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:

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!