“But you look so good” is what people usually say when they find out that I struggle with debilitating chronic illnesses. It is true- I wear fashionable clothes, I do my hair, I put on makeup and I have a smile on my face. Underneath it all though, is someone who is trying to live her best life with the cards she was dealt with. I grew up in southern India and none of my family members knew what Chiari malformation was. It wasn’t until I came to the United States, had a baby and hit a complete rock bottom that I found out that a condition called Chiari even existed.

The journey to diagnosis was much like putting together a jigsaw puzzle especially when my medical providers did not take me seriously. The pain came first-it started in my fingers, head, neck, knees and gradually, over the course of two years became fairly debilitating. I was initially misdiagnosed with Rheumatoid arthritis since I have a family history with it. I was on sulfasalazine, prednisone, and hydroxychloroquine for over a year. While the prednisone helped with the pain, the combo of drugs just made me sicker and sicker. I went down to 95lbs and got extremely depressed. It was easy for everyone to simply say that I was homesick and that my pain was imagined. Yes, I was depressed but not due to homesickness. It was legitimate and severe pain that existed but could not be seen or measured by a test. I was sent to a psychologist and then to a psychiatrist. I was just “the immigrant who was dealing with immigrant-related depression and anxiety.” When multiple medical providers went with that same narrative, I started questioning if it was all indeed in my mind and psychosomatic. I was starting to accept that living like that was going to be my new normal. I was training with my figure skating coach to be able to make to adult nationals. Skating gave me joy because it was an artistic sport that I could distract myself with. But eventually, the pain took that joy away from me.

During this time, I became increasingly bitter and angry with everyone around me including my family members. I felt alone and invalidated over and over. In hindsight, I can understand why it was hard for my family to believe my pain too. No one knew about the monster that was causing it. Things got even weirder when I was pregnant- my body reacted negatively to pregnancy. I had gestational diabetes, polyhydramnios and mysteriously, two rib fractures that perplexed everyone. I was induced at 39 weeks with a labor that lasted over 30 hours and ended with fourth-degree external and internal tears. While my rib fractures and severe tears were a red flag, they were apparently not a red flag enough to warrant a deeper look at what was going on. It was still easier to stick to the hysterical, angry woman of color/ immigrant narrative. The physical scars from my fractures and tears healed and the emotional scars were temporarily masked by the joy of my new baby girl. Days turned into weeks into years and pain was my normal. Having no pain was a red flag at that point.

Slowly, my esophagus deteriorated, and my lower esophageal sphincter completely gave away. My nasal septum deteriorated somehow, I developed a near-constant tremor. People were starting to see some outward signs of what I had been complaining about for years. After a motor vehicle accident, my symptoms took a drastic turn for the worse. I had to fight with my primary care doctor to get an MRI. It took me 7 years at this point to learn that if I did not fight for myself, then no one would. And I wanted my baby girl to have a mom around.

From the diagnosis of Chiari 1.5 to surgery was about 3 weeks. I liked my surgeon and the care team, so I was comfortable with the surgery. Recovery was long and painful though. I had meningitis twice after the surgery, developed chronic migraines in addition to trigeminal and occipital neuralgia. The diagnosis of central sleep apnea, MCAS and EDS came a year later. My brain stem was traumatized massively. I had to go to vestibular physical rehabilitation therapy to fully walk independently again. A year after my Chiari decompression, I had the Nissen Fundoplication to fix my esophagus. Four years past surgery, I am still recovering and learning how to manage my conditions. My care team now consists of – Anesthesiologist, Physiatrist, Neurologist, Pulmonologist, Gastroenterologist, ENT doctor, Massage Therapists, and Mental Health Counselors in addition to my close family. A huge part of learning how to manage these conditions has been figuring out what my physical limitations are and listening to my body cues. It has taken a long time to learn that it is ok to say no and that it is ok to have a LOT of mental energy but have little to no capacity to do things physically. I gave up figure skating because that kind of physical activity was causing me extreme pain. I get intense urges to skate every now and then which I give into occasionally but as the years have gone by, I am much better at gauging the pain and deciding not to do it. Loud sounds and bright lights trigger a lot of pain now, so I am better about avoiding places that I know are noisy and overwhelming. I have found peace in hiking through the wonderful trails of the pacific northwest. I have cut down on social commitments to prioritize my health over anything else. Some weekends, I do nothing except sleep all day… I have had to learn that that is OK. My husband has been a huge support and my pillar to lean on for the 11 years that we have been married. If I am tempted to do something against my better judgment, he reminds me to know my limitations. I have made peace with the fact that some people might find it impossible to hard to understand what living with Chiari and the comorbid conditions is like and that I cannot control how other people see it. My job has been a stable and joyful part of my life, but it took me a long time to accept that too since my original goal was to go to medical school. I realized my personal limitation about not being able to make it through medical residency. I want to let everyone know that there is hope at the other end of the tunnel… even though it is not in the form of a cure.

“Vi måste operera din hjärna och det måste ske nu”!

Vad gör du egentligen om någon säger så till dig? Hjärnan. Den där delen högst uppe som liksom är allt som du är. Någon måste skära i den och fixa något som är fel. Och att det finns ingen tid att tänka efter. Hur ska du kunna stanna nu och säga: Men vänta lite, det finns saker som inte går ihop och jag behöver få svar. Jag hoppas att när du har läst detta så är det just precis vad du gör, och du gör det med en stark röst för om du läser detta så är chansen stor att även du är en krigare och för denna fighten så kommer det behövas.

Våren 2017 var jag den lyckligaste människan på jorden. Vi skulle äntligen bli en liten familj. Vi hade längtat och planerat i flera år för en liten människa och till slut kröp pluset fram på stickan. Jag trodde inte jag kunde vara lyckligare än då. Tiden gick och vi levde i vår lyckobubbla. Jag var van vid att få ont i huvudet när jag skrattade, hostade eller nös och trodde att detta var något som hände alla – men helt plötsligt började jag få riktigt ont i huvudet. Läkaren på vårdcentralen sa att det hörde till graviditeten och jag skulle inte oroa mig. Jag försökte ignorera symptomen men de eskalerade snabbt med dålig balans, jag tappade styrka och koordination i armar och ben, såg dubbelt och hade stora svårigheter med minne och koncentration. Till slut gick jag till läkaren igen som nog ändå tyckte att jag skulle till akuten för och magnetröntgas. Jag kunde ju ha fått en hjärnblödning.

Efter röntgen satt vi där jag och min sambo i vårt lilla rum på akuten och skrattade och skojade som vi alltid gör. Sambons ADHD gjorde att han klättrade på väggarna och roade mig för att jag skulle slippa tänka på smärtan. Tanken slog mig: “Vi kommer bli världens lyckligaste familj, han och jag och våran lilla bebis.” En AT-läkare kom in, tittade konstigt på mig och frågade hur jag mådde. Jag svarade att jag gjorde mitt bästa trots att det var jobbigt. Han började göra några tester, sa ingenting men kollade sen på mig igen och sa: “Du har en missbildning i hjärnan. Det är därför du mår som du gör”. Han förklarade att min lillhjärna låg i kläm och att jag inte fick åka hem förrän han pratat med specialister på ett annat sjukhus. Så de la in mig. Jag visste ingenting om resan som nu började, inte ens vad det var jag hade. När jag låg i sjukhussängen så försökte jag googla mig fram till svar och undrade mest vad framtiden skulle föra med sig.

