Like any marriage, we’ve had our rocky moments. We’ve both showed our ugly sides more than we like to admit. I’m not sure when he changed, but somehow along the way in our 27 years of marriage, my husband morphed into this amazing man who is EXACTLY what I need in every way!

My husband doesn’t have the same responsibilities that most husbands have. He works full-time to provide for his family and does more than his fair share of household chores. But my husband has a wife with several chronic conditions. He has a wife that has been in some level of pain almost every day for over 18 years! He’s seen me through ten surgeries since we’ve been married, eight of them being in a five-year period. That is enough to exhaust the strongest of men, but exhausted or not he has stood by my side through all of it.

I first became symptomatic following a car accident. We were both 29 with three young kids (ages 2, 5 and 8). I was initially diagnosed with double whiplash, but when that failed to get better, I was left with no reason for why I couldn’t hold up my head. It felt like my neck lacked the strength to hold up my head for more than an hour at a time and on some days, not even that. To add insult to injury, I went without a diagnosis for over a decade. It couldn’t have been easy to stay by my side when all the doctors were saying that they couldn’t find anything wrong with me.

Over our decade without answers, I continued to decline. I started having severe cognitive decline and memory loss. I’d have periods of time where I lost my ability to walk and use fine motor skills (so I couldn’t write or pick up small things). He helped me walk to my classes and even helped me do my homework when I couldn’t write. He didn’t have the answers, but he knew that I desperately needed them, and he was determined to stay by my side even though by doing so it was putting an unfair burden on him. He didn’t have the help-meet that he needed in life. He just had this sick, scared wife, with no means to any end to her suffering.

When I was finally diagnosed, the choice for decompression was an easy one. It was 2010 and I was now 39 years old. I had lost 10 years and the possibility of getting my life back and engaging as a wife and mother again was something that I couldn’t pass up. We never expected this “easy surgery” to take the turn that it did. We knew that there was a chance that it would be unsuccessful at relieving all of my symptoms, but never in a million years did we think that decompression would open Pandora’s Box on my need for surgeries. From 2012-2015 I needed seven additional surgeries. Each surgery had its own recovery and complications. And with each additional surgery, I became increasingly dependent on my husband for help, but not once did he complain. This was affecting his life too, but not once did he concentrate on that. Not once did he think of it as being unfair to him to have to care for me, even though it really was. I’m now covered with scars on my left side from my neck to my groin, one night he raised my pajama shirt and gazed intensely upon them. He started kissing them. When he got to the scars covering my stomach, I pushed him away and with tears in my eyes, I told him that I wasn’t comfortable with him touching me there. He looked me in the eyes and told me that he didn’t see me the way that I see myself. He said that when he sees my scars, he’s reminded of all that I’ve had to fight through and what a strong woman he’s married to. “I wish that you could see yourself the way that I see you,” he exclaimed. All my insecurities just melted with his words of affirmation.

This man who vowed to love me for better or worse, richer or poorer, in sickness or in health, really meant it, and those vows were continually put to the test every day of his life. And he wasn’t seeing the polished-up version of me that others tend to see. He saw the frustrated me, the defeated me, the hopeless me. The me that went from one pair of pajamas to another. The me that gained over a hundred pounds on nerve meds that ultimately made me worse. The me that started to stutter and slur and hated myself for it. The me that drooled in front of people and whose nose ran uncontrollably when I sat down to eat. The me that lost control of both her bladder and bowels and that had to wear a diaper for years. The me that no longer could wipe herself or shower without his help. The me that took so much of my pain and frustrations out on him, when he’d take all of it upon himself in an instant if he could save me from it. Not only does he not complain or criticize, but he became my biggest cheerleader in life!

He listened to me as I read studies to increase my knowledge on what I’m facing. He lets me yell to him about the incompetency of doctors. He’d make small jokes to help me make light of the seriousness at hand. When he forgot things, he’d say that he was having a “Chiari moment.” I’d remind him that he wasn’t the one with Chiari and he insisted that it was sympathy pains. When he developed a herniated cervical disc and we looked at his MRIs together, the first words that came out of his mouth were, “well, I don’t have a Chiari Malformation,” as he pointed at the cerebellar tonsils. I often feel so unworthy of his love, yet if you talked to him, he’d tell you how unworthy he is of my love.

There’s no denying his real role in my life; he’s my hero!

*This article is dedicated to my husband, Johnny (my hero and the love of my life) and to all the other couples trying to hold a marriage together through this crazy fight we face.  

They keep telling me I’m a blessing.

That I’m lucky to be alive.

That although I’m sick I’m blessed to be here every day.

I’m blessed to spend time with my kids.

And although people tell me this everyday like it is some sort of affirmation, I don’t feel blessed.

I don’t feel blessed when I walk to my car and my heart beats so hard and so fast that I feel like I’m the star of the movie Blow.

But I am always reminded at least my heart beats… a blessing.

I don’t feel blessed when my five-year-old rubs my back every day as I throw up every speck of food I ingest.

But at least I have food…. blessed.

I don’t feel blessed that I can no longer provide for myself financially because even getting dressed is a chore.

But at least I have people whose lives I can greatly burden with my illness…. blessing?

I don’t feel blessed when I hear my beautiful kids playing and I can’t drag my body out of bed to play with them.

