Hurray for my arms!!!

Let's all give a cheer for my poor little arms. They held out and I was able to get what will hopefully be my last plasma-exchange yesterday. For this round anyway.

The Real Deal...

It has been a very long few weeks, and we are all pretty worn down. So many people have written and called to tell me that they are thinking of me, and many of you have called me an inspiration. These are wonderful, flattering words, and I truly appreciate them. It isn't that I am always smiling and thinking that things are just fine. I am certainly a very positive person, but there are times when even I get down. I suppose I wouldn't be human if I didn't feel some anger towards this disease and what it has done to me and to my family. There is huge guilt that comes along with accepting help- but I don't have a choice.

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When it comes down to it, I hate the disease, but I know how to live with it. More than anything HATE the drugs that come along with the disease. I hate the way they make me feel and look. But I know they are a temporary, necessary evil, so I stick with them. This most recent exacerbation has been so hard because we tried using the prednisone and then the IVIg (five times) and none of that worked. So since February I have been taking drugs that I hate that haven't done what they needed to do. And then I had to truck down to Boston every other day for 2 more weeks for 5 more treatments. It is just a lot to handle. That includes involving all the fantastic people who rally when I get into trouble- and not being able to be the full time mom and wife that I love being. Plus, this exacerbation is following the last one in November when I ended up in ICU, which also took several months to get out of. Basically, the past 3 years have been a series of one crisis after another.

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I hope that I don't sound like I am whining here- it is what it is. I am just saying, people see me and so many say, "wow, you LOOK great" and I know they are surprised. That is one of the things about this disease that is hard to live with. People can't see what is going on inside of me, and how much of a struggle some of the things that everyone takes for granted are for me. Like using a hairdryer, or writing a letter, or chewing a bagel. And I know that the steroids have made me gain weight like they do to lots of people- lets face it I was way to thin to begin with, but hey, 30lbs is 30lbs even if you started out being too thin. It is still a shock to your system, and it is hard to look in the mirror at a face you don't recognize as your own.

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My Mom gave me such a boost last week. She kept telling me how proud she was of me and how I handle all this (and she gave me lots of hugs- which was just what I needed last week). After leaving my primary care appointment when I found out about the high blood pressure, I had gotten a lot of information to process, not all of it good- and I just asked my folks to hang in there with me. Mommy commented after I had moved through my thoughts and made sense of it all that she thinks it is great that I am able to take whatever they throw at me and deal with it. It is true- I deal with one thing at a time and make it work for me. I keep getting thrown these curve balls, and I have to keep making it work. There is no alternative, and I will not be walking around complaining all the time. I do want to say thank you to those of you who listen when I do need to complain, or cry or just yell. You all know who you are- and I cherish each and every one of you!

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Enough of that....Now for some explanations and good news. I am feeling much stronger after the last treatment, and if this cold doesn't throw a wrench in it (once the body starts producing antibodies to fight infection, it usually produces MG antibodies too), I think this series of pheresis is going to hold me for a while. I had a tough night last night- these darn steroids keep you from sleeping, and the cold didn't help- and I still feel stronger today than I did yesterday. In the past, a series of 5 would keep me strong for about 3-4 weeks, and then I would have to start the process all over again. The big difference this time is that I am taking the cyclosporin, and the goal is to get me off the steroids and on the cyclosporin, hopefully not needing to go in for pheresis at all, or very rarely. I am down to 25 mg of prednisone, and tapering as planned. I wish we could go faster, but I am going to listen to the Guru- who is a huge fan of what is in my mind a ridiculously slow taper. (I am pretty sure he has never taken prednisone lol).

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The best news of all is that my last cyclosporin level showed that I have gone from a 55 to a 301!!!! The optimal dose is between 100-400. That means once I am off the steroids and the pheresis has worn off, we will know FOR SURE if the drug is going to work or not. I actually cried when Janice, my nurse, told me. And the most excellent thing is, if this doesn't work, which IT WILL, we have a plan. We will be using the pheresis if I get into any trouble- and I am to call the minute I start to feel it, not wait until I am sounding like I have had a case of wine at 10am. Hopefully that will keep the pheresis down to a minimum- like maybe one or two treatments rather than 5. If I continue to need the pheresis on a regular basis, then we can assume with conviction that the cyclosporin is not doing what it needs to do, and then we move on to the cytoxan. I am not going to get into details about that, since I am certain that I am not going to need it.

