Spinal Fusion #3

Let’s start off on a positive note! The shunt I had put into my brain to drain my spinal fluid has been phenomenal! We have adjusted the settings slightly, but we are at a good place now and I have been so happy with it! My headaches when laying down or bending over are virtually gone and it has been such a relief. I thankfully have not had any complications with my shunt at all, which has been a huge blessing!

I did end up having my fusion revision back on 4/30/18 (about four months ago). All things considered the surgery went really well. The plan was to replace the cervical screws on the left, which we knew from imaging were loose, and then extend the fusion down to C4. Previously the fusion was down to C2, but since we had failed twice at getting a stable fusion on the left we decided to go down further to try to anchor the fusion better. Our hope was that we could still leave all of the right side hardware in place since it was so stable and just add on to it in order to extend it.

When my wonderful neurosurgeon got into the operating room plans changed just a little bit. It turned out that the skulls in my skull on the left side were also very loose. The surgeon had to take those out and replace them in a new location on my skull (the third spot now). I also had a congenital fracture/deformity on my C3 vertebra. This is not a big deal in itself and did not cause any structural issues, but for a fusion you need to place hardware two level below the instability. For this reason, we ended up needing to extend my hardware down to C5 instead of C4 as we initially had planned. We were able to leave the right hardware intact and just extend it down further, which was what we had hoped for!

The surgeon was very happy with the surgery and we were hopeful that this would be the last fusion I would endure. Unfortunately, less than two months after this surgery (6/29) I was rear ended while on my way to a doctor’s appointment. It was an extremely minor accident. My car needed some cosmetic repairs, but was not too bad off. My service dog was scared, but physically absolutely fine. Any other healthy adult would have been just fine. Me? I spent the day in the emergency room. Although it was just a tiny tap and I had my cervical collar on we needed to make sure my neck was ok. Now the rods and screws we put in during the surgery are like a scaffolding. They are there so that bone can grow on top of them and the bone is what causes a solid fusion. Bone growth is a lengthy process that can take a year or more to be completed. So at not even two months out the bony is no more solid than Jell-O. It does not yet hold anything together. The concern was that the impact could have loosened one of the screws since the bone isn’t solidly holding my fusion together yet.

Well, they did an x-ray in the ER and it looked different from the x-ray we had done post-op. The next step was to do a CT scan. Unfortunately, the CT scan did show that the screw in the C5 vertebrae on my left had changed position. It wasn’t clear how much, but thankfully we were able to wait for my surgeon to get out of the OR with another patient so that he could view the scan. He felt comfortable with sending me home and just watching the screw’s position over time.

I had a follow up appointment with my surgeon the beginning of August. We did more x-rays on that date. The good news is that the screw did not change positions from the end of June to the beginning of August. This means that the screws positon in not progressively getting worse. The bad news is that we are not sure what this change in position means. Since the screw is at the bottom level and is used as an anchor it could cause the whole construct above it to become unstable over time. It could also be that it does not affect any of the other screws and is below the level of my instability so it doesn’t cause any issues. Time will tell on that.

I go back to the neurosurgeon the beginning of November and we will do more x-rays then to determine if there is any change. Right now we don’t really have a plan. My neurosurgeon is working with other surgeons to come up with a just in case plan b. Right now I am still in my cervical collar 24/7. I have been in it for an entire year next week (9/17) and will be in it for the foreseeable future.

Other than that my symptoms have been really good! I have had a great return of sensation to my right side, which is exactly what we were hoping for! I have been having a lot of pain from the hardware on the back of my skull. Anytime I lean my head against a head rest or a pillow I get a terrible headache on the back of my head. My surgeon is hopeful that this will go away with time, but right now it is quite bothersome.

There was another fairly major complication with my surgery, but I will save that for another post.

Please pray that we don’t need to do any more interventions and that my symptoms start decreasing.

Neurorsurgeries- Monitoring and Shunt

Hi everyone! The neurosurgery saga continues! This is a long update, so if you have any questions please let me know! 

If you all remember I was supposed to be in a cervical collar for six months in order for us to see if we could get my craniocervical fusion to stabilize since my screws from my fusion revision last February (2017) came loose again. I made it to 4.5 months and was feeling so rotten that I decided to call my surgeon and get in sooner. I saw him last month (2/6/18) and he ordered a number of imaging tests on my neck and spine. I had a couple of different x-rays done, a CT scan, a full spine MRI, and a bone density scan.

After we got all the results back we decided to move forward with a more extensive cervical fusion. The x-ray showed that everything looked the same as it did back when we imaged in September. We then did a flexion extension x-ray in which they took images of my chin tucked into chest and bent my head back as far as it would go. This shows differences in your neck with movement. The results of this x-ray showed that despite my loose screws my fusion was holding everything in place well enough that nothing is moving. Next, we did a full spine MRI. This shows the spinal cord and discs in your spinal column, which you cannot see on an x-ray or CT scan. This showed my tethered spinal cord release in my lower back was still ok and that my cord did not reattach itself to the spinal column (which there is always a chance it will). It also showed no pockets of fluid have developed in my spinal column (called syringomyelia), and that my Chiari 1 malformation (the back part of my brain (cerebellum) descending into my spinal column) still had plenty of room from past surgeries we have done to give my cerebellum more room. The one thing this MRI did show is that I have a pocket of cerebrospinal fluid (CSF) called a pseudo meningocele in the very back of my skull. I did not realize that I have actually had this for a while now. This pocket of fluid got slightly bigger since my last MRI a year ago, but it definitely changed shape. I will talk more about this pocket in a minute. Finally, we did a bone density scan to ensure that I did not have low bone density causing my fusions to fail. This test came back perfectly normal for my age and sex.

