Rituximab

It's been a while. For a long time, I just didn't feel like doing anything--even something so simple as getting on the Internet and blogging. I finished the round of plasmapheresis I'm April using the new permacath, but my body was so traumatized from the incident that I didnt feel any kind of functional difference from the round of plasma exchange. After about a month, I was finally about to sleep again, but it was a long several weeks. When the time came for another round of plasma exchange, I was met with resistance from both the local doctors and the insurance company...it took 2 weeks to get the ball rolling again. But here I am now--I just finished my 9th round of plasmapheresis treatments on Monday, and I'm feeling pretty good. With the new clinical trial medicine, and my body being free of the destructive antibodies, I'm better than I've been for months. I go up and down so fast, though. I've only felt good for about 4 days now, but I'm so grateful for the reprieve. I know, however, how temporary it is. The antibodies regenerate fully in about 3 weeks, so if I feel decent for a couple of weeks, I'll be lucky. So, in an attempt to stop the antibody reproduction, I am starting a new therapy today--Rituximab. The purpose of this drug is to kill the B cells which produce the harmful antibody. It is as chemotherapy commonly used to treat lymphoma, but I've been assured that it is mild--I should not expect the usual "chemotherapy" side effects. I've been here in the chemo room for 1.5 hours, and have been told not to expect to leave for another 5 hours...if all goes well. If this therapy is effective and I can tolerate it, I will take it once a week for 4 weeks, then I'll stop for many months--until I need it again. This, in conjunction with the the 3,4 DAP is my best bet for any kind of cessation of symptoms. If this works, I should feel better in a month. And while I know that sounds like a long time to wait, considering the 3 years I've been looking for answers, it seems a small price to pay--IF I can feel better and begin life again. I'm hesitant to get my hopes up too high--it seems that I'm always disappointed when I dare to hope that a new medicine or treatment will produce positive results...but I guess that's all there is in life--hope for a better future. N So, here's hoping.....

Well, hello there.

Well, as part of this new treatment plan, I am going to continue the plasmapheresis for as long as necessary.Even though The 3,4 DAP has been helping with symptoms, my baseline was pretty weak-- mornings were especially rough, as well as any time the DAP was wearing off. It was time for another round if plasma exchange. I could've done this last week, but with buying a new residence, I didn't really prioritize it. So, I called in last Friday to set it up, and they told me to come to the hospital on Tuesday morning to get my central line placed.

This wasn't a big deal....I'd had it done 6 other times in the last 8 months....but they don't like for me to come alone. So, I called my friend and asked her to sacrifice half of her day to come sit with me at the hospital. Funny, how I remember telling her that we'd only be here half a day and it wasn't a big deal at all. I left her watching Dowton Abbey on Netflix and said "bye."

I remember meeting the anesthesiologist in holding and assuring him that I didn't need any real sedation...Propofol would be fine for the 1o min or so I'd need to be out. I said goodbye to him, and went to sleep.

The next thing I remember is in clips and phrases: "What is Miriam (my cousin who is a recovery room nurse) doing here, isn't it her day off?" "Yes, but she's her cousin." "Let's get that second unit of blood into her a soon as possible." "Where's her mom?" "She's being picked up from work." "Amd her dad?" "Still in Panama City."

That's all I remember from the recovery unit. Apparently, at that point, they put me under again and removed the ventilator. I woke up again in the SICU, but the next few days were blurry. I remember that just about every member of my whole extended family came in at one point or another. I think I must've thought, at that point, that I had been in serious trouble, but I didn't understand it until they moved me from the ICU into a floor, and I got to chat with Miriam more in-depth. They moved me to the floor on Thursday night, and that is when I learned what happened.

When my surgeon tried to put in my vascath, there was so much scar tissue that he had a little trouble getting the catheter inserted. Well, suddenly the catheter slipped and punctured the pleural cavity. Then, things got really serious as my chest cavity filled with blood, resulting in a hemothorax, which let to a collapsed lung. The surgeon then made a split second decision that saved my life-- he inserted a chest tube up into the chest cavity which helped drain the blood, causing the lung to reinflate, which put pressure on the hole in the chest wall, helping it to clot off.

The next few days were a blur of pain medicine as I had to live with a chest tube the size of a garden hose stuck in my back and shoved up against my shoulder. They finally removed the chest tube yesterday morning, but I hadn't eaten for 3 days, and had to push through nausea yesterday to be able to keep food down. I report, though, that today I am able to eat and do feel better.

When Miriam stayed with me on Thursday night, she told me that I scared the surgeon and the anesthesiologist pretty bad...they weren't sure I would make it. She said it boiled down to this: I was about 30 seconds away from dying, and the call to insert the chest tube made by the surgeon saved my life.

