Tuesday, June 23, 2015

June 23 - Dear Nicholas Sparks (Guest Blogger Julie)

My husband David and I had been married for 2 ½ years when we decided to have a baby. I was 34 and had a 9-year-old son (Jimmy) from my first marriage, and David was 33 and had a 13-year-old son from his first marriage. The baby was due June 6th and I would have a c-section in late May/early June.

Because of my age, I was concerned and cautious with this pregnancy. But other than morning sickness, things seemed fine. We had an ultrasound on December 23, 2002. I was 16-17 weeks along. The ultrasound technician did not say much during the scan. She said everything would be sent for analysis and my doctor would get a report. I wonder now if that is their policy, or if she just didn't want to say anything to us.

At Christmas we showed off our ultrasound pictures, and I started to relax. Then on Dec. 27, the hospital called saying my doctor wanted me scheduled for genetic counseling and a Level 2 ultrasound as soon as possible. I was terrified and called my OB/GYN. They said to calm down and not to worry (too late!). The ultrasound showed the baby might have a hernia or a hole in the diaphragm, or the diaphragm was missing. My first feeling was relief that it was something "physical" and not"mental." I figured they could fix something physical. Next I checked the internet using the only terms I could think of: fetus hernia diaphragm. The only thing I found was Congenital Diaphragmatic Hernia (CDH). What I read was not comforting!

At the hospital on Monday, I was a wreck. The genetic counselor (Beth) said there were other suspicious things which the doctor had not mentioned. There was a concern the baby might have a cleft lip, cleft palate, a missing kidney, and a larger than normal head. And yes, they were looking for CDH. Beth told us babies with one abnormality usually have more. She talked about all kinds of potential problems. Beth was very open and honest with us.

The ultrasound confirmed the CDH. The good news was they didn't see any of the other defects. I would need to have Level 2 ultrasounds frequently, and in a few weeks they would do a fetal echocardiogram. Mostly we heard that they couldn't tell us much yet because it depended on how well the baby's lungs developed. The liver was in the abdomen, but it could move up or down throughout the pregnancy. I had an amnio done, and they also did a FISH test so we could have results in 48 hours. Both results came back fine. There were no problems with the baby's chromosomes. Beth warned it did not mean there was nothing else wrong. There could still be problems which don't show up on ultrasound or amnio. But it was still a relief.

We had an Echo on Feb. 5. They didn't see any major heart defects (no holes or anything). Because the heart was pushed over and turned, some things did not go in a normal route, but blood was flowing to and from the heart the way it should. There were some things they couldn't see well though, because the stomach was in the way.

I read everything I could about CDH. I read every Cherubs story. I was frustrated because there didn't seem to be much difference between the babies who lived vs. the ones who died. I did read that babies with heart problems in addition to CDH would most likely die. That scared me. Although they didn't see anything terribly wrong with our baby's heart, it wasn't completely normal.

We met with a surgeon who explained what we could expect with the CDH repair. She was very optimistic based on the reports she'd seen on our baby. She said she'd guess his chance of survival would be 80-90%. She even thought he might not need ECMO. She said babies on the bad end of the scale generally do poorly, and ones, such as ours, on the good end tend to do much better. We felt very positive after talking to her.

At about 6 ½ months the baby was lagging in weight (11th percentile), but he was growing consistently (the lag wasn’t getting any worse). I started having Biophysicals once a week to keep an eye on things. My chart was now marked as Diaphragmatic Hernia and IUGR.

Thomas Jefferson University Hospital in Philadelphia (an hour away) was the closest hospital with ECMO, so I transferred my care to the MFM group at Jeff. At our first appointment, we had an ultrasound and another Echo. We were told the hole in the diaphragm was very large and that the baby had severe dextrocardia, meaning the baby's heart was pushed way to the right (practically against the ribs). We talked to a Neonatologist, toured the NICU, and saw a baby on ECMO. The docs reviewed the ultrasound results, and said the stomach was still up, along with a significant amount of bowel, and the liver was still down. The baby was still smaller than they'd like, (15th percentile now), but measurements showed everything was proportionate, meaning he would just not be tall (David is 5' 6" and I am 5' 1" so that wasn't surprising). They were concerned about his limbs possibly being short, and his head looked lemon-shaped, but they could not give us any indications of what this meant! Also, I had polyhydramnios, which explained why the baby was so active. I started having NSTs twice a week.

