Dear Pediatrician: Summary of Ethan's Hospital Adventure

"Hi, I'd like to schedule my one week discharge from the hospital appointment for my son. His birthday you ask? Why yes, it is 7-2-2015. Yep, that is correct. Seven as in July. Oh yeah, I understand today is October 20th, you see, my son has been in the hospital since birth and let me tell you about his miraculous life so far..."


Dear Pediatrician,

This is my son, Ethan Michael Chamberlain. He was born July 2nd with a congenital heart defect. He has several heart anomalies and my cardiologist has classified his diagnosis as Shone's Complex. He has been in the hospital for 3 1/2 months and has had 1 catheterization, 2 EEGs, 4 total surgeries (2 open heart, 1 sternal wire removal, 1 g-tube placement), 4 MRIs, 6 intubations, 14 echocardiograms, and over 60 x-rays. Let me tell you about the beginning of his life so you can see just how far we've come!

Surgery #1
On July 6th Ethan entered the OR for his first heart surgery for a PAPVR repair, COARC repair, and an ASD repair. The surgeon entered through a thoracotomy on Ethan's left side and began the planned procedures. There was a tare in his heart and Ethan began to CODE. Quickly the thoracotomy was sewn up, Ethan was flipped to his back, his chest was cut open, the surgeon broke the sternum bone, and bypass was set up to get blood flow to his vital organs but Ethan had already gone 13 minutes without sufficient flow. After the procedures were complete, the doctors couldn't stop Ethan's bleeding. Ethan entered cardiac arrest several times and the doctors continued to work to revive him. After 20 hours from the time Ethan entered the operating room, the cardiologist presented 3 options. The doctors could put him on ECMO (a long term heart and lung by pass machine whose main side effect was bleeding), they could stop reviving Ethan and see if he made it, or they could continue until he didn't come back.

Michael and I chose to continue trying and we turned to each other, our families, and the Lord. I owe the nursing staff that night for allowing my mom to be with me past visiting hours. My mom was a strength to me as I cried out my soul for my first newborn baby. She held me in a way only a mother could while Michael and I tried to come to grips with the information presented. We called my uncle who is an anesthesiologist in Utah at 2am to get his opinion and family comfort. I am grateful for his insight. We turned to the Lord. You see, I believe in God. I believe God is a God of Miracles. I believe that God's power is on the earth and I know it was through the priesthood power, God's power, that my son was healed. Michael was allowed to enter the operating room and give a priesthood blessing and he did. To the doctors in the room it was just a prayer, but I know it was a powerful blessing of healing. When Michael came back from the operating room he reported that during the blessing he had said Ethan could return to his Father in Heaven in peace but he had the choice to stay on the earth with us. Michael left it up to Ethan according to God's will.

After the blessing, we did the hardest thing I have ever done in my life. We gave the ok for the doctors to stop reviving Ethan and then we waited. We waited for the nurse to come in with the time of death. As we waited I said my good byes. Good bye to my son, good bye to my dreams and plans, good bye to the life I had fantasized over for 9 months, good bye to my body that just gave birth. I started thinking about what I would do next. How would I stop my milk supply? Who do I call to plan a funeral? When should I find a job and start work? ... It was too much and so I focused on my eternal family. I was grateful for my temple marriage and knew I would see my son again, if not in this life then in the next. I was grateful that God had blessed me with 3 precious days with Ethan before his surgery. I was grateful that I was able to hold him for 3 hours that morning instead of going home to sleep. I was grateful that I had no regrets. I spent as much time with my son as I could before his surgery, I even had my dad take over 200 pictures of him before surgery, and there was nothing I wished I had done. I was at peace. Still broken and hurt as I waited for his time of death but at peace. And then... there he was. A team of doctors and nurses that had been with Ethan the whole 21 hours of surgery was sprinting through the halls pushing monitors, IV poles, and a warmer with my baby on it! He did it!!! I can't describe my emotion because I was numb. I had hit the lowest low and he was in a critical condition, I didn't want to get my hopes up. I had already said good bye, how could I say good bye again?? But there he was!! After the priesthood blessing, Ethan stopped bleeding and the doctors took the opportunity to work with Ethan and get him on the path to recovery and brought him up to the PICU.

