Is it POTS or Panic?

Now that my class is over in research methods, I can finally post a copy of the paper I worked on all of last semester. I ran into trouble when my computer crashed and I lost ALL of my work, so it wasn’t as good as I had originally planned but I still got an A on it.

So here it is – my research proposal.

Hope you enjoy.


Is it POTS or Panic?

Differentiating Between the Diagnostic Criteria for

Postural Orthostatic Tachycardia Syndrome and Panic Disorder

By The Undiagnosed Warrior

Abstract

The objective of this study is to identify features that could assist the medical community in correctly diagnosing and treating patients by differentiating between Postural Orthostatic Tachycardia Syndrome (POTS) and Panic Disorder (PD). According to Dysautonomia International (2014), approximately 85 percent of POTS patients have been told by at least one medical professional that their symptoms are “all in their head”. POTS patients are commonly misdiagnosed as having Panic Disorder (PD) because these two conditions share a vague presentation symptoms associated with autonomic dysfunction. Since the characteristics of POTS can be attributed to a number of differential disorders, the ability of a doctor to determine a definitive diagnosis for patients is becoming difficult and the consequences of misdiagnosis are monumental.  In testing physiological and psychological measurements, this study is expected to  identify specific characteristics associated to each disorder and, therefore, can be used to create concise diagnostic standards to be utilized by the medical community. Three study groups will be evaluated: POTS patients, PD patients, and a healthy control group. Subjects will be compared using formal medical diagnostic testing, including Tilt-Table Testing, Magnetic Resonance Imaging (MRI), and blood serology. Psychological measurements will be evaluated through self-assessment using the Panic Disorder Severity Scale (PDSS) and the Wahler Physical Symptoms Inventory (WPSI).  In identifying the key factors differentiating POTS and PD, this study could essentially contribute to the improved process for clinical diagnosis.


Is it POTS or Panic?

Differentiating Between the Diagnostic Criteria for

Postural Orthostatic Tachycardia Syndrome and Panic Disorder

Postural orthostatic tachycardia syndrome (POTS) is an exceedingly under-recognized form of dysautonomia, which is the umbrella term used to define a wide variety of conditions resulting from a malfunction in the autonomic nervous system itself. Dysautonomia can cause a severe range of symptoms as the autonomic nervous system controls the majority of the body’s primary automatic physiological processes, such as the ability to maintain heart rate, blood pressure, and even digestion. According to Dysautonomia International (2012), it is estimated that over 70 million people in the world live with some form of dysautonomia. Despite the prevalence of dysautonomia, it takes most patients years to get diagnosed due to a general lack of awareness between the public and medical professionals, leaving patients feeling abandoned in their health care (Dysautonomia International, 2014). Although there is no cure for any form of dysautonomia at this time, including POTS, research into these disorders is currently underway in hopes of developing better treatment plans for patients, in addition to improving the overall diagnostic methods currently utilized by the medical community.

The average amount of time for a patient to receive a diagnosis of POTS is about six years and approximately 85 percent of POTS patients have been told by at least one medical professional that their symptoms are either “all in their head” (Dysautonomia International, 2014) or they were sent for a psychiatric evaluation before they were diagnosed as having POTS.  Not surprisingly, POTS patients are commonly misdiagnosed as having Panic Disorder (PD) because the two conditions share a vague presentation of autonomic symptoms. In current practice, patients with POTS experience extreme delays in diagnosis or misdiagnosis due the complexities of their symptoms. The current diagnostic criteria needed to obtain an official diagnosis is defined by either an increase in heart rate of at least 30 beats per minute or the presence of a heartbeat exceeding 120 beats per minute occurring within a 10 minute period upon standing. In children and adolescents, a change of 40 or more beats per minute is needed since the variability of heart rate differs between adolescents and adults (Dysautonomia International, 2014).

The “gold standard” in diagnosing POTS is through the use of a tilt-table test, which monitors a patient’s vital signs as the table moves from a supine or lying down position to an upright position. A simpler and cheaper version of this test, called the “poor-man’s tilt table test”, is also useful in establishing a diagnosis and entails a similar process of measuring a patient’s heart rate and blood pressure while the patient is lying down, sitting up, and in a standing position. Additional methods include other forms of autonomic nervous system testing, such as Quantitative Sudomotor Axon Reflex Test (QSART), Thermoregulatory Sweat Test (TST), gastric motility studies, blood serology, and other forms of cardiac evaluation.

Although the severity of POTS can differ between patients, the more common presentation of this condition include the physiological indications of autonomic dysfunction, such as heart palpitations, dizziness, shortness of breath, decreased concentration, headaches,  gastrointestinal disturbances, fatigue, hypovolemia, blood pooling into the lower extremities, and episodic syncope or pre-syncope (Khurana, 2006). The symptoms of POTS are the result of the movement toward an upright position, like sitting or standing, and the onset of symptoms can either be acute or chronic depending on the underlying cause of the disorder. Given how often the triggering agent of this condition (sitting up or standing) occurs throughout events of everyday life and depending on the frequency and intensity of experienced symptoms, POTS can cause a great deal of dysfunction for those affected by this disorder.

According to Dysautonomia International (2012), approximately 25 percent of POTS patients experience such severe symptoms of autonomic dysfunction that they are considered to be permanently disabled.  Despite the intensity and significance of symptoms, POTS is namely an “invisible illness” because patients outwardly maintain a normal appearance. Since the disability in POTS patients is not initially clear to those around them, patients often are left feeling misunderstood by their family, their friends, and even those within the medical community. Delays in diagnosis are unfortunately quite common within the POTS community, especially during the early stages of this disorder.

According to Dysautonomia International (2014), about 34 percent of patients first noticed their POTS symptoms during their adolescent years (ages 13 to 19), although the average length of time between onset of symptoms and a formal diagnosis was approximately six years. Additionally, while only six percent of the POTS population received a diagnosis during their first physician visit, about 27 percent of patients needed to visit with 10 or more doctors and another eight percent had appointments with more than 20 doctors before they obtained their diagnosis. (Dysautonomia International, 2014). Doctors in the field of cardiology diagnosed the study subjects most often, with primary care physicians being the second most common category of doctor to make a diagnosis this condition. However, family practice or primary care physicians only identified the symptoms of POTS and provided a diagnosis in 12 percent of patients. Ultimately, the lack of resources and available doctors specializing in this disorder has continued to make it difficult for a patient with POTS to receive both the proper diagnosis and management of this condition. For example, one study showed that POTS patients had to travel quite a large distance from home just to receive treatment for this condition, with 50 percent of patients traveling over 100 miles, 21 percent over 500 miles, and eight percent over 1,000 miles (Dysautonomia International, 2014). Essentially, the lack of treatment available to POTS patients is a direct result of the limited number of doctors with the knowledge to both diagnose and treat autonomic nervous system disorders.