So, I've been in the hospital since Tuesday...I'm afraid I scared my friend that came with me pretty badly, as well as my family. I do, however, think I have survived this experience. :D

Oh- they had to go in again on Wednesday morning and place the central line, which didn't get put in on Tuesday...this time, we went with a PermCath, so I won't have to have it replaced with every treatment.

Ready or Not.

I just got done with a 3 week full evaluation at the Mayo Clinic and I finally--after 3 years of waiting and wondering--have a diagnosis and treatment plan. I feel like it may be my best shot of ever recovering. However, it's gonna be a long hard road.

I have Lambert Eaton Myasthenic Syndrome (LEMS). Odds of getting it? 1 in 4,000,000. Odds of getting it without associate lung cancer? Half of that. So, how lucky was I? I was the 1 out of 8 million people. So, to the other 7,999,999 of you out there that I saved from having LEMS, you're welcome. :)

For the next 10 months, I'm going to dose off the prednisone. And I've already stopped taking my immunosuppressant, Cellcept. After 2 months off, I can start Rituximab, which only has to be taken once a week for 4 weeks every year. However, for the next 2 months, my immune system will be allowed to run rampant. The plasmapheresis should help a little, but there's no knowing how these next 2 months are going to play out.

As far as symptomatic treatment is concerned, I'm still going to take Mestinon and add to that 3,4 Diaminopyrimide (DAP). This new medicine helps out a lot, but the side effects are highly unknown. It isn't approved by the FDA and I can only take it as part of a clinical trial. The " up" side? It's free. The "down" side? The personal side effects I've noticed are pretty intense, but I think they will become less severe over time.

Recovery will take a lot of time--we've discovered that I have some pretty severe muscle atrophy as a result of the long-term prednisone use. This contributes to the problem I have with walking. In 4 or 5 months, I should be on a low enough dose of Prednisone that muscles will no longer be affected, but then we're not sure if therapy will be effective in regaining muscle control.

It's just a whole lot of changes at once, and I'm not sure I'm ready.

Mayo Days 3-8

Tests. Consultations. Lots of sitting, waiting.

I apologize for not keeping the blog very updated. It didn't take long to get weary and worn down. After a few days, I began napping in the afternoons and sleeping longer nights, so I wasn't on the internet much.

So far, no real conclusions have been drawn...I am slightly anemic (which can be fixed by taking a supplement for about 3 month) and I need a little more Vitamin D. These are not big things, so we haven't found any real answers yet.

I am meeting with a new doctor tomorrow...his specialty is neuroimmunology. I have to wonder, since this problem is obviously neurological AND autoimmune, why did it take me a week and a half up here to get in to see this guy?! It seems to me that this should've been the first person I talked to. Anyway, we'll see what he has to say.

Mayo day 2

Funny story--when I got here yesterday and was checking in, they asked me to step on a scale. The number that popped up on the wall was 169.9.....I almost had a heart attack! I mean, how in the world? Then, I realized that this measurement was cm...it was my height. :D

Tonight I'm weaker than normal--the neurophysiology department asked me to not take my Mestinon for 24 hours so that the results of my EMG tomorrow won't be skewed by the presence of any latent medication. I understand that. It just makes it more difficult to move until after noon tomorrow. :)

We didn't do anything exotic today...I had an MRI of the brain and did a little shopping.

Tomorrow, we're have 2 scheduled procedures and 1 scheduled consultation, then we're going to stage a "sit in" at the neurologists' office for an appointment. :) We have an apt. scheduled for that office, but it's not soon enough.

We shall see.

Mayo Day 1

The Mayo Clinic is awe-inspiring. I mean, I don't expect them to heal me in the few days I'm here, but I have confidence that if anyone in the world can figure out what is going on...it'll be the guys here.

The clinic is just this huge (insanely large) building with 3 wings and 20 floors (including a Subway level which connects it with all the surrounding buildings). Each wing of each floor specializes in something. So when I checked in this morning with the receptionist at internal medicine, I was in the system. By the time I had finished giving my history to the doctor a couple of hours later, I had my itinerary for the day. They just printed it for me and I went from floor to floor getting labs and tests. When I got to a reception desk and told them my name, I was already in the computer and on the schedule. It was so efficient. *sigh* If only all healthcare facilities could see how this place runs....I'm swooning at the organization. LOL

I spoke to my coordinating physician this morning and he has referred me to several specialists which I will see as soon as they are available. He also ordered several tests, labs, etc. Once I have seen everyone and done everything he has asked me to, then we will meet again to reevaluate. He gave me the basic initial reaction I've had from all my doctors: this is a complex case that may or may not have myasthenic properties due to the facts that I don't have the classic MG symptoms and am not responding well to aggressive MG treatments. He did add, however, that their goal is not to treat the symptoms so much as determine the underlying cause for them. I appreciate that--that's what I want, too. Find the cause and fix that, and hopefully the resulting symptoms will resolve themselves.

