February 14, 2009, was more than just Valentine's Day for my husband Richard and I, it was the day we had been waiting for. My doctor's office called me to inform me that something showed up in routine blood work. I tested positive for HCG and was pregnant with our first child. Seeing as we had been trying for about eight months to get pregnant, the news brought instant tears to my eyes and was the best valentines day present one could eve ask for. The first trimester of the pregnancy was stressful. I had developed a subchorionic bleed and was afraid I was going to miscarry. I bled off on and on for the first 13 weeks or so. By week 14 everything calmed down and I was starting to relax. At our 20 week ultrasound we chose not to find out the sex of our baby, we wanted it to be a surprise. At this visit we were referred to a high risk OBGYN with better ultrasound equipment. They could not see the baby's fourth chamber of the heart and he/she had micrognathia, or a small chin. They told us not to worry, they were sure it was fine, they just wanted to make sure. The following Tuesday, our worries were put to rest, they found all four chambers of the heart and determined that the baby's chin was a result of having Daddy's features as he has a very undefined chin. At about 26 weeks pregnant I began to swell, the doctors monitored this closely and determine I was just a sweller. By 29 weeks I had a started to get headaches where I would see floaters. The doctors did a 24 hour urine test where they measured the level of protein. I was teetering on pre - eclampsia, however, did not have it. I was put on limited activity. By 32 weeks to say I was huge was an understatement, I had been monitored weekly by my OBGYN and was gaining weight rapidly. At this time I had an ultrasound. I knew instantly that something was wrong, the ultrasound teach was very quiet and we were led into an examine room to be seen by my OB. During this time the nurse took my blood pressure and weight. Not only had I gained 11 lbs. in a week, but the pressure was 145/90. Things were not good. Finally, my OB came in, I remember saying to him if he was about to tell me something was wrong to get out, I did not want to hear it. He handed me the box of tissues instead and told me to sit. I instantly began to cry. It appeared that the baby was about three weeks behind in growth and my fluid level, which should be around a 21 was at 57, which is why I looked like the Octomom and why I was gaining weight so rapidly. He told me I needed to go to the hospital for monitoring. Once at the hospital, the nurses put me on the monitor. Because I had so much fluid, they had a hard time keeping the baby on the monitor. They tried to give me a benadryl to relax and possible sleep so they could track the baby. However, this only backfired as I was nervous ad scared. The next day I saw the same high risk doctor in the Hospital. He told me that due to all the fluid it was hard to see the baby. However, he felt the baby had pyloric stenosis and wasn't swallowing like he/should of and that when the baby was born he/she would need a minor surgery to repair it. Feeling a little relieved, although not totally convinced I was informed that the monitoring looked good and as long as the 48 protein urine test looked good I could go home and remain on bed rest. The next week was busy. I did get to go home, the protein had actually dropped since the original test. we had appointments scheduled with the head of the NICU, my OB and a Neonatologist. The head of the NICU was shocked with the diagnosis given to me and informed me that pyloric stenosis is not typically diagnosed until about 5 weeks after babies are born. She also informed us that the Neonatologist had an emergency, we rescheduled that appointment and off to my OB I went. He did a biophysical ultrasound on me because they could not keep the baby on the monitor. My OB informed me that he thought that they could be wrong about the diagnosis of Pyloric Stenosis and thought possibly the baby has a duodenal atresia, where the baby's intestines were intertwined. He told me that he thought I would make it until at least 37 weeks of my pregnancy. Boy, did this baby make him look like a liar, my water broke that night. I went to the hospital and awaited the arrival of my baby. However, although I contracted, nothing happened and I was put on antibiotics and was told to sit tight. After about a week, I was going nuts. They could not keep the baby on the monitor and in turn it stressed me out because the nurses were always freaking out. My OB came in and informed me that they were meeting with a few neonatologist and the head of Children's hospital to determine whether or not to keep me on antibiotics and hooked up on the monitor until 37 weeks or to induce labor at 34 weeks. When I as informed of this I lost it. I think everyone on the perinatal ward thought I was dying, I was so distraught. To make me feel better they moved me into a much bigger room ( I had tons of family and friends visiting) and to allow me off the monitors one hour a day to go sit outside and have dinner with my husband. 