This weekend I went with my daughter Miranda to a Girl Guides’ event at the Southlands Riding Club/Farm in Vancouver.  My title here has the word ”sleepover” in quotes because there wasn’t a whole lot of sleeping going on, ha ha.

Here's the barn at the farm.

I went with Miranda because she couldn’t go if I didn’t.  Because of her Fibrodysplasia Ossificans Progressiva, my girl has safety risks which the other kids don’t have, and also, she needs help with dressing herself.  You can’t just delegate that kind of stuff to one of the Guides’ leaders, so it’s off to these things I go…

Anyway, the sleepover was at a really nice place which boards horses, gives riding lessons and runs a bit of a farm with goats, pigs, chickens and organic produce.  Miranda’s group, the Sparks, arrived there at 3 PM yesterday – 13 girls, 4 leaders and 2 mom volunteers – to stay overnight and do all kinds of farm/camp-out type stuff.

Miranda with her Spark friends.

When I go to these events, I’m in a bit of a unique position – unlike the other adults there, I’m not jointly responsible for a bunch of girls, but rather I’m there to focus on Miranda and make sure she can participate safely.  For instance, when all the girls were running around in the grassy area burning off energy yesterday afternoon, I kept a close eye on my girl to make sure she didn’t step in any holes, or slip in mud, or lose her balance and fall in the pond.  Another thing I had to do was brace Miranda as she walked up a narrow ramp to approach a goat in a milking pen, and help her position her body so that she could reach the goat’s udders despite her rigid spine, fused shoulders and partially fused elbows.  I also helped her roll out her bread dough this morning to make cinnamon buns (ditto the arm problems for this task).

As the parent of a girl with FOP, I’ve had to learn how to quietly and quickly assess any given situation and figure out how to get Miranda through it with minimum intrusiveness but maximum caution and also maximum participation.  It’s a fine balance, sometimes, but it’s either that or Miranda doesn’t get to, you know, be a kid and have kid experiences – which would be really heartbreaking.

It was all a lot of fun.  After meeting horses, milking goats and running around yesterday afternoon, the girls ate their brown-paper bagged suppers, did some crafts and enjoyed singing and smores at a campfire.  At night, we all slept squished in close together in a loft in the barn, right above the horses.  It was a late bedtime, and then an unfortunately early wake-up caused by a zealous cock-a-doodle doo-ing rooster ($#&^@ bird!!!).

This morning, after getting dressed and organized, the girls got to make and then eat some cinnamon buns which were baked in an outdoor stone oven over an open flame.  Following breakfast, they met the pot-bellied pigs and 2 more goats, and got to brush and comb a very nice, placid pony.  By 11 AM, it was time to go home, and Miranda’s dad Pete and brother Owen came and picked us up.

Right now I’m honestly a mess – I think I had maybe an hour of sleep last night, because I SERIOUSLY underestimated how cold it would be to sleep in a barn in May, and because of all the croaking frogs, peeing/neighing/bickering horses and all those girls tossing and turning (and that one girl who got homesick and started bawling in the middle of the night didn’t help either).  The other adults said they had pretty much the same experience too…  But anyway, I figure them’s just the breaks, and I’ll sleep it off tonight.  Miranda had a lot of fun, and mades some good memories – which is what it’s all about.

Miranda pausing while learning about baking bread outdoors. She and her little pals were mighty giggly (ie, overtired) after sleeping maybe 6 hours last night...

Last Saturday night, my husband Pete and I went to the Rare Disease Foundation “Rare Finds” gala event.  Holy smokes, it was amazing…

Before I tell you all about it, I’ll begin by explaining that the Rare Disease Foundation is a Vancouver-based charity focused on, you guessed it, rare diseases.  The organization has two purposes – to provide a local community and support group for families dealing with rare disorders, and to provide “micro-grants” to health care providers and researchers needing just a bit of money to get a rare disease-related care and/or research initiative off the ground.  I’ve been involved with the parent group side of RDF for a few years now, and I must say that it really is terrific.  OK, so my RDF peeps don’t experience the specifics of Fibrodysplasia Ossificans Progressiva like we do at our house, but they’ve got their own rare disease concerns, and we can all relate well to each others’ experiences.  I’ve made some valued friends through that group.

Anyway, back to the gala…  This was RDF’s major annual fundraising event, and this year it was held at, wait for it, the Vancouver FERRARI AND LAMBORGHINI (did I spell that right?) DEALERSHIP!  What a totally amazing venue…  They had moved most of the cars off the showroom floor, but they left two of them behind for us to drool over admire.

