Johnson, Matthew Ryan

[left:7ce87935d0]http://www.cherubs-cdh.org/Album/new/johnson3.jpg[/left:7ce87935d0]My husband and I found out that we were expecting our second child in October of 1997. We were so excited, we had been trying for a while, and I finally went on a fertility drug called Clomid. The pregnancy was going so perfect; I had absolutely no problems. We did a test called Tri-Screen to determine if there were any birth defects. The test came back high risk in Down Syndrome, we were devastated. We went in to my OB/GYN and I had an amniocentesis, and an ultrasound at 16 weeks. The ultrasound showed nothing abnormal, but he said we would have to wait for the amnio to come back to be certain. Fortunately all the chromosomes were normal, and we found out we were expecting another boy! We had already decided on names before I even got pregnant. His name would be Matthew Ryan. Four weeks later we went in for our regular 20-week ultrasound. I knew something was wrong by the look of the technician's face. He brought our OB/GYN in, and they told us that our baby's stomach was up by his heart, and his heart was pushed over to the other side of his chest. They suspected CDH, and that he could possibly die from this. My husband and I were again completely devastated. They sent us to a specialist to get a second opinion, and they confirmed again that it was definitely CDH, and that some of his liver was up also which made his chances of surviving very small. They gave us three options we could terminate the pregnancy, and there is no possible way we were going to that. Option two would be to go full term, and see what happens. They gave him an optimistic 20% chance of surviving. The doctor explained to us in great detail about ECMO, and what could happen on ECMO. Option three would be to have fetal surgery. They would make an incision from hip to hip, then poke three or four different holes in my uterus for scopes. They would then put clips on his throat to keep the amniotic fluid from escaping his mouth, and then that would make his lungs grow by forcing the intestines, stomach, and liver down. We went out to the University of California San Francisco where they did this surgery and met with Dr. Harrison and his team.

The team had to decide whether we would be a candidate or not, because they only did this surgery on the worst cases, and unfortunately we were a candidate. We decided we would go through with the fetal surgery, because we believed and still do to this day that was the only chance our son had at surviving, and I would do anything to give my son a chance at life, even if that meant sacrificing mine. We also felt that if we didn't go through with the surgery, and our son died we may never be able to forgive ourselves. But if our son died even with the surgery we could at least live with ourselves knowing that we tried everything in our power to save him. We live in Colorado so this also meant me living in San Francisco until he was able to come home. This was an experimental surgery, and we were not sure our insurance company would pay for it, luckily they did. Matthew would be the 12th to have this surgery done with the endoscopic scopes at UCSF. They did the surgery about four weeks later, I was 26 weeks. The surgery went wonderfully the only problem was that the machine that clips his trachea had jammed. It tore his trachea, which they said they wouldn't be able to tell how bad it was until he was born. Matthew and I had done wonderfully, and they started seeing lung growth just a few days after the surgery, which they said was remarkable since they usually see it after at least a week. After the surgery I would have to be on bedrest until Matthew was born, we got a room at the Ronald McDonald House a week after the surgery. Three days after we arrived at the House my water broke. I was very scared because I was only 27 weeks along. I managed to hold off for three more weeks. On May 14, 1998, I was 30 weeks along and developed an infection so they had to take him by C-Section. This C-Section is different than most, they put me under anesthesia so I don't remember anything but what they told me. When he was delivered still attached to me they took the clips off of his trachea, and quickly stitched it up. They said the tear was not as bad as they had originally thought. Then they cut the cord, and quickly intubated him, and he was rushed to the ICN. The first thing I asked when I came to was "Is my baby alive?" The nurse said that he was doing better than they had expected him to, Matthew was on a conventional ventilator. Matthew had 50% of his left lung, and almost 100% of his right lung, so the fetal surgery had worked. We were so happy, but Matthew was not out of the woods yet. They wouldn't let me see him until I was totally out of my anesthesia. They brought me a picture of him, but I was so loaded up on all of the drugs that my vision was blurry, and I couldn't even see what he looked like. They finally wheeled me into the ICN to see him, and he was beautiful. All I could see was my son; I didn't even see the tubes and wires going in out of his little body. The second that I saw him I thanked God for sending him to us, we are so blessed. He was so tiny he weighed 2lbs. 10oz. and was 15 inches long. Later that night they had to put him on a high frequency ventilator, he was on that for about a week and a half in total. They would put him back on the conventional, but then he would fail, and have to go back to the high frequency ventilator. He did that a few times. A week after he was born he had his CDH repair using a Gortex patch. We were very fortunate that Matthew was not born with any other birth defects, because we were told when there is one birth defect there could be others. We were so afraid when he would stop breathing and turn blue, and the doctors would have to do CPR on him. That has happened to us so many times that I can't even count. No baby should ever have to go through something like this.

The next few months were spent weaning him off of his ventilator. My husband and my other son would come out every other week. That was so hard being away from them, but we managed somehow.

