As I sit down to update my journey, I am crushed that we’re still figuring things out (and nothing really was as I was initially told it would be), yet at the same time, I’m so thankful that we’re continuing to figure things out. Nobody should have to fight a fight like this (every symptom, every diagnosis), but all of this just increases my resolve to change it before anyone else in my family (or yours) is having to fight it! What we fail to change in our generation, our children and their children will face in theirs!


Looking back, I have always had symptoms of Ehlers-Danlos Syndrome (EDS). As a child, I was in the school nurse’s office for stomach problems at least once a week. I was “double-jointed” and my friends always asked me to do maneuvers that I thought everyone should really be able to do if they tried. I was athletic early on, a tom-boy. I particularly loved playing softball (or baseball with tennis balls was the absolute best), but my ankles rolled when I started to run. Despite the fact that I was the only player that twisted their ankle multiple times in every game, I didn’t think there was really anything abnormal about me. Later, as an adult, I had repeated miscarriages and complications in all of my pregnancies, but the doctors conveniently came up with different explanations for each “rare occurrence.” It couldn’t possibly be all those rare mishaps, but EDS explained it all.

My mother passed away from a brain aneurysm the day after my 18th birthday. She was just 37 years old when she died. As a child she had a lazy eye and scoliosis of the spine, so an eye patch and back brace were a normal part of her childhood attire. She suffered from migraines throughout her adulthood, but nothing was more tale-telling than reading her journal after she passed, with multiple entries about repeated headaches and neck pain. Decades after her death, my maternal grandmother (my mother’s mother) developed multiple brain aneurysms over the course of a decade. Each time one appeared, she had it filled with titanium coils. I always admired her fight for life.

Me and my mom (1971).

The first headaches that I remember started immediately after giving birth to my first son in 1992. It was a cesarean section at an Army hospital in Fort Ord, California. Instead of an epidural, they gave me three spinal injections to numb me from the chest down. At my postpartum check-up, I complained of daily headaches when upright. My primary care doctor ordered a CT scan, but because it was just a few years after my mom had died they looked only for brain aneurysms and found none. I was still having those orthostatic headaches six months later.

Me and my eldest son, Johnathan (1992).

The Accident that Shook Everything

In 2000, I was a Bible College student and stay-at-home mom of three happy and active children (ages 8, 5, and 2). One September night, I was in a car accident that changed all of our lives. My neck was never the same again. My initial symptoms were head/neck pain, but all radiology reports indicated that everything was “unremarkable.” I tried everything they offered to me: rest, acupuncture, acupressure, steroid injections, osteopathic and chiropractic care, nerve stimulation units, physical therapy, pain meds, etc. Nothing worked long-term. Then in 2005, my neurological problems started intensifying. I began having bouts of partial paralysis in my legs and hands. I would just wake up one morning and out of the blue, I would have no fine motor skills. I would wake up feeling as though I had no thigh muscles to support me when I walked or tried to step up a step, and I had difficulty coordinating my footsteps. My primary care doctor at the time did blood tests and concluded that my “potassium level was on the low side of normal, so it must have been from potassium shock,” and he thought that no other tests were warranted. I started having vertigo whenever I was at any elevated height, even just a step or two up, like my brain couldn’t figure out how to balance with visual changes in height (I’d take a step up or down like the step was much higher or lower than it actually was). I also started having noticeable memory issues and intermittent trouble processing information. They tested to see if I was having small seizures in my sleep. When that was ruled out, they referred me to the memory clinic for further cognitive testing. They had no cognitive baseline to compare my results to, but said that I “tested higher than 89% of the population, so I should be happy,” and that I should just try reducing stress in case it was stress-related. They didn’t understand that it didn’t matter to me “how I compared to others.” I was only 34 years old and something was very wrong with me; I wanted answers that had nothing to do with the general population. In 2006, my eyes started twitching all day, every day, until the muscles just wore out and I could no longer hold them open completely. Oddly, one of my college professors inquired about my eyes and recommended that I have it investigated because it “could be neurological in origin.” When I did talk to my doctor about it, he saw the recommendations of the Memory Clinic and attributed it to stress as well, without any testing.


My Chiari Diagnosis

Finally, in 2010, ten years after the car accident, another MRI was done at my insistence to check for aneurysms once again (because I still was having excruciating head/neck pain and trouble holding my head up). I received an email from my primary care doctor that they found a cause of all of my symptoms. It was a condition called Chiari Malformation and the neurosurgery department would be contacting me to make an appointment. The neurosurgeon (who became my neurosurgeon) checked through my MRIs and said that the Chiari Malformation was evident on my first MRI after the accident ten years earlier. I was told that it was congenital and that it is commonly believed to be a result of prenatal drug use or lack of proper prenatal care (which was devastating to hear, but not all that unlikely as I was born in 1971. It also ended up being very wrong “textbook information” that they tell us all). Desperate for a measure of relief, I underwent a full decompression surgery a few weeks later. Missing the fact that part of my brain was in my spinal canal was 100% the hospital’s fault, but in hindsight, I really wish that I had done more research before surgery. I had comorbid conditions (many of which my doctors hadn’t even heard of, didn’t fully understand, and more importantly, they didn’t know the connection between these comorbids and my herniated tonsils). Initially, I felt quite a bit better. The release of pressure in my head helped my headaches. It was short lived though. Those undiagnosed comorbids caused my decompression to ultimately fail, although it all unfolded over several years.

My preoperative MRI (2010).

Post-op Complication: Pseudomeningocele

When I was released from the hospital following decompression surgery, I was instructed not to lift, push, or pull anything for two weeks so that my dura patch would have a chance to adhere. The problem was, I could feel fluid squeezing out of the patch far beyond that two-week limit. I developed a pseudomeningocele (blue box above), which can be normal immediately after surgery before the dura adheres, but as long as there is no active leak, the body should absorb the fluid and the pseudomeningocele should quickly resolve. My neurosurgeon tapped some of the fluid out with a syringe twice and we waited patiently to see if it would subside on its own. It did not subside and in December 2012 (just over two years post-decompression), I developed acute vertigo. Everything was spinning and rocking, non-stop. It didn’t matter if my eyes were open or closed. I was waking up vomiting in my sleep from the dizziness. I couldn’t walk at all without falling hard to my right. I had no sense of balance at all and it didn’t just come and go, it was constant. Another MRI was done and it showed that my cerebellum was absorbing the fluid from the pseudomeningocele (so the cerebrospinal fluid was inside my brain, not just surrounding it; see light blue circle in image above). The decision was made to put in a subgaleo-peritoneal shunt (SP shunt), which runs from the pseudomeningocele to my peritoneum. They expected that it might take up to six months to fully drain from my cerebellum, but I woke up from the anesthesia with no signs of vertigo. I believe this surgery saved my life, but as with all shunts (especially amongst those with EDS, which I had not yet been diagnosed with), the shunt was destined to cause problems all by itself.

Postoperative MRI (2012)

My Many Shunt Revisions

In April 2013, an unrelated CT Scan revealed that my shunt was no longer in my peritoneum. My NS scheduled for a general surgeon to “tie in” my shunt so it would not happen again (surgery #3). We went several months without complication until that November. The tied in shunt pulled out of my peritoneum again (it was excruciating). Hoping gravity would help in the matter, my NS did an incision just under my right rib cage and dropped it down into my peritoneum (surgery #4). Shortly thereafter, radiologist reports started showing a concern for the location of my brain and I was diagnosed with “Sagging Brain Syndrome.” So my six-week post-op appointment (which my NS did faithfully after every surgery) became my pre-op appointment for my 5th related surgery. This time a non-adjustable valve was attached to the shunt (at my chest) in hopes that by slowing down the amount of CSF being drained by the shunt, my head could retain more fluid and my brain could once again lift and become buoyant. Five months later I developed a hernia and upon closer examination (during surgery), it was found that my peritoneum was literally falling apart from all the trauma of the shunts; so my hernia removal surgery became a reconstruction surgery where my abdominal wall was pulled together with mesh, while carefully ensuring that the shunt didn’t come out (surgery #6). The shunt never moved again. As my brain continued to sag, the choice was made to replace the valve with an adjustable valve and in November of that same year, I was having surgery #7. The valve was adjusted to its slowest possible setting in hopes of finding a balance where it drained enough to keep the hydrocephalus at bay, yet retain enough CSF to lift my brain and keep it lifted and out of my spinal canal (so we could establish flow to the spinal canal and avoid the possibility of a syrinx).


Diagnosis: Ehlers-Danlos Syndrome

Despite my concerns that I might have a connective tissue issue and being told over-and-over again that I “didn’t look like someone with Ehlers-Danlos Syndrome,” I was finally diagnosed with it in May 2015. After finally finding a neurologist who understood the role that our connective tissues can have in a Chiari Malformation, I was given a referral to a geneticist. It still wasn’t as easy as it should be though. The geneticist did not know much about Chiari or Ehlers-Danlos related conditions (although he didn’t initially admit to that), so I had no idea at that point what was and was not related, and neither did my doctors. I received a call from the geneticist’s assistant and I agreed to send her pictures of my hypermobile maneuvers from the Beighton Scale. I could do all but bend over and put my hands flat on the floor with my knees straight, but I was able to do that when I was younger (and thinner). I was given a 9/9 on the Beighton Scale and was told that he would just mark my chart as diagnosed “hypermobile” and that he didn’t need to see me. I honestly didn’t know any better at this point, but I was about to learn something very important. I sat there thinking about what this “hypermobile” diagnosis would mean for me and decided to look more into EDS for myself. I read about the high risk of aneurysms, organ tearing, miscarriages, etc. and I was back on the phone with that assistant within twenty minutes. She asked if she could call me back, and within the hour the geneticist had decided that he needed to see me. He set up an appointment with me within twenty-four hours and asked if it was okay if he had a few others (doctors and medical students) there as well, since they’re a training hospital and they “don’t really come across patients with Ehlers-Danlos” (he should have told me that from the beginning). I agreed. Despite his lack of knowledge on EDS related comorbidities, he did know exactly where on my body to look for characteristics of EDS (all of which I thought I didn’t have). For instance, my skin isn’t unusually elastic, except in my upper arms and upper thighs. My skin isn’t translucent (I’m olive complected), except for on my breasts, back, and inner forearms. My skin isn’t unusually soft, except on my back. Now concerned that I might have Vascular Type EDS (vEDS), he decided to have me tested for that. The test was easy on my part but expensive on theirs. They drew blood and had it refrigerated and shipped to a lab in Washington state. It took thirty days for them to make sure that there was no mutation in my COL3A1 (collagen 3; alpha 1) gene, which has a median mortality age of 48. Initially, I felt devastated, since I was already 44. I decided that I hadn’t fought through all that I had, to only live a few more years. Thirty days later, the test came back indicating that I didn’t have vEDS and by default, I was diagnosed with Hypermobility Type EDS (hEDS). I was relieved, but the geneticist assured me that I still needed to be cautious. Since EDS symptoms are known to cross the type boundaries, and we already knew that vascular complications ran in the family (with the aneurysms) and with me personally (my peritoneum tearing), it technically made me “hEDS with vEDS crossover symptoms” and I’d probably have to explain that to my doctors for the rest of my life, so they remain aware of my potential to have additional vascular problems.


