Love, Life, and Chronic Disorders

Life, love, relationships… It’s all difficult enough without adding stress. That stress can come in any form: job loss, money trouble, stress at school, stress at work, kids… These are all stresses that people face regularly. Toss in a flu here, strep there, and you have life! My guy and I were fully prepared to face these types of stresses together! No problem… Easy peasy.

For M and I, life has been a little bit different… From car accidents, to sickness; from root canals to surgery, we have packed in plenty of stress in our almost 6 years together! We have seriously “done the time”. Yet, we know that there is more to come. We have hurt, we have yelled, and we have laughed. Most importantly, we have loved. We have learned. And we are ready for just about anything!

When someone has a chronic illness, or illness of any kind, really, sometimes the face of the sick is the only thing we see. But it is so important not to forget about those ever so important and supporting partners on the other side of illness. Often times, these partners are the bread winners, sometimes they are equal partners, and in some instances, they are equal sufferers. No matter the case, dealing with a chronic illness takes a serious toll on a relationship.

Although M and I are learning to cope and deal with each situation one day at a time, it wasn’t always this way. For a long time, it was tense. Neither of us understood what was wrong with me. I thought I was crazy; he thought I was crazy. I ended up paranoid that everyone could see the “crazy” written on my face! I was constantly tired or nauseous, and although this was tough for me, M started to become frustrated that I was no longer the active Cheerleader and fun-loving girl that he met. I’m sure he was convinced that I was depressed or just anxious… I know it crossed MY mind more than once. All I wanted M to do was understand! If he would just understand how poorly I was truly feeling!

He not only knew how I was feeling, he was freaked out because he knew I wasn’t okay. No… He didn’t know if I was really sick or really crazy, but he knew I wasn’t okay. And being the “fixer” that my husband is, he was angry that he couldn’t just make it better for me. Being selfish and completely focused on myself, I never even considered that M might be worried or frustrated or scared. I thought he was being a jerk. After one fateful appointment, during which Brandi (mentioned previously as my godsend) mentioned that there could be something going on with my heart, I delivered the news and my frustration to M and that was when I saw it for the first time: He was so not okay with what was going on. Things were stressful and tense while we waited through this test and that test to finally receive a diagnosis, and our relationship was changing. Being just engaged, I knew that if he could stick by me through all of this now, we could make it through just about anything! Finally, after I received my POTS diagnosis, we were married and ready to take on the world. Through our first year of marriage, we faced enough of a challenge in adjusting to just being married! After struggling and all the tension, my POTS seemed to finally be under control.

Then… It started…

The twitches, the spasms, the pain…

It didn’t all start suddenly, or out of nowhere. It was all very gradual. Some days, I would have a spasm in my arm that would last a while, but didn’t seem to be bothering anything. I figured… People spasm sometimes. Muscles work hard all day long. Sometimes my eye would twitch, but I figured my eyes were tired; after all, I never wear my glasses like I’m supposed to and I’m on the computer a lot. As the days and weeks went by, the twitches and spasms would last longer and longer and I would feel what I can only best describe as “bone pain”. I’ll admit that I’m not the best communicator, especially when it might lead to my vulnerability or show my weakness. So, when I decided to alert M of my new symptoms, I waited until my arm was in the middle of a pretty intense spasm and tried to make light if it, even though it had been going on all day and I was seriously freaked out. I simply said, “haha… Check that out! Been doing it all day. THAT can’t be good! Haha!” So, I don’t know why I was so surprised when M replied with, “huh… Weird” and then turned back to his T.V. show. And with the pain, I wasn’t much better in my communication. So, I don’t know why I was so surprised when M looked at me and said, “it can’t be that bad”. I had dug my grave. If I told him the harrowing truth now… That I wanted to scream with pain when I woke up every morning, and that my twitches were intense and making it difficult for me to do certain things, of course he was going to think I was crazy! Finally, I told him I was going to the doctor. It should be of no shock that he responded out of anger. “Why didn’t you tell me it was that bad!?” I was preparing to go on a trip to visit a friend… “If it’s really that bad, maybe you shouldn’t go tomorrow.” I responded with anger right back. I saw this as a challenge. I went anyway. Even though it was a wonderful trip, in hindsight, maybe I shouldn’t have gone. I’ll never know. All I know is that I wasn’t fair to M.

I think that, a lot of the time, when a person is sick, that person expects his or her partner to just deal with it quietly, while sitting on the sidelines. I think we tend to push our partners away, by trying to make light of the situation instead of just being real about it all; being vulnerable and honest that we really are kind of terrified. For those of us who are incredibly lucky, our partners stick around anyway and MAKE us communicate. As a lucky one, my M has not only done this, but he has found a way to incorporate this part of my life into his; he has learned new ways of reading me. Now, he can tell just by looking at me if I’m having what we zebras call a “painy” day or a “POTSy” day. Even still, it’s not always easy. Even still, I live in the land of denial and swear that there is a lighted, flashing, glittery sign on my head reading “crazy” and show girls in straight jackets doing the can-can next to it. The simple fact that I have thought this image up might be disturbing in itself, but I hope you enjoyed it!

Well… This brings me to the end of our 6th encounter. I hope I have enlightened you all a little bit, but I will admit that I have a limited perspective, for I can only report what I have experienced from my perspective. If you want to know everything… You will have to talk to M.

Zebra love and hugs to all!

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Types and My Zebra Friends!

As I sat here today, pondering exactly what I would write about, I realized the importance of communicating with all of you who are reading my posts, exactly what goes in to the typing of EDS once a diagnosis is received. However, I feel that it is also important (as an advocate of my fellow zebras) to inform you of all of the possible co-morbidities that these friends of mine are afflicted with daily. At the very beginning of our correspondence, I wrote of POTS and my struggles with it. But I want it to be clear, that although it is difficult, my “suffering” pales in comparison with so many! 

