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.

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
· 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

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

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 that 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 on 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 hours of manual labor 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!

2013 Year In Review

This year brought a lot of major life changes and new beginnings for me. Mostly positive ones. I moved to a new home in a different location and am now (finally!) comfortably settled in here. I am living within walking distance to a major doctor's office, lab and my physical therapy clinic. I'm also extremely close to groceries, gas and restaurants. The convenience of not having to drive everywhere has simplified my life a great deal. The appointment running has calmed down a bit too with the exception of physical therapy and dentistry that is. The dental work I've had done and am still having done has tried ever fiber of my being this past year. I only have seven more fillings and five more crowns to go before I am completely cavity-free. I have had a grand total of three root canals, one crown and three fillings done this year. The physical, emotional and financial stress of dental work is enough to drive anyone clinically insane. I realize I have to keep plugging away at fixing my teeth to avoid future root canals and extractions, as those are not an option for me.

My physical therapy appointments though tough, are also rewarding. I don't look at physical therapy as just another appointment, I view it as an integral part of my weekly routine. I am already seeing results in the strength department. Luckily I build muscle pretty quickly which is helping to stabilize my ankle along with the rest of my wobbly body. I am lucky to live right next door to such a great physical therapy clinic with a therapist who also happens to suffer from hypermobility and TMJD herself. Doing physical therapy as a teen saved her from having TMJ surgery and that experience was what led her to the field. I feel optimistic about the progress that I am making and feel myself getting a lot stronger each and everyday. I only go twice a week but have an intense home exercise program that I do daily, sometimes in my living room and sometimes at my apartment complex's on-site gym, which is another nice feature of the new place.

The first month after I moved was fraught with major challenges concerning air quality. I had selected and moved into a ground floor unit, but unfortunately the complex was built on a wetland and as soon as the rains started in my apartment reeked of a strong, sickening mold smell. So much for life in a "luxury" apartment. A home humidity monitor I purchased revealed that the internal humidity reached 75% everyday. A normal internal humidity level is between 30-50%. Anything above 60% provides ideal conditions for mold growth. I fought with the complex to let me move to another unit. An option which was totally feasible considering it was brand new construction and only a quarter of the units were occupied. Their corporate management initially denied my request calling the mold smell a "subjective" complaint since they couldn't pinpoint the exact source of the mold. Upon conducting a bit of careful research and calling the city building inspector it turns out that the problem was much larger than bleach or exhaust fans could have ever fixed. In order to remedy the problem, the building would have had to have been torn down completely. Because apartments are technically classified as commercial space (even though residents inhabit them as their homes,) the city allows them to build on concrete slabs without crawl spaces. They didn't allow the concrete slab to cure long enough in the construction process which resulted in a subsequent sponge-like effect on the foundation every time it rained. Every time it rained that damp moldy smell crept up into my home from the concrete floor, through my carpet and into the living space.

I finally moved into a new apartment in the same complex at the beginning of October after enduring a month of hell in a moldy apartment. I am now living on the second floor of an apartment that doesn't smell of anything other than whatever I have been cooking in the kitchen. My sinuses are much better now and I can actually breathe out of my nose in the mornings again. Something I had wondered if I would ever be able to do again. Although sinus surgery may become an inevitability down the road due to a deviated septum and bone spurring, for now I have dodged a major bullet by avoiding surgery. And I am happy to put it off for as long as possible unless it becomes a necessity. My new apartment feels much healthier and I can breathe easy here which is a huge relief.

I am doing decent in the health department at the moment (aside from a nagging, two-week old mystery upper right quadrant and back pain that landed me in the ER for bloodwork, an abdominal ultrasound and a HIDA scan to no avail and is currently being investigated by my primary care doc). My health is something I do not take for granted at all. For the most part my POTS is controlled and doing much better now that I've removed myself from the moldy environment of the other apartment. Earlier this year I had a scary bout with benign positional vertigo that finally subsided after taking a month round of Augmentin antibiotics for my sinuses. It was a scary time for me. Every time I layed on my right side or flat on my back I would get the spins. This continued for a few months with my doctor telling me it was "just some inner ear virus," and an ENT insisting it was benign positional vertigo spurred by spending too much time in a dental chair as I had a scary incident where some heavy duty dental drilling back in the chair spurred a major attack. Whatever it was, it is no longer happening to me now and I am so thankful. I had a follow-up with a new ENT the other day and luckily they were not able to trigger the vertigo in his office. Because of my history with rotational vertigo during dental work though, he wants me to undergo a more sophisticated test called a VNG early next year to measure eye movements during different positions and then conduct a repositioning procedure in the office to resposition any inner ear crystals that may have become dislodged.