Overall, I am very impressed with this facility and hope for the best. :) If we can make progress, these doctors will be my new heroes.

The story part 3

So, we wait, and we wait, and life is pretty miserable about now, but we wait some more...

3 weeks later and still no change. Same symptoms and now I'm getting consistently more tired all the time.

It just so happens that I have an amazing Family Doctor. I went to see him and he was so concerned and spent so much time trying to help (as a matter of fact, he's been with me this whole time, still is my doctor, actually). We finally decide that in order to run all the test he wants to run, we'd be a lot more efficient if I were admitted to the hospital.

So, here we are, mid-September, and I am now officially on a leave of absence from work and in the hospital. *At this point, I have so much faith in my doctors and the practice of medicine that I'm POSITIVE something will be found. I'm extremely confident that this would be the start of the end of my troubles.

They run every test imaginable. I had MRI and cat-scans of the head and brain, the back, the abdomen. I had so much blood work that they used up all my veins they could access and put in a PICC line for the duration of my stay. I had spinal taps and all kinds of uncomfortable neurological tests where they stick needles in you and shock you with electrodes. I had swallowing tests and physical therapy tests. If you can think of a diagnostic test, I had it. I had ultrasounds of the abdomen and heart. And the list goes on.

I spoke to a nutritionist and an immunologist and an infection diseases doctor (because EVERYONE that knew I went to the Philippines just insisted that it was a bug I picked up over there). I had a neurologist and a cardiologist and a radiologist. I had the largest team of doctors that I could have ever imagined.

And guess what? every test was normal. Every one. No one could find anything wrong...except the eye doctor, and it was pretty obvious that my pupils were dilated and unresponsive. But everyone kept saying they'd never seen anything like it. No idea what was going on, etc. They did, however, tell me that my eyes would never recover because they were dilated because the nerves were damaged. Too bad. GAH! I was devastated! How do you so callously tell a previously-healthy 25 yr. old that her vision is impaired for life? Jerk.

Well, the next step, after a full week in the hospital and no answers was to head to the University of Alabama in Birmingham to see a Neuro-Ophthalmologist who might be able to help. This trip was gonna be fun, though, because UAB wasn't in-network with my insurance.

The story part 2

The next step was to see my optometrist--I mean, my eyes weren't focusing, right? Tests were mostly okay...my pupils were a little sluggish to respond to light, but nothing to worry about. I also had a test that showed maybe some slight neurological anomalies, but, again, nothing to worry about.

So, I'm off to see a neurologist in Enterprise. Well, 15 minutes with this, this, JERK and he says it's all in my head...did I want to see a psychologist? Well, fine, then. Um, no--to the psychologist.

Well, by this point, all my running around is doing is costing me money. No answers. No help. So, for a while, I just trudge along as best I can. In addition to the dry mouth and dry eyes, I am not really able to digest food...everything just gets stuck in my esophagus or intestines. I am more constipated than I have ever been in my life (sorry for the ickiness...just the truth). And things just get worse.

Then, one day in late August, I wake up and realize that I can't see very well...I look in the mirror and my eyes are dilated and fixed. No response to stimulation. So everything is fuzzy and I am overly sensitive to light. At this point, my whole family is terrified...what is going on?

We run in to see an ophthalmologist--since my last run-in with an optometrist was a bust--and it takes him about 10 seconds of looking in my eyes and listening to my symptoms to tell me that I've ingested some kind of poison. My body systems are slowing down and it's because I've been exposed to something toxic. Don't worry, he says...in 10-14 days, you should be fine.

Whew! What a relief!

The story

I took a little time today to chat with a friend and I gave a brief synopsis of what all has happened to my health over the last 2.5 years. It was good to remember a bit about where I've been vs. where I am today.

It was 3 years ago this month that I got my first full-time (40+ hr/wk) job. This means that I have just graduated from BYU and road-tripped back home. I am living my myself for the first time EVER and I love it! I just found out that I am going to be sent to Atlanta for 2 weeks for training for work, where I will get my own hotel suite in a nice hotel, and I'm feeling pretty special.

Fast forward 5 months. I'm loving work. I have a boyfriend who loves the outdoors as much as I do, I'm making money...life is good. It's the 4th of July week of 2009 and I'm down in Destin, FL spending the week sailing and hanging out with Danny's family. This is the first time that I noticed that I was kind of tired--more so than usual, even for playing outside in the heat. So I think, maybe I'm a little dehydrated. I bought some Gatorade and slept a little more.

