Passion

Is it literally possible to have more passion?  I am here to tell you.  Yes.  Yes it is possible.

I always felt passionate for my kids, my family, my work, being athletic, etc.  I never really charted out on paper where to direct that passion.  I stumbled upon great ways to take all those thoughts in your heart and your head and direct them in a certain direction.


There are two things that made me realize that you can turn passion into tangible actions and results.

1.) The Passion Planner

and

2.) The book Creating Innovators by Tony Wagner.

I have a 10 minute commute each day from dropping one of my sons off at school to work.  One day last November, I caught the "What's Trending" segment by Carla Marie on Elvis Duran and the Morning Show.  It's actually right here: http://www.elvisduran.com/articles/whats-trending-461825/whats-trending-november-18th-12981210/

I love to look at my home and work schedules in one month views.  I bought the Passion Planner then set my passion to work.  I dove right into creating "mind maps" in the Passion Planner.  It is a great tool for plotting out what I really want in one year, 3 years, my lifetime, etc.  I had used mind maps a lot but never on some of my own personal goals.  Here's what I wrote for the One Year goal (yes I wrote in pencil, ha ha!):


The Passion Planner calls this a "Game Changer".  Then I took that goal and popped it out further. One of the branches of the Mind Map looked like this:

This was only one piece of my mind map.  I brainstormed ideas for getting my kids involved in sports, how to help Alex (my severely affected son), how to be more fit and healthy myself, etc.  In my overall 1, 3, 5 year, and lifetime plans I have other goals like Financial Health, Luxuriating Our Home, my career, etc.

I look at these goals every week incorporating them into my daily, weekly and monthly activities.  Thus our recent launch of our non-profit, raisingrareboys.org.

Why do I have a 1 year goal to have a healthy family when my kids have a fatal, rare genetic disease?
Doesn't seem very smart now does it?  You can sit and wait for someone else to save you; a pharmaceutical company, the U.S. Food & Drug Adminstration (FDA), the National Institute of Health, etc.  They may not make it happen, and they certainly can't do it alone.  You have to have a part in your own destiny. We have to take part in doing something. Our kids are counting on us to do something. Our young children's lives are counting on us to do something to help them.

When Alex was diagnosed with Hunter Syndrome at about 18 months old, we were given choices.

1. Treat him with Elaprase knowing that it cannot pass through the blood brain barrier to treat his brain and central nervous system (CNS),
2. Try a Bone Marrow Transplant but that carries the risk of morbidity and may not also be effective enough to produce enough enzyme to keep his brain and CNS healthy, 
3. Try to get into a clincial trial just starting (the Intrathecal Trial that Nick is in today) while treating him with Elaprase. 

There were other choices like do nothing, etc. but above are the main three.  

We made both the right and wrong choice.  At the time, data indicated that we were possibly facing a mild gene mutation but couldn't be sure.  75% of kids with Hunter Syndrome face Central Nervous System involvement so we knew that as well, so CNS is going to need some support.  We met all sorts of kids who on the surface looked like they were doing well, and some severe.  We decided to treat with Elaprase but discussed involvement in the trial but didn't pursue it fast enough.  By the time we called, Phase I was full and it was moving into Phase II where it would have stiff IQ testing requirements.  So, here we are. We didn't have any experience with clinical trials.  The safety phase just seemed too scary.  We made the wrong choice for Alex, and because of it he is suffering more, and it could shorten his life span.  We made a life altering choice.  It sounds like I am beating myself and my husband up but we made the best, supported decision we could make at the time and hindsight is always 20/20 as they say.

For every negative reaction there has to be an equal and opposite action (Newton's law).  We have to fill the pipeline.  We have to support research, we have to support current progress in gene therapy. We have to fill the pipeline with treatments for all kids with MPS, including Alex.  This is the reason for the launch of our non-profit.  We have to do it.  We have to do it for Alex.  It's the only way to right our failure.

