A few days ago, I saw a video clip on Facebook with a title something like, “Sh*t people say to people with disabilities”.  As you can guess, it was inspired by those numerous other clips with similar titles (“sh*t white girls say to black girls, etc).  It was funny, and all too true.

Watching it made me think of the irritating things people have said to me, as the mother of a child with a disability, or to my daughter Miranda.  There are two comments in particular.

The first one is along the lines of “Your daughter is an inspiration”, or, even more extreme, ”Her purpose in life will be to inspire everyone she meets.”  Sigh… You know what?  Miranda doesn’t WANT to be an inspiration.  She certainly didn’t choose to be born with Fibrodysplasia Ossificans Progressiva.  She doesn’t want people to think of her as this “incredible, brave little soul, courageously carrying on despite a tragic condition”.  Given that she was born with FOP, and given that she became symptomatic and diagnosed with her disorder at age 2, Miranda has known no other life but that with FOP.  She doesn’t particularly LIKE discussing her FOP.  Miranda is a little girl who wants to talk about her dolls, her art class, the movies she wants to see, her favourite food and all the other numerous things which interest 7 year olds…  Just like any other kid, that’s what Miranda wants to focus on – not her status as a “disabled person”.  And that’s what I want to focus on too!  Calling her an “inspiration” really puts her in a separate category to everyone else, and zeroes in on difference.  So, what’s a better alternative to saying that she’s “an inspiration”?  How about saying that she’s a great kid, with a terrific life ahead of her?  (actual comment from someone in the early days post-diagnosis – I could have kissed the friend who said that!).

The second one that bugs me is, “You’re so strong”, or “I could never do what you do.” (ie, this is a comment directed at me personally as the parent)  There are so many ways in which this type of comment is bothersome.  When people say that to me, what I want do is snap back with, “Well what the $#%^& was I supposed to do with this diagnosis?  Run away?  Lose my head?”  I just did what I had to.  There’s a great expression I really like, which is “You don’t know how strong you can be until being strong is the only option.”  As far as I’m concerned, there really was no choice except to carry on as a parent and do the best I could.  This type of comment carries the suggestion that maybe most people would have just lost it and permanently fallen apart when faced with the outright “horror” of parenting a child with FOP!  For me, this one is almost bordering on rude.  I know it’s not meant that way at all, but, well, nevertheless that’s my reaction…  Now, in fairness, I know that there are some parents who actually aren’t equipped to deal with a child who has disabilities.  I saw examples of that when I worked in child protection law – I remember one particularly hard case of a single mom who voluntarily relinquished her parental rights to her severely disabled child when she just couldn’t cope (and good for that mom for recognizing her limitations).  There are those cases, and they’re sad.  But, while I have no statistics to cite, I would venture to guess that most parents who have kids with disabilities simply carry on – just the way all parents do.  You do what you gotta do, whatever hand you’re dealt, and I’m not sure “strength” has much to do with it.  So again, what’s a better comment?  How about, “You’re a doing a great job as a parent.”  Simple, and complimentary!

Don’t get me wrong, I understand that people mean well when they say these kinds of comments.  When I hear them, I smile and thank them for their well wishes.  I’m not going to be rude to someone who just doesn’t know what to say, and comes out with one of these little gems.  I understand that these comments are meant to be positive.  But that doesn’t mean I don’t silently curse inside my own head at the time I hear ‘em…

Miranda, who'd rather you compliment her pretty dress or new hair cut than tell her she's an inspiration.

So tell me – if you have FOP, or a child with FOP or other special need, which remarks bug you?

Something I have to constantly adjust is the way I talk to Miranda about her Fibrodysplasia Ossificans Progressiva.

When Miranda was first diagnosed at age 2, my husband and I agreed that we’d be honest with her, but at an age appropriate level.  Of course, “age appropriate” is a shifting guidepost…

I think it’s probably fortunate that my girl can ease into her understanding of FOP.  Given Miranda’s young age at diagnosis, this is one, uh, “luxury” that we have.  I can’t begin to imagine how hard it must be for those people who have normal childhoods, and then at 16 or 17, become symptomatic and find out they have FOP.  At that age, it’s going to hit you like a ton of bricks.  There aren’t a lot of people in that category – most kids with FOP are diagnosed before age 5 – but I know of a few who had that experience, and my heart goes out to those folks.

