Me vs Gravity: Life with Dysautonomia

         October is also Dysautonomia Awareness Month! Wahoo! Dysautonomia is an umbrella term for several conditions that result from a dysfunction of the autonomic nervous system (ANS). Many people know of the ANS from health class as being the system that is in charge of the "fight or flight" response, and you'd be mostly right. Yes, the ANS is in charge of "fight or flight" but more broadly, it is essentially in charge of regulating the automatic functions of your body. There are nine different types of dysautonomia: POTS, OI, AAG, Pandys, NMH, NCS, PAF, FD, and MSA. Learn more about them here. For this post I will only be talking about POTS and my experiences with POTS. I have secondary hyperadenergic POTS (hPOTS).

What is POTS?

As stated above, POTS is a neurological condition in which the ANS does not send the proper signals to the body to regulate blood pressure and heart rate (primarily, POTS affects the whole body).  Normally, when a person stands, gravity pulls blood to the lower extremities. The nervous system senses this and sends a message to the blood vessels to constrict, sending more blood back to the heart and head. With POTS, this message to constrict does not get sent. Therefore, instead of the heart rate increasing by a normal 10 to 15 bpm upon standing, it can increase by more than 30 and can even double (when first diagnosed mine went from 68 bpm lying down to 189 bpm and remained that way for 10+ minutes). By affecting circulation, it also affects cerebral blood flow. Symptoms include orthostatic intolerance (dizziness), chest pain, headaches, GI cramps and dysmotility, inability to focus and concentrate for long periods of time, inability to read due to blurred vision, difficulty with recall, blood pooling, extreme fatigue, nausea, tremulousness, insomnia, loss of consciousness. 

What causes POTS?

While there is some knowledge about comorbid conditions that are commonly seen with POTS and certain types of POTS have associated causes with the start of symptoms, it is unknown what is the true cause. For example, one type of POTS can occur post-concussion and we know that but not everyone who gets a concussion gets POTS. There is currently no cure and treatment is based on symptom management. 

Common Misconceptions

• POTS is caused by anxiety. POTS symptoms can mimic anxiety/panic disorders patients are often misdiagnosed or POTS is missed entirely because symptoms are deemed to be caused by anxiety. See research here. 
• POTS is caused by deconditioning. See research here. Exercise (for those allowed by their doctors) can be a crucial part of a POTS patient's treatment plan but is not caused by deconditioning and cannot be cured by exercise alone. 
• POTS is just getting dizzy sometimes. POTS is a complex neurological condition that effects every patient different though almost all, if not all, have multiple organ systems effected. 

Lingo

• brain fog: cloudy feeling that hangs around and causes you to do stupid shit like flush the toilet three times, forget if you took your meds or just thought about taking your meds, and being unable to form a coherent sentence

What my POTS is like

***Disclaimer: This will by no means be an extensive list of symptoms or experiences and this is just how POTS effects me. There will also be crossover between EDS, GP, MCAD and possibly LQTS.***

       I was formally diagnosed with POTS in 2015 though I had symptoms beginning in early middle school years. To save my fingers I am just going to talk about my current life with POTS and will probably do a more extensive post later, I just tired and lazy.

       Remember the last time you had the flu and ached everywhere, slept all day and don't remember half of what happened? Now imagine waking up feeling like that every day (and more! yaaay), this is one of my main struggles with POTS. Several factors contribute the fatigue including medication and adrenaline surges/crashes but on any given day my energy level is equal to or less than a sloth on Ambien. I take naps nearly daily, sometimes multiple times a day just to keep up with my peers. I have trouble filtering out stimuli, recalling things, producing speech, following multistep directions, and reading for comprehension.

        Another big problem I have is blood pooling. POTS alone can cause bad pooling but with the stretchiness of my veins from EDS, it is much worse. I also have Raynaud's syndrome so my hands and feet are always cold! The picture to the left is an example of blood pooling in my hands (on medication). This is what happens if I stand with one hand raised and one arm relaxed for two minutes. The white hand was raised and therefore has less pooling (gravity does all the work). After about a minute of sitting my legs look like my left hand. Now imagine a 11 hour car ride or a 3 hour lecture. This is why many POTSies wear compression stockings and socks to help our bodies circulate blood or why lying down with our legs up helps. 

       While POTS impacts many things in my life it does not mean I can't do fun stuff, learn, and enjoy life I just need everything to slow down and take breaks. I have very limited energy and most is spent on school, existing, medical appointments, personal hygiene (showers are EXHAUSTING), and keeping myself alive. I have to worry about getting places without steps, lying down in the middle of the mall to keep myself from passing out, obsessing over hydration and medication schedules to maintain baseline and prevent things from spiraling out of control, and pretend to be a functioning adult.