Dagen efter kom överläkaren in till mig. Han berättade att min lillhjärna buktade ut 13mm från skallbasen och att de helst av allt velat skicka mig på operation på en gång eftersom jag hade så svåra besvär, men med tanke på lilla bebisen så fick det lov och vänta tills den var säkert ute. Utanför mitt fönster började världen göra sig redo för sommaren och i kalendern stod det Juni 2017.

”Arnold Chiari” (Chiari typ 1). Så sa de att det hette och google förklarade för mig varför jag hade mått som jag gjort. Allt stämde ju in och armerad med kunskap så kändes det ändå lite tryggare att komma hem medans vi väntade ut bebisen. När jag surfade runt på nätet så hittade jag en grupp på Facebook som var för just sådana som mig och jag hade plötsligt några att bolla detta med. Några som gjort samma resa som jag skulle göra. Man kan säga att jag höll mig uppe den där tiden hemma med tankarna på att jag gjorde det för någon annans skull. För vår lilla familjs skull. Så när jag i vecka 20 kom in på ett ultraljud var jag inte alls beredd på vad som hände. Vårt barn hade inte utvecklats som det skulle och den enda utvägen var att låta kroppen stöta bort vår dröm. Jag föll rakt ned i ett mörkt hål och jag minns faktiskt inte alls så mycket av den tiden. Det var för mycket att hantera sorgen av både min egen sjukdom och att vårt barn aldrig fick bli. Världen kändes grym och jävlig.

I oktober fick jag träffa min första neurokirurg och vi fick direkt veta att det var ett allvarligt fynd och att jag borde opereras så fort som möjligt. När jag satt där så försökte jag komma ihåg alla tips på frågor jag fått från min grupp på Facebook, att jag skulle fråga om retroflexed odontoid, bindvävsstörningar och instabila nackar. Men ingenting kändes som om det hade fastnat fast jag hade studerat på alla dessa komorbiteter och jag hade inga argument när läkaren sa att det var en operation som gällde. Det var inte det att jag inte trodde att de där sakerna gällde mig, tvärtom hade jag flera symptom på EDS men neurokirurgen sa att om jag hade haft en bindvävsstörning så hade de märkt av det innan och att jag behövde en dekompression och duraplastik just i detta läge. I efterhand så kan jag se att detta var ett avgörande ögonblick och jag önskar att jag kunde gå tillbaka och ändra historien. Så du som läser detta nu, lyssna noga. Jag vill att du bestämmer dig för att kräva att din läkare utesluter alla eventualiteter som kan orsaka en lillhjärna att ektopera för det är inte alla som har Chiari som är födda med en för liten posterior fossa, som många kirurger kommer vilja få dig att tro. Jag vet att det kan kännas svårt och sitta där mitt emot någon som ska ha all kunskap och att du vill kunna lita på att det de säger är rätt och riktigt, men komplikationerna av en sådan operation OM du faktiskt har chiari av någon annan anledning är inte värda det. Tro mig, eller för all del – fortsätt läsa så ska jag förklara.

I mars 2018 tog läkarna bort 2,5 cm skallben och 2cm på min atlaskota. När jag vaknade så mådde jag bra, förutom att de glömt stänga mitt ena öga innan de la mig nedåt på britsen så det gick sönder (Yes, sånt händer). Det tog ett tag innan det läkte och jag har idag ärr på hornhinnan som aldrig försvinner. Trots ögat så kände jag mig ändå okej, men det hela varade bara i två veckor innan jag fick en kemisk hjärnhinneinflammation och fick ligga inlagd på sjukhus igen. Väl hemma så väntade och väntade jag på att få må bättre, men det hände helt enkelt inte. Huvudvärken var fortfarande jobbig, jag kunde inte ligga på rygg eller bakhuvudet utan att känna som om jag skulle svimma, jag var svag, yr och en hel del neurologiska besvär i form av känselbortfall och smärta. Läkarna försökte alla möjliga mediciner, men allt gav mig biverkningar och ingenting fungerade egentligen. Jag fick välja mellan bisarra svåra biverkningar och att stå på morfin och som du kanske vet så är inte läkare alldeles för förtjusta i opiater mot långvarig smärta. Hur mycket läkarna än försökte justera medicinerna så var det ingenting som jag kunde ta. Jag hade ont.

”Det måste gå ett år för att du ska kunna läka innan vi kan ta några beslut om din hälsa”. Jag fick höra detta flera gånger när jag försökte prata med läkarna om hur jag mådde. ”Du är beroende av morfin”, ”Du är narkoman”, ”Din smärta är inte på riktigt, du bara inbillar dig”, ”Du är botad nu och ska inte ha så här ont, din hjärna hittar på”. Detta är saker jag fick höra gång på gång. Efter en träff med min neurokirurg så fick jag en röntgen med flexion och extension för att kunna se över RO, CCI och EDS och fick snabbt veta att ingenting var fel på bilderna. Jag mådde bra enligt alla bilder. EDS-utredning skulle ta tid och det ville inte läkarna göra förrän efter mitt ”läknings år” gått. Vad jag än frågade läkarna om så fick jag samma svar. Det är inget fel på dig eller du måste läka klart. Men min kropp ville inte läka och jag skrev flera meddelanden till min kirurg utan att få svar.
”Hjälp mig, det känns som om jag ska dö”, i december 2018 skrev jag ett långt meddelande med alla symptom, hur ont jag hade och att jag trodde att min kropp höll på att ge upp och det tog inte lång tid innan han faktiskt ringde upp och vi pratade länge. Det fanns inga anledningar till att jag skulle må såhär dåligt, det enda bilderna hade visat var ett litet bråck som inte skulle påverka och en inkapslad vätskecysta. Han kände sig rådvill och sa att han skulle ta upp mitt fall med några andra kollegor och kirurger på en konferens för och se om någon annan hade någon tanke. Igen påpekade han att jag inte kunde vänta mig någon åtgärd innan mitt läkningsår var över och jag kände mig frustrerad. Jag va botad sa dom, men ändå kändes det som att jag höll på att dö succesivt.

Min främsta fiende var nu tiden och den sniglade sig fram. Snart var det februari 2019. 11 månader efter att jag fått min första operation. Jag kunde inte längre klara av en vardag, jag sov mestadels under dagarna och jag hade så väldigt ont. Min sambo tog hand om hemmet och mig. När jag låg på rygg så svimmade jag och livet var outhärdligt. Jag skrev återigen ett meddelande till min kirurg och denna gång tog jag helt enkelt farväl. Jag kände hur livet rann ur mig och det fanns snart inte mer tid kvar. Han ringde upp nästan på en gång och berättade att de nu tänkte göra en till operation. De hade fortfarande inga indikationer och ville göra några tester innan jag fick operationstid. Jag fick göra neurologiska tester, en magnetröntgen och en lumbalpunktion för att kolla trycket i skallen. Magnetröntgen fick jag göra under narkos eftersom att jag inte kunde ligga på bakhuvudet och tryckmätningen visade att mitt tryck låg lite högt. Neurologen tyckte att jag var tjock med ett par kilo extra för min längd så de la inte någon vikt vid resultaten men efter testerna så fick jag tid för operation igen. April 2019.

”Hade jag sett dessa bilder på någon annan patient så hade jag inte gjort någonting. Egentligen finns det inget problem”. Det berättade neurokirurgen för mig när jag skrevs in medans han också berättade vad de skulle göra. Gå in, ta bort bråcket, utöka duraplastiken och sätta en titanplatta för att framhäva kompressions-effekten som kom sig av att jag låg på rygg. Han hade kunnat säga vad som helst så länge de gjorde operationen och jag mådde bättre, för mitt liv var inget liv – det var ett väntrum till döden. Och det visade sig att det var just och precis det.

