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.

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.

What we found out at Children's Mercy.

As soon as we got to Ema's bedside at the Children's Mercy NICU, we were given a wealth of information about her condition. Everyday we learn a little bit more about what's affecting her. I will try to explain what is going on as best as I can so everyone can understand.

Em's Congenital Heart Defect (CHD) is a particular type known as an Interrupted Aortic Arch (IAA). The aorta is the big artery coming out of the left ventricle (one of the four chambers of the heart), and it is responsible for carrying oxygen rich blood to the body. The aorta has three branches before going down to supply the lower part of the body with blood. Those three branches are located on an arch as the aorta begins to head downward. Below is a picture of a normally formed heart (left) compared to an IAA heart (right).

One question that we had initially was why didn't anyone notice Ema's special heart sooner than Saturday night when we took her to the ER? The answer to that is is kind of complicated, at least to those who are familiar with the anatomy of an adult heart. Below is a diagram of a fetal heart.

The area that is labeled "ductus arteriosus" is present in fetal babies, but if you refer back to the previous image, it is absent in normally formed adult hearts. A few days after a baby is born, the ductus arteriosus closes and normal aorta function continues. With Ema, the ductus arteriosus is the only thing supplying blood to the lower parts of her body. As with normal babies, that ductus closed a few days after birth, which was some time Saturday evening when we got her home. Before that, her heart problems were undetectable.

Right now, Ema is on medication that keeps the ductus arteriosus open. Every other problem Ema is experiencing right now is stemming from the period of time when the ductus was closed. Each of her IVs contains medications to remedy and normalize all of her bodily chemicals and blood gas levels.

Another defect with Ema's heart is the "hole" that the cardiologists told us about in Wichita. They explained the hole to us a little bit more thoroughly in Kansas City. Again, if you look back up to the first image, you'll notice that in between the  right ventricle (RV) and left ventricle (LV), there is a wall, called the ventricular septum. That is where Ema's hole is. Her heart looks a lot like the image below.

In Ema's heart, that septum has a pretty large hole in it. Right now, this is kind of a good thing, actually. The area of blue in the diagram is blood that doesn't have oxygen in it, because it just entered the heart after passing through the body. The red area is blood that has just come back from the lungs, so it's nice and full of oxygen. Because the blood that is going to Ema's lower half is coming out of the pulmonary artery, the only way it gets oxygenated blood is when it passes through the hole in her ventricular septum. However, when the doctors do surgery, they will need to patch this up.

Doctors are keeping her healthy and allowing her to recover until she is strong enough for surgery. At the same time, doctors are also trying to screen to figure out exactly what caused the heart defect. We should have a pretty good idea this Friday what we can expect of the next several weeks. We do know that Ema will be in Kansas City for at least 4-6 weeks.

I should leave you with some happier news. When we left the hospital last night, Ema's eyes were open ans she was wiggling around quite a bit. She grabbed my finger and held it for several minutes. This was the first time we had seen our little girl's eyes since Saturday, so it was very nice for us.

Flying to Kansas City.

It took what seemed like hours before we were able to see Ema in the NICU at Wesley. By this point, she had four IVs; one in each arm, one in her belly button, and one in the top of her head. She was hooked up to a ventilator through a tube in her mouth. She was stable, and they were able to conduct a few tests that they couldn't do in Newton.

A cardiologist and neonatologist (a doctor specializing in ill and premature newborn infants) met with Becky and I to tell us what they had found. The explanation was that Ema was born with a congenital heart defect. This is basically a catch-all term form abnormalities in the heart that are present from birth. The cardiologist tried to explain the exact nature of the deformity. Based on the test they had done (which I believe was an echocardiogram) they believed that the problem was with Ema's aorta. A chest x-ray in Newton revealed that Ema's heart was enlarged. That information, with the results from the cardiac echo, led the cardiologist to believe that Ema's aorta was severely narrowed at some point after it left the heart. We later found out, after a more precise and accurate test, that this was not exactly the case. The cardiac echo also found what the cardiologist described as small holes (approximately three of them) in Ema's heart. This also sounds pretty frightening. Essentially, what this means is that passageways between the different chambers of the heart are not tightly sealed, which allows for some level back flow and blood mixing. Though this is clearly a concern, it is not as concerning as the problems with the aorta, which, at this point, hasn't been completely identified.

What we did learn from this meeting, was that the best cardiac surgeons are in Kansas City at Children's Mercy Hospital. As soon as Wesley could get Ema stable, they were having her flown to Kansas City. We left Wichita shortly after 11:30am and prepared to head to Kansas City.

