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Being “Built Wrong”

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Alexa Simmons was a 22 year old woman from Colorado who lived courageously despite all that Ehlers-Danlos Syndrome threw at her.   Ehlers-Danlos syndrome is a group of disorders that affect connective tissues, which are tissues that support the skin, bones, blood vessels, and other organs. Defects in connective tissues cause the signs and symptoms of Ehlers-Danlos syndrome, which vary from mildly loose joints to life-threatening complications.  Alexa fought EDS for over 10 year but finally lost the battle on October 6 2011. 

This is Alexa’s story in her own words.  She was a friend, a daughter, and a sister. She will be greatly missed and will always be loved.   She did so many wonderful and thoughtful things for so many aflicted with EDS and other chronic diseases around the entire globe — speaking as her dad … I am not sure whether I will ever fill the hole in my heart — but I will try as she would have wanted all of us to carry on –  She will continue to live on in all of us for the rest of our lives.  We love you — sweet Lexa Lou!

Foreword

Foreward

Scott Simmons (Alexa’s Dad)

The following story was written in 2010 by my daughter Alexa who passed away on October 06 2011 from complications due to Ehlers-Danlos Syndrome (EDS).  Most people do not even know that EDS exists and, for that matter, most doctors unfortunately do not understand it and often diagnose it as a psychiatric issue versus a disease/illness.  I hope this story increases awareness of EDS and related disorders – perhaps it will inspire people to understand that many diseases are not observable and that they need to be more compassionate towards others.

On her Memorial Page – we posted the following

“Alexa Simmons was a 22 year old woman from Colorado who lived despite all that Ehlers-Danlos Syndrome threw at her. She was a friend, a daughter, a sister and a zebra. She will be greatly missed and will always be greatly loved.

She is survived by her mother, father and sister as well as maternal grandmother and grandfather and paternal grandmother and an enormous number of friends.  She did so many wonderful and thoughtful things for so many afflicted with EDS and other diseases around the entire globe — speaking as her dad … I am not sure whether I will ever fill the hole in my heart — but I will try as she would have wanted all of us to carry on — best of love to Alexa’s relatives, friends and all she came in contact with.  She will continue to live on in all of us for the rest of our lives.  We all love you — sweet Lexa Lou!”

So – now on to Alexa’s story in her own words—I hope it gives you a new perspective on life and those who you love.

 

Alexa’s Story – In Her Own Words

Being “Built Wrong”

One Woman’s Struggle with Ehlers-Danlos Syndrome

(Written by Alexa Simmons – A Courageous Woman who left us too early)

            When I was born I had ten fingers, ten toes, and although a bit on the small side (at 5lbs and 19 inches) I was deemed 100% healthy.   Two years later my “baby” sister was born and we had the perfect family.  My parents fully expected the two of us to be able to enjoy all of the privileges of a modern day suburban child.  They didn’t just expect us to get to enjoy all of these experiences they KNEW we would.

            My parents were partially right about me getting to enjoy all the suburban experiences I deserved.  Today at age 21, while I have graduated high school with a 3.2 GPA, there are many things I have missed out on.  I didn’t get to go to homecoming or prom.  I have yet to experience my first boyfriend, date, or kiss and unlike many of my peers I remain a virgin. 

The majority of my life is lived from the four walls of my bedroom in my bed; I’m a prisoner in my own home due to my health.  I hardly ever leave the house because I’m too dizzy, tired, in pain, just plain sick.  I am “living” with Classical Ehlers Danlos Syndrome and many of its cohorts.  These related disorders include but are not limited to Dysautonomia, Chiari I Malformation, Tethered Cord Syndrome, and the list goes on.

            I did get to experience many of the suburban child’s pleasures…  I was in Girl Scouts, Indian Princesses, and was always popular having to decide one friend to have a sleep over with between two or more or more friends.   I will always cherish these memories of my “previous life”.

            My “new life” began in the second week of March 2001 –  It always astounds me how much of that last day I’m able to remember.  I can recall that it was a typical March day, snowy and foggy.  I can remember going to piano lessons with my little sister, and then stopping by Chipolte on the way home.  Most importantly I remember the beginnings of the stomach pains that would change my life irreversibly…  It was diagnosed as appendicitis.  And we were clueless at the time that the emergency appendectomy would be the end of my pain and troubles.