”Petra, detta är bland det värsta jag har sett” – orden kom ur hans mun när han träffade mig på uppvaket och han fortsatte och berätta att jag hade haft massiv ärrvävnad som de inte kunnat se på röntgen, den värsta han sett i hela sin karriär. Hade de väntat längre med en operation så hade jag blivit hjärndöd. När de öppnade skallen så hade jag ingen pulsfrekvens i hjärnan och inget flöde alls på csv vätskan. Ärrvävnaden och bråcket hade satt stopp för allt, och som om det inte var nog så hade allt också vuxit fast i varandra. Hinnor, lillhjärna och ryggmärg hade blivit som en enda stor smet.

Nu var jag återigen botad och skulle åka hem, även om jag redan på sjukhuset fick starka smärtgenombrott som var nästan omöjliga att hantera så tyckte läkarna att jag nu var fixad och skulle vara hemma. Jag önskar att jag kunde säga att läget har blivit bättre. Att läkarna nu lyssnar på mig och förstår att ärrvävnaden kan växa tillbaka men så är inte fallet med alla. En del tycker att jag är nu botad och ska må bra. Idag är det 6 månader sedan jag opererades och jag börjar nu vara i samma situation som för ett år sedan. Jag har väldigt ont, börjar tappa neurologiska funktioner och min vardag finns inte. Mina ”måsten” består numera av ”Jag måste lindra smärtan” – inget annat får rum på min planering.
För ett tag sedan ringde min kirurg och frågade hur jag mådde. När jag berättade hur jag hade det så sa han att de nu inte vågar göra något mer kirurgiskt. Jag tog då återigen upp frågan om EDS och sa att det nu var dags att utreda det eftersom att jag har så många symptom som tyder på knasig bindväv. Du kanske tror nu att han genast förstod vad jag pratade om och var med på noterna? Nej. Hans svar var: Du har väl inga andra besvär än din Chiari? ”Va? Nu räcker det! I två år har jag pratat om en utredning för att ta reda på vad som egentligen är fel på min kropp, har du inte lyssnat på mig ett dugg” Det tog stopp. Det fick räcka med okunskap och ignorans och jag väntar nu på en utredning för bindvävsstörning.

I dagsläget när jag skriver detta så vet man inte någonting egentligen gällande mig. Varför allt är som det är och varför jag blir så sjuk. Jag är ett stort frågetecken hos sjukvården, så jag gör vad vi måste göra i denna situation. Jag läser på, pluggar och kämpar. Vågar fråga och är jobbig. Jag tar hjälp av personer som går i samma skor som jag och utan communityn på Facebook så vet jag inte vad jag hade gjort. Jag svarar på frågor jag inte borde behöva svara på när jag kommer till vårdcentral eller sjukhus ”Vad sa du att du hade sa du?” , men jag hade inte orkat kämpa om jag inte haft känslan av att jag inte är själv. Jag tror inte att någonting kan hända mig nu som jag inte redan gått igenom, men skillnaden nu är att jag har mer kunskap och erfarenhet men jag är också utmattad, trött och stundtals nedslagen av att behöva slåss. Att gå igenom så pass stora och allvarliga operationer utan att bli frisk är inte helt lätt att hantera alla dagar. Man ska ju liksom inte bli lika dålig, om inte sämre – efteråt. Man ska ju bli botad.

Nu när du har läst ända hit så vet jag att du förstår att det är så viktigt att du krigar redan från början. Oavsett om de säger att du måste opereras nu nu nu – KRÄV svar. Se till att alla diagnoser som kan vara av vikt är utredda. Har du en läcka i ryggen, i kraniet, kanske har du bindvävsproblem eller för högt tryck i huvudet. Kanske är det någon anledning till att din lillhjärna håller på och trycka sig ut ur det där hålet och kanske är inte den anledningen att du är född sådan. Ta råd av de som vet och lär dig. Jag vet att det är jobbigt, jag vet att du inte vill och jag vet att det finns dagar då du bara vill lägga dig ned och gråta och slippa vara stark. Så – gör det då – det är okej, men sen ställer du dig upp igen och fortsätter kriga.
Tiden är inte alltid vår vän, oavsett om det gäller väntetid på hjälp, väntetid på operation och recept eller bara den tid du får utstå dumma frågor och människor som egentligen ska hjälpa dig som misstror dig. Tiden du måste lyssna på någon som tror att du inbillar dig eller tiden det tar innan du hittar en läkare som lyssnar och tror på dig och när du väl har den läkaren och hen lovar dig att allt kommer blir bra så är det lätt att säga “Ja” utan att tänka, men det är då tiden är som viktigast. Det är då du ska vänta INNAN någon har för alltid förändrat hur din skalle ser ut och kanske skapat värre problem än du hade innan. Så ta din tid och se till att du har alla svar innan så att du kan spendera din tid med att läka och leva ditt liv igen efteråt.

“We need to perform surgery on your brain, and we need to do it now!”

What would you do if someone said that to you? Your brain – that thing on the top of your head that is all that is you. Someone needs to cut into it and fix what is wrong and there is no time to think. How are you supposed to say, ”Stop, I need to think. There are questions without answers and I need answers.” Well, I do hope that once you have read this – that is exactly what you will do and you will do it with confidence and a strong voice. Why? Because if you are reading this, chances are you are a warrior too, and to fight this fight successfully, you will need to see yourself as a POWERFUL WARRIOR!

It was spring 2017 and I was the happiest gal in the world. We had planned and longed for years and we were finally becoming a family. I could not have been happier. At that time, I was used to getting headaches when I coughed or sneezed, but I figured this happened to everyone. I would never have thought that something was wrong, but all of a sudden I started getting real headaches and I went to the doctor’s office. They said that it was just the pregnancy and that I didn’t need to worry so I went on with my life. However, the symptoms were escalating fast and I was losing my balance, strength, and coordination, and I had major issues with remembering and focusing. When I finally went to the doctor again, they sent me straight to the ER to get an MRI to rule out a stroke.

As we sat there in that tiny room, we were laughing and giggling. My boyfriend tried to keep my spirits up and with ADHD, he was pretty much bouncing off the walls. I looked at us and thought ” We will be the happier family. Him, me and our little baby.” An intern doctor came in through the door and gave me a weird look. ”How are you doing?” he said. I said I was doing my best to not think about the pain. He started doing some tests, but he didn’t really say much. When he was done, he looked at me again and said ”You have a malformation in your brain. That is what is causing your pain and symptoms.” He explained that my cerebellum was pinched and that I couldn’t go home until he had conferred with specialists at another hospital, and then he admitted me. I had no clue about the journey I was about to take and I honestly didn’t even understand what it was I had. I tried Googling that night and I was wondering what the future would hold in store for us.

The next day the head of the department came to see me and told me that my cerebellum was protruding 13 mm from my skull and that they would have wanted me to have an operation immediately, but because of the baby – they would have to wait. Outside my hospital window, the world was getting ready for summer and the calendars now read, ”June 2017.”

