The Dance

I have titled this update “The Dance” because that is exactly what this life seems to be.  Our dear friend and nurse coordinator, Lucy, used this analogy for our results from last week with Rhett.  I have been thinking a lot about this comparison over the last week as I have been digesting the information we received.  I once referred to this life as a roller coaster with all the ups and downs, but a dance is also a way to describe what having a medically complex child is like.  Rhett is always the lead, and we just follow whatever moves he makes.  Sometimes he keeps us on the same pace as life seems to go along smoothly, and other times he likes to change up the pace a bit leaving us trying to catch up to figure out how to best meet his needs.  Overall, we had mostly positive results from all the appointments and procedures in Cincinnati, but he did decided to throw us a curve ball which is something he tends to do.

I will go through the appointments we had last week to update you in the order that things happened.  On Monday, Rhett had another video swallow study.  This is where we feed him different thicknesses of food mixed with barium to see how he is swallowing the food by an x-ray.  Since I am pregnant, I was not able to feed Rhett for the swallow study.  This is the first time that I was able to stand back and actually watch the x-ray as he was eating.  As I was watching, I could tell he was doing a great job.  Every time he has had a swallow study in the past, he has always slightly aspirated.  This means that even though he would swallow most of his food correctly, there was always a trickle that would go into his airway which is not where we want it to go.  That is why he has always been approved to practice eating, but most of his nutrition would need to come through his g-tube.  Since he would slightly aspirate, we did not want him to aspirate enough to turn into pneumonia.  Over the last year, we have increased how much he eats by mouth and decreased how much he receives by the g-tube.  This test was done to give us an idea if he is ready to continue to be weaned off his g-tube, and those were the results that we received.  Rhett did not aspirate on any consistency of food.  This means that he was able to swallow thin liquids, regular purees, and pudding correctly without any trickling into his airway.  Rhett passed his swallow study completely for the first time ever, and we can continue to increase his food intake by mouth and also begin to work on him learning how to drink liquids.  This is one step closer to being g-tube free, but as long as Rhett has surgeries in his future, we will keep the tube in place.  I am hoping to keep the tube, but get him to be 100% oral to where we do not use the tube.  The tube is a comfort to have in times of sickness and surgeries, especially since he will need continued work on his airway.

We also had an eye appointment on that Monday.  Rhett’s vision is fine as far as we can tell, but we are monitoring his exotropic strabismus which is where his eyes will sometimes drift outwards.  This is basically the opposite of being cross eyed, and we are monitoring this because he may eventually need a procedure done to correct this.  He is currently able to bring his eyes back to center when they begin to drift, but if his eyes get to the point that he can not bring them back, then we will have to move forward with a procedure.  I hope that his muscles will get stronger to where his eyes will correct on their own, but from my understanding there is a very slim chance of that happening.  So this is another procedure we will have to look forward to in the future, but it will be up to Rhett to let us know when he needs to have him done.  I think you all are able to see how Rhett is always the lead in this dance of navigating the world of CHARGE Syndrome.

On Tuesday, we decided to take a little time for some fun since he only had one appointment that afternoon.  We tried the zoo that morning, but it was a little too cold for Rhett to be outside for very long.  Since it was also too cold for many of the animals to be out, we decided to wait and try the zoo again that afternoon.  Rhett is currently obsessed with lions, hippos, and cheetahs, and I really wanted him to see the real live animals.  That afternoon when we went back to the zoo, Rhett was able to see two out of his three favorite animals.  The cheetahs would not come out until spring, but he did get to see the lions and hippos.  He absolutely LOVED seeing them!  The look on his face was amazing.  He had the best time, we got some great pictures, and he was signing away.  It does my heart good to see us finding some fun for Rhett in between all the not fun stuff.  

In between our zoo trips, Rhett had a pulmonary clinic appointment.  We do not have a pulmonary doctor that we see at home, so our Cincinnati doctor always likes to see Rhett prior to an airway scope.  We did trial Rhett on a non-modified speaking valve, but he was unable to wear it safely.  We trial him every so often to see if his airway narrowing is growing with him in hopes that he will not require a reconstruction of his trachea.  Although he did not pass the non-modified speaking valve trial, Rhett checked out great for the appointment.

