Not Porcelain Dolls

So three weeks have passed since surgery and I'm off running in therapy (ha! if only I were running). While I do have timed goals per physical therapy standards, I am torn between staying on schedule or getting ahead of schedule.  Staying on schedule is safe and slow and improves my odds of total success from the reconstructed ACL surgery.  If I move ahead of schedule, I risk injury of the same ACL but if I do not accelerate my progress, I also run the risk of injuring other extremities.  How do pro-athletes decide to push ahead?  Granted, pros have personal trainers working with them every day and they are not as susceptible to new injuries during recovery as I am. Would I be irresponsible if I forged ahead of schedule?

In my "This Path" blog, I mention that athletes choose their high risk paths and of course, I only refer to regular athletes.  What do I mean by regular athletes?  I label regular by those who are otherwise healthy and have no physical limitation that is not an every day event.  There are other athletes out there who have found a means of surpassing limitations they were told they had.  I do not dare call them challenged for their definition of challenge is on a competitive level.  Porcelain dolls they certainly are not.  For instance, Amy Purdy  having both her legs amputated from the knee down in order to save her life, became a snowboarder after her amputation and also went on to compete on Dancing with the Stars finishing with an impressive second place standing (literally...standing). Doug Henry, a USA multi-AMA champion motocross racer, was paralyzed from the waist down in 2007 and yet still gets on a dirt bike! (Click here for more Doug Henry news).  Don't believe me?  Just check out the pictures that I shot of him in 2011 at Red Bull's Day In The Dirt where he was quite competitive staying ahead of Jim Gibson for several laps.  Needless to say, I was in awe of his competitive drive.

November 2011 Copyright Roni Faxon

Jim Gibson and Doug Henry. November 2011 Copyright Roni Faxon

There are many more permanently injured athletes who do not give in to their injury but there is one whose circumstances hit closer to home and that is, Jeff Black.  Who is Jeff Black?  He is a body builder and not just any bodybuilder. For you see, his bones break frequently due to Osteogenesis Imperfecta, but his unbreakable spirit keeps him going to the gym and to competitions.  

The wonderfully, inspiring athletes mentioned above are not on a shelf like porcelain dolls just sitting pretty and not meant to be played with or taking part in what life has to offer and so, neither will I.  I am unbreakable; I will not be alive to simply watch the world and all its beauty from a shelf but instead, I will live life to its fullest for as long as God grants me a new morning to face.

So why am I so frustrated?  I want to be normal; is that selfish?  Is that vain?  

Having read Jeff's blog today, I couldn't possibly take my recovery as scheduled.  I will not take unnecessary risks but I will not baby myself either. I have to shake this frustration, but only physical activity helps me do that so like Jeff, I will push past the pain to get back to my prior activity level...I am unbreakable.

This Path

In my previous blog, I said that my girls and I are blessed that it isn't a cancer gene we carry. Likewise, we are also blessed that the type of O.I. we have is not the most extreme form.  The Discovery Health Channel did a segment on children with O.I. and follows the jouney of some with with an extreme form of O.I. ( Children of Glass).   For some, the signs and challenges are very pronounced and heartbreaking while for others like me it is not.  I often remind myself of those who have suffered more but the reminder does not take the frustration away completely.  The last three years have been full of injuries sustained from the simplest of things.  Stupid things.

"So what?" you ask, "there are several athletes that experience multiple injuries throughout their career and they're not writing some self pity blog".  Well true that.  Ryan Villopoto (four time Supercross Champion) is in month five from his knee surgery and already back on a bike.   Football players from the time they are in high school and on through the NFL suffer from repeated injuries so why the pity party?  "Suck it up" you say.  Here is the difference; athletes choose their path.  Athletes choose a journey containing high risk of injuries and long periods of recovery.  Their choice makes it highly probable that they will get injured...but it was their choice.  My family did not choose this path; my parents did not choose to have their kids afflicted with O.I and while I chose to have children (not yet knowing that I could pass it down), I also did not choose to have my kids afflicted with it, nor did my kids have a choice in picking their DNA strands.  We inherited this path and there is no walking away from it.

