That all changed during my CCSVI angioplasty this past March. After the procedure, I had what seemed to be a dysautonomia reaction when the sheath was being removed. That freaked me out a bit and made me focus on the EDS side of things again. Thankfully Dr. Sclafani had an EDS doctor to recommend. I called her and four months later got to see her.

I highly recommend Dr. Francomano in Towson, MD. That was possibly the best doctor’s appointment I’ve ever had, and I’ve had a lot of them. She had reviewed the 30+ page questionnaire I completed and the test results she requested. She did a thorough examination. She noted all of the things I expected her to find and plenty more. I learned that my knees go 20 degrees past straight, that the whites of my eyes are quite grey, that my shoulders have hypermobility, that my feet are flat (but only when I stand), that my pinkies extend past 90 degrees, many of my reflexes are 3+, and several other things. I learned that my crumpled ears are an EDS thing, too. She respectfully disagreed with her colleague at Penn and confirmed what I already knew; I have EDS.

Next I need to learn more about how to manage my health. To ensure she puts me on the right path, she ordered several tests. I need to do a Tilt Table study to figure out my blood pressure drop problems and the dysautonomia. I need to have an upright MRI to check my disks in the cervical spine while looking straight ahead and then looking down to see if there’s any disc damage that would explain the hyper reflexes. I need to do a sleep study to figure out if I’m getting enough REM sleep. Lack of REM sleep could explain my chronic fatigue and insomnia issues. I’m also going to get another echocardiogram to serve as a baseline. I don’t have the results of the one I had in 2008 and haven’t been able to track down the results. Lastly, I’m going to have orthotic inserts made for my shoes. Little Sister has DAFOs (actually she’s graduated to Sure Steps) that extend past her ankles, but the doctor thinks I’ll be fine with just a shoe insert. The hope is to keep my ankles from collapsing. Hopefully all of this won’t cost a small fortune!

One of the most interesting things to me was her interest in the connection between MS, EDS, and CCSVI. It seems that more and more doctors are starting to connect the pieces and looking outside of their small boxes.  Cardiovascular health has long been an area of focus for EDS doctors, but until now they have focused on the heart’s valves and arteries. However, if there are issues here, why couldn’t there be problems with the veins? Veracious veins are a known issue with EDS, so why not explore our other veins? It’s an exciting time to have these diseases. Hopefully doctors will start working together in an interdisciplinary way and begin to connect more dots. I’m so fortunate to have found doctors like Dr. Sclafani and Dr. Francomano who are the kind of people who will do just that.

Lesson learned: never trust a first doctor’s opinion. My MS diagnosis was delayed by 7 years and my EDS diagnosis was delayed almost 3 years. Go with your gut and continue to seek answers!

The folks at the urgent care center were really nice. The nurse took my baseline blood pressure (100/70) and my pulse (88 or something like that).  They check things both sitting and standing.  Then the doctor came in with the first Gilenya pill. Nice guy with a good sense of humor. He asked if I had any heart issues and I warned him of my dysautonomia experience and history of low blood pressure. Then he handed me a pill and said to savor it since it’s worth $125. I swallowed it and was off to my waiting area, a room at the end of the hall with a reclining chair and TV with DVD player, but no cable.  They even gave me an iPad to play with during my stay.

During the first hour, the nurse checked my pressure and pulse (all checks are both sitting and standing) every 15 min. Hour two was every 30 minutes. I went 60 minutes during hour three.  Hours four and five were every 30 minutes again with a final check at the six hour mark. My pulse and blood pressure both dropped, but I didn’t feel dizzy or sick in any way. My pressure dropped to 84/60 (or something in that ballpark) and my pulse went into the 50′s, but I felt fine. I think my body is used to compensating for low blood pressure. For a very long time, my normal was around 90/60. By the sixth hour I was back to 100/64. The doctor called my neurologist to make sure he was okay with how things went. The neuro gave the green light for me to continue on Gilenya. The doctor urged me to stay very hydrated. I needed to drink, a lot!  Unfortunately I learned that my decaf coffee didn’t count, which makes me very sad. The dehydrating chemical in coffee is still in decaf.