In 2014, I still have plenty of work to do. I would like to make more strides in the health and fitness department with an emphasis on healthier eating. I definitely have some work to do in that department. I am fortunate to have a wonderful boyfriend who enjoys cooking for me, the problem is he enjoys cooking tasty comfort foods without worrying about things like nutritional content or calories. Oh well, I can't complain too much. I know how lucky I am to have someone who cares about me and takes good care of me. I am finally in a healthy relationship, one that I don't have to change myself for. Compared to my verbally abusive ex, this experience has been night and day and I am so thankful.

So to sum it all up, this year has brought some health ups and downs, but overall I'm in a good place health-wise. I'm in a good place location-wise after finally moving into a healthy apartment. And I'm in a great place relationship-wise with someone who loves and respects me. Next year I intend to get my body in peak physical condition and hopefully start to renew old hobbies one by one without destroying my body in the process. For now I will have to say no to ballet but may take up pilates and yoga again in the new year.

Liebster Award!

I am honored to have been nominated for a Liebster Award by one of my favorite new bloggers and fellow potsy Brittany at Fabulously Faint. Admittedly I don't know much about the award except for the following: 

The Liebster Award is used by bloggers who have 200 follows or less on bloglovin' to promote each other and to make more friends in the blogging community and attract more attention to their blog. Each nominee must first list 11 random facts about themselves and then answer the 11 questions proposed by the person who nominated them. Lastly choose 11 bloggers and ask them your own 11 questions.

I in turn nominated eleven of my favorite bloggers! They happen to all have dysautonomia and/or related conditions.

Brooke at Growing Older, Growing Up?

C.M. at Life Unknown

Erin at My Life as ErinJ0

Kingsley at Life As Grike

Michelle at Living with Bob

Lauren at POTS Grrl

Hannah at Hannah's Dysautonomic Life

Candice at Infectiously Optimistic

Michele at Dysautonomia Normal

Jessica at Falling Apart At The Seams: My Life with Ehlers-Danlos

Anomie at Anomie Fatale: The Agalma Femme


11 Random Facts About Me!

1) I have Postural Orthostatic Tachycardia Syndrome, Ehlers Danlos Syndrome and Temporomandibular Joint Disorder.

2) I am obsessed with 90's pop culture nostalgia: toys, junk foods, music, television etc.

3) I adore the color pink and always have. Because some things never change.

4) My all time favorite book is The Great Gatsby.

5) I have perfectionistic tendencies and am very OCD about germs and cleanliness, though I have never been officially diagnosed.

6) I hold three bachelor's degrees including a degree in English Literature.

7) I loathe technology and hate trying to keep up with all the latest and greatest gadgets. I resisted the whole touchscreen smartphone craze for a LONG time. 

8) I am a dog person. If there are 20 people in the room and there are any dogs in that room, the dogs will always come sit on my lap. This phenomenon has happened on many occasions. We get each other and speak the same language!

9) I used to produce magazine style shows and host talkshows for government television.

10) I have naturally curly hair that is extremely hard to tame. I prefer to wear it straight but everyone else prefers it curly.

11) I used to dance ballet, jazz and modern dance and still really miss it. Ballet was my favorite.


Here are my questions courtesy of Brittany at Fabulously Faint:

Question #1
If you could only eat one food for the rest of your life what would it be?

Fruit Tarts. Undoubtedly fruit tarts. Anyone who hasn't tried one of these fine delicacies absolutely needs to. I would happily eat them everyday for the rest of my life. Fruit, custard, chocolate. All in one pretty little package. What's not to love?

Question #2
What's the most exciting/adventurous thing you have ever done?
Although I'm about as far from an adrenaline junkie as you can get, I'd say the most exciting thing I've ever done was meeting my favorite band and having a drink with the lead guitarist a few months before I got sick. I could've died happy that night.

Question #3
Describe yourself in three words.
Creative, kind and funny.

Question #4
Who inspires you?
Chronic illness survivors! That includes a lot of strong people. And anyone who stands up for injustice especially in the face of adversity! It takes a lot of strength to do such a thing. There are too many people I admire to name just one!

Question #5
What is your favorite piece of clothing?
My pink peacoat. It personifies me!

