Tuesday, June 13, 2017

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!