Visit http://csfinfo.org for more information and a growing body of research on Chiari. Be sure to wear purple to help spread awareness during the month of September!
Jan 27, 2017
In the middle of difficulty lies...opportunity?
As I sit quietly awaiting a CT scan of my diseased sinuses wearing a 24-hour holter monitor, a neck brace and a mask over my face to protect my weakened immune system in a waiting room full of strangers, I wonder, when did my life get so complicated? Just under ten years ago I was having the time of my life, working and going to school full-time while enjoying an active social life. I have to be totally honest here: I really, really miss those good ol’ days, before chronic illness struck out of the blue and overnight. Since 2007 I have endured countless doctor’s appointments, invasive tests, and probably had hundreds of tubes of blood drawn out of my body. Diagnosis after diagnosis has accumulated on my medical record, which has now become a dauntingly long and complicated list. I Just. Want. To. Be. Normal. Again.
In the midst of the worst sinus infection of my life I reflect upon these things. Two and a half months of antibiotics and on my third one now and we’re deciding whether or not it’s really time for surgery, which has been delayed for a few years due to a host of new and complex conditions arising. Sinus surgery has not exactly been top priority until now, because the antibiotics have stopped working. I am terrified at the prospect of surgery, but my facial pain is so severe and unrelenting that I am desperate for some relief. A whole year of steroids have wreaked havoc on my immune system and my body needs a break from fighting the giant sinus infection that is my life.
I’m certain many of you can relate to how I’m feeling. Frustrated, overwhelmed, angry, perplexed, scared and a bit sad that I’ve had to endure so many maladies over these past ten years while the majority of my peers have been living it up: traveling, partying and achieving major milestones. It was definitely not easy losing most of my twenties to chronic illness. 30 was the worst year yet due to getting hit by yet another scary and sudden illness called Guillain Barre Syndrome, which stole my ability to walk within three days time, and which I’m still not completely recovered from. I still have some residual nerve damage in my legs and probably my arms, but at least I can walk again and open a package of cheese for myself. So far 31 isn’t shaping up to be so grand either. My chronic sinusitis is clearly kicking my butt and with that I’ve had some frighteningly familiar neuromuscular symptoms return. I could scream from the sheer frustration of still being a reluctant passenger on the seemingly never-ending doctor merry-go-round.
But, if the words Albert Einstein so eloquently spoke are true, does opportunity really lie in the middle of difficulty? Perhaps I should be approaching this surgery optimistically, as the goal is to help me breathe better, which may end up improving many of my other symptoms as well. It’s all about shifting my perspective of the operation from fear and dread to optimism and opportunity.
I hope you, dear reader, have been doing well during my absence. It’s the beginning of a new year and I wish you improved health and happiness!
Mar 9, 2015
Survival Mode
The past few months I've been stagnating in survival mode due to horrendous, persistent pressure headaches at the base of my head and upper neck. Throw in some charlie-horse muscle spasms on the sides and back of my skull and in my upper neck that are palpable to the touch and it's a recipe for intense pain, the kind that keeps me awake at night. When I finally do pass out from pain and exhaustion, the pain promptly awakens me again in a mere two to three hours. Subsisting on two to three hours of sleep per night is enough to render even the sanest of people completely mad. This lack of sleep, coupled with intense chronic pain really gets me down at times. I feel like a zombie most days and on my "good" days where the pain is bearable and I can attempt to distract myself from it, I get overzealous and overdo it because I never know how long the low-pain stretch will last and I feel the need to take advantage of those times whenever I can. However, on the low-pain days, all my body seems to want to do is catch up on sleep, but my mind is telling me I need to get out of the house and enjoy whatever I can before the headache becomes hellish again. The worst part of all this is, my doctors still can't seem to tell me with any certainty whether this head pain is a result of my 5mm chiari malformation or more due to my atlantoaxial instability (right lateral subluxation of C1 on C2). Or it could possibly be occipitoatlantoaxial instability (meaning my ligaments are so lax due to EDS that they're failing to hold my head on straight). But again, not even neurosurgeons can pinpoint with any certainty what exactly is causing the intense pain.
