A Silas Update: CHOP, diagnoses, surgeries and progress

           Hey y’all.  Here are some bits and pieces of what has been going on since the summer.

 

           Over the last several months we’ve sort of been in the middle of our own quiet chaos trying to plan ahead for Silas’s appointments and future surgeries, while figuring out what has been needing to happen for the new diagnoses he has been given.  It’s been a whirlwind of emotions but we have buckled down and are doing really well with things so far.

            When Silas was first diagnosed with CHARGE syndrome at 14 months old, I was still trying to get adjusted to him as a tube fed baby with hearing loss; this was a really big deal for us and we thought this was all there was to find out with him, and I remember I mourned for months while trying to be publicly positive. We also were trying to heal and move on from the stress of his first year of weekly doctor and therapy appointments, surgeries and hospitalizations, thinking “Oh my gosh!  When will this end?”  They say the first year is the hardest. Maybe they are right? I don’t really feel that the surprises have slowed down necessarily, and it seems our load has only grown (and our love, too).  Though once Silas was diagnosed, I believe we were able to accept things and find our groove; we were able to adjust so much in terms of how we approach things, our perspective, and how we handle what we are given.  <- That part hasn’t been easy!  I also think I’ve been able to hand over so much of my worry and fear to God since receiving the news of his syndrome, and visualize why God’s presence is a priority in the center of this journey as we’ve moved forward.  It isn’t my journey to control and we have an Almighty God who knows what He is doing.

            Silas has truly been a blessing to our family, in that he has helped us slow down a little, search eagerly for the prize in even the littlest moments and appreciate things on a level we had no way of understanding before. Each of us in our own way have grown because of his presence in our lives, and I believe each of us have had a big impact on Silas’s achievements. Ryan has been such a strong spiritual leader in our household, which I have needed on a daily basis as I navigate how to care for my family and their needs. He has also been the sole provider of our family, as I have to stay home to care for Silas’s many needs. Carson has been so strong, such an amazing role model for Silas, while keeping every moment so incredibly fun for all of us. I hope his spirit always stays young. Silas has been expanding his toddler wings, and testing our patience daily while exploring his options as a now walking, babbling, temperamental almost-3-year old. Many days feel very much “normal”, and many days I am discouraged by what I wish could be a little easier to understand. But please, do not get me wrong! I absolutely love this life and my family because of exactly who they are and what they bring to my life. Please don’t feel discouraged for us, or feel sorry for us. None of us want that. Please ask about Silas’s diagnosis’s in an effort to better understand who he is and why he is such an amazing kid, and not out of sympathy. Pray for us, not because we need attention from anyone, but because we need God’s healing and loving presence in our lives daily, as this journey is something that we are still navigating and will be navigating for a very long time.

         As most of you know, we recently returned home from a trip to The Children’s Hospital of Philadelphia at the end of July. We were really amazed with the outpour of love and financial help that was given to us, which helped us in so many ways and will continue to do so in the future. The trip to Philadelphia for one week was very expensive! There is no way we could have gotten through that week without the support of so many. We are returning to Philadelphia in November for a couple of clinical appointments and a surgery. Surgery for Silas is really hard on him no matter the procedure as he has some heart issues and a fragile airway. He is having his tonsils and adenoids removed this trip, and will be having several scopes while under sedation. Because of how unique Silas is on the inside, we were told we will need to stay locally to the hospital for at least two weeks following this procedure to monitor bleeding, so please pray for us as we make difficult but necessary decisions involving Carson, as he will more than likely have to stay home so that he can make it to school every day. We will be gone almost three weeks, and this is going to be hard on all of us. I am already dreading being without my oldest for this long and being many hundreds of miles away.

We get to meet with Silas’s ENT tomorrow to talk more about the plans for the future, and we are really excited to see her as she is a huge part of our family and a big reason for why Silas has gotten such great medical care. We will get to talk with her about the plans ahead for Silas involving his surgeries out-of-state.