Question #6
If you could switch lives with someone for a day who would it be?
Someone who is in perfect health and optimal physical condition. I would take advantage of being in a healthy body for a day, that's for sure!

Question #7
If you could choose any career what would it be?
An interior designer! It wouldn't even feel like work to me, I would likely enjoy every second of it!

Question #8
What is your guilty pleasure?
Reality TV. Shows like Keeping Up With The Kardashians and Dance Moms especially.

Question #9
What is your dream vacation?
Australia. I have always, always wanted to see the Sydney Opera house on New Year's Eve and to hold a koala bear!

Question #10
Who are your style icons?

Anna Nalick. I was at her concert recently and we happened to be wearing the exact same ankle booties! Her style is unpretentious yet pretty and feminine. Timeless, not trendy. She always utilizes vintage or eclectic finds skillfully. She is not flashy but classy and refined and doesn't feel the need to dress trashy.

Question #11
What is your biggest achievement?
Being Valedictorian and voted most likely to succeed in my high school yearbook. It was flattering that my peers had such a favorable opinion of me and nice to be valued for my intellect. Especially these days now that POTS-induced brainfog has seemingly clouded my once sharp intelligence. If nothing else, it's comforting to know I was once a brainiac!

It's EDS Awareness Month!

No, it is NOT erectile dysfunction syndrome awareness month, nor is "EDS" an acronym for that! EDS stands for Ehlers-Danlos Syndrome and May marks a special month of awareness for this life-altering genetic connective tissue disorder which often results in extreme joint hypermobility and frequent dislocations among myriad other symptoms. I did not learn I had this condition until long after my POTS diagnosis. Many people with EDS also suffer from dysautonomia as a secondary condition. My POTS and TMJ are likely both linked to EDS as an underlying cause.

As a former ballet dancer I have always been very flexible. And that was a major attribute growing up. But never realized I had stretchy skin until an orthopedist pointed it out. And more importantly, I never realized that these seemingly harmless traits would predispose me to other serious health issues like POTS. I am very fortunate that my joints seem to be holding up okay so far (knock on wood!) and feel for all the EDS patients who have already had multiple joint replacements at my age or younger. EDS manifests differently in each patient although most of us share those hallmark traits of flexible skin and joints. Some people have very severe cases which can result in disability and even death, and others are plagued by milder cases that don't impose a major impact on daily life.

If you are extremely flexible and suspect you or someone you know may suffer from EDS, please talk to your doctor about it. And consider getting a referral for genetic testing. Diagnosis can lead to more comprehensive, tailored treatment and can help doctors and surgeons determine safer means of treatment. For instance, when I was struck with appendicitis and found myself in an emergency situation, I was able to inform the surgeon and anesthesiologist of my EDS before surgery. They took extra special care of me because of my EDS diagnosis, paying careful attention to the position of my jaw during surgery, as it is prone to dislocations. Knowledge is power. Take ownership of your health to help your healthcare team provide you with the best care possible!

Year in Review

Although I don't write a Christmas letter or anything of the sort, I figure my dear blog readers tune in from time to time throughout the year and are probably all too aware of the health goings-on in my life. So I will try my best to keep this brief and not too negative. It has been one heck of a year to say the least!

Let me start with the good. Certain aspects of my health have improved significantly. My resting heart rate is good! Normally anywhere between 56 and 66 beats per minute on any given day. And there have been many days where my standing heart rate hasn't been so bad either! (70's, 80's, 90's, I rarely reach the 100's anymore!) I am always thrilled to check my blood pressure monitor and see relatively normal numbers there too. It helps me breathe easier when I think about how those same numbers used to look a year ago. Can I say my POTS is gone? Well, no. There are days when my HR still spikes upon standing, I still get the occasional dizzy spell and brain fog seems to plague me on a daily basis. But when I compare my symptoms and my numbers to what they were 5 years ago the difference is dramatic. And there are even some days where I even feel somewhat normal.

In terms of chronic pain and limitations however, this has been the worst year of my life. The TMJD pain is unbearable half the time and it impacts my life severely. I have had to drastically modify my diet to soft foods exclusively and master the art of being quiet which has proven very difficult both emotionally and functionally. It is really tough to not be able to make phone calls to doctors or insurance companies. Not to mention not being able to speak to friends and family very often. I have had to shell out $1300 for a home ultrasound machine like the kind they used on me in Physical Therapy after my insurance company cut off physical therapy completely. It is about the only thing that brought me any real relief. I am currently going through the appeal process with my insurance company about covering the ultrasound machine as durable medical equipment for a chronic condition.

