Sometimes, Murphy Is Wrong

I made it!  The catheterization was Monday.  Considering how much I whined about it, I felt honor bound to report back.  It’s over, and—wonder of wonders!—this one was not traumatic.

In an effort to be helpful many people told me they’d had a cath and it was easy, piece of cake, like rollin’ off a log, no worries, all of which made me want to punch them in the nose!  Sure, I understand that two people can have the exact same experience but perceive it completely differently.  But still, I wondered, how can you live through THAT and say it was OK?  Well, now I understand that the cath I had Monday—the cath my friends had and talked about—that is the kind of cath you’re supposed to have.  The cath I had last August was clearly the cath from Hell.

Dooley, the Polar Bear with Funny Hair

I am so glad I had my arsenal of distractions in place.  Dooley came with us and was nearly as good at comforting as my husband is.  I listened to the relaxation CD in the car on the way and plugged in Mamma Mia! once I was settled in a bed in the waiting area.  The nurse collected some blood and then let my husband come back to wait with me—an unexpected bonus that was very helpful indeed (especially when the procedure was scheduled for 10:30 but didn’t begin until after 1:00).  The nurse let the doctor know I had some questions (and may have given him a heads-up about the “she’s a wreck” part, too), so he came back to talk to me before the procedure and assured me there would be a sedative.

I didn’t plan it this way, but it occurred to me later that when your doctor enters your little curtained enclosure and finds you clutching a teddy bear in one arm and your husband’s hand with the other, he gets a pretty good idea where you are mentally.  I could see the confusion on his face and the nurse’s and could only speculate at what they might be thinking—She’s had a cath already, right?  It’s the “virgins” who are usually beside themselves, what happened that she’s had one and is panic-stricken?

I downplayed this part before, but during the last cath, I was certain the doc was trying to force a foot-long piece of rebar down my vein.  There was no pain, but that’s what it felt like, a lot of pressure and extremely uncomfortable.  I remember feeling a trickle on my neck, and someone said, “Oops, a little liquid there.”  (Yes, I’m pretty sure he said “oops.”  That’s what you want to hear during your first cath ever, right?)  He wiped it away, and when he pulled his hand away, I saw blood on his fingers.  MY blood.  That’s the stuff I discounted because I assumed everyone has those experiences.  Of course it’s hard threading the catheter, of course there’s blood.

But there was more to it than that.  Last year I didn’t know what was going on.  To be fair, I was still half out of it from the procedure I’d had just before, but they didn’t give me anything for the cath itself, so when I was lucid, I was also scared.  It felt strange to be scared but not have symptoms of fear—no butterflies or racing heart.  I asked questions of the nurses and was given dismissive answers.  And then there was the incident I mentioned before, when I opened my eyes and didn’t see anyone around.

This time, however, there was no runaway blood.  While he was threading the catheter, I felt the doctor’s hand lightly on my neck—no pressure at all—and then he said, “Yes, that was a little complicated.”  I don’t know if that’s the way it usually is, or if he was extra careful because he knew how upset I was, but either way, it was much better this time.

The nurses were great, letting me know what they were doing as they were doing it.  When one nurse came along and announced, “I have cold soap for your neck,” another piped up and added, “She doesn’t have a needle—no needles yet.”  I’m OK with needles as long as I don’t have to watch, but it was really thoughtful of her to let me know it wasn’t time yet.

When it came time for the sedative, the nurse let me know it was coming, and about fifteen seconds later, I felt like pudding.  With the best will in the world, I could never get myself that relaxed on my own.  I may have slept through part of the procedure—it seemed to go fast—but I remember most of it, including the discussion about nitric oxide.  I had let the doc know ahead of time that when a mask is placed on my face, my brain gets a message that I’m being smothered, and I panic (and struggle).  We made a deal: he said we could use a cannula if I promised to breathe only through my nose.  We did, and I did, and it worked.

Best of all, they all checked in with me.  They place a drape over your neck and the side of your head (which works well because you’re covered and therefore sterile, but it’s not over your face or in your peripheral vision) and you turn your head sharply to the left.  The nurses put themselves in my line of sight and asked how I was doing, and the doc checked in, too.  The other thing that was helpful is that he let me know when he got the catheter threaded and again when he was nearly done, so I had a feel for where we were in the process and how soon I’d be outta there.

