A bit of an Update

It has been way too long since I’ve posted. I’ve been struggling with symptoms affecting both my mind and my body and I just haven’t had the brain power/energy to finish a post! My illness is a physical illness, but it stems from my brain and my autonomic nervous system so I have both neurological and physical symptoms, many of which are “invisible” to anyone who doesn’t know about them.

I’ve written before about how my Dysautonomia causes severe brain fog—this includes problems with word finding and sentence formation, short term memory loss, trouble focusing/short attention span, and a lot of day dreaming/zoning out. Right now my Dysautonomia is flaring because I had a virus and I’m not getting the full 2 liters of IV fluids I am supposed to get because of a back order that is in place due to the hurricanes that took out a major supplier in Puerto Rico.

Not only is this flare causing me to have extreme brain fog, but I’m having other symptoms as well such as falling asleep or losing consciousness while sitting or standing due to lack of blood flow to my brain. This is a common problem for those with NCS (one of the types of Dysautonomia that I have) but it is not only terribly annoying and embarrassing, it’s debilitating and limiting because I can’t drive or plan anything that involves standing or sitting for too long, and it’s hard to be around other people because I can fall asleep mid-sentence or even worse, in the middle of someone else’s sentence! Let’s just say I won’t be going on any first dates any time soon 😉

Because of my flare of Dysautonomia as well as an increase in severity of my migraines, I also struggle with overstimulation or hypersensitivity to sound, noise, touch, and smell. Overstimulation is something that a lot of people would think of in relation to autism or ADD in children, but it’s something I, as an adult, struggle with every day. Any loud or repetitive noises or bright, colorful, or flashing lights can send me into a terrible episode of overstimulation that leaves me in full body pain and spasms as well as with a migraine that doesn’t respond to medication. Some days my skin hurts to the touch like there’s a bruise spread across my whole body. Before my diagnosis my family thought I was just crazy and picky about noises, but now we know my brain really just can’t handle a lot of these noises, lights, etc.

Winter is always a challenge for me because I deal with intense pain flare ups due to the cold, lots of migraines, and my GI system always gets even worse than normal once I hit November/December, this year just seems to be throwing a few curve balls at me with the neurological symptoms being so significant on top of the normal flares.

Luckily I’ve learned how to adapt and work around most of these symptoms so I’ve still enjoyed getting ready for Christmas and our Christmas day was lovely and (relatively) peaceful. It’s so nice having my family home for an extended break—having company and my care team here makes things both easier and much more fun J

I hope to start being able to use my brain a little more so I can update on some more things and also share more about my Newbie Tubies Project and how I’m hoping to get that going by the New Year!

Thanks for reading, Happy Holidays!

Recovery and Discovery: A New Idea

My recovery process from having my new feeding tube placed (switching from a GJ to two separate tubes, a g and a j tube) has been really challenging. Due to some surgical complications and my connective tissue disorder, healing has been difficult and I’m still in a lot of pain. I’m lucky, though, because I have an amazing support team at home who are here for me and care for me no matter how long it takes; not everyone has that.

Because I’ve been having such a rough time healing and I’ve been in bed for so much of the last 4 weeks I’ve had a lot of time to think; through the online support communities I’ve seen so many people go through these diagnoses and tube placements alone. I just can’t stand to think of how terrible it must be to have to be your own support system in times like this; for two weeks I couldn’t even get out of bed or walk on my own, I still can’t bathe on my own or prep all my meds, feeds, and fluids. I’m dependent on my parents for almost everything, for individuals who have to have tubes placed and don’t have support systems and don’t know much about feeding tubes (who does if you’ve never had one, been on the online pages, or had a loved one with one?), this can be an extremely scary and challenging adjustment.

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My support system 😉

What I’ve decided to do is start an organization/nonprofit that sends packages to new tubies—people who are getting their first feeding tube placed—so that we can give them some comfort and some of the “tubie essentials” to get started with. This would include things like tubie pads, microwaveable heating pads, cute masks, pill crushers/sorters, journals to write symptoms in, allergen free, natural soaps, bath bombs, etc. I’ve compiled a list with more products, but we are looking for anything comforting for someone who just came out of a tube surgery (no food!).