They told me I had something called ”Arnold Chiari” (Chiari Malformation Type 1) and Google explained to me why I had been feeling the way I did. Everything fit and I felt a bit safer going home, armed with the knowledge that I could be helped. Browsing Facebook, I found a group of likeminded people and my world suddenly expanded and I felt like I belonged. But the one thing that kept me going was the thought that I was doing this for someone. Not me, but for our tiny family. I had no idea what was coming. One day I was going in for a scheduled ultrasound in our second trimester and the next I was no longer going to be a mom. The doctors told us that our baby wasn’t developing as it should and that they had to let my body reject it. I fell into a deep depression and I honestly can’t remember much of what happened during that time. Dealing with the loss of our dream and my own illness was too much to bear, and I just shut down. The world was a cruel and harsh place.

It was October when I got to meet my first neurosurgeon and I was told that what they had found was considered an important finding. They really wanted me to get on that table as soon as possible. Sitting in front of this highly ranked doctor, I tried to remember all the things that I was supposed to ask. Things I had learned in the group and on Facebook. The retroflexed odontoid, connective tissue disorder connections and unstable necks. But my mind felt like a non-stick surface and I couldn’t remember any of it. It wasn’t that I didn’t think it applied to me – actually the opposite. But every possible idea that I shared with my new neurosurgeon fell on deaf ears, as he would sternly tell me, ”all you have is Chiari and surgery is going to fix that.” I felt intimidated and was afraid to rock the boat. If I had a connective tissue disorder, they would have known, right? I needn’t worry about knowing this, I just needed a duraplasty and decompression and all would be fine. In hindsight, this turned out to be a pivotal point in my fight (and I hope those reading pay particular attention to this point). This is the point where I should have stopped and stood up for myself. Nobody knows our bodies better than we do, we know when something is wrong. I should have listened to my body and trusted my gut. I should have not agreed to brain surgery without additional testing to rule out the possibility of comorbids pathological to Chiari, and not just assume that the only cause of my tonsillar descent was an underdeveloped posterior fossa. Many surgeons say this and are unwilling to test us for any other pathologies before they alter our cranial anatomy. As patients, we believe our surgeons, even when we know that they are simply unwilling to test any further. They believe what is in their textbooks and I am here to tell you that sometimes, they’re wrong and their assumptions are just not correct. I know we want to believe them, but the complications that can arise if we’re right and they’re wrong are not worth it in the end. Believe me, and if you don’t, please just keep reading.

They removed 2.5 cm of my skull bone and 2 cm of the lamina from my atlas vertebra (C1) in March 2018. For two weeks I was fine until I developed chemical meningitis and was hospitalized. Well, fine is maybe an overstatement since they did forget to close my eye before the operation and it actually dried up and stuck to the operating table, scarring my cornea for life (yes, that is a thing). I waited and waited for that moment when I was supposed to feel good again. But it never came. I couldn’t lay down on my back or the back of my head without feeling like I was going to faint, my head pain was awful and I had several neurological symptoms. The doctors tried different medicines and painkillers but nothing worked or it gave me bizarre side effects. No matter what they tried, the pain wouldn’t subside.

”In Sweden, you have to be a year post-operative before we can make any kind of decisions on your health.” This is something I was told so many times and it was so frustrating. I was told that I was an addict to opioids and that I was imagining my pain. ”It isn’t real, you just think it is going to hurt.” Or, ”You are cured and there is no reason you should be in all this pain.” I was fed so many misconceptions and lies during this period, but I had to keep fighting in hopes of getting my life back. Giving up was not an option. After having new MRIs done to look at my retroflexed odontoid and the possibility of instability, I was told I was fine. The pictures were perfect and I wasn’t sick. I was told that an investigation to look into Ehlers-Danlos would take to long and my doctors didn’t think it was important to do before my year was up. They considered me ”well” and I felt worse than ever. If I was cured, why did I feel like I was dying?

In December I wrote to my doctor ”Please help me, I feel like I am about to die.” That must have triggered something in him because he called me on the phone and we talked for a long time. He told me there were no reasons he could see to explain my symptoms. I had a small herniated disc and an arachnoid cyst. Nothing that would cause my pain and symptoms. Even so – he said he would check with some colleagues to see if there was something that could be done, but he was adamant about not touching my brain until I was a year out from my first surgery.

Time was my worst enemy. I couldn’t believe how slowly it went by. February 2019 came and it was 11 months after I first lay on that table. I couldn’t manage the day to day life, I slept all through the day and I was in grave pain. If I tried laying on my back, I would pass out. My boyfriend took care of our house and me, and life was not a life worth living. Once again I tried contacting my doctor. This time I simply wrote a goodbye letter, I knew I was dying. He called me right away and told me that they were going to open me up again. There were still no indications in the images that something was wrong so they asked me to perform some tests before they would schedule me. I did neurological tests, a lumbar puncture, and a new MRI. I had to be put to sleep during the MRI because of my issues and the tests did show my pressure was a bit too high. However, the neurologist thought I had a couple of extra kilos for my height and attributed everything to me being slightly overweight. My second surgery was scheduled in April 2019.

”Had I seen these images on someone else, I wouldn’t have done anything. There is really no visible problem.” My doctor told me this when I was admitted for my surgery. He told me they would remove the herniated disc and the arachnoid cyst, extend the duraplasty and transfer a titanium plate to combat the decompression effect I had of lying on my back. I didn’t really care what he told me they would do – I would have let them put horns on my skull if he thought it would’ve helped. My life wasn’t a life – it was a passage to death’s door, and I didn’t even know how right I was.

”Petra, this is the worst case I have ever seen” – the words out of his mouth when he saw me in the ICU after surgery shocked me. He continued to tell me that I had massive scar tissue that wasn’t visible in the images and that it was the worst case he had seen in his entire career. Had they waited to perform the surgery, I would have become brain dead. I had no pulse frequency in my brain at all and my CSF did not flow. The scar tissue and the herniated disc were now blocking cerebrospinal fluid and it had all grown together like a giant lump of bad juju. Membranes, cerebellum and the spinal canal were like a big jumble.

I was once again cured and sent off to go home and heal. Despite having major issues with pain control that could not even be managed in the hospital, they figured I was fixed and ready to go. I wish I could tell you that it has gotten better, that the doctors finally started listening to me and realized that scar tissue can go back, but they still don’t. Most of them feel like I am now cured and should be fine. I am 6 months out and am starting to feel the same way I felt before surgery last time. I’m in immense pain, losing neurological functions and my day to day life is nonexistent. My pain management is all that is on my schedule and I am functioning on a day-to-day basis. My neurosurgeon called to check up on me a while ago and when I told him how I was doing he said that they didn’t dare to perform any more surgeries on me now. So I asked him about my thoughts on a connective tissue disorder. I was ready for him to give the go-ahead for an investigation. But to my surprise, he said, ”But you don’t have any more issues than your Chiari, do you?” I was done. For two year I had tried to convey what I thought was going on and told him about me and he hadn’t heard or registered a word of what I said. Enough was enough, and I told him that he needed to listen to me and start helping me feel better and find out what was wrong. I am now waiting to look further into my connective tissues and Ehlers-Danlos Syndrome (EDS).

Nobody really knows what is wrong with me and how to handle it. I am a great enigma with my doctors and I can not trust anyone to be my advocate and do the research, so I do it myself. I’ve learned to ask tough questions and not give up and I have also learned to ask for help from people in my position. What are they doing and how can I apply that to my life. Without the community, I would be lost. I would not know what to do when I get to yet another doctor or nurse who asks, ”You have WHAT?” I don’t think I would have had the strength to keep on fighting if I didn’t know that I wasn’t alone. I am pretty sure nothing else can happen now that I haven’t already been through. I have more knowledge and experience, but I am also more worn out, exhausted and sometimes just jaded from having to constantly fight. To go through two major surgeries without any relief is not easy peasy. Sometimes it just sucks – I am supposed to get better from treatments, not worse. Right?