My Poor Mess of a Neck

The electric shock feeling in my spine (Lhermitte’s Sign) that I’d had intermittently for years, became an all-day, everyday thing, and much stronger in intensity. The MRI revealed that the herniated disc I had between my C3/4 was getting worse. The disc was removed with cadaver put in its place and the discs were fused together. My 8th surgery (ACDF = Anterior Cervical Discectomy and Fusion) wasn’t related to Chiari, but it was related to the EDS. We knew that my cervical spine was really bad from the beginning, but it got worse. I am now actually diagnosed with Degenerative Disc Disease in all three levels of my spine, but my neck has by far taken the brunt of it all. The ACDF, while 100% necessary, compromised the discs adjacent to it, and every disc from C4-7 is either bulging or herniated (Subaxial (cervical) Instability), so additional surgeries are likely to be needed.


Learning to Advocate for Myself

Over the past several years I have become an enthusiast of Chiari related research and MRIs (out of medical necessity more than anything). It became apparent to me that I absolutely needed to know everything that was going on in my body in case my doctors didn’t. When I first started, I’d print out studies and lay in bed with multiple high-lighters. I had such brain fog that I’d lay there crying at the fact that I was reading and rereading the same paragraphs over again, but I knew that I had to learn it despite how impossible it seemed. I prayed a lot for God to help me with my understanding and He did. I also started looking at the medications I was taking, the supplements I was taking, and what the ideal doses were for me (especially those that would help with inflammation and cognition), and other natural remedies. The first thing that I removed was all of the nerve meds that they had me on for peripheral neuropathy. I was maxed out on Nortriptyline (a tricyclic antidepressant) and almost maxed on Gabapentin (both of which had caused me to gain an incredible amount of weight over the years). When I informed my primary care doctor that I wanted to go off of them all, he thought it was a bad idea because of the severity of my neuropathy. I insisted though and asked him to help me to wean myself off of both of them in healthy intervals, and let’s “just see.” With the first down-dose, I physically felt a reduction in inflammation. It took me many months to wean off and get them out of my system, but in hindsight, I think this was the single best decision that I could have made. The longer I was on supplements instead of the nerve meds, the more my brain-fog improved, and I now believe that I have regained all that I’ve lost cognitively and then some.


Syringobulbia

In 2016, I was reviewing some of my old MRIs and I saw a large CSF filled hole in my lower medulla oblongata (lower brainstem). It was obvious in all MRI series since 2015, yet I was told that all was stable. After researching it, I asked my neurologist to take a look and see if it could be Syringobulbia. She referred my question to my neurosurgeon and he confirmed that I had an 11mm cyst in my brain stem. This type of cyst happens when there is a blockage of cerebrospinal fluid and is most frequent when the brain stem is also herniated below the foramen magnum (Chiari 1.5). It explained a lot of the problems that I was having, that we had thought to be unrelated. For instance, and I had a decreased sensitivity to temperature for years, never feeling hot or cold; and never having the automatic reactions that I should have had in response to temperature, like sweating and shivering. I could comfortably be outside in heat above 100° without breaking a sweat, or be outside in shorts and a tank-top when it was a chilly 30° morning without ever shivering. I also developed tachycardia and I am now medicated to keep my heart rate down to a safe level. My neurosurgeon ordered a new MRI in April 2017. The size of the syrinx had decreased to 9mm but was draining down my spinal cord forming an additional syrinx (Syringomyelia).

Syringobulbia. Left – Syrinx in 2015 measuring 11mm in diameter. Right – Syrinx in 2017 measuring 9mm in diameter.

Consulting a Specialist

After all that I had been through in my fight, in April 2017, I decided to pay out of the pocket and have an online consultation with a Chiari Specialist in New York, who specializes in Chiari with EDS (the best $300 that I’ve spent in my fight). I sent him my pertinent medical records and copies of my MRIs in advance, wanting to find out what my doctor did right, and what he did wrong; and what course of action should be taken at that point. My expectation was that he would give me reasons why I should go to New York to see him, but that’s not at all what he told me. He told me what my doctor did right and that he didn’t disagree with the course of action that my neurosurgeon wanted to take. He said that my brain had sagged as low as it really could, but that since my high/low pressures had balanced out, and I was feeling better than I had in years, my syringes really should dictate our next course of action.

In March 2018, following an exceptional year (at least where my head and neck are concerned) new imaging was done. My neurosurgeon asked me to come in to review it. It gave me a chance to tell him about the specialist’s opinions. My MRI showed that the Syringobulbia had decreased another 2mm. I asked him what that meant for the cervical syrinx, and that had almost completely disappeared. I asked him to go back to my images and correct me if I was wrong, but “the only reason that a syrinx (in either location) would dissipate like that was if I was finally getting CSF flow down my canal (despite my severe brain sag).” He agreed and I think he was a little surprised to see me think on my feet and figure that out in front of him (where I wasn’t having to ask anyone or look it up). He also confirmed that I had an Acquired Chiari, secondary to Intracranial Hypertension. He applauded me for learning all that I had and said that he wished that he had checked my pressures before decompressing me, as it may have changed the course of action that we had taken. And we agreed to wait a year and see where the syringes (syrinxes) are. As I left his office that day, I felt such a sense of relief, that we were finally getting CSF flow like the decompression in 2010 was meant to do.


My Extensive Epidural CSF Collection

In 2022, my neurosurgeon contacted me telling me that he was retiring and he’d like to have one last MRI of my entire spine (he added the brain to the request at my request). Unbeknownst to me, he ordered a CSF Leak Protocol, which consists of less slices, but they’re specifically looking for leaks. The images showed an “extensive extradural CSF collection from C7-L4, consistent with a CSF Leak and probable dural tear or CSF Venous Fistula.” They followed up with a Dynamic CT Myelogram. A Dynamic is different than a regular CT Myelogram, as they do it over 2-3 days, and they insert the contrast little by little into my spinal canal, and watch carefully for it to leave the spinal canal. CSF leaks and dural tears aren’t uncommon amongst Ehlers-Danlos patients, and usually happen in the front or back of the canal. CSF Venous Fistulas on the other hand are a much newer phenomenon, and they usually happen on the sides of the canal (more often on the right side). After two days of grueling tests, they found no active leaks or evidence of fistulas and surmised that what they saw on the MRIs to be “residual artifacts” from a leak that I had in the past… a leak that could have pulled my brain down into my spinal canal in the first place.

Sagittal and axial views of my thoracic and lumbar images showing the residual artifacts of an extensive extradural CSF collection.

It’s been a long road, hard road. I still battle inflammation and I’m definitely not done with surgeries. Eventually, I will need a ventriculoatrial (VA) shunt to hopefully resolve my high-pressure issues and enable us to remove my over-draining SP shunt that is making my brain sag. But for right now, I’m just enjoying feeling so much better! I praise God every step of the way, as I know that He’s there making a way out of no way. I have no idea why He took so long or why others haven’t seen the same results (because He loves them as much as He loves me), but I don’t have to have all the answers. I’ll just praise Him through the course of my journey, as He’s never let me go through it alone!

*I dedicate this story to my family: John (husband), Ron (dad), Johnathan (son), MyKaella (daughter), Jojo (son) and my daughters-in-law, Violet and Sarah. Thank you all for all your help and for standing and kneeling beside me throughout my entire ordeal. You’ve been there for me and loved me through this long haul and I praise God for each and every one of you. 


Originally written in 2018. Updated April 2022.

Tired of your doctors telling you, “If you just exercised, you’d feel better!”?

Well, they may not be all wrong. Just hear me out! The truth is we all just want to feel better and fight to get as much of our lives back as we can. That is never going to be as easy for us as it would be for someone who doesn’t have all of the challenges that we deal with.

Not only is moving necessary for our circulatory systems, and better blood flow means less inflammation, but as Elle Woods says, “Exercise creates endorphins. Endorphins make people happy.” It’s actually true. And whether you’re chronically ill or just sedentary, a slight boost in happiness can change your entire outlook on life. Okay, so I am also not saying go out and pick up CrossFit. Or marathon running. We all know our bodies most likely cannot tolerate that. So how do you exercise safely, while still providing yourself the benefits of movement? Each one of us are different, but my goal is to give you the framework of how to start moving in some way, and then you build on that foundation.

Start small. I want you to remember that for the rest of your life – especially when you are faced with something difficult or are challenging yourself to start moving. Write down a goal (i.e., walk to the kitchen and back, or walk to _____ and back) and put it somewhere that you will see it. Once a day, accomplish that task. Most importantly, listen to your body. If it is day 1 and you accomplish your daily goal and the next day, you cannot move, that goal may be too much. How can you accomplish that daily goal the next day? Do you need to modify what the goal says? Or how you treat your body before or after? Does it involve extra braces, or heat or ice after? The first few weeks will be the hardest. You are re-learning your body and how it moves. Try not to reduce the goal (of course, if symptoms are such that you end up at the hospital, then please – re-evaluate the goal). If you want to walk outside, try to plan a route that will take you by things you enjoy – a friend’s house, a pretty park, etc. If you cannot walk outside, and choose to walk inside (your home, a building, etc.) that is perfectly fine too.

