Go Go Go!!!

Susanne was not excited when her alarm went off around 5:45am this morning. This was a rude awakening. She started off on the wrong foot last night because she didn't even get into bed until around 1:00am. She thought that she would at least get around five hours of sleep, but that dream was crushed right away. Paxton woke up crying around 2:00am and didn't settle until an hour later. Susanne was up the entire time consoling him and trying to figure out what the problem was. Moppy ended up staying up as well for moral support.

Somehow they managed to get out of the door on time. They were even able to catch a ride from security as a courtesy because they left before 7:00am. Once they arrived at the hospital, they had a few minutes to spare to grab some breakfast before their crazy day began. Paxton looked exhausted, which didn't bode well for the busy schedule he had lined up.

Paxton's first appointment was with the cardiology service. He got called back almost immediately to get weighed. When Susanne pulled him out of his stroller, she quickly realized that a bomb had gone off in Paxton's pants. It was the worst mess Susanne had dealt with yet. He had managed to explode out of his diaper and cover his entire back with fecal matter, soak through every inch of his fresh, new outfit, and stain his car seat cover. It was so bad that his baby blue onesie was now a new shade of brown. It was just mind boggling to think how this exactly happened. He literally had been in that outfit for less than an hour. It was difficult for Susanne to know how to approach this disaster. After assessing the situation, Susanne realized that he truly needed a bath from head to toe, but that was not going to happen. After Operation Clean Up occurred, the nurse could finally get his weight. It took so long to take care of the problem that the nurse had to go do other things until Susanne was ready.

Today, he weighed 17lbs 7oz. Susanne was pleasantly surprised that he had gained weight from the last time especially since he had just dropped a few ounces in his diaper right before the weigh in. What did he think this was....Biggest Loser?

Moppy was tickled to see that Paxton already required a wardrobe change. She was shocked to see the aftermath. Susanne had the evidence stowed away securely in a scented bag. Hopefully, the smell would not follow them all day.

Paxton's first scheduled test was an ultrasound of his heart. He was so overtired that his patience wore thin within the first fifteen minutes of them starting. He was crying, sweating, and eventually turning blue because he was so mad. The little tricks that usually worked to calm him down, were ignored by Paxton. Nothing seemed to help. He didn't care for the singing, the "shhhhhh"ing, the humming, his favorite toy, the fanning, the words of encouragement, or the soft, soothing touch from his mother. The ultrasound technician was very patient and understanding. She was wonderful with Paxton. She gave him time to fall asleep and get comfortable before proceeding any further. He was only able to shut his eyes for about twenty minutes before he had to be woken up for the next test.

Next, Paxton had chest radiographs taken. They were interested in comparing his heart and lungs to the films that were taken before his heart surgery that was done back in October. Paxton did well for the positioning of the x-rays. He stuck it out for the whole process, but was not thrilled. He was being such a trooper for being so exhausted.

After they were finished in Radiology, he was past due for his next feeding. Moppy and Susanne made sure to give his medications on time, but waited for the first break between appointments to set up his feeding. Once he was set up, they moved to the Craniofacial and Plastic Surgery department. They were early, but this gave Paxton time to recover. It was sad to see the number of children that were in the waiting room. They were all dealing with different problems, with each one being more heart breaking than the next.

The craniofacial surgeon informed Susanne that he wanted to schedule Paxton for his cleft palate repair as soon as the cardiology service gave Paxton clearance for surgery. The surgeon didn't want to take any risks with Paxton. He wanted to make sure that his heart was stable after his major operation in October. Hopefully, the cardiologist would give them the green light. It was ideal for Paxton to have this procedure done before he was 12 months of age, but in his situation he felt it was safe to do it later as long as he had it done before 14 months of age. The doctor spent some time educating Moppy and Susanne on the procedure that Paxton would have done. Paxton would have to stay in the hospital again and would be monitored under the cardiology service post-operatively. Susanne felt great to have a plan in place. It was always nice to know what the next step was going to be.