But at least I have my hearing so I can listen…. blessing?

I’m often frustrated that I have to take 9-15 pills a day, as I throw up the sour taste of the meds.

I don’t feel blessed, but I’m told I am because I have access to health care.

I’m supposed to feel blessed when my angels fall asleep in the car, but I don’t because I’m 99% sure carrying them in the house will kill me.

All these blessings but everyday feels like hell.

I’m alive. I’m breathing. But I can’t touch any of the things that make me happy.

All these blessings and all I can think is that I’d trade all these blessings in for one last day.

Give them all up, just for one last day that I can feel normal.

That I wake up and I don’t want to scoop out my eye balls and pull out my brain just for a little relief.

That I wake up and feel energized. That I can cook my kids’ breakfast and smile and laugh.

That I live just one more day without this sickness.

I’ll trade all my blessings just for one day of no disease.

One day for my children to remember me laughing and hugging. Not vomiting. Not crying. Not laying on the floor asking them to quietly play around me because my head will explode.

I don’t want my blessings anymore.

They’re supposed to be some beautiful gift from God.

I can still see and hear all the things and people I love. But I cannot participate.

And somehow that’s a gift.

And I cannot be appreciative of them while they rob me of who I am.

I’m sick of this disease called a blessing.

 

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I’m in an abusive relationship. It’s not a romantic one at least, not in the traditional sense of the word.

When I fell in love with her, she reminded me of a goddess. She was beautiful and kind. She never took no for an answer. She was unstoppable. She was an inspiration. To me and to everyone around her. She made me feel the highest high.

As time went on, I watched my goddess like creature slowly turn to stone. She suddenly became cold. She wanted me to stop caring about everything: my friends, my family, my hobbies, my career. She just wanted me to stay inside with her and listen to her. She wanted all my attention.

It started with bashing other people. Telling me why they didn’t deserve my time. She blanketed her insecurities by saying I was better than dealing with other people’s problems and to put myself first which really meant her and her illnesses.

Once I isolated myself from the people that I once would have laid my life down for, she turned it on me. There was no more self-love. Instead she instilled self-doubt. I wasn’t good enough to have friends and family, and love and happiness. I deserved her miserable and toxic company. All this emptiness was my fault and now I must live with it and her forever.

After she attacked my mind she started on my body. I had bruises everywhere. She’d trip me and push me into the walls. I was so sore and tired. I couldn’t eat. I lost so much weight.

All the confidence she once gave me was gone. I didn’t care anymore what I looked like. I didn’t do my makeup or change my clothes for days. Why should I? She is the only one who loved me and now she’s turned on me. I’ll stay in my sweat stained shirt forever. Maybe it will keep her away from me so I can be in peace.

It didn’t. Clothed or unclothed, it didn’t matter where I was, what time it was, or who I was with. She was always in my ear whispering about how much I’ve changed and how ashamed of me she was. Her presence made me vomit. She gave me headaches that lasted for days. I wanted to kill her.

I started to hate her. I hated her for making me hate myself. And every time I would get close to telling her to stay away from me forever, I would get flashbacks of that goddess. The girl who laughed in the face of fear. The girl who made me feel the best I’ve ever felt. I know she’s still there. I can’t give up on her.

When did she get so callous? Maybe if I can track down what triggered her abrupt change, I can help her get back to herself. What made her so abusive?

I need her to get back to who she really is. Who is she anyways? She is me. I am her. She is the reflection in the mirror that I refuse to look in the eye. She is my body before and after this disease. She is my greatest love and my mortal enemy. But can I live without her? How can I end this cycle? I can’t. I can’t walk away. I must continue to be destroyed and only hope that goddess will reappear. That her glow will radiate into my soul and warm me. That I can look at her and see love and not despair; that I can love this broken diseased soul that makes me who I am.

 


FOR ALL MY NON-CHIARI FAMILY MEMBERS AND FRIENDS… I really NEED you to listen to this and just try to understand! 


I know there’s absolutely NOTHING I can say to help you understand the pain that I go through; what it feels like to have pain all the time, in places that I didn’t even know that I could have pain in. To feel like someone is grabbing the back of your skull at your neck and literally trying to pull your skull right off of your head. I know there’s NOTHING I can say or do, to tell you how discouraging it is to have doctors tell you that they have “no idea why you’re hurting so bad,” and suggest that you should see a therapist, because obviously, if they don’t see it, it must be just psychological. And while I know your intentions are good and I know that you love me, I CANNOT think of anything to say to help you understand how incredibly hurtful it is to have those that are supposed to love you the most, those shoulders that are supposed to be there for you to lean on, tell you that you’re having a pity party, or  how they “wish you wouldn’t talk about it all the time” (and sometimes it’s not said that nicely either). And for MY KIDS and HUSBAND to have to carry so much on their shoulders and to feel like they have nobody to talk to about it, because everyone’s “tired of hearing about it.” And worse, since it’s genetic, for them to see how you treat me in my pain, and fear that if they’re diagnosed, that you will treat them the same.