But first, let us discuss the types of EDS. Bear with me, as I am going to be throwing a lot of information your way. Also keep in mind that it is more than possible for an EDS patient to be “typed” with more than one form of EDS. 

*For anyone who might be curious, I am getting all of this information from the Ehlers-Danlos Syndrome Network: ehlersdanlosnetwork.org

Classical Type (Previously I and II): “The Classical Type of EDS  is characterized by highly elastic, soft, and doughy skin; unusual scarring; and loose joints.  This type of Ehlers-Danlos Syndrome combines the types formerly called I and II.  Ehlers-Danlos Syndrome, Classical Type is a subtype of Ehlers-Danlos Syndrome.”

– “People with the Classical Type have smooth, velvety skin that is stretchy, fragile, and easily bruised. Wounds often split open with little bleeding, heal slowly, and leave characteristic thin, wide scars (“cigarette paper” scars). People with this condition also have loose joints with an unusually large range of movement (hypermobility). As a result, joints are prone to dislocation, sprains, and the early-onset arthritis. Non-cancerous fibrous growths on pressure points (such as elbows) and fatty growths on the forearms and shins are also common.  Other manifestations include weak muscle tone in infants due to hypermobility, which can make them seem “floppy” and delay the development of motor skills such as sitting, standing, and walking. As many as half of the people with Classical Type Ehlers-Danlos Syndrome have a condition called mitral valve prolapse, which affects blood flow between the chambers of the heart.” 

– This is the most common form of EDS and is typically inherited. 

Hypermobility Type (Previously Type III): I don’t feel as though I need to expand on this type too much, as I have provided the most information on this, using my own story, but I will give you the explanation as described by the Ehlers-Danlos Syndrome Network: “The Hypermobility Type of EDS is characterized by loose joints and chronic joint pain. This form of Ehlers-Danlos Syndrome was formerly called type III.  Ehlers-Danlos Syndrome, Hypermobility Type is a subtype of Ehlers-Danlos Syndrome.”

– You will remember that one of the key indicators of this type, is hyper mobile joints, but it is not necessarily the first symptom to rear its ugly head. 

Vascular Type (Previously Type IV): “The Vascular Type of EDS is characterized by possible arterial or organ rupture as a result of spontaneous rupture of vessels or organs due to the result of even minor trauma.  The Vascular Type of EDS is the most serious form of Ehlers-Danlos Syndrome.”

– “People with the disorder have thin, fragile skin that bruises easily. Veins are visible beneath the skin, particularly on the chest and abdomen, and hands and feet may have an aged appearance. Unlike people with other forms of Ehlers-Danlos Syndrome, people with the Vascular Type have skin that is soft but not overly stretchy. Facial features are often distinctive, including protruding eyes, a thin nose and lips, sunken cheeks, and a small chin.”

– …”tearing of tendons and muscles, painfully swollen veins in the legs, lung collapse, and slow wound healing following injury or surgery. Infants with the condition may be born with hip dislocations and a foot disorder called clubfoot, which causes the foot to turn inward and downward.” 

– Spontaneous rupture of internal organs is a very real possibility for people with Vascular type Ehlers-Danlos Syndrome.

– This is a very tough form to diagnose, but is the most dangerous of all of the types of EDS.

– As you read, you may notice that there are symptoms and criteria that span over several types of EDS. 

– Hypermobility of joints IS a symptom that coincides with the Vascular type of EDS. 

Okay… are you getting bored yet? Stick with me. There are a few more types to go through. I understand that it is a lot of information to take in all at once, but it is very important to me to share the plight of my friends! 


Kyphoscoliosis Type: “The Kyphoscoliosis Type of EDS is characterized by generalized joint laxity and severe muscle hypotonia (weak muscle tone) at birth.  The muscular hypotonia can be very pronounced and leads to delayed gross motor development.  Individuals with the Kyphoscoliosis Type of EDS present with a progressive form of scoliosis at birth. The phenotype is most often severe, frequently resulting in the loss of ambulation in the second or third decade. Scleral fragility may lead to rupture of the ocular globe after minor trauma.”

– “Tissue fragility including atrophic scars and easy bruising. Spontaneous arterial rupture can occur. 


Other Findings May Include
  • Marfanoid Habitus (Marfan like features)
  • Micro Cornea (abnormally small cornea)
  • Radiologically Considerable Osteopenia (diminished amount of bone tissue)

The Kyphoscoliosis Type of EDS is the result of a deficiency of lysylhydroxylase (PLOD), which is a collagen-modifying enzyme. This type of EDS is inherited in an autosomal recessive manner. The Kyphoscoliosis Type of EDS can be diagnosed through a urine test.”
 
I confess that I have much less knowledge, myself, about these last few types of EDS, so I have no personal input in regards to each one, but it makes them NO less important than any of the others! 
 

Arthrochalasia Type:  “The Arthrochalasia Type of EDS is characterized by congenital hip dislocation which is present in all biochemically proven individuals with this type of EDS. 


Severe generalized joint hypermobility with recurrent subluxations are seen in individuals with this type of EDS. 

Other manifestations of this type may include
  • Skin hyperextensibility with easy bruising
  • Tissue fragility including atrophic scars
  • Muscle Hypotonia
  • Kyphoscoliosis
  • Radiologically Mild Osteopenia. 

The Arthrochalasia Type od EDS is caused by mutations leading to deficient processing of the amino-terminal end of proa1(I) [type A] or proa2(I)[type B] chains of collagen type I. It is inherited in an autosomal dominant manner. A skin biopsy can also diagnose this type of EDS.” 
 