I wish I got to stay there longer than six hours! It was the most relaxing and productive day I’ve had in ages. I sewed on about a dozen Girl Scout patches, enjoyed watching The West Wing, my awesome husband brought me lunch from Saladworks, and I got to play with an iPad all day. For the profit Novartis is making on Gilenya, it would have been nice if they threw in a massage, but I shouldn’t complain. It was a great day.

I did the first dose on my own this morning and feel fine. Along with drinking a lot, I was advised to take the pill in the morning. I generally prefer to take medication in the evenings, but blood pressure generally gets lower at night, so that it not the smartest time for me to take Gilenya.

I asked the doctor how many patients he’s seen and it’s been well over 100. I asked if anyone had a reaction to the first dose. He said there was one gentleman that wasn’t honest about having chest pains. This person had a reaction during the first dose and was told to stop taking the medication. Everyone else he’d seen was fine. That was comforting.

So far, I definitely like Gilenya! I’ll try to keep you posted on how things are going.

There was a fridge with blue containers

Stocked with hated Rebif shots

and a woman sick of dealing with…

Evenings wasted each week – three

The need to drink water til the constant need to pee

Heat packs and popping two Aleve

Itchy shot spots nothing could relieve

Shot spots that were also black and blue

Constant fears of waking with the flu

An auto-injector that’s too powerful

A medicine’s whose effectiveness is rather doubtful

Goodbye hate and fear.

Goodbye drinking til my pee is clear.

Goodbye Aleve.

Goodbye time thieve.

Goodbye auto-injector contraptions.

Goodbye injection site reactions.

Goodbye flu-like side effects.

Goodbye mounting skin defects.

Goodbye six full sharps containers that live on top of my fridge.

(Oh wait, I’m stuck with those since no one will take them from me!)

GOODBYE REBIF!

Here I come, Gilenya! Hope you and I get along well!

(Obviously inspired by, “Goodnight Moon”)

It’s time that we part ways. I’m moving on as you don’t fit well in my life.  You are far too high maintenance.  You waste three nights a week and the occasional next morning.  If you were as easy to inject as insulin, maybe I’d keep you around, but you’re not.  Before injecting, I need to make sure to drink at least 2 liters of water, sometimes closer to a gallon.  If my pee isn’t clear, I won’t inject.  I have to take 2 Aleve, warm the injection site with a heat pack, rub on alcohol, wait for that to dry a bit, then finally inject, endure the pain, put heat on again, and ensure to go to sleep within an hour so that you don’t make me sick. For months you gave me the flu.  Yes, it was gone by around 11 the next morning and yes, it’s a rare occurrence now, but I still need to plan for the chance you’ll wreck my sleep and the next morning.  You’ve also abused me.  I am tired of the itchy red spots that turn black and blue.  I bet people think my husband has been abusing me, but no, that’s all you, Rebif.  I’ve had enough.  I can’t even tell if you are doing any good and studies are questioning if you are of any use.  I’ve thankfully had no disabilities (unless you count Optic Neuritis) since my symptoms arrived in 2001.  I took nothing until 2008 (not intentionally, but that’s another story) and can’t say I’ve noticed any improvements since starting injections for MS.  You can take it personally if you want, but it’s not just you.  I dumped Copaxone before you.  You CRAB (Copaxone, Rebif, Avonex, and Betaseron) drugs just aren’t my type.  You’re cramping my quality of life.  It’s time to try something new.  I’m going to give Gilenya a try.  Hopefully the pill will be more my speed.

Take care,

Ivy

Today I think I found out why.  I finally had the ultrasound of my thyroid.  When I had my CCSVI ultrasound, Dr. Sclafani found nodules on my thyroid, so I followed up with an endocrinologist who ordered the dedicated thyroid ultrasound to make sure everything is okay.  After the ultrasound technician was finished she asked me if I knew that I had something going on with my jugulars.  I explained that I had just had angioplasty for CCSVI.  I said to the best of my knowledge, the right jugular is open and doing well.  I explained that the left side has a valve issue.  She confirmed what I thought was the case.  The lower part of the jugular was open and looked normal, but the part higher up was definitely not normal.  She didn’t expand on her comment.  I asked to get a copy of the imagery, so I’m picking that up on Wed.