When Miranda was age 2 to 4, we mostly just told her that she had to be careful, because if she fell, she could get an “ouchy FOP bumpy”.  If we gave her medicine for a flare-up, it was to “make her bumpy feel better”.  Her headband was to protect her head from “bumpies”.  That was all she needed to know.  Also, we didn’t worry about her meeting older people with more advanced FOP, because we figured that at that age, she wouldn’t grasp the significance of it – and we were right, she didn’t.

From age 5 to 6, we told her frankly when she got FOP flare-ups that her body was making a new bone.  We explained that this is because of her genes, and we told her what genes are all about.  We wanted her to understand that everybody has genes, and lots of people have “things” caused by genes.  And so, for instance, we told her that her brother and dad have strabismus (“lazy eye”) for which they need glasses, and that’s also from genes.  This was, and is, in an effort to make our Miss M not feel that she’s so different (hey, at this age, she doesn’t have to know that FOP hits only 1 in 2 million people).

As Miranda’s been shifting from age 6 to 7, she’s been wanting to do more things that her friends do.  In every such instance, we have to think carefully about the safety of the activity in question, and figure out if she can do it.  If not, then we have to clearly explain to her what risks are involved, and why it’s not safe for her.  Luckily for us, she’s a smart child and seems to grasp things correctly.  More pleasantly, we can also tell her that our scientists are working on a medicine to “stop” FOP, and that they think it might be ready in a few years, and we can explain why it’s so important for us to do fundraising for FOP research (NOTE – not that she’s always happy to be the focus of FOP attention – lots of times she’s not).

At age 7, what doesn’t Miranda know about FOP?  There’s one real big thing, the fact that absent any new treatment, her FOP will gradually affect more and more parts of her body, further limiting her movement (all her current limitations developed at age 2 and 3, before she could remember life without FOP – ie, this is her “normal”).  She’ll eventually learn about that one, but now’s not the time for it.

Generally, it seems so unfair to me that a child should have to know about any of this.  Miranda is a little girl who plays with dolls, draws pictures of mermaids and discusses Pokemon cards with her brother, and yet she has to know about this distressing genetic disorder.  I SO wish my sweetie didn’t need this knowledge.

Miranda, a fun-loving little girl, hanging out with the mascot at the Cypress Ski Hill. Wouldn't it be nice if she could be as carefree all the time as she looks here?

A few days ago our National Geographic magazine arrived in the mail.  This month, February 2012, the cover story was about dogs, and how the incredible variety of breeds of dogs has been found to be attributable to just a few genes.  If I may summarize the article, it seems that despite the fact that dogs come in so many different shapes, sizes and colours and with such varying hair lengths, hair textures, temperaments, skills, etc, there’s not actually much genetic variability among dogs.  Their DNA is mostly the same from dog to dog.

So what does that have to do with Fibrodysplasia Ossificans Progressiva, which is the subject of this blog?  I’m getting to that, so don’t “hound” me (ha ha, sorry, I couldn’t resist).  Thinking again about dogs…  Say you focus on the dog’s coat.  The author of the article, Evan Ratliff, points out that among dogs, “three or fewer” genes determine whether a dog has “a coat that’s long, curly, wiry, or a combination.  If none of the three genes are mutated, the dog will have the short, smooth coat of breeds like beagles and basset hounds…” (p. 43)  Wow, and more wow.

So, generally speaking, the specific example of dogs shows that a change in a single gene can have a big whopping effect.  Which now brings us to human variability, particularly in the realm of genetic and chromosomal disorders.  Unlike the canine example described above, many human disorders have been found to be caused by the interaction of a bunch of genetic mutations, or by deletions or substituations of entire genes, and even by abnormalities of entire chromosomes.  I understand, for instance, that multiple sclerosis is thought to be due to the interaction of a number of genes, and probably with environmental contribution as well.  Another example is that of Down’s Syndrome, which has been known for some time to be caused by an extra chromosome (each chromosome containing many, many genes).

