Faces and Stories of Chiari and Syringomyelia
To read stories, just click on the name.
This page was last updated: June 14, 2010
I'd like you to meet my daughter Alyssa, a very bright 6 year old.
She competed in a National Math Pentathlon Tournament during her kindergarten year, and was reading at a 3rd grade level by the end of the year. She can also write and count to 1,000.
To look at her, she pretty much looks like a normal 6 year old. She loves crafts, singing, books, and kitty cats. She even tried her hand at Karate, but her balance is just too bad. However, that doesn't stop her from picking on her little brother, James.
Alyssa loves everyone, is willing to do anything to help, and brings a smile on everyone's face, but she's also extremely sensitive, and cries about everything and anything. Her teacher, last year, said she cried at least once every day.
Alyssa also has Syringomyelia.
She was born October 14, 1997, and seemed to be a perfectly healthy baby girl. She developed at an average progression, and was walking at 13 months, although she did seem to fall quite often.
The "falling pattern" continued, even at 5 years old, but we thought she was just clumsy, and getting in a hurry. Then, some of the members of our church noticed her foot turning in slightly. So, just to be on the safe side, we took her to our family doctor.
He had a son that had a brain tumor, and apparently had some of the same "symptoms" as Alyssa, so he suggested she have a MRI. This was in September of 2003, and that's when they found the syrinx.
At first, they determined it was a mild case of Anencephaly, which is what my first child died from. I started searching the Internet, but could never find a "mild case", so I called the neurologist to check the spelling. She spelled out "Syringomyelia". A little confused, I asked her what that was, and she explained that it was in the same category as Anencephaly, with the Anencephaly being the worse case scenario, and the Syringomyelia not being nearly so bad.
In continuing my search, Dr. Frim's name kept coming up, so we decided to go to him for a second opinion. He confirmed the SM diagnosis, and WE decided he was definitely the doctor for Alyssa. He has run many tests, but has yet been able to determine the "cause". She continues to see Dr. Frim on a regular schedule, with a "wait and watch" approach.
As far as Alyssa goes, she doesn't know much about her condition other than she has something wrong with her spine, and she has way too many doctor appointments. Her symptoms at this point are daily pain, and she continues to fall a lot.
As of now, James isn't showing any signs or symptoms of Syringomyelia, but since research has determined that it can be hereditary, we will continue to keep an eye on him.
Jessica
In my last face to face, I mentioned Jessica. She and Laura Slyman both have pacemakers. It was through an on-line pacemaker support group that they met. While talking with Laura, Jessica recognized many Chiari type symptoms, and prompted Laura to have a MRI. Jessica was a vital part of helping Laura find a proper diagnosis and treatment. Jessica still continues to help others on the internet by listening and giving support.
Jessica started her Chiari journey at the age of 20. Symptoms started early in 2002 after coughing from pneumonia. It wasn’t until she rode a roller coaster that symptoms really started to progress. Symptoms varied and included horrible headaches, neck pain, dizziness, major swallowing problems, loss of gag reflex, balance problems, Nystagmus, nausea, drop attacks, bradycardia, tachicardia, shortness of breath, numbness and tingling in her extremities, and low back pain.
In August of 2002, Jessica had her first posterior fossa decompression at OU Medical Center in OKC. Symptoms didn’t improve, so a decompression revision was done in April of 2003. It is through her persistence, research, and knowledge that she was able to find The Chiari Institute. Symptoms still continued to progress so she sought a second opinion at The Chiari Institute in NY. After several tests and exams, a craniocervical fusion was recommended for cervical instability. Jessica had a cranioplasty and fusion done in January, 2004.
Heart irregularities continued to cause problems and it was recommended that she have a pacemaker. This was done at Oklahoma Heart Hospital in July of 2004. Now that she has a pacemaker, she is unable to MRI’s. She has to have CT mylograms every time she would need a MRI.
Symptoms persisted, and she was diagnosed with Pseudotumor Cerebri, and a VP shunt was placed in November of 2004 at OU Medical Center in OKC.
Jessica continued to have problems, so returned to TCI. She had extensive testing, and this was going to be a long stay in NY. They revised her craniocervical fusion in November, 2005. TCI discovered by pulling up on the head (fusion in extraction) the patient has significant relief in increased intracranial pressure. After the fusion revision, she found she didn’t need her VP shunt any longer and it was ligated (tied off) in December, 2005.
Due to Ehlers Danlos Syndrome, Jessica has poor wound healing. During this time it was found she had a MRSA infection. They performed a debridement surgery which involves opening the wound up and cleaning out the infection. She then received IV antibiotics and the wound was closely watched to make sure it didn’t open again. Unfortunately, symptoms continued to be a part of her daily life. Her mother had to return home, and she was in the hospital on her own. It was decided that the fusion wasn’t stable, and revision surgery took place in January, 2005. During the surgery, they found a broken screw which had been allowing movement in the area. After the revision, Jessica started to feel better almost immediately. She needed to stay in the hospital until her wound was totally healed, and sutures were removed. After being in the hospital for over two months, she was finally able to return home.
Jessica works as a manager in a lingerie department. She returned to work shortly after being released from the hospital. She started back part time for a couple of weeks, then was able to return as full time. Jessica appealed to her insurance company to pay out of network, and was able to win the appeal. She still finds it difficult to work full time, but without her job, she wouldn’t have insurance.
Prior to her Chiari diagnosis, Jessica was very active in the children’s ministry at her church. She used to teach preschoolers twice a week at church along with other activities within the church. She still continues to teach on week of vacation bible school each summer.
Due to many surgeries, she has lost many of her friends. Working full time pretty much takes all of her energy, and she is unable to stay out late like many of her friends. Medications also make her tired.
Jessica started college, but was unable to finish. This has been the hardest aspect of being sick for so long. She hopes that she will be able to return to college in the future, but without a full time job and insurance, this would be difficult.
She feels much better than she did prior to her last stay in the hospital. She is still taking quite a few medications, and hopes to slowly wean off some of them in the future. She lives with her parents, and would like to be out on her own in the near future. She has always kept a positive outlook, knowing there had to be something that would help give her her life back.
