One of the complications that many of us EDSers/chiarians deal with is high intracranial pressure. The cerebral spinal fluid accumulates in our brain, builds up and puts too much pressure around brain tissue. There are different treatments, depending on the cause. For some, having surgery to correct whatever is blocking the flow will lower the pressures. For some, medications such as diuretics will lower the pressures. And, for some, shunts are inserted to drain the excess fluid and keep the pressures down.
I have high intracranial pressures, also known as intracranial hypertension (IH). I have too much fluid circulating in my brain and its making me more sick. I take 2 different medications, Lasix and Diamox, for this. There are plenty of risks to both of these meds. And, there are side effects that come with Diamox, including neuropathy, that drive me crazy. But, I'd rather have neuropathy in all of my extremeties than deal with high pressures. Why? Let me tell you what it feels like when the pressure is up.
Have you ever had a migraine? One of those miserable headaches where you feel every heart beat and want to puke and just need a dark room? That's sorta what this feels like to me. I get a headache. It starts like a migraine. But, then it grows. My entire head pulses. It feels like something is pressing against my skull, my face, from the inside. Eventually, I have trouble holding my eyes open. I can't stand to eat and I want to puke but know that would make me worse. I have a lot of trouble thinking clearly. My reflexes slowly leave. As it gets worse, this "migraine" moves through my body. Everything else starts to behave a little bit wrong. My heart and vital signs are affected, I breath much deeper, I develop tremors and twitches. It starts to feel like I really am dying. Or, hurts bad enough that I want to. I've tried everything from migraine meds to pain pills to ice and heat and dark and sleep. Nothing fixes this pain except one thing....diuretics. Pills meant to pull the excess fluid from your body. They move some of that fluid from my body and my pressures come down and I can function again.
So, when you hear mention of someone's pressure in their head, this is sometimes what they are talking about. High intracranial pressures, aka intracranial hypertension, aka idiopathic intracranial hypertension, aka pseudotumor cerebri.
Friday, April 11, 2014
Friday, April 4, 2014
Why Maryland?
I live in Kentucky. When I tell people that I travel to Maryland for care and treatments, they look at me pretty funny. Its rare that anyone asks me why, though. Oh, they probably ask behind closed doors. But, they don't ask me. No doubt people have said things about the costs of what I do, how I could afford it if I did it here, what kind of doctor I see that requires so much money. So, I'm going to explain it all. I'll tell you why I, and a whole lot like me, travel very far to do what we do.
If you've read some of these blogs or know me, you know that I have Ehlers-Danlos, chiari and a host of other disorders. Before you knew me, did you know what Ehlers was? No? Its likely that your physician didn't either. Ehlers is a multi system disorder, though. It affects my skin, bones, heart, GI system, nervous system and more. So, what do I do if my doctor doesn't understand it and your doctor doesn't understand it? I have to look for one that does. Most of us EDSers have been on that mission for years. We find doctors that say its only a joint disease, doctors that say we shouldn't have pain with it, doctors that say it has nothing to do with our heart, our thyroid, our brain. Some say its a somatic disorder, meaning its imaginary. Some say there is no treatment. And some just refuse to discuss it. I've met most of those. I called tons of doctors between home and Ohio just looking for one that could help us. I found a primary who knows more than some but still not everything. She's a great person but I have more needs than she can deal with. I found a geneticist in Cincinnati that gets it but it takes months to see him and he doesn't manage all of our care. Still, he and my PCP were enough if EDS had been my only problem with no complications.
Then, one day I learned that I had chiari. A piece of my brain was falling into my neck and no one understood that. I'd probably need a neurologist so I called them. I couldn't find any who would treat me. Some didn't know what it was, one said everyone has it. I saw one doctor who said my chiari was caused by my fat. Wow. I'd gotten so fat that the adipose tissue must have ran out of room and been shoved up into my head. Maybe that's what forced my brain into my neck. I kept calling people, reading doctor's online bios, researching things on the internet. And that's how I heard of Dr. Fraser Henderson. I was told that he was the greatest neurosurgeon, he specialized in chiari, he understood EDS. BUT he was in Maryland. Google maps told me that Maryland was 600 miles from me. No way was I traveling that far to see a doctor. Yeah. If you get sick enough, you will eat those words.
I continued to decline, lose some muscular function, have more neuro problems. I saw a dead end coming with my job because I was becoming so ill. So, the hubby and I discussed it. Over and over. I know I made him crazy. But, eventually, we decided we would go to Maryland. We'd make it into a vacation. I'd see this miracle performing doctor, the kids could see DC, maybe travel to the beach. One day last July, we packed up a rental truck and went to Maryland. The trip didn't turn into the vacation we planned because this doctor found things wrong that no one else had and ordered more tests and evaluations. I cried at that appointment and I never do that. He is the most compassionate doctor. He understands more details about EDS than I do and I have it. He found all of my problems within an hour. I was in shock. It wasn't long before my first surgery was scheduled with him. And then another one. Those surgeries gave me back the use of my legs and arms. So much of my pain was gone. He fixed what others didn't acknowledge. That's worth traveling so far isn't it?