You don’t understand, but my brothers and sisters with Chiari do. They understand it all, because they’re living it too. I know you don’t understand the bond that we have, but even though we’ve never met face-to-face, we’ve cried so many tears, and prayed through so many surgeries together; we’ve lost friends together while doctors still try and claim that Chiari can’t kill you (even with death certificates that say Chiari in hand). We’ve lost friends that just couldn’t take the pain anymore, and we cry together because we know that those thoughts have been our own and we struggle to find a way to keep pressing on, together! I know you don’t understand the bond, but it’s real.


I’m not saying all this to make you feel bad, God forbid you feel bad, I’m saying this because I STILL NEED YOU. You weren’t put in my life by mistake and what I’m going through physically and mentally is frightening and heart-breaking and I need you here by my side. You think you don’t have time to see me through all these surgeries and diagnoses, I don’t either. There’s so much more that I wanted to do with my life and now I just want to hold my head up without pain. I NEED you to change your heart towards me and all that I’m going through. I need you to call me, and just love on me. I need you to remind me of all that I am, despite my pain – that I’m stronger than I often feel like I am. I need you to remind me that there is still value to me still being here on earth. Because in those dark moments, when I look at all I’ve lost and everything that my family has lost through this fight, I need to hear it in someone’s voice besides my own. I need you to remind me how important it is that I fight this vigilantly, so that if God forbid one of my kids have to fight this fight, I will be the best possible advocate I can be for them. They’ve missed out on so much with me being sick. And should my fight on earth come to an end, I need you to stick to them like glue, and help them know how fantastic they are and that they’re not alone. Remind them that their stronger than they think, just like their warrior mom! Remind them that they have a purpose and a destiny to fulfill on earth, and to not to let anything stand in the way of that. If they get knocked down, pull them up again, every single time! Don’t worry, I’m not planning on going anywhere, this isn’t a suicide note or anything. I still have every intention of changing this fight of mine and winning it. But it’s hard sometimes and I really need you to fully understand how much I NEED YOU! Help me win this!


***Michelle originally wrote this on Facebook in 2016, with tears streaming down her face. The response from the Chiari community was astounding. What was astounding was not that so many liked it, but that so many Chiarians resonated with the heartbreak of it. It has been slightly edited for publishing. It was originally written with explicit language, that we at Chiari Bridges felt was a “most accurate” representation of the raw emotions that so many of us feel when we encounter these types of struggles in our family dynamics. However, this “clean” version is being created, for those to that are not comfortable sharing content with “strong language,” but still relate to the article and wish to share it.

The original (explicit version) of this article.


FOR ALL MY NON-CHIARI FAMILY MEMBERS AND FRIENDS… I’m going to cuss, but I really NEED you to listen to this and just try to understand:


I know there’s absolutely NOTHING I can say to help you understand the pain that I go through; what it feels like to have pain all the time, in places that I didn’t even know that I could have pain in. To feel like someone is grabbing the back of your skull at your neck and literally trying to pull your skull right off of your head. I know there’s NOTHING I can say or do, to tell you how discouraging it is to have doctors tell you that they have “no idea why you’re hurting so bad,” and suggest that you should see a therapist, because obviously, if they don’t see it, it must be just psychological. And while I know your intentions are good and I know that you love me, I CANNOT think of anything to say to help you understand how fucking incredibly hurtful it is to have those that are supposed to love you the most, those shoulders that are supposed to be there for you to lean on, tell you that you’re having a pity party, or  how they “wish you wouldn’t talk about it all the time” (and sometimes it’s not said that nicely either). And for MY KIDS and HUSBAND to have to carry so much on their shoulders and to feel like they have nobody to talk to about it, because everyone’s “tired of hearing about it.” And worse, since it’s genetic, for them to see how you treat me in my pain, and fear that if they’re diagnosed, that you will treat them the same.


You don’t understand, but my brothers and sisters with Chiari do. They understand it all, because they’re living it too. I know you don’t understand the bond that we have, but even though we’ve never met face-to-face, we’ve cried so many tears, and prayed through so many surgeries together; we’ve lost friends together while doctors still try and claim that Chiari can’t kill you (even with death certificates that say Chiari in hand). We’ve lost friends that just couldn’t take the pain anymore, and we cry together because we know that those thoughts have been our own and we struggle to find a way to keep pressing on, together! I know you don’t understand the bond, but it’s real.


I’m not saying all this to make you feel bad, God forbid you feel bad, I’m saying this because I STILL NEED YOU. You weren’t put in my life by mistake and what I’m going through physically and mentally is frightening and heart-breaking and I need you here by my side. You think you don’t have time to see me through all these surgeries and diagnoses, I don’t either. There’s so much more that I wanted to do with my life and now I just want to hold my head up without pain. I NEED you to change your heart towards me and all that I’m going through. I need you to call me, and just love on me. I need you to remind me of all that I am, despite my pain – that I’m stronger than I often feel like I am. I need you to remind me that there is still value to me still being here on earth. Because in those dark moments, when I look at all I’ve lost and everything that my family has lost through this fight, I need to hear it in someone’s voice besides my own. I need you to remind me how important it is that I fight this vigilantly, so that if God forbid one of my kids have to fight this fight, I will be the best possible advocate I can be for them. They’ve missed out on so much with me being sick. And should my fight on earth come to an end, I need you to stick to them like glue, and help them know how fantastic they are and that they’re not alone. Remind them that their stronger than they think, just like their badass mom! Remind them that they have a purpose and a destiny to fulfill on earth, and to not to let anything stand in the way of that. If they get knocked down, pull them up again, every single time! Don’t worry, I’m not planning on going anywhere, this isn’t a suicide note or anything. I still have every intention of changing this fight of mine and winning it. But it’s hard sometimes and I really need you to fully understand how much I NEED YOU! Help me win this!