Then this summer I read Creating Innovators by Tony Wagner.  I saw this book through discovering the AltSchool in San Francisco. They have a recommended reading list (http://blog.altschool.com/the-future-of-education-our-recommended-reading-list). What an amazing little trail, to find a great book.  If you are looking for how to raise young innovators, you like education reform, or are against standardized testing this is the read for you.   There are a ton of take aways for parents, e.g., offering a buffet of interests to your kids.  You can use a lot of the suggestions at work and in the corporate environment too.  The entire summer I fostered a boring environment.  My oldest son went to Invention Camp and then I bored him the rest of the time to facilitate him making things in my house, exploring nature, golfing, playing baseball and fishing like crazy. Let's call this Project Bored-Him. No offense to his babysitter (she was on board with the mission) and Invention Camp was amazing! He loved it! The other thing that this book taught me was about the differences in people who are innovators, how differently they think and what innovation needs to thrive.  It helped me further organize our passion for saving kids with Hunter Syndrome.  No one should have to live with the MPS/ML diseases they are terrible, hands down.  I thought about one quote from the book every day for a whole month about sometimes when we're trying to prevent something bad from happening to our kids, or protecting them from something bad, we might not be able to prevent it at all (that's major paraphrasing) and we shouldn't let that get in the way of their experiences. I think our kids will have their lives shaped from living with this disease and how we've dealt with it relatively positively. What if I can't save my kids?  What if there isn't enough time to save them?  That weighs on my mind but we have to push forward, standing still doesn't feel like the right thing to do.  Throughout the book, it talks about Passion, Purpose and Play.  I think our Long Drive II Survive competition that we started last year embodies just those principles! That's why last year it was so fun, and we hope to keep it going annually!

So off we go launching into our new non-profit; our success will be measured in lives saved, lives improved, and lives touched.  So now you know, why now.  This is why, and maybe some of the tools the Passion Planner and this book can help you too.

Amy & Her Awesome Family

Summer

Surviving our Summer

For Mid-westerners, the summer is precious.  You don't survive it.  You welcome it.  It's the season of slow starts to days that seem to last forever in a good way. It's endless sunshine but always strange weather. Summer is the revival of festivals and outdoor concerts, Cedar Point. Countless hours at the pool, giggles, running, bubbles, lightening bugs in the dark against the starlit sky, campfires, s'mores and all things that make childhood great.

Not exactly our summer.  The way we survive living with Hunter Syndrome is we strive to get past the first obstacle then the next then the next. We know that sometimes things are temporary and will get better. When you start weekly infusions you get past them until they can be done at home and not in a hospital. Your kids do not want to go to the hospital weekly. You get past monthly clinical trial visits in Chicago to the extension study where it's hopefully closer to home. You cherish people who help you, who are selfless and who think of you first.  We had several sweet, selfless people help us this summer.  You tell yourself that you just need to keep going! You just survive it. You compartmentalize it.  You arrive at it. You watch it go.  You make the best of it and then you hope that nothing unexpected throws things into more of a tizzy than it already is, and in the midst you have a life and somehow it works.

This is what we did, and we made it through!  We made it through June, July, and now we're making our way through August.  In June, Hunter Syndrome and living a normal life collided creating a pace that was an all out sprint all month long. As parents we feel the weight of getting everything done that Hunter Syndrome requires; weekly infusions, planned and unplanned surgeries, bouts with random illness, clinical trial visits, finding child care for special needs, planning regular summer activities around Hunter Syndrome, while working and taking care of our family...when you list it out or blog about it...it's unbelievable. During the summer our careers were also in overdrive, but we just kept going.  With two children with Hunter Syndrome, both having such disparate experiences it's starting to feel like an ever revolving door, in and out to this and that. We also did our best to have fun, and give the kids a great summer but it was a struggle to keep the balance! We missed spending time with our friends, catching up with old friends, and celebrating our wedding anniversary.  If we missed you or something was canceled, know we still think of that and miss you. We'll make up for those misses as we're still so grateful for our lives, for our boys, for the opportunity to fill a clinical trial spot, and to keep driving to keep our boys as healthy as possible.

Surgeries and Clinical Trial Visits

In June, Alex's surgical needs became a top priority.  When a child goes in for surgery, especially a medically complex child, lots of appointments are needed, letters, and paperwork are required from specialists to essentially clear the child, and OK the surgery.  With Hunter Syndrome, you have lots of specialists.  It is a lot of work for the parents.  Especially if you're due for a followup or the physician wants to see the child for an updated look at their health.  These clearances are required from everyone; genetics, pediatrics, all specialists, as I said it's a lot for Alex and it's a lot for us.  I continue to be so impressed with some of his physicians who always put us first, and think of our needs and do whatever they can to help ease our worries and medical load of appointments.  I am so thankful for those physicians. They are the ones who really get it.

