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



  1. Vicky & Dave,

    We can't even begin to imagine what you are both going through. It's amazing, because I had never heard of this until you enlightened us. Even in all of the literature I read when I was pregnant with Rozzie & Chandler, nowhere did it say that this could happen. The saying "no news is good news" really rings true. You & Dave are very strong.

    We will pray for you and Isaiah every day.

    -Christine & Justin

    1. I am right there with you! I had heard of dwarfism but not OI! It's such an extremely rare disease.

  2. Thank you both so much for sharing your journey. I must admit I have shed tears for your dear family and am in aw of how strong you both are. A day doesn't pass that I don't say a pray for your family.

    Anna and George