A Broken Heart

35,000 children are born with heart defects every year (American Heart Association)
Only 1% of those children have truncus arteriosus (many sources, including the Herma Heart Center where Turnip may be treated)
That means about 350 babies are born in the U.S. with the same condition as Turnip every year. (330 according to the Morbidity and Mortality Weekly Report from jan 6, 2006)
Surgery usually happens at 6-12 weeks (EPA, page 4)
15-50% of children require another surgery within the next five years (i.b.i.d.)
All heart valves are replaced after 12 years(i.b.i.d.)
Newborns with truncus arteriosus stayed in the hospital for an average of 30 days during 2003 (CDC, table 2)
The hospital charges for this stay (not including lab tests and doctor fees) ran to $200,000 (CDC, table 2)
In a small study published in 1996, 6% of infants died before surgery and 10% died during surgery (EPA, page 4)
In a national survey, 20% of newborns with truncus arteriosus died in the hospital in 2003 (CDC, table 1)

Thursday, October 20, 2016

not quite sticky enough

Today we visited a pediatric hematologist to see if she could come up with a reason for small boy's nose bleeds (I was also sort of hoping she would come up with a reason why he would not be able to get a mechanical valve).  Small boy had some fancy pants blood work done a few days ago, and we went in so she could explain the results. 

It turns out that there is a reason why small boy's nose bleeds last so long.  Blood has lots of different ingredients: some of them help carry around oxygen, some of them help fight germs, and others help plug any blood vessels that get leaks by forming a clot.  There are lots of different ingredients that help blood clot.  Small boy has a problem with two of them. 

 Sweet Gum Balls from my yard. First are the platelets.  Platelets are like sticky sweet gum seed pods.  When a blood vessel gets a hole in it, they stick to the edges of the hole...and then they stick to each other and form a temporary plug.  Small boy's platelets are not as "sticky" as expected.  That means they form a less stable plug more slowly than expected. 

Then there is factor IX.  Factor IX is one of many ingredients that create the "glue" that coats the platelets after they form that plug.  The "glue" helps strengthen the plug made out of the platelets.  Oliver has less factor IX than expected.  This means his platelet plugs have a harder time staying stuck together after they form. 

We don't know why small boy's platelets are less sticky than expected or why he has less factor IX than expected.  His unusual heart anatomy may be making his platelets less sticky.  He may be using up more factor IX to make up for his less sticky platelets (Use more glue to hold the flimsier clots together).  He may have a mutation in the gene that codes for factor IX.  We don't know.

What we do know is why he has such long nose bleeds.  We also have more information to think about as we consider how to proceed with his next surgery.   There is a medicine (DDAVP) that may help when he gets long nose bleeds, but overall, having an explanation for why he gets the nose bleeds does not prevent him from getting nose bleeds...or from getting a shiny new mechanical heart valve for his birthday this year.