I choose to exercise and keep healthy hoping that my muscles will fill in where my tendons and ligaments fail me.  I choose this because without being strong, I would need to rely on others on a more permanent basis.  I'm blessed with an incredible husband who has been my crutch through all of the injuries but being an independent person, I get tired of asking for things; asking for a glass of water because I can't carry a glass and keep both my crutches in hand, asking for help to get out of bed because I've been weakened by the most current injury, asking friends and family to drive me to work because my driving foot is incapacitated, etc.   If it's tiring for me to ask it certainly must be tiring on the people being asked to help.  I don't want to tire anyone.

Had I not started this blog most of you who know me personally would never had known that I was afflicted with O. I. Perhaps you would have continued to think of me as unlucky or clumsy.  With the exception of my small stature and the blue in the whites of my eyes, there is no exterior sign that would give anyone reason to believe that I am fragile.  Some of my friends kidded around with me and said that exercise was too dangerous and used my injuries as a reason for them not to exercise.   While I did laugh at their reasoning and joked along, inside I was telling myself that perhaps I should reconsider and join them. Little did they know how dangerous the exercise truly is for me but without it, I would be broken. Exercise gives me a sense of accomplishment; that Rocky at the top of the stairs feeling...I am not breakable feeling.

My path now is to heal the reconstructed knee without adding stress to the other extremities.  The healing process becomes a balancing act for me; how do I maintain the upper body strength to remain on crutches for the next six weeks without stressing the ligaments in my wrist?  How do I rely on my weak, left ankle to bear the majority of the weight while the right knee heals?  How do I comprise the weight bearing to the left of my body without aggravating my s-curved back?  Oh yeah, that's another trait...I have scoliosis of the back...a curve that would make most street bike riders happy to see on a mountain side road.  

These last two weeks after surgery have been complication free but then again, the exercises up to now have been just about getting some movement back in the knee.  I start physical therapy next week and with that may come new injuries to the other leg.  The people at physical therapy know me about as well as my friends know me and they probably see me more than my friends see me.  My path is one that runs in circles and the view is getting old but I am not breakable.

I Can Do This

O.I. a rare disorder;  so rare that there are only a handful of doctors on the west coast that have treated patients with O.I yet not rare enough to have affected only one of my parents’ offspring but all three of us.  Rare enough that information on adults with O.I. is not as comprehensive as information on children with O.I. but not rare enough to have missed being passed on to two of my daughters with one more affected than the other.  

When all four of our girls were little, they were into lots of different things so when they reached high school, it wasn’t surprising that they wanted to continue their active lifestyle.  Three of them were in high school together while the baby was farther behind in middle school.  The high school girls were in drill, dance, and cheer.  Knowing that I had passed down the O.I. gene to two of the girls, I guess I was irresponsible for allowing them to participate in physical activity.  But, I wasn’t breakable during high school so why would I hold them back?  The stronger of the two (or at least the one I thought was stronger) broke her hand while at a sleepover during a pillow fight.  A pillow fight!  When my second oldest announced that she was going to be a flier on the cheer team my first thoughts were, “oh no! she’s fragile!”.  But wait!  She’s the one that has almost the exact traits that I had at her age.  I’m not breakable, she had not been breakable so why not let her be a flier?  First time she’s dropped, she breaks her ankle and requires surgery to correct the break.  I find myself explaining to the pediatrician that O.I. runs in our family but he assures me that anyone under the same circumstances could have broken their ankle in a similar way.  Cheerleading, I was told, had a high risk for injuries and was even considered by some, more dangerous than football. So, she went on and completed her high school years with injuries sustained along the way, including a second break to her foot.  Irresponsible on my part?  Perhaps, but she survived and so did her other sister who shared the same broken gene.