One day, when I was 7 months, I started having contractions. They were 7-10 minutes apart, so David called the Doctor. We were told to go to our local hospital to be monitored because it was much closer than Jefferson. The monitor showed I was contracting every 3 minutes. I was only a fingertip dilated and not at all effaced. They ran tests and started an IV for fluids. Results of a fetal fibronectin test were negative meaning there was 99% probability that I would NOT go into labor in the next two weeks. But I kept contracting. I guess the hospital didn't want to be responsible for sending me home since my official doctors were at Jefferson, so at 3 a.m., I was transferred by ambulance to Jeff. At Jeff they did another cervical exam (still no change), and talked about options. I was exhausted. David noticed the contractions were slowing down, and when I dozed they completely stopped. Finally they did an ultrasound to check on the baby. He was fine, and his heartbeat had been perfect the entire night. At noon on Friday they finally let us go. A couple of weeks later, I had another bout of contractions during an NST and was sent to triage. After a couple of hours, I was sent home with a prescription for a urinary tract infection.

We had a special Oxygenation test on May 2nd. This was a way to determine how well the baby's lungs were developed. First they measured the baby's oxygen intake while I breathed room air. The graph showed a lot of resistance getting oxygen to the baby. Then I breathed oxygen through a mask for 10 minutes and they checked the level again. We could immediately see the difference in the graph. We were told it was the best outcome they'd ever had with this test, and they felt the baby would do well. We were ecstatic. Then I met with another surgeon to talk more about what would happen when the baby was born. He said there were several indications which made him think our baby would have a very good chance.

The big day finally arrived, and Sean Anthony Feaster was born at 9:28 a.m. on Wednesday, May 28th. He weighed 5 lbs 4 oz and was 18 ½ inches long. Even though we had been continually told we wouldn't know what we'd be dealing with until he was born, things could not have gone more differently than we expected.

When the c-section started, the Doctor never said he started cutting, nor did he tell us when the baby was out. There were so many people in the OR, and I couldn't tell what was happening. The nurse came and told David not to worry about not hearing the baby cry. She wasn't sure if we realized they would intubate immediately and he wouldn't be able to cry. David said, "He?" She said, "Oh, you don't know what you had?!" She went to check, and when she came back, David turned to me and said, "You were right. It's a boy." We were both crying.

We were told David would be able to see Sean briefly and take a picture. But then someone said, "Get that camera from Dad." They grabbed it from David and took pictures for us. I think I knew then that something was wrong. Otherwise they would have let David see him. They handed us two pictures, and we continued to cry. Sean was taken to NICU, and David and I were taken to the recovery room to wait for information. They said it would take about an hour to get Sean situated, and then they would let David go up to see him. Over an hour passed, and we were told it would take a little more time. So we waited some more. We tried to act calm, but we were very worried.

Then the Neonatologist came into the recovery room with another Doctor and a social worker. I knew that was not a good sign. The Doctor said Sean was very sick. He had a significant amount of bowel and part of his liver in his chest, and he had a problem with his heart! Immediately I thought of my research which said most babies with both CDH and a heart problem would die. Sean had Tetrology of Fallot (TOF). We were told the team of Neonatologists almost lost Sean at one point, but he was now fairly stable. Jefferson was not equipped to handle cardiac problems, and Sean needed to be transferred to A.I. duPont Hospital for Children in Wilmington, Delaware (which is only 20 minutes from our home in Newark, Delaware) immediately and placed on ECMO. We were confused. We were at Jeff because we thought they were the only ones in the area who had ECMO. As it turns out, the Nemours Cardiac Center at A.I. does have ECMO, but it's only used for cardiac patients. And until now, there had been no indication Sean had a heart problem.

We were devastated. The docs asked if we had any questions. I said, "Is there ANY chance I can see him before he goes?" David told them I had not seen Sean at all, and David had only caught a glimpse. I hated to delay Sean from getting the medical attention he needed, but I wanted so badly to see him. When they brought Sean in, I was so happy and so scared. He was beautiful. We were able to touch him, and the social worker took pictures with a digital camera. I told the transport team,"Whenever you need to go, just tell us, because if you're waiting for me to tell you to leave, it won't happen." I knew Sean had to leave, but I couldn't bring myself to tell them to take him. It was so hard to see him go. I was terrified he would die before he got to A.I. David left to follow Sean to A.I. I felt so lost and scared, but I wanted him to get to Sean too. Sean needed his Daddy a lot more than I did. At the time I thought nothing in my life would ever be harder than what I just experienced. Unfortunately I was wrong.