Right now, this project is in the “just a dream/just getting started” period as we try to find people willing to donate products to our cause. We are asking small, spoonie geared businesses as well as local businesses who make things like soaps, hats, blankets, etc. So, if you have any interest or know someone who might, please let me know! There’s absolutely no pressure to donate, though!

I will also be putting the profits from my paintings into this project (once I turn a profit!), so if you’re interested in looking at my art, please do! It’s posted on my blog in the lifestyle section under “My Art” 🙂

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I wanted to share this with you all as it will be something I’m working on a lot for now, so I’ll try to keep you posted! This is a way for me to help others and be productive while hardly leaving my room—as long as we find donors! So thank you so much for reading and I can’t wait to see where this is next time I update you!

 

Recovery: The Real Challenge with Surgery

The past two weeks have been even more challenging than my “normal” for both my body and my mind. I had surgery two weeks ago to place a new/additional feeding tube and we ran into some challenges and now I am trying to heal and recover.

I’ve had to spend more time in bed since getting home from my surgery because I’ve been unable to move much on my own. For the first week I couldn’t sit up, stand, walk, sit down, go to the bathroom, brush my hair, or do anything for myself. Being 100% dependent on other people is really hard, regardless of the fact that I was already disabled and very dependent on my parents for so much even prior to surgery.

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Kevin needs his new tube placed!
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Baxter alerting me.

I have to admit that the first week or so post- surgery is all a blur. There was lots of pain, many doctors, a painful car ride home (or two), a lot of sleeping, medications, ambulance, pain, another ambulance, an awful ER, pain…. But what I do remember is that both of my parents were right there by my side the whole time. There was never any talk of hiring a nurse to do the hard work or asking another family member or close friend to come help so my parents could go back to work. Every day I had at least one if not both of my parents there taking care of me, no complaints or mention of using up their sick days.

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3 tubes?!

For the first many days I slept 20+ hours a day with the help of pain medication, nausea medication, and sedatives, which all together helped make me more comfortable. After my ER trip on Monday/Tuesday I stopped taking the heavy pain medications because they delay gastric emptying so I also stopped sleeping and instead started having major insomnia again. By Wednesday/Thursday I was starting to walk on my own and eventually getting out of bed by myself, too. Although I run out of energy quickly and my pain levels are still severe, every step forward is worth a celebration.

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Our one and only trick or treater 🙂 My favorite visitor!
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Always blessed and spoiled by my parents’ coworkers!

Having such a supportive and involved family made all the difference for me; I didn’t have to worry about keeping up with meds or finding a good nurse or anything because my parents and sister were on 24/7 “Rachel Duty” for as long as I needed them. We also have an incredible community that supports me by sending cards, flowers, and gifts but they also support my parents at work and through facebook and texts/calls of support and well wishes. Being the parents and care takers of a young adult as sick as I am is no easy task and it’s extremely important to have that support.

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Mom sleeping in the ER.

Surgery is tough, but recovery is hell. Waking up each morning in major pain and knowing it’s not going away isn’t easy on anyone physically or mentally, but each small improvement or sign of progress gives me hope. Life is precious and every day that your body is functional and pain free is a gift; I encourage you to take advantage of every day and live life to the fullest, always follow your heart and do more of what makes you happy. Find joy in every day.

Xoxo

A Day with Dysautonomia

I’ve been dealing with dysautonomia since I was a teenager, really even longer before my diagnosis in high school. It started out with shortness of breath and heart palpitations before moving into full on syncopy and fainting spells and eventually complete autonomic dysfunction. Today, although my dysautonomia is in many ways managed better than it has been before, it still affects my body and routine every day.

Although my fainting spells have been minimized by doing twice-daily saline infusions, if you look deeper you find that my dysautonomia is still very much present. I struggle daily with common symptoms like dizziness, accelerated heart rate, and fatigue, but I also have symptoms that are less talked about.