I sure hope that someone, somewhere develops a stent that is safe for use in jugulars.  It seems like that might be my only hope of having proper blood flow.  It’s pretty disappointing, but I’m not giving up hope.  Dr. Sclafani and other brilliant doctors who are dedicated to helping us are bound to come up with something!

I discovered that my skin could generally tolerate just one type of band-aid, Curad’s Sensitive Skin Bandages.  They became hard to find, so I bought a case of them on Amazon, fearful that I’d be stuck using gauze and paper-tape in the future should they ever stop making them.  I’ve tried other brand’s “sensitive skin” band-aids with zero success.

I can use the Curad bandages on my fingers without problems, but it seems that they aren’t quite good enough for other parts of the body.  I came home from angioplasty with guaze and a clear plastic-like cover over the incision site.  I left that on for a few days and then changed over to my Curad sensitive-skin bandages.  That went well for a couple of days, but then I started seeing irritation.  I could start to see the imprint of the bandage on my skin.  The incision hadn’t healed well enough yet for me to feel comfortable going without some kind of covering, so I left the band-aids on.  Stupid decision.  Here’s the result:

Angioplasty incision, surrounded by reaction to band-aid

You can kind of see the outline of the band-aid, even though it’s been off for almost 24 hours.  That looked worse about a day ago.  The lovely rectangle around the incision is from whatever they use to affix the gauze to the flexible bandage.  That part looked even worse last night.  If you look closely at the bandage, you can see a shiny outline surrounding the gauze.

Curad Bandage: Note the shiny area around the guaze

It is the shiny area that made the raw rectangle around the incision.  Lovely, eh?  Yesterday afternoon I changed back to using gauze, but gave up on that and decided it was time to just give up on bandages.  I’m trying to leave it open today, but when a wound is at the very top of your leg, it’s a hard area to air out when it’s 40-degrees outside and I need to be sitting at my desk all day.

I hope one of these days someone will make a latex-free, sensitive skin band-aid that doesn’t destroy my skin.  (Note that the Curad Sensitive Skin bandages are NOT latex-free.  Perhaps that’s part of the problem, but it really does seem to be the adhesive that caused the problem.  It’s hard to tell.)

~UPDATE~

A couple of days ago I spent my lunch break at CVS in their first-aid area.  I looked at tons of various bandage options and decided to try two things.  I bought a box of latex-free fabric bandages as well as a box of gauze pads and paper tape.

When I got home, I put the latex-free bandage on a random spot on my thigh to see what it would do.  So far, it’s red around the outside of the bandage.  While I still don’t know for sure if I have a latex allergy, my skin definitely has issues with adhesive.

On the angioplasty incision, I put gauze and paper tape.  Not only is paper tape really difficult to keep on given the wound location, but the parts that did stick made my skin red!  Isn’t paper tape supposed to be one of the safest things on skin?  Go figure it would be a problem for me!

My husband came up with an idea.  He covered the offending part of the Curad Sensitive Skin bandage with gauze.  Since it takes a few days for the outer edge of those bandages to mess up my skin, we figured this would at least buy me some more time for the incision to heal.  I just wish the darn thing would develop its own scab already.  I’m pretty sure the Lovenox I’m on is slowing the healing process.  It’s been two weeks and the area is still rather raw.  My fingers are crossed that his solution works.

I first had angioplasty to open my jugular veins this past summer.  Things were great for several months.  The best part was that I had more energy.  The fatigue was basically gone.  Around Christmas I noticed the return of some symptoms.  I was getting really tired again, my arm strength had decreased, the toe cramps were back, my arms were falling asleep again, and the tingling above my upper lip was also back.  I was pretty sure my veins were closing back up.

I was fortunate enough to have been on Dr. Sclafani’s wait list since last year and heard from his office at just the right time.  I drove up to Brooklyn to have my ultrasound and sure enough, my jugulars were refluxing again.  We set up a time for angioplasty.

I must admit that I was more nervous this time.  Unlike last time when I was heavily sedated, I’d be awake through this procedure.  I had talked with Dr. Sclafani after the ultrasound and was very impressed with his knowledge and passion.  I had heard great things about him from people I trust, so I knew I was in good hands.  That helped calm my nerves.  His nurses were also great at keeping me calm.