A local neurologist also thinks Botox injections would help quell the muscle spasms in my jaw and facial muscles, but insurance does not want to cover that either because it is not FDA approved for anything other than the treatment of migraines (which I am not having). Although my medical insurance does cover TMJD, they certainly don't seem to cover any of the treatments that have worked for me. So the battle continues. Sadly, this type of battle has become all to familiar to many of us. Having to fight for the basics all the while fighting our own battles against our ailing bodies. My wish for the new year is that less people will suffer and struggle with their insurance companies. That we will not have to fight as hard to receive adequate treatment and relief.

May my friends and readers experience improved health and  relief from chronic conditions in 2013. Keep on fighting the good fight and never never never give up!

Being Human is Hard Sometimes

I have a huge aversion to vampires. I have not seen a single Twilight movie and have a feeling I'm not missing out on much. I don't buy into the whole "vampires are cool" craze. There are just too many darn vampires on TV these days. So I found myself surprised when I could not stop watching the UK version of Being Human. It came highly recommended by a fellow potsy with great taste in television, so I decided to give it a try. Aside from being permanently scarred for life by a few gory scenes, I actually gleaned quite a lot of good out of this show and found myself empathizing with the main characters' struggle to be "normal" humans.

Being Human features a werewolf, a ghost, and yes--a vampire--as roommates living together in Annie the ghost's old house which George the werewolf and Mitchell the vampire rent from Annie's former fiancé (Annie the ghost is invisible to most people). Later on Annie remembers that Owen her despicable sociopath of a fiancé was the one who pushed her down the stairs, resulting in her untimely death. Annie is definitely the character I related to the most. She cannot be seen and heard by most people and leads an isolated life mostly confined to the house. The characters all seem to spend a lot of time at home, however George and Mitchell are out in society working at a nearby hospital and masquerading as human.

As irony would have it the "monsters" and ghost are not the true evil characters on the show. It is the Catholic priest, the misguided professor and the sociopath fiancee (all real humans) who are perhaps the most evil and seem to lack any sort of profound moral compass. Although they make big mistakes, the non-humans are actually good-hearted and consistently try to help people in spite of their own issues.

Here is how I personally relate to each of the main characters:

Annie: I wouldn't be surprised if a lot of POTS and EDS patients easily relate to Annie the ghost. She spends most of her time at home as a quiet observer of the goings-on around her. She is invisible to almost everyone and cannot be heard either. She cannot really "live" her life as she is a ghost and had all her earthly dreams and goals ripped from under her when she was pushed down the stairs. Her afterlife lacked purpose and she is often struggling to find her place in the world. She is arguably the nicest character on the show and genuinely goodhearted and wholly undeserving of what happened to her. She also wears the same outfit in every episode. Comfy clothes and cute Ugg boots.

George: George is a good-hearted werewolf who wants desperately to lead a normal life. He has to hide his secret from friends, coworkers and even tried to hide it from lovers. Although I don't deem his problem to be quite as severe as the vampire or the ghost's considering he only has to deal with spontaneously transforming into a werewolf one night a month during the full moon, it is still a huge disruption to his life. I relate to George because I too transform into a very unpleasant person during the first few days of my period and therefore I try to lay low and avoid people during that time so I don't say something I'll later regret.

Mitchell: Although I find it hard to relate to Mitchell much at all, a part of me does feel sorry for him. Sympathy for a vampire? That doesn't sound like me at all, but it could have something to do with the fact that he is really, really, ridiculously good looking. Although he has some bad relapses, he tries hard to be a good vampire and not feed on humans anymore. Mitchell perhaps most strongly resembles the drug addict the show's writers had originally intended for his character. He also struggles to form meaningful and lasting relationships with the other characters except for George and Annie that is.

The first three seasons of Being Human (only 8 episodes each!) are currently available on Netflix. I highly recommend this show if you like sci-fi or drama. Be prepared to shut your eyes for a few scenes if you have a weak stomach like me. Other than that it's a great show that can teach you a lot about humanity, values, and perhaps most significantly, the struggle to maintain normalcy with an unwanted affliction. Many of us with chronic illness can relate.

