Category: BC Residents and news

BC Residents and news, Low pro food / cooking, Managing the diet

Wish list

Posted on by Amanda Horner

I get asked alot about what products I like and am currently filling out the survey for BC residents on what items we would like to see with our subsidy and I thought I would share my ” wish list” with you. I have tried all these products before. They make my diet way easier and give me more variety and easy options for work and school. You will notice there are no pre made breads or pastas on my list. Aproten is my most favorite pasta and I have that already. With breads, I have better success making my own then any pre made. I find them tough and don’t always come in good condition. I consider them a waste of money. I prefer to make my own with wheatstarch, or the cook for love recipes, and of course the taste connections. SO instead my wish list is items I cannot make myself.

Here is my wish list , if I could choose what foods I would like to see included that are currently not included under our subsidy.

These do not include the products I love and have access to already. Special products now referred to as the BC Medical food nutrition program, does a fabulous job and has a lot of selection already!

Taste Connections :

Low protein bread mix,

multi baking mix,

versa mix,

wheatstarch ,

sugar cookie mix,

cake mixes ( strawberry and lemon)

I have been asked why I prefer taste connections of cambrooke, its not that they are better, I love cambrooke am a proud supporter . They also have great customer service too. However Taste Connections products as I have said before are made with more whole nutrition, no chemicals or GMOs and are made by Malany. They also imate “regular” food textures so closely anyone can eat them and you cant tell the difference. They don’t taste like medical or imitation and the mixes are so easy to work with. If you have never had “regular ” breads or bakery products you would not know the difference and thats fine. however I use to cheat a lot and miss those textures, and the taste. Taste connections is the closet I have ever found!

Cambrooke Foods:

GO pockets ,

Bigger bagels,

ravioli,

mini pizza pockets,

pita pockets,

tweez ,

cozy morning cereal.

Cambrooke foods is my go to store for those pre made foods I cannot make myself, or do not have the time to make. They are so convenient its like going to a grocery store. They are supper easy to take to work or school and traveling. with way less time and energy! These are my go to products that I miss from them.

PKU perspectives:

La Tiara Taco Shells,

Country Sunrise BACON MIX 

Country Sunrise SAUSAGE Mix 

Wai Lana Chips

What I love the most about PKU Perspectives is their country sunrise products and the “meat alternatives” that have given me the opportunity to try new things , things I would never have dreamed I could have before with a normal looking meal when I sit down. Like camping making low protein smokies or burgers. I am not a big fan of the cambrooke pre made meat alternatives but love the textures of the pku perspectives ones and the ease of making them on my own!

Toronto Specialty food shop:

Frostline Non-Dairy Ice Cream Mix – Vanilla

I have actually never had this, and usually opt for a lower in protein ice cream option, or i splurge and have regular ice cream as i have never found a suitable replacement to the texture and taste, so wouldn’t it be nice if this was my answer for those summer time ice cream cones!!

So there it is, my wish list of products I would like to see covered in BC along with our products already covered. Do you agree disagree? what are your go to foods? Would you like to share a review or experience on a food I have listed on my wish list? Would love to hear feed back on how you like an item and maybe see your review on my blog? email me with your submission.

Please also dont forget to fill out the survey for the BCMNP through CanPKU. The link is in my previous blog post and on fb! It is 2 questions and very quick and easy. You input is very valuable and needed! Nicole Pallone is meeting with the team on thursday so the sooner the better!!

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BC Residents and news, Low pro food / cooking

Special Products Distribution centre update.

Posted on by Amanda Horner

just had mini heart attack from a misunderstanding. I had hear the BC Children’s hospital Special products distribution center was closing down, then I read on the website that the retail side of SPDC is closing and panicked. So to clarify after speaking with the staff at SPDC, they are not closing down! They are shifting focus to the metabolic foods . They will not be selling retail items like breast pumps, feeding bags, syringes and what not, they are continuing with the BC metabolic food fund. Currently they have a lot of back order specially from cambrooke that has not come in yet. They have no estimation of time on when they will be in and the current October order form is not available online as the whole website and systems are being updated right now. The website will be a metabolic food website. Please check back in a few days if you are wishing to place your October orders!

ahh panic over and hopefully crisis prevented for anyone else whom may of been confused!

