Never a dull moment

Well my spirit is strong but my body is weak and so it goes with myasthenia. I saw my neurologist on Tuesday and a few changes have been made. First, he has decided to continue treating me with the IVIG but at 1/2 the dose as I seem to tolerate that much better. I was worried he would want to change me to plasmapherisis but given I have such bad veins and a congenital heart defect the IVIG seems to be the way to go. I was worried it wasn't working as well but he said that the fact I went so long with the reaction to the port stitches and only ended up in the hospital after I reacted to the Claritin, it was a good sign that it is doing as much as we can hope for at this time.

Also, the cyclosporine finally started showing up in the blood labs....hooray!!! This means I don't have to move to another major immunosuppressant but I am still not functionally where he would like to see me so he increased my dosage again. This is never fun because the first 2-3 weeks I have severe nausea and stomach cramping not to mention a need to visit the restroom every hour throughout the day. Hopefully that will taper off as I get use to the dosage.

We also broached the subject of having a thymectomy again. I am hesitant as it is major surgery but am getting closer to surrendering in the hopes that it will work and make me stronger. The evidence is not great in that 75% of patients do not see an improvement but I agreed to have a consult with the thoracic surgeon to weigh out the pros and cons. Please keep me in your thoughts and prayers as this is a major decision for me and my family.

Lastly I found out that my labs have been off for the past few weeks. My red blood cell count, my hematocrit, and my hemoglobin are all low and keep dropping. If my hemoglobin stays low the neurologist said a blood transfusion may be needed. At this point I welcome it as I have been so tired and even weaker than normal lately and it is supposed to really give you a boost.

So that is about it, I know it is alot of information but it was a productive appointment. I continue to have to use the walker daily and my vision is constantly affected by this beast of a disease. On good days I have no depth perception, on bad days I have double vision. They say this is the one symptom that tends to not go away with medication. I was lucky in the beginnning but I think the myasthenia is winning the battle in that regard. I will continue to do my physical therapy which consists of trying to strengthen my diaphragm, wrists, and ankles. The recent flare up set me back quite a bit in regards to overall weakness but I am hopeful that I will start to see some improvement soon.

Ta Ta for now and happy Friday!
Jen

Another hospital stay, urgh!

Well, I managed to stay out of Club Med for 9 whole months. I had gotten pretty used to dealing with my illness at home with my weekly IVIG. Sure I have had daily symptoms of weakness and vision issues that are a bother but I was getting used to managing my new life. Unfortunately the beast had other plans and a myasthenic crisis decended upon me on Monday.

For those of you who don't know, a Myasthenic Crisis is when the illness affects the muscles involved in swallowing and breathing. I have had three of these since my illness began, scary stuff when you can't communicate to the doctors what is going on but I will start at the beginning.

As you know, in September I had a port put in for my weekly infusions. It has worked wonders in not having to get jabbed multiple times trying to find a vein that will work with the IV but the incision never stopped itching and has been red the entire time. Not hot or oozing, just red. Last week my IVIG nurse said I should really let the surgeon know about the itching and redness so Monday morning I went in and showed the nurse what it looked/felt like. She spoke with the doctor and they said I should take Claritin for 7 days and call if it got worse or if after 7 days it didn't improve.

40 minutes after taking the Claritin, which I have never taken before, I couldn't open my eyes, lift my arms, or speak/breathe very well. My husband put a mestinon under my tongue and the weakness slightly subsided. An hour and a half later I was in dire straits, any attempt at breathing resulted in an awful sound and I was like a wet noodle. My hubby put me on my walker and wheeled me out to the car and rushed me to the ER. He explained what was going on and they rushed me back and gave me IV prednisone and ordered a NIF test (this tests the strength of the diaphragm muscle). The prednisone helped quite a bit and my NIF score was 20. Anything under 20 and they ventilate you and put you in the ICU which I never wish to experience so I was teetering. They admitted me to the hospital and ordered three days of IVIG and observation.

