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!

 

 

 

 

 

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.