Now once again having just looked at the current page that is up through the BC childrens website, SPDC list 2 list for low protein food, one stating covered foods such as low protein staples (baking mixes, bread/cereal products, sauces, protein food replacements ) and then a list for

Non-staple Low Protein Foods may be purchased. This greatly concerns me as I am worried all the “GOOD” food will fall under this category, like the cambrooke foods, such as the eggz, tortillas, or the maddys muffins and cakes. or any of the pre made cambrooke foods we have been asking for them to bring in . Looks like I have some more investigating to do , to bring you all the answers. Hopefully the new website that will be up in a few days will provide some clarity as all links are currently broken and I cannot investigate further that way.

I know cambrooke has been trying to work with the BC government to have their foods subsidized and delivered to our doors, but I have heard no update on their progress , I choose to remain hopeful.

You will know, as soon as I know.

Amanda

1
BC Residents and news, CanPKU, Events

BC PKU Day 2014

Posted on by Amanda Horner

IMG_3363 IMG_3357 IMG_3358 IMG_3359 IMG_3361 IMG_3362 IMG_3356 IMG_3354 IMG_3353 IMG_3351 IMG_3350 IMG_3349 IMG_3348 IMG_3347 IMG_3346 IMG_3343 IMG_3342 IMG_3341 IMG_3340 IMG_3339 IMG_3338 IMG_3337 IMG_3336 IMG_3334 IMG_3333 IMG_3332 IMG_3331 IMG_3328 IMG_3327 IMG_3325 IMG_3324 IMG_3323 IMG_3321 IMG_3320 IMG_3319 IMG_3317 IMG_3316

 

This past weekend was BC PKU day 2014, on April 5th. At BC Children’s Hospital, in the Chan Auditorium. This was our 4th Annual BC PKU day hosted by Canadian PKU and Allied Disorders INC. Though it was one our smallest in attendance it was one of my favourites.

We left Kamloops at 430 am in the morning and drove down to pick up Katie at the hotel and head to the event. We got there just in time for the 9 am start. I set up a booth for CanPKU merchandise that included the BC Campagin t-shirts, CanPKU awareness Bracelets, the “PKU and the Brain” book published by CanPKU, as well as stickers and magnets.  I assisted briefly with registration table and of course took photos at the event.

This year though I had the pleasure of enjoying more of the event as a guest and I leant a lot! We had some fabulous speakers this year and I feel that the day flowed really well. Each speaker was perfectly placed that they flowed from topic to topic, breaks where scheduled well and the food was the best ever!

I was also one of 5 of the Adults who attended. That was great to see that the numbers of adults in attendance is slowly increasing! Including myself Ian ( Male PKU Adult, originally from Ontario. Now living in Vancouver ) Katie ( Female PKU adult from Kamloops and my good friend ) Kim ( Female PKU Adult from Pentiction,  my friend and fellow nurse) and Tamara ( Female PKU Adut from Surrey BC ) all attended the event. I always enjoy socializing at the these events and answering questions for the other families so having other adults there and hearing about there difference experience is always exciting.

Our first speaker was Chad, We met a few years ago when he first attended one our events with his husband after adopting a baby with MSUD also known as maple syrup urine disease, another rare metabloic disease similar to PKU. Their son follows a low protein diet and drinks a special formula and does blood dots too. However it is 6 times more rare than PKU, and it is also deadly! There is NO coming off diet, instead of having high PHE levels, their son cannot break down the amino acid leucine. Instead of causing the same side effects of PKU, with in 4 days it causes brain swelling, coma and death!!  Chad joined our team of advocates and helped us lobby for coverage of the low protein foods and thus attended the event this year to talk about his advocacy and awareness journey. Chad and his family have accomplished so much and have raised so much awareness for MSUD but also funds for BC Children’s Hospital. Chad is a a wonderful speaker and his talk was very captivating and educational. His talk gave the day a strong start that was carried on forward by the remaining speakers. 

