Day 78. Wrinkles in Time

Time has had a tendency to wrinkle in on itself more than usual, lately.

That is the short version of my excuse for letting the weeds grow up around my blog for eight or more weeks. It also explains the wrinkle in the number of days. My reasons for taking so long to write again are legion; some are good and some not good at all. It got to the point where I skipped so many days, I could not stand to see the minuscule number I had left. What a relief it was, then, to discover a mathematics error tonight (a relief, but not a surprise). So, the sharp among you will note that time appears to have folded in upon itself and I have more days to live than it appeared….and yet it still is slipping horribly, frighteningly below one-hundred, and toward zero.

So, here I am writing again, like it or not. I haven’t written regularly for several months now for a hundred reasons. I’ll limit my excuses to four big ones here:

  1. Time slipping, slipping, slipping into the future.  When I last wrote, I returned to the blog after a long time to accept the nice blogging awards for which Michele Berger nominated me. Something about the difference between Day 121 (three digits) and Day 81 (two digits)—verged on paralyzing me. One main impetus for my starting this blog was to prove Dr. Mean, the surgeon who predicted I wouldn’t outlive my 48th birthday, wrong, to show him the diametric opposite, that I am living fantastically well. However, those numbers humbled me. Put yourself in my place, reader.  I know I am in the business of proving that idiot doctor wrong. But what if, just for a moment, he is right? Eighty-one days are not many; that isn’t even a quarter in the finance sector. I am allowed these occasional tidal waves of fear.
  2. Narrative Strategies Workshop. I was using what I learned in the workshop I wrote about (Day 121) helped me to decide that I’m not going to let being sick keep me down. So I won’t fall apart about this 81 day thing. Hell with that!  However, focusing on being a well person takes a lot of energy.  I have been really trying to do that.  I have a well mind, but I am frustrated that almost every day, my body is (so) sick.
  3. Mindfulness-Based Stress Reduction (MBSR) Meditation. I started a Mindfulness-Based Stress Reduction (MBSR) Meditation course, one that I found just as helpful as the Narrative Strategies course, though it took quite a different approach. I couldn’t understand why I was so incredibly busy.  Yet, it took my observant friend Ellen to point out how much time MBSR took. We attended a two-hour course weekly, plus read several (lengthy) textbook chapters and a handbook chapter every week; on top of that, we meditated for 30 mins to 1 hour daily. What might have been a relatively insignificant after-work investment of time for the average person for me involved every extra moment of my time, every day of the week. The ribbon of time looped in and tangled on itself. Sometimes I would find myself back at the beginning of what I had done, not having started in the least because I had not done it correctly at all (or had slept through the exercise, more often than not). Meditation is tricky like that. Still, it did—and does—help, not as much with my pain as with my stress about being ill.
  4. Suffocation by pillow, parts A & B.  

               A.    Suffocation by pillow: Pulmonary Emboli. It’s always emotional when I go to have my CT angiogram at John’s Hopkins University Medical Center, which is supposedly the mecca of treatment for people with Ehlers Danlos Center. This test examines and creates images of my arteries. It is important to keep in mind that one ruptured in my left leg once, and some “shifty” things remain there. But the issues of greatest concern are in my abdomen, an aneurysm in my superior mesenteric artery, as well as several on my renal arteries.  This is bad stuff.

When I went to see the doctor a few weeks ago, it felt like opposite day. Ordinarily, the ribbon of time for me is like a regular timeline, but on this day, it meandered like it was traveling on helium, divorced from gravity, and thus from the need to travel forward. Usually, I see the doctor, the Chief of Vascular Surgery, who is so busy I wait for hours to see her. By the time my appointment comes up, she is in a foul mood and if I have questions, she is unkind, belittles me, or tells me I should have asked the questions of my internist (when they are very specialized questions about my disease that are appropriate mainly for a specialist at one of the best hospitals in the world, supposedly, i.e., my internist—understandably—doesn’t know the answer, which is why I am seeing this doctor).

However, on the day in question, I had my scan, came upstairs to her office, and the doctor saw me immediately. I was stunned. I was even more stunned to find her in a good mood, so much so that I couldn’t remember to say any of the things I wanted or needed to.Since I had been called to her office so quickly, the CT angiogram of my body had not rendered yet. Time, as it had been doing that day, slipped off of its normal timeline. So, instead of what we did normally, she said “Let me show you last year’s scans instead.” Speaking very solicitously, she brought out the scans, mentioning that last year she  had only ordered a scan of my legs (meaning they had omitted my abdomen, where the most dangerous aneurysms are!). “Let’s look at your legs. See, you can see just these little clots. Nothing to be worried about at all.”