The reality is that there are too many obstacles that hinder doctors from identifying a definitive diagnosis. First, the primary characteristics of POTS can easily be attributed to a number of differential diagnoses, including those of a psychological nature. Therefore, in what should be a relatively simple and straightforward process, the diagnosis of POTS is then missed because the majority of medical professionals have not been properly educated or trained to detect specific disorders of the autonomic nervous system and many of them will not encounter a single patient previously diagnosed with dysautonomia, even after multiple years of practicing medicine. Determining a formal diagnosis is further complicated by the fact that the standard diagnostics ordered by physicians generally doesn’t account for modifications associated to postural or autonomic processes that are essentially typical of POTS. When labs and other testing return within normal limits and a diagnosis cannot be confirmed, the next option for physicians is to provide a referral to either a specialty clinic or for a psychological evaluation. For patients with POTS, it is usually the latter.

According to a study by Dysautonomia International (2014), approximately 83 percent of patients reported that they had been diagnosed with a psychiatric disorder at one time or another while seeking treatment for their symptoms. Since both POTS and panic disorder stems from the autonomic nervous system, causing similarities in the presentation of symptoms, it is also not surprising that the majority of patients were first diagnosed as having an anxiety disorder. Sadly, a large percentage of patients report that a medical professional had inferred that their symptoms were “all in their head” (Masuki et al., 2006). Although some patients seemed to show improvement following diagnosis and treatment of their POTS condition, a greater part of the subjects stated they either they felt the same or they got worse following intervention protocols. Researchers ultimately concluded that more awareness and education about POTS is needed throughout the medical community since “the lengthy delays that POTS patients experience in obtaining a diagnosis results in longer periods of suffering without proper treatment, increased costs to patients, and wasted healthcare resources” (Dysautonomia International, 2014, p. 21).

The likelihood of being misdiagnosed continues to grow every year in the United States, as the amount of pressure placed on the current healthcare system has doctors working harder than ever before. Current estimates imply that approximately 12 million individuals, or about one out of every 20 people, are misdiagnosed each year and perhaps even worse is the number of patients that never receive a diagnosis at all (Singh, Meyer, & Thomas, 2014). The consequences following misdiagnosis are monumental; a patient’s overall health or condition may not only deteriorate, but they could be at risk of losing their life as well. Not to mention that treatment between different diseases and disorders will almost always differ, especially in comparison to techniques involving physiological and psychological management.

The inability of doctors to generate an accurate diagnosis is mainly attributed to the deficient amount of time essential to thoroughly review a patient’s history and perform the physical exam, as well as order the necessary diagnostic tests to confirm potential or suspected diagnoses. Even then, some issues can remain undiagnosed if the labs fail to provide any valuable answers or the symptoms themselves are easily associated to a multitude of conditions, essentially making it nearly impossible to substantiate an appropriate diagnosis. Furthermore, the process of validating differential diagnoses is much more complex in patients whose underlying condition is either quite rare and, therefore, is not well understood by the medical community, or the presentation of symptoms involves a mixture of both physical and cognitive indications, where the origin of disease is virtually indistinguishable.

Take somatization disorder, for instance. According to Smith (2013), “somatization is a mechanism in which a patient with emotional pain exhibits that problem as if it were a purely physical problem, but no physical problem can be found. These complicated clinical problems don’t neatly fit into a specific diagnosis” (p.5). On the other hand, there is also a variety of biological disorders commonly associated with psychological symptoms. In multiple sclerosis, for example, patients may experience the loss of memory, verbal fluency, concentration, visual perception, and the ability to perform higher cognitive functions. Additionally, the etiology in a number of conditions is not currently known at this time (e.g. irritable bowel syndrome or chronic fatigue syndrome) and diagnosis can only be obtained through a process of exclusion (Smith, 2013). As a result of these intricacies in the developmental diagnosis, a substantial number of patients are mislabeled as having a psychiatric illness.

This is particularly prevalent in patients suffering from a variety of chronic symptoms, such as POTS or other rare disorders. “According to the National Institutes of Health (NIH), a disease is considered rare if it has a prevalence of fewer than 200,000 affected individuals in the United States and there are roughly 7,000 rare diseases currently identified” (National Organization for Rare Disorders, 2015, p. 1). Although POTS is still commonly considered a rare condition by the medical community, current estimates show that POTS may actually affect anywhere between 1,000,000 to 3,000,000 Americans, contrary to reported statistics on this disease (Dysautonomia International, 2012). The primary reason for this discrepancy is the direct result of the failure to diagnose or the misdiagnosis of this condition.

Patients with POTS are most often misdiagnosed as having PD primarily because of the resemblance of symptoms that two conditions share with one another. Like many other chronic conditions, both POTS and PD can initially present with seemingly vague and indiscriminate symptoms that could easily be misinterpreted as beings caused by a number of other disorders, both physical and psychological in nature. In both POTS and PD, patients often experience heart palpitations, shakiness, dizziness, nausea, shortness of breath, and abdominal distress. These symptoms, along with many others, occur in a chronic condition similar to POTS and PD because as a way to compensate for either a dysfunction or imbalance of other systems within the body. Prior research into the other forms of dysautonomia outside of POTS, specifically pure autonomic failure and vasovagal syncope, also exhibit either really high or really low autonomic response, causing similar symptoms (Umeda et al., 2015).. Therefore, it is essential to evaluate the activity resulting from the autonomic nervous system in order to fully understand the complexities with each condition.

The autonomic nervous system is one of the primary systems in the body and is responsible for the functioning of the majority of internal organs, including such structures as the “blood vessels, stomach, intestine, liver, kidneys, bladder, genitals, lungs, pupils, heart, and sweat, salivary, and digestive glands” (Merck Manual, 2015). Given the magnitude of the autonomic nervous system, it is further broken down into two smaller parts: the sympathetic and parasympathetic nervous systems. The sympathetic nervous system is primarily responsible for activities in response to perceived danger or emergency situations. For instance, the fight-or-flight response experienced during a panic attack occurs as the result of sympathetic activation. Alternatively, the parasympathetic lowers the sympathetic response in order to conserve energy. In parasympathetic activation, for example, a person’s heart rate will decrease and digestion may stall in order to restore the energy consumed during sympathetic activation. Essentially both systems balance one another to maintain appropriate response in both internal and external influences.  From a psychological perspective, however, it seems as though POTS differs substantially from the majority of the diagnostic criteria outlined for Panic Disorder (PD).

Where PD really begins to differ from POTS is in the psychological indications associated primarily emotional manifestations of PD, including the extreme feelings of fear or anxiety, losing control or going crazy, and even a fear of dying. According to the DSM-IV, the diagnostic criteria of PD either with or without agoraphobia includes the presence of both  recurrent and unexpected panic attacks that are not directly the result of substance abuse or a general medical condition (American Psychological Association, 2013). Additionally, individuals with panic disorder are often concerned and worried about the potential for a future attack, which can also lead to a variety of behavioral changes in an individual as a way to compensate or cope with the fear associated with panic attacks. For example, patients with panic disorder with agoraphobia avoid a place or a situation for fear of the embarrassment associated with panic.

Gabor (1996) states that the most common form of PD is what Gabor has termed as the cardiac attack, which is often associated with symptoms of dizziness or lightheadedness, similar in presentation to many of the symptoms associated with POTS. The characteristic symptoms of PD, such as feelings of fear or panic, don’t come until much later when treatment is sought for this disorder. Gabor (1996) also mentions that the symptoms of PD can initially present similar to a variety of other disorder, also analogous to POTS, including the presentation of an abdominal attack that is typically marked with bouts of nausea and a variety of other gastrointestinal manifestations. Although patients with POTS may experience feelings of fear or anxiety similar to those in PD, it is common in individuals with any type of chronic illness because of the uncertainty in life, particularly in regards to their health and future.

