.

After watching his amazing tedx talk on the device that was posted to youtube back in december i had a few questions so i wrote an email to nathan , and he has allowed me to share his responses on my blog.

Amanda :

From what I understand , the test turns purple in the presence of PHE. However since we area always eating protein and a limited amount of phe is always in our blood, this would mean that the test should always read positive? What I would like to know, is have you developed a way to accurately detect and read in numbers just how much phe is in the urine? Like reading? it is not enough to just have the presence detected, for treatment, we need to be able to read how much is present. Like on the home tests being developed with blood, they have a screen that produces a digital number. Will your device be able to do the same?

Nathan:

First off, I need to preface my answers to all the questions with a bit of a disclaimer. Due to the nature of a TEDx talk, it is hard to delve into the chemistry in sufficient detail to cover all the aspects of the project. As a result, many of the obvious questions that would arise from my work, especially for a PKU patient, would not be found online in the talk, but are questions I have definitely considered and I include in my other presentations (competitions, Science Fair, interviews, ect.)

Anyway, the answer to your question is both yes, and no. You are absolutely correct, any Phe will start the chemical process and produce nanoparticles, and thus the colour change. Having said this, the reaction is not a runaway train after that (bad analogy, my apologies). Only so many nanoparticles will be produced as there is Phe in the solution. This means a gradient will be produced depending on the amount of Phe in the sample. The more Phe, the darker the solution; I’ve included a picture that demonstrates this more easily than I can in words (Figure 1).

With this in mind, I was planning on developing something along the lines of a pH chart for people to read their levels. It could be a small piece of paper or something equivalent, and it would show a gradient of purple, and what concentration each colour corresponded to. This would give people the ability to attach a numerical value for blood concentration to their visual test, and thus determine how much is present.

Alternatives to the chart include using solution conductivity. Since the solution produces gold nanoparticles, the conductivity of the solution would increase as the amount of Phe increases. This way, I could imbed a program that converts solution conductivity to an actual value, taking away the risk associated with visual interpretation of a chart. While I acknowledge that this would be a much better method to go about the test, as a high-school student, it is slightly out of my reach at the moment to pursue and develop electronic integration to my test, therefore I am more included to pursue the chart at the moment. However, this may change as I continue to work on it.

Amanda :

my second question is : Many of the home tests in developed by the NPKUA and previously by Biomarin, have had trouble with accuracy when phe levels like mine are in the low range. Does your device operate similarly? how can you improve the accuracy for lower range levels? Idealy PHE levels in anyone should be 2-6 mg / dl so that would be considerably lower then say someone who is suffering from high levels. My highest level I ever had was 25 mg/ dl.

Nathan:

As I mentioned in my answer to the first question, there is a lot of more complex experimentation I leave out of my presentations, and this is one of them. As part of my research, I did tests regarding a concept called the Limit of Detection, or the LOD. Essentially, this is a test that determines how sensitive my test is, and gives me a value of how low the concentration of Phe can be for me to know if the colour change is because of Phe or if the colour change is due to experimental error.

My LOD was below that of the low-end range of Phe levels in PKU patients, according to published literature, meaning my test works for all concentrations of Phe. However, I am not 100% sure how the accuracy changes at those lower levels – that will take more research to determine. I am optimistic, considering the reliability of gold nanoparticle production with Phe, that the accuracy should not change very much.

Amanda:

There are 2 systems for reading PHE results. In canada we use the milligrams per deciliter and in the states or the uk we see that they use the umol/ L system . where levels range is 120-360 . What system would your device use?

Nathan:

Regardless if I pursue the chart system, or the conductivity system, since mg/dl to µM/L is a simple conversion, it would be very to make my test useable in both locations, with both sets of units included.

Amanda :

have you been approached or applied to either the NPKUA, CanPKU or the government for any sort of assistance in developing your device or plan to?

Nathan:

I have not been approached nor have I applied to any agency or organization for assistance, and this is for a few reasons. The main reason is that I am working on a slightly different project at the moment, which may replace the device I developed and presented in my TEDx talk. I am trying to port the chemical aspect of the test to a specially designed piece of paper, and as a result, remove the need for the device. Because as much as I like it and am proud of it, it is large and fairly cumbersome. As an extension, this new test would, theoretically, include the capability to test for types of cancer, Alzheimer’s, and other metabolic disorders like PKU. It is still in the research phase, and as a result, I can’t speak to whether it will be successful or not. If all goes well, this new platform will be the method of testing for PKU.

If it is not successful, I will consider returning to the device, and trying to optimize parts of that system. With this in mind, I have not sought out funding or support yet because, quite honestly, I am not sure what will work best and as a result, what I may try and develop further.

Amanda :

And lastly, if it is ok with you, once you have replied, may i publish this conversation to my blog? with your permission of course. I think these are all questions we have had since you have entered our world and it has spurred many conversation on social media.