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Photo explanation... For those of you who are interested in what is going on in the photo above, in everyday non medical speak, the tubes in my arms are attached to dialysis needles going into the crook of my arms. The hardest thing about that is the needles are made of steel, so you absolutely can't move a muscle during the treatments. Very hard when you are taking diuretics, by the way! I am an old pro at it, though. I am usually floating by the end of the 2 hours (depending on how much time it takes to hook me up, I can be sitting like that for 3 hours or so). Jan and Danata keep telling me we can use a bedpan- but I am not willing to go there yet. I digress.

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Usually, the right arm is the arm that they draw the blood from. I think they do that because it is my stronger arm, and I have to use a little ball to pump the veins every once and a while. The left arm gets really tired when they draw from there. The blood is then moved through all the tubing to a centrifuge where the plasma is separated from the red and white blood cells. That big yucky looking bag hanging on the machine (right side) is the old plasma- filled with those nasty antibodies that are causing these symptoms. The problem is that the plasma also has all kinds of good stuff in it- proteins, etc. That is one of the reasons that we do the exchanges every other day- to let my body play a little catch up.

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After the plasma has been removed, albumin (and some saline) is then added to the red and white blood cells and the new "clean" blood is then pumped back into my left arm. The albumin is hanging in those bottles. The white thing that looks like it has lines on it is the heater. We spend a lot of time turning that thing on and off during my treatments, because I tend to get very warm- then cold. The treatment leaves me feeling puffy and tired, but otherwise I generally don't have much in the way of side effects. It is kind of like the feeling you get right before you get the flu. Wipes you out, but it passes, usually within a few hours. It is worth it- especially compared to the IVIg, which gave me migraines and terrible flu-like symptoms. For now, it is the ideal treatment, because IT WORKS!!!!

Thank you all again for all your love and support. I always say this, because I really feel your positive thoughts and prayers. It is helping, so keep it coming!

A week ago tonight

I hesitated to post this, but Jason insisted. Most people say they can't see the puffiness from the steroids, but I completely don't agree! It is mostly just in my face and belly- and is wayyy more pronounced when I eat potato chips or anything else even resembling the taste of salt. heehee. One slice of pizza and my eyes practially swell shut from the fluid retention.

Jason arrived home, much to our pleasure, a few days early last week. Here are the kiddos and I in one of our many gifts from Japan. We are so happy to have him back home- even if things are still kind of reeling from all these treatments, drugs and trips to Boston.

Lots to say

Hi everyone- I feel like there is lots to say- but I am kind of out of energy to discuss it. Just a little tired today. Yesterday I had my 4th in what is going to turn out to be 5 exchanges. As you might remember, I was hoping to only have one. I have been able to stay pretty up most of the time, but I am kind of drained today. Hey, it happens to the best of us.

We had a little trouble getting to my veins yesterday- that is the first time I have ever had that happen, so I consider myself pretty darn lucky. I think it is fantastic that we haven't ever had an issue before. It makes me feel blessed that I don't have to have a central line to have this done whenever I need it.

There are two nurses down at St. Elizabeth's who take care of my pheresis treatments- it is a very specialized field, and they are wonderful women. Danata was running the exchange yesterday, and she didn't hesitate a second to call for someone else to run a different kind of line so we could complete the exchange. If they hadn't gotten it on the last stick, I would have ended up going home and coming back for another treatment on another day. It is a testiment to how good she is that she called someone who works more often with the other lines, rather than just to keep fishing. I am grateful to have both her and Janice taking care of me on a regular basis.
The big thing is that I am feeling much better and that I will be having one more exchange, problably on Saturday morning.