I struggle with temperature regulation, being unable to stay warm if I go anywhere with a temp under 65-70 degrees, even if I’m bundled up and only there for a couple of minutes; this includes grocery stores, cars, outdoors, and my own house during the winter. Something as simple as changing my clothes can send my body temperature drops as low as 91 degrees F – hypothermia is 95 and below. I joke about my “hibernation” during winter, but it’s partially true, being that cold and unable to warm up is not a fun –or safe– feeling. On the other hand, if I’m fatigued or talk too much (no joke), I run low grade fevers and have to put myself in a “time out” to let my body rest and recover.

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I also struggle from severe adrenaline rushes. My blood work shows high norepinephrine and dopamine levels which you would expect from someone who is overly excited or even scared, maybe from sky diving or being in a high speed car chase? Well, I get them from standing up or over exerting myself. When your body has to work extremely hard just to keep you on your feet, it sometimes goes into overdrive. These adrenaline rushes either leave me hyper during the day or up all night, but either way, I’m exhausted and weak when they’re over.

My dysautonomia has also contributed to my digestive tract failure and my chronic pain. Although I have other conditions as well, these are all comorbid and interact with one another making it harder to treat. I’m on 24/7 nausea medication and daily pain medication as well. I struggle with daily migraines and occipital neuralgia. I rely on tube feeds and IV fluids to keep me nourished, hydrated, and able to stand up without passing out.

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Although there are many other symptoms I struggle with, these are just a couple that are currently having a big impact on my life.

Dysautonomia is an umbrella term for a multitude of conditions and needs more research and awareness. There are way too many people suffering from this condition who deserve treatments and a cure, but we have none.

To each of you reading this, thank you. Please continue to read, share, and educate others on these conditions so that one year we can write about the cure.

Why I Continue to Avoid TPN as a Young Gastroparesis and Generalized Dysmotility Patient

I’m about to hit my 3 year mark of being tube fed. I never would have imagined that I would be 21 and fed through a tube in my gut, but it isn’t my last choice as far as alternative nutrition options go.

I’ve been asked and offered many times to go (back) on TPN, or total parenteral nutrition, which is nutrition that goes straight into your veins. This option gives you full nutrition—protein, fats, vitamins, etc—and can be tailored to your exact needs.

Because my intestines and colon are delayed (they don’t process food or feeds at a “normal” rate), I don’t get in enough feeds to meet my calorie goals and I haven’t been able to gain back any weight. My BMI is considered extremely low and some of my doctors would really like me to go back on TPN. For those who don’t know much about TPN and for those who have never had to make the choices I have, I’m going to try to explain to you why I – as well as many of the other girls in the same situation—want to avoid TPN for as long as possible.

Although TPN is complete nutrition, and it probably sounds like a great option to many of you reading, it comes with many risks. TPN requires you to have a central line or a long term IV that goes deep into a large vein and then straight to your heart. These can get infected easily and lead to sepsis, which if not caught in time can be life threatening.

While tube feeding is much more natural and forces your GI tract to at least try to function, TPN leaves the GI tract to shut down completely. For someone with dysmotility (lack of movement), this can mean there is little to no chance of returning to tube feeding or eating if another treatment option becomes available after they start TPN.

TPN also puts you at a higher risk for glucose abnormalities and liver dysfunction. The damage to the liver can be so serious it can cause you to be unable to run TPN or even require a transplant if not caught in time. While on TPN, you are required to do weekly blood work, blood sugar monitoring, and weigh ins. TPN can also cause volume overload, metabolic bone disease, and reactions to lipids (fats) such as nausea, headache, back pain, sweating, and dizziness.

So there are many, many undesired side effects from TPN. But aside from side effects, TPN is scary because for those of us with gastroparesis and intestinal dysmotility, TPN is our last option. To go on TPN means to admit that our intestines are no longer functional enough even just for tube feeds. It means we can’t eat, we can’t tube feed, and we may not return to either.

Yes, some people go on TPN and come off of it able to tube feed or even eat again. Some people only use TPN to supplement their tube feeds or oral intake. Everyone’s case is different and TPN helps so many people live a more “normal” life because it does provide full nutrition; it can boost your energy and help you regain strength and muscle that is lost from malnutrition. TPN saves the lives of many starving patients with gastroparesis and generalized intestinal dysmotility.

