Backstory & Diagnosis

I'm pregnant!
It was January 25, 2010 and it was before 5 am. I was getting ready for work, and James was still fast asleep. But I had to have him take a look. I woke up to show him, to see if he saw what I did. Am I imagining that second pink line, or is it really there? His eyes confirmed what mine had been hoping for see for a few months now. We didn't know he was Ewan yet, but that's the day we knew he was with us. I took a second test. In the coming days, I took a third, a fourth, and a fifth.

we are pregnant
January 25, 2010

There was no doubt in our minds: we were having our first baby.

My pregnancy progressed has many do: with nausea and vomiting and semi-regular profile shots of my thickening belly. We discussed names, the boy names (for some reason) coming with particular difficulty. Quite literally, Ewan was the only boy's name discussed that we both really liked. If our baby was a boy, he would be Ewan Eliezer.

First ultrasound
I was scheduled for an ultrasound at 20 weeks and a day on May 19, 2010. It was a routine scan, and our desire was chiefly to discover if we could expect a boy or a girl. Ewan was not shy and showed us definitely, even to our unexpert eyes, that he was all boy.

But we also received some less delightful news. Some abnormalities were showing up with his heart. What we thought would be a 45-minute appointment turned into three hours. They talked to us about congenital heart defects, about Down's syndrome, and about amniocentesis. I was scanned and re-scanned, and I had my blood drawn. The doctor talked to us about abortion. Without getting political about it, this has been and always will be something that is unequivocally not an option for us. Period. We let the doctor know this in no uncertain terms.

We went home heartbroken. This is one of those things that typically lands squarely on the list of Horrible Things That Happen to Other People -- only now we were the other people. We cried for days. When we called family and friends who were delightedly anticipating our news (Boy? Girl?), we had to reveal the other news we received: We're having a boy, and his heart is badly broken. In the days that followed, the shock eased and hearing repeated stories of misdiagnosis via ultrasound, we learned to hope.

I was referred for additional testing with a pediatric cardiologist, and on June 1, 2010 the diagnosis was confirmed. Our heartbreak was renewed. The information we received was far more specific in terms of what he saw, and what the treatment options were. He was frank with us: This is severe, he said. He mentioned abortion too, and we affirmed again without hesitation: that is not an option.

The diagnosis
Ewan was diagnosed with a congenital heart defect called Tetralogy of Fallot, which consists of a combination of four defects in the heart. He also had Pulmonary Atresia, a condition in which the pulmonary valve is sealed or not present. It is estimated that Tetralogy of Fallot occurs in as many as 5 out of every 10,000 births.

heart: side by side

After we received the diagnosis, we heard a lot of, "I know so-and-so who was born with Tetralogy of Fallot, and s/he is fine now. " But it's important to note that there are several forms of Tetralogy of Fallot, and within those forms there is a broad range of severity. Think of it this way: if someone said to you, "I'm bleeding," it could mean they had a paper cut, or it could mean they have a gunshot wound. That's the kind of range of severity we're talking about. Ewan was diagnosed with the most severe form, which was further complicated by the Pulmonary Atresia. Tetralogy of Fallot typically includes a combination of four defects in the heart:
Ventricular-Septal Defect: This means there is a large hole between the right and left ventricles of the heart. This creates a situation where oxygen-poor blood is mixing with the oxygen rich blood.

Pulmonary Stenosis: A narrowing of the pulmonary valve. The pulmonary artery's function is to carry blood away from the heart and to the lungs to be oxygenated. In a normal heart, the aorta and pulmonary artery are the same size. In Ewan's heart, they had difficulty even finding the pulmonary artery on the first ultrasound. The fetal echo at 22 weeks showed a very small pulmonary artery.

Right Ventricular Hypertrophy: A thickened right ventricle. The right ventricle wall is thickened because it has to work extra hard to get blood through the pulmonary artery.

Overriding Aorta: In a normal heart, the aorta leaves the heart from the left ventricle. With an overriding aorta, the aorta is leaving the heart from right over the ventricular septal defect.

This, of course, changed many of our plans in terms of how and where Ewan would be born (we had been receiving our prenatal care from midwives and had planned on delivering at a birth center), and our idea of what it would be like after he was born.

Why and how does this happen?
The short answer is this: nobody really knows. It took some considerable time after the diagnosis before I was ready to engage in any kind of research, and even then what I found was not particularly enlightening. A percentage of congenital heart defects may be genetic, but this still does only a little to explain how it happens. According to Lucile Packard Children's Hospital at Stanford, 5-6% of CHDs may be related to a chromosomal abnormality, 3-5% can be explained by a single gene defect, and about 2% are due to environmental factors. Some CHDs are a combination of these factors.

