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.

June, July, August, September & October Update

We are way behind on writing an update on June. I kept putting off writing, thinking that June might have some significant progress after her surgery that I would be able to write about. Unfortunately, things have been status quo in regards to June's digestive system.

June, July & August

We actually had a wonderful summer! We got out more since we didn't have to worry about germs and viruses with the warm weather and being outside. Most of June and July were spent outside at parks and the pool. June absolutely loved going to the pool. She enjoyed the kiddie pool, but was very taken with floating in the big pool too. While we would hold her upper body, she would kick her legs like a frog and laugh. June also enjoyed being at the park with the rest of the kids. June doesn't get much social interaction, so it was great to have her out around others. We enjoyed going to a wedding as a family, and June tore up the dance floor. We even managed to have a fun day at the St. Louis Zoo, celebrating birthdays with family and godparents without any medical emergencies.

In July, we went to Chicago for testing. We attempted a mini family vacation since we had a couple days of testing. The first day, we met with a pediatric pulmonologist in the north Chicago suburbs. Since June wasn't sick, it wasn't necessarily a productive appointment, but our surgeon wanted us to get connected with one since she is so sick so often in the winter months. The pulmonologist explained that June's constant illnesses could be explained by a couple reasons, and that he would want to meet with us more often closer to the winter months. Last winter, we used June's breathing treatments as a reaction to her getting sick. The doctor recommended that we start her breathing treatments at the end of October/beginning of November and give them twice a day until April. The rationale behind this was that the medicine would stimulate June's airway to keep it open. With June's tracheomalacia (where the tracheal support cartilage is soft that the trachea is able to partly collapse - in June's case, this is where her esophagus was detached from her trachea), a common cold, runny nose, or cough quickly becomes a serious upper respiratory infection leading to June not being able to breath. Last year, June was sick from November to April - we would get her over an infection, only to have her catch another one. She ended up on an oral steroid every month and even multiple times a month. Her pediatrician told us that the negative side-effects of oral steroids being prescribed so often is that they can stunt a child's growth.


After the appointment at the pulmonologist, we headed to Chicago for a speech appointment. At this point, they don't think June is aspirating, but they wanted us to continue to add Thick-It to her water just in case. After our appointments, we went to the Maggie Daley Park. Sawyer had a blast running around on the playground, and June enjoyed the swings. While swinging, someone told us they were giving away pints of ice cream (gelato). June loves ice cream, and can actually it eat, so it didn't take very long to convince her to ditch the swings for some vanilla gelato. For dinner, we were able to meet Andy (Jason's childhood friend and groomsman), Alicia, and kids for pizza.

The next day, the girls dropped the boys off at the park again and headed to June's surgery appointment. We had June's tubes changed. June hadn't been vomiting or spitting out her food for about three weeks, so it seemed like the nissen had finally loosened and everything was going as planned. Of course, the next morning, June vomited five times as we were trying to leave the hotel, including right in the middle of the lobby floor (facepalm). After the appointment, we were able to meet up with Jeanne and meet baby Collette since they were on the Northwestern Medical Campus for a class. We then headed to Lincoln Park Zoo with Kelsy and had dinner at the Rainforest Cafe with Spike and Kelsy. It was awesome to see the possibilities that we have ahead of us when it comes to taking a vacation. I had an entire suitcase dedicated to June's medical supplies and still managed to forget her feeding bags. Luckily, we were able to get some bags from our surgery appointment that we could use to feed June. So note to self, triple check the packed bags!

Throughout the summer, the vomiting has continued. We would have two to three weeks of nothing, and then one day, June would vomit more than seven times. Although we haven't made much progress with June's eating, we started to see her excel with her speech. By the end of summer, June was stringing a couple of words together for short sentences. June also recouped her lost physical skills from her hospital stay in the spring and got back on track.

