Tuesday, May 28, 2013

To Dupont or not to Dupont

That is the question.

I was truly hoping (dreaming) that we'd walk out of Dupont confident in pushing to have Isaiah there.  Of course I was also dreaming that the specialists would look at the ultrasounds and say everyone else is crazy and Isaiah is fine.

But we're in reality, and in reality, Isaiah has severe OI.

We had our appointment in the "yellow module" of the hospital.

We met Dr. Bober, he's a geneticist and pediatrician who treats children with OI...but baby isn't even here yet.  Dr. Bober went by the reports from our last appointment at CHOP so he spoke mostly about type 2 OI.

He helped us understand that it's not that Isaiah won't have room in his chest for his lungs to develop (his chest is actually normal sized).  It's that his ribs are so thin and easily breakable that just breathing could break them.  And with breaks, comes pain.  Can you imagine trying to take deep breaths with multiple broken ribs?  That is where the issue is.  He may be in too much pain to take deep enough breaths to sustain life.  Don't get me wrong, the ribs are also healing crooked, but as of our ultrasounds on May 2, his chest size is in the normal range.

He also spoke to us about the plan for after Isaiah is born.  He recommends getting a treatment called Pamidronate (Those familiar with this treatment call it PAM) as soon as Isaiah is stable enough for it.  PAM is known to strengthen bones, help with pain, and lessen fractures after it's in the system of someone with OI.  His first dosage would be smaller than normal and then he'd begin full dosage treatments at 8 weeks old and every 8 weeks after that.

However, that's only if Isaiah can get to a point that they can insert that IV.  When he's born, we might have tough decisions to make that affect his quality of life.  If Isaiah can't breath on his own, do we want to put him on a ventilator at all?  If he can initially breath on his own and then develops respiratory issues, what about then?

We have a lot to think about.  We're now waiting for them to talk to the OB that Dupont is working with [to deliver babies right there at the hospital].  We're planning to have our next ultrasound with him so that we can meet him and see if we're comfortable with the idea.  One big negative for me about having Isaiah at Dupont is that after the c-section they would transport me to Christiana Hospital.  What if he does die and I'm not there? This makes my heart hurt.

On a brighter note, we did get to meet two families from our OI support group before our appointment!  We met both moms and their kids with OI.  The kids were spunky and adorable (and so were the moms! ha).  Their moms were positive and so obviously in love with their beautiful kids.  It was great to chat with them about their experiences...especially because they helped settle my worry about Isaiah not moving too much/having the hiccups like crazy (they both had similar experiences and know the difference between OI babies and non-OI babies as they both have other kids).

When we got home, I went and read over Dave's post that he made after our last appointment at CHOP.  He had shared that we were told that they believed it's type 2 and lethal and were at our lowest point.  Reading through over 20+ responses of how they had been told the same doom and gloom diagnosis's....and yet they have their kids ranging from days old to in their 20s.  They are alive.  They are breathing.  They are happy.

It's inspiring and strengthens us.

At the same time...I had recently found a mom (in the support group) who is pregnant with a child suspected of OI either type 2 or 3....She was due just 2 months before me.  Sadly, she lost her snowflake today.  If you see Dave and I have changed our profile pictures to snowflakes, it's for baby Jake and his family.  RIP baby Jake. It is snowing on Facebook for you.  I hope you are at peace and without pain up there in heaven.  <3


Monday, May 27, 2013

Good Intentions

Dear Family and Friends,

We love how much you love us.  We love it beyond words.  We want to be sure you know something...

Your children are wonderful.  Your children are beautiful.  Your children are a part of our lives.

Please don't be afraid to invite us to see you or bring your children to an event/activity because we're going to be there.

We love seeing them.  They make us happy.  They give us inspiration.

Do not worry; our sad moments most often come after difficult doctor's appointments.  Or when we're by ourselves in the car and torture ourselves with music with too much meaning behind the words.  But when we see you/your children, we feel happiness.  We feel optimistic.  We feel love.  We feel loved.

Thank you for being so considerate of our feelings, but knock it off. :)
Vicky and David

Wednesday, May 22, 2013


Dave and I were thinking (dangerous, I know!)... it's time to move on from posting on our shutterfly share page.  Our story is spreading fast and if we're going to ask you to pray for our baby, we wanted to give you an easy website to get the latest information about everything.

So here we are. OIbelieveinIsaiah.blogspot.com

A big motto for people with OI is "O.I. CAN!"  Well, Dave and I don't have the disease, our baby does.  And simply, we believe.

We believe our son is going to defy the odds....I should probably back track.

For those that don't know Dave and I, here we are:
We've been married for 5 years, together for 8 years.  

We are first time parents-to-be to this beautiful baby- Isaiah David (cuteness alert!):

We may be biased, but he's obviously the most adorable baby in the universe.  

We'd been hoping to start a family for some time.  We found out about our pregnancy a few weeks before Christmas of 2012.  This gave us the opportunity to tell many family members face to face, so although it was early, we shared our exciting news:  http://youtu.be/su5hjOPkG8c

After our announcement to family, we slowly rolled out the news to friends...and before we knew it we were revealing our surprise to Facebook with this post on our shutterfly page: http://dacky.shutterfly.com/ournewhouse/152

The weeks went by, everything seemed to be progressing as it should.  Baby's heartbeat was always "perfect".  I had zero morning sickness.  I was enjoying almost everything about pregnancy (except the growing pains....ladies who have experienced it know exactly what I'm talking about here).  We went to our 20 week ultrasound like 2 kids on their way to Disney World.