  Wednesday was Rhett’s anesthesia day, and he had a lot of things going on while he was under anesthesia.  We do our best to combine as many procedures as possible while he is under anesthesia so that we can minimize the amount of times he goes under.  He has already surpassed the number of times many people have to go under anesthesia in their lifetimes, and he is only 3 years old.  He always handles it like a champ, but my nerves are always on pins and needles while he is in the OR.  While Rhett was in the OR, he had his ear tubes replaced, his airway and nose scoped, his hearing checked, and a kidney procedure done to stop his kidney reflux.  From all of these procedures, we received mostly great news.  When they scoped his nose, our ENT confirmed that his nasal passages continue to remain open enough to not require any dilations.  Now onto his airway.  Remember, when it comes to his airway, Rhett is always leading the dance.  I will begin with the positive.  The inflammation that we have been working on was finally gone.  This means that the meds and breathing treatments Rhett has been on for the past year is finally paying off.  Now we will pray that the nasty inflammation will continue to stay away.  The next set of news we received was not necessarily negative, but also not exactly what we would like to hear.  Rhett’s lingual tonsils are very large, and would most likely obstruct his airway when he is asleep if he did not have the trach.  This means they have to be removed before the trach can come out, which means another surgery for Rhett in the pretty near future.  Also, if you didn’t know, there are multiple sets of tonsils.  Rhett has already had his regular tonsils removed, and these lingual tonsils are located right at the base of the tongue around his vocal cords.  During this scope, we also got the confirmation that Rhett will definitely need to have his trachea reconstructed before his trach can be removed.  This is the surgery we have been waiting for, but we have now changed our plan for the way the surgery will be done.  Originally we had planned for a single stage reconstruction where the trach would be removed at the time of surgery, but now we are planning for a double stage reconstruction.  This means that he will keep the trach in after his airway is reconstructed, and then we will begin the process of weaning him off the trach slowly.  While he will most likely be fine breathing through his upper airway when he is awake, we want to make sure that there is no obstruction while sleeping.  We want to make sure he is 100% ready for the trach to come out so that there is no chance he will be in danger living without it.  Now I will move on to the kidney procedure, which went well.  This was a noninvasive procedure to correct his kidney reflux.  While the procedure went well, we will not know if it was successful until later on.  Now for the news that hit me the hardest.  We did a repeat hearing test under anesthesia to see how his brain is responding to sounds from his right ear.  The reason we decided to add this test on was because about a month ago Rhett had his hearing checked from a booth test, and he was not showing much response from that ear.  I thought he was being a stinker and not wanting to participate for the test, but I was wrong.  His right ear showed significant hearing loss from what it originally was.  His right ear went from mild/moderate loss to moderate/severe loss.  This means that Rhett is having some progressive hearing loss, which is not uncommon for some people who have CHARGE Syndrome.  This makes me extremely sad that he is losing his hearing from his “good” ear, but I know this won’t slow Rhett down.  We have since then turned his hearing aid up, and I am noticing a greater response to sound.  His hearing is not bad enough to require another cochlear implant at this time, but if it continues to regress, that might be an option we can discuss in the future.

So now that I have given you all this information, I am sure you are wondering what our future will look like in regards to procedures.  Here is the current plan we have in place.  We will return to Cincinnati in 6 months to remove his lingual tonsils, repeat the hearing test, and have a follow up appointment to check on his eyes.  Please pray that over the next 6 months his hearing will remain stable, and he will not continue to have hearing loss.  Unfortunately there is no reason he would be having loss, it is just something that sometimes happens.  I’m telling you, Rhett is the leader in this dance and is notorious for his body to do funny things.  Also pray that his eyes remain stable, and that we do not get to the point of discussing surgery just yet.  I also ask that you pray that his airway inflammation remains gone, and that the lingual tonsil procedure will be exactly what he needs to eliminate some crowding in his upper airway.  After we do the lingual tonsil procedure in 6 months, we will move on to the double reconstruction surgery.  Our plan at this time is to do that surgery in a year from now.  We want to give Rhett plenty of time to make progress with his eating skills, and not bombard him with too many procedures since he finally has a safe swallow.  We are hoping to do the first stage to the double stage reconstruction next year over spring break. 

So for now, we will enjoy life without traveling to Cincinnati for appointments.  We will work to improve his feeding skills, and enjoy our summer with friends and family at the lake.  This will also be a nice break from traveling to transition into our family of 4, and allow Rhett to adjust to being a big brother.  I feel like we are all in an excellent place right now, and I am excited to just enjoy Rhett growing and learning.  I want to thank all of you who continue to pray for Rhett and our family.  It is always so appreciated.  So for now we will keep on dancing, and CHARGE-ing on!!