This second cath was a completely different experience than the first.  I felt looked after and protected, and it wasn’t scary.  There aren’t enough words to describe the sense of relief that came over me when it was finished.  A good thing, too, because this procedure is how the docs monitor my condition, so it was not my last one.  Now I know I won’t have nearly as much anxiety next time, whenever that turns out to be (not that I’m volunteering to do it every month or anything).  What a relief!

Retail and Other Therapies

When describing how apprehensive I am about my heart catheterization, I hope I didn’t give the impression that I am rolled into the fetal position, helpless.  “Being a grownup” clearly doesn’t work for me, so this time I’m trying the “asking for help” route.  I mentioned the great tips I got from folks in my online support group.  I’m going to share the wisdom that they and others kindly provided.  Surely I am not the only anxious person around.  Maybe this can help someone else.

Remember my miserable experience with the MRI?  I had called ahead and asked if their machine was an “open MRI,” and was assured it was.  But when I got there, it definitely was not.  When I told the tech administering the MRI that I was claustrophobic, do you know what she told me?  “Close your eyes.”  (She clearly had not yet found her true calling as a sadist.)  I didn’t even know until months later that it is possible to ask for pharmaceutical help in dealing with such procedure-induced stress.

Asking for help in this current “crisis” is paying off in huge dividends, with the first wave of advice overwhelmingly in favor of asking my doctor for sedatives during those days leading up to (and even the morning of) the cath.  My cardiologist questioned me carefully and then prescribed the requested sedative pills.  He told me I’d get a powerful sedative just before the procedure, so I asked for something to help me sleep the two nights before the test (I’d already been losing sleep worrying).  Sedative?  Check!

Another great suggestion came from the mother of another PH patient: to go out and do something fun the day before, not just as a distraction but also as a fun place to which my mind could return during the procedure.  My husband and I went to an intertribal conclave of Native Americans on Saturday.  We bought genuine Navajo art, including a necklace and two pairs of earrings for me.  Can you say “retail therapy”?  We ate buffalo cheeseburgers and fry bread and watched amazing dancers in colorful costumes.  Everyone was very friendly and we had a great time.  On Sunday, we went to church, where I lit a candle of concern for myself and asked my friends for any good vibes they could spare me.  Afterward we went to lunch with good friends.  When I finish here, I’m going to don jammies and watch movies for the rest of the night.  Two days of distraction?  Check!

“In the Beginning” described my struggle with migraine headaches.  During that episode my neurologist suggested a physical therapist to help me control the corporeal ramifications of stress.  The therapist used deep-tissue massage to relax my “board-like” shoulder muscles and taught me relaxation and “body scan” exercises.  While preparing for the cath, I checked with her for further suggestions.  “Focusing on my breathing is not going to be enough!” I told her.  “What do you suggest for big-time stress?”  We chatted for a while and decided on a course of action.  I would—and did—return to my t’ai chi classes to refresh my memory of essential movements.  Then, we decided, if I could not have the distraction of music in the lab, I could focus on t’ai chi movements in my head.  At her suggestion, I also bought a CD to help with daily stress-busting for the days leading up to the cath.  It’s called Relaxation Body Scan and Guided Imagery for Well-Being by Carolyn McManus, and I love it: the world’s most soothing voice, and an unobtrusive harp in the background.  Since the CD arrived, I have played it every night before going to bed; it redirects my thoughts and has helped me sleep.  (Yes, I own this CD; no, I did not get it for free in exchange for this endorsement.)  Refresh memory of t’ai chi?  Check!  Actively pursue relaxation?  Check!

I asked the support group if anyone had been allowed to bring music into the cath lab with them—some were allowed, others were not.  I haven’t heard back about music at my lab but I’m bringing a battery-operated CD player and ear buds just in case.  I’ll play the relaxation CD in the car on the way there.  My choice for the cath itself is the soundtrack to Mamma Mia!, which is about seventy minutes long and unflaggingly cheerful.  Music for distraction?  Check!

It occurs to me a few other things are directly within my control.  For example, I have had other procedures where the nurses chat to each other about their weekend or the boneheaded things their boyfriends do, completely ignoring the draped patient on the table.  I know, they do these procedures all day long, every day; it’s boring, and I don’t begrudge them their banter.  But for me, that just makes a bad situation worse; I feel somehow more vulnerable.  So I do intend to take matters into my own hands: even if I have to tug on a sleeve to be noticed, I will say, “Hi, I’m nervous, would you talk to me, please?”  The nurses and techs I have met are obviously in the right business; they are caring and compassionate (the MRI tech being the only exception, and she worked at another lab), so I have every confidence that I need only make my request known and they will help me.

