Searching for a Savior- Part 1

I first started looking for a boyfriend when I was in third grade. I remember an elementary school dance was coming up, and I was describing my outfit to a friend. I explained that it was “short and tight.” She exclaimed how inappropriate that was for our age. I instantly felt ashamed. Why would I at such a young age want to draw the male gaze, to be the object of desire?

As I reflect on that exchange, I realize that as children, we are not capable of knowing how to get our needs met. What I wanted was to be loved, to be cherished, to be protected, to be valued. I had also come to understand that the ultimate goal of life for a woman was to be loved by a man, and to live to please him.

I also recall that I would fall in love with fictional characters, as I found them in books and movies. I didn’t understand why I had these feelings at the time, and wondered where they came from. But as I think over the collection that I daydreamed about, they were your classic “good guys,” and archetypal father figures. Characters like Atticus Finch in To Kill a Mockingbird, and Jean Valjean in Les Miserables, and so on. As I was going about my days I would imagine that they were looking over me, watching out for me, proud of me.

The way I searched for love, happiness, acceptance as I got older, my methods of trying to find it evolved. I ordered a book entitled “The Get Him System” out of the back of a teen magazine, though I was barely a teenager myself at this time. “Finally I can get someone to love me” I thought to myself as I waited the seemingly endless amount of time for it to arrive. I don’t recall many of the details of the book, but I do remember that it recommended that you smile at boys, and subtly touch their arm as they talk to you. This method proved challenging as I had begun to struggle with socializing with the kids at school. It had become apparent that I was a bit of a wreck, and no wanted much to do with me. This only compounded my loneliness. My search became more and more desperate. I hungered for love as ferociously as I craved oxygen. Like a drowning person, fighting to get a breath of air, I searched for this person that would care for me.

I became more and more aggressive. I tried to appeal to the lust that I knew that men had for women. I began to throw myself, and my body at the prospects that I found. When a guy I dated came over to my house for the first time, I showed him a page of a Cosmopolitan Magazine that depicted various sexual positions. I was surreptitiously suggesting that I would do anything he wanted if he stayed with me. I was in 9th grade. My ploy wasn’t successful, and he broke up with me soon after. He didn’t want to take advantage of my offer. As I look back, I can only think of how forward and inappropriate this must have seemed. I feel sorry for that frantically lonely girl that I was.

At a high school homecoming dance, I spotted my next target. This guy was a friend of a girl of circle of friends that barely tolerated me at best. As I pursued him, he didn’t stop me, and I took this to mean that perhaps he felt the same way. As the dance went on, I held his hand, danced with him, then finally worked up the courage to kiss him. I thought perhaps I had found my person. This person that I so desperately needed. My best friend informed me not much later that he didn’t like me. I felt so embarrassed and ashamed. I had made a fool of myself yet again. The next day was the homecoming parade. As I started working on our float, no one would talk to me. It turns out this boy was someone another girl was interested romantically. I was disgusting, everyone hated me, no one wanted me. I would have to keep searching.

End Part 1

Management of a complex, multi-system chronic illness

Hey everyone. In my attempt to raise awareness of this illness- hypermobile Ehlers Danlos Syndrome and all of its comorbidities, I wanted to document what it’s like to live with and manage this illness. Ever since I left teaching in disgrace due to this terrible illness, I’ve made studying it and managing it my life’s work. It has taken exactly ten years to amass these diagnoses that are listed below. The frustrating thing is that despite the fact that I know the illnesses that I suffer from, and work relentlessly on treatment, I am still extremely ill. Here is the list of diagnoses, symptoms, and treatments that I have put together since 2011 when I was first diagnosed with Myalgic Encephalomyelitis.

Diagnoses/Symptoms/Treatments

Diagnosis: Hypermobile Ehlers Danlos Syndrome (HEDS)

-Description: Heritable genetic disorder of the connective tissue

Symptoms/Comorbidities: Hypermobility, MCAS, POTS, Immune Deficiencies, Fibromyalgia, low blood sugar symptoms

Treatments: Gabapentin, Magnesium*

Diagnosis: Myalgic Encephalomyelitis/Chronic Fatigue Syndrome (ME/CFS)

-Description- Myalgic encephalomyelitis/chronic fatigue syndrome (ME/CFS) is a disabling and complex illness. People with ME/CFS are often not able to do their usual activities. At times, ME/CFS may confine them to bed. People with ME/CFS have overwhelming fatigue that is not improved by rest.

