Misdiagnosis of
Fibromuscular Dysplasia in Women
Kari Ulrich RN
Aspen University
Professor Bonnie Kehm, PhD, RN
January 20, 2017
Abstract
This
paper explores gender bias as a contributing factor in misdiagnosis of fibromuscular
dysplasia (FMD), a vascular disease in females. It has been established that over 90% of
diagnosed FMD patients are women. In a disease that is vascular in nature, it
is reasonable to look at comparisons in female patients with cardiovascular
diseases. Although FMD is different from
atherosclerosis, FMD and heart diseases share similarities in risk factors. The articles reviewed included current
literature on FMD and gender bias in health care, including cardiovascular
disease. The sources were used to understand
the bias towards women’s health care historically in addition to gender bias in
heart disease. As research about gender
disparity in women’s health become available, the sharing of results are
essential in clinical practice as well as indoctrination in medical
training. Indeed, as a consequence of
these comparisons and consideration of the history of gender bias in health
care, gender bias is undeniably a factor in the misdiagnosis of FMD in women.
Keywords:
Fibromuscular Dysplasia, Gender Bias, Women
Misdiagnosis of
Fibromuscular Dysplasia in Women
Recognizing a vascular event in
women with fibromuscular dysplasia (FMD) is imperative in avoiding
misdiagnosis. When a woman arrives at
the emergency room complaining of a headache and neck pain, most physicians
will formulate a list of differential diagnoses, usually thinking of common
causes while ruling out the most dangerous ones. Physicians will do a proper workup and imaging,
and when the results come back negative, they often diagnose women with stress
or anxiety. Significant clinical signs
are there, so why are health care providers missing them? Women with undiagnosed fibromuscular
dysplasia are often sent home only later to suffer a heart attack, carotid
dissection or stroke. Although
fibromuscular dysplasia is thought to be more prevalent than breast cancer,
misdiagnosis in these women is a critical concern (Burton, 2009). Women with fibromuscular dysplasia
have an increased risk of an adverse event through misdiagnosis due to gender
disparities.
Literature Review
Several
articles have been written about fibromuscular dysplasia (FMD), but gender bias
has not been considered a factor in the misdiagnosis of FMD. This review examines information on FMD and
gender bias in health care. The journal
articles are peer reviewed and were retrieved online from Google Scholar and
ProQuest. One newspaper article was used,
which gives a perspective from the patient point of view.
Fibromuscular
Dysplasia
The current
literature establishes that FMD is a disease that predominantly affects the
female population, and symptoms are often dismissed (Olin et al., 2014). Gender bias may play a critical role in the
misdiagnosis of FMD, as evident in the literature on women’s health care. Similarities of gender bias as it relates to
women diagnosed with cardiovascular disease are compared to women diagnosed
with FMD.
FMD is
defined as a rare, non-inflammatory vascular disease that affects mid to distal
arteries. It is different from
atherosclerotic disease, which is caused by inflammation. However, inflammatory biomarkers have been
found in a cohort of FMD patients (Ganesh et al., 2014). Medial FMD is the most common classification
of FMD and accounts for greater than 90% of the diagnoses today (Olin et al., 2014).
FMD is not a well-understood disease due
to little to no natural history recorded in the past 75 years (Shivapour,
Erwin, & Kim, 2016 p. 376). The limited
knowledge of FMD contribute to physicians not recognizing FMD manifestations.
The current
literature suggests that FMD is underdiagnosed.
The prevalence of FMD in the general population has been mainly based on
renal transplant donors with limited information from cerebral angiography
studies and autopsy studies. Information
from the Cardiovascular Outcomes in Renal Atherosclerotic Lesions (CORAL) trial
showed that of the 58 participants who were screened for FMD and conversely
diagnosed with FMD during the trial, 44 were female (Shivapour et al., 2016, p.
378). The exact prevalence of FMD in the
general population is unknown although Brinza and Gornik (2015) have noted that
90% of FMD patients are women (para. 1).
Research is needed to understand epidemiology in the general population.
FMD can
affect any vascular bed but is commonly seen in the renal and carotid arteries. Symptoms of FMD vary depending on the
vascular bed involved. Typical symptoms include
a headache, high blood pressure, dizziness, and pulsatile tinnitus. FMD should be suspected in young women who suffer
arterial dissections, transient ischemic attacks, or myocardial infarctions as
these afflictions may be life threatening (Brinza & Gornik, 2016). Out of seven FMD patients featured in a Wall Street Journal article from 2009,
two were diagnosed at autopsy after suffering from cardiac-related deaths due
to FMD. The remaining five patients
stated that their symptoms had been dismissed by health care providers as being
stress related or psychosomatic (Burton, 2009). It should be noted that not all patients are
symptomatic, and some patients are diagnosed incidentally when being tested for
unrelated ailments.
Gender Bias
The
Institute of Medicine (IOM) reports that historically, women’s health needs
have been neglected (Wenger, 2012). Women
make up approximately 50% of the population and are major consumers of
healthcare. Nevertheless, until
recently, women have been excluded from the medical curriculum (McGregor et al.,
2013). For example, the medical school
curriculum does not cover gender bias, and there is minimal teaching on rare
vascular disorders such as FMD. Women’s
health initiatives have been addressed over the past few decades. In the early 1990s, medical institutions started
to address the problem by incorporating women’s health curricula into medical
education (McGregor et al., 2013).
Lack of
familiarity with FMD, predisposition of females to this disease, and vague
presenting symptoms can lead to misdiagnosis of FMD. According to Wenger (2012), females are 52%
more likely to have a delay in emergency care for cardiac symptoms than men
(para. 24). Similarly, Brinza and Gornik
(2015) describe a major problem with the care of FMD patients, noting that the
time from the start of symptoms to the time of diagnosis is approximately seven
to nine years (para. 6).