Last year’s cath was traumatic, partly because they had a hard time getting the catheter down my vein (afterward, the doc called me “Leatherneck”!).  So I’m going to ask if they can possibly use my wrist this time.  At one point during the last cath, I opened my eyes and couldn’t see anyone, didn’t hear anyone talking or even breathing.  I know it was unreasonable—I had a tube in my vein, of course they wouldn’t have left me alone—but it really scared me.  This time I’ll ask ahead of time if someone can stay in my line of sight for reassurance.

The issue of sedation for this cath is uncertain.  One person said I’d be sedated but would be aware and would remember everything.  Another said the sedation is heavy enough that many people sleep through the whole thing.  But I have also heard of patients who were advised they would be sedated, showed up expecting it, and were told they couldn’t have it because it affects the pressure in the lungs (which might be OK for a heart disease cath but not when the pressure is exactly what they need to know).

My arsenal of distractions is in place.  I’ve done my best to ameliorate the stress.  There’s nothing left now except to hope for an uncomplicated procedure—and the blissful oblivion of really good drugs!

Fear

My cath is scheduled for October 26 at 10:30am.  I’m dreading it.

I had an MRI a few years ago, part of the process of getting the migraines treated.  I was really nervous about it but told myself,  So they put you in a big tube, so what?  You’re a grownup, you can handle this. I even drove myself to the radiology lab in Baltimore, more than an hour away.  Bad idea.  It isn’t just that I’m uncomfortable in small spaces; loud noises bother me.  Never mind the amazing diagnostic technology, an MRI is a lot of very loud noise in a very small space, and the combination of the two was overwhelming.  Afterward, I hid in my little dressing room in the medical center for two hours because I could not stop crying.  I don’t mean sup-sups and sniffles, I was crying so hard that I couldn’t drive.  It was two more hours before I stopped trembling.  This is how I learned that little pep talks aren’t so effective for me.  Nothing like firsthand experience.

I already mentioned that my sole experience in the cath lab was not the most fun I’ve ever had (understatement alert!).  I’ve been anxious about this upcoming cath since the moment my cardiologist told me I’d have to have one.  After my appointment, the first thing I did was appeal to one of my online support groups for advice.  The response was overwhelmingly kind and supportive, and the folks there shared a lot of great tips (I’ll summarize those in another post in case it’s helpful for someone else).  A few people, trying to be helpful by putting on psychiatrists’ hats, asked what it is exactly that worries me about the procedure.  They meant well, and I’m not upset with them, but it’s an infuriating question.

Mostly what worries me is that they are going to thread a very narrow TUBE  through my jugular VEIN into my HEART.  Isn’t that cause enough for concern?

If I were hanging on to the roof of a twenty-story building by my fingernails, or if someone were holding a gun to my head, or even if I were going in for major surgery, no one would question my right to be apprehensive.  Why are invasive procedures not automatically on the list of stressors?

Perhaps a better question is this:  Why do I feel like I have to defend my fear?

It’s the people who are not afraid of these procedures who should be regarded with suspicion.  “You’re not scared?  Why not?  What’s the matter with you?”

Some people are afraid of spiders or elevators or clowns.  Me?  Invasive procedures.  Yes, I have asked for help—practically everyone I know who might have some insight or advice has heard from me.  But my apprehension is compounded by the fact that I also feel silly for having to ask for help dealing with this.  I know I’ll be apologizing to the nurses in the cath lab because I’m so nervous.  Why?  It’s not my fault!  But I still feel like a ninny.

I’m beyond reassurance.  At this point, only proof that “you’ll be fine!” will do.

Please Pardon the Interruption

If you are someone who stops by here occasionally, you have probably noticed that I haven’t posted in a while.  A month, to be exact.  That’s because I’ve hit a bit of a jam commonly known as writer’s block.  Something happened last month that has been fully occupying my attention, and I want to write about it, but it would interrupt the narrative flow, which offends the anal-retentive editor in me.  In my most recent entry, I was still talking about what happened last year, so won’t it confuse my gentle readers to inject current events?  I keep telling myself, Finish telling the story, get everyone up to date, then you can come back and talk about this latest thing. The problem is that the rest of the story won’t come.  So I have decided that my gentle readers are smart people who can handle a burp in the timeline.  And indeed you are.  So here’s what has happened in the last two months.

