Merry Go Round broke down...

In Memory of Alexa Simmons

2011-10-14T22:50:00.000-07:00

I have spent the last week trying to figure out what to write about Alexa. I wanted to be able to explain everything she meant to me, but every time I sat down the words would not come. For someone who is used to being able to write pages of papers and stories without effort, this was particularly annoying.

I did not know how to start. How does one summarize an entire relationship in words? How can I capture what Alexa meant to me and to the whole EDS community in a blog post?

I did know one thing - I did not want to reduce Alexa to a "Lifetime Movie Special." I hate nothing so much as the idea that everyone with a chronic illness or disability needs to be "inspirational" so as to help others understand the importance of life, why they should value the time they have, blah, blah, blah. Alexa was a person, not a Hallmark Card.

So who was Alexa? She was my friend. She was friend to so many. She was a real person, with a real personality. And she actually was inspirational. Alexa dealt with so much in life, especially the last year, but she still maintained a largely positive outlook. Sure, she got frustrated, she had fights with people, she had days when she was down or depressed. But she never stopped hoping.

I never met Alexa in person, but I still felt close to her. She called me, we chatted, we understood each other. I talked about her to people all the time. Whenever I had a question about the neurological problems associated with EDS, she was the first person I would turn to. I felt like she knew everything. She was so well-read and so quick, so clever.

My funniest Alexa story is also my saddest. I was at Disneyland and I was in line to ride Jungle Cruise. I use a wheelchair, and I was in my powerchair that day. My powerchair weighs 300 lbs, and I use the wheelchair boat on Jungle Cruise so that I do not have to transfer. I was chatting with Alexa and distracted, and I proceeded to drive my powerchair right off the side of the ramp! So I was sitting there with one wheel hanging off, watching two Cast Members (who still give me crap about it!) try to lift me back on. It was just hilarious.

The sad part is that I never told Alexa that story. I kept forgetting, and I thought that I would have more time. Every time I forgot, I thought I would tell her later. Later never came, and now it never will.

I loved Alexa. I recently sent her a stuffed animal, the same one Crys my wife got me the last time I was in the hospital Alexa sent me the cutest note ever on Facebook thanking me. She always seemed surprised when people thought of her. I don't know anyone who knew her who did not love her.

So now what? Now I live, and I live, at least partially, for Alexa. I admit I have been sitting on my rump feeling sorry for myself recently rather than being more proactive about my health. So now it is time to get my ass in gear. I cannot bring Alexa back, but I can at least honor her memory by actually doing something. Time to get back involved in my life. I always thought there was more time, but there is not. Sometimes, the time is gone, and I do not want to have regrets for things undone. It is time to LIVE.

Alexa, I will always love you and remember you. I cannot believe yet that you are gone. I had a question yesterday, and my first thought was to ask you. I will miss you for always.

I'm back!

2011-10-13T21:16:00.000-07:00

So I am actually going to start posting again. I have been very unwell recently, so I was taking a bit of a break from, well, everything...

But now that I am starting to reconnect with the world, especially since I am starting down the road to surgeries... Time to start actually being part of the world again, interacting with people, if only on the internet!

When you want FEWER medications!

2011-04-21T14:21:00.000-07:00

So we hear a lot in this country about how doctors have been doling out pain medications like Skittles and how patients everywhere are clamoring for more pain meds.

So why am I here having problems taking LESS meds??

I have a new (now likely fired) Pain Management doctor. I saw him for the first time on Monday. He was very late for the appointment (over an hour) and was not the doctor I was told I would be seeing. He, in just a half hour, managed to insult my knowledge of EDS, my old PT (who I wanted to move to Anaheim with me), all of my doctors, and pretty much everyone else in the world. I told him with Darvocet off the market, I need a new day-to-day medication. I use Vicodin for the bad nights and Aleve for the little aches and pains.

So what does he offer me? METHADONE! That's right, what they give to heroin addicts.

Yes, it is helpful for some people, but this is generally considered a drug you "graduate" to, not one you start with. He also said he never, ever prescribes Darvocet because it is a neuro-toxin. Ok, I've heard of the heart problems, but neurotoxin? That is a new one!

So he wants me to be on Methadone twice a day, Vicodin twice a day, Naproxen (Aleve) twice a day, and baclofen (a muscle relaxant). I told him that I am uncomfortable taking that many meds, and that I am going to have to skip doses frequently so that I can drive and work, and he had no answer for that.

He asked me what I thought about nerve injections. I told him that they make me much worse. He refused to believe it and scheduled me for an occipital nerve block, because, according to him, my "chiari malfunction" should not be causing headaches. The fact that he called Chiari by the wrong name gave me a lot of hope, let me tell you.

He also decided that I have bursitis in my knee and that I need a cortizone injection. I refused this, as cortizone causes scar tissue in me (welcome to EDS) and it made everything worse the other times I have had it. He ignored this as well and told me I am going to have a cortizone injection.

I called him the day after the appointment because I was not comfortable with taking methadone and I wanted to try something else first. I told him I need something I can take and drive and work at the same time. I was thinking Tramadol or Tylenol-3 or something. His response:

"You can take the medication or you can be in pain."

When I told him I still was not going to take the methadone, he said "Ok, then, don't take it. Have a good weekend and see you at the follow-up." The follow up is almost 5 weeks away.

So he left me with absolutely nothing. What a great help, eh?