In order to differentiate between POTS and Panic disorder, it may be easier to study the pathophysiological abnormalities to identify specific characteristics that medically define each of these disorders. For instance, neuroimaging studies have identified specialized regions in the brain that are likely responsible for the dramatic nervous system response initiated in PD patients. It was theorized that POTS patients should also display similar abnormalities in the brain as well. One study did find significantly lower amounts of gray matter in POTS patients in comparison to the healthy control group, primarily in the right middle frontal gyrus, although the volume of gray matter in the striatium and the right middle temporal gyrus were higher (Umeda et al., 2015). Additionally, the quantity of white matter in the left middle temporal gyrus was elevated in POTS patients as well. More importantly, though, Umeda et al. (2015) determined that there was actually a negative correlation between left insula volume and anxiety or depression scores, furthering the idea for autonomic dysfunction to result from abnormalities in the brain. Still, historical research identified inappropriate neurological activity associated with the flight-or-fight response, common in PD patients, mainly occurs in the amygdala and this part of the brain has yet to be identified in any of the physiology of POTS.

Heart rate, on the other hand, may be an easier feature to study. Both POTS and PD are associated with cardiac abnormalities, like heart palpitations and shortness of breath, stemming from problems with regulations of the autonomic nervous system. . The implementation of assessing heart rate variability of patients with PD could potentially supplement other forms of diagnostic testing, including serological and biochemical measures, to confirm a more accurate diagnosis of these disorders.  For instance, a study by Ito et al. (1999) identified a significantly higher heart rate in LF, HF, and TP levels during the tilt table testing. Former research has shown that these levels may indicate over activity of the sympathetic nervous system, resulting in the increased potential for panic-like symptoms to occur. Surprisingly, though, no differences were noted between respiratory measurements of patients in either the tilt table test or when in a resting position. Ito et al. (1999) speculated that this is likely due to the activation and response of both the sympathetic and parasympathetic nervous system, which often accompany one another to maintain homeostasis within the body. Based on the results of this study, Ito et al. (1999) concluded that reactivity of both systems of the autonomic nervous system may be prevalent in the early stages of PD, which could easily account for any discrepancies found in the autonomic responses of individuals with a longer history of PD as illustrated in earlier research studies.

Another study by Cohen et al. (2000) found that PTSD patients had both higher and lower heart rate changes than those in the control group, which they believed to “reflect a basal autonomic state of hyeractivation” (p. 7). Additionally, they found that subjects in the PD group also had higher and lower heart rate variability in comparison to individuals of the  control group, but they showed less fluctuation overall than those with PTSD. The PD and PTSD experimental groups both demonstrated a significantly higher low-frequency (LF) and a lower high-frequency (HF) component of heart rate variability than those in the control group as well. During periods of recalling stressful events, the PD subjects displayed greater intensities of sympathetic activity and decreased parasympathetic activities, which coincided with the findings in the control group. The PD subjects had also demonstrated “the expected increase in sympathetic activity upon standing, indicating that this component of baroreflex response improved with treatment, so that is was comparable to that seen in healthy control subjects” (Cohen et al., 2000, p. 9). Ultimately, the results of this study provided support to the researcher’s hypothesis that there would be marked differences between the nervous system’s response in those with either PD or PTSD

Psychological variations may also assist in demonstrating measurable differences in the presentation of POTS and PD symptoms. For instance, although POTS patients often mention past experiences with some symptoms commonly associated with panic attacks, Khurana (2006) theorize that these feelings corresponded to  increased orthostatic intolerance since resolution of symptoms occurred directly after testing had completed or when the subjects were placed back into supine position. Also, the POTS patients had no prior family history of PD, as well as the fact that many of the common cognitive symptoms accompanying PD (such as fear of dying or feelings of detachment) were not reported by the patients with POTS either, indicating negative correlation between the disorders. As expected, no symptoms were noted in any member of the control group of this study. However, the most interesting and influential finding of this study was that personality patterns were different between the disorders, with POTS patients scoring within normal limits, along with the control subjects, when evaluated during personality assessments.

In a different study, Masuki et al. (2006) hypothesized that the orthostatic stress experienced by patients with POTS was not a result of anxiety. In order to test their theory, the researchers used a number of psychological assessments often used for clinical diagnosis of anxiety disorders. To measure mental stress, the researchers administered the Stroop colored word test on a computer while measuring the subject’s heart rate, arterial pressure, and forearm blood flow. Additionally, psychological variables were evaluated using multiple questionnaires, including the Anxiety Sensitivity Index (to measure anxiety related sensations within the body), the Body Vigilance Scale (also to measure body sensations), and the Coping Strategies Questionnaire Catastrophizing Scale (to measure levels of catastrophic thinking patterns) (Masuki et al., 2006). In concluding the study, Masuki et al. (2006) found that patients with POTS demonstrated minimal variability from those of the healthy control group, especially in relation to either mental stress scores or the psychological indexes used primarily to diagnose anxiety disorders. The main different between POTS patients and healthy subjects was only present in terms of physiological responses during orthostatic testing. Essentially, these findings indicate that POTS is very much a physiological disorder and, therefore, not a result of an anxiety disorder, despite their similarities.

Summary

POTS patients experience a variety of problems associated with the etiology of their condition. About 85 percent of POTS patients having been told that their symptoms are either “all in their head” or they were sent for a psychiatric evaluation before they were diagnosed as having POTS. Often physicians misdiagnose or fail to diagnose POTS due to the presentation of its symptoms. The problems associated with misdiagnosis are obvious, leading to delay in care and even death in some cases.

POTS patients are commonly misdiagnosed as having Panic Disorder (PD) because the two conditions share a vague presentation symptoms associated with autonomic dysfunction. Since the characteristics of POTS can be attributed to a number of differential disorders, the ability of a doctor to determine a definitive diagnosis for patients is becoming difficult and the consequences of misdiagnosis are monumental.  Past studies evaluating both POTS and PD have provided insight to why these conditions are often confused with one another.

Essentially, it is hypothesized that investigating the symptoms associated with the primary response of the autonomic nervous system in both PD and POTS will identify key features as to the etiology of each disorder. Through measuring both the physiological and psychology factors mutually associated with these disorders, the results of this study could essentially contribute to improvement in the process of clinical diagnosis, giving back hope to the patients and trust about receiving appropriate treatment.

Method

Participants

This proposed study plans to measure and define specific criteria that could easily differentiate a diagnosis of either POTS or PD. Subjects in this study will be recruited primarily through physician referral from the University of Colorado (UCH) Anschutz Medical Campus located just outside of Denver, Colorado. Three groups of volunteers will be evaluated for this study: patients with POTS, patients with PD, and a control group. The POTS group will include a total of 20 patients given a formal diagnosis of POTS by the Cardiac Electrophysiological program at UCH. The PD group will consist of a total of 20 participants as well, 10 of which have been diagnosed with panic disorder with agoraphobia and 10 without agoraphobia, all of which will be referred from UCH’s outpatient psychiatry services. The final set of test subjects will be the control group, consisting of healthy patients recruited through the primary care avenue within the UCH network.

