My heart drug is an orphan?

As you know, December and my procedure did not go even remotely like we thought it would, it has been extremely difficult getting over that experience.  I work at it every single day, squashing back the daily fear and anxiety that has become an all to familiar addition to my daily life, some days it works, others not so much.  At the 6 week check-up on my new device, the subject of switching to a new experimental drug was approached, a drug that was used to treat another disease that was recently found to be somewhat successful at treating patients like me with Idiopathic Ventricular Fibrillation, my answer was and emphatic NO.  I was no where near either physically, but most of all mentally ready to venture into uncharted territory once again, just the thought of another shock put me over the edge, the thought of it would almost put me into hysterics,  there was no way I was having any part of the unknown, I took the “wait and see” attitude and decided to take my chances on the bumped up dose of my existing medication, Quinidine.


That was January 23.  On that day, I took my amended prescription to the pharmacy and asked them to fill it right away.  Quinidine is a special access drug, meaning that every time I need to have it filled, there is special paperwork that has to be forwarded to Health Canada in order for the hospital to bring it into the country for me.  I never know how long that process is going to take, so I always leave myself a lot of leeway just in case there is a snag somewhere, it’s happened before, lesson learned.  After waiting a couple of weeks without a call saying “come and get it”, I called to see what was taking so long and to my surprise was told that they were no longer able to get a supply,  it was on back order.  *face palm*  back order?  ffs.  I took quick stock of my supply and figured I would have enough to last me for 2 weeks, plenty of time I told myself.  Every day I did the math, after all, I know all too well from my experience at Christmas that I would be in trouble about 24 hours after stopping the drug, there is no way I wanted that to happen again.  When I got down to 5 days left, I had a discussion with one of my Cardiologists as to what the game plan was and his explanation of the situation did not make me feel any warm and fuzzies.  This first part I already knew, the drug that keeps my heart from going all arrhythmic is on the worldwide shortage list, it is an old school drug that has been replaced by new and supposedly improved drugs which my heart does not like and do not work for me.  In short, my drug does not make enough profit for pharmaceutical companies so they can just arbitrarily decide to stop making  it, never mind that it is saving some lives.   It’s also referred to as a Orphan drug. These drugs help people with rare diseases such as mine.


Here is a little excerpt from Wikipedia “Since the market for any drug with such a limited application scope would, by definition, be small and thus largely unprofitable for pharmaceutical companies, government intervention is often required to motivate a manufacturer to address the need for an orphan drug.”  According to an article in the Vancouver Sun dated January 18, 2013, “Canada is one of the last countries in the industrial world without a policy”.  The government’s Health Canada website says in a press release from October of 2012 that “a modern framework for the designation, authorization and monitoring of orphan drugs that will provide a significant benefit to Canadians with rare diseases and spur research and innovation in Canada.” and “The proposed framework is in the final design stages and will soon be targeted for public consultation. Comments and feedback gathered during the consultation will be incorporated into a final version of the proposal.” but there is no update to this page which leads me to believe that this framework has never gone any further in it’s development.


So, back to my conversation with my Cardiologist.  They recently stopped production of Quinidine in the US  where my supply was coming from, leaving them with two European suppliers to approach.  He worked the phones talking with Health Canada as well as some higher ups in the drug companies still making my drug and the first supplier, in Italy I believe, was unable to supply Canada, they just did not make enough, he then called the 2nd company, in Egypt, the plan was for them to supply the drug and we could expect a supply to arrive “in a perfect world” the day after my personal supply was to run out.  In the meantime, the pharmacy had a number of tablets that had expired and the doctor agreed that we could use that supply to hold me over until fresh supplies arrived.  I picked up a prescription for 28, enough to last me 7 days which based on the information, would be enough.  Today I am down to 8 tablets, I will be out as of 11 pm tomorrow night.  Needless to say, I have been freaking out, my bag is packed and I was ready, willing and able to park myself on the doorstep of the Emergency Room until they admitted me starting tomorrow night.  I was awake at 5 am this morning, very high anxiety, I could not sleep.  I finally got up and I watched the clock tick down until I could call my Cardiologist’s office to let them know that I was almost out again, WHERE IS MY SUPPLY!?!?!  At 1 pm today, I got a call back, they had found another 50 expired tabs in stock, the pharmacy would call me when they were ready to be picked up.  I was also told that they were still working on a regular supply, honestly, I really don’t want to have to get on this emotional anxiety roller coaster again, which is highly possible if they don’t get a reliable source worked out somehow. *fingers crossed*


The past weeks have been tumultuous and stressful, I have not been able to focus on very much, it’s so hard not being able to control what is going on and making any kind of short term plans hinge on will I be able to meet the commitment.  I take one day at a time and take the fun when I can.


When I think about all the stupid shit I used to worry about before all this happened, I give my head a shake, I had no idea that there were far more important things coming, like, “will I be able to get the drugs that keep me alive? It really puts things into perspective, and I want to remind you again to be grateful every day you open your eyes and never take anything for granted.  Oh, and laugh LOTS!  even if it is at yourself!  and LOVE like nobody’s business!!!


I want so bad not to be a hard luck story going forward, I am trying hard to stay positive, and to see the silver lining, but man, some days it is just not possible.  I will continue to fight, I will continue to lend my voice any where it can be heard for the cause of the orphan drug and I will continue to share my story in hopes that it will help someone else.


“get on with your life” they say…well, I’m trying to, I just have to keep dodging these roadblocks.


Thankfully I am resilient.