September & October

The month of September swiftly brought us back to reality. By the middle of the month, June was very sick. She was coughing so hard, she could barely catch her breath between episodes. Nights were rough - many nights, we were back to her sleeping on my chest while I sat propped up in a chair so she could breathe. It started on a Friday when she woke up slightly warm. At this point, we had already started doing breathing treatments with a steroid twice a day, but June was starting to develop a harsh cough. Although June only had a temperature of 99°F, I had a sinking feeling we were heading toward something more serious. Our pediatrician gave us amoxicillin and an oral steroid. After a week and a half of medicine, she wasn't getting any better, in fact, her cough was getting worse. She was coughing so hard she was vomiting several times a day, and she wasn't able to keep food down because the thick mucus was clogging her esophagus.

We ended up on another oral steroid. Since she was so sick, we also made a trip to Springfield to see June's pulmonologist. (Oh, yeah, we decided to change June's pulmonologist to one closer to home since we anticipated a rough winter. Our surgeon recommended a pediatric pulmonologist in Springfield who she works with on difficult cases residing south of Chicago.) He decided that we should get some testing done with June. The first round included a sweat test to see if she had cystic fibrosis and blood work to test for autoimmune disorders. Since we were headed up to Chicago in October, he wanted her labs done at Lurie's Hospital. He also discussed ordering an airway clearance therapy vest. The vest is designed to assist with the mobilization of retained secretions that, if not removed, may lead to increased rates of respiratory infection, hospitalizations, and reduced lung function. It would simulate the respiratory therapy June receives when she is hospitalized. The therapy vest is very expensive (in the range of $15,000-$20,000), so we are hoping we can get it cleared through insurance and prevent future hospitalizations.

With the exception of a day or two of reprieve, June was sick for most of September and all of October. At the end of October, we headed to Chicago for June's surgery check-up. After talking to June's Speech and Feeding Therapist about tube feeding, we decided to ask to switch from Pedisure to blended diets. In her experience, kids' bodies created a lot less mucus on blended diets (potentially too much diary?). We met with a dietician, and she was on board. While Pedisure has all June's vitamins and nutrients to supplement her inability to consume enough by mouth, it has a lot of unnatural ingredients. June's new blended diet, although pre-made, would be from all organic ingredients.

At this point, we are desperate to try anything that will help June stay healthy and out of the hospital. We are also worried about being able to provide June a "typical" life of a toddler. We want her to be able to go to preschool and be enrolled in some activities, but right now, that doesn't seem possible. Last year, June was sick from November to April - she was on an oral steroid every month (which is concerning because she is building up a tolerance and it can stunt a child's growth). This year, her illness have started earlier and so has the medicine even though June has limited exposure to other kids.

At the end of October, we also had June's re-evaluation for Early Intervention services. June did really well in her evaluations. She is communicating well above her age, so we decided to stop speech services. June's speech therapist is also her feeding therapist, so she will continue to work on speech during her feeding sessions, especially on days when eating isn't going well. June is also caught up in her physical abilities. We are continuing physical therapy service over the winter because when June was hospitalized last winter, she lost about 10% of her physical abilities.
If June is hospitalized this winter, we want to make sure she has services in place to help her gain lost skills. Obviously, June is still behind on her feeding skills, so she will continue to receive feeding therapy twice a month.

Thankfully, June managed to get healthy in time to enjoy some Halloween fun before she caught another cold:

April & May Update

The last two months have been a whirlwind as I wrapped up the first school year at my new job, so I am way behind posting updates on June. Unfortunately, her progress has seemed stagnant to us. While she has been able to keep her J (intestine) tube feeds down at night, she has continued to vomit multiple times a day during meals. Some days she won't vomit at all and eat a lot of food (in June's case that means eating two meals and a snack a day), and other days, she can't keep a puff down, vomiting seven to ten times a day. At our April check-up, we were told that the vomiting was normal as her esophagus was recovering from the surgery. The surgeon also explained that she made the nissen very tight so it may not be letting food through. June came home from her hospital stay with RSV, so they thought  all the vomiting was because of the congestion from her illness that she couldn't get up or down. 