I had an echocardiogram (nicknamed “echo”) in August.  Those of you who have had children know about this stuff; an echocardiogram is a sonogram, but instead of pointing the wand at Baby, the technician points it at your heart.  It’s really amazing technology!  I can’t believe they can put a hunk of plastic on your chest with a little bit of warm goo and get a television image of your heart working.  You can even hear the valves flapping and the reassuring whoosh-whoosh of your blood.

I was really looking forward to my echo.  Over the past three years, I have had many tests that were invasive, scary, uncomfortable, or all three, but you can give me sonograms all day long.  Noninvasive, dark room, warm goo, whoosh-whoosh, what’s not to love?  Plus, I have been feeling good lately.  I mean, really good.  That’s been true for a while, but over the summer, I realized that I finally regained my stamina.  I don’t have to budget my energy anymore; I can actually do more than one thing in a day and not be completely exhausted.

The echo estimates the pressure in the pulmonary artery.  Because I’ve felt better and because I’ve regained some stamina I knew my pulmonary pressure was going to be lower than it was last August.  Still, I was curious to know how much lower.

But at my follow-up appointment with my cardiologist in September, he reported that my systolic pulmonary artery pressure is estimated at 78.  Anything over 75 is considered “severe.”  The tech pointed out some small improvements to my heart during my echo, but the final report reads, “No significant changes from previous study dated 8/20/2008.”  So the cardiologist wants me to have a heart catheterization (familiarly known as a “cath” but, believe me, this is not a procedure with which I want to be familiar).  Remember, an echo only gives you an estimate; a cath will tell you exactly what the pressure is.  Apparently echos can be off by as much as twenty points, so the docs need to get in there and find out exactly where my pressure is (and if there is something else causing my high pulmonary pressure).

I am not happy about this turn of events for two reasons.  First, the cath I had last year was miserable, sufficiently so that I am having considerable anxiety about facing another.  Second, I don’t understand what this discovery means for me.  Why do I feel this good if my pressure is still so high?  (Not that I’m complaining!)  It might be a mistake, if the echo is inaccurate.  But if the echo is accurate and the pressure is still high, what then?  Another drug?  Wait and see?  Will I slowly return to feeling as badly as I did last year at this time?  Borrowing trouble, I know, but it’s hard not to be worried.  The medical community brags about how advanced echos are these days; can they really be that far off?

The cath is scheduled for October 26.  I don’t know what time yet.  But if you happen to think of it, any positive vibes pointed in my direction will be appreciated.

In the End: The Proverbial “Last Straw”

Amid all the tummy troubles, in October 2007, I noticed a wound on my right shin.  Looked like a blister.  I put Band-Aids and wound cream on it, I uncovered it so it could “breathe,” I did everything I could think of for that darned thing but it Would.  Not.  Heal.  After about three weeks of this I finally, reluctantly, called my doctor’s office.  You can probably imagine how tired I was of dealing with health issues by this time.  Oh, please, can’t I ignore this?  My usual doc didn’t have an opening but they fit me in with her partner.

From three feet away, he declared, “Oh, yes, that’s a venous stasis ulcer.  Do you have a physical therapist you’re comfortable with?  OK, good, here you go, bye-bye!”  Five minutes later I was out on the sidewalk with a referral for physical therapy thinking, “Venous what?”

Fortunately the therapist had more time for me and explained what was going on.  Lymphedema.  Sometimes the lymphatic system breaks down and is unable to do its job, which is getting lymphatic fluid out of the extremities (arms and legs).  As a result, fluid can build up in those extremities.  With the excess fluid, sometimes venous stasis ulcers (which are little blister-like bubbles of a fluid called “lymph”) form, and if they break open, it is very hard for the wound to heal because the circulation to that limb is so slow.

Job One was healing the ulcer.  The therapist wrapped my leg from ankle to knee in bandages that were soaked in calamine lotion.  “It’s old-fashioned,” he said, “and a little inconvenient.  But we still do it this way because it works.”  The bandages stayed in place for a week or two, so I had to shower with a garbage bag on my leg.  That was a pain in the posterior but, as advertised, it worked: when he removed the bandages, the wound was healed.