The What, Where, How, When and Why: Amino Acids

I have grown increasingly intrigued by oral and injectible amino acid therapy ever since stumbling upon lessflexible.com. Amino acids are complicated and I don't claim to know the first thing about them, but I do wonder if they could help improve the strength of our stretchy connective tissues as they did in one young woman's experience. She claims she is less flexible since starting a carefully tailored and administered amino acid therapy regime with her doctor. I would love to be less flexible, as would most EDS sufferers. Amino acid therapy an area that I believe warrants further research in relationship to connective tissue disorders. I am happy to welcome guest blogger Martina from Gracewell Healthcare who graciously tackled the basics of amino acids. 

Essential Amino Acids	Non-essential amino acids
Histidine	Alanine
Isoleucine	Arginine
Leucine	Asparagine
Lysine	Aspartic Acid
Methionine	Cysteine
Phenylalanine	Glutamic Acid
Threonine	Glutamine
Tryptophan	Glycine
Valine	Proline
	Serine
	Tyrosine

The What, Where, How, When and Why: Amino Acids

This guest guest post was generously contributed by Gracewell Healthcare

What: They Are/The Benefits of Them

Amino acids consist of a mixture of carbon, hydrogen, oxygen and nitrogen. The human body naturally stores twenty of these acids. However, another nine must be obtained from food sources. It is absolutely essential that we have every single one of the amino acids, as they each perform a special function. In general terms they combine to form proteins, which are important for the building and maintenance of muscles and processing of the brain. Amino acids have also been found to play a role in weight reduction.

Where: To Find Them

There are a wide range of foods and drinks that are high in protein. Meats such as pork and beef fall under this category and contain the complete variety of amino acids. Less fattening alternatives such as fish and chicken are also complete amino sources. Those individuals who refuse to consume meat or dairy products can still maintain the protein levels by eating vegetables, nuts and whole grains. Health experts advise the consumption of at least 60g of proteins every single day. Some people may even opt to maintain the healthy diet with supplements.

How: To Consume/Recognise/Identify Them

In scientific terms amino acids are formed from a central carbon atom, to which the Carboxlyic acid, Hydrogen, Amino and variable 'R' groups are attached. Amino acids can join together in the formation of dipeptides and polypeptides. They have fairly complex names such as phenylalanine, which is known as a provider of energy and tryptophan, which can combat sleep deprivation and depression. As previously mentioned, the standard amino acids form part of our natural DNA structure. The essential amino acids must be consumed as food or drink.

When: They Come Into Play/Their Function in the Human Body

A high profile scientist by the name of Dr. Elson Haas has stated that around one fifth of the human body is made up of proteins. They are a major constituent part of the eyes, skin, muscle and brain. Without these proteins human beings wouldn't exist. Apart from keeping us alive the proteins and amino acids that they are made up from play a key role in the processing of messages from the brain and generation of energy. People who are unable to maintain their amino acid levels may experience depression, insomnia and extreme lethargy.

Why: They Are Important

It is essential that humans consume foods and drinks containing amino acids on a daily basis. That's because the body uses them in a variety of means, including the building of organ and muscle tissue and development of hormones such as adrenaline. It is definitely worth taking supplements that include vital aminos such as Threonine, which prevents the onset of illness and disease, and  Leucine, which allows for the processing of vitamins, minerals and protein. Anybody who fears that they may not be consuming enough amino acids or requires assistance on the selection of supplements is advised to see a medical expert. 

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A Country Girl's POTS/EDS Anthem

Ironically, one of my best friends put this Rascal Flatts song on a mix CD for me on my 22nd birthday right before I got sick. Since then, the lyrics have taken on a whole new meaning. I relate to music much differently now than I did before I got sick. That's what life experience and struggle bring to the table. A deeper understanding and appreciation of language and lyrics. 

You feel like a candle in a hurricaneJust like a picture with a broken frameAlone and helpless, like you've lost your fightBut you'll be alright, you'll be alright
‘Cause when push comes to shoveYou taste what you're made ofYou might bend ‘til you break‘Cause it's all you can take
On your knees you look upDecide you've had enoughYou get mad, you get strongWipe your hands, shake it offThen you stand, then you stand
Life's like a novel with the end ripped outThe edge of a canyon with only one way downTake what you're given before it's goneAnd start holdin' on, keep holdin' on
‘Cause when push comes to shoveYou taste what you're made ofYou might bend ‘til you break‘Cause it's all you can take
On your knees you look upDecide you've had enoughYou get mad, you get strongWipe your hands, shake it offThen you stand, yeah, then you stand
Every time you get upAnd get back in the raceOne more small piece of youStarts to fall into place, yeah
‘Cause when push comes to shoveYou taste what you're made ofYou might bend ‘til you break‘Cause it's all you can take
Yeah, then you stand

