We have a plan...

                   

Owen did so well yesterday at his Cardiology appointment. The highlights were:
1. When they called his name to come back for his appointment he ran up to the MA and said "That's me! That's me!" and gave her a hug.
2. He was perfectly still for his 35 minute ECHO thanks to the monster truck DVD we brought and his awesomeness

3. He was also perfectly still for his quick EKG. These have been more challenging for us in the past as  he really does have to be still to get a good reading. He was so still (with minimal holding down on our part) and it read within 2 seconds...A FIRST!
4. He put on gloves and checked Dr Rivera's heart first with the toy stethoscope we brought then with the doc's stethoscope.

Owen's oxygen levels have been stable at 83-86% for a while. This is consistent with his anatomy and he really won't improve. The challenges with a lower oxygen level for a prolonged period of time are many. The body is in a constant state of trying to adapt. His body makes more red blood cells, so his blood is thicker, and his hemoglobin and hematocrit levels are chronically elevated. This is why he takes 1 baby aspirin daily to thin his blood from the thick red blood cells. The risks are mostly here-- he is at a higher risk for stroke for this reason and other as well.

The other challenges is developing normally with a decreased oxygen level. He currently does not have any developmental problems but we have also been working on his development for years!

So MD usually recommends the next surgery between ages 3-4. He has mentioned this to us in the past but I also thought we would monitor for failure first and then fix Owen up. The problem with that mentality is that heart damage can occur and then he could go into surgery in a weaker state.

So, given the fact that our upcoming fall season is a little busy with you  know, a new baby, and the fact that Owen is stable now he was agreeable to us moving forward with things in the Spring.

So he will first have a cath in Februaryish and then the Fontan procedure in April/Mayish at UCSF.

The question of the years still needs to be answered....
What about the feeding tube?? The one we haven't used for a year!? Well Riv was willing to sign us off from a cardio standpoint and defer to GI to take it out but with an actual surgery on the books he thinks it wise to leave it in. It doesn't cause any issues, but it would be more of a milestone to get it out! So we are with the tube for another 6-9 months.

Here is some info I just cut and pasted if you want a review of Owen's anatomy, past procedures, etc. Owen's summary/my additions in RED.

Operative stages for achieving a Fontan circulation This final surgery is the goal of the previous two.

The type of operation needed in the newborn period is quite varied depending on the specific type of single ventricle cardiac defect. In some babies there is not enough blood flow into the lungs, resulting in cyanosis. In these babies, a tube graft is placed from an artery (usually the left subclavian or left innominate artery) to the pulmonary artery. This is called a systemic to pulmonary artery shunt or Blalock-Taussig (BT) shunt. Owen had this placed 8/13/2010

In other babies, the flow of blood into the lungs may be excessive, placing an extra burden on the ventricle and exposing the pulmonary arteries to dangerously high pressure. In these babies, a procedure will be performed to restrict blood flow to the lungs. This is done by placing a piece of material or a “band” around the pulmonary artery.

Rarely, a baby with a single ventricle anomaly will have “just right” flow into the lungs so that an equal amount of blood flows to the body and the lungs. These babies do not require intervention in the newborn period. This was why the team waited so long for the first surgery, as they wanted to be sure that he really needed it.

Whatever is needed in the newborn period, the aim is typically to balance the blood flow between the lungs and the body, achieving stable oxygen levels and adequate heart function.

The second stage for most children with single ventricle anomolies is undertaken around three to six months of age. The operation is called a “bi-directional Glenn” or sometimes a “hemi-Fontan.”Owen had this in Spring 2011... Need to look up the date again...

During the Glenn operation the large vessel that drains blood from the head and upper body back to the heart (the superior vena cava) is taken off the heart and sewn directly to the pulmonary artery. If a prior BT shunt was present, it is removed. If a pulmonary artery was previously placed, it may be removed but can also be left in place in some situations.

The Glenn operation has two major advantages in most children. First, because the connection is a direct one between two blood vessels, rather than made of artificial matter, it has the ability to grow with the child.

Second, it removes some of the work of pumping blood to the lungs from the single ventricle so that the ventricle will no longer have to pump all of the blood to the lungs in addition to all of the blood to the body, which places it at risk for early heart failure. In most cases this stage is tolerated the best of all the stages with a survival rate of 95 percent or better.

After the Glenn operation most children will have oxygen saturation levels of 75 percent to 85 percent. His saturations have met goals after this procedure.

The third and final stage in the reconstruction of a single ventricle heart defect is theFontan completion operation. This operation is usually performed at 2 or 3 years of age, based on the child’s size and clinical status. The shunt placed here is a large one that is meant for kids >15 kg. It is supposed to last decades.

During the Fontan operation, blood returning to the heart from the lower half of the body (via the inferior vena cava) is connected directly the blood from the pulmonary arteries. Up until now this blood has bypassed the lungs resulting in oxygen levels lower than normal.

After a Fontan operation, oxygen levels will be nearly normal (90s). The two most common methods of performing the Fontan completion today are the “lateral tunnel” and the “extra-cardiac” techniques.

In the lateral tunnel method, a tunnel-like patch is placed inside the atrium so that blood returning from the inferior vena cava is directed through this tunnel. A connection is then made between the end of the tunnel / top of the right atrium and the underside of the pulmonary artery.

In the extra-cardiac method, the inferior vena cava is taken off of the heart and a synthetic tube, usually Gore-texTM, is sewn directly to the top of the inferior vena cava and to the underside of the pulmonary artery, routing the blood flow outside of the heart. We are hoping for the extra-cardiac method as the less trauma the heart itself can have the less likely for him to have irregular heart rhythms. 

Ok so there you have it... Yipes. We are dreading this since we know what to expect per se, but the experience does come in handy. It will be interesting with an infant, dividing our time between two kids who we want to be with so much. The time to prepare is nice but also gives us a lot of time to think about it all. Thank you to all of the continued support, love, and care for us and "baby O" who is not so much a baby anymore! He read (memorized) me the entire The Very Hungry Caterpillar tonight, so special. I'll try to post that video if I can be so technical tonight.