John had an appointment yesterday with a plastic surgeon that specializes in facial defects. Just like we hoped and prayed it would, we left this appointment with some viable surgical options and the CT scan did provide some clarity. 

We know he has lots of scar tissue. The piriform aperture (nose holes in the skull) are supposed to be above 11mm. 

John’s were 3mm when he was born and his first initial surgery made them some bigger, but they’re still less than 10mm, so could be opened up more. 

 

The nasal septum (as I’m sure you all know) is supposed to be straight. Tons of kids and adults have deviated ones but generally doesn’t cause a ton of problems… for that reason, they generally do not correct deviated symptoms in children or kids.

What John has is……unique. 
His septum starts straight but very promptly turns about 90 degrees to the right, and connects to the right side of his nose, rendering his right nostril useless. It’s completely blocked. This explains why we’ve seen zero progress in his breathing over the last few months. Ironically, three months ago he had a huge hole in his septum that was initially concerning, but was actually helping him to breathe better at the time. That hole has since closed up.

 

So what he has is just 1 nostril, that’s still too small, and is mostly occluded with scar tissue that can’t safely be removed for many many years. It’s incredibly hard to keep his nose completely clear because of all the scar tissue and even with constant saline and suction, he is oftentimes still unable to breathe very well. In a given day, he cannot finish one entire bottle and at this point is almost entirely G tube dependent. It’s often times hard to get him to settle down to sleep and on the worst days he can’t take a bottle or pacifier so it’s very hard to comfort and settle him.


The options are proceed with surgery to 1. Removed obstructed septum and straighten it out, hoping and praying it doesn’t result in a collapsed nose or even more scar tissue and 2. Make his piriform aperture a little bigger. 

The doctor seems hopeful this is a safe option and the chance of scar tissue and collapse is low, but unfortunately always a possibility. He would have to keep a stent in the right side just for a week since it would be an actual rhinoplasty, and would possibly stay in the hospital for that entire week depending on how he’s doing. If his nose were to collapse, we would be looking at multiple surgeries to try to reconstruct his nasal structure and probably a trach anyway. 

 

 

Or we can do nothing. 

But entering cold and flu season with an unstable airway that’s easily obstructed at baseline could very easily end with us having no choice but to trach him anyway.

Although we are thrilled that he’s been stable enough to stay home for a few months, the day-to-day maintenance and his daily struggles in regards to his breathing are not something that are sustainable. The condition he was born with is generally something that kids can outgrow, but the secondary issues that were now dealing with are not something he will ever outgrow. The scar tissue cannot safely be removed until he’s well into adolescence, and his extremely deformed septum will never be fixed without surgical intervention.

Throughout this whole journey, it’s been very hard at times because it seems like we’re only dealt less than ideal options. If we do nothing, we run the risk of severe illness and even further compromise to his breathing that already isn’t very good. If we proceed with the surgery, there are always risks that it doesn’t go as planned. 
However, like I said, his normal daily life is not something that sustainable long-term, and we feel like we have no other option but to proceed with the surgery. We have full faith in his Doctor, who is extremely intelligent and very good at his job. He took extra time to spend with us on a Friday afternoon to talk through all the details, and made it very clear that although it is his recommendation to proceed with surgery, we are his parents and therefore the decision lies 100% with us. 

If you’re looking for specific prayers to pray, what we need now is for:
1. surgery goes as planned
2. zero/minimal scar tissue postoperatively
3. that his nose is able to remain structurally intact once the blockage is removed, and we can avoid total reconstruction. 
4. Extra strength and patience for Andy and me, because these decisions are so heavy, and the coming days are going to be so long. 
5. For Collins, who unfortunately has to be the second priority most days. 😞



The surgery will likely take place in the next 2-3 weeks, and we will keep everyone updated when it’s close to time. 

In the meantime, John is still the most happy and loving seven month old around and it’s so unfair that he’s had to endure so much. Thankfully, the Lord blessed him with the most tolerant demeanor (like his dad) and he is taking everything in stride, with a smile on his face. 

Thank for reading and walking this journey with us. 🩵