Thanks to chemo Victoria Beckham and I share the same en vogue look.
Our lovable monkees
Making dough for our Girl Scout Daisy Troop.
I have spent a good bit of time today reading my past entries. I recently joined a website called facebook, and was able to get in touch with so many old friends! Kimmy and Colleen from high school, Tracy, my maid of honor and college roommate, and Charles!!!, my dear friend from all the way back in kindergarten. What a powerful tool.
Reconnecting with people I have been so out of touch with for so long gave me reason to reflect, I suppose. So I started thinking, how long have I had this blog? What have I written about? So many friends have commented on our journey, and I appreciated it, but I didn't really think about it until today. You see, we wake up everyday and just go about our business. Both Jason and I, and I suppose our kids following our example, not thinking about how huge Myasthenia is in our lives and how much it has defined us. We try very hard to NOT make MG the focus of our lives, but it still is. I believe it is a lot like having a third very annoying, upsetting, disruptive person in your marriage. Or an evil house guest that won't leave. lol I am not complaining. This is just the way it has been. Thankfully chemo has lessened the burden tremendously. I keep thinking back to how it was last year. I hardly believe we survived.
I thought this might be a good time to review what the disease is, and what has gone on all these years. My beginning blog entries are very technical, and the more recent ones don't really talk about the MG at all!
A Bit of History....
In March of 1995 I was diagnosed with myasthenia gravis. It is an auto immune, neuro-muscular disease where, simply put, my immune system has falsely attacked my muscles. Only the voluntary muscles are hit, so the heart still functions just fine. The muscles become easily fatigued because the full "message" that they need to move is not getting across because the receptors are covered by antibodies.
I was in my second semester senior year at Villanova, engaged to be married, working as a student teacher, volunteering in South Philly, the vice president of glee club, the president of ski club- the list goes on. What I am saying is, I was busy. When I started to lose weight I thought it was just because I didn't have a lot of time to sit down to eat and when I came home from teaching and needed to lay down I just thought I was doing too much. I didn't take the time to consider that I was having a hard time chewing and swallowing or that my muscles actually hurt they were so fatigued. One day I woke up with a sore in my mouth. I trooped off to the dentist thinking it was my wisdom teeth coming in. He gave me a prescription for what turned out to be an ulcer in my mouth. By that evening, my speech was very slurred. Jason and I laughed it off, blaming the meds. I thought my tongue was swollen. I took the weekend to rest and I did feel a little better. I thought it was so weird that when I woke up in the morning I could talk and by the afternoon I could barely get a word out. Plus I was certain that the shortness of breath I was experiencing was due to my tongue being "swollen".
The following week I returned to my 8th graders with a slightly less smiley smile. I just couldn't get my smile to work! Half way through my first class of the day, my cooperating teacher called me out into the hallway and told me to go down to the teachers lounge and call an ambulance or something- my lips had turned blue and I was gasping for air. Of course I tried to shrug it off, but I did go sit down, called Jason and we reluctantly headed to the emergency room. After scaring the crap out of me, the intern sent me off for a CT scan to determine how severe the "stroke" was that he was certain I had suffered. Thankfully I was cleared, and shipped off to see a neurologist the next day. By the time of my appointment, I was quite certain that I didn't need to see a neurologist, but a psychiatrist. What was happening to me was just way too weird.
March 19th 1995: Within about a minute of meeting me, the Neuro said he thought he knew exactly what was wrong and had me sign a waver to do a test, called a tensilon test, right there in the office. I remember being a little frightened by the waver... all these scary words about heart attack and stroke. Regardless, I really wanted an answer and this would give us a quick one. Dr. Reid had me lay down on the examining table and injected the tensilon into my vein. Within seconds, my heart felt like it was going to beat right out of my chest and dance across the floor and I could TALK. Totally, completely, easily talk. It wore off in 30 seconds or so. Although I was glad to be rid of the heart palpitations, I was devastated that my ability to talk was also gone. The doctor gave me a few minutes to recover and met me in his office where he proceeded to give me the diagnosis that would completely change my life.
Myasthenia Gravis. He told me all about it, and I took meticulous notes. I was happy. Yes. Well, not happy exactly. Relieved that I didn't need to go check into a psych ward. Relieved that there was a real thing going on- and it had a name. I was given a prescription for Mestinon (first given and easiest drug to take and to tolerate for MG)and I was sent along my way with a follow up appointment in a month or so. I completely believed I was going to be just fine. This might be a minor inconvenience, but with the support of my family, and Jason of course, I would be fine.