My best bet, no doubt, would probably be to travel to a place like The Chiari Institute of New York where they fix the instability and decompress the chiari in the same 6-hour procedure, taking a team approach with two surgeons present in the operating room, one to fuse the upper vertebrae and one to decompress the chiari. That means (hopefully) only having to go under the knife once and ensuring that my bobblehead is stabilized. However, New York might as well be Mount Everest to me right now as travel has become just about impossible lately. Even car rides in my trusty Aspen Vista cervical collar have become almost unbearable. Any movement really triggers and worsens my neck and head pain, which would suggest instability is likely the pain-causing culprit. This is not to say that my chiari is not causing me any problems. My balance has become really bad this past year or so. I used to be so graceful, so poised back in the olden days when I danced ballet. Now I am so clumsy I am tripping over my own feet, dropping things left and right and I have to concentrate really hard on basic tasks like walking or carrying something. I know that is not normal, not for me, not for anyone. I used to enjoy going on walks even after getting sick with POTS, but lately I fear that I look like a drunk person trying too hard to keep my balance and I wonder if people think I'm a drunk when I'm out in public...
I know that it's definitely time to do something. I've been told by my physical therapists, neurosurgeons and primary care physicians that unfortunately physical therapy is never going to remedy the problem, no matter how diligent I am with my exercises. My ligaments are too lax thanks to stupid EDS hypermobility. And while physical therapy can help to strengthen the surrounding supportive muscles and hopefully preserve the rest of my neck function and help provide stability to my neck's lower vertebrae (I currently have four bulging discs in my c-spine and some mild arthritis between all my vertebrae), the upper vertebrae are probably going to require a cervical fusion procedure to correct the instability. The prospect of a risky neurosurgery makes me sick to my stomach.
In terms of major surgery I've only had an emergency appendectomy, and fortunately I didn't have too much time to ruminate on the issue or be scared as I wasn't really given a choice. I do remember asking the on-call surgeon if I could just go home, research the procedure for a bit and then return the next morning for the surgery. He informed me that I could do that, but then he'd see me again in a day or two at most with a ruptured appendix and have to perform an even more painful surgery with a much longer hospital stay. So I consented to have a laporoscopic appendectomy that same night, and honestly, it was the best decision I ever made! I was in the hospital recovering for one day and then they released me the next night. Luckily the nurses and anesthesiologist were all very careful with me because of my dysautonomia and EDS. I was well-hydrated and there were no complications. I took some extra strength Tylenol at home and didn't even need the narcotic pain meds they prescribed. It hurt, sure, but in a few days the worst of the pain had subsided and I was able to get on with my life and start eating and drinking normally again.
I have a feeling that neurosurgery is a whole different ballgame though. I don't like the thought of anyone cutting into my neck and head. I just don't. It's icky. Necessary, but icky, and frankly very scary. There's too much important stuff up there. I have thought long and hard about what will happen to me if I don't have surgery though and the possibilities are also scary. I can't get in any more car accidents or sustain any type of major injury as it could easily be game over for me. Internal decapitation does not sound like a good way to go. So although it will be scary and painful, I have decided I need to stop being so afraid and proceed with surgery at the soonest opportunity. I do not want to live perpetually in pain and a neck brace. It would be nice to not have to wear this thing indefinitely. And even nicer to be out of pain. I can barely remember what it feels like to be pain-free but I know it would be amazing and I could accomplish so much more with my time and get so much more out of life.
Most importantly, if I get my health back I can devote 110% of my energy and focus to helping others who are still in pain and suffering from chronic, painful and misunderstood conditions like dysautonomia, EDS and chiari: the trifecta or "The Sara Syndrome" as the brilliant Dr. Rekate has coined it. Also, if you haven't seen it already, last week The Today Show featured a news segment about a young boy suffering from the debilitating effects of chiari malformation, dysautonomia and EDS. Please watch his story and share with your friends and family. This kind of national, mainstream media coverage is a huge deal for patients like us.