By day two my NIF was up to 25 and day three it was up to 30, then it went back down to 22, and then back up to 25. I was able to convince them to release me and let me have the 3rd day of treatment at home. I missed my kids and husband and my own bed and sleeping without people waking me up all the time blah, blah, blah.

So now I am resting and recovering. The best guess is that I was having an allergic reaction to the stitches under the skin and that the Claritin didn't agree with the MG and that pushed me into a crisis. So, no more claritin for me, I can deal with the itching until the stitches dissolve though I have no idea how long that will take:)

A few notes for those of you who haven't been admitted for a crisis/exacerbation. Make sure you have a binder/folder with a list of all of your medications, your neurologist's contact information, any test results from prior issues, any allergies or sensitivities, and a durable medical power of attorney so if you get ventilated you have someone who can speak for you. I also recommend requesting that you are able to self medicate your mestinon. I take mine every two hours and if it is late it is bad news, for the nurses about 80% of medications can wait 15 - 30 minutes without making the patient very sick but that isn't the case with myasthenics and mestinon so if you are strong enough make sure to insist this. Also pay close attention to the other meds they are giving you. Two of mine were incorrect. One they wanted to give me two extended release tablets of a med that I take 2 times a day and that would have resulted in an overdose. The other was the calcium I take. I take 500mg of calcium 3 times a day and they sent up Tums. While tums has calcium, the antacid part coats the stomach blocking the absorption of my much needed meds.

I hope this helps someone. For now, I rest and recharge. Stay strong.
Jen

Report on the Port

Hi all my faithful readers and new friends! I hope today finds you well. I am trying to soak up every last bit of sun before it is gone. I love this weather!!! High 60's to low 70's, the brightest/bluest sky imaginable, bright white puffy clouds...ahhhh. Not too hot, not too cold, and the perfect dose of natural vitamin D.

I wanted to report on the port for those of you considering getting one. Mine has healed up nicely. The treatments are now about an hour shorter because the meds run smooth and steady and there is only one poke. It is perfect and I love it. I always thought ports went out of the skin but I was wrong, it is completely under the skin so no maintenence on my end either...sweet!!!

In other news, I have a confession to make. I cannot sew! I know, what kind of mother am I? I have, however had a sewing machine in my possesion for the last 3 years, it is the old Kenmore type that is built into a table, so I have been using it as a table and the dust on the machine is crazy! But, my IVIG nurse said, hey we are together for 6 hours anyway, want to learn to sew...would I? Yeay!!! I now have a new hobby, and homework:)

That is about it for an update, I hope you all find yourselves well and happy and strong!
Jen

Med changes, job changes, surgery, oh my...

So you were probably all thinking...hmm, Jen has been quiet, she must be feeling great! Well, I was, and then bam, I found out that I was losing my job. The job that I have loved for 3 years, in the field I have specialized in for over 13 years. As you can imagine my stress level went through the roof and my MG did not react well at all. Things will be okay and everything happens for a reason so I will not go into detail here other than to say I am at peace with it and I am reassessing what this means for the future. And on to med changes...

I saw my neuro in late August and he said that I am really not improving at a rate that he would like to see. That was actually good news for me because it means there is hope for more days without symptoms. My average day lately has me with double vision about 50 percent of the time, needing the walker for at least a few hours, and periods of swallowing and speech difficulty. I have also had fatigue greater than I can put to words and pain in my back and neck that does not easily relieve itself. I stretch, I walk, I do deep breathing, I take meds, I am still in pain.

So for the med changes, I am now on double the dose of IVIG every week. Instead of a 4 hour treatment (which I loved because it flew by) I now have a 6 hour treatment every week. Also, my veins just can't take the constant IV's so on Monday I had surgery to have a port placed. The surgery went well but the anesthesia caused a pretty severe MG flare for the next couple of days. Today I feel like I am back to my "normal" weakness level so that is a good thing. My doctor also upped my Cyclosporine dose back to 100mg a day and the stomach issues are back with a vengence..yuck! He said if that continues then we will switch to a different long term immunosuppressant or try Rituximab.