What if you are in a wheelchair or are unable to leave the bed? Well, that is fine too. You can still get your exercise in. You can do leg lifts while lying in bed. Pick a goal to do ___ on each leg. Or if you do not have use of your legs, do upper body movement (just be careful of pulling on your neck too much). Safe upper body movements while laying down include arm lifts (straight out in front of you like you would be a zombie).

Be sure that any movement that you do should be within a pain free range. If you are doing arm lifts and it hurts your shoulders or neck (or instability causes your shoulder to sublux), reduce how high you are lifting your arms. The same principle applies with your legs.

One of our admins is going on a trip soon and they wanted to be able to walk around, so they start walking 1 lap around the block (a small block) each day. After the first day, the pain was pretty bad, so she added some neck bracing, and additional leg braces in order to try it again on day 2. Day 2 she was able to complete without issue! She takes her time and is not concerned with HOW LONG it takes her to complete the walk around the block. She only cares that she DOES it.

That is all you should remind yourself. To pick a small goal and COMPLETE it to the best of your ability. You will be SO proud of yourself for completing that goal and I will be cheering you on and always here for questions and to help!

The week before Christmas in my home is filled with gift wrapping, cookie baking, dogs barking, people talking (loudly)… On a normal day, we feel the pain in the stiff neck, the backache, the headache, (the list could go on). When we are surrounded by stressors, they can take a toll on a person, especially a chronic pain patient, so it’s important that we “just take a minute” when it’s needed.

Christine Miserandino created a theory called The Spoon Theory as she struggled to explain her chronic pain (from Lupus) to a friend. The theory basically goes like this. Every day we get twelve spoons. Every activity we do uses a spoon or more. For example, a shower may use three spoons, leaving us only nine for the rest of the day. During this time of year, we have to be careful how we use our spoons, so we aren’t trying to borrow spoons from the next day/week, because the old adage is true… what we do today, we pay for tomorrow.

I’m learning to let go of things that I simply can’t do anymore or ask for help when it’s available. If you have family, ask for help and put them to work. Let them wrap the gifts or do the cookies. Allow them to help. I’m not very good at that. I know how I like my stuff done, but I’m learning to be gracious even if it’s not all ‘my way.’ I saw a meme the other day that said, “the first Christmas wasn’t perfect so it’s okay if yours isn’t either.” There are no truer words.

Many of us struggle with sensory overload. We can’t deal with loud noises, flickering lights, crowded places, etc. I have chronic tinnitus. It’s always there. So, when it gets too loud for me – when people are over-talking each other, the TV is blaring, grand-kids are running, and the dogs are barking – I excuse myself and I go to the bathroom and take a few minutes to just ‘be.’ They really will not follow ya to the bathroom, so take your moment and breathe, and allow peace to come in. Do that as many times as you need to. I promise it makes a difference. If you are asked, just say, “Well, I have to do what I got to do,” and let that be that.

I hear many times of families who just aren’t very understanding or supportive. We will not convince them in one visit. Michelle Cole wrote an amazing article called Dear Family. It’s on how to tell families and friends what we need from them. I would encourage all to read that. Should there be that one family member who just starts on how yoga helps or how we aren’t praying enough, whatever their “answer” is for our issues, as much as we would like to come back at them, don’t! It’s not the time, and it’s not worth the spoons, so for their sake and the sake of others there (and for your sanity), just let it go. We know the truth. We know some will never understand no matter what, because honestly unless it happens to them, they can’t know. Enjoy your day no matter the naysayers.

Pace yourselves. Do a little each day. Epsom salts baths are a Godsend for me because they help tired sore muscles and joints. If your body says to ‘lay down,’ listen. Our bodies dictate what we need and we have to be mindful. Remember, stress exacerbates our symptoms. As best you can, try to be as stress-free as possible. I know it’s hard. I really do, but none of us need or want a flare (or to spend the next week in bed). Remember, if you can’t do anything (if you can’t do the first cookie, wrap the first gift, buy the first gift), that is okay too! It really is! Above all, give yourself grace! Oftentimes, we extend grace to others but leave ourselves out. Don’t do that! Give yourself grace and while you’re at it, give yourself a little holiday hug!

I pray we all have the best Christmas, remembering why it’s celebrated in the first place. As the admin of our Chiari Prayer Group, my prayer is “May God bless you and keep you. May The Lord make His face shine upon you and be gracious to you. May the Lord lift up His countenance upon you and give you peace!” (Numbers 6:24-26)

Ever feel alone in all you’re facing? It’s one thing to read what people are going through, but to see them as they share how they’re going through the same struggles that you are can be game-changing!

Starting in December, the tribe will be hosting online support meetings on the first and third Tuesdays of each month to share and learn about all we’re dealing with.

A valuable resource for patients and their families, looking for support and a sense of direction!

 
My sister asked me how she could pray for us specifically. Here’s what I’ve come up with.
 
 
 

As we lift up a warrior fighting EDS, Chiari, and/or Comorbids,
We are believing You for:

Knowledgeable doctors/surgeons
With hearts for the patients that are trusting them
Ears to hear them
And a willingness to unlearn and relearn

CSF leaks to seal
Cranial masses to disappear
Narrowed venous structures to widen
And cranial pressures restored to normal

Sticky filums to release
Stretched spinal cords to retract
Conus Medullaris’ to rise
And elongated medullas restored without consequence

Collagen restored without mutation
Intravertebral discs moving back into their rightful place
Laxity issues resolved
Straightened odontoids and clivus bones
And craniums to rise

Spines to straighten
Cerebellar tonsils to rise
CSF flow restored
And syringes (syrinxes) dissipated

Muscles reconditioned
Paralysis reversed
Vision completely restore
Ringing in the ears to cease

Habitual good night’s rests
No insomnia, painsomnia, chronic fatigue, or narcolepsy
Breathing issues corrected
Restless legs calmed

Nerves decompressed
Even vagus restored
Motility perfected
Inflammation gone
And pain a thing of the past

Depression replaced
Hearts seasoned with grace
Families restored
Where no one’s needs are ignored

Thank You, Father,
That despite all we’ve endured,
Your grace continues to be sufficient.
That none of this has taken You by surprise;
You knew all we’d face, yet You still chose each of us and call us Yours.
Our hope is in You and You alone,
The Author and Perfector of our faith,
We stand on Your promise that You still have a plan for each of us!
We might not know what it is or understand how we’ll get there,
but You are a good Father, and we trust You completely.

In Jesus’ Name, Amen!


(Note: This prayer was written to become a collaborative prayer, that we can add to as needed.)

WHEN A DOCTOR TALKS ABOUT THE “MOST SERIOUS” COMPLICATIONS SURROUNDING CHIARI MALFORMATION, THEY USUALLY SPEAK OF PARALYSIS OR DEATH. WHILE BOTH OF THESE ARE FAR LESS COMMON THAN THE ARRAY OF OTHER SYMPTOMS AND COMPLICATIONS, THEY BOTH CAN INVOLVE THAT OF A SYRINX.

The word syrinx (seer-inks), plural syringes  (seer-en-geez), means cavity or cyst. Syringomyelia (seer-ingo-my-el-lee-uh) is when the cyst forms in the spinal cord (myelo usually refers to the spinal cord), and when the cyst is in (or ascends up into) the bulbar region of the brainstem (the medulla oblongata) it is called Syringobulbia (seer-ingo-bulb-e-uh). While these cysts are technically the same cerebrospinal fluid-filled cysts, because they are damaging a different part of the body (each with a completely different function), the location of the syrinx has everything to do with the symptoms that it can cause.

Hydromyelia:
At the center of the spinal cord, there is a hole known as the central canal which runs from the fourth ventricle down the length of the cord. Its role is to carry a microscopic amount of cerebrospinal fluid down the center of the cord from the fourth ventricle. The small amount of fluid in the central canal is usually considered normal. When the central canal has too much cerebrospinal fluid coming in from the fourth ventricle it widens the canal and that abnormal widening that follows the central canal is known as hydromyelia (hydro-my-el-lee-uh) or syringohydromyelia (seer-ingo-hydro-my-el-lee-uh).

The Central Nervous System (CNS) is an amazingly complex network. The brain, brainstem, and spinal cord each play a role in communicating to coordinate everything that happens in our bodies (even when no cognitive thought is required). The messages to and from the brain are sent through the brainstem, down through the spinal cord, and out to our nerves. When that signal is blocked, it can cause interruption from that point down. When it’s thin, it interrupts the signal in the middle (gray matter), as it widens it interrupts the signal more and more, so it’s harder to get signal through the cord at the widest point of the syrinx.

The spinal cord runs through a canal known as the spinal canal. The spinal canal holds cerebrospinal fluid that among other things, nourishes the spinal cord and helps protect it from injury. A syrinx is generally known to be caused by a blockage of cerebrospinal fluid, which is why it is most commonly seen amongst those with Chiari Malformation. When the cerebellar tonsils descend into the spinal canal, like a cork the tonsils block the flow of cerebrospinal fluid. Even when the tonsils fail to descend, they can rest on top of the foramen magnum and still block the flow of cerebrospinal fluid (see Chiari Zero below). There are other conditions that can cause similar blockages as well, so it is not exclusive to Chiari malformations. Anything that causes stenosis (narrowing) of the spinal canal (the cord: canal ratio) can also block cerebrospinal fluid and lead to the formation of a syrinx – conditions such as bulging/herniated discs (from degenerative causes or trauma), spinal cysts/tumors, edema/inflammation of the spinal cord or surrounding membranes (from trauma or conditions like meningitis/arachnoiditis), and/or irregular curvatures of the spine (scoliosis).

