Tuesday, March 22, 2016

#Wheelchairproblems

       In honor of Bertha's retirement (my current chair), and the arrival of my new chair (name TBD) I thought I would make a post about some of the pros and cons of being a wheelchair users along with some illustrious stories and bits-o-wisdom.
RIP Bertha Unkown-2016
Bertha fixed with 6 washers and a screw
   
    Thanks are also due to Bertha for she has taken me places that otherwise would have been out of reach, and allowed me to get one step closer to being a normal college student.
     A couple of days ago me and the boyfriend attempted to go visit the Air and Space Museum in DC. We drove and parked at the metro and metroed into DC. Someplace in the literal TWO blocks between the station and the museum, the screw that holds one of the small front wheels in disappeared. This means that the pin that goes through the wheel (essentially the axel) was not held in place. #howdoesthisevenhappen  (by the way this was Sunday....when it was snowing in March and cold as shit)  We had to call a Lyft driver, hope that they would have a car big enough to fit my chair, and get a ride to a hardware store a couple miles away (second time this has happened). Upon loading the chair, one of the spacers must have fallen out too. Thankfully the fellas at ACE Hardware in NW were happy to help us out. That being said, by the time this whole ordeal was over, the museum was closed so we headed back to VA. #wheelchairprobs

Pros:

  1. Good parking spots
  2. Nobody looks at your butt...nobody can see your butt
  3. Your shoes stay clean and nice for way longer
  4. You can get places really fast (as long as its downhill/flat)
  5. People all of a sudden become more chivalrous 
Cons:
  1. You're lower than everyone else
  2. You can't always reach top shelves in stores 
  3. Molehills really are mountains
  4. Sometimes even accessible places are stupidly unaccessible
  5. Clothes are not designed for the sitting figure

Wednesday, March 16, 2016

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.

Saturday, March 12, 2016

On Memories of Easier Days

     I was looking through pictures on my computer desperately searching for this one picture I needed for a project. After a couple of minutes I found the picture and immediately quit the application, hoping I had escaped in time. A sharp pain starts in my chest. I remember climbing mountains, and going on adventures. I remember not having to plan my life around medications, doctors, and energy fluctuations. Spontaneity. Freedom.  Carefree. Worry-free solitude. I have always been some degree of sick, in some degree of pain, and had some restrictions, but nothing compared to this. I live with constant anxiety about flare-ups, falls, canceling plans, and being alone when needing help. It's exhausting. My 21st birthday is rapidly approaching and planning the celebration has been yet another reminder of limitations. Sure, you can break those limitations: eat whatever you want, do something physically demanding, or go to a new place where you are unsure of what you might encounter but theres a catch. Breaking rules and limitations with my body can sometimes leave me dealing with the aftermath for days or weeks following. It can mean anything waking up in the ER with no memory of what happened to simply being unable to get out of bed for days. So, is it worth it?
        For many people a 21st birthday includes hanging out with friends and family, and having their first (legal) alcoholic drinks. Alcohol is definitely out of the question because of the potential for serious medication interactions (RIP Julie). In my family, tradition is to have a family dinner at the restaurant of your choosing and then desert and presents afterwards at home. For me currently, eating causes pain, nausea, vomiting, and other TMI GI issues. I could go out and eat something I've missed and spend days 2-6 of year 21 dealing with the repercussions, or I could plan something not food, alcohol, or high energy centered. Some 21st eh?

I miss not having to worry about spoons, building accessibility, supervision, and germs. Those were easier days.
   
   
      

Monday, March 7, 2016

An Open Letter to my Caregivers and Friends

Dear lovely humans,
    There are a couple things I want to tell you, remind you, or just share with you. First off, thanks. I know I'm not always easy to be around, or to care for, or to love. Now you may try to make the point that no one is an you would probably be right. It is hard for me to accept help and cope with not being able to do the things I used to or should be able to do at my fruitful age of 20. Its hard to watch my peers doing things that seem so out of reach. Its hard to live in a society that values productivity when your accomplishment of the day is getting out of bed.
     So thank you. Thank you for coming up with ways to make me feel useful even when I can't get out of bed. Thank you staying home with me and watching a movie even though it seems like thats all we every do. Thank you for spending hours researching my conditions just so you can understand my world. Thank you for respecting when I want to be alone. Or when I cancel plans for the 1,000th time. Or when I go into a communication dead-zone. Thank you for sitting with me in the middle of a public place when I get a dizzy spell so I don't look stupid. Thank you for standing up, and speaking up for me when I couldn't. Thank you for spending countless hours in doctors offices, ERs, and hospitals. Thank you for being patient.
      Apologies are also called for. I'm sorry for lashing out when I've had a bad day. I'm sorry for being unpredictable. I'm sorry for the days when the illness takes over. I'm sorry I can't be your partner in crime for hikes or horror movies anymore. I'm sorry for the middle of the night panics, the scary calls from EMS saying they're taking me to the hospital but can't tell you why. I'm sorry for causing you pain when bad things happen. I'm sorry sometimes I live in my own world.
      Thank you for helping me get through the deep valleys of depression and isolation. Thank you for being there to help me celebrate my accomplishments and joys. I am constantly amazed by the unconditional love and support you guys show me (except you mom, that's in your job description).
                                
Thanks,
Julie
  

When Your Wellbeing Gets Lost in the System

          I have been very fortunate to have a some brilliant minds working to keep me alive and improve my quality of life. Two of my specialists are so good at what they do that people come from all over the world to seek out treatment from there. I merely have to get a 30 minute ride. I honestly don't know where I would be without them. That being said being a patient of these doctors who are at the top of their game and are leaders in their field has some serious downfalls.
Here are just a few:

  1. Months if not years of wait time for a new patient appointment, if they're even accepting new patients
  2. Long periods of time in between visits after becoming an established patient
  3. They're stupid busy between conferences, surgeries, case load, and teaching
  4. They don't communicate with the rest of your team whether due to their schedule or their ego this can become a big problem
  5. I can't remember the last specialist who was knowledgable about my rare diseases who took insurance
  6. Their offices are usually administrative nightmares
  7. You usually have no one to go to (with cases like mine...rare meets complex and misunderstood) for a second opinion. You're second opinion become Google which can be dangerous.
  8. You will wind up having to try and get urgent medical care from Emergency Departments which are neither equipped to deal with your..."specialness" or too open to the concept of you knowing more than them about your conditions/ treatment needs.
        As great as it is to have some brilliant minds treating you, you wind up bearing a huge load on your own. Anyone with rare/chronic illnesses can relate to the feeling of their care getting lost in the system. I have been utterly dumbfounded by how unequipped our healthcare system is to deal with the chronically ill. According to the CDC, as of 2012 about half of the adult US population had one or more chronic illnesses. Thats roughly 120 million people. One in four Americans have two or more chronic illnesses.
      I wanted to write about this for a couple reasons, but the main reason being in response to an incident that happened a couple of weeks ago. Over the years I have slipped through the gaps in the system here and there but most of the time no major harm resulted in it, just frustration and longer waits. This time was different.
      It started like many incidents nowadays do, I had passed out in the shower and woke up in the ambulance three hours later (it was also 3am) with IVs and monitors galore and a big shrug given when I asked what happened. Upon my arrival to the ER I was fairly quickly assed by a resident and hooked up for monitoring by a nurse and left there. Anyone who has spent enough time in ERs knows that the worst thing that can happen is still being there during shift change. 0600 roles around after a couple of hours staring at the ceiling (I was in a cervical collar and left lying flat on my back) and one tech coming in to take an x-ray I realized there was no way I could fly the coup before shift change. The doctor comes in to tell me my ECG was abnormal and they're waiting on blood work results (which they never drew). He told me my new doctor's name and that he will come in when he gets here and repeat the ECG. If its abnormal again they'll admit me but if it's normal they'll send me home to follow up with my cardiologist. 0900 rolls around, no doc, no repeat ECG, no bloodwork. I call the nurse. She takes the blood seemingly pissed off that I was checking up on things trying to get answers. 1130: nurse comes in and hands me my discharge papers saying she can't find any of my four doctors to talk through them with me but they need the bed so they're discharging me. I ask about the repeat ECG. She shrugs. I get frustrated and leave.
          I call my cardiologist and he agrees to see me later that day. He does an ECG and it was once again abnormal. He reviews my medications and asks me about one that I started the week prior per orders from my GI doctor. "This medication has a blackbox warning for causing a prolonged QT interval which is very dangerous. Your doctor should have never prescribed you this. You should not have been discharged. You need to stop this medication immediately and go see an electrophysiologist."
Note to self: need to find new GI doctor.
Note to self: call cardiologist when before taking any new meds
Note to self: stand up for yourself in the ER
Note to self: you need to be on your toes, you're health and safety is your responsibility

        Several doctors appointments later and the issue was addressed with relatively few repercussions (lucky compared to what the situation could have been). I now have to get a heart monitoring chip inserted above my heart to collect data and try and figure out if there is another underlying condition.
        It scares me to say I got lucky because that means things could have gone way worse. I'm constantly worried for the day that I won't get as lucky. When will it be? Will I be alone? Will I get to the ER but have it be dismissed?





Thursday, March 3, 2016

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 8th 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.