A few weeks ago we traveled once again to our home away from home to Cincinnati. With it being the week of Thanksgiving, I knew I needed to get an update out to everyone. We have so much to be thankful for from this trip, although things did not totally go 100 percent the way we wanted it to go. But then again, does it ever? This is all part of the wild ride known as CHARGE Syndrome where you come to expect the unexpected and unpredictable.
During this trip, Rhett had several follow up appointments for his eyes and ears, and then he went under anesthesia for the doctors to take a look at his airway since his lingual tonsils were removed in August. He got a great report from the eye doctor, and will continue to monitor his eyes every 4-6 months. He still has the drifting with his eyes, but it is staying stable in how they drift. He has great control over the drifting, and is able to bring his eyes back to focus when they begin to drift. We are so thankful for this news in that he will not require surgery anytime soon for his eyes, and he may never require surgery if they continue to stay stable.
Rhett also had consultations with the ENT that specializes in ears and the audiologist in preparation for a Cochlear Implant for Rhett’s right ear. We were able to talk through some things with the ENT and audiologist both, and we feel that the 2nd CI is in Rhett’s best interest based on the regression of hearing in his right ear. The team has met since our appointments, and they agree that Rhett will receive more benefit from a CI for the right ear instead of a maxed out hearing aid. Rhett’s hearing aid is currently maxed out, and there will be nothing more we can do for that ear with the hearing aid alone. Planning a surgery for your child is always super nerve wracking, and I am not taking this decision lightly at all. With much therapy, Rhett is currently doing well responding to and understanding some sound which makes me nervous to mess anything up. Even though he is hearing and understanding some sound, he is currently missing out on hearing the majority of speech sounds which makes listening and hearing very difficult for him. With the CI we are hoping to close some of the gaps he has with hearing and understanding those sounds, and to give him the best chance possible for “normal” hearing in that ear. I say “normal” because no matter what we do, Rhett’s hearing will never be normal. My hope is to get him as close to normal as we possibly can. I am asking for prayers as we pursue this 2nd Cochlear Implant surgery. Rhett will continue to need a lot of therapy to help his brain learn how to hear with a Cochlear Implant on that side. We are hoping there will be some benefit in this learning process since he has been getting some sound to that ear all along. While we would love for Rhett to hear and understand speech and also learn speech, that is not our main goal for doing the CI surgery. Since CHARGE is such a multi-sensory impairment syndrome, my main focus is to give Rhett as much input to the senses that he does have. Just because we implant that ear does not mean that Rhett will all of a sudden be able to hear, listen, and understand. This will be a long road of learning for Rhett. Please pray this is the right decision for him. We are currently waiting on the approval from the insurance company so that we can schedule surgery. I know that the insurance company has already denied him once, so now the appeal process will begin. Please pray this process to get the surgery approved by insurance will go quickly so that the surgery can be scheduled for as soon as possible. The quicker we can schedule surgery the better.
During this trip Rhett also went under anesthesia for scopes of his airway with his airway ENT and Pulmonologist. His airway continues to look crowded, which is not uncommon for people with CHARGE Syndrome. In fact, the Pulmonologist said if he saw Rhett’s airway without knowing his diagnosis, he would be able to diagnose him with CHARGE based off how his airway looks. Even with the crowding in his airway, both doctors agreed to allow Rhett to complete the capping trial. The capping trial is where Rhett would put a piece over the trach which forces him to use his upper airway to breathe. This is the next step towards getting the trach removed, so we were thrilled they were allowing him to give it a try. They told us that many times CHARGE kids can surprise us and are able to manage breathing just fine, even when the pictures look like they should not be able to. After the scopes, Rhett was admitted to the hospital for the capping trial. The trial began the next day after we put a smaller trach in, and then we put the cap over it. Rhett did amazing that whole day and kept his O2 levels in the high 90s which is really good. We knew the challenge would be at night once he was asleep and relaxed. We were worried that his airway would relax and become obstructed while asleep. Rhett went to sleep around 8:30, and continued to keep his numbers up. Dale and I watched a movie and monitored his levels. We finally decided to sleep a little bit around midnight since his numbers were so high. I slept with Rhett in the hospital bed, and around 3am I could hear him becoming a little junky in his chest. His numbers dropped a little bit, but were still in the 90s which was good. I patted on his back in hopes he would cough and clear whatever junk he had while he was asleep. He would not cough, and over the next 30 minutes his O2 levels continued to get a little lower and lower. His numbers got to 85 and the nurse and respiratory therapist came in to check on him. They also tried some things to try to get Rhett to cough, but he continued to sleep right through everything we did. At one point we did take the cap off to see if his numbers would pop back up, which they did not. That was actually a good sign because if they had popped back up then that would indicate obstruction. Rhett really just needed to cough. Since he would not cough, we did end up having to take the cap off to suction out his trach. Once he was suctioned, his numbers went back up in the high 90s where they needed to be. Because we had to take the cap off to suction, Rhett did not pass the capping trial. I refuse to say that he failed because I think it was a huge victory that he was able to manage fine for 15 plus hours. Now it is a matter of getting him stronger, and making sure he coughs to clear secretions instead of suctioning. I am so thankful to know that he is able to breathe out of his upper airway, and that he didn’t fail immediately after putting the cap on. The plan is to return to Cincinnati at the end of February to scope again and do the capping trial again. Please pray that Rhett will get stronger over the next 3 months so that he is able to be capped for the entire 48 hours with no problems.
In the meantime, we will get to enjoy the celebrations of this Christmas season without having to travel back to Cincinnati. We get to celebrate Rhett’s 4th birthday and focus on him being a kid without the stress of planning the not fun stuff. He has come a long way with his growth and development over this year, and I can’t wait to see how much he will continue to thrive. Rhett is coming such a long way with his sign language for communication. Although he is not where he needs to be, he is making strides with his communication. He can sign many different songs, and can even sign the entire alphabet (when he wants to). I share this because I want to praise God for all that Rhett is. Even though everything seems to be a little more difficult for Rhett and he has had to go through much more than most adults experience in their lifetime, Rhett continues to be such a happy kid. He is truly amazing. We give thanks to the Lord this Thanksgiving and everyday for blessing us with such a gift.