It wasn’t all standing around and chatting and looking at cool cars, though.  There was some incredible food…  Chefs from a bunch of premier Vancouver restaurants volunteered their services to prepare appetizers and/or desserts at a bunch of booths which were set up in a circle, and gala-goers got to sample the wares at each booth and then vote for favourites by depositing a little marble into a glass jar located at each table.  Interestingly, I found out later that the winners were a chef who prepared a sort of high-end version of a smore, and a chef who prepared, if you can imagine, mini yak tacos (yes, that was yak).  I didn’t vote for either of these, but they were good. 

There was also, of course, the obligatory silent auction, with lots of very cool things for sale.  I was sorry to advise my kids later on that the billing got too high for us to get the hockey stick signed by all the Vancouver Canucks - too bad.  However, Pete and I did successfully bid on a gourmet dinner for 4 to be delivered to our home.  Looking forward to that, yum yum.

Pete and Karen, swanky gala-attending types and big spenders on silent auctions...

There were lots and lots of glittering, wealthy-looking people at this gala, which is just what RDF needed…  Celebrity watchers noted the presence of 2 or 3 of the “Real Vancouver Housewives”.  This is all good, because I understand that the RDF raised about $115,000.00 from this event!  Wow and more wow, such a fantastic result.

Apart from being a great cause, it was lots of fun.  Pete and I got to hang out there with some of my RDF friends, all dressed up and sparkling.  These are parents of kids with disorders such as Prader-Willii Syndrome, PVNH, Maple Syrup Urine Disease, Dup 15q, undiagnosed disorders and more.  These are all fantastic and cool people, and I’m glad I know them.  Also glad we all got a fab night out!

My girl Miranda, the reason why I'm involved with the Rare Disease Foundation.

PS – Update on last week’s blog – luckily, Miranda didn’t suffer any FOP-related effects from the heel injury which I talked about.  She made it through the 7-10 days post-injury with no FOP flare-up developing.  The pain and swelling from the injury completely went away within a couple of days, and didn’t recur.  PHEW…

We had a nasty little FOP scare this week.

On Tuesday, my daughter Miranda went to school as usual, and that day I went to my employer’s office (mostly I work from home).  Later in the day, I was walking to the parkade to leave and pick my kids up, and my cell phone rang, but I lost the call as I got in the elevator.   I figured the person would call back, but he or she did not.  I headed on my way.

I got to the school and walked over to Miranda’s classroom just as the bell rang.  Miranda’s first grade classroom door opened, and out popped Miss M saying, all excited, “Mom, guess what!  Today in gym class I sprained my ankle!”

I had a moment of pure terror…  For a kid with Fibrodysplasia Ossificans Progressiva, even a minor ankle strain can cause a swelling in which some of the soft tissue metamorphosizes  into bone and locks the joint.  This is totally scary crap…  When I collected my wits, I asked the teacher and Miranda’s educational assistant (EA) what had happened.  It turned out M had been in gym class (last period of the day) and standing on a gymnastics mat when another child jumped onto the mat and it slid a little bit, causing Miranda to lose her footing and step down hard on her right foot.  She didn’t fall, but the the teachers could see right away that her foot was starting to swell and that she had to limp.  At that point, they got some cold packs on it quickly and iced it for a while (exactly the right thing to do).  The principal tried to call me – which was the call when I entered the elevator – but didn’t make a huge effort because she knew I’d be on my way at that point anyhow.

Yikes.  Yikes.  Yikes.  Miranda has had no FOP bone formation beneath her waist, and we’d very much like to keep it that way.  I got her home and got her the first dose of a 4 day regime of Prednisone, which is the only thing known to sometimes prevent an FOP flare-up (no guarantees).  I also e-mailed our FOP expert, Dr. Fred Kaplan, to see if he had any recommendations for a sprained ankle.  He suggested getting it checked out by a doctor, because there was a distinct chance Miss M could have suffered an undisplaced fracture (!) of the lower end of the fibula.  This REALLY freaked us out, and so we made a doctor’s appointment for the next day.

The rest of that day, Miranda said her foot didn’t really hurt all that badly – though I could see some swelling.  She wanted to do all her normal things, and I had a hard time keeping her sedentary.   By the next day, she said it hurt only a little bit, and the Prednisone had taken the swelling almost completely down.

My husband Pete took Miranda to the doctor later that day.  Our doctor examined the foot and ankle and determined that actually this was not an ankle injury, but rather an impact injury to the heelpad of the foot.  Further – good news – she was confident that there was no fracture and didn’t recommend an x-ray.  She said just to take it easy and not put a lot of pressure on the foot.