Following Chad was Dr. Jerry Vockley. MD and PhD. ( read his profile at : http://www.chp.edu/CHP/Vockley,+Gerard,+MD,+PhD)

Dr. Vockley spoke to our group about Latest PKU research , Current Trials , Peg Pal and the new PKU Guidelines that where just published not to long ago. Dr. Vockely worked on the new guidelines and is also working on Peg-Pal. Of course just a few of many of his projects. I learnt alot about Peg Pal at this years event and am hopeful with the information we received that this really may change the face of PKU. Though it has not been approved for trails in Canada and is only being studied in America. They are having promising results. Unlike Kuvan, Peg Pal is a daily injection similar to insulin to diabetics. Peg Pal works by breaking down the PHE in your body and making it non toxic. It is not based on weight such as KUVAN. Peg Pal comes in amounts such as 10/20/40 mg injections and the doses are regulated by the medical staff.  Peg Pal is suppose to work with ALL forms of PKU and for ALL PKU patients. Not just non – classical.  They have seen results from Adults who have had levels around 20-30 mg/ dl go on PEG Pal and with PEG PAL, and a low protein diet the levels where brought down to around 3 to 4 mg/ dl and lower. So I will be closely following the progress and hope to learn much more about it.

As well as Peg Pal , Dr. Vockley talked to us about possible future treatments for PKU including stem cell transplants and liver transplants. Things you think of as “futuristic” and to “sci-fy” but are actually a real possibility. This does create excitement for myself and many adults. Currently these therapies and PegPal are not being tested in paediatrics. When Peg Pal switched to Paediatrics it will be dosed accordingly to weight.

I have had the pleasure of being apart of 2 talks by Dr.Vockley now, once in 2010 and our BC PKU Day and of course now. The man never ceases to amaze and awe me. An incredible PKU hero in my mind! I am looking forward to see what he does next!

After Dr. Vockleys speach, local Dr. Sylvia Stockler from BC Children’s Hospital spoke briefly about KUVAN and how to become part of trails and their current research project. They have some coming up that they need volunteers for. Unfortunately nothing for adults. I really wish our clinic for the adults where partaking in studies and research development specially for treatments. I find it so frustrating that they do not. Dr. Stockler had a guest speaker with her as well .

Than it was time for our annual group photo! Even with such a small turn out we still manages to get one this year. Cole was feeling sick and tired this point and had not eaten so he was to shaky to be our photographer for the group photo this year. Kim’s mom Bev, volunteered to take our group photo. Here is the one from my cell phone edited with instagram:

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We had 40 registered guests this year and 10 sponsors, speakers and vendors.

After our group photo was the lunch and social time. The morning had been pretty long with no break so we where all pretty hungry and egar to eat! Poor Katie had got food poisoning from the hotel restaurant and was unable to eat any of the delicious low protein food options.

I made myself a low protein sandwhich , grabbed some cookies and fruit, but then I was grabbed by Jen and told about the low protein tortillas that one of the vendors was making fresh on the scene! to my delight , It was Malathy Ramanujam from Taste Connections! She was making low protein vegetable meat balls, and fresh tortillas. WOW! They where amazing! I ended up eating 3 tortillas, with 3 “meat balls” in each one! I was so stuffed!! The “meatballs” where made with grated vegetables like beets and carrots, with bbq sauce and a little flour to bind. This is so now my new favourite dish and I was ecstatic to learn that at the end of the day she would be hosting a cooking demo for us! Even all the non – PKU family and friends tried her dishes. Even Cole! and he loved them! 

During the break I tried to float around and greet as many friends and families as I could and catch up. It was great to see everyone mingling and chatting. I had a great chat with our Cambrooke rep Eric Parker at his table about the formulas, new products and the future of Cambrooke therapeutics and our new BC food subsidy program. Needless to stay I will have some more news to announce at another time in my blog regarding our talks so please check back!

After the break we had 2 more speakers and the cooking demo.

Immediately following the break our next speaker was  Dr. Kendra Bjoraker , Neuroscientist . Who was speaking to us about PKU and the brain. Growing up and developing with PKU. Education and parenting of a PKU child, and an insight into the workings of a PKU patients brain. Our weakness, how we learn , how we grow and develop and many other interesting aspects. I found her speech particularly engaging. Partly because she use to be a special education instructor and had different techniques during her talk, diagrams, and props. Dr. Bjoraker spoke to us about how the PKU brain works, about working memory, processing speed, social, emotional and psychological aspects and side effects. I found I related to much of what she had to say and wondered to myself if doctors and my family had of know much of this information when I was younger if my childhood and education would have been different and how it would have benefited from this knowledge. I do not consider my learning difficulties as a disability or that I have disadvantages , but a lot of what she said struck deeply with me as well as a few other adults. I have hard to learn along the way what they now know to be for PKU brains and development and I found myself nodding and agreeing about statements that where made. Examples of how I learn best, where my weakness lie, how I process information, make decisions, and how my high levels effect me. I found myself saying a lot ” yes that’s me! ” I had to laugh when she talked about attention span and how we tend to drift or do a few things at once, because I was sitting there very visibly in the back row on my cell phone. Though I was live tweeting the event! 