2 days later my OB informed me they had all decided that it would be detrimental to my health to keep me on antibiotics for 3 1/2 more weeks and that because I was still filling up with water and leaking that I could be prone to infection. They also felt like the baby would grow better outside of the womb. at 34 weeks exactly I was induced. Nothing happened...literally nothing, the pocosin dripped and I contracted but no dilation occurred. At 12:30 my OB was done with his appointments and came and checked me, he informed me that he was going to have me get an epidural because he needed to go in and break the rest of my water and it would be extremely painful. After, two failed attempts at getting my epidural, it finally took and my OB broke my water. I will never forget the face of the nursing student who was in the room....she was shocked. The amount of fluid that came out of me was alarming. You see when my water initial broke, it was just a little poke in the bag so it kept filling up.. When my water was all out the baby's heart rate began to drop. Reaching 70 at one point. My OB got his scrubs on, called Akron Children's hospital and prepared for a c-section. Emily Anne Birdwisa was born on September 16, 2009 weighing 3lbs 4 oz. She was immediately taken into the room next door. They brought my husband into the room and informed him she wasn't breathing and needed to be intubated. Richard came back in the OR while I was getting put back together and tried not to scare me. It was written all over his face, something was seriously wrong. After I was taken to recovery one of the neonatologist came in and informed us of the situation. Emily had a blackness over what would have been her right side, they were not sure what to call it at the moment but told us that she could not survive with just one lung. He told my husband that someone needed to come with him to the Hospital so that in case the worst happened someone was with him. They told me they would call with updates. Before, being rushed to Akron Children's Hospital, I got to see our beautiful baby girl briefly, she was a fighter. Honestly, the next five hours were a blur. My sister's and brother drove up to see the baby and to be with me. Each time one of them saw me they tried to hold it together but couldn't hide the fear from their faces. Before my husband came back the NICU called, Emily tested negative for the three big Trisymony's and was stabilized on the ventilator. The next day I quickly got permission for my OB and finally went to go see my beautiful baby girl. She was gorgeous. Jet black hair, rosie complexion and feisty as all hell. That night they informed us she had pulmonary hypertension and was probably not going to survive. Again, I could not contain my emotions and my husband and I melt to the floor with the news. The next morning we were told they wanted to do a surgery that allow her to swallow and poop normally. Her lungs were not her only issues, the doctors were right, she did have intestinal issues. After much discussion with the surgery and the head Neonatologist we decided that the following Monday we would go ahead with the surgery. We had Emily baptized in the NICU that day. The weekend was pretty dull. Emily had tons of visitors and was stable for the most part. In fact I held her numerous times, even with all her tubing. She would stabilize the most when she was doing skin to skin with me. That Monday we consented to the surgery and the long wait began. My whole family was there, Emily had both sets of grandparents, aunts, uncles and parents, we were quite the group in the waiting room. After ungodly hours the doctor came out told us he fixed Emily's duodenal atresia and finally gave us the diagnosis of CDH. Emily had a RCDH with her spleen and liver pushed up over the right lung. He told us he would wait a week or so, let her heal and do her repair the following Monday. Most of the week was uneventful, we were waiting for Emily to poop. That Friday, when we got to the NICU we knew something was wrong. Emily was agitated and was not happy. The doctor heard Richard and I make the observation and informed us she agreed, that the parent's always knew when something was wrong. They started her on antibiotics just to make sure if she had an infection it was getting treated. The weekend was rough, she pulled out the NG tube in her nose and had to be put on a paralytic to keep her from raising her heart too much and to keep her calm. By Monday all her test came back negative for infection, however, her pulmonary hypertension was getting worse and her CO blood gas levels were high. By Tuesday morning they had put Emily on an Oscillator machine in hopes that her CO2 levels would improve. Although for a short time this appeared to be the answer, her CO2 quickly began to rise and they put her on Nitric Oxide during this transition he Oxygen levels dropped into the 70's and never really bounced back. By Midnight her heartbeat was 220 and she was fighting hard. After much discussion with the doctor we knew it was evident that Emily was going to pass, it was our decision to allow that to happen on the Ventilator or in our arms. At 3:15am September 30, 2009 we took Emily off the Ventilator and held our baby girl for the first time without any tubes. Richard and I told her how we fell in love and how we prayed for her existence in our lives for a long time. We told her we loved her and thanked her for giving us the best gift in the world of being her parents. Our family trickled in one by one to hold her and to give her a kiss. At 4:05 Emily passed away in our arms. Emily taught us how to be parents and the importance of honoring her life the best way we could, by bringing awareness to CDH. Emily is one of the many that are undiagnosed. She was one week old before we had a definitive diagnosis. We were unable to plan for what was to come and were unable to fully understand what CDH meant. I truly believe that Emily is our angel and wanted us to truly appreciate her life. We feel Emily's presence in our life often and know she is watching over her sisters and brother.
This story was written by Emily's mom, Katie Birdwisa.
You can help CDH families by donating to CHERUBS at www.cherubs.org.