Dermatosparaxis Type: The Dermatosparaxis Type of EDS is characterized by severe skin fragility and substantial bruising. Wound healing is not impaired and the scars are not atrophic. The skin texture is soft and doughy. Sagging, redundant skin is evident. The redundancy of facial skin results in an appearance resembling cutis laxa. Large hernias(umbilical, inguinal) may also be seen. The number of patients reported with this type of EDS is small.


The Dermatosparaxis Type of EDS is caused by a deficiency of procollagenI N-terminal peptidase. It is inherited in a autosomal recessive manner. A skin biopsy can diagnose this type of EDS. “
 
One more, folks… stick with me! 
 

Tenascin-X Deficient Type: The Tenascin-X Deficient Type of EDS is characterized by

joint hypermobility, hyperelastic skin, and fragile tissue. Lacking multiple shrinking (atrophied) scars in the skin that is often seen in the Classic Type of Ehlers-Danlos Syndrome. Inherited as an autosomal recessive genetic trait (not seen in family members or only in one generation of members of the same family).”
 
Okay… we have covered them all! Unfortunately, I must admit that a simple definition and diagnosis criteria goes nowhere near really explaining each type of EDS. To really understand the gravity of each situation, one would have to speak with a sufferer. 
 
Moving on… 😉 
 
 
In the past few months, I have been so lucky to make some new friends! These are friends who I can really open up to about my troubles with my EDS, POTS, pain, just about anything really! Of course I do still talk to my husband about it, and he is ever so aware of the realities of what I (really, we) deal with. However, there comes a point of frustration (on both our parts) when he can’t REALLY understand me. This is also a reality. He can sympathize… sure! But what really counts, is that he doesn’t really know how I feel. So I have turned to my support group of wonderful friends and fellow zebras. These ladies (and a few gentlemen) are wonderful, caring, compassionate, smart, and frustrated people! Collectively, we can joke about our illnesses and talk about just about anything in life, but at the end of the day, we are still sick; at the end of the day, someone needs surgery; at the end of the day, someone is having seizures, or starting another round of chemo or radiation, or physical therapy. What’s really sobering at the end of the day, is that there are so many mothers and fathers who share this with their children. I can’t imagine. To have an entire family in pain… I’m sure that they share special bonds, but I can’t imagine how I would feel if I had to watch my husband suffer like I do. 
 
Personally, my additional health issues include: POTS, Raynaud’s, IBS, GERD, Reflux, Dysfunctional (or Dry)  Vocal Chords (though I still try to sing), asthma, and non-celiac gluten sensitivity (I am gluten free). 
 
This may seem like a lot, but the fact that I have learned to just live with it aside, what I struggle with is light compared to some of my friends. 
 
Here is a list of just a few of the illnesses that they suffer from in addition to EDS:
 
-**Chiari Malformation: (from the Mayo Clinic) “Chiari malformation (kee-AHR-ee mal-for-MAY-shun) is a condition in which brain tissue protrudes into your spinal canal. It occurs when part of your skull is abnormally small or misshapen, pressing on your brain and forcing it downward. Chiari malformation is uncommon, but improved imaging tests have led to more frequent diagnoses.” 
 
**there are subtypes of Chiari Malformation not listed in this post
 
– Multiple Sclerosis (MS): (from PubMed Health) “Multiple sclerosis is an autoimmune disease that affects the brain and spinal cord (central nervous system).” >>> This is a VERY basic definition of a very debilitating disease. 
 
– Marfan’s Syndrome:(from the National Marfan Foundation) “Marfan syndrome is a disorder of the connective tissue.  Connective tissue holds all parts of the body together and helps control how the body grows.  Because connective tissue is found throughout the body, Marfan syndrome features can occur in many different parts of the body. 

Marfan syndrome features are most often found in the heart, blood vessels, bones, joints, and eyes. Sometimes the lungs and skin are also affected.  Marfan syndrome does not affect intelligence.” >>>Olympic Gold Medalist Michael Phelps suffers from Marfan’s. 

– Fibromyalgia: (from the Mayo Clinic) “Fibromyalgia is a disorder characterized by widespread musculoskeletal pain accompanied by fatigue, sleep, memory and mood issues. Researchers believe that fibromyalgia amplifies painful sensations by affecting the way your brain processes pain signals.” 

These are just a few of the additional disorders and diseases that my friends suffer from. One of my friends has had to have surgery on her abdomen and GI tract, because of extreme malfunction; another friend has a tumor that is slowly (or perhaps not so slowly) killing her…

This disorder may be tougher to deal with some days more than others, but I still have to count myself blessed when I get to be at home in my own bed at the end of every night. My friends are brave, strong, tenacious, incredible… I could go on. Mostly… they are MY heroes! 

I love my Zebras… 🙂 

 

 

A Day in the Striped life of a Zebra

As you read my title for this post, I’m sure you thought, “what in the world!?” well… Allow me to explain. 

Have you ever heard of Occam’s Razor? This is a principal in the medical field, which states that the simplest or most obvious explanation is almost always the correct one. Another way “they” put it is: just because you hear hoof beats, it doesn’t mean “zebra”… Most likely it’s going to be a horse. Makes sense, right? Wrong.

For a person with EDS, it is assumptions like these that can be dangerous. We ARE the zebras. We live our lives in the realm of “that can’t be true.” A few days ago, I posted about the details of POTS and EDS, and I included a slideshow of me doing what we call “bendy tricks”. 

