Learning How To Take No For An Answer

Being an advocate is hard. 

In public, we celebrate the victories. We laud partnerships, and announce accomplishments. We mark honors, and unveil new opportunities. What we don't do is draw attention to our failures. 

For every email sent, there are five more unanswered. For every sponsor whose logo bedecks the ballroom banner, there are ten more who said no. For every grant awarded, there are twenty more for which one was unqualified to apply or deemed unworthy. For every yes, there are a hundred replies no.

To fight for a cause is to be beleaguered by it, for even successes beget problems. To fight for a cause is to work one's self out of a job, for true success means that one no longer is needed. 

Confession: sometimes I wonder what it would be like if I just stopped. What if I went off the radar, stopped pushing, got quiet, took up sewing instead? I could cancel all my doctor's appointments, quit taking my medications, move out even further into the country, write with pen and paper, and see the world from a perspective no farther than the tip of my nose. There would be handmade afghans, and kittens, and tomatoes, and a screen door slamming shut. Life would be as Southern as Eudora Welty, and one day years down the line I'd fall asleep in a lawn chair in the sun and just not wake up.

Or I'd get all bunkered up in a holler somewhere and just be lonely as sin, while wishing I could do more with my life than just waste away into a shell of a bitter old shrew. Either way. I'm not much good at happy mediums. 

The point is this: the next time you are dealing with someone who doesn't have to do what they're doing, who is giving his or her own time to a cause, who is asking you to consider helping—think just a little longer before you answer. Some people say, "Well, we just get asked to help all the time, and we can't say yes to them all." Granted, that may well be true. Consider though what you're being asked for and by whom. Consider the relative magnitude of what you'll give and what those in need will get. And above all else, approach those who are fighting for a cause with an attitude of respect and a willingness to problem solve—perhaps if you can't help you know someone who can.

Medical Research and the Gaps Between Cause, Treatment, and Cure

I am sick, and I am tired.
I am sick of being an anomaly.
I am tired of fighting against my body.
I am sick of reading headlines about unnecessary, yet well-funded, research.
I am tired of not being considered a sound research investment.
I am sick of competing for attention.
I am tired of being sent home without an answer.
I am sick of doctors who think they know more about a disease than the patients who live with it.
I am tired of doctors who know nothing about my disease at all.
I am sick of money being wasted collecting data that is too vague to provide answers.
I am tired of feeling as though any true breakthroughs will have to come from the patient community.

Every day fibromuscular dysplasia patients connect with one another in a Facebook group facilitated by the international, nonprofit organization FMD Chat, of which I am founder. Every day this group of patients checks in on one another. Every day group members discuss how they're feeling—physically and emotionally. Every day we share test results.

Every day we wonder why no doctor seems more interested in the similarities that are found in our stories. Every day we hope that rather than doctors calculating the percent of patients reporting certain vascular events or peering at biopsies and DNA samples that a doctor will ask us how we feel. Every day we hope for a treatment—much less a cure. Every day we are disappointed.

It grows more difficult not to become bitter.

Fibromuscular dysplasia (FMD) is a noninflammatory, nonatherosclerotic arterial disease that is most commonly seen in women. It may present at any age, but is more commonly discovered when the patient is between the ages of 20 and 60 years old. Just nearly a year ago, on January 30, 2012, I wrote about a FMD patient registry—which so far has cost more than $180,000—and at the time had yet to reveal any information that was necessarily "news" to the medical or patient community—at least those who were familiar with the disease. Of the first 339 patients enrolled, patients reported high blood pressure (66 percent); headaches (53 percent); rhythmic ringing in ears (30 percent); dizziness (28 percent); a whooshing sound in the ear (24 percent); neck pain (22 percent), according to data released at the International Symposium on Endovascular Therapy. 