Some of Jessica’s hobbies include scrapbooking, and she’s currently making scrapbooks for her three nephews. She also enjoys cross stitching; it’s easier, and can be done from a reclining chair. Both hobbies give her something to do when not at work, and she finds joy in creating new projects.
Laura Slyman
House eposide "The Choice" Aired May 3, 2010
Laura is 17 years old, and lives near Seattle Washington. I was fortunate enough to meet Laura at The Variety House in February. She had just had her decompression/fusion and had just been released from the hospital.
Laura has had symptoms for as long as she remembers. Heart issues took priority when she was 14 years old. She was passing out when walking, and dizziness. These symptoms pointed to cardiology, she had a pacemaker placed when she was 15. We now know the cardiac symptoms were neurologically related.Laura joined an on line support group for people with pacemakers. This is where she met Jessica. When Laura explained her symptoms Jessica mentioned that she could have Chiari Malformation. Jessica recommended Laura go to TCI for an evaluation. After a year and a half of no answers, in October 2005 Laura went to TCI where she was told she had Chiari, EDS, a retroflexed odontoid, and cervical instability. If Laura hadn’t joined the support group she still may be suffering from CM/EDS.Laura and Jessica hope to meet when they both go back to TCI for follow up exams.Laura had her decompression and fusion (fused to C5) surgeries done late Feb. 2006. She was released on the 9th day to The Variety House. TCI wanted to remove her sutures before they returned home because of wound healing issues with EDS. Recovery has been slow for Laura, she had severe muscle spasms. She said it took a long time to be able to sit up for 5 minutes. She has had kidney problems since her surgeries, including a stone. She has been going for physical therapy to gain a bit of neck rotation and for Thoracic Outlet Syndrome. The PT has been working on breathing techniques and stretching, which has helped with the TOS symptoms and her range of motion has doubled to, on average, 20 degrees.The previous symptoms are gone, but new ones surfaced. She now has back and leg pain, numbness and tingling in her pelvic bone area. More tests were run to find she has Tethered Cord Syndrome. She is currently waiting for a date for TCS surgery.Laura has two younger brothers, ages 14 and 9. She said her oldest brother has symptoms of EDS, and her younger brother has been diagnosed with migraines. She’s concerned because she was 9 when her headaches started, and they were diagnosed as migraines. Chiari has changed Laura’s life in many ways. She said that her faith has become stronger. She also said that it has changed what she can and can’t do in school. The only thing it hasn’t changed is she still doesn’t like to clean her room. Laura will graduate from high school in 2007. She is planning on looking at colleges this summer. She would like to become a pediatric neurosurgeon or cardiologist. She’s always wanted to work with children in medicine, but having gone through what she has, her passion has changed to neurosurgery as well. She will make a wonderful pediatric neurosurgeon.Laura has been grateful to church members for being so supportive. She has taught first communion classes for the past 4-5 years and sang in choir for almost a year, until her symptoms caused her to not be able to stand for the whole hour. Her faith has brought her through her past surgeries and will be stronger for her tethered cord surgery.Laura’s hobbies are playing the violin and guitar, read and write, and called herself a fashionista and a shopaholic. What teenage girl isn’t a shopaholic? She likes to hang out with her friends, ski, play tennis, and loves volunteering in many different ways, from being on the administrative team for a world wide online pacemaker support group to working with children, and is working on setting up a Chiari run for her Senior project.This story shows how powerful the Internet can be. Two young ladies were able to find each other, and this has changed Laura’s life. I have full confidence that Laura will meet her goals in the future. She is an extremely gifted young woman.Emily
Emily was born Dec. 27, 1997. At 9 lbs. 1oz, she seemed healthy, but we immediately realized something wasn't right. She wouldn't wake for feedings, and after having her home only one day, we were in the emergency room with her weighing a mere 6 lbs.
Every test imaginable was done, including a CAT scan, but the doctors couldn't find anything explaining her sleepiness and inability to eat. After a week, we were sent home, and told to follow up with our primary pediatrician.
Life at home turned upside down. If Emily wasn't sleeping, she was screaming. She had great difficulty feeding, and would normally vomit up what she did manage to get down.
After a battery of gastro tests, the doctors couldn't find anything wrong, so they assumed it was allergies, and put her on an imported formula that cost around $1,000 a month. However, the only way the insurance would cover the cost was if we let her starve on the other formulas, to the point of being hospitalized.
With no idea of what was wrong, we just stumbled through with her. She began speaking, then walking, and was soon complaining about pain in her legs, and her arms going "fuzzy". She had never slept well, and her nights were plagued with fits and screams.
The doctors began to think that perhaps it was Arthritis. Once again, the testing resumed, some of which was very invasive. They also did more gastro testing because of the vomiting and reflux problems. And, again, nothing could be found.
The pain and mood swings were then coupled with Emily falling or her legs giving out, and we were sent to the first Neurologist. He did massive amounts of blood tests, convinced it was something chemical. He refused to do an MRI because "her symptoms weren't consistent enough". When nothing showed up, he then suggested she was Bi-polar. Needless to say, we didn't go back to see him.
Our primary doctor was now looking at us like we were insane. Emily was a bright, cheerful girl, and in spite of her problems, he felt there was nothing wrong.
As more problems appeared, including an onset of seizure-like staring spells, we again sought out a Neurologist. This time, an MRI was ordered, and a Chiari Malformation with a herniation of 12-13mm and partial spinal fluid blockage was found. As strange as it seems, we were greatly relieved to have a diagnosis, and finally, some treatment and relief for Emily.
Her diagnosis was made in February 2002, and she had her decompression surgery in September 2002. At that time, her symptoms were extreme fatigue, pain in all limbs, numbness (or "fuzzies" as Emily calls it), headaches, vomiting, fevers, extensive periods of sleep, mood swings, staring spells, bowel and bladder accidents and long periods of uncontrollable crying, being incoherent during these spells.