We were stronger than we ever thought we could be. I got to finally hold my son for the first time after a month, I will never forget what that felt like, I can't even explain it. It was just so wonderful my son had even made it this long. The doctors said he was doing wonderfully, and that we would probably be home by our due date which was July 21st. That day came and went they had tried to extubate Matthew a few times, and he even pulled it out himself a couple of times, but he just couldn't support himself. They did what is called a bronchoscope. They looked down Matthew's trachea with a scope, and found that he had a floppy airway, which they call tracheomalacia. They said he would eventually grow out of that as he gets stronger, so they decided to transport us back to Denver. I was sad to leave I had been there for four months, and had made friends, but I was so excited that we would finally be coming home to our family. We were transported by jet to the hospital where the surgeon that recommended Dr. Harrison was. We had to get used to new nurses, doctors, surgeons, and the way they did things. They tried to extubate him numerous times; the longest he lasted was three days. That was so wonderful not to have a tube down his throat. They did another bronchoscope, and found some scar tissue blocking his trachea caused by the fetal surgery. It is like breathing through a straw, and having 75% of it blocked. They would have to do surgery to repair it. This surgery would be bigger than any surgery he had done. Because of where the scar tissue was located they would have to open him up like open heart surgery, and he would most likely have to go on a heart and lung machine. Then they would have to break his sternum to get to where they needed to take out the scar tissue, and reconstruct his trachea. His trachea was curved so they also had to straighten it, and they used some cartilage from his ribs. We were terrified, but I believed he would do fine. He did, and he came out of surgery fighting, literally. They didn't even have to use the heart-lung machine (ECMO) he was so strong during the whole thing. The surgery was successful. They tried to extubate him, and he still could not do it. They looked again, and found that he had severe tracheomalacia. They decided to do a tracheostomy. After they put in his trach, it was like he was a different baby. He could do so much more now. Matthew had been refluxing, which they said was normal for CDH babies. They did a Nissen-Fundoplication, and also put a G-Tube in. After that surgery he started to gain more weight, which is what he needs to eventually get his trach out. He has tolerated his feeds, and we are currently working on giving him fruit and vegetables. In the last week he has tried some crackers. He doesn't take much, but it is a start. He is getting better about gagging when it goes down his throat. He is sitting without support now, and we are trying to build up strength in his legs for him to stand. They say developmentally he is where he should be for his corrected age. Matthew is a little over 11 months now, and just came home for the first time. He had been ready to come home for a couple of months, but in Denver there was a huge shortage of home nurses. Matthew was home for four days, and then had to go back in the hospital. He got a blood infection, and then we learned his patch came loose. Some of his bowel and colon were up in his chest. He recovered from the surgery, and came home after two weeks in the hospital. If there is any advice that I can give it would be to be there for your baby, and give them the love and strength that they need. With your love and support behind them they will overcome anything. Through the eleven months of my son being in the hospital I have missed two days, and that was because I was sick, and didn't want him to get infected, otherwise I have been at the hospital every day of his life. The one thing that kept us going was Matthew's smile. He came out of his 16 surgeries with a smile. I would recommend if you know that your baby has CDH before he/she is born to tour the N.I.C.U., and possibly see a baby on ECMO. We did, and I think that helped us out so much, it wasn't such a shock when the time came because we knew what to expect. I feel very fortunate that we had found out that Matthew had CDH during the pregnancy, because I feel it gave us time to prepare as much as we could. It would be nice to hear from parents who are thinking about doing the fetal surgery or who have had it done, and what your baby's outcome was. I would also like to hear from parents who have children with a tracheostomy, if you could give us some advice, and how long your child had it, or will have to have it. We believe miracles can happen, because Matthew is living proof of that. The name Matthew means "Gift of God", and we thank God every day for his tremendous gift to us. I don't think I deserve such a gift. That gives me even more reason to protect and love him with all of my heart and soul.



Update 10/4/99



Since I wrote Matthew's story too much has happened. Matthew came home from the hospital in April 1999. He had to go back in the hospital a week after he came home. His patch had broke so they had to repair his hernia patch. He came home a week later, and was able to spend his 1st birthday at home with all of his family. It was so wonderful. Matthew had no problems for the next three months. On July 10th Matthew stopped breathing, and his ventilator alarm never went off. He was taking a nap, and we usually checked on him every 15 to 20 minutes. My husband did CPR until the paramedics came. They were able to get his heart beating again. Matthew suffered severe brain damage, because he was without oxygen for at least 10 minutes. They said he would probally never come out of his coma. We waited two days to see if there would be any changes, and it was only getting worse. His brain was swelling more and more. On July 12, 1999 we decided to let him go. That was the hardest thing we have ever done. I hope none of you will ever have to make that awful decision. We knew we did the right thing, and we know Matthew is in a better place. In heaven there are no tubes, surgeries, or pain. He will be with us in our hearts forever.



Written by Matthew's mom, Melissa Johnson (Colorado)
1999