Just as David arrived at A.I., a surgeon called me saying Sean was very ill and needed to go on ECMO immediately. He couldn't wait for David to get to the unit and wanted my verbal ok to go ahead in order to save Sean's life. A social worker (Judy) at A.I. called to tell me Sean had been baptized and kept me updated while Sean was in surgery. Sean was successfully put on ECMO and was safe for the time being. Not long after going on ECMO, they were able to turn the flow down 50%. Sean was able to do half of the work himself! We knew then how strong he was.

A.I. is an amazing hospital. The Cardiac Unit has rooms for parents so they can stay at the hospital. The room was set up so that when Sean was well enough he'd be moved there, and we could help take care of him. The room had beds, a bathroom, a TV/VCR, and a telephone. They provide parents with meals and other necessities. It was a wonderful set up for people like us with very sick children. We were able to literally live at the hospital.

David called me often. He told me about the people caring for Sean and how great everyone was. He said he sat with Sean and told him how much we loved him, and how I wanted to be there and that he'd bring me soon. There were times when he couldn't sleep and would go sit with Sean in the middle of the night (another advantage of being at the hospital 24/7). David said when he sat with Sean, an hour felt like five minutes. But to me, sitting in another hospital unable to be with Sean, five minutes seemed like five HOURS.

I was desperate to get to A.I., but because of a problem with my blood pressure, I didn't get out of the hospital until Sunday. Saturday night David came and brought pictures of Sean. He looked so different than he did the day he was born. For the rest of my life, I will regret missing those first days with Sean. There's no way to describe how it feels to be separated from your baby. I asked David about Sean's limbs and head and neck. He said, "Julie, there is nothing wrong with him on the outside. He looks perfect." A geneticist saw Sean and said he looked perfectly normal on the outside.

When I finally got to A.I., David said, "Do you want to go see Sean?" What a question! I was nervous, scared, and excited. David took me to CICU and there was my Sean! Even through all the tubes and wires, he looked absolutely perfect. Sean's left eye was swollen shut because his head had been turned since he went on ECMO, and his head was flattened from always lying on that side, but he was still adorable. I leaned close to him and said, "Hi Sweetie," and he opened his eye! David told Sean, "See, I told you I was going to bring Mommy." I didn't want to stop touching him. I wanted to make everything better. I didn't want to ever leave him again.

Jimmy came to visit that night. I took him to see Sean, and it was the first time I had both of my sons together. I loved it. Jimmy was thrilled to have a little brother. He often asked me if the doctors knew yet if Sean was going to live. We took the camera so Jimmy could have a picture of Sean to take to school. And I took a picture of Jimmy and Sean. Thank God I did because it is the only picture I have of the two of them together.

The next day was Sean's CDH repair. The surgery went fine. They put Sean's organs where they belonged and repaired the hole in his diaphragm. Sean had virtually no diaphragm on the left side. There was only a small lip, so they used Gortex. The next morning, the doctors made adjustments to Sean's settings to see what he could tolerate, and tried clamping off the ECMO lines to see if he would be able to come off. I saw Sean turn purple and it looked like he was struggling. He was not ready to come off, but they were able to turn the flow down. Now Sean was doing 75% of the work himself! His blood gases were great after the CDH repair. He was putting out urine which meant his kidneys were working. We were thrilled to hear he pooped for the first time a couple of days after the surgery. It meant his intestines were functioning too! The nurse said sometimes Sean would even try to take his own breath in addition to the ventilator, which seemed like a good sign.

I spent as much time as I could with Sean. It was so hard to get up and leave him. Mostly I held his hand or stroked his forehead, and sometimes he grabbed hold of my finger. At times the nurse would tell us not to stimulate Sean because of his heart rate. That was so hard. Whenever David talked to me or the nurse, Sean would open his eyes or try to move. He knew his Daddy's voice. Sometimes he would lift his arms and get so feisty that they had to strap his arms down. He was strong and he was a fighter. He opened his eye(s) a lot. I know he saw and heard us. We told him what a good boy he was and how strong he was. And we never left CICU without telling him we loved him and that we'd be back later.