My best bet, no doubt, would probably be to travel to a place like The Chiari Institute of New York where they fix the instability and decompress the chiari in the same 6-hour procedure, taking a team approach with two surgeons present in the operating room, one to fuse the upper vertebrae and one to decompress the chiari. That means (hopefully) only having to go under the knife once and ensuring that my bobblehead is stabilized. However, New York might as well be Mount Everest to me right now as travel has become just about impossible lately. Even car rides in my trusty Aspen Vista cervical collar have become almost unbearable. Any movement really triggers and worsens my neck and head pain, which would suggest instability is likely the pain-causing culprit. This is not to say that my chiari is not causing me any problems. My balance has become really bad this past year or so. I used to be so graceful, so poised back in the olden days when I danced ballet. Now I am so clumsy I am tripping over my own feet, dropping things left and right and I have to concentrate really hard on basic tasks like walking or carrying something. I know that is not normal, not for me, not for anyone. I used to enjoy going on walks even after getting sick with POTS, but lately I fear that I look like a drunk person trying too hard to keep my balance and I wonder if people think I'm a drunk when I'm out in public...
I know that it's definitely time to do something. I've been told by my physical therapists, neurosurgeons and primary care physicians that unfortunately physical therapy is never going to remedy the problem, no matter how diligent I am with my exercises. My ligaments are too lax thanks to stupid EDS hypermobility. And while physical therapy can help to strengthen the surrounding supportive muscles and hopefully preserve the rest of my neck function and help provide stability to my neck's lower vertebrae (I currently have four bulging discs in my c-spine and some mild arthritis between all my vertebrae), the upper vertebrae are probably going to require a cervical fusion procedure to correct the instability. The prospect of a risky neurosurgery makes me sick to my stomach.
In terms of major surgery I've only had an emergency appendectomy, and fortunately I didn't have too much time to ruminate on the issue or be scared as I wasn't really given a choice. I do remember asking the on-call surgeon if I could just go home, research the procedure for a bit and then return the next morning for the surgery. He informed me that I could do that, but then he'd see me again in a day or two at most with a ruptured appendix and have to perform an even more painful surgery with a much longer hospital stay. So I consented to have a laporoscopic appendectomy that same night, and honestly, it was the best decision I ever made! I was in the hospital recovering for one day and then they released me the next night. Luckily the nurses and anesthesiologist were all very careful with me because of my dysautonomia and EDS. I was well-hydrated and there were no complications. I took some extra strength Tylenol at home and didn't even need the narcotic pain meds they prescribed. It hurt, sure, but in a few days the worst of the pain had subsided and I was able to get on with my life and start eating and drinking normally again.
I have a feeling that neurosurgery is a whole different ballgame though. I don't like the thought of anyone cutting into my neck and head. I just don't. It's icky. Necessary, but icky, and frankly very scary. There's too much important stuff up there. I have thought long and hard about what will happen to me if I don't have surgery though and the possibilities are also scary. I can't get in any more car accidents or sustain any type of major injury as it could easily be game over for me. Internal decapitation does not sound like a good way to go. So although it will be scary and painful, I have decided I need to stop being so afraid and proceed with surgery at the soonest opportunity. I do not want to live perpetually in pain and a neck brace. It would be nice to not have to wear this thing indefinitely. And even nicer to be out of pain. I can barely remember what it feels like to be pain-free but I know it would be amazing and I could accomplish so much more with my time and get so much more out of life.
Most importantly, if I get my health back I can devote 110% of my energy and focus to helping others who are still in pain and suffering from chronic, painful and misunderstood conditions like dysautonomia, EDS and chiari: the trifecta or "The Sara Syndrome" as the brilliant Dr. Rekate has coined it. Also, if you haven't seen it already, last week The Today Show featured a news segment about a young boy suffering from the debilitating effects of chiari malformation, dysautonomia and EDS. Please watch his story and share with your friends and family. This kind of national, mainstream media coverage is a huge deal for patients like us.
