A New Diagnosis

It has been a busy couple of months, my friends.

I’ll start with the biggest news first. Steph has had her chiari decompression and spinal fusion surgery. Things have not gone smoothly for a whole number of reasons that I will leave for her tell of when she is healed up and ready. For now, send up prayers for healing and comfort. A small cerebral spinal fluid leak developed in her dura at the surgery site this week. The collection of spinal fluid caused an infection which formed an abscess, which in turn caused her incision to open back up. This necessitated emergency surgery to clear the infection, remove and soak her bone graft in an antibiotic solution, and patch her dura (the thin layer of tissue between the skull and brain). She has had a PICC line (a thin tube inserted in to one of the main veins leading to the heart) inserted for an intensive round of antibiotics and a 4-5 day hospital stay. She’ll have another six weeks of iv antibiotics with an at home nurse once she is released from the hospital, and a round of oral antibiotics following that- possibly indefinitely. An infection that close to your brain is more than a little scary. Steph is a warrior, you guys. She is steadfast, faithful, witty and wonderful. She has incredible intuition, a strong voice, tenacity and a heart full of righteous fire.

There is something really beautiful about living a shared experience with another person. An automatic kinship. An understanding where words fail to explain. Never before had I met another person who needs to pause band practice to pee as often I do. This is a gift.We are too busy living in the moment to ever remember to take pictures when we’re together- so this is what I’ve got.

On to my last few months:

Not long after my last neurosurgery appointment in September we (my husband and I) learned of a neurosurgeon who specializes in EDS who was in the process of being recredentailed at the hospital where I received most of my specialty care. We had been keeping tabs on him and trying to get moved over to his service. This was slow going- no one at the hospital really seemed to know what was going on with his schedule, when he would be there, if he was seeing patients, if he was actually working there at all.

At the beginning of October I started having some strange new cardiac symptoms. My heart rate was up to 160 beats per minute just from standing and brushing my teeth. Tachycardia is normal for me with standing but my heart rate rarely rises above the 130’s. My heart rate while at rest was down in the 40’s.

I went to physical therapy and was sent home because my therapist didn’t believe it was safe for me to be exercising. She advised me to get an at home pulse oxidation monitor and to document my heart rate and oxidation levels so that I would have more information to give to my neurosurgeon at our next appointment.

The next day I had a really bad headache and just a general feeling of being unwell. My heart rate was at 38 beats per minute and my pulse oxidation was was bouncing between 92 and 96 percent. I was exhausted, not able to think clearly and needed a fair amount of help moving from my chair to my bed.

Craniocervical instability can cause bradycardia (abnormally slow heart rhythms) and fluctuations in rate which return to normal when the head is properly supported. We called the neurosurgeon to see what we should do and were advised to go to the emergency room and to follow up with my cardiologist. Our local hospital staff are really great with EDS patients- they know a decent bit about EDS and the possible conditions and complications that can come along with it. They know they don’t have the equipment and specialists available there to treat us and are honest and up front about it. I had been to our ER before for heart rate related issues and the most they did was give me IV fluids.

I had the equipment at home to monitor my vitals and could self hydrate so we opted to wait until the next day to seek medical care as I already had an appointment with my cardiologist scheduled. Also- it’s flu season and I am not about that.


I was there for around 4 hours, having tests done and being monitored. She spent 30+ minutes with me going over new symptoms, and doing a thorough physical examination. She did not believe my new cardiac symptoms were related to my POTS or my heart at all really- she asked if I had a recent MRI and upon hearing of my last neuro appointment a) wished that I had a disc of my images to go look over right there and b) told me to find a new neurosurgeon and listed a few to try and get appointments with. She wants to hear that a neurosurgeon has a plan for me at my next appointment with them.

It is hard not to question yourself and not to wonder if maybe you are being dramatic or crazy and this was a validation I needed to keep seeking the doctors I need.

After having both my cardiologist and physical therapist confirming that craniocervical instability was something that needed to be further looked into and addressed I was finally placed on the service of the EDS neurosurgeon I had requested an appointment with.

I saw Dr. R a few days ago and the appointment went really well. He listened to me and took notes on my symptoms. He asked questions and he answered mine- not just quick “because I said so” answers. It didn’t bother him when I questioned him.

He doesn’t think tethered cord is something to worry about right now- I might have it, I might not, but either way it isn’t high on the priority list. It was high on my priority list. Tethered cord can cause irreversible incontinence issues and I would really love to not have that problem- which I let him know. He explained why he doesn’t believe tethered cord is an issue that needs to be addressed right now and it made sense to me.

He went through my images and did all the measurements needed to evaluate craniocervical instability in an EDS patient.

He taught me that protractors are useful to some people beyond grade school!

He gave me a diagnosis.

Craniocervical Instability. With a Harris measurement of 16mm (normal is 12mm or less) and 8mm of sliding movement of my skull based on positional changes (normal is 4mm or less), and borderline instability findings with rotation of my head. https://www.ehlers-danlos.com/2015-annual-conference-files/Henderson_0.pdf

He sent me to be fitted for a cervical collar right away- it’s beautiful. You’re jealous.

He is getting the ball rolling on the issues in my head that need to be investigated and fixed before we talk about fusion surgery. There is restricted flow of cerebral spinal fluid which may require a shunt, and he put in a referral for me to got see the best vascular neurosurgeon for EDS patients to make sure there are no issues in that regard. I have to do a lot of physical therapy for my cervical spine- which I’m not excited about because I’ve done it before and it didn’t help me the first time. It actually hurt a lot, but not as much a surgery hurts so here is hoping I have more success this time.

I am really thankful for Dr. R. I am also really thankful for my other neurosurgeon, Dr. D. Dr. D is still my doctor and he is working to fix the pressure in my head. He is by the book, meticulous and doesn’t take his job lightly. He ordered every test and scan I asked for and facilitated my other care- even when we were not in agreement about what my needs were.

Dr. R recognizes that not all patients and circumstances were included when the book was written so sometimes you have to be part of writing the next chapter. I need them both. They are both good doctors and good humans.

“I hope you’re proud of yourself for the times you’ve said ‘yes,’ when all it meant was extra work for you and was seemingly helpful only to somebody else.” -Fred Rogers

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