Syringomyelia:
Syringomyelia does not usually run the entire length of the spinal cord like the central canal (but it can). Instead, it often seems to spontaneously appear. As the CSF increases in a syrinx, it can either lengthen or widen. The length doesn’t matter much (radiologists note the location because they report what they see and try to be precise). But a neurosurgeon that knows about syringes, knows that what really matters is the diameter of a syrinx (which is where the risk of paralysis can come into play). Symptoms tend to vary based on where the syrinx is located in the spinal cord. The highest point of the syrinx (where the initial disruption starts) and the widest point of the syrinx (location of the greatest disruption) should always be considered because there are different nerves branching from the spinal cord at different intervals. A syrinx interrupts the communication from the top of the syrinx down, so a syrinx that is higher in the spinal cord can have an impact on a larger range of the body. Someone with a syrinx in the lumbar region of the spinal cord will usually have communication problems from the waist down, but someone with a cervical syrinx is likely to have problems from the neck down. A syrinx in the upper thoracic spinal cord (or above) will often include the arms since the peripheral nerves that lead to the arms branch off around the T1 vertebra. The wider the syrinx, the greater the interruption of communication. Therefore, a syrinx should always be gauged by its diameter and not its length. Symptoms generally include (from the syrinx down): muscle weakness, pain, and spasms in legs; pain, tingling, burning of arms; muscle wasting (atrophy); loss of reflexes; loss of pain senses, loss of temperature sensation, numbness, pain, and stiffness in back/shoulders/upper chest (cape-like area); stiffness of muscles; muscle contractions (fasciculations); bowel & bladder dysfunction; scoliosis; paralysis (rare).

Syringobulbia:
Syringobulbia exists when a syrinx forms in the medulla oblongata (the bulbar region of the brainstem) OR when a syrinx in the cervical cord extends upward into the medulla. A syrinx in the medulla can cause medullary issues or in extreme cases, death (since the medulla is responsible for things that happen autonomically (automatically) for survival – such as breathing, heart rate, swallowing, gag reflex, etc.). Dysautonomia is an umbrella term used to describe any dysfunction of the Autonomic Nervous System (ANS), both Sympathetic and Parasympathetic divisions, often accompany any damage to the medulla. When talking about Dysautonomia, many tend to think of Postural Orthostatic Tachycardia Syndrome (POTS), but POTS is but one symptom of Dysautonomia in a long list. The damage from Syringobulbia is not generally isolated to the medulla, but to the cervicomedullary junction (where the cervical spine meets the medulla), it can also affect the cranial nerves causing symptoms such as facial sensory loss (unilateral or bilateral); extraocular muscle palsy; nystagmus; palatal palsy; atrophy of the tongue; dysphonia (vocal cord paralysis); slurred speech; indistinct speech; drooling; tongue fibrillation; oropharyngeal dysphagia; impaired gag reflex; hearing loss; tinnitus (ringing in the ears); alveolar hypoventilation; Sleep-Disordered Breathing (SDB); Central and obstructive sleep apnea; Anhidrosis (inability to sweat normally); Inability to burp (Retrograde Cricopharyngeal Dysfunction – RCP-D).

Common Treatment Options:

Monitoring The Syrinx:
Both Syringomyelia and Syringobulbia tend to be progressive, but in some cases, patients report having no symptoms and imaging proves it to be relatively stable in size. In this case, monitoring is generally recommended. A neurologist or neurosurgeon should carefully monitor these patients to track changes in the diameter of the syrinx (which should include regular imaging) and/or any evolution in related symptoms.

Surgical Treatment Of The Underlying Cause:
For symptomatic patients, or when the syrinx is progressing in diameter, or when the syrinx is so wide in diameter that it is stretching the diameter of the spinal cord from the inside out, treatment is essential. This generally involves treating the cause of the blockage of cerebrospinal fluid. When syrinx exists in a symptomatic patient with Chiari Malformation, a posterior fossa decompression surgery is usually recommended, with the desired result being to re-establish the flow of the cerebrospinal fluid to the spinal canal (so it no longer reroutes to the spinal cord and/or low-lying medulla).

Surgically Draining The Syrinx:
A surgical shunt is commonly used to surgically treat a syrinx when: the underlying cause is unknown or when treating the underlying cause has proven ineffective at reducing the size of the syrinx in a patient that is symptomatic.

When you start to educate yourself on a condition like Chiari, your vocabulary will be challenged. Most of us study with a medical journal article opened in one tab and medical dictionary in the next. Amongst all the medical terminology you will tackle, there are probably a few terms as important to your understanding of Chiari than comorbidities and pathological/etiological cofactors. When two or more conditions tend to co-occur, they are said to be comorbid with one another. It makes no inferences of a causal relationship between the conditions, only that they co-occur. This co-occurrence deduces that a correlation exists, but when the nature of that correlation is not known, they are just said to be comorbidities. When a “causal relationship” is known or suspected, the conditions start being discussed in terms of pathology or etiology, which are similar, but not exactly the same thing.

An etiological cofactor exists when the “root cause” of a condition is known or believed to be known. That “root cause” is the etiological cofactor. When an etiological cofactor can cause a series of events or conditions that can become “direct causes” for other conditions, that series of events creates a pathology. Conditions along the path are called pathological cofactors. Understanding these cofactors is imperative in understanding Chiari and all of the comorbid conditions that accompany it.

ETIOLOGICAL COFACTOR:

Chiari Malformation often seems like a beast that wreaks havoc on our bodies on every level. Indeed it is, but as you can see from the diagram above, it really is not the “root” of everything that is going wrong. There is a bigger beast at work in so many of us, and its name is Ehlers-Danlos. It is not by chance that so many of us with Chiari have so many other conditions in common (especially conditions like Degenerative Disc Disease, arthritis and other connective tissue problems). It is not by chance that so many of us have a history of miscarriage and similar familial histories. It is not by chance that Chiari is more prevalent in females than males. And it is definitely not by chance that Chiari is running in families and they cannot find a definitive genetic link. They cannot find it because they are not looking at the beast hiding in the background.

Ehlers-Danlos Syndromes are a group of inherited disorders involving a genetic mutation in one or more of our bodies’ collagen. Collagen is the most abundant protein, making up 1/3 of the proteins in the human body, affecting our bones, skin, muscles, and connective tissue[1]. Collagen is often described as a “cellular glue” that helps hold the body together. When that glue fails to hold, everything seems to go awry; before and after birth: skulls can under-develop in utero, organs tend to prolapse, and bones begin to shift as joint laxity increases (including the bones/vertebrae at the craniocervical junction). Ehlers-Danlos is a primary “root cause” of Chiari Malformations and a majority of the other problems we have. The list in blue is far from being a complete list of conditions caused by EDS. They are commonly accompanied with Chiari because they can cause or attribute to a Chiari malformation (pathological cofactors).[2]

PATHOLOGICAL COFACTORS:

Cranial Settling occurs when the skull has dropped and the odontoid (C2/axis) enters into the foramen magnum (Basilar Invagination). This drop can further compromise the craniocervical junction and as it pushes everything down, it increases the likelihood of an Acquired Chiari Malformation.

Craniocervical Instability (CCI) & Atlantoaxial Instability (AAI) usually occurs with cranial settling and Basilar Invagination (BI). The settling and/or softening of tissue can cause a shifting of the C2 (resulting in CCI or AAI) and the cerebellar tonsils (which are already inclined to prolapse) simply drop down with each shift affecting ones ability to tilt/rotate their head.[3]

Intracranial Hypertension (IH – High Intracranial Pressure) occurs when your intracranial pressure (ICP) becomes elevated. This elevation can happen for a variety of reasons.

  1. Space Occupying Masses (cysts, tumors or hydrocephalus) take up space inside the skull causing a “mass effect.”
  2. When no mass effect exists, many doctors look no further and give the diagnosis of Idiopathic Intracranial Hypertension.

Because the area of the skull is fixed in an adult cranium and partially fixed in that of a child, the elements inside the fixed space (CSF, blood volume and brain matter) tend to get pushed out wherever they can (the only place that they can escape without breaking through the dura is through the foramen magnum and the brain matter that’s closest to the foramen magnum is the cerebellar tonsils).[4]

Tethered Cord Syndrome occurs when the tissue inside the epidermis adheres to the spinal cord or filum terminale. While this tethering can happen anywhere along the spinal canal, it is most common in the lower lumbar and/or sacral spine. When this adhesion happens it creates a pulling down of the spinal cord and consequently, the brainstem located at the top of the spinal cord and the cerebellar tonsils just get pulled down with it.[5]

Intracranial Hypotension (Low Intracranial Pressure, often involving a CSF Leak) usually involves a cerebrospinal fluid leak or an over-draining shunt, we will highlight the former. Ehlers-Danlos patients tend to have weak dura matter. Tears/holes in the dura can happen anywhere in the dura surrounding the brain or spinal canal and they can happen completely spontaneously (without a known cause). When the leak occurs in the spinal canal, they can create a suctioning effect where cerebrospinal fluid (CSF) is being pulled down and out, causing the intracranial pressure (ICP) to drop. The cerebellar tonsils that are already prone to prolapse (due to EDS) end up getting suctioned downward with the CSF.[6] Cranial leaks often happen when high pressure is left untreated until the high pressure causes a leak in the dura mater. In cranial leaks, fluid usually leaks through the nose or ears (less common), and you can often taste the metallic taste of the cerebrospinal fluid in the back of your throat. While both spinal leaks and cranial leaks can cause low pressure and low-pressure symptoms, and while both can start, stop, and start again spontaneously, there is an increased risk whenever there is an opening where cerebrospinal fluid leaks outside of the human body (if cerebrospinal fluid can make it out of the body, microscopic bacteria can make it inside the same opening where it can enter in the meninges).[7]

Posterior Cranial Fossa Hypoplasia (PCFH) is the only etiological cofactor listed above that is definitely congenital. The role of collagen in bone development has been long-standing, especially its known contribution to certain conditions like Osteogenesis Imperfecta. However, more recent studies are discovering the role collagen plays in congenital posterior fossa anomalies. Posterior Cranial Fossa Hypoplasia is the most commonly “acclaimed” cause of Chiari malformations, but studies show, that even when all of the other causes above are factored out, only approximately 52% of those left (that fail to meet “the diagnosis criteria” for any of the above), have a small posterior fossa.[8]

COMORBIDITIES: 

While all of the conditions listed in the diagram are comorbidities, some are etiological/pathological cofactors of an Acquired Chiari (even though nearly 100% of us are told that our Chiari Malformation is congenital) and others have Chiari Malformation as their etiological/pathological cofactor:

Syringomyelia occurs when cerebrospinal fluid (CSF) is obstructed and a CSF filled cyst/cavity forms inside the spinal cord. This cyst is directly related to the obstruction of cerebrospinal fluid that can be caused by Chiari Malformation, Spinal Stenosis (a narrowing of the spinal canal, spinal cyst/tumor, a herniated disc), or irregular curvature of the spine (scoliosis). When that cyst/cavity extends into the medulla oblongata (the lowest part of the brain stem), it is called Syringobulbia, and it comes with a new set of symptoms consistent with the damage being done to the brain stem. So when Chiari Malformation exists with a syrinx, and there is no stenosis or disc problem in close proximity below it, the Chiari Malformation should be listed as the etiological cofactor for the syrinx. If more than just the Chiari Malformation is believed to be causing the syrinx, each would be more accurately described as a pathological cofactor.