Perfect Turnout Comes With A Price

As any ballet dancer knows, perfect turnout is considered paramount to being successful in the ballet world. Here is an example of picture perfect turnout:

Now, for many of us with EDS, this position comes naturally. It always did for me. As a child my ballet teacher was often impressed by my innate "perfect turnout" and would use me as an example to demonstrate for the rest of the class. A few of my peers were able to easily emulate this ideal turnout, and others could barely get their feet to point anywhere but straight forward. They were probably the "normal" ones in the bunch. Little did my teacher know that I was simply a genetic freak anomaly who didn't have to work to acquire this turnout at all. I loved ballet. It came pretty naturally and I was able to use my body as a vessel for self-expression. Had I have known that I may have been doing more harm than good to my body though, perhaps I wouldn't have let my teacher work me so hard. Perhaps I wouldn't have danced ballet at all actually.



Everytime I watch Dance Moms and see Brooke Hyland engage in her picturesque contortions I can't help but wonder if she has EDS and want to warn her to slow down now because her body will thank her for it later. I worry that any child who exhibits extreme hypermobility may be at risk for extreme pain, suffering and even surgeries later in life. Especially if the hypermobility is encouraged by demanding teachers (like Abby Lee Miller!) or even parents who don't realize the potential consequences of overstretching their children.



Perhaps someday the medical population will realize that EDS is much more common than the literature recognizes and start testing for hypermobility routinely at physicals. They should definitely start screening for it at ballet schools, gymnastics gyms, etc. If nothing else, early screening may enable parents to help their kids protect their joints through good practices and bracing at an earlier age, thereby prolonging the life of a problematic joint.

Instead it is often considered cool to share these party tricks with others. In fact, our culture values hypermobility on display. Everytime I watched street performer and self-described extreme contortionist hiphop dancer "Turf" do his thing on America's Got Talent I couldn't help but root for him. Not only was he a likable guy, it also seems likely that he will endure some medical problems down the road as a result of his dancing now.


Disclaimer: It probably goes without saying, but just in case you're new to this blog I should mention that I am not a licensed physician and therefore not qualified to make medical diagnoses for any of the aforementioned individuals based on what I have seen them do on TV. They may or may not have ehlers-danlos syndrome.

From Worrier to Warrior

Worrier: A person who torments oneself with or suffers from disturbing thoughts, cares, anxieties; one who frets.

Warrior: A person who shows or has shown great vigor, courage or aggressiveness; soldier.

Seems it has been awhile since I posted a general health update on all the random happenings of my crazy body. And a lot has been happening. The year was off to a crappy start with an emergency appendectomy that seemed to set the tone for the rest of the health craziness that would ensue. I was just relieved I awoke from surgery and that it had been a completely routine procedure free from any major complications. I had some MAJOR tummy troubles before (probably just my appendix going bad) and after the appendectomy but I saw a GI doc who gave me some medication to take briefly that seemed to do the trick and I am now on a once-daily acid-reflux medicine called Dexilant. It is a great drug. Not quite strong enough to knock out all of the acid when I eat junk foods, but on the days I forget to take it I notice what a big difference it is making. And unlike Prilosec which I had a hard time remembering to take twice daily, I only have to take Dexilant once daily (and believe me, that's hard enough to remember!) The caveat? It is an EXPENSIVE drug. Luckily I am now enrolled in a prescription discount program sponsored by the manufacturer so I get a 30-day supply for $20 instead of over $200. I hate the fact that I am now on a daily prescription as I try not to take anything since my body is hyper-sensitive to meds. Luckily I haven't experienced any noticeable side effects from the Dexilant so I will continue my daily regime in addition to improving my dairy-free diet which I am hoping to gradually make more alkaline in the near future.

For several weeks after the appendix surgery I was worried I might be experiencing gastroparesis symptoms as so many other potsies do, but my ravenous appetite has now returned and so I am convinced it was just acid reflux coupled with a little post-surgical constipation. Although I did not take any narcotic pain medicine after surgery, apparently constipation is almost a given side effect of any abdominal surgery even among the general population. I even received my first (and hopefully last ever) enema in the ER this year a few weeks post surgery. Talk about uncomfortable. However it was also comforting to discover that I am not the only one who has had to resort to such extreme and unpleasant measures after abdominal surgery, for once I was dealing with a COMMON problem. Which was refreshing for a change. And my scars are tiny and healed very quickly. They are barely noticeable anymore.

