Posted by Karen

In this blog, I spend lots of time talking about how our family lives and copes with our daughter Miranda’s FOP.  I try to convey the sense that despite FOP, Miranda has a terrific life and is just like any other little girl.  And that stuff is true…  It really is.  I love my little sweetie and have great optimism about her future, just as I do for my son Owen.  Miranda is a wonderful, happy, energetic and bright little girl, and I think she’ll have a great life.

But…  The fact that we’ve integrated Fibrodysplasia ossificans progressiva  into our lives, and adapted to it, doesn’t mean I don’t wish, with all my heart and soul and every fibre of my being, that it was GONE.  If I could ascribe intent and purpose to a gene, I’d say that the word which best describes FOP’s motivation is cruelty. 

When Miranda was a baby, she was (physically) just a typical infant like any other, except for her oddly bent large toes.  She stayed that way for a mere 2 years, and then FOP’s evil intent kicked in and started modifying her body.  Bit by bit, FOP has been robbing Miranda of her ability to move normally.  It started with her neck and shoulders - the first flare-ups blocked her ability to turn her head to the left and to raise her arms beyond shoulder level.  It wasn’t long before flare-ups raced up and down her spine, locking her back rigidly straight, and then moved up into her left shoulder, stealing almost all movement in that joint.  Swiftly thereafter, FOP moved on to Miranda’s left elbow, leaving her unable to fully straighten her arm.  Less than a year later, it locked her right shoulder down forever and fused her right arm in an L-shape at the elbow.

Miranda has adapted tremendously well to all of this - she’s had no choice.  However, anyone spending any time with Miranda can’t overlook the destruction FOP has wrought on her body.  She can walk all over a shopping mall, but at the food fair she has to kneel awkwardly on a four-legged chair to eat her food because she can’t stretch her “good” left arm up high enough to reach her food if she sits down properly.  To put on Miranda’s jacket (which has to be 1-2 sizes bigger than the rest of her clothes), you have to slide it up her right arm, pull it as far over the right shoulder as possible, stretch the left armhole forward and down, and then shimmy it down her left arm.  When walking with her, an adult must hold her left hand, because the right hand can’t make it up high enough to reach an adult level hand.  Miranda lays down on a bed on her back, and then can’t get up without rolling over onto her front, pushing her knee as far up on the bed as she can, and then pushing hard with her leg to leverage her rigid torso upward.  Miranda is fully toilet trained, but can’t go to the bathroom by herself because her arms can’t move enough to get her pants on and off by herself.   Miranda has to drink with a straw, because she can’t bend either her back or her arm enough to drink the bottom third of the liquid in the cup.  Miranda has to ride a low-to-the ground, specially configured tricycle, and can forget about ever ice skating, because of the risks she faces with physical activities.  I could go on and  on, but I’m sure you get the picture.

And despite all this, I think of Miranda as not too badly affected by FOP…  Yet.  As an adult, she will likely lose her ability to walk, feed herself and chew when FOP takes away the movement in her hips, knees, “good” left elbow and jaw.  I know lots of adults with FOP who face those very restrictions.  They face them with strength, grace and amazing patience and ingenuity, it’s true, but I have no doubt what the answer would be if you asked any of them if they’d like to have movement restored.

Which brings me to…  My dream.  My dream is for a medication to stop, or even just minimize, the ongoing progression of FOP.  I want it so bad I can taste it.  Which is why I was overjoyed when the Canada Revenue Agency finally approved the application of the Canadian FOP Network to become a registered charity…  Hooray!!!!!  Now that the CFOPN is a registered charity, it should help us tremendously in Canadian fundraising efforts to help researchers  find a cure. 

A few days ago, our little family sent a letter the following out to friends and extended family to tell them how to help accompish the dream…  Thanks for reading, and for remembering Miranda, Erin McCloskey and everyone else with FOP.

Dear Family and Friends,

As you may know, Karen has been busy this year helping establish a brand new, not-for-profit Canadian organization to raise funds for research into Miranda’s genetic disorder, Fibrodysplasia ossificans progressiva (FOP).  Our group is called the Canadian FOP Network, and we are delighted to announce that the CFOPN is now a registered charity!  This means that if you make a donation to the CFOPN for $20 or more, the CFOPN will provide you with a receipt which you can use to obtain a deduction from your Canadian income tax.

We hope you will consider making a charitable donation to the CFOPN in the spirit of the Christmas season, or at any time throughout the coming year.  The CFOPN(www. cfopn.org) donates the majority of the funds we raise to researchers working to find a cure for FOP.  Our most urgent and fervent hope is that one day, before too long, a medication will be available to prevent the progression of the devastating effects of FOP. 

Each and every FOP flare-up makes us sick at heart…  At Miranda’s young age of 4.5 years, she already has a rigid spine, unmoving shoulders and restricted elbows.  In her day to day life, she has to take care not to suffer even mild traumas, because such events put her at risk of further loss of mobility.  We cannot begin to tell you how much it will mean to us when scientists develop an effective treatment for FOP.  It will literally transform Miranda’s life, and the lives of all other FOP sufferers.

You may ask, “What are the chances research will lead to something?”  The answer is that chances are very good!  Scientists at the University of Pennsylvania “Centre for Research into FOP and Related Disorders” discovered the genetic mutation which causes FOP in 2006.  Since then, they have made tremendous progress in understanding the functioning of the mutant gene, and they are confident that they are on the right track to solving the FOP puzzle.  Dr. Kaplan, one of the primary researchers at the UPenn lab, told us personally that the FOP mutation is one which, because of its configuration, is an ideal target for pharmaceutical intervention.

To donate and help find a cure for Miranda, you can go to the CFOPN website at www.cfopn.org, where it’s possible to make a donation on-line.  Another option is to donate via cheque made out to the Canadian FOP Network (send to: CFOPN, 101 Brixham Crescent, London, ON  N6K 1K9).  Finally, you can also make a donation at any branch of the TD Canada Trust (direct payment to Branch 2864, Account no. 5002365).  Please indicate that your donation is “In honour of Miranda Friz”. If you want any further information, please don’t hesitate to contact Karen at karen.munro@shaw.ca or 604-299-8460. 

Thank you so very much for your kind consideration.  Please remember, her future depends on it!

Love Karen, Peter, Owen and Miranda

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