- CASE FILE
Been told I'm dying from Restrictive Cardiomyopathy and Group 2 Pulmonary Hypertension. Don't qualify for a heart transplant.
ABOUT THIS CASE FILE
From 1998 to 2004, I used prescription eye drops preserved with Benzalkonium Chloride (BAK). BAK is known to be toxic to corneal nerves. Despite frequent exams for new-onset eye pain, the drops were not suspected. But the drops were determined to have caused the pain. The long-term use left my corneal nerves permanently damaged and cause chronic neuropathic eye pain.
In 2013 I was treated at a Pain Clinic for the eye pain. They prescribed Oxcarbazepine (Trileptal), an anticonvulsant. This, despite my stated concern that I had a prior adverse reaction to Gabapentin (Neurontin) and that another anticonvulsant seemed needlessly risky. The physicians assured me I'd be fine. I wasn't.
In addition to the short-term adverse effects, serious on their own, a few weeks after ceasing the drug I suffered an episode of DRESS Syndrome. The leading cause of DRESS Syndrome is ... anticonvulsants. It took months to recover. It was deemed 'near-fatal.' I ended up at the Mayo Clinic.
Several months after leaving the Mayo Clinic I developed a number of sudden-onset cardiac symptoms -- breathlessness, lightheadedness, severe exercise intolerance, near-syncope, and outright syncope (fainting). I was diagnosed with atrial fibrillation.
But I wasn't so sure it was that simple.
Five cardiologists and electrophysiologists assured me that it was, indeed, "just atrial fibrillation." I pressed on.
I self-referred to Harvard (11/2017) where I underwent Invasive Cardiopulmonary Exercise testing which revealed severe heart failure and Group 2 pulmonary hypertension. No treatment. It was suggested that I try to find the exact cause.
I self-referred to the advanced heart failure clinic at Johns Hopkins (7/2018) where I underwent a heart biopsy. They found evidence of acute inflammation that had improperly resolved, leaving in its wake ... fibrosis (scar tissue). My heart was pathologically stiff.
I was seen by one of the world's foremost experts in rare cardiomyopathies (Luisa Mestroni - Fall 2018) who declared that what I have is a Restrictive Cardiomyopathy and that it was causing Pulmonary Hypertension. When I asked her if it was "all downhill from here," she responded with, "You can always pray for a miracle."
I later self-referred to a heart failure specialist back at the Minnesota Mayo Clinic (5/2019) who did additional invasive testing and another heart biopsy. He specified that the exact diagnosis was Restrictive Cardiomyopathy and WHO Group 2 Pulmonary Hypertension. He assured me that this was a direct consequence of the December 2013 adverse drug reaction (DRESS Syndrome). It is a known consequence of DRESS.
He referred me to the heart transplant team for an evaluation.
I have been athletic and a consistent exerciser for my entire life. I have run marathons and could always run for hours with no difficulty. By this point I could neither walk up stairs without serious difficulty nor jog -- slower than I've ever done in my life -- for more than a couple of hundred strides. I feel as though I've been poisoned. The fatigue is nearly crippling. I have lost about 90% of my premorbid functional capacity.
But all of the exercise meant that I am 'too fit' to qualify for a heart transplant. I simply did too well on their treadmill and bicycle tests.
And this is before we consider my complicated history of adverse drug reactions. A transplant requires a laundry list of toxic medicines, many for life.
The problem with my eyes affects my vision dramatically. Any near work (ie, reading, computer use, most hobbies) is extremely difficult for me. The pain in my eyes also makes it very difficult to be in the elements (ie, wind, sun, dust, heating, air conditioning). I basically now watch TV and walk the dog, ordinarily wearing black-tinted safety goggles to protect my eyes.
I have basically been told that I need to wait until my body's "compensatory mechanisms --" what has allowed me to exercise despite a fatally damaged heart -- begin to fail and my organs begin to shut down. At that point, we can re-address the idea of a heart transplant.
Meanwhile, when I asked the Mayo heart failure specialist if my lungs might also have been damaged in the same way my heart was -- and from the drug reaction -- he said it was "a definite possibility." Nearly five years after the initial drug reaction, I am still trying to get a definitive diagnosis. I will be seen by a Pulmonologist at a Respiratory Center of Excellence in a few months. It is a definite possibility that my lungs are also garbage, and that what my future really holds is a heart-lung transplant.
I've been told to watch myself die, and then -- and only then -- will we look seriously at the prospects of a major organ transplant. Until then, I am seriously ill and severely limited. I do believe that I will soon lose my ability to continue to exercise (ie, walk the dog), and that will launch the anticipated downward spiral. Meanwhile, I fight mightily to take the best care of myself that I can, while ensuring that my family understands the gravity of my situation.