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)

Monday, December 12, 2011

quarterly cardiology appointment

Yup,  we have reverted.  At today's visit Oliver's numbers weren't so great.  Instead of dropping Oliver's final med, our doctor decided to increase the dose.  Instead of waiting six months, we will be going back again in three.  Instead of an echocardiogram, at his his next visit Oliver will have an MRI to get a more detailed look at what is going on inside of his chest.

From the echo today, it looks like Oliver's conduit will need replacing sooner rather than later.  His truncal (aortic) valve still looks serviceable, but his conduit is narrowing both above and below the pulmonary valve and his left pulmonary artery looks small.

I knew there was something wrong when Oliver's echo lasted an hour. 

On the upside, he isn't blue, doesn't struggle to breath and doesn't lack energy...yet.


Thursday, September 1, 2011

FDA trials

It has happened--what I was waiting for.  This month, the federal government gave scientists approval to begin testing home-grown heart parts in the U.S.  Two scientists at Yale University have created blood vessels using patients' own cells.   They started implanting these vessels in patients 10 years ago in Japan.  Now, the FDA has given approval for them to start placing these blood vessels in pediatric patients in the U.S.  This is just thrilling.  This is the one breakthrough that I have wished for for Oliver.  If he could get a living vessel grafted onto his heart the next time he goes in for surgery, there is the chance that he would never need another surgery---because the living vessel would grow with him. 

Tuesday, June 7, 2011

semi-annual cardiology appointment

Yesterday, we went in to see the Dr. Wilson.  Here's the run down:

Weight: 27 # (20%ile)
Height: 87.8 cm (20ish %ile)
Head circumference: 49.5 cm
BP: 99/52
Heart Rate: 97

Status of all parts (both homegrown and replacement): no change from the last two echocardiograms (the best we could hope for).

What was different:

*Oliver did not have his oxygen saturations measured
*Oliver was hooked up to leads during his echocardiogram so that they could record a trace of the electrical activity of his heart at the same time they assessed the blood flow and structural integrity of his heart.  This was just because he is getting bigger and they felt he would not mind them/pull them off.
*Oliver's cardiologist noticed that Oliver is starting to outgrow the dosage of his one last drug...and he's going to let him keep outgrowing it.   Oliver may be off all meds by his birthday.  The doctor wants to make sure there aren't any bad things that go wrong in the next six months.  This one last medicine (enalapril) is an afterload reducer. From what I understand, it reduces the blood pressure in Oliver's blood vessels so his heart does not have to work so hard to push the blood through these vessels and out to his body. 
*The doctor said that Oliver should get antibiotics before he goes in to the dentist, just as a precaution.
*The doctor said that Oliver has passed through one stage of rapid growth (infancy) without any surgical interventions and now has entered a stage of steady growth.  The biggest strain on the heart comes during periods of rapid growth (infancy & adolescence).  So, the doctor said Oliver's heart parts show every sign of carrying him through until his next stage of rapid growth...  Which is a whole lot longer than I ever dreamed.  Of course, there is always the caveat that his one homegrown valve could suddenly start misbehaving or something else could go wrong.  But, I say, why invite trouble?

Saturday, June 4, 2011

Questions from a comment:

A reader posted these questions as a comment on an earlier post.  I'll answer from my experience, but if anyone else has more to add or something different to add, please do.

1. What is the possible earliest time when you can find out your baby is having TA?
2. What is the survival rate for people with TA ? I mean what is the average life span?
3. If I had a baby with TA, what are the chances for TA to repeat in my future pregnancies? Provided me and my partner are genetically doing good (we've got genetic test done). Is there any such rule that defines the possibilities of TA in a couple, or it just so random to predict whoz next ?

What I have learned/experienced/heard:
1. My son was diagnosed at 20 weeks gestation.  I don't know if it can be diagnosed earlier.
2. Survival rate: If you look at the header of my blog, 80% of babies who get operated on, survive their operation.  Average life span: I'm not sure.  Since the first surgical correction of TA was done in 1968 (I may have the dates wrong), we wouldn't have any people who were older than 43 right now...I"m aware of a 22-year-old--and there are others you can find on the yahoo group listed on the left side of my blog.  In fact, that might be a good place to re-post your questions.
3. If you have had one child with a congenital heart defect, it increases the odds that you will have another one--not by a huge amount--but some.  I'm not sure of the numbers.  I know of several people who have more than one child with a CHD--even though they got a clean genetic bill of health.  See: "you'd never guess" and "last roommate" in the blog list to the left.

Monday, March 28, 2011

Calvin's tulips

The tulips we planted in Calvin's honor quite some time back are finally pushing through the dirt.  They didn't do so well last year-- I think it was either the rabbits or a lack of water or sun. So, this year, I have pushed the big barrel out from under our overhang.  At least I can control their access to sunlight and water, if not the appetites of the bunnies.

Friday, March 25, 2011

Oliver's doctors

Today, Oliver graduated from another medical service.  For the past two years, we have been making semi-annual pilgrimages back to Milwaukee to see their developmental team.  We had another appointment scheduled for Monday.  However, our insurance denied coverage for the appointment.  When we called the Herma Heart Center, they confirmed that as Oliver was doing so well as of his last check at their developmental clinic, there is really no reason for him to continue to receive evaluations.  Now, we are down to:

1) a pediatrician
2) a cardiologist
3) a surgical team

Oliver sees the pediatrician with the same frequency as any other kid.  He sees his cardiologist every six months.  Hopefully, he won't see his surgical team again for many years. 

Friday, March 18, 2011

what went wrong

This semester I am teaching Zoology at a local college.  Next week we will start our unit on development.  Consequently, for the past few weeks, I have been updating my understanding of how animals (including humans) develop.

On page 389 of Scott Gilbert's Developmental Biology textbook, there is a picture of what went wrong with Oliver's heart.   I can't stop looking at it.  You see, during development, human hearts go through a stage where there is only one outflow vessel from the heart: the truncus arteriosus.  This is a normal stage of development.

In normal development, that common vessel will eventually divide in half and give rise to the aorta and the pulmonary trunk.  In Oliver, and in other children with Truncus Arteriosus, that common trunk, or truncus arteriosus, does not divide in half.  For some reason, the cells that usually separate that common trunk into two vessels do not migrate into their proper place and divide the single tube in two--and so, the developing embryo is left with a single outflow vessel: the persistent truncus arteriosus. 

This process is similar in many other animals, and has been studied in chickens.  In the enlarged image, you can see the cardiac nerual crest cells migrating to the conotruncus, a.k.a. truncus arteriosus, of a chick embryo where they will form an internal dividing wall to create the separate aorta and pulmonary trunk.  For some reason, this did not happen in Oliver.  And it sucks.

Sunday, February 6, 2011

Saturday, January 1, 2011