            From March 2001 to June 2001 I would not only undergo an appendectomy, but I would also have my gallbladder removed in May and my abdomen STILL hurt.  It was then that my doctor decided to perform an endoscopy; a diagnostic procedure where a tube with a camera on the end is inserted through your mouth and into your stomach.  My endoscopy came back showing that I had severe gastritis and an ulcer from all the pain meds I ate during the months that it took to diagnosis why I was in such agony.

            Treatment for my stomach was started tout de suite.  By mid June 2001 I was feeling better…  Not like I had felt before the mess in spring 2001 had started but I was back to playing outside and spent my summer with my friends at the local water park.  The agonizing pain was gone.  We had thought that the horror of spring 2001 could help to be put behind us with a trip to Playa Del Carmen, Mexico.

            However this good period would only be temporary.  In March 2002 I once again came down with the horrific abdominal pains, constant vomiting, and a severe headache that began at the base of my skull radiated up the left side of my face and down my neck.  I spent two weeks at the hospital having every digestive test done.  The doctors even had me undergo surgery again just to be sure they weren’t missing anything.  I was beginning to understand what it felt like to be a lab rat.  When the doctors could find nothing wrong they told us to go to the Children’s pain clinic but said they doubted anything would help as they felt the majority of my problems were psychosomatic.

            My mom did take me to the Children’s pain clinic and pretty much as soon as I walked out of the doors to go home I was a 12 year old zombie.  I would fall asleep no matter what I was doing…  My eighth grade class got to go see “A Christmas Carol” at the local theater and I slept throughout the entire show and on the bus ride home.  I was falling asleep in class and would forget to do the most mundane of tasks, such as taking a pizza out of the oven.  My mom knew they were not the answer and she began to take me from specialist to specialist to help figure out the source of all my pain.  We were drowning in a whirlpool of chaos, just when we were ready to let go a life raft was finally thrown our direction.

            I’d had a sleep study that came back showing “unspecified restrictive lung disease”…  This got me sent to a pulmonologist or lung doctor.  She had no idea at the time why my oxygen saturations were dropping at night but she did think she knew the source of my ailments.  She felt I was suffering from Chronic Fatigue Syndrome and she referred me to a doctor who just so happened to be researching chronic fatigue at National Jewish.

            As soon as this doctor walked into the exam room, there was a different aura surrounding him.  Immediately I knew that if he didn’t know what was causing me to feel so sick then he would dig deep to at least figure out a large chunk of my health puzzle.  While he didn’t feel that I had Chronic Fatigue Syndrome he did feel I was suffering from orthostatic intolerance or the inability to remain up right.  Orthostatic Intolerance is a hall mark symptom of dysautonomia (dysfunction of the autonomic nervous system).  Because this disorder affects your Autonomic System everything that the human body does that we don’t think about is messed up.  It can cause headaches, fainting, seizures, bowel and bladder issues, and the list goes on…  It was like that list of symptoms was made for me. 

            There was only one test to prove that I had dysautonomia…  A tilt table study.  During a tilt table study the patient is laid on a table at 180* for 30 minutes so the doctor can get a good baseline blood pressure and heart rate, they are then strapped to the table in case they faint and the table is tilted to 70*.  The table is to remain tilted for 45 minutes…  If there is any significant change in your heart rate or blood pressure while tilted then the test is considered positive.  My tilt table was scheduled for April 25, 2003; the day after my 14th birthday.  To me this day felt like judgment day.  Remarkably I failed with flying colors…  Within ten minutes of the test my blood pressure and heart rate had bottomed out and I fainted.

            While there is no cure for dysautonomia the symptoms can be treated.  I was immediately started on florinef (a steroid to help increase my blood volume), salt tablets (I’m supposed to get between four and ten grams of sodium a day), and potassium (as eating all of that sodium can deplete your potassium stores).  Within several days of this regime I felt better, while I was still in constant pain, the difference in how I felt was like night and day.  Shortly after my diagnosis this doctor moved on to the CDC in Atlanta….  Once again the psychological conclusions began all over again.