We were relieved there was no fracture, but of course this means nothing where FOP is concerned.  Even a minor impact can unleash the destructive power of this disorder.  We kept Miranda as inactive for the rest of the school week as possible, and used a rental wheelchair to wheel her to and from school and for the teachers to push her around the school in.  By Friday, Miranda’s foot swelling was gone completely and she said her foot felt fine, so she was chomping at the bit to end her inactivity…

…which included going to her first softball event of the season (gulp).  Though we were concerned, we decided to let her go to softball, but she was under strict orders NOT to do any running that day.  The first day wasn’t a game, but rather a first day registration, softball-type exercises, etc kind of thing, so she was pretty much able to follow my rules.  Still, I was kind of worried about how long the event was – she was on her feet for about 2.5 hours – but by the end she said she felt fine.

Miranda in her softball gear, with the Vancouver Canadians baseball team mascot, who was the special guest for the day.

Here we are on Sunday, and the Prednisone’s done.  So far, no obvious FOP flare-up, but Miranda really won’t be in the clear until about Friday of this coming week.  We’re cautiously optimistic that nothing will happen, but only time will tell.

Wish us luck.

April 19, 2012 marks the 5 year anniversary of my daughter Miranda’s diagnosis of Fibrodysplasia Ossificans Progressiva.  That day in 2007 was an unbelievably horrible day – easily the worst day of my life.

It was also one of those pivotal days where our world changed.  There are a few of those in a person’s life – the days when your children were born, the day you got married, etc.  This particular day was one I never expected to have; there was no familial history of FOP.  It’s not a genetically recessive disorder which can quietly “run in families”, so it came right out of the blue at us (as happens in 99% of cases of FOP).  Nope, it was just chance that Miranda turned out to be that 1 in 2 million person with muscles which turned to bone.

FOP hit Miranda hard and fast – there was no easing into it.  She was diagnosed about a month after her first flare-up on her neck (she’d had a few on her head earlier), and shortly thereafter it spread like wildfire.  Within about 5 months, she went from being a little girl with free and easy movement….

Here was Miranda at age 2, before FOP locked her arms - look how she could dive into all that pasta...

This is one of my favourite early photos of Miranda. In fact, I think this is the last picture we have of her with full arm movement.

…to a child with a fused spine, a left shoulder locked in place, a left arm which wouldn’t straighten at the elbow, and a right arm which couldn’t go higher than chest level.  Less than a year after that, the right shoulder locked in place and her right arm became permanently bent in an “L” shape.

Miranda with left elbow now limited, but right not yet affected.

For the first few months of all this, it was pure hell.  Miranda was in pain, she was losing movement, and we could do nothing but watch it happen.  Additionally, we had to undergo a tremendous mental and emotional adjustment.  FOP was constantly in our thoughts.  We had so many fears about the future.

But - eventually we started to pull ourselves out of the hole.  We reached a point where, though we were still anxious about the future, we knew we could carry on with life.  Guess what?  Our kid was still here,  She was still happy.  Maybe she couldn’t move her torso or arms very well, but she was enthusiastic about all the things 3 year olds do.  Also, we had tremendously helpful support from the international FOP community, and many numerous examples to follow people with FOP who lead happy and interesting lives.  How could we not try to do everything possible for Miranda?

Our happy girl, age 2.5 years.

We’ve had some tough times over the past 5 years, and some stressful events, but we’ve handled those occasions acceptably well (at least I think we’ve done well).  Something which continues to be hard is that we have to keep watching Miranda’s body change.  Even if she’s not having active flare-ups – and she hasn’t had any now for 7 months, knock on wood – the existing FOP bone pulls on parts of her body and changes her posture as she grows.  Also, there are still lots of scary future FOP events which continue to worry us (we try not to think about those things if we can help it, but sometimes you just can’t help it).

Yes, FOP changed our world, and permanently.  But you know what?  Over all, it’s not been as bad so far as we thought it might be.  Here’s a snapshot of Miranda today:  our girl is 7 years old, and in grade 1 at school.  She’s smart – she may have an independant education plan because of her physical concerns, but she has a grade 3 reading level and thrives so well in school that she’s doing some advanced learning activities beyond the usual grade 1 curriculum.  She has friends who she likes to play with, and many of the kids in her school know her and greet her in a friendly way when they see her.  She’s in Girl Guides at the Sparks level, and she’s taking piano and adapted swimming lessons.  She’s about to start T-ball (my heart will be in my throat when she plays, but so be it).  And, she doesn’t like to be reminded about her FOP, though it’s always a concern for us.