Dysautonomia occurs when damage has been done to the brain stem or Vagus nerve. Whenever either of these is damaged, often from compression at/near the craniocervical junction, the autonomic nervous system can begin to dysfunction.

Confused? If you understand the causal relationships but find yourself wondering if a comorbid condition is an etiological or a pathological cofactor, think of it in terms of a domino effect. Only the first domino is the real etiological cofactor. All of the dominoes in between (on the path) are pathological cofactors. The important thing to remember in this array of medical terminology is that while everything is definitely not Chiari, it almost always shares a connection to it, and that is why so many of us have so many conditions and symptoms that doctors call unrelated! It is imperative in our fight that we know “what” we have and “why” it is happening. With such a broad spectrum of symptoms (like we all have), we must educate ourselves and not just believe the limited knowledge of our doctors.

*Revised November 2019

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References: 

McIntosh, James. “Collagen: What Is It and What Are Its Uses?” Medical News Today, MediLexicon International, 16 June 2017, <www.medicalnewstoday.com/articles/262881.php>.

Quake. “The Chiari Malformation Ehlers-Danlos Connection (Short Version).” Chiari Bridges, 7 Dec. 2017, <www.chiaribridges.org/chiari-malformation-ehlers-danlos-connection-short-version>.

3 Hawkeye. “Overview: Craniocervical Instability and Related Disorders.” Chiari Bridges, 6 Dec. 2017, <www.chiaribridges.org/craniocervical-instability-related-disorders>.

4 Quake. “Brain Under Pressure – Understanding Intracranial Hypertension.” Chiari Bridges, 10 Dec. 2017, <www.chiaribridges.org/brain-pressure-understanding-intracranial-hypertension>.

5 Storm. “The Tethered Cord – Chiari Malformation Connection!” Chiari Bridges, 15 Dec. 2017, <www.chiaribridges.org/tethered-cord-chiari-malformation-connection>.

6 Argent. “Overview: Cerebrospinal Fluid Leaks.” Chiari Bridges, 10 Dec. 2017, <www.chiaribridges.org/cerebrospinal-fluid-leaks>.

7 Pérez, Mario A et al. “Primary Spontaneous Cerebrospinal Fluid Leaks and Idiopathic Intracranial Hypertension” Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society vol. 33,4 (2013): 330-7. doi:10.1097/WNO.0b013e318299c292, <https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4040082/>

8 Quake. “Overview: Chiari Malformation.” Chiari Bridges, 6 Dec. 2017, <www.chiaribridges.org/chiari-malformation>.

 

HOMEPAGE: <https://chiaribridges.org/> (go to article)

ABOUT CHIARI (TAB)
  • Understanding Your Head and Neck Pain: <https://chiaribridges.org/understanding-your-head-and-neck-pain/> (go to article)

  • Overview: Chiari Malformation [Revised]: <https://chiaribridges.org/chiari-malformation/> (go to article)
  • What’s In A Name? An Expansive Review of the Name and Definition of Chiari Malformation: <https://chiaribridges.org/whats-in-a-name-chiari-malformation/> (go to article)

  • One Painful Fight – Get Ready To Rumble!!!: <https://chiaribridges.org/painful-fight-get-ready-rumble/> (go to article)

  • The Diagnosis – Round One: <https://chiaribridges.org/diagnosis-round-one/> (go to article)

  • The Important Questions to Ask Your Neurosurgeon [Revised]: <https://chiaribridges.org/important-questions-for-your-neurosurgery-appointment/> (go to article)

  • Overview: Chiari Comorbidities & Etiological/Pathological Cofactors [Revised]: <https://chiaribridges.org/overview-chiari-comorbidities-etiological-pathological-cofactors/> (go to article)

  • Overview: Chiari Treatment Options & Potential Pitfalls: <https://chiaribridges.org/chiari-treatment-options-potential-pitfalls/> (go to article)

  • Overview: Complications Associated With A Chiari Decompression: <https://chiaribridges.org/complications-associated-with-chiari-decompression/> (go to article)

  • A Bruised Mind – Chiari & Depression: <https://chiaribridges.org/bruised-mind-depression/> (go to article)

  • Finding Hope In The Seemingly Hopeless Chiari Fight!: <https://chiaribridges.org/finding-hope-seemingly-hopeless-chiari-fight/> (go to article)
COMORBIDITIES (TAB)
  • Overview: Chiari Comorbidities & Etiological/Pathological Cofactors [Revised]: <https://chiaribridges.org/overview-chiari-comorbidities-etiological-pathological-cofactors/> (go to article)

  • The Chiari Malformation Ehlers-Danlos Connection: <https://chiaribridges.org/chiari-malformation-ehlers-danlos-connection-2/> (go to article)

  • Overview: Craniocervical Instability and Related Disorders: <https://chiaribridges.org/craniocervical-instability-related-disorders/> (go to article)

  • Brain Under Pressure – A Guide to Understanding Intracranial Hypertension:<https://chiaribridges.org/brain-under-pressure-a-guide-to-understanding-intracranial-hypertension/> (go to article)

  • Overview: Cerebrospinal Fluid Leaks: <https://chiaribridges.org/cerebrospinal-fluid-leaks/> (go to article)

  • Spine Pulled Tight – A Guide to Understanding Tethered Cord Syndrome: <https://chiaribridges.org/spine-pulled-tight-a-guide-to-understanding-tethered-cord-syndrome/> (go to article)
WARRIOR STORIES (TAB)
  • Listing of stories: <https://chiaribridges.org/warriors/> (go to article)

  • The Michelle Cole Story – A Chiari Warrior’s Journey: <https://chiaribridges.org/the-michelle-cole-story-a-chiari-warriors-journey/> (go to article)
MISCELLANEOUS
  • Donations: <https://chiaribridges.org/donate/> (go to article)

  • Volunteer: <https://chiaribridges.org/join-the-cb-volunteer-team/> (go to article)

  • Share Your Story: <https://chiaribridges.org/submit-your-story/> (go to article)

  • Submission Agreement: <https://chiaribridges.org/submission-agreement/> (go to article)

  • The Chiarian’s Glossary: <https://chiaribridges.org/glossary/> (go to article)

Colton had just finished his Chiari Decompression. He was headache free and doing great! His neurosurgeon came to me and said, “This is a genetic disorder and Emmalyn should be checked.” Two weeks later I took Emmalyn into the Chicago area to be sedated for her first MRI of the brain and spine. Three hours later I met Emmalyn in recovery where she came out of anesthesia smiling. Emmalyn was three years old and asymptomatic, not even a headache. Colton’s neurosurgeon called me the next day and let me know that she was only 5mm herniated, but in the spinal MRI, she had two separate syrinxes. A smaller one in her cervical spine and a very large one in her thoracic spine. Her opinion was to decompress Emmalyn right away as she was very concerned about the two syringes and explained, “With the decompression, it should allow the syringes to dissipate.” So, we scheduled her first decompression for two-weeks later, on July 31st, 2012. Emmalyn went through her first decompression like a champ and was released from hospital after three days. This is when Emmalyn’s headaches started. It seemed like once a day she would be getting a headache. Two weeks into recovery Emmalyn was sitting in a chair and said that she felt sick to her stomach, so she ran into the bathroom, I followed and as she was heaving her head went deeper into the toilet. I picked her up and her eyes darted to the right and she couldn’t talk to me. I immediately called 911. When the paramedics arrived, they swept her from my arms and rushed her to the ambulance. They kept her in front of our house in the ambulance for about ten minutes before they came and told me she was having a seizure; they didn’t want to jostle her head, so they called the helicopter. We were told to meet the helicopter at the hospital, which is forty-five minutes away. My husband and I jumped in our van and I don’t think I have ever seen my husband drive so fast; we beat the helicopter there. When the helicopter landed, we were in the ER to greet her when she came off the elevator, and when she did, she was covered in blood and screaming. I looked to the helicopter nurse and she said, “Two minutes before landing she came to and she wanted her mom and pulled out her IV.” She had a forty-five-minute seizure. We are in “small-town,” USA, so the ER did a quick MRI, bloodwork, but no EEG and at the time I didn’t know she should have had one. So, they said she was fine and released her from the hospital. Of course, after speaking with her neurosurgeon the next day she had us in the car to make the five-hour drive for her to be admitted. After three days of tests, they concluded that she had chemical meningitis and put her on medication and anti-seizure meds for six months. Let me tell you that was the scariest time of my life, but Emmalyn took it like a champ. In three months, we had repeat scans, her decompression site looked good, but her syringes didn’t change in size, so we chose to wait and see.

In 2013, Emmalyn developed leg pain and started having incontinence. She potty trained early and never had problems. After going through more imaging and urodynamics they said that she didn’t look like she was tethered but they were sure that she was. They called it Occult Tethered Cord. So, Emmalyn underwent a tethered cord release on September 27, 2013. She only spent one night in the hospital and was up and doing all well, so they let her go home. After the release, her incontinence subsided but the leg pain continued.