So let’s turn now to FOP.  If you have it, as my 7 year old daughter Miranda does, you know well what this genetic disorder is about.  But not everybody knows everything that FOP does, so I’m going to run through a checklist.  In no particular order, here’s what you get from FOP:

1. Differences in the big (or “great”) toe.  Just about everyone with classical FOP has big toes which are missing the first joint down from the tip of the toe.  Or, maybe you could say that the joint is there, but it’s abnormal and non-functioning, so people with FOP can’t bend their toes at that joint.  Additionally, the big toes are slightly (and sometimes not so slightly) smaller than would be expected, and angled inward toward the other toes (sometimes just a little, sometimes a lot), causing a bunion-like protrusion on the ball joint of the foot.  This big toe business is an absolutely characteristic feature of FOP, and often leads to diagnosis before anything else is observed.  (NOTE – there are a small number of people with “FOP genetic variants” which cause everything from very little toe difference at all to big toes which are completely missing).  To have a look at some FOP toes, click on this link to the International FOP Association’s website:  http://www.ifopa.org/en/symptoms.html

2. The thumbs of people with FOP are often a bit different as well.  When Miranda was born, her thumbs were clasped into her palms – sort of in imitation of her big toes.  Some people with FOP have thumbs affected this way, and some seem not to.

3. Subtle differences of all the fingers and the wrists.  I can’t exactly pinpoint what’s going on here, but when I look at the hands of people with FOP, things seem a little unusual.  The thumbs seem to be rotated a bit, and the finger joints seem to be a tiny bit larger than you would expect.  The heels of the hands, right at the wrists, seem to protrude a bit as well.  This stuff is really not very noticable, but if you know what you’re looking for, you see it.

4. FOP causes “exostoses” (small bony bumps) on the shin bones just below the knee.  They’re asymptomatic, just…  Present (in many, but not all people with FOP).  I remember noticing these on Miranda before we got her diagnosis, and I pondered what they meant. 

5. Hips.  Apparently people with FOP have abnormalities in the hip sockets which you can see on x-ray if you know what you’re looking for.  I also know one woman who thought her 10 year old daughter’s gait looked unusual from behind, and she pushed her doctor to look into it – this lead to a train of inquiry which resulted in an FOP diagnosis 2 years before the condition became symptomatic (call me impressed by THAT very observant mom).

6. Ribs and vertebrae.  The rib joints of people with FOP are slightly different to the norm, and neck vertebrae in people with FOP are often fused or partly fused right from birth.  In Miranda’s case, she could move her neck well, except for bending her neck backwards.  She HATED “tummy time” as a baby because she couldn’t get her head up off the floor, leading a physiotherapist at the time to think she had weak neck muscles.  Said physiotherapist was astonished when, a few months later, Miranda was able to sit up completely normally and on schedule.

7. Hearing problems.  About 50% of people with FOP have a degree of hearing loss, some to the point they require hearing aids.  It was originally believed that the hearing loss was caused by abnormalities in the ear bones, but more recently there’s been suspicion of a neurological contribution.  Miranda has one normal ear, and one ear with mild hearing loss at some tones.

8. Gastrointestinal problems.  A percentage of people with FOP seem to have issues with vomiting.  Miranda hasn’t had this problem, but Suzanne McCloskey, who shares this blog with me, has blogged in the past that her daughter Erin had to be investigated for this concern.

9. Seizures.  A minority of people with FOP have been documented to have a form of seizure problem.  This isn’t well understood, and is being researched.  We haven’t had this problem with Miranda.

10. Body hair.  Specifically, less of it.  You can see this particularly in the eyebrows.  Most people with FOP seem to have eyebrows which are kind of sparse, and sometimes even non-existent.  I don’t notice this very well in Miranda, but I’ve seen it in many others who have FOP.  I remember one woman with FOP commenting that she didn’t have to shave her legs often, because her leg hair just didn’t grow much.  (OK, so this disorder is good for at least SOMETHING.) 

11. Skull and facial shape.  There seems to be a characteristic FOP facial shape, which I would describe as long-ish and kind of narrow.  When people with FOP get together, many of them look related.

12. Finally, the kind-daddy issue of them all, the one that dwarfs all the rest of the stuff above into minor insignificance – FOP causes progressive swellings in the muscles, ligaments and tendons which become very warm, sometimes reddened and usually painful, and within those swellings, pieces of the tissue are destroyed and replaced by bone.  This eventually fuses the joints and prevents movement.  People with FOP are described as developing a “second skeleton”.  Bone imbalances then also lead to scoliosis, which is present in almost everybody who has FOP to one degree or another.  To make matters worse, flare-ups of FOP can be caused by even minor traumas, meaning people with FOP have to live carefully.  I’ve blogged often about Miranda’s FOP movement restrictions, which include a rigid spine, largely unmoving shoulders, a right elbow fused in an “L” shape and a partially fused left elbow.

