Summer's CDH Story - Left CDH - Liver Up
Summer was born via c-section on January 23rd, 2015. She was our dream come true. On September 5th, 2014 my husband and I headed to Evergreen Hospital in WA State for our 19 week anatomy scan. We had heard their machines and facility were wonderful so we decided to make an experience out of it and go check it out since we also wanted to give birth there. We were anxious to finally found out if we’d be welcoming a baby boy or a baby girl in the coming months. During the ultrasound our little one wasn’t cooperating according to the tech so she went to get the doctor. All I could think in that moment was how bad I wanted to leave here knowing for sure what we were having! Come on baby! The doctor came in, quickly flicked the ultrasound wand down, and declared that she’s definitely a girl.
We had a few seconds of bliss and excitement and then he said but... and everything he said after that BUT was in a foreign language to me. Her stomach was up next to her heart. There’s a hole in her diaphragm. Reduced lung growth. Other organs in her chest. He said she won’t be running any marathons. One of us asked... ok so is that it? Just maybe no sports? She’s ok right? And then he told us that CDH has around a 50% survival rate, there’s a possibility that she has other physical or chromosomal defects, and that today was just the start. He said I’ll give you a moment and when you’re ready we will meet you in the genetic counselors office to get started. I couldn’t control my sobs and tears were streaming down my husband’s face. How is this happening? What are we going to do?
In the coming weeks we met with many doctors and fortunately met our daughter’s future surgeon, Dr. Riehle, at Seattle Children’s Hospital. She answered my entire list of questions, walked us through everything we would be doing throughout the remainder of the pregnancy and after the delivery. She referred to me as a mom already and she eased everything inside me as we prepared ourselves for our new world. We found out that our girl had a large hole in her diaphragm and her stomach, intestines and 10% of her liver were up in her chest cavity. She had very little left lung and her heart was pushed so far over to the right that it was touching the wall of her chest on that side.
We transferred all maternal/fetal care to UW Medical Center where I would be giving birth and our baby girl would transferred 1 mile away to Seattle Children’s Hospital once she was stable enough for transfer. On Summer’s birthday she was still completely breech so they took me in for a c-section. My husband and I held on to the hope that she would come out crying but the room was deafeningly quiet as she was pulled from me and rushed to the resuscitation room with my husband at their heels. She was immediately intubated and her numbers started climbing as life entered her fragile body. My husband came back to me and let me know that she was ok. Eventually they wheeled her into my surgery in an incubator and let me see her perfect face. Shortly after she was sent to Seattle Children’s Hospital one mile away. My husband went with her and soaked up all the information over the next two days while I recovered.
I left UW - Medical Center and headed their way (a quick 5 minute drive) after two days and the next day she was having her repair surgery at 4 days old. After surgery she was struggling with her blood gasses and they had to put her on an oscillator. After about 12 hours she was back on her regular vent and on the road to recovery.
The first time I ever held my first born baby was when she was 8 days old. She was still intubated and she was covered in tubes and wires, but the moment was amazingly perfect. At two weeks old she was extubated and she graduated from the NICU to the surgical floor at one month old. After 6 weeks in the hospital we took our love home with an NG tube for feeding. Feeding was her biggest hurdle and for a long time after coming home we struggled to get her enough calories because she was consistently vomiting her feeds. She was on a 24 hour pump and anytime we tried to give her more food or more calories so we could take her off the pump for an hour or two, she would get sick.
At three and a half months old Summer was admitted back into Seattle Children’s Hospital for her constant vomiting. We were told often before this that she is just a reflux-y kid but I followed my instincts and pushed hard to get her admitted. By the time she was fully admitted to the medical unit she was severely dehydrated and producing only crystalized urine. She was throwing up every hour and the vomit had quickly turned from milk to bright yellow bile to green waste. After 24 hours she was transferred to the surgical unit after more advocacy from me and she was given a repogle tube to constantly suck everything out of her stomach and stop the vomiting. After 3 long days inpatient we got in touch with her CDH surgeon who immediately realized that Summer had a bowel obstruction that was likely caused by scar tissue from her CDH repair surgery.
The next day our brave little hero headed back into surgery to repair her bowel obstruction and to place a g-tube so we could go home with no NG tube. We spent 2 weeks in the hospital at this time and then we went back to being home as a family and restarted our new life. The first year was a tough one as every sickness seemed to cause Summer trouble with her breathing and/or her feeding. She was and still is a reflux-y kid and that took some getting used to but she is an absolute trooper through it all.
She was hospitalized 3-4 more times in that first year from bronchiolitis, colds and RSV but has never been hospitalized again after her first birthday. She was able to get her feeding tube removed at 2.5 years old after lots of feeding therapy and lots of hard work.
Today, Summer is a happy, generally healthy four year old tiny hero. Medically, she is a rock star and powers through her many tests and procedures and her many bouts of sicknesses. She is prone to bronchiolitis and has rough winters but we have remained at home through it all. Summer has been diagnosed with asthma, for which she takes both a controller inhaler and a rescue inhaler, GERD (acid reflux), for which she takes omeprazole, pectus excavatum, chronic constipation, and her main diagnosis is pulmonary hypoplasia (underdeveloped lungs). We recently found that her right lung is doing 78% of the work and her left lung is doing only 22%.
Summer is entering her second year of preschool this year and is keeping up with her peers beautifully. She loves to talk to anyone with a listening ear, she loves outer-space and learning about the planets, and she loves her family, especially her little brother. She loves mac and cheese, sweets and a wide variety of foods after struggling so long to eat and drink. She is incredibly strong in so many ways and we are so thankful to her team at Seattle Children’s for giving us the chance to love on our little girl.