I know EDS is not well known, but people with chronic pain who need to function is hardly a new concept. After how rude he was to me, after it took 3 phone calls just to get him to call me back, after he scheduled me for procedures that I had refused, he is certainly not going to see me for a follow up! And I am certainly NOT going to allow him to start sticking medications and needles into me!

So, why is it so hard to get a doctor to prescribe LESS in the way of medication? I know I will never live pain free, but I do want something to HELP. I am not looking for a cure, just a helping hand.

Adventures in a new health insurance system

2011-04-06T19:49:00.000-07:00

So, here I am with a new health insurance which means a whole new set of doctors! I had my first appointment with my new GP (who shall henceforth be known as GP Dude), and I admit, I did not think it went well. There was an insurance problem, and so I ended up having to postpone my appointment from morning until the last appointment of the day. And he did seem rather overwhelmed by me. He was telling me how I had to "take things in order" and was more or less refusing to give me the referrals I needed, including the all-important neurosurgical consult. I was not interested in redoing all the testing, and I really need to be seen for my neuro problems immediately.

Well I left that day planning on never going back. Then I got a call yesterday saying that my referrals were ready. He had given me referrals to rheumatology, endocrinology, neurology, pain management, AND neurosurgery. So i guess he is not useless after all.

Now I am worried about the new neurosurgeon. He treats Chiari, but I don't know if he will do anything about issues that do not appear on an MRI. Here's hoping he can do something...

Bendy Humor

2011-03-15T19:16:00.000-07:00

You know you're a bendy when...

Your 7th grade student subluxes his shoulder, trying to gross you out and get out of class, and you just dislocate yours, just to outdo him! Needless to say, this student got little sympathy from me. I should mention the student was in no pain and is of Asian descent, which means he is naturally more bendy than I am.

Also, in happy news, I got a second interview for a job I really want! It is a temporary part-time position, so I should do just fine working there. Here's hoping I get it!

Illness, Genetics, and Identity: Part One

2011-03-13T17:57:00.000-07:00

There is an expectation in our society that a sick person always wants to get well, to be better. Publicly working for a cure to your disease is acceptable. Pride in your disease is not acceptable. After all, who wants to be sick?

And here we have a dilemma. What about those of us with a genetic illness? It is literally a part of us, it is written into the code that makes us who we are. Are we supposed to want to get rid of our illness like people with an acquired or random illness?

Do I want to get better? Yes, I am working hard to improve my health and my quality of life. I am working on finding surgeons to help reduce or eliminate some of my neurological problems so I can live a better life.

Do I want to get rid of Ehlers Danlos Syndrome? If I could, would I change my genetic code, so I could be more like everyone else?

I don't know.

EDS is written into my genetics, as much as my height, my eyes, whether my hair is curly... It has become a part of who I am, who I believe myself to be.

I can't tell people that I want to have EDS. Usually the reaction to a person wanting their disease is frustration, disgust, and disbelief. We all know the person who tells EVERYONE about their illness and their suffering. But that is not me. I don't want EDS for the attention it gets me. I was always able to get people's attention before I knew I had EDS.

I don't know as I want EDS. I know that I am not going to have my own children since I do not want to pass on EDS. But it is part of what I am, and at this point, I don't know who I would be if I suddenly got rid of my EDS.

This musing is obviously a work in progress. Perhaps at some point in the future I will have a better idea of where my EDS fit into my identity.

Who would have thought the tram could be used as a teaching tool?

2011-03-11T21:47:00.000-08:00

So I go to Disneyland. A lot. And most of the time I drive there. To get from the parking structure to the tram, you either take a shuttle or walk (or roll) - which means I would have to cross a street and tire myself out before ever getting to the park.

Luckily, the resort provides a tram, which almost all people take. In the front of these trams, there is a car which is designed to accommodate wheelchairs and scooters. It has built-in tie-downs and the bench folds up. Each end of the route it takes has 4 loading zones, two of which can accommodate wheelchairs. There are specially built ramps which allows me to board without having to get out of my chair.

The waits for these cars can be long, but that is just part of life with a wheelchair. Disney has added a "courtesy van" with a ramp that parties with wheelchairs can take to the parks. It picks up and lets off in generally the same area, so that is not an issue. When riding the tram, at least half the time I run into problems with Cast Members putting too many people with strollers in with me, or even forgetting about me entirely, and trying to unload me where there is no ramp. I have also had other guests get annoyed with me because my wheelchair takes up too much room and they cannot fit their strollers (nevermind that there are 5 cars they can sit in, and only one row for me).

So, why do I feel an aversion to the van? Partly it is that it is, quite literally, the short bus. Regular guests do not have to and are not allowed to take it, and we do not even wait in the same area. There is nothing "Disney" about it. In a place where even the toilets are themed, an unthemed van just makes me feel like a burden.

Also, I have been told repeatedly how much easier it is. Well, it is not easier for me. It may be easier for parties with large groups or groups with multiple wheelchairs/scooters/walkers/etc. But I travel alone most of the time. The ramp has a 35 degree incline (3 times a standard ramp) which I cannot get up on my own. I do not allow people I don't know to push me, so this is problematic for me. Also my chair, the Quickie Q7, cannot be tied down easily due to its minimalist frame. Usually I transfer, but this is even more difficult on a shuttle bus. Also, I do not do well with temperature transitions, and going from a very hot outdoors to a very air conditioned shuttle can actually throw me into seizures. So it is not easier for me.

In fact, the only people it is easier for are the able-bodied people who want to load their strollers into the area for wheelchairs, or those who don't want to wait for me to be boarded. For some reason, I do not feel like making their lives any easier.

