The VSD

As October moved along, Nathan's health continued to make good, but small, improvements. Unfortunately, because of a heart condition known as Ventricular Septal Defect (VSD), Nathan was really unable to make any remarkable progress.

A VSD is a hole in the wall of the heart (the septum) that separates its left and right sides and occurs between the two lower chambers (ventricles).

In a normally functioning heart, the left side of the heart only pumps blood to the body, and the heart’s right side only pumps blood to the lungs. When a VSD exists, a large amount of oxygen-rich blood from the heart’s left side is forced through the defect into the right side. This blood is pumped back to the lungs, even though it has already been refreshed with oxygen (see diagram above).

Unfortunately, this causes the heart to pump more blood. The heart, especially the left atrium and left ventricle, will begin to enlarge from the added work. High blood pressure may occur in the lungs’ blood vessels because more blood is there. This increased pressure is known as pulmonary hypertension. Over time, increased pulmonary hypertension may permanently damage the blood vessel walls.

In order to have the heart function properly and reduce the pulmonary hypertension, the VSD needs to be closed. VSD closure is usually performed by sewing a patch over the hole to close it completely. Eventually the normal heart lining tissue grows to cover the patch and it becomes a permanent part of the heart. This option requires open-heart surgery.

A second option for closing the VSD involves "plugging" the hole with a special device through a procedure in the catheterization lab. The procedure is less invasive than open heart surgery, but had never really been tried on someone as tiny as Nathan before. So there were inherent risks involved with this as well.

Talk about being stuck between a rock and a hard place and a fire breathing dragon!

If we did nothing, Nathan's heart would sooner or later over work itself and eventually stop.

Or we could subject him to the rigors and risks of open heart surgery.

Or we could try a new procedure that has a small proven success rate, but absolutely zero results (good or bad) for someone Nathan's size.

So we were again left with an extremely important decision to make regarding Nathan's life.

Snow Cone Belly

As we mentioned in the previous post, when Dr. Scaife performed Nathan's hernia repair, he discovered that all of Nathan's insides that he had taken out of his chest (liver, stomach, intestines) didn't fit into his abdomen. Since they had never been in there in the first place, his abdomen hadn't grown to fit them.

As a result, Dr. Scaife temporarily housed a portion of Nathan's "guts" in a surgical silo, a polypropylene mesh covered on both sides with a sterile transparent adhesive film that is constructed right there in the operating room. The silo is sterile, soft, flexible, sturdy, internally smooth, provides a reasonable barrier, and allows visibility of the bowel. The silo is inserted to an open wound in the patient's abdomen and "reduced" as the patient tolerates. The patient is also given a large number of antibiotics to prevent infection since the silo is in an open wound.

However, because Nathan was still on ECMO, the silo posed a another potentially dangerous problem in addition to possible infection:

Patients on ECMO are given blood thinners to help prevent the ECMO machine from clotting. However, the blood thinners also prevented Nathan's wound from clotting, meaning that it was constantly bleeding. A a result, Nathan's silo was heavily bandaged with gauze. The gauze was replaced as needed as it became saturated with blood. He also received regular blood transfusions to replace the blood he was losing.

But with all the gauze, there were times that Nathan's tummy looked like a giant snow cone. Hence, we called him our Snow Cone Belly Baby while we waited for the day he would come off the ECMO.

**For those brave souls, you can view pictures of the uncovered silo, by clicking here. But I caution you, they are NOT for the faint of heart or stomach!

He Just Looked So Perfect

Around 4am, one of the nurses brought in a wheelchair so that I could transport Bekah to the NICU.

As we entered the room, it seemed very small and crowded. There were a couple of other babies, a few nurses, a couple of doctors, and the Life Flight team already in there. We just wanted to see our baby, but it felt like we were intruding or in the way. We found an open space and positioned ourselves as close to Nathan as we could without being in the way. We were right across from him, maybe 5 or 6 feet away.

Nathan just lay there motionless, the ventilator doing his breathing for him. But I couldn't help but notice as we looked across at him, that he just looked so perfect. Inside, his body was so messed up; but outside, he was beautiful. Looking at him, you would never know that he was so sick and clinging to life.

We weren't there very long before the Life Flight team was ready to load him up and transport him across the way to Primary Children's Medical Center. They wheeled in what we affectionately termed "The Space Shuttle" to transport Nathan.

Unexpectedly, the Life Flight team leader asked me if I was LDS. I replied that I was. She then asked if I would like to give my son a blessing before they took him away. Even though I wasn't expecting it, I eagerly accepted the opportunity and moved over next to my son. He was even more perfect up close!

I gently laid my hands on his little head and prayed Heavenly Father would watch over him and that angels would be with him.

After that, they gently loaded him into the Space Shuttle and invited me to accompany them across the way.

Compassionate Care and Compassionate Prayer

If life were a movie, after our busy day at the hospital meeting doctors and getting the MRI, it would soon be time to have the baby. But real life isn't like that. We still had at least 6 weeks before Nathan was likely to be born. Or at least we hoped that he would wait that long! The longer he waited to arrive, the better it would be for him. That was the good part of having so much time.

Ironically, the bad part was having so much time. The meeting with Dr. Yoder weighed heavily on my mind over the next few days, especially the part where he discussed "compassionate care."

Since the day we had decided to move forward believing that our son would survive this whole ordeal, it was somewhat of an unspoken rule around the house that you don't bring up the "What ifs," even though I know we both thought about them, probably more than we wanted to or would admit to at the time, as if that would be showing a lack of faith in God and His healing power.

Relatively soon our son would be born. This was closer and closer to reality. How long would he live? Minutes? Hours? Days? Would there be time for me to hold him? Or would his mom be the only one to experience that? I couldn't take that away from her. Would he know I was his dad? Would he know how much I loved him?

