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!

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

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.

Should People With Chronic Illness Become Pet Owners?

A big thanks to guest blogger Sandra Scott of Simple Living for weighing in on an important topic I have been pondering for quite sometime myself. Although I don't have a dog or cat yet, it is definitely something I'd like to do within the year.

So you want to become a pet owner. The rewards almost always outweigh the amount of work and the risk that your new furry companion may ruin your favorite shoes, rug, or sofa. However, there are a few important factors to consider before committing your time, energy, and income to a new pet.

For starters, consider your personal energy level. If you suffer from a chronic illness, you may also experience pain or debilitating fatigue at times. Would you be able to walk a rambunctious dog everyday? Or would a calm and cuddly feline companion be more suited to your unique needs? Also consider the potentially negative effects of pet dander and fur on your health. As long as you are able to keep your house and pets clean, pet dander probably won’t pose a significant threat to your health (unless you are asthma and allergy prone). If you do happen to be dander-sensitive, consider a cute turtle or even a beautiful array of tropical fish.

Every pet is different, and some require more maintenance and attention than others, but every pet is worth it in their own special way. If you find it difficult to meet your dog’s grooming needs, there are plenty of places to take them in for doggy beauty treatments fairly affordably. That said, it is still important to factor your pet’s potential grooming and veterinary needs into the monthly budget along with their food to determine whether or not you can really afford an animal.

Although experts can’t seem to agree on exactly why this phenomenon occurs, it has been proven that pet owners enjoy better overall health and longevity than their petless peers. I would like to think it has something to do with the power of unconditional love and companionship. Even when your health fails, your pet will remain a loyal companion by your side no matter what. They expect love and attention in return, but as long as you are able to adequately meet the basic needs of your pet (food, water, clean shelter, and some exercise) then getting a creature companion may be a feasible and wonderful option, and may even serve to improve your overall health. Or at the very least, improve your mood. Who wouldn’t love to see a wagging tail and happy grin every time they’re feeling blue and under the weather? As far as I’m concerned, there’s no better way to brighten a day.

Milkshakes and Muscle Relaxants

That about sums up my day...not as fun and 'relaxing' as one might assume by the title. Although some new health problems have cropped up this year, I felt like I finally had a handle on my intense TMJ headaches. In fact, I thought I was out of the woods in that department. No such luck. I haven't had a doozy of a headache like this since last year!

Not sure why I have one right now...except for the fact that I am almost positive that some higher power is playing a sick cosmic joke on me...it sure seems like I'm being punished for doing the things I love most: eating and talking. Which I have to refrain from entirely when I get a TMJ headache like this. And forget trying to have a social life or be successful at any sort of job. As if POTS weren't enough to deal with on it's own...thanks Universe for being such a sadistic jerk. One major, debilitating health problem is more than enough to deal with and already more than I can handle.



There's no real rhyme or reason to what brought this headache on (no excessive talking or chewing lately) but it started around the same time last year and lasted for MONTHS. Last year I saw a neurologist who suggested a misdiagnosis of 'new daily persistent headache syndrome' without so much as feeling the giant muscle knots that feel like marble-sized cysts sitting right beneath my scalp. My mom can feel them, my friend can feel them, I can feel them. All with our bare, untrained hands. It's creepy. Nobody else has muscle knots like that behind their ears and on the sides of their heads. These knots are movable, get bigger and smaller, and are somewhat relieved by massage. Feels like awful menstrual cramps in my head. When someone presses directly on a knot I get goosebumps on my arms from the intensity of the pain. And that's saying a lot considering I have a high pain tolerance (I didn't even take any pain meds after returning home from my appendectomy).

The amount of vigorous massage it would take to break these suckers up is unbelievable. And frankly the flexeril has done nothing but relax all my other muscles and not touch the ones on the sides of my head. My primary care provider informed me that there is really "no good muscle relaxant for those small muscle groups." Fan-freaking-tastic. The only thing I have yet to try to remedy these awful muscle cramps is botox.

