I felt a lump in my throat as I padded down the stairs, having just kissed my darling twin toddlers goodnight. “I love you, Mommy,” my youngest called after me. “I love you, too, sweet boy. Get some rest,” I replied rather hoarsely, as my throat was swelling with a wail I couldn’t let out. “I’ll see you boys in the morning.” But would I live that long?
That is the question I lay awake with until the wee hours of the morning, unless I self-medicate with over-the-counter sleeping aids. And even then, I toss and turn, sitting upright to check my pulse on the heart rate monitor my wife insisted we purchase the day our insurance company decided it wasn’t going to cover my life- saving surgery.
On Jan. 2, I was diagnosed with Brugada syndrome, an incredibly rare genetic heart disease that sharply increases my chances of dropping dead from sudden cardiac arrest. That’s when your heart suddenly stops beating—not like a heart attack, where blood stops pumping to your heart and you’ve got some time for someone to bring you back to life. With cardiac arrest, someone must be performing CPR within mere seconds for you to have a remote chance to survive. That’s a lot of pressure for my twin 2- year-olds and my graphic designer wife to live under. And it’s quite a heavy wet blanket of fear for me to try to exist under. Though I’ve managed to face most days optimistically, I must admit that it’s been a struggle to keep that oft-seen smile throughout this ordeal.
Eleven months ago, I lost my good-natured 36-year-old brother, my only sibling, after a 15-week hospitalized battle with severe acute pancreatitis. I was by his side throughout it all, working with both the hospital and hospice to ensure he had the best care. I took on that role because four years prior to that, we lost my 61-year- old sweet father to what we believed to be a heart attack. My mother, through choices of her own, isn’t in my family picture anymore. So it’s just me holding up the fort for my withering tree of biological relatives.
It would be far less stressful if I had a defibrillator implanted in my chest, which would shock my heart if I ever go into cardiac arrest. That’s the understood course of action for someone with Brugada syndrome: to surgically place an ICD (implantable cardioverter defibrillator) inside the chest, wiring the heart with tech-savvy skill to send electrical shocks of power to jolt my heart back into normal rhythm. I’ve heard it’s much like a horse kicking you in the chest. I’ll take that over death any day.
So why did Blue Cross Blue Shield of Georgia decide mere days before my scheduled surgery in late January that my ICD “is not medically necessary” and merely “investigational?” Because some mystery determiner of my fate didn’t care for my results on one of the tests I had done in December called an EP study. The procedure, which was performed by my electrophysiologist, a respected cardiologist specializing in the electrical workings of the heart, involves threading two catheters from the groin to the heart and watching it on multiple screens while simultaneously injecting medicine meant to provoke abnormal rhythms in the heart. In other parts of the world, ajmaline is used. It’s not legal in the United States, so procainamide is used instead.
The EP study is just one way to view the inner workings of a person’s heart, but it is not the deciding factor for a diagnosis of Brugada syndrome. The American Heart Association, the Oxford Journal and Dr. Brugada have all published studies indicating that there are numerous Brugada patients who do not have a positive reaction to the EP study, and there are cases of false negatives as well. A more indicative factor for Brugada syndome is the unmistakable pattern on the EKG, which I have without a doubt. It’s a cove shape, recognizable at once to electrophysiologists and apparent to anyone else once their attention has been drawn to it. The Brugada pattern appears on multiple EKGs of mine. It is my understanding that no one has this distinctive pattern on their EKGs if they don’t have Brugada.
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The specific EKG readings of a Brugada patient were discovered in the early ’90s by Dr. Ramon Brugada, a cardiologist from Spain, during his and his brother’s investigation into a dizzying number of sudden, unexplained deaths. It was a joyous moment, I’m sure, when the brothers realized that there was a link, not only on the EKGs of patients with Brugada, but also in a lot of other scientific evidence that you can read about on brugada.org, such as sodium channel blockers and even a possible gene mutation.