Symptoms: Extreme fatigue, systemic weakness, unrefreshed sleep, insomnia, cognitive impairment- “brain fog”

Treatments: See below

Diagnosis: Postural Orthostatic Tachycardia Syndrome (POTS)/Dysautonomia

Description:  is a blood circulation disorder characterized by two factors:

  • A specific group of symptoms that frequently occur when standing upright
  • A heart rate increase from horizontal to standing (or as tested on a tilt table) of at least 30 beats per minute in adults, or at least 40 beats per minute in adolescents, measured during the first 10 minutes of standing

Symptoms: Lightheadedness/rapid heartbeat upon standing, low blood pressure, low blood volume, frequent thirst

Treatments: High salt diet, salt sticks salt supplements, compression garments- socks and pants

Diagnosis: Mast Cell Activation Syndrome

Description: MCAS is a condition in which the patient experiences repeated episodes of the symptoms of anaphylaxis – allergic symptoms such as hives, swelling, low blood pressure, difficulty breathing and severe diarrhea. High levels of mast cell mediators are released during those episodes. The episodes respond to treatment with inhibitors or blockers of mast cell mediators. The episodes are called “idiopathic” which means that the mechanism is unknown – that is, not caused by allergic antibody or secondary to other known conditions that activate normal mast cells.

Symptoms: Hypersensitivity to meds, supplements, chemicals, foods, temperature/barometric pressure changes, light, stress, allergy shots

Treatment: Zyrtec, Chlortabs, Montelukast*, allergy shots, low histamine diet,

Diagnosis: Immune Deficiencies (Common Variable Immune Deficiency, Selective Antibody Deficiency, Hypogam), Chronic Epstein Barr Virus

Symptoms: Chronic upper respiratory infections, activated Mast Cells

Treatments: Cuvitru subQ immunoglobulin infusions, Valacyclovir, Azithromycin, Gaia Olive Leaf

Diagnosis: Asthma

Description: A condition in which a person’s airways become inflamed, narrow and swell, and produce extra mucus, which makes it difficult to breathe.

Symptoms: Trouble breathing, shortness of breath, tight chest

Treatments: Flovent inhaler, Levalbuterol Inhaler, Monteukast*, Magnesium*

Diagnosis: Vasomotor Rhinitis

Description: Vasomotor rhinitis is a condition in which the blood vessels inside your nose dilate. This swelling can cause nasal congestion and increased mucus drainage.

Symptoms: Swelling in airway- lungs and sinuses

Treatments: Flonsase nasal spray, Neilmed bottle sinus rinses w saline and baby shampoo

Diagnosis: Craniocervical Instability and Atlantoaxial Instability

Description: Craniocervical Instability (CCI), also known as the Syndrome of Occipitoatlantialaxial Hypermobility, is a structural instability of the craniocervical junction which may lead to apathological deformation of the brainstem, upper spinal cord, and cerebellum

Symptoms: neck pain, sleep apnea, facial pain, balance problems, muscle weakness, dizziness, vision problems, reduced gag reflux and difficulty swallowing

Treatments: Hard cervical collar, C-04 spinal fusion (12/21/2020)

Diagnosis: Headaches/Migraines

Description: Migraines are thought to result from changes in levels of brain chemicals in certain regions of the brain. Migraine episodes can be disabling

Symptoms: Nausea, vomiting, and extreme sensitivity to sound and light.

Treatment: Tylenol

*Medicine/supplement that treats multiple symptoms/illnesses

Vasomotor Rhinitis

Hi everyone! Happy Labor Day Weekend. I just wanted to share some of the health stuff that I’ve been working on. One of the major issues that’s been happening is that when the weather changes, it sets off all kinds of inflammation in my airway. From my sinuses all the way through my lungs.

So with the weather all over the place in eastern NC, my sinuses will swell up constantly and give me terrible headaches, like someone bashed my face/head with a mallet, and I’ll have trouble breathing. It’s crazy how sensitive I am. I’ll managing all day, then when the weather starts cooling off around 4pm, I’ll be so fatigued I’ll literally have to stop whatever I’m doing and lay down.

Montelukast and nebulized meds barely make a dent. Then all the swelling and stuff must set off my mast cells because I’ll feel tremendously weak, fatigued, drowsy, irritable, etc.
I saw my local allergist and asked for Dupixent, which is a biologic injectable med that helps calm down certain types of inflammation. He says I have to do a steroid inhaler first. So I’ll try that and see how that works.

I also have to go see an ENT to try to get the swelling in my sinuses under control. I’ve tried all kinds of nasal sprays, but nothing really touches that either. So I’ll see what they say.

I’m still working on restarting IVIG. It’s a long process to get it started bc it’s so expensive. So it’s lots of paperwork, and insurance to deal with. But my dr says once I’ve been on it for awhile again, it should help with the hyper sensitivity and inflammation.