Just so everyone can appreciate what we had been through to this point, here's a brief timeline/recap:

• 7:30pm Saturday - Ema comes home from Newton Medical Center
• 12:30am Sunday - We take Ema to NMC ER
• 4:30am Sunday - Ema leaves NMC via ambulance for Wesley Medical Center
• 11:30am Sunday - Ema is flown from Wichita to Kansas City Children's Mercy Hospital
• 11:30am Sunday - Becky, Donna, and I leave Wichita to pack some clothes for KC. 
• 4:30pm Sunday - We arrive in Kansas City and see Ema at Children's Mercy. 

By this point, we had not gotten a chance to shower or sleep. Just so you all can appreciate the full picture.

Something's not right.

After Ema was born, we spent two normal, sleepless days in the hospital. Ema got to meet several family members and new friends, including a skeptical older brother, and members of a very prestigious high school debate team. Everyone immediately noticed what mommy and daddy knew all along, that this was a special beautiful baby girl.

Finally, on Saturday evening, we were ready to take our baby girl home and start our new life as a family of four. We got home around 7:30pm. Almost right away, we noticed something was not quite right. Ema had been very fussy, and she was not wanting to eat. You might be thinking that a fussy newborn is perfectly normal. I don't mean to boast, but our babies are wonderful. They don't really get fussy (this can be verified by any number of family members). After a few hours of trying to get Ema to eat, we also noticed that she was cold. Becky took her temperature and decided to call the hospital. Like a good husband, I was asleep.

Shortly after midnight, we were in the ER at Newton Medical Center. Dr. Jantz was informed about Ema's condition and got to the hospital before we did. As Ema was getting checked, Dr. Jantz and the ER nurse heard a murmur. This basically means they heard something that did not sound like a normal heart beating. Dr. Jantz quickly had us walking to the special care nursery. He had an assumption about what was going on, and he acted on it (assumption is my word. Dr. Jantz is a skilled professional and probably knew exactly what was going on). Within a very short amount of time, Ema was hooked up to an IV that was administering a drug. This drug was opening up Ema's aorta, which is the large artery supplying the body with oxygenated blood. I am convinced that this was the second action of the night that saved Ema's life. For those who weren't counting, the first was when Becky realized something wasn't right and called the hospital.

After a few tests in the special care nursery and other life saving medications, the neonatal transportation unit from Wesley Medical Center was contacted and heading to Newton. By 5:30am we were sitting in the waiting room of the NICU at Wesley Medical Center. This was just the start of a very long day.

A difficult pregnancy.

Before I can go into detail about Ema's current condition, it's necessary to give some background information. After Becky and I found out we were pregnant, the first thing we prayed for was a pregnancy free of complications, because we had already gone through a difficult pregnancy and birth with our son, Grahm. Several months went by with what we thought were answered prayers. However, by around week 24, Becky had a sonogram that revealed she again had polyhydramnios (too much amniotic fluid in the uterus, where baby lives before birth). Because our first pregnancy involved poly and resulted in a premature birth, the doctors working with were taking every precaution to make sure this baby would go full term. One of the interventions they decided was to have weekly biophysical profiles, which involved a weekly sonogram, and a monthly visit to a prenatal specialist in Wichita.

Even with this intervention, we still had a few hiccups along the way. Becky was admitted to the hospital at 29 weeks because of contractions. This happened at least two more times before week 37. Nobody wanted to risk Ema coming early, since her brother spent two weeks in the NICU (Neonatal Intensive Care Unit) at Wesley.

As weeks 37 and 38 passed by, it looked like were finally going to enjoy a stress free, and full term, birth. Again, this was not to be the case.

Becky's Cesarian Section was scheduled for Friday, November 11th (11/11/11) a day we picked out back in August. On her last weekly biophysical profile before the C-section, we got some less than great news. Ema seemed to be doing fine except she wasn't moving. Because he heart rate and breathing motions were normal, the doctors weren't immediately concerned, but they wanted us to come back early Thursday morning so they could check again. After all, babies like to sleep, so maybe Ema was just sleeping.

We arrived at Newton Medical Center at 6:30am for Becky's sonogram. By 7:45am, doctors had explained to us that while Ema was moving around this time, she was not showing any breathing movements. This sounds pretty scary, like she was suffocating, but remember that babies don't really breath in the womb. Breathing movements really just show that she's prepared to take her first breaths when she enters the world. Still, this was a problem because those movements should have been there.

The doctors decided that Ema was going to be a day early.

As Becky got prepared for surgery, I quickly, and not so calmly, informed our family. Without going through all of the details, Emaline Belle Buchta was born at 9:49am on Thursday, November 10th. She weighed 5 pounds 14.9 ounces, and was 18.5 inches long. She was healthy and everything was just fine. It was a welcome relief for us. After our experience with Grahm's birth, it was nice to be able to hold our baby girl right away, and to have her in our room free of tubes and machines. Everything was normal, and we were happy.