Now as adults, they are faced with similar ailments that I had starting right about the same age.  The one daughter that shares the trait closest to mine, has lost hearing in her right ear while my other daughter has suffered injuries to her lower extremities over the simplest of events.  So not fair!  Why couldn’t this rare disorder have remained rare enough to have missed my girls?  While I am grateful that we have been blessed that it is not a cancer gene, there are still times when this affliction becomes so frustrating!  We have healthy lifestyles, we exercise and try our best to eat right but even exercise can hurt us.  But we do it anyway; We are not breakable.

Having been a fan of motocross since the age of sixteen, I was thrilled and hesitant when my husband surprised me with my first dirt bike as an anniversary present.  And of course, just shortly after presenting it to me he acknowledged that my father would be angry with him for doing so and of course, my Dad was upset but I assured him that I would not race.  I would just use it to have some trail fun out at the desert or ride around in the pit area while at the races with my husband.  So on my first time out, I managed to ride around without falling and ended up with a greater respect and love for the sport.  I took a riding lesson (yeah I did that in reverse) and had a blast gaining more confidence even though I did fall over once during training.  But I didn’t break...I survived with not even so much as a bruise from the fall.  “Yay!”, I thought, “maybe I have finally outgrown the O.I. Maybe the supplements and exercise have paid off.  I AM NOT BREAKABLE!”.

My time on the bike was short lived because the next year, I managed to roll over my left thumb during an exercise move known as the burpee and sprained it good.  The bruise sustained from that injury covered the palm of my hand and wrapped around my forearm.  My left hand, used for the clutch on the bike, was braced for several weeks.  But I didn’t break.  I continued attending the races with my husband shooting pictures of the races as I had all along.  When the brace came off, we went out to a practice track that had plenty of open space for me to putt around on my bike and while the thumb was still sore, I managed to complete the riding day with just one fall but no new injury.  “Great!  I can do this!”  Why would I think otherwise?  I had two separate incidents where I fell while on my bike and didn’t sustain an injury.  I wore all the safety gear, and clearly it was doing its job.  I can ride, I am not breakable, I will be fine.

Somewhere along the way, I managed to lose sight of my healthy eating and gained weight.  Weight gain from fat is not good for anyone let alone an O.I. patient with a weak structure so I bumped up the exercise, cut back on certain foods, and focused on muscle gain.  The weight loss was slow, the muscle gain was slow, the pain in the joints and the back was discouraging but the spirit was willing.  I had to lose weight...it was a matter of being breakable or not.  I kept on and thankfully my oldest daughter, and one that was not affected by O.I., was a bodybuilder so I got a lot of advice and encouragement from her.  I got to within eight pounds of my targeted weight goal, ten points of my targeted muscle mass and then disaster strikes.  I fall while walking across a parking lot on my way to a favorite restaurant.  I bang my left knee and right foot throbs.  But I’m not breakable.  My husband helps me up and we continue into the restaurant because it’s my cheat day after all, and I’m hungry and want my cheat day.  We have a few margaritas (I have more than a few to help the pain) and proceed home when done.  Through out the night it becomes evident that my foot will need some real medical attention so in the morning we proceed to the weekend clinic and find out that I broke my foot.  But wait! I didn’t break it because of the O.I., I broke it because it’s a common injury when people fall off a curb or dance and lose their step.  So common is the break that it even has a name...it’s the Jones Fracture.  “Aha!  I’m still not breakable!  IN YOUR DREAMS RONI!  Hello!  You just broke your foot!”, go the stupid voices in my head.

Six weeks go by and I finally get to start putting weight on my foot again.  Another three weeks before I’m able to start walking without a limp.  During this period I gain weight again so faced with a freshly healed foot, I invest in a stationary bike to keep working towards my goal even though I was starting from scratch (gained ten pounds!).  All goes well over the remaining months of the year and I finally get back on my dirt bike but it feels like I’m starting all over again.  I’m uneasy, keep stalling it, and have a real rough time believing that I will ever get to the level that I need to be at to trail ride.  Ugh! stupid injuries!