I saw dark spots there, none very large, but lots of them; I counted at least six before she took the image away. They were dark blotches, like irregular inkblots made by Sharpie markers, in the crotch where a  small artery, it appeared, had branched off. Nothing to worry about, she assured me, and from the tone of the meeting, I felt not just comforted but encouraged. Okay. We were being so positive; it never occurred to me to question why they didn’t scan my abdomen last year!I told her that I was so disappointed not to see the present day’s scan because I had set a goal that the aneurysms would be smaller, if not, the same size. She said I looked great, and that was such an admirable goal; she couldn’t wait to call to me to share in my good news. Then, seemingly attempting an informal girlfriend-y tone that seemed entirely inappropriate, she said things like, “So, what is up with you?” and “How have you been?” It was just out of character. Incidentally, she never even waited for my answers.

The doctor waited until the end of the week to call me with results. Actually, she called me back that very night with the results: The message was, “This is Dr. Deadbeat. I have your records. Please call me back.” Not good.  Had it been good news, she would have left a message.

I called her back many times until Friday afternoon, when she finally got back to me. “Emergencies” had kept her occupied. Coming from a medical family, I take a cynical view of such emergencies because I know that sometimes what constitutes them can mean running to the DMV, UPS, or talking to one’s sister on the phone.

However, she did call back finally and tell me, “I have good news and bad news.” If possible, time stopped doubly here. Why would a doctor say this? I wanted to rip her lungs out and just get to the negative first. “Just say it, just say it, just say it, just say it.” I didn’t speak these words aloud, of course. I said them in my head, hoping some part of her would hear them. Soon enough, she continued, her way.

“The good news is that none of the aneurysms grew. Now, the bad news….Heh-heh!…. Last year when we didn’t take pictures of your abdomen, we probably missed this … “ The first sentence took about 45 minutes, whereas the second sentence took all of five seconds. She went on, though. “We probably missed the pulmonary emboli in your lung. Those are small blood clots that have reached your lung and settled there. They’re probably nothing, but you will probably want to consult a pulmonologist, or see your primary care physician first. She can advise you about how you should proceed.”

I must admit, I was shocked and extremely disappointed. I believed that I had “thought” away my disease and disease processes. So now this diagnosis really trifled with my new narrative of being a well person. And here’s another new disease that could kill me.  could easily be the thing that kills me.  I am humbled. Again.

I am also furious at this doctor. So much of what she did was shady.  She should have alerted me immediately about the clots in my legs. Those could have been treated. The syndrome is that bloodclots in the leg mean a DVT (Deep Venous Thrombosis), particularly for someone like me). Part of that syndrome is that the clots can come loose and lodge in the arteries of the lung and cause all kinds of trouble if they cut off blood supply. That’s called a Pulmonary Embolism.  I got two of those.  So if she had been on top of things, I would not have had two of those.

It makes me wonder about how “nice” she was being; I wonder whether in fact she did see the images and wanted to chat me up just to make me feel like she was her gal pal? Her behavior was so strange that that is the only explanation I can decipher. Someone of her stature has no excuse for overlooking that. People wait for months or years to get to see the doctors at what some people call one of the finest hospitals in the world.  I wonder what kind of care they are getting at just an average hospital.

I know that I have routinely gotten better care at Washington Hospital Center in D.C., at Greater Baltimore Medical Center, and at Virginia Hospital Center.

Anyway, no wonder I have been feeling like I’m being suffocated by a pillow.

  1. 4. Qi Gong & Lungs. Eve Soldinger, my Qi Gong Practitioner diagnosed this weeks before the CT Angiogram.  Several times, she asked me what was wrong with my lung as she stood on my right. “Lungs?” I asked. “That has to be asthma.  Now and again I am short of breath and I have to use a rescue inhaler.  If it gets out of control, I have to use a nebulizer, rarely. “No, that isn’t it,” she said.  That’s how good she is.  These remain small and for the most part asymptomatic (just every 20th breath—when I breathe very deeply—I feel like I have water in my lung, and have for a while).

So what does this all mean??  I don’t know.  I am moving much more slowly (than what? Than 40 days ago) and I am frustrated.  But (on the positive side) I have been writing some short stories, and it may just be that I haven’t had the energy until Carlos & Bob came back to town.  Yes, that probably is it indeed!

366 Days. Speed.

Traveling at the speed of sound

I don’t just have one rare disease.  I have the divine gift of two.  Thank you, Jesus.

I have adrenal insufficiency, a disease that about 1 in every 100,000 people get.  Other people who play with those kinds of odds win the lottery.