Here we have Miranda and her friend Erin McCloskey, circa November of 2010. In both girls you can see some of the effects of FOP.

That’s really rather a lot of stuff.  I’ve got a list of 12 things above, and I know there are even a few more things (I didn’t even mention that FOP is known to have an immune system connection, for example).  But guess what – all of this crap is caused by a SINGLE genetic mutation.  One gene.  ONE.  And not only that, it’s caused by just a single pinpoint difference in one of the amino acids which make up the gene.  That teeny, tiny, miniscule change causes all the effects noted in FOP.  It just boggles my brain.  It’s almost unbelievable to me, except that I have absolute and complete confidence in the UPenn scientists who discovered the mutation and announced it in 2006.

A poster of the human genome at the FOP research lab at UPenn. The poster has the FOP mutation identified, but of course it's far too small to see in this photograph.

It also makes me think of the basic explanation of chaos theory – “if a butterfly flaps its wings in Japan, it causes a hurricane in California” (or something to that effect).  One little amino acid gets substituted for another at a baby’s conception, and the result is differences in toes, joints, body hair, hearing, movement and the body’s injury repair mechanism.  The genetic basis of FOP really is… well… amazing.

Sometimes I blog about challenges we have with finding activities, especially sports, which my daughter Miranda can do.  Yes, Fibrodysplasia Ossificans Progressiva has its difficulties, but for lots of things there’s little or no FOP complication.

This past week, for instance, Miranda and her class participated in a Chinese New Year celebration at her school.  The kindergarten and grade 1 kids had practiced various songs and presentations for some time, and they got to show of their hard work at an assembly.  Miranda was clearly excited about it – she must have asked me about 20 times if I was going to come and watch! 

It was really cute.  First, some of the kids dressed up like a Chinese dragon and did a parade through the gym.  When that was done, some of the other kids (including Miranda) sang a song in Mandarin.  I was impressed how well they did, singing in another language…  After that, various smaller groups split off and presented their own things, one by one.  Miranda’s group did a “Chinese fashion show”, where the girls walked out in pairs to the front of the gym and modeled beautiful Chinese dresses, smocks and parasols – apparently one of the teachers had taken a trip to China earlier that year and had brought back all of these items to donate to the school (wow).  When it was Miranda’s turn, she proudly walked out with her little friend, the both of them wearing lovely outfits.  Miranda was grinning very hard; she was so delighted.  After all, what 7 year old girl can resist a chance to play dress up?

After a few more small group presentations, they all gathered again to sing more songs, and again, Miranda participated with gusto.

Back in 2007 when Miranda was first diagnosed with FOP at age 2, I was so scared of so many things, and doubted that Miranda would be able to be a fully active member of her community.  The pain and devastation which I felt (understandably) clouded my ability to see things in perspective…  I’m so glad my fears were not realized.  Miranda has proven herself to be, first and foremost, a kid like any other, and a happy participant in many school and other activities.

Here's a picture of Miranda and her friends which appeared this week in the Burnaby Now newspaper.

It seems we spent a lot of time this week talking and thinking about sports.  More specifically, my daughter Miranda’s participation in same.

In Canada, sports are encouraged for everybody, able-bodied and those with disabilities alike.  Miranda, at age 7, can’t help but absorb that.  But…  Miranda’s  Fibrodysplasia Ossificans Progressiva is a disorder which makes many sports challenging or impossible.

The discussion in our house started with one of Miranda’s birthday presents from last weekend.  The gift was a cute figure skating outfit, complete with authentic looking skates, for her doll.  Miranda LOVED this present, and spent much time dressing her doll in it and playing “figure skating competition”.  Obviously this got her mind ticking, because a few days ago, she said casually to me, “Mom, I saw a thing on the internet where you can get pretend skates that you can wear, but they don’t really skate.”

This ripped my heart out.  I know Miranda saw no such thing on the web, but rather was thinking about being unable to go ice skating.  Ice skating is just too risky (IMHO) for people with FOP – much likelihood of falling on hard ice (especially as a beginner skater) and causing an FOP flare-up which produces new bone and more limited movement.  Additionally, existing FOP bone in Miranda’s back severely restricts trunk movement, and you need to be able to twist, turn and bend your back when you skate.  And so, we’ve pretty much written off skating.  All I could do was commiserate with Miranda, and try to distract her with something else.  But geez did I feel bad for her.