But it is more than that. I want to be included. Most lines in the parks are not mainstreamed, which means I am usually sitting somewhere by myself waiting to board rides. I do like talking to people, and I have met some of the coolest people while on the tram or waiting for it. More importantly, I have answered dozens of questions about EDS, spreading knowledge and awareness.

There are so many times that using a wheelchair or having EDS or being sick have made me be separate from everyone else. This is one thing that I can do with everyone. Anyone parking in that structure can take the tram, and I like being able to do what everyone does.

I have tried explaining this to Cast Members, and they don't get it.

I cannot change my genetics, and I don't want to. That does not mean I don't want to do what everyone else does, if in a somewhat different way.

So in the meantime, I am going to continue riding the trams and explaining why I like doing things the way everyone else is expected to do them.

Time to start writing again...

2011-03-09T21:00:00.000-08:00

So I took a two month break from doing any real "health" stuff, and it was great. But now I am getting proper health insurance again (April 1), so it is time to go back to dealing with my health issues!

I saw Dr Francomano in February, and she is really stressing that I need to deal with my neurological issues. Not much else can be done until we fix that. She did give me a Aspen Vista collar to help deal with the neck pain. I am slowly getting used to wearing it, but it does seem to help.

I also have a list of all the doctors that I need to see:
GP (I have an HMO, so she better be cool with writing referrals!)
Geneticist
Rheumatologist
Endocrinologist
Orthopedist
Neurosurgeon
Opthamologist
and of course the fun of Yearly Exams.

And I am sure I am missing something. I really do have to stop moving so that I don't have to start over anymore!

I still need to apply for state aid for the copays which are substantial for hospital-related things, but at least it is a start!

Why can't I just be me?

2010-11-15T16:33:00.000-08:00

I was told the other day while I was at Disneyland that I was beautiful. And the person did not mean physically (although I like to think they meant that too). She was talking about my "attitude." And what was my great accomplishment, you ask?

I went to Disneyland.

Yes, as a Spoonie, this is a little complicated. I have to have a series of medications, a handicapped parking tag, my custom $5000 wheelchair, five layers of clothing to deal with the Reynaud's, a Guest Assistance Card which helps me avoid queues in direct sunlight, an in-depth knowledge of how touring Disneyland with a wheelchair works, and an abundance of spoons that day.

But I still went to Disneyland. By myself.

For some reason, this was just too much for this woman to handle. In fact, I seem to astound many people every time I go to Disney by myself. Granted, the womasn who told me this used a power wheelchair and oxygen, so she understands being a Spoonie, but still...

She, as so many do, ask what my disease or disability is. And, as I almost always do, I explain about EDS, taking the opportunity to educate someone about this rare disorder.

I have a very upbeat attitude about my disability in public. Actually, I have a fairly upbeat attitude most times. There is absolutely no cure for what I have and very little research is being done (though we are working to change that). The odds of me ever being "well" is slim to none. So therefore I have resolved not to be whiny and depressed in public. Yes, of course, I get depressed, I cry, I rail at and about doctors, and I complain to my wife when something hurts or I had a bad day... But I know that I will hurt and be sick whether or not I go to Disneyland, so I might as well go to Disneyland!

This woman really thought that my attitude was "beautiful." I have also heard that my attitude was "brave." The other thing I have heard is just sheer incredulity. Some people just do not get that I go out by myself. They grab my wheelchair to "help," they try to help me transfer... They just cannot understand a disabled person doing things on her own.

I go to Disneyland, not the moon! I go on Peter Pan's Flight, I am not joining the Army!

Why do I have to be "brave" or "unbelievable?" I am not trying to be an inspiration. I am not trying to be courageous. I am just trying to be ME! Why can't I just go to Disneyland without turning into a Hallmark Channel Movie?

My very first hospital stay!

2010-11-01T14:40:00.000-07:00

I know it sounds hard to believe, but until this weekend, I had never been admitted to the hospital.

I went to the ER about 1AM on Sunday after two days of unrelenting nausea and vomiting. I was also starting to have some problems breathing, and ended up hyperventilating on the way to the hospital so badly that I lost feeling in my limbs and my muscles were all cramped up. In the ER, they took my medical history and, of course, I had to explain what EDS was. They gave me ativan through the IV, which helped my breathing, but not the nausea. I asked for something for the nausea, and all they did was give me more ativan (which is STILL not an anti-emetic!)

As I was heading out the door, they told me my potassium was too low and handed me two massive supplement pills, which were a little hard to take given that I was throwing up!

They never seemed to get that the nausea did not go away, and by rehydrating me with the IV, it had given me something to throw up, So we did not make it 3 blocks in the car before I was puking again. We returned to the hospital so I could get cleaned up and ask for advice (after all, this is why I had gone in the first place!)

They told me to go down the street to get a prescription filled for a sublingual anti-nausea med, Zofran. Well I threw that up within 5 minutes too... So... BACK to the hospital!

When I got back to the hospital, they decided to admit me. I was so out of it, in so much pain, that I did not care. They gave me a new IV, anti-nausea meds, and Fentanyl, which is by far my favorite in-hospital pain killer. They admitted me, moved me upstairs to a room (luckily my own room), and then started asking me even more questions. I was pretty stoned, so heaven knows what I said!

All day yesterday I was nauseous, and required anti-nausea meds 4 times. Even with this, I was only able to handle 1 cup of apple juice and 1 small amount of milk. Anything else was making me sick. They gave me a hard time about wanting my pain meds (they wanted to give me dilaudid, but I knew darvocet would be enough). Eventually I got enough pain relief that I could sleep.