The surgeon decided to use a swallow study and esophagram to exam the function of her esophagus. June is very familiar with the room where they perform the studies, and she was not thrilled to be there. She didn't cooperate for the swallow study, but they did see her aspirate (breathe into her lungs) thin liquids, so they recommended that she no longer drink water. They weren't sure if the aspiration was because she was upset or if there is something wrong with her swallow. She was sent home with instructions to drink liquids the consistency of smoothies or nectar. We bought Thick-It, which is a product that helps people with dysphagia drink liquids. The dry mix thickens liquids without adding flavor or texture so she can drink water without it going into her lungs. The esophagram showed that her esophagus isn't working "normally". The surgeon said that this isn't surprising - her esophagus (and any TEF baby's esophagus) doesn't contract regularly because the nerves from the two different sections never learned how to communicate correctly; however, her esophagus function did seem to be struggling more than they originally thought. They plan to keep an eye on it, and depending on her progress, will determine next steps that need to be taken. Worst case scenario is that June's esophagus will need to be replaced with a section of her intestines, but we were assured that she was a ways away from this procedure.

In May, we traveled up to Chicago for another appointment. June was still vomiting multiple times a day but was no longer sick. We have gotten really good at knowing when it is coming so we can dive across the room and catch it, and we were able to catch it on video so we could show June's surgeon. When they saw the video, they agreed that what was happening was not normal. We also mentioned our concern about how often June gets sick. This past winter she had RSV twice and pneumonia. She had some sort of a respiratory infection every month to the point that she was on some sort of an oral steroid or inhalation steroid most of the time from November to April. We are not sure how we are going to manage school for June because she has very limited exposure right now and is constantly sick during the winter months. 

At the May appointment, we went through our cleaning routine with the surgeon, and she suggested that maybe June isn't getting sick from exposure but from aspirating, which is then complicated by her inability to fight off any sort of bug. They decided they want to admit her again this summer to perform another bronchoscopy to look at how June is swallowing. We've been noticing that she is gagging on her own spit throughout the day, and the surgeon feels that there is something "off" about June's swallow. June's doctor also discussed having another echocardiogram (ultrasound of her heart) done and setting June up with a pulmonologist. Unfortunately, we are having some issues with getting in to see some of these doctors until October, so we are hoping for some guidance on how to everything scheduled in a more timely manner at our appointment next week. Despite all of these complications, June still continues to express interest in food and wanting to eat. We try to make meal times fun and inclusive, but we are very concerned her eagerness to eat will start to subside since she vomits anytime she eats more than a few bites. A couple weeks ago the vomiting stopped for two weeks, and we thought the nissen was finally loosening, but this past week the vomiting came back with a vengeance. There are days I wish we had some sort of a diagnosis because that might mean more structure and some sort of a procedure to follow to treat her. It seems like as soon as we find a solution for one thing, something else develops that wasn't previously presented to us as a concern or possible complication. I don't think a diagnosis would make anything any easier - it just might provide us with some more resources. With all our time in hospitals, we have noticed the committees of medical professionals or parents who have children with certain diagnosis that have dedicated amazing energy to efforts such as resources and treatment for those suffering a similar diagnosis. It seems that the TEF community is smaller and more variant, and we just haven't had the time to research as much as we would like since we are just trying to get through the day-to-day. We did get connected to another woman in the area who has a daughter who has followed a similar path to June. The little girl is a couple years older, so we are hoping this might provide some more guidance on what to expect as a parent. I met her briefly one evening, and she gave us a wealth of information already to look into. We like our doctors, but I think it is hard for them to understand why we are so concerned - they see a lot of different cases varying on degree of complexity, while we just see what June is going through and our inability to provide her a somewhat normal day. Luckily, we have a supportive community that has been overwhelming and amazing in what they have provided our family. We cannot thank everyone enough for their contributions to June's medical expenses and meals. Your words and follows are encouraging enough, but the support we received after June's most recent month-long hospital stay helped alleviate some stress in our lives.