That said, it doesn’t make it any less scary. Losing the ability to eat is one of the most confusing and complicated things you can imagine. Going from eating orally to being fed through a tube is one of the strangest and hardest adjustments I’ve had to make, but knowing I could lose the ability to feed even through a tube in my gut is even harder to accept.

TPN is a miracle for so many people, but it is also a nightmare for many of us. It’s what can help us live, but also what can put our lives at risk. I’ve been on TPN before, and I fight my body (and sometimes my doctors) every day not to go back to it. You can’t understand what it’s like to go through this until you’ve been the patient, but I hope that everyone—doctors, nurses, family member, friends—can try to understand how hard it is on the patient to make these decisions and all someone needs during that time is support and love.

Learning to Live in Today

This week, while the class I began school with started their fourth year of college, I started my third year of medical leave.

People often ask me if it makes me sad to see posts about college or to drive through grounds when I’m headed to the hospital, but mostly what I feel is disbelief. How has it been so long? Does life really move on so easily without me? Will I ever get to be “normal” again? Do I even know what that means and could I return to it if I tried?

At a young age we start to understand that our lives follow a guided path; sure, everyone’s is different and we all stray from that path at times, but in general, it is set up for us. We grow up being loved and cared for, we learn right vs. wrong, we go to school and hopefully graduate. From there, you either get a job or “further your education,” aka more school. Some people get married, some have children, some do neither. The order isn’t the same for everyone, but we all make plans and in general, most people end up following some variance of “the path,” as I’m calling it.

Well, my path was altered in high school. I got (really) sick when I was about 16. It took me years to get real answers, in reality, I’m continuing to seek more answers to this day, but since getting sick, my life has been anything but “normal.” I spent time on homebound from high school, I did one year of college before withdrawing on medical leave, I’ve lost countless friends because of these illnesses, and I’ve lost any firm perspective on what my future may hold. However, I’ve also grown and become a stronger and wiser person.

Do I wish I were starting my fourth year with my friends right now? Of course. But I’ve learned that we can’t always predict where we will be in four years or four months or even four days…

You don’t have to fit anyone else’s mold. Yes, go to college; study whatever you want! Or, take a gap year. Travel. Volunteer. Be an actress, an athlete, an architect, a doctor, a musician. Be a stay at home mom, a stay at home dad. Be you.

Most importantly, don’t hold back. Splurge where ever you can, big or small. Do all you can to live in the moment and enjoy every possible second. Today, right now, is all you have. Now don’t go spending your family’s life savings on lottery tickets or a trip to vegas using the excuse “Rachel told you to,” but buy yourself something you’ve been wanting when you get your paycheck, just because you earned it. Take your parents or your family out to eat just because they deserve it. Do something just because it makes you or someone else smile, do it just to make memories.

Life is beautiful, but it is short and unpredictable. Throw caution to the wind and always follow your heart.

Battling Gastroparesis: Happy Awareness Month!

In December of 2013 I was diagnosed with gastroparesis. Like majority of people, I had no idea what that was or what the diagnosis meant for my future.

When I got my diagnosis I was given minimal information about the condition, and because I didn’t know what it was back then, I didn’t know I wasn’t getting the full picture. I was told I had delayed movement in my stomach, it wasn’t emptying food like it should be. They told me gastroparesis is a chronic condition but since mine is what they call “idiopathic” or without findable cause, it was likely post-viral and would go away within a year or 18 months.

This discussion, my original gastroparesis diagnosis, lasted only minutes, and I was left to figure out most of it on my own. My parents and I left that hospital thinking I just had to get through this flare up and then it would hopefully go away for good. I had no idea on that day 4 years ago just how much gastroparesis was going to change my life.

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Some more info about GP– symptoms especially! And yes, that is my tube and a donut tubie pad!

Since my diagnosis in 2013, I have had countless tests and tried endless treatments, medications, diets, and therapies. Gastroparesis is extremely difficult to treat and there is no cure. In 2016 we found out that my dysmotility (movement disorder) had moved into my intestine and colon as well, so that became a major complication. Luckily at this point my parents and I had become experts on my conditions; after my original diagnosis we started to learn how to do our own research, we joined online support communities, and we went to see specialists who could give us more information about my conditions and prognoses as I was diagnosed with more conditions down the road.