Procedure

Upon receiving approval and funding for this proposed project, screening for final selection of participation in the study will take place. In order to be eligible for this study, all participants must undergo both a physical and psychological exam prior to the start of this study in order to exclude any underlying conditions or comorbid disorders that could potentially influence the outcome of this research. The physical exam will include several forms of medical testing, such as an electrocardiogram, urinalysis, and common blood screenings. The psychological screening will then evaluate patient on elements of personality and anxiety levels by utilizing the Psychological Screening Inventory (PSI) and the Beck Anxiety Inventory (BAI) (Benet, 2014). Individuals found to have comorbid disorders, whether biological or psychology would then be excluded from participating in this study. The pre-exam process will aid in the overall control of possible extraneous variables that could potentially damage the outcome of this study, such as alcohol and drug use, for instance.

Both biological and environmental controls will be put in place to help ensure validity in the results of this study. Measurements will be taken at a set time during the morning hours over a 3 days period. The will be asked to not eat, drink, smoke, exercise, sleep, or consume caffeine at least 4 hours prior to beginning the study, since each of these elements could potentially affect metabolic activities within the body and, therefore, influence the overall results. Also, participants will be placed in a supine position for a minimum 15 minutes prior to the start of any orthostatic testing to decrease autonomic activity resulting from external factors (e.g. stress from driving to the study) and to habituate them to a laboratory setting. Environmental controls will also include the pre-exposure of participants to the sounds and equipment in the laboratory for approximately 15 minutes before any measurements are taken for this study to lower levels of both anxiety and arousal often stimulated in unfamiliar environments. Also, a consistent temperature of 72 degrees Fahrenheit will be maintained in the room during testing, since research has shown that this specific temperature is optimal for the majority of the human population. All of these outlined procedures are necessary to assure both the accuracy and strength in the experimental design.

Measures

            This study will evaluate the three subject groups on a variety of physiological and psychological measures in order to compare and identify the essential differences between groups in hopes of classifying specific factors that can later be used to either diagnose or differentiate between the characteristics associated with both POTS and PD. Physiological variables will be evaluated using specific medical diagnostics, including the use of a tilt-table test, an MRI, and blood serum analysis.

A tilt-table test is often ordered to evaluate orthostatic intolerance and other potential causes of fainting. Following the initial control measures listed above, patients in this study will begin in a supine position for a minimum of 20 minutes. Heart rate, blood pressure, and respiration will be recorded throughout the study. Before moving each patient, blood will be drawn to measure baseline amounts of catecholamines, specifically epinephrine, norepinephrine, and dopamine. Measurements will continue to be taken on all variables at intervals of five minutes following tilt into an upright position. Subjects will be left upright for a total of 45 minutes and variables will once again be documented upon returning to a supine position. Any symptoms experienced throughout the test will also be freely recorded as reported by each subject. If clarification is needed, subjects will be asked to further specify experience of feelings.

In addition to testing for orthostatic intolerance in the tilt-table test, this study will utilize an MRI machine to determine if any regions of the brain show any type of abnormality or either an increase or decrease in activity which may potentially identify any of the two disorders of interest. Patients will be placed into the apparatus following initial control procedures. The first set of imaging will be taken in silence to get a picture of the brain without stimulation. Once this is complete, the patient will then listen to an assortment of music through the use of headphones, including classical music, pop music, country music, and heavy metal. Each genre is meant to represent common emotional responses known to either stimulate or diminish activity in different parts of the brain.

The psychological measurements of this study will include two types of assessments commonly used to evaluate the severity of symptoms as they are perceived by each individual participant. The two forms selected for this study include the Panic Disorder Severity Scale (PDSS) and the Wahler Physical Symptoms Inventory (WPSI) (Benet, 2014). Formatted as a self-reported questionnaire, the PDSS is a common instrument used by both medical and psychiatric professionals to assess and diagnose patients based on the levels of symptoms consistent of patients with PD. To review a list of questions measured by this questionnaire, please refer to table 1. Alternately, the WPSI is a test that is designed to measure both the degree of both physiological and somatic symptoms self-reported by each participant of the study, with focus on differentiating between potential psychological disorders and those of a somatic nature. Again, to review an example of questions reviewed in the WPSI assessment, please refer to table 2.

Discussion

Ultimately, the results of this study should support our hypothesis that there would be marked differences between the nervous system’s response in those with either PD or POTS when evaluated through neurological, psychological, and biological testing. It is expected that this study will clearly show how autonomic symptoms should differ, seeing as though POTS is a physiological dysfunction while PD is more consistent with fear and a catastrophic thought process. The medical testing proposed in this study is also expected to back up this idea.   By highlighting both the similarities and the differences between POTS and PD, the results of this study could help by easing the process for obtaining a clinical diagnosis while ensuring that patients with either disorder are not left misdiagnosed. Additionally, any contributions to the limited literature available on POTS can hopefully one day lead to a cure for this condition. Nevertheless, the main focus of this study still remains in isolating definitive features to differentiate POTS from PD.

Although this proposed study takes extra measurements to ensure validity, it is not free from limitations. For instance, despite the fact that the proposed research does consists of a relatively moderate population size given the complexities of this study, there is still the potential for bias in the selection of  participants since all of the patients are being recruited through physician referral of patients receiving treatment in one specific state – Colorado. Additionally, while this project attempts to eliminate patients with comorbid disorders during the initial selection process, the medical diagnostics used in screening may not identify every possible disorder that could potentially influence the results. Finally, self-reporting on the psychological assessments will be completed by paper and hand-scored for this project, and, therefore, could be subjected to operator error. Despite these limitations, though, this project opens up the possibilities for future research into the nervous system response seen in patient with both PD and PTSD.

Prospective research is going to be needed to better understand and implement the initial findings of this study, particularly in identifying additional differential diagnoses and comorbid conditions. Earlier studies have identified the possibility that patients can, in fact, have a dual-diagnosis of both physical and mental health related disorders, including patients with both POTS and PD. For instance, in a case study by Kataoka (2001) described a patient that not only met the diagnostic criteria both POTS and PD, but also demonstrated both disorders through a variety of testing. During a tilt-table test, the case subject began to experience symptoms almost immediately after initiating the head-up tilting of the table and the patient’s heart rate immediately increased from 79 beats per minute up to 140 beats per minute, confirming the diagnosis of POTS. Most interestingly, though, was that there seemed to a correlation between the number of symptoms experienced and the amount of time that the patient was placed in a head-up position. At the 21-minute mark, the patient had a “panic disorder-like reaction”, which consisted of crying, hyperventilating, numbness in the extremities, pre-syncope, and feelings associated with fear (Kataoka, 2011).

