Ziebert, Avery Mae

[left:3256a17581]http://www.cherubs-cdh.org/Album/new/ziebert-avery6.jpg[/left:3256a17581]It was the first weekend in July, 2005 when I found out that I was expecting a baby. My very first one! I knew right away that I was having a little girl, although I had to wait until my 19 week ultrasound to confirm it. I remember my 13 week ultrasound. It was the first time that I saw a picture of my baby. I was so happy that I cried. I couldn’t believe that there was really someone in there!

I felt great throughout my pregnancy, so I didn’t expect any problems. I was tired, of course, but I was working 2 jobs and taking four classes at the University. I didn’t have any morning sickness or any other complaints at all. I did everything by the books, staying away from caffeine and alcohol. Nobody really ever thinks that there will be problems. That’s why on October 19th 2005, when I was 19 +3 along, I was ecstatic to be going in to find out the sex of my baby. The technician confirmed what I had known all along. I was having a little girl!

She was taking a long time looking at things, but I assumed that it was just because she was a friend of a friend, and wanted to give us lots of good pictures. Even when she left the room and brought the doctor in, I wasn’t worried. It wasn’t until they told me that the baby’s stomach looked ‘a little higher up than usual’ that I thought something might be wrong. Still, it didn’t sound that bad, but when I saw the look on my mom’s face, I started to cry. I was confused. What did this mean? Instead of the three days that it normally takes to get back ultrasound results, I was told to go home from the hospital and make an immediate appointment with my OB for the next day.

The next day my OB explained what the problem was. My little girl had what was called a Congenital Diaphragmatic Hernia. Because her diaphragm (the muscle under her ribs) had failed to close properly, all of her abdominal contents such as her stomach, bowel and liver, had migrated up into her chest. This in turn, pushed her heart to the right side of her body, and the biggest problem was that it had prevented her lungs from developing properly.

I was immediately referred to a Maternal-Fetal Specialist, as my pregnancy was now considered high risk. We live in Canada, but discussed going to Philadelphia to have fetal-surgery done. This would mean that Avery would have been operated on while she was still inside of me. Ultimately, we decided against this option as the surgery is still in the experimental stages and has only a 50/50 success rate. Babies born with CDH have a survival rate of 60% right now, so our chances of saving Avery seemed better if we waited until after she was born.

Windsor is equipped with a NICU, but not a large enough one to handle the severity of Avery’s defect. I had to travel to London for specialist appointments before Avery was born. Here, I had an MRI done. This was supposed to tell me Avery’s lung to head ratio (LHR) which should have given a better indication of her chances. A LHR of 1.4 or better is considered pretty good. A LHR of under 1 is basically considered hopeless. From the MRI that I had done, two different doctors got two extremely different results. One told me that Avery’s LHR was 0.6 and one told me that it was 1.5! These numbers were completely different, and so I was back to waiting again.

I decided to deliver Avery in Toronto, because they offered a machine called Extracorporeal Membrane Oxygenation or ECMO. ECMO is a heart-lung bypass machine that will not cure a baby’s lungs, but it will allow them to rest for a time if they need to. Apparently, ECMO is rarely used in Canada for babies born with CDH but it is frequently used in the States. I wanted it available to Avery in case she needed it. I prayed that she wouldn’t. But just in case, I wanted every resource available to my daughter. I anticipated an approximate 3 month hospital stay and then I would take my daughter home with me. I anticipated long-term problems which would correct themselves by the time Avery turned eight. I did not anticipate the death of my newborn.

I felt so helpless throughout my pregnancy because there was nothing that could be done until after Avery was born. When she was born, she would have surgery to repair the hole in her diaphragm and move all of her internal organs out of her chest and back into her abdomen. Then, hopefully, Avery’s lungs would begin to grow. She was happy and active while she was in my belly. She was always kicking and hiccupping and I would spend hours watching my stomach move around like waves. Avery didn’t need to use her lungs in there. She should have stayed inside of me until she was 18! At 37 weeks, in the middle of February 2006, I packed my bags and left home for Toronto. I was due on March 12, 2006, but like many first babies, Avery took her time. I believe that she knew that she was safe inside of me and that she didn’t want to come out and face the cruel world that we live in.

On Friday, March 17th, 2006, I went into labour at Mount Sinai Hospital. My labour was quick and excruciatingly painful. I didn’t have an epidural until I was already at 10cm. Luckily, it kicked in when it was time to push. Avery was transverse (sideways) and her heart rate began rising to high levels, so she needed to be delivered with forceps and I had a double episiotomy. When she came, though, at 10:17pm weighing 6 pounds and 14 ounces, it was absolutely incredible. My heart swelled with pride and joy. Avery arched her back, clenched her tiny fists and opened her mouth wide trying to scream. No noise came out. Avery had too little lung function. I never did get to hear my baby cry.

Avery was held up for me for a millisecond and then whisked away by the resuscitation team. I cried. I can’t believe the emotions that I was filled with. I was so full of love and happiness and hope for my daughter. I knew that she was sick, but I wasn’t thinking of that then. All I could think about was how incredible it felt to have given birth. Everyone important to me was there, and I wouldn’t have had it any other way. I’ll never forget the pain, physical and emotional. But Avery was worth every second of it.

Since I hadn’t been able to see Avery right away, the staff took pictures while they were working on Avery and brought them in for me to look at. The very first thing that I noticed about Avery was her lips. Everyone in my family has those lips, and my daughter was born with them, too! I haven’t always liked my lips. I will love them forever now. It is amazing that someone can grow inside of you and come out looking just like you!