            On July 4, 2003 I passed out while riding my bike and broke my wrist.  My pediatrician had no idea how to handle my autonomic issues so she sent me off to see an adolescent medicine doctor; he immediately yanked me off my medications.  This is when my seizures began.  Every time I went to see this doctor he would lecture me on how common it was for a tall, skinny teenager to just faint and go into convulsions when they stood up.  He refused to even glance at my off the chart blood pressure and heart rate readings, and blew my tremoring off as a nervous habit.  The last time I saw him I attempted to throw a 64 oz. diet coke from 7-11 in his lap.

            In  July 2004 my family had no choice but to make the trek from Denver to Toledo, Ohio to see the wonderful wizard of Dysautonomia.  To many autonomic dysfunction patients Toledo is Oz and the doctor is their great and powerful wizard.  He felt that I was through the worst of it and I should be feeling 100% by my early 20s.  At this appointment he also diagnosed me as having a collagen defect known as Ehlers Danlos Syndrome, only he called it “benign joint hyper mobility syndrome”…  This did explain why I was so flexible, had stretchy skin, and suffered from joint dislocations.  The day after my appointment I was able to go to Cedar Point for six hours, riding the last roller coasters of my life.  Little did we know that by August 2004 I’d be using a wheelchair to get around.

            It was mid August 2004 and I was psyched to be starting my sophomore year of high school.  I had always been a great lover of school and learning.  On the first day of school I passed out twice…  It was then that the school gave us an ultimatum, either go on “homebound” or attend school in a wheelchair.  Me, being well me, I chose the chair…  I couldn’t imagine not being able to attend school again.  Of course I also didn’t realize how much more my health would decline during the year.

            I would miss chunks of two to four weeks of school at a time, and the truancy letters were piling up.  Many of those blocks of time that I would miss school it was due to the horrific “ice pick” headaches I deal with, or I was so fatigued the thought of getting out of bed took a Herculean effort on my part.  Many days were spent in the ER.  And due to a pair of inflexible teachers I nearly flunked two classes that were required for graduation (I squeaked by with a “D” in each class which were changed to Ps so it wouldn’t affect my GPA).  We knew it was time to start “homebound”…  I was devastated that I would no longer get to spend my days at school in classroom learning but even I had to admit that I could no longer “fake it”.

            I didn’t even bother to pop in at lunch to say “hi” on the first day of junior year in 2005…  I was heartbroken that we had to go this route but knew in my heart it was for the best.  I was on oxygen, and by now I had a hard time just sitting up without passing out.  Not only that but I was always tired and many days would be spent sleeping.  I did attempt to go to French class, but I’d always be lost by what the class was learning that day due to the chunks of class I’d miss at a time.  And I was struggling with English due to “brain fog”; it could take me upwards of a week just to turn out a three page paper. 

            And my doctors were perplexed by my consistent decline, which didn’t fit at all with my diagnosis of Dysautonomia as it typically is not progressive. Over Thanksgiving 2005 I was watching “Mystery Diagnosis” on the Discovery Health Channel when they began to talk about a young lady my age that had dysautonomia and Ehlers Danlos Syndrome (what the doctor in Ohio had referred to as benign joint hyper mobility syndrome) and she too continued to decline in health and had horrible headaches…  She could’ve even passed as my sister as we are both tall and lanky with long faces.  Eventually she ended up being diagnosed with Chiari I malformation, and Cranial Cervical Instability. 

The next day when the show reran we hooked up every TV to record this show.  My mom watched it and agreed that this girl’s story was pretty much identical to mine; we then sent the tape off to my primary care physician who also noticed the resemblance.  She immediately ordered a MRI of my brain and cervical spine.  The radiologist read the scan as negative but the area that really needed to be looked at to diagnose Chiari Malformation was obscured by my braces.  Still we sent the films up to a neurosurgeon that claimed to specialize in Chiari and had received several glowing reviews from my friends.  He called us almost two months after we had sent him my films.  He thought he could see an abnormality but we would have to have my braces removed and get new MRIs in Milwaukee for him to be sure. 

My braces were removed pretty much the day after that phone call and within two weeks we were on a train headed to Milwaukee.  The whole ride there I was in tears due to the pressure in my head…  When we had gone from Denver (the mile high city) to sea level my ears had never popped.  It was agonizing.  I had my MRIs done and the noise from the machine made my headache even worse.  I was in pure misery.  I laid on the floor of the surgeon’s office with my feet up on a chair so I wouldn’t pass out.  When this doctor came in he brought my films and pointed out several anomalies that could be fixed….  First I did have Chiari Malformation, along with an arachnoid cyst in my cerebellum and I had multiple bulging discs in my cervical spine at 16.  Before he would go in and operate he wanted to make sure my symptoms were coming from my brainstem compression caused by the Chiari.

