Saturday, February 25, 2017

Surgery for Craniocervical Instability (CCI): Part 1

NEW! People You Should Know Podcast: had the wonderful opportunity to share my story for a new podcast. Click here to take a listen Megan King- Ehlers-Danlos Syndrome Make sure you listen to the other podcasts that are featured. There are so many amazing individuals with amazing stories that you'll most definitely want to take a listen to.

WARNING: I'm not going to sugar coat anything with this post. I'm giving full warning this is a post that might be difficult for some to read. To be completely honest, this post wasn't the easiest for me to write because what happened was traumatizing. In fact it has been the scariest year yet. There need to be resources though because there are many people in the EDS (Ehlers-Danlos Syndrome) communities who need this surgery. I think it's important to write about so people can ask questions. I also think it's really important for them to be able to read about this surgery from a patient's perspective. I know I would have liked to read about someone who went through this. The thing to keep in mind and remember is everybody has different experiences with this surgery. This was mine. I'm giving full warning there is one graphic picture from surgery. I will give ample notice before you get to that photo. 

August 25, 2016 It was halo removal day, and I was 100% horrified because I felt my skull slide forward in May 2016 when I was being fitted for my CTO (cervicothoracic orthosis aka a really sturdy neck brace). All that kept going through my head was, "What is going to happen to me when all the bars to the halo are removed?" My appointment was at 9:00 a.m. In the car I put music on to try to distract my brain, in addition to doing a word search puzzle as we made our way to Chicago. It wasn't working well when my CTO was sitting directly to my left. When my parents, and I arrived at the hospital, Every. Single. Step. was filled with much dread, and fear as I walked the hospital hallways leading to my neurosurgeon's office. The nerves were making me feel so sick to my stomach. 

As I waited in the lobby for the medical assistant to call my name, the anticipation was building like one getting ready for the first giant drop on a roller coaster. When my name was called, I wish I could have sprinted the other direction. I did not want my halo removed. I didn't run; I reluctantly walked back to the room. Shortly thereafter, Dr. F walked into the room with his neurosurgery fellow (Dr. V), and a medical student. 

Before starting the removal, Dr. F discussed the process to my mom and I. They would loosen/remove the bars, the vest, and get me into my CTO. I told Dr. F I did not have a good feeling, and didn't want my halo removed. He said I've grown an attachment to my halo because it has helped me so much, and then jokingly said I couldn't keep it on forever. I voiced my concerns but the only way we would know for sure if I was unstable was to remove my halo. Dr. F asked if I brought my CTO and I pointed to the chair. At that, Dr. F moved a chair to the center of the room and had me sit down.

I reluctantly sat on the low back chair directly across from my mom keeping eye contact with her the entire time. My hands were clammy, and my heart felt like it was going to pound out of my chest. Dr. V started to unloosen the bars while Dr. F went to grab an extra wrench since I accidentally forgot mine. In the time it took Dr. F to grab the additional wrench all hell broke loose. When Dr. V loosened the final bar I felt my skull-C2 slide into my throat. I simultaneously started gagging, and flew my L hand to my chin to shove my skull backwards. I then tilted the chair on its back legs to let gravity keep my head back. Dr. V asked what was wrong, and told me not to lean back so I don't fall. When I could finally speak, because I was crying & couldn't catch my breath, I told her I'm leaning the chair back to allow gravity to help keep my head back because I felt something slide forward. She asked me to bring the chair forward and when I did the same exact thing happened; I felt my skull-C2 slide, I started gagging, I pushed my skull back with my L hand again, and leaned the chair back. She then tightened the back bars again on my halo. Dr. F came back shortly thereafter. I looked at my mom with panic, tear-filled eyes; she looked back at me the same way with panic (minus the tears or else they'd have her leave the room).

This is not a video of my neck. I found it on Youtube. This "just" shows instability at C1-C2. This is exactly what I felt if it showed the skull sliding forward too. Pleasant, right? (Not a graphic video. It's just a movement X-ray) Video of: C1-C2 Instability Video