We have to wait to make sure my arms are up for the fifth treatment- my blood levels are bouncing right back, but my arms are pretty bruised and we don't want me to get down there and then not be able to do the treatment. I also have a cold, so we it isn't a good idea to go ahead if I am not feeling up to it. If I have a fever the 5th is off- until I am better. I am following all the instructions, not lifting anything at all and icing- and now heating my arms to help "plump them up". The hardest thing about it, other than the discomfort, is trying to tell little Thomas (who isn't even 2 yet) that I can't pick him up. When he asks, I just sit down on the floor with him and give lots of hugs. He is a real snuggler, so he seems pretty content with that most of the time.

Katherine has been fantastic through all of this. She just seems to think it is totally normal for me to be spending all this time away from them in hospitals. I was thrilled out of my mind yesterday when she asked if I was going in to the hospital and I said yes. She asked if Nannie and GrandDad would be babysitting, and when I said yes, she said, "yippee!!!" It made my heart soar. It is really great that she is adjusting so well. I am proud beyond words of Katherine, Thomas, my parents and Jason for all their constant love and support over the past 2 weeks. (not to mention the past 12 years and most especially the last 3) Everyone in the family, our neighbors and friends have been beyond supportive, and I do feel the good thoughts and prayers everyone is sending. Thank you all so much. And a special thanks to cousin Margo for keeping me laughing.

It surprises me that after all these years, I can still be learning new things about the disease and about the treatments. Yesterday I was happy to hear that the reason I see so many different types of doctors down in Boston is that once Dr. Gorson orders the pheresis, I am then pretty much controlled by the hemotology/oncology unit. Makes sense, but I never thought about why I was being managed by anyone other than Dr. Gorson. Dr. Parameswaran has primarily been managing my day to day treatments, and she has been great, too. She has such a calming demeanor- and she even laughs at my jokes. Not to mention she TOTALLY knows what she is talking about. Wonderful combination. St. E's is a teaching hospital, so lots of docs come and go, but they are almost all really great. And if they aren't, heck, I am on steroids, so I just tell them that I am not thrilled with the care I am being given at the moment. This disease is too hard, and the drugs do so many crazy things to you to keep your mouth shut, I guess.

I was going to try to post some pictures of this last treatment, but I just couldn't bring myself to do it... I just don't look like myself, and I am pretty sure if I did post the one with me and Danata that she would never ever forgive me. We both look drugged. ha. The other pictures of me- my ego won't let me post. I will find some better ones to share with you all!

Thank you all again for your unending support and good thoughts.

3 Down maybe only 1 to go!!

So I wore my glasses to drive myself down to Boston today. At a stoplight I paused to look in the mirror and put to on some lip gloss.... couldn't at least one of you have told me that I am growing a mustache?!! Ha. Good ol' prednisone. At least I am not thinking about driving my car into people who cut me off in parking lots. Lemmy see- mustache or murderous thoughts? I guess mustache is the preferable choice.
So I am laughing about this with my nurse down in Boston, and she looks at me and says, "Oh no one would ever notice that. Just don't mention it. Umm, Then again, maybe you should get some bleach!" HA It was pretty hysterical. If I wasn't hooked up to the machine I would have busted a gut. I know after the last fun I had with the wax lady (the red mustache that lasted for 3 weeks was definitely NOT worth getting rid of like 10 hairs) I will never do that again!!!!

On a serious note, I have now had 3 plasma-exchanges, and I am really starting to turn the corner. Feeling very positive that the cyclosporin is going to kick in before I actually do kill anyone on the prednisone. Maybe someone should ask my family if that is true or not. But my Mom and Dad are still here, and I am pretty sure I haven't insulted anyone lately. LOL.

I did get to have a wonderful pop in visit from Guru Gorson today. He was really not expecting that I would need any more than 2 pheresis treatments, so I guess that he wanted to see me for himself. Either that or he just couldn't stay away from my bubbly personality. We are still sticking with the plan (see April 13th's entry). I should say here that it normally takes 5 plasma-exchanges to bring someone out of crisis- and I was very close to a severe crisis last week. Once I get shortness of breath, we are in pretty deep. The concept is that 5 exchanges removes approximately 90% of the anti-bodies causing my symptoms. This isn't anything you can measure- it is just what usually works for people in crisis. As I said, last time I needed 7, but I was really at my worst that time.

