More Fusion Issues

Merry Christmas everyone! I know it has been a long time since my last update and I have had a number of people ask how I am doing. Overall I am doing pretty well!

I had a craniocervical revision done back in February of this year to replace loose occipital screws from my original fusion I had done back in December of 2013. The surgery went well and there were no complications. All of my imaging looked great and the surgeon was able to replace the loose screws and secure the fusion as we needed. I was in the hospital for two days and had good pain management. I was in a hard cervical collar for three months following this surgery. At three months post op imaging of my fusion (CT scan) was done to assess how well my body had responded to the surgery. The imaging at that point looked great! My screws were solid and looked like they were embedded into the bone as needed. I then was able to start weaning out of my cervical collar, which after three months was very exciting for me! After a couple of weeks I was not wearing my collar at all anymore except for on long car rides and I was feeling pretty well!

My neurosurgeon wanted to do another round of follow up imaging (an x-ray this time) three months after this appointment, which ended up being about 7 months post-op. Unfortunately this imaging did not go nearly as well as we would have liked. The x-ray showed that my cervical screws, which were secure before my surgery in February, were both loose on the left side. The occipital (skull) screws that had been replaced during my revision in February were still secure but now the lower  cervical screws were both loose on the left side. Thankfully the right side of my fusion was still secure and had good bone growth so it was just the left side we were worried about. At this point my neurosurgeon but me back into a hard cervical collar 24/7 and I waited about two weeks for an appointment with him to discuss our options.

At this appointment we discussed two possible options and of course neither of them were without their downfalls. The first option was to jump right in and do surgery. Since I had already had two fusions we felt that at this point I would need a more involved surgery that included fusing more levels of my spine. Right now I am fused from my skull down to my second cervical vertebra. This is the only area of my spine currently that is unstable so at this time I do not need any vertebrae further down my spine fused. With my current situation, however, we agreed that this fusion has been unstable for so long and we had now twice attempted to attain a solid fusion that we would need to go down another vertebra and include my C3 (third cervical vertebra) in the fusion. This can lead to many potential complications. I would have to have C3 included on both sides of my fusion. As the right side of my fusion is solid and has adequate bone growth it would be a risk to alter that at all. If we add onto the right side of my fusion it may cause it to become unstable as well. Additionally, including lower levels of my spine has the potential to cause what the neurosurgeon calls a domino effect. Basically we are permanently changing the way that my spine moves and sits. As your spine is all connected changing the way that the top of the spine sits may change the way lower portions of my spine are positioned. The more levels that are fused higher up the higher my risk of needing more fusions down the line on lower levels is.

Given all of the downsides there are to surgery we felt that we should first pursue a more conservative route. The alternative to another fusion revision surgery was to brace my neck and see if we could artificially hold it still enough to promote bone growth. Bracing would include me being in a hard cervical collar for six months. Our hope is that with holding the neck still for that long we will be able to encourage bone to form around the fusion, the ultimate goal regardless of the approach we take. This bone formation is what solidifies a fusion and how you know that a fusion was successful. The risk with this is that we may go six months and still end up needing to do more involved surgery if there is no bone growth after that time. Ultimately we decided that this is the best route to go for me and my current situation.

I am currently three months into wearing my cervical collar, so I am halfway there. I am just waiting at this point to do another round of imaging. I haven't scheduled my appointment yet but I will get imaging done and see the neurosurgeon sometime in March, putting me at about 13 months out from my revision surgery. We will determine at that point whether or not the bracing has allowed my fusion to solidify and bone to form or if we will need to move forward with another surgery at that point.

Besides my fusion issues my health has been very stable! It has been a huge relief to be so stable right now as school has been very busy! During the fall semester I was at a high school three days a week doing clinical work toward my degree in speech-language pathology. It was a nice change for both Reilly, my service dog, and myself, but exhausting none the less! I am so pleased that I will get to be at the same site three days a week during the spring semester! I am finishing up my graduate program and will graduate with my Master's degree in August. I can't believe this season of life has gone so quickly! Thank you as always for your continued prayers, it means so much to me and my family! I hope you all had a wonderful Holiday season and have a terrific 2018!

Surgery Date and Description

I just wanted to give a quick update with the date and details of my upcoming craniocervical revision surgery.

My surgery will be this coming Friday February 17th at 1:15 p.m. It was supposed to be the following week, but it was moved up for logistical reasons on the side of the hospital. The projected hospital stay is 4-5 days but I can almost guarantee that I will not be in the hospital that long! I have never stayed more than two nights in the hospital after a surgery and I have had much more invasive surgeries than this one, so I can't imagine I will be in the hospital any longer than two days this time. My hope would be to come home on Sunday, but they may force me to stay until Monday because they don't like to discharge on weekends.

The surgery is booked to be about 4 hours long, which in the grand scheme of things is relatively short. Both my original decompression surgery and my first craniocervical fusion were about 7 hours long, so this is significantly shorter than those. Obviously this time may increase if the surgery is more difficult for any reason, but 4 hours is their rough estimate.

