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.

Some Pictures

Before Ema was released from Newton Medical Center (and before we knew anything was wrong), a professional photographer took some pictures of Ema. I wanted to share some of them with you. Enjoy!

November 30

We are happy to report that Ema is making a lot of progress. She is still free of breathing aids. She has been waking frequently, and is very alert when she is awake. She has started taking breast milk, though she is breathing too quickly to bottle feed. Instead, she has a feeding tube through her nose (which she has already taken out by herself at least once). I had to leave Kansas City and head back to work, leaving Becky there by herself. The following is Becky's medical report for Ema as of Tuesday, November 29th:

The next two weeks will be very important for Ema's future:

Ema had her first immunology consult today, 11/29/11. Blood was drawn so that her lymphocyte numbers can be tested and evaluated. Earlier imaging tests showed that Ema has a small or missing thymus, which is a gland that produces T-cells, an important immune system cell. This imaging test suggests that Ema will very likely have low levels of immune cells, and thus a compromised immune system. The blood test that is now being done will determine how severe Ema's immune deficiency is; She will either have a "partial DiGeorge", where some thymus tissue is left and some T-cells are produced, or she will have "complete DiGeorge" where she has virtually no thymus and no T-cells. Whichever she has will mean a great deal for her future; Partial DiGeorge will leave her with more infections and colds and things, and a need for regular trips to an immunologist for follow-up, but is not usually life-threatening. Complete DiGeorge can be very serious, because her ability to fight infection will be so compromised. We will know by the end of the week which she has, and will determine then a plan of care for her future.

Ema also saw her cardiologist today. So far she is doing well, gaining weight and remaining stable. Another echocardiogram is scheduled for next week, and depending on those results surgery will likely be the week after. They will be looking for how big her aorta is in relation to the rest of her body, to determine whether it will be able to sustain blood flow under the new pressure. If is determined to be too small, than she will likely go into heart failure after surgery, which would likely mean being on constant IV medication to maintain balances.

Ema also saw a geneticist today. The genetic counselor explained that people with DiGeorge vary widely on the particular symptoms that they present with. Some individuals never know that they have the deletion, because they never show symptoms. Some people have heart problems, like Ema, and nothing else. And some individuals have a variety of mix- and - match symptoms. We really won't know until later how severe all of her symptoms are, as some of them don't present themselves until later, like any possible learning disabilities. They also suggested that Ema receive care from the early education program once she is home in Newton, so that Ema's parents have another resource to use to measure and evaulate Ema's development for signs of problems, and also to connect them with any therapies she may need that they provide, like speech therapy, occupational therapy, or physical therapy. They also suggested that Ema's parents be tested to see if they have the deletion, so that they can make decision about other children. Ema will have regular visits with a genetic counselor in the future.

Ema also saw an endocrinologist for the first time today. They plan on running bloodwork for her to determine the function of her endocrine system, the system that deals with her hormones. Doctors already know that several of Ema's levels are a little abnormal, such as her calcium level, which is low. The bloodtests will help determine if any of her other hormones are abnormal, so that they can begin to treat the deficiencies and plan for the future. Because of her calcium levels, we already know that she will need calcium supplements once she is no longer receiving IV nutrition.

Ema still is getting feedings every three hours, and has even slowed her breathing enough to receive one bottle feeding! She has begun to be used to her feeding routine, and is waking up hungry a few minutes before her scheduled feedings. This is a good sign that she is processing her food and that she is developing an independent feeding routine, something that most babies develop normally.

She also loves her binky, and the new mobile that her parents got her!

General update.

Ema has been making a lot of progress in the past few days. She is not hooked up to any breathing aids. She is down to two IVs. We've been able to hold her. She is even going to try bottle feeding today.

Ema has also undergone several tests. She has had a kidney ultrasound, a CT scan of her heart, two head ultrasounds, at least two echo-cardiograms, and blood tests everyday.

Doctors found a small bleed on her brain. That was the only serious damage from the lack of blood flow when we brought her home from the hospital initially. Additional scans found that her body's natural processes are beginning to clear that up. They don't anticipate any lasting damage from this bleed.

Because Ema is so small, the cardiologists and surgeons have decided to wait a month before doing surgery. They also want to wait until the bleed on her brain is completely healed. There is also an issue with the size of her aorta. They would like it to get a bit bigger so they can be sure it will handle the pressure of the new blood flow.

Thank you for all of the prayers!

DGS

The three letters that are the title of this post have terrified me since Sunday evening. But before I talk about that, I want to talk about something that every parent already knows.

Parents worry about their children. We worry that our kids get enough to eat. We worry that our kids are healthy. We worry that our kids are safe from everything bad in the world. As a relatively new parent, I can tell you that I never knew just how scary the world was until the moment I became a parent. But all of these are things I can protect my children from.

DGS stands for DiGeorge Syndrome. It is a type of Velo-Cardio-Facial Syndrome (VCFS). It is a genetic disorder classified by a deletion mutation of genetic material on the 22nd chromosome. Approximately 5% of cases of DGS are inhereited, the other 95% are spontaneous. DGS is associated with a long list of symptoms that include interrupted aortic arch (the heart condition Ema has), immune system deficiency, cleft palate and/or lip, developmental delays and disabilities, certain facial characteristics like wide-set eyes and low-set ears, and a few others. Not every person with DGS will have every symptom. The severity of these symptoms varies from person to person as well.

This is what we could not protect Ema from. On Friday, we found out that Ema tested positive for DiGeorge Syndrome. We are hopeful that hers is a mild case, but ultimately it doesn't matter. I got to hold Ema Friday night at around 11pm. It was the first time I had held her since last Saturday. Ema is perfect. That's all we need to know for right now.

A few pictures.

I know people are probably anxious to see what Ema looks like. These pictures are in chronological order.