At first, the surgery seemed to be a miracle, with most of her symptoms being relieved. But, as time goes on, her symptoms are returning, along with an increase in falling, and temporary losses of vision.
We are now consulting with a Pediatric Chiari specialist. He feels that her symptoms will continue to worsen, but at this time, doesn't want to surgically intervene.
In spite of everything she has been through, and her daily pain, Emily continues to be such a nice little person. She leaves a lasting impression on everyone she meets.
Since this story was written, Emily has had to have several other procedures performed, the latest being a shunt revision this past August 2004. All the shunts prior to this one have failed to maintain a manageable CSF pressure, so the last shunt is one that will have to be manually opened and closed in order to maintain a tolerable pressure. This is going to be quite an adjustment for both Emily and her parents, but at this point, it's their only hope of Emily living a near "normal" life.
However, while Emily was still in the hospital, recovering from this surgery, she began passing blood in her urine. She was referred to an urologist for this complication, but we haven't received a report as to how this is going. But, as for the shunt, it is working well.
Unfortunately, with many Chiari patients, these "complications" are quite common. Because of the pressure that is put on the brain, any part of the body can become "victim" to this condition.
- Karen Cullen, Emily's MomChristian
I'd like you to meet my AMAZING son, Christian. Christian was born on October.12.2000 at 7lbs 4 1/2/ oz., and overall, he was healthy. He was born with hip click,which is were his left hip would pop in and out of place,so he was in a harness type thing for the first few months of his life,and he had a heart murmor,which had to be watched by echocardiagrams,but it wasnt considered serious.
He has had asthma his entire life,which he has battled pretty well,he has had pnumonia numerous times,but alwasy seems to do great and get through it.
Christian grew normal and developmentally great and still has to this day.He had no problems until about January, 2005, at which time he started having really bad headaches. We took him to the pediatrician and she just suggested tylonol and watch him over a long period of time and after so long with no break, she sent him to an eye specialist to rule out that it was his eyes causing his headaches. In April, 2006, after problems with his eyes was ruled out, she finally ordered a catscan w/ contrast and it came back totally normal.
From there she ordered an MRI of the brain done at Kosair Childrens Hospital in May 2006. It came back abnormal and she sent us to see Dr Moriarty in Louisville, Ky and he diagnosed Christian with ACM 1. A word that was totally foreign to us.
I did alot of research and found alot of help and support through the Wishes and Rainbows website and the Yahoo Chiari groups.With out that I dont know were I'd be.. HONESTLY!!! Christians decent was 11mm and the Nurosurgeon ordered decompression surgery.
Christian also had no gag reflex at all.We went for another mri,this time of the spine on August 8th 2006 at Kosair and thankfully it came back good.We saw Dr. Moriarty again on Aug.23,2006 to discuss the surgery and it was scheduled for SEPTEMBER 22,2006 st Kosair Childrens Hospital in Lousville, Ky. We spent 24 hrs in I.C.U and 24 hours in a regular room and went home on day 3.
I could not ask for a better Dr.or place for my child to have gone. They treated us great, and he did great.Christian got his stitches out on Oct 4,2006 and returned to kindergarten on Oct.9, 2006 --- and he is doing GREAT. He has been headache FREE ever since the surgery.
He now has some other issues we are dealing with, but we are not sure they are Chiari related. He went for an ultrasound of his kidneys and bladder on Nov 17, 2006 and it came back abnormal, so now we go Monday Nov 27 2006 for a V.C.U.G at Kosair to see exactly what's wrong.The dr's are saying maybe he has reflux of his kidneys. Im clueless.We also have to see an urologist, but not sure exactly when.
Christian had the vcug done and it came back that he has stage 2 kidney reflux but not being treated right now. he see's a urologist a few times a year to see how he is doing. they put him on a 4c's diet.. no caffiene..citrus..chocolate..or carbonation.
He did ok, but thats just to much on a 6 year old.
He has been doing good with his chiari since his surgery,but about a month ago his headaches returned.. but only every once in a while.. thank God.. and not as bad as before. He is doing great in school and graduates kindergarten on May 24,2007.
His neuro ordered an MRI when his headaches returned and it came back good; no bone has grown back.. THANK GOD!!!!!. as you know i was diagnosed and had decompression myself on March 6th and I'm doing good as well. Christian's pediatrician is checking into the Ehlers Danlos Syndrome (EDS) and wants to have him evaulauted. Im pretty sure he has it.. he has all the symptoms.. as far as brusing really bad and really easy and also the double jointedness.
He goes back to the neuro on May 22nd for a follow up.. so I'll keep you informed. Thanks to everyone for reading our story, and the help and support from all.
Misty Chism
Shane
Shane
My son's name is Shane; he is 10 years old and has Arnold Chiari Malformation and Syringomyelia. Not only do we battle these conditions, but he also has Klippel-Feil Syndrome. This syndrome consists of Dextracardia (heart on opposite side), missing a rib, born with an extra digit on one hand, deafness, and respiratory problems. He has asthma and has had pneumonia nine times.
We have struggled since birth with his health, seeing countless doctors and more specialists than we can keep track of. It was not until he was 7 years old that we found out about the Chiari. He would have horrible headaches every day, pain in his legs and we were starting to notice nerve damage. In one year, he became deaf in his right ear. We saw 10 ear specialists, all with a different diagnosis.
In February of 2002, we felt we were not getting the answers we needed here in Colorado, so we contacted the Mayo Clinic in Minnesota, and off we went.
It was not an easy journey!! Insurance would not cover it, but we knew we had to do it. So, thanks to our wonderful family and friends, and even a few strangers, we raised enough money to go. It was at that time we learned he had Klippel-Feil Syndrome, along with the Chiari Malformation and Syringomyelia. After seeing several specialists there, they finally put a name to all of his birth defects!
It was such a relief, finally learning what his problems were, but we also knew that road ahead was going to be a long and difficult one. They strongly recommended he have the decompression surgery as soon as possible. We could not afford to have the surgery there, so the doctors recommended a neurosurgeon here in Colorado that could perform the surgery. We flew home; met with the doctor, and a week and a half later Shane had his first decompression surgery.
