S-T-R-E-S-S-E-D and the Fontan

I am STRESSED to the MAX! We are just five days away from leaving for Ann Arbor, and we're feeling the pressure. I'm wading my way through an ever growing mound of laundry, dishes, and toys... trying to keep my focus on what absolutely needs to get done (writing this blog entry is not one of them, but it's a little break for me :) ...and trying to keep the summer fun for the kids despite our upcoming hurdle. But we're all feeling the stress; the kids are acting out (some more than others,) I'm cranky and snapping at the boys, and we're coming down with colds. The boys are watching way too much TV, and I'm letting them... they can't stay outside too long with temperatures flirting with 100 lately.  I have some great pictures and stories of our summer adventures, but those will have to wait until we're in Michigan and I have time to post them. Instead, I'll update about Zachy and his upcoming surgery.

Last week Zachy and Samuel came down with a nasty cold. Fortunately after about a week it seems to have run its course, and we're praying for Zachy to be in the clear as he heads into surgery. Yesterday he went for his 18-month checkup. Currently he weighs 21 lbs. 5 oz., which puts him in just the 2nd percentile for weight (though he is 35% for height.) The good news is that he is still growing, and I would expect that to pick up after this next surgery.

Steve, Zachary, and I fly up to Detroit early Sunday morning. We are on the waiting list to get into the Ronald McDonald House, but we had to make hotel reservations just in case. The bigger boys will stay back home with family. THANK GOD for family! I don't know if there is ever any way we can express our appreciation for all they do to take care of us. They have big plans for the boys, and have promised a camping trip to boot! On Monday Zachy goes in for pre-op testing, which includes x-rays and a sedated echocardiogram. Tuesday he has his heart catheterization to get pictures, take measurements and pressures, and assure that he is a good candidate for surgery. Wednesday is the big day, though we still don't know what time he will be scheduled.

The goal of the Fontan procedure is to ease the workload on Zachy's heart, which only has one effective pumping chamber. The first stage of this surgery was completed last summer, and the final operation is expected to go smoothly.  The following explanation is taken from U of M's website:

The Fontan procedure is the third stage of the repair. It is done when the child is between 18 months and 2 years of age. A heart catheterization is done before the Fontan.

Why is the Fontan procedure done?

The Fontan is done so that almost all the blue blood coming back from the body goes to the lungs. After this stage, most children are much "pinker" because now nearly all of the blood pumped out to the body goes to the lungs first.

How is the Fontan procedure done?

The patch that was placed in the right upper chamber is removed (1).
A wall, called a baffle (2), is built in the right upper chamber. The baffle guides the blue blood coming from the lower body into the blood vessels that go to the lungs (the pulmonary arteries).

A small hole, called a fenestration (3), is made in the baffle. This allows a small amount of blue blood to go across the baffle into the right upper chamber. This hole works like a pop-off valve in case the pressure in the lungs gets too high. The size of the hole may vary. For most children, a small hole is made that will close by itself over time. In some children, a larger hole is needed. Closure of large holes is usually done six to twelve months later during a heart catheterization.  Click here for a video explanation of the Fontan.

Keep in mind that the illustrations I post are slightly different from Zachy's heart, since he has such a unique little anatomy.  Still, they do a good job to explain his overall defects and the corrective procedures.

We expect Zachy's hospital stay to be between one and two weeks, and we hope to return to Wichita sometime in mid-July.  Is this his final surgery? many people ask us.  I wish I knew!  The Children's Hospital of Philadelphia addresses the question in this video.  But no doctor or surgeon can really answer the question.  All we know is that this is the final planned surgery to get his heart to work as efficiently as possible.  These surgeries have only been around 20-30 years, and there are not many survivors from early on.  Some of these patients now in their 20's and 30's go on to experience problems such as leaky valves, arrhythmias, protein-losing enteropathy, and heart failure.  Zachy would need additional procedures if these problems arise, and could very likely require a heart transplant somewhere down the road.  In the meantime, though, we're quite thankful that his special mended little heart is pumping hard and strong!