Our last speaker was  Denise M. Ney, PhD, RD. Who spoke to us about GMP and Whey Protein in the new formulas such as Cambrooke Restore, Bettermilk, and others. I found this speech particularly informative as I drink bettermilk. We learnt about the benefits if GMP. As well as the harvesting process, how the formula is made and why . Denise conduct research in mainly 2 areas, gastrointestinal physiology, and the nutritional management of PKU. Which lead to the development of our GMP formulas. That is actually natural  protein found during the process of making cheese. After hearing her talk, It is a wonder the hole group of participants with PKU would not all want to  switch to a GMP formula immediately. I know it has done wonders for me. I have benefited from it greatly.

After Denise’s talk we had a short break that I spent talking to Eric from Cambrooke foods about some of the information I learnt from Denise.

Following the break was our cooking demo with Mal from taste connections. It was also probably my most favourite part of the day. Not just because of the great tasty food, but also because Mal was a great speaker, very engaging. Also because I have a special interest in low protein food, new recipes, products and am always looking for new ideas to make. I was particularly delighted to learn about how nutritious the taste connections products are. Not are they simple to make, convenient and do not require the addition of more ingredients but also they include ingredients I am familiar with, can pronounce and are developed my Mal! Mal had everyone try her recipes. Not just our PKU attendees. We where all speechless! Not ever have I seen such flexible doughs, easy to use products and textures so very evenly matched to regular food! I fell in love with the fresh English muffins as well as of course the tortillas. I am so impressed! In the hour that Mal spoke with us she demonstrated many different recipes. She made the meat balls, the tortillas, chocolate cake pops, English muffins and more. It was so simple, mostly add water. And it was all cooked quickly enough that we got to eat all! ( what was left over I got to take home too!! ) I cannot wait to ask SPDC to bring in these baking mixes so I can give it a try. She made it look way to easy! But wow the textures of the food, specially the muffins where moist soft and delectable! I am now a new fan of taste connections and really cant wait to get our BC Subsidy on board with covering this product.

So as you can see, yet again BC PKU day 2014 was a true hit and in the end I am so happy we where able to attend. Recent events in our personal lives almost prevented us from attending this year, but as always it was so well worth it and I would have been devastated to miss out. A huge shout out and thank you’s to CanPKU for hosting this annual event, and to all our terrific speakers!

I cannot wait to see what BC PKU day 2015 has in store for us! Not sure how they will top this one!

For those of you who did not make it, I hope to see you there next year!

 

 

 

 

4
BC Residents and news

Pedal 4 PKU.

Posted on by Amanda Horner

I am so excited about Avery’s Ride for PKU and so honoured to have her Uncle Kevin take on such an amazing fundraising and advocacy event.  This is sure to catch the eye of the government, the people, and the media. I am sure it will bring much more needed awareness to PKU and am looking forward to helping out any way That I can.

 

I am offering to host Kevin if he shall chose. I also and offering to host and organize an event in Kamloops when Kevin rides though. Maybe If i am in shape enough by the time he reaches here Cole and I can meet him on our bikes!

I also hope to Collect Donations to donate to Kevin and CanPKU.

 

I am also going to reach out to Thompson Rivers University and my nursing student friends to see if they would like Kevin to guest speak on PKU , while he is in town.

There are many ways we can all show our support to Kevin, Avery , PKU and CanPKU. For more details on how you can support this event or on the ride across canada please check out the website at : http://www.rideforpku.ca/

One of the major ways you can all help is by using your Twitter account (if you have one) to spread the word.  Please post one or more of the following tweets to spread the word

Suggested tweets:

  • ·         We are happy to announce Avery’s Ride for PKU! Ride starts in #Victoria June 7th. Follow her Uncle’s journey here @Averysridepku #cdnhealth
  • ·         #PKU uncle will cycle across #Canada in 71 days to raise awareness and $25,000 http://bit.ly/1czLeYi #cdnhealth #cdnpoli #rarediesease
  • ·         @Averysridepku will travel coast to coast to make a difference for those living w #PKU. Find out more & support here http://bit.ly/1czLeYi
  • ·         “a cross-Canada bike ride would be a great way to advocate, raise awareness & fundraise for those living w #PKU” @dube_kevin #cdnhealth
  • ·         “There is not a single province/territory in this country that doest need to improve #PKU funding in some major way” @johnadams51677
  • ·         Help support @Averysridepku!! Find out more here http://bit.ly/1czLeYi#pku #rarediease #cdnhealth #cdnpoli