I was wheeled in to see Avery approximately 2 hours after she was born, when she had been stabilized enough to transfer. She was so much tinier than I thought when I had looked at her pictures! I got to spend about 5 minutes with her and take some pictures before she was taken through an underground tunnel to the Critical Care Unit at the Hospital for Sick Children. That night, I didn’t sleep much. I was worried about my baby, all alone, but I had to trust that she was with good people who could care for her.

In the morning, I was wheeled through the tunnel in a wheelchair to see Avery for the first time. I remember when I was pregnant and I had a tour of the Critical Care Unit. This was not the same. This time it was my baby hooked up to all of the machines. She was on the oscillator, a high-frequency ventilator to help her breathe. She had tubes and lines everywhere, but she was so beautiful! She had the longest eyelashes! The first couple of days of Avery's life, she seemed to be doing well. Everything that had to happen before we talked surgery seemed to happen at lightning speed. Avery's medications that needed to be were weaned down and turned off, and she made the transfer to the conventional ventilator easily, even doing a small amount of the breathing work herself!

On day 5, Avery opened her eyes for the first time! That day, she stared at me and she gripped my finger so hard. I never wanted to take my eyes off of her. She was feisty, probably beginning to realize that she didn't like what was happening to her, and she kept trying to pull the tubes out of her nose! She would make her 'cry' face when she was being suctioned, or when she was upset about something and she had to be put in baby restraints. These things are the things which broke my heart, but at the same time it showed me what a fighter my girl was.

On day 7 Avery had her hernia repair surgery done. I wasn't actually that nervous for the surgery. I had been told that the surgery itself is not the hardest part. It's what comes after it. I found out later that Avery's Apgar score at birth was 3, 6. Those numbers are out of 10 and an 8 is considered good, so these were not good at all. The hole in her diaphragm was described as 'huge.' A pig-skin patch was used to repair it, with the hope that the patch would grow with Avery. The surgeon joked that he did so much to her, even taking out her appendix as it was in the wrong spot, that he figured he'd give her a haircut while he was at it. Of course that was just a joke.

Avery really did have her first haircut when she was 13 days old and an IV needed to be placed in her head. She had been having a rough time since surgery. She never got back on the conventional ventilator. She remained on the oscillator at high settings. The doctors were getting worried and talk about ECMO was beginning, although in 7 years it had been used on less than 5 CDH babies in this hospital. Sick Kids sees approximately 10 CDH cases per year, so I knew that things were getting bad. Our days were filled with instances of desaturations, belly ultrasounds, echocardiograms, tweaking of numbers and medications and trying to drain the fluid off of Avery who had become increasingly puffy. Avery's nurses had given her the nickname 'Diva' because she was proving to be so high-maintenance.

On Saturday, April 1st, 2006, Avery had a particularly bad desaturation. This was the worst day of my entire life. I never knew that a heart could hurt so much, that I could feel so much pain. Hearing the news that my daughter would likely die, I just sobbed into my mom's lap. I was told that 3 to 5 minutes without adequate oxygen is too long. Avery was without adequate oxygen for an hour. It was without question that she had suffered brain damage. I just didn't know how much. Being her mother, I was ever hopeful. I thought that sometimes a baby's brain can find new pathways and overcome something like this. I thought that even if she was going to be challenged in her life, maybe it wouldn't be that bad, and certainly I wouldn't love her any less. But I didn't know how bad it was.

Avery had also been put onto ECMO. The cannulas of ECMO are supposed to go into the side of one's neck. Senior cardiac surgeons were unable to properly fit them into Avery's neck. So her chest had to be opened and the two tubes had been placed directly into Avery's heart. When I first saw her I was in shock. Because her neck and chest had been opened, and ECMO requires that the person be on Heparin, a blood thinner, Avery had blood bubbling out of her neck and chest. Her open chest was covered only by a piece of gauze labeled 'open sternum.' Because of the very real possibility of brain damage, Avery was being kept ice cold. To assess her neurological function, she had to be lifted from the paralyzers that she had been kept on for all but the one day that I had seen her eyes. Her eyes were now open again, but this time they were different. This time they were staring blankly and non-reactive. I put my face directly in front of hers and imagined that just maybe she was making a connection with me. But I think now I knew. This was the end.

On Monday, April 3rd, 2006, the doctors were going to perform 3 tests on Avery to check her brain function. If these tests came back and told us that there was even a tiny bit of function, then it might mean that Avery one day would be able to lift her head by herself. If these tests came back and showed nothing, then that was it. There would be no possibility that Avery would even be able to do that. The tests came back and showed nothing. All 3 of them were nothing.

I don't feel like I had to make a decision for my daughter at that point. Had those tests left us with any hope, I may have felt differently, but Avery wouldn't have lived like that. I knew that what was happening was right and that is why although this day was a horrible day, it didn't compare to the shock and despair that I felt on Saturday. My baby was going to die today, but she would finally be at peace.

We asked her to do so much in her short 17 days here on earth. She was put through such a great deal and she did so well. She tried so hard, she was so strong. It wasn't fair to keep asking her to do more. The doctors sent me to a special room while they disconnected Avery from her ventilation and ECMO and fixed her up. I regret to this day that I wasn't with her when she died.

When Avery was brought to me, my heart actually lightened. She was stunning. She looked perfect. Peaceful and serine. As hard as it was for me, I know that it was right for Avery. I can only pray that she is happy now and not in pain anymore. Someone told me once that the less time a soul is on earth, the better a person they were. Avery didn't need to prove herself here. She truly is the brightest star shining down from Heaven.



Written by Avery’s mom, Lauren Ziebert (Canada)
2006