Over the next two years, we monitored Emmalyn and her headaches continued. In 2014, we did our next repeat MRI and it showed that Emmalyn’s syrinx in her thoracic spine had gotten a little longer in length. It concerned her then neurosurgeon and she made the decision that we should do another decompression in hopes to reduce the size of Emmalyn’s syrinx. On July 1st, 2014 Emmalyn went for her second decompression. Once again, she came through everything like a champ. One thing about Emmalyn she is a fighter!

For the next year Emmalyn struggled with more headaches and leg pain, so more imaging was done. She had developed scar tissue that was blocking her CSF flow once again. After the imaging, the decision was made that in November, she would undergo yet another decompression to clean up the scar tissue so that flow could be reestablished. Emmalyn underwent her third decompression on November 2nd, 2015. She sailed through surgery and the surgeon came out to talk with my dad and me. She explained that Emmalyn’s future could be complicated as she was developing a lot of scar tissue and that would make things more complex because it could continue to block the flow. The pain after these surgeries is something, they don’t prepare you for and after this surgery, it was worse than the last two. She came out swinging and hated everything. They got her pain under control, but it seemed like more pain medication was needed this time to keep it that way. This time, she spent four days in the hospital before returning home.

After the surgery, her pain got a little better. Then on December 29th, our world got turned upside down. We were at my niece’s house in Wisconsin and my mom had run her hand down the back of Emmalyn’s head and she yelled for me to come over there. Emmalyn had a large lump on the back of her head that was squishy. We rushed to the ER in Madison and they took her straight to MRI and that is when we found out Emmalyn had developed her first pseudomeningocele. It was very large. They only let us leave with her when I promised to get an appointment with her neurosurgeon within the next week. On a good note, Emmalyn won her first American Girl Doll Grace while in the ER. She was so excited. It brought a smile to her face in between the bad headaches she was having. At this point in life, Emmalyn had lived with two years of headaches and had become known as the girl with a smile. She always smiled through her pain.

On January 5th, 2016 we took the five-hour drive back to her neurosurgeon. She took one look at the back of her head, and the imaging and she said we would be going back into surgery the next day to repair. Emmalyn was an add-on to their schedule, so she wouldn’t be going to surgery until noon. Keeping a five-year-old with no food or drink after midnight to noon is quite a task, but when the prior surgeries took longer than they thought, going until 5 pm was more than a task. She was mad, tired, hungry and beyond over it. They came to get her and even with all she had been through, she smiled and said, “love you all, see you when I wake up.” She was always so easy going into the operating room. After three hours, her neurosurgeon came out and said there was a pin-sized-hole in the dura. She showed me a picture, and she repaired it with a patch. Once again in true Emmalyn fashion, she woke up swinging, mad and in pain. By this point, they had her pain regimen down to a T, which is SO important. Her recovery went well, and she was out in three days and sent home.

Once again, her headaches and leg pain continued, but she was able to be upright again. In the middle of February, the back of her head became squishy again and her headaches started to get worse when upright. I called her neurosurgeon, and as always, more imaging was ordered, and as I suspected, her pseudomeningocele was back again. She delayed surgery as she wanted to do some research. So, Emmalyn laid down for most of a month. On March 31, we returned to the hospital for her sixth surgery. She went in and did the repair and placed an EVD drain, to check the pressure and drain CSF fluid. The drain was placed for a week, and it was the happiest and pain-free I had seen Emmalyn in over two years. Her pressures were all above 28, which meant high pressure. She made the decision that a VP shunt needed to be placed. Emmalyn went in for her seventh surgery on April 6th. Her Neurosurgeon came out and talked to me and my parents, she let me know that if this patch was to blow and another pseudomeningocele was to appear that at this point she wouldn’t know what to do next, that Emmalyn was too complex for her. I wanted to cry in fear for the first time. My dad said then what, whatever it was we would make it happen. Thank God for my dad, my rock. She said that she would refer us to a specialist she knew of in NYC. I prayed right there this night for god to take care of my little girl and heal her. After she woke up, the pain wasn’t as bad. Shunt placement seemed to be much easier than decompression. She stayed for two more days and was released to return home.

Emmalyn’s headaches continued, and one month later her pseudomeningocele reared its ugly head. I called her neurosurgeon and she did imaging and this leak was bigger than the other two. It consumed the whole back of her head. She made the referral to the specialist in New York City, NY.

After sending all her imaging, op notes, doctor’s notes, and everything else. We consulted with the specialist’s office. An appointment was made to go to NYC and see him on June 10th, 2016. My sister, Emmalyn and I boarded the plane for our first trip ever to NYC. We met with the specialist, who went over all of Emmalyn’s imaging, talked with all of us, and checked Emmalyn over. He let us know that Emmalyn was a very complex case, that her first neurosurgeon had removed too much bone and most likely cause her to have Craniocervical Instability (CCI). He also said that he suspected that she had a connective tissue disorder called Ehlers-Danlos Syndrome (EDS), and that was why healing had been hard and why the dural patch was not holding. His surgical plan was to go in and once again patch the leak in the dura, clean up the scar tissue, place a titanium plate to hold where her brain was slumping, place an EVD drain again for a week to make sure the VP shunt was needed, and because of the EDS aspect to be closed by a plastic surgeon with muscle flaps to make sure it would hold. Before he would schedule surgery, he wanted to speak with her former neurosurgeon in depth to know what he was in for when he cut open the back of her head. He said that we could fly home and he would be in touch on when surgery would be scheduled.

Emmalyn struggled for a whole summer with severe headaches as we waited for surgery to be scheduled. Finally, at the beginning of September, we received the call that we would be returning to NYC on Sept 15, 2016, for pre-op and surgery on the 19th. Dreams come true and prayers answered. We would have to be in the city for two weeks, as she would be in the hospital for a little over one week. We returned to NYC and everything went as planned. She and I walked to the OR. She had a smile on her face, and she said, “I love you, mommy,” as she went under. As always Emmalyn woke up swinging and in so much pain. We were at a new hospital with nurses and staff that didn’t know her pain regimen, so momma bear kicked into action. At first, they wouldn’t listen when I told them that the only thing that worked for her for the first twenty-four hours after surgery was morphine, and then she could be switched to Oxycodone. Once I was finally heard Emmalyn’s pain was taken care of. Within forty-eight hours Emmalyn was up and walking with her drain and feeling great. Her pressures were still above 28 the whole week so the decision was made to put the VP shunt back in place with the valve set at 1.5. She returned to the OR on September 26th for the shunt and was discharged from the hospital the next day. The next sixty-eight days were the best days of my daughter’s life; she was finally pain-free!

On the 4th of December 2016, Emmalyn’s leg pain returned, and two days later her headaches returned when upright. I emailed her new neurosurgeon immediately. He ordered stat MRIs of the brain and complete spine. I received a copy of the MRIs on disc and looked at them when I got home, and my worst fears came true – the pseudomeningocele had returned. I emailed her neurosurgeon a few images and he called me right away. He explained that he believed the leak was back and he wanted us on a plane ASAP. We flew back to NYC in two days and my parents followed three days later. When we arrived, he admitted her immediately through the ER. He explained surgery would take place on December 14, 2016, for another pseudomeningocele repair. On the 13th of December, he came into her room and switched gears, he believed that her shunt was malfunctioning and there was not a leak. I told him that her symptoms were the same as every leak before and he said he was very sure it wasn’t; so, on the 14th they would do the surgery to check her shunt. He took her into surgery on the 14th and come out and said that there was nothing wrong with the shunt, but he dialed it to 0.5 to help with the CSF and we could return home after post-op.

We returned home in time for Christmas and Emmalyn’s headaches continued to be horrible. I kept in constant contact with her new neurosurgeon about it and he kept saying let’s wait and see. We ended up having some insurance troubles as his office no longer excepted our insurance, so we had to take out an additional private policy for Emmalyn to be able to continue to see him. After all that was solved, we returned to NYC at the beginning of March for an ICP bolt test. Emmalyn returned to the OR on March 2nd, 2017 to have an ICP bolt placed to once again check her pressures as he believed her current VP shunt wasn’t aggressive enough. Her pressures were still a little high but not like before. She was in the hospital for forty-eight hours with the bolt and then it was removed at her bedside. She screamed through the removal. He sat down with us at post-op and talked to us in-depth that he still believed that there was not a leak and that he wanted to place a new VP shunt, with no valve, but instead she would have an anti-siphoning device behind her ear to slow it down when she was upright. He believed this was the best course of action for Emmalyn. He is the specialist, so of course, why would I question it. So, we scheduled her next surgery for March 22nd.

We returned to NYC at the end of the month and she went back into her first surgery of two on March 22, 2017. In the first surgery, he placed an EVD drain to drain CSF and to check pressures again. Her pressures were normal but on the higher size so he proceeded to surgery number two on March 29th. He removed the EVD drain and placed the new no valve shunt and the anti-siphoning device that could be changed when needed. It was a very painful surgery for Emmalyn as the placement of the anti-siphoning device was behind her ear in a very tender spot, and he also let me know she was the first child he had ever placed this in. She had recovered and returned home. Her headaches continued.

We had to come back in May for imaging as no one in our area would do MRI’s on her now due to liability issues with the shunt. The pseudomeningocele was still present and her headaches were still strong. He made the decision to give it until July and if things hadn’t improved, he would open the back of her head and check for the leak.

We returned in July and nothing had improved. We sat down to discuss the next surgery and he switched gears again. He said after a discussion with his colleagues, he still didn’t think there was a leak. He then let me know he thought adding a lumbar shunt would help the situation to take care of the pain and the pseudomeningocele. Once again over my better judgment, he is the specialist, so we scheduled surgery, and her lumbar shunt was placed and set at 1.5. It was an easier surgery for her, so her hospital stay wasn’t as long. The next few days at the hotel were horrible her head pain was so she couldn’t even walk or be upright in bed without a debilitating headache. After speaking with her neurosurgeon, he said take her to the ER and have it dialed up to 2.0. Upon arrival at the ER, they checked her shunt and after her previous MRI, the resident dialed it to 0.5 rather than 1.5 (so it was virtually wide open). The current resident dialed it to 2.0 in front of me. Things improved. Recovery from this developed a whole new symptom – stomach pain. Now she had daily headaches, leg pain, and stomach pain, but through it all remained smiling as much as she could. He wanted her back in a month, for new imaging, to see how things were doing.

