She's doing so well.

Ema is doing exceedingly well! She is now in her own room, which means we can stay with her every minute of the day. We can also have as many visitors as we'd like. At this point, she is not on any IVs. She is hooked up to the standard monitors, but I haven't heard them alarm in days. These are all very good signs that she is a healthy baby. The next step is to get her breast feeding. She still has an NG tube (a feeding tube through her nose). She is well ahead of schedule for recovery. It looks like we'll have her home in a week or two. Thanks for all of the prayers and support!

The surgery went well.

I missed it because I came down with the flu (just my luck).

Here's Becky's update:

Emaline had her first cardiac surgery today, the Norwood procedure that we described in earlier posts. The day started off slowly. I got to the ICN at 6:45am and Ema was awake, so I picked her up and rocked with her for awhile. She was in a pretty bad mood because she hadn't had anything to eat since midnight (for Ema a bad mood just meant that she would periodically cry, which normally she hardly ever does).  Several members of the surgical team came and talked with me during the next few hours, but it wasn't until about 8:45am that they had me put her back in her bed so they could transport her to the operating room. They let me walk with her down to the OR doors, where I kissed her goodbye and another nurse took me to the surgical waiting room. Leaving her there in the hallway I wondered whether I would ever see her again.

 The procedure took several hours, and every hour a nurse would come and update us on her progress. From the very beginning they seemed to be almost surprised at how well Ema was doing. They came and told us as she was put under, had IVs added and medication started, when the surgeon began the operation, as he added the shunt and sewed all of her new connections, and as she was preparing to go off of the bypass machine that had been her heart during the surgery, they warned us that it might take several tries before her heart would be able to work by itself. However, just a short time later they came back to tell us that she had been able to come off the bypass the first time without any problems.

The nurse then took us the PICU waiting area while they finished up and got her settled into her new room. Some time later, I was able to go back and see her. Because her heart may swell and there may be a buildup of fluid at her incision site, the doctors still have not closed her incision, but have it covered with a dressing and have a tube coming out of it to drain the fluid. They are very optimistic about how it is looking, because she isn't draining very much and has had such a strong heartbeat. They are telling us she may be able to be closed up fully as early as tomorrow or Saturday. She is still on a respirator and coming off of that will be the next step after she is closed. During all of this, she will remain asleep. I think that is the scariest part, because she looks so still and death-like. Even so, I can see her heartbeat beneath the bandage on her chest and it is comforting to know that her heart is stronger than it was just this morning.

She is very pale and blue-tinged (cyanotic) and she will remain this way until her third and final surgery that will completely separate her oxygenated and non-oxygenated blood. Again, this is slightly scary to see, but we just have to remember that this is normal for babies that have been through what Ema has been through.

When I woke up this morning it felt like the waiting was over, but I guess it has really only begun. We have crossed the first step and Ema has done so well, I know that is an answer to prayer! However it feels like we have many, many steps to go before Ema is safe.

About the surgery.

Here is an explantation of the surgeries that Ema will have to have:

The first is called the Norwood procedure, and that has already been explained to us (the combination of the aorta and pulmonary artery and placement of the graft.


The second is called the Gleen procedure, also called a Bidirectional Glenn procedure or Hemi-Fontan (see also Kawashima procedure), involves redirecting oxygen-poor blood from the top of the body to the lungs. That is, the pulmonary arteries are disconnected from their existing blood supply (e.g. a shunt created during a Norwood procedure, a patent ductus arteriosus, etc.). The superior vena cava (SVC), which carries blood returning from the upper body, is disconnected from the heart and instead redirected into the pulmonary arteries. The inferior vena cava (IVC), which carries blood returning from the lower body, continues to connect to the heart.

At this point, patients are no longer in that delicate balance, and the single ventricle is doing much less work. They usually can grow adequately, and are less fragile. However, they still have marked hypoxia (because of the IVC blood that is not fed into the lungs to be oxygenated). Therefore most patients are referred for another surgery.



The third is called the Fontan procedure, also called Fontan completion, involves redirecting the blood from the IVC (inferior vena cava) to the lungs as well. At this point, the oxygen-poor blood from upper and lower body flows through the lungs without being pumped (driven only by the pressure that builds up in the veins). This corrects the hypoxia, and leaves the single ventricle responsible only for supplying blood to the body.



Some patients require long-term blood thinners. The Fontan procedure is palliative, not curative. But in many cases it can result in normal or near-normal growth, development, exercise tolerance, and good quality of life.[4] In most cases, patients will eventually require heart transplantation.[5]

Thymus and Surgery

Sorry for the delay between posts. Here's a general update on how things have been going.

First, the support continues to be not only overwhelming (in an amazingly good way), but also life changing. Hopefully I'll get an opportunity to explain more later.

Second, Becky got to spend a few days at home this week. Grahm and I had been missing her very much, and I know she was getting terribly homesick. We spent Saturday night and most of Sunday back in K.C. Grahm will spend the rest of the week in K.C. with Becky.

Third, Ema gave me the biggest smile while I was holding her this morning. Thank you for the prayers, and please keep them going this week. We will need them.

Here is the medical update from Becky:

The last couple of weeks have brought both good and bad news for Ema.

The good news is that we got Ema's immunology results back, and they showed almost normal levels of T-cells, the cells that fight infections. This means that Ema's immune system is slightly compromised due to her DiGeorge syndrome, but is not life-threatening. She will have more infections than most other kids, but other than that it will be manageable. This alone is great news for all of us, and definitely an answer to prayer.

The bad news is that the echo-cardiogram that Ema had on Monday of last week showed that the valve that connects her aorta to her heart is still on the small side for what the cardiac surgeons would like. This means that Ema will have to have the valves that connect her aorta and pulmonary veins (the veins that lead to the lungs) to her heart combined into one valve that leads only to the aorta, and an artificial valve put in place to lead to the pulmonary veins. The reason why this is not good news is that it will mean several additional surgeries in several years to replace the artificial part that won't grow with her as she grows. Doctors think she will need to have it replaced in about 1-2 years, and than again in about 8-10 years. This will, however, allow them to fix all of her heart defects and should return her heart function to normal.

During that same echo the doctors noticed that the blood flow through Ema's ductus (the special duct that is currently supplying Ema's lower body with blood) was not as good as they would like it to be. This meant that on Friday, Ema's medication that keeps the ductus open (Pros tins) was increased to open the ductus further. This medication increase makes it more likely that Ema will have breathing problems, so her team has to keep an extra careful watch on her breathing to make sure she doesn't need any assistance. It also made them temporarily hold Ema's feedings for Friday, but as of Saturday they resumed them.

Aside from the other things that have been going on, Ema has been doing very well. She has been getting regular feedings and they are beginning to have the desired effects, because Ema already weighs 6 pounds 9 ounces! This is great weight gain for the amount of food that she is allowed to have, so her team is very happy with this gain.

Ema's surgery is scheduled for sometime on either Thursday or Friday of this week. We are hoping it is on Thursday! The sooner that Ema's heart is fixed, the sooner we can take her home and introduce her to all the people who have been thinking and praying for her recovery.