While we have once again realized that this latest surgery is still not the cure-all for June, there are some positives. June is now down to continuous feeds for only 12 hours, so she is essentially on them only during the night. Although she looked adorable in her little backpack, we are happy that some of her constraints are off. She has also been able to maintain her weight. She went from a malnourished 16 pounds to 23.5 pounds in two-and-a-half months. And although it is still a little big on her, she graduated to 18-month clothing before her second birthday. The warmer weather has also allowed us to get out more with June. The summer is in full swing, and June has been going to watch her brother attempt to ball t-ball and soccer as well as taking her dogs for walks (we provide a lot of entertainment for the neighborhood with mommy walking two dogs and two kids) and going to the pool.

She is even getting to participate in an activity herself - the Fun Runs. She loved her first run. It took her a little longer than the others since she took off in the opposite direction, but she was insistent on running the whole thing herself and crossed the finish line in style with a dress, flamingo crocs and flamingo sunhat! 

We also made it to see her cousin Abby graduate from high school (a rare and sometimes nerve-

racking event to be around so many people at an indoor event and not know their health status :)):

We are hoping for a summer full of answers, but hopefully, one we can enjoy as well!

Surgery Update

Let me start with the most important news: WE ARE HOME!!! June was discharged yesterday afternoon - a month after she was first admitted. This was the longest stay we have had since back in the NICU days and triple the travel time. Whew!

Time to backtrack to the day of her surgery and everything that has happened since we've had little time to post much of an update. June's surgery was scheduled for Thursday, March 9. She was put on the schedule for 12:45 (the last scheduled surgery for the day) so our surgeon would have plenty of time to address what needed to be done, especially since this was the first time June's surgeon had operated on her GI tract. We also had no idea how long the surgery would be since they didn't know if they would be cutting her open and performing the surgery laparoscopically. June had a fun morning playing on the 12th floor in the Family Life Center and walking the halls. She started getting hungry, and unfortunately, she started pointing the the cabinet (where she knew we kept the pretzels) and signing for "more food please". At 12:30, we went down to wait in one of the surgery prep rooms. We met with the anesthesiologist and surgeon to answer last-minute questions. They took June back at 1:30. They gave her of dose of "happy juice" so she wouldn't get upset when they took her back. We would get a call from our surgeon's nurse when they made the first cut and then every hour with an update. There was a still a lot of unknown with this surgery, so the update calls were a welcomed relief. At 2:30, the nurse called to say that they had just made the first incision. This incision was at her belly button to insert a camera so that the surgeon could look at her insides. The last hour had been surgery prep and positioning. At 3:30, I got the call that they ended up needing to cut June open instead of performing the surgery laparoscopically (by camera). The incision would run from her naval to her sternum. At 5:00, I got a call that they were starting to close June up, which would take about 45 minutes to an hour.

Waiting to go into surgery

The surgeon came out to meet with me at 6:15 pm. Everything had gone well. June's stomach was on the smaller side but not the smallest stomach she had seen. She was able to pull the stomach up around her esophagus to tighten the opening so that it would be harder for June to vomit. Imagine taking a towel and using your forearms to wrap it up around your shoulders. She also moved the interior placement of June's gastrostomy (G) tube. The button on the surface of June's stomach stayed in the same location, but the balloon inside June's stomach that holds the tube in place was pushing on her pylorus (connection between her stomach and intestine). June had an atresia (blockage) at her pylorus that was repaired in the NICU. The surgeon decided that the balloon pushing on the repair was putting unnecessary pressure on it, and the balloon might be blocking the stomach's ability to drain properly. We also discussed what the future would hold for June. It will be important for June to learn to take small bites (no hot dogs - ever) and wash down the food with sips of liquid. June will need lots of time to eat meals in slow, small bites.

