September is Chiari Malformation Awareness Month, and since I’m a ‘chiari zipperhead’ and an author, I thought I should take a few moments to share my story and  tell how a chiari malformation affected my life.  

My story begins nearly four years ago. I started noticing that every time I took a shower, the three middle toes on each foot would start to feel cold and tingly and they’d turn completely white. It was annoying and sometimes painful, but it would go away, so I didn’t really care about it too much. Now I know it was something called Raynaud’s phenomenon.

Then as time progressed, my limbs would go numb. Usually it would be my left side but sometimes all four limbs would go numb at once.  Sometimes it was so bad, I couldn’t move.  I literally would be walking one minute, and the next minute, something would happen and no matter how hard I tried, the message from my brain to my legs no longer worked and I could not move at all. 

I had strange nerve pain in my arms. The only way I can explain it is that it felt like I had scratched my fingernails down a chalkboard and I was feeling the agony in my arms. I started having migraines. They were rare, but intense.

I finally went to see my doctor when my entire left side went numb while I was driving my car to the grocery store. Thank goodness it was my left side so I could still control the car, and thank goodness my kids weren’t with me!  I was terrified. 

My doctor suspected MS or a tumor.  An MRI was ordered to rule these out and of course I was scared of the results. Fortunately, my MRI came back completely normal.  We had no idea what was wrong. 

My husband, who is a musculoskeletal radiologist, decided to share my MRI with a friend who was a neuroradiologist.  He looked at the study and said, “She does have a minor chiari malformation, but it’s only a 5mm herniation. That could be causing some of her symptoms, but it’s pretty rare and her symptoms aren’t classic.”

What’s a chiari malformation? The definition from the National Institute of Health is as follows: Chiari malformations are structural defects in the cerebellum, the part of the brain that controls balance. When the indented bony space at the lower rear of the skull is smaller than normal, the cerebellum and brainstem can be pushed downward. The resulting pressure on the cerebellum can block the flow of cerebrospinal fluid (the liquid that surrounds and protects the brain and spinal cord) and can cause a range of symptoms including dizziness, muscle weakness, numbness, vision problems, headache, and problems with balance and coordination.

My cerebellar tonsils were herniated, or pushing down into my spinal canal 5mm, which is practically normal, but still considered a malformation.  A lot of people with chiari malformation have much larger herniations, but what’s interesting is there doesn’t seem to be a direct correlation between the size of the herniation and the manifestation of symptoms.  People with small herniations may have hugely debilitating symptoms, while others with larger herniations may live a perfectly normal life.

Because my symptoms weren’t classic, meaning I didn’t have chronic, debilitating headaches, we didn’t pursue the diagnosis. I had been under a lot of stress at the time, so my doctor thought maybe it was a manifestation of depression. I didn’t think this was possible, because, quite frankly, I had been extremely depressed and suicidal previously in my life and I knew what it felt like. Yes, I was stressed, but the stress I was facing at the time was nothing compared to what I had survived before and I just couldn’t believe that that was what was wrong.  

We tried a host of medications. I tried Lyrica, amitriptyline and a few others.  I had horrible side-effects, including truly suicidal thoughts.  It was lovely so I refused to take them anymore.

I felt that living with my symptoms was easier than living with the side-effects of the meds I was taking. So, I decided to change my diet and exercise and things seemed to improve. I had read that symptoms like those I was experiencing could be caused my aspartame. I was a diet soda addict. I stopped drinking diet soda, and my symptoms improved greatly.  I thought, “Wow!  I’m cured.”

Life went on, Then about two years later, something weird started to happen.

It’s kind of embarrassing, but I feel the need to share it.  Every time I went to the bathroom, I’d get this crazy pain along the back of my skull. It would start at the base of my head and branch up, clenching my brain.  It literally felt like someone took their hand and squeezed the back of my head. At first, this pain would disappear as soon as it came and it only happened occasionally.  I kept it a secret.  Again, it was weird, but it didn’t do any harm and quite frankly, I was too embarrassed to tell my husband. (LOL, now I’m telling the world... **sigh** Oh, how things change!) 

Well, it became more and more frequent and sometimes it would be so intense, I felt like I was going to pass out on the toilet.  Vain little me thought, “Now, that would be embarrassing!” so instead of allowing my kids or husband to find me passed out on the toilet, I decided to share this new symptom with my husband. 

Like I said, he’s a doctor.  He hides things well.  Not that day.  I could see the worry in his eyes and soon he was typing away on the computer.  He said I was experience something called an exertional occipital headache, and it could only be caused by a couple things- a tumor or chiari malformation. I went in for another MRI, and once again, there was no tumor.  John asked the radiologist about the chiari malformation.  He said it was there, now it was 6mm, but he wasn’t convinced it was causing the symptoms. 

Well, soon after that I went to the bathroom, the imaginary hand gripped the back of my head, and it never let go.  The pain lasted for four months.  Nothing, NOTHING stopped it. Imagine living every second of your life with a headache and never, ever having a break from that headache for months!  It was hell! 

My doctor tried to put me on the same medications I was on before, but I refused. I couldn’t do that to myself again. 

My symptoms kept progressing, not only did I have a headache that wouldn’t go away, I couldn’t look upward or I would nearly pass out.  I had to walk, looking at my toes, so I could walk at all.  I kept having dizzy spells, almost falling over. 

I went to a new doctor and explained what had been going on. I told her that my husband thought my chiari 1 malformation was causing my symptoms, and he knew to prove this, I needed a special MRI to see if the flow of my cerebral spinal fluid was being blocked. 

She looked at me and said, “I have no idea what you’re talking about, but I’ll follow your husband’s lead.”

Well, I’m glad she did. I had my MRI and I had a cerebral spinal fluid blockage.  My information was sent to Johns Hopkins, and I sat and waited.  I assumed, because of all the waiting I had done up until this point, that I’d be waiting another four months.  I was wrong.  I waited two days.  My neurosurgeon called close to 6 pm that Friday night and asked to see me Monday.  The urgency scared me, but I was relieved.

He said everything I had experienced over the past four years was, most likely, caused by the malformation. I was approved for surgery and it was scheduled.  It was called a decompression, or an occipital craniectomy and C1 laminectomy and the surgery took place at the base of my skull. In simple terms, the neurosurgeon cut a hole in my skull to create more room for my brain to allow the cerebral spinal fluid ample room to flow. They put a titanium mesh over the hole so my muscles could be reattached. They also gave me a C1 laminectomy, cutting off the back portion of my first cervical vertebrae. The scar that was left on the back of my head looks like a zipper.  All chiarians receive one and that’s why we’re called “zipperheads.”

This type of surgery is not a cure, however it does helps treat the symptoms. There is no cure for chiari malformation.

The surgery was hard.  Recovery was hard, but I had a wonderful group of doctors, family and friends supporting me.  I couldn’t have done it without them.

The pain from the surgery was intense, but nothing compared to the pain that it treated. Eventually, I healed and felt incredible. I was thrilled! I had my life back! 

           About three weeks after that, I started having pain at the base of my skull again.  I freaked out, but I sat down and focused on the pain.  I realized that the pain was outside of my skull, not inside like it was before. I was having post-surgery muscular pain.  An orthopedic massage therapist and friend, told me that the residual muscle pain after surgical trauma can last for a really long time and recommended that I have some massage therapy.  It worked.  The pain is gone and I no longer have to take muscle relaxants.

            So there it is...my story.  It’s a long one, and if you read it all, thank you!  One in 1000 people have a chiari malformation.  It is as common as MS, but few people even know what it is.  Spread the word, and maybe you can help someone in pain.