***Michelle originally wrote this on Facebook in 2016, with tears streaming down her face. The response from the Chiari community was astounding. What was astounding was not that so many liked it, but that so many Chiarians resonated with the heartbreak of it. It has been slightly edited for publishing. What was astounding was not that so many liked it, but that so many Chiarians resonated with the heartbreak of it. It has been slightly edited for publishing. It was written with explicit language that we at Chiari Bridges felt was a “most accurate” representation of the raw emotions that so many of us feel when we encounter these types of struggles in our family dynamics, and therefore the decision was made to publish it in its raw form.

We have published a “clean version” of this article.


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During my pregnancy I remember being very unwell, writing in my journal, “there is something wrong with pregnancy, I know something is wrong with my baby”, at only 10 weeks gestation; proving that a mother always knows. At around 30 weeks gestation my unborn baby started to get chronic hiccups followed by trembling, lasting several minutes and several times per day. I begged doctors to run further testing, pleading something was wrong but it all fell on deaf ears. Baby Eric’s heart rate was strong and steady, my fundus was measuring right on time with gestation, so my concerns were dismissed. At 36 weeks gestation, I started having consistent and lasting contractions. After several hours I proceeded to the ER department, concerned I was in preterm labor. Stress tests were run, and the nurses dismissed me, asking me to come back the next day for a follow up. While running a stress test the following day after food, juice and changing positions, the doctor was called as Eric didn’t have enough variables in his heart rate. I can still hear the Doctor yelling at the nurses asking why I had been sent home the night before demanding them to get on the phone with the hospital in the next community and to make sure a neonatal team was on standby, because they would be expecting a new Mom tonight. I was terrified, as all my worst fears were coming true. I was transported immediately, where I was met by a huge medical team. Everything was moving so quickly, myself and my partner were in shock and terrified not knowing what would come next. After an emergency ultrasound, Doctors explained that we had a very tiny baby with a great deal of amniotic fluid and something was seriously wrong with my baby; exclaiming that an emergency C-section would have to be performed. At this point we didn’t understand what this could mean, or why I had so much amniotic fluid. This was why my fundus was measuring well and everything went undetected. My partner and I were living every parent’s worst nightmare. Thinking back, I recall crying and holding my boyfriend’s hand as they carefully lifted a very tiny completely blue baby hand from my stomach. When we all saw my breathless baby like a flip of a switch the operating room became chaotic. Doctors were in a mad rush trying to resuscitate my baby, who had no vitals and wasn’t breathing. I was hysterical, so the doctors sedated me. My boyfriend was becoming increasingly agitated, so they demanded that he leave. I can still hear the nurses voice yelling at him, “you are taking up time we need to save your baby, you need to trust us and cooperate, OUT”, before the anesthesia flushed into my system.

After several hours I regained consciousness, family and doctors surrounded me trying to explain what they knew about baby Eric. They explained Eric and I would have to be immediately flown out to a larger center where they could properly care for my baby. Eric couldn’t support his own airway, he couldn’t suck, swallow, or move due to hypertonic muscles. He was having several seizures, all pointed to brain damage. At this point they had no idea what was wrong and could not conclude whether my baby would live. I recall saying I wanted to go see my baby and trying to get up out of the hospital bed, but the nurses told me I couldn’t see him, because I had just gone through a major surgery and he was too sick; we both had to rest for transport. When the nurses told me that, I flipped, telling them that come hell or high water, I was going to see him. I got as far as swinging my legs over the bed before I vomited everywhere. The nurses finally clued in that I was going with or without their help, so they laid me back down and wheeled me to the NICU to see him. I couldn’t even see my baby for he was so small and leads encased his body where he layed. They had his chin strapped up as every time it fell backward it would close off his airway leading to oxygen desaturation. They didn’t have the resources to intubate a baby at this hospital, so they decided to fly us out to Vancouver Children’s Hospital.

The nurses and doctors kept me sedated for the first 4 days of being in Vancouver as every time I came to, I would start screaming for my baby and they would find me wandering the halls trying to find my baby. On day 4, they finally let my partner wheel me down to the NICU to see him. It was the scariest place I had ever seen. Rows and rows of incubators filled with tiny preemies with all kinds of machines keeping them alive. The nurse told me Eric was the biggest baby they had at 4 lbs. 11 oz. This gave me false hope, believing if he was bigger than all the others, he must have a fighting chance to survive. After several days of Eric in the NICU covered in wires, leads, and intubation tubes we still had no answers as to why our baby was born so ill. Eric was having up to 35 or more seizures per day, he was poked and prodded many times a day and underwent several tests; spinal taps, MRI’s, CT Scans and had several neonatal experts tirelessly working to find the cause of his many issues. Any parent who has been through this knows and understands the emotional roller coaster that comes with this experience. One day they tell you that your baby is improving, and they say he will beat this battle, and the next you’re told he wouldn’t survive the next 24 hours telling you to say your goodbyes; waking up all hours of the night, if you sleep at all, to pump your milk for a baby that isn’t there. You are walking to the hospital in the middle of the night just to be next to him because you can’t bear to be away. You are begging, pleading to God for your child to be healed. After 3 weeks they finally let me have skin to skin cuddles and started teaching us how to care for him. This isn’t the way it’s supposed to go; you’re supposed to go to the hospital, have a baby and take them home, right? Instead you walk around numb, you don’t remember the last time you ate, or showered, you just feel like you’re having an out of body experience, as if you’re watching this happen to someone else. You’re scared to leave their side for even a minute because what if it’s that minute that he leaves this world and you’re not there?