The doctors tried clamping the ECMO again one day. Sean handled it for about five minutes then started to have problems. It was progress. He just needed a little more time. Three days after the surgery, the doctors decided to do Sean's TOF repair. They had hoped to get him off ECMO first, but he still was not able to do all the work himself. They didn't know how much of that was due to the heart problem, so they wanted to fix it and see what happened. David and I felt fixing the TOF would improve things tremendously. Sean was already doing most of the work himself, so it seemed logical that the boost the heart repair would give him would be more than enough to get him off ECMO. We knew ECMO saved Sean's life when he was born, but we also knew he needed to come off soon.

The surgeon repaired the hole in Sean's heart with "a rather large patch," and said, except for the fact that Sean's heart was still on the right, things went routinely. The Doctors let Sean rest over the weekend and kept him pretty heavily sedated. As usual, whenever they lowered the meds, he would start to move and get mad!

On Saturday fluid built up around Sean's heart, and they had to drain it. During the procedure the Doctor nicked Sean's lung. Air built up in his chest, so they put in another chest tube to get the air out and keep his lung from collapsing. Sean was very swollen from his surgeries. His kidneys stopped functioning after the heart surgery, so he was given Lasix and they turned the ECMO back up. They tried feeding Sean breast milk, but he did not digest it.

On Monday, they tried changing Sean's settings again, and he did not do well at all. He got VERY sick. It took all day for them to get him back to where he had been in the morning. It was so scary. It made me wish he was still inside me where he had been protected and safe. Tuesday morning an Echo showed Sean's heart still wasn't pumping blood properly. One side of his heart was larger than the other, so one was getting too much blood and the other not enough. They wanted to take him back to the Cath Lab and put a hole in his atrium. The blood flowing through the hole would be blue blood, and with his lung problem, Sean really needed oxygenated blood, but this was the only option they had. They wanted to do the procedure right away and were unhooking Sean's equipment to take him to the Cath Lab while I signed the consent form. Seeing my tiny, sweet baby going in for another procedure was heartbreaking. The procedure went ok, and Sean's blood gases were stable throughout the afternoon. That evening the Doctor said there weren't any major changes in Sean's status but they hadn't expected immediate results.

Sean's blood gases were fairly stable throughout the night - not great, but consistent. His chest tube was draining a lot of blood, but no one seemed concerned, so I tried not to worry. I noticed he hadn't been putting out any urine at all. He was still very swollen so an attachment was put on the ECMO unit to remove fluid. Wednesday evening Sean was still the same. I never asked what the next step for Sean would be. I was too afraid of the answer.

Thursday morning we were shocked when we saw Sean. The swelling was gone, and he looked so much smaller! It was a drastic change from the night before. He looked tiny, and his eyes were huge in his little face. Unfortunately, Sean's blood gases were not very good, and his bilirubin levels were rising, which was a sign his liver was not functioning properly. The nurse said they weren't planning anything special for Sean that day. Just then another baby was being transported in, so I had to leave CICU.

I was in our room when a nurse popped in and said a Doctor was looking for me. The Doctor came to our room and I started to panic. Part of me thought she wouldn't tell me anything bad without David there, but I was wrong. She said the Doctors got together that morning and discussed Sean's case. Everyone who had worked with Sean was involved in the meeting. They even called Doctors at other hospitals to get input. Sean wasn't getting any better; in fact he was getting worse. She said Sean had been on ECMO for the magic two weeks. They tried Nitric Oxide several times, but Sean still had too much lung resistance. His kidneys had shut down days ago, and now his liver was shutting down. His blood gases were not good, and his blood pressure was low. All of these were very bad signs. She said there was nothing else the doctors could offer him. The more she talked, the harder I cried. She went on to say we needed to make a decision. We could wait until things got worse and the Doctors would make the decision to take Sean off ECMO, or wait until something happened such as a stroke or a blood clot which would kill Sean, or make the decision ourselves to remove Sean from ECMO. She said some parents can't bring themselves to make the decision so they just wait and let God make it for them. And some parents don't want to see their child go through anything else. She knew the decision would be especially hard for us because Sean was still opening his eyes and looking at us. As far as they could tell, Sean's brain was still ok.