2 weeks later, I'm at work and I'm struggling. I'm beginning to think I'm sick. I'm sleeping about 12 hrs/day (every minute I'm not at work) and my eyes are having difficulty keeping my computer screen in focus, not to mention that my mouth is so dry ALL THE TIME that I feel like I've been sucking on cotton balls (I had to keep water at my desk, so I could swallow some before I had to speak to anyone).

I make what is to become my first of MANY doctors visits. I walk into a facility, and the doctor there runs labs and tests, but has no idea. So, I go home to wait for the results of the bloodwork. All clear. Little did I know, this was the first time of many that I would hear this statement and baffle my doctor.

To be continued...

Today

I've been having plasmapheresis treatments since June--to help keep symptoms under control. We started them in preparation for the thymectomy that I had in July 2011. We've been continuing with the plasmapheresis as needed since then. We kept hoping that each round would be the last one needed...but that doesn't seem to be happening.

For the first two rounds, we had to travel to Atlanta...which was a big deal since each round takes 10 days and we had to come up with hotels and food for those days, not to mention the gas to get there and back. Then, finally, we found a dialysis place that would do it here--so much less trouble. However, each round requires the surgical placement of a central line, then I have to keep the line in my neck for the course of the treatment, which is both painful and inconvenient. The treatment itself is painless, but it leaves me feeling drained. The whole process is extremely inconvenient.

The benefit is simply about 3 weeks of functionality. That's it. All this trouble for 3 decent weeks. The process of plasmapheresis is simple in concept: pull blood out of the body, separate the plasma from the red blood cells, discard the plasma and replace it with artificial plasma (albumin), return the blood. This process gets rid of the antibodies that my body is creating that is causing a disruption between the nerves and muscles. So, I get temporary relief, but only until my body can create more nasty little antibodies.

We've been doing this as a means of being able to function. The medicines I'm taking, without the plasmapheresis, just keep me alive; I can barely move when I'm on them alone. The plasmapheresis helps enough that I am able to move a little. But I can't do it forever. It's rough on the large veins in the body. And all doctors that I've seen are out of ideas. I've been to over 30 different physicians over the last 2.5 years.

Today, I got an appointment at the Mayo Clinic in Rochester. I feel like it's my last option. If I go up there, and they can't come up with a plan to fix me, then I don't know what else to do. And I can't NOT go just based on the premise of being scared they won't find anything... So, here we go. Let's see what they can do.

Monday

Mondays are hard for me. I think it might be because Sundays are so long...and it takes a lot of effort to get up, get ready, and be at church on time. Not to mention that being there, actively involved takes a lot of energy. I enjoy my Sundays, but they take a lot out of me.

I'm just going to write a little here about how I feel and some of the struggles and events that occur concerning my health. This is not a pity-me party or a bid for sympathy. I've just been asked a lot recently what is wrong with me--you know, what are my health problems. How do you put 3 years of hospitals, tests, doctors, surgeries and failed treatment attempts into a sentence or two?

I have Myasthenia Gravis (MG), we think. I have seen over 15 neurologists, and this is our best guess. We think it is some rare form of MG, in association with something else not-yet-diagnosed. I have a few of the classic MG symptoms, but not all. And some very MG-specific symptoms don't present with me. And some of the medicines for it are helping a little, but not much. So, no one knows.

What do we know?

It is auto-immune.

Without high-powered steroids and immuno-suppresants, I am completely bed-bound, too weak to even lift a finger without help. (We know this because during one of my stubborn streaks, I quit taking the medicine and had to spend 12 weeks in bed with a full-time nurse at my side).

Even with the meds, my activity is extremely limited and my strength is almost non-existent and is used up quickly. Example, on Saturday evening, I was cutting my fingernails and I struggled to be able to squeeze the fingernail clippers.

There is a breakdown in communication between the nerves and the muscles. Over the last 2 1/2 years, this has led to a severe muscle atrophy (due to periods of forced inactivity). I am unable to exercise to rebuild muscle, because the muscles don't work well, and when they do work, using them repeatedly is more detrimental than helpful. For example, I had a long day at church yesterday, but instead of that helping to build stamina and muscle, I have been virtually unable to move today. Any time I push myself, I pay severely for it.

That is what we know. We don't know how to help. We don't really even know what's wrong. I have seen doctors all over the US, and even been to Mexico to try treatments that are illegal in the States. Nothing helps. I have tried spiritual healings and blessings. I have tried dietary changes and supplements. I have tried holistic medicine--that almost killed me.

We are working on getting an appointment to see someone at the Mayo Clinic. It is the only option left to me, at least that I see at this point. As I type this, I'm sitting in my chair with my head resting back on my chair, because I don't have the strength to hold it up. I have to psych myself up to try to muster the strength to get up and move into the bedroom. This is typical for me in the evenings.

And yet, I truck on. What choice to I have, really? Just one step at a time.