My dr told me that I’m trying to do too much on my own and my GP should coordinating my care. It’s funny bc my GP is great, and does pretty much all the tests and meds that I ask for, but I’ve been at appts with diagrams and charts teaching him about hEDS. Then I go to all these specialists- allergy, ENT, pulmonologist, cardiology, endocrinologist and I have to teach them hEDS too. 🤯🤯🤯 That’s a lot for a sick person to do, y’all.

All the things that come with it

****Health update: hi everyone, I wanted to do an update on my health journey. I’ve been working with a slew of new doctors for the last year or so, since receiving diagnoses of hEDS, MCAS, POTS, asthma, two different types of immune deficiencies- SAD and hypogam, and CCI.

While I was thrilled to finally learn which conditions were underlying my decades long battle with illness, it created even more questions. The new highly specialized specialists often have theories about the issues that you are experiencing, so they order multiple tests and refer you to other specialists for things outside of their area of expertise. Few recent appts have nailed down solid answers, but require more tests, labs, and specialists. This isn’t uncommon for patients with complex chronic illnesses. I’ve started keeping a binder to organize the information related to my various health conditions.

Another difficulty is having so many doctors, and coordinating the information between them. For example, I have an Allergy/immunologists near NYC that I see remotely that is highly specialized in my constellations of symptoms. But to carry out the testing that this doctor wants done, I have to work with a local Allergy/immuno. The local doc is very limited in his knowledge of my conditions, and continues to insist that I invest $800 for a test for a tick borne illness, bartonella. He believes that MECFS is caused by bartonella. That’s a whole other issue, the medical community with their severe limited understanding of MECFS. This doctor is nice enough, his lack of understand of ME is a bit infuriating. But I need to work with him to carry out the testing that I need. I feel very confident in my diagnoses that took decades to get, and here comes this doctor insisting that it’s bartonella. I’ve been tested out the wazoo for bartonella, and other tick borne illnesses. If bartonella caused MECFS, wouldn’t the millions of sufferers have discovered this?

But, moving forward, the way that I understand MECFS, is that it’s a disease that revolves around the body attempting to maintain homeostasis. When someone either has a bad infection, or some other trauma to the body, the body in a certain percentage of people goes into a hibernation mode in order to be able to carry its most important metabolic functions in order to keep itself alive. Think of it like your phone going into low power mode. It’s trying to save its battery.

In many cases, it’s not one thing that causes MECFS. It’s a number of problems occurring and piling up, and causing the body to go into low power mode. The more issues that you can fix, the more you can convince your body that it’s not dying, and not only that it doesn’t have to hyper react to everything, but that it can turn off low power mode.

I’m still unclear about a few facets of my particular case. I know that I was born with hEDS, it’s a genetic illness. But I am unclear if I always had immune deficiencies, or did the immune deficiencies occur as my body rolled into MECFS.
I tend to think the former, as if I had a healthy immune system, I would have been able to suppress the mononucleosis infection like a healthy individual can.

Bill Reed, my NYC doc says that it’s my unbalanced, disregulated immune system that’s setting off the MCAS, or hyper sensitivity. I don’t completely agree that’s the sole factor. I think there are many issues that my body is struggling with, and is pulling me deeper into the illness.

Bill, myself, and my other doctors have formulated a plan that I feel really good about. 1) I’ve completed the lab work that Bill wanted. He was looking to see how my immune system is working now that I’ve been off of immunoglobulin for about a year and a half now. Many components of my immune system are lacking. I will likely need to start immunoglobulin replacement, this time intravenously.
Also, my progesterone and testosterone are undetectable. I’ve told doctors for years that my hormones are a major factor in this. As I felt 70% better when I was nursing my kids. My PCP was the first to listen and fully check my hormones.
2) I had to get my sinuses cultured, as it’s easy for bad bacteria to colonize in there bc my immune system can’t fight it off. 3) Once I’ve been swabbed, I’ll start preventative antibiotics to help my body fight infections that it can’t fight on its own. I also I to start putting antibiotic ointment in my nose for the same reason.
4) I need to find a decent local neurologist to look for small fiber neuropathy. Essentially my immune system has been so overactive that it’s been attacking my nervous system. This will also be treated by restarting immunoglobulin replacement.
5) Last In this plan I’ll restart allergy shots, as I’ve done better some allergy seasons while on them. More contacting old doctors offices and retrieving old records.
Oh, and last I was also recently dxed with asthma and started nebulizer treatments today. My doctor said my breathing test improved significantly after I did the breathing treatment.
This is what I spend most of my life on, you guys.

A Fall from Grace- Part 2

As I was explaining in part one of this story, when I was teaching, it was the first time that I felt like I was getting my life together. It was helping to build my previously non-existent self-esteem and confidence. It helped me realize that I deserved better than being abused and gaslit in a terrible marriage. I was able to get out, despite his lies to our friends, threats, and manipulation.