They kept me last night, planning to discharge me this morning, The doctor came in, seemed not to understan that I was having very bad pain, vertigo, and dizziness, due to the pain in my neck (which is not new, but was very bad this time). He disregarded it when I told him it was not due to the nausea, but then grabbed my head WITHOUT gloves on, and YANKED my head very hard in all directions (like 5 times in total). This was TERRIBLE and made everything worse. He obviously did not know what EDS was, and that you have to be very gentle with my joints ESPECIALLY my neck!

He left, telling me he would still discharge me, and told me to see my doctor by Friday, I responded that I cannot see a doctor until after the first of the year because of insurance. He seemed concerned by this, and told me he would give me the names of some free clinics.

Yeah. Right... Like any free clinic around here can handle someone as complicated as me!

Well, a half hour later I was trying to get from wheelchair to bed and a wave of vertigo hit me so hard that I fell out of my chair. The nurse and PCA picked me up, and paged the doctor. It took him OVER AN HOUR to respond to the page. He had also decided to order more bloodwork on me, since it was clear I was not "better."

So here I sit, planning to go home tonight, still with the neck pain and possibly a neck injury caused by a combination of throwing up, the doctor yanking on me, and falling down.

Here is hoping that I can convince them to give me some meds to calm this down - so far all I have gotten was my Lyrica and Antu-vert, which just made me sleep.

Will update as I get more info...

When getting diagnosed with a chronic disabling illness is a GOOD thing...

2010-10-12T21:14:00.000-07:00

Most people would not understand when I say "Congratulations" to someone who was just diagnosed with EDS, but that really is my first reaction. One of my friends was just diagnosed after dealing with pain and uncertainty, misdiagnosis and not being believed - and she fought this battle for most of her life.

Most of us with EDS get several misdiagnoses before finally finding out what is wrong. We are told my doctors, friends, coworkers, and even family members that we are exaggerating, hypochondriacs, too sensitive, have a low pain threshold, attention seeking, lazy, weak, clumsy, klutzy, depressed, anxious, mentally ill, and just plain lying. We fight for recognition not only to prove to others that we are really sick, but to prove to OURSELVES that we were right all along.

You see, after a while you begin to believe what people are telling you. I've had a doctor tell me that there is no such thing as occult tethered cord, despite the fact that I know people who have had surgery for it AND I had an article describing it. I've been told that having joints hyperextend cannot be painful.

Sadly, the battle does not stop with a diagnosis - all this means is at least know you know the direction you are fighting. I once had a rheumatologist tell me that I did not have EDS because I was not bendy enough. This was AFTER being diagnosed by the world's top expert in the disease, and without him doing a physical exam. Apparently he is such a good doctor that he can disregard a diagnosis from the world expert (who arrived at the conclusion after 3-4 hours of exams) and not even need to touch me. In reality, he is the son of a tenured professor in rheumatology at that teaching hospital, and likely got his job because of his father. Needless to say I did not go back to see him.

So the battle will continue. Having a diagnosis makes some things easier, and it is a HUGE boost to mental wellness to know it is NOT all in my head. So, yes, it is strange, but I will say congratulations to someone who just got a diagnosis after years of pain. After all, not having a diagnosis does not magically make the pain disappear.

I should say something profound

2010-10-06T17:55:00.000-07:00

but sadly, all I have to say is OW! I hate weather change headaches. The good news is that it is sunny again. The bad news is that my head is going to explode.

So now my plans are to go to Disneyland tomorrow - all I have to do it get up in the morning to drive Crys to work so I have the car so I can go ride some rides!

The loss of assurance

2010-10-05T14:01:00.000-07:00

One of the hardest things my illness has taken from me is the assurance that my body is going to do what it is supposed to do. I never know when I get up in the morning whether this will be a day when I can do whatever I need to around the house, or if I am going to be stuck in bed all day. I don't know if my body will digest food properly, if I will be able to stay awake, if I will be able to feel my hands and feet.

I remember when I was little when I did not have all these problems... I would get out of bed every morning during the summer, run outside, ride my bike all day, play with kids and the neighbors' dogs. I knew that when I got up that morning, my legs were going to work, I would be able to eat and go to the bathroom with the assurance when it would work... I got tired when I was supposed to, and rest made me less tired.

I don't expect to ever get that assurance that things will work the way they are supposed to. But I do hope that one day I stop being surprised when something doesn't work. I know that I cannot control it, and I am doing everything I can to prevent the problems I can. So why do I get so surprised when something else goes wrong?

Official Disability Employment Awareness Month

2010-10-05T10:01:00.000-07:00

The statistics on employment for people with disabilities is very depressing. For those people with disabilities who are able to work, we are much more likely to be without a job. Employers are much more likely to discriminate or even blatantly break federal law, even in an interview.

As a note - an employer is allowed to ask if you feel you are able to do the job with or without reasonable accommodation. For example, I can work as a teacher as long as I have my wheelchair - this is a reasonable accommodation. It is NOT discrimination for someone to say I cannot be a firefighter, since there is obviously no physical way I can do it.

It IS illegal for an employer to ask on an application or in an interview (even when you are employed in most circumstances) what your disability is, why you need a wheelchair, how "sick" you are, etc.

I had an interview yesterday and it was perfect! They followed the laws perfectly and the head of the company really did not seem to care at all that I happen to come with my own furniture. I was very impressed.