When Dr. F came back in the room, he was not prepared to see me in tears. In a very concerned voice he asked, "What's wrong?!" Dr. V told him that I felt slippage in my upper C spine, and I started gagging. Dr. F had a significant look of concern on his face. He said we have to loosen the bars again and remove the vest to see if I could withstand the CTO brace. He came on my left side, while Dr. V was on my R side, and they loosened the bars. Yet again my upper C spine slid forward, I shoved it back, and leaned my chair backwards. At that point Dr. F immediately came behind me and held my skull up as Dr. V got me into my CTO brace. When it was on, Dr. F let go of my skull. I went back to leaning my chair backwards because I started to gag again. It was then when my mom told Dr. F that I've been nauseous every time I eat, I throw up every time I brush my teeth (not from hitting a gag reflex), I'm burping constantly, and I always have the hiccups. Dr. F found this information (which I told my mom not to mention because I didn't think it was a big deal) quite interesting. He said those symptoms indicate that my nerves are hypersensitive in my upper C spine. After a couple minutes of being in the CTO brace, my R shoulder started to tremor/shake. Dr. F saw this, and said he wanted to see if I was able to stand/walk. 

When I heard the words, stand and walk, come out of Dr. F's mouth, my body filled with trepidation. I told him there would be shaking down my R leg like I got when my skull slid forward in May. He said he just wanted me to try. Dr. F was on my L and Dr. V on my R. I was in the shoulder brace that was made in MN so they couldn't lift me easily. I pushed myself up with my L leg, and the R side of my body felt completely disconnected. I had weakness. My R leg was spastic and shaking all over the place. I couldn't put weight on my R leg, and couldn't even get my body upright. I was so scared because my symptoms had never been that bad before. Dr. F and Dr. V both grabbed me under my arms to get me back into the chair. Then all of a sudden I had the hiccups, and started burping. I started to cry...again. To say I was overwhelmed is the biggest understatement ever. I think I scared the medical student too. She looked horrified as all of this was happening.

This was my walking on June 2, 2016. This video, of me, is absolutely nothing in comparison to what I experienced August, 25, 2016 Neurological Symptoms Down R Leg

After this series of events unfolded, Dr. F put me back in the halo brace, and explained what was going on. He said my craniocervical junction is very unstable, and my muscles/ligaments are too weak to support my head/upper C spine. I was given two options 1.) fuse the rest of my neck (skull-C2) which would mean I would never have mobility in my neck again, or 2.) wear the CTO brace, do physical therapy, and IF everything went according to plan I could maybe get a year at most before being back in surgery. I told Dr. F if surgery was inevitable then just do it and be done. In my head I was thinking, "My skull keeps sliding forward, I'm getting neurological symptoms, and I can't even walk". Dr. F had me come back in the afternoon so I had time to process everything, and let him know what my decision was.  

When I went back to my appointment in the afternoon, Dr. F didn't really give me an option. He said surgery was needed, and I agreed with him 100%. Surgery was set for 5 days later. Talk about a crazy week. Drive from Colorado to Minnesota. Be told you need scapula surgery. Drive home. See Dr. F the next day. Find out you need neck surgery again, and then have it 5 days later. I emailed Dr. E my shoulder/scapular surgeon about what had happened, and he said he was scared about that, and wished me good luck. 

In hindsight I wish I had a short video of this day as words don't adequately describe how horrible this day way. 

August 30, 2016 It was surgery day...again...number 27. It's not possible to write every thought that was going through my brain. I was so scared though. When I had my halo application April 5, 2016 I never thought it would be the last day I would move my neck again. This surgery held significantly higher risks than any other surgery I've had. I knew I needed it though. At this point, all I knew was a plate was being secured to the back of my skull with screws and C1 and C2 would be stabilized. I didn't know how far down the fusion would be because Dr. F had to connect the new hardware to my hardware from four months prior. 
At my first appointment with Dr. F he told me he loves dark chocolate. Before this surgery my parents, and I stopped in the hospital cafeteria and bought some 80% dark, sea salt Ghiradelli chocolate. This made him a very happy man. This picture was right before meeting the amazing anesthesiologist, and being wheeled back to surgery. To my surprise, the med student who was at my appointment on the 25th was shadowing anesthesiology this day. My parents and I were told Dr. F had the OR booked long, and to expect a pretty long surgery. 
These two have been by me for every surgery. I'd be lost without them. I can a see their nerves when I look at this picture. 
Very appropriate quote right before being wheeled back to surgery!

The time had come where I was wheeled back for surgery. I told the anesthesiologist I did not want any amnesia meds beforehand. I like to remember knowing I was brought back to the OR. Getting me intubated was extremely hard. I was still in the halo, but now I was hard fused C3-T2. They gave me some meds to relax me but EDS patients tend to metabolize them a lot faster. The doctor used a camera to get the tube down my throat. They covered my face with a drape, but not enough to cover my eyes. The anesthesiologist was showing the med students where my vocal cords were on the monitor. As he explained, I listened and watched. I found it pretty fascinating. Then they went to put the tube down my throat. Before doing this they numbed my mouth, and back of my throat. They tried intubating me 5 times that I remember before I grabbed the anesthesiologist's hand, and said to versed me (amnesia meds). I was gagging so bad, and it was throwing my shoulder blades out, and I was feeling my muscles tear from them. I started crying because the pain was so bad. That's the last thing I remember before waking up in ICU. Pain. Lots and lots of scapular pain. 

