Our little snowflake

Some updates on Thomas...
After a pretty healthy and festive December at home with everyone, Thomas caught a corona virus and bronchiolitis the first weekend of 2015. This landed him in the hospital, again, since it made it very hard for him to breath on his own. His oxygen saturation was as low as 72% at one time and his breathing rate was as high as 70-75 breaths/minute (normal saturation is 98-100 and a good respiratory rate for him should be in the 30's). In the hospital, they gave him some of the heliox (helium/oxygen mixture) and had him on high-flow oxygen until he was stable. He was in the hospital for 3 days. He was discharged Sunday night and we had to be back in Cincinnati on Monday morning for our second Myelomeningocele clinic. Last clinic, which was our first, I wrote about how it was comforting to meet all these doctors who would be a major part of Thomas's care as he grows, like meeting family (ok, like meeting in-laws.......in a good way!). Thomas has been through so much since that first clinic appointment. We have been in the hospital 7 times and had 3 surgeries. I remember at that first appointment thinking how easy this was, all the doctors had a very positive outlook on his future and what he would be able to do. The lovely thing, looking back on the hospital visits, is that I was right, the various doctors from our clinic were like family. They always came to check on us when he was admitted and were there to answer questions or to help advocate for Thomas as we navigated a path through it all. First up this clinic visit, was his urologist. This visit we had a formal urodynamics done and a renal ultrasound to look at his kidneys. For the formal urodynamics, they filled up his bladder and put some electrodes on him to measure the pressures in and around his bladder as it filled and emptied. His bladder only was able to fill with 15mL and it seemed like it could only actively hold 7 mL before the pressure rose. This is very small and leads his urologist to believe that his bladder is very rigid and not expanding and growing like it should. So as it fills, instead of expanding to hold more urine, it just spasms and since his muscles don't work together due to the nerve damage from his spina bifida, the  muscle to release the urine down his urethra (sphincter) doesn't get the message that the bladder is full and it should open and this just causes the pressure to increase. When the pressure is increased it has to release somehow and so the easiest way (if he isn't cathed) is for the urine to go back up the tubes leading to the kidneys (ureters) in order to relieve the pressure. This is bad for the kidneys and can lead to kidney damage and even kidney failure. On his ultrasound it appeared that the left kidney is slightly swollen so it has sustained some damage since his original ultrasound done at birth. In order to protect his kidneys from any further damage, he put Thomas on a drug that is a muscle relaxer and will relax his bladder so it can expand to hold more urine, hopefully decreasing the pressure and keeping the urine from going back up the ureters into the kidneys. We have also been asked to up our frequency of cathing to ensure his bladder doesn't get too full. While we don't like to hear that his kidney has changed since his last ultrasound, it is good that we caught this early and can adjust our tactics to help protect from further damage. We will do another follow up in 3 months to see how he is doing on the ditropan (the muscle relaxer for his bladder). This drug, while completely necessary and kidney saving, is not awesome in it's side effects. Many patients experience constant over heating, other have had constipation, dry mouth, blurred vision, fatigue, nausea, and weakness. Looking over those side effects and comparing them to what Thomas already has going on, it doesn't seem like something that will be very helpful; weakness - he already has significant weakness that we are working hard to overcome so he can just function on a very basic level; blurred vision - he already has some poor vision and we are trying to track down how much vision loss he has sustained through all of this; constipation - he has bladder issues as it is and we are expecting him to have bowel issues as well once we start solid food. So to say the least, I was not excited to get this news. It seems like he can't catch a break. Next up was the physical medicine/rehab doctors. They observed Thomas and played with his legs and said that he seems to have decreased movement and strength since our last meeting. This is something that I have been thinking also so you would think that it wouldn't be much of a surprise to hear someone else say it out loud, but this one hurt. All along, through all the hospital stays and surgeries, for some reason, I kept thinking that once we get over these hurdles, he will be ok and function like a typical kid and maybe feel included, but to hear that this part has decreased and his prognosis of mobility might change, broke my heart. If he has significant vision loss AND has mobility challenges, possibly putting him in a wheelchair, that just seems like such a difficult route for anyone to take. Last up was the developmental pediatrician. He came in and we discussed the stridor and he had concerns about Thomas's weight. He hasn't put on much weight recently. This could be concerning because it could be stemming from the stridor, since he is working so hard to breath and just get enough air, he is burning through his calories and not able to gain weight, but his lack of weight gain could also be from the various hospital stays where they don't allow me to feed him for a few days, so hopefully it is the latter and he bounces back, otherwise we will have to address the stridor soon. I know this is looming, but was hoping to put it off as long as we could. To address the stridor, we will need to find the best route, which basically means pick your poison. There are a few options; a tracheostomy, which is a hole from the outside of your neck into your windpipe to create an airway;  cordotomy, which means to cut the vocal cord to make more room for the air, but this will affect the voice; arytenoidectomy, which is cutting of the cartilage on the back half of the vocal cords and also affects the voice and could add a risk of aspirating; the other one that I have only heard of and still need to learn about is the posterior anterior cricoid split. After meeting with all of our doctors, our nurse comes in to see if we have any questions. I asked her about all the different routes for helping with the stridor and what others have done and also about how to best know if it's his vision that is holding him back from looking at us and recognizing faces or something else and what kind of experience others in clinic have had and she said that unfortunately spina bifida is a snowflake defect, no two are a like and Thomas is definitely forging his own path. While I am proud of him for being his own person and a beautiful unique little snowflake, this also just made me feel lonely. I have written before about how I crave familiarity with him and want him to be like other children with spina bifida so we can see what path they took and have confidence in our decisions knowing that others have had success. Had I spoken with someone who chose to do the prenatal surgery, we may have opted to do it. Had we met someone who had the stridor and done the decompression when it first presented, we may have saved some vision loss and vocal cord damage. I know hindsight is 20/20 and there is nothing we can do about it now, but I don't want to make those mistakes again, I want to be fully informed and understand EVERY detail of EVERY option. Next up we have hypertension clinic on Jan 12 and a neuro follow up on Jan 13 and a feeding team clinic on Jan 15. Hopefully those appointments bring good news! Will post again soon.