The neurosurgeon felt the surgery was a success, and we had definitely noticed improvements. Over the next two years, he had hardly any headaches, and less pain in his legs, although there was no improvement in his hearing. But, all in all, we felt things were going pretty well.
On February 25, 2004, he had his regular scheduled MRI, and met with the neurosurgeon afterwards. That was when we got the news that things were worse than before the surgery. On March 4, 2004, Shane had his second decompression.
Our wonderful neurosurgeon feels this one went extremely well. He found that scar tissue had hardened near the patch that was put in with the first surgery.
His recovery has been much better than the first, and at a month and a half out, he is back at school and doing well. We will have another MRI on June 2nd to see how well the surgery really went.
Every day is a struggle. This condition not only affects your child, but also affects the family. To see the pain your son is going through, to spend the countless hours just praying to God to watch over him, and wondering every day what tomorrow will bring is extremely difficult. I have learned to have faith, but it hasn't been easy to maintain all the time. The emotional stress is unbearable, but then it is accompanied with the financial stress. With time off from work due to doctor visits, surgeries, and such, normal living expenses start backing up and the medical bills and other unexpected expenses start rolling in.
Thanks to my incredible mother, who just happened to be in the right place at the right time, we found out about Wishes and Rainbows. They were able to provide us with a little assistance to help with the finances, but more importantly, we are able to get Shane's story to you.
It's extremely important to us for Shane's story to be heard, but also for us to bring awareness to Arnold Chiari Malformation and Syringomyelia. Misdiagnosis causes so many extra problems, and can cause symptoms to become permanent due to lack of immediate attention. It's only through research and awareness that this can come to an end.
- Shane's MomPage under construction
Page under construction
More faces and stories coming soon
Kristen
I was diagnosed with Chiari Malformation and related conditions 4 years ago after a whiplash episode and Ehlers Danlos Syndrome in 2008. I was in my 3rd year of college. My diagnosis changed my life for the better although there are some for the worst. Everything happens for a reason as I say! I have had the chance to help others with these conditions and find it very rewarding. Throughout the past 4 years, my career choice has changed and my career goal is to now become a Respiratory Therapist and possible Sleep Tech.
*To get through the hardest journey, you must take only one step at a time, but you must keep stepping*
To read more see my blog: www.chiariwarrior.webs.com
Hitesh
I'm Hitesh Kakkad. I live in India. My story is coming soon.
Paula
I'm Paula. I live in Maryland. Married mother of 2. My Chiari and Syringomyelia was diagnosed after having my second child in 2000. I developed a pain behind my ear that wouldn't go away. I went to the hospital. They sent did a CT scan on me, then sent me home. The doctors told me to go see my neurologist. After going to my Neurologist, two weeks later he order MRI's and CT Scans. Within one week I found out I had Chiari. My Neuro told me that he couldn't do anything except give me steroids that could possibly help. Then telling me I couldn't die from it, it's natural, I was born with it. He then gave me a list of things I could no longer do.Which I call the "NO" List. Like riding a bike, no amusement rides, no planes, no running and the list went on. I lived that way for years dealing with the pain. Hiding it from everyone. Ignoring my Chiari like it didn't exist. My family didn't show an interest so I thought It wasn't anything major. I did learn over those years that doing certain things did make a huge impacts on my chiari, like cause and effect. A headache here and there. No dangerous red flag signs.
Years later on November 7, 2009 I was rear-ended at a stop light. Little did I know my life was about to change. I went to the hospital with only a REAL BAD headache and back pains. The ER did full spine X-rays and give me pain medines. They never checked out my head. I was diagnosed with whip lash. Days later dealing with awful headaches I went back to my neurologist for more answers as to why does my head and back hurt so bad.After more MRI's and CT's. I found out my herniation went from 4MM in 2000 to 15MM after my accident. I was told it's time to start thinking about surgery. My neurologist washed his hands of me. So I went looking for another one. After visiting 5 Neurologist. I finally found one that would atleast give me his time of day. The search was on for a surgeon. Everyday I kept feeling worse and worse. My arms started to getting weak. I couldn't walk very far with being exhausted. I went back to work. Blacking out at my computer, not being able to remember things. I was out of breath for no reason. Then I started to have sleep apnea every now and then. It was one thing after another. I started to have panic attacks. Going to the hospital because I felt pains in my chest and in my arms. I started getting depressed. I was refered to see a shrink, I didn't. I felt like I was choked by an invisible turtleneck. It got to the point I was afraid to go to sleep, because when I layed down it got worse.
After seeing several Surgeons.Writing Neuro departments for help or referrals to doctors at hospitals. I got no where. I filled out papers for TCI. The Chiari Institute of Long Island, NY. They accepted me and my journey begain. I got a lot of test done at home before going to NY because of my insurance, it was cheaper for me. I had my first appointment with Dr. Shelat and Dr. Insinga. I wasn't ready emotionally for surgery. Then within 30 day's after my appointment Dr. Insinga call me concerned with my health. We talked and he told me to call when I was ready. I already made up my mind they knew what they were doing. They educated me more than anyone else had ever done. I was scared of surgery.Then a few days later one of my best Chiari friends, Kelly Morrill -Davis passed away. At that point I said it's time. I was going to do it for Kelly. In her honor.
November 13, 2009. I had an posterior fossa decompression and a cervical laminectomy (removal of C-1) at North Shore Jewish Hosptial in L.I. NY. My invisiable tutleneck was gone. I was in the hosptial Friday - wednesday. I stayed at the Variety House til that Saturday, just to insure nothing happened by my fault after I left the hosptial. I met wonderful people at the Variety House. I started Physical Therapy about 12 weeks after surgery. It's 6 months after surgery. I am still going to Physical Therapy. I have a long way to go to get my atrophy back in my hand. So far surgery was a success. I still have syptoms like headaches and vertigo every now and then. (more coming soon)
Felicia
“It took me some time to realize that something was truly wrong with me and for that “wrong” to turn into true grief. I was seven years old and all I knew was that my head hurt!”