Now for a brief explanation of the surgery. I will post the same picture that I posted in my last update just to help explain the surgery.

The hardware on the left side of my skull will be disconnected from the hardware that is in my cervical spine. The skull hardware will then be removed from my skull completely.

The ideal place to attach hardware to is the center of the back of your skull. This piece of bone was removed from my skull during my Chiari decompression surgery to make room for the cerebellar tonsils (part of the brain) that was herniating into my spinal column. So since this piece is missing my original fusion was done next to this spot, slightly further over than what is shown in this picture. Well as you move laterally out from the center of your skull it gets thinner and thinner. We have no choice but to move over since the spot that my first fusion was done did not fuse properly. We hope that this will be alright, but it may prove challenging for the surgeon to get the screws solidly into the bone since the bone will be thinner.

Once the new screws and hardware are secured to my skull the rods connecting this new hardware to the cervical hardware already in place will be reattached. After this a substance called bone morphogenetic protein (BMP) will be laid down. This substance helps to induce the formation of new bone over the hardware. Ultimately I need to form bone over the hardware to secure it in place. This new bone formation is what causes the fusion to be solid.

My nerves will be monitored throughout the entire procedure because they will be working so close to my spinal cord and brain stem. This will help them ensure that my fusion holds my head and spine in the correct alignment and that they do not damage any nerves during the procedure.

I have done all of my pre-op appointments over the last couple of weeks including getting blood work done, having a physical, and meeting with anesthesia and everything should be good to go for Friday. I am just working on getting all of my school stuff taken care of before then, but honestly I could not be more ready for this surgery! My energy is extremely low, I have lots of headaches, and my neck is bothering me more and more. My body has been telling me something is wrong neurologically for the past 8 months and just this past month or so I have felt like my body is exhausted. I am so grateful that my surgeon got me in so quickly, but that in itself is worrying. I was scheduled so quickly because he is treating my case as an emergency case. He is worried that the loose screws may come loose and dislodge. My brain stem is also under pressure again because of the instability present in my craniocervical joint. This could cause permanent damage, more than it did prior to my last fusion, so we need to act quickly to avoid any further damage. My surgeon is having to cancel many of his surgeries due to there being no rooms available at the hospital (it is a children's hospital and sadly this flu season has been extremely difficult on very young children in my area), but he refuses to cancel mine because he sees how critical my case is.

All prayers and well wishes are much appreciated and I will be sure to update as soon as I can!

Loose Craniocervical Fusion

So I have failed miserably at updating everyone recently! I had finals for school (which went very well!), family came in town for Thanksgiving, we went out of town for a friend's wedding and Christmas, and I had the stomach flu (TWICE).

So unfortunately I have some not so great news to update with. As I had updated a couple of months ago I have been having headaches since May of last year. They came on out of the blue and we were not sure why they developed or where they came from. Last I had left everyone we thought that my intracranial pressures (ICP) were high and were pursuing testing to determine whether or not venous stenting would be an option for me. This procedure would have been done if there was a venous abnormality present causing my ICP to be high. After having a special type of MRI done, called an MRV, to check my veins the vascular surgeon did not see any reason why my pressures would be high and saw no vascular abnormalities of any kind.

At this point we were left with two options. Go back to our original plan of pursuing more invasive testing of ICP or continue delving a little deeper to see if there was something else going on that we hadn't considered. Now this local neurosurgeon trained under my wonderful neurosurgeon, Dr. Rekate, in New York and is one of the only surgeons to perform both vascular surgeries and more traditional neurosurgeries. After he heard my neurological journey and symptoms through the past several years one point really stuck out to him: I had immense symptom reduction after my craniocervical fusion. Despite needing another neurosurgery (my tethered cord release) I was like a completely new person after my fusion. My symptoms were dramatically reduced and I was able to function again as a "normal" human being. I had no headaches after this surgery and my neurological symptoms like ringing in the ears, pins and needles, weakness in my limbs, and double vision almost completely disappeared too. With all this in mind this neurosurgeon decided that he wanted to run further tests to check on my fusion and ensure that it was still doing well. We thought that since I had such a sudden reemergence of symptoms that it was possible that something was wrong with my fusion.

The problem with this theory is that I have had numerous MRIs recently and they have all showed that my fusion looks great! The first and easiest test to check on my fusion was a flexion and extension x-ray. This is an x-ray taken with my neck bent all the way until my chin touches my chest and then another x-ray is taken with my neck bent backwards as far as it will go. The two images are then compared to determine whether or not there is a shift of the hardware of my fusion. Ideally no movement of the hardware should occur, which is exactly what we saw on the x-rays.
Now after the x-ray we were left with two more tests that we wanted to run. Thankfully we were able to have both of the scans done at the same time which helped to decrease the time we had to wait. The first scan we did was another MRI. The doctor wanted a special series of images on the MRI machine to view my venous structures in more detail. After reviewing this scan, he is even further convinced that I do not have any venous abnormalities and that that is something we can rule out.