So, I commend you for reading through to this point and hope that by doing so, you now know how important it is for you to suit up for battle. You need to be the warrior from the get-go. No matter if they tell you it needs to get done yesterday – you make sure you have your answers before you agree. Get every answer from your list: Could it be from a spinal leak? A cranial leak? Do I have a connective tissue problem? Could my pressure be causing it? Could it be something else than a congenital malformation that is causing your brain to escape your skull? Study and learn all you can, ask for help and be the pain in the *ss patient if you need to be. I know you don’t want to. I know it is rough and that some days you just wanna lay down and give up. So do that – for a day – and then stand up and fight again! We wait for a lot of things. We wait for urgent care. We wait for prescriptions. We wait for testing. We wait for imaging. We wait for a doctor who will believe us and when one finally does and promises some relief with surgery, we figure we’re done waiting. But THAT IS THE TIME TO WAIT and get all the data before you’re on the other side of surgery, where your anatomy has forever changed and they are telling you that you are healed. I know you think that you are pressed for time, but take time and make sure you have all your ducks in a row and do your very best to make sure that nothing is missed, so you can spend time healing and living life again!

When I first started getting hit with symptoms, I was a divorced, single mother of three amazing kids; responsible not only to provide for them but to see them through life, unscathed by life’s situations, and showing them that there was nothing that if they worked hard at something, nothing could hold them back. I had just started to expand in my career as a self-taught auto technician. I was a woman making a place for herself in an industry traditionally dominated by males. July 3, 2015, was the day that my life forever changed. I was brought to the hospital with stroke-like symptoms. I was having visual problems. I couldn’t walk or talk. I had no idea who I was or where I was. The whole right side of my body basically stopped working and the right side of my face was droopy. I was brought to the ER and before the doctor would even try to figure out what was wrong with me, he ordered a series of drug tests. I passed every test, so he finally admitted to me. Once on the neurology floor, more testing was done. They performed an MRI, MRA, EKG and told us that all results were normal. I later discovered that was not the case.

One doctor refused to believe that I was not on drugs. She noted in my file that while she has no evidence to support it, she believed that I am on a drug that they hadn’t screened for, based solely on “my age, single mom status, and prior good health.” She also noted that they found a Chiari, but that based on my symptoms, she believed it was irrelevant (an incidental finding). I would love to see her now and show her just how very wrong she was. I firmly believe that what she put in my medical chart is why I have had such a difficult time getting the care that I need and deserve.

A few days later, I followed up with my PCP. She went over my MRI results with me and pointed out that they found a Chiari Malformation with a 19mm herniation of my cerebellar tonsils. She told me of changes in my white matter that the radiologist said needed to be “further evaluated” and referred me to my first neurologist, who I met within August. He ordered a visual evoked potential and an EEG. Both come back normal, so he diagnosed me with migraines, even after hearing my symptoms, which frustrates me even more as I know that it is not migraines causing these issues.

At this point, I switched my neurology care to another hospital. They went over my history with me and ordered a lumbar puncture to rule out Multiple Sclerosis, which showed banding in my spinal fluid. On September 14, 2015, I was officially diagnosed with Multiple Sclerosis and opted to begin treatment and was to start on Plegridy. As I started the full doses I started breaking out into hives. The docs didn’t seem to think I should worry, so I called the drug manufacturer and they said it should be considered an allergic reaction to the Plegridy, and to discontinue using it and advise my doctor.

After this experience, I switched care back to the first hospital for neurology to get a second opinion. The new neurologist ordered a new brain MRI and one of my cervical spine. There were no changes to my brain MRI, but my cervical imaging showed a syrinx. They weren’t sure if the syrinx was of any significance. So, she referred me to the only MS specialist in North Dakota whom I would meet with, in May. The MS specialist took a complete history on me and ran a bunch of blood work to rule out other illnesses. When those illnesses were all ruled out, she diagnosed me with Radiologic Isolated Syndrome (which means that they saw similar characteristics to MS in my imaging, without MS symptoms). While in her care I continued to get worse, with symptoms progressed to include pins and needles feeling in my hands and feet, occipital headaches that drop me to the ground, cognitive decline, fatigue, weakness, some random numbness, and muscle spasms. She ordered a new MRI and once again no changes were indicated. She began to question if my Chiari was behind my growing number of symptoms. She tried to refer me to Mayo, but my insurance declined her referral.

Eventually, I started having issues walking and my gait was becoming increasingly unsteady, so I return to a local neurology clinic. They did an MRI on my brain, cervical and thoracic spine. They found a syrinx in my thoracic spine and once again they doubted the significance, along with a slight scoliosis convex. When asked what a syrinx was, they told me that it was “an old MS lesion.” I later learned that a syrinx is a cyst inside of the spinal cord caused by a blockage of cerebrospinal fluid and it damages the spinal cord from the inside out – often associated with Chiari Malformation.

During this care for MS, I kept having what they thought were MS relapses, roughly every three to four months. Each time they ordered new MRI images and treated me with high doses of IV steroids for five days in a row. Never once did this imaging ever show an actual MS-relapse or MS activity. I continually had issues with every medication that they put me on to help “try to delay the progression of the MS” (the MS that I never had). In November 2017 I started Ocrevus, which was just FDA approved that year. Around this time, I started having strange symptoms and thought them just to be side effects of the medication, not realizing that something else might be causing it all. I met with my neurologist before my second full dose and I told her everything that I was experiencing. We opted to take me off the Ocrevus and they repeated the MRI yet again. Again, the MRIs show absolutely nothing new for activity and she admits that she doesn’t know what to do for me. I am three years in at that point and never once have they seen any MS activity.

I made an appointment with yet another neurologist. I met with him on March 2019 and he immediately pulled my MS diagnosis. He instead decides that I have migraines and anxiety. He believes that anxiety is why I am completely numb all over my body. He disregards the Chiari and the syrinxes when asked about them stating that they do not cause any symptoms that aren’t of any significance. I left this appointment more frustrated than I was before and began losing hope that I was ever going to be able to figure out what was going on with me. How am I ever going to get the proper treatment when I am consistently blown off whenever I ask about a condition that was noted from day one?

I began working more closely with my PCP. I went over the last three years of my medical journey with her and told her that I felt that we really needed to dig into this Chiari Malformation that has been called out in my imaging since July of 2015, especially since I had many symptoms that may be from it. I told her about a neurosurgeon that I had been told about in Sioux Falls, SD, who specializes in Chiari. We also talk about a connective tissue disorder known as Ehlers-Danlos Syndrome and start comparing my symptoms (of which I had several). My PCP sends in referrals to the neurosurgeon, a genetic counselor, and a rheumatologist. (Because with Chiari you will more than likely have several comorbidities.)

In June we traveled down to the specialist. He went over my MRI images and stated that my herniation was 19mm (which was almost quadruple the amount that they get concerned about). That coupled with my symptoms led to discussing the need for me to have decompression surgery. Finally, after four years we know the true culprit of what was wrong with me, my brain is literally falling out of my skull. We leave with a bunch of literature for the surgery and I call his office back Friday and tell them my decision to go forward with the surgery and we started planning for me to have surgery in early September. Just as we thought everything was on the right course, my insurance drops a bombshell on me. I received a call from the specialist office, and they tell me that my insurance has declined my surgery stating that I can have it done locally by the same incompetent neurosurgeon that I met who couldn’t even measure my Chiari correctly. I have appealed this decision twice and both times I was denied. I am now pushing for a State Fair Hearing.