Most of you know that we've worked hard to raise large amounts for deposits. There is a reason, though. Yes, he requires large deposits such as the $4000 I had to pay upfront for my tethered cord surgery. But, he is an out of network provider with my insurance. That means that after they pay him, he can balance bill me the rest. All of you know how much that can be, right? But, he cuts us slack. If we pay the deposit, we don't pay him another dime for that surgery. We don't come out in dept. We save thousands and thousands in the long run. And, we have the privilege of working with a surgeon who saves us, not butchers us.
This month I go back to see him again. I have to stay a few days longer than we expected. I have to have more tests ran. But, this time its all relating to my brain. Once again, we can't afford this trip but it has to be done. I much prefer to suck up the cost, beg a little, give up a lot and live. Chiari kills. I trust him to keep me alive.
If you've read some of these blogs or know me, you know that I have Ehlers-Danlos, chiari and a host of other disorders. Before you knew me, did you know what Ehlers was? No? Its likely that your physician didn't either. Ehlers is a multi system disorder, though. It affects my skin, bones, heart, GI system, nervous system and more. So, what do I do if my doctor doesn't understand it and your doctor doesn't understand it? I have to look for one that does. Most of us EDSers have been on that mission for years. We find doctors that say its only a joint disease, doctors that say we shouldn't have pain with it, doctors that say it has nothing to do with our heart, our thyroid, our brain. Some say its a somatic disorder, meaning its imaginary. Some say there is no treatment. And some just refuse to discuss it. I've met most of those. I called tons of doctors between home and Ohio just looking for one that could help us. I found a primary who knows more than some but still not everything. She's a great person but I have more needs than she can deal with. I found a geneticist in Cincinnati that gets it but it takes months to see him and he doesn't manage all of our care. Still, he and my PCP were enough if EDS had been my only problem with no complications.
Then, one day I learned that I had chiari. A piece of my brain was falling into my neck and no one understood that. I'd probably need a neurologist so I called them. I couldn't find any who would treat me. Some didn't know what it was, one said everyone has it. I saw one doctor who said my chiari was caused by my fat. Wow. I'd gotten so fat that the adipose tissue must have ran out of room and been shoved up into my head. Maybe that's what forced my brain into my neck. I kept calling people, reading doctor's online bios, researching things on the internet. And that's how I heard of Dr. Fraser Henderson. I was told that he was the greatest neurosurgeon, he specialized in chiari, he understood EDS. BUT he was in Maryland. Google maps told me that Maryland was 600 miles from me. No way was I traveling that far to see a doctor. Yeah. If you get sick enough, you will eat those words.
I continued to decline, lose some muscular function, have more neuro problems. I saw a dead end coming with my job because I was becoming so ill. So, the hubby and I discussed it. Over and over. I know I made him crazy. But, eventually, we decided we would go to Maryland. We'd make it into a vacation. I'd see this miracle performing doctor, the kids could see DC, maybe travel to the beach. One day last July, we packed up a rental truck and went to Maryland. The trip didn't turn into the vacation we planned because this doctor found things wrong that no one else had and ordered more tests and evaluations. I cried at that appointment and I never do that. He is the most compassionate doctor. He understands more details about EDS than I do and I have it. He found all of my problems within an hour. I was in shock. It wasn't long before my first surgery was scheduled with him. And then another one. Those surgeries gave me back the use of my legs and arms. So much of my pain was gone. He fixed what others didn't acknowledge. That's worth traveling so far isn't it?
Most of you know that we've worked hard to raise large amounts for deposits. There is a reason, though. Yes, he requires large deposits such as the $4000 I had to pay upfront for my tethered cord surgery. But, he is an out of network provider with my insurance. That means that after they pay him, he can balance bill me the rest. All of you know how much that can be, right? But, he cuts us slack. If we pay the deposit, we don't pay him another dime for that surgery. We don't come out in dept. We save thousands and thousands in the long run. And, we have the privilege of working with a surgeon who saves us, not butchers us.
This month I go back to see him again. I have to stay a few days longer than we expected. I have to have more tests ran. But, this time its all relating to my brain. Once again, we can't afford this trip but it has to be done. I much prefer to suck up the cost, beg a little, give up a lot and live. Chiari kills. I trust him to keep me alive.
Saturday, March 29, 2014
Overachievers 'r us
Why do we all feel the need to overachieve? We push ourselves so hard and miss out on so many parts of our life. I went through nursing school when my kids were very young and now I look back and see how much I missed. But, I was doing it for the good of my family. I was doing it so that I could give them a better life.