The only change in the plan is that in the past I have waited until the symptoms start fairly early in the morning (about 2 hours after I wake up is my general rule) to call a neuro and get some kind of treatment. I am now going to be calling the Guru as soon as I start having symptoms in the hopes that I will get in there, get some clean blood and be able to only have one or 2 treatments every month or so until the cyclosporin has had the time to prove itself. Which it will.

I am taking my bloated self to bed now. All my love to all of you. Thank you for all your good thoughts and prayers. Hugs. Claire

PS- a couple of sites that might be interesting for you:
A site with overviews of MG and all the Meds: http://www.myasthenia.org/
A site about plasma-exchange: http://www.healthline.com/galecontent/plasmapheresis

Ups & Downs

Well, the great news is that my Blood pressure has finally started to come down. My primary care doc put me on a diuretic, so that has really helped a lot. The fact that the diuretic is working leads us to believe that the BP issue is from the prednisone, not the cyclosporin. Great news.
The other news is that I am going to go ahead and do a full course of pheresis (plasma-exchange). I have had 2- and I am responding well, as usual. Normally, 5 exchanges remove approximately 90% of the antibodies that cause my symptoms. For now we have 2 more scheduled, and we will see how I am to decide if I need another.
I will keep everyone updated.

Happy Plasma Exchange Day

Hi Everyone. Thanks for all the positive feedback on the Blog. I haven't really had a chance to write back to all of you who have so thoughtfully taken the time to email me. Jason has the Laptop, so it is hard to sit long enough to write back to everyone. I am in the process!

Today I went down to St. Elizabeth's for a plasma-exchange. They decided after I arrived that we could do a little larger exchange, which is a great thing, as far as I am concerned. The more of these evil little anti-bodies they get out, the better. I had become pretty weak, so I was really happy to get hooked up. I really hate getting short of breath. It pretty much sucks. LOL Thankfully we have plasma-exchange available- and we know it will work.

My wonderful nurse down in Boston reminded me how bad I was last November, so it is pretty easy to keep things in perspective right now. I am home and resting comfortably with a lovely glass of merlot at the moment, rather than being in ICU. Last time I had to have 7 exchanges (I only remembered 6) and this time I had one and planned another for Saturday. I am really keeping my fingers crossed that this will keep the MG antibodies in check for a few weeks. If I need to go down once a week, though, until the Cyclosporin kicks in, that is what I will do!

Another bit of good news comes from my GP today. She said that my most recent bloodwork shows that the blood pressure issue is most likely related to the steroids, not the cyclosporin. We have started me on a diuretic to help alleviate the problem. This info is coming from the fact that my kidneys seem to be functioning really well, despite the cyclosporin.

So I stopped at the infamous CVS- people must be starting to talk about me and my pharmacist!- and picked up yet another script. Ahhh well, at least this one will help with the huge head issue. By the way, my parents went out last night and got another blood pressure cuff- if anyone is interested Brookstone has some nice ones that are really accurate. So hopefully I will be up all night long peeing. And maybe tomorrow when I wake up I will recognize the face I see in the mirror. I certainly recognize the heart and the fight I have come back to finding in the last week!

Wednsday April 18

The day started out well today... I thought we might be able to skip the plasma-exchange with some extra rest and ultra positive thinking.

I spent the morning with my kiddos and the wonderfully helpful folks. We started out returning the infamous blood pressure cuff to CVS- I swear that thing caused my blood pressure to skyrocket while providing me with no information what-so-ever, other than the repeating message, "error 3". Then we headed to lunch and brought the monsters home for a nice little nap.

I was so happy to be able to get out for a bit- I went and had my frizzy prednisone hair highlighted and cut to a very flattering shape around my poochie prednisone cheeks :) Remember that show, "third rock from the sun"? That is me- I am the big giant head. Incoming message from the big giant head... "steroids can make you nuts!!!" Just kidding. Kind of. This course is actually going much better than the others. I have a good attitude and I am really so hopeful that the blood pressure is from the prednisone, not the cyclosporin. This one is going to be it, folks. It is going to work.

