A dear friend of mine who has Chiari, recently learned she also has Ehlers-Danlos Syndrome, or EDS for short. She and I bonded when I found out I had Chiari, and had no clue what it was. She helped me a lot to understand what I was going through, and pointed me in the right direction to find a good Neurosurgeon. We even had our surgery’s by the same doctor one month apart. She has been kind enough to share her story, which will be featured soon. Until then, I would like to share a little information about what EDS is.
Ehlers-Danlos syndrome is a group of inherited disorders that affect the connective tissues, primarily skin, joints and blood vessel walls. Connective tissue is a complex mixture of proteins and other substances that provides strength and elasticity to the underlying structures in the body. People who have Ehlers-Danlos syndrome usually have overly flexible joints and stretchy, fragile skin.
Symptom severity, like in Chiari, can vary from person to person. Some people with Ehlers-Danlos syndrome will have overly flexible joints but few or none of the skin symptoms. Different sub types of Ehlers-Danlos syndrome are associated with a variety of genetic causes, some of which are inherited and passed on from parent to child.
What are the signs and symptoms?
Overly flexible joints
Stretchy or fragile skin
Fatty lumps at pressure points
Tearing of tendons or muscle
Severe muscle and joint pain
There is no cure for Ehlers Danlos Syndrome. Close monitoring may be helpful.
For more information, please visit ED National Foundation
Life with Chiari is helping to spread awareness about the Ehrick Garion’s Act (a.k.a. Ehrick Garion’s Law) The act will bring about awareness, education and research for Chiari Malformation. Its so important for people to realize this condition is real. It has real complications, and very real consequences.
What is Ehrick Garion’s Act?
Ehrick Garion’s Act is an awareness act created to advocate for people with Chiari Malformation and their families. The goal of the act is to have state and/or federal legislation passed which will require greater education and training of health care professionals to recognize, advise, and treat patients especially children with Chiari, and to eliminate the challenges for Chiari patients to have their disabilities recognized by state and federal agencies.
What will this law do?
This law will protect families who have lost children/spouses/parents due to lack of diagnosis, withholding diagnosis, complications due to delayed diagnosis, medical malpractice due to lack of knowledge of Chiari and related conditions.
Why did Kolya start this initiative?
9 year old, Ehrick, died of a brain hemorrhage, because his doctor told his parents, “It’s nothing. It’s not a life or death situation.”
8 year old, Garion, died in his sleep of central sleep apnea, because his doctor withheld his diagnosis from his parents.
A Message from the creator:
“I created Ehrick Garion’s Act in 2012, officially launching in November of 2012. I’ve heard story after story (including my own) about the injustices in medical community, academic institutions (from kindergarten to college), government agencies (primarily Social Security), workplace, etc. I knew there had to be legislation to prevent these things from happening. I’m currently getting my degree in Medical Sociology to understand the medical community (how doctors think, how insurances think, etc.). With this information I can advocate more effectively. I wrote a research proposal on Chiari and Mental Fatigue, which was accepted by the Eastern Sociological Society and I presented on it in February of 2014 in Baltimore at their annual conference.”
Kolya Lynne Smith, creator
Want to contact Kolya?
Email her at: firstname.lastname@example.org
Visit her website: chiariact.com
I had to edit this post. I had to cancel my walk this year. My surgeon recently informed me he thinks I may have EDS. I have a lot of doctors appointments and testing coming up because of this, and I would not be able to dedicate the time and resources needed for the event. I am truly humbled by the outpouring of support that I have received.
I can’t tell you how good I feel this week. I feel like all my struggles are coming to fruition. I have fought so hard for recognition of Chiari and its finally starting to happen.
I got to go an have lunch with my husbands co workers today. I never get to have outings like that, and it was so much fun! Everyone he works with are such nice and lovely people. They all are always asking about me and how I am doing.
I spoke with Stephanie Marks Buchanan, Legislative Director for Senator Aument today. She said that Senator Aument will be doing my resolution to recognize September as Chiari Awareness Month this year. Retired Senator Brubaker did this last year. chiari resolution
As always, stay positive!
I wanted to share my Chiari story with you. Everyone has their own journey with Chiari, be it a patient, parent, or caregiver. I hope my story can help someone else.
I found out I had this condition in February 2013. I had the worst headache of my life, and thought I was having a stroke at 29 years old. I went to the ER and they did a cat-scan. The Dr. saw a mass at the base of my brain that was herniating into my spinal column. She said it was either a brain tumor, or Chiari, something I never heard of before. After being discharged from the hospital, it was a very long two weeks until I got in to see a neurologist. The neurologist confirmed it was Chiari Malformation, but blew me off. He said he didn’t think it was all that serious.