Sure… Some of that was pretty interesting to look at, but in all reality, it was painful. I finished my personal little photo shoot, posted my blog and then found myself completely spent the rest of the day. Thanks to my provocation, I found myself unable to perform even the simplest of tasks without the interruption of arthrytic like pain. Even typing or lifting a glass to take a drink was met with great struggle. Although this instance of pain can be blamed on me completely, there are more days out of the week that I wake up to this kind of pain than not. 

Here’s an example for you… 

Yesterday, I was going to post about EDS. Unfortunately, I woke up with some pain, but then, as I was putting some lotion on my hands, I dislocated a pinky. Don’t worry! I was eventually able to remedy the situation, and honestly… It was such a tiny pain. It was just so out of nowhere! 

Now, let me explain to you how my journey has lead me to a place of waiting for an official EDS diagnosis… 

 As long as I can remember, I have been both “flexible” and “double-jointed”. Why the “”? Well… There wasn’t a very large part of my life during which my actual muscles were flexible, and I know this now. Also… Apparently there is really no such thing as being “double jointed”. I have come to learn that this is something that doctors will tell patients when they just aren’t sure what to tell the patient or how to explain the phenomenon. However, when your bones, both voluntarily and involuntarily, are going in and out of “place”, there is a problem. A real one. 

When I was (I think) a junior in High School, I suddenly started having a pain in my ribs that was extreme and, at times, unbearable. Sometimes, it just felt like my ribs were getting “stuck” on something. Other times, it felt as though a metal rod had been shoved into the middle of my abdomen from the side and it was not separating my upper body from my lower body, as that rod was twisted. It would absolutely take my breath away. It was horrendous! I finally saw our doctor who told me that I have mild scoliosis. Great, doc, but how does that cause pain in my lungs and ribs? His explanation was pleuracy – a phenomenon, during which a rib will “catch” on the lining of the lung and tug when there is movement. Fun, huh? He told me that the residual pain shouldn’t last long and that I would be fine. Fantastic! I was cheering and really didn’t want to miss out! Two years went by and sometimes I would still feel that odd pain, but it wasn’t something that I couldn’t handle. 

Soon, I was a freshman in college. Yay! Freedom! I came to school on a vocal scholarship, as a performance major and I could not wait to perform at the collegiate level! Things were going really well and I was enjoying learning all the “ins and outs” of the music I loved so much! Then Icame down with bronchitis right before our big holiday choral performance. I pushed through and I made it, but the pain was back. From what I had heard from other doctors as well as my own research, pleuracy was not something that should be persistent OR recurring. I knew something wasn’t quite right, but I had no idea what to do about it at 18 years old and out on my own for the first time. So… I drank extra tea and used a heating pad, which got me through, but the pain was almost permanent at that point and, unfortunately, I started to become completely accustomed to the pain. Soon, I started to cheer again. It was okay, but not like High School. I always loved running, but I found that any time I ran, I would eventually be overcome with coughing fits, which I recently learned are actually bronchial spasms. (my bronchial tubes literally spasm) The pain I was in after each practice, required significant recovery. I simply thought I was weak. One day, during practice, a girl fell right on my head and Ia sprained my neck. 100 pounds of falling cheerleader will do that to you, I suppose. I just always hoped my head didn’t hurt her back too much! :/ Anyway… Soon after this incident, with a neck brace… I decided it was probably best for me to step down from the squad. It was upsetting, but I could tell that my body was getting tired and I just couldn’t do it anymore. I didn’t want to hurt anyone else or hurt myself worse. 

My Sophomore year of college, things were good. I was living off campus with a friend and we were having fun, but financially, things weren tough on me. By the end of the semester, I had decided to move back home for a semester and “figure things out”. However, I wanted to finish tha semester with a bang first! The end of November rolled around and then it was the first of December, 2007. My best friend and I decided to go up to Colorado Sprints to do some Christmas shopping and I was going to take some boxes to my parents’ house so that I would be ready to leave after finals. Instead, early that morning, on the first day of December… A friend’s birthday… My best friend and I found ourselves rolling in her husband’s truck down La Veta pass. As the driver, she sustained the most visible injuries. Me… My spine was compressed and I had shrunk (shrank? I never know) almost 3 inches total. Basically, we got lucky. But for me, this was the beginning to a pain I had never known before. 

After the accident, my back was obviously damages and I vaguely remember trying to walk around the house, but it just always hurt so much! I don’t remember much more about my recovery, since my memory was severely affected. 

Now… Let’s fast forward to the winter of 2008. That summer, I had suffered from my first rounds of kidney stones. Now, with the weather cooling down again and back in the beautiful Valley that I live in, I started to notice some serious pain. I was told that after my accident I had sufferedserve damage  to the right side of my body. This made the pain even worse, limiting muscle movement in my right hand, as it does now. As it got colder and colder in our dessert, frozen tundra, I soon found myself unable to even function unless I took at least 5 tylenol pm every night! Obviously, this was not good for me… Especially my stomach!  I tried taking warm baths before bed and I even bought myself a heated mattress pad… Which is still amazing, but just not enough; neither then, nor now. (Of course, now I’m more used to it.) 

I went to a doctor, because I didn’t know what to do and said, “I think I must have chronic pain or something.” To which he was the first of many to reply, “chronic pain isn’t real. It’s all in your head and I would just give you placebos.” Okay, jerk. Well… Your fake meds aren’t going to help my REAL pain! I didn’t see a doctor again until the wonderful Brandi, who I mentioned in my last post! 