The last four years have been one hell of a ride when it comes to my health. My health problems have made it far more difficult to continue working on cars. As my symptoms wage war on my body, I am now forced to work on light duty and have been for the last two and a half years. I know that my days of working in a shop are coming to end as I just can’t handle the physical requirements of the job anymore. My quality of life in the last year alone has declined sharply. I used to be the energetic mom who could coach a sports team after working all day in the shop and still have the energy to keep up with the housework, now that is not the case. I manage to push on and get them to their activities, but I’m exhausted to the core. When this all began back in 2015 my kids were 8, 7, and 5. They are now 12, 11 and 9. At times I feel like I am a horrible mother because I miss the mom that I used to be. I miss the days when my kids weren’t worried about my health and when we could make plans with other families and keep them. I have lost so much of who I am thanks to the ignorance of some members of the medical community. I am losing faith in the medical profession in general. Male doctors have been the worst as I go through this journey, as women seem to have to first prove that it’s not psychosomatic before we’re worthy of being helped, even with imaging shows something to the contrary. When I present them with proven facts about Chiari Malformation, it still gets dismissed and it is extremely frustrating. The longer I go without receiving proper treatment, the more likely it becomes that some of this damage will become permanent and to me, that is not acceptable. I am fighting for my life and I will not back down until I receive the proper care, I can’t!

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 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.

The first headaches that I remember started immediately after giving birth to my first son in 1992. It was a cesarean section at Silas B. Hays 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.

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 stimulators, 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. I also started having vertigo whenever I was at any elevated height, even just a step or two up (depth perception). 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 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, and 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.

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 Type 1 and the neurosurgery department would be contacting me to make an appointment. The neurosurgeon (who was to become 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, some 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 the Chiari malformation. Those undiagnosed comorbids caused my decompression to ultimately fail, although it all unfolded over several years. In the end, it was a great deal of research, that unveiled all that we were really dealing with.

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, 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, but 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). 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.

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).

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, and 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. 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 that 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 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), that my doctors would have to remain aware of that possibility.

The electric shock feeling in my spine (Lhermitte’s Sign) that I’d had 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 EDS. From the very beginning, we knew that my cervical spine was really bad. Well, 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 under it, and every disc from C4-7 is either bulging or herniated (cervical instability). Eventually, my entire cervical spine will likely need to be fused.

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, even if (and in case) my doctors didn’t. When I started, learning, 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.

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 was not indicated on my radiology report or made known to me. 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 brainstem. This type of cyst happens when there is a blockage of cerebrospinal fluid and is most frequent when the brainstem 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, I had a decreased sensitivity to temperature for many months, 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).

After all that I had been through in my fight, I decided to see a Chiari Specialist that specialized in Chiari with EDS and in April 2017, I paid $300 to have an online consultation with Dr. Bolognese in New York (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 now. 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 syrinxes 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 Dr. Bolognese’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 officially changed my diagnosis to 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 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.

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 Amanda (like a daughter). 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. 

I was what you would consider a “typical developing child” growing up, I did not have any health issues and was able to enjoy much of my childhood. My journey to finding answers in regard my health began at 15 years old, when I began rapidly losing my vision in my left eye. I dealt with severe headaches and the doctors struggled to draw a connection to my declining vision. I went from 20/20 vision to 20/400 in my right eye and 0 vision in my left. I was considered legally blind. I had to relearn how to navigate life with very little vision.

Little would I realize then, that this would be the beginning of a long road with specialists, procedures, frustration and even more frustration. I underwent eye injections to try and reduce the inflammation, leaking blood vessels in my eyes and optic nerve issues. My case stumped some of the biggest hospitals and specialists in the Bay Area. Eventually a doctor had noticed that for over 5 years every brain MRI listed “low lying cerebral tonsils” and decided to dig deeper into this issue. I received a diagnosis of Chiari Malformation Type 1 and had a full CSF blockage. Soon after, I required my first decompression surgery to help make more room to allow CSF to flow and taking pressure off my optic nerves. Things did not change, my positional headache was worse than ever, blindness, nausea/vomiting, joint pain, neuropathy, etc. Nothing improved from surgery.

Fast forward a few years, I was told that a second decompression surgery was required, which I agreed to. It resulted in a rip in my dural patch causing a cerebral spinal fluid leak at the surgical site. After these two decompression and a CSF leak repair surgery, my vision had improved significantly yet I was worse off symptom wise than I was when I initially began noticing changes in my body. Sadly, I was told from my specialists that there was nothing more they could do for me. They referred me to the headache/face pain clinic. After many failed attempts at managing my pain with medications, my doctor mentioned that my symptoms resembled a spinal fluid leak and that there is a doctor who is navigating research and I should be evaluated.

My new leak doctor requested many tests to evaluate for a potential leak, the first being blood work and prolactin levels, brain to spine MRI’s with and without contrast, CT , digital subtraction myelogram, MR myelogram, and the list goes on. After a few months of investigating we were able to confirm that I suffer from spontaneous intracranial hypotension, meaning that I have multiple leaks or suspicious areas in my spine, that happened spontaneously (without known trauma). My doctor mentioned that my Chiari diagnosis is what is classified as an Acquired Chiari Malformation.

The reason I am writing about my story here today is to spread awareness and bring recognition to spontaneous cerebral spinal fluid leaks, because this is something that I will face for the rest of my life. I have now had two decompression surgeries, two CSF leak repairs with hemi-laminectomies and duraplasty, seven epidural blood/fibrin glue patches, and sadly with even more procedures/surgeries to come. In my case, my Chiari was not congenital; it was acquired due to my low cranial pressure from a chronic leak in my spine. Nearly ten years after the onset of my problems, I have very important answers that would’ve been extremely useful before agreeing to have decompression surgeries. My hope is that if you are reading this, and have been diagnosed with Chiari Malformation, you will take the time to consider the possibility of leaks (even if you were told that it is a congenital Chiari Malformation. If I could help one person with sharing my story, someone like me, who is struggling to navigate their care with a map that is upside down, backwards and jumbled, it is worth the time in telling my story! We must be persistent, continue to advocate for ourselves, and truly be willing to learn to educate those around us. Even with some temporary success from surgery, my spontaneous leaks can occur at any time and for any reason or no reason, they do not discriminate.

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I had a rough journey with these conditions and I am sharing it to help show how complex we can be and how much we need the medical community to step it up a notch (or ten)! I grew up in Denmark, where I lived when I was diagnosed and had my first surgeries.

I exhibited various pains already from early childhood. As very young child, I would scratch big wounds in my head to my parents’ great frustration. I also suffered from headaches at a very early age, but my older sister had done the same, so when teeth correction helped her it was also hoped it would with me – and it did. Then started the pain in my back, shoulders and arms and that pain slowly moved upwards and developed. I was sent back and forth between physios and rheumatologists but all I got was scoliosis and hypermobility. I was told it was nothing to worry about and just do some physiotherapy. I was in a bad shape when I finished my teens and despite trying, I couldn’t really manage a job. At one point, I was seeing a chiropractor for misplaced hips and he wanted to check the x-ray that the public system had done of my neck and which was deemed clear. Here I had my first “real” diagnosis, Klippel-Feil malformation of c2-c3, which I was then told, could not cause any problems and was common (which it absolutely isn’t).