We wouldn’t allow for anyone else to care for him, we did all his range of motion therapy, we did his bath time, and we did all his personal care alone because if a nurse or doctor touched him in a clinical manor he would be riddled with more seizures. After a month, the nurses started insisting we start taking better care of ourselves, so my boyfriend and I started alternating night shifts. My nights alone with him were my favorite, I would hold him all night and rock him. He would look up at me with his deep blue, soulful eyes and tell me all the secrets of the world. He had the oldest spirit I had ever saw, full of wisdom and love.

After about a month we had a meeting, and it was then that the doctors told us we had a failure to thrive baby, his cells didn’t migrate to the right place at the right time, that his cerebellum and brainstem were severely underdeveloped. The statistics 19 years ago was that one out of one million babies were born like this and they had yet to find out what caused the illness. Our baby couldn’t regrow the parts of his brain that didn’t develop. He had gestational arrest at 32 weeks, he had scoliosis, spina bifida occulta, epilepsy and severe brain damage. Our child would never move on his own, suck or swallow or be able to interact and that he would most likely die of aspiration pneumonia. He would live his life in hospital more than out. I have worked with special needs adults with this type of quality of life, I could play the tape to the end, and I knew hanging on to him would only be for selfish reasons. I understood that my baby was in pain, I could see in his beautiful blue eyes, as I said before, a mother always knows. It was at this time Eric’s father and I decided to sign a” Do Not Resuscitate” order. It was without a doubt the single hardest thing I have ever done.

We called our family to let them know of our decision and if they wanted an opportunity to say goodbye to Eric, they would have to travel down to do that as extubating was set for a week away. The doctors were certain he would pass away shortly thereafter. The family gathered, and we had our son baptized, pictures were taken, everyone had time alone to say what they needed to Eric, and he was extubated. Eric surprised us all; he just kept right on breathing, and he could support his airway after all. The hospital put us in a family room with our son, so we could spend as much time as we could with him in a less clinical environment before he passed.

Within 2 weeks, it was clear this child was a strong fighter and wasn’t ready to give up quite yet. We had another meeting and it was decided we would take him home. We wanted his big sister to have time with him and show him what a home was like. We took a 2-week crash course on neonatal nursing. We had to learn how to do his lung physio, how to suction him, and how to work a feeding pump and so much more. Eric was brought home February 10,1999. We did his 24-hour care until March 9th when he took a turn for the worse. He was diagnosed with aspiration pneumonia. This left me in a panic; I wasn’t ready to let him go, and I wanted him sent back to Children’s Hospital and be treated. We had an amazing Doctor who came to the hospital and took me for a walk to discuss why we made the DNR code and why we made the right choice for our son. I took my son home that day knowing we were running on borrowed time. His breathing became very shallow, he turned blue from lack of oxygen and on March 11, 1999 at 3:15 am our darling boy went home to be with his creator. The year following is a fog, I remember very little. I was deeply depressed but I knew I had to keep moving forward for my daughter, she needed me. I know she was hurting too but I was so consumed with my own grief, that I couldn’t reach out to her, I couldn’t handle both her grief and mine. My daughter and I have had to take a lot of time since to heal together. With that being said, the pain of losing a child is not something that you can run away from or attempt to forget; I relive my sons small time on this earth every single day.

Fast forward to 2005, I became very ill. It took 13 years for me to find out I had Chiari 1 Malformation and EDS and another 3 years too learn I too had Spina Bifida Occulta and Tethered Cord. I started to learn all I could on these conditions and joined many support groups. I was reading up on all the different types of Chiari when I came across Chiari Malformation type 4. It was described as one of the rarest types, when a baby is born with an underdeveloped hind brain or cerebellum. WHAT?! Did I just stumble across the reason as to why my son was born sick? Did I just stumble across the name of the disease that took my son from me? I printed out the info for Chiari 4 and Eric’s medical info and took it to my Doctor. The doctor and I had a long discussion and he agreed this was what my son had all those years ago, I just don’t think they called it Chiari 4 back in 1998. I was relieved to know, but angry. It took 18 years for me to find out what had happened to my darling baby and that was after all, genetic. They ran genetics on Eric but told us it was negative. Little did we know then that h-EDS was what I had and that they do not know the genes that cause it. I also learned many of us suffer from the MTHFR Gene where our bodies reject folic acid and B12, two vitamins I had always been deficient in and it can cause neural tube defects in babies.