Coming out of surgery

Going under anesthesia is a huge event for a child with Hunter Syndrome, we work with physicians to prioritize and "piggy back" all the tests, procedures and surgeries that will occur with each anesthesia scheduling.  We have a backlog of preventative surgeries where the condition is just rearing its head...e.g. carpal tunnel, and surgery is required sometime but not at this exact moment. We also have a regimen of testing that requires anesthesia.  We essentially carry around an app, a planner and a backlog list of things we need to get done.  Genetics does a great job helping us with this, we really couldn't survive long without them.

Heading home, not feeling great

So Alex went in for surgery in June.  Getting a tune up on the ears that were causing uncomfortable pressure, and again that fluid creeps in every time those tubes fall out.  Thus, he's a candidate for permanent tubes.  So fingers crossed.  We're hoping this addresses the can you hear, how clear can you hear dilemma that we've been dealing with for some time (almost 2 years), and makes him more comfortable as well.  Hearing aids are reprogrammed with the right levels, and we're ready to rock those puppies out getting some good quality sound into those precious ears.  On Facebook, I gave a compelling account of how hard it is to jam hearing aids into Alex's ears.  It's like tackling a football player, and taking those tiny little things and shoving them in a very physically strong, strong willed child.  A basket hold or strong bear hug is required, and it is not for the weak.  We are on it though, he hears way better with them on! We are keeping the faith in this department and trying to bolster total communication! He also had hearing testing, an ABR, new ear molds, etc.  So it's go time...we have to help Alex develop more language if he can!

Next were his teeth.  Like many Hunter Syndrome kids, Alex chews things wearing away the enamel on his teeth.  It's horrible.  He had tons, and tons of dental work.  Gladly he did his follow up here in Cleveland without general anesthesia which is nothing short of a miracle, and shows that he is alert, he knows what's happening, and he's willing to comply sometimes.  Our master plan for Alex is to keep working on the issues caused by Hunter Syndrome improving his overall quality of life.  We watch clinical trials for him, and continue to hope that treatments progress and become available that can help him.  Alex isn't declining rapidly, it's just that he isn't moving forward and developing at the same pace as his peers. So, it's a slow decline.  He has been through so much this summer we tried to let him rest as much as possible.  He also had to deal with some random illness throughout the summer that was likely tied to just the regular issues Hunter Syndrome creates with their intestines.

Not one summer day goes by that we don't think about what we can do for Alex. For us he remains a puzzle, that we're trying to complete because he's worth it and he's completely adorable.

What we build at clinical trial visits from Legos and a Coffee carrier!
"...one small step for man, one giant leap for mankind"

Nick had a clinical trial visit each month.  We either drove or flew into Chicago for these visits trying to make them as "fun" as possible.  It is heartbreaking for even business travels to see Nick board and de-board a plane.  He sobs for his daddy.  When his older brother Jack was able to attend, it was a nice distraction from the difficult clinical trial visits.  Nick is 4.  This is very hard for him even if it's not the worst procedure we've ever been through.  Nick continues to do amazingly well!  He hits his milestones, and you would never know he has Hunter Syndrome.  Two scars from his medi-port and intrathecal port are the only indications at this point that he has Hunter Syndrome.  In August, we have two visits and our remaining schedule for the rest of the year is very busy with multi-day visits pretty much every month till the end of the year.  It will not be easy but we will get through it.  We're still holding out hope that this gets approved soon.  All Hunter Syndrome kids need access to more treatments, especially those that treat the brain, and central nervous system.

Flying to Chicago

Then there was a whole lot of Fun and normalcy

Jack who is nine kept pushing us as a family to get out even though we were exhausted.  We saw fireworks for the first time all together sitting outside of our car.  We went bowling.  Joined the Zoo, and visited it.  Went to the Lego store every clinical trial visit, and joined their VIP program.  We saw a piano that plays without anyone playing it in O'Hare (Nick's personal favorite).  We swam, we swam, and we swam some more.  The boys golfed and golfed and golfed.  Jack did really well. Nick and Alex get some great hits too. There was a lot of baseball played.  Alex went to special needs camp. Lots of running, jumping, digging and playing.  All done in and around Cleveland and Chicago.  No vacations but as much fun as we could handle!  Some things we are still cramming in despite school starting, like a visit to Cedar Point.  All in all, we did it.  Summer still leaves me with a bit of sadness as they start school.  My husband never understands why I say I miss them when they go back to school but I do.  I will miss them until they come home off the bus!  So cheers!  We did it.  Let's revel in the fact that we got through summer, and we did pretty well!