She was the first doctor to really pay me any sincere attention. Not once did I ever have her walk into the room and look at me as she wracked her brain to remember who I was. Not once did she have to look at my chart so she could remember my name. She always knew who I was and what kind of care I needed! (she recently moved out of the state, and yes, I cried) 

Let us fast forward a few more years to AFTER I received my POTS diagnosis…

2011…

My POTS was finally getting under control, and I was ecstatic! I was finally able to be active without feeling like my heart was going to beat right out of chest. However, my pain was starting in again, and it was getting worse. I wouldn’t have thought much of it, but it was Summer time! I just didn’t get it! Fall time rolled around and it was the week before I was supposed to be taking a bus across several states to Louisiana to visit my best friend and my godsons. I woke up one morning to the left side of my face twitching. It wasn’t a slight twitch like one might experience in the eye when tired… It was intense. It started in my lip and eventually moved up my cheek and into my eye. Now… It wasn’t painful, but it was disconcerting, to say the least. Soon… I started to notice a tingling sensation that I would get up my face to my head and down my neck into my back. Again… Painless, but irritating. The day before I was to leave for Louisiana, a spasm in my arm started that lasted all day long. This spasm was painful, and overwhelmed my entire arm, so that I could hardly us it all. I decided to mention this to Brandi. She immediately decided to look at MS, given some of my other symptoms. She had blood drawn for everything imaginable; something I had grown accustomed to, yet I still asked the sweet lab tech girl to leave some for me to take home with me! (she obliged) Out of twelve vials of blood, the only significant result was my vitamin D level, which was low.  Brandi immediately started me on prescription supplements, which admittedly helped for a while. However, this “while” didn’t last nearly long enough. After about a month, the spasms were back with a vengeance and a new symptom had started to take form; from time to time, at completely random moments, I started to feel an intense burning sensation on my skin, as though someone was holding a hot curling iron to my skin. It is still something that occursr regularly. 

Back in late October, early November, I was introduced to a group of folks who suffer like I do. I can’t tell you how amazing it felt to hear people tell me I’m not crazy! They went on and on about something called EDS. After receiving more information and doing my own research, I started to realize that this was exactly what I had been dealing with! However, I was still hesitant to say that this was probably what I have. In these last few months, my pain has most certainly gotten worse. Bones slip out of place regularly, but mostly I can just phenagle them back into place with no trouble. Every once in a while, I will have trouble with a hip and hobble around for a few days, but I am learning how to cope. Sometimes I’m scared. A lot of the time, I’m okay with things and am just determined not to let it ruin my life. I just wish my doctors felt the same way. The first time I mentioned this to a doctor, he told me I was probably just depressed and that he didn’t really know anything about EDS, so he didn’t think I could possibly have it. He left me with a referral to a neurologist (whom I have yet to see)  and a complex that I am crazy. He never even checked the mobility of my joints. Ignorant…

The second time I mentioned this to a doctor, it was well received, with the admission that he didn’t know ENOUGH about EDS, but that he would like me to see a geneticist. After making it through a job change with my husband, we are just waiting on the health benefits to kickon and it’s off I go! Hopefully, one day soon, I will wake up without pain. Still… There is no cure. 

So this is my journey.

My journey began…

Blog number 3… here we are.

I hope you are all finding this easy to read, access, and understand. That is my goal through all of this. These rare conditions are difficult enough to understand that even doctors don’t know what they are all about, so I hope that I am being clear and concise. It is my hope that if any of you have any questions at any time, you will let me know and not be afraid to ask! I wish people had asked so many more questions these last few years of my life! 🙂

Now I would like to begin to share some of my story with you all… This will be a story full of frustration, doctors I dislike, doctors I like and, in the end, hope. Here we go…

My first truly frustrating, negative situation with a doctor happened roughly a year after I was in a terrible car accident with one of my very best friends. This accident may have been the beginning of everything for me, but there is no real way of knowing. (At least, that’s what I’ve been told.) This car accident left both the driver and me with a lot more emotional scarring than anything else, but the nerve pain was intense! The bone pain… felt like bone pain, but the nerve pain… it was unlike anything I had ever felt before! I even found a few weeks after the accident, that my intestines were bleeding. It was terrible pain and I could hardly eat. Our accident was December 1, 2007.

Fast forward to Summer of 2008…

After spending a few months in Colorado Springs with my family to recover from my accident, I finally came back to the San Luis Valley at the end of February. Unfortunately, I was not well enough that I could go back to school, and I knew I would also be needing to re-do that fall semester, since I had missed all of my finals. It was difficult getting back into a version of life where I was independent. After my accident, I became severely dependent on my parents… physically, emotionally, mentally… you name it. I needed them. Now, I was expected to go back to adulthood and learn how to be an adult again! I was nervous beyond belief, but my husband (then boyfriend) was my rock. By mid-May, I had found a new job, we had found an apartment in town with a friend, and I was preparing for Summer classes. Life was good! I was a busy girl that Summer and well into that Fall semester. I was working 40 hours a week and going back to school full time. Suddenly, out of no where, I started feeling nauseous. Every day, I would wake up and feel like I had been punched in the stomach, I was in pain and I was beginning to vomit regularly every time I ate. Soon my back started hurting. Finally, it was as though I couldn’t breathe without feeling pain, let alone walk! I saw a few doctors… one said I had a UTI and just needed some antibiotics, but after I finished my treatment, I was still in pain. She suggested I see someone else. I went to another doctor, who so obviously did not believe me. He looked at me as though I was crazy… I had lost roughly 15 pounds by this point (3 weeks since the start of my symptoms), but still he looked at me like I was crazy. He kept asking me questions which indicated his assumption that I was causing myself to lose weight but just wanted to “score” some pain pills. In a move that I can only imagine this doctor hoped would scare me away, I accepted a shot of nausea medication with a HUGE needle going right into my hip. The pain was excruciating but I was totally fine with it! Anything to make it stop! He decided (reluctantly) to order a CT scan of my abdomen at that point. It was scheduled 4 weeks out. This was absolutely not going to be soon enough. I left that office with a bruise on my hip, limping from the pain, 15 pounds lighter and feeling like I was crazy. The pain got worse with each day, until finally I decided to walk myself down to the ER (I was working in the hospital) after I got off of work one evening. The ER doctor was kind enough and said that he thought I had gall stones and that I should avoid fatty foods, red meat, and dairy. This blessed man sent me home with pain meds. Finally, I had my CT scan! Upon my follow-up, I fully expected to hear that I was just crazy! Instead, this is what he told me (I will NEVER forget): “Well… it looks like you have a few kidney stones there… 4 in total, and it looks like you’ve passed one before these. Still… you shouldn’t be in this much pain or discomfort.” Seriously!? You have got to be kidding me! He was so smug… I simply chose to blow off the rest of what he said. I got my pain meds and nausea meds, and I left… hoping to never see this man again. And I didn’t. He never bothered to schedule a follow-up. He did, however, send me to Colorado Springs to see a surgeon, who was expecting to take out my gall bladder… I had been told I was going for an endoscopy! What a mess this doctor was! (His office too, obviously!) Still… this was only the beginning.