 

I will do my best to Blog about the Ride , events and details along the way! So be sure to check back  :)

 

Your PKU Friend Amanda C

 

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BC Residents and news

Important information for BC Residents

Posted on by Amanda Horner

In regards to the new food subsidy  program announced in November 2013 by the BC Health Minister Terry Lake , our BC Children’s Hospital Special Product Distribution Center now has a food list of covered foods on their website as well as order forms. As well as important FAQ’s you need to know regarding the program.

With the high orders of new food orders SPDC has been receiving they have noted on the website that it is not possible to respond to every email that your order was received. They ask for you to be patient with them and give your order 1-2 weeks to arrive. If it does not arrive in this time to call them immediately.

The new order form is really quiet easy. It is available online, it is an Excel document with a list of available products. You enter in your information and the quantity of each item that you want and it tallies the amount on its own. Save the document and email it to SPDC.  They are no longer taking emails as orders.

Also if there is any products you would like to see available though the program to email them your suggestions. I have asked for the country sunrise products specially the omelet and egg mix. SPDC will do their best to try and get the products in.

If you have any questions regarding the program, the new ordering excel sheets and process, please call SPDC or you can email me and I will do my best to help you.

Please see the list below for more information:

http://www.bcchildrens.ca/Services/ClinicalDiagnosticFamilyServices/NutritionServices/SpecialProducts/LowProteinFoods.htm

 

 

 

0
BC Residents and news, News

B.C. to subsidize food costs for those with rare genetic disorders – BY KIM PEMBERTON, VANCOUVER SUN

Posted on by Amanda Horner

The PKU disorder limits a person’s ability to break down proteins often found in wheat, dairy, meat and fish products.

 

More than 200 British Columbians diagnosed with rare genetic disorders such as phenylketonuria (PKU) will get a break on their food bills after the province announced Thursday it will provide a monthly food subsidy of up to $250 to pay for low-protein foods.

There are 209 patients being treated for metabolic disorders in B.C. of which approximately 170 have PKU. The PKU disorder limits a person’s ability to break down proteins often found in wheat, dairy, meat and fish products. A person with PKU must eat specially modified, low-protein breads, pastas and other foods along with fruits and vegetables, which can be expensive.

Newborns in B.C. have been routinely screened for the brain-threatening disorder since the 1960s. Every year, two to three children are diagnosed with PKU, but it is treatable.

Provincial Health Services already supplies the metabolic formula that PKU patients need for proper body development. And with Thursday’s announcement of the food subsidy program, British Columbia joins six other provinces in providing people with PKU financial assistance for low-protein foods.

Kamloops nurse Amanada Cosburn said the news also means she and her husband now feel they have the option of having a child, since as a PKU patient she didn’t want to try to be a mother until she felt certain she had the means to ensure her health.

“I’m thrilled. As an adult with PKU it has been my dream for this to come to pass. It will drastically impact many lives and make it easier to comply with the diet to keep my brain safe,” she said.

Cosburn explained that she works as a casual nurse and before that she was working at minimum paying jobs so found it difficult to afford the special food she needed.

Nicole Pallone, the vice-president of Canadian PKU and Allied Disorders said the food subsidy program will improve the health outcomes of many patients, but more is needed.

“Obviously, we’re thrilled. It’s a great step in the right direction,” she said.

Pallone said the organization is advocating for the provincial government to spend $2.8 million in total to improve the lives of people with PKU. Part of that request was to cover the $700,000 estimated annual cost of the food subsidy program and also to increase services at the one adult PKU clinic and one child PKU clinic in B.C.

The remainder of the requested money would go toward paying for a new drug called Kuvan, which is now publicly funded in Ontario and Saskatchewan and for some patients in Quebec.

“We are delighted the minister of health has listened to our needs in terms of the necessary medical foods, and we are hopeful that the ministry will continue to improve the health outcomes for PKU patients by providing public funding of Kuvan,” she said.