In fact, I started pondering whether I COULD find a way to get Miranda skating.  I know that for her, sitting on a sledge and being pulled around the ice just doesn’t cut it.  I wondered…  Does anyone out there help folks with disabilities skate?  I tried an internet search.  The one really relevant thing I found was a registered charity which creates special adapted ”frames” to help children with disabilties be able to skate by holding them firmly upright while on the ice.  Sounds great, but…  Downside – I couldn’t see any evidence that they sell these things, but rather use them in their own skating programs.  In the USA.  Sigh. 

The next thing was also a few days ago.  Miranda said to me, out of the blue.  “Mom, I’m 7 now.  You can play floor hockey after school when you’re 7, like Owen does.  Can I sign up for floor hockey?”  (!)  To the depths of my feminist soul, I hate the response I gave her, but it was the first thing which came out of my mouth in a desperate attempt to not have to tell her YET AGAIN that she can’t do something because of FOP.  I said, “Umm, Miranda, you know that almost no girls play that, right?  It’s almost all boys.”  She then backtracked pretty quickly, and said maybe she didn’t want to play floor hockey after all…  Gah, I hate myself for that one, but I knew that Miranda “I’m-a-girl-and-I-only-want-to-do-things-girls-do” Friz would quickly abandon floor hockey without me having to mention FOP.  (I feel, in a complex way, both guilty and relieved at the same time.)

Finally, maybe because of all this other talk, I started thinking again about T-ball this spring for Miss M.  She told me recently – hmm, maybe it was even this week as well? – that she wants to play.  OK, this is the one team sport she can play in a quasi-safe manner, albeit decked out in Klingon battle gear for safety, ha ha.  She played last year in our local “Learn To Play” league, which for kids that age isn’t even games, but just doing drills to learn the basic skills. 

Still, even with helmet, knee and elbow pads, etc, my heart was always in my throat last year watching her play.  I wondered if maybe a “special needs” league would be a better idea.  I talked to one of their coaches, and he was very encouraging.  However, he said one thing which made me pause.  He said, in a nutshell, that the most important thing about the sport is for the kids to be with their friends.  Paradoxically, that’s what sold me on putting Miranda back in the regular league.  That’s where her classmates play, and she loved last year that she was on the same team as them.  And so, I told my husband Pete that I figure M should do the next step up in “Learn to Play”, which is T-ball.  He shares my concerns, but agreed.  We’ll just have to suck up our fears, grit our teeth and let her play.

Here's Miranda (with purple lips from eating candy, LOL) watching a university hockey game with her brother Owen, dad and me (the photographer) last night at our local arena. OK, so she can't play hockey, but she can have fun watching it.

If you or your child has FOP and you have any stories, thoughts, etc about playing sports (post-diagnosis, of course), I’d love to hear ‘em, so please leave a comment.  Thanks a bunch.

Today Miranda is 7 years old.  7.  SEVEN.  I can’t believe it!  Where did the time go, and how did my girl get so big?

This whole weekend has been, as it should be, all about Miranda.  We kicked off the festivities on Friday evening by letting Miss M pick where we go for supper.  She went back and forth on the decision all day, and in the end, finally picked Boston Pizza.  That turned out to be a pretty good choice, because the waitress did her up a nice little brownie with whipped cream and gummy worms and the words “Happy Birthday Miranda” spelled out in raspberry and orange coulis and chocolate sauce.   That was such a sweet thing for her to do, and Miranda was DELIGHTED!  Neat way to start the weekend.

Next up was her birthday party, on Saturday afternoon.  Picking the venue for Miranda’s party always requires a bit of thought…  Certain places are just not good choices because of Miranda’s Fibrodysplasia Ossificans Progressiva.  A jungle gym style party venue, for example, isn’t the best option, because Miranda’s arm FOP arm restrictions limit how much she can climb on things.  Bowling is also a problem – that arm thing again.  Last year we did gymnastics, which sounds on the surface like a wierd choice, but was actually great because of all the soft floor mats (ie, allowed lots of running around, which M doesn’t usually get to do because of FOP).  This year, the choice was a swimming pool party.  Miranda so loves the water…

We had a bit of a glitch at the beginning.  When we arrived, we learned that the birthday party we had arranged and paid for had somehow been booked by the city parks and recreation department at a different pool!  I still don’t know how that happened…  Fortunately, the pool we were at was very accommodating and found space for us to have Miranda’s party at that facility.  Miranda and her 6 pals swam (with lots of parent supervision) for the first hour, then went to the party room for pizza, cupcakes and presents.