A few months later, I passed yet another stone.

A year later, I had SEVEN more! For those of you who have lost count, I had kidney stones 4 times in two years! No one ever bothered to figure out why or how. But this time… I met Brandi. She was PERFECT! I know… it sounds like I fell in love! She was a Nurse Practitioner, and she was what I had been searching for my whole life! Upon learning that I had SEVEN gigantic kidney stones, she decided to stay in touch, and made several personal calls to me, after hours just to make sure I was doing okay until I could see a urologist. That woman did everything for me! Let me take a quick rabbit trail…

For those who may be wondering “what constitutes a HUGE kidney stone?”… I have the answer. Typically they average 1-2mm in diameter. With these seven stones sitting in my kidneys, etc., my SMALLEST one measured roughly 3mm in diameter. This guy was lonely… the rest of my stones measured anywhere between 5mm and 7mm in diameter! Because of this, my doctor set me up with a urologist who saw me and instantly decided I needed to have a lithotripsy as soon as possible. Basically… He wanted to put me on a table, knock me out, shove a tube down my throat, and beat the CRAP out of my kidneys with a huge, and very loud machine, in order to break the stones up so that I could pass them more easily. I could go into much more detail here, but the recovery was a b!*$H!

I sincerely thought things could not get worse than this!

I was so wrong…

I had gotten engaged in 2009, so 2010 started with excitement and a Wedding on the horizon! We scheduled the big day for later that year and started the planning! Suddenly, I woke up on Easter Sunday with the BIGGEST big toe on my right foot that you could imagine! It looked like it was broken! I had no idea how I could have broken my toe in my sleep, but I limped my way through brunch with the in-laws and headed for an orthopedist later on that week. Long story short… I wore a gigantic boot on my foot for nearly 2 months! Why? I had an extra bone in my foot of course! Apparently this uninvited stranger was attempting to make a break for it through my toe! Shots of some kind of incredibly painful steroid or something helped to remedy the situation, so I could avoid surgery. Then… it happened…

That pain in the abdomen… the nausea… the weight loss… the vomiting… the pain in the back. They had returned, right? The kidney stones??? Wrong… I had gall stones. In the middle of all of this, I had started having these attacks at night. I used to have them when I was younger. I would wake up in the middle of the night, heart pounding, sweating, absolutely terrified. It felt like every movement that I made was magnified by 100! I was never sure if I was still breathing or not, all I knew was that I was going to die, and I wished and prayed that it would finally just happen. These attacks were MISERABLE. Then, it started to happen during the day… I would get ridiculously dizzy, my heart would start racing, and all of a sudden I would black out. Then the chest pains started and out of no where, I was exhausted. No amount of sleep was enough. Summer of 2010, I had my gall bladder removed. As much as my doctor wanted to address the other problems I was facing, I really needed to get that thing out of me! The surgery was a breeze… in and out in about 45 minutes. Recovery was a little difficult. I didn’t seem to be healing very quickly, but my surgical compression stockings always made me feel about 10 times better! I would soon learn that this was indicative of my POTS.

After two weeks, I had recovered from my gall bladder surgery and I was back in the office with the INCREDIBLE Brandi… she was amazing through everything! She kept in touch with me, and if I ever needed to see her suddenly, I was told to come it “right now”. Brandi wanted me to see a cardiologist who came to town every once in a while from Denver. “Not much of a bed side manner, but a very smart man”, she said. I saw cardio number one and he immediately dismissed all of my symptoms as anxiety. After telling him that I had been on anti-anxiety medication, he agreed to run a few tests on me. First he did an echo (ultra-sound of the heart), and then he wanted me to go to Denver for a Tilt table test, which would (unfortunately) have to wait a few weeks. The weekend of my Bridal shower, I went to Colorado Springs, saw family, had a blast, and then on Monday, my little sister drove me to Denver t0 have my tilt table test done. The doctor was specifically testing me for NCS or neurocardiogenic syncope. My test was negative for NCS, but I did have significant response to postural change (change in position from laying down to standing up). The PA (Physician’s Assistant) that the cardiologist had left me with completely dismissed everything else that had happened during the test. Thankfully, a concerned nurse wanted me to know about something that she called POT Syndrome. That day, I went home and looked it up right away! It fit! When I got back home, I took this to Brandi and she wholeheartedly agreed with my suspicions. She agreed to do some research and asked me to come back in a few days to follow up again. When I did, she decided I should see a new cardiologist soon, but wanted me to see one of the other Internal Medicine doctors who specialized in cardiology. He started me on beta blockers. (meant to decrease blood pressure, heart rate and decrease overall symptoms of POTS) The adjustment to these meds was difficult, but after a while, they really did help. Unfortunately, they didn’t help for a very long time on their own, because, unfortunately, my blood pressure was already low, so these meds absolutely exhausted me by dropping it even further. For the time being, there was nothing much to be done. My sleeping problems had started again. It’s possible that I had become so afraid of having an attack at night that I couldn’t fall asleep. In any case, Brandi decided I needed something to knock me out, as we had tried more regular sleeping pill prescriptions to no avail. Soon… it was time to get married! 🙂

My beautiful Winter Wedding went off without a hitch, and M and I started the beginning of our Happily Ever After!