Miranda, age 7

We’re so proud of our Miranda that we could burst.  She’s such a great kid.  Her future will be different in some ways than we though it would be when she was born, but she’s still got so much potential.

Today is Easter Monday, which explains why my blog is a day late…  We were busy at our house yesterday with coloured eggs, chocolate bunnies, cooking food and other such Easter-y activities – no time for regular Sunday blogging!

I commented last week that my daughter Miranda is like the Energizer Bunny these days…  And that carried on this week too, which is good, as the past few days have been pretty busy.

On Saturday, Miranda went to her friend Sara’s 7th birthday party at Omega Gymnastics.  My husband Pete went along with her, and he said that Miss M had a TOTAL BLAST.  What’s that, you say gymnastics sounds totally wrong for a kid with Fibrodysplasia Ossificans Progressiva?  I’d agree if you’re thinking parallel bars, swinging from your arms, handsprings and that sort of stuff, but leaving that aside, there’s lots to do at a gymnastics place for Miranda – which is why we even had her own 6th birthday party there.  In fact, a gymnastics party is actually great for Miranda – the floors are all covered with those thick gym mats, which means she can pretty much run around to her heart’s content, and even play TAG with the other kids (normally, tag is just completely out for her – even on grass, too much potential for injury).  Additionally, the gymnastics place has a big pit filled with soft, squishy foam blocks, which is perfect for any kid, including one with FOP, to leap into with wild abandon. 

OK, I didn't get a shot of Miranda and her dad at the gymnastics place, but here they are the next day.

On Saturday night, we painted Easter eggs.  Miranda and her brother Owen (age 10) competed to see who could get the most brightly coloured egg.  Miranda ended up doing 4 purple ones, because she liked the purple colour so much.

On Sunday morning, Miranda and Owen were up by about 7:15 AM (groan from parents), and engaged in a rambunctious and energetic Easter egg hunt.  Luckily, the Easter Bunny hid all the eggs around waist height so that Miranda didn’t have to reach too high – her reach is pretty limited due to her fused shoulders and elbows.  Later, we all went over to the kids’ farmor’s (farmor is Danish for grandma) house, where Miranda showed off her stylish, pretty dress, went for a walk with Owen, Farmor and Auntie Anna, and ate a big pile of Easter ham (which is her ALL-TIME FAVOURITE).  Not to mention that she and Owen also scarfed down way, way too much Easter candy.

Here's Miranda doing a "fashion pose" and showing off her pretty dress (complete with sullen fashion model expression - kind of scary how kids pick that stuff up).

It was a really fun Easter…  The kind of family holiday which will make for great memories in the years to come.  That’s important for anyone, but I think it’s extra valuable for people with FOP.  Of course, no-one knows what the future will bring, but people with FOP have a pretty good idea about that subject (in short – progressive fusion of joints and increasingly limited movement).  What we don’t know, however, is when those life-altering physical changes are going to occur.  And so – we try to live for the moment, and milk every moment for all it’s worth.

Bunny Miranda with her bunny Easter basket.

Miranda’s been totally full of beans lately.  I attribute it to spring!  OK, so I griped a lot about the change to daylight savings time a couple of weeks ago, but since then, Miranda’s been a bit like the proverbial Energizer bunny.  Which is just fine - I’m not complaining.  

It probably helps that Miranda’s Fibrodysplasia Ossificans Progressiva has been quiet for a while.  She hasn’t had any new flare-ups since last September, knock-on-wood-long-may-it-continue-that-way.  In fact, we haven’t even had to give Miss M prednisone for any falls or other traumas in a few months either.  Yay!  This is all good.

Anyway, much of the fun began a few weekends ago when Miranda, her brother Owen, her dad Pete and I went for the first bike ride of the spring.  It was one of those rare but oh so glorious early spring days in Vancouver where there was NO RAIN (just imagine, wow).  Miranda got on her Freedom Concepts Discovery tricycle and Owen on his regular bike, and Pete and I followed along on foot.  We found a wonderful trail, not too far from home, which was perfect for Miranda and her bike.  Miranda had a really great time, as did we all.  I was really impressed with her stamina – she rode her bike for close to an hour, with not much by way of break time (though in fairness, Pete and I did help push her up some hilly bits).

