Bentley graced us with his presence on March 29, 2016. After hours of pushing without any progress, I was questioned if I would like to have a C-section performed. I was in excruciating pain and was more than ready to have the labor over with so I opted to proceed. They had to “yank” him from my pelvic bone as he was starting down the birth canal and got stuck. He had pinched my sciatic nerve in the process which explained the pain I was experiencing during labor. The pediatrician immediately noticed an odd shape to his skull. She wanted to wait before discussing anything in further as abnormally shaped skulls are not uncommon following labor. It was the next morning when the pediatrician returned and started throwing names out there that I couldn’t even begin to comprehend at that time. Bentley’s head had almost formed to by pelvic bone. There were actual indention’s on his head where you could tell he favored to rest his head. She provided us with documentation in regards to what she was talking about so we could better understand what she was talking about. In other words, his head wasn’t going to improve on it’s own. We agreed to discuss details further at Bentley’s one week and I needed to chose a children’s facility to have the condition further evaluated as the local hospital did not treat these conditions. The conditions mentioned were deformational plagiocephaly and craniosynostosis.
I was fortunate enough to have a friend who’s nephew had gone through the same thing when he was born. I was forever grateful to have someone to turn to for any questions I may have had. Knowing that this child was now 8 and seeming to be doing fine, I asked where he went for his care. Gillette Children’s Specialty Health Care in St. Paul. I didn’t need to hear anything more. At Bentley’s one week appointment, the doctor proceeded with setting up the referrals to have Bentley start to be seen. Within a week we had an appointment set up to get a better understanding of what was happening with our son. They started with us being seen to determine exactly what was going on with his head and to see if it could be corrected without surgery. The doctor that we initially met with said he would be surprised if it was more than deformational plagiocephaly but wanted to ensure so he scheduled a CT scan. My husband and I hadn’t even made it home (about an hours drive) before we received a call from the hospital with a doctors appointment to meet with the cranio specialist and a neurologist. It was that day we found out that the condition was not treatable and that Bentley would need to have surgery. Bentley was diagnosed with right and left lambdoid and right sagittal craniosynostosis. Ugh.
Bentley had a rather prominent flat spot on his head which would cause him to favor leaning his head in that direction. This was the right sagittal area of the skull I am referring to. Because of him favoring that direction for turning his head, he had a condition called torticollis. The tenon on one side of his neck was “stiff”. At that time Bentley began PT work to loose up that tenon. The surgery was scheduled to happen in October, when Bentley would be 6 months. Both because of how evasive the surgery would be, because they needed to repair 3 of the sutures, and also they wanted Bentley to have full control of his head, in case a helmet would be needed post op. In August, we had the pre-op MRI so the surgeon’s could determine exactly what would need to be performed at the time of surgery. After the MRI, we did a series of, I believe they were called iron shots but I am not completely sure. The shots were to help reduce the risk of blood transfusion during the surgery. They were once a week for 3 or 4 weeks. It wasn’t long and the date of surgery arrived.