Dr. Sclafani spent time talking with me and my husband for a while before the procedure.  Although admittedly not a neurologist, he did a basic neuro exam, which was pretty telling.  He was able to see that my right eye drifts and doesn’t exactly follow the way I want it to.  We talked about other symptoms and experiences, including Ehlers-Danlos Syndrome.  He had recently seen another EDS patient, so he was up to speed on the basics, but it’s still new to him.  We talked briefly about something called Dysautonomia and his suspicions that this might be something that affects me.  It was the first time I had ever heard that term.  I figured I’d ask him more about it after the procedure.

The procedure itself went pretty well.  I even got to pick the music we’d listen to.  In a way, I was glad to be awake.  It was good to be able to talk with him and to be part of the decision making process.   Not to scare anyone off from doing this, but there was some pain involved during the ballooning, but nothing worse than labor pains and Lamaze breathing came in handy.  My right jugular was still open from last time, which was good to hear.  The valve was a little off, but not enough to be a concern.  The left jugular was a problem once again.  He was able to get into it without the problems the first doctor had, but he wasn’t able to get the balloon to fully inflate.  We talked about some options.  One would be using a larger balloon and seeing if inflating it more would work, but he was worried the vein couldn’t handle it.  We also talked about doing a stent.  Stents worry me.  I don’t think they have made ones that are right for veins yet, so I passed on that option.  We decided to leave things as good as he could get them with the conservative approach, which was still more open than the vein was, and see what happens in three months.  I’m pretty sure I’ll be back on the table for the left jugular!  After that he checked for May-Thurner, but didn’t find any sign of issues with the iliac vein.  Then it was on to the azygos.  Last time, the doctor didn’t see anything wrong with the azygos.  Thankfully Dr. Sclafani has started using internal ultrasound to really see what is going on.  From the outside, the azygos looked wide open, but from the inside he could see that it was much smaller.  He ballooned the azygos in two spots.

Recovery was much different this time, too.  My husband was allowed to come back right away and a nurse was with me most of the time.  I still haven’t mastered drinking while laying down, but did figure out that if I turned my head to the side, juice wouldn’t go up my nose.  After resting a while, it was time to take out the sheath, the tube they use to thread everything in through.  That’s where things took a turn for the worse.  He pulled off the adhesive and probably underestimated how stretchy my skin is because it didn’t pull off as easily as it does on other people.  The “just rip off the band-aid” approach doesn’t work on me.  I need to remind docs to hold down my skin as they go.  After that, he started to pull out the sheath.  I instantly got nauseous.  My pulse and blood pressure plummeted.  I started to sweat.  It was awful!  He got it out and as he was holding pressure on the insertion area, my body started to cooperate again.  I almost went into shock.  Not good.  We started talking about Dysautonomia again.  It does seem like something I need to learn more about.  Apparently my body doesn’t respond as it should to things like adrenaline.  Most people feel pain and their pulse increases.  The “fight or flight” instinct kicks in.  Not for me.  My body shuts down.  It’s kind of scary.

Dr. Sclafani’s instructions were to go back to the hotel and relax and to order in dinner, so we enjoyed pizza in bed.  While lounging, I decided to do some research about Dysautonomia.  Dysautonomia is where the automatic nervous system malfunctions.  There’s a whole host of things associated with it and people with Ehlers-Danlos Syndrome seem to have this more often than others.  I haven’t yet learned enough about it, but decided it is time to finally find an EDS doctor.  I haven’t located anyone in the Philadelphia area, so it’s time to widen my radius.  I have heard some folks mention someone down at Johns Hopkins.  That wouldn’t be a bad drive.  I’ve been focused so much on the MS, that I’ve been ignoring the EDS, thinking it wasn’t a big deal.  Then a thing like almost going into shock from something pretty simple happen and it makes me rethink things.  I feel like I have a lot of puzzle pieces scattered on a table and I can’t seem to form a complete picture.  There are pieces that are clearly MS, some that are clearly EDS, and then a bunch of others that seem to be both or neither.  I wish there was a doctor out there that could put the whole thing together for me and to help me make sense of things.

I can’t figure out what it is about fall that brings about a decline in my health.  What is it about this time of year that throws my body for a loop?