Now there is a national campaign to educate people as to the laws and difficulties for people with disabilities to get employed... and they have an awesome website!

http://www.whatcanyoudocampaign.org/

I am glad that SOMEONE is paying attention to the problems we face!

At least someone is trying to do something...

2010-09-28T15:53:00.000-07:00

Dr Hal Dietz on the research being done on connective tissue disorders, including EDS.

Dr Dietz Video, via Johns Hopkins

Finally we are trying to find a way to prevent problems instead of just fixing them once they become a danger!

Something I posted on Disboards...

2010-09-27T20:09:00.000-07:00

I just posted this over on the Disboards, where I am a moderator on the disABILITIES forum... And I thought parts of it would be pertinent over here...

Lately I seem to have answered a lot of questions about accepting the need for a mobility aid at Disney. There is nothing wrong with those questions, and I completely understand the difficulty in facing the need for more assistance. Here are a few words of advice I have learned after three years on this board:

1. There is nothing wrong with getting a wheelchair, walker, or ECV and only using it part-time. I know a lot of people feel like they are "cheating" if they park a wheelchair or scooter and walk, as if other people are judging them. The important thing to remember is that YOU know your body best, not the random strangers. Use a wheelchair when you need it, and walk when you don't.

2. A wheelchair is a tool, not a judgment. Using one in Disney is NOT a reflection of your disease or your daily needs. Most people walk 6-10 miles a day in Disney, and a vacation is NOT like your daily life. Therefore, using a wheelchair is Disney does NOT mean you have to use one at home.

3. I hear a lot of the time how using a wheelchair is letting the disease win. And I always argue, NOT using the wheelchair is letting the disease win! If I stayed at home in bed all day because I was afraid or ashamed, if I did not go to Disney and enjoy my honeymoon, then THAT is letting the disease win!

4. A lot of doctors will urge people not to use a wheelchair, to keep walking, because that is better for the person's health. And the doctors are likely right. However, if you speak to most doctors about Disney and 6-10 miles a day, they will suggest using a wheelchair! They are giving advice for daily life, not for a commando vacation! Always follow doctors' advice, since an unneeded or improper mobility device can do more harm than good, but if you need something, then use it!

5. Most important: There is a difference between surviving your Disney vacation and enjoying it! In daily life, you will "push through" pain or discomfort, because it is important to maintain as active a life as possible. Vacation, however, is NOT the time to be in pain! There is no shame in asking for a stronger prescription for the vacation or using a wheelchair to avoid pain or distress. You want vacation to be a time you spent with family having a wonderful time. Your most vivid memory should be of your favorite ride or finally meeting Mickey Mouse, not the evening you spent in the hotel room in incredible pain while the rest of your family went out.

In this list I have used the word "wheelchair" because that is what I use. You can easily substitute "scooter," "ECV," "walker," "GAC," or whatever assistive device you use.

Oh the wonders of melatonin

2010-09-27T17:40:00.000-07:00

I had barely slept in days due to a stomach bug, so I decided last night that I really needed to try melatonin, a natural sleep aid.

I think that is going to become a nightly ritual for me - it let me sleep through pain without giving me the morning-after hangover narcotics and sleep aids do. I generally wake up several times a night because of pain or neurological problems. Last night I slept through all of it. There is nothing I can do to stop myself from being in pain, so something which lets me get restful sleep is very useful!

I am realizing now how many supplements I am taking. I am taking B12 (low B12), Vit D (low D), Vit C (immune booster), Zinc (immune booster), Magnesium (helps with muscles, and I don't eat a lot of magnesium rich foods), and now I am adding melatonin.

There is a new EDS treatment being tried in the UK where they max out a lot of vitamins and minerals and supplements, trying to boost systems of the body failing due to EDS. I wonder how many of my pills are on their list. I want to bring up that study with my new docs when I get to see them.

Letting go of expectations

2010-09-22T16:43:00.000-07:00

I've decided one of the most important and perhaps the hardest part of being a spoonie is letting go of expectations. This is not to say you should not have hopes or dreams or plans or obligations, but you have to let go of expectations. For example, if I can hope that a new doctor will have some answers about how I became so ill so quickly, but if I expect that, more than likely I am going to be disappointed and angry. If you expect as you get older that you will not lose any physical abilities, then you will be very disappointed. If you expect that people will respect you simply because of an education or advancing age, you will be disappointed.

The trouble is, disappointment often leads to anger.

I hope that I get more answers soon, and I hope that I am well enough to raise a family, and I hope that one day I can put the wheelchair and the pain meds away for good. To be without hope is to live a life of despair, a life without meaning. An expectation, however, is an outcome that you have already decided will come to be. We will never know what will come next, spoonies more than other folks, and to constantly expect certain events or outcomes is very frustrating.

So to live a life full of joy, and peace (and of course occassional anger, sadness, and frustration), you have to let go of expectations and learn to live with hope and dreams.

New geneticist

2010-09-21T15:40:00.000-07:00

So... I am getting information in the mail about a new geneticist at a Marfan's Syndrome and Connective Tissues Disorders Center at Cedar Sinai in Los Angeles. After reading so many conflicting things and getting no answers, I am glad to finally have a new set of doctors.

It's not that I don't trust Dr. Francomano - I absolutely do. I just am not sure about the neurological portion of it. I have neuro problems, but I also have an as-yet undiagnosed autoimmune condition. And after reading so many bad things about The Chiari Institute, and how they were making up surgeries just to make money, I admit I am a little nervous about trusting their method of dealing with neurological problems in EDS.