When I kind of came too, I knew there had been issues getting me intubated. I still had a tube down my throat, but my eyes were shut. I was so sleepy. I heard a voice to my left, which I found out later was Dr. F, who told my parents he was going to keep me sedated all night. As soon as I heard those words I knew things were different than surgery in April. The next thing I knew it was morning because the medical staff was reversing the sedation, and I was waking up a little bit. 

August 31, 2016 I was alert but was confused. My wrists were tied to the arm rails of the bed (not because I was being combative). At first I didn't know why they were tied. Then I realized I was still intubated. I couldn't talk, and I was panicking. I'm sure my eyes said a lot. The tube makes you gag. I looked at my mom to see if she could get the restraints off me. She talked to the nurse, and reassured them that I wouldn't pull the tube out of my mouth. Fortunately they untied my wrists, but they kept their eyes on me like a hawk...which is what they do in ICU. The vent was obnoxiously annoying making this whooshing/squeaky noise as it pumped my body full of oxygen. 

Might be hard to tell but I'm fully awake in this picture. Before the tube could be pulled, I had to prove I could breathe on my own for 30 minutes. Every time I stopped breathing the clock started over. It was incredibly hard. I lost track how many times the clock was restarted. Every time I'd doze off to sleep I'd stop breathing. Finally, I succeeded and they could remove the breathing tube. 

Complete relief, and exhaustion that the breathing tube was out. It hurt so much to get the tube out, and to get the "sticky pads" off my cheeks, and back of my neck. As soon as the tube came out I had to say my name. I didn't have a ton of surgical pain...yet. I was still pretty sedated, on heavy pain meds, and slept the majority of the day. 

This is the cartoon version of what I had installed. Directly below this picture is an actual surgical picture. If this type of thing grosses you out, keep on scrolling past. 

Top is my skull with a plate attached to it. This was a posterior craniocervical fusion. C1 has wires around it. The new hardware was attached down to C5 which is directly below the bronze horizontal piece. The horizontal crosspiece adds additional stability. 

X-ray of my brand new hard fused neck skull-T2...24 screws later, 2 new wires, a plate attached to my skull, and two rods. Surgery was 7 hours long.
The x-ray below is what my neck looked liked before this most recent  fusion. It looks alien-like or something futuristic to me. 

This is where I am going to wrap up this post up. I'm going to have to break the CCI (Craniocervical Instability) Surgery into more than one post because there is just too much to write about with the hospital stay, and what occurred in the coming days. Keep your eyes out for part 2.

To all my friends who are fighting, recovering from surgery, raising funds to get this particular surgery KEEP FIGHTING. You have many people rooting you on, and it will get better! Feel free to ask questions :) 

* *If you have a Facebook account, click here and "LIKE" my page: Meg's EDS Medical Journey
* *Please SHARE. There is NOT much information on Craniocervical Instability (CCI) at all. People who are symptomatic need to be more aware, and especially medical professionals. It's far too common for people with EDS to develop CCI and there aren't enough specialists that treat both. Sharing spreads awareness about EDS (Ehlers-Danlos Syndrome) CCI and life in a halo. I know there are many who would appreciate it. 
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* *As always, if you wish to communicate, you can email me at To those that are shy to email me, I can tell you now, you most definitely are not the first. 


  1. Thank you sweetheart!! First I just wanted to say that you have to love yourself and not put too much pressure on yourself. Let people love you and take care of you. I am still working on that myself. Second I appreciate you speaking out. You are brave. Like your quote said. About me: I've had a decompression for Chiari and a cranio-cervical fusion that had to be redone because a rod broke, also I've had a VP shunt placed for hydrocephalus and a tethered cord surgery. I also have EDS. I'm still in pain every day and have been ill most of my life. I'm 46 years old and don't remember much of the past 10 years of my life due to trauma and the surgeries. Thank you again for sharing this. It means a lot. I wish nothing but love and health and peace to you. Take care... Saryn
    My email...

    1. Hello Saryn, thank you for your lovely message. I will send you an email :)