Exercising continues, the weight loss is experienced, the muscle gain is visible, and I write to my favorite DVD trainer and let him know the results and my newest goal.  I’ve reached the fit level and next aim for the athletic level.  The trainer writes back with a, “good job! keep us posted” and then...while visiting my parents in El Paso, Texas and taking a fast paced stroll around their neighborhood, I feel a little sting in my left ankle. It was nothing big that stopped my stroll, it wasn’t anything that made me think it was more than just a pinch of my hiking boot so I continued on my walk.  As time went on during our stay, it becomes more difficult to walk.  By the time we finally get back to San Diego, I’m bracing it and giving it a rest from physical activity.  I finally get in to see the Doctor months later and find out that yet again, I tore a tendon.  But this one had to be different, this one didn’t tear all the way through...it’s just a partial tear so there will be no surgery.  I am instead, married to an ankle brace that I am to wear forever whenever I am doing something extremely physical.  Fudge!  There goes the side lunges!  There goes the jumping jacks and other plyometric workouts!  Fudge! There goes dancing in heels! DOUBLE FUDGE!   Oh no that will not happen! I AM NOT BREAKABLE!

I maintain my HIIT (high intensity interval training) on my stationary bike but have to filter out the extreme DVD training videos; all of which had power moves that the ankle could absolutely not handle.  So I create my own free weight workout and continue my fitness goal.  I start feeling pretty strong again and wean out of the custom brace into a more flexible brace and find that it works okay.  For race day, I put the custom brace back on, I’m out on the track taking pictures, my husband comes by to pick me up and take me to my next shooting location, the next race gate drops, I swing my camera around, the bike wiggles, and so does my leg from the knee down...POP, POP, POP!  “WTH!”  My husband jumps off the bike and ask what that was he heard.  Through tears I answer, “that was my knee...I think I just tore something”.

Back at the pit area, I ice and wrap the knee and can walk okay as long as I’m careful but notice that stepping in and out of our Ma’rita bus (RV) is making my knee feel very unstable.  I continue taking pictures but from a farther distance and take all the pictures during the awards ceremony while standing. I’m okay; I am not breakable.

Six weeks later, I finally get an MRI and I’m told that not only did I tear the meniscus, I tore the ACL completely. Looking at the MRI, the doctor shows me all the damage sustained including the bruise to the bone but, it didn’t break.  Because my bone density was softer than any patient he had ever operated on, the doctor had to use wider than normal screws to attach the graft that was to be my new ACL.  He didn’t want to drill deeper into my bone with a normal screw because he was afraid of weakening the bone further.

So that brings me to where I am today...after surgery with collapsed muscle that seems to just sludge over the knee cap, knee the size of a cantaloupe, bruises of different colors and new goals to meet over the next six months.  I can do this; I am not breakable.

Introduction

Unbreakable, the movie by M. Night Shyamalan with Bruce Willis and Samuel Jackson pairing as opposite polar points…one breaks easily while the other is unbreakable.  The movie follows the path of both individuals and introduces the audience to a rare condition called, Osteogenesis Imperfecta (O.I.).   Hi, I’m Roni and I am unbreakable and I will be writing about my experience with O.I. as a means of venting and educating people in general.

For years my brothers and I would find ourselves injured. So what? You ask; most active kids do find themselves recovering from cuts, bumps, and bruises but for my family, the bumps, bruises and broken bones didn't take much effort.  My brothers broke their bones often while I just bruised.  For a long time, we never knew why.  My family passed off my brothers' E.R. runs as just part of the course for boys but my baby brother was in his first cast just months after being born when he trapped his leg in the crib.  I vaguely remember going to some massage specialist that in my smokey memory seemed more like a tribal Shaman.  He would massage my brother's ankles and perhaps mine as well.