If he doesn't make it, how do you arrange for a funeral? Where would we bury him? How do you deal with all that?

All the questions I had avoided seriously asking myself and many more now flooded my head.

What do you do? I did the only thing I could do --- I prayed.

But what do you say?

I had prayed for Nathan countless times in the last two months. But now it was somehow different. I don't know how to explain it very well, but it was just different now.

Before, even though Nathan was sick and we had known all along that there existed the possibility that he might not make it, that possibility was now more of a blatant reality. The abstract and intangible was now way more real and tangible.

How do you plead to you Heavenly Father for your child's life? What do you say? Do you foolishly think you can barter for his life? Does your child "deserve" to live more than any other child? Are you more privileged or special than someone who has lost a child that you think God "owes you?"

I didn't know what to say to Him. But I knew He understood my heart. I knew He understood how much I loved my son. I knew how much He loved His Son too. So I couldn't say to Him that Nathan deserved to live, but I could say that if he did, Bekah and I would teach him to be a good and righteous man, to love God and his fellow man, and to serve them both. That was all I had to offer. And if in His infinite and perfect mercy and wisdom that meant that Nathan got to live, then we would do our best to keep that promise. But if not, then I prayed His mercy would be enough to keep my heart soft and curb any bitterness or resentment that I might have if He decided that He needed Nathan on the other side of the veil more than we He needed him here with us.

What is a Congenital Diaphragmatic Hernia?

The wide, flat muscle that separates the chest and abdominal cavities is called the diaphragm. The diaphragm forms when a fetus is at 8 weeks’ gestation. When it does not form completely, a defect, called a congenital diaphragmatic hernia (CDH), is created. This is a hole in the muscle between the chest and the abdomen.

The majority of CDHs occur on the left side. The hole allows the contents of the abdomen (stomach, intestine, liver, spleen, and kidneys) to go up into the fetal chest. The herniation of these abdominal organs into the chest occupies that space and prevents the lungs from growing to normal size. The growth of both lungs can be affected. This is called pulmonary hypoplasia.

While in the uterus, a fetus does not need its lungs to breathe, because the placenta performs this function. However, if the lungs are too small after the baby is born, the baby will not be able to provide itself with enough oxygen to survive.

The depiction above is of a SEVERE CDH.

Meeting with the Specialist

The doctor’s office helped us set up an appointment with a perinatologist, a maternal-fetal medicine specialist that deals specifically with the care of the mother and fetus at higher-than-normal risk for complications.

The appointment with the perinatologist was scheduled for the following week. It was a loooong week--full of anxiety, fear, and the emptiness of the unknown.

The following Wednesday (May 9, 2007) we met with Dr. Melissa Herbst, a perinatologist from St. Marks Hospital in Salt Lake City at Timpanogos Regional Hospital in Orem, Utah. The appointment consisted of an ultrasound – a really, really long ultrasound! Dr. Herbst spent a considerable amount of time with the sonogram, viewing as much of our baby’s anatomy as she could.

As she looked at the various sonograms, she informed us that about 1 in every 2,500 babies in the United States is diagnosed with CDH. The severity of CDH varies depending on how much of the contents of the abdomen have migrated into the chest cavity.

Her verdict on our baby? SEVERE.

His stomach, intestines, and part of his liver were in his chest. Because of the severity of the case, Dr. Herbst elected to refer us to one of her colleagues, Dr. Robert Ball, a leading specialist in maternal-fetal medicine and Fetal Therapy Initiatives.

THE BEGINNING....When we found out our baby was sick.

We’ve been debating how best to introduce our blog. We feel that it’s important for everyone tounderstand the past, so that you can better understand the present.

The dilemma that we come across, though, is how much of the past do we explain. Well, we think we’ve come up with something that will work, so here goes.

We figure the best place to start is the day that we found out that Nathan was going to be sick:

On May 1, 2007, Bekah was scheduled for her monthly OB visit and the 20-week ultrasound. As I’m sure many of you can attest to, when it’s your first child, you mostly just want to know if you’re having a boy or a girl. That’s the BIG DEAL of the ultrasound…or so we thought!

After learning that our life would be blessed with a little boy, the sonographer (in the kindest way she knew how) informed us that according to what she was seeing on the ultrasound, some of the baby's organs were appearing to be out of place.

What does that mean? How do you respond to that? Why do I suddenly feel empty?

The sonographer then informed us that the doctor would explain more to us in just a few minutes. As we were being escorted from the ultrasound room to the doctor’s office, the sonographer quietly, but sincerely, said “I’m sorry.”

We sat anxiously, nervously, and emotionally in the doctor’s office, holding each others’ hands for support. The few minutes we waited seemed like forever until the doctor finally came in.

So what’s going on? How sick is our baby? What do we do now? And a thousand other thoughts run through your head. You want to ask them all, but you don’t know where to start. You want to focus on every word the doctor is going to say, but your head is so full and swimming that it’s hard to focus on anything.

The doctor informed us that our son had what is called a Congenital Diaphragmatic Hernia (CDH). However, due to the restrictions of their ultrasound equipment, they were unable to determine the severity of the hernia. The severity of the CDH would go a long way in determining the odds of our son’s surviving at birth.

WHACK! Punch in the head. Kick in the stomach. A little of both.

In less than 30 minutes, the joy of finding out that we were going to have a son was dashed with the news that our little boy was going to be sick. Just how sick, we didn’t know yet. But when we woke up that morning the farthest thing from either of our minds was our son’s mortality. And now here it was trying to beat down our door. And there was no where to hide.

So what happens next?