Hi, I am a 25-year-old female. I live in Chicago Illinois. I found out this past June of 2009 that I have a neurological disorder called Arnold Chiari Malformation (a.k.a. ACM or Chiari). That following July 31st of 2009 I had surgery for the malformation called posterior fossa decompression or also known as sub occipital craniotomy or foramen magnum decompression (each doctor puts it in their own text). It is a surgical procedure performed by a neurosurgeon to treat the symptoms of Chiari. Chiari is a malformation of the brain. It consists of a downward displacement of the cerebella vermis and the medulla through the foramen magnum, sometimes causing hydrocephalus as a result of obstruction of cerebrospinal fluid outflow. The cerebrospinal fluid outflow is caused by phase difference in outflow and influx of blood in the vasculature of the brain. Chiari cause’s headaches, fatigue, muscle weakness in the head and face, difficulty swallowing, dizziness, nausea, impaired coordination, paralysis and many “many” more symptoms (these are just “some” of what I had). But as I have learnt each patient suffers from completely different symptoms. The surgery I had or known as posterior fossa decompression etc. is when removal of a portion of the skull or posterior fossa decompression (the release of pressure), a small section of bone at the back of the head is removed to make more room for the cerebellum and relieve pressure. During surgery, the Dura (covering of the brain) was also enlarged with a patch created from synthetic material (some doctors use your own skin, mine used this patch). The enlargement creates more room for the brain and the spinal cord and helps to establish the flow of cerebral spinal fluid. A cervical laminectomy (c1) was also performed to remove the bone forming the back of the vertebra. Both the posterior fossa decompression and the cervical laminectomy were done at the same time and through the same surgical incision in the back of my head and upper neck.
And this is how it all begun… How it all is now, and how it will/may be in the future…
I have complained of “headaches” since the age of 7 (It could have been earlier in life but that is when I truly remember the pain setting in). That’s when my mother also really seemed to notice and took me to the doctor. We left there only to be told that I was a “girl” and these are the natural thing(s) that happen to young girls. As the years went on, the bizarre symptoms increased and they begun to really scare me. We were going from doctor to doctor just trying to find the culprit. My heart was tested, my eyes, blood. All sorts of test but you name it they all came back “normal”. My problems were blamed on puberty. Then at the age 13 when I started passing out cold and not remembering where I was (still suffering from headaches and much more as time went on) my father took me to the doctor (my parents were divorced at this point) in Nebraska at an Air Force base. They checked me out from top to bottom but their main concern was epilepsy. I remember thinking… EPILEPSY. YIKES… But yet again, that was not what my problem was they told my father and step-mother that it seemed to be that I was just passing out from anxiety and put me on Zoloft (this is a very typical thing that most doctors tell families when all along their child has had Chiari). They said that I was young and just about to enter high school and probably just letting stupid teen things get to me. They never “NEVER” explained the head pain though. So I took the Zoloft thinking ok this must be the problem… I must just be freaking myself out and passing out from “this” anxiety (after them explaining to me what anxiety was because I had no clue what that meant). I probably stayed on Zoloft for a little over a year before I started getting really sad and depressed. After finally letting my mom know how this medicine was making me feel my mom decided to take me to another doctor. They said its not a great idea to have someone that young on a medication like that and that it was meant for at least 18years of age and older. With that being said I stopped taking the Zoloft. The big question I am sure you are asking yourself is “did my head still hurt after I started taking the Zoloft?” YES. “And did it hurt after I stopped taking the Zoloft?”… YES. The headaches have been something that I feel like I was always told I just had to deal with. After complaining and complaining over and over and years and years again my mother took me yet again to see another doctor he told me it seemed I just had migraines and gave me a migraine prescription. This didn’t help and I only took this once because it made me see swirling colors. (Not something anyone would look forward to when trying to take away a headache and it also seemed to make it worse) Years and years went on and I just learnt to deal with taking tons of Excedrin and other over the counter medication. But I started to go down from there. I started having really horrible days head wise and then the next day I was fine. That was until two years ago, the past October (2007) I was rushed into Northwestern by ambulance for fainting. That very morning I called my mom crying on my way to work. I told her that I had been having my horrible headaches for week’s straight. But they just kept getting worse and the pounding I could hear and pain in my shoulders I told her that I was driving to work and that I don’t know how I even got to the car or how I even got ready because my head was hurting so so bad. And when I say so so bad I mean on the pain scale 1being nothing and 10worst it was a level 9-10. I told her that it felt like a fist was in the back of my head pushing harder and harder. And that my shoulders where hurting so badly like I carried a 300 pound man (which is still one of my main symptoms to this day). As I was crying I told her that I knew and have know that there is something horribly wrong with me for some time. That I had just tried to play it off but things were getting worse and worse the older I was getting. Things were becoming unbearable. I told her that I didn't care if it was a tumor or if I was only left with one day to live I just wanted an answer. After getting to work and not even being there 10 minutes I passed out. That’s when I was taken by ambulance to Northwestern and had a full day of test after test. One test in particular that they did that means the most to this story was a CT scan. They came in the room after they had read it over and said that there was something that looked a little “weird” but no worries, my chances of anything being wrong were .2percent. I asked what looked weird? They said my cerebellum but not to worry it was something that could only be wrong in small children. And that they were 100percent sure that my problem was “just anxiety” (this is where I should have lied I guess right… They ask you a million questions and yes I told them at one point I suffered from anxiety) A lot of Chiari patients get told time after time that they are just suffering from depression or anxiety. It is a proven in a lot of reading that I have personally read myself. So the two years pass. I am back on Zoloft since the hospital funness back in October 2007. With now being told I should see a therapist. And my life is passing by but my head is still hurting tremendously. I had two choices bad days where I had to call off work and stay in bed all day or days where I just dealt with it and tried to make it seem that it wasn’t a problem. Those were the TWO ONLY things I could choose from. That’s when I decided I needed to start from scratch and see a new doctor. That’s when I met with Dr. Brian Boholst at Northwestern memorial hospital. He is a primary care physician. I started out wanting to tell him nothing of my past. But when he started asking me things I decided it would be best if I expressed to him what had happen in the past and that I was very concerned that I had a tumor or something more was going on from all the pain in the back of my head. I just kept calling them horrible