The next step was reducing the size of my legs so I could wear compression stockings.  My therapist began a regular schedule of very gentle massage designed to “wake up” the lymph nodes so they would move more fluid out of my system.  Once my legs came down to a more normal size, he measured me for compression stockings, which were made especially for me.  My first pair went from toes to hips and fastened around the waist.  No matter how loosely I secured them, I always felt sick when I wore them (they aggravated the IBS), so my second pair went from toes to knees.  Not as much coverage as I needed, but at least I could wear them every day.

Lymphedema does not go away.  It can be managed, but you’re stuck with it.  Not only is there no cure, lymphedema requires daily attention.  You have to wear compression garments every day and keep them clean.  Experts emphatically recommend that patients put lotion on their skin every day without fail (because dry, cracked skin is a hazard) and keep a watchful eye for ulcers and signs of cellulitis (“a common, potentially serious bacterial skin infection” according to the Mayo Clinic).  This can be a challenge because lymphedema so often hits obese people.  You must be hyper vigilant about protecting the limbs from injury.  And fer cryin’ out loud, don’t go outside without bug spray!  (I hate bug spray.)

I understood that eventually I would learn to live with it, but at the time of the diagnosis, the very thought of having to live the rest of my life with a high-maintenance chronic disease was deeply discouraging.  I had lymphedema and IBS to deal with and could only wonder what would be next.  Was my body falling apart before I was done using it?

Sources:  my therapist, the National Lymphedema Network, Lymph Notes, and Lymphedema People.

In the Middle

During these years, I often complained to friends: “Just when I get one thing fixed, something else pops up!”  I had my last migraine on February 4, 2007, and my first bout with “tummy troubles” (we didn’t know what to call it then) on February 8.

I had never been so sick.  Painful cramps, fever, intestinal distress “at both ends,” as they say.  That first time, I was trapped in our guest room for two whole days.  I camped there so I wouldn’t wake my husband in the middle of the night but also because the loo was only a few steps from the bed.

The first time it happened, I thought it was a stomach flu.  The second time it happened (just ten days later), I thought I was simply unlucky.  The third and fourth times?  There was definitely something going on.  My doctor ran a battery of tests and couldn’t find anything, so she referred me to a local gastroenterology office, where I was looked after by a very thorough and professional physician’s assistant (PA).

She ran every conceivable test.  I was at the hospital so often that I (only half joking) asked a nurse if they had an awards program (they don’t).  I found out, among other things, that I digest food very quickly (within about an hour!), don’t have any problems with my esophagus or stomach, have a perfectly healthy gall bladder, and did not have Lyme Disease or parasites.  Lacking any other diagnosis, we settled on irritable bowel syndrome (IBS).  (In lighter moments, I often quipped, “Sometimes it’s irritable, sometimes it’s really ticked off!”)

Usually, the meds seemed to keep things under control, but then I’d have another flare-up, and we’d tinker with the meds again.  This went on for about eighteen months.  I cannot begin to describe to you the frustration this situation caused.  It wasn’t just the fact that I was occasionally (and unpredictably) imprisoned by my body’s requirement that there be a bathroom nearby.  It wasn’t just the discomfort and “yuck” factor of being sick.  I made social appointments only with caveats (“Assuming I’m feeling OK that day”).  I often had to cancel or defer commitments at the last moment, or I simply didn’t attend events I’d been looking forward to.  I didn’t dare plan anything with any sort of long lead time.  Even though it was obviously not my fault, I worried that friends would think I was avoiding them, or that colleagues in community service pursuits would label me as hopelessly unreliable.

Toward the end of my eighteen-month-long ordeal, I was having less of the intestinal distress, but there were a lot of foods I just could not eat (called “food aversion”): foods either tasted really bad to me or the texture made me want to spit them out.  I didn’t even want my favorite foods (cheese or sweets).  I was surviving almost exclusively on Carnation Instant Breakfast, skim milk, yogurt, and produce.  I was also filling up very easily (which is called “early satiety”); for example, I would eat a banana and half a glass of milk and be completely full.

I saw the PA again in August 2008.  It was obvious she was completely frustrated.  People go into medicine because they want to help people, and my body was simply not responding to anything she tried.  The shocking thing was when she told me I’d gained twelve pounds since I last saw her (which had only been six weeks).  She couldn’t believe it when I told her how little I was eating. She sent me for a chest x-ray and wrote in her notes that there was something systemic going on that was causing my problems.  She was right, and three weeks later we knew what it was.