Also surprising was the fact that the whole time this exaggerated reaction was occurring,  the patient’s measurements of catecholamine blood serum levels increased along with her vital signs: epinephrine increased from 82ng/ml at baseline to 269 ng/ml; dopamine increased from <10 ng.ml to 28 ng/nl; and norepinephrine showed the most drastic change from 301 ng/nl to 585 ng/nl (although the serum concentration and the age on onset did not exemplify the diagnostic criteria for an extremely rare form a POTS known as hyperadrenergic POTS)  (Kataoka, 2011). Also, measurements of arterial blood gases throughout the study suggest that severe hypocapnia and a higher pH occur alongside the presence of an experienced panic attack (Kataoka, 2011). Based on the results of this study, Kataoka (2011) ultimately concluded that:

The etiology of the syncope was orthostatic tachycardia syndrome complicated by panic attack-associated hypocapnic hyperventilation, which presumably caused a greater degree of deep cerebral hypoperfusion than would be expected with orthostatic tachycardia alone, ultimately leading to the patient’s symptoms, including syncope. (p. 91)

Given this information, it may be wise to further assess these variables to see if there are changes across larger populations of study.

Finally, another approach that may be effective in evaluating both POTS and PD is to examine how cultural differences and expectations influence the presentation of symptoms in these disorders.  Although there have been a number of studies in more recent years that examined cross-cultural differences and their influence on anxiety disorders, this topic of study has not been actively documented in relation to patients with POTS.  However, one study did take a similar approach by assessing the presence of orthostatic intolerance during panic attacks. Hinton, Pollack, Pich, Fama, & Barlow (2005) chose to study Cambodian refugees due to alarge number of individuals of this population being treated in mental health clinics all over the United States. Cambodian refugees are predominantly diagnosed with Post Traumatic Stress Disorder (PTSD) and Panic Disorder (PD) at significantly high rates due to events occurring prior to arriving at the United States. According to Hinton et al. (2005), “trauma seems to predispose to panic attacks, initially, during the trauma, by conditioning arousal to specific interoceptive and exteroceptive cues, and subsequently, by producing persistent arousal” (p. 301). Additionally, the researchers believed that orthostatic intolerance occurred during these attacks based on the presentation of the perceived nature that the panic attacks were culturally specific, perceiving feelings of dizziness or instability as a primary symptom.

The results obtained by Hinton et al. (2005) did identify the positive correlation between orthostatic panic and catastrophic reasoning as the researchers originally hypothesized. Current amounts of perceived panic were elevated in the majority of refugees, demonstrating no difference between genders. Also, the SCL-90-R showed high levels of not only PTSD and PD, but generalized anxiety, phobic anxiety, somatization, and depression as well. According to Hinton et al. (2005), associated flashbacks and catastrophic cognitions not only mediated an increased orthostatic panic response but also seemed to be an indicator to overall psychological functioning as well.


References

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Cohen, H., Benjamin, J., Geva, A. B., Matar, M. A., Kaplan, Z., & Kotler, M. (2000). Autonomic dysregulation in panic disorder and in post-traumatic stress disorder: Application of power spectrum analysis of heart rate variability at rest and in response to recollection of trauma or panic attacks. Psychiatry Research, 96(1), 1-13. doi:10.1016/S0165-1781(00)00195-5

Craske, M., Wittchen, U., Bogels, S., Stein, M. & Lebeu, R. (2013). Severity Measure for Panic Disorder – Adult. American Psychiatric Association. Retrieved on October 11, 2015 from http://www.appi.org

DSM-IV. American Psychiatric Association. (1994). Diagnostic and Statistical Manual of Mental Disorders (4th ed.). Washington, DC.

Dysautonomia International. (2014). Physician Patient Interaction in Postural Orthostatic Tachycardia Syndrome [PDF document]. Retrieved from Lecture Notes Online Web site: http://www.dysautonomiainternational.org/pdf/PhysicianPatientInteractionInPOTS.pdf

Dysautonomia International (2012). Ten Facts Doctor’s Should Know About POTS. Retrieved on October 4, 2015 from http://www.dysautonomiainternational.org/page.php?ID=180

Gábor, S. P. (1996). The cerebral and abdominal forms of panic disorder and their differential diagnosis. European Neuropsychopharmacology, 6, 205-206. doi:10.1016/0924-977X(96)88267-8

Hinton, D. E., Pollack, M. H., Pich, V., Fama, J. M., & Barlow, D. H. (2005). Orthostatically induced panic attacks among Cambodian refugees: Flashbacks, catastrophic cognitions, and associated psychopathology. Cognitive and Behavioral Practice, 12(3), 301-311. doi:10.1016/S1077-7229(05)80052-5

Ito, T., Inoue, Y., Sugihara, T., Yamada, H., Katayama, S., & Kawahara, R. (1999). Autonomic function in the early stage of panic disorder: Power spectral analysis of heart rate variability. Psychiatry and Clinical Neurosciences, 53(6), 667-672. doi:10.1046/j.1440-1819.1999.00623.x

Kataoka, H. (2011). A case of orthostatic tachycardia syndrome presenting with panic attack during tilt table testing. Cardiology, 120(2), 91-94. doi:10.1159/000333131

Khurana, R. K. (2006). Experimental induction of panic-like symptoms in patients with postural tachycardia syndrome. Clinical Autonomic Research, 16(6), 371-377. doi:10.1007/s10286-006-0365-0

Low, P. (2015). Overview of the Autonomic Nervous System. The Merck Manual of Diagnosis and Therapy Online (Professional Edition). Retrieved on October 11, 2015 from http://www.merckmanuals.com/home/brain-spinal-cord-and-nerve-disorders/autonomic-nervous-system-disorders/overview-of-the-autonomic-nervous-system

Masuki, S., Eisenach, J.H., Johnson, C.P., Dietz, N.M., Benrud-Larson, L.M., Schrage, W.G… Joyner, M.J. (2006). Excessive heart rate response to orthostatic stress in postural orthostatic tachycardia syndrome is not caused by anxiety. Journal of Applied Physiology, 102, 896-903. doi: 10.1152/japplphysiol.00927.2006

National Organization for Rare Disorders (2015). Resources and Frequently Asked Questions. Retrieved on October 4, 2015 from https://rarediseases.org/for-patients-and-families/information-resources/resources-faqs/

Singh, H., Meyer, A.N., & Thomas, E.J. (2014). The frequency of diagnostic errors in outpatient care: Estimations from three large observational studies involving U.S. adult populations. BMJ Quality & Safety, 0: 1-5. doi: 10.11136/bmjqs-2013-002627

Smith, C.W. (2013). The real problem with misdiagnosis. Journal of Participatory Medicine. Retrieved on October 4, 2015 from http://www.medscape.com/viewarticle/782258.

Spector, P.E. & Jex, S.M. (2007). Physical Symptoms Inventory: PSI Page. Retrieved on October 11, 2015 from http://shell.cas.usf.edu/~pspector/scales/symppage.html

Umeda, S., Harrison, N. A., Gray, M. A., Mathias, C. J., & Critchley, H. D. (2015). Structural brain abnormalities in postural tachycardia syndrome: A VBM-DARTEL study. Frontiers in Neuroscience, 9:34.  doi:10.3389/fnins.2015.00034


Table 1

The Panic Disorder Severity Scale (PDSS)

PD

Table 2

Physical Symptoms Inventory (PSI).