I couldn't believe what she was telling me. We always knew it was a possibility, and we had seen some of the signs, but it still seemed unreal. I kept thinking, "Is this really it? Is this the end? It can't be!"I didn't want to give in. I said, "If we wait, does anyone think there's any possibility Sean might get better?" I had to know if there was ANY hope at all. If there was the slightest glimmer, I would cling to it. She looked me straight in the eye and said, "No." Everyone had exhausted every option. There were no more tricks to pull out of the hat. She said Sean's heart surgeon (Dr. Norwood) was the most aggressive Doctor she's ever known. He will try anything and everything, and even he couldn't come up with anything else. As I thought about this later, I realized the Doctors probably knew taking Sean back to the Cath Lab on Tuesday was a long shot. I'm pretty sure they didn't think it would work, but it was the only thing they could think of to try. I take comfort in that because I know the Doctors really did do absolutely everything they could think of. Now Sean was basically being kept alive by "heroic" measures.

I called David and he said he'd be there right away. It seemed to take forever for him to get there. Finally he walked through the door and put his arms around me. Neither of us said anything. We just cried. When we pulled apart, I was not prepared for the look on David’s face. But I completely understood the look because I felt the same thing: complete sadness, total devastation, and helplessness. David said, "I don't want to lose my son." We went to see Sean. I looked at him and thought, "How can I let him go?" "What will life be like without him?" My heart was breaking. Thinking back, I admit Sean did not look good that day. Maybe it was because he was so thin and frail looking now that the swelling was gone. He didn't seem as strong as he had before. I don't remember him trying to lift his arms at all that day. He opened his eyes, but he looked tired and worn out. I was hoping the Doctor would have something new to say. But there hadn't been any miraculous change in Sean's condition.

I told David what the Doctor said about our options. David said no matter what we chose, it would still be decided by God. He was right. I had gone over the options all morning. Part of me thought, "Why make Sean go through anything else?" But when I thought of actually letting him go, I didn't think I could go through with it. No matter what we chose, the outcome would be the same. Sean would die. We would never bring our baby home from the hospital. David said, "I think we only have one choice." He had told me that while I was at Jeff, he had promised Sean he'd never let anything else happen to him. He cried as he reminded me of this promise to Sean. We couldn't let Sean go on knowing there was no hope. We would ask the Doctors to remove Sean from life support. Sunday was Father's Day, and I thought maybe David would want to wait until after that, but he didn't. He was adamant about not wanting to risk ANYTHING else happening to Sean. We didn't want him having a stroke or throwing a blood clot. Sean had been through so much. It would have been cruel, and it would have made David break his promise to Sean. We would call everyone to come say goodbye. After everyone saw him, we'd spend time alone with Sean. We thought it best to wait until evening to remove Sean's support. It would be quieter and there would be fewer people around.

Telling people was horrible. My Father was so upset he couldn't bring himself to come say goodbye. Jimmy took it hard too. I took him to see Sean, and before we left, Jimmy went to Sean and squeaked out, "Bye Sean" and started crying. I knew it was the last time I'd see my sons together.

The afternoon went by quickly. I was looking forward to things quieting down and being alone with David and Sean. As the last of the family left, it started to storm. It had rained almost every day while Sean was in the hospital. I kept thinking the sun would finally come out the day we knew Sean would be ok. So it was fitting that there was a storm on Sean's last night. Sitting with Sean was bittersweet. He was so beautiful. His skin was so soft. He had his eyes open quite a bit. We took pictures of each of us with Sean. And the nurse took some off all three of us together. Then we just sat watching him, holding his hand, touching his face, rubbing his arms and legs, and trying to memorize every detail.

The Doctor explained what would happen. He said we could be there with Sean. It wouldn't be easy to watch him die, but I could never have walked away and left him by himself! Then they would clean him up and bring him to our room for us to hold. The Doctor told us to take our time and let him know when we were ready. I couldn't imagine us having the emotional strength to tell him we were ready to let Sean go. How do you choose that moment? While we were sitting with Sean, another baby was being brought in. The baby must have been very sick. Practically everyone in the unit was helping to get the baby stabilized and settled. There was a lot of activity around the baby, and I couldn't help but think this was probably exactly what it was like when Sean was first brought in. Normally we would not have been allowed in the unit while all this was going on. But no one ever asked us to leave. They just pulled the drapes around us.