After they finished with that appointment, they had to venture back over to cardiology to discuss the test results. Susanne was so delighted to see the cardiologist that had taken such great care of Paxton during their previous visit to Children's Hospital Boston. He was excited to see them too and he was beyond thrilled with Paxton's overall progress. Paxton's blood pressure appeared to be well managed with his medication. Also, Paxton's oxygen levels looked great. They ranged between 89 and 90. This was the best that it had been for the majority of his life. This meant that he had great perfusion to his tissues and the Glenn shunt was doing its job efficiently. The ECG was unremarkable overall. There was no evidence of any arrhythmias so he was able to discontinue one of his medications, Amiodarone. At the end of the appointment, he showed them the difference in the pre-op and post-op radiographs. There was a remarkable improvement with the over heart size. Also, the lungs looked clear and there was no evidence of aspiration. The cardiologist informed them that he was unable to fully assess certain aspects of the heart ultrasound that was completed earlier. Some of the important views that he needed to evaluate where skewed because Paxton was moving a lot during his mental breakdown this morning. He could say for sure that the tricuspid valve had trivial regurgitation compared to previous free flow through that valve. The Glenn shunt was patent and getting a sufficient amount of blood to the lungs. The left side of the heart was functioning close to normal. The areas that were still in question were the right ventricle and the pulmonary artery. The cardiologist wasn't sure that either of them were doing much of anything, but he couldn't completely tell from the views that were taken. His plan was for Paxton to return to have another ultrasound of his heart when he came back for his cleft palate repair. Paxton would be under anesthesia, so this would allow him to get what he needed to get a true handle on how Paxton's heart was doing. He also wanted Paxton to come back in three months for a follow up MRI of his heart and another ECG. Once he had all of this information, he would be able to determine how well Paxton's heart was doing overall. Unfortunately, he felt pretty confident that Paxton would require another heart surgery, but he just wasn't sure what at this point.

As they finished up with cardiology, it was time to feed Paxton again. They found the ophthamology department first before getting him all set up. Paxton was seeing a neuro-ophthalmologist for the evaluation of recent light sensitivity and assessment for Moebius syndrome. When Paxton was finally called back, they first discussed his extensive medical history. This always took up a lot of time. Afterwards, Paxton had a series of tests done. The doctor looked at how well he was tracking objects, assessed his pupillary light response, tested to see if he would look towards a pattern if one eye was covered...etc.. Also, he had a direct and indirect fundic exam performed, intraocular pressure assessed, and evaluation of his lens. The ophthalmologist was actually able to assess the severity of his farsightedness. Currently, he was positive 4. All babies were born farsighted, but it should correct itself by now. She was interested in a follow up appointment in 3 - 4 months to reassess his vision. She was comfortable with saying that his cranial nerve 6 was intact and functioning well. It was the cranial nerve 7 that was still in question. She thought that there was definite evidence of function, but there were some other minor concerns that made her want to investigate further. She witnessed his ability to smile, frown, and have a furrowed brow when crying. She did also see that when he smiled, he had visible nasolabial folds. This would not be possible if cranial nerve 7 was not working. She took multiple pictures to document his different facial expressions. Scott had previously raised concern about his left eye possibly being lazy. His left eye tended to prefer to migrate towards his nose. She did not have any concern with this observation because he proved that he could move his eye normally in all directions. Also, she mentioned that before the nasal bridge develops, the sclera, the white part of the eye, can be less visible. The only other thought was that because he was farsighted at this time, he may cause his eyes to move towards his nose to try to help focus on objects. She thought that this would improve as he aged. All in all, she did not feel like he had Moebius syndrome. She did, however, want to consult with a neurologist that she knew personally that specialized in Moebius patients. She would send the pictures from today's visit along with her exam findings to see what her opinion was. If she was suspicious at all for this syndrome, then the recommendation would then be to schedule an appointment with her to assess Paxton. The doctor was also going to relook at the brain MRI that was done in October. If they took images as far down as the brain stem, then she should be able to confirm if cranial nerve 6 and 7 were normal in appearance.

Finally, they were finished for the day. They had not had time to eat or drink anything all day. They were half starved. They ate dinner before leaving the hospital and took some time to shut off their brains before heading back to the Yawkey house. They decided to walk back despite the bad conditions outside because they felt like they needed the fresh air. Even though it was already dark out, it was well worth the risk.

When they got back, they got Paxton situated and in bed. They spent the rest of the night getting everything back in order before sitting down to try to recover from the exhausting day. They would all sleep well tonight.