I decided to embrace this new empowered phase of my life. I threw myself into my work, that I had come to love. I began dating, which I was still terrible at- my self esteem clearly still needed some work. I started working out, eating healthier and dressing better. I asked my doctor for antibiotics to help clear up acne that had plagued me since adolescence, that I tried everything under the sun to clear up.

Not long after, I started noticed that I wasn’t feeling right. My stomach hurt, and I was having some trouble breathing. Like someone was giving me a great big bear hug around my lungs, and they couldn’t fully expand. I ended up going to the hospital, where they diagnosed it as an anxiety attack. I wouldn’t put it together until after I left the hospital that I was having a bad reaction to the antibiotics.

But even when I stopped taking them, the problems persisted. I started getting a terrible burning sensation in my chest, that radiated down my arms. It was alarming, because it was so painful and came in went in attacks. I couldn’t make sense of what was making the attacks come and go. It felt like I was being electrocuted, like I was hooked up to a car battery. I started being terrified of whatever was bringing the attacks on. I became convinced that it was something in my environment that was causing these symptoms. At this point, I probably really wasn’t acting like I was ok, especially at work. I had started seeing doctors, who were stumped as to what was going on. That led to referrals to specialists that I’d have to wait months for. I started missing work. I would hear from coworkers that others had mentioned that they thought I had lost my mind. And I kind of was. How could someone be ok when they would have awful attacks of feeling electrocuted almost daily?

I would be teaching classes where I would feel an episode coming on, and I’d have to do my best to not let it show that something terrible was happening to me. I’d be in the middle of a lesson and someone would come in the room to tell me something, or drop something off, and a part of me would wonder if they were there to help me. But that was ridiculous, I knew that no one could help me. I was alone in the nightmare, while everyone else was going on with their lives.

One day, at the start of a class, I had such a bad attack that I had to leave class and another teacher had to take over for me. This was starting to happen a lot. My coworker’s patience with me was starting to wear thin. I no longer felt that comradery with them. I was no longer part of this elite group that I had become so proud of. I was locked in this excruciating, lonely, terrifying unbelievable battle with this phantom.

While once attacks, the pain stopped leaving. It became persistent, and relentless. I got no break from the electrocution. I called my parents, who were in Florida and told them they needed to come, and quick. Something was seriously, seriously wrong, and I couldn’t manage it by myself anymore. They packed up, and made the 13 hour drive on that same day. We decided it was time for me to take a medical leave of absence from teaching. I would go to NY with them, and work with doctors there I hopes of getting to the bottom of this. I would have no way of knowing that this would be just the beginning of a decades long battle, that would reveal a devastating genetic illness that runs strongly in my family.

Please stay tuned for Part 3.

A Fall from Grace – Part 1

In honor of MillionsMissing 2021, I wanted to write of the way I slowly but surely lost my teaching career due to the scourge of ME/CFS.

When I began teaching high school right out of college in 2004, I did not really have any of the skills that I needed to be successful. I had virtually no self confidence, little self respect, or self worth. Needless to say, I did not have a very good start in the classroom. I was shy, and had a hard time gaining the respect of my students.

As I began to see what I was doing trying to befriend my students wasn’t working, I started modeling my teaching style after more successful educators. Their no-nonsense boundaries, discipline when needed, and passion for their their subject matter earned the respect of their students. As I adopted these behaviors, things began to change for me, not only in the classroom, but in my life.

My classroom was no longer the hostile environment it once was. There was no longer a power struggle, because it was clear that I was in charge. My teaching style became more interactive, and engaging. My passion for history began to shine through, and the students came along with me. They enjoyed the interesting stories, activities, music, and videos that I would make a part of my lessons. My coworkers began to feel more like family. It was the first time that I felt welcomed and valued in a family environment. Especially the history department, who worked closely together to make it one the best, and most celebrated in the state.

As my confidence grew as a professional, things began to change in other parts of my life. I found the courage to leave my extremely abusive marriage. I decided I deserved better than someone that was openly and relentlessly cruel, mocking, and belittling to me. When I finally got him out of my house, and we were formally separated, as North Carolina law dictates that you must be separated for at least a year before a divorce can be grated. I was on top of the world.

It was the first time in my life that I felt confident, proud of myself and my accomplishments, and free from wanton cruelty and abuse. I felt like this massive weight had been lifted, particularly escaping a man that hurt, and threatened me. The rush I got from teaching was like nothing else, the fact that I felt that students were learning so much from me, my coworkers valued me. I had never experienced anything like this before.