We had our ultrasound.  We were on cloud 9.  After that, we met with a doctor (what was normal protocol...), where our world came crashing down.

"We have some concerns about what we saw on your ultrasound today.  Usually we like to see growth in the 50th percentile, your baby's arms and legs are in the 5th percentile.  The long bones are curved when they should be straight.  Is there any dwarfism in your family?"

Dwarfism? Just the opposite.  We've been joking since the beginning about how our baby may end up being a giant since we both have some seriously tall siblings.  

We went home.  We cried.  We racked our brains trying to figure out what to make of the appointment.  We googled "curved long bones during 20 week ultrasound".  This gave us hope.  Babies grow at different rates.  Maybe the arms and legs will sprout and grow and  the baby will be "normal"?!?!

We scheduled an appointment with a high-risk pregnancy specialist.  They squeezed us in for 2 days later due to the "urgency of our situation".

We still hadn't found out the gender of our baby.  We had asked the ultrasound tech to write it down instead of telling us.  We had big plans.  After the appointment we had thought about canceling our "baby day", but we just couldn't, we had hope that the specialists would see something different.

We went on with our plans:

(yay, finally got one of the videos to embed!)

It's a boy! Our boy.  Our son.

Our appointment with the specialist came quickly.  We spent way too long in the crazy busy waiting room.  Then we had an ultrasound that lasted an hour and a half.  (Baby was stubborn!)  The doctor not only confirmed the idea of skeletal dysplasia (dwarfism is a type of skeletal dysplasia), but he informed us that he was extremely confident that our baby has Osteogenesis Imperfecta.  There were healing fractures in our baby's arms and legs, but at the time, his chest and spine looked great.  His head was too easy to change the shape of with the ultrasound thingy, which is another sign that he has OI.

We had another tough day but we quickly rallied our hearts; we shared the news with immediate family.

 To think that just earlier in the week we had visions of first words and first steps, and now we wondered, will he be able to walk? 

We found oif.org.  We read about OI.

We accepted this diagnosis. We still have our son.  We knew, though, that our plan to deliver him close to home may have to change.  This disease is so rare, not many doctors and nurses have experience delivering a baby with OI.

I called CHOP the very next day after the appointment with the specialist.  The doctor had recommended talking to doctors who specialize in OI, so I did just that.  We made an appointment with the genetics specialists there and learned more about the disease.

We couldn't keep this news to just a select few...we knew we had to share and spread the word.  We needed support.  We needed faith.  We needed prayers.

We put together a video to share the news with everyone...

We found a support group for parents of kids with OI.  That group is FULL of over one thousand members, many of which have kids with severe OI that CAN walk...they just learned a little later than other kids their age....but their first steps were a miracle and are inspiration to us.

Weeks later we went back to CHOP for an entire day of many appointments....appointments with genetic counselors, ultrasound techs, OBs who specialize in special delivery cases, and more.  We had a ridiculously long day.  And at the meeting at the very end of the day, it became agonizingly longer.

The doctors saw curved long bones. They saw fresh fractures in his arms and legs.  Devastatingly, the doctors saw fractured and crocked ribs.

We saw our baby hiccuping.  We saw him yawn. We saw him waving and sucking his thumb.  We saw him moving his little mouth like he was talking.

We found out at the meeting that Isaiah's condition is possibly worse than thought at 20 weeks.  They believe he has type 2 or possibly a very severe type 3....

And they believe it's lethal.

They believe it's lethal, because, although his chest was measuring correctly at the time, it's "likely" that because of his broken ribs, he won't be able to take full deep breaths when he's out of the womb- his lungs just won't have the room.

Is there a word for worse than devastated? Because that's how we felt.

Luckily, we have the OI support group. We posted our "diagnosis".  Within 24 hours we received well over 25 comments telling of similar diagnosis's....and yet their babies are alive.  Some even with type 2!  Some are just not as severe as the ultrasounds made them look.  They range from only days old to 17 years old.  

And just like that, our hope returned.

The doctors advised that we have an amniocentesis done to verify exactly which type of OI Isaiah has.  After a lot of thought, we decided against it.  We'd rather have the hope.  We'd rather the doctors not have a "confirmed type 2 diagnosis" because if they did, they'd treat the delivery differently; I'd become the priority and we'd have a vaginal delivery that would likely cause Isaiah even more fractures, and we could "spend the time we have with him alive, with him".

Listen, we want them working their tails off on our son.  We don't want him living off a ventilator, but we want to give him  a CHANCE, because darn it, we believe in him.  We believe in his strength.  We believe in his ability to breathe.

There is no giving up on Isaiah from anyone except Isaiah.

We're still talking with CHOP, but as per the advice of so many OI parents, we're also talking to AI Dupont in Delaware.  They have more experience with OI.  We're hoping they'll be more positive on the diagnosis and have the hope for him that we have.

I have more to share, but I don't want to scare you away thinking I am going to write novels in every post.  This post is an introduction to our story.  Some posts in the future may be about the past and our excitement for our first baby.  Some posts may be about our doctors appointments and our plans for delivery.  Some may just  be brain dumps; some when we're having good days, some not so much.  

We hope you'll follow us in our journey in bringing Isaiah into this world.  We hope that if you believe in prayer and a higher power that you will pray for Isaiah.  Pray for his ability to breathe.  Pray for his strength and bravery.  Pray for his will to live. Pray for our continued strength and optimism.  Pray for the doctors and that they don't give up on him.

Most of all...pray for a miracle.  If we have thousands truly praying for Isaiah, I believe God will hear us.  

O.I. Believe in Isaiah, and we love him with all our hearts...