Next, he wanted to have a CT scan done of my fusion to view it in greater detail. A CT scan takes a number of different x-rays taken at various angles and combines them together to form a computer generated image of the part of the body you want to see in greater detail. While MRIs are good to view soft tissue structures, like the brain and cartilage, it is not ideal for viewing my fusion because the metallic hardware shows up as bright spots on the images and can obscure the structures around it. The CT scan, on the other hand, is my better at showing us my fusion in detail because it shows the bony structures and the hardware is much clearer on this type of scan.

Thankfully this CT scan did provide us with some answers that we needed!

First, let’s start with a picture of what my hardware looks like:

(You are looking at the back of a skull and the first three cervical vertebrae of your spine)

This is the type of hardware that the new, local neurosurgeon uses, which is similar to, but not exactly the same as, what Dr. Rekate uses. I have screws (the larger purple pieces at the bottom of the piece of hardware) that go into the top cervical vertebrae, C1, as well as the second vertebrae, C2, as is shown in the picture. I also only have two screws (the small purple eyelets on the skull) in each side of the hardware that attaches to my skull instead of the three shown in the picture. Bone is supposed to form around the rods that go down from the skull to the cervical vertebrae, which further stabilizes the fusion and ensures the craniocervical joint is fully stabilized. Also, how fitting is it that my hardware will be purple?!?! For those who don't know purple is my absolute favorite color!

With that visual and explanation you can better understand what the results of the CT scan told us. The CT scan showed that on the right side all of the screws were firmly in place and that bone was formed completely and securely over the entire rod that goes down from my skull to my vertebrae. This side looks perfect and exactly what we would expect to see from a secure, solid fusion. The problem, however, occurs when we look at the left side of my hardware. The screws in my cervical vertebrae are solidly fused and secure, but the screws in my skull, on the other hand, are another story. These screws are fairly short, at only 6 mm long, which is about ¼ of an inch. These screws should be completely through the bone of my skull and the head should be flush with the bone. This is how they are on the right side, but on the left they are only 3mm in the bone. This means that they have loosened and are halfway out of the bone they should be in. In addition to this there is no bone formation on the rod as there should be. This means that the hardware is not solidly fused and the left side of the craniocervical junction is unstable.

We do not know why the fusion has failed on that side and the screws have come loose. I could have bumped my head and loosened the screws or they could have just wiggled loose over time. We believe that there was good bone formation initially, but for any number of reasons the bone has been reabsorbed (the body just breaks it down and metabolizes it) and the screws have wiggled loose and are not holding my skull securely to my spine anymore.

Now my symptoms are not presenting the same as they did when I needed my first fusion. The largest complaint I have had is headaches that are present on the top of my head. They feel like my head is going to burst and the pressure is incredibly intense. I have also had some blurry vision and ringing in the ears. These symptoms all lead us to think that I have pressure issues. The headaches and pain I had prior to my initial fusion were all in the back of my head, not up on the top and involved far more neck pain and discomfort. We are not 100% sure why my symptoms are presenting differently this time. There are a couple of guesses we have 1) my symptoms are not from the fusion becoming unstable and this is a completely separate issue to what is actually causing my symptoms (even if this is the case I still need to address the fusion not being solid because the loose screws in my skull are at risk for dislodging completely from the bone) 2) The nerves on the back of my head never regained function after my last surgery so I have a numb spot on my head. The symptoms that I previously felt on the back of my head may be being referred up towards the top of my head. 3) since the instability is isolated to a higher location, only my skull vs my skull and my vertebrae, and it is only on one side of my head I am feeling it differently than before. I do not have the neck pain and fatigue that I did before because half of my fusion is still solid and my brain stem is not being nearly as compressed as it was previously.

Given those thoughts we cannot know which is truly the reason for my new and differing symptoms. At this point we have to address the fusion being loose because like I stated before it is hazardous to leave loose screws in. My brainstem may also begin to be compressed again by the instability, which is not something we want to go through again. This will be addressed by performing a fusion revision. The hardware on my skull on the left will be removed and replaced with a new hardware and reconnected to the vertebral hardware. The vertebral hardware will not be touch and the right side won’t be touched wither.

The question of whether or not there are additional issues going on, specifically issues with my intracranial pressures, is something that we will have to table until after I heal from this fusion. If my symptoms are resolved after I have healed from the surgery we will know that the loose hardware was the cause, but if they do not then we will pursue further testing of my ICP. I will go into that more in another post, but unfortunately we cannot do the ICP test during this surgery and subsequent hospital stay as we are worried that the post-surgical pain I will be in will give us inaccurate numbers and thus cause the test to be invalid.

Well now that I have written you all a novel of an update I will write a subsequent post shortly with the information about the surgery and details pertaining to that for those that did not want to make it through my novel to just get the date and essential details! Thank you as always for all of your prayers and well wishes, they mean the world to my family and I!