I began dating again, repeating an impossible to break habit of mine of inviting men that treated me badly into my life. A habit that I would determine that I would need profession help to shed, as I could not end this cycle on my own. And yet, I was back on the market, in search of the love and affection that I had always been in desperate search of.

As my confidence grew, I was also attempting to improve my appearance. I had been going to the gym with a friend to tone up, treated myself to some new clothes and makeup, and fatefully asked the doctor for some antibiotics for stubborn breakouts that I had struggled with since adolescence. Breakouts that I wouldn’t learn until decades later were due to an immune deficiency as part of an inherited genetic illness, Hypermobile Ehlers Danlos Syndrome.

The Journey Continues- Investigating Impaired Immune Response

Hello readers. I’ve been wanting to write an update of what I’ve learned in journey of my health. It’s difficult because I’ve been fighting a cold Mina gave to me for about a week, I just can’t seem to beat it.

I’ve continued to work with Bill Reed, Dr Maitland’s PA (who accepts insurance, thank goodness.) Due to work with him, and my own research, I’ve been able to continue building my understanding of my illness, and the way it’s played out over the last 20 years. Due to the genetic disease I was born with, I have always had an immune deficiency. That is why I became so ill when I contracted mono 20 years ago. My body didn’t have what it needed to fight the virus, and keep it suppressed like a person with a healthy immune system does.

The immune system is a lot more complex than I ever realized, and I still can’t claim to know all the ins and outs. I used to think there was over active and underactive. But people like me who have Selective Antibody Deficiency, have both over and underactive immune function. When faced with an infection, the part that needs to attack the pathogen is lacking, so there is no proper defense against the virus or bacteria. But in an attempt to compensate, the body deploys the immune response that it does have at it’s disposal. That part of the immune response is not equipped to fight the invader, but what it does do is attack vital organs, nerves, connective tissue.

So now, a person is fighting a pathogen, and the body’s immune cells begin to attack the body itself, friendly fire in a matter of speaking. The immune dysfunction also triggers the mast cells of the body. Mast cells are the alarm cells that are spread through every system of the body- nervous, digestive, urinary, circulatory, the skin. When the mast cells begin to overreact, they also begin to attack your body. You develop a hypersensitvity, and cannot tolerate foods, alcohol, medications, stress, weather and temperature changes, hormonal changes. It’s just such an unbelievable mess.

Bill Reed believes that it’s the immune dysfunction that’s making my body such a mess right now. Or, one of the main reasons. Hormonal changes seem to contribute to the problem immensely. I’ve explained to any doctor that would listen that when I was nursing the girls, allergy seasons did not make me intensely ill. Many dismissed me and told me hormones had nothing to do with it. Which is ridiculous, as a simple google search pulls up many credible articles that explain how hormones, particularly estrogen make allergy symptoms worse.

Anyway, it no surprise in many in the chronic illness game that so many doctors don’t know anything, and hormones can play a major role in these illnesses. The fact that I improved while nursing gives us some clues, but because nursing suppresses both estrogen and progesterone, we don’t know which one is aggravating my mast cells.

So here’s where my treatment has been going, I’ve added Chlor-tabs at night, an OTC antihistamine. I’ve started Nasalcrom, to calm down the mast cells in my nose and sinuses. I’ll be using nebulized Ipratropium bromide for asthma (we’re trying to narrow down the breathing issues between asthma or vocal cord dysfunction.) We’ll then take another run at the immune dysfunction after that. I was doing immunoglobulin replacement but it WAS THE WRONG KIND. HYQVIA was the brand I was using, subQ. which was wrong wrong wrong wrong. The Hy portion of the medicine softens the tissue to make it ready to receive the plasma, but you don’t give this to someone with a connective tissue disorder, who already has tendons and ligaments that are too lax. Also, I should have been doing intravenous, and not had it going under my skin.

Can you imagine what it’s like to navigate all of this? All while so ill that I can sparsely take care of myself, let alone two little kids that are home full time since the pandemic?

I’ve had to start working with my local allergist, in conjunction with Bill because Bill is in Tarrytown, NY and I do telemed appts. I go to my local allergy/immuno, Dr Lucas, for the hands on tests like allergy tests that require your physical presence. Dr Lucas is nice enough, and wants to help. But he’s not familiar with hEDS and the comorbidities that travel with it. That so many people with Ehlers Danlos Syndome also have ME, POTS, MCAS, SAD, ME/CFS. SO it’s up to me to teach him about it, to get him to believe me. He keeps suggesting that I get a $700 specialized Bartonella test (a tick borne illness.) I’ve been tested out the ass for tick borne illnesses, I explain in more polite terms. I feel confident in my diagnoses, I say, while screaming in my head that I know what I’m sick with. I speak as quickly as I can, as I know appt time is limited, trying to describe the connection between hEDS, POTS, MCAS,the whole freakin thing. And again, he recommends the Bartonella test. Somehow, I am a (desperately ill) patient who is teaching their doctor. Sending him articles and research, hoping I don’t cross the line and have him abandon me, as other doctors have done.