I got back to see a snoozing June at 7:00 pm. We got her back up to the room and settled in her bed. Since they were able to remove the breathing tube, she did not need to go to the Pediatric Intensive Care Unit and could go back to the room we had been staying in. They did, however, give her a nasal cannula because they wanted to make sure she took deep enough breaths and didn't stop breathing because of the pain from cutting into her abdomen. The first night was very rough for June. She was in a lot of pain - anytime she moved or her bed was bumped, she screamed and shook. There were some hours that she screamed out in pain every couple minutes. She was on a continuous morphine drip and had a pain button that I could push for her every eight minutes. Some hours she was able to tolerate the pain with just the continuous drip, but there were several hours where I had to stay up to push the button every eight minutes. June was also very congested, and she started vomiting thick secretions that she would gag and choke on because she couldn't swallow them with the swelling in her stomach or throw them up because she was in too much pain to wretch.


In the morning, our day nurse helped tremendously with getting June more comfortable. She got her pain medication switched to a different narcotic that made a difference in less than an hour. She also contacted the doctors regarding June's respiratory problems. They prescribed breathing treatments every six hours to thin the secretions so June could breath and have an easier time of geting them out of her body. Once June's pain was under control, we got her out of bed and into my lap so we could cuddle for seven hours. The next night was rough for June too. She spent most of the night gagging and vomiting. She had a desaturation of her oxygen levels after one session, so they increased her oxygen and took a chest X-ray.

Each day, we started seeing more and more of our little girl's lively personality coming back. She still struggled with the thick secretions that were lodged in her throat. She ended up with the nasal cannula being put on at night because that is when she struggled the most with breathing. After 48 hours (Sunday), they tried giving her Pedialyte in her G tube. Two bags were connected to her G tube with a splitter - one held the Pedialyte and the other was where excess Pedialyte, gas, and air could escape. All of the Pedialyte ended up in the excess bag, so they stopped feeds. The next day (Monday), they tried again to give her Pedialyte. The same thing happened again; however, after they gave her erythromycin (the medication we give June four times a day so her stomach nerves are stimulated and work properly), her stomach started processing the Pedialyte. On Tuesday, they switch her to Pedisure and started working the rate up slowly. June was also cleared to eat soft foods, such as yogurt, applesauce, mashed potatoes, and smoothies. June didn't eat much but would have a few bites three times a day. We hit 35 milliliters an hour by Friday, and that is where the problems arose. June started having breathing issues and was vomiting again. On Sunday, she was vomiting Pedisure, which she shouldn't be doing because the Nissen was suppose to prevent vomiting. We had been set to be discharged on Monday (March 20), but with the new developments, June's discharge date was pushed back. June's surgeon switched her feeds to her J tube. Since June was expressing interest in eating, her doctor didn't want to suppress that interest if she continued vomiting. The goal is to get her enough calories to continue to grow while taking pressure off the stomach. We also started draining her stomach 24-7.

On Wednesday, we met with June's surgeon. She felt that June was getting close to being ready for discharge. They were planning to keep her one more day (which would be two weeks out from surgery) but were willing to let her go that day if we were comfortable with the plan. We were more than excited and willing to bring our baby home that day. To give you an idea of where June should be, our surgeon said that typically by two weeks out from an abdominal surgery, kids are cleared to eat regular food. June is still on a very limited soft food diet because her stomach cannot handle the stretching at this point. She will also never end up getting continuous feeds in her stomach because the surgeon doesn't think with her stomach size that she will be able to handle it. June receives feeds 20 hours a day to get the calories she needs and can eat foods (yogurt, applesauce, baby food) for breakfast, lunch, and dinner. Her stomach is also drained 24-7. To make matters a little easier (since the last time we were on continuous feeds June was just taking her first steps and now she is running wild with her brother), June now carries around a backpack with her food and drained stomach contents. She actually does really well with the bag and doesn't notice that is on most of the time. We have a long road and a lot of work ahead of us, but the moments that have followed since we brought June home on Wednesday, March 22 is more than worth it.

June loves her new flamingo hat - it is perfect to hide from the nurses:

 Home At Last!

She's ready to roll: