Health, Unveiling Invisible Illnesses

Dysautonomia International Education Day

Dysautonomia International Education Day in Clearwater, Florida 11/09/2019


The Education Day in Clearwater was great. I definitely would love to host a local (Brevard County) get together soon. I would love to have more time to talk and get to know the other warriors. Here was my quick take on everything:

It was pretty cool to hear that 30 different universities are currently studying dysautonomia. Lauren Stiles said the push for research funding comes from us patients wanting answers. It is assumed that 1-3 million people have POTS. Dr. Laura Pace (neuro gastric MD) says “we need to treat the cause, not the symptom.” Amen! She also had an amazing point that some of us (especially with Mast Cell) can develop eating disorders as a protective mechanism from symptoms. She had a case of a young girl that was diagnosed with anxiety and eating disorder but after Pace’s testing, she actually had severe gastroparesis and POTS. She is a huge advocate for the Smart Pill.

Dr. Raj was awesome! He was very funny and witty. He had pointed out that many patients do better on low dose beta blockers vs. the typical higher doses that are prescribed. I completely agree as my doctor originally had me on a very high dose, which bottomed out my bp and made my heart even more tachy. I do much better with low dose. Dr. Raj also briefs The Faces of POTS journal (2019, B.H. Shaw, L.E. Stiles, et al.) and points out that 89% of patients missed school, 28% were homeschooled, and 25% dropped out of school as a result of their POTS (B.H. Shaw). Dr. Raj recommends exercise tips, suggesting every other day or 4x per week for 30 minutes. He suggests if you are done and quit in 13 minutes then you need to go slower, pacing yourself. He does not suggest upright exercises. Behavioral therapy is also important in dealing with chronic illness; Dr. Raj states, “ultimately people have different strategies for learning to cope.” Yoga, therapy, SSRIs, mindfulness etc. are different options in coping (2019, Dr. Raj).

Dr. Laurence Kinsella (neuro) had great recommendations for CBT (Cognitive Behavioral Therapy) to help with coping. I also love that he mentioned “symptom snowball” which makes diagnosis tricky because of the long list of symptoms that we have. He discusses the longterm effect of meds used for migraine treatment and suggests Migreleif as a holistic approach, along with an elimination diet. He also suggested a free course to help with sleep management: free mindfulness course.

It is great to have a community driven for change, giving answers to the patients and education to health care providers. Dysautonomia International is not only raising awareness and providing education, but they are advancing medicine in a grey area that leaves millions in the dark. They are shedding light and discovering much needed answers, with much more to come.

Print Outs:

10 Facts About POTS

What is Dysautonomia?

10 Facts About Dysautonomia

Health, mental health, Unveiling Invisible Illnesses

I am not strong

I am not strong.

I am not this tough, battling warrior that some may see me as. I am scared. I am depressed. I am angry. The cards I have been dealt leave me no choice but to try to roll with the punches. I may do so gracefully on the outside, but on the inside I often find myself questioning, “why?” as I carry around the grief of living with a chronic illness.

Every day I wake up in pain and discomfort. Some days it is just my normal everyday life and I accept and move on. I get up and start my day, tucking the pain away. I ignore my reality of a failing heart and the dozens of risks that hang over my head. I sweep under the rug all of my nervousness and worries and I focus on what is good in my life. People think that may be admirable but really, it isn’t healthy. Also, what other option do I have?

We are always told to focus on the positive, while ignoring the dark and negative aspects of life that exist for all. For me, the only way out is through. Acknowledging and dealing with the darkness is healing. Society teaches us to suppress ourselves and our feelings, which leaves us depressed, hiding behind a smile. We are pressured to be put together and strong no matter the circumstances.

Other days, when I wake up, I can’t tuck away the pain. I can’t pretend that I don’t have this horrible genetic condition that eats away at me, that I forever have to live with. I look in the mirror, before my exhaustion is covered up with makeup, and I see how how hurt and tired I really am. I see how sick I look. I begin to hide it, first with my morning meds and then with makeup. I cover it all up.

You wonder how I am so busy? I have to be. I have to keep myself so distracted because the moment I sit down and stop moving, I feel it all: mentally and physically. When my mind has no distractions, I cannot help but feel the storm come. I think about, “what if I die?” and “I am so sick and tired of being sick and tired.” It’s not fair. My mind will go into dark places. And I just have to tuck it away. I have to “be strong” because that is what everyone wants to see, right? No one wants to see someone complain or pity themselves.

I don’t give up because I fight for my kids. I fight for my husband and my family. I fight for others that may one day be in my shoes. I fight for advocacy and healthcare equality.

I still have someone in my life who haunts me, tells me how much of a burden I am. “You always have something wrong with you. I can’t keep up with all your surgeries. What, am I supposed to carry around a calendar?,” he screams over the phone just 5 minutes before surgery, due to a delay and miscommunication in last minute changes (with the schedule with our child). Sometimes I let the past (and ongoing) emotional abuse of this ex get to me. I find myself questioning how much of a burden I am to others. This is often a question the chronically ill deal with. There is always someone without empathy that has a heartless opinion about you and your health.

Then, you have those “healers” who have the cure for you. “Try this holistic approach if you want to cure yourself and be free of illness and magically live healthy forever.” Apparently these people don’t know that I already eat a strict, clean diet without preservatives, dyes, additives, artificial ingredients. I am a certified herbalist. I don’t drink alcohol or caffeine. I take herbs and supplements. I am very knowledgeable on natural remedies, which I use for most ailments. What people don’t understand is that their basic education does not cover a vast amount of information on the human body. Sure, you can change your lifestyle, diet, and start supplements to reverse or mend many issues. But at the end of the day, it is not going to fix my heart and it isn’t a one size fits all answer. My heart is anatomically unable to be altered by herbs. This isn’t a lifestyle thing, stress or cholesterol induced issue. PLEASE FUCKING STOP sending your unsolicited “cures” to me and others. It is absolutely horrifically disrespectful and insulting.

——

It’s currently noon. My neck is stiff and I have yet to brush my hair or teeth. I glare at my heart meds on the dresser that I still need to take. My back is in pain, spasming and out of place. I am dizzy and know that my heart rate will shoot up and my blood pressure will drop as soon as I get up. Nausea and headache to follow, as I hold onto something to keep from falling. But, I will put myself together. I will suck up the pain, anger, frustration, sadness and make myself look strong with a pretty dress and red lipstick.

But I am not strong.

This is just my life.

Health, Unveiling Invisible Illnesses

Sweet Dreams with an Invisible Illness

When your bed is your sanctuary, a safe place but some moments steal that from you.

After a long day, collapsing into bed is pure bliss. Your head rests on the pillow and the soft comforter melts over you. As your eyes become heavy, suddenly your heart flutters with each breath. Your chest gets tight, as if someone is crushing you or you swam to the bottom of the deep-end pool. Your left hand and mouth feel like needles and pins while your chest begins to ache. The pain in your left arm makes it impossible not to worry.

You sit up and take slow deep breaths, taking your blood pressure and discovering that it is high. Some moments your blood feels cold as it runs through your body, so you turn on your bedside heater and grab your robe; this isn’t the first time you have felt this way so you know what to do by now. After a few starling palpitations you decide it’s time for emergency medication. You are still trying to prevent going to the ER, where they will simply question your mental health and ask, “are you having an anxiety attack?”

“You’re too young for…”

“No, that’s not common for your age…”

———-

As I sit up, I can’t help but wish my body didn’t betray me so often.

Let me rest.

Let this pass.

Why?

My muscles twitch and spasm throughout and inside my body, like a symphony of fireworks. The ringing [in my ears] is so loud that even as I distract myself with the TV, it still rings louder. Occasionally the chest pain strengthens and waves of vasospasms in my chest put the fear in me of what could happen next. I try lying down again until I shoot up after another intense heart rhythm.

I’m so tired.

Please.

As the meds kick in, I wait to lie back down in fear that another episode will come. One by one, I turn off my heater, remove my robe as my blood circulates better, take a deep breath to see if the tightness has released, slowly inching back into my pillow, and then I drift into sleep only to hope that I wake up to start my day tomorrow.


*From 2017-2019 I went to the ER 54 times. I still continue to end up in an ER once every month. I decided to document/blog (after) an episode that sometimes sends me to the ER; fortunately I was able to manage through it tonight and avoided an ER trip. This is another reason I am grateful that Mayo Clinic found the myocardial bridge, which often causes coronary artery vasospasms at night.

Thanks for listening and learning with me to spread awareness and hope for others that may one day be in my shoes. Never take your health for granted.

Health, Unveiling Invisible Illnesses

Mayo Clinic Update

We are done for the day and just had the evaluation with the cardiologist. In a nutshell: it takes a village. My aortic valve is slightly worse but my heart isn’t in bad shape to need surgery YET so that is great news, for now. However, I did have an elevated NT-Pro BNP which is indicative of heart failure but ever so mild and more to be used as a baseline.

I will be back September 5th for more tests. I will finally get my cortisol and metanephrines tested. I will have a CT angio and a 7 day heart monitor. The role for this doctor will mainly be to monitor my heart valve. We are ruling out any other structural abnormalities and then this information will be very helpful to the new neurologist that I will be seeing out of state in Arizona, unless Nashville opens up (first choice). The answers I am mostly looking for will be there, to better understand and treat my dysfunctional nervous system. The full genetic sequencing is another piece of the puzzle. There are a lot of pieces.

The valve is one issue but my nervous system is what causes the other heart problems, as well and many other issues. This is an ongoing process. It’s is an up an down roller coaster. At times, I am excited to get answers and the Cinderella hopefulness to find a way to magically be better. Oftentimes I find the sinking feeling of reality and logic settling in my stomach that there is no cure, just management. It’s impossible to accept and why I still try to search for more answers.

My health is like a domino effect: one issue causes another, then another… There is such a huge list of issues connected to connective tissue (disorders). This also makes it difficult to understand and diagnose, because it’s essentially a giant cluster fuck.

I will always continue searching because science advances, awareness spreads education, and advocacy feeds it all. I will continue to fight for myself but also for the future of others that will stand in my shoes one day. I hope the darkness in my life fuels the light that other seek.

Thank you for listening and for your support. Feel free to subscribe or to reach out if you ever need any help. It is my passion to lead other patients in the direction they need.I know exactly what it feels like to be lost, medically neglected, and dismissed.

Helpful Links:

http://www.dysautonomiainternational.org

https://www.ehlers-danlos.com

https://www.healthline.com/health/mast-cell-activation-syndrome

The Invisible Diaries

https://vimeo.com/292473119

Health, Unveiling Invisible Illnesses

Why I Am Going Out of State for a Doctors Appointment

After seeing a neurologist for about nine months, we came upon the solution for me to see a different neurologist, in the same office, since my doctor felt she couldn’t help me. Her specialty was migraines and seizures and she was not familiar with the dysfunction of the autonomic nervous system. I felt it might be easier to stay in the same office since maybe the two doctors could communicate or have access to more information than leaving the practice altogether.

It has now been a little over a year with my newer neurologist. In this year, I have seen him every month at times. One appointment was to order an MRI without contrast to rule out certain diagnoses. Then, the next appointment was to follow up on that test and to order something else. It felt like it dragged on and on, leaving me to wonder why everything wasn’t just all tested at once. I could sense the uncertainty, and while he admitted that he isn’t familiar with dysautonomia, he sympathizes and ensures that he will help somehow.

The first neurologist ordered an EEG and she said everything was delayed but showed no signs of seizures. This was two years ago, before my pacemaker. After leaving that test, my heart rate dropped and I passed out in the hallway. Fortunately, I had a heart monitor on at the time and called my electrophysiologist and explained what happened. They looked at the episode and determined my heart rate plummeted.

Now, two years later, my new doctor decides to do another EEG. I find myself nervous, wondering if the testing triggered my episode last time. But I also find myself frustrated and here’s why: two appointments ago, my neurologist said that my (dysautonomia) episodes sound like seizures. He offered me seizure meds and I quickly declined. I avoid medication unless absolutely necessary or given a proven diagnosis. I stated that I would never take meds for a guessed diagnosis and that I was sure these episodes were a result of autonomic dysfunction, or dysautonomia, which had also been diagnosed by my electrophysiologist. Dysautonomia is common with Ehlers-Danlos patients. He admits again he is not knowledgeable in EDS or dysautonomia. “Let’s just try another EEG.”

Keep in mind that there are 12 million misdiagnosis per year. Having a complex illness makes it tough to get proper treatment and management due to the lack of knowledge in rare diagnoses. As a patient, it can be hard to walk away because we feel almost desperate to get care but also hopeful that we will make progress over time; maybe the doctor will come around, research, or learn more. Starting over is time consuming and you already put in so much…just like a relationship. Sometimes hope keeps us there longer than we should stay.

Finding a specialist (there are only three clinics in the country) that specializes in Autonomic Dysfunction for me is a must. Looking back, the last two years was a waste of time. The last two years, my diagnosis was never understood by my doctor, nor will it ever be. I was nearly fitted into his specialty of seizures, only to be added to the 12 million misdiagnosed, because that was his specialty. That was what he was comfortable with. Any many patients would have trusted his judgment, taken the pills and felt they were being cared for. Not me.

As I leave from my EEG test, I know that it may be the last time I come to that office. I chose to humor my doctor and myself with the test because it never hurts to rule out a diagnosis (again). I know that months from now, I will travel outside of the state to see a doctor that truly understands what is going on with my nervous system. After spending my entire life having notes in my medical records of “unusual symptoms” that no one could piece together, for the first time ever I will have a doctor that has that missing piece of the puzzle.

Unveiling Invisible Illnesses

If I Only Had a Heart

The #myedschallenge for today is “if you could rid yourself of one EDS symptom, what would it be?

Without a doubt, I would rid myself of heart issues. I will gladly take the pain, muscle aches and spasms, joint hypermobility, subluxations, migraines, gastrointestinal issues, brain fog and so on. I have always known my life with heart issues.

I remember playing on the softball team in 3rd grade, complaining of my heart racing. “Keep running, kid. That’s normal,” my coach said. It wasn’t until I was 15 when we realized my heart rate was hitting the 300s and I had SVT and WPW. I remember being in my 20s and dancing with my friends, periodically going to the bathroom stall to slow down my heart. I just wanted to be normal.

Between multiple surgeries, hospitalizations, ER visits and doctors appointments, these heart issues have robbed me of so much of my life. These heart issues affect me every day.

My heart valves leak and have hypertrophied, requiring an inevitable 2nd open heart surgery one day. I have already had 4 cardiac ablations and refuse to do a 5th as it would make me 100% pacemaker dependent. My aortic root is dilating, a common issue with EDS. While I worry about my heart rhythms, blood pressure, arrhythmias, and valves, I also have worry about the possibility of aortic dissection.

I put my hand on my heart, and though battery operated, I am still so grateful that it pumps. I am grateful that I am here to be a mother and a wife, to share my story, to raise awareness, advocate and educate. I am grateful to breathe in the warm Florida air, even while my heart races and my chest aches. But what I wouldn’t do for a healthy heart….

Health, Unveiling Invisible Illnesses

I am not convinced

“I’m not convinced.”

Those were the words out of my decade-long relationship with my trusted electrophysiologist. I saw her on and off for 10 years during the moments I had insurance. I had 4 cardiac ablations for supraventricular tachycardia (SVT) from a congenital heart disorder called Wolff-Parkinson-White Syndrome, which is an extra electrical conduction pathway between chambers that cause arrhythmias. My heart rates would go up to 300s and drop down to the 30s. After four cardiac ablations and still having arrhythmias and fast heart rates, I could not take meds to slow down my heart since my rate would drop low too. I spent years in that position… in limbo without treatment and a chaotic heart.

My valves began to deteriorate as well, causing even more issues. I had open heart surgery for an aortic valve repair in 2011 and will need a replacement in the future, requiring open heart surgery again.

My trusted doctor, told me that it sounded like I was dealing with something that was too rare and not likely possible. She wasn’t convinced I could have another rare disorder. She denied me treatment. I was afraid to sleep at night, afraid that I wouldn’t wake up. Did you know you can pass out in your sleep? I finally collected my most recent 50 page heart event monitor report from the VP of the device company (my doctor would not give me the reports) and took it to another doctor. He ordered a Tilt Table Test and induced an episode and found that I had a severe cardioinhibitory response and confirmed that I needed a pacemaker, wondering why it took so long.

Two weeks later, my life changed. My heart rate doesn’t pause, stop or plummet and I can take meds to keep my heart rate from going too high. The pacemaker even kicks in to reduce arrhythmias.

The puzzle pieces all came together after seeing specialists and understanding why I was having a dysfunctional nervous system and irregular heart, chronic pain, chronic fatigue and an array of health issues. Genetic testing, research and being my own advocate helped more than anything. It took my entire life to get answers. I learned that I have Ehlers-Danlos Syndrome, a connective tissue disorder that causes many of my health issues on top of WPW Syndrome. Having WPW made is harder to see that something more could be going on because everyone was focused on that.

I never want anyone else to ever have to go through what I have gone through. I never want anyone else to be medically neglected, dismissed or too rare for their doctor to be convinced. There is an entire world of people suffering in the dark. My mission is to change that. I raise awareness for those people that feel alone, lost and ignored while they fear for their lives, praying to wake up the next morning.

Thank you for listening!

Health, Unveiling Invisible Illnesses

What is Ehlers-Danlos Syndrome?

Ehlers-Danlos Syndrome is an inherited heterogeneous group of thirteen subtypes in which abnormal collagen synthesis affects connective tissue: skin, bones, ligaments, blood vessels, organs and tissue. Collagen is a major structural component of the body. Danish dermatologist Edvard Ehlers recognized the condition in 1901. In 1908, a French physician named Henri-Alexandre Danlos suggested the features of the syndrome. Weak and structurally abnormal collagen may result in flexible and loose joints, poor wound healing, fragile blood vessels, ruptured or prolapsed organs, dysautonomia and many other various health conditions.

The inheritance patterns vary based on each subtype. Autosomal dominant inheritance means that just one copy of an altered gene can produce the disorder. For other subtypes, the disorder can be inherited from one affected parent and some even develop new gene mutations that occur with no family history. The gene mutation for COL1A2 can be found on chromosome seven, altering the collagen found in most connective tissue. This particular gene is associated with cardiac-valvular EDS, atypical Marfan Syndrome and Osteogenesis Imperfecta. There currently is no known gene linked to the most common subtype, hEDS; however, recent testing and research has been launched to discover the gene associated.

The launch collaborated with Ehlers-Danlos Society will collect enough data to gain more knowledge of this rarely diagnosed disorder. Different genes are associated with different subtypes and symptoms can range from mild to life threatening. 

For formal diagnosis, a referral to a geneticist is a start. There is test called the Beighton Scale, which has helped assess hypermobility since 1998. Medical history is also important for diagnosis. There is also a list of diagnosis criteria for each subtype that require a patient to meet a certain amount of history and symptoms. Vascular EDS can be potentially fatal, which makes proper diagnosis and subtype classification important.

Ehlers-Danlos Syndrome is not curable but symptoms may be managed with a team of specialists. It is a systemic disorder, meaning that multiple systems may be affected. Many patients see a range of specialists such as pulmonology, electrophysiology, cardiology, gastroenterology, neurology, pain management and so on. Specialists involved depend on the patient’s specific symptomatic issues that are a result of the connective tissue disorder. 

The current statistics for the more common subtypes are 1 in 2,500 to 1 in 5,000. Some of the rarer types only have a handful of documented cases. However, recent clinical studies show that EDS is more common and not so much rare as it is rarely diagnosed. This makes awareness, education and advocacy so important, in order to better understand and diagnose this disorder. 

 

 

 

 

References:

 

The Ehlers-Danlos Society. What Are The Ehlers-Danlos Syndromes? Retrieved from https://www.ehlers-danlos.com/what-is-eds/

 

National Library of Medicine. (April 2019). Genetics Home Reference: Ehlers-Danlos Syndrome. Retrieved from https://ghr.nlm.nih.gov/condition/ehlers-danlos-syndrome

 

Health, mental health, Unveiling Invisible Illnesses

Rare Disease Day

It is Rare Disease Day so obviously I am jumping on this moment to raise awareness. I have Ehlers-Danlos Syndrome and a rare type, called cardiac-valvular EDS or cvEDS.