OK, a plug now on the cupcakes.  Ours were made by the fabulous Amy Kelly, who does business in the Vancouver area as “Amy’s Southern Cakes”.  These little cakes were so terrific – vanilla with confetti sprinkles in the cake, and piled high with pink buttercream icing, and each cupcake had a letter on it…

In case you can't tell, the cupcakes spell out "Happy Birthday Miranda".

They were THE BEST!  The kids loved them, and a few of the kids asked for seconds (which is a real mark of approval).

Miranda and her pal (* I offered to take off the helmet, but she said she wanted to wear it. Okee dokee.)

Anyway, the girls all had a great time, and then before we knew it, the party was done.

Miranda with her mom and dad and a couple of friends

Finally, today is Miranda’s actual birthday.  She woke up to a pile of birthday presents stacked up on the dining room table, and was really excited!

Just before plowing into the gifties...

Between gifts from her friends and presents from family, I think our girl got pretty much everything she wanted. 

We’re going to round out the day with Miranda’s favourite homemade supper, macaroni and cheese with ham chunks stirred in.

Yay!  I thinks it’s been a very happy birthday.  Here’s to many more.

A week or so ago, a friend of mine posted on Facebook that she was doing that exercise where you think of a single word which best describes what you hope for/want to achieve this year.  I thought about that, and had an immediate response.  The word I thought of?  Confidence.

I pondered this a bit, and realized that I can think of lots of ways in which confidence will assist in my professional and personal life, and it also fits very well in the context of dealing with my daughter Miranda’s Fibrodysplasia Ossificans Progressiva.  In fact, the word “confidence” is another way of stating the second half of my personal philosophy regarding FOP:  “plan for the worst, but believe the best can occur”. 

The “worst” is pretty bad…  If FOP takes its natural course, Miranda’s hips will fuse, such that she is permanently locked in either a sitting or standing position.  Her wrists and the rest of her remaining elbow movement (lots of it is gone already, thanks to FOP) will be lost, making her unable to do much with her arms.  Her jaw will be frozen in place, making eating difficult and possibly requiring the removal of teeth.  Worst of all, FOP bone can so restrict her chest movement that her lungs become compromised and her lifespan is shortened.

As I said, all of that is horrible.  If I walked around thinking about it all the time, I’d lose the ability to function as a rational adult.  Instead, I choose to accept that those possibilities are out there, and accept that we may have to deal with them, but to focus on what’s good about our situation and have CONFIDENCE that things will continue that way.  There are so many reasons for confidence…

First, it looks likely that our FOP experts are going to figure out how to crack the FOP nut.  In fact, they’ve already done it at the laboratory stage, and they’ve even succeeded in doing it in multiple ways.  2010 and 2011 were chock full of new scientific studies having been released in which various scientists announced discoveries of new medicinal compounds which largely or completely block the effects of the FOP gene.  OK, so none of that stuff is at the stage of human testing yet, but much of it is clearly headed in that direction.  It could very well be that within a few years, there will be a drug available which neutralizes FOP, and Miranda will get to avoid all the really bad effects of advanced FOP.  I can have confidence the research will continue, and I need to remember that when times are difficult.

Second, I have a great kid who adapts well to new circumstances.  Can I have confidence that she’s going to continue to thrive in school and socially?  Absolutely!  Miranda’s been such a champ this far; I can’t think why this wouldn’t continue.  Miranda loves school, is engaged in learning, is taking piano and art lessons (the latter to begin next week), has friends she plays with, and has a fun, gregarious personality.  I think this girl will go places.

Third, the world is a changing place.  New technological advances mean that people with disabilities have ever increasing likelihood of being able to work and otherwise engage meaningfully in society.  This isn’t the world of 100 years ago, when it wasn’t uncommon for a person with FOP to either stay permanently at home with no social contact, or to make a living by joining a circus freak show (yes, this happened).  Even if the worst happens, and FOP continues to affect Miranda unchecked through her life, she can still have a great life.