Now, I am in grad school, striving to be a nurse practitioner. It doesn't matter that I'm not well enough to work now. I have hopes that I will be, eventually. Still, I have bulldozed my way through school while I was sick. I have held on, with a 3.5 gpa I might add, through 4 terms and 2 major surgeries. I know that I'll need my brain surgery, the decompression, soon because I am getting worse. I am completely torn about taking a break from school for it. Why? Why would I not automatically put myself and my health before my education?
Lets list some reasons.
I feel like I am a failure. I feel like a quitter. I feel like I'm letting people down. I feel like people will see how weak I am. I feel like I have to prove myself. I feel like a lazy bum.
Does any of that make sense? No. My logical brain knows that it doesn't. Yet, those are the hang-ups that I have. And, I bet a lot of people know what I'm talking about. I bet a lot of people push just as hard as I do. So why do we do it? Why do we have to be the best? Why do our kids have to be the best? Why do we expect perfection today?
I've got to try and learn to take care of me first, family second and then school. Time to figure out and apply my priorities.
Now, I am in grad school, striving to be a nurse practitioner. It doesn't matter that I'm not well enough to work now. I have hopes that I will be, eventually. Still, I have bulldozed my way through school while I was sick. I have held on, with a 3.5 gpa I might add, through 4 terms and 2 major surgeries. I know that I'll need my brain surgery, the decompression, soon because I am getting worse. I am completely torn about taking a break from school for it. Why? Why would I not automatically put myself and my health before my education?
Lets list some reasons.
I feel like I am a failure. I feel like a quitter. I feel like I'm letting people down. I feel like people will see how weak I am. I feel like I have to prove myself. I feel like a lazy bum.
Does any of that make sense? No. My logical brain knows that it doesn't. Yet, those are the hang-ups that I have. And, I bet a lot of people know what I'm talking about. I bet a lot of people push just as hard as I do. So why do we do it? Why do we have to be the best? Why do our kids have to be the best? Why do we expect perfection today?
I've got to try and learn to take care of me first, family second and then school. Time to figure out and apply my priorities.
Tuesday, March 25, 2014
Zebras AND Pandas?
My body has become a zoo. Zebras and Pandas everywhere. I bet you wonder what I'm talking about. Think I've taken too many meds or have started tipping the bottle. So, I'm going to explain why you'll find more and more of these black and white animals around me, on me, in my house, etc.
Most don't know that a Zebra is the nickname for a person who is a medical mystery. In medical school, or nurse practitioner school as my case is, you learn a saying. I remember reading it in one of my books during my very first term of grad school. It goes something like this:
Most don't know that a Zebra is the nickname for a person who is a medical mystery. In medical school, or nurse practitioner school as my case is, you learn a saying. I remember reading it in one of my books during my very first term of grad school. It goes something like this:
If you hear hoof beats think horses, not zebras.
What they mean is, when a patient comes in with a complaint, look for the easiest and most obvious reason. Don't get excited and waste time and resources looking for rare diseases. Those of us with Ehlers-Danlos use the zebra as our sign. Its on our awareness ribbons, we tend to collect zebra patterns. We have a rare disease. If a physician isn't looking close enough, they will see pieces. A fluctuating or high heart rate. Chronic pain. Arthritis. Hypermobility. Gastric issues. So they will diagnose easy things such as tachycardia, fibromyalgia, arthritis, irritable bowel syndrome. But if you pull the whole puzzle together, you'd find a rare disease, a zebra. Sometimes the symptoms are not just small issues. Sometimes they are puzzle pieces.
Where do Pandas come into this? Well, a handful of us EDS zebras have been found to have odd symptoms that couldn't be explained by EDS. One of our genius doctors started looking deeper and made a discovery. Pandas! Really, its an acronym for pediatric autoimmune neurophysciatric disorder associated with strep. Its an autoimmune disease that, until lately, was diagnosed in pediatric patients. Turns out, though, that there are some of us out there that have it as adults, have probably had it for years and years, untreated. It presents as anxiety, depression, OCD, mood swings, tics, tourettes, tremors, seizures, and a lot more things that I can't think of. It looks like a psych issue. But, if you look at our blood, you'll find that we have a problem. When we are exposed to certain disease, and its different triggers for many of us (strep, lyme, coxsackie, mono, pneumonia, etc), our body creates antibodies. In a normal person, an antibody is the way the body remembers and recognizes that disease on repeat exposure and can make what it needs to attack and kill it. In a person with Pandas, we make the antibodies long after the disease is gone. Those antibodies become confused and see the brain as the bad bacteria or virus. So, it sends armies of antibodies that somehow cross the blood/brain barrier and attacks our brains. They are literally trying to kill it like a disease. This triggers the odd behaviors. I mean, wouldn't you act odd if you had an army of antibodies climbing over and attacking your precious brain cells? Treatment varies, depending on the trigger and how long you've had the disorder, among other things.
