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 and when I finally do pass out from pain and exhaustion, 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 my 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 pretty bad 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. This is not the way I want to live in pain and perpetually in 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.

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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 one another'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?

The most common form of Ehlers-Danlos syndrome (EDS type 3) occurs in an estimated 1 in 5,000 people, according to Medline Plus. It is a genetic disorder that affects connective tissues throughout the body. The pain of EDS varies from mild to extreme, but can become chronic over time and require lifelong physical therapy as well as surgical and pharmacological treatment. There are many types of EDS, which may have multiple comorbid conditions. For instance, some individuals with EDS type 3 also have a hindbrain herniation called Chiari malformation (CM). Some research suggests that having EDS may dispose people to developing an acquired form of Type I CM that can be difficult to manage appropriately even with advanced surgical intervention.

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

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.

· Chiari Malformation (2013, August 21). Retrieved from
· Chiari Malformation and EDS (2014, April 15). Retrieved from
· Ehlers-Danlos Syndrome (n.d.). Retrieved from
· Ehlers-Danlos Syndrome Myths and Facts (2009). Retrieved from
· What is Ehlers-Danlos Syndrome? (2006, May). Retrieved from
· Underlying Causes of Dysautonomia

Nov 10, 2014

I Need This In My Life!

This just might be the greatest music video there ever was. Apparently 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.


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, bravest on earth.

October is Dysautonomia Awareness Month! What are you doing to raise awareness?

This October marks another great annual Dysautonomia Awareness Month. It is always so awesome to see the patient community rally together and do amazing things to help raise funds to support research and help raise awareness in their communities. It's inspiring to see young kids participating alongside their parents, making noise for turquoise! Thankfully, turquoise is one of my favorite colors! I love to wear it and also have plenty of bright turquoise throughout my house. I even painted a turquoise pumpkin for Halloween this year. If you can, get out there and raise some awareness in your community. Tell someone about dysautonomia. Help educate a new medical practitioner who may not know what it is. Find a fundraising idea and donate the funds to research. Or purchase something on through this page and part of every sale automatically gets donated back to dysautonomia research (it doesn't cost you a penny extra, amazon donates their share of the sale!)

There is lots happening online as well. Check out for a daily dysautonomia factoid. I have learned SO many things I didn't know. There's also a live Tweet-A-Thon happening on October 23rd.

Oct 2, 2014

2014 In A Nutshell

On the eve of a very important appointment with a very important neurosurgeon, I find myself brimming with anxiety over the occasion. You'd think after seven years of health drama doctor's appointments wouldn't even phase me anymore, but the reality is, sometimes they still do. Especially the important ones. So I figured what better way to spend my evening than to engage in a little blogtherapy and update you all what's been happening in my life so far this year and also explain why I haven't updated this blog in ten months!

Let's start off with some happy news! In February I got engaged! The proposal was magical and perhaps I should save that story for a separate post! We were both so thrilled that we started wedding planning almost immediately but then we both took a step back and realized that planning a huge wedding was starting to get really overwhelming (and really expensive!) really quickly. The more I thought about it the more I realized that a wedding is merely one day and that the marriage itself is what really matters. So we went from wanting a huge outdoor wedding and reception to now wanting a very small, private, low-stress ceremony and will probably skip having a formal reception altogether. It just doesn't make sense to exhaust all of our physical and financial resources on one single day when the time, money and energy expended could be better invested in creating our home together. I've basically stopped wedding planning for now and I'm so much more at ease. We've agreed upon the perfect small venue and I am going to wear a gorgeous gown. I also want a cool, Cake-Boss style cake. But other than that our wedding will be no-frills, no fuss.

In April we started fixing up my fiance's house to put on the market. Boy was that a job! It really tested our patience as things just kept going wrong and the house proved to be a lot more work than any of us expected! Several weekend work parties, one dead cat (long story!) and countless manpower hours later, the house is finally listed on the real estate market and being shown! Cross your fingers for us that it sells soon so we can move forward with the next chapter of our lives.

In June I finally had an upright MRI of my brain and cervical spine as ordered by my primary care doctor for the wonky discs in my neck that kept going out. The MRI results confirmed my biggest fears: that I do indeed have a Type 1 Chiari malformation as well as cervical instability secondary to Ehlers-Danlos syndrome. I always knew Chiari was a distinct possibility because of my EDS, but I figured since my POTS has gotten so much better over time that I surely I didn't have any more comorbid conditions to worry about. Well, I was wrong. My Chiari measures a mere 5mm but I am symptomatic with headaches, balance issues and tingling extremities and have been for over a year now. So far I have seen a local neurosurgeon, had a Skype consult with Dr. Rekate of The Chiari Institute (who was wonderful and a wealth of information!) and have one more consult with the head of neurosurgery at the local University tomorrow. Dr. Rekate told me he trusts this surgeon's skill so I feel a bit better in knowing that he's highly regarded by his peers. However my cervical instability is perhaps more of an issue than the Chiari itself so I need to make sure my local doctors take it seriously and address it accordingly.

In July and August, I ended up getting sent for several additional tests: an MRI of my thoracic spine, an MRI of my lumbar spine, cervical x-rays in flexion and extension, a 3D CT scan of my cervical spine, and a cine MRI of my brain/c-spine. Am I ever sick and tired of MRI's! The good news is that my MRI's showed NO evidence of a syrinx or a tethered cord. Tomorrow I will receive the results of the cine MRI from the neurosurgeon and boy am I nervous. The cine MRI is a specialized MRI that assesses the cerebral spinal fluid flow or extent of the obstruction from the Chiari. I am hoping he tells me I don't need surgery, or at least not urgently. The prospect of brain/spine surgery scares the bejeezus out of me. And I would really like to focus on things like getting married and finding our new home. A surgery would certainly disrupt my plans. But will have to see what he says tomorrow and take everything one step at a time.

Can't believe it's already October which also happens to be dysautonomia awareness month! This month I look forward to getting 3 teeth crowned (ugh!) and watching copious amounts of Gilmore Girls on Netflix. It's also my mom's 60th birthday which is kind of a big deal. Not quite sure how to commemorate the occasion but she has been a huge support to me my whole life and especially these past few months as I have suffered some pretty rough days health-wise and dealt with yet another life-altering diagnosis. Wish I could buy her a really cool, expensive gift! Or send her on a vacation somewhere tropical and relaxing!

As for November and December, we are hoping my fiance's house will finally sell so we won't have to maintain it over the winter and I am really really hoping to avoid neurosurgery. I should know more about that tomorrow. Until then, thanks for reading! I have really missed blogging and missed hearing from my lovely readers as well! Hope you are all doing well and enjoying improved health and wellness!