Nina's CCI Battle
Awareness For Ehlers Danlos & Cervical Cranio Instability People
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What is CCI
What is CCI Cervical Cranio Instability?

Cervical Cranio Instability is a complication many young children and especially young women can suffer from. Its a very serious life threatening complication that happens because of the EDS III. The reason CCI is very susceptible for people with EDS is because the ligaments in our necks are stretchy and therefore are not strong enough to hold up our heavy heads (or skull). As a result of the head being so heavy it wobbles, the skull slides and moves around and is unstable, because of this movement it causes brainstem compression, which damages the brain stem and top of the spinal cord. This has catastrophic implications for the sufferer.

Because the the head wobbles, and can sink onto the neck, the condition can also be nick named a pumpkin on a toothpick.

Nina's individual case to put simply, is her head is to unstable on her neck.  From the front of her neck her ligaments are weak and this is causing the front part of the skull to sink and the downward motion compresses the brain stem. From the back of her neck, her ligaments are again to weak, but this is causing the bones to move further apart, causing a huge gap from the back of her skull to her neck. This has the opposite problem, there is a huge wide gap from her skull to her neck. The ligaments have dislocated and there is no protection to her brainstem. Many people who suffer from this type of neck dislocation do not survive. Its a matter of time when this will happen for Nina if she can not get surgery. Here are some photos to show you the lengths we are prepared to go to just to feel better and live our lives again.


   

     

   
 



This is my good friend from America who so kindly let me demonstrate just how wicked spinal issues that are caused by EDS really are. My friend, unlike me has all three common EDS complications to deal with, Chiari, Instability and a Tethered cord. Of all of which have been diagnosed and operated on by the wonder Dr Fraser Henderson in Maryland USA. Others have visited the Chiari Institute in New York where there are a whole specialist team both for adults and children.

My friend in this picture wanted me to use this on my website to demonstrate the difference 3 years can make, and what a downhill struggle she has had to cope with. She will not mind me saying she was a wonder, active member of the community, and she was (is) a fantastic sculpture artist. EDS is wicked when it strikes any part of the body, but in particular the spine, because this is where the spinal cord and brainstem are protected in!! Early diagnosis is key in taking into account preventative PT, surgery and leading a sensible lifestyle whilst not giving into EDS, just respecting it is there and not pushing your body too far. This is why i feel so strongly in raising awareness with my campaign.





The next part of this page are a lot more in depth and complicated and far more medical!!! I feel it needs to be written to help others.

Underneath are my MRI scans. Both standing on the right and laying down on the left.





The importance to having a stand up MRI when you have EDS is to see the effect that gravity has on your body against gravity. Our skull and ligaments are supposed to move no more than 2mm between positions. From my diagram and lines drawn you can clearly see my skull is shifting more than it should. By 4mm in fact.


    



This is the fusion that is needed to correct c1-2 bone.

 You can see the metal rods


The red line is also demonstrating how the cerebellum is moving quite dramatically from the back portion of the skull (the occiput).  This movement is all caused because of the instability or "wobbling" of the head. It is no wonder many patients suffer from nausea, balance, dizziness, gait, movement problems, when this part of the brain is responsible for these functions and its moving and being squeezed by any movement! The distance from the back of my skull to my C1 is excessive also, so much so it is called an Atlanto Occipital Dislocation, there is not a great deal of information on the internet about this because the majority of people who suffer from this type of injury do so in car accidents etc. and they die instantly. Mine has slowly happened over the years and that is why I am still alive, although I am suffering greatly. In essence I am living with a half broken neck!!

These symptoms for me over the last 8 years since the age of 22 were indeed gradual. I had terrible constant nausea and IBS type symptoms originally. Then as things steadily got worse by the age of 27 I was diagnosed with POTS (please refer to other related conditions to learn about this). But basically the brainstem compression that has occurred with me had damaged the spinal cord. The spinal cord of course controls and regulates many functions in the body such as heart rate, blood pressure, digestion, temperature, all these autonomic bodily functions are interrupted by the brainstem compression and in my own case POTS was probably caused by this. My POTs effects me in the way that I have blood pooling in the lower limbs of my body, my heart has to pump much faster than a normal person's in order to oxygenate the brain. My very fast heart rate I believe, is down to the damaged nerves or "messages" that are sent through the brain stem and spinal cord.   POTS can be caused by many different things, but in the case of EDS suffers, I believe that CCI causes so much damage to the spinal cord that POTs is often inevitable with time.  CCI can actually mimic the symptoms of POTS and Chiari, it worth remembering that.

If the Grabb Oakes measurement picture underneath it measures more than 8mm, it is indicative of surgery or fusion to relieve the brainstem compression. Mine measures 8mm on the lay down and 9mm on a stand up MRI. Yet here in the UK, they do not listen to this proven medical evidence and criteria, I was ignored.  I believe there is only (one) standup MRI/open MRI in the UK, which is in London and waiting times and the criteria that needs to be met to have an open standing MRI, is extremely lengthy and there is no guarantee if you have EDS that you qualify for a standup MRI on the NHS. My advice for others viewing their own scans to check for what looks like a pannus build up around the C2 bone. Pannus build up is like cartilage. It will only be there if movement or instability is happening. A healthy spine does not have a pannus build up around its C2 odontoid. Just another warning sign to look out for.