I drove home from an appt where I was frantically describing the inns and outs of hEDS, and was so sick and exhausted when I got home that I clocked myself in the face after accidently left the microwave door open as I cooked dinner. This to me, reveals the strange duality of patients like myself, with poorly understood, complex illnesses. Am I a teacher of doctors, or am I a patient? Why am I in such a position to be both?

On top of all this, I am still healing for the fusion surgery. The site it still swollen, I am told by other patients their swelling went down around the 5/6 month mark. I am 4 months out.

I try to bare in mind, the generations of people that had even less knowledge, less resources than we have now. They didn’t have patients communities on the internet, they didn’t have Dr Ron David at Stanford, they didn’t have MEAction, or Solve ME/CFS Initiative. We are building off of their knowledge, and their work. The books they published, like Osler’s Webb. I know that I’m riding on the shoulders of giants. I many have my own tiny little blog, and social media accounts, but I want to contribute in some meaningful way to this pool of knowledge, and this important work in hopes that future generations will not have to suffer as we do. With the fear, the sadness. the uncertainty, the dismissive or underinformed doctors. So with that, I continue. I will continue to write of my findings and progress as it unfolds.

A Twenty Year Medical Voyage

Long post. And I’m super foggy, so bare with me I’m proud that I’ve come a very long way in the last few years of coming to understand my very complicated illness. I ordered a book on it a few months ago, and it’s 700 pages. My body is still ailing, and also recovering from a major surgery. But I think of the decades I spent with no idea what was wrong with me, or how to help myself.

The knoweldge I have now is the culmination of two decades of terrifying, debilitating symptoms of many kinds that often came and went. I’d go through periods of time where I’d be bedridden/house bound, unable to walk across a room, to periods of time of feeling relatively normal and able to pursue other things I wanted in life. There were times I would think I was cured, only to have to bottom fall out, and end up completely debilitated again for reasons that I couldn’t make any sense of. This lack of stability wreaked havoc on my mental health, and made me absolutely terrified of everything. Even during good periods of time, I couldn’t relax and enjoy it completely because I knew it would probably not last.

I’m going to get into the specifics of illnesses like mine. This is a long post, so I appreciate if you’ve read this far. I write about my illness as my understanding of it evolves. So some of this I have described before.

I suffer from an inherited genetic condition known as Ehlers Danlos Syndrome. There are 13 different types, and I suffer from the Hypermobile type. It is ofte abbreviated as hEDS. As I have come to learn the wide spectrum of symptoms, I have realized this condition has plagued the health of my family for generations.

hEDS is a disorder of the connective tissues of the body. More specifically it affects your collagen, which your body, and every system in it made of. Collagen should be springy, and snap back into place like a rubber band, but faulty collagen like those that have hEDS stretches and doesn’t snap back as it should. It stretches more like silly putty. So just like a house that is built with faulty materials, it is only a matter of time before you will start to experience issues.

One of the issues that is easiest to spot in the constellation of symptoms those with hEDS experience is Hypermobility of their joints. The joints of the body move farther than they should, and often partially dislocated (subluxation) or fully dislocate while doing normal, and every day activities. An easy diagnostic symptom to spot is the ability to bend the thumb all the way back to the arm, and band at the waist and touch the palms of the hands fully to the floor. I recall in the 8th grade, I was dancing around with my friend, Gina, when my knee cap entirely dislocated. I popped it back in myself, and it was excruciating. I had absolutely no idea that it was the sign of a devastating genetic condition that would change the course of my entire life.

I was fully evaluated by an EDS specialist about a year ago, and she discovered that BOTH of my shoulders were dislocated. She asked me if I knew that my shoulders were dislocated. I had no idea. She asked me if I wanted the popped back in, and of course I did. I hear of other hEDS patients that fingers dislocate opening jars. Their hips dislocate climbing into bed (which happens to me on occasion) etc. This is a symptom of the faulty connective tissue that I described.

People with hEDS have comorbidities, or other illnesses that travel with this genetic condition. I will describe them here.

A frequent complication that comes with hEDS is a condition called Orthostatic Intolerance, or a more severe form of this, Postural Orthostatic Intolerance Syndrome (POTS.) It’s an intolerance of standing up, due to a curculatory condition. The collagen that comprises the blood vessels is too loose. When a sufferer goes from laying or sitting down to standing, all the fluids in the body does to their feet. Their heart starts pumping faster in order to try to keep their blood pressure from plummeting. When going to a standing postion, the person will feel light headed, and their vision may become cloudy, or they may black out all together.