          We’ve recently reached out to a cardiothoracic surgeon at St. Louis Children’s Hospital and are in the early planning stages of when to approach Silas’s heart problems. This is going to be a very major surgery, but are happy we have found a doctor who has done this procedure somewhat routinely, as it is a very rare problem with very little knowledge in the medical field of how to correct it. There are some big risks involved, especially for a child such as Silas, but this doctor has voiced his confidence to us which is reassuring. This surgery will be to dissect his Kommerell’s Diverticulum (aneurysm), reroute his left aberrant subclavian artery to the left carotid artery, and divide his vascular ring. Here is a detailed picture below of what the surgery, minus vascular ring division, will entail for any visual learners like myself:

            What is the Diverticulum causing for Silas? The diverticulum presses up on the back wall of Silas’s esophagus, causing and indentation which will make swallowing solid foods pretty impossible for him without choking. It is also considered an aneurysm, and could very likely grow bigger over time which is not a good thing if you think of what aneurysms do. There isn’t any indication this is happening at the moment, but it is always possible. The longer this sits where it is, the more permanent damage it does to his already impaired esophagus.  Why are they rerouting his left subclavian artery to the carotid? Because Silas was born with a uniquely placed subclavian. The blood is flowing from the heart out to the aorta, and with where Silas’s subclavian is placed this causes blood to flow backwards, which isn’t a great thing if you think about it. We aren’t suggesting this is the cause of Silas’s weak left arm and his daily blue spells that cause him to pass out, but we aren’t denying this is the reason either. Silas also fatigues very easily, which could be from these vessel issues as well. We are really hoping that these symptoms resolve after surgery, but we can’t 100% tell what the surgery will fix until it is done and we give him time to adjust. Here is a picture of Silas’ esophagus, showing the diverticulum compressing the back wall vs. a normal esophagus.

Silas’s Esophagus

Normal Esophagus

             He also has to be scheduled sometime after the New Year for his major airway surgery. Both  the airway and heart surgeries are going to require an even longer recovery period staying locally to the hospital, so please pray for as much stability in our lives as possible on the road ahead, and of course keep Silas in your prayers that his surgeries go as planned with no complications.

             We recently received the results of Silas’s sleep study from early on in September, and it did show that he has severely obstructed sleep apnea. It showed that Silas’s oxygen saturation levels drop very severely in to the 60’s in his sleep, and that he is only getting about 7% of his REM sleep (20-25% is what he should get). This means he has been extremely sleep deprived for a while. Please pray that in a couple of months the tonsillectomy and adenoidectomy will help with this diagnosis and give him good results in the future so that we do not have to consider any machinery to help him sleep at night.

              One of his future appointments will be visiting his endocrinologist next week to go over his recent lab results in regards to his growth hormone levels, and we will be talking about the possibility of Silas needing growth hormone therapy. This has been heavy on my mind since Silas was a baby, as his growth hormone levels last year were the lowest they possibly could be without him being considered “deficient.” At the first of the year, we checked in with his doctor and the goal for him was to grow, grow, grow this year so that we could hopefully rule out the need for growth hormone shots, but Silas has not grown a whole lot since then. There is a possibility she will say “absolutely no need for shots,” or yes to shots as he continues to grow over the years, or that there is a possibility for a trial run with shots to give him a boost. I’d really love to hear that he has grown a lot more than I am able to notice and that we don’t have to go this route. Please pray that as a team we will make the best decision for Silas. Growth hormone treatment for us is not about how tall we want Silas to be. It is about his bones growing to their fullest potential so that he can be as healthy as he possibly could be in his adult life.

               Silas has made a lot of progress in all areas. He is picking up on speech, pitch and trying very hard to form words, though he is still very, very much non-verbal. He is doing SO well using signs, but it becomes more difficult in the situations where he needs to express his emotions. He is walking really well on level surfaces and we are slowly working towards uphill and downhill climbing, stairs and unleveled surfaces such as the backyard and parks. He can’t make much progress with eating until after his surgeries, but he is getting to the point where he is bored with purees. I am hoping he stays interested in food so that trialing solids and liquids after his surgeries will be fun for him (and for the rest of us.) We recently had his evaluation with the school system, and are transferring out of the infant-toddler program by his third birthday which is going to be a big transition for all of us. He will still receive his therapies at home, but we will be limited as far as family support and guidance goes as he enters in to the school system with an IEP. I am nervous but looking forward to what the future holds for him.

“Oh God, be greater, than the worries in my life.
Be stronger, than the weakness in my mind.
Be louder, let your Glory come alive. Be magnified.” – The Messengers