I have both Ehlers-Danlos and Pandas. Not only is everything made of connective tissue inside me trying to fall apart, but my brain is being attacked! So, there you have it, zebras and pandas.
Oh....don't forget to share my page. And mention to your friends that there is a link to our fundraiser at the top of the page. Zebras and Pandas can be quite expensive!!
Saturday, March 22, 2014
The decline (aka my whine for the day)
I hate Ehlers-Danlos. And I am sinking.
We were talking this morning, my family and I, about how this disorder has consumed my life. Sometimes I wonder, if I never knew I had all of these things wrong, would I be this sick? Yes, the logical side of me knows better. I was this sick and that's how I found out I had these things.
I go back to Maryland next month to follow up with the wizard, my neurosurgeon, Dr. Fraser Henderson. The man performs miracles. We will look at the results from my last surgery, which I do consider successful. And we will look at the symptoms that I have now and decide if we'll do the brain surgery. We have been putting it off. Some people's chiari or cranial instability are life threatening by the time they get to Dr. H. Mine was not. Well, it could be, has potential to be. But, my brainstem was not crushed like some. Mine has looked a little different on each set of imaging. So, we've tried other things first to see if it takes some of the pressure off of my cord and brainstem, hoping to avoid the big surgery for a little while. I don't know what else there is to try, though. Or, if he thinks that I am ready. I don't want the surgery. But, I want to go live again.
I'm getting more and more sick, again. For a while, I think I was feeling better. Now, I am waking up with headaches and nausea every morning again. Headaches are lasting through the day. I'm having horrible pressure inside my head some days, despite being on medications to decrease that. The pressure is probably high intracranial pressures. That's what I'm being treated for, anyway. My vision is getting worse, blurred or doubled more than its not. The bounding heartbeat, that feeling, is coming back, despite the beta blockers that had controlled it. I have neuropathy in my hands and feet. My neck has started popping when I move it and the pop makes me sick through my head. My reflex in my left pupil is becoming sluggish. And the tinnitus (which is either spinal fluid or a compressed blood vessel) is getting a lot worse in my left ear. I need a magic pill that fixes all of that. I do NOT want brain surgery. I do want to be better and work again.
The not working part, that's killing me. More than anything else, I'm going crazy because I can't work. I want to be a nurse. I want to have a paycheck. I want to contribute. I want us to stop sinking financially.
On a good note, I just finished my first year of grad school. Despite all of this. I don't know if I'll make it through or not. But I refuse to stop trying until there is no choice. I have got to think that I can give my family more, make a better life.
We were talking this morning, my family and I, about how this disorder has consumed my life. Sometimes I wonder, if I never knew I had all of these things wrong, would I be this sick? Yes, the logical side of me knows better. I was this sick and that's how I found out I had these things.
I go back to Maryland next month to follow up with the wizard, my neurosurgeon, Dr. Fraser Henderson. The man performs miracles. We will look at the results from my last surgery, which I do consider successful. And we will look at the symptoms that I have now and decide if we'll do the brain surgery. We have been putting it off. Some people's chiari or cranial instability are life threatening by the time they get to Dr. H. Mine was not. Well, it could be, has potential to be. But, my brainstem was not crushed like some. Mine has looked a little different on each set of imaging. So, we've tried other things first to see if it takes some of the pressure off of my cord and brainstem, hoping to avoid the big surgery for a little while. I don't know what else there is to try, though. Or, if he thinks that I am ready. I don't want the surgery. But, I want to go live again.
I'm getting more and more sick, again. For a while, I think I was feeling better. Now, I am waking up with headaches and nausea every morning again. Headaches are lasting through the day. I'm having horrible pressure inside my head some days, despite being on medications to decrease that. The pressure is probably high intracranial pressures. That's what I'm being treated for, anyway. My vision is getting worse, blurred or doubled more than its not. The bounding heartbeat, that feeling, is coming back, despite the beta blockers that had controlled it. I have neuropathy in my hands and feet. My neck has started popping when I move it and the pop makes me sick through my head. My reflex in my left pupil is becoming sluggish. And the tinnitus (which is either spinal fluid or a compressed blood vessel) is getting a lot worse in my left ear. I need a magic pill that fixes all of that. I do NOT want brain surgery. I do want to be better and work again.
The not working part, that's killing me. More than anything else, I'm going crazy because I can't work. I want to be a nurse. I want to have a paycheck. I want to contribute. I want us to stop sinking financially.
On a good note, I just finished my first year of grad school. Despite all of this. I don't know if I'll make it through or not. But I refuse to stop trying until there is no choice. I have got to think that I can give my family more, make a better life.
Wednesday, March 19, 2014
The Impact of Illness...