Speaking of skin, next month I have to see the dermatologist to get a "suspicious" looking toe mole biopsied. I knew all those years of daily flip-flop wearing would eventually catch up with my feet. No one ever puts sunscreen on their toes. It is an overlooked area of the body. I also have a few other spots they will probably want to biopsy since I have a family history of skin cancer. I doubt it is anything serious, but my insurance deductible finally having been met dictates that it's time to knock that appointment out of the way. Last Valentine's Day my mom had a basal-call carcinoma removed (successfully) from her face and that served as a big warning sign that it's time to pay more attention to my skin. My grandpa is also currently undergoing various skin cancer treatments including a new laser-light treatment to remove pre-cancerous growths all over his head. I am really hoping that this new treatment will clear it up for him because it's a painless, non-invasive approach and he has undergone painful removal procedures several times already.

Truth be told, random but important appointments like the dermatologist visit have taken low priority these past few months as I've been struggling with the worst pain of my life from intense TMJD headaches. I seem to be stuck in the bargaining stage of grief where I can't help but wish it were any other joint affected but my TMJ. That is the one I need to use the most in order to speak, feed myself, function. It is so hard to get by in the world without talking. People take that ability for granted everyday. I know I did. This pain really plays head-games with me since it is worst after speaking or eating (basically the two things I live for). It feels like my body is punishing me anytime I try to be myself and reclaim my lost identity by making a phone call or eating a burger (with a knife and a fork, swallowing the bites whole). My jaw goes out and the pain sets in. Punishment for doing the things I enjoy. Heck, just for doing the things that are necessary to sustain life. A food-loving girl can only survive off of smoothies and soy yogurt for so long. And I tend to lose all my marbles when I'm starving and not eating the kinds of foods that my body demands. Throw in days upon weeks upon months of pain-induced sleep deprivation and it's a recipe for disaster and dysfunction. Just getting through each day has proved to be quite a challenge the past few months and the pain has reached a point where the intense menstrual cramps I am accustomed to getting every month pale in comparison (although they have not changed in intensity at all). In fact, if anything my periods have been getting progressively more painful over time too (will deal with that one later as a trip to the gynecologist is not high on my priority list at the moment either). It's just that I know from past experience that my period represents a temporary state of pain, which makes enduring it much more bearable. There is an end in sight. TMJD pain has been discouraging because I seem to get the pain episodically but unfortunately these flare-up episodes last for months on end with no apparent rhyme or reason and I never know when the pain will finally cease. If the smell of BenGay didn't redden and burn my eyes so badly I would be rubbing it all over my face, head and neck on a daily basis.

Needless to say I have been "doctoring" for my TMJ issues again out of sheer desperation and have finally stumbled upon a few knowledgeable practitioners who didn't dismiss my pain with a prescription for valium and a box of tissues (as my gem of a PCP did at the beginning of this flare-up). I am now seeing a physical medicine doctor who specializes in pain management. He has a solid understanding of the complexities of the musculoskeletal system and has seen a few other EDS patients as well.

I am also seeing a physical therapist who has worked with Dr. Tinkle in treating his EDS patients. My jaw must have dropped open in disbelief when he not only knew what EDS was but had successfully treated it before. I will be attending physical therapy sessions with him 2-3 times weekly and receive an ultrasound treatment to each TMJ which so far has helped immensely considering I've only had two sessions. I'm optimistic that ultrasound therapy will provide some relief as my pain seems to be muscular in nature. I am also undergoing the needles in acupuncture again twice weekly for now but hoping to wean off to once weekly soon.

I gave massage another go last week with a knowledgeable practitioner who had actually had a cervical fusion surgery a few years back. I was also impressed with how well she knew the body but I am going to hold off on massage a bit longer as I think I'm doing too much right now and the sheer number of appointments every week alone is exhausting. Massage is supposed to be relaxing but I'm too stressed out by all these appointments to fully enjoy it. Once I wean off the acupuncture I would like to incorporate massage into my weekly regime. Thank god I had that appendectomy and my insurance is finally kicking in to cover these treatments (until the end of the year, that is!) I am dreading the day January 1st rolls around when I will have another deductible to meet before I can receive treatment of any kind.