Since Dr. Brugada’s a-ha moment, the arrhythmia specialists the whole world over have been learning about this rare disease. Currently, it’s believed that 1 in 10,000 people have it, it’s inherited from one parent, it usually occurs in men, it’s most often seen in Southeast Asia, people who die from it tend to be around 40 years old, and it often strikes in the early morning while the person is sleeping. I’ll turn 36 on March 1, two days after the year anniversary of my brother’s death. I don’t want to die young. I want to watch my children grow up; to teach them to love to read and, from them, to glean patience and how to better enjoy the small details; to hold hands with my wife and share a watery glance when our boys graduate high school and then college; to help my brother’s children never forget their dedicated father who left this earth too soon; and to soak up the sunshine, walk on crisp leaves and welcome the stomach cramps from laughing with my friends.
I have a responsibility to myself and to my family to strive for living. And I believed that my insurance company had a responsibility to honor the comprehensive plan my self-employed wife and I have been earnestly shelling out hundreds of dollars for on a monthly basis.
But what has happened instead has surprised me. After I read the rejection letter from BCBS, I called my doctor’s office. They were as surprised as I was, though they’d received the letter a day before I had, and had already attempted on numerous occasions to get in touch with BCBS to demand answers, a peer-to-peer review (in which my doctor would speak to a doctor employed by BCBS) or to file an appeal. During the last two weeks of January, voicemails for the insurance company were left by my doctor’s office and by me amidst perilously long holding times, during which an automatic recording would say over and over that the long wait times were due to the implementation of the Affordable Care Act. One time, the recording told me that because of the Affordable Care Act and the increased call volume, they knew they’d be unable to answer my call that day and then I heard a dial tone. It was only 1 p.m. I was enraged and quite scared of the implications. Finally, during the first week of February, the wheels have slowly begun to turn as my electrophysiologist (EP) was at last able to speak to the doctor who turned me down for my ICD. And yet, that wasn’t enough. Because surprisingly, the doctor who decided my fate is not qualified in electrophysiology, but instead is a general cardiologist. I don’t think I’m going out on a limb by thinking that this doctor likely does not know that much about my rare disease, and, of course, I’m reeling that he gets to decide whether or not I shall live based on something he isn’t informed about. My EP is now waiting for an electrophysiologist employed by Blue Cross Blue Shield to review my case.
Will I die waiting for a simple procedure that’s afforded to people with much more mild heart issues than mine because of this mysterious denial? If only my insurance company would listen to the extensive expertise of my personal doctor, the one who knows me better than anyone of the anonymous reviewers who aren’t immersed in specialty cases for rare life-threatening diseases like mine.
It does not seem to matter that I might have a familial history of sudden cardiac death or that I’ve had frequent bouts of dizziness and an unexplained galloping heartbeat. Why does an American company that doesn’t personally know me get to make money off of my hard-earned monthly premiums to what I thought was medical coverage in case of emergency while I sit here with my family wondering if this is my last breath? Sadly, I’m learning quickly that insurance isn’t guaranteed medical coverage at all, despite the slick speeches delivered by a president I still admire, but rather a sick and greedy conglomerate that takes and takes and takes, and sometimes doles out pennies for situations it cannot legally extricate itself from.
Since the denial, I’ve spent hours on the phone with attorneys and my doctor’s nurses and administrative team, as well as opening an investigation with the Insurance Commissioner of Georgia and filing my own personal appeal with Blue Cross Blue Shield of Georgia. My doctor and his staff at Piedmont Heart, an affiliate of Piedmont Hospital in Atlanta, Ga., continue to valiantly work to have my surgery authorized because my doctor believes—as six of his colleagues do—that I could drop dead at any moment because of this disease that may have also killed my father. But BCBS claims that, because of the Affordable Care Act, under which no American can ever be denied insurance, it cannot help me survive this deadly disease in a timely manner. And so I continue to wait in fear for the chance to live.
Kirsten Ott Palladino is an award-winning writer and editor, and the cofounder and editor-in-chief of EquallyWed.com, an online same-sex wedding magazine where this article was originally published. She lives in Atlanta, with her wife and children. Reach out to her on Twitter @kirstenop.
Photos: Maria Palladino, Our Labor of Love, Maria Palladino