Sarah Pette's Pulmonary Hypertension Blog

Sarah's ManiPHesto

Posted on February 19, 2013 by Sarah

Two years ago today, I was diagnosed with a rare, life-threatening, and progressive disease called Pulmonary Hypertension (PH).  The short explanation is that, for an unknown reason, the blood vessels in my lungs are closing, causing my heart to fail under the increased pressures.  I think there has been a lot of confusion from friends and family about what exactly all this means, so on this second anniversary I thought I'd try and explain a bit about what's been happening.

John and I left El Salvador in June of 2010.  I had noticed that I'd begun slowing down, but I blamed it on being overweight and out of shape and resolved that in the New Year I'd try to be healthier.  January rolled around and I caught H1N1 flu; I then caught another severe strain of flu a few weeks later.  While this in no way contributed to my PH, it sort of kicked it into high gear.  For weeks I thought I had pneumonia because I felt fine when I wasn't moving, but I couldn't walk without holding on to the wall, I couldn't roll over in bed without being severely out of breath, and I couldn't bend over without getting lightheaded and gasping for air.  John finally convinced me to go to the ER on Valentine's Day, 2011, where I then stayed for a very long time.

It can take years to get diagnosed with PH because it is so rare, but as I was dying of heart failure they managed to move quickly and diagnosed me in four days.  Within a few hours of diagnosis I was in an ambulance to another local hospital that had specialists in my disease, and a few hours after that was in surgery to have a catheter implanted in my heart.  It is a white silicone tube that comes out of the right side of my chest and is attached to a medical pump about the size of a paperback.  (Sexy!)  The drugs I get through this continuous IV are vasodilators that keep my blood vessels open so that my heart doesn't have to work so hard and will last longer than through next week.  This drug also has a four-minute halflife, meaning that if the pump malfunctions or the catheter is pulled out, my arteries immediately begin to close and my life is in immediate risk.  These drugs are also so rare and expensive that only one hospital in the area even has them.  Fun times.

I remained in the hospital for about three weeks, but, unbeknownst to me, I had become septic in that time.  This means that I had a systemic blood infection.  About a week later I was back in the hospital, this time with a 105 degree fever and having seizures due to the infection.  This was one of many nights I was sure I was going to die.  After another three weeks or so I was released back into the wild, only to be back shortly with another hospital-acquired infection - this time, pneumonia.  I think I was only hospitalized for a week or so that time, but it all sort of runs together.  My parents and John were with me through the duration of my time in the hospital.  My mother actually had to come and live with us for a few months because I was too weak to walk on my own.  I spent almost the entirety of this time curled in a ball, trying to make it to the next breath.  In total, I was out of work for four months, and even then was only working a few hours a week for some time after that.  We were only able to do this trough the incredibly generous leave donations from our government friends; during that entire time, I never missed a paycheck.

I spent the next year in and out of the hospital and in and out of heart failure.  I did not eat any solid food for three months besides the occasional grape or two, and subsisted on Kool-Aid, ginger ale, and Italian ices.  In retrospect, someone really should have put me on a feeding tube, but they were too concerned with my heart and lungs to worry about my stomach.  During this time, I lost approximately 45 pounds.

I am currently on 14 medications to try and control my disease.  The most notable is carried around in my medical pump and infused directly into my heart via catheter; I am on it 24/7 and, until science comes up with something better, will be for life.  This means that it's difficult to shower and I cannot swim, which breaks my heart.  I am actually on Cialis as well, as it was invented as a vasodilator that just happened to have a very profitable side effect in men.  (They tried me on Viagra, but it gave me headaches.  And no, you may not have any. ;)  I'm also on so many diuretics that I know the location of every clean restroom in the Washington metro area.  I take pills around the clock, not to mention that I'm addicted to IV drugs.  My parents are so proud! 

In the course of trying to combat my disease I've had my meds changed a lot.  Every time this happens, it makes me incredibly sick and I am unable to eat.  Having seen my brother go through chemo, I would compare it to that except my meds are on continuous infusion, so I don't get a break from the side effects.  There was another big adjustment in May of 2012, and between May and October, I lost an additional 75 pounds, purely through an inability to eat. 

All of these drugs cause tremendous side effects.  I am always red because my blood vessels are so dilated, and strangers comment on this a lot.  (Nice sunburn!  Gee, it must be cold outside!)  I struggle with severe nausea, and my muscles are constantly sore; I have a lot of headaches, and often just feel like I have the flu.  Sometimes these are the good days.  The IV meds also have the unfortunate side effect of causing severe nerve pain in my legs and feet, so I can't stand for very long.  I can't easily explain this to strangers, though, so I get a lot of nasty looks when I use my handicapped parking.  The disease itself keeps me from being able to bend over, run, go up stairs quickly, or even walk some days.  Who knew your heart and lungs were so important?

A few weeks ago, John and I celebrated a year without hospitalizations.  (We don't even count ER visits at this point.)  Unfortunately, I was just hospitalized again last week with Norovirus, which apparently didn't care that I already had enough weird health crap going on and didn't need a viral stomach flu.  I'm now trying to rebalance my medications and get back on track.

Currently, my doctors are trying to keep me healthy enough that I don't need a lung transplant, though it has been put on the table.  That was a bad day.  Many people nonchalantly mention transplant like it's a cure, but the truth is that lung transplant patients don't live very long.  It's not a cure, but merely a way to get a few more years.  However, I'm convinced that hearing this news somehow turned something around, because since being confronted with the possibility of transplant I have been doing much better (though the addition of a third class of PH drug to the regimen might have had something to do with it...).  I'm almost up to the functionality of a very lazy, out of shape regular person!  This has been a huge improvement and a huge relief for now, but since the disease is progressive, there is always fear looming in the background of what might be next.

There have been a lot of advances in the treatment of PH, and more drugs are being developed every day.  I also live directly across the street from the FDA, so I can yell at them every day on my way to work to hurry the hell up.  Therein lies my salvation: the long and the short of it is that without a cure, I'm not going to have a normal life expectancy.  I already cannot have children, as my heart is not strong enough.  Since it's so hard to get around some days, it's questionable if I can be a mother at all, since running after a child who's headed for the road could overload my heart and kill me.  That news practically does the same some days. 

All of that said, my only chance for something resembling a normal life is for more money to be spent on the development of new drugs.  John has kindly posted my story on his website in an effort to help raise awareness and funding for research.  My friend Cati has also set up a donation page on my behalf where you can donate to PH research in my name:  The best place to find out more about the disease is through the Pulmonary Hypertension Association.

Thank you to everyone who has donated in the past, and to those of you considering donating now.  Better living through chemistry!  Thank you also for taking the time to read my maniPHesto, (sorry sorry…), and don't ever hesitate to write or call with questions or just to say hi.  I'm pretty easy to track down – I don't exactly run very fast now do I?  Without the love and support of you all I couldn't have made it this far, and it wouldn't have been worth it anyway.