October 17, 2016, bright eyed and bushy tailed we headed to Gillette Children’s Specialty Healthy Care to experience what was, as of right now, the hardest day of my life. I handed my baby over to individuals that I had no other choice but to entrust them with my son’s life. While the surgery only lasted a few hours, it seemed like the longest time, EVER! Bentley was a rock star! He was conquering everything with flying colors once he came out of surgery. His levels were incredible. He wasn’t too crabby. He ate…and ate…and ate. We finally had to stop the eating as we didn’t want to make him sick. He was discharged from ICU the following morning. Thankfully, the brunt of his surgery was performed near the back of his head so his eyes never completely swelled shut. He had one “bad” night. They said every child that has this surgery has this “bad” night, it just varies from child to child. My dad, Bentley’s grandfather, came to visit and he was able to calm him down. His heart rate was in the 200 rage, bouncing between 180 and 210, causing the machine to go off A LOT. He had a minor fever and was just over all, fussy. Nothing, with the exception of grandpa, seemed to calm him down. Unfortunately, grandpa needed to go home. I eventually fell asleep sometime during the night. At this point I was that exhausted. I don’t even know if you want to call if fall asleep or that I just passed out. They were watching him very closely to make sure the vitals didn’t get any worse than they already were. The nurse that took over (they switched shifts) knew exactly what to do. When I awoke to a calm kid and normal sounding monitors, I was in shock. “What did you do!?” While the nurse that was on staff taking care of Bentley initially was very well educated and did everything that she had been trained to do, had never experienced this yet. This nurse had with other patients so she knew exactly what Bentley needed. It was just the right combination of this and that. Something along the line of the oxygen level, fluids, and meds were not “in sync” with one another. I was so glad to see him content and comfortable again. Another thing we kind of had to put our foot down on was Bentley sleeping on his stomach. This was the way that he had slept from the day he was able to turn over on his own. They didn’t want him to and I finally asked his doctors. There was no risk in the wise of the surgery recovery but there was a chance is eyes would swell shut. At this point, I was willing to try anything to help him calm down. Allowing him to sleep on his stomach also helped. Later that day he had his turban removed. Everything was progressing awesome, and ahead of schedule! We were told to anticipated him being hospitalized until Friday. (The surgery was on a Monday.) At the rate Bentley was going, he would get to leave the next day, Thursday. We got him up to play a lot. Went on a number of walks. He loved it! It was almost like we were almost back to normal. The swelling was already starting to go down. He met with both care teams the following morning, Thursday, and they said they didn’t see any reason to keep him any longer. Yay!!!!
Bentley was back to his usual self within the week. We kept him home from daycare for the next week, which we probably didn’t have to. At his 1 week post-op appointment, we discussed a helmet. While the helmet wasn’t required, there was always the risk of it providing a better turn out wearing it, than not. It was one of those, it’s not going to hurt situations. He wore the helmet until the following February, having weekly checkups to adjust the helmet. In February they said they could not longer adjust the helmet but that it looked good and that Bentley was being discharged from helmet therapy!!
March of 2018, he had an un-sedated, fast brain MRI. The results were not good. Bentley had hydrocephalus and a Chiari malformation as a result of the cranio, both of which appeared worse after surgery. His neurologist proceeding to schedule a sedated MRI just to ensure the results. Bentley must have moved during the fast brain because with the sedated, everything looked improved. They sent us home, wanting to be seen again in a year, same routine. May of this year, 2019, Bentley had another sedated MRI. I requested to do the sedated up from as the risk of movement was still there and I didn’t want to go through both again. His fluid levels and Chiari malformation have stabilized and are continued to be monitored. Neither are showing to be a cause for concern and as long as that is the case, we are going to consider this to be the “norm” for him.
In order to stay on top of the fluid and malformation, Bentley needs to have the pressure on his eye sockets checked yearly as well – papilledema. While the fluid looks fine and stabilized, there is still the risk of pressure. He does not have any pressure at this time. This test will help to determine that it stays this way. He is also severely far-sided, which is a possible side affect of the condition and very possible for him to outgrow. Bentley just celebrated his 3rd birthday in March and is doing incredible. He is progressing exactly how he should be. Tackling his milestones just as he is supposed to. At this time, there are no concerns with his developmental progress nor does he show any signs of having any problems. As far as the eye can see and the doctors can tell, our child is a normal 3 year old boy. Let’s hope that continues.
Now – pertaining to the fluid (hydrocephalus) and Chiari malformation. Just because they are stabilized now does not mean they won’t cause problems down the road. As I mentioned with the eye appointments, there is always a risk for pressure. If this does become a problem down the road, he may need to have a shunt placed. Not a huge deal. I have heard of this more and more since it being mentioned and it’s a lot more common than you would think. As for the Chiari malformation. This isn’t really known to start causing issues until closer to being a teen. This is when children typically start suffering from headaches. While it is something that we have been made aware of, it is not something we have gone into detail on. Right now we are just monitoring it to ensure it doesn’t get any bigger than it already is. If it does begin to cause a problem, we will discuss in further detail at that time. Our care team doesn’t like to scare you with too many details unless it’s absolutely necessary. I have done some research and came across things like medication and surgery but I am going to think positively and just pray we never have to go there.
I know reading some of these stories helped in our adventure and I hope our helps you with yours as well!