This is a post that is not a particularly cheery one, so be forewarned. Part of the purpose of this blog is to document the ins and outs of Marfan, and I feel that needs complete honesty.

I’ve spent the better part of 2 of the past 4 days in the Emergency Room. Saturday morning I woke with a migraine that turned into the worst headache of my entire life. Concerned it might be a brain bleed (even the illogical becomes logical with Marfan), Mark and I dropped Menininho off with friends and headed to the ER. Several hours later we learned I’d had my first cluster headache.

The good news? According to Wikipedia, cluster headaches “may be the most severe pain known to medical science.” That means I was TOTALLY justified when I called Mom on the way to the hospital and said something to the effect of “Mommy, I’m probably dying so I just wanted to tell you I love you and Mark’s taking me to the hospital.” The bad news? They’re kinda chronic. And when I called my Marfan cardiologist, he told me I was his first Marfan patient to have them (and he has hundreds of Marfan patients). Cluster headaches aren’t directly related to Marfan, but they’re related to migraines, which people with Marfan tend to get at a higher rate than the general population.

So, knowing that I’d be going to get more cluster headaches over the next few weeks, I was anxious to get ahold of some medication. My cardiologist approved a particular drug for me that we’ll call Q. My general practitioner (GP) called in the prescription for me last night, just in time for me to get the beginnings of another cluster headache. The medication worked like a dream, except for one thing. After about a half hour, my neck began to tighten. I got dizzy and couldn’t hold my head up. I was terrified.

Mark called the hospital and, to make a long story short, it turns out I was never supposed to be taking the medication! However, neither my doctor nor the nursing staff had bothered to make me aware of the late-in-the-day change of plans.

After a fitful night of sleep, I called my doctor’s office and complained. Because the pain woke me up 2 hours early, I had PLENTY of time to summon all my righteous anger and figure out exactly what I was going to say. There was a lot of blame shifting, but I feel like the issue was resolved as well as it’s going to be. I asked when I could expect my neck to start feeling better, and the nurse said she’d call me back.

When she did, she told me I needed to go to the ER right away because my GP was concerned I was having a vertebral artery dissection, which Q has occasionally caused in patients with Marfan. A dissection is a tearing of the artery and in Marfan it’s very rare to get dissections outside of the aorta, the main artery running from the heart. Any of my Marfan readers know what kinds of thoughts run through your mind when your doctor says “dissection.” They’re life threatening. There is a moment of panic (“Is this the Big One?”) and then you go into the Zone and get done what needs to be done.

“Mark!” I called from the bedroom. “Doc thinks one of the arteries in my neck dissected. Time to head to the ER. Call a sitter while I get dressed.” And looking back, we were efficient. In a matter of minutes we’d found a sitter for the baby, gotten all of us dressed, emailed my cardiologist to let him know what was going on, packed Menininho’s diaper bag, and rushed out the door.

Despite the nurse’s assurances that I would get in for a CT quickly, I found the ER to be mostly a waiting game. It took almost an hour for a doctor to see me (who told us he didn’t think I was having a dissection), and then another hour for him to page my cardiologist. It was decided I probably wouldn’t need a CT because I wasn’t actually having symptoms of an arterial dissection. My GP was totally wrong. But, it took another 3 hours of waiting and not being allowed to eat (“just in case”) before I was sent home. And of course my cardiologist came down and advised me that if I’d just called him directly in the first place, I could have bypassed the entire ER “experience.” (Which, by the way, included a neighbor with a gunshot-wound-infected-leg and a hypothermic drunk who was prone to shouting.)

My neck still hurts.

So, I don’t write this as a woe-is-me post. Please don’t take it that way. Most of the time I view Marfan as a blessing. If I had the opportunity for God or a magician or whoever to take it away and make me totally healthy, I’d turn them down. I am happy with the person that Marfan has made me and I am grateful for the opportunities that I’ve had working with this foundation. But Marfan IS a chronic, life threatening illness and some days really do SUCK. And I think it’s important to acknowledge both the good and the bad, and then try to turn the bad into a learning opportunity.

So, to my “Marfriends” and myself:
1) Have a better emergency plan than I did (or just use the NMF’s, which is useful even to those without Marfan).
2) When possible, consider bypassing the other doctors and go straight to your cardiologist whenever there is ANY remotely cardiac issue.

And to anyone who actually read this post to the end, go buy yourself some chocolate; you deserve it!!!