Having a chronic illness changes everything in your life. Having one that kicks in unexpectedly, becomes very serious and changes everything that you do changes everything about you.
How has EDS and all of the illness that followed changed me? Wow, where do you begin. Lets start with the social impact.
When you are ill, your friends are there. But, sadly, as things progress, many begin to drop out. At first, I was hurt and angry. At a time in my life when I needed it most, almost no one was there. With time, I've let most of that hurt go and attempted to understand. Maybe many people just don't know what to say. Maybe some just can't handle hearing about your illness so often. And maybe they just assume that other friends are checking in on you. We are humans. It is easy to not think about what isn't in front of you. But, for those of us who are ill and left behind, its a lonely life.
There is a huge mental impact. I have had a job of some sort since I was old enough to work. I've always earned money, except for a short time when my children were very young. Maybe it wasn't much, but I was out there doing it. I've always been a multitasker, enjoyed the chaos around me. Working, college, kids. Now, I am very dependent on everyone else. I miss working. I was a nurse. I cared for others. I helped save lives. Now, I have to bribe and pay my kids to help me clean the house because its almost too much. I had to reevaluate how I saw myself. I had to find new self worth. I'm still working on that one. Not there yet. I have, however, learned to see people differently. I like to think that I've learned to be more kind, a little more understanding. I'm certainly trying to.
And then there is the financial impact, itself. That one can be huge! I have had two surgeries since November. We have made four or five trips to Maryland for visits and surgeries in less than a year. Maryland is 600 miles from us. There is the cost of gas, motels, food, physicians and scans and the surgical costs. I've costs my family thousands of dollars. I am the woman who will hardly buy new shoes for herself because my kids may need something instead. So, giving this money over to care for myself has been VERY hard. And where does it end? Financially, we will slowly sink. I've not been allowed to work. Yesterday, we reevaluated and it was decided that no, I am still not released for work. They feel that it wouldn't be safe for me or my patients. Not until we fix my brain. I currently draw disability insurance from work. It is equal to about half of my previous paycheck. None of my bills go down, though. So, we have to make it on a lot less money AND come up with the money to travel and pay my medical bills. Thankfully, there have been some wonderful people, family and friends, who have done so much to help us. But, it is never ending. I have a trip coming next month and right now, I'm not sure how we'll manage it. But, we will.
With a serious illness, you have to consider the possibility that you'll die. No one likes to talk about that part. I am a nurse, though. I am not a stranger to death. Just this year, I have saw so, so many stories of others with chiari who have passed away. Young people. People who should never have died. This could be any of us. We have to fight a system that doesn't understand our illness. And, in my case, I had to come to terms with the possibility. I am a realist, I can't sugar coat it for myself. So, you accept the knowledge that it can happen, you fight hard to be sure that it doesn't and you live the best that you can.
So...there is a little insight for those that haven't been in these shoes. A glimpse at the more private side. Because, if I'm not going to let you truly know everything about Ehlers-Danlos and my other disorders, what is the point in doing this?
How has EDS and all of the illness that followed changed me? Wow, where do you begin. Lets start with the social impact.
When you are ill, your friends are there. But, sadly, as things progress, many begin to drop out. At first, I was hurt and angry. At a time in my life when I needed it most, almost no one was there. With time, I've let most of that hurt go and attempted to understand. Maybe many people just don't know what to say. Maybe some just can't handle hearing about your illness so often. And maybe they just assume that other friends are checking in on you. We are humans. It is easy to not think about what isn't in front of you. But, for those of us who are ill and left behind, its a lonely life.
There is a huge mental impact. I have had a job of some sort since I was old enough to work. I've always earned money, except for a short time when my children were very young. Maybe it wasn't much, but I was out there doing it. I've always been a multitasker, enjoyed the chaos around me. Working, college, kids. Now, I am very dependent on everyone else. I miss working. I was a nurse. I cared for others. I helped save lives. Now, I have to bribe and pay my kids to help me clean the house because its almost too much. I had to reevaluate how I saw myself. I had to find new self worth. I'm still working on that one. Not there yet. I have, however, learned to see people differently. I like to think that I've learned to be more kind, a little more understanding. I'm certainly trying to.
And then there is the financial impact, itself. That one can be huge! I have had two surgeries since November. We have made four or five trips to Maryland for visits and surgeries in less than a year. Maryland is 600 miles from us. There is the cost of gas, motels, food, physicians and scans and the surgical costs. I've costs my family thousands of dollars. I am the woman who will hardly buy new shoes for herself because my kids may need something instead. So, giving this money over to care for myself has been VERY hard. And where does it end? Financially, we will slowly sink. I've not been allowed to work. Yesterday, we reevaluated and it was decided that no, I am still not released for work. They feel that it wouldn't be safe for me or my patients. Not until we fix my brain. I currently draw disability insurance from work. It is equal to about half of my previous paycheck. None of my bills go down, though. So, we have to make it on a lot less money AND come up with the money to travel and pay my medical bills. Thankfully, there have been some wonderful people, family and friends, who have done so much to help us. But, it is never ending. I have a trip coming next month and right now, I'm not sure how we'll manage it. But, we will.