Say some prayers for my poor battered skin. It turns out the more you are exposed to chemicals the greater the chance for an allergic reaction. I am allergic to tape, tegaderm, opsite, and band aids. My skin actually blistered and tore off with the tape removal and I look like a burn victim in spots...not fun on top of everything else.

Other than that, and I know it is alot, I am hanging in there, keeping my spirits up. The family is doing great. School is in full swing so we have been busy with homework and what not. I hope all of my readers are well and enjoying the fresh fall air.

Before the blog...my journey to diagnosis

So, since I have been horribly inactive I thought I would share the full, unadulterated story of what I went through on my journey to diagnosis. Warning - This Post Is Looong! I have to confess, life has been so busy and so crazy lately that I am taking this from an email I communicated to a newly diagnosed contact but it dawned on me that I didn't start my blog until after my January hospital stay so I would post it here...this is my story...

I was delivered the news that I have bicuspid aortic valve disease unexpectedly last June when I was hospitalized for what appeared to be a stroke. While performing all of the tests to see why the left side of my body was not working and the right side of my face was extremely weak (drooping eyelids, slurred speech, drooping mouth) they performed a bunch of scans/CT/MRI/blood tests and... a chest echo. I was discharged from the hospital with a tentative diagnosis of either anxiety due to stress (though I revealed that life was going quite well aside from the fact that I was hospitalized on my 10 year wedding anniversary) or complex migraines (which was funny to me since I have suffered migraines since I was 16 years old and this “stroke” event did not have the vision auras or any head pain…at all that always accompany a migraine).


Two days after my 3 day hospital stay I saw my GP for follow up and found out that they discharged me without even reviewing the echo. To my surprise, out of all the tests run, this was the only one that was abnormal. It showed a Bi-Cuspid Aortic Valve with insufficiency, mitral valve insufficiency, and tricuspid valve insufficiency with evidence of mild pulmonary hypertension. So, off to the cardiologist I went. The cardiologist performed a TEE which confirmed the BAD with
insufficiency but showed only mild mitral and tricuspid insufficiency and no pulmonary hypertension. He said, you are fine and your heart is unrelated to the stroke like event. I had done some research and had many of the symptoms of pulmonary hypertension: fatigue, shortness of breath, and a feeling of pressure on my chest when I did the slightest of activity. In addition to that I had extreme residual weakness of my eyes and my arms/hands and I was determined to get to the bottom of it considering the fact that I have two young children and I am the primary breadwinner while my husband is back in school. I called a friend of mine who is an RN and she said there was a local Pulmonary Hypertension clinic and gave me the number. Two days later I was in the office of the pulmonologist who saved my life, though neither one of us knew it at the time.


My pulmonologist performed a six minute walk test and after 2 minutes my oxygen SATS dropped to 80, they put me on supplemental oxygen for the remainder of the test and they didn’t go above 88. After the test was finished and I was sitting for 5 minutes they shot up to 100%. He took off the oxygen and they stayed at 100% until he had me get up and walk again, then they fell right back down again. So, he said, there is definitely something wrong with you and I promise to get to the bottom of it. I was prescribed oxygen to use anytime I wasn’t sitting in a chair or laying down, but also when sleeping. Then he one by one ordered the million dollar work up. I had a Chest X-Ray, a Chest CT with contrast, a nuclear pulmonary V/Q scan, a whole body V/Q scan, and a right heart cath. All came back normal so thankfully I did not have pulmonary hypertension. Unfortunately we had no answers except
for the fact that I had an oxygenation problem, weakness, and a Bicuspid Aortic Valve. I also found out that my aortic root had enlarged since a prior CT was performed in 2007, but it certainly wasn't causing these problems. After researching this I found out that people with Bicuspid Aortic Valves are born with connective tissue issues that make them more prone to Aortic Aneurysms, like I needed one more thing to deal with!