Years passed by with various periods of severe pain flares, flares that felt nothing like the pains I had before. But no doctor was really believing me. I had a spontaneous collapsed lung a couple of times in this period and ended up with surgery for this (I woke up under this surgery which later also turned out to have a significance). At 25, one day I had a sudden and severe onset of symptoms – a pain in the back of my head feeling like two stabbing knives. This did not resolve and after several attempts with various medicines, that I only got sicker from, I finally saw a new rheumatologist whom again treated me with harsh accusations of laziness and psychological imbalance. I can assure you he was the one bringing on my tears that day, despite the extra severe pain I had been in for weeks. I was placed in the care of the hospital physios and after a while, it became clear to them that there was something really wrong and they got me to see another rheumatologist, who in turn took their word and referred me for an MRI. I had only just turned 26 when I was diagnosed with Chiari 1 Malformation and Syringomyelia – in my full spine.

I did a bit of internet research as information in Danish was very sparse and realized there was a certain number of bad outcomes due to something called Ehlers-Danlos Syndrome, Retroflexed Odontoid and Basilar Invagination. I asked my neurosurgeon, who was supposed to be the best in Denmark at this point, about these things – he claimed I didn’t have any. However, he did agree to refer me for Ehlers-Danlos evaluation. Here I was told I did not have that either. However, I was bordering on a similar connective tissue disorder called Marfan Syndrome, which they still could not diagnose me with due to my heart and eyes not being faulty. I had my first decompression surgery in December 2006. It was rough. I reacted badly to anesthetics and to the morphine and I also lost a lot of spinal fluid. I could not raise myself up the first month which I thought was normal. Slowly, I got better, and imaging showed my syrinx shrinking. Two and a half years later, though, I started experiencing dizziness and nausea and though my first surgeon didn’t believe me, imaging finally showed a big collection of fluid outside my spinal cord originating from a hole in the duraplasty used to close after tonsillar cauterization at my first surgery. I tried talking to the surgeon about concerns of Klippel-Feil and instability, that I had read about, but they would not hear of it and said that for now they would just focus on this issue. So, this was repaired, and I moved to Spain with my boyfriend at the time. I was placed on a disability pension from Denmark and that enrolled me in the Spanish public healthcare. I did, however, in the meantime follow up on my concerns and contacted a specialist, who had written about the Klippel-Feil and Chiari connection, and he straight away stated I had some severe issues with my odontoid and needed it removed and my neck fused to my skull. My first meeting with a surgeon in Spanish health care came up and he just looked at my imaging two minutes then stated my problems were way bigger than Chiari and Syringomyelia. He also diagnosed a severe retroflexed odontoid and Basilar Invagination – so severe he had a hard time understanding how I could breathe, let alone walk. But given my reasonably good condition, he opted to postpone these surgeries as they are big and not without risks.

A couple of years of enjoying the benefits the climate change gave me (and likely putting my head in the sand) went by but then I could no longer ignore the fact that I was getting worse. I was in a rough period with other matters of life, so it took a while before I realized I couldn’t escape the changes in my body. I started losing weight amongst other things and after a quick detour of fear of stomach cancer, I finally realized that everything that was going on was related to my brainstem compression. So, I went back to the neurosurgeon. He ordered some testing but before it could be done, I ended up admitted urgently after I stopped breathing one night. From here started a roller coaster. I didn’t feel right about their suggestions and the surgeon that was going to operate didn’t feel very secure himself even. I ended up getting transferred to a private hospital in Barcelona that calls themselves a “Chiari Institute.” Had I known what I do now, I would never have paid the fee for a filum release, but the doctor claimed this was what I needed and well… It was worth a shot in this urgent situation. He then sent me home, claiming I was cured. I didn’t feel right and breathing through the night was still a problem, so I started sending my imaging to experts around the world and working on getting referred to another hospital in Spain’s public health with higher expertise. All these experts claimed I wouldn’t have long to live unless I had this odontoid approached.

An American expert, however realized I had even more going on and that my gut feeling about the first Spanish hospital was correct – when I confronted them with these things they backed out. He also explained to me why he thought I indeed had this Ehlers-Danlos Syndrome too. Fast forward, long and rough fights with health care and the Danish system that refused to take any responsibility despite the first doctor who didn’t see a bone poking more than a centimeter into my brain and almost crushing my brainstem, another public health Spanish doctor who was mortified they had diagnosed me like they did in Denmark and changed my Chiari 1 diagnose to a Chiari 1.5, I finally found some Spanish doctors in private care, that I would trust to take on my case – and that my parents could just find the money for, through a loan on their house. Ideally, I would have gone to this doctor in America, but price and decline didn’t allow.

October 2016 I finally had a partial odontoidectomy and a fusion, which beyond doubt saved my life. It was a rough ride, for both me and the surgeons. They had to deal with complications related to my anatomy, to the mess the first surgeon in Denmark had left – he had indeed damaged my muscles more than I ever knew – and to the problems relating to the soft tissue. I do know they did a great job, but due to all the mistakes, how complicated my case was and is, I am unfortunately not done. I have ongoing issues and though some of these could have been avoided with the right approach from the beginning, some are just the way it is with these conditions.

I hope that my story will inspire other to take a step back and get better investigated – by real specialists – before letting anyone start cutting. Also, I hope it will serve to see how much we need the medical community to step up and keep up to date with research. These are serious conditions and doctors all over the world are literally playing with our lives. Please help spread awareness – educate yourself and others and ask for raising the standard of care for these complicated conditions.

 

My introduction to Chiari malformation I (CMI) begins in 1994. I had been married about 7 months and we had just celebrated our first Christmas together as newlyweds. Shortly after the new year, I developed a bad headache that eventually evolved into losing my eyesight in one eye. I went to the eye doctor, who immediately sent me to the hospital. I was diagnosed with Pseudotumor Cerebri and Papilledema, which are known to often accompany Chiari (co-morbid conditions). At the time, we were told that it was likely due to a virus. I had five failed lumbar punctures and finally a successful sixth in radiology, was given Diamox, and the problems went away. Nothing was ever said about Chiari or an abnormal MRI. I also had no idea that I could or should get a copy of the MRI from the hospital, so I could keep my own records. I wasn’t even given any reason on why it might be necessary. I trusted my doctors and they helped resolve the problem with my sight. Little did I realize that it was only the beginning and I was in for the fight of my life!

From 1994 until 2005, I had few further significant issues. I continued to have headaches which I treated with Excedrin and ringing in my ears (tinnitus), which was generally attributed to the aspirin in the Excedrin and sinus issues. In January of 2005, I started having jaw pain. I saw a number of dentists and doctors who couldn’t figure out the problem. I eventually ended up seeing a doctor specializing in pain management who indicated he thought it was Trigeminal Neuralgia (which is another co-morbid disorder of Chiari, but its connection wasn’t made known to us). I was sent to a neurologist, who pointed out I had a 2cm (20mm) herniation, which he said wasn’t enough to worry about, but it was a Chiari malformation. Again, he was the doctor, so we just trusted that it wasn’t something to worry about. The pain worsened:  it started lasting for longer periods of time and increased in both intensity and frequency, so I went to the ER on the advice of the neurologist for pain relief. When I arrived, the neurosurgical group that I had an appointment with had left word for me to go to their office, so I could see them. Since my appointment wasn’t for another several weeks, we were thrilled to get in so fast. We were also told I had Trigeminal Neuralgia and that Chiari wasn’t related and that Chiari couldn’t kill you; although they informed me that I would need surgery. I was scheduled for a decompression surgery almost immediately and thought I was cured. The neurosurgeons indicated that a 2cm (20mm) herniation was quite significant and that I was “in good hands.” The decompression was somewhat successful in that it resolved some of my symptoms, but the relief was short lived.