The journey you go through when living with gastroparesis and generalized dysmotility is extremely taxing on both your body and mind; it’s exhausting and disappointing to try and try again and often get little to no relief. But, we have to keep trying in hopes that one day we will find the right treatment and hopefully a cure.

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As part of awareness month, I want to give you a glimpse at what it’s like going through testing and treatment with gastroparesis, so I’m going to list some of the tests, procedures, and treatments I’ve tried over my time with GP.

I was originally diagnosed with an Upper GI series, an endoscopy, and a 90 minute gastric emptying scan. Since then, I’ve had 3 more 4 hour emptying scans, multiple endoscopies, countless EKGs, lots of ultrasounds, endless x-rays, a breath test (SIBO), esophageal manometry, anal manometry, smart pill test, CT scans, MRIs, and so much more. And these are only the tests that have to do with GP—not my other conditions.

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I’ve tried physical therapy, cognitive behavioral therapy, acupuncture, dry needling, chiropractors, and essential oils. I try keeping up with walking and core strength and I keep a positive mind set—no one can tell me I’m sick because I’m depressed! 😉 I’ve even read mindfulness books and watched documentaries on how to “heal your body,” although I wasn’t 100% sure about that one!

When I was able to eat, I’ve also tried a lot of diet adaptation. I was on the BRATS diet, low FODMAP diet, gluten free, dairy free, a gastroparesis diet, a liquid diet more than once, and I’ve been on both TPN and tube feeds. As of now I am completely dependent on my feeding tube for nutrition and my port for IV fluids daily.

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I stared with a picc line for TPN, then I had an NJ tube for a trial run with tube feeds before quickly deciding to have a GJ tube placed surgically in March of 2015. Since then, I’ve had my tube changed out over 26 times in IR due to either clogs, having it flipped into my stomach, or just needing a new tube (every 3-5 months). I also had a port a cath placed in August of 2016 and have that accessed 24/7 for fluids and nausea medication.

I won’t even list all of the medications I’ve tried because that’s just a ridiculous number and I don’t think you or I have the attention span for that. But you name a motility agent, a nausea med, a non FDA approved trial drug for GP, or pretty much anything used to control symptoms or promote motility or hunger and I’ve almost definitely either tried it or discussed it with my doctors and ruled it out as an option.

 

Over these (almost) four years I went from being able to manage my symptoms with a specialized diet and nausea medications to not being able to eat at all. Gastroparesis and generalized dysmotility are cruel illnesses, and paired with my genetic condition, Ehlers Danlos Syndrome, they’re relentless and grievous. My condition has progressed to a very serious level and I’ve tried most of the options available to me.

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Learning to embrace my medical devices 🙂 there should be no shame or shyness towards life saving devices like tubes and central lines!

Almost a year ago I saw my motility specialist at Cleveland Clinic, a man who is considered to be the best of the best, and he gave me three options. We’ve ruled it down to one option, but sadly insurance isn’t thrilled with it. 9 months later and we are still fighting for it. But we won’t give up. Gastroparesis won’t win this fight.

Happy Awareness Month! Keep your eyes out for more posts from me and others as we try to spread awareness and work our way towards a much needed cure!

Xoxo

R

 

Desensitized to The Diagnosis

Yesterday I got a new diagnosis. But in all honesty, a new diagnosis doesn’t phase me much anymore. When I was 16 I got my first diagnosis, Postural Orthostatic Tachycardia Syndrome (POTS), but I had no idea that 5 years later I would have more diagnoses than I can count on both hands.

In 2016 I was diagnosed with Ehlers Danlos Syndrome, a genetic condition that affects connective tissue and collagen in your body. For me, this diagnosis explained a lot. I had already been diagnosed with Dysautnomia/POTS/NCS as well as Gastroparesis and generalized dysmotility in my colon and intestines. I had been struggling with unexplained chronic joint and nerve pain and I finally had an answer; although EDS wasn’t an easy answer and it meant I will likely never be completely free of illness, I finally had answers.