Oh, also one more totally random finding worth mentioning that I probably haven't shared yet. This year so far I have had multiple x-rays (which revealed nothing except for constipation), two or three abdominal CT scans (found appendicitis), one head CT scan (they found a sinus infection on that one, told them I didn't need a CT to know that...) and an MRI of my TMJ's. I am supposed to get a full upright MRI of my head, neck and spine to check for an acquired chiari malformation and all related issues but I have decided to wait until after my birthday to do that because I'm not sure if I'm emotionally equipped to deal with another bad test result right now. Earlier this year my doctor decided to order an ultrasound of my neck to examine the lump in my throat I felt upon swallowing (which reminds me I have not yet scheduled the endoscopy I am supposed to get either. TOO MANY TESTS!) Anyway I did not expect them to find anything at all on the neck ultrasound. Figured it would just be another futile test, but to my chagrin they did find a small thyroid tumor. At 3 mm it is still too small to needle biopsy so it is just something I will have to have monitored yearly to make sure it doesn't grow any bigger. If it does then I will have to have a biopsy. But for now it is not dangerous and highly unlikely that it is any type of cancer. The endocrinologist I saw seemed pretty confident that thyroid tumors are an extremely common incidental finding and most of them are not harmful. Nonetheless it was and still is difficult for a worrier like me to forget that it is there...

Which brings me to my closing thoughts. The chronically ill are a very special population. We are not just worriers. We are also warriors. Can we be both simultaneously? Yes, although the two concepts would seem at odds to most people. We assume both roles everyday without even realizing it. Being a warrior is hard work. Not worrying about our health is even harder. I cannot imagine any of my "normal" friends or relatives enduring the endless medical tests and treatments we have. It takes persistence. Patience. Mental and physical fortitude. In between doctors' appointments, we often forget to live. Or at least I do. My goals have gone from hosting my own talkshow to simply making it to my next doctor's appointment on time. But does it really matter if I am on time? Or even in one piece when I arrive? Not so much in the grand scheme of things. I have accepted the fact that I am imperfect. All the labels and diagnoses mean very little to me anymore. I just want to be happy and pain-free, warts and all. And to transform my inner worrier into a proud warrior for good. Just like the musically-gifted Mr. Mraz has done...

Adrienne McGuire's EDS Story

I am thrilled to welcome an amazing guest poster to Defying Gravity, Adrienne McGuire. Check out Adrienne's other compelling reads at Daily Path.

I’m 37 years old and was diagnosed with Ehlers-Danlos Syndrome Type III just last year. I have two children who are both very active and require a lot of physical and emotional attention, which can be a real challenge when you’re suffering, which I have been for most of their lives. I saw many different doctors over the years, all of whom proclaimed that they could find no reason for my pain. Some chalked it up to anxiety; others called it fibromyalgia. But I knew there was something else going on.

Hindsight is 20/20; isn’t that what they always say? Looking back now, I can see that my EDS and POTS problems started when I was young with several fainting spells and a distinct inability to withstand any amount of heat without passing out or nearly passing out. I had horrible shoulder and back pain as young as 13 and have ever since. My neck has been in and out of spasm since age 20. At age 22 my entire body began to twitch, which I now know was from overuse. By 25, my legs were constantly aching so badly I was in tears daily. And that was eleven years before my diagnosis.

Last year I had to say goodbye to the old me and say hello to the new me with limitations when a rheumatologist told me I have the hypermobile form of EDS along with a case of POTS that fluctuates with hormones, as do my other EDS symptoms. As I educated myself about the disorder, I realized that the only way to control the symptoms was to slow down. I had to explain to my children that I can’t do all of the physically active things that we used to do, and that was really tough. I mourned my lost abilities to run, hike, carry my kids, and play on playgrounds. I had a mental break and ended up in the emergency room several times. That was 9 months ago. Today, I am moving forward.

Though I lost some abilities, I gained so much in the way of mental strength and focus. I could no longer work as a legal assistant but I became insanely determined to create a life for myself that worked. By pacing, therapy (both physical and mental), trial and error to find effective medications and supplements, meditation, and learning how to ask for help, I have managed to look at my life with a positive spin. I now wear silver ring splints all the time and other supports as needed for wrists, knees, elbows and neck. I take medications that control my POTS symptoms which means I can get out more, albeit in short bursts. I recently talked to my doctor about getting a wheelchair for long outings. But, overall, when I look at my life now, with a disability, I am so much happier and grounded than I ever was before.

Adrienne McGuire is a writer, website consultant and wellness enthusiast who abandoned the corporate ladder to create a life that worked for her.  Her journey down the road less traveled took her to www.dailypath.com, where she is now an integral part of the writing team.