Medical terms

What is CCI/AAI?

CCI/AAI is a rare complication of Ehlers-Danlos Syndrome.

The term CCI, or Cranio (skull) Cervical (neck) instability, means it goes wrong in the upper cervical vertebrae. That is between the skull and the first cervical vertebra. However, it can also go wrong in the vertebra below it, in which case it is called AAI, or Atlanto (upper cervical) Axial (second cervical) instability. The ligaments that should normally hold all the vertebrae together are too weak. As a result, the vertebrae are not held properly and shift. The neck becomes unstable. This can also happen under these vertebrae in the neck, chest or lower back.


Neck instability causes compression of the brain stem, spinal cord and nerves, causing vital functions in the body to malfunction. Think of breathing that normally occurs automatically. We don't have to think about adjusting those when we sleep or run. Think also of reflexes, blood pressure, heart rate, seeing and hearing, sweating, digestion or body temperature. These and dozens more human body processes can all be disrupted when the brain stem is compromised as is the case with Annemarie.


Debilitating disease

In addition to muscle weakness, Annemarie suffers from dizziness, breathing disturbances, memory problems, fatigue and sensitivity to light, sound and touch. In short, due to the compression of the brain stem, stimulus processing in a broad sense is a major problem with her. The disorder is progressive. In advanced stages, patients with CCI lie in a low-excitation room because the entire nervous system is overloaded. Without treatment, further deterioration cannot be prevented and will eventually lead to death.


Life-saving surgery

A fusion in which the affected vertebrae are fixed can provide relief and thus give life back to the person in question. This is what we would like for Annemarie. She will lose a lot of movement in her neck forever, but will get her life back. The operation consists of lifting the head up relative to the vertebrae and securing the vertebrae in this way with rods, screws and donor bone. Space is created so that the brain stem is no longer compressed and the nerves become free. This surgery is life-saving and is performed only when medically necessary.


We hope you will make a donation to make this life-saving surgery possible. Every little bit helps so that Annemarie will soon have a future again.


What is (EDS) Ehlers- Danlos Syndromes:

Ehlers-Danlos Syndromes (EDS) is an umbrella term for a group of rare inherited connective tissue disorders. There are several types, including hypermobile EDS and classic EDS. These include joints that are extremely flexible causing many problems in the body. One of these problems is CCI/AAI. Joint dislocation is commonplace for many of these patients. Organs can also be affected by the poor connective tissue and sometimes stop functioning altogether.


Main features include hypermobility, stretchy soft skin, rapid bruising and delayed wound healing. Because of these features, pain and fatigue are complaints that are common, although symptoms vary from person to person.


The cause of this progressive condition is a deficiency in the substance collagen. Collagen ensures that tissues are held firmly together. Unstable neck (CCI/AAI) occurs in 1 in 15 people with EDS.