crippling migraines. He started out with giving me some exercise’s thinking maybe I was tensing up and needed to learn how to un-tense myself. He also gave me some migraine medication that did nothing for me. I did the exercises and nothing changed. The pain was more of the problem when I did the work out moves. Then the next visit he wanted me to start journaling when I was having these episodes that were the worst. Like the times that these “headaches”were bringing me to my knees in the mornings. Not to mention causing small black outs and a lot of other symptoms. Through journaling I did find the same times happening with the dropping feeling and swooshing feelings they were happening the most in the am. After going and seeing him a third time he decided it was time for an MRI. He gave me a list of names of neurologists to use thru Northwestern and that is when I called Dr. Shephard. I had the MRI and then about a week or so later had the appointment with Dr. Shephard. Meeting him for the first time he came in the room told me what I had “Arnold Chiari Malformation” that it was very very bad one of the worst cases he had ever seen. It was 1 ½ inches outside of my skull. He gave me a few answers to questions I had and then gave me a name and phone number to a neurosurgeon that was the best for my condition, told me to get the CSF flow and (c-spine) cervical MRI and to take them to this doctor. That was when I met this doctor. His name was Dr. Chandler. He had examined me and we talked about the best choices for me. The best choice he thought was to relive some pain and that he thought it was best if I had the surgery. We scheduled for July 31st of 2009 and I went home with a long list of things to do before and so on. My mind was in the air thinking, worrying and a huge sense of just scared. But I was so relived that there was truly a problem and that I was truly hurting because I had something wrong with me. “It took me some time to realize that something was truly wrong with me and for that “wrong” to turn into true grief. I was seven years old and all I knew was that my head hurt!” July 31st I went into the operating area and that was it until I woke up from the surgery. I remember being druggy and funky feeling but as soon as I was in my room and my family was in there and I realized I MADE IT, it was all ok from there. But then not to long after having the surgery for Chiari I found out something more was going on. I was back in the hospital one week after the surgery for a total of 8 days in the ICU. For the reason being that my incision had internally ripped and was causing my spinal fluid (CSF) to leak out in between the scalp and skull. First I had a spinal tap to make sure there was no infection dwelling in my spinal cord causing this. When that came back normal I then was told I had to get something called a lumbar drain. That was put in my lower spine (lumbar) and was placed by an incision and the right placement to slowly get rid of the excess spinal fluid. The fluid they had to get rid of was the size of a soft ball sitting on the back of my head. This pain of the drain being drained every hour was the worst pain I think I will ever encounter in my life. There is no way anything any human could go through that could be worse than that. I was on so many painkillers and on top of that a drip IV of dilaudid (analgesic narcotic with a lot of similarities of morphine). After spending this dreaded week in the ICU I came home in a lot of pain with a ton of pain medication. That’s when I started to have this all over fatigue in my arms and legs, I had had fatigue before but I had never had it to this extent (I am using the word fatigue so lightly, my fatigue has become pain that leads to screaming it hurts so bad) and a lot of other weird unexpected pain all over my body. I made an appointment with Dr. Chandler to talk about all of this pain. He then decided that something “weird” was going on and that I needed to see an orthopedic surgeon. Millions or MRI’s to nerve conduction test and so much more I had then again been taken from one doctor to another specializing in whatever they though was “NEWLY” wrong with me. Been told I had frozen shoulders to horrible nerve damage to many other things and doing physical therapy three times a week. I am still at this point in physical therapy and it has been almost ten months. Nothing I was expecting and nothing I was told could be a possibility. Then with hearing one story to another and only being told that they have no idea what was going on or “I was a medical mystery” or “you are just a puzzle, and a super hard one that is” and pushing me on to a new doctor to new doctor for another test. Then later I met Dr. Deutsch he was my second opinion doctor (a neurosurgeon like Dr. Chandler). I went to him about 5 months after the surgery. I had-have been in so much pain that Dr. Chandler didn’t seem to be getting where I was coming from and wanted to go outside of that box and try someone else. That is when I met with Dr. Deutsch. He looked at my before MRI of the actual Chiari malformation and the current MRI and said well… You had one of the worst Chiari malformations that I have ever seen and I completely understand why your surgeon took out as much as he did skull wise. But now you are left with no option but to try and take away the pain with another surgery. Being Craniocervical fusion. I really struggled with the thought of being a paralyzed person for the rest of my life. I though my gosh this guy is a quack. Who in the world would want to do that? It felt way to permanent. I took up until now to actually research it and think of it as an option because the spinal pain is so bad. So I now lean to The Chiari Institute. This would lead me to be in the hospital for a few months and unable to do anything for a year at least (being in a whole body neck brace). I think hey if its going to give me twenty years of a pain free life why not? Right? He also mentioned me getting tested for some neuromuscular problems. He was defiantly concerned about my shoulder and how it was wasting away because I wasn’t able to move it properly. That’s when some time later I finally met with a rheumatologist and she said well… You have fibromyalgia. She touched me in all these spots. Told me I had 18 of the trigger point’s that were tender and that was that. Fibromyalgia is a chronic condition characterized by widespread pain in your muscles, ligaments and tendons, as well as fatigue and multiple tender points — places on your body where slight pressure causes pain. And most recently I have been told by the same rheumatologist I have Ehlers-Danlos Syndrome (wow, I wish all this bad news to stop). Ehlers-Danlos syndromes are a group of disorders, which share common features including easy bruising, joint hypermobility (loose joints), skin that stretches easily (skin hyperelasticity or laxity), and weakness of tissues. The Ehlers-Danlos syndromes are inherited in the genes that are passed from parents to offspring. They are categorized according to the form of genetic transmission into different types with many features differing between patients in any given type. The fragile skin and loose joints is often a result of abnormal genes that produce abnormal proteins that confer an inherited frailty of collagen (the normal protein "glue" of our tissues). EDS causes so much more pain for Chiari (muscles are constantly overworked trying to hold us together, since our 'glue' is faulty). They recommend a good amount of physical therapy to work on muscles. I think of these two diagnoses as an additional pain factor and just what I don’t need (or want). With being told thing after thing and more and more I will not let it stop here in Chicago where my condition is “rare”. I now have currently applied for a special hospital called “The Chiari Institute in New York”. Having a doctor that actually specializes in something you have (Chiari) and something so “RARE” this has became my only option. Hopefully you have gotten the hint of why so badly I MUST GO!