It was wonderful to spend some time on me for a change- me that didn't include a hospital and drugs. And the haircut looks great! I will have to figure out how to post photos next.

About mid-way through my hair appointment, I realized that I was having more than just a bit of trouble talking. It was getting a bit hard to catch my breath. That is the point of no return for me, so I headed home and called down to Boston. I am going in tomorrow for a little tune-up. Keep your fingers crossed that one plasma-exchange will get me back on my way to health! If not, I will get a few more, and buy myself some more time until the increase in the cyclosporin kicks in.

Also, please keep Jason in your thoughts and prayers in his mission bringing home injured soldiers this week. Love and miss you Big!!!

Tuesday April 17th

Today was one of those wild days where all the time flew by... I had to go get my blood drawn to check on the cyclosporin levels as well as checking the levels on my kidney functions. Before we could even leave the house, the phone rang about 300 times. Then we had to get Kate to school and then Tom to sleep in the car so I could go to my GP appointment while Mom and Dad drove around keeping Thomas asleep. And then off to Katherine's parent-teacher conference that the teachers were kind enough to re-schedule because I was in the hospital last week and missed the previously rescheduled appointment. Then drop the kids and folks off at home and off to CVS to my wonderful pharmacist to get some more awesome steroids and a blood pressure cuff. It was a whirlwind day.

Of course I get home and the cuff doesn't' work... as if my blood pressure isn't high enough. I need a blood pressure cuff that doesn't work. Ha. It is actually kind of funny when you think about it. Thankfully, the kiddos were in pretty good moods, so they took a big jacuzzi tub with me and then happily trotted off to bed.

I am still processing the appointment. It was very rushed, because there was a mix-up and the wrong Claire was taken in before I was- what are the chances that there are 2 claires waiting for appointments back to back, and I am in the bathroom when they call the first one (which should have been me). I had about 20 minutes to meet my doctor. The poor woman has been following my case for almost 2 years and has yet to meet me. I have tried at least 4 times to make it in to see her, but with all the specialists, etc. I have never made it in. She was very understanding about it. It is just that when I am really bad, I am dealing with Doctors all the time- and then when I am not, I just can't fathom calling a doc for a regular checkup.

The long and short of it is that my blood pressure is still going up, so I am going in for an EKG and I am to monitor my blood pressure 3 times a day. I will probably end up going on some kind of med for it. The hope is that the pressure is rising from the steroids and/or the IVIg not from the cyclosporin. I am also going in for a DEXA scan to check my bones for osteoporosis from the pretencion. We really had almost no time to talk, so I will be going back in to chat further with her. The plan was for me to head in on Thursday for the EKG, but I have been taking a quick turn for the worse, so I will be headed to Boston on Thursday for a plasma exchange instead.
So Friday will be the EKG.

Update Friday the 13th

Hey everyone.

I have finally decided to do something about keeping everyone informed about my health without getting on the phone with each and every one of my dear friends and family. I absolutely must start off by saying thank you to all of you for all your thoughts and prayers over all these years. I completely believe that I have felt your good thoughts over the years, and that it has helped immeasurably. I am hoping that keeping this log will get everyone the details that you all deserve and need to hear while keeping me from talking about Myasthenia Gravis 24-7.

As most of you know, the past 3 years have been extremely difficult. Before the kiddos, we kind of just led our lives and had this crazy disease to "mix it up a little bit". It was difficult and scary at times, but nothing like it has been recently. I am not planning to make this a big vent session for me, but I do want to be honest. I have been through just about every drug available to use for MG, and none of them have done what they needed to do without major side effects. I am sure in later postings I will go futher into this, but right now, I just want to get the information out there about my most recent appointment down in Boston with my neurological guru, Dr. Gorson.

To sum it up briefly, I have taken Imuran (twice), CellCept and now Cyclosporin. I also have been through dozens of courses of plasma-exchange and Intravenous Immune Globulin (IVIg). I don't even want to count how many times I have taken massive doses of Prednisone. I also take Mestinon every 4 hours while I am awake. The long and short of it is that there is nothing that keeps me from getting into a crisis situation if I get a little cold or one of the kids doesn't sleep for a night or two. That doesn't include any physical or psychological stress that any normal human being faces on a regular basis.