Nathan:

Yes, I would be happy for you to share my answers. I have been in contact with a few other members of the PKU community as a result of places I have posted my talk, and I would love for these answers to reach people like them.

BC Residents and news, News, Social Media

PKU and Poltics.

Posted on September 25, 2015 by Amanda Horner

With the coming Canadian election approaching us on October 16th 2015 I wanted to take a moment to talk about voting and politics.

As a young adult I never had any interest in politics. I just didn’t understand , never did the research of get involved. I had no interest and found it all confusing and daunting.

As I grew older and got more involved in the PKU community I began to see things differently. The more I got involved and the more I heard the voices of others around me, I learnt the importance my voice could carry if used right. My experiences in public speaking, awarness and advocacy efforts with CanPKU taught me a lot about politics and how to work towards change. I’ve attended parliament with CanPKU and spoke at the legislator to politicians, I have written campaign letters and done petitions and I have done my research into each party and what their platforms are.

It was not until the last provincial election that I really got involved. After years of watching and listening and researching and learning how to talk to politicians how to ask and how to deal with them I reached out to our MLA candidates about why I should vote for them in the provincial election? Just how would they help someone like me? Most of my letters and emails went unanswered or dismissed. I requested meetings with each member. One MLA called me and we talked on the phone. However one MLA did answer my request. Our now BC Health Minister Terry Lake met with me for 2 hours! It was such a positive experience and meeting that The honourable Terry Lake earned my respect and my vote. After the election he was named the health minister and shortly after we received low protein food subsidy for all PKU patients in BC.

Since my experience with Terry, I have followed his career as health minister not only for PKU but because I am a nurse. I work as an part time Licensed practical nurse in an extended care hospital for the elderly. I follow the issues in health care and the politicians promises and platforms on how they will or how they plan to make a difference.

I follow other social issues now, things that effect myself, my PKU community, my health care community, my fellow nurses, my family, and what effects cole and I personally. I have taken time to understand my beliefs and views . I know what issues I think need to be changed. We watch the news and watch the debates when we can.

I really believe the more voices that join together and tell our government what we want the more change we can make in our beautiful nation. I find it irritating and frustrating that so many people choose not to vote. I am writing this post today to encourage everyone to get involved. to take the time, be interested, do the research the parties , the leaders and the platforms. Find where you stand and who you side with. Look at the issues that effect you , or that you are about. What drives your passion, and understand the way voting and government works.

All to often I hear people say they won’t vote because they don’t like the options, that it comes down to the lesser of the evils, or that there voice won’t matter. All voices matter. The more of you that vote depending on what party you choose, the better the chances of that party getting into power there is.

There are some great links online to help you understand.

Here are 2 of my favorites.

Strategic Voting 2015 Canadian Federal Election

http://www.strategicvoting.ca/

and :

Canadian Election Quiz, I side with.com

https://canada.isidewith.com/political-quiz

i found the second link to be extremely helpful as it showed me what issues where the most important to me and how much I agreed with each party in percentages.

Dont get me wrong, there are pros and cons to each party. There are things I like about each and things I hate about each. I am currently torn between 2 parties but am leading more closely to another.

When making my decision I also look at the party leaders. Who I feel would make a better leader. I also look at the past track records, and development of past issues and how they where changes or not changed. I also look to the future as some things we face today we have never faced before. I look at the promises and the facts. I also look critically at the adds, specially the attack ads. I have no respect for the attack adds. It seems to me only one party is truly advertising and has not openly attacked another party in an add. I look at facts, science, and my community. I look at what affects me at work, at home, and in my province.

Voting is a right, a responsibility and an honour years ago women did not have. So please take the time to research and exercise your vote. its simple and has more impact than you can know right now!

It is time for Change in Canada and I am calling on all of you to get out in vote!

BC Residents and news, CanPKU, Events

Kamloops Walk For PKU May 30th 2015

Posted on May 31, 2015May 31, 2015 by Amanda Horner

WOW! what an incredible day!

The weather was a gorgeous 31 degrees and we had a sucessful walk where we raised $1287 dollars for CanPKU. We had around 25 walkers. 3 adults and 2 kids with PKU. Tons of food and lots of fun!

After the event, the other 2 pku adults ( Brittany from kelowna and Amanda my friend from kamloops) came over for dinner. I made us all low protein spaghetti. We hung out and shared our pku experiences, while bonding and getting to know brittany . We made summer plans to meet more often! maybe even a BC Adult get together in the future?

Amanda Brittany and I drank Davids tea and played mario wii. It was a nice way to unwind after a busy day.

Now the house is clean and quite and I am off for a bubble bath!!

To see more photos from the event please visit my facebook fan page!