In closing, I would like to say, no parent should have to wait 18 years to find out what took their child from them. As a parent, no matter how your child passes, you will blame yourself. I had to work through some pretty hard moments and learn to forgive myself for this unknown genetic disease that not only took my son Eric, but now has also made my oldest daughter Tricia ill. This is unacceptable to me. This is why it’s so important that we raise awareness and educate our doctors, so no mother or father has to wait 18 years to understand their child’s conditions or death. This is way the work that WTF and Chiari Bridges does is so very important to me and why I am always asserting that we be tested for all comorbid conditions. Rarely is Chiari just Chiari, so please take the time to undergo all the proper testing for all the comorbid conditions.

 

Eric Michael Nault November 28th, 1998 – March 11th, 1999.

Thank you for taking the time to remember my dear Eric with me,
Amy Schmalz

Zona McGee was blessed with a vibrant and beautiful little boy on July 2nd, 1993. She named him Ryan Andrew and fell in love with his sweet face the second she first held him. He looked just like her! Her husband, Kevin, and daughter Crystal, eight-years old at the time, were over the moon as well.

Zona had been battling a genetic kidney disorder her entire life, causing both of her pregnancies to be considered high-risk, so when her second delivery and birth went well, she was elated. She counted her blessings and relaxed into the day-to-day tasks of being a young mother. Her life was happy, and she adored her children very much.

By her beaming account, Ryan was a gregarious and charming little boy. “If there was something to explore, he would be all in it,” she told me. He had more energy than she knew what to do with and she kept him busy with stimulating activities and adventures. He was sweet, kind, and loved to make people smile, especially his sister, with whom he shared a unique bond.

Watching her children grow into responsible young adults made Zona immensely proud. She had many wonderful memories of her family through the years. Because her kidney disease is genetic, and other family members suffered as well, she had worried that her children may inherit the gene, but neither did. Ryan was a very bright, energetic, healthy child. Throughout his childhood, Ryan’s well-child checkups always received A+ reports.

However, when he was 15, his physician noted an incidental finding of minor scoliosis. Because Ryan did not complain of pain, the doctor decided to just keep an eye out for future changes. Ryan’s teenage years were happy. As he matured, his high energy settled down, and he became more introspective. He often spent his free time gaming on the computer, broadcasting on his YouTube channel, playing guitar, and enjoying life with his friends and family. Music was a major part of his world. He loved everything about it: listening, writing, and playing. He often shared his songs and favorite bands with his proud family.

Ryan was a good student and took a local job after graduating from high school. He loved working and earning his own money. By all accounts, Ryan was a completely normal, functioning, and on-target, young adult. He was doing what most recent high school grads do – living life, having fun, and trying to decide what profession he might go into.

He was also heavily involved in organ donor awareness. Although Zona had been doing remarkably well for years, during this time, her condition began to rapidly decline, and she was put on a kidney donor list. She had a calling to act and started a blog, which led to a passionate fight to spread awareness about the importance of donating organs and tissue. Ryan fought alongside her and made the decision to become a donor himself. Little did they know, Ryan’s decision would end up saving many precious lives, including his own mother’s.

In May of 2013, Ryan began experiencing minor, intermittent headaches that were uncomfortable, but not debilitating. Zona made the logical assumption that Ryan was not wearing his glasses often enough. She continued to encourage him to wear his glasses, but the headaches became more severe over the next month. He visited his doctor who also believed it was due to his eyes, and advised Ryan to take Tylenol for the “migraines.” Stress was also considered, but the doctor was not a bit concerned.

Sadly, glasses and Tylenol did nothing to prevent or relieve the pain. His headaches were constant and progressively painful, making it difficult for Ryan to function. He was in agony, but he tried to minimize his suffering as to not “burden or stress out his family.” The doctors assured them that “nothing serious” was going on with him. The doctors were horribly premature in that assessment of Ryan’s symptoms.

One Friday, during a particularly bad flare up, Zona became very concerned that Ryan may have been suffering from a sinus infection and planned to take him to a walk-in clinic that following Monday. He never made it to that visit. On Sunday, May 12, 2013, Mother’s Day, Ryan was stricken, out of the blue, with an unbearable headache. He also complained of a stiff neck and collapsed in the bathroom. Zona and Crystal, who both heard a “thud” found Ryan unresponsive on the floor, and rushed him to the hospital.

They waited anxiously, for tests to come back, wondering if Ryan’s symptoms pointed to meningitis. The doctors mentioned that he, “May have a brain tumor”, before all the results were back. The family was terrified. However, after an MRI scan and lab results were completed, the doctor came in and told Ryan that “luckily” it was not a tumor or bacterial brain infection, but that they had found a Chiari Malformation.

Zona anxiously queried, “A what?”

Her question was ignored, as her child was an adult. She asked again, but the doctor turned from her and explained the test results to Ryan directly. He told Ryan that a Chiari occurs when the brainstem becomes herniated, but they could easily “fix” it with brain decompression surgery. He also told him that unless he agreed to surgery, his headaches would worsen, and he would just keep coming back to the E.R. He was presented with consent forms and was informed that they wanted to do the surgery the following morning.

Ryan had never dealt with illness or pain and he was terrified. After consulting with Zona, who felt that they should take some time to get more information, Ryan made the decision to undergo the operation. He just couldn’t bear the pain any longer; it was that debilitating. They were promising him relief and his symptoms were so severe, he trusted them and signed the consent forms. Zona was beside herself with worry. She thought to herself, “We just found out that he has something I have never even heard of, and they want to saw through his skull? I need more time!”