During the past 30 days did you have any of the following symptoms? If you did have the symptom, did you see a doctor about it?

During the past 30 days did you have?

No Yes, but I didn’t see doctor Yes, and I saw doctor
1. An upset stomach or nausea
2. A backache
3. Trouble sleeping
4. A skin rash
5. Shortness of breath
6. Chest pain
7. Headache
8. Fever
9. Acid indigestion or heartburn
10. Eye strain
11. Diarrhea
12. Stomach cramps (Not menstrual)
13. Constipation
14. Heart pounding when not exercising
15. An infection
16. Loss of appetite
17. Dizziness
18. Tiredness or fatique

All scales are copyright Paul E. Spector and Steve M. Jex, All rights reserved, 1997.

A Highly Underdiagnosed Form of Dysautonomia

This semester, I am taking a course in physiological psychology. Although this class entails an incredible amount of work (as my teacher is the head of the whole psychology department at my school), it definitely appeals to me since the majority of the course material involves learning about the functions occurring in the human body, the chemical and biological processes that take place, and how all of this influences the brain and individual behavior. Needless to say, it’s right up my ally. One of our weekly assignments is to submit a reaction paper to a research article that relates to the particular topic of the week. So last week, the topic was on the human nervous systems. Obviously, I chose to review an article on POTS and Dysautonomia. I figured that it was a great way to spread awareness to both my professor and fellow classmates on this condition, seeing as though we were only learning about a FUNCTIONING autonomic nervous system. Anyways, I thought I’d share it, even though the article I reviewed had to be torn apart due to some inconsistencies in the writing. Nevertheless, I  figured it was still relevant since I finally got my official diagnosis of POTS after so many years and many of my friends/followers also live with some form of dysautonomia. Hope you enjoy!


dysautonomia


Postural Orthostatic Tachycardia Syndrome:

Reviewing Research on a Highly Underdiagnosed Form of Dysautonomia

            Health officials in the U.S. define a rare disease or disorder whenever it affects fewer than 200,000 people. However, it has been estimated that over 500,000 people in the United States are living with a debilitating condition known as postural orthostatic tachycardia syndrome (POTS), despite the fact that this disorder is still considered medically to be a rare condition. This is primarily a result of a doctor’s misdiagnosis or failure to diagnose. More often than not, patients receive a psychological diagnosis first, mainly as having some form of anxiety disorder, long before they obtain an actual diagnosis of having POTS. The average number of years it takes to get a diagnosis of POTS after onset of symptoms is about four years, alt hough I personally lived with this condition for almost 20 years before receiving my diagnosis two weeks ago. Given the limitations that this disorder can cause in an individual’s quality of life, more recognition and awareness in the medical profession is essential, including research into developing a diagnosis and treatment plan for this disorder. In the article, Postural orthostatic tachycardia syndrome: an under recognized disorder, the authors discuss the clinical manifestations of POTS syndrome, as well as clinical testing methods and patient outcomes. (Pandian, Dalton, Henderson, & McCombe, 2007).

POTS syndrome is a form of dysautonomia, which is a term used to describe medical conditions caused as a result of a dysfunctional autonomic nervous system. The autonomic nervous system is responsible for most of the automatic functions in the human body, including heart rate, blood pressure, temperature regulation, digestion, and even breathing. In dysautonomia, however, anyone of the autonomic functions can be dysregulated. In POTS, for instance, symptoms are defined through orthostatic intolerance where an individual develops “excessive tachycardia and symptoms of cerebral hypoperfusion on standing” (Pandian et al., 2007, p. 529). Often, those with POTS will experience dizziness and fainting shortly after they stand up, due to the increase in heart rate and decreased in blood pressure caused by blood pooling away from the brain and into the lower extremities instead. Given that POTS is an autonomic disorder, patients can additionally experience other wide-spread symptoms, including symptoms of gastrointestinal disorders, migraines, hot and cold intolerance, chronic fatigue, exercise intolerance, hypoxemia, and shortness of breath. While there is no cure for POTS, researchers are investigating therapies to reduce the amount and level of symptoms experienced.

While a lot information is still unknown about POTS, the researchers note in the article that there are several ideas as to the possible causes of this condition. This was most interesting, as many research studies have not focused on the potential for varying causes of POTS. One thought is that POTS patients have a “form of autonomic neuropathy manifested by an inability of the peripheral vasculature to constrict adequately in response to orthostatic stress”, which would explain the increasing heart rate and lowering blood pressure (Pandian et al., 2007, p. 529). Other views mentioned in the article focused on neurotransmitters and hormones as being the primary cause of POTS. For instance, one idea is that POTS is a type of β-adrenergic receptor hypersensitivity. Another is that “the serum catecholamine levels are often significantly increased when upright” (Pandian et al., 2007, p. 529). Finally, other theories mentioned in this article included patients having a genetic predisposition to developing the disorder, POTS being secondary to other disorders (like diabetes, sarcoidosis, or Sjorgen’s syndrome), or POTS being an unrecognized autoimmune disorder. Personally, I believe POTS, like many other neurological and physical disorders, can be explained by the combination of environmental, biological, and genetic situations.

In order to study symptomatology and medical testing on POTS, Pandian et al. (2007) studied 250 patients from 2003 to 2006. Researchers’ recorded demographic details, such as age and sex, as well as the patient’s individual symptoms, including duration, severity, and frequency of symptoms. Tests were performed to test level of autonomic dysfunction by using a tilt table study, heart rate response to deep breathing, and the Valsalva maneuver test. Researcher were able to assess outcome on the patient’s ability (or disability) to function normally; by being able to stand up without symptoms for 30 minutes, to maintain work, and ability to participate in recreational activities, without worsening of symptoms. The results of this study determined that the duration of symptoms lasted anywhere from three months to 20 years. “The common presenting orthostatic symptoms were light-headedness (100%), palpitations (90%), pallor (90%), weakness (80%), and clammy skin (80%). The mean heart rate increment during the tilt study was 51.7 14.3 b.p.m.” (Pandian et al., 2007, p. 531). Other symptoms noted by patients during the study were dry eyes and mouth, decreased saliva and sweating, nausea, bloating of the abdomen, cramping, diarrhea, constipation, weight gain or loss, satiety, and vomiting. Finally, out of the 250 participants, only five of the patients were functioning normally at follow-up visits as defined by the outcome criteria and four patients were considered worse off than when the study was initiated. Some of these patients were managing their POTS with pharmaceuticals, including beta-blockers, antidepressants, fludrocortisone, and pyridostigmine. Pandian et al. (2007) concluded that not only is POTS an under-recognized disorder presented by a variety of symptoms, but it also has the potential to be treated, although with inconsistent outcomes.

Overall, while I believe that this article provided a great deal of detailed information regarding the symptoms associated with POTS, there was quite a bit of data that was lacking as far as the actual research that was performed. The tests used on the patients are diagnostic tests that are considered current protocol in the diagnosis of POTS, and by using patients who have already received this diagnosis, the findings in this study are relatively insignificant, other than they confirm a patient’s original diagnosis. Also, in the introduction researchers mentioned the potential causes of POTS syndrome, but those ideas were not investigated in this study. It would have been interesting to compare the measurements of symptoms with the various testing they ran to other studies, such as DNA or blood testing, to determine the genetic or neurological causes of POTS. Considering I recently had my cortisol levels ran, which came back as being particularly low, my personal theory is that the neurotransmitter and hormone connection must have some sort of significance in the development of both POTS and other forms of dysautonomia, in addition to having a genetic predisposition to the disease that is environmentally influenced.