This new center has their own cardiologist, orthopedist, ophthalmologist, pretty much everything.

Now my biggest fear is that I will go in there and they will say I do not have EDS! Oh well, a new set of fears and challenges!

Health Insurance Catch-22

2010-09-21T12:14:00.000-07:00

I currently have COBRA for insurance which covers me in the state of Connecticut, and for urgent care outside of Connecticut. Because Crys was laid off and I was unable to work, we had to move to California. I called Anthem today to find out what kind of coverage I have out here. The answer is...

I dunno.

Yes, they really have n0 idea what to do with me. Apparently I can go to an ER or Urgent Care Center, but the odds of them knowing how to handle me and my EDS is not very good. I asked three different people at Anthem, "What happens when the Urgent Care Center tells me I need to see a geneticist (EDS Specialist)?" The answer was "I don't know." They have no idea what to do with me.

And just to make matters more fun: I cannot get state health coverage until I have a doctor to certify that I am disabled. I cannot get a doctor until I have insurance.

This is one of those moments that I hate life. It does not help that my pain levels are as high as they have been in a long time, or that I still have no income.

Guess I just have to hope I have no major medical problems for the next 3 months...

Diagnoses: Collect them all!

2010-09-16T19:18:00.000-07:00

So here I am in California, looking for work, and still not certain whether or not I can even do a full-time job. I am still figuring out everything going on with my health. I know there is a clinic out here that can deal with EDS-related issues, so I am hopeful that I will continue getting answers on that. I will not, however, have health insurance that covers me out here until December, so I guess I will just have to wait on that one.

I need to pursue a proper diagnosis for my autoimmune condition. I have been told it is possibly lupus, and we are treating it like lupus, but I really do need a name for this.

I also need to pursue a proper diagnosis for my mental health and sensory problems. I guess I have never considered them to be a medical problem the way I do now, but I can tell they are definitely affecting my ability to work.

I am so sick of being in limbo, of not knowing what comes next... I have no idea what the next step in my life will be. Hopefully I will have better luck with doctors out here than I did in CT.

So here I am...

2010-09-09T20:42:00.001-07:00

... in California. Now I just need to find a job. I am definitely nervous about working again, as I have not had a full-time position since 2007. I do just fine sometimes, and at other times I can barely sit up.

There is a Marfan's and CTD Clinic in LA, so I am hopeful that they may actually be able to help me. At the very least I will not have to explain what EDS is! They have their own orthos and cardios, so I will need to see both of them as well. And probably an endocrinologist. And a geneticist. And a rheumatologist. It seems like I get to see the entire medical community of Southern California!

EDS in the news!

2010-08-02T09:58:00.001-07:00

EDS on ABC News

http://abcnews.go.com/Health/Wellness/ehlers-danlos-bendy-joints-stretchy-skin-pain/story?id=11279484

We are not throw-aways

2010-07-26T13:42:00.000-07:00

One of my friends was just discharged from the hospital after being admitted for extremely low BP and heart rate issues... And she was just discharged two days later in worse condition than she was admitted in. Her doctors do not know what is wrong with her, and therefore just sent her home.

Why do doctors think this is an acceptable answer? Why do they think that because we are already damaged or sick or ill, it is okay to just disregard our problems? Why do my friends, spoonies all, get sent home from doctors and hospitals because they can't figure it out? In the last 18 months I have gone from walking with a cane to requiring a wheelchair, and most doctors and hospitals will not even give me an appointment. Another of my friends is suddenly losing the ability to breathe, and they just do not seem bothered.

We are not throw-aways. We are worth saving, we are worth fixing, we are worth the effort.

Why does no one seem to be bothered by the incredible apathy in the medical system?

Do I have the right to a diagnosis?

2010-06-10T17:29:00.000-07:00

I've spent the last several weeks trying to decide the answer to a question:

Do I have the right to expect a diagnosis?

I have some form of autoimmune arthritis, but we do not know what type.

Two weeks ago, my rheumatologist said to me that I was being unrealistic to expect a diagnosis, and that I should get used to the idea of never having an answer.

So, is it? Is it unrealistic to believe that there is an answer to my questions out there? In my mind, a diagnosis means that I may be able to get better treatment, better pain control, and a better quality of life. Right now, we are just trying medications, hoping to figure out whether or not it would help.

I believe that I DO have the right to answers. I do not expect them to happen NOW, but I am not okay with simply giving up.

Just because our diseases are rare does not mean we should just suffer. It is painful not to know what happens next, not only physically but mentally.

So, yes, I do believe I have the right to a diagnosis. But I also have to accept that it may take a while.

Why is it never simple?

2010-05-24T10:46:00.000-07:00

I went in for Tilt Table Testing this morning because I stand up and my vision blacks out and I fall over.

So I went in for a Tilt Table Test, and the put in an IV and hooked up the BP cuff and EKG things, no problem.

Then I laid there in the freezing cold waiting for the doctor, who had apparently gotten distracted...

When he came in, he made a few jokes about me being short, which I just thought was rude.

Then he slowly set the table upright, stopping several times on the way, wanting to make sure I adjusted (standing is difficult for me). In all it probably took 60-90 seconds to stand me up, and they never did have me completely upright. I was leaning back about 15 degrees.

Then they had me stand there for 20 minutes, to see if I passed out.

i have never passed out from standing too long, my problem are always within 30 to 90 seconds of standing up.