But she didn’t have time. She also did not have the respect of the doctor simply based on Ryan’s age. This infuriated her, as while Ryan was over 18, she felt that his family should be able to have their questions answered as well. But she didn’t fuss. They were coming to prep him, so she hid her fear and frustration to be strong for her son. When they wheeled the gurney away, she had no idea she would never see her son the same way again.

During Ryan’s first surgery, a temporary shunt was placed in an attempt to drain excess cerebrospinal fluid. When that failed, the surgeon made the call to do decompression surgery. Ryan went into this operation a very healthy and fit young man, but he came out with obvious signs of brain damage. Though it was clear that Ryan wasn’t well post-op, his family never again saw the surgeon who performed his decompression. There was no follow-up.

Over the next few months, Ryan was rushed to the E.R. on several occasions due to cyclic, intractable, vomiting along with severe head and neck pain. During these dozen-plus visits to the E.R., he was turned away multiple times and labeled “drug-seeking, weak, and dramatic.” This was a slap in the face, as Ryan was advised to blindly have the surgery to prevent him from returning to the E.R. This painful irony was not lost on Zona, and it only added to her trauma and confusion.

Ryan was having seizures, yet he was told he was faking them. When Zona protested, she was told by a nurse entrusted with Ryan’s care, “Your son is not having seizures. What is wrong with you? Do you want him to have seizures?”

In all the E.R. visits, there was ONE brain scan. The family was told the surgery was successful and whatever was going on, if anything, was completely unrelated to his decompression surgery at their hospital.

On the last visit to this particular hospital, the chief neurosurgeon refused to treat him neurologically and ordered a psychiatric evaluation instead. After speaking with Ryan, the psychiatrist said, “I do not believe you are crazy, but you are a bit of a wimp.”

Zona was livid and chased everyone out of his room. She then immediately took Ryan to another, smaller hospital. It was obvious to the triage nurse that Ryan was in serious trouble. He was gaunt, having lost 30-plus pounds in three months. He had nystagmus, and his vital signs reflected the pain and distress he was experiencing. Scans showed brainstem slumping and his neck, literally, had no support. Zona was told that “too much bone had been removed,” and that he needed emergent intervention.

The doctors recommended immediate surgery to correct the horribly botched decompression. However, they suspected that he had meningitis, due to a fever, so they wanted to confirm and aggressively treat that before opening him back up. They began I.V. antibiotics and Dilaudid.

Again, Zona helplessly waited by his side for more test results. She was slightly relieved that there was finally a team of people looking after her child who believed them, but as she watched her child dozing from the pain medication, she barely recognized him. He was thin, with hollow, sunken eyes, and his weak, frail arms were drawn to his chest. Hands clenched in fists, he laid in a semi-fetal position. She wanted to know how this had happened. It had been a surreal, awful three months, and she wondered if there would be enough time to save him. Tragically, there was not.

Ryan suffered a fatal seizure the following morning that collapsed his brainstem, and he never woke up. He was pronounced brain dead, August 11th, 2013.

After Ryan’s death, it was discovered that he did not have an infection at all. According to an independent attorney’s assessment of Ryan’s medical records, there were at least nine opportunities for the health professionals, whom Zona trusted, to save Ryan’s life. The investigator called Ryan’s treatment barbaric and inconceivable. He, like all of us, want to know how this young man was so callously discarded and left to suffer until his untimely death. That question will never be answered. It is incomprehensible how the “professionals” who did have contact with Ryan, not only shunned him, but covered up evidence of medical injustice in order to protect their establishments, surgeons, and other health care workers.

Ryan’s severe, post-decompression, decline was obvious. Ryan was aware that he was in critical condition. He knew he was not going to survive. Before he died, he told Zona, “Make sure everyone knows what happened.” She did that and more.

Zona wanted the world to know who Ryan was and about the loving gifts he left behind. The medical establishment failed Ryan and his family, but he remains a true hero. Upon his death, several families received the gift of life through Ryan’s organ donation-including his mother, Zona. The day after Ryan died, she was in surgery receiving her child’s kidney.

There are times when Zona, naturally, wondered what she could have done differently, but the answer to that is simple: Without advocacy and awareness, hindsight is 20/20. There was nothing more she could have done. She tried everything in her power that she knew to do at that time. Because this was being reiterated by the doctors, she had faith that he was in good hands and that he would recover fully. There was no playbook she could consult on how to advocate for her child. She was in a surreal state of shock, disbelief and fear. She had no frame of reference to show her that the medical professionals in charge of Ryan’s care were terribly wrong, and negligent in the very least. She had to believe the doctors whose opinions and advice we are taught to trust. After all, doctors are the ones who have the medical degrees and they know best, right? No.

From the time Ryan was decompressed to the day he died, Zona made several calls to get Ryan help, but nobody would listen! Zona’s desperate attempts to alert the medical staff of Ryan’s worsening condition were to no avail. Nobody helped! Nobody cared! Nobody listened.

Zona took the pain from this unimaginable nightmare and turned into an unrelenting drive to educate other families who are caught up in the trap of medical injustice. She became a fierce advocate for patient safety and rights with the hope that no other child will suffer the way Ryan did. She also helped parents navigate the medical system so that they may learn from her experience. She vowed to work feverishly to bring about awareness and authorized a documentary about Ryan’s life and struggle with Chiari Malformation.

