When it isn't PH

I have this long-running joke that I drew the short straw in my family gene pool. In my twenty-nine years (ok, ok, thirty-one!), I have received diagnoses of tetralogyof Fallot (a congenital heart defect, you can read my story here), systemic lupus erythematosus, immune thrombocytopenia purpura, cervical cancer, acquired angioedema and other autoimmune diseases.

A few weeks ago while perusing Pinterest, I came across a quote that said,

“God gives us only what we can handle. Apparently God thinks I’m a bad-ass.”

Truer words have never been spoken… er- written.

Through all the things I’ve endured in my life, I’ve never had that ‘woe is me’ mentality. Quite the opposite, in fact. My mom has a photo of me in a hospital bed after my second heart surgery, arm stretched out thrusting my pretend sword in the air yelling just like He-Man, “I have the power!”

He-Man was my hero (no She-Ra for this gal!), and if he could defend Castle Grayskull from the evil Skeletor, than I could beat whatever came my way. And that is essentially how I’ve always tried to live my life.

It wasn’t until recently, two words were spoken by quite a few of my doctors that I began to get fearful of my diseases, and what they could lead to.

Pulmonary Hypertension.

As a pediatric cardiac ICU nurse, I know all too well about pulmonary hypertension. In my clinical experience, there were never any good outcomes.  That’s not to say that there aren’t any, but since I worked with such a fragile patient population, I unfortunately didn’t get to see any.

I also knew I was starting the pulmonary hypertension workup with two strikes already against me: a congenital heart defect and lupus, which are both causes of PH.

Once I reported to my rheumatologist that I was progressively getting more short of breath, it was a whirlwind of specialists and tests galore. I went to a pulmonologist, my cardiologist, my hematologist; I did PFT’s, chest CT’s and more blood work. All of it came back abnormal, everyone had his or her own opinion, but nothing could definitively say it was pulmonary hypertension or not.

All involved parties decided that a cardiac catheterization would finally put the question to rest. I hadn’t had a cath since 1987, right before my last open-heart surgery. I’d say I had a pretty long run before I needed one again.

Once the catheterization was scheduled, I had a few weeks to prepare. When faced with the possibility of a new diagnosis, I’m usually digging through my nursing textbooks, doing Google searches, and reading online discussion boards. Yes, I’m fully aware of my neurotic tendencies, but this time, I was actually staying away from most online resources (except the links sent to me by PHA!) to try and maintain a little sanity.

It wasn’t until 6 days before my cath that I got my hands on the radiologist’s final report of one of my CT scans. I’m thinking it was a big, fat mistake that I was given a copy, but I got it nonetheless. I went straight to the bottom and read ‘Conclusion: probable pulmonary artery hypertension.’

My calm, collected, make jokes demeanor went right out the window. I just knew it was pulmonary hypertension. And apparently, so did the radiologist.

During the pre-admission testing the Friday before my cath, I found out I would recover in the Cardiac Procedure Recovery Unit (CPRU), or the Cardiac Intensive Care Unit, which is actually where I’m a nurse. It never crossed my mind that going to the CICU was a possibility, further heightening my nerves.

You would think I would have remembered that all PH patients recover in the CICU after any type of procedure, but the more distressed I became, the less I remembered I was a nurse. All I could focus on was knowing where I woke up determined the outcome.

I’ll skip the details of my weekend before Monday’s catheterization; just know that my wonderful husband did everything he could to help ease my mind.

In the cath lab, the doctors and nurses were wonderful. I was given pain and sedation medications, but I was so nervous and fighting them. I was too scared to fall asleep, for fear of waking up in the CICU. Eventually, one of the nurses said, “Marla, go to sleep.”

So I did.

I woke up to a nurse practitioner telling me she was going to removed the catheter from my groin, and the next thing I know, I became very dizzy and the voices around me began to sound farther and farther away. I vaguely remember hearing someone yell, “I need Atropine and some help in here!” as an oxygen mask came down on my face.

Attempt number two at waking up, I was scared to open my eyes. I didn’t know where I would be.

I opened them.

CPRU.

Not only did I not have pulmonary hypertension, I didn’t require any type of intervention. I was shocked and relieved. And happy; oh, so happy.

Throughout my 6-hour flat-time recovery, my husband and sister kept me entertained, spoon-fed me, and coworkers came to visit. How wonderful it was to not have them taking care of me in the CICU!

A few doctors and a nurse practitioner from the Adult Congenital Heart Defect team came in and out, too. They all said the same thing; I had them quite nervous I would be going home with a pulmonary hypertension diagnosis.

Not this time.

I have the power!

By Marla DeFusco