In a month nothing improved, she just continued to get worse. So, in August we returned for imaging and an appointment. The imaging showed that the pseudomeningocele had almost gone away. He was so happy. I was too, but her symptoms had not gone away at all and now the added stomach pain was causing even more suffering. So, he said we should return again the next month, and they would externalize the shunts to see if the stomach pain would go away and if it did, we would convert the VP to a VA to get the tubing out of her stomach.

We returned in September. He externalized her VP shunt but not the LP. Her stomach pain didn’t improve so he said it wasn’t the shunts, so he took her back into the OR to place her shunts back in place. Although I explained her headache pain was not better and I still believed she had a leak from her dura. He said he was positive there wasn’t a leak.

We returned to Illinois. Her symptoms continued to be debilitating. I emailed him at least five times a week for answers and he quit answering. So, I called his office and they would set up phone call appointments that he never kept. All attempts to contact him were ignored for three long months, while our eight-year-old Emmalyn suffered. Until the day I emailed his college about the problems she was having, asking, begging for anyone to give us a second opinion, and what do you know he called me ten minutes later. He promised he would come up with a plan, and said he thought she needed pain management as everything surgical was stable. After a week of hearing nothing, I emailed him one last time. Asking him to open the back of her head and check for a leak, if there wasn’t one, I would concede to pain management. His office called the next day to set up surgery.

We returned to NYC on December 10th for surgery on the 11th. He told me surgery should be less than two hours as he didn’t believe he would find anything. He came out to the waiting room five hours later. He took me outside the waiting room and explain to me that when he opened the back of her head, he found many holes in her dura that were causing a leak. He explained that he went further up on her head and harvested her own tissue and sealed the dura again. He told me that he believed this would secure the dura and we would never have a leak again. He said that the plastic surgeon was closing her up, and I should see her in about an hour. He assured us that he would be up the next day to talk further. What a punch in the gut. Emmalyn woke from surgery in so much pain, but the wonderful PICU team that knew her so well jumped into action. A lot of these nurses have at this point gone from just nurses to being like family to us. Our stay after this surgery was rough, she was hospitalized for ten days and couldn’t be upright longer than forty-five minutes without morphine. Her leaks were sealed, but with two shunts over-draining, her low-pressure pain was beyond belief. Only morphine by pump or IV were helping her pain, but they wanted her off of the morphine and on oxycodone before they’d release her. Her neurosurgeon was in everyday checking on her. Emmalyn’s only request was to be home for Christmas. We were eventually able to get her switched to Oxy and they said she could fly home on the 21st of December. He didn’t want to remove the shunts just yet as he wanted to keep all CSF off the back of her head so the patch would seal. We would return at the end of January to address the shunt issue.

Emmalyn came home for Christmas and spent it lying down, as the pain was at its worst when she was upright. She spent the next month that way until we returned at the end of January to return for surgery to have the lumbar shunt removed on January 31st, 2018. After it was removed her pain was still bad when upright, so her surgeon decided to go back into surgery to externalize her VP shunt and clamp it off to see if things improved, and they did somewhat. So, the decision was made to take her back into the OR again to remove her VP shunt. After three surgeries in ten days, Emmalyn came out with no shunts at all!

We returned home and as always headaches and leg pain had continued. We returned to NYC for post-op and imaging at the beginning of March. All imaging was done, and no leaks were found in the back of the head, but they noticed a “kink” in her brainstem and that her two syrinxes continued to be large. Her neurosurgeon believed everything was stable in her brain, even though the thoughts of CCI were still there. He began to focus on her spine. He sent her for a prone MRI (where she was laying on her stomach), and her surgeon said that she did not look tethered, and we were put back in the “wait and see” category.

Due to insurance reasons, we were unable to see our neurosurgeon for a while, so we were sent back to our original neurosurgeon. After consulting with her she sent us to a new neurosurgeon in her office that specialized in CCI. After doing a flexion MRI, CT, and additional testing the decision was made that Emmalyn needed fusion. She went in on June 20, 2018, for fusion surgery from 0 to C4. Recovery was very rough and wearing the collar wasn’t much better. After her fusion surgery, her headaches seemed to get better, but her leg pain was at an all-time high.

Our insurance issues were resolved and our neurosurgeon that did her fusion thought it best that we return to our neurosurgeon in NYC because he knew her case best. So, we returned in September for more imaging and the next steps. In the process, Emmalyn’s scar on her side where her lumbar shunt was placed was very painful and very large. We consulted with our plastic surgeon and he decided that the scar needed to be revised along with the one on the back of her head, revision surgery was decided to be done on October 2. After our neurosurgeon in NYC reviewed her brain and spine imaging, her thoracic syrinx was still very large, he came up with the plan to go in and check for a tethered cord. He really believed she was not tethered, and he stated if she wasn’t then they would consider shunting her syrinx at a later date. On October 2nd Emmalyn was taken back into surgery for scar revision and exploration of tethered cord. After being in the OR for forty-five minutes our neurosurgeon came out into the waiting room to talk to me. After shaving the back of her head for the scar revision they saw that the screws from her fusion were ready to come through the back of her head. He said he brought his fusion surgeon in and he decided they would probably have to remove the top part of her fusion. I agreed to do what needed to be done to fix the situation. After four hours of surgery, he came out and explained that the side scar had been revised, and after exploring for a tethered cord, he considered her “a complex tethered cord,” and he untethered everything. He then explained that they were keeping her intubated overnight so they could do a CT to make sure she was fully fused before removing the top part of her fusion. Seeing her intubated was one of the hardest things in my life. She woke up once and was so scared and tried to talk. We explained what was happening and she went back to sleep. They did the CT overnight and made the decision she was fused and removed her top fusion the next morning. She came out with no collar and was told she did not have to wear one. We were sent home two weeks later.

The weekend after we returned home Emmalyn started complaining of a bad headache and stated that she heard a cracking noise in her head, and her head felt wobbly. I immediately emailed the two neurosurgeons. The plan was to get a CT on Monday. We found out the donor bone in her fusion had broken. The plan was to put her back in the cervical collar for it to heal. In just a few days of being in the cervical collar, the headaches were horrible, and her incision opened and looked infected. After talking with the neurosurgeons and talking with Emmalyn it was decided to return to NYC to have the top part of her fusion back in. On November 5th she went back in the OR once again to have the top part of the fusion put back in place. It was then that we learned just how extensive the infection had been, and our nightmare battling it began. After fusion surgery, she was placed back in the collar and the plastic surgeon that closed her stated that part of her incision had a blackness to it and needed to be revised and would have to be back in the OR in two weeks for revision. After the revision the infection returned. They put her on antibiotics and sent us home right before Christmas, after eight weeks in the city. Emmalyn’s headaches continued and did not get better, but the incision started to look better. Her round of antibiotics ended, and her incision opened again, so I sent pictures to her plastic surgeon and he wanted us back to NYC as soon as possible. We returned on January 2nd, 2019. We saw plastic surgery, infectious disease, and neurosurgery. Infectious disease was concerned that her hardware was infected, but neurosurgery said it wasn’t. After three weeks in the city, it was decided to take her into the OR and take the infected part off and see how deep it went. It was determined to be superficial, but they decided to keep her on the antibiotics. Her headaches continued to be bad, so the decision was made to keep her in NYC for the next month to monitor her. Her incision healed well on the antibiotics, so they discontinued the antibiotics. On February 27th she was taken into the OR for another ICP bolt, to check to see if high pressure returned. When upright her pressures were at 0 to -5 and laying down, they went as high as 8. Our neurosurgeon let us know that her pressures were normal, and a shunt would not help. After the surgery was done and we were discharged her incision opened, yet again. We went for her post-op visit with the neurosurgeon and he took one look and said her hardware had to be infected and needed to be removed. After speaking with her fusion surgeon, he stated there was no way we should be removing the hardware so soon, as she was not fused. So, they decided to take her in the OR and do a complete washout and cultures and put her on antibiotics indefinitely until the hardware could be removed. On March 11th she was taken into the OR once again and had a complete wash out with antibiotics. After the cultures returned with no answers, her infectious disease doctor put her on Cefadroxil 500mg twice daily until the hardware could be safely removed. After three months in the city, we finally returned home.

Emmalyn’s headaches continued as always. It had been her way of life for seven years. Now the new symptom of nausea started and never left. We returned in May to have her hardware removed. After it was removed her upright headaches came back strong, but her incision healed perfectly. We were there for another six weeks after surgery to be monitored and because of the low-pressure headaches that I knew all too well. I asked our neurosurgeon for a CT myelogram and he refused, saying he “still believed she had instability issues and there was definitely not a leak.” After going back and forth with him for a long time he refused to listen. I decided it was time for a second opinion. I reached out to another specialist and he had many questions and agreed to see her. I let her neurosurgeon know that because all he was willing to do is have her see pain management, we were headed for a second opinion. We left NYC and didn’t look back.

Her new neurosurgeon in California was very thorough in her initial appointment and understood how much she had been through and he wasn’t going to do any intervention unless it was needed. He did a flexion MRI that showed she was fused, so instability was not the problem. He started by putting her on a medication to raise the pressures in her head, which helped some, but the headaches were still bad when upright or active. We returned home while he pulled a team together for further testing. We returned to California in September, where we met with a pain team and physical therapy. It was decided she needed a CT Myelogram as they were convinced there was a leak somewhere. After the Myelogram, we met with her neurosurgeon and it was determined Emmalyn had a leak at L1 in her lumbar spine. He recommended an epidural blood patch to repair the leak. We received a call from the pain team that the leak specialist agreed. We returned to sunny California on October 8, 2019, for her blood patch on October 9, 2019. On October 9th Emmalyn went into the OR for her blood patch. She had two blood patches placed due to the presence of scar tissue at her L1 and L2 from her tethered cord surgery. They placed one there but also did a second patch coming up from her tailbone to make sure that it would seal. She struggled for a few days in the hospital with rebound high-pressure, so her neurosurgeon put her on Diamox until we could figure out her new normal.