© Copyright (c) The Vancouver Sun
0
BC Residents and news, News

BC Metabolic Nutrition Program for People with Inherited Metabolic Diseases

Posted on by Amanda Horner

BC Metabolic Nutrition Program for People with Inherited Metabolic Diseases
After an extensive review, the Ministry of Health has approved funding to help patients with Phenylketonuria (PKU) and other Inborn Errors of Metabolism (IEM) with the cost of low-protein foods.

On January 1, 2014, B.C. will join at least eight other provinces in providing some form of food subsidy to PKU and IEM patients.

British Columbian patients with PKU and IEM will receive up to a $250 monthly subsidy to help with the high cost of low-protein foods.

The program will be managed by the Provincial Health Services Authority as an add-on to their already-existing supplement program, which supplies a free metabolic formula to PKU patients. A program based clinical nutritionist will work with the patient(s) to determine the required patient dietary needs (e.g., caloric intake and low protein foods required).
What does the program cover?
The BC Metabolic Nutrition Program (BCMNP) covers the cost of certain outpatient drugs, nutritional supplements and specialty foods used to treat PKU and other IEM.
The program covers approved:
IEM formulas (medical foods) and nutritional supplements (e.g. L-amino acids).
As of January 1, 2014: Special low protein foods up to $250/month distributed by the Special Products Distribution Centre (SPDC) located at BC Children’s Hospital.
Am I eligible?

Talk to your doctor, an eligible person must be:

Resident of BC with a valid BC Services Card or Care Card;
Diagnosed with PKU or IEM and be under the medical care of a doctor from BC Children’s Hospital Biochemical Diseases Clinic or Vancouver General Hospital Adult Metabolic Clinic; and,
Be prescribed the IEM formulas, supplements, and low protein foods as a medical treatment.

 

 
Frequently Asked Questions (FAQs):

Q: If I ordered less than $250 of approved low protein foods one month, can I order more the next month?
A: Yes, there will be some flexibility as to how the funds can be spent. Eligible people are allowed a maximum benefit of $3000 per year for approved low protein foods, but due to expiry and ‘best before’ dates, it will be advisable for an eligible person to order only the amounts that they can consume before these dates.

Q: What low protein foods will be covered under this program?
A: Initially the approved low protein food list will include shelf stable low protein foods that are currently available at the SPDC. By January 1, 2014, a list of approved low protein foods will be posted to the SPDC website: http://www.bcchildrens.ca/spdc.

Q: Will I be able to order perishable foods such as approved frozen low protein pizza or bread?
A: The funded approved product lists will be reviewed periodically by an expert advisory committee. Once logistical and storage issues have been managed, additional foods will be added to the approved list.

Q: Will eligible people have input as to which low protein foods are included in the approved list?
A: Yes, a process will be developed for eligible people to put forward suggestions for new low protein foods to be added to the approved list. These requests would be reviewed periodically by an expert advisory committee.

Q: Where can I learn more about the program?
A: You can ask your Dietitian or Physician from BC Children’s Hospital Biochemical Diseases Clinic (www.bcchildrens.ca/Services/SpecializedPediatrics/BiochemicalDiseases/default.htm) or Vancouver General Hospital Adult Metabolic Clinic (www.vch.ca/403/7676/?program_id=451) for more information. Once the program is operational in January 2014, a list of approved low protein foods will be posted to the SPDC website: http://www.bcchildrens.ca/spdc.

 

 

0
BC Residents and news, CanPKU, News

Let the Happy Tears Fall,

Posted on by Amanda Horner

I am terrible with secrets, and I have been holding a big one for weeks! My heart is over joyed and pouring out happiness and excitement to finally be able to share this great news!!

The British Columbia Health Minister Terry Lake announced today, a $250.00 subsidy for low protein foods. This subsidy is for patients with metabolic disorders in BC such as PKU and Maple Syrup Urine disease who rely on these specially modified medical necessary low protein foods to maintain healthy blood levels and prevent mental retardation.

I have been working towards this goal since 2008 when I first made the decision to open up my life with PKU to the world and use my voice to become an advocate. I have always drempt of this day. I remember in 2008 when I first found my passion for PKU and organized my first walk a thon for PKU at Jaun De Fuca rec centre with the help of my friends Nick and Tyrai, Laura, Nick and Daphne. This walk a thon drew the attention of CanPKU president John Adams, and its where I first met little baby Roise and her mother Nicole Pallone, whom later became the vice president of CanPKU. When I was organizing that walk a thon, my goal was to raise money for a low protein food bank.