Here is a comprehensive list of symptoms you may suffer from when you have CCI




Occipital Headache, especially at the occiput (back of the head)

 

  • Sore neck
  • Facial pain and tingling or extremities
  • Gagging
  • Low Blood Pressure
  • Tachycardia Arrhythmia
  • Postural Orthostatic Tachycardia Syndrome
  • Breathing Problems
  • Tingling in hands and feet
  • Numbness in arms and legs
  • Arm and leg weakness
  • Hyperactive Reflexes
  • Dysesthesia
  • Lateral Nystagmus (involuntary rapid movement of the eyeball)
  • Poor Temperature Control
  • Severe Sleep Apnea
  • attention disorders
  • Memory disorders, Weakness of memory
  • palsy
  • vertigo
  • balance disorders, stumbling, problems coordinating walking, problems of coordination of hands and arms
  • visual problems (seeing stars, reduction of the visual field)
  • tinnitus (ringing in the ears)
  • pain in the following areas: the mandibular articulations, ears, and eyes
  • Often the symptoms are accompanied by severe drowsiness / stupor and agitation, vegetative faults, esp. of the cardio-vascular system: unsteady erratic pulse, tachycardia,
  • In some cases: vigilance is (extremely) reduced, consciousness disorders (patients tell, they are not really awake, like a hit fly, no more in this world living, in a diffuse world, anything is unreal, I am living like in a dream, ...) - reaching from fatigue over somnolence to comatose states.
  • disturbed, changed or reduced self-perception
  • breathing disorders, autoregulation of breathing defective
  • nightly scaring (waking up, not able to breath, to see, to move, in some cases completely blindness (for a period of time) - this sometimes with great anxiety to die

 


C1-C3/C4
  • Headaches and migraine-like pain
  • neck and scalp tension
  • pressure and pain behind eyes
  • blurring of vision
  • dizziness
  • light-headedness
  • fainting
  • facial pain and numbness
  • ringing in ears
  • ear pain
  • jaw pain
  • reoccurring sore throat
  • nasal congestion
  • sinus trouble
  • loss of co-ordination
  • disorientation
  • symptoms of dyslexia
  • generalized malaise
  • childhood fevers
  • vertebral artery insufficiency
  • insomnia (loss of sleep)
  • problems with memory
  • depression
  • irritability
  • loss of concentration
  • symptoms of allergies and hay fever
  • weakness in legs
  • difficult breathing
  • heart arrythmias
  • functional heart conditions
  • asthma and allied conditions
  • certain types of arm pain, angina-like pain
  • numbness in legs and feet
  • leg pains
  • bed wetting in children
  • abdominal cramps
  • fatigue, esp. when standing
  • urinary difficulties
fatigue and leg weakness



So how do we fix CCI?

Well I will just stick to explaining how you can try and correct CCI.
In America there a handful of pioneering surgeons in independent hospitals that are specialising and perfecting surgical techniques for EDS related brain and spinal problems.   Many of us can suffer from what I call the dreaded threesome.

  • Chiari Malformation 

  • Cervical Cranio Instability

  • Tethered Cord Syndrome


Thankfully I only need surgery for my CCI, others are not so lucky. Again please refer to the (related conditions page for more information)
So how do we fix this instability from the skull (occiput) to the C2 vertebrae?

Well, the operation itself is straight forward in the fact that it is two metal rods carefully placed to stabilize the head on the neck, and so brainstem compression cannot happen. Once the patient has had this spinal fusion, hopefully the metal rods fuse successfully to the bone and the support is in place for many years to come. Although this operation takes many hours and has to be carried out by a very capable surgeon, major veins, nerves and arteries must not be hit, and it is very intricate surgery.

The only way to get a dx for the conditions we are discussing are as follows;
 
Pay privately to go and see Prof Rodney Grahame in London, he is the UK's ;eading expert in EDS and he is currently sending out patients scans to Dr Henderson in America so he can advice on whether surgery is needed. If it is then you need to be able to fund the surgery yourself in the USA.

Contact The Chiari Institute in New York yourself, you can arrange to have a skype consultaion with one of their leading expert surgeons via Skype.

Contact Dr Bolognese at the Chiari Neurosurgical Center at NSPC and arrange for a similiar Skype.

Contact Dr Theodore of Arizona and arrnage for communication via email.

This is unfortunately the only way to often get a true diagnosis as the NHS does not screen for CCI and very rarely checks for Chiari. We need to appeal to the government for rare diseases and campaign for the UK surgeons to give us the surgery we deserve! So please visit my online petition and sign it!!

 

My Story/Latest NewsWhat is EDS What is CCIMy campaignMy VideosRelated ConditionsDonateOn Line PetitionContact Me/Link