In the midst of all these tests I went back to the Neuro who saw me at the hospital, after an EEG he decided it must be complex migraines and wanted to put me on a beta blocker. My old doctor tried that on me and it landed me unconscious in the ER with a blood pressure of 70/50. So, you can imagine my dismay at his recommendation. I said, I am happy to try your theory if you would be willing to run some additional tests first. He was so offended that I would not try the beta blockers and that I challenged his diagnosis that he dropped me as a patient. I walked to my GP’s office in tears (they work in the same building) he told me the guy was a pompous “fill in the blank” and not to worry about him, we would figure this out together. Apparently the neuro I saw went to Harvard and thought he was a big
fish in a little pond and had quite the reputation for upsetting or blowing off patients...just my luck.


The next week I was scheduled for a pulmonary function test which was ordered by my pulmonologist. Note this is the least invasive and least expensive all the tests ordered. At any rate, I was alarmed when my pulmonologist called a few nights later to inform me that the test showed diaphragm weakness due to a neuromuscular disorder. He referred me to a different neurology office and said we may be on the
right path. I went to the appointment and after performing the exam the doctor took a step back and said “I think you have Myasthenia Gravis” this was due to the fatigable generalized weakness and the ptosis from the eye strength test. She called in four residents to show them what it looked like and explained that while there is no cure there is treatment, I said sign me up! However, she wanted to
confirm with the blood test. When the blood test came back negative she was very aloof and said, well I don’t know what it is, maybe vasculits or something. When I asked if I could try a trial dose of Mestinon or Prednisone she said no, there is nothing more I can do for you. I later learned, when gathering my medical files that she suspected anorexia. I went from 150 pounds to 125 pounds and shrinking in two weeks and by the time I saw her (almost 2 months after my hospital stay) I was 115 due to the fact that it was extremely difficult for me to swallow! Note that for a woman who is 5’6” 115 is not considered underweight though the rapid rate at which I was wasting away sure scared the heck out of me. Okay, so that was late August.


I spoke with my GP and he referred me to the University of Michigan. He personally called the head neurologist to discuss my case and by early October I was in Ann Arbor and hopeful for some answers. After going through my 3rd neurological exam, my second EMG, my first single fiber EMG, and more blood tests, the head of Neurology diagnosed me with “suspected sero-negative generalized Myasthenia Gravis.” The only thing he was scratching his head at was the rapid rate at which
my oxygen fell with any level of activity but he did start me on
Mestinon (this was later confirmed to definitely be due to the MG but more on that later). My dad had taken me to that appointment and I was so anxious for any type of relief that I made him drive straight to the nearest pharmacy in Ann Arbor. I took my first mestinon and 30 minutes later I did not sound like I had marbles in my mouth and I could read the highway signs. I said, “dad, this is it, they finally
figured me out!” I was ecstatic!


The prescription called for me to take 30mg of Mestinon every 4 hours and titrate up until I had side effects…i.e.I ran to the bathroom with what was second only to the urgency of a colonoscopy prep. That dosage was 60mg every 4 hours and so my journey began. A few weeks later I called U of M because while the Mestinon helped me during the day, after I slept I could not move my arm to turn off the alarm clock
and was so weak that a few times I fell after getting out of bed. I was also starting to feel kind of dizzy. The neurologist prescribed Mestinon Timespan for bedtime so the Mestinon wouldn’t wear off while I slept. It worked like a charm for my morning strength but the dizziness was still there, I felt like I had just stepped off a boat, or had a few too many cocktails.


