A Reason to Blog

I’ve been trying to come up with a reason to blog for a while now. Unfortunately, I now have a main topic area. Just about 5 months ago I was diagnosed with Ehlers-Danlos Syndrome (EDS) and just last week found out I have Multiple Sclerosis (MS). Apparently I’m hitting the genetic jackpot left and right. My mind has been overwhelmed with the news and perhaps blogging will bring some clarity, allow some place to vent every now and again, and to have a place to track how things are going.

I’m not sure where this will lead or how often I’ll get to post, but here goes nothing….

Ivy

White House Petition on EDS

The other day I stumbled upon a petition regarding Ehlers-Danlos Syndrome on the White House’s petition site.  If you live in the U.S., please sign it to help us raise awareness and recognition.  Thanks!

Gilenya Update

So, now I know why blogs about Gilenya were difficult to find!  There’s really nothing to report or complain about!  I started Gilenya over seven months ago and must say I love it.  I can almost forget that I have MS.  I pop a pill once a day and go on with my life.  I don’t need to set aside three nights a week for shots nor do I have to worry about keeping the next morning open in case of flu-like side effects.  Of course there’s no way to know if the pill is actually doing anything positive, but there are zero negatives to date.  I’ve not had any relapses, the eye doctor has monitored me for signs of macular edema (so far, so good), and my heart is great (had an EKG and echo cardiogram and the ticker is fine!)  My optic neuritis hasn’t improved, not that I expect it ever will, but it’s not gotten worse.  I’m not due for an MRI until this summer, so I won’t know until August about my lesion status.  Definitely cannot complain!

The hardest thing about Gilenya is remembering to take it!  It’s quite easy to forget, especially since it is so simple to take.  During the first two months, I missed a dose each month.  But, that brought me to a Sunday start-day for my pill packs and I really wanted to keep it that way to make things easier, so I came across a fool-proof method to remember to take the medication.  Put it with my dark chocolate stash!  Pick something you do every day or crave every day.  Put your Gilenya pack with it and you’ll never forget to take it!  I have a serious dark chocolate addiction.  I keep small squares of it in a kitchen cabinet.  So, that’s where my Gilenya lives.  It’s right next to the bag of chocolate.  I indulge in a square a day and that’s when I take the pill.  So easy!

I also owe updates on CCSVI and EDS, so I’ll try to get those thoughts together soon.  I feel like I’ve been hibernating from blogging all winter.  It’s time to get back to writing again!

Gilenya – Week One

I’ve been on Gilenya for a week now and must say that I love it. I feel more “normal” than I have in years. My mood has improved, too. It’s fantastic to not be doing injections. Pop a pill each morning and go about my life. No need to prep. No side effects after. It’s just great! My blood pressure is low, but I don’t notice it. If a doctor hadn’t taken it for something else and saw it was 100/60, I’d have no idea. It’s not far off from my normal 100/70, so I’m pretty confident that things are going fine. I did have a headache mid-week that lasted a day, but responded to Motrin. I get occasional headaches that come and go, so I don’t think I’m able to blame that on the new medication. So far Gilenya gets 2 thumbs up!

EDS Confirmed

When Little Sister was diagnosed with Ehlers-Danlos Syndrome (EDS) the summer of 2008 I was very focused on what that meant for her and to a lesser extent me. One of the first things I did for me was try to find an adult doctor so that I would have someone following my progress. However, the doctor I found at the University of Pennsylvania said he disagreed with my daughter’s doctor and said I didn’t have EDS since my skin was not stretchy. This was despite the fact that I bend in ways most adults cannot. I can put my legs over my head, I can bend my thumbs to my arms, and I can hyper-extend most joints on my body.  I have a classic “cigarette paper” scar on my leg. Those were the most obvious thing, but as a doctor he probably should have picked up on more things.  A few months later I received my MS diagnosis and that has occupied most of my time from then forward.

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!

Gilenya – Day One

I had the most relaxing, wonderful day yesterday. Novartis arranged for me to do my first Gilenya dose at a local urgent care center.  I was told to arrive at 8 a.m. and plan to stay for 6 hours.  I could bring snacks, books, DVDs, etc., but couldn’t leave until the 6 hour observation was over. That sounded like a nice get-away to me! I packed season four of The West Wing, some snacks, 2 books, my laptop, and my daughter’s Girl Scout vest with all of the badges I needed to sew and off I went.

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.

Goodbye Rebif

In a modest white house,

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”)

Dear Rebif

Dear Rebif,

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