Also recently, Miranda had her best friend from school over to play.  The girls got into the dress-up clothes, with M wearing her blue sequiny dress from last Halloween, and S wearing Miranda’s witch costume.  The girls got dancing around to a recording of Thriller from a Halloween disc we have, and I got such a funny video of it (which I would love to post, but should not as I haven’t asked permission from S’s parents to post images of their daughter on-line).  Anyway, here’s Miranda being fabulous in her dress-up dress…

Yesterday, Miranda and I baked the traditional Easter sugar cookies, complete with blue and pink icing (those being the only colours we had).  She always thinks it’s fun to stir up the ingredients, though is a bit clumsy at it and has a tendency to flip stuff out of the bowl…  Still, the end result was pretty (and yummy).

OK, I confess that these aren't actually the cookies we made this year. This is our batch from 2011. But the ones this year look JUST LIKE THIS, I swear.

Today was another bouncy day for Miranda.  She and Owen, still in their PJs, got wrestling around on the living room floor this morning, giggling up a storm.  *Caveat* – wrestling isn’t a great idea for people with FOP, but our Owen is very careful with his sister…  He’s learned from experience what’s safe for her, and never takes things too far (such a great brother).

Right now, Miranda’s off with her dad to watch Owen’s spring ball hockey assessment, no doubt asking Pete a billion questions about everything she sees at the arena (as is typical for her).  After that, Pete’s taking the kids to Tim Horton’s for donuts, which Miranda is much looking forward to – T Ho’s is a fave of hers (good Canadian girl that she is, ha ha).

Hoping very much for continued spring fun with my girl!

PS – Addendum to last week’s blog – check out the cool mini-tongs we bought for Miranda to use for eating popcorn, goldfish crackers, etc…

She just needs a wee bit of practice with these puppies...

PPS – IFOPA members, there are still a few more days to get your nominations in for the Jeannie Peeper Awards.  The JP Awards recognize extra-special contributions to the FOP community, such as big fundraising efforts, raising awareness of FOP and inspiring others with exemplary efforts.  For more details on how to nominate, e-mail me at kmlegal@shaw.ca or go to the IFOPA website at www.ifopa.org. 

Miranda doesn’t want us to do everything for her.  No way!  She wants to do things herself, just like any other kid.  Totally understandable.  She’s 7 years old, and what child at that age wants to have to ask for everything?

However, Miranda’s Fibrodysplasia Ossificans Progressiva means she isn’t exactly like any other kid…  She has restrictions which make some activities of daily living more challenging.  She has to find different ways of doing some things, and as she grows older and taller, she develops new techniques.

So what’s the source of the problem?  Well, the biggest and most troublesome thing is that due to extra FOP bone, Miranda’s shoulders are locked down pretty tightly, and her elbows are also partially restricted.  In fact, Miranda has virtually no movement in her left shoulder, which is her worst affected major joint, and her right is only a little better – she can move her right arm out from her body by maybe 20 degrees, and she can reach forward just slightly.  As if that wasn’t enough, her right elbow is locked so that her arm is in a permanent “L” shape.  She can stretch it a very small bit, and has some ability to move her forearm toward or away from her body, but that’s it.  Finally, her left arm can’t fully straighten at the elbow.  She can bend her forearm up normally, but not straighten beyond about 120 degrees.  (Aside – when Miranda was first diagnosed almost 5 years ago, I was hopeful that she’d be like a fair number of people with FOP and maintain elbow and some shoulder movement well into her teens.  That wasn’t to be – all of the above restrictions had happened by the time she was 3.5 years old.  Sigh…  But at least nothing has worsened since.)

Despite the shoulder and elbow problems, however, Miranda’s wrists and hands work just fine.  She can hold a pen and write normally, and has typical fine motor control.  Plus, she still has some strength in her arms, so can carry things if they’re not too heavy.  This means her arms continue to be pretty useful.

Take eating…  Miranda has touble with “finger foods”.  Until you watch someone with a locked shoulder try to eat something by hand, you don’t realize how useful that shoulder movement is…  On her own, Miranda can get her left fingertips only up to the bottom of her chin.  I say “on her own”, because she has a way around this problem.  What Miranda does is squeeze her left arm between her body and a table edge, and this leverages her hand closer to her face.  Ingenious, I think!  Harnessing the power of physics, pretty smart…  Still, I think we need to get her a pair of small size tongs of some sort – it would make eating things like popcorn and small crackers or candies a lot easier.  (Mental note, need to buy.)  As for drinking, that’s easy…  She just uses a straw.