One of the biggest and most debilitating and frustrating co-condition is called Mast Cell Activation Syndrome, or MCAS. Mast cells are large allergy cells that are ubiquitous in the body. They are often just under your skin, and are spread throughout your connective tissue. They are intended to maintain homeostasis in your body. If there is an injury, or pathogen, or toxin, the Mast Cells precipitate an action to correct the threat. For example, if there’s a cut or an injury, the mast cells release a chemical (called a mediator) to precipitate clotting. If there is a pathogen, the Mast Cells release chemicals to direct the immune system into action. If there is a toxin, or venom, the Mast Cells release chemicals that break down the affected tissue.

Mast Cells are certainly necessary, but the problem comes when the Mast Cells become over-reactive. As our world changes, and our bodies are dealing with many new chemicals in our environment, and our foods and water, these Mast Cells are activated and begin to negatively impact our bodies, and health. Especially for those that are genetically predisposed for Mast Cells conditions, like those with hEDS. An Infection, like I had 20 years ago, can activate the Mast Cells in your entire body, and make them overrreact to everything. Think of it like a car alarm that goes off every time the wind blows.

Once the Mast Cells are activated in a major way, it’s like Pandora’s Box and anything can stet them off. I would have zero clue why I would be so sick, after periods of being relatively symptom free. I have discovered the following things set off my Mast cells: infections, weather and temperature changes, hormonal changes, certain medications and supplements, high histamine foods and certain preservatives and food dyes, stress, and my biggest trigger POLLEN.

So for example, when Lilly (my oldest daughter) began daycare, the expose to the extra germs she was bringing home set off my Mast Cells in a major way, totally destabilizing by health at the time. It wasn’t just the germs my body was dealing with, it was the Mast Cells going crazy. The worst I’ve gotten it is when combinations of factors have occurred, like when I went through hormonal changes after giving birth, then having pollen season hit.

And Mast Cells are everywhere in the body. If they activate in your brain, you get brain fog, irritability, memory issues. If they activate in your gut, you get digestive issues. If they activate in your skin, you get hives, rashes, itching. If they activate in your nerves, you get neuropathy. I’ve had Mast Cells activate in different systems at different times, and sometimes everywhere at once. Many of the meds and supplements to calm and control the Mast Cells, you guessed it, they activate my Mast Cells. My body rejects them. Many of them have made me violently ill. Doctors are not taught about this, or barely taught about this, so sufferers are pretty much on their own when it comes to treatment.

Cervical spinal issues. Ugh I’m getting tired. Here goes. Your head is heavy. Picture like balancing a bowling ball on an upright baseball bat. You need healthy connective tissue, tendons and ligaments to hold it in place. If you have a connective tissue disorder, well, your head starts falling off. Pulled over years by gravity, your weak tendons and ligaments can’t hold it in the proper position. Also, your Mast Cells have been breaking down your connective tissue even further due to their over activity.
Your head gets pulled forward, so far forward that the bones and structures of your orthodontic cavity start crushing your brainstem and spinal cord (Craniocervical instability and cranial settling.) Because your those parts of your brain and spine control your autonomic nervous system, heart rate, blood pressure, urination, breathing, those systems start getting all the wrong signals, because your brain is getting injured from the instability and compression.

Where the ME (Chronic Fatigue Syndrome) plays into all of this, no one exactly knows. Your body’s failing and just starts to shut down. Any form of exertion, physical or mental, sends of farther into crisis mode because your body can’t produce the energy to sustain it. Your body’s gone into powersaver mode, because it knows it needs to store the last few molecules of energy it has for the basics, eating, bathroom, metabolic autonomic functions.

So, my body’s falling apart, my head (was) falling off. Every system is in crisis. Sounds terrible. And it is. But now I understand it. 20 years I’ve been at this. Doctors, research, activism and protests of the medical community, patient support groups.

There’s a path I see. My head and spine are fused. I will work on my immune deficiency with a doctor knowdgeable in hEDS, I avoid chemical and environment triggers, eat low histamine foods. Keep trying to tame those wild, misbehaving Mast Cells. Educate and create awareness of hEDS. And live my life somewhere in between.

An open letter to my ME specialist

Dear Doctor,

I am writing to let you know that I have been diagnosed with Mast Cell Activation Syndrome, Orthostatic Intolerance, and Craniocervical Instability, and am in the process of nailing down an Ehlers Danlos Syndrome diagnosis.

At my appt with you last summer, you told me there was nothing more you could do to treat me. I have suffered incredibly with illness since then. I decided to discontinue my work with you because I didn’t believe that nothing more could be done for me.