Per Dr. Jeffrey Olin's presentation at ISET, "there has been virtually no new information in the last 30 years" regarding FMD, as the result of "small case series, case reports (the majority of published papers are single case reports), nothing on pathogenesis, little on genetics, nothing on imaging, little on methods of angioplasty, no new classification since Mayo Clinic 40 years ago." In saying as much, Olin does a bit of a disservice to his own prior publications on FMD and publications by those doctors oft associated with FMD. However, the 2012 conclusions drawn from the registry were widely familiar—FMD is often found by accident after a stroke or transient ischemic attack; it should be looked for in young patients with high blood pressure or migraine-type headaches; it can be diagnosed with angiography, CTA, or MRA; angioplasty can be used to open up affected arteries if done early enough; lood thinners and antiplatelet drugs may be prescribed; FMD can lead to a tear in the artery or permanent kidney damage; it is unclear what causes FMD; FMD is an underdiagnosed disease.

For patients to enroll in the registry they must travel to one of a handful of participating centers. Of the 339 patients included in the ISET report 148 were enrolled at the Cleveland Clinic in Ohio; 68 at Mount Sinai in New York; 35 at the University of Michigan; 32 at Alliance Health in South Carolina; 20 at North Central Heart in South Dakota; 18 at Ochsner in Louisiana; 18 at the Mayo Clinic. Data indicates that 328 procedures were performed on 171 of the patients enrolled, with 54.3 percent receiving Percutaneous Transluminal Angioplasty; 21.3 percent receiving Percutaneous Transluminal Angioplasty and Stenting; 8.8 percent receiving a vascular bypass; 4.9 percent receiving endovascular repair of an aneurysm; 2.7 percent receiving open surgical aneurysm repair; and 7.9 percent in the "other" category for therapeutic procedures. Of these 171 patients, 73.2 percent's target vessel was the renal artery. 

Such findings are of clinical value for those unfamiliar with FMD, and the conclusion that FMD presents in the carotid and/or vertebral arteries as often as the renal is indeed intriguing and aligns with the conclusion that "the most common presenting symptom/sign was hypertension followed by headache, pulsatile tinnitus, and dizziness." Patients among the FMD Chat community are well-acquainted with these symptoms; however, there remains a significant gap between recognizing the prevalence of these symptoms and treating them. 

Hypertension in FMD is caused by renal artery stenosis, which causes the kidney to emit renin, a chemical that raises the blood pressure. This hypertension often is treated with anti-hypertensives widely used in the general population. The problem is that FMD related hypertension often is resistent, requiring multiple medications and high doses to manage. These drugs—as with all drugs—come with their own sets of complications, and to many patients fulfill the cliche "the cure is worse than the disease" in terms of physical effects, as patients typically do not feel hypertension, known as the silent killer, while anti-hypertensive side effects such as swelling, coughing, chest pain, irregular heart beat, fainting, nausea, and shortness of breath are much more noticeable. Headache related to FMD becomes complicated by the fact that patients generally are advised not to take NSAIDs, which can cause kidney and stomach damage. Those with migraines are further restricted if prior vascular events such as uncontrolled high blood pressure or stroke contraindicate vasoconstrictors such as the commonly prescribed Imitrex. The Federal Drug Administration has approved no drug to treat tinnitus, and, based on what patients have reported within the FMD Chat community, dizziness typically appears only intermittently, making it a somewhat vague symptom to treat in any manner other than a directive to "stand up slowly." 

Granted, only about 250 of the approximately 7,000 rare diseases in the world have FDA approved treatments; however, treating symptoms of a disease is a different exercise than treating the disease's underlying cause. The FMD community is in dire need of measures to improve patients' quality of life. Research into the disease's genetic factors, while needed, does nothing to help patients currently living with the disease. And so a year after first issuing my plea, I say once again, "More must be done across the related specialities to address the issues that many FMD patients struggle with on a day-to-day basis including crippling fatigue and pain, the inability to work and lost income, depression and anxiety, isolation and fear. These issues are imminent threats to patients' overall health, and without a concerted effort to holistically treat patients there may well be fewer patients willing and able to participate in research studies."

What has changed in this year's time is that FMD Chat, then a fledgling organization, has grown, and that I am making it a personal mission to improve current patients' quality of life. Part of this mission is selfish—I am an FMD patient, and I want better care for myself. However, I also want better care for the hundreds of FMD patients who have made FMD Chat part of their support network and for all the patients FMD Chat has yet to reach. This mission is about making a difference and caring tenaciously for a group of patients in need, a group of patients that I have come to consider my friends and family.

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