Four weeks after my tentative diagnosis and med trial I had a follow up scheduled with my pulmonologist and new cardiologist. To my amazement I passed the 6 minute walk test without oxygen…bye, bye oxygen tank! I literally happy danced myself out of the office (albeit weak and out of breath, I didn't care I got to ditch the tank)!They said my diaphragm must have been so fatigued that it caused atelectasis which caused the lack of oxygenation. They said it was a pretty good confirmation of MG. So I got through November until Thanksgiving. I don’t know if I caught a bug or was just doing too much given how good I felt compared to how bad it was for 6 months
but I spent the first week of December hospitalized with a crisis. My breathing was severely affected and so were my legs. I was given 5 days of IVIG at the hospital by the pompous neuro previously mentioned who apologized to me a gazillion times and at discharge I was given a walker and In home nursing care for four weeks. At the
end of the nursing care which included physical therapy I was able to walk unassisted for most of the time. One week later I was in Ann Arbor for follow up with my fantastic neuro who confirmed the diagnosis and added prednisone and regular IVIG to my treatment schedule..


Unfortunately, I must have picked up a bug somewhere because a few days later I felt lousy, and I mean lousy. I checked my oxygen sats (I ordered a pulse ox off amazon.com when I was on the oxygen tank to monitor what my readings were) and my pulse kept shooting up and my oxygen kept dropping. I called my neuro right away and he said I was headed for a crisis fast and said I needed to go back to the
hospital. I said I didn’t want to go to the same one I had been at previously so he called the other major hospital in our city and spoke with a very kind, non pompous, 72 year old neurologist who followed his orders to a “T” and offered to assist me with any local needs I may have under the direction of my specialist in Ann Arbor. He was amazing, in my room at 8am every morning to check up on me and chat. I had 3 days of IVIg and started on bi-weekly in home treatments after
that.

The rest of the story is available here...on my blog.

Hugs and blessings,
Jen

Radio Interviews, IVIG, Gardening, Naps, Oh MY...

So I didn't' post as promised. The interview, while fun as all get out, was also quite a bit of an energy sucker...note to self, don't schedule radio interviews 2 days pre-IVIG... Anywho, I made it through the interview and it will now air on Wednesday June 23rd at 10AM on WPRR. The best way to listen is to download it for free on iTunes under The Johnnie Tuitel Show. It will be available a couple of days after the air date. We discussed Myasthenia (by the end of the interview both Johnnie and Angie Kay could pronounce it), we discussed "invisible disibilities", and we discussed funny topics, it should be a great show.

IVIG - Went so well that I think I was only awake for about 30 minutes of it. On the flip side, I am still quite tired and my nose is stuffed so I may be catching a bug. I just have absolutely no oomph, no get up and go, and my eyes are having less and less normal periods which is really having an effect on driving.

Gardening - Okay so I have a self proclamed "black thumb" however, this year, I have been able to keep 2 house plants alive for more than a week (actually going on 6 months), and my flowers outside not only look good but are growing at an exponential rate. I finally figured out how to dead head. So, I may not be a complete and total tool when it comes to gardening...woo hoo.

Naps - need 'em, love 'em, gotta have 'em. It is a necessary evil when you are weak and tired 24/7. I never, NEVER used to nap, now I am napping almost daily. Okay, Update-complete.
Out - Jen

Feelin' groovy..da, da, da, da

I just get so giddy when my IVIG does the trick! I am feeling awesome! Energy..ahhh, strength...ahhh, vision...YES! And, biggest news of all....my baby turns 4 on Wednesday. Cinco De Mayo! Happy Birthday Miss Cindy Sassafrass.

I am feeling blessed beyond words. I am coming up to one year with this mess of a disease and I can say today I am feeling just great. I hope and pray it last through to the next treatment. The weather is certainly helping. Cool enough during the day to keep the doors and windows open and warm enough at night to keep the windows open. The fresh air is, well, refreshing. I just love this time of year. The lilacs bloomed too, I have the fragrance of fresh cut lilacs throughout the house. If anyone tries to say the mind and body are not connected, they are flat out wrong. That is not to say a positive attitude can heal, nor a negative one kill but they certainly have an effect.