After Emmalyn’s lumbar blood patch on October 9th, she had five days with no pain, and the low-pressure headaches (headaches when upright) returned. (Epidural Blood Patches are much less invasive than a surgical dural repair, but they often take multiple attempts to try and seal the leak.) We went home and Emmalyn continued to suffer until we returned on November 20th for a second lumbar blood patch. Her second blood patching offered no relief at all. After being in California for a week we were sent home again to see if it got better over time. They didn’t and Emmalyn started to get discouraged (which is unlike her). After talking with the doctors, the decision was made to return to California on December 10th for a third lumbar blood patch. The third patch offered her one day of relief before her horrible headaches returned. It seems like after every blood patch the headaches would come back worse. After the third blood patch failed the leak specialist decided it was time to try fibrin glue patch as the blood wasn’t sealing. We returned to California on January 14th (causing us to miss her brother’s 13th birthday, which was a hard one for us both, but he understood the urgency to get his sister better). On January 15th Emmalyn was admitted for her fibrin glue patch in the lumbar spine. Unfortunately, it didn’t help, and her headaches came back right away. Feeling pretty defeated the doctors decided to try a patch in her cervical spine as she is known for leaks in that area. We returned home for a month and returned to California on February 4th for a cervical blood patch. She was admitted on February 5th for the patch and the next day was her 11th birthday. She developed a high fever and cough. It was a scary time as they didn’t know what was happening. They ended up admitting her to the hospital and started running tests. The fever kept coming back and it ended up after three days in the hospital she was found to have bronchitis. The fever went away and on the third day she was able to get up and her headache was gone. She was discharged and after two days her headache returned. The bad part about all these blood patches is afterward the patient must lay flat for seventy-two hours as to not blow the patch. It is a difficult process but when it works it is amazing and it’s disheartening hoping for relief each time, only to see her still in pain.

After the fifth patch, the doctors had serious discussions about what was next. Emmalyn’s headaches when upright were worse than they have ever been. After a lot of discussions, it was decided for Emmalyn to return to California on April 1st for another CT Myelogram (to check for remaining spinal leaks) with a Lumbar Puncture (to check her opening pressures), and surgical repair if necessary. The onslaught of COVID-19 hit. With so much unknown, we decided it would be safer to drive to California, rather than flying. Emmalyn and I rented a vehicle and hit the road on March 26th. We didn’t stop much and tried to do two states a day. The ride was a hard one on Emmalyn and the headaches were horrible, but we arrived in California on March 28th. Her Lumbar Puncture revealed that her opening pressure was twenty-four, which is a little on the high side, and not low like they expected. To make it even more confusing, the CT myelogram revealed a small leak still in the lumbar spine. The decision was made to do an EVD drain to drain CSF and recheck her pressures, to address the high-pressure issue first. On April 1st she was admitted for an EVD drain placement for seventy-two hours. She was put in the PICU (Pediatrics Intensive Care Unit) and at first, things weren’t improving at all. They dialed up the drain and as it was draining her headache began to improve by the last day her headache was at a two-out-of-ten, something we haven’t seen in an awfully long time. It was so great to see a genuine smile on Emmalyn. They removed the drain on Saturday afternoon, and she was discharged to the Ronald McDonald House, where we were staying. By Monday, her upright head pain returned with a vengeance. The decision was made to place a VP (ventriculoperitoneal) shunt with a Certas Programmable (adjustable) Valve.

Surgery on April 8th went as planned and Emmalyn’s headaches were all over the place. She ended up being admitted to the hospital for eight days. After a few adjustments, they set her valve to a six and discharged her back to the Ronald McDonald House. Unfortunately, Emmalyn’s upright headaches were still horrible. It was time to bring the leak specialist back into the picture. After many conversations, it was decided to do a Cisternogram, a CT test with nuclear medicine to find a leak. They had to prepare for the test so it couldn’t be done until May 5th. This time, we stayed in California, where she’d be close enough for valve adjustments as needed, and due to the pandemic, we didn’t know what travel restrictions would be implemented. Two more weeks of bad headaches.

Emmalyn went in for her Cisternogram with a Lumbar Puncture to check her opening pressure (which was normal… so the high-pressure was no longer a factor due to the shunt). The Cisternogram is generally a forty-eight-hour series of tests – beginning with an initial CT, another three hours later, another after six hours, then twenty-four-hours, and finally one at forty-eight-hours to check everywhere for a leak. Emmalyn did great and after the twenty-four-hour test, they said they didn’t need to do the next one. After a round-table discussion of doctors regarding the results of the test, it showed that Emmalyn had a leak at L4-5, and this time, they wanted to surgically repair it. On May 13th we went in for Emmalyn’s 38th surgery. Due to her constant leg pain, they also decided to do an ultrasound of her spine while in there to check her Tethered Cord area. She went in for surgery and four-and-a-half hours later the surgeon was out to speak with me. He found the leak and was able to repair it and upon the ultrasound of the spine, found that her spinal cord was complexly tethered again, stating “it was in a ball and he had to untether it.” He let me know that her EDS is severe and that her scar tissue is massive, so he went back in to try and repair all that he could.

Emmalyn was unable to walk after this surgery. That was something that I, as her mom, wasn’t emotionally prepared for, and she wasn’t either. We were prepared for the pain, as she has been through that many times, but on day three after surgery, it was time for her to get up and walk and couldn’t. With all that Emmalyn has been through, never has not been able to walk afterward. This time when her physical therapist got her up to walk, her legs would just give out on her. It was so hard and scary for her and me both. She would cry in frustration and pain, as her headache was still there and remaining at a ten-out-of-ten, around the clock. Her back was hurting worse than her head and now, she couldn’t walk. After a week in the hospital, she was able to walk with a walker, her back pain was still horrible and her headaches still present, so her surgeon decided another MRI was needed. She was taken for her MRI on Wednesday evening, and after a few hours, the neurosurgeon resident came to talk to us. He let us know that either there was a leak or a seroma was present by her lumbar spine and they would have to take her back into surgery the next morning. Emmalyn yelled at the resident and told him, “No, none of this is making me better it is making me worse.” He was patient and kind and told her if we didn’t do surgery it would continue to get worse yet. With Emmalyn’s blessing, I signed the consent and they took her back into surgery the next morning. After three hours they came out and told me it was a seroma and it was taken care of. By the next day, Emmalyn was ready to get up and her back was feeling somewhat better, but she couldn’t walk again. At this point, we didn’t know how long she would need the walker. We were in the hospital for another five days and over the course, she improved walking with a walker and her back pain subsided, but her headache was back all the time, and laying down wasn’t taking it away. We stayed in California until June 9th, where at this time Emmalyn was back walking on her own, another MRI was performed to check the seroma and it had fully dissipated, but her headaches were still 10/10 and even Dilaudid didn’t help. It was decided it was time to go home and give her a break from surgeries and time to heal, so Emmalyn and I hit the road. We decided to take a long route home to see friends and the Grand Canyon. (Since this has been such a long battle for all of us, I try to find ways to break the monotony of it all whenever I can if she’s up to it.) On day three of the drive (after seeing the Canyon), we stopped to rest and visit a friend, but Emmalyn was in so much pain, she was in tears and said she couldn’t continue with the drive. We had a carload of stuff, so the decision was made to pack everything in boxes and ship them, buy plane tickets out of Denver and fly home. Flights for Emmalyn are horrible, the pressure makes her headaches so much worse.

We were home for a month with no relief and returned to California on July 6th for a follow-up. It was decided to do a flexion/extension MRI to check her cervical cranial junction and to check where the cerebellum had become adhered to the brain stem. After the MRI, her neurosurgeon called and let me know it was time to surgically go back into the back of her head. He said that her cerebellum was slumping too low into the skull causing traction to the brainstem, also her 4th ventricle was severely dilated, and would likely need to be stented, but it would take a couple of weeks before it could be scheduled. We flew home on July 14th and returned to California on July 27th for what we’re hoping to be her final surgery.

We are now back in California, getting ready for Emmalyn’s fortieth surgery. The surgery is on July 31st, exactly one day after her very first surgery eight years ago. At preop, her surgeon was extremely optimistic that this surgery will help her to feel better and hopefully let her have a more normal life. The surgery is expected to be five-eight hours long as some of this is unknown territory and decisions will have to be made once she’s opened for surgery, as an MRI only tells the surgeon so much. I have every faith in her surgeon that he will do what needs to be done to get Emmalyn better.

The road with EDS, Chiari Malformation, CSF leaks, Tethered Cord Syndrome, Craniocervical Instability, and all the comorbids she’s faced, is such a long road for anyone that has it, but don’t give up, because the right surgeon or doctor will come along and hopefully be able to help make it better! Emmalyn’s story has been long and hard, and more than any little girl should have to endure. It’s impossible to go through forty surgeries in the eight years of her now eleven-year life and have a short story to tell. She’s stronger than any child should have to be and despite all the pain, she tries to maintain a cheerful disposition that brightens everyone’s day. Emmalyn wanted to share her story in hopes that it might help other children in their fight. We hope that her story will help other families understand the importance of advocating for their child, even if it means getting second/third opinions! Don’t believe everything your doctors say, research it for yourselves and push to get the medical care that your child deserves. If a surgeon is willing to do surgery but is unwilling to run tests, walk away, and get another opinion! Ask your child what they’re feeling and when they’re feeling it, as well as any changes that might be helping to relieve the pain (even if the relief is slight) because those are important details; and believe what they tell you even if you’re the only one that believes them. And beyond everything else, don’t give up and don’t give in! Fight it like you’re fighting for your child’s life because that is exactly what you’re doing – that is the fight!

*Originally published 10/2019, updated -7/2020.