Over the years my involvement in the PKU community has evolved and I have been an active advocate with CanPKU and there fabulous BC Advocate Team. We have all worked tirelessly on making this dream a reality. I could not be more thrilled today.  I feel like everything I have done has lead to this moment and I cannot help but cry happy tears. I feel like this will impact so many lives, and change them for the better. BC PKU residents can finally achieve the quality of life they deserve.

Though the  journey is not over, and there is still much work to do with our government and funding for KUVAN, this is a huge step forward and I am thrilled our voices have been heard, our hard work has paid off!

For myself, this means the worry of complianing with diet is lessened, it will be easier to maintain my blood phe levels in safe range. My abilty to control my levels will not be direclty effected by my finaces. It will be easier to acheieve my health goals and to one day have a baby of my own.

For years I have put off having a baby because of the pre conception diet women with PKU are required to be on for 6 months prior to conception and the entire pregancy. You see, whatever my blood phe levels are , are double in the uterus and are toxic to a fetus. So if my levels are high, If I cannot afford low protein foods and keep them low, my baby could be born mentally retarded or deformed. The risk has always been to high for Cole and I to justify. I never was confident that I could maintain my levels low enough to be safe , because I could  not afford the low protein foods I required to stay on diet.

This announcement today, changes that and gives me the confidence and makes my dream of mother hood a real possibilty.

I am truly blessed and grateful to Minister Terry Lake, Bill Bennett and CanPKU for their support and hard work, to the BC Advocacy team and all my friends and family who took time to learn about PKU , to support my efforts and campaigning. To all of those who took the time to read my blogs, to share my story, like my page, Sign the petitions, read and share my radio interviews, news interviews and the CanPKU media Campaign. All of you helped make this possible. You all helped change my life , and all 177 PKU patients in BC and the 32 other BC Patients who require low protein foods such as those who suffer from Maple Syrup Urine Disease.

 

Now I know that everyone has alot of questions so I will do my best to answer what I can with the information that I have.

This is a monthly subsidy that is applied to the BC residents account at BC Children’s Hospital Special Products Distribution Centre. It is open to all 177 PKU patients and the 32 others who require them. There is no enrolment application, it is not a pay now be reimburse later.

This program will be through the same program that already funds our low protein formula. All orders HAVE to go through Special Products distribution centre. You call in and order up to 250 dollars and it is covered! Anything after that is our responsibility. You have 250 a month. So my plan is to one month order all my baking mixes, and the next my pasta and other foods.

I know everyone is worried about the limited stock at SPCD and that they don’t have the cambrooke pre made foods, or country sunrise products. We are already talking to the staff at SPDC on how to remedy this and possibly get them more space or a new location as well as freezers and more stock!

The program and its leaders will be taking a lot of feedback from the PKU community , no doubt there will be some kinks to work out along the way and the first little while will be trial and error as the program grows.

This is all the information I currently have , Please feel free to contact me with any questions or comments and I will try my best to get them answered.

So for today, Celebrate! and spread the news!

The new program starts January 1st 2014!

 

Thank you again to everyone who made this possible. My heart is so full of joy, I am so happy! We all should be proud today!

 

 

 

 

 

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BC Residents and news, CanPKU, News

CANPKU News Release!

Posted on by Amanda Horner

Patients celebrate B.C.’s decision to fund medical foods to protect against brain-threatening disease

– Access to Kuvan still needed as third component of PKU Comprehensive Brain Protection Strategy –
Vancouver, B.C. – November 28, 2013– Canadian PKU and Allied Disorders (CanPKU) is delighted with the B.C. government’s decision to provide funding for special low-protein medical foods for patients affected by Phenylketonuria (PKU) and similar inborn errors of metabolism (IEMs). Medical foods play a critical role in preventing devastating neurocognitive, psychiatric and physical symptoms, and in some cases even death, caused by PKU and 24 other rare, inborn metabolic disorders. The foods are one of three medical components of CanPKU’s Comprehensive Brain Protection Strategy for People with PKU submitted to the B.C. government in February 2013.
In PKU, the body is unable to process an essential amino acid found in dietary protein, called phenylalanine (or “Phe”), and the resulting accumulation of Phe in the blood is toxic to the brain. If left untreated, symptoms can range from mild cognitive impairment to severe mental retardation. As such, patients must adhere every day of their lives to a complicated and highly-restrictive low-protein diet to stave off Phe’s harmful impacts. Medical foods help PKU patients maintain their blood Phe levels at acceptable levels, protecting their brains.
Patients with other IEMs are affected by different amino acids in protein, and low-protein medical foods help them to maintain proper growth while on the severely restricted diet. One such IEM called Maple Syrup Urine Disorder (MSUD) can cause death if a similar diet is not adhered to. Medical foods, which cost on average 10 times more than regular foods, are not regulated by the Canadian Food Inspection Agency, but instead by Health Canada as medical treatment.