Thanks for reading and make noise for turquoise (dysautonomia awareness color)! 

Finally got around to making that awareness video... 2 years later...

WARNING: Might just be one long rant...

        Okay... so I wanted to touch on a bunch of different things that don't have another home. I'm feeling very brain foggy/disorganized so if none of this makes sense... then it'll probably at least be entertaining.
#1: How long it takes to get anything done in the medical world
      Let me preface this with I ABSOLUTELY know this is not all on the medical professionals. There's shit with insurance, documentation guidelines, hospital rules and regulations, and just overweighted caseload that influence this issue. But seriously, it gets ridiculous.
Some examples:

• I met with a new GI July 22nd. He seemed on top of his game, familiar with my conditions, and agreed that action needs to be taken to get by nutrition etc back on track. He had lots of medical history, former testing, current summary... everything he needed. He ordered a SmartPill test (camera pill you swallow and it takes pictures and does testing as it goes through your GI tract... pretty cool) and an upper and lower scope (sticking cameras up your arse and down your throat...yum). I had already had a gastric emptying test to diagnose gastroparesis (delayed stomach emptying), but we had no idea the motility of my intestines or if they absorb any of the nutrients. Fast forward 4 weeks until my scheduled SmartPill test swallow. I had to go off all GI meds for a week (which means not only more of the daily yuck but near constant acid coming up my throat), get a ride to the hospital, fast beforehand, take off work etc etc. I get there and they're (nurses) doing the pre-procedure checklist. They get to question #3 "do you have any implanted devices?"... "yes... but the doc knows about it... we talked about it"  "Let me give him a call"  *comes back 10 minutes later* "I'm sorry, the implant disqualifies you from doing this test, I have to cancel it... the doctor will give you a call (lol jk)". Okay, so waste of time. I messaged my doc later (shock and awe... he never called) and asked if we could schedule the only other test one can do to get the same data as the SmartPill. His response: we will talk about it next appointment. So you're thinking aight... that's okay. Wrong. Not only is his first available not until mid-November, but his office won't schedule any follow-up appointment until after I complete, and get the results back from the scope scheduled for September 14. So basically I'm SOL until at least December timeframe.... assuming that visit won't just mean going over EGD results and him ordering the same test I asked him about months earlier. Don't worry guys...it's only nutrition. 
• It took my PT 3 weeks to write and sign a sentence saying "patient needs new custom wheelchair back to maintain posture and for support". 
• It has taken my doc 5+ weeks (he was on vacation for one but still) to write and sign a prescription, note, and LOMN saying "patient needs custom bilateral AFOS". 
• I've been waiting for records from one doctor for 13 months despite several verbal and written reminders to him and his staff.

#2: My two cents on the Epipen bull:

          As someone who is literally allergic to life (sunlight, heat, cold, stress, talking, meds etc) and must always have Epi on my person and has unfortunately had many uses of the autoinjectors I am pissed. But not just by Mylan (jerk face mcugly butts) jacking up the price of Epipens (500%), but the fact that I have multiple friends who have to make the choice between life-saving medicine, therapy, and treatments and feeding themselves or their kids. 

And we aren't even talking about those without insurance. Most of my friends are lucky enough to have some form of health insurance (even if its crappy) and are still drowning in bills and medical debt. There is not a single day that there isn't a medical bill arriving at my house and that is just ERs, hospitals, and doctor's bills. Plus medicine, devices, PT, testing, surgery, and prolotherapy (not covered by any insurance as it is "experimental"). I have been incredibly blessed with kick-ass insurance (although still a PITA) and to be able to stay afloat in the bills. Despite all kinds of preventative and prophylactic treatments, there is still only so much you can do to keep ahead of things and emergencies still happen. Epipens also only are viable for a year. They come in a 2 pack but most need more to keep one at school, one in the car, and one on your person. For most, it is now cheaper to go to the ER for anaphylaxis treatment than to use their Epipens. Honestly, all I got is "fuck you Mylan". No excuses, epinephrine is cheap, autoinjectors are relatively cheap. Not to mention their tax evasion strategies. 

#3: Depression

Since the people who 1) even read this blog and 2) read all the way til now and aren't asleep from boredom are probably the people who care, I saved this one for last. As some of you have noticed, I've been dipping into another depressive phase. Depression and me are buddies now so it's cool. It is also normal and a big part of chronic illness so don't go panic on me. So don't be alarmed if I'm a) super clingy and annoying or b) super avoidant and grumpy gills ... that's kinda just how it goes. See previous post RE: things you can do to help.  

Aight. That's all for now....