Nov 28, 2014
I Am Thankful for The Chronic Illness Community
Every year on Thanksgiving I take a minute to reflect upon what I am thankful for. I have found hope, solace and support among the chronic illness community of spoonies, zebras, potsies and zipperheads! I have met some of my best friends here and it's comforting to know that we can relate to each other's struggles on a personal level. It's also a major victory when one of my chronic illness friends has a good day or their health improves. We all know not to take those days for granted and what a major milestone health improvements can be. Even the smallest health victories are worth celebrating. It's amazing to have friends of all ages, from all different walks of life, from all over the country and all over the world sharing the same conditions. Although these cruel conditions are often what unites us initially, I have found that there is often so much more that we share. We are much, much more than just our illnesses and are certainly not defined by them. I often talk excessively about my condition to my chronic illness friends simply because they really get it, and they can relate. But I have found that the majority of us share many common interests and attitudes beyond our shared symptoms. If you are reading this, I am thankful for YOU!
The Sara Syndrome
This presentation by Dr. Rekate really resonated with me. It captures the typical experience of patients suffering from dysautonomia, joint hypermobility, chiari malformation and craniocervical instability and the associated sensitivities and autoimmune problems we struggle with.
Nov 15, 2014
Ehlers-Danlos Syndrome and Chiari Malformation: What’s the Connection?
Genetics and Birth Defects
While EDS develops before birth, CM in EDS patients can be acquired later in life as a result of the unstable connective tissues throughout the neck and spine pulling the brain downward. Cranio-cervical Instability (CCI) and Tethered Cord Syndrome (TCS) are features found regularly in EDS patients that may contribute to the occurrence or severity of the CM. EDS is considered an inherited disorder because it is caused by genetic mutations that affect collagen. Collagen is a building block of proteins that gives connective tissues their strength. According to the Lister Hill National Center for Biomedical Communication, the following gene mutations may lead to EDS:
· COL1A1 and COL1A2
· COL3A1
· COL5A1 and COL5A2
· ADAMTS2
· TNXB
· PLOD1
Although CM may indeed have a genetic component in the congenital form, the only genetic link in the acquired form is the presence of connective tissue disorders like EDS. While considered an uncommon disorder, CM typically appears during childhood or early adulthood, although symptoms can surface at anytime and may be triggered or exacerbated by accidents, falls or blows to the head. During this stage of development, CM can become apparent because the skull cannot adequately contain the cerebellum and this crowding at the base of the skull can disrupt the essential flow of cerebral spinal fluid (CSF), as well as impinge important nerves and even disrupt blood flow. While CM is largely considered a congenital condition by most neurosurgeons, there is a growing body of evidence that it runs in families, just like EDS, and furthermore, that people with EDS may be predisposed to developing CM, although they were not necessarily born with the CM as a congenital abnormality.
Similarities and Differences in Pain
Perhaps the biggest similarity between EDS and CM is chronic pain. People with EDS are often misconceived as being clumsy or as having imaginary pain because of the fact that they may look completely healthy on the outside. The reality is that the range of pain spans from mild to severe, but can occur anywhere along the body where connective tissues are present. If you suffer from EDS, you may bruise easily and have unusual scars after a wound heals. Your skin may be unusually stretchy, and you might have prominent, visible blood vessels. EDS is also known for causing joint pain and frequent joint dislocations and subluxations throughout the body, which can necessitate the need for joint bracing and careful physical therapy to retain independence and mobility.
CM causes several different types of chronic pain, including a severe characteristic headache at the base of the skull, as well as pain throughout the neck, spine, and throat. It can also trigger severe nerve pain throughout the body. Individuals afflicted with CM may experience serious neurological deficits, trouble swallowing, tinnitus, vision disturbances and balance disruption that can lead to trouble walking and/or clumsiness.
Does EDS Cause CM?
Several studies suggest there is a complex connection between the two disorders that goes beyond the typical “cause and effect” association. According to the Hypermobility Syndromes Association, the causes of CM in a patient with EDS may be different than those in a patient without EDS. Furthermore, EDS patients seem to be more prone to Type 1 CM, which is the most common form of the disorder. The Association reports that a study of 2,813 Type 1 CM patients found that about 357 of them also had EDS symptoms.