Hypermobility is very common with EDS. There are many, many other health issues that fall under the umbrella due to this collagen defect. Imagine your joints are like rubber, frequently popping out of place from even just a hug or rolling over in bed. Sometimes these joints stay out of place or wear down. It is a painful disease to many.

This does not only affect joints but can also affect your organs.

We are all different and we call ourselves Zebras because in the medical field, doctors and nurses are trained that when they hear hooves to look for horses not zebras. This mentality has caused me to go undiagnosed and medically neglected for my entire life, up until I had genetic testing last year. Despite my heart issues and frequent ER visits, being young and seemingly healthy has had me labeled as drug seeking or having anxiety attacks.

The reason why is because EDS and dysautonomia (dysfunction of the autonomic nervous system) does not show up on routine blood work. I have never done drugs, besides cannabis, and even after open heart surgery and a broken sternum I did not even finish my pain meds prescription. I have been treated as if I were an IV drug user, because in my area that is the only reason someone of my age would have this extent of damage to their heart. I am so incredibly thankful to now be taken seriously with a diagnosis, but it is bittersweet because this syndrome is progressive and for me, my heart is always at risk. In my recent echo, I have developed a dilated aortic root. This is beyond scary to me because EDS, especially cvEDS comes with aneurisms. Dealing with this type of diagnosis as well as chronic pain and illness is mentally exhausting.

I am passionate about awareness is because it took so fucking long to be heard. I suffered for so long not taking proper care of myself and not knowing the correct treatments. I have been called a hypochondriac by exes and have hidden behind a mask for years. I want others to know they are not alone and I want medical professionals to see us.

Health, mental health, Unveiling Invisible Illnesses

Waking Up in Pain

Mornings are hard. When you think of Sundays, you think of sleeping in and waking up to the sun finding it’s way to to you. You think of sitting up, a nice stretch and a moment to admire the open window sharing hints of a beautiful day.

The reality is that you wake up from pain. And you have woken up several times already but you hurt too much to go back to sleep and the sun is up now, so you may as well get out of bed. Lying in bed hurts. It isn’t this relaxing thing where you can leisurely sprawl out in bed and feel like you are on a cloud, melting into your mattress. No, you have to move because one position makes your tailbone go numb and another hurts your collarbone and lying on your stomach makes your back feel broken.

So, now you get up and everything pops back into place. Almost everything. Your left hand and lips are tingling and numb but it only last a few minutes. You walk to the bathroom, holding on to everything you pass for stability so that you don’t fall. Even when you sit down, reaching to wipe is excruciating and demoralizing. It breaks you just glimpsing into the future, wondering if are going to need help wiping your own ass one day. Then, as much as you want to crawl back into bed and melt into your significant other, you quietly walk out of the room so you can find something to do and walk off the pain of sleeping.

Your head is killing you and you are nauseous as if you are hungover. As you walk to the kitchen, everything fades away and you can’t see. Your body starts to feel fuzzy and go numb, just like before you pass out. You don’t typically fully pass out so you know you can just keep walking through it as long as you hold on the way there. You are a pro and have smiled and held conversations while on the brink of passing out but you know it passes and this is your norm.

When pain levels are high, your morale is low. Your dreams and ambition slide over to the back burner. Sometimes they even get put away into Tupperware to decay in the back, hidden behind the fruit. Sometimes the sunshine creeping in through the window looks so far away. But you just get through this. You hold on to the idea of your next good day, whenever that may be. You find hope in new remedies and whatever ways you can try to have control over your health. You find hope in the people who support you and your loved ones.

*On the pain scale of 1-10, I have never been under a five. My one is a five. I am not wanting sympathy but what I am wanting is to be honest about how I feel instead of hiding with an autopilot response of “I’m fine.” I want to raise awareness for people like me so they don’t feel alone, and for people without chronic pain so that they can understand their loved one or friend. We live in a world of sucking it up and smiling through the pain. We are suppressed and depressed with a bottled soul. It isn’t right. Be real and let others be real so they they aren’t lost and alone.