BC Residents and news, CanPKU

Dare to dream- update on PKU home testing device; Victoria Teens test himself, in hopes of helping others.

Posted on May 24, 2015May 24, 2015 by Amanda Horner

Great things come from Victoria BC ( not just me LOL) this teen is one of them!!!

This is so amazing!!! This teen is from my home town of Victoria BC , Where I was born and raised . ( I relocated to kamloops in summer 2012)

and he is responsible for helping making my life long dream as well as many others come true!

Gotta admit it was both surprising and very exciting to log on today and too see this in my email today from my mom.

Not just that its about PKU and its featured in my hometown paper the Times Colonist . I never thought id see PKU and Victoria connected in the news in this way!!

Very proud Victorian moment!

I plan on contacting this young man and hope to meet him when I go home to visit this summer!

The Article in my hometown paper the times colonist today is as follows :

Nathan Kuehne went to the Canada Wide Science Fair two years ago and nothing has been the same since.

It’s not so much that the Glenlyon Norfolk School student won a silver medal for research on the optimum amount of charcoal in soil for plants.

It’s more that he got his first look at the type of research being done by other students across the country.

“Kids had developed new ways to create batteries,” he said. “Kids had actually developed treatments for cancer.

“I hadn’t been exposed to that kind of high school research before.”

Here were average high school students devoting hundreds of hours outside the classroom to research that could really make a difference to people’s lives, he said.

Inspired, he returned home and embarked on work to develop a urine test for tumour-based cancers under the tutelage of Fraser Hof at the University of Victoria.

In his understated way, Kuehne, 17, said the project was “largely successful,” picking up a gold medal in his Grade 10 year.

“The problem was it required an expensive machine to run,” he said, whereas his goal had been to develop an at-home test.

He proceeded to modify the test in Grade 11 and found that, while the new version didn’t work for cancer, it was effective at monitoring phenylketonuria, or PKU. Those with the genetic disorder are unable to break down the essential amino acid phenylalanine, which is commonly found in protein-rich foods and some artificial sweeteners.

The disease affects about one in 12,000 newborns in North America, according to the website of Canadian PKU and Allied Disorders Inc., a non-profit organization. If left untreated, the disorder can lead to developmental disabilities and other neurological problems.

Once Kuehne discovered that his test could be used to monitor phenylalanine levels, he learned there was a desperate need for an at-home device that would allow people with PKU to monitor their condition and prevent symptoms. Other tests can take weeks to give results, while Kuehne’s takes less than 10 minutes.

“That motivated me, but also gave me an acute sense that this is something that could really make a difference,” he said. “People are suffering without a test like this right now.”

So last summer, while other young people his age were at the beach or otherwise enjoying their vacation, Kuehne was putting in eight-hour days in Hof’s UVic laboratory.

“To excel at science fair, you have to be willing to try experiments that you don’t know if they’re going to succeed,” said Erin Dallin, Kuehne’s chemistry teacher and mentor at Glenlyon Norfolk.

“There were times when it just didn’t work and he came back to the lab the next day and kept going. So that’s a lot to ask of somebody who is a teenager.”

Kuehne’s drive and determination led him to a first-place finish in the Vancouver Island Regional Science Fair and another gold medal at the Canada Wide Science Fair, where he finished in the Top 10 among 469 competitors.

He recently filed a patent application for his self-diagnostic test and hopes to publish his work in a peer-reviewed journal.

“I hope to keep working on it,” he said.

The son of a university professor and an accountant, Kuehne plans to pursue a career in engineering or medicine with a focus on research.

“I love exploring new boundaries,” he said, “but at the same time, I like seeing them applied to people.”

lkines@timescolonist.com

– See more at: http://www.timescolonist.com/news/local/victoria-teen-tests-himself-with-hopes-of-helping-others-1.1945269#sthash.8ftW1N7G.dpuf

A note to Nathan:

Words cannot describe how much this meas to me. As an adult from victoria living with PKU! This is history in the making and will have such an impact on lives like mine. Truly a dream come true and nothing I thought i would see in my life time. You simply cannot know how this will effect and change our lives. I have been waiting for this. Feels like a dream!

Amanda Cosburn LPN and PKU Adult.

Since posting this post, Nathan’s Science Teacher Erin has contacted me through my pku facebook page and we are planning to meet up when I travel home to Victoria in June. I look forward to shaking this young mans hand and thanking him. This one grade 11 youth is potentially responsible for changing my life, for impacting many lives and the course of PKU treatment .

I am PKU Strong

I am PKU Strong

My life with Phenylketonuria

Category: BC Residents and news

Camp MagniPHEque

3rd Annual Kamloops Walk For PKU

Kamloops Walk For PKU

Interview with Nathan

Kamloops Walk For PKU May 30th 2015

Dare to dream- update on PKU home testing device; Victoria Teens test himself, in hopes of helping others.