With a serious illness, you have to consider the possibility that you'll die. No one likes to talk about that part. I am a nurse, though. I am not a stranger to death. Just this year, I have saw so, so many stories of others with chiari who have passed away. Young people. People who should never have died. This could be any of us. We have to fight a system that doesn't understand our illness. And, in my case, I had to come to terms with the possibility. I am a realist, I can't sugar coat it for myself. So, you accept the knowledge that it can happen, you fight hard to be sure that it doesn't and you live the best that you can.
So...there is a little insight for those that haven't been in these shoes. A glimpse at the more private side. Because, if I'm not going to let you truly know everything about Ehlers-Danlos and my other disorders, what is the point in doing this?
Tuesday, March 18, 2014
Dealing with doctors...
I want to help you all understand what its like to visit a doctor when you have a rare disease. First, I had to get a cup of coffee in me while I thought about what I would say. Like I said before, I am new to this.
All of my friends and family know that I see a neurosurgeon all the way in Maryland to treat my condition. Many of you may not understand why I travel that far. You see, he is the FIRST that I've met who "gets it". He truly understands Ehlers-Danlos, chiari, cervical instability and the multitude of other ailments. He is a diamond in a gravel pit.
When we started this journey, we saw a lot of doctor's who discredited any suggestion that I made. But, I was just learning about connective tissue disease and really didn't have a foot to stand on. It all started with Ashley and her many trips to the orthopedist for her constant dislocations. Each time they just said that they didn't know what was wrong or why she kept stretching and tearing these ligaments. They would put her back in braces, back on crutches and send us on. Until the day we say the "new" doctor. I don't even remember his name. I should find out. He was new at the practice, though. For this visit, I had no babysitter, so I brought all of my kids. He was looking at Ashley's knees, then looked at my family of tall, thin children and asked if we had Marfans. I had only heard of Marfans, really knew nothing about it. He said that he really believed that may be our problem and for me to find out. And so began the journey.
Where does a parent start with that? At home with their primary physician. We saw an awesome Nurse Practitioner named Jodi (and, I ALWAYS recommend the NPs over MDs but I am biased). She knew a bit about Marfans. She began looking at my kids and finding things that I just hadn't noticed, as a mother. Yes, they were a little taller than average. Yes, their arm span was longer than their height. Yes, they all had a high palette, crowded teeth, history of speech problems. They had long legs, very long fingers. Yes, they were all double jointed. Three of them had scoliosis to varying degrees. A couple of them had irregular heart beats. The were things we hadn't picked up or put together. Until now. They met enough criteria (Marfan's symptoms) to consider more testing. So, they were scheduled to see a pediatric cardiologist and a geneticist at UK medical center.
This is where the fun starts. The day rolled around and we went to see the cardiologist. Let me say right from the start that he was a jerk. He wanted to know why we even thought we had Marfans. I explained their story, their symptoms. He said nope, he knows they don't have it. They are fine, just a little tall. But, he'll do an echo on each one just in case. So, one by one I went in with them while they had an echo. After, he tells me that they all look good. The twins had a slightly larger than normal aortic root but that he thought it was actually normal for their age and that the problem was they were too thin, which distorted the percentile. Otherwise, all fine. No abnormalities. We don't have Marfans. Silly idea. Go home.
Next was the geneticist. Because I brought 4 kids to be examined, they split them into two rooms with two physicians. I was with the girls. The lady, who I hear has moved on to practice elsewhere, thankfully, looked them over. Again, I was asked why are we there. Again, I go through the story. She checks them out, says sure they are a little hyper mobile, but they are fine. That I was wrong, their measurements are fine. This is when I began to learn to stand up for us. I told her that no, they are not fine. I measured them myself. I told her to not "eyeball" it and actually measure. So she did. And had to admit that I was right. They are off. So she looks them over again. Finally, after several tests, she tells us that they do not have Marfans. She says my oldest has Ehlers-Danlos. She said she had some subtype, she wasn't sure which, and not to worry about it. It was no big deal, there was nothing we could do. Then, she says that the oldest was gaining a little weight and she just needed to exercise and monitor that. And she sent us home.
It was almost a year before I figured out that Cincinnati Children's Hospital has a connective tissue clinic and a specialist in EDS. During that year, I kept pushing for answers but no one had any. So, I took Ashley to Cincy. There, we met an awesome doctor who knew exactly what was going on. He spent an hour with her, checking her, asking questions. And that day we found out that she (and probably myself as well) had EDS-hyper mobility type. We've saw him a couple of times since, over the last couple of years. He is still just as great.