I only wish I didn’t plague him with all of these ins and outs, ups and downs of my poor health. How much happier everything could be! But we cope, and we get through.

2011…

I finally saw my new cardiologist. He is AWESOME! Immediately seeing that my symptoms were not being managed properly, he started me on a low-dose steroid. This was intended to keep my heart rate down, but bring my blood pressure up. It also addressed the issue of severe chest pains that were almost to the point of angina (pain like that of a heart attack, that might lead one to believe that a heart attack is occurring… typically a symptom of underlying coronary heart disease). The steroids were a bit rough to get used to, as they caused intense swelling everywhere as well as weight gain. In the Summer of 2011, I found that wearing compression stockings was very helpful in keeping the swelling down in my hands, arms, feet and legs. I should mention that because my body temperature is poorly regulated, heat in the Summer and cold in the Winter, affects me differently than most people. In the Winter, my “bones hurt”, but in the Summer, my hands and feet swell more than any other time of the year, as blood pools and my heart rate spikes. Compression stockings help to reverse this; they encourage the blood to flow back UP my legs into the rest of my body, cooling me down, and bring my blood pressure up and my heart rate down, although hydration is KEY.

Fast forward to 2012… This year has been a good POTS year so far. I have been able to keep my symptoms under control for the most part, with a few issues here and there, but mostly all good stuff! 🙂

I know I packed a LOT into this post and that I haven’t even addressed the issues I face as far as EDS goes, but I think we all have more than enough to process for the time being! I apologize for the length, but I do thank you for taking the time to read through this!

Zebra love to you all!

So, what does it have to do with me?

Today I would like to share some of the “unknowns” of EDS and POTS.

In my slideshow, which you are probably looking at right this very moment, I have included some photos of myself and the various EDS (or bendy/zebra) “tricks” I can do.

I want to apologize for the quality… it’s hard to take these of myself.

I would like to address a few common misconceptions in this post, and I would like to start with the ones concerning EDS.

First:

– People assume that being “bendy” as we call it, is cool and not a huge deal. Well… it’s awesome if you’re in cirque du soleil, or have joint flexibility by itself! Unfortunately, for those with EDS, intense pain is a normal part of the day.

             ** As you may or may not have read in my first post, two days ago, EDS involves random dislocations or subluxations… yes… this is very true. While all EDS patients deal with different problems with different joints, I personally deal with problematic hip, shoulder, knee, and wrist joints regularly, as well as week ribs. I.e., a rib may pop or “slip” if I turn to the side while taking a deep breath, sneezing, or yawning.
Second:
 – People tend to “figure” that EDS is easily treatable. Unfortunately, this just isn’t the case. Because EDS is so rare, many doctors assume that patients are faking it, drug seekers, or in the case that a diagnosis has been obtained, doctors assume that the pain couldn’t possibly be “that bad”. In the case of a friend of mine, she was considered to be in such poor condition that no one would touch her at all! The fact of the matter is that doctors are practicing. And that is why those who are afflicted with rare conditions such as EDS tend to know the most about it… even more than their doctors in many cases.
Other stuff about EDS and symtpoms:
– Numbness, tingling, twitches, intense muscle spasms, arthritic like pain, nerve pain, burning sensations, stabbing sensations, and so much more… these are all regular issues for “EDSers”.

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Now… let’s talk about POTS. 

What people tend to believe:

– That I am lazy

– That I am ALWAYS sick

– That I am a hypochondriac

– That I am faking it

– That I am dramatic

– That it can’t be THAT bad

The reality:

– I am always EXHAUSTED. On a regular basis, my blood pressure stays low enough, that I may as well be sleeping.

– I am always nauseous. Because of poor regulation of my blood pressure, blood flow to my GI tract (intestines, stomach, etc.) is constricted. Sometimes the side effects of this rear their ugly heads in the form of intense nausea, kidney stones, gall stones, kidney infections, gluten intolerance or allergy (nonceliac sensitivity or celiac’s disease), and so much more.

– I am almost always dizzy or experiencing severe vertigo, or what we refer to as a “brain fog”. In fact, sometimes, I feel like “Davey after Dentist”: “Is this real life!?” “Is this forever!?” Unfortunately, dizziness is a common symptom of POTS and the meds that I have been prescribed to treat my other symptoms, include dizziness as a side effect… so… there’s not really any getting rid of it completely.

– I am always uncomfortably hot or uncomfortably cold. Because POTS is a form of dysautonomia, this mean that my autonomic nervous system is basically malfunctioning. This means that my body is unable to regulate body temperature as well as heart rate and blood pressure. This also means that sometimes, I honestly am not sure if I’m hungry or not and sometimes I have trouble breathing (this is treated with inhalers, like asthma).

– If I really want to feel okay and keep my blood pressure UP and my heart rate DOWN, I should drink at least 2 gallons of water a day… I visit the restroom a LOT!

– I have chest pain and/or palpitations almost all day, every day.

