I Haz Hurt Feet

It took two weeks of titrating but I am now at my new dose of Remodulin (100 ng/kg/min).  It’s been moderately uncomfortable while my body adjusts to this new dose: foot pain, headaches, fatigue.  Or perhaps those are just the symptoms of being so tantalizingly close to the end of the school year BUT NOT THERE YET.  The Nuneses: not the best with patience.

I’ll continue to straddle this line between the medicine providing therapeutic results (opening up the blood vessels in my lungs) versus withstanding the increasing side effects.  At some point, the line of return is diminished as the harsh medicine disrupts other body functions; it doesn’t much matter if blood can pump through your lungs marginally better when the extra fluid that collects in your body continues to put strain on your heart.  Or if you’re in constant pain.

I saw Dr. Edelman, my transplant pulmonologist, last Friday (June 8^th).  He had talked to Dr. Ralph about my May visit and concurred that the next step is needing right heart catheterization (RHC) to see if my increase in cruddiness and the extra fluid around my heart has changed some of my numbers (mostly my cardiac index and right atrial pressure).  If those numbers have changed for the worse since my last RHC in September, Dr. Edelman will apply for an “exception” with UNOS (United Network for Organ Sharing, the national organization that gives a score to each patient needing a transplant) to get me a higher place on the waiting list.

Applying to the UNOS Lung Review Board for an exception is necessary because of all the dozens of factors that UNOS uses to give each transplant individual a Lung Allocation Score (LAS), it doesn’t include these two tests which most effectively illustrate the heart failure I’m in.  So my LAS is very low although my disease is in end stages.

Here’s the two big things we’re looking at with my RHC:

1)      The cardiac index (CI) measures how well my heart is pumping blood through my body and is measured in liters per minute.  To qualify for a UNOS exception, your CI has to be 1.8 or lower; mine has been 2.0.

2)      The right atrial pressure is the pressure in the right atrium of the heart. In normal folks, the right atrial pressure is less than 5mmHg.  I am at 9 mmHg and to qualify for a transplant exception, I need to be at 15mmHg.  This test is interesting because it is influenced by dehydration so I will withhold my twice-daily diuretics for 48 hours before the RHC and that should put more of a strain on my heart due to an increase in fluid which will give a more accurate picture of my dire straits.  I am a little nervous about this part as I’ve battled with fluid retention many times before and twice have ended up in the hospital for a week at a time as they pump me full of IV diuretics to get the fluid off. 

Once we get the results from the RHC, if they are at those thresholds, we’ll have the conversation with Dr. Edelman about applying for the exception.   My reaction has been pretty overwhelmed at the idea of moving from the very bottom of the list up to the 90^th percentile.  I’ve kind of liked hanging out with the bottom feeders…I’ve done all the work-ups, all the tests and have felt that when/if I need to move higher on the list, I’m ready to go but that I’m not getting The Call anytime soon.  Then to jump all the way to the top, to expect a call likely within a couple or few months…it’s just a lot to take in.

Other stats:  There’s about 58 people on the waiting list. My LAS score of 31.2 puts me down there in the bottom of the pack (most LAS scores are in the 30s and 40s but the scale is 1-100 so super critical patients can get scored high and be assured they are at the top of the list).  UW has done 18 transplants so far this year which is behind their hoped-for schedule of one a week but it’s been trending up in the past couple weeks.  Of course, in my rather uncouth manner, I suggested that was because of the Seattle shootings we’ve had lately.  I didn’t get a chuckle.

I also attended the Lung Transplant Support Group at UW on Tuesday.   There were pre and post transplant folks and their caregivers there.  It's mostly heartening to hear other folks share their experiences and worries -- lots of inspiration, though some discouraging things, too.  Out of maybe 25 patients, I was the youngest in the room and the only one who has Pulmonary Hypertension.  

My right heart cath is scheduled for Thursday, June 28.  Will post more after my doctors interpret my results!