I seem to be going on more about my brothers but it's for reason; they did suffer more than I did when growing up. However, it seemed that at least one of my friends in the fourth grade recognized that I was different when I attempted a cherry drop from the monkey bars and failed miserably landing on my head. I distinctly remember hearing my friend run over from a different area of the playground yelling at the girls who "allowed" me to attempt the drop, "why did you let her do that? She's fragile!" Wow! Up to then, I never thought of myself as fragile.  I remember thinking while blood streamed down my face and I was escorted to the nurses office,  “I’m not fragile! That was not me, she was mistaking me with my brothers. My brothers break not me!”  Like most kids with bumps on their head, I survived.  There was no injury to the neck, no injury to the back...I was not breakable!

When I was finally in high school and made marching band (positions were earned) I managed to step on an uneven part of the practice field and pulled something in the back of my left leg. I kept marching in spite of the pain because I was not about to give up my spot and become an alternate (one of those kids that just sat on the side lines). The next day I had an ugly bruise directly behind my knee but off to practice I went anyway. The bruise grew over the course of several days and covered the entire back side of my leg from my ankle clear up to the base of my buttocks. My friend, Jackie, pointed out the extreme ugliness of it so I covered it up by wearing pants.  There was no doctor’s visit and I survived just fine again...I was not breakable!

It was my sophomore year in high school when my brothers were finally diagnosed with Osteogenesis Imperfecta and everything for them changed. They were quickly pulled out of all P.E. classes and couldn't participate in after school sports. The Orthopedic doctor finally asked my mom if there were other kids in the household and she told him she also had a daughter.  I was taken in to my brothers’ next office visit and as soon as the doctor saw me, he confirmed that I was afflicted with it as well.  “What! not me! I AM NOT BREAKABLE!” But the whites of my eyes gave it away, apparently I’m a walking billboard for O.I.  The whites of my eyes known as the sclera, is greyish/blue rather than white; a common trait of O.I. patients.  I was tasked by my mother with looking up O.I. at our school library but it was only a high school library and there really wasn't much known about O.I. back then anyway; there still is little, public knowledge for it is a rare disorder. So rare that I often feel I am the one educating my medical caregivers about the condition and its symptoms.

Through out the years, my brothers kept suffering from broken bones.  My middle brother suffered the most and had other, extreme symptoms.  Like Diabetes, O.I. has different grades of affliction and in some grades also affects soft tissue and organs.  My middle brother ended up having open heart surgery twice because his mitral valve was not functioning as it should.  Just last year, my baby brother had the same surgery.  “What’s my fate?”, I wonder.  I’ve brought this to my primary care physician but he seems to think that it’s not something I need to worry about.  I do live a much healthier lifestyle and have for many years but still, the history is there in my family so it is a concern.  

As I have aged, my bruising has become more severe, my spraining of ligaments and tearing of tendons occur more often.  At first, I was open to the idea of it just being a natural, aging process because remember, I am not breakable, but as more research is done by the O.I. Foundation(O.I.Foundation Web Page), more information becomes available for me to use as a resource.  My connective tissue does not absorb collagen type II like a normal person so my afflictions are not the same as the normal person of the same age.  I have added multiple supplements to my diet to assist but there is nothing that fixes my affliction...I can at the most, just minimize the occurrence though lately, I can’t seem to even do that much.  And then, there is my loss of hearing.  Also a common trait of O.I. and one that seems to get worse with aging.  I have worn hearing aids since I was in my late twenties and every ten years, I need an upgrade.  Will I eventually lose my hearing all together?  Only time will tell.

My CalVmx friends (CalVmx - California vintage motocross racing) have seen me in some sort of brace year after year and I’m not a racer.  My injuries have occurred from the simplest of things.  I have had multiple surgeries to correct for torn ligaments and tendons and even tried surgery to correct for my hearing loss.  I am not breakable...I tear.