In the Beginning

I’m going to back up a little and explain how I got here.  You don’t just wake up in a hospital with extreme shortness of breath.  My journey started about three years before I landed in the hospital.  I don’t know if my particular path is unique or not, but it is not unusual for a PH patient to experience years of misdiagnoses and apparently unrelated health issues before the docs finally figure out what’s really going on.

Perhaps I should emphasize this point: no doctor has ever drawn a line between my early problems and pulmonary hypertension, but it seems to me extremely likely that they are related.

I had been getting bad tension headaches for a couple years but very rarely, maybe only two or three times a year, and they always yielded to three Tylenol and an early night.  During the summer of 2005, I was working a lot (I’m a freelance book editor) and serving as president of a local community group.  It was a very stressful time, and frankly I wasn’t surprised that I was getting those headaches more frequently—and that they were increasing in intensity.  I started seeing a general practitioner (GP) about them.

In May 2006 I had my first granddaddy of a migraine.  I had always had sympathy for people who get migraines regularly, but that was when I first truly understood what it’s like.  I started getting them more regularly.  They were of the “textbook” variety and included the full range of misery: visual disturbances, sensitivity to light and scent, and—well, let’s just say I kept a bucket handy.  Migraines arrived with more and more frequency and greater intensity, pain that brought me to tears, pleading for stronger meds, something, anything that would make them go away.  I began to understand why people coping with chronic pain consider suicide (thank goodness I wasn’t quite that hopeless yet myself).

In August, I had a migraine every day for sixteen days in a row.  I had already put my volunteer activities on a back burner, and I reluctantly stopped working as well.  Dishes and laundry piled up.  I simply couldn’t function.  Even on days without a migraine, I always felt exhausted.

Throughout all this, my GP and I tried a variety of meds.  Nothing really helped.  I kept asking her, “Why does an otherwise reasonably healthy thirty-eight-year-old woman suddenly start getting migraines?  Why?”  She wasn’t so concerned about the why (mostly, I think, because the possibilities were endless); she focused on trying to make them stop, which was fine with me!  But having no success, she finally threw her hands up and referred me to a neurologist.

The neurologist discovered my blood pressure was through the roof, which surprised the daylights out of me—it had always been so good that nurses would compliment me on it!  She started me on blood pressure medication, with additional orders to stop all pain medication. “Not even a baby aspirin!” she instructed.  (Turns out I was having rebound headaches in addition to the migraines.)  I had my last “textbook” migraine on Superbowl Sunday, 2007 (wish I had known that then; I would have thrown a “going away” party).  From there, the headaches tapered off slowly over the summer.

Migraines: fixed!  Time to move on with my life, right?  Nope.

Happy Anniversary to Me. Not.

It’s my anniversary.  Not one I’m celebrating, though.  So maybe “anniversary” isn’t the right word.  Let’s say it’s a landmark.

One year ago today, the sky fell in on me.  I was alone in my hospital room at the Hershey Medical Center when a member of my cardiac team of doctors entered.  His face was very serious as he sat beside my bed and apologetically told me there was no cure for me.  Nothing they could do to “fix” me.  They had looked under every rock and could not find the cause of my condition, so there was nothing they could treat.

He kept talking, but to this day I cannot remember what he said, except that he kept asking if I had any questions.  I was so stunned, I could only shake my head “no.”  All I could see was a white fog.  Thoughts were swirling around in my head so fast I could not grasp them.  How can there be nothing?  They can treat so many conditions these days!  Why not mine?  I was so sick, would it be like this for me forever?  Not being able to climb the stairs or cross a street, passing out when I cough?  Are you telling me I’m going to die?  Are you telling me I’m going to die?

I did not have the courage to ask him that question.

As soon as the doctor left, I called my husband.  I don’t remember what I said to him, except that I fell apart on the phone, and he promised to come right away.  My sister called to check on me and I cried my heart out to her, too.

A social worker arrived a few minutes after that and caught me crying.  “Are you OK?” she asked.  “No.  I just got some very bad news.”  She launched into Professional Comforting Mode: “Yes, it’s natural to cry when we blah blah blah….”  I thought, I am aware that my behavior is normal.  Shut up. But I said, “What can I do for you?”

