Countdown! Many people are bewildered when I explain my condition and upcoming surgery.  So, I will try to concisely explain. In a nutshell, I have a genetic condition called, Ehlers-Danlos Syndrome.  This condition causes problems with connective tissue throughout my body.  Because connective tissue is EVERYWHERE, including around organs, I will always struggle with chronic illness and pain in some form. The area of my body that has been most dramatically affected is at the connection between my head and neck.  Compromised connective tissue there has created a syndrome called, craniocervical instability (see quote below from NSJH Newsletter ).  Over a period of years, this has caused functional cranial settling (skull rides lower at the top of the spine) with basilar impression (odontoid bone in front of the brainstem abnormally protrudes through the opening at the base of the skull, and pushes against the brainstem).  Oy.

Craniocervical instability (CCI) is a condition where the cervical spine is unable to maintain adequate alignment and support of the cranium during certain movements, causing the head to “wobble.” The underlying cause of CCI is structural: one or more bony and/or ligamentous anomalies involved in the craniocervical junction prevent normal range of movement of the head and neck. In many instances, the instability reduces the cervical spaces through which the brainstem and spinal cord pass, causing pressure on the delicate soft tissue, with neurologic and vascular consequences as well….CCI tends to worsen over time, eventually causing irreversible damage to spinal nerves and muscles and loss of physical strength and mobility….

Because my symptoms have become so severe (I have been pretty much bedridden for two years), I will be having a craniocervical (or occipitocervical) fusion surgery.  This should pull my brains out of my butt, and back in my head where they belong!

…Days before the surgery, the patient goes through several hours of advanced dynamic MRI. This pinpoints the location of millimeter-thin compressions that are causing brain and spinal cord symptoms. We also measure several critical craniocervical angles against norms; these provide a basis for intraoperative surgical stabilization and fixation. The surgery itself is very delicate, typically taking 5-6 hours. After the patient undergoes general anesthesia, the surgical team makes one opening, approximately 10-15 inches in length from the top of the head to the back of the neck. As the surgical field is highly trafficked with vertebral arteries and veins, the brainstem and critical cranial nerves, each step in the realignment of bones, the remodeling of angles and in the relief of compression points requires exquisite surgical precision. A unique feature of our approach is the use of intraoperative ultrasound during surgery, in addition to continuous brainstem and spinal cord monitoring. This guides precise adjustments during our realigning and stabilization of the cranium on the cervical spine. Postoperative recovery in the hospital takes 4-5 days, followed by rehabilitation and a gradual return to normal activities.

Most people who have craniocervical instability also have a Chiari malformation.  Since I do not appear to have this, my surgery will be somewhat simpler than the typical patient.  At last, an aspect of my self that is less complicated than the rest!  Almost all of the posts I have read from other patients report dramatically improved quality of life.  Here is one I stumbled upon yesterday (MedHelp.org):

…JUST 3 wks ago…I was still lying in a recliner with awful sx, desperately awaiting our trip to MD to finally have my decompression/craniocervical fusion surgery…just about all of my pre-op symptoms are resolved. …I was a walking time bomb! My brainstem & spinal cord were so compressed that when he did the sensory evoked potentials before surgery it took way longer than it ever should for the electrical message to reach my feet, which indicates spinal cord issues. It also took him 30 pounds of traction force to lift my head off my spinal column so that it wasn’t compressing everything. He used a rib to help build up my skull so that my skull won’t fall down on my brainstem/spinal cord and once everything was in perfect alignment and just where he wanted it, he used plates and rods and screws to hold it all together. He was able to put my clivo-axial angle back to perfect so that there is no more brainstem compression. He removed one of my nerves that would have been compressed by the hardware to avoid future problems. I haven’t noticed anything at all so guess it’s not an issue! He went down the list of all the symptoms that I had pre-op to see how they are doing…..I think just about every one of them have resolved. I told him about how amazing the dreams are that I’ve had since surgery….I literally haven’t dreamt in years so the surgery has obviously helped my sleep cycle as well. I hope that means less fatigue!…

Symptoms resolved?  Will my symptoms resolve like they have for other patients??  Improved quality of life…enjoying the world with my family…riding my bike…going to the movies or out to dinner…doing volunteer work like my grandmother before me…preparing Friday night dinner, or even a sandwich…riding in a car to look at Christmas lights…picking up a dropped ice cube…doing laundry…going for a stroll around the corner to visit neighbors…maybe even driving myself to the grocery store.  There are so many things I want to do. My hope is to live, love, share, laugh, cry, learn, and experience the gift of life in a manner that will generate joy for me, and those around me.  During these difficult days before my surgery (33 to go), I try to keep thinking about the hopes I have for the future…with the knowledge that they will probably happen. Thank you for your prayers.

‍