I had an SSEP and BAERS to test my nerve conduction.  My SSEP came back very positive indicating there was a blockage very high up in my spine, which was caused by the Chiari.  The surgeon immediately recommended we go ahead with a Chiari Decompression.     

My surgical date was June 7, 2006 and I was nervous as hell…  My stomach was in knots. I was worried I wouldn’t get any relief from the operation and I was doomed to live a life of pain.  My parents tell me I was back in the OR for over 6 hours.  My Foranum Magnum was enlarged to make more space for my brain, scar tissue was removed, and part of my first vertebrae was removed as it was impinging on a major artery that was supplying blood to my brain.  As soon as I awoke the pain was excruciating but my hands and feet were warm and dry as opposed to cold and clammy as they typically were.

After my staples were removed we were given the “go ahead” to drive back to Colorado.  We got home on a Sunday and Wednesday I was in the local hospital with my now open wound oozing, and me running a 104* fever and in so much pain I couldn’t transfer from my wheelchair to the gurney without help.  This visit pneumonia was decided to be the cause of my problems…  They gave me a bag of IV antibiotics and sent me home with a z-pack and some percocet as I refused to go back into the hospital.  The next day I was back in the ER and this time I was diagnosed as having Bacterial Meningitis and a MRSA infection in my wound.  I spent an additional eight days in the hospital having another brain surgery to clean everything up, and then I went home on a PICC line so I could have antibiotics continuously dripped into my system.

As soon as I began healing from the infection that easily could’ve taken my life, I began to do things I hadn’t done in years.  I went to the mall and the movies without my wheelchair and I was feeling pretty good.  The autonomic issues were definitely still present but this was as normal as I would feel.  However I started having crazy high blood pressure spikes that led my doctors to think I had a pheochromcytoma…  All of my image studies were clean; I just had high adrenaline levels due to my autonomic issues.

Unfortunately the good times once again didn’t last.  My mom took me to see a neurosurgeon in Iowa who said I had Cranial Cervical Instability and he also wanted me to see his geneticist to confirm my diagnosis (although he knew as soon as he walked into the room that I had a connective tissue disorder.).  His geneticist did confirm my Ehlers Danlos Syndrome and told me I had one of the most involved cases she’d ever seen.  Nothing can be done for EDS…  I just treat the dislocations and sprains as they come along….  My elbow can dislocate just by me reaching in my closet for clothing.  The Iowa surgeon was going to do a Cranial-Cervical Fusion but ended up backing out.

We also found out that during the initial brain surgery a bovine pericardium patch was placed over my dura to allow more space for my CSF to flow better was recalled by the FDA.  Currently we are looking for a surgeon to help my head/neck by replacing the bovine patch as it was recalled and is most likely why I contracted bacterial meningitis.  And we also need a surgeon to fuse my skull and first two vertebrae together because if I fall (which I do a lot of as I faint) I could break my neck.

As I said at the beginning most of my life is lived from my bed and it is…  I’m in constant pain that even the strongest painkillers don’t touch.  Currently I’m awaiting a pain pump implantation in hopes that with less pain I could deal with my other symptoms better.  Recently I started putting my make up back on everyday as a way to cheer me up.  It’s worked greatly as now when I walk past a mirror I don’t just see a sick, pale girl with huge circles under her eyes but I see a pretty girl with ivory skin.    

Even if I get the neurosurgical help that I need I will always be “sick” but hopefully I will be able to ditch the wheelchair.  I hate the way people stare at me when I’m in a neck brace and wheelchair…  I would rather they just come up to me and talk to me, rather than stare like I’m some zoo exhibit.

It is so hard living in a small community with what is considered to be an “invisible disability”.  To most people there is no disability unless they can see it….  During my good period after my decompression I wanted to go into Costco with mom, so she parked in the handicapped spot as it was mid-July and I am very heat intolerant.  When we got out of the car we were accosted by an elderly man who said we took that spot from someone who really needed it, like him.  I’m standing there with an IV coming out of my arm and a large healing incision on the back of my head/neck which he could’ve seen as my mom had pulled my hair out of the wound.  If I was in a wheelchair at the time or missing a leg he wouldn’t have said a thing because my disability would’ve been clearly visible to him.