Last November we faced the worst crisis I have ever experienced. I went through 6 plasma-exchanges, combined with the used of steroids and the introduction of cyclosporin. Since then I have been going into Exeter Hospital about every 2-3 weeks for IVIg while weaning off of prednisone. I was fairly stable until about a month ago, when I was forced to re-introduce the steroids at 6o mg a day (huge dose for my size). In the past 2 weeks I have been through 5 IVIg treaments- unfortunately, the drug just isn't working the way it did in the past.

At the moment, I am decreasing the steroids while increasing the Cyclosporin. We have just increased the dose to 150 mg twice daily, which is the ideal for my weight. There is still lots of room for me to increase the dose, provided the side effects don't become too bothersome or dangerous. Guru Gorson has recommended decreasing the prednisone by 5 mg every2 weeks. I would have liked to go down by 10 mg bi-weekly, but I am going to do my best to follow my doctor's excellent advice and wait the 6-13 weeks for the higher dose of cyclosporin to kick in. There are several other drugs I am taking to combat the really fun stuff that prednisone can do to you in high doses- and I am confident that I am going to manage the decrease the way he recommended.

If I should get into any trouble while we are waiting to see how miraculous the cyclosporin is going to be, I will be heading down to Boston for more plasma-exchanges, rather than continuing to try the IVIg here in Exeter. The side effects have become pretty intense, and it doesn't quite cut the mustard- so it isn't worth doing anymore. We have tried it extensively- and the only way to come to this conclusion was to really give it a full chance. I am kind of sad that I won't really be using my local neurologist anymore, because he is a wonderful man, and a fantastic doctor. Plus the nurses in both Short-Term Stay and the Endo unit here in Exeter are beyond wonderful and I have become close with several of the other cronic patients- and I will miss them. They are a huge part of why I have been able to stay so positive through these often difficult times. Exeter doesn't have plasma-exchange, and Boston is really the best place for me. It really isn't that big of a deal for me to head down there for treatments- Gorson says not to let anyone dare tell me that I can't drive myself down for treatments. I have a proven track record with this, and I handle the exchanges remarkably well.

Gorson and I are hopeful that this drug will work well for me. The biggest issue at the moment is that my blood pressure has been rising- I have an appointment with my primary doc on Tuesday to address that and a few other minor side effects that can be caused by cyclosporin. The blood pressure thing might not be that big of a deal- both prednisone and IVIg can cause the same issue, so it might not even be related to the new drug. There is another side effect that is bothersome, but not seriously problematic. The drug can cause the hands and feet to have tingling sensations and there is an odd feeling concerning temperature- it is kind of like the feeling that one gets after being out in the cold for a long time. Other than that, my renal functions need to be closely monitored, but so far all my levels are looking good. The excellent thing about cyclosporin is that it can be measured quantitatively- I have my blood drawn regularly, and once the level reaches a certain number, we will know if the drug is working or not. These are not big deals- but I know some of you want details, and this is it :)

I have filled most of you in on the Cytoxan, which is the drug many websites call "the last resort" drug for Myasthenics. This sounds scary, but it really isn't nearly as bad as it sounds. It is chemotherapy, but it is given in very low levels, and I would be monitored very closely. That being said, we have decided to hold off on this until my cyclosporin levels have reached the optimum level. Doc Gorson was very positive about the Cytoxan, though. He has used it quite extensively in patients with MS, and he is very confident about using it in my case if the cyclosporin doesn't do what it needs to. Plus he mentioned that there are a few other drugs that are out there and on the horizon that can also be used if the Cytoxan doesn't help either.

Overall, this is all very good news- I hope that comes across through all this medical mumbo-jumbo. Sorry if this is too many details for some of you. Please feel free to check in on the blog anytime- I will be updating it as I get more information. Both Jason and I are feeling extremely positive about the current plan of action. We have a plan, and it is a very solid one.

Again, Thank you to all of you for your thoughts and prayers over the years, and particularly to those of you who have been so helpful and wonderful over the past weeks.