I had gotten a surgery date of April 6^th, but got a call at the beginning of this week that they had a cancellation and wanted to change my surgery date to Friday 3/9/18! I had to rush around to get everything scheduled that I needed to and was able to get into clinic this past Tuesday to discuss an exact surgical plan and sign consent forms. Once we started discussing the surgery my surgeon admitted that he was not 100% sure that this surgery was going to fix my symptoms. We always know there is a chance that surgery will not help, but he said he felt like he was missing something. He had talked with many other surgeons and other medical professionals and just felt like there was something else we weren’t looking at correctly. I love my surgeon and he highly values my opinion and knows that I am well educated on my condition and the nervous system. We discussed all of my imaging, past history, surgeries, and symptoms. The one thing that has always stuck out to me is that I have symptoms of having high pressure in my brain. I have headaches that get worse with laying down. I had a lumbar puncture done to check my pressure and it was considered borderline. The problem with lumbar punctures is that they only take a reading of the pressure in your brain and spinal column at one instant. It is like taking your blood pressure: you may have a good reading in the morning but have high blood pressure in the evening. The medication used to treat high intracranial pressure is a medication called Diamox. It essential pulls the extra fluid off your brain and spinal cord and drains it. I am on this medication but I can only be on a super low dose. This medication lowers the CO2 levels in my blood, so we cannot increase the dosage at all. The dose I am at is not high enough to control my symptoms or conclusively tell us if the symptoms I am having are from high intracranial pressures.

In order to conclusively determine whether or not I have high pressures in my brain we did something called invasive intracranial pressure monitoring. The specific type of monitoring we did is an external ventricular drain (EVD). During an EVD a small tube is place into a ventrical in your brain. The ventricles are where CSF flows through your brain. This tube is hooked up to a system that allowed them to monitor the pressures in my brain and also drain off extra fluid to see if decreasing the pressures in my brain provided me symptom relief. 

This surgery was actually done last Friday and we have been in the hospital since. I was in the neurological ICU until Monday. We monitored my pressures for those three days and determined that my pressures were normal. They went up when I laid down, but were still within the “normal” range. At this point the surgeon was not sure what he was going to do.  We decided to open my drain to see if lowering my pressures would help relieve my symptoms. My drain was opened on Monday and within an hour enough fluid was drained out of my brain to provide relief for the headache that I have gotten every single time I lay down for the past two years. After having the drain open for almost 48 hours my surgeon made the decision to go ahead and place a shunt later today. 

This is my CSF after one day of draining. As you can tell it has the color and consistency of water.

A shunt is a device that pull fluid from within my brain and drains it down a catheter into my abdominal cavity. It is implanted within my brain and there will be a valve that can be programmed in order to set the pressure that my surgeon wants in my brain. He is thinking to set it low because that is where my symptoms seem to be best resolved. He then will feed the catheter down through my neck, down my chest (around my port), and into my abdominal cavity. The fluid is then absorbed by my body. This type of shunt will allow my fluid to only drain when my pressures go high, which is important since my pressure is not high when I am sitting or standing. The surgery will be short (between 2-4) hours and recovery should not be too bad. The biggest risk from here on out is infection. I will finally get to go home from the hospital after 24 hours of antibiotics, which should mean I will be released Friday morning. This has been my longest hospital stay yet and I will have had two surgeries during this stay. Joseph and I are ready to get home! The other difficult part about this stay is that my service dog Reilly is not allowed to stay with me. He stayed with Joseph’s brother and family for a couple of days and then has been with my parents for the rest of the week. I have never been away from him for more than one night in the last 3+ years, so I am VERY ready to be reunited with him!!!

After I am healed from this shunt surgery my surgeon and I will go back in and have a fusion revision and extension done. I will make a subsequent post about that, but we are thinking that could be as soon as 6-8 weeks from now. Thank you all for your continued prayers and well wishes throughout this journey!

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!

Vascular Neurosurgeon and Headache Update

After discussing in depth with my phenomenal neurosurgeon we have decided to go a different route than what I had expected to deal with my headaches! Since the medication to decrease my intracranial pressure (Diamox) has been helping so much we are almost certain that my pressures are high even though my lumbar puncture did not show extremely elevated pressures. We know my headaches are worse at night and the lumbar puncture was done in the morning, so that was one reason that the readings from that test came back borderline and not high. Given all of these factors we now have two options. The traditional treatment would be to place a shunt to drain excess cerebrospinal fluid from my brain/spine into my abdominal cavity where it would be reabsorbed. A shunt is device that will drain this fluid to reduce the pressures in my brain. Prior to this treatment I would need to have more monitoring to ensure that my pressures are as high as we believe they are.