I have no idea how long it was before my new specialist arrived at my room to introduce himself.  It felt like an eternity, but it was probably only a few hours.  My mom and husband arrived while he was asking about my symptoms.  Every now and then for months afterward, one or the other of them would say, “Yes, your doctor mentioned that,” and I’d ask if they were talking to him behind my back!  “No, he said it that day at the hospital….”  I don’t remember that conversation, either.  Turns out, shock is a great memory scrubber.

I still wish there had been less time between the “we can’t fix it” announcement and the “but we can control the symptoms” conversation.  But, honestly, it wouldn’t have mattered.  I was strapped into the harness, launched on a frightening journey, no brake pedal in sight.  I can’t imagine that any amount of propitious timing could possibly have prepared me for it.

What Is Pulmonary Hypertension, Anyway?

Idiopathic Pulmonary Arterial Hypertension.  What a mouthful!  Let’s break it down one word at a time.

“Idiopathic” means the doctors don’t know why it happens.  The common joke (made by the doctors themselves) is, “The idiots in pathology don’t know why.”

“Pulmonary” means it’s in the lungs.

“Arterial” means it’s in the arteries; in this case, the arteries in the lungs.  Did you know your lungs had arteries?  I think I knew in the fifth grade, when we had a health unit on the heart.  We learned the whole path your blood takes on its journey through the heart and lungs, but of course I’d forgotten that until last year.

“Hypertension” means the blood pressure is too high.  You have probably heard about systemic hypertension, maybe even have it yourself, probably taking pills for it.

When you put all these words together, it means that the pressure in the arteries of the lungs is much too high, and the reasons for it are a mystery to the medical community.  The heart squeezes blood from its right side into the pulmonary artery.  Because the heart is meeting so much resistance, it has to work harder to get blood where it’s supposed to go.  What happens when you exercise a muscle?  It gets bigger.  That’s great for biceps but not so great for the heart.

So the heart is working really hard to pump blood and having a terrible time.  Not much blood is making its way into the lungs to get oxygenated.  The result is that, with any exertion, the patient gets short of breath extremely easily.  There can also be dizziness, chest pain, swelling in the legs and ankles, fainting, and all-over tiredness.  I also had blue lips and a really bad, dry cough.

My sources for this entry:

1. my doctors,
2. the Pulmonary Hypertension Association’s website page titled “What is PH,”
3. the Pulmonary Hypertension Association’s brochure “Understanding Pulmonary Hypertension,”
4. and Remodulin’s® website page titled “PAH Overview” (Remodulin® is the PH drug I’m taking, more on that later).

Introduction

My name is Mary. I am 41 years old and live with my husband and our furry kids—a dog and four cats—in south central Pennsylvania. A year ago, I was diagnosed with Idiopathic Pulmonary Arterial Hypertension (IPAH, also affectionately know to its hostages as PH). IPAH is rare, affecting about two people in a million. (Somehow I don’t feel as though I won the lottery. Wonder why that is?) It is also incurable (unless you consider a lung transplant a cure), progressive, and ultimately fatal.

It takes a certain amount of hubris to imagine that anyone, possibly not even the people who love me best, will be interested in what I have to share in this space. If you’re not, I’ll try not to take it personally. All I can say is that starting a blog is an urge I have fought for about nine months, and I finally decided to do it—if for no other reason than to get the darned thing off my back.

To be honest with you, I have no idea why I have this emphatic compulsion to write about my experiences with pulmonary hypertension. I do have a few theories. Maybe recounting how hard the last year has been will make it easier to appreciate how good I feel now. Possibly this blog will connect me with “kindred spirits” who really understand what a chronic illness does to you—not just the nitty-gritty physical consequences but the mental as well. Perhaps so that, when I shuffle off my mortal coil, there will be some evidence that I was here. Who knows? Could be all three.

The one thing I’m sure of is this: When talking to people kind enough to ask after me, I feel honor bound to accentuate the “up” side of having this disease. As in, “It’s very serious, but I’m responding really well to the meds!” These people are friends who care about me, and I so appreciate their support. I don’t want to be a downer. And really, I am doing so well these days that expressing optimism is not an act. But sometimes I hit a wall and I just cannot be cheerful. I have created this place as a space for me to be honest about what is going on in my head. I won’t always be chirpy Little Mary Sunshine. But here is our contract, our covenant, between me and you, the reader: When we meet I will answer “I’m great!” Here you will find the truth—good, bad, indifferent.