Additional Tube Placement
Ethan was in such critical condition after he stopped bleeding that the surgeon didn't even place all the tubes for recovery from surgery. On July 8th the PD Catheder, sponges, chest tubes, different sized retractor, and pacer wires were all placed. On July 10th he was awake!! Then, July 13th, one week after surgery, his chest was closed. July 14th the PD Cathedar was removed, July 15th the upper chest tube was removed, July 16th the IJ (central line) and pacer wires were removed... slowly but surely he started to heal and stabilize!!

Vital Organs (Heart, Brain, Kidneys)
His vital organs all took a HUGE hit during surgery. The doctors were very concerned with his heart, brain, and kidneys. For his heart, he had 6 echocardiograms in the first month alone. His heart function, as measured by BNP level, was at 2,970 (normal is below 99). For his brain, he had 3 MRIs and 2 EEGs. The findings included a venous thrombosis on his left vein, issue of old blood or calcification throughout the brain, and a small right carotid artery. For his kidneys, they were severely injured and full recovery was questionable but there was hope because he produced a couple drops of pee a few days after surgery but not even close to normal.

Feeding
A week after surgery, for the first time in his life, Ethan was fed breastmilk through his ng tube on July 14th. It was the best feeling for me because I felt like I was finally useful! I could feed by baby!! However, on July 20th Ethan developed chylothorax and he had to be fed a special formula called Enfaport for at least 6 weeks as projected by the doctor.

Extubation #1 and #2
Then, Extubation #1 took place on July 17th and only lasted 2 hours. Extubation #2 took place on July 25th and he got as far as a nasal cannula on the wall, no extra support. We were so excited and started talking about leaving the ICU to another floor on July 31st but the chylothorax came back. His heart function was worse that day with a BNP level at 3,059 and his breathing quickly got worse until we found ourselves intubated on August 1st.

Catheterization
Since we didn't know what went wrong with extubation #2 and why Ethan had to be intubated again, we did a cetheterization to get a better idea of his heart function. The big takeaways included: severe LVEDP, arch obstruction, and pulmonary hypertension. Another surgery was recommended to fix the aortic arch however, the surgeon preferred not to do the surgery because of how Ethan handled bypass during the first surgery and because of how fresh the incision was. The doctors explained that we could either do surgery or get a trach to help with breathing and wait a few months until the incision from the first surgery healed to do the second surgery.

Extubation #3
Because of the concerns with a second surgery we chose to extubate one more time. Deep down, I knew Ethan could breathe on his own. I didn't want a trach so if he didn't last being extubated we would proceed with the second surgery. He was extubated for the 3rd time on August 17th and he made it 1 week before his work of breathing increased and we had to intubate again. So that meant we were going to do the surgery but we knew that he needed the surgery and going into surgery, we didn't have any doubts or thoughts that he might not need the surgery. We had tried and we knew that he needed this surgery.

Surgery #2
On August 26th Ethan went in for his second heart surgery to do an aortic arch augmentation with a homograft patch. This surgery only took 9 hours instead of 20 and was a success - he came back with all his tubes for recovery and his chest CLOSED!! It was so successful that we thought we might be home two weeks later. By August 28th he had his central line out, September 1st surgeon removed pacer wires and right chest tube, September 3rd surgeon removed center chest tube and by September 9th the left and right Pleural catheders were removed.

Infection
Unfortunately, we didn't make it out of the hospital 2 weeks after the second surgery because Ethan's incision got infected. We first noticed the infection on September 1st so we hit it with antibiotics but it didn't start oozing until September 2nd so we could collect a culture. The culture came back negative which meant we couldn't identify the infection and would need to continue broad antibiotics. If we knew what it was we could target that specific bug and narrow the antibiotics, but we didn't so broad strong antibiotics it was. BEcause of the location of the infection right on top of the sternum, we had to assume the bone was infected. A bone infection would require at least 6 weeks on antibiotics. However, since the bone had foreign material (Stainless steel wires from surgery) the antibiotics were to be continued over 3-6 months. So 3-6 months on a broad antibiotic only distributed through an IV meant a long time in the hospital or being sent home with a PICC line. We did a wound vac on August 5th - August 9th to clean out and help close the incision but because the bone was possibly infected, we had to continue antibiotics.

Extubation #4
Before discovering the infection, we extubated 5 days after the second surgery on August 31st. By September 3rd he was intubated again. That was unexpected because the second surgery was supposed to make it so his heart didn't have to work as hard and he could breathe better. Because he had to be intubated  again, we questioned if the issue was more lung related than heart like we had thought. So the cardiologist was going to do a trach but we again pushed for one more extubation. One of the other cardiologists pointed out that we should treat extubation #4 like his first extubation since it was the first extubation after the most recent surgery. And he was right.