The alternative to this procedure, which is a very new idea that has very little research done on it, is a venous stenting. They are finding that in some EDS patients the veins in our brains are clogged. This clog causes a blood to back up and raise the pressures in your brain. A stent can be placed in these veins to open up the blood flow allowing the pressures to come back down in your brain. This is a  new procedure that there is little research done on it in EDS patients. There is one doctor at the University of Virginia that is doing this procedure on EDS patients and he is having great success. Here is an article about that doctor and his use of stents in patients who have EDS.

http://www.uvaphysicianresource.com/venous-stent-procedure-providing-relief-to-some-ehlers-danlos-patients/

The good news for me is that there is another doctor, a vascular neurosurgeon, who is starting to look into this procedure at Cincinnati Children's Hospital. I am located not even 15 minutes from there, so obviously that is extremely convenient for me! To make things even better this doctor trained with my phenomenal neurosurgeon Dr. Rekate in New York, so he is very familiar with EDS and Chiari. I am not yet sure if I even have venous insufficiency or am a candidate for this procedure. What we need to do first is to get a full brain MRI as well as an MRV. An MRV is a special kind of MRI that looks specifically at the veins. This will show us if there are any clogged veins or not. If there are clogged veins then I will go ahead and set up a consultation with this doctor in order to develop a plan and hopefully schedule a procedure to place the shunts. I will be having the MRI/MRV done tonight and then the results will be sent to the doctor and I should hear back from him in a couple of days. I will also send a copy of the disks up to Dr. Rekate in New York so that he is able to view the scans himself as well. I have had to be off of the Diamox, the medication that lowers my intracranial pressure, for the whole weekend so that we can see what my brain looks like with the high pressures and my headaches have been severe all weekend! This is just one more confirmation that it is my pressures that are high that is causing the headaches. I am looking forward to being able to resume the Diamox tonight after the scan!

The stent procedure is a much less invasive surgery than a shunt surgery and is much less risky. Shunts can become infected and often need to be revised, meaning surgery is done again to replace or fix parts of the shunt. They are not an ideal solution, but if that is the route we have to go then I will pursue that option as well. The stent procedure carries many fewer risks and complications, so please pray that this is the solution we are able to pursue.

I have some other updates coming up as well, but I wanted to update on this as we now have more information. I will update again after I hear from the vascular neurosurgeon following my scan tonight and we are able to begin to develop a plan.

Headaches after Fusion and Decompression

As I mentioned in my last post I have been having terrible headaches the past several months. They are different than the headaches I had before my brain and neck/spine surgeries. They are on the top and feel like the top of my head is going to pop off! I did not have any headaches for about 2.5 years after my decompression revision and fusion, where they made more room for the back of my brain and fused my skull to the top two vertebrae in my neck. I am extremely luck and feel very blessed that I did not have any headaches because this is simply not the experience of many patients! That being said, these headaches kind of blindsided me because I had gone for so long without them that I was caught off guard by them!

After a visit with my primary care physician (PCP) we decided to order the CINE MRI, as I discussed previously, which is different than a traditional MRI because it looks at the flow of the fluid that surrounds your brain and spinal cord (cerebral spinal fluid-csf). My PCP thought that it appeared that my CSF flow was blocked by what appeared to be scar tissue from my previous surgeries. We sent the disk off to my neurosurgeon in New York so that he could take a look at it. He does not think that scar tissue build up is the issue and feels that there is something else going on.

In order to find out what more is going on he wanted to do a lumbar puncture. A lumbar puncture is exactly what it sounds like: a needle is used to puncture your lumbar (lower back) spine. They are able to do lots of labs with  test, but for me they wanted to see what the pressures of my CSF were. Your CSF pressure is the same throughout your brain and spinal cord, so they can measure the pressures through this lumbar puncture. My pressure was 16, which is borderline high. It not super elevated, but it is higher than you would expect to see.

Since my pressures were elevated somewhat we decided to start a medication to reduce the intracranial pressure. This medication is called Diamox and must be started very slowly. After building up to the full dosage of this medication (250 mg 3x a day) it has been helping significantly with my headaches! This is great news.... but there is a side effect that has come with this medication (of course it couldn't be that easy!).  I have been having horrible pins and needles in my hands and feet that have been occurring since I have gotten on this medication.  This is an extremely painful sensation and I say it feels like little elves are shoving tiny needles into my hands and feet. The good news is that we do not think this symptom will continue after I discontinue the medication, but the bad news is that we think this side effect will continue for as long as I take the Diamox. Of course we must now decide what to do about this since this side effect is becoming intolerable.

It is looking like I am going to have to discontinue the medication, which of course is not ideal because it is helping my headaches so much! I have a Skype appointment with my neurosurgeon in New York tomorrow afternoon so hopefully we will get more information from him then! Please pray for us to get guidance and peace for whatever the next step may be.