Common
misconceptions of FMD are found throughout historical literature and have been
noted by Olin et al. (2014). There is a
misconception that all coronary, carotid, and renal disease is inflammatory in
nature. This misconception may lead
physicians to miss the diagnosis of FMD by relying on tests that show
inflammation. As such, physicians may
forgo imaging that would demonstrate FMD.
Cardiovascular events often present differently in women than in men. Coronary FMD has recently gained attention in
the medical literature as a predisposing condition for spontaneous coronary
artery dissection (SCAD). Brinza and
Gornik (2016) state SCAD presents as an acute coronary syndrome. Furthermore, Wenger (2012) notes that 50% of
women with acute coronary syndrome have no evidence of obstructive disease in
their coronary arteries (para. 14). Coronary
FMD may be underdiagnosed due to the appearance of the artery. Olin et al. (2014) describe coronary FMD as a
focal narrowing whereas medial FMD has a string-of-beads appearance.
The history
of gender bias in women’s health care influences how women with FMD are treated
presently. Gender bias has been evident in
the accounts of patients and families, describing how they were dismissed by
medical professionals (Burton, 2009). Gender bias in clinical practice continues to
be a challenge today (McGregor et al., 2015).
Acknowledgment of gender bias in women’s health care and continued
education are critical factors in reducing misdiagnosis of FMD.
Discussion
Understanding
Fibromuscular Dysplasia
In order to
understand the risk of adverse events for women presenting with fibromuscular
dysplasia (FMD), there needs to be an understanding of the disease, itself. As discussed in the literature review, FMD is
a non-inflammatory vascular disease that affects mid to distal arteries. FMD can affect any vascular bed and is
commonly found in the intracranial and renal arteries. FMD is currently considered a rare disease,
although several leading experts argue that it is not rare but rather overlooked
(Brinza & Gornik, 2016 p. 45). The
importance of knowing the difference of FMD compared to an inflammatory disease
such as atherosclerosis is imperative in making a correct diagnosis. For example, an FMD patient complaining of
neck pain, headaches, or dizziness may be worked up for carotid artery disease,
especially if the physician hears a bruit.
The physician may proceed with an ultrasound of the neck thinking that
atherosclerotic plaque is the cause of the bruit. With carotid artery FMD, the distal portion
of the carotid artery must be visualized, or the diagnosis would easily be
missed. As Olin et al. (2014) point out,
atherosclerosis occurs at the proximal portion of the artery, unlike FMD, which
is seen at the mid to distal arteries (p. 1056). This scenario could happen in any of the
vascular beds. Interestingly, FMD can
affect men, women, and children but is more prevalent in young, and middle-aged
women.
Gender Bias a
Consideration in Misdiagnosis
Gender bias
cannot be ignored as a factor for misdiagnosis of FMD. History suggests women have been
underrepresented in health care as far back as the pre-1940s. McGregor et al. (2013) have noted that women
had been protected from clinical trials since before World War Two, and they go
on to say that this was out of fear of harm to a woman’s unborn child (p. 2). However, this is not the entire reason. Women’s hormones played a role. It was thought that because women had
fluctuations in hormones, men would be more suitable as subjects to represent
both sexes (McGregor et al., 2013). For decades;
women were seen as having uncontrollable hormones, and in turn, this led to
stereotyping women as hysterical and anxious when presenting with symptoms such
as a headache, dizziness, or chest pain.
Since it takes, on average, seven to nine years to be diagnosed (Brinza
& Gornik, 2015), female patients have been left to believe that their
symptoms are imagined. According to
Burton (2009), a female patient described going to the emergency room
experiencing severe abdominal pain and was told that her symptoms were all in
her head, she was later diagnosed at another hospital with intestinal ischemia
secondary to FMD. Another female patient
explained that it took three visits to two hospitals before she was finally
diagnosed with a carotid dissection. It
would be another year before she was diagnosed with FMD as the cause of the
dissection. These experiences are common
in the female FMD community. Dr. Olin, a
leading expert in FMD, states that one of the biggest mistakes made by
physicians is telling patients that their symptoms are all in their head
(Burton, 2009).
Reflections on the
Future
Women’s
health is starting to be a priority in both medical curricula and clinical
practice. McGregor et al. (2013) state
that changes are being made at the federal level, with the establishment of the
National Centers of Excellence in Women’s Health in academic medical centers,
focusing on gender disparity in the education of physicians (p. 3). Dr. Olin et al. (2014) list, as one of the
top research priorities, studying the prevalence of FMD in the general
population of women aged 16-65 (p. 1069). Acknowledgment of bias in women’s medical care
and knowledge of FMD as a disease that is prevalent in women is vital to
decreasing misdiagnosis.
Conclusion
Do women with
fibromuscular dysplasia have an increased risk of an adverse event through
misdiagnosis due to gender disparity? Gender
bias in healthcare is evident throughout literature. What about gender bias in a disease that is
predominate in females? Could this
explain the delay in diagnosis that patients with FMD experience? The validation that comes from finally being
diagnosed is helpful, but the years of damage that has already been done to the
body, mind, and spirit, over a period of years, cannot be repaired so easily. Provider awareness of gender bias when women
present with symptoms of FMD will likely improve how patients are perceived. The acknowledgment that gender bias plays a
role in the misdiagnosis of FMD will be a factor in reducing the risk of an
adverse event. Nevertheless, until
funding of clinical research is secured in both women’s health and FMD, there
is little hope of improving the quality of life in this cohort of patients.
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