So they laid me back down and said I was fine. They took the straps off me and the EKG, but left the BP cuff on. Then they had me sit up.

I sat up quickly and I was dizzy and my vision blacked out. I almost fell over, and I was sitting! The BP cuff, which was going every minute and a half, started up after about 30 seconds.

In the 90 seconds between laying and sitting, my BP dropped from 111/6- to 87/64.

I told the nurse THIS is what happens to me, and THIS Is what I was trying to show them. She said she wanted to check it herself, so she did that, about 3 MINUTES after I sat up! I was fine by then!

And in the meantime she had given me apple juice and opened by IV all the way!

She said that the test showed my heart had no problems with me sitting or standing up. YES. I KNEW THIS! WHAT ABOUT THE PART WHERE I ALMOST FELL OVER AND MY BP PLUMMETED!

She did not write down the part where my BP dropped, and the doctor had left.

I am so sick of doctors! So we know essentially I have orthostatic hypotension, but I STILL do not have a dx. The nurse said I probably do have dysautonomia, but this particular test did not show it, and since the doctor was gone, oh well...

GAH! Why can't it ever be simple!

so I have not been here in a month...

2010-04-29T12:46:00.000-07:00

I just realized I have not posted in a month... Since I was last here, I have my new powerchair! It is a Quantum Q600 with power tilt and lift. And it is a lifesaver. I have named it the "Doom Buggy" which is why they call the cars in the Haunted Mansion at Disneyworld... Pictures will follow.

I am also on a new med, which has thrown a monkey wrench into the medical works, as it were. I started on Plaquenil about 3 weeks ago, and it helped! The problem is that none of my blood work has shown that I had any sign of inflammation. I did have the highest sed rate I have ever had, but it is not high enough to indicate a problem.

Plaquenil is an anti-malarial drug which is a Disease Modifying Anti-Rheumatic Drug (DMARD), which helps RA, lupus, and other autoimmune diseases. I have had a lot of signs that I had an autoimmune disorder before, but the fact the drug has helped a lot indicates that there really is an autoimmune problem.

So now we are trying to figure out which disease it is. I am going to see my rheumy, neuro, and geneticist in the next month... I am curious about a particular type of AI called spondylitis. I have greatly reduced curves in my back and reduced mobility in my back. Even Dr Francomano thought it strange that I could not come within 10 inches of touching my toes.

So now we are both closer to answers and still far from the answer... So we will see what is going to happen next!

accessibility fail...

2010-03-25T14:24:00.000-07:00

Here is my "accessibility fail" story for the week...

I went to an upright MRI place this morning. I called them yesterday to let them know I use a wheelchair and I will need to use my wheelchair cushion to sit on, since I cannot sit on a hard plastic seat for two hours.

They told me there was no problem. And there wasn't with the part where I needed the cushion. The problem was getting into the building. I get to the building and...

There is a STEP. A stair.

The building is NOT accessible.

They had to come get me, since I could not get in the building on my own. Luckily I still use the manual. If I had used the powerchair, I would not have been able to get in.

Why do you put an MRI center in a non-accessible building?

Why do you not tell me that the building is not accessible?

GAH! Accessibility fail!

and the verdict is...

2010-03-25T13:48:00.000-07:00

Who knows.

In the last two weeks I have seen a neurosurgeon, a neurologist specializing in spine things, a neurologist specializing in neuromuscular disease, and someone doing an upright MRI.

My problem now is that no one is coordinating care. I am all over the place. I have a new rheumatologist next week.

Most doctors believe that I have a peripheral nerve problem. Dr Francomano believes the root is central. No one seems to have any idea what is going on.

Some people are freaking out, others don't think this is a big deal.

Few of them are even bothering to look up what EDS is. The neuro I saw today kept asking why I did not have a genetic test to prove that I had EDS. He kept saying "you only have a clinical diagnosis, why did they not do genetic testing?" I told him no less than three times in an hour that there IS NO genetic test for my type!

The EMG and NCS are supposedly fine, but I do not have the report yet.

The MRI was "normal" according to the neuro on first glance, but he noticed at least two new lesions which are NOT the ones that were there before. My neck is still bending the wrong way. There is still the retroflexed odontoid and pannus. He did not look at the flexion or extension MRIs.

I am supposed to have a LP done. This will have to wait until after graduation.

So the culprit could still be a rare form of CIDP or something, or what Dr. F thought it was... but... right now, we still know nothing...

Either he was really helpful, or really not...

2010-03-16T11:27:00.001-07:00

So I had my appointment with Dr. A (new neurosurgeon) today. He was either very helpful... or really not.

First of all, I was taken back an hour late. He looked at my MRIs from months/years ago, and said essentially "there is nothing going on." He acknowledged the retroflexed odontoid and pannus, but said he doesn't think it is causing a problem. He also said I do not have tethered cord, because it did not show up on the MRI.

He is ordering an upright MRI (though he did not think it would show anything - even though I know it will). He is also consulting a colleague, to see if I can get an in-depth EMG. He also thinks I need an EEG and a LP.

He seems to think it is a neuromuscular process. He has also put MS back on the table.

He never did a physical exam.

I am not sure if he was helpful in getting me a new neuro consult and an MRI, or if he was blowing me off. He did not ask for details, he did not read the reports...

Oh well, I have another NS appt next week. Maybe this guy will pretend to care!

of course....

2010-03-10T13:00:00.000-08:00

So now it looks like they did not put in the pre-auth properly, and so now we are hung up on getting the wheelchair. Again.

