375 Days. What is VEDS?

In 2008, after being sick for a several years with an illness nobody could explain, I got an unpleasant surprise: an aneurysm ruptured in my leg, causing a lengthy recovery and permanent nerve damage and severe pain, the kind that awakens me from sleep screaming sometimes or collapses me on the floor, holding the leg.  The result, however, was that I was finally diagnosed with a genetic disease, Ehlers Danlos Syndrome, Vascular type (VEDS), a rare disease (about 250, 000 people total are thought to have it).  At least I knew why I was sick.

EDS causes a defect in the body’s formation of collagen that can cause hypermobility in joints (being able to bend the thumb down to touch the bone under the wrist, for example, and all varieties of unusual double-jointedness, depending on the way the disease affects the individual), and constant pain.  But more seriously in VEDS, that malformation also occurs in blood vessels, which frequently causes them to balloon, forming aneurysms, and dissect, which is to tear.

Existing treatments for aneurysms are advanced, requiring no traditional surgery. Stents can be threaded through a small incision in the groin and brought (using a micro-camera) to the site and used to replace the vessel that is damaged. This is remarkable, and the best treatment available, since no specialized medication is available for VEDS. Avoiding surgery is critical, since doing surgery on someone with VEDS is comparable to trying to sew on wet toilet paper.

Unfortunately for me, though, according to my doctor, my aneurysms are in places where stents would not be effective.  Several of my aneurysms are on the renal arteries (near my kidneys), and the most dangerous one is on my superior mesenteric artery (SMA) (below the aorta).  This is where arteries are huge.  For comparison, the aorta is the size of a garden hose.  Making the tiniest surgical mistake on the SMA would be immediately fatal; the surgeon explained that in the case of fixing the aneurysm surgically, the risk outweighs the benefit.  If it ruptures, I will die immediately, before anyone possibly could fix it.  Thus, my predicted shortened lifespan.

Here’s the good news:  unlike many people my age, I have low blood pressure, which means less pressure on the aneurysm to grow.  Having consulted with a nutritionist, I am a vegan (gluten-free as well) and I eat a reasonable amount of protein and take many (excellent quality) vitamins and nutritional supplements for maintaining my health (and I’m happy with the brand I’m using). Not eating garbage, especially meat with hormones of all kinds injected, is very important.  I believe taking these important steps will lengthen my life beyond that doctor’s flip prediction.

What do you think?  Do you agree that my diet may have an impact on my lifespan?  How about my use of nutritional supplements?  Do you use them?

375 days.  Tick, tick, tick.

376 days. The clock is ticking.

What would you do if you found out your days were numbered?

Would you quit your job and travel the world?  Could you?

Would you speak more kindly to your family?

Would you finally learn to paint?

Would you write that book you have been meaning to write?

I have had to consider these questions deeply (and on the surface) for the past three years.

Almost exactly three years ago to the day, when I was 44, a doctor I had never met walked into the examining room where I sat, greeted me briefly and said (in the voice of Bill Lumberg from the movie Office Space), “Uh, yeah.  I’ve seen the pictures you brought in, the MRA, and I just want to be up front about this.  An aneurysm this size, on a woman your age.  Look, you probably can’t expect to live much past 48.”  In tears, for the most part, I missed the rest of his callous speech. He is the expert in the field, having literally written the textbook on how to do surgery on people with my disease; so it was hard for me to dispute his opinion. Then, to add insult to injury, he told me that because I was crying so much, I was probably depressed.

Wouldn’t you be?

He did do me a great service that day by making me very angry.  I intend to prove him wrong.

So, knowing all these things, have I changed my life considerably?  Well, yes and no.  Even though I had just completed my Ph.D. and was optimistic about my job as a college professor at Northern Virginia Community College, I had to stop working.  That was devastating.

I have gotten much closer to my family, and I try to remember to really look at them (I guess to memorize their faces for eternity) and understand them a little bit better than I ever have before.

My friends are a different story.  It’s hard to keep in close contact with friends when you’re sick all the time, and you never know whether you will have to cancel plans because you just don’t feel well.  But I’m making a real effort to socialize on different terms.  I’ll talk more about that in an upcoming post.

Heidi's Watercolor 8-19-11

After going through a period of real angst, though, I realized that I had a great deal of time on my hands to do what I had always wanted to do:  write.  Writing is very hard for me since I had my aneurysm.  It takes a very long time.  I had to go from writing a sentence a day to a paragraph a day to a page or two a day (I can’t do much more than that without becoming exhausted).   But I don’t love it any less.  I also have always wanted to learn to paint.  So I took an online watercolor class with my friend John, and I found that I love to paint.  Now I have a big box of paints and a number of books on the subject.  I’ve framed some paintingsand given them as gifts (which means the poor recipients are stuck with them and have to hang them up).

So this is what I’m doing so far, 376 days and counting.  I want to prove Dr. Mean wrong, but just in case….some days, as I try to finish my book, and I don’t feel well or don’t feel like writing, I tell myself, “Hurry, hurry…no time to spare.”