What about light switches?  Our Miss M just can’t reach any of those with her hands; not even close.  We have a reacher for her, and she’s working on learning to use it on lights.  She does use it sometimes.  But often, she’s in too much of a hurry to look around for the reacher.  And so, she just stands underneath the switch and pushes it up with her head.  That’s a recent innovation – it was only a little while ago that she got tall enough to do that.  Downside to this though is that she can’t usually also get the switch DOWN with her head…  She so she walks into a room, turns the light on, and then leaves afterward with the light still blazing, LOL (but how’s that different from most 7 year olds – not much, I’d say).

Clothing is really tough, especially from the waist up.  Miranda isn’t independent with her clothes, not even close.  But, as she gets older, I can see her abilities improving in some ways.  Recently, for instance, she was wearing a loose sweatshirt and she wanted to take it off.  She used her right hand to hold the left sleeve, and pulled her left arm out of the sleeve.  Then she used the left hand to pull the right side down as far as possible, and managed to manoeuvre the right arm out of the right sleeve.  She was pretty proud of herself at that point – only her head was still an issue.  I then suggested she use her reacher inside the shirt to push it up over her head.  She did it, and voila, got her sweatshirt off all by herself!  Very cool.  I think that as time goes by, she’ll probably be able to figure out how to do more of her own dressing.

I tell ya, figuring out how to deal with the restrictions of FOP is an interesting process.

Here's our Miranda, doing another modified activity - biking. Her three wheel bike is sturdier and safer, and has a handle system which moves close to the body to accommodate her short reach.

If you have FOP and you’ve found a simple, useful way to modify your environment, leave a comment and tell me about it.

PS – IFOPA members, nominations for the Jeannie Peeper Awards are open until April 12, 2012!  Don’t forget to nominate people you admire for the Community Achievement, Emerging Leader and International Leader awards!  Go to the IFOPA website at www.ifopa.org, or e-mail me at kmlegal@shaw.ca for more information.

When Miranda was first diagnosed with Fibrodysplasia Ossificans Progressiva about 5 years ago, one of the many, many upsetting thoughts I had was that she’d be deprived of the opportunity to have academic or work-related achievements as an adult.  For that reason, among many others, I bawled like a baby when, about a week after the diagnosis, an adult with FOP reached out to me via the e-mail newsgroup we had just signed onto and said, “Hi!  My name is (such and such).  I have a masters degree in (blah blah), I’m employed as a (professional title), and I live by myself in my home which I own.”  I still get a lump in my throat thinking of that moment.

For me, and for my family and my husband’s family, academic and work achievements are a valued thing, so to learn that an FOP diagnosis didn’t necessarily mean forgoing accomplishment was a ray of sunlight into an otherwise very dark time.  To this day, I’m still delighted every time I hear about young adults with FOP doing interesting and admirable things.  I’m going to tell you about 3 of them who’ve been doing or have begun doing cool stuff in the recent past.

First, we have Ms. Loubena Harbaoui of Paris, France.  Loubena is in her twenties, and is studying at the Unversite de Cergy Pontoise for her doctorate (yes, doctorate) in law.  She also has a master of laws degree with a focus in criminology.  While doing this very difficult and challenging academic work, Loubena has also been a driving force behind a group in France which exists to help promote the artistic endeavours of people with disabilities.  They’ve put on concerts and shows, produced CDs, and much more.  Loubena’s FOP has advanced to the stage that she has done all of this as a power wheelchair user.  An article about her formidable achievements was published in a French periodical a few months ago, and in the article she was described as “indefatiguable”.  Sounds about right to me… 

Next, I come to Mr. Vince Whelan, also in his twenties.  Excuse me, that’s DR. Vince Whelan – OK, I’m not sure if he’s actually graduated yet, but in any event, Vince is in the 2012 class at the University of California at Irvine’s School of Medicine.  Vince will be the first known physician with FOP in the WORLD.  How fabulously awesome is that?  It was announced this week that he will do a residency in pediatric medicine at the UCSF Fresno.  He wants to be a pediatrician and work with children diagnosed with serious and rare health conditions.  Here’s a link to an article about Vince and his newly announced residency:  http://www.ocregister.com/news/whelan-345021-years-students.html  (NOTE – in the article there’s a picture of Vince with his mother, Carol Zapata Whelan, who wrote a book called  Finding Magic Mountain about her family’s experience with FOP.)