I am stuck in bed every other day (basically I can only sit up for a short while in day, and then I need to spend the whole next day in bed). The wheelchair would get me out of here.

Gah! When will things actually start going right!

Wheelchair!

2010-03-07T16:00:00.000-08:00

I may have actually gotten a powerchair! this chair may literally save my life, because it can take the weight off my neck.

I am wondering at what point do I say "I am at a crisis" and go to the hospital. I keep having trouble breathing, I get more and more dizzy, my entire face has started going numb. Unfotunately the hospital near me does not know how to handle me.

I have three appointments coming up, so perhaps I can get to the point where I know what is happening!

Oh well... at least I am getting my wheelchair!

What comes next?

2010-03-04T13:25:00.000-08:00

I find myself not knowing what happens next...

I know I should apply for disability, so that I can at least pay bills and such, but the whole process scares me.

I actually have two neurosurgeon appointments, one week apart. I am hoping that one of the two may be able to do something...

I can barely sit up at all, and I cannot hold up my head. I cannot feel the right side of my face. I am having break-through seizures.

At this point, I just need to get through the day.

Why is it so hard to find a neurosurgeon?

2010-03-01T16:27:00.000-08:00

I have been trying for the last month to get a neurosurgeon. Dr. Francomano has referred me to a wonderful doctor in another state who is not on my insurance, so that is not happening. So I have been trying to call hospitals all over this part of the country. And NO ONE would see me.

Mass General, Beth Israel Deaconess, Yale... none of them would even let me in the door.

It turns out my best bet is Hartford Hospital - the odds of them having ever seen someone like me is slim, but if I can get an appointment, that will make me happy!

Dr Francomano :)

2010-02-25T12:27:00.000-08:00

First of all – finding a place to park is a pain in the butt, and it took a while to walk/roll all the way through the hospital to where the appointment is. Suggestion: make sure there is someone to help you if you need to carry things or use a wheelchair.

I got to the room, checked in, did paperwork (HIPAA stuff), and then they take my picture. I hate pictures, but they only use it for medical records, unless you let them use it for other things.

They take you back to a room. Ask for blankets, as it is cold. A genetic counselor comes in to ask some basic questions in the beginning, things like when you were diagnosed with anything previously, medications, pain levels, etc. This only took about 20-30 mins for me, then they bring that to Dr. Francomano. She goes over it, so she knows where to start.

There was a resident there the day I was there, so there were two people poking me. They then asked me lots of question and literally looked over me head to toe. I learned things like I have a particular eye shape and soft ears, which mean nothing functionally, but are something a geneticist notices.
She measured the angles of all my joints, and discovered I over 18 degrees past 180 in both elbows and knees! (10 degrees is considered hypermobile). We know my hips are hypermobile (can dislocate one on command), and after it was all done, I have a Beighton score of 6/9, but she thought it would be 7/9 if my hamstrings weren’t so tight.

My skin has stretch marks and scars, but it is not stretchy really. She felt all over for bumps or nodules, (have none right now, but have in the past).

She did basic neuro tests, since that is one of my major complaints. I failed most of them (messed up for the first time on finger to nose), showed major muscle weakness, could not tandem walk at all, fell over on the “close your eyes with your arms out” test, loss of sensation in some places, etc. She also looked at my urodynamics study results, which showed neurogenic bladder.

She had me stand up (with someone ready to catch me) to look at my spine. Apparently I am losing or reversing curves all over the place.

She then left the room, after letting me get dressed (i was in a gown the whole time), and she put everything together into a document. She came back in and went over everything.

She told me that I have the hypermobility type, as my skin is not terrible, though she acknowledges I do scar pretty badly.

She also said that based on my urodynamics study and symptom, she believes I have an occult tethered cord, and a possible Chiari. She asked if I had any MRIs, and luckily I brought the CDs with me. She looked at my lumbar MRI, and said that something looked “weird” on it, but since it was from over a year ago, it is not great for diagnostic purposes. She looked at my neck/head MRI and stated she saw a retroflexed odontoid (top bone of spine) with pannus (scar tissue) and a possible Chiari malformation (1-2mm herniation max). She then referred me to a neurosurgeon in Bethesda, MD. I am looking for a doctor in Boston, as that is a bit of a schlep for me. I will go down there if nothing else works up here.

A retroflexed odontoid means that the top bone of my spine bends toward the spinal cord (it looks like it was broken and then healed wrong, though I never had any trauma), and scar tissue has formed around this. For most people, this causes no problems, but for those of us with EDS, since everything sags and compresses when we stand up/sit up, it is poking my brainstem and compressing the spinal cord. Pannus is scar tissue which has formed around the “point” which further restricts the spinal canal.

It is possible I do not have Chiari, but that the tethered cord is pulling down on the brain.

All in all, it was a PERFECT appointment. She really took her time with me, but it still ran largely on time (so the person after me still was seen). She gave me prescriptions for ring splints, two kinds of PT, new braces, referrals to other doctors, and the title of a book for “at home” treatment of myofascial pain.

So here is my list of diagnoses either given or confirmed by her:
Ehlers Danlos Syndrome: Hypermobility Type
Occult tethered cord syndrome
Retroflexed Odontoid with Pannus
Possible Chiari Malformation

So now it is off to the neurosurgeon!

First post in a two year old blog...

2010-01-09T15:14:00.000-08:00

I am going to get this going soon, I promise! I am going to see Dr. Clair Francomano (the world expert in EDS) in February, so I will be posting lots of information about that!