Miranda, age 6 in 2011 - a kid with a great future!

Confidence.  Yep, that’s my word for 2012.  If you’re challenged by FOP or something else, do you have another good word for 2012?  I’m curious, so leave a comment and let me know…

Ah, it’s New Year’s Day…  A day for looking ahead, and hoping for a great year to come.  On the downside, seasonal holidays are only 1 day longer – d’oh!  Our family has had a really fun break, and while we’ll be refreshed and ready to get back to the regular routine, I’ll be sad to see it end.

One good bit, for example, was that from December 26 through 29, we had our son Owen, age 9, signed up for his first ever set of snowboard lessons at Cypress Mountain.  He went 4 days straight, from 8:30 AM to 3:30 PM.  (OK, the part where we had to get up early to drive him each day wasn’t so hot, but we managed.  )  Our routine was for one of us to ferry him to the ski hill, then return and spend the rest of the day with Miranda. 

Unfortunately, snowboarding and skiing are activities that Miranda, with her Fibrodysplasia Ossificans Progressiva, just… can’t… do.  What I mean is that my husband Pete and I have considered the risks, and decided that these snow sp0rts pose too many difficulties and risks for Miranda.  First, there’s the movement part of it – to ski or snowboard, you have to be able to shift your torso and move your body freely in order to keep balanced and going forward.  This is impossible for Miranda due to her rigid spine, unmoving shoulders and (mostly) locked elbows.  She just wouldn’t be physically able to do it.  Second, there are the dangers associated with these sports.  I remember that when I learned to ski, there were multiple occasions when I wiped out spectacularly, with one ski going one way and the other going the opposite direction, causing multiple bruises and muscle strains – all of which would be bad news for a person with FOP, who is likely to suffer an FOP flare-up with more resulting bone formation.  Yikes.

But though Miranda can’t do these sports, we wondered if there was anything else she could maybe try at the ski hill.  Pete looked into it, and he learned that   there’s a snow tubing area at Cypress.  It turns out that if you’re age 6 or older, you can go to the snow tubing place and pay a fee to rent a big inflated inner tube, get towed up a hill and slide down the hill in the tube.  I was kind of leery about this…  Miranda is 6 years old, so is the youngest age at which you are allowed to participate, and besides, I wasn’t sure how she’d manage with the tube.  Still, we decided that the three of us would go up to Cypress one day while Owen had his lesson, and that Pete and I would take turns skiing and checking out the tubing hill with Miranda.  We figured that if the tubing looked OK, she could go tubing, but if not, then surely she could at least play in the snow.

We went up on December 28, and we decided that I would ski first and Pete would spend the morning with Miranda.  It turns out that he took her to the tubing area, and from the get-go, she was VERY keen to give it a try.  Pete scoped it out, talked to the attendants about risks, etc, and decided to make it work.  He got a free ticket for the tubing area as he was helping Miranda (this is Cypress’s policy for attendants with people who have disabilities), and he paid for a standard tubing experience for Miss M. 

It turns out that Miranda loved snow tubing!  Pete says that she made it to the bottom the first time, and then shouted, “That was SO MUCH FUN!  Can we do it again?”  She then went up and down the hill a whole bunch of times, laughing all the way.  Pete sat in his own tube and held on to hers (which, strictly speaking, wasn’t necessary, but it made her feel safer).

Miranda lasted a bit over an hour at this, which sounds like plenty to me.  After that, they went back to the ski lodge and got some lunch, and then Pete and I switched off and he went skiing.

I’m so very glad that Miranda had such a great time doing snow tubing.  She raved about it afterward, and says she wants to go back and do it more.  So why was it so great?  I have no doubt that Miranda had a blast, but I don’t think that’s the whole story…  It was at least as important that she had the chance to do a fun snow sport just like any other kid.  In fact, she even saw one of the other children from her grade 1 class at the tubing area, and though she didn’t specifically say this, I bet she was proud to have been seen by him doing snow tubing.   

Miranda even got to sport one of those nifty little ski tags on her parka.

On a totally separate topic, Happy New Year to you and yours! 

Today is Christmas Day…  “It’s the most – wonderful - ti-i-i-i-me of the year!” 