Finally, there is Ms. Louise Wedderburn of the United Kingdom.  Louise is 18 years old and a recent high school graduate.  She is at the very beginning of her career, which she intends to be in the fashion design.  She will be having the opportunity to attend London Fashion Week with design industry experts, and that experience will be showcased in a British documentary about herself and her life with FOP.  Here’s an article about Louise published this week:  http://www.televisual.com/news-detail/Channel-4-Education-announces-a-raft-of-new-commissions-_nid-1363.html

Go Louise!  I expect to hear great things from her in the future.

All of this is so wonderful.  I wish these 3 young people the very best as they move ahead in their chosen careers.  I’ll be behind them cheering every step of the way, and telling my daughter about them.

PS – Speaking of fantastic people in the FOP community – IFOPA members, don’t forget to nominate people you think are worthy of Jeannnie Peeper Awards!  For more information, e-mail me at kmlegal@shaw.ca or go to the IFOPA website at www.ifopa.org. 

I get a goodly number of ideas for blogs from bits and pieces I pick up through the Fibrodysplasia Ossificans Progressiva and rare disease communities.  Just this week, for instance, a germ of a thought started when an FOP mom I know commented, in response to one of her child’s earliest FOP flare-ups, that she hoped she’d be better able in the future to recognize (and maybe minimize?) some FOP progression.

It occurred to me that I remember thinking that way.  In fact, I used to be pretty determined about the very idea…  This is consistent with my personality, which has always caused me to react to challenging situations by diving into things head first, roping up as many details as possible, and doing as much work as I could to minimize the stressful aspects.  That’s a polite, positive-spin way of saying I was (and still am) a control freak, ha ha.  (people who know me well are probably reading this right now and snickering at the truth of it!  )

So in the early days shortly after my daughter Miranda, then age 2, was diagnosed with FOP, I remembered believing fervently that once her rampantly raging out-of-control first set of FOP flare-ups came to an end (note – she had SEVEN active flare-up sites at one point 6 months after diagnosis – a total nightmare), I’d then watch her like a hawk during the quiet times.  I thought that at the first minute when I’d see a teeny tiny hint of a new flare-up, I’d dose her up quick with steroid medication, which is the only thing known to sometimes stop flare-ups, and in doing so I’d prevent the FOP beast from further damaging her body.  I thought I could do it.  I KNEW I could do it…

And so I took a 2-pronged attack – I both watched over Miranda closely to try and prevent traumas (which can lead to flare-ups), and I examined her little body closely every day to look for new swellings.  Did all this vigilance work?  I’d say only to a limited degree.

As regards the safety issue, yes, I probably made her environment a bit less risky for tripping hazards and the like.  But you know, I can’t be watching Miranda 24-7.  It just can’t be done; I’m not superhuman.  Nor would it be healthy for my girl to have her nutso crazy mother hanging over her like that all the time.  Plus, kids need independence and need to try new and different things, not just sit quietly on a soft (and thus harmless) chair all the time.  As such, there have been plenty of instances in the last 5 years when I just haven’t beeen able to prevent things from happening to Miranda.  In fact, I’ve lost track of the number of times she’s fallen and skinned her knee/bumped her head/bonked her shoulder/smacked her elbow, etc etc etc.  Oy, makes me sick remembering some of those.

Watching my girl closely for flare-ups has also been only partly successful.  I did give her steroids sometimes shortly after physical traumas, and I think maybe thereby prevented some flare-ups (but I’ll never really know because hey, maybe there wouldn’t have been a flare-up anyway in those instances – GAH).  There were also other times when I gave her steroid meds according to the directions, and flare-ups happened anyway.  One time which I clearly remember she bonked an existing, old FOP bony knob, and even though I got her the ‘roids very shortly thereafter, a swelling grew a few days later regardless (sigh).

As for watching for the spontaneous, non-trauma related kind of flare-ups, that really didn’t work well at all.  At least a couple of times I thought Miranda had a new flare, and I gave her a one day dose of prednisone, only to figure out the next day that I had misjudged (and so obviously I gave no more meds).  Other times, I’ve tried to pay close attention, but then by the time I was sure it actually was a new flare-up, and not a false alarm, it was too late for the steroid, as the first dose has to be given within 24 hours of a new lesion.  And you know, you can’t just go about dosing prednisone here there and everywhere, because the stuff is serious, has side effects and shouldn’t be given frivolously.  (NOTE – if you’re reading this right now and thinking, why didn’t she get a doctor to look at Miranda if she wasn’t sure about a flare-up herself?, my answer to you is HA!  The only doctors in the world who could determine a flare-up better than an FOP parent could – and even this is debatable – are in Pennsylvania.  Local MDs don’t have FOP related experience, because it’s just too rare.)