More studies are needed to determine precisely how EDS causes CM. The only way to accurately determine the severity and cause of your condition/s is to seek appropriate testing and evaluation from a team of specialized medical professionals including neurologists, neurosurgeons and geneticists. EDS Type 3 can be diagnosed by a geneticist who administers a Beighton Scale Test while CM can be diagnosed by a neurologist or neurosurgeon through an MRI of the brain and cervical spine, preferably an upright MRI in those with suspected connective tissue disorders. Ideally, you should also seek out a doctor that specializes in chronic pain disorders to help manage the daily pain since medical testing isn’t the only assessment of pain disorders.
Kristeen Cherney is a freelance health and lifestyle writer who focuses on preventive measures for a better quality of life. Cherney holds a BA in Communication, and is currently finishing her MA in English.
Resources
· Chiari Malformation (2013, August 21). Retrieved from http://www.mayoclinic.org/diseases-conditions/chiari-malformation/basics/definition/con-20031115
· Chiari Malformation and EDS (2014, April 15). Retrieved from http://hypermobility.org/help-advice/chiari-malformation-eds/
· Ehlers-Danlos Syndrome (n.d.). Retrieved from http://www.nlm.nih.gov/medlineplus/ehlersdanlossyndrome.html
· Ehlers-Danlos Syndrome Myths and Facts (2009). Retrieved from http://www.ehlersdanlosnetwork.org/myths-facts.html
· What is Ehlers-Danlos Syndrome? (2006, May). Retrieved from http://ghr.nlm.nih.gov/condition/ehlers-danlos-syndrome
· Underlying Causes of Dysautonomia
http://www.dysautonomiainternational.org/page.php?ID=150
Nov 10, 2014
I Need This In My Life!
This just might be the greatest music video there ever was. Apparently CouchCar.com is a thing, a SoCal thing. Props to Taylor Locke Music for showing the world how to travel in comfort and convenience. I can't stop watching this!
One Lovely Blog Award
Thanks so much to Mary over at A Body of Hope for nominating me for the One Lovely Blog Award! Here are seven facts about me:
1) I have a growing nail polish collection and recently started dabbling (quite literally!) in nail art.
2) My favorite season is fall. I love all the warm hues, crisp cool air and pumpkin spice everything at Trader Joe's!
3) Earlier this year I was diagnosed with Chiari malformation and craniocervical instability secondary to Ehlers-Danlos syndrome. A few more serious diagnoses to add to the list. I am trying my best to cope with these both physically and emotionally.
4) I am currently writing a book. So many readers have told me I should write a book since I started this blog years ago and I am finally taking their advice!
5) I have mild OCD tendencies, particularly when it comes to organization, cleanliness and germs. I have never been officially diagnosed but even as a small child cleaning my room put me at ease and everything had to be in its place. As a result, I had the neatest, prettiest pink room on the block!
6) Earlier this year I experienced a startling revelation that I am no longer as young and cool as I used to be...I am still listening to stuff I listened to as a kid in the 90's and 2000's, and I still think it's much better than this newfangled music kids these days listen to! ;)
7) I wear yoga pants just about everyday. Today marks the first time in two months I have put on a pair of jeans. Why? Because yoga pants are much more comfortable, and the type I wear provide subtle compression which helps with hypermobility!
I will be contacting my nominees via Facebook shortly! I look forward to reading your posts.
Kristina
Oct 18, 2014
I love to hear from you!
A BIG thank you to Mary over at A Body of Hope for making this for me! I love to hear from my readers and read your blogs! If you have a blog of you're own please share it with me! It is always great to hear from you guys and see what you're working on. There are so many talented people out there! Thanks again, Mary!
So True!
A friend and fellow dysautonomia warrior posted this on her Facebook this morning and it really resonated with me! People in the chronic illness community are among the strongest and bravest on earth.
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