Miranda, age 6, and her brother Owen, age 9, have ripped into their presents and are now having a great time playing with new treasures.  Later on today, we’ll eat turkey, sweet potatos and all the fixings, and even more importantly, will love and enjoy each others’ company.  Miranda will even play “Jingle Bells” for us on the piano. 

Before I rejoin the festivities, I’m going to take a page today from my friend Yolaine’s book, and say a very special and heartfelt Merry Christmas to all my friends who live with the effects of rare diseases.  This past year, I got to know a wonderful bunch of people in the rare disease community – families newly coping with Miranda’s genetic disease, Fibrodysplasia Ossificans Progressiva, and others with different and varied conditions such as Dup 15, Prader-Willi Syndrome, Maple Syrup Urine Disease, disorders yet to be identified and many more.  Every one of these conditions brings heartbreaks, but they don’t define ourselves or our children.  It’s so very, very important for us all to appreciate that, and what better time to do so than Christmas?  Christmas is a special time, a chance to focus on the things we value and cherish about ourselves and our families, and to set aside (as much as possible) our health worries. 

And so, my Christmas wish for all my rare disease friends is for a wonderful, happy, relaxing and joyous day.

Miranda and Owen on Christmas Eve

Before I go, one more thing – a big and profound THANK YOU to my cousin John and his wife Anne, who made an extremely generous and warm-hearted donation to the International FOP Association in honour of our Miranda!  I am touched beyond words at this sign of their love.

Finally, Merry Christmas all!

I’M BAA-AACK!  And happy to be blogging again, after 3 weeks off to deal with a…  time-consuming project I had to do through work (end point was Friday Dec 16).  The early part of December was a tough slog for me, and now I’m ready to relax!

However, the past month wasn’t a complete washout in terms of enjoying the holiday spirit with my kids and husband – a couple of weeks ago, I took a break and we all went to the “Starlight Stars on Ice” kids’ holiday party.  I’ve written about the Starlight Foundation before, as has my co-blogger Suzanne.  It’s a really great charity which exists to plan and carry out fun events for children with serious illnesses/medical disorders and their families.  My daughter Miranda (age 6) qualifies because of the progressively disabling nature of her Fibrodysplasia Ossificans Progressiva.  Anyway, every year Starlight does a great Christmas bash, complete with lunch, yummy holiday treats, face painting, Santa Claus, presents for all the kids and culminating in ice-skating.

This is the third year we’ve been, and it’s lots and lots of fun.  Miranda particularly liked the very colourful clown who did face painting and balloon animals/objects…

And Owen (age 9) thought it was a hoot that the clown could put temporary Vancouver Canucks green colouring in his hair…

Owen with green stripes in his hair, and Miranda with a candy cane painted on her cheek. M is also wearing balloon fairy wings.

Pete got in on the action too with a green beard.

Owen wasn’t so big on all the arts and crafts stuff, but that was RIGHT up Miranda’s alley.  She decorated two gingerbread cookies, made paper Christmas tree ornaments, coloured a Christmas picture and did two rounds of “spin art” (a spinning machine with paper mounted in the middle; the child squirts paint into it and makes an abstract and colourful painting).

It’s a good thing Miranda enjoyed the crafts so much, because unfortunately, she couldn’t take advantage of the skating.  Skating is just too much of a no-no for Miranda with her FOP – for one thing, a fall on the ice has excellent potential to cause an FOP flare-up (painful swelling leading to movement-limiting bone creation), and for another thing, Miranda’s spine is so rigid due to FOP bone that she wouldn’t be able to bend and twist her torso in the way necessary to skate effectively.  In past years, the Starlight party was at a different place with a rink which had hockey sledges in which a child could sit and be pulled around the ice, which is what we did with M, but go figure, the Vancouver Olympic Speed-Skating Oval had no such sledges…  Since Miranda couldn’t go on the skating rink, the good Starlight folks were happy to let her stay in the arts and crafts room during the skating part, and she was happy to stay there and do more art.  (Aside – in the past, Miranda has been sort of lukewarm about the sledge anyway – even in the context of Starlight, which has kids with all sorts of disabilities, including in wheelchairs, M thought she stood out when she was in the sledge.)  My husband Pete and I took turns skating with Owen and looking after Miranda.

Me and my girl (yes, that's a bit of red in my hair too!).

The Starlight party was a great start to the yuletide season.  Always so much fun.  Here’s hoping the rest of the holidays are just as fun…