So at the end of the day – has being an anal-retentive, type A personality, hyper vigilant style person had the desired effect?  On balance, I gotta say probably not.  Will it stop me from being that way?  Not likely.  It just means I recognize what’s going on.  D’oh!

Miranda on her recent 7th birthday. I can't control her FOP, but I think she forgives me.

PS – IFOPA members, don’t forget to nominate folks in the FOP community who are worthy of Jeannie Peeper Awards!  The JP Awards recognize personal merit for fundraising, spreading awareness and other admirable efforts regarding FOP.  Check out the IFOPA website for more information!

Two years ago, the Internation Fibrodysplasia Ossificans Progressiva Association began a new annual tradition of recognizing great leaders in the FOP community.  The Jeannie Peeper Awards acknowledge and thank some fabulous people who have done so, so very much to raise awareness of FOP and funds for FOP research.

If you are involved with the IFOPA, you will for sure know who Jeannie Peeper is.    For everyone else – Jeannie Peeper is a woman with FOP who founded the IFOPA.  In the mid-1980s, having recently finished a university degree in social work, Jeannie met a doctor through the National Institutes of Health, Dr. Michael Zasloff.  Jeannie found out that Dr. Zasloff had other patients with FOP, and she was immediately interested to make contact.  Jeannie had never met or communicated with another person who has FOP.  Hey, in the 1980s, there was no widespread internet usage, and no FOP contact group, so I guess this shouldn’t be surprising…  Anyway, with Dr. Zasloff’s help, she met her first other person with FOP when she was 31, and she formed a pen-pal club with 11 founding members.  The group gradually gathered more members, and things began to snowball as more and more people got involved.  Today, the IFOPA is a registered charity with close to 700 members around the world, and which has raised $15 million in funds for the FOP cause, almost all of which has been directed to FOP research.  (The source for this information can be found at the IFOPA website at: http://www.ifopa.org/en/board-of-directors-and-officers.html

Jeannie Peeper, founder and president of the IFOPA.

When our family first got my daughter Miranda’s FOP diagnosis almost 5 years ago (!! 5 years!!), the IFOPA was so very, very important in helping us cope and maintain our sanity.  I just can’t imagine what the world was like for FOP families before the IFOPA.  It must have been so exceptionally hard…

Anyway, the Jeannie Peeper Awards were created in recognition of the fact that over the years, many other people have also contributed a great deal to the FOP community, and those people should be applauded and thanked for their tremendous efforts.  I was on the JP Awards committee last year, and it was a fascinating (and humbling!) experience learning all about the great deeds by people in our community.  This year I’m the chair of the committee, and I expect it to be just as interesting a process this year.

There are 4 Jeannie Peeper Awards, as follows (descriptions of the awards courtesy of the IFOPA):

- President’s Lifetime Leadership Award – Those who receive this special award are honored by the IFOPA President (Jeannie herself) for an enduring history of exceptional leadership in the FOP community.  Recipients of the award, personally selected by the IFOPA President, have been active in promoting philanthropy by example or word in the FOP community thereby encouraging others to assume leadership roles and increase FOP community involvement.  This award may also be given for a record of meritorious service to the FOP cause in political, entertainment, sports, medical or other similar arenas.

- Outstanding Community Involvement Award – This honor is presented to an organization, family or individual for promoting and maintaining a high standard of FOP community involvement that includes fundraising and/or awareness activities.  To be eligible for this award, a major fundraising project must have been completed or exceptional FOP community service performed within the past five years.

- Emerging Leader Award – This honor is presented to an individual age 29 or younger who has demonstrated exceptional commitment to improving the quality of life in the FOP community and/or whose personal achievements in the face of adversity are outstanding.  The recipient is one whose volunteerism or courageous example serves to encourage and motivate others.

- Outstanding International Leadership Award – This honor is presented to an individual, family or group that has shown oustanding leadership by making significant contributions to the FOP cause in awareness activities, fundraising or other meritorious services in the international community (ie, beyond USA). (Americans are however also eligible for this award if their relevant service has occurred in the international community).

As committee chair, I would so love to see lots and lots of nominations!  It really helps the committee to know who the FOP community thinks are deserving of these awards, so please, please nominate anyone you think is worthy.  You have all of March in which to submit a nomination.  To learn how to do it, go to the following:

http://www.ifopa.org/en/news-and-events/jeannie-peeper-ifopa-awards.html

Looking forward to seeing your nominations!