The EDS Bible

Finally it's back in stock! This book is really the EDS Bible. It is written by Dr. Brad Tinkle who is largely considered one of the fathers of EDS and is very well respected in the EDS community.

http://www.amazon.com/Hypermobility-Handbook--Management-Ehlers-Danlos-Syndrome/dp/098257715X/ref=sr_1_1?ie=UTF8&qid=1458184415&sr=8-1&keywords=joint+hypermobility+handbook

           I have been waiting for months for the book to come back in stock (no idea why) and it is finally here! I did my first breeze through it and it looks amazing. It not only details EDS, the many conditions that are comorbid (come along with), and research but also treatment plans, PT options, medication and supplement suggestions and much more! Highly recommend for EDSers and caregivers as well as medical persons who want to learn more about EDS. I will post again later once I have read more.

Backstory

     Hello! My name is Julie. I am, in many ways, your typical  21 year-old college student. I am a junior at George Mason University. I love traveling, languages (learning my 8^th now), movies, photography, nature, fixing things, and art. I live in the dorm on campus during the week and on the weekends I stay with my mom and work. During the school year I work part time as a support staff person for kids with special needs in camps and classes. My hope is to become an Occupational Therapist. I love it because it combines the problem solving and MacGyver-esque fixing with helping people and medicine. I also have been volunteering at a local free clinic doing medical interpretation as well as some administrative tasks for almost 8 years now.

I have an older brother who is now 23 and was my partner in crime throughout childhood. My mom and I are pretty close, we both like similar music, playing card and board games (when I let her win ;) ), and watching movies. As kids, my parents took us on several international trips, but as we got older the traveling radius got smaller and smaller. That is until I caught the travel bug again in 2011. I convinced my mom to go back to Ghana with me. It was the first of many a life changing trips. Now we are all probably familiar with the new fad of “voluntourism”, and going to developing countries to “make a difference”. Well I was young and naïve and I thought I could change the world in the month I was there. Needless to say, I learned an incredibly important lesson about expectations and going with the flow. I think this has helped me in dealing with my illnesses. 

The next year I received a State Department Scholarship to study Arabic is Muscat, Oman for the summer. First off, I learned that having a severe food allergy to onions in a country whose cuisine is a combo of Middle Eastern and Indian is complicated. The first day of the program in-country I met with the Indian cook for the program, told him about all my allergies and how important it was that I don’t consume anything with ingredients I’m allergic to. The cook responds flabbergasted, “but ma’am, with no onions…no flavor”.  Needless to say having an anaphylactic reaction in the middle of the desert is nerve racking. After graduating high school in 2013, I made my way to Peru to volunteer in a clinic for one last hurrah before college. 

At the top of Mt. Machu Picchu 

 So that’s the typical young adult part… but who wants to be typical anymore?

 I spent my freshman year studying Nursing at the University of Alabama Birmingham (UAB). Due to my work at the clinic and my good grades I was pre-accepted into the School of Nursing, just as my grandma had done there many years before me. I loved college, I loved my nursing classes, I loved my independence, and I loved the possibilities of what my life could be. Two weeks into school I woke up at 3:00 am in the ER. I didn’t remember getting there, what I was doing before, or going to sleep. I frantically pressed the call button until the nurse came in and explained to me that I had a seizure and have epilepsy. She paged the doctor and he came in, explained that they were going to start me on anti-seizure medication and have me follow up with a neurologist. Epilepsy. The nurse came back with my discharge papers. I laid there frozen for a few minutes till things caught up. I panicked. I remember calling my mom, my aunt, and my friends desperate for someone to pick up the phone in the middle of the night and tell me it was going to be okay. Finally, my uncle, a doctor in Oregon, picked up. He calmed me down and told me to hand the phone to my nurse or doctor. He talked with them and helped me make a plan. I had no idea that this was just the beginning. It was just like that moment in Ghana where I realized things were changing and there was only so much I could do.

            The rest of my freshman year was a battery of tests, hospitalizations, therapy sessions, and doctors visits. They ruled out epilepsy eventually with continuous EEG monitoring and passed me off to a psychiatrist who passed me back to the neurologist who eventually passed me back to the psychiatrist. You get the picture. By the end of the year I felt like I was 89 years old, not 19. After my freshman year ended I had to make one of the toughest choices of the year: drop-out of UAB, leave my friends and nursing school slot and move back to Virginia with my mom for treatment, or to continue trying to balance school (somehow managed a 4.0 my freshman year despite my health continuing to decline) and treatments with no support system within 500 miles. I chose moving back. I enrolled for online classes at my local community college and started making calls to specialists and doing my research. At that point, I was still having seizure like episodes, syncope, digestive issues, more frequent joint dislocations, severe migraines, breathing problems and severe allergies. Nobody could figure me out. 