Dealing with trying to make other people actually understand my condition is probably one of the most annoying things one could ask to try and describe when you have Chiari and all these other complications. I can’t. I can't and that’s why… People ask me a lot how bad the headaches are and my way of explaining it is that they are very very bad. There is no other way for me to fathom how to even explain to them how horrible they are. I tell people that if I were to wake up and had never felt a headache before and feel what I feel everyday I would probably die right then and there from the pain freaking me out or giving me a heart attack. Its something that I feel I have had to just deal with and watch it get worse and worse through time. But see the things is you learn to deal with pain after time until it gets worse and then you eventually learn to deal with that new pain that goes right on top of the old pain. Dealing with these headaches has been hard on me in a lot of ways. It has caused me to not only stay home and in bed on horrible head days but it has made me very “very” depressed. Why depressed? My life sucks, I am nothing that I had once dreamed of being. I am hurt. I am in pain and nothing seems to help. Leaving me thinking what am I truly worth. Or do people even believe me... That this could really be THIS BAD? Which leads to many other thing. Doctors always just sent me away and told me that there was nothing wrong with me or it was just normal. Like oh you must have had a panic attack. But no I have a neurological disorder. And now that is something that I think my mind and heart is STILL soaking in. And another hard question is "well you had the surgery so you should be fine now." Yes I had the surgery but that didn’t cure my pain. That surgery was to help me NOT become paralyzed or deaf, blind or able to swallow or talk. That’s the main thing about this disorder… You will always have it. Chiari is never cured. It will always be a problem and it will always lurk around within me. You have to keep in mind the surgery was not to fix the pain it was to stop the progress of the Chiari paralyzing my body in certain limbs if not all and so on. Surgery is to relive the progress of Chiari! But With that being said I feel like my head and my aches and my pain have just been a spiral down and I don't ever think I am going to feel normal again or have a normal life anytime soon at least.
Right now I am currently working as a hairstylist 10-15 hours a week But that’s depending on how my body is feeling the day of. (Bad head day… Or an ok head day) See one thing about my conditions is that I can’t tell the night before or maybe even 5 hours before work of how I am going to feel right before I go into work. It’s like a bomb being dropped on you instantly. I have had to leave weddings from this feeling because the pain instantly becomes so bad. I honestly feel like working 10 hours a week (or up to three days a week) is way too much for me to even handle. The stress of pushing myself to work if it is last minute is my main concern. Standing my clients up and calling off and them getting mad is extremely stressful and not to mention I am not doing my job. It is wearing me so thin. The pain in my spine from one end to the other and standing for those few sort hours has me "OVER DONE." It’s to the point that I am so weak that any second this overload that I have bottled up is going to cause me to have a break down. Another scary thing about my life is if I feel this terrible at 25 what’s 30 going to have in store for me (so depressing beyond words). But I just have to pray to God and to NOT think so far into life that way. But it’s so scary. It’s such a chore to get myself up to get myself ready and to go to work. I feel like the only energy I have is forced. I am forcing myself to work and it’s leaving me in pure exhaustion (which yet again is what I mean by the overload breakdown). I feel the only reason I have not given up is one because of my FAITH but also because bills just don’t pay themselves when you tell the bill collectors "hey well I am sick". YEAH, I am sick I AM! But yes I do have to pay the bills still. Not only medical but everyday essential to life to live bills. I need to eat, a roof over my head, pay for my schooling loans, and the list goes on and on (as every adult should know)… I have a life that I can't just give up on I will let my disorder kill me before I give up. As much as I physical should stop working I financially can't at this point. But I feel like I am on my last leg of that physical breakdown. I used to work 40+ hours. Then a few months before the surgery (July 31st 2009) I was working about 30 hours . After the surgery I had four months off to heal. I honestly don't feel like I have healed at all or I guess the best way to put it is I feel like I will never be the same Felicia that I once was even though I had pain when I was 7,16 or 18 or even 20. This disorder has just eaten the life right out of me and gotten worse and worse through these past few years.
My daily routine isn’t much. Its me feeling so weak and so lifeless all the time. I currently am sleeping anywhere from 12-16 hours a night. I wake up a lot throughout the night. I am either in pain or just discomfort of how my head is positioned or how my body is laying and turned there is a lot of spinal pain from top to bottom. Its a horrible feeling knowing that even the one thing that should make you feel rest is still keeping you awake, feeling down, fatigued and in PAIN that one thing is sleep. I usually will force myself to wake up two hours before I have to go to work, working my five-hour shift coming home only to fall on the couch with pure exhaustion. Lying down is my only way to actually relax or relive some pain. As much as I am still in pain it’s the most comforting situation I can be in. I am on multiple medications to help with the pain but as everyone knows all medication have their side affects. I feel like the drugs might dissolve the pain a little bit but they don't do anything to restore my function for the day. When there is pressure on my brainstem (spinal area), my body does not work. The Neurontin that I take causes A LOT of problems. Some side effects being extremely sleepy (sitting and nodding off), a dizzy nausea's trippy feeling. It has also recently impaired my vision and reaction time. I can’t focus or get anything done (just like this letter-paper I am writing, It has taken me 5 weeks to get this far). I feel like I am not giving my best focus when I try to drive or do anything that requires me to be alert and able to see clearly. While driving my eyes constantly come in and out of focus due to the medication but that's not extremely new Chiari has been causing this problem for years but not anywhere near the everyday extreme it does now. I have had my eyes check multiple times only to be told no, there's nothing going on. This medication also makes it that I cannot think quickly enough to speak. I talk and then pause for a long while before I can find the words to communicate with. I forget things very easily now. I have horrible balance, say the wrong words when talking, and it makes me feel silly in how I act. I am very worried about my accuracy at work. I have to be on top of things and persistent and precise and feel confident in what I am doing. The “focusing” or poor concentration feeling is by far the worst symptom. But WITHOUT this medicine I am left in such horrific pain that all I can do is cry and moan in excruciating pain. So that’s where you must ask yourself which one of the two will you choose.Medication lowering you pain about 20-30 percent or horrible pain lasting all day long? And I say take the medication! Throughout the day I would say my head pain stays at a constant 4. (Even with taking up to 4 fioricets in a days time). But that’s not the end of it. It can at any point in time jump up to an 8. Anything after an 8-9 on the pain scale means I am going to the E.R. because something is wrong. Which I have had to do many times from the Chiari. One thing I have learnt about my condition is I know when something is wrong. I just know. So I am the best to judge nobody else.