You probably won’t understand this unless you’re chronically ill, but after receiving that diagnosis, the ones that followed haven’t been surprising to hear or hard to accept. EDS has a lot of co-morbid conditions, and as mine has progressed and as I’ve seen more specialists, I’ve collected a nice array of conditions. Because I know my illness and understand what it can cause, I’m prepared for all of the co-morbid conditions it can bring on.

It’s never good news when I get another diagnosis, but I like to think that a diagnosis simply means we are moving forward towards treatments and answers, it doesn’t actually change anything symptom wise. I’m the same as I was before the diagnosis, I just have more answers and another syndrome/condition on my records. Usually a diagnosis actually brings me more relief because doctors actually start treating a conditions once it has a label and is no longer just unexplained symptoms.

I don’t mean to minimize the severity or seriousness of chronic illness; every illness I have I take very seriously and we treat each to the best of our ability. But after being sick for so, so long and being diagnosed with so many things, there is a desensitization to the process. Maybe it’s a protective mechanism, a coping mechanism, or maybe it’s just because it becomes your life, but just because I’m progressively ill doesn’t mean I have to let each diagnosis set me back.

A Battle With The System: Fighting For Treatment

Nine months ago my motility specialist gave me three treatment options. My digestive tract paralysis had progressed from my stomach into my intestines and colon and there just isn’t much they can do for that.

Option one– a specific medication –was quickly ruled out due to risks with another condition I have and the third option is not doable either, so we were left with one option.

Our one treatment option was IVIG therapy, or IV immunoglobulin therapy. This is a treatment that focuses on rebooting the immune system and can sometimes help reset some of the issues with the central nervous system. It’s used to treat immune deficiencies and other conditions that can lead to a weak immune system. For me, the goal is to boost my system in hopes that my digestive tract will be positively affected. There are no guarantees and it’s only about a 50/50 chance that it would make any difference at all for me, but it is our best and only real option right now.

It’s been nine months since we put the prescriptions in for that and I’ve been denied by insurance twice. My illnesses aren’t on their list of conditions that require IVIG for treatment and each round of IVIG costs $10-15,000, so it’s not easy to get approved for patients like me.

That said, this is my only option for treatment that may help me improve, not just keep me comfortable. Even if all it does is help me tolerate my tube feeds better and have less pain or nausea, it would be a huge victory. This is what my doctors think I need. So being denied the opportunity to try it is really upsetting; sadly, we see this happen a lot in the chronic illness community.

Our medical system is a money making business, so a lot of medications and treatments take pre-authorization, out of pocket co-pays, repeated appeals, and some are not covered at all. But for those of us with severe, chronic and progressive illnesses, this can make it hard for us to live any semblance of a “normal” life.

I am so thankful to have good health insurance, but the hoops I have to jump through and the delays in my care are extremely frustrating at times. My parents and I spend hours each month calling the insurance agency and calling doctors and pharmacies to advocate for the treatments I need. I’m lucky to have people who fight for my care when I’m not strong enough to do it myself, not everyone is that blessed.

If our doctors prescribe us a medication or treatment option that they think is vital to our health care, insurance agencies should not be so quick to deny it. The lives and well being of patients should be the first concern of every part of our medical system.

A Word From Many: Ehlers Danlos Syndrome

As part of Ehlers Danlos Awareness Month I asked a large group of women with EDS to describe their journey with this condition in one word. Whether it be their most common symptom, an adjective that explains how their life has been affected, or an emotion that describes what EDS means to them, I just wanted to hear what EDS is to each person. I took all of these words and put them together to share in hopes that they will show how brutal EDS can be to so many people, but also to show the incredible strength that it brings to it’s sufferers and as a reminder that no one fights this alone.

So again, these words come from over a hundred different women– not just me! Many of the words were suggested by more than one person; the most commonly used ones are in the largest print. I don’t share these seeking pity, but because those living with EDS live complicated lives full of so many symptoms, emotions, and stressors that go unseen. Awareness month may be over, but every day we will continue to fight for better treatments, more awareness and understanding, and we will continue to fight for our lives.

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