Surgery Number 17

It's been five years since I have updated June's blog. Thankfully, that's because we have had five years of our new "normal". However, this summer took some unexpected turns which led to some new discoveries and questions. And when it comes to June, we have learned there are always more questions than answers.

Since it has been so long, I'll start with a quick recap of our journey with June and then share the recent findings:

We found out very early (24 weeks in utero) that June was going to be born with some complications. June and her twin were competing for resources the first 12 weeks after conception and both very behind. I was being very closely monitored since I was pregnant with twins who were behind in growth and because of a previous miscarriage. Although June managed to power through, her stomach was not showing on any of her ultrasounds (and there were oh so many). The doctors believed she had Tracheoesophageal Fistula - a section of her esophagus was missing so she was not swallowing amniotic fluid so her stomach would show. It is very difficult to diagnosis a TE Fistula in utero but with her lack of a stomach showing and increased amniotic fluid (leading me to measure 38 weeks by 24 weeks), it was definitely a probable cause. In 10 weeks, I had three amniotic fluid reductions (a needle the length of your arm from your elbow to wrist place in my stomach to pull amniotic fluid so I didn't go into premature labor) and a couple close calls for going into labor early. We welcomed our beautiful baby girl four weeks early on July 23, 2015, and the fun began. 

June had surgery to repair her TEF at 40 hours old. We were told everything went as planned and we would be heading home in 4-6 weeks. Unfortunately, everything did NOT go as planned and it took 117 days (four months) to bring our girl home. In that time, we had so many close calls: three emergency surgeries, a wound vac (that was new for the NICU), code Blues (first skin-to-skin contact resulted in several emergency people in our room resuscitating her), seizures, and fun discoveries (like three holes in her heart). 

My past blog posts from 2015 detail these fun times in detail so I'll summarize what we learned. Our daughter does not have a diagnosis. She has several similar physical birth defects to babies who are born with a trisomy chromosome without the third 13th, 18th or 21st chromosome.  She was born with three holes in her heart, but her heart has figured out a way to function with those holes so she did not need
surgery. She was born with large ventricles in her brain, but they never filled with fluid so they did not

need to be drained. She is missing a set of ribs. Since a section of her esophagus was missing, her stomach did not fully develop. It also was cut into so many times during her surgeries that it does not function at a "normal" level. June was tube fed for many years (first in her intestines and then her stomach) to compensate. Her intestines did not correctly rotate, and she had a couple other blockages along her GI tract. An infection was sewn into her midsection that lead to her abdomen to split open from one side to the other and from her chest to her naval at just a month old (reason for the wound vac).  We have worked with many specialists throughout the years including pediatric surgeons, cardiologists, neurosurgeons, neurologists, physical therapists, feeding therapists, pulmonologists, ENTs, and geneticists. June has been a patient at Peoria's Children Hospital, Lurie's Children's Hospital and St. John's Children's Hospital, and we are pros at packing for hospital stays that last at least a week and securing
childcare for our older son Sawyer in record speeds. I started this blog to share with our world that June is quite simply the most amazing miracle we could have dreamt up. We are so lucky that so much spunk and fight can be encapsulated in such a petite, 38-pound seven-year-old. She is our idol. 

Fast-forward to 2020 and Covid. The last two years have been filled with anxiety and sleepless nights for everyone. At what point do you take precautions so you can protect life? When do those precautions take a toll on living your life? For everyone the answer is different. The last few years have taught us that there is a delicate balance between protecting and pursuing life. June is one of those who are at a significant risk when it comes to contracting Covid. It could be fatal for her. Unfortunately, June has to fight an uphill battle to live, and now she is fighting an uphill battle in society to live. 

Luckily, our precautions have paid off so far, and we have not had to worry about the effects Covid could have on June. However, this spring, June's oxygen levels started dropping. Long story short, she was chronically wheezy and after a couple of visits to Pulmonary, they decided she needed to be hospitalized. 

We spent a week in the luxurious St John's Hospital resort in Springfield during June. Lots of testing was done with no answers. Several treatment plans were also attempted but no results. They decided to do a test of her lung function. Very grim doctors came to tell me that June's lungs were only functioning at 33% as she sang along to Sing 2 on the hospital TV. No one could figure out how she was functioning at the capacity she was. Finally, we were released because she was not making progress.

At our follow-up pulmonary appointment we found out that someone had entered June's height as 4'6" so that drastically impacted her lung function results. Her lungs are actually functioning at a 66%, which is still very low but much better than expected.

Around the same time, I was rubbing her back and noticed that she was very crooked. An X-ray showed
that June has a 33 degree curve in her spine currently. We were told that anything over 14 is significant, and she was officially diagnosis with scoliosis. In July, we went to an orthopedic surgeon. June has never had a back workup done (which probably should have happened when she was born because of her enlarged ventricles in her brain), so the next steps to determine if her scoliosis is primary or secondary. Primary scoliosis is caused by her spine not being formed correctly, tethered cord (spinal cord being caught in the spine), or fluid on her spine. All of these will require surgery. Secondary scoliosis can be caused by a chest cavity surgery (June's TEF repair on her first day of life). The MRI and another X-Ray will help determine which type of scoliosis it is and to proceed with treatment.

The rest of the summer was not as eventful as the month of June, but June Bug continues to struggle with breathing, especially at night. 

Tomorrow we are headed up for June's 17th surgery. She is having her feeding tubes removed after seven years, some repair done to the scars from her first surgery (her muscle is sticking to the bone), an EndoFLIP (a procedure that measures the area across the inside of her esophagus and the pressure inside), an Endoscopy (to look at the inner lining of her esophagus and take some biopsies), and an MRI workup of her back. We are going to have a consult with pulmonology in Chicago to talk about next steps with her lung function as well. 

We hope that the next few days will provide us some insights on why June is struggling and what we can do to help her. I'll post more this weekend.