I knew something was wrong with me, and I wasn’t going to take his nonchalant brush off as an answer. I started doing a lot of research, but to my surprise, there was very little information about Chiari out there. I found out there is an institute in NY that deals solely with Chiari patients. It is called the Chiari institute. I applied to be a patient in April of 2013, and in May of 2013, I went to see my new Neurosurgeon, Dr. Paolo Bolognese. He ran quite a few tests, MRIs, Cat scans, blood work, physical, a few hands on tests, and what seemed like a million questions. He said I was right to push for treatment. Had I waited, in six months, I would have been either permanently paralyzed or dead.
May 28th my husband and I packed our bags and made the five hour drive to New York. We were both terrified the whole time. We checked into our hotel, and I didn’t sleep a wink all night. I kept begging my husband to just cancel the surgery and just go home. I had never been more scared in my life. May 29th, 2013 the day before my 30th birthday, I was in surgery to repair my skull and brain. I was hooked up to IVs and Dr. Bolognese came in to reassure us everything was going to be ok.
One of the scary parts for me was being wheeled into the operating room fully conscious. It was terrifying to see how blank and sterile everything was, and seeing the trays of tools that were about to go into my head. I was transferred to the operating table and knocked out. My head was screwed to the table to keep it in place.
There were a lot of difficulties, and my 8 hour surgery turned into a 14 hour surgery. My doctor had to pull me out of surgery several times during my fusion placement because the area of my skull where the screws were supposed to go was too thin. I ended up having a decompression, duraplasty, electrocautery tonsilar reduction, a plate covering the space my skull is missing and craniocervical fusion from my skull to my C4 vertebra. Dr. Bolognese sewed me back up and I spent my 30th birthday in a medical coma. I was continuing to have complications, and they thought they might have to take me back to surgery.
When I finally started to come around, the first face I saw was my poor, tired, completely worn out husband. He had been up almost 40 hours by that point, waiting for me to come out of surgery and the coma. He never left my side for a second.
I ended up being in the hospital ( Long Island Jewish in NY) 5 days. During that time I had a wonderful set of nurses and physical therapists. I was up and walking with in two days. The only thing I really struggled with was getting my pain medication on time, and vomiting so much!
The ride home was the worst thing I had ever experienced. It was extremely painful and I was horribly uncomfortable the whole time. I ended up being in a large plastic neck brace for six months. I had to walk with a cane, I had to relearn how to feed myself due to the fusion, and I had to relearn a whole new way of doing things for myself.
After coming home, I found out I was permanently disabled. The surgery and the condition its self has left me with severe nerve damage. I am in constant, high levels pain every single day. I have lost the feeling in my hands and feet. I have to walk with a cane, and if I am going to be out any period of time, I rely on a wheel chair. Sounds and lights bother me a lot. I can no longer go to movies with out being in a lot of pain. I can no longer drive, and my memory is shot. But, none of it matters, because I am alive, and I am able to help others through an especially difficult time in their lives. I didn’t want it to stop me, or others with this condition.
I joined a Facebook Group for people with Chiari, and since then I have counseled a lot of other patients about what to look for on their MRIs, how to find the right doctor in their state, how to keep track of symptoms, help them fund raise, and just be a shoulder to cry on. I know how hard it was for me when I was diagnosed, and I had no one to turn to. I want to be that person for other people. Its a very scary thing to go through, and I want to do all I can to make it a little easier.
I talk about Chiari to anyone who will listen. Its rarely heard of, but not that rare at all to have. I always tell people, if somethings wrong, if your headaches are unusual, get checked, Its a simple, painless test and it could save your life.
An update on me: I recently found out I have EDS. Please see my other post on this condition.
Hello and welcome to Life With Chiari!
My goal with this blog/site is to help spread awareness of Arnold Chiari Malformation, and to help others who have been diagnosed with this condition find answers and connect with other Chiarians.
There is a FAQ section for the most frequently asked questions. If you can’t find an answer there, please feel free to email me directly at email@example.com and I will do my best to work with you to get an answer to your question.
Over the coming weeks as this site takes shape, I will be adding more sections like doctor resources, patient resources, what to expect after surgery, ect. Every week I will try have a feature writer from the Chiari community to tell you first hand about their personal journey with Chiari. Some will be patients, some will be parents of children with Chiari, and some will be caregivers who have loved ones have Chiari.
There is a lot more to come, so please check back every week to see updates on fight for awareness and new stories from the Chiari community.
As always, keep a positive attitude.
We may not have control over much when it comes to this condition,
but we can stay positive and do our best to help others understand our daily struggle.