Also, Pandian et al. (2007) use the phrase ‘under recognized’ in their title and throughout their paper as a problem being associated with POTS, although they did not pursue studying the actual number of individuals with POTS that may not have received a diagnosis as of yet. While the researchers were using a true statement, since lack of a diagnosis has been identified in a variety of other research, I would have liked to have seen a random selection of participants, consisting of the general population, also studied using current diagnostic tests for POTS. Still, any research or information that brings awareness to this highly underdiagnosed and limiting health condition benefits all of those affecting by POTS, including the ones still left undiagnosed.

References

Pandian, J. D., Dalton, K., Henderson, R. D., & McCombe, P. A. (2007). Postural orthostatic tachycardia syndrome: An under recognized disorder. Internal Medicine Journal, 37(8), 529-535. doi:10.1111/j.1445-5994.2007.01356.x


Oh doctor, doctor, I must have gotten this sick somehow. I’m going to ask you a series of questions, and I want them answered on the spot, right now.

Patience has never been a virtue of mine, as many of my friends and family can attest to.

But I feel that I have been extremely patient with my doctors, maybe more than I should be.

I wasn’t planning to do an update until I received the final confirmation from my doctors,

but it seems like that won’t be happening anytime soon,

so I figured that no time is better than the present.

It’s been just about a month since my abnormal tests came back. I have left multiple messages for both doctors, one every week, hoping for a callback or at least an idea as to whether or not I need to come in for an appointment to discuss the test results in person.However, all I got was silence. I literally felt my blood boiling each day that went by that I didn’t receive a call. 

I’d whine about how:

“They’re delaying my care.”

“Sure, just because I’ve been sick forever, no reason to rush or anything.”

“I don’t know how doctors can get away with not calling when tests are abnormal.”

And, of course, the classic:

“I’m going to die before they ever figure out what this is.”

I was feeling sorry for myself, to say the least. I felt alone, abandoned, and lost as to what to do next. It was really starting to mess with my head. I hated being this way but I hated how these doctors were making me feel even more.

I felt like they were completely disregarding everything I have gone through

in my search for a diagnosis.

The years of life I have lost due to being sick.

The hours spent driving to appointments.

The amount of time in hospitals and doctor’s offices.

The high levels of radiation and all the discomfort in medical testing.

The countless pills prescribed, which often caused more side-effects than actual relief.

The procedures and surgeries, which also didn’t fix my disease.

The friends and family I lost, because they didn’t understand.

My career goals and aspirations that have been placed on hold.

Everything I once was and what I could be.

My whole life is on hold!

And here, right in front of you, are these abnormal tests

that provide answers to my chronic, undiagnosed illness

that I’ve fought so hard, for so long, to find

and you can’t pick up the phone to call me? 

Maybe I am being impractical,

but a month spent waiting seems way too long.

That’s a month to complete more testing or get second opinions.

Four weeks of treatment or management.

30 days of my life lost, left waiting.

FINALLY, a nurse from my GI, Rheumatologist/Immunologist, and Cardiologist’s office called, but she only caused  more confusion and frustration than before. She tells me my fecal cultures came back normal, which I already knew. She then says my cortisol levels were low (again, I’ve had my test results for weeks) and the doctor would like me to rerun it to be sure. She’s going to mail the paperwork so I can at least have my blood drawn in town, instead of driving over an hour. Great, thank you. She also tells me to stop taking my lupus medications… wait, what? The GI doctor and the Rheumatologist/Immunologist agree that I should stop taking them, because I’m not autoimmune. Um… ok. How come she originally, as did all the other doctors, think it was seronegative autoimmune disease? Specifically, Lupus?  I’m not in the mood to argue, I’ve been feeling terrible. I ask if I stop them forever, she says yes and the conversation is over.

She calls again yesterday, just to tell me to stop taking the Lupus medications… again. Yes, you told me the day before. Both doctors think it may be causing my painful and urgent diarrhea.Um, I have had on and off urgent diarrhea for… like… ever. But ok. So it’s not because it can’t possibly be autoimmune? I’m confused. She asks if the medication has helped. I said I’m over the initial side effects. I’ve had constipation for the past few days, so don’t think it’s causing diarrhea, and I haven’t had any extreme photosensitivity as in the past weeks, but still having outbreaks of rashes. I don’t know what is causing what anymore. My symptoms are too random and sporadic. She says to call next week or the week after, let them know if being off the medication makes me feel better or not, and then we will restart it if it’s not the problem. Wait… so let me get this straight? Now you’re taking me off it, to see if it’s having side effects I have had forever, only to restart it and have to adjust to it once again? Are you kidding me? Well, it may be affecting your cortisol levels, so they want to see if I stop it if it’ll change my blood test.  I am beyond confused and frustrated at this point. I went and redid my cortisol blood test this morning, so I guess we’ll just wait and see what that tells us.

Still haven’t gotten a call from the vascular surgeon. My CT Angiography was the one I was MOST worried about and  I fretted every night about him not calling me to discuss the findings. . Finally fed up, having left four messages now, I called and scheduled an appointment to discuss. I don’t know whether or not I trust him to treat at this point, given the lack of respect of promised phone calls with no answers, but he may just not know what to do or say about the finding. This is the doctor who didn’t believe in Nutcracker Syndrome at all for his own, valid reasoning, but admitted my original CT Scan showed the most convincing case of Nutcracker Syndrome that he has seen in over 30 years. He ran the CTA to “prove him wrong”. On the order form for the test, he even wrote “to exclude Nutcracker Syndrome”, instead of “evaluate for Nutcracker Syndrome”. He was really convinced I couldn’t have it, it’s too rare, and most vascular surgeons don’t think it’s a real thing.

Well, guess what? I proved him wrong. Not only that, they found two more (even rarer)  vascular compressions. The radiologist noted both May-Thurner Syndrome and Superior Mesenteric Artery Syndrome, although the Nutcracker Syndrome is the most extensive. So perhaps, maybe he is lost as to what to do or say at this point, I don’t know. But I’d rather him tell me that if that is, in fact, the case, rather than be silent about it. But I have an appointment now, so he can’t ignore me. So we’ll see how that goes.

I also had my consultation with cardiology last week. It had gone way better than expected and I really liked the doctor. He not only listened to me, he caught things other cardiologists had  missed in the past, and had my notes completed (and accurate) by the end of the day. I was fearful for this appointment, as I have not had the best luck with cardiologists in the past. They always say they hear a “murmur” or “valve issue”, order tests, and then call me crazy. This has happened on multiple occasions, both in my teens and early twenties. So you can see how I’d be nervous about going straight into an appointment saying “I think I have POTS syndrome and so does my neurologist and the immunologist (although she seems to have forgotten EVERYTHING she told me in my last appointment, so maybe she doesn’t think I do anymore, who knows)”. I show him the letter from the neurologist and my ‘poor man’s tilt table test’ results. He says that it looks like I have POTS, but he wanted to have some “orthostatic fun” in the office just to see. He measured my heart rate and blood pressure while laying down, sitting up, and standing.  Sure enough, my blood pressure dropped really low and my heart rate increased up to 150. Yep, he’s pretty convinced that it is POTS, but because of the missed information in previous cardio tests, he wants to rerun them again just to make sure it’s not something “easier” or misdiagnosed.