That leaves 85-90% of CHDs with no known or identifiable cause.

When I reviewed the risk factors for mothers that increase the risk of having a baby with a Congenital Heart Defect (CHD), I had precisely none of them. I exercise regularly, have a very healthy diet, and am not related to anyone who has a defect like this. I was taking all the right supplements well ahead of my pregnancy, not drinking excessively (or even regularly), not having certain medical conditions or taking certain medications ... you name it. This is both comforting and aggravating.

It really can happen to anyone.

When the doctor at Seattle Children's Hospital spoke with us, he said it as honestly and truthfully as he could: We don't really know why this happens. It just happens.

Ewan’s Life
Read the birth story.

Ewan was born at 9:49 am on Saturday, September 18, 2010 after 17 hours of unmedicated labor. He was perfectly beautiful, and it was difficult to comprehend how anything could be wrong with him. After spending about a minute with him, he was taken away to the NICU so he could receive the support and medicines he needed. Later that same day, he was transferred to the Neonatal Intensive Care Unit at Seattle Children’s Hospital.

1 day old

Our days in the intensive care unit are hard to summarize. There were ups and downs, victories and setbacks. I didn’t get to hold him that much – once when he was just a day old and intubated, with more tubes and wires coming out of him than I could count, and once more when he was four days old. That time, he was alert and aware and looking into my eyes. I didn’t want to let him go, ever.

4 days old

When he was five days old, he was sent to the catheter lab (more commonly referred to as the "cath lab") for a routine procedure. The goal of the procedure in the cath lab was to get the most accurate possible pre-surgical map of his heart so surgeons could develop a plan for his treatment. While in the cath lab, his oxygen saturation levels deteriorated to a level that required emergency surgery. They quoted us a 30% chance of death.

That was at 5 pm on September 23, 2010.

That night was one of the longest of our lives. As surgeons came out to the waiting room to update us, their faces got increasingly long. The first shunt didn’t work. The second didn’t work. If the third didn’t work, there was no backup. Somewhere around 3 am, they told us things were looking better. And so Ewan came out on ECMO (a machine that oxygenates blood for a patient so that the heart can rest), but alive.

First time seeing him after surgery

The days that followed meant more procedures, trials off of ECMO, and more waiting by the bedside. More talking to doctors and asking pointed questions. James wrote down everything they said, asked the questions I couldn’t think of. I learned just to be focused on being Ewan’s mama. I treasured every moment by his bedside.

First time seeing his eyes open after surgery

On Saturday, October 2, Ewan appeared to be doing really well. He was awake and alert, and focusing on me with those gorgeous blue eyes as he always did when awake. He moved his mouth as if to form words. I stayed by his bed, talking to him, singing to him, adoring him. I had so much hope, even with all the support his body was receiving.

The next day, everything had changed. He had not opened his eyes all day. He was not neurologically responsive. Tickling his feet had always yielded a dramatic response before and on this day, not even a twitch. We learned that he had been suffering from a blood infection. He was on a broad spectrum antibiotic, but clearing the infection would be difficult with the ECMO circuit (the infection tends to stick to the plastic tubing). Oxygen saturations were up, but not perfusing. And his intestines had started to fail.

So there he was: heart and lungs supported with ECMO, kidneys supported with a dialysis unit, fighting an infection, intestines failing, liver failing, kidneys failing, and quite likely had some severe and irreversible brain damage. He was receiving maximum oxygen support, but it wasn't perfusing, causing his brain and other organs to be starved of the oxygen they needed to function. The most optimistic doctor we had stood by his bedside and told us: We don’t see kids come back from this. The only hope we have is not a medical hope.

And so we made the heartbreaking decision to disconnect support. We could have chosen to let the infection take its course and allow his organs to fail completely and wait for that dreaded phone call in the middle of the night, or we could say goodbye on our own terms. We could surround him with the people that loved him and in a peaceful setting, send him home. And that is what we chose to do.

One of our final moments with Ewan
So surrounded by friends and family, bathed in peace and prayer and love, we sent Ewan home to Jesus just after midnight on October 4, 2010.

Our hearts will never be the same.
Read about Ewan's last night with us.

ewan's marker

Ewan Eliezer Petermann
September 18 - October 4, 2010

What now?
You're welcome to follow this journey as we navigate our way through remembrance and grief. I'm not one for hiding anything, so I promise to be raw and real with you about what I see, how we feel, and how we're coping (or not) with all of it. I don't know how it happens, but I am a firm believer that there is a two-fold power in telling our stories: In the telling, we are healed and in the hearing, someone else finds that they are not alone. And to the person who needs to know that, it really does matter.

Peace be with you.