When I was your patient, I put my hope, faith, and trust in you. I spent thousands and thousands of dollars and drove 10 hours each way to see you each year. I feel that it was within your scope to either diagnose, and educate me with these conditions, or refer me to someone who would.

In the nearly five years that I saw you, I did not receive one referral, and you barely ordered any follow-up lab testing after my initial appointment.

I see in my notes from 2016, that you diagnosed me with hyper-mobility. You wrote in my notes, along with a laundry list of other things, but failed to explain to me that was likely a significant finding. You told me nothing of the possibility of EDS, didn’t recommend or refer me to a specialist.

At my first appointment in 2016, you decided against a tilt-table test. I have just been diagnosed with OI. I need extensive treatment to manage this condition that you didn’t diagnose, and told me nothing about.

When I complained of severe exacerbation of illness during allergy season, you chalked it up to “sensitivities.” When I described all of the issues that would ultimately be diagnosed as MCAS, you offered no help.

I asked you last summer of the possibility of cervical spinal issues, you did a minute or so of neurological tests, then said that you didn’t think I had that issue. Well, thank God I decided to peruse it, because I have been diagnosed CCI and need to wear a cervical collar to manage it.

You are supposed to be the very best in this field, but I feel that you did not do your best by me. It is through sheer grit, and determination that I have continued to survive with this cruel and until now undiagnosed and untreated collection of illnesses.

I have persevered and relentlessly pursued answers, along with the remarkable ME patient community. With these resources, I have finally managed to find the appropriate and dedicated level of care that I was previous lacking.

In closing, I feel that it is wrong to take the money of desperately ill individuals if you are unable or unwilling to do your best by them.

Thank you,
Lauren

Mast cell activation syndrome

I wrote this as an open letter to my girls in case they experience the health issues that I have, but I’m not around to help them.

Girls, a big thing that you need to know about that I’ve suffered terribly from is mast cell activation syndrome. Mast cells release histamine and 200 other chemicals when we’re exposed to allergens, pollen, certain foods, chemicals, etc. It’s not that we necessarily have too many mast cells, it’s that they overreact to triggers. Mast cells can be in your tissue, that’s why blood and urine tests can come up normal.

I’ve been really sick with this for decades, and only now in 2020 am starting to understand it. I’ve become severely ill on many pollen seasons. To the point where I go weeks, even months barely able to walk, think, take care of myself. I’ve seen countless allergists, but none of them have been able to diagnose me.

When I started investigating cervical spinal issues, I have leaned that there are a collection of diagnoses that go together. Hyper-mobility/ connective tissue disorders, mast cell activation syndrome, me/CFS, and spinal issue. Also pots, but I don’t have that.

Here are the symptoms that I’ve had that lead me to believe I have MCAS: severe illness during pollen season, but normal allergy tests. Photo sensitivity and flushing in my face, I’m completely alcohol intolerant, I’m sensitive to hot and cold temperatures, I have really low blood pressure. Also, I’m hypersensitive to many things. Meds, chemicals, foods. I can’t be around carpeting without itching like crazy. I have chronic sinusitis, and perpetually swollen lymph nodes. My hair is thinning and my face swells on occasion.

I did a 24 hour urine test for MCAS, and it came up normal, but I’ve learned this test is unreliable.

I’m still very sick right now, I weaned Mina off of nursing around December and got really sick when the pollen hit in March. I’ve noticed that hormonal changes and spikes in estrogen make my symptoms a lot worse.

Here’s the things that have helped: a low histamine diet, montelukast (prescription.)

I have just started a bioflavonoid called Quercetin with brolemain, it’s supposed to be very effective in stabilizing mast cells. It calms them the hell down and stops them from overreacting to everything so much. I’ve only taken it for less than a week now, but I am very hopeful that it will help.

Last thing for now girls. If you do have health issues, you have to be very careful about doctors. Even if you do see “specialists” a lot of them can treat simple versions of illnesses. I’ve seen half a dozen allergy/immunologists in the last decade, but none of them could diagnose me with MCAD.

When I started having severe neurological issues 10 years ago, I had an MRI and nerve testing, but couldn’t diagnose me with the cervical/spinal issues I have.

Even the ME expert I saw for five years, supposedly one of the best in the field couldn’t or suspected and didn’t bother to diagnose me with MCAS, a connective tissue disorder, and cervical spinal issues. He also charged me $10 a minute and I spent thousands and thousands of dollars to see him.

Patient communities are really the best resources for great support, and for gaining info and ask questions about your symptoms and diagnoses. I would have never known anything about the possibility of cervical/spinal issues if not for the ME/CFS online community. Be very careful to look at reviews of any doctor before you see them. I pray that you never suffer with the health issues that I have, but if you do, you need to know these things.