– Migraines and complex migraines are a normal part of life. Most people know about or have had a migraine. However, if you have never heard of or had a complex migraine before, let me lay it out for you: The first time I had a complex migraine, I woke up feeling just fine. I was getting ready for work when I saw a black tunnel closing in around my eyesight. When I woke up on the bathroom floor with a bump on my head and still unable to see, my husband took me to the ER. I was “blind” for close to 3 hours, and while I was at the ER, the entire left side of my body went completely numb. The doctors explained to me that having a complex migraine is like having a mini stroke, for which they continuously checked me. Apparently, once that “door” has been opened, the symptoms tend to hang around a bit. While my experience was scary, I know people who are afflicted like this several times a week!

– When I get scared or excited, it hurts my chest a little. (lol) Because my heart rate is so poorly maintained, whenever I even think about something exciting and my heart “jumps”, I get a burning sensation all the way across my chest, I get short of breath and my “heart hurts”. (This is the easiest way to explain it.) Soon after, I usually have an intense pulsation in my head. It feels like someone could SEE my head pulsing. It’s not necessarily a bad thing, nor does it mean that I can’t “handle” being excited or scared, it’s just another ODD reality of my life! 🙂

– If I stand still for much longer than five minutes at a time, my blood pressure will drop (almost quite literally), and my feet will swell and turn bright red. This is called blood pooling. In the slide show, you will see a picture of my foot, in which it is bright red. This happened after I did dishes one afternoon. Followed closely by the red foot, you will see my foot again, but in more of a white-ish grey-ish color. This is called Raynaud’s***.

*** Raynaud’s, as explained by the Mayo Clinic: “Raynaud’s (ray-NOHZ) disease is a condition that causes some areas of your body — such as your fingers, toes, the tip of your nose and your ears — to feel numb and cool in response to cold temperatures or stress. In Raynaud’s disease, smaller arteries that supply blood to your skin narrow, limiting blood circulation to affected areas.”

Alright… well… that was a LOAD of information today! I want to thank anyone who reads this just for taking the time to do so! I hope I offered some perspective into the life of someone with EDS and/or POTS!

Much love! 🙂

Definitions/Welcome to my blog!

Welcome to my blog! 🙂

I’m new at this, so I hope you can all bear with me.

Coming soon after this post, I will be hoping to share photos, articles, videos and so much more to help all of you understand the life of a POTSie Zebra. In other words… patients who live with POTS and/or EDS. Let me define some terms for you first!

POTS: Postural Orthostatic Tachycardia Syndrome (form of Dysautonomia)

From the Mayo Clinic: ” POTS affects the autonomic nervous system, which controls involuntary body functions. In POTS, the nerves that regulate blood flow are out of balance, so blood doesn’t go to the right places at the right time. The result is a variety of symptoms, including postural orthostatic tachycardia — an overly rapid heartbeat that occurs when shifting position from lying down to standing up.”

-There is much more that POTS involves, but for now, you get the jist.

EDS: Ehlers-Danlos Syndrome

From the Mayo Clinic: “Ehlers-Danlos syndrome is an uncommon group of inherited disorders, caused by genetic mutations, that affect the proteins that support the skin, bones, joints, blood vessels and internal organs (connective tissues). Ehlers-Danlos syndrome is usually grouped into six major types. While there is no cure for Ehlers-Danlos syndrome, Mayo Clinic doctors can help manage your symptoms and prevent further injury to your joints, skin and internal organs.”

-I will be mostly discussing type 3, which is also known as Joint Hypermobility Syndrome.

The Beighton Scale is something else that I will be discussing rather regularly on this blog. This is a scale that is typically used to diagnose EDS patients.

Because the lettering is so tiny on the picture above ^, I will “break down” the points system for you.

1 point is given for the ability to touch the floor with your palms without bending your knees.

1 point is given for each thumb that is able to be bent to touch your forearm. (2 points total)

1 point for each elbow that can be bent backwards more than 10 degrees. (2 points total)

1 point is given for each knee that can be bent backwards more than 10 degrees. (2 points total)

and 1 point is given for each pinky that can be bent backwards by 90 degrees or more. (2 points total)

**some patients are able to bend all 4 fingers back 90 degrees or more and this may also be used as a measurement.

9 points total may be given

in order for a patient to be classified as hypermobile, he or she must score at least 4/9 points using the Beighton Scale. (this is what geneticists and most doctors use)

>>>Personally, although I have yet to receive an official diagnosis from a doctor, I have a score of 8/9 on the Beighton Scale. (photos to follow)

***As I post, I will occasionally come back to this blog to add more terms that need defining, but POTS and EDS are, for obvious reasons, the most important for now! 🙂

Much love to all of you who come by and read all that I have to babble on about! Thanks! 🙂

***New Definitions:

NCS (neurocardiogenic syncope; also known as Vasovagal syncope) as defined by the Mayo Clinic: “Vasovagal syncope (vay-zo-VAY-gul SING-cuh-pee) is the most common cause of fainting. Vasovagal syncope occurs when your body overreacts to triggers, such as the sight of blood or extreme emotional distress. The trigger results in vasovagal syncope — a brief loss of consciousness caused by a sudden drop in your heart rate and blood pressure, which reduces blood flow to your brain.”

Beta Blockers as described by MedicineNet: “Beta blockers, also known as beta-adrenergic blocking agents, are drugs that block norepinephrine and epinephrine (adrenaline) from binding to beta receptors on nerves. There are three types of beta receptors and they control several functions based on their location in the body.”

Low-Dose Steroids/Corticosteroids (Specifically, Fludrocortisone) as described by MedlinePlus: “Fludrocortisone, a corticosteroid, is used to help control the amount of sodium and fluids in your body. It is used to treat Addison’s disease and syndromes where excessive amounts of sodium are lost in the urine. It works by decreasing the amount of sodium that is lost (excreted) in your urine.”