However once I had gotten my headaches under control I didn't think I would have to deal with this intense pain ever again. I should have known better. I am afraid that if I go in and get botox in these muscles now that it may freeze them in their knotted position and leave me in pain for the three months that the botox lasts. No doctor seems to know much at all about botox for TMJ and they sure as heck do not know how botox works in an EDS patient.

To say I'm annoyed right now would be an extreme understatement. Narcotic painkillers don't even touch this and the muscle relaxants don't do much either. Wish I knew someone who was confident enough to try to put my jaw back into place or break up the muscle knots somehow. Desperation is setting in. I am scared that it is going to be a repeat of last year with a months-long headache. The majority of my spring and summer last year was spent in excruciating pain thanks to these stupid muscle knots. Is this common in EDS? Why do I only get them around my jaw? And most importantly, how do I get rid of them? Any suggestions?

New Daily Persistent Headache Syndrome

Yesterday I saw the headache neurologist I have been waiting patiently in pain for over two months to see. He gave me about 15 minutes of his time, asked me a set of very cut and dried questions and cut off most of my answers before I could finish a complete sentence. Luckily my mom was with me and was able to tactfully yet forcefully interrupt him by saying "can I ask you a question?" and got a few questions answered that way. I know a lot of doctors suffer from what I will term "no bedside-manner syndrome" but this guy takes the cake. He was completely deficient in people skills. I was completely unsatisfied with my appointment I had waited so long for. And even more disappointed that my follow-up appointment is another seven weeks away. He wants me to chart my headache pain level on a scale of 1-4 (4 being debilitating pain) until my next appointment and then he will decide what medication to try me on. Gee, sure would have been nice if the office gal would have asked me to chart my symptoms over two months ago when I made my appointment and I would have had plenty of "data" for him and would not have to suffer for another 7 weeks without any medicine to offer relief.

I left his office feeling a little defeated and also feeling like this guy didn't even let me tell him half my symptoms. He told me to go home and google new daily persistent headache syndrome, he said he is almost positive that I have that, but wanted me to read up on it and let him know if my symptoms matched or not or if I disagreed with his diagnosis. Which I absolutely do. Not only are the daily persistent headaches characterized by "mild to moderate bilateral pain" (hello, mild to moderate pain doesn't even phase me, but the intense pain that literally gives me goosebumps and produces muscle knots the size of ping pong balls behind my temples certainly does) they are also not known to have a specific cause. I can pinpoint the day the headaches started and a triggering event. I'm sure it's no coincidence that I've had horrible headaches centered around my jaws since September of '07 after that fateful impacted wisdom teeth extraction when the oral surgeon informed me that I would probably end up with a bad case of TMJ after the surgery (my car accident 3 weeks later didn't help either). Of course, the surgeon neglected to inform me I would end up with dysautonomia, but who could have ever predicted that. It gives me the shudders whenever a friend or relative says they are going to get their wisdom teeth pulled. I wish I would have never, ever had mine out as I blame both my TMJ and POTS on that stupid surgery. I went in young and healthy, and came out a few hours later in the body of an 82-year old woman.

Unfortunately I think yesterday was a waste of an appointment. A bullshit diagnosis that my pre-med friends in college could have more accurately diagnosed. And even more unfortunately for me, that leaves me back with the same old TMJ diagnosis which is not only a very stubborn pain that's hard to knock out, it's also a condition most medical insurance companies will not cover at all. Most dental insurance companies will cover a little bit, typically $500 a year with a $5000 lifetime maximum. Which is hardly enough to cover the cost of one nightguard let alone more effective but costly treatments like botox or even surgery.

I am sure new daily persistent headache syndrome is a very real condition with very real sufferers. I just don't think I am one of them. I would be willing to bet money that that's one condition I don't even have. It almost seems futile to go to most doctors anymore when my POTS friends are so much smarter and offer better advice. I'm talking to you here, Claudia. Thanks to all my friends in the dysautonomia community for being both smart and supportive. Too bad all of us aren't doctors, we'd be a heck of a lot better at it than the ones practicing now.