I know that Chiari, fibromyalgia and Ehlers-Danlos syndrome (EDS) have completely 180-ed my life (not to mention there is probably more they will diagnose me with in the near future, just because that seems to be the trend). Chiari has taken away all my joy. Its not only heartbreaking its so depressing. You feel so alone. It's so hard to believe this can happen to such a young person. Its so hard to think that I am already disabled at this age or that I am disabled. Its also so hard to ask myself what does the future hold for me. I know that I will never be the same Felicia I once was. I will never get to do all the things that I have once loved. That leaves me truly broken. I have become that person that push’s people away, and I have became very good at it. This includes my own family and my husband who loves me dearly, who have stayed by my side throughout this entire ordeal. This is most definitely the most heart aching thing that this whole disease has brought on me pushing away my loved- treasured ones. I feel I will never feel normal and will always be in some sort of constant pain. Its come to the point that I don’t even want to take my two dogs out to even go potty because I am so weak. Or walking them, and this used to be something that my husband and I did everyday on a daily bases. Every night we were out walking them. But now I cant even pull myself together to even do that. I used to find tons of joy riding my bike to work but that is also something that I have no interest in even thinking about doing. My body is to weak to try and peddle the bike all that way. These were once my two easiest forms of exercise and things that were simple that I liked to do. I cannot do too many things in one day because of exhaustion and knowing that if I overdo it the next day will be horrible, I cannot get up and go on little sleep.
You can also follow my “blog” (an online journal) for more information. Or email me if you have any questions.
FeliciaKayDehmlow@gmail.com
http://thisismefe.blogspot.com/ .....by Felicia K.Dehmlow
Kathleen
Kathleen's story coming soon. Be sure to read it first in the June 2010 Newsletter.
Rachel
My name is Rachel. I live in Pennsylvania. I am a 34 year old married mother of three. My Chiari journey began shortly after my first child was born. I was complaining of migraines and leg numbness. When I was sent for an MRI, I was diagnosed with Chiari Malformation at the age of 25. My neurologist sent me to a neurosurgeon who explained the surgery and told me that it didn't appear I needed it, but to keep an eye on things. Looking back now and knowing as much as I do, thank God I didn't need it as I have an inkling he never actually had a patient with Chiari. All of his information was very old. Overtime, as I had questions and new symptoms arose, when I attempted to go back to my neurologist, he apparently left the country to broaden his studies. I made an appointment with his partner, who informed me that I never did have Chiari, that I was misdiagnosed. He was one of five neurologists over a five year period who also informed me that I never did have Chiari, it was a misdiagnosis. So, I went on with my life assuming they were all correct. After a few years of fear, the term Chiari Malformation became a distant memory in the back of my head.
During my second pregnancy many years after my diagnosis, I had severe back problems towards the end. My migraines got worse during the pregnancy and my leg numbness was becoming increasingly worse. Since no neuro would believe I had Chiari, it was just left untreated. Same issues with my third pregnancy. Only this time, after the pregnancy, not only did the symptoms not subside, but got worse. Before I knew it, I had over 30 symptoms that were associated with Chiari Malformation (pressure issues, pain the back of head, vision and hearing issues, leg, arm, face, jaw, upper/lower back, hip, neck and any other pain you could think of, numbness throughout body, horrible insomnia just to name some). Years prior, I could not find any information about Chiari on the Internet, but decided to try again. This time, not only did I find a ton of information, but a couple support groups as well. I joined some support groups, asked for a neuro recommendation, assuming I would have to travel to get some actual answers, and find out there are actually highly recommended ones in my area! I was so happy to find this out and I even got an appt the very next day! But on the other hand, was pretty positive, that like the other neuro's, was just going to be told I was misdiagnosed and never get to the root of my problem.
After weeks of updated testing, it was confirmed that I had Chiari Malformation. Although my herniation was not big, I did suffer many symptoms and my quality of life was miserable. It got to the point where I could no longer give my little ones a bath or dress them. My surgeon highly recommended surgery. I felt relieved to have an actual diagnosis from a real doctor who dealt with Chiari, once and for all.
I had my surgery on January 7, 2008. I was sitting up within hours of surgery and walking around the hospital that evening. I was home two days later and off pain meds the same day. I was doing really well recovery wise, but unfortunately I developed a staph infection and had to have surgery to clean it out the following month. I continued to battle staph infections on and off for seven long months and was so relieved when that finally ended. Having surgery was the best choice I could have ever made. It gave me my life back. Almost every chiari symptom has gone away. I still get the feeling like my head will burst when I get very stressed out in life, but I have luckily learned to keep that in check so it has happened very few times. If it weren't for the Fibromyalgia, my life would basically be back to normal!
I owe so much to the other Chiarians who helped me find a doctor and get my life back on track again. I wouldn't be where I am today without them. The Chiari community is a wonderful and supportive community. I am proud to part of them!
Please feel free to look me up on facebook: Rachel Reitlinger Sell
Some of us sport hardware (wheelchairs, braces, etc) but most of us don't. We look like everyone else.This is what they call an invisible disability. So the next time you see one of us, please understand the energy it takes to smile, laugh and PRETEND.