After a while, we went to take a walk and get clothes for Sean. When we got back, everyone was still busy with the other baby. I was glad because it meant we had more time to spend with Sean. It was getting very late, and after a while, we decided to go to our room for a bit The nurse said they were wrapping things up with the other baby and would call us as soon as the Doctor was free. Soon the nurse called and said the Doctor was ready. It was just after midnight on Friday, June 13th.

When we got to CICU, David walked directly over to Sean without washing his hands first. I was surprised, and then I realized there was no reason to wash up this time. The Doctor asked if we wanted to have an autopsy done. Sean's little body had already been through so much. I didn't want him being cut open again, but we agreed to let the geneticist have a tissue sample. I briefly wondered if the geneticist would use the sample to see if there was anything else wrong with Sean. If there was something else, I wasn't sure I wanted to know. He would always be my perfect baby.

The Doctor told us once they gave Sean the pain medication, we could not change our minds. And then it was time. For months, I had prayed this moment would never come. For weeks, I had begged God to let Sean get better. Now all I could do was pray for a miracle. Maybe, just maybe, Sean would be able to breathe and his heart would work on its own once the machines were turned off. I knew the chance of that happening was minute, but I still wished for it. There was nothing else I could do except be there for my baby, my Sean, and comfort him as he passed away.

We stood on either side of Sean and each held one of his hands. I heard the Doctor tell someone to turn off the machines. We cried as we held onto Sean and told him how much we loved him. We touched Sean's face, his hands, his arms, his legs, and his hair. I don't know the exact moment when Sean died. His eyes were open the whole time. That made it hard, but I was also glad Sean saw us as he died and knew we were there with him. Suddenly his chest moved and he seemed to be trying to take a breath. I said, "It's ok, Sean." I wanted him to know it was ok for him to let go. He didn't need to keep fighting for us anymore. He could finally rest. David must have felt the same way because he said, "It's ok, Sean. You go be with Jesus." It seemed like we were only there for a few minutes, but it must have been longer because the nurse and Doctor told us we needed to go. I didn't want to walk away yet. I hated the thought of leaving him. But we did. For the first time, I left CICU without telling Sean I'd be back later.

The nurse brought Sean to our room and said she thought we'd like the way he looked now. I was afraid to look at him until she was ready to hand him to us. She gave us a memory box and started to hand Sean to me, but I told her to give him to David first. For the first time, we looked at Sean and saw him with no tubes or wires or tape. He was so cute! He seemed smaller than he had in his bed. He looked like he was sleeping. He looked like the angel he now was. The nurse took pictures of the three of us then left us alone and said to call when we were ready for her to take Sean back. David held Sean briefly then gave him to me. He felt wonderful. Finally, after all this time, I had my son in my arms. We took turns holding him and used up a roll of film. I couldn't hold Sean close enough or long enough. I knew I would never have enough time with him. I didn't ever want to call the nurse. I wanted Sean with me forever. But eventually I let David call her. She came and took our baby away. I would never see Sean on earth again. I would never touch his face again. I would never hold his hand again. I would never get to do any of the things I had dreamed of doing with him.

Several months later we met with the geneticist. He found that Sean had 13 ribs, a hemi-vertebra or butterfly-shaped T7, and some other minor variations including a transverse palmar crease and a fifth finger clinodactyly. Sean's chromosomes were fine, however, which rules out a lot of things. There is the possibility that Sean had Fryns Syndrome, but the geneticist thinks it is unlikely. Sean did not have all of the features consistent with Fryns. Due to all the blood transfusions, Sean was unable to be tested for 22q11 deletion, but David and I were tested and our results were normal.

We had a beautiful funeral service for Sean. He certainly made an impact on many lives during his short time with us. David and I love talking about Sean, and we think of him constantly. I've heard people who've lost a child say the baby who died is not the one they were meant to have. I don't agree. If I wasn't meant to have Sean, God wouldn't have given him to me at all. What I am trying to accept is that for some reason God decided Sean wasn't the baby I was meant to keep. But there's no doubt in my mind I was meant to have him. Sean will never be with me physically again, but he will always be in my heart.

Sean's mom, Julie (Delaware)

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