Now, this is just the beginning of our story. Since then, we have saw many doctor's and been in the ER a few times. Each time, I have to explain that we have Ehlers-Danlos and give a quick, summarized lesson about it and what it does. I have to explain the connection to whatever we are having treated. I have to tell them what to look for. And then, most of the time, I am discredited. They say "no, EDS makes you hyper mobile. It doesn't cause pain. It doesn't cause GI issues. It doesn't affect the heart. It doesn't cause your meds to work wrong.". Because I am a nurse and have had a few years now to learn about this, I can argue my case. It doesn't usually matter, though. I only have RN after my name. They have MD. So, they are omniscient, all knowing. They are wrong, though. I have learned, for most things, to seek out the best physicians, those most familiar with EDS. Usually we accomplish this through support groups, facebook groups and EDS friends that I've made a long the way. If a doctor doesn't factor EDS into our situation, I move on. EDS factors into everything, now. No matter what is wrong. Many patients spend years and years fighting physicians, trying to find out what is wrong with them. They spend years being given wrong diagnosis, psychiatric diagnosis, called Munchhausen mother's or patients. These are strong people who keep fighting. We were fortunate that our journey took years, but not nearly as many years as theirs.
I usually only take my family to the best, now. It means we travel. But, it means we are cared for properly. And, that is how I found my physician in Maryland. I only saw one quack neurosurgeon before him. A man in Cincinnati. A real idiot. Then I found Dr. Henderson. He has made it his life work to understand us, to understand EDS and the way it affects our bodies. The man is a real genius. Truly, I've never met anyone like him. I am both intimidated and fascinated by him. We really love him. I have learned so much from him about myself, about the way my body works. Someday, I'll talk my daughter into seeing him. I've joked before that the man can sniff the air around us and tell what's going on in our bodies. Piece by piece, he has put me back together. And I never knew I was so broken until he fixed me and the pain was suddenly gone in whatever part of my body. Some of the things we have, such as chiari, can be life threatening. But, I have faith that he won't let me die. He'll get to it, he'll fix it when it is time.
Over the years, I've saw a lot of doctors who said that I was wrong, either about myself or my children. But, I am not. I refuse to take their answer. Don't assume that you have to take the diagnosis that they give you. You don't. Argue to have that removed from your record. Fire them. They may be MDs, they may be more wealthy or more educated. But, guess what? You are still paying them. They are hired by us. They can be fired by us. When you know they are wrong, move on to the next. When the money runs out, fundraise. Beg. Swallow your pride. Because you deserve to live.
I'll wrap up this book chapter with that, because today I can't see. Everything that I type is doubled. That is just a mild part of what I go through. But, my eye doctor says I am fine. ;)
If you can, click the link over there to our fundraiser and help us. Or share it on whatever social media you use. I won't stop fighting to live and to fix my family as they need it. But, it does cost money. A lot of money. Lets just say, I could have nearly paid off my mortgage by now.
All of my friends and family know that I see a neurosurgeon all the way in Maryland to treat my condition. Many of you may not understand why I travel that far. You see, he is the FIRST that I've met who "gets it". He truly understands Ehlers-Danlos, chiari, cervical instability and the multitude of other ailments. He is a diamond in a gravel pit.
When we started this journey, we saw a lot of doctor's who discredited any suggestion that I made. But, I was just learning about connective tissue disease and really didn't have a foot to stand on. It all started with Ashley and her many trips to the orthopedist for her constant dislocations. Each time they just said that they didn't know what was wrong or why she kept stretching and tearing these ligaments. They would put her back in braces, back on crutches and send us on. Until the day we say the "new" doctor. I don't even remember his name. I should find out. He was new at the practice, though. For this visit, I had no babysitter, so I brought all of my kids. He was looking at Ashley's knees, then looked at my family of tall, thin children and asked if we had Marfans. I had only heard of Marfans, really knew nothing about it. He said that he really believed that may be our problem and for me to find out. And so began the journey.
Where does a parent start with that? At home with their primary physician. We saw an awesome Nurse Practitioner named Jodi (and, I ALWAYS recommend the NPs over MDs but I am biased). She knew a bit about Marfans. She began looking at my kids and finding things that I just hadn't noticed, as a mother. Yes, they were a little taller than average. Yes, their arm span was longer than their height. Yes, they all had a high palette, crowded teeth, history of speech problems. They had long legs, very long fingers. Yes, they were all double jointed. Three of them had scoliosis to varying degrees. A couple of them had irregular heart beats. The were things we hadn't picked up or put together. Until now. They met enough criteria (Marfan's symptoms) to consider more testing. So, they were scheduled to see a pediatric cardiologist and a geneticist at UK medical center.
