Once I am caught up, I will be updating on a daily or as need basis, but today I am going to pile a week’s worth of info into one post. Fortunately, my parents had come to town in order to stay at our house and take care of pets as we had planned to be in the hospital a few days with the scheduled C-section, so when we went into crisis mode we were able to focus on Ren while they made sure everything was taken care of. Within an hour of delivery, we were getting information via x-rays and physical observation so we had to  hit the ground running before they even had Devona stitched up.

I followed the neonatal team out of the OR and up the the NICU (neonatal ICU) where an x-ray of Ren’s chest immediately showed signs of fluid in his lungs, as well the fact that his ribs were very thin and slightly bell shape, which concerned the neonatologist more than the fluid because this is often found in babies with SMA. Ren had been placed on a ventilator and was stable so I returned to the OR to check on Devona to let her know he was ok, but that we were definitely looking at something very similar to Atreyu. The blurriness of the day to day started at this point.

Over the next couple of days we learned that Ren had very low muscle tone and was not managing his oral secretions with swallowing, but it wasn’t a danger to him bc he was intubated. At some point, the neonatologist told us that he didn’t expect Ren to ever come off of the ventilator. This is the kind of comment that makes your knees weak and anger begin to build inside of a new parent. Of course you never want to do or try anything that will endanger you delicate little baby, but accepting this a a reality was not something we were willing to do.

Over the first few days of life, Ren continued to breathe over the ventilator which led to a steady reduction in the settings  of the machine. Initially, the vent was set to 40-50 breaths a minute, providing highly oxygenated air. I had not yet learned what all the setting meant on his machine, so I don’t know the actual concentration of the O2 that he was getting. Ren is a fighter and he was just getting started showing us how deep that well is in him. By day 3, Ren had the settings on the machine reduced to 21% O2 (this is the same as room air that we all breathe) and only 15 breathes a minute. He was consitently over breathing the machine by 30-40 breathes a minute. As amazing as this was, our little guy wasn’t done showing us what he could do. Over the next 24 hours, his team had turned his machine to settings that offered no breathes, but still provided some pressure on his inhalations and exhalations. He was essentially breathing with a CPAP that helped with apnea, but it was still using the tube that went into his lungs and prevented him from talking to us.. The next morning we were telling Ren how amazing he is and how he had blown us all away, talking about how maybe someday we would get a chance to try an external CPAP because then he would no longer have a tube in him and he might be able to tell us a story or two with that little voice we were dying to hear. As continued to stand in awe of our little guy, the neonatologist was making his rounds with his team and the all gathered around to discuss Ren as we listened in. Staying informed is so important, when it comes to advocating for your child, but it can also beat you down when you hear things that aren’t what you had hoped. After all, this is the same doctor that told us that Ren would most likely be on the ventilator for all of his life. As we listened on while the head neonatologist listened to numbers from the resident, we were caught completely off guard when he announced that he wanted to give Ren his biggest challenge yet; he wanted to pull the breathing tube and give our little tough guy a chance to do things all on his own. Of course we assumed that we would be moving to an external CPAP at this point, however Dr. Sharnberg was singing a different tune than he was only a few days earlier.

“I don’t think he needs it,” he said. “How do you feel about that?”

Obviously, we were overjoyed, but nothing compared to what was coming. The second they pulled the tube up and out of him, he let out his first battle cry.

“I am mighty Ren and I am going to shock the world,” he said.

Well, that’s what we heard anyway. Of the countless tears that had been shed over the last 5 days, these were the first tears of joy. For th next 24 hours, everyone was talking about how amazed they are at how he is chugging along and breathing all on his own. If this is what he could show us in several days, what else could we expect from our little warrior?

When it comes to these situations, triumphs are often times accompanied by setbacks. It was Friday  around 5pm (26 hours from coming off the vent) when he had just finished being examined by a geneticist. Our best guess (doctors included) is that while lying on his back, secretions had built up in his throat and he failed to clear them himself, leading to him struggling to breathe. It only took seconds before he was turning blue and a team of medical personel were rallying around him. Within minutes, he was intubated (ventilator tube back in) and back to a nice pink color. I will spare the details, but watching a team of doctors and nurses working on your child while alarms sound is one of the more helpless and horrifying experiences you can experience.

Throughout the week different doctors had weighed in on what they thought was going on with Ren, including a neurologist that believes it is “a standard case of SMA,’ which we overheard him tell the neonatologist. Although it is hard to deny that there are signs that point to SMA, there are also plenty of things that keep it from being “standard.” Only time and test results with settle this debate. Blood draws were ordered for the SMA tests (despite both of us testing negative as carriers.) These things can get scary when they are talking about taking 8mL of blood from such a tiny little guy. In order to obtain quantities like that, it requires tapping into arteries rather than veins like most blood draws. This is where as Ren’s biggest advocates, we  stuck our foot in the closing door of orders and got involved. No one knows more about our stroy than us and becasue of the knowledge we have acquired through the years of genetic testing on ourselves and doing our best to educate ourselves as well as we could, we were not satisfied with the test the neurologist ordered. SMA is a tricky thing and comes in different types and locations. The standard test looks for a deletion of SMN1, which is what we were tested for and don’t think this is much of a possibility. There is also a test for what is called Atypical SMA which looks much deeper and in other places, and can diagnose X-linked SMA which is associated with UBA1 rather than SMN1, and can be passed to a boy by his mother. Once we got in there and started throwing around our opinions and wishes, the doctors agreed that the Atypical SMA test was a good idea. Had we not been there to be Ren’s voice and advocate, they would not have done this. The SMN1 test doesn’t take nearly as long and we are actually waiting for results to come down as I type. The results are in and we are just waitng for them to make their way through the channels of communication. You will learn to be patient in situations like this. The Atypical SMA test will not be back until June 18th. I am going to publish here so that I can get back to Ren and Devona and wait for the results, but will pick up here later tonight.

Here are a couple pictures that were taken immediately after his breathing tube was removed.