Pulmonology Breathing Sprints
Before trying another extubation, we consulted pulmonology. The pulmonologist pointed out that Ethan had been intubated most of his life and didn't have the muscle strength to keep his lungs open on his own. So we came up with a "weight lifting" program to help strengthen his lungs. All the respiratory therapists freaked out when we mentioned breathing sprints without any pressure support. It is difficult to breathe through a straw which is what the breathing tube is without a little pressure support but the idea was to strengthen his muscles and the only way to do that was to test it. So we did a slow and steady two week program that did these no pressure support sprints 3 times a day. We started with 20 min and then increased it by 10 minutes every day, then thirty minutes every day, then eventually an hour every day until by the end of the two week program he was breathing on his own 18 hours out of the 24 hour day.

Extubation #5
We extubated him after the 2 week program on September 21st and he slowly worked his way from high flow down to the nasal cannula on the wall. He was on room air the whole time too which impressed several doctors and nurses. He didn't need extra oxygen! The 21% oxygen in the air was enough to keep his oxygen concentration in his blood at 100%. That was really exciting! 3 days after extubation, Ethan started bipap at night on September 24th. This was an idea that one of the doctors had just to give him a little break and extra support at night so he could continue on the next day. Well, it worked!

Surgery #3
Two days after being extubated, we noticed a sternal wire poke through the skin on September 23rd. The surgeon came in to take a look at it and said that he needed to surgically remove the wires and would have to do so in the operating room. It was a bummer that the wire didn't poke through 2 days earlier when he was intubated but we worked around it. Since the surgeon was going to be in the incision removing the wire poking through the skin, he decided to remove all the sternal wires to reduce the foreign material. He scrapped off a sample of the bone to be tested for bone infection and it came back negative. This sternal wire removal surgery got Ethan off the antibiotics that were only distributed through the PICC line. So we were one step closer to not needing the PICC line. The only thing left was milrinone.

Milrinone
Ethan had been on milrinone most of his life. When we tried to ween him off milrinone (a heart medication) in the past, his x-ray increased in cloudiness or his heart function decreased. So this time around, during the month of October, we did a similar weening process as the breathing and did a slow ween over 2 weeks decreasing from .75 to .65 to .5 to and so forth until he was completely off milrinone. We checked his bnp level which had dropped to 224, the lowest it had ever been, which meant his heart function was more normal than it had been in July. Oh it was amazing! He has been off milrinone since October 14th and is doing so well!

Feeding
So Ethan can breathe on his own, he is no longer on any IV medications like the antibiotics and milrinone, he was tube free except for the ng tube. We've been working with Speech to get Ethan to eat with a bottle. His first bottle feeding was September 23rd and he drank 6 mL of breastmilk. Oh, we started breastmilk back up after enfaport on September 17th. That was huge!! I was so excited again and he started to put on some baby fat in the legs and arms. So exciting. He likes the breast milk much more than enfaport and he is less gassy which is so nice. Well, in order to go home, Ethan needs to be eating. The most he has taken has been 20 mL which isn't enough to support himself at home so he needs to be fed through an ng tube. I did some searching and determined that Ethan would do better with a g-tube. Inserting a PEG was not feasible for EThan because it is considered a somewhat "Blind" procedure and with all of Ethan's surgeries and scar tissue, his stomach and intestines may have moved and there is a fear of puncturing the intestines rather than the stomach. So the only way to get a g-tube would be another surgery.

Surgery #4 and Extubation #6
The fourth surgery was g-tube placement occured today October 20th. It only took an hour and he was intubated for the procedure but came back to the room extubated. Hurray! So now Ethan has a g-tube button on his belly where the stomach is and that will be how he gets his feedings and medicine. This is the first time since birth that he doesn't have anything on his face!! WOW!! He looks so good and cute. IT'S AMAZING!!!

So there you have it. We are being discharged from the hospital on Thursday October 22, 2015. We will be going home with 9 medications, a feeding pump, and a bipap machine. We are excited to meet with you next week. Ethan has come so far! He is a fighter and one incredible kid. He's my hero and a miracle and I sure do love him! We have been through a lot and Ethan is a totally different kid than he was back in July after his first surgery. His vital organs have bounced back and he is right on track developmentally, we will continue to work with him and love him and will appreciate your help and guidance as his pediatrician.

Thanks,
Amanda Chamberlain
(Ethan's Mom)