If there is one thing I wanted to get out by writing this it’s never give up when you feel something isn’t right with your health.  YOU know your body better than anyone else and doctors aren’t Gods.  If you see one doctor and you don’t like what you hear then move on to another doctor.  Get a second, third, hundredth opinion if you feel you need to.

 Also please remember that just because that person stepping out of the car parked in the handicapped space looks totally normal, don’t judge.  You don’t know the pain that they may be going through; their doctor gave them that placard to park there for a legitimate reason.  You can’t see a person’s blood pressure drop and their heart rate shoot up when they get up.    

And, for those afflicted, try to always keep the hope and the humor.

 

 

Closing Thoughts

Closing Thoughts

Alexa is now gone – she left our lives on October 6 2011. The 22 years went so quickly – and I think of all of the things that I forgot to say while she was alive.  What a tremendous child, daughter, friend and woman.  We were so lucky to have this gifted woman in our lives for 22 years – although the time was short, we know that we were lucky to know such a wonderful angel. Alexa was amazing in terms of reaching out to hundreds of other people afflicted with EDS – she touched so many people over her life via Facebook, Skype and other social media.  We miss her greatly – but we hope that this story helps to educate others in EDS and promote others to have a more compassionate attitude to the medically challenged. 

If you want to contribute to research on Ehlers-Danlos — we have created a memorial fund in honor of Alexa at the following URL:  http://www.firstgiving.com/fundraiser/InMemoryofAlexa/scottsimmonsfundraisingpage

In closing … I need to tell you that losing my wonderful daughter seems so unnatural — parents are not supposed to say goodbye to their children at the age of 22  – but the same time, we believe she is in a better place – free from the pain and suffering she lived through for the last 10 years.  Angie, Liz and I will heal in time as will all of her friends and relatives … our memories of Alexa will live on to the end of our days. 

 

To our Lexa-Lou – wherever you might be

You are so missed – there is an empty space in all of us that will not be soon filled

You were taken from us far too soon — but you touched so many in your 22 years

We bid you farewell sweet girl — you were an inspiration to so many –

We will love you forever

 

    19 comments

  1. justa335
    justa335 said:

    Thank you, sir, for sharing the story of your amazing daughter, Alexa, with us. No doubt she will continue to amaze and inspire others with this story on this site. Thank you once again.

    2 years ago
  2. Writer no longer with us said:

    What suffering and bravery Alexa conveys with her story. Thank you for publishing this, I feel privileged to have read it.

    2 years ago
  3. dtheherring
    dtheherringSupporter said:

    I’m very moved that this story is out there and Alexa’s life is part of the thousand threads that connect us all to the world.

    David

    2 years ago
  4. data
    data said:

    Thank you for publishing your daughter’s story. Her bravery if the face of so many difficulties is inspirational. I recall yet again, “There but for the grace of God go I.”

    2 years ago
  5. scottsimmons said:

    To all – -thanks for the comments — I am touched by them. It is odd – on one hand, I feel so touched to have had Alexa in my life for 22 years … and yet, I feel like she was taken far too early. I plan to do another version of this with pictures of Alexa and perhaps a longer segment from me on being a parent for a chronically ill child. My wife is an angel and was basically Alexa’s primary caregiver for the last 10 years … how she did it … I do not know — her doctors (for the most part) were useless and believed that their sole ob was to prescribe drugs and shuttle her to the next doctor (I have a number of doctors who would qualify for a kick in the chops) …. and, in the end, it is unfortunate as I really believe she could have lived longer with better medical care but that is a moot issue for Alexa. Once again — thanks for the nice comments.

    2 years ago
  6. phantazum
    phantazumSupporter said:

    I have read this 3 times and it still brings on the tears – but I am ashamed to confess that it is not the tragedy the story relates but rather the way it is written as it comes from the very deepest part of the human soul.