That weekend, Jason and I had our Pre-Cana training to get ready for our wedding. Mestinon in hand we packed up and trotted off to class. Then the itch started. And the hives. And the burning... ohhh it was miserable. Turned out I was allergic to the Mestinon. Frightened and not sure what to do, I called the neuro and was told absolutely not to stop taking the drug, but to take benedryl along with it until I could get back into his office. I was so miserable, even with the benedryl that I had to stop the drug. I returned to Villanova, ready to take on the world. MG or not, I was determined to be fine. Immediately following my diagnosis, my parents started calling around to find the top specialist in the field. We were able to get an appointment at UPenn that very week. Dr. Schotland took one look at me and wouldn't let me leave the hospital. I guess that was the moment I started to realize just how serious this was. Like, really? I am in danger just going back to my dorm to get some pajamas? Oh yes, Claire. You could stop breathing. You are about to end up on a respirator at this very moment. I started to wish I was crazy instead of having this disease.
After several more tests, they put me on prednisone and signed me up for plasma-exchange. All day long, different med students, interns, residents, nutritionists, speech therapists, occupational therapists- pretty much everyone in the hospital except the cooks in the cafeteria- came to "visit" me. MG is rare, particularly in this extreme form. I did my best to take it all in stride. They were learning from me and I was learning from them. I still have pages and pages of notes on how to deal with living with MG and the benefits and side effects of prednisone. Ahhh prednisone. I could write a book about the lovely effects of that EVIL drug. Instead I now choose to ignore the whole saga of my 14 year on and off relationship with steroids.
Plasma-exchange on the other hand- now there is a treatment that WORKS. It is a miracle for me. The doctors couldn't believe what happened that first time they hooked me up to the machine. (there is a previous entry on pheresis- it explains it all and even has a picture of me hooked up- sunday, april 27, 2007 Hurray for my arms!!). Midway through the treatment, my speech started to clear up. I felt a million times better. Not perfect, or even close, but so much better. Dr. Schotland, who is famous for his treatments of MG, and was most definitely not a young man, came flying to my room to see this insanely fast turn around. The positive effects didn't last very long, maybe an hour or two, but they served to invigorate me. I now knew, without a doubt that I was going to make it through this, and I had a treatment that would keep me healthy. The docs at HUP removed my thymus gland (involved in the development of your immune system, but should shrivel to nothing in early childhood) and told me I was eventually going to get it all worked out and find a treatment that would work long term for me. I had no idea that would take this long, but...
The story goes on from there... plasma exchange is not a long term solution. It is something that works when a patient is in crisis, holds it's own risks and isn't all that convenient. 5 exchanges remove approximately 90% of the antibodies causing the symptoms. Being as special as I am, I respond very quickly during the first 2 exchanges, and then my body seems to "make up time" and goes even more haywire than it was to begin with, so I do very well with 7 exchanges, rather than 5. Given that the pheresis can only happen every other day (because they take out good stuff along with the bad stuff), I spend at least 14 days in treatment to get about 3 and a half weeks of feeling good before the antibodies start to take over again. So plasma exchange was out for a long term answer, but is great for me in a crisis.
So the different drug therapies began to be discussed. I've been through many, many courses of months on steroids getting me well enough to try other drugs that haven't worked. Ephedrine, Imuran, Cell Cept, Cyclosporin, IVIg, the reintroduction of Mestinon... all with some hope, only to have our dream of me being healthy destroyed, or delayed (?) again and again. Ask my family, ask my true friends. I have felt sad and angry at times. I have NEVER EVER stopped believing that this drug, this treatment, this positive thinking and energy flowing through my body is going to make me well.
We tried everything there was. Many of them several times. Last year we finally got to the point where there was no other choice but to completely destroy my immune system and start over. An immune system overhaul. A rebooting of my system. So we tried chemotherapy. Every single treatment I have tried, I have believed would be the answer. This time I not only believed it, it had to be. We were kind of at the end of the road. If this didn't work, we weren't sure where we would turn, and Jason and I were pretty wiped out from all the years of me being in and out of ICU.
And here is where my superwoman complex comes in. You see, I need to be well. I need to believe that I can handle anything thrown my way. Not all on my own, of course. Somehow through this, I have a fantastic marriage, 2 insanely cool kids, a great little pug dog and a cat who loves me so much she keeps me awake many nights looking for a hug. Having wonderful family surrounding us and awesome neighbors and friends has made all of our burdens so much easier to carry. All of the positive energy and prayer that you all have shared with us over the years has been one of our greatest blessings, and I am certain that it is one of the biggest reasons I am doing as well as I am right now. Thank you all.
At the moment, I have completed 8 monthly treatments of Cytoxan. Initially they told me that I would be given a low dose. Turns out I got 2-3 times the dose a lymphoma patient gets. Still, I am amazed. I have at least 2 more years of chemo remaining, but now it is once every 3 months instead of monthly, and next year it will hopefully be once every 4 months. And I have hair!! How cool is that? Head of hair aside, I am infinitely better than I have been. Everything is easier. I still have to go in every other week for 6 hours of IVIg and lots of trips to the hospital for blood tests and check ups. Now when I am feeling good, I am actually doing well! I am swimming again, not resting every day (many days, but not every), and truly taking care of my kids and my husband the way I always imagined I would. I planned on the chemo being a cure. So far it isn't- but we still have a ways to go, and I am thrilled with the results so far and know that we can only get better from here.