“We commend B.C., and particularly the leadership of Health Minister Terry Lake, for recognizing low-protein medical foods as a key treatment to improve IEM health outcomes. This is a decision that will go far to help the children and adults in this province who work hard each day to protect their brains and lives from these diseases,” says Nicole Pallone, from Sparwood, B.C., who is vice president of CanPKU and mother to a five-year-old daughter with PKU. “This announcement marks B.C.’s most significant improvement in funding for PKU treatments in 50 years – and our community is so thankful to all who contributed to this decision.”
According to the Ministry of Health, each patient in B.C. who requires this treatment will be entitled to a subsidy of $250 per month to spend on special medical foods, starting January 1, 2014. Prior to this decision, B.C. only provided a nominal $40 monthly stipend for foods, which was only available to patients on social assistance. Aside from medical foods, B.C. IEM patients receive synthetic amino acid formulas which continue to form the basis of the complicated medical diet. These formulas are high in protein, vitamins and minerals, but are specifically manufactured to exclude the amino acids that cause harm to patients with each condition.
Access to third treatment in PKU Comprehensive Brain Protection Strategy still needed
While this funding decision marks a monumental step forward for PKU treatment in the province, one critical treatment remains inaccessible to patients who depend on B.C. Pharmacare. Kuvan (sapropterin dihydrochloride), the first and only Health Canada approved drug therapy for PKUi, is now funded in Ontario and Saskatchewan, as well as Quebec where the government funds Kuvan on a case-by-case basis for women with PKU who are pregnant or plan to become pregnant.ii Kuvan is covered for some patients with private health insurance.
Following the approval of Kuvan in 2009, negotiations with the drug’s manufacturer began with the drug programs in Ontario, Saskatchewan and B.C. Much to the disappointment of PKU patients in B.C., it was the only province to walk away from these negotiations. It remains unclear to the patient community why B.C. funds Kuvan to treat BH4 deficiency (originally called malignant PKU) and as a diagnostic tool to determine whether newborns with high Phe levels have PKU or BH4 deficiency, but not for its Health Canada approved indication to treat PKU.
“It is our sincere hope that B.C. continues to improve PKU patients’ health outcomes by providing access to the remaining essential treatment tool – Kuvan – to ensure that the brains of adults, adolescents and children are protected,” says John Adams, President and CEO of CanPKU, whose adult son has PKU and has been successfully treated with Kuvan for more than six years. “We are grateful to the Ministry of Health for its renewed support for patients and families in B.C., but our mutual work is not yet done to bring treatment of PKU to national and international standards.”
To date, Kuvan is publicly funded and accessible to patients with PKU in Austria, Belgium, Denmark, France, Germany, Greece, Italy, Japan, Netherlands, Norway, Slovakia, Spain, Switzerland and the United States.iii
About PKU PKU (phenylketonuria) is a rare inherited, brain-threatening metabolic disorder, observed when the body is unable to process phenylalanine (“Phe”), an essential amino acid found in dietary protein. The resulting accumulation of Phe in the blood is toxic to the brain, and if left untreated, symptoms can range from mild cognitive impairment to severe mental retardation. Approximately 1 in 12,000 to 15,000 infants in Canada is born with PKU. All provinces and territories, including B.C., offer newborn screening tests to determine if a child is born with PKU. If PKU is detected, the appropriate treatment must be initiated immediately and maintained throughout life to ensure normal brain development.
About Canadian PKU and Allied Disorders Inc. Canadian PKU and Allied Disorders Inc. is a non‐profit association of volunteers, dedicated to providing accurate news, information and support to families and professionals dealing with PKU and similar, rare, inherited metabolic disorders. Our mission is to improve the lives of people with PKU and allied disorders and the lives of their families. By allied disorders we mean other rare, inherited metabolic disorders also detected by newborn screening. For more information, visit http://www.canpku.org and download our comprehensive resource for patients and families, PKU and the Brain.

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