    2 years ago
  7. Joleen711 said:

    Dear Mr. Simmons,
    Your daughters story has touched my heart. A dear friend had shared some of Alexias story with me but this tribute to her, and reading her own words really brought me to tears. I struggle with many health issues including Chiari, TC, CND ( and more), and still trying to get back on my feet. Alexias story, I pray will touch and help many people.It has touched me. There are so many who do not have the compassion, love and support from family and friends. Alexia was so blessed to have you and your family by her side and her strength was incredible! My prayers go to you all as you continue to move through the healing process of loosing your precious daughter. I can’t even imagine. May you feel comfort and peace as you go through each day…one day at a time. God Bless You

    2 years ago
  8. littlekasino
    littlekasino said:

    Thank you for sharing this, I am ashamed to admit I ignored the existence of EDS. Alexa’s words didn’t only move me, but educated me too. Through her story I came to know a very brave woman supported by a very brave family. I hope many others like me can read this and be both touched and educated, so that nobody should face again the ignorance and stubbornness Alexa had to face in order to get treated.

    2 years ago
  9. Writer no longer with us said:

    Thank you for sharing this with us, no doubt the doing so was a double-edged experience, pain and at least something positive in the knowledge that your lovely girl’s story needs to be told. You have done that very well, both your own introduction and end piece, and you have presented your daughter’s words in a way that she will be proud of as she looks down with love on her family and friends, that is for certain. Again, thank you. JMH.

    2 years ago
  10. CaVenz
    CaVenzSupporter said:

    My heart goes out to you and your family and, of course, your daughter. Thank you.

    2 years ago
  11. Monica said:

    Like everyone else, I am extremely moved. What Alexa has written clearly shows how mature, patient and balanced she was. Pure grace in the face of so much uncertainty and pain. I have to add that Alexa was blessed with a wonderful family. I have known her father Scott for many years and think the world of him. Scott is one of the most dedicated and caring people I have ever known. He shared stories of Alexa with me, and even though I have never had the good fortune to meet Alexa, Angie or Liz, I know that Alexa was loved deeply by a wonderful family. Thank you for sharing this story Scott. It has changed a part of me.

    2 years ago
  12. Writer no longer with us said:

    Fortitude and courage shine through Alexa’s powerful literary voice, showing us just how talented she was. As a parent of a child with an ‘invisible’ illness who also died before I was ready to let him go, I empathise with Alexa and her family and feel honoured that I have been permitted to share her thoughts. I also admire Scott’s courage in sharing this with the world. Thank you!

    2 years ago
  13. pollykins said:

    Through tears
    Thank you for posting this about such a remarkable person.

    2 years ago
  14. Soulhaven
    Soulhaven said:

    Only Alexa could tell this story. An eye-opening and touching read.

    2 years ago
  15. chipsareonfire

    What an incredible and strong young woman. x

    10 months ago
  16. Celticlily said:

    Thank you for sharing this. Alexa’s words and strength are inspiring, I hope that though them she will continue to do good in the world.

    10 months ago
  17. PatriPoeSupporter said:

    Alexa exhibited wisdom and courage well beyond her years. What an inspiration and voice for the many people out there suffering from illnesses that are misdiagnosed. Thank you for sharing her journey.

    8 months ago
  18. adrian said:

    That was so touching and really brought tears to my eyes .
    Its like my whole world stopped for a moment!
    She was a brave girl.

    8 months ago
  19. Catgrass said:

    I’m so sad, shocked and amazed at Alexa’s story. What an unbelievably brave young woman. She’s lucky to have had such a wonderful family. I wouldn’t want to imagine what someone would go through with her health issues if they had an unsupportive family. You should be so proud.

    I’m waiting for genetic testing for ED myself but if I do have it it’s nowhere near as bad as Alexa’s. I gave my doctor an article about ED about 7 years ago to show her how my symptoms matched up. She just just kept it. My neurologist of 16 years didn’t know anything about ED aside from looking at a few tests of the Brighton scale so he didn’t follow up either. Now I can’t work but at least I made it to 46 – with 16 years of chronic pain 24/7, fatigue, TMJ, ulcerative colitis, etc mind you. I was finally diagnosed with fibromyalgia 5 years ago.

    My own father, a family doctor, constantly told me it was just stress. I can only be grateful that my condition, whether or not it is in fact ED, never approached the severity of what Alexa and your whole family went through. I hope you are done with tragedy in your lives. Wishing you all the best.

    5 months ago
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