On Sunday, May 14th, 2017, Mother’s Day, Zona McGee, succumbed to metastatic lung cancer that she acquired from the anti-rejection drugs she was given to save Ryan’s kidney. Her death is a tremendous loss to our community. Her family and friends miss her more than any of my words can express.

Zona’s worst fear was that Ryan would be forgotten and that her promise, to let everyone know his story, would end with her life. I promised we will never let that happen. We never will.

Rest in peace, beautiful angels.

Please support Zona’s Visions:

Zona’s Blog Zona-Life On The Waitlist

Ryan’s Awareness Page: Ryan’s Voice Chiari Patient Awareness

Please support the documentary Writing For Ryan and view the documentary trailers and share the website link as well.
www.chiaridocumentary.com

Gianna Soares
Writing For Ryan
Updated 01-21-2018
For the exclusive use of Chiari Bridges, as per Zona’s request.

After years of having our symptoms dismissed, having our pleas for help and understanding seemingly fall on deaf ears by our doctors (and many times our friends and family as well), it can be a relief to finally have a name for what has gone so horribly wrong with us. The relief is short-lived however, as we begin to realize the full scope of all that is really wrong with us. Although surgery can be extremely successful for some, many of us are left with some degree of symptoms or complications to deal with. For those of us who also have a connective tissue disorder, such as Ehlers-Danlos Syndrome (EDS), the issues with our tissues can seem endless. The thought of “getting healthier” can seem like a daunting task. We do not have control over every aspect of our health, or every aspect of our fight, but we are not powerless! We do have control over some lifestyle choices, that can help improve our day-to-day lives.

MAINTAIN A POSITIVE ATTITUDE:
The single most important item within our control is our attitude! We don’t have to ignore our reality or turn a blind eye to the negative aspects of our conditions to have a positive attitude. We can choose to frame things in a positive light. For example, if I am no longer able to walk as far as I could this time last year, I can look upon that situation with an air of defeat… or I can remind myself that I was also unable to walk that far three years ago, but with determination, with time I made progress! It may be unfair that I must start over again, but I am worth every ounce of effort that it takes to do so. I can acknowledge the unfairness, and then choose to focus on making progress towards my goal. A positive attitude is not going to will Chiari or EDS away, but it can improve our experience of living with these conditions.

CHOOSING FOODS WITH YOU IN MIND:
With connective tissue disorders, and the myriad of effects they can have on our bodies, eating healthfully can feel like walking through a minefield. Mast cell issues can cause sudden or intermittent allergic reactions to a wide variety of foods. Dysautonomia can require us to consume large amounts of salt (and still may end in nausea and vomiting). We are more prone to gastroparesis, gastric dumping, Irritable Bowel Syndrome (IBS), and other gastrointestinal problems that limit our food choices. Despite all these challenges, most of us can make food choices with optimum health in mind. Many EDS experts recommend eating whole, nutritionally dense foods, and taking supplements to help mitigate the vitamin and mineral deficiencies many of us are prone to, due to malabsorption. If inflammation is an issue, we can avoid dairy, sugars, refined flour, fried foods, and replace them with foods that are known to reduce inflammation, such as: salmon, blueberries, beets, broccoli, spinach, and foods cooked in turmeric, ginger, garlic, and olive oil. Most importantly, we can educate ourselves on our various conditions and what the experts on those conditions recommend, discuss this information with our own doctors and develop an individualized plan for ourselves, and apply this knowledge to our everyday life. Knowledge is key with conditions such as ours! While eating well is not going to shrink our cerebellar tonsils or cause our bodies to make collagen differently, it can help improve energy levels, and reduce pain and other symptoms.

MOVING IS ESSENTIAL TO MOBILITY:
Despite the pain and the fear, we can choose to move every day and strengthen our bodies as much as possible. Deconditioning is a real issue for many of us who have had such debilitating pain and other symptoms, that even after a successful decompression surgery, we may find ourselves unable to function normally again. And while we may never be 100% again, we usually can gradually improve our strength and endurance through a good physical therapy and exercise program. Experts agree that strong muscles help reduce many of subluxations and soft tissue injuries that are common to us. It isn’t always easy to find the motivation to get up and take a walk or to do those exercises your physical therapist assigns, but we must remind ourselves that we are worth the effort, and that even the very slightest bit of progress, is still progress. As the adage goes, “A journey of a thousand miles begins with one step.”

We encourage you to fight for better medical care, for more research, for doctors and loved ones to listen to you. But with that also comes a responsibility to do everything you can to take the best care of yourself possible. To follow your doctor’s recommendations (once you find a good one), to eat well, and to stay as active as you possibly can. But you don’t have to go this alone! If no other positive thing comes out of being diagnosed with Chiari or any of its comorbidities, we do promise you this; the Chiari community is full of amazing, inspiring, loving, encouraging people who will stand in your corner and cheer you on through all your challenges, even if no one else will. And we here at Chiari Bridges will be there along the way with tips and advice on living your best life possible with Chiari and all its ugly friends. Remember, pain is inevitable, but suffering is optional!

 

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