I had one friend in the area, very little interaction with the outside world, and lots and lots of interaction with the medical world who gave me diagnoses of things like Munchausens, Conversion Disorder, “Just Anxiety”, Attention Seeking, “Abdominal Pain”, etc. I grew more and more frustrated and sick. Finally, a friend from high school mentioned Ehlers-Danlos Syndrome (EDS) and Dysautonmia/POTS (Postural Orthostatic Tachycardia Syndrome). She had been diagnosed in middle school. Having no idea what she was talking about I started googling the conditions. Things started to make sense. I was a pretty sporty kid, but spent most of the time on the bench due to injuries. A normal scrape for most people took months to heal and always scarred for me. Sitting still caused pain. Standing up made my head spin and I frequently passed out. Eating made my stomach hurt. Even after 2 years of braces and a permanent retainer on my teeth they still shifted right back to where they wanted to be. A shoulder injury in middle school put me in PT for two years with little improvement. Wait…normal people don’t have joints that pop in and out easily? I printed out the information on EDS and POTS and made an appointment with my primary care. I finally felt like I knew what was going on. I wasn’t making this up. This is real. My primary care breezed through the information sheets, looked up at me and told me, “you don’t have EDS, we would have seen it by now”. Seen it… an invisible illness… she didn’t see it. It felt like a dagger in my chest, but I didn’t give up. I made an appointment with the cardiologist my friend sees who is one of those world-renowned types who appears on talk shows and has people who traverse national boundaries to see him.  

Three months later after even more tests, he looks at me and says, “you definitely have POTS and EDS and a complicated case of it. I also think you have Mast Cell Activation Disorder and Cranio-cervical Instability (CCI). EDS and POTS have no cure but we will do our best to help you manage your symptoms.” I felt relieved and somewhat happy. Someone finally believed me. Ehlers- Danlos Syndrome is a group of connective tissue disorders in which the body produces weak and faulty collagen which is the “glue” that holds your body together. All of my joints dislocate very easily. I am extremely hypermobile. I bleed, bruise, and scar very easily. Additionally, my veins and organs are prone to rupture. I am in constant, severe pain. Since collagen is everywhere in your body, it effects so all body systems. POTS, MCAD, CCI, Gastroparesis, Arnold-Chiari Malformation, Migraines, Depression, and Anxiety (all of which I have) are just a few of common comorbid conditions that piggyback off EDS. POTS is a condition that affects my autonomic nervous system which controls heart rate and blood pressure. Normally, when a person stands, gravity pulls blood to the lower extremities. The nervous system senses this and sends a message to the blood vessels to constrict, sending more blood back to the heart and head. With POTS, this message to constrict does not get sent. Therefore, instead of the heart rate increasing by a normal 10 to 15 bpm upon standing, it can increase by more than 30 and can even double. By affecting circulation, it also affects cerebral blood flow. Symptoms include orthostatic intolerance (dizziness), chest pain, headaches, GI cramps, inability to focus and concentrate for long periods of time, inability to read due to blurred vision, difficulty with recall, extreme fatigue, nausea, gastroparesis (paralyzed stomach), tremulousness, insomnia, loss of consciousness due to loud noises/ flashing lights and much more. It can also present with seizure like episodes from extreme adrenaline rushes and hypoxia. At last, the real answer behind my non-epileptic seizures. MCAD causes hyperactive mast cells which release histamine bursts causing reactions such as hives, flushing, difficulty breathing, and anaphylaxis. I can react to just about anything (even heat and cold), and what I react to can change. I was newly diagnosed with Long QT Syndrome which can cause heart arrhythmias, syncope, and seizures. I have an implanted cardiac monitor that continuously monitors my heart for 3 years.

Fall of 2015, I started school at GMU and have enjoyed it. It allows me to have a part-time college experience close to home, and close to my medical team. I use a manual wheelchair with a portable power assist wheel to get around. I work 3-4 hours each weekend and in my free time I go to doctor’s appointments, color (when my fingers don’t dislocate too much), watch movies, go for rolls if the weather is nice, and hang out with my friends. On a good day, I can tackle the world. On bad days I need help with mundane tasks and can’t get out of bed. Did I mention my conditions fluctuate? Living with several rare diseases is like having Latin be your first and primary language. The language you operate in, think in, dream in. Now you know English too, but at times English seems so foreign. Naturally, those who understand you best as you would be those who also have Latin as their first language. They understand your struggles in trying to communicate your experiences to others, they understand that you're basically operating on a dead language. If you want to talk to the pope, you're set. Rare diseases and chronic illness can be isolating and overwhelming. They influence my life and can control it, but I refuse to let them define it. After all, I am a professional patient.