I plan to post a new video tomorrow on my Vlog on a good day, if all goes well, to show just how normal I am when I don't have symptoms. Here's to looking at the bright side of life. Thank you for all your prayers and support.

Much love,
Jen

Two days downhill, on the upswing again

Had a little setback on Friday. See, when you are chronically ill and exposed to medical supplies over and over and over again, you can develop an allergy. This happened to me this summer with the IV tape called Tagaderm. It is the clear thin plastic square tape that goes over the IV tubing. My skin reaction starts with my arm turning a red white and purple marbled appearance and then developing tiny red bumps that end up spreading to my chest abdomen and thighs. Benedryl doesn't touch it, only high dose prednisone, which we learned makes me weaker. Low prednisone makes me stronger, too difficult to get into right now. At any rate here is what happened...

Cynthia arrived at 9am, ready to roll, she did another stand up job of painlessly getting the IV in and I was READY. I was really falling back into the MG abyss and wanting to reap the rewards of the IVIG. She was able to push me up to a higher flow rate so we were finished by 2pm which was wonderful as well. Gave me a few hours rest before my toddler was home from the school carnival.

I'm getting off track, back to the reaction. For some fun reason, even though I am allergic to the Tegaderm, it is included in every IV starter pack. The pharmacy had mailed the tape I'm not allergic to but it was in a separate bag. At about 12 noon I went to the bathroom and my whole arm was marbled. Cynthia removed the bad tape and replaced it but it was too late. So then I had to have a mega dose of Benedryl. Between the benedryl and the allergic reaction (which set my immune system off, which increases the muscle receptor attacks) I ended up super duper weak and fatigued. Thank you Pat for being a single dad for 28 hours. That is about how long I slept give or take a few times to wake up and use the restroom, take my meds, or eat. I'm feeling much better and I am certain tomorrow will be a good day.

Will post more later. Have a Blessed Sunday!

Home Renovation -

Well, not a renovation per se but there are changes coming today. I am sitting in my recliner waiting for UPS to show up with my hospital equipment for treatment. I spoke with the pharmacist from the in home IVIG provider yesterday and found out that I will be receiving the following:

An IV Pole
IV tubing and needles
A sharps container
IV Benedryl
An Epi Pen
Tape that I hope I am not allergic to
My medicine
A laundry list of things I don't remember

This is all a bit overwhelming for me but it is the way my doctor and I wanted it so I'll just have to roll with the punches. I had to laugh on the phone yesterday when the pharmacist told me how to handle the medicine. For those of you who do not know, IVIG is a human blood product. Basically, they filter out healthy immune cells from donors to put in my system and override my messed up immune cells that are attacking my muscles. Anyway, the pharmacist said to refrigerate it until 12 hours prior to infusion and then let it warm in a safe room temperature setting. She said do not microwave, put it under warm water, boil it in a pan, or (and here is where I laughed out loud) put it in a diaper warmer. I said "what???" She laughed too and said these were all things other patients had done and that it would destroy the medicine...oh, it never ceases to amaze me.

It reminded me of a story my aunt shared with me. She is a labor and delivery nurse and she used to volunteer at the free clinic once a week. There was a teen girl who had gotten pregnant and was surprised because she said she was using jelly. My aunt asked what type and she said Smuckers grape flavor. I don't claim to be the smartest sandwich in the basket but these stories always give me a good laugh.

In addition to my new treatment plan, I am attempting to go back to work on Monday. This was supposed to happen three weeks ago but my unexpected hospital stay put a cramp in my plans. I am praying for strength and stamina as our family needs my income and I need to get out of the house for my sanity. I am going to ease back into it but it didn't go so well the last two times. I am a bit more optimistic now that I have a solid medication protocol, we'll see...