This is where the fun starts. The day rolled around and we went to see the cardiologist. Let me say right from the start that he was a jerk. He wanted to know why we even thought we had Marfans. I explained their story, their symptoms. He said nope, he knows they don't have it. They are fine, just a little tall. But, he'll do an echo on each one just in case. So, one by one I went in with them while they had an echo. After, he tells me that they all look good. The twins had a slightly larger than normal aortic root but that he thought it was actually normal for their age and that the problem was they were too thin, which distorted the percentile. Otherwise, all fine. No abnormalities. We don't have Marfans. Silly idea. Go home.
Next was the geneticist. Because I brought 4 kids to be examined, they split them into two rooms with two physicians. I was with the girls. The lady, who I hear has moved on to practice elsewhere, thankfully, looked them over. Again, I was asked why are we there. Again, I go through the story. She checks them out, says sure they are a little hyper mobile, but they are fine. That I was wrong, their measurements are fine. This is when I began to learn to stand up for us. I told her that no, they are not fine. I measured them myself. I told her to not "eyeball" it and actually measure. So she did. And had to admit that I was right. They are off. So she looks them over again. Finally, after several tests, she tells us that they do not have Marfans. She says my oldest has Ehlers-Danlos. She said she had some subtype, she wasn't sure which, and not to worry about it. It was no big deal, there was nothing we could do. Then, she says that the oldest was gaining a little weight and she just needed to exercise and monitor that. And she sent us home.
It was almost a year before I figured out that Cincinnati Children's Hospital has a connective tissue clinic and a specialist in EDS. During that year, I kept pushing for answers but no one had any. So, I took Ashley to Cincy. There, we met an awesome doctor who knew exactly what was going on. He spent an hour with her, checking her, asking questions. And that day we found out that she (and probably myself as well) had EDS-hyper mobility type. We've saw him a couple of times since, over the last couple of years. He is still just as great.
Now, this is just the beginning of our story. Since then, we have saw many doctor's and been in the ER a few times. Each time, I have to explain that we have Ehlers-Danlos and give a quick, summarized lesson about it and what it does. I have to explain the connection to whatever we are having treated. I have to tell them what to look for. And then, most of the time, I am discredited. They say "no, EDS makes you hyper mobile. It doesn't cause pain. It doesn't cause GI issues. It doesn't affect the heart. It doesn't cause your meds to work wrong.". Because I am a nurse and have had a few years now to learn about this, I can argue my case. It doesn't usually matter, though. I only have RN after my name. They have MD. So, they are omniscient, all knowing. They are wrong, though. I have learned, for most things, to seek out the best physicians, those most familiar with EDS. Usually we accomplish this through support groups, facebook groups and EDS friends that I've made a long the way. If a doctor doesn't factor EDS into our situation, I move on. EDS factors into everything, now. No matter what is wrong. Many patients spend years and years fighting physicians, trying to find out what is wrong with them. They spend years being given wrong diagnosis, psychiatric diagnosis, called Munchhausen mother's or patients. These are strong people who keep fighting. We were fortunate that our journey took years, but not nearly as many years as theirs.
I usually only take my family to the best, now. It means we travel. But, it means we are cared for properly. And, that is how I found my physician in Maryland. I only saw one quack neurosurgeon before him. A man in Cincinnati. A real idiot. Then I found Dr. Henderson. He has made it his life work to understand us, to understand EDS and the way it affects our bodies. The man is a real genius. Truly, I've never met anyone like him. I am both intimidated and fascinated by him. We really love him. I have learned so much from him about myself, about the way my body works. Someday, I'll talk my daughter into seeing him. I've joked before that the man can sniff the air around us and tell what's going on in our bodies. Piece by piece, he has put me back together. And I never knew I was so broken until he fixed me and the pain was suddenly gone in whatever part of my body. Some of the things we have, such as chiari, can be life threatening. But, I have faith that he won't let me die. He'll get to it, he'll fix it when it is time.
Over the years, I've saw a lot of doctors who said that I was wrong, either about myself or my children. But, I am not. I refuse to take their answer. Don't assume that you have to take the diagnosis that they give you. You don't. Argue to have that removed from your record. Fire them. They may be MDs, they may be more wealthy or more educated. But, guess what? You are still paying them. They are hired by us. They can be fired by us. When you know they are wrong, move on to the next. When the money runs out, fundraise. Beg. Swallow your pride. Because you deserve to live.
I'll wrap up this book chapter with that, because today I can't see. Everything that I type is doubled. That is just a mild part of what I go through. But, my eye doctor says I am fine. ;)
If you can, click the link over there to our fundraiser and help us. Or share it on whatever social media you use. I won't stop fighting to live and to fix my family as they need it. But, it does cost money. A lot of money. Lets just say, I could have nearly paid off my mortgage by now.
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