The disease occurs when adrenal glands (just above the kidneys) don’t produce enough cortisol. Cortisol performs vital functions of the body, from maintaining adequate blood pressure, to slowing inflammation, to balancing insulin response, to regulating metabolism of protein, carbs, and fats.

We hear a great deal in the media about having too much cortisol in the body, which can cause a large belly and a host of other problems.  The body must maintain a delicate balance of hormones indeed since, while high cortisol levels can be dangerous and lead to cardiac problems, low cortisol levels also can be fatal, and quickly.

Before I knew about my cortisol issues, I could not get out of bed.  I would wake myself by alarm, but it was like awaking from a coma.  I always woke up relatively early in the morning. Even though I don’t have an official job, I work for a very difficult boss:  myself.  I’m the worst task master/mistress of them all.  So I can never call in sick.  I had breakfast and then while I still had the oatmeal bowl in hand, fell asleep, drooling on my chest and dozing for hours, only waking up if something particularly loud happened on TV.  I knew something was wrong, since I had already had nine or ten hours of sleep while normal people slept.

Several doctors had told me nothing was wrong; it was just pain or my medication that was making me tired, or my EDS.  Finally, though, when my leg was infected, my brilliant infectious disease doctor (Dr. William Furlong at Virginia Hospital Center) suggested a test of my cortisol level.  It was 0.2 (normal range is 6-23).  It seems that I was uncomfortably close to Acute Adrenal Crisis, which sends a person into shock; one can die easily in this condition.  For that reason, I wear a medical alert bracelet so that it is clear in an emergency that my body needs a jolt of hydrocortisone (something that a normal body produces in a crisis that mine does not).

I consulted a fantastic endocrinologist this week.  If you need one, I recommend Dr. Jason Wexler, who practices at Washington Hospital Center.  I wanted to ask him about the hydrocortisone I have to take for my condition.  Truthfully, it is speed — with some side effects one would expect, and some unexpected ones as well.  For example, the jitteriness is terrible (shaky hands, etc.); it’s awful after three p.m. when the crash comes.  Predictable as well are mood swings — the higher the dose, the more labile the emotions.  But unlike speed, it makes a person feel hungry, and by hungry, I mean gnaw off the table leg and eat the baby famished.  I gained 19 pounds the first few weeks I took the meds (at a higher dose than I am on now).  I have to stick to a 1000-1100 calorie/day diet so that I do not gain weight.  The most unseemly side effects are that #1, it can cause the skin to be extremely fragile, and #2 it can cause aneurysms to form and rupture.

Well, hell.  That’s just no fair to someone like me.  And since I have taken the medicine, I have had four skin ruptures on my legs.  It makes a girl panic just a bit, then, about the aneurysm situation.

I was worried that I was on the wrong medication (a number of steroids are available to treat my condition.  However, Dr. Wexler did a great job of explaining the way each of the steroids is metabolized, and in this way he convinced me that hydrocortisone is the best of them, since it leaves the body quickly and is least likely to have those skin and blood vessel aftereffects. That’s the best we can hope for, since it is a life-or-death medication.

Another good idea Dr. Wexler had for me had to do with my one-a-day problem.

I can only schedule one activity per day because that is all I can tolerate without being fatigued.  He explained that the dose of medication I am taking is ⅔ the strength he would give for a person of my stature.  So, I could have a few different options.  One would be to add back 5 mg.  (I don’t want to do that because I will almost certainly gain weight.)  The other idea, though, would be to add back ½ of a 5 mg pill in the late afternoon on a day when I do extra things, as a kind of a jolt.  I had thought of trying that before, but I had assumed that it would be a drain on the body and dismissed the idea.

So, I have tried it three times.  The first time I did it, I didn’t have a huge day, but I did run one extra errand.  Having the extra jolt worked, and it didn’t throw me off the next day, when I returned to the lower dose.  Success.  However, when I tried it this Friday I had different results.  I had lunch out, and then had dinner out, a very big day.  Not only that, but going to dinner with friends was a long haul on its own merit.  We went shopping at the Yes! Organic Market right next door to the restaurant after eating, which was fun.  I ended up buying two bagfuls of stuff, and then I had to carry them a block away to the car — way too much for me.  I was in terrible shape by the time I got to the car.

I was sick all night.  I think it was a combined misery of: too much in one day, too much walking, too many heavy things to carry, perhaps some food that I was allergic to.  Those variables together whipped up into a heapin’ helpin’ of pain central.  I was up all night moaning and groaning and wishing I was dead, a big shame, since I was getting up this morning to go to lunch with my angelic Mom and Nathan and Greg and the boys in Fredericksburg.  Oy.

So speed can only speed you up.  Nothing can speed you up fast enough to zip past the  pain.