I stopped taking the Gabapentin for Trigeminal Neuralgia but began having trouble with balance issues and nobody was sure why. I was told that the Chiari had nothing to do with any of this and that I was “just lucky” that all my conditions were minor. My surgeon considered Microvascular Decompression (MVD), but said I was “too tight” and the surgery wouldn’t be a good idea. In January 2007 and November 2007, I had rhizotomies performed to deaden the nerve. The rhizotomy only worked for about six months before the pain returned. I had a repeat rhizotomy in November 2007 and that has been successful to date. I was still having a lot of symptoms (that I now know to be Chiari symptoms) but they continued to assume them to be due to the Pseudotumor Cerebri, even though I didn’t have my pressure checked or any sign of a papilledema. In June 2008, I had a Ventriculoperitoneal (VP) shunt put in. It was ligated (tied off) in July 2010 since I had lost weight and the symptoms had switched from what was presumed to be high pressure to low pressure symptoms. Due to my new low-pressure symptoms, they tried several blood patches in an attempt to repair what was assumed to be a leak, even though it was never found on any of the testing. Initially, the blood patches worked well, but over time the blood patches were less effective, and I started getting headaches again.  Eventually, the doctors gave up on blood patches as they weren’t helping the headaches and finally, I stopped getting headaches (which we later found out wasn’t uncommon for those who have had long-term CSF leaks.

Despite my lack of headaches, I started having neurodegenerative problems: trouble maintaining consciousness, hypersomnolence, severe balance problems, bouts with confusion and cognitive changes. My neurologist became convinced that I needed a second decompression. He discussed my case with my neurosurgeon who initially didn’t think that another decompression would help. He was convinced to perform the surgery and I had the decompression in January 2012. The decompression was very successful for 23 days. After that, I declined rapidly. Over the next year, I developed multiple lung infections, which they presumed to be from my history as a smoker, but in reality, it was due to dysphagia. In January 2013, I was admitted to the hospital for yet another lung infection and by May, I was given a feeding tube. My wife was brought into a meeting with my neurologist where he said I needed to go to a nursing home and that my death could be imminent. I went to the nursing home and did better than they expected. Instead of dying at the nursing home, I improved and was released to go home a month later. I continued to improve enough to have the feeding tube removed that July.

Once home, my condition continued to decline. My wife continued researching and we decided to go and see a Chiari specialist since her research indicated it was the only real way to proceed, especially with a difficult case. In June 2014, we met with one such expert who was able to explain why the first two decompressions failed. Ehlers-Danlos Syndrome and Craniocervical Instability were two of the missing pieces to my puzzle. It was an eye-opening experience and finally, my issues made sense. We discussed several options for surgery, and eventually settled on a date, October 30, 2014. I had my third decompression and this time, a spinal fusion. It was a wild success! Many of my symptoms were resolved immediately. While it was not a cure, it certainly helped me substantially in restoring many functions and my quality of life.

However, in September 2015, I went into a coma while hospitalized for a reduced state of awareness (which coincided with weaning off the Decadron) and the local doctors said it was another failed decompression. In October 2015, I had yet another MRI. While going over the radiologist report, my wife noticed something none of my doctors mentioned, it stated that I had severe Intracranial Hypotension. My wife sent my MRI images to a leak expert and my NY neurosurgeon for further assessment.

In January 2016, I was in another hospital across the country having imaging for the known CSF leak. After the testing was done, a leak was found in my lumbar spine and a location they felt was suspicious on my thoracic spine (where I had old stress fractures and incredibly thin dura, believed to have been causing leaks). There were also bone fragments next to the fractures, so the doctor double reinforced my spine in that area and performed a dural reduction surgery in parts of my thoracic and lumbar spine. I got better until August 2016, when I started experiencing symptoms of a CSF leak. Upon consultation, we decided a blood patch would be the place to start and it resolved my symptoms quickly.  As of August 2018, the blood patch has continued to keep me from leaking and no leak symptoms have occurred.  While I’m going to be at risk for leaks, and likely leak periodically due to Ehlers-Danlos Syndrome, I will take the results I’ve seen thus far.  It’s also interesting to note that while I’ve had a number of unsuccessful patches, this time the post-patch recovery protocol was different in that I laid flat for 2 hours post patch, then 3 days lying flat except to go to the bathroom and eat.  I’m convinced that this protocol helped immensely, as the weight of the CSF Is much greater when upright thus increasing the chance of a leak.  Also, a blood patch doesn’t end the leak repair, it merely starts it.  Once the clotting effect has finished, the next stage occurs, which includes tissue growth to repair the opening.  With Ehlers-Danlos Syndrome, this process is often diminished and requires longer than the normal recovery time, which is why I believe many of us have had failed patches.

I am still continuing to heal, and likely will never be returned to my old self.  I have been discharged from physical therapy as of December 2017, however I still exercise every day for at least an hour.  I know that this is a key part of my healing; there have been a few occasions when I was unable to do my exercises for a few days I notice difficulty in doing my daily activities.  In addition, I will not likely be able to work as a paid employee ever again, however, I have been able to provide some help to a variety of people.  I enjoy helping others and this works well as if I have a less than optimal day, I can just let anyone who needs my help know that I won’t be available to help them.

My recovery hasn’t been without trouble, as I returned to the hospital once, in the spring of 2018, for what was presumed to be a gall bladder problem.  Since I’ve suffered issues with kidney stones, it’s not surprising to me that I have a lot of gall stones.  I suspect it has to do with some of the gastrointestinal issues that hEDS brings, but there is nothing definitive.  After 2 days of pretty significant pain, the pain subsided and nothing more came of it.

Overall, there have been a number of positive outcomes and I wouldn’t change the decision to have my third decompression & fusion.  This has granted me the ability to lead a life, which while not “normal,” is fulfilling.

I am not, and never will be, completely healed. Many of my symptoms have resolved to the point where I can tolerate them and at times, don’t even notice them. While there is no cure for Chiari or the Ehlers-Danlos Syndrome causing it all, there is more healing possible. They are difficult disorders for many reasons and one of the biggest issues is the way it presents itself; for each person, it can be entirely different, making the diagnosis very difficult. I will always have to be monitored for leaks each time the symptoms present themselves, but for now, I find myself thankful to be alive and so very lucky to have the support I do, especially from my wife, my hero!

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*Updated August 2018

Bridges are built as a means to assist people in getting over the things that obstruct their path. We are constantly facing obstacles when it comes to our health, so we decided to build a bridge! Chiari Bridges is a non-profit network built by Chiarians for Chiarians. Our goal is to provide information, substantiated by published studies, in a way that is easy to understand. We encourage readers, to not only read our articles, but look up every study for themselves and read that too. Print it out and sit down with a highlighter; read it as many times as necessary until you understand; then use it to talk to your doctors. Our doctors are under no obligation to read our articles, but they should be willing to read officially published studies from journals, especially those specific to their profession. As many of our authors are patients as well, all authors sharing original pieces will be writing under pseudo-ghost names, to protect them from being black-listed by their doctors. Chiari Bridges is not affiliated with any physician or medical group. You should in no way use this site as a replacement for diagnosis, treatment, or medical from a qualified medical professional. The information provided on our website is strictly for educational purposes, in order to help further the understanding of Chiari, and its associated conditions. Our goal is to share our collective experiences and the information we have gathered, with those in need of answers, that it may increase your knowledge and guide you in conversations with your doctors.