5 Attachment(s)
Johnson, Lilliana "Lilly" Michelle
When I first found out that my baby girl was diagnosed with CDH I searched for information and I found the Cherubs website that was full of information and stories about both survival and loss. I had really hoped that I would someday be writing a story of survival. Unfortunately that is not the story that I am here to tell today.
My dear, sweet Lilliana Michelle was due on April 4, 2015. When we went for our anatomy ultra sound on November 3, 2014 I started to become concerned because the tech kept looking at her chest. Then when the high risk doctor came in I knew something was very wrong. I for the first time heard the words congenital diaphragmatic hernia. Having no idea what CDH was, I asked very basic questions. The doctor explained in very general terms about the condition and what tests she thought I needed to get done, but did not tell me about the prognosis. When I asked her about when I needed to get the tests done, she asked me, much to my shock, if I wanted to terminate the pregnancy. I couldn't understand how/why she could ask us that question when she had just seen our beautiful little girl wiggling all over the screen. I left the office thinking she had a simple hernia that would require surgery. Little did I know what a CDH diagnosis really meant.
After leaving the office I started the google search. It was then that I was hit with the knowledge that this hernia that the doctor had described actually had the chance to be fatal. I spent that night crying and frantically searching the internet for information for hope. I registered on the Cherubs website and found the cherubs group on Facebook. I reached out asking other parents what I needed to know and what questions I should ask. I also found a website that talked about a doctor named David Kays who had a better survival rate than what I was seeing on other sites. In response to my post on the Facebook page, I received many messages telling me to go see Dr. Kays. Then I got a message from Joy, Dr. Kays' program manager so I gave her a call. She provided me with a better understanding of the condition and all of the information I needed to make a decision about treatment. After briefly considering the best local hospitals in my area, it was clear that going up to Dr. Kays in Gainesville was the right choice. After testing we learned that Lilly did not have any additional chromosomal disorders, but her CDH was very severe. All of her organs, intestines, stomach, and liver were in her chest. It was going to be a battle, but Dr. Kays believed that she had a chance at survival and he promised to do everything he could to make it happen.
We had planned to start Lilly's induction on March 23, 2015 but my little princess had other plans. I had an excess amount of amniotic fluid during my pregnancy, which is common with CDH and the pressure had caused my water to leak so Lilliana made her appearance in the world on February 27, 2015. She was tiny at only 4 lbs 1 oz. and I was so worried but Dr. Kays assured me that he had done surgery on smaller babies so her size while a concern, was not an insurmountable complication. Lilly struggled very much on her first day of life and was placed on ECMO before the night was through. The ECMO was a life saver. It allowed her to rest and grow strong. Dr. Kays had initially planned to complete her surgery that Wednesday while she was on ECMO, which is a risk, but he later changed his mind because she was improving and he thought that he could wean her from the ECMO before surgery which would be less risky. She was weaned off of ECMO on Saturday and had her Surgery on Sunday.
I was thrilled that she was able to get off of ECMO before surgery, but even more so when Dr. Kays came out of her surgery and told me that had we done it on Wednesday that she would not have survived. Her tissue was so fragile that it tore and bled during surgery which would have been the end if she had the blood thinners from ECMO in her. Luckily he was able to stop the bleeding, but that was not the end of the concern. Lilly was so small that Dr. Kays could not get all of her organs into her abdomen and close up her stomach muscles. Even with only her skin closed it was a very tight fit and he was not sure that her organs would be able to function. He advised us that it was critical that she urinate within the next few hours because it would be a sign that things were working. He told us that Lilly had almost no diaphragm, approximately 2%, and so she required a large patch. Additionally the next 48 hours would be critical. He advised that if she needed ECMO again, that he did not think she would survive because she was just too weak. I will never forget the excitement on the doctor's face when my little girl gave him a sign that things were working. He was yelling "we have pee!"
From that point on, Lilly began to improve slowly. Day by day her numbers got better, medications were weaned, and ventilator support was decreased. We celebrated as she graduated from the vent to high flow and down to the regular O2. I was thrilled to get to hold her for the first time on March 23rd, which was her due date. Then we started feeding. This was the long and tedious part of the battle that I did not expect. We spent a long time with P/T working on getting Lilly to eat enough by mouth to gain enough weight to get discharged. On May 20, 2015 Lilly was released from the hospital. I could not have been more excited to take my girl home and reunite our family.
On Friday March 29, 2015 I was due to return to work from maternity leave so the night before my mother came and spent the night as she was going to be the one to take care of Lilly for my first day back at work. I sat down to feed Lilly and was amazed at how well she was eating. Then all of a sudden she spit up and as I wiped her mouth I realized she was not breathing. She immediately went limp and started turning blue. I screamed to my mother to call 911 and I frantically began CPR. After what seemed like forever I was able to get her breathing again. She was rushed to the local hospital where they said that they thought she had aspirated while eating and recommended the nissen fundoplication surgery to keep her from refluxing. She was transferred back to Gainesville where Dr. Kays performed the nissen surgery and also gave her a g-tube so that we could make sure that she was getting enough nutrition. The last Saturday in June she came home again, just in time to celebrate 4th of July with the family.
Back at home we settled into a routine. I returned to work. Things seemed to be getting back to our new normal. I work three Saturdays a year so since I had to work on Saturday the 15th of August, we decided that my mom and dad would get their first sleep over with all three of the grandkids. That Friday night I came home from work then went out for pizza with my parents and the kids. After dinner they dropped me off at my house. I fed Lilly and changed her diaper in the car, gave the kids kisses and sent them off. I had no idea that would be the last time I saw Lilly smile up at me with her big brown eyes. When my mom got home and settled in, she fed Lilly again. This time she stop breathing and went limp after the bottle. My dad was able to get her breathing again while my Mom called 911 and called me to let me know what had happened. It is over an hour to the town where my parents live so my brother in law drove me since I was too frantic to drive. The nurse at the hospital called me to ask questions and told me that they had intubated her and were working to get her stable.
When I arrived at the hospital I ran through the halls of the ER to get to her and as I entered her room I instantly knew that something was terribly wrong. They were bagging her. At some point after they had her intubated, her heart stopped. They were trying to get it started again. I held her hand at her bedside while the doctors and nurses tried everything they could to get an IV in so that they could administer drugs to help their efforts, but it was to no avail. After what seemed like a very long time, they did an ultrasound of her chest to show me that there was no activity. The nurse just looked at me and shook her head. I screamed. After such a long stressful battle I could not believe that I had lost her so suddenly and without much warning.
I always said that my biggest fear in life was losing one of my children because I did not understand how any person could continue on after that. It has not been easy, but day by day and moment by moment I get by. I am all too familiar with the deep, soul crushing sorrow of missing one of your children who is forever lost to you. Still I am forever thankful for the 169 days that Dr. Kays gave me with my sweet little Lilly. There are far too many CDH parents who never get the chance to take their babies home. There are many who hold their children for the first time when they make the gut wrenching decision to stop treatment and say good bye. CDH is not just a hernia. It is a very serious health condition that takes the lives of hundreds of children a year and has lasting impacts on those children to make it through their first year. In Lilly’s honor I will continue to spread awareness and provide support where I can to other families. Thank you for taking the time to read her story.
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