In my echocardiograms from 2005 and 2007,  he noticed that there was what he called “abnormal electricity” shown, but the EKG didn’t catch it, so it was dismissed. It happened again in my 3D echo from last year. Also, the 3D echo from 2014 showed I had pericarditis, which is a typical sign of autoimmune (particularly Lupus), but, of course, need to rule out other possible causes as well. And finally, my halter monitor from 2007 showed abnormalities and heart beats exceeding 160 bp, which was also dismissed during that time. So he ordered a 3D echo again, to see if the pericarditis has cleared on its own or if it’s gotten worse. He also wants to evaluate the possibility of a hole in my heart (which many people are born with, although it usually clears up on its own as you get older) since they can’t confirm the cause of my hypoxemia, other than the mild sleep apnea that was confirmed through my sleep study last year (although he doesn’t believe that is what is causing it, because again, it was very mild and happens sporadically during the daytime as well). So I’ll be back in the hospital tomorrow to complete all of these cardio tests. If  all the differential diagnoses are excluded, then he will be referring me to the dysautonomia clinic for further treatment, but was comfortable enough to put down Postural Orthostatic Tachycardia Syndrome as an official diagnosis. Again, something I have been saying since I started looking for answers. Finally! So we’ll see how testing goes tomorrow and I guess go from there.


So what does this all mean?

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It’s more than just ONE cause, obviously.

There are multiple conditions feeding off of one another, 

making my conditions not only rare,

but also complicated to treat and manage.

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Here’s what is on the table (so far):

Dysautonomia:

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More specifically —> Postural Orthostatic Tachycardia Syndrome (POTS):

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Nutcracker Syndrome:

“The nutcracker syndrome is quite a rare condition. It is due to the compression of the distal segment of the left renal vein (LRV) between the superior mesenteric artery (SMA) and the aorta (also called left renal vein entrapment).  This syndrome needs treatment when symptoms are disabling” (Hartung, O., 2009, p. 246).

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Superior Mesenteric Artery Syndrome (SMAS):

“Superior mesenteric artery syndrome (SMAS) is a digestive condition that occurs when the duodenum (the first part of the small intestine) is compressed between two arteries (the aorta and the superior mesenteric artery). This compression causes partial or complete blockage of the duodenum. Signs and symptoms may include abdominal fullness; bloating after meals; nausea and vomiting; and abdominal cramping that may be helped by lying in certain positions.” (NIH Office of Rare Diseases, 2014)

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May-Thurner Syndrome:

“May-Thurner syndrome (MTS) is caused when the left iliac vein is compressed by the right iliac artery, which increases the risk of deep vein thrombosis (DVT) in the left extremity. DVT is a blood clot that may partially or completely block blood flow through the vein. Even though DVT itself is not life-threatening, the blood clot has the potential to break free and travel through the bloodstream, where it can become lodged in the blood vessels of the lung (known as a pulmonary embolism). This can be a life-threatening condition” (ClevelandClinic.org)

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Symptoms and Causes of Low Cortisol Levels:

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A lower than normal level may indicate:

  • Addison disease, in which the adrenal glands do not produce enough cortisol
  • Hypopituitarism, in which the pituitary gland does not signal the adrenal gland to produce enough cortisol
  • Suppression of normal pituitary or adrenal function by glucocorticoid medications including pills, skin creams, eyedrops, inhalers, joint injections, chemotherapy

Other conditions for which the test may be ordered include:


Is this a final diagnosis?

Knowing how thing have gone in the past, it’s  highly doubtful.

Maybe there is more to the story…

Maybe there is less significance…

Only time will tell.

Again, I am left waiting…


Now if only I can get all my doctors organized and working together, maybe I can clear up what this all means and what needs to be done next. Surgery? Medications? Again, who knows… 

While I DO know for sure that NONE of these diagnoses will ever be “cured”, I’m hoping we can at least find a way to manage everything so I could hopefully live a semi-normal life again.

I still have to do a hydrogen/methane breath test next week, as well as upcoming appointments the week after with dermatology (to run biopsies on my skin rashes, hopefully to “catch” the autoimmune disease that’s hiding in my skin) and a follow-up with the vascular surgeon. Plus the results from the cortisol testing I did today and the cardio tests tomorrow. Let’s hope we can get the pieces put all together and figure out what’s next as far as treatment goes.

I guess I’ll just have to wait… something that is unfortunately becoming entirely too common at this point, but at least we’re getting somewhere… slowly. 

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Life’s a game made for everyone…


I struggled with this post, unsure how I wanted to approach it… but really,  I’m not sure exactly what to feel myself. I’m in a whirlwind of emotions right now. And I’ve started new medications, which are wreaking havoc on my body now as well. It’s like my world has sucked me into a whirlwind and spit me right back out again. And there’s so many questions left up in the air and so many more answers I am still searching for…

But it looks like we have several possible diagnoses


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i·ro·ny1
ˈīrənē/
noun
 
  1. the expression of one’s meaning by using language that normally signifies the opposite, typically for humorous or emphatic effect.
    ““Don’t go overboard with the gratitude,” he rejoined with heavy irony”
    synonyms: sarcasm, causticity, cynicism, mockery, satire,sardonicism

    “that note of irony in her voice”
       
    • a state of affairs or an event that seems deliberately contrary to what one expects and is often amusing as a result.
      plural noun: ironies
      “the irony is that I thought he could help me”
      synonyms: paradox, incongruity,incongruousness

      “the irony of the situation”

My post from May 31  is only more proof that living life with a chronic and undiagnosed illness is both a comedy and tragedy wrapped all in to one. 

Monday, after meeting with my immunologist/rheumatologist, I finally received a partial diagnosis and started on a new treatment plan. Sure enough, they say it’s Lupus, despite what previous blood tests have shown. Not only is it ironic that this comes one day after my post about NOT having Lupus, but May was Lupus Awareness month and I got my diagnosis on June 1. I dunno if it’s coincidence but life is really funny, I tell ya.

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They’ve run some more tests they sent out to a special lab that we are also waiting for further confirmations about the extent of autoimmunity I have. So far, based on general blood tests, the lupus does not seem to be affecting the majority of my organs, which is good news and gives me a lot more options as far as treatment goes. They honestly believe it’s a secondary condition that came out with all the crazy symptoms lately from all the stress on my body for being so sick for so long. We unfortunately have no answers as to what caused the anaphylaxis reactions at this point, but because I had three in a week, she’s put me on an aggressive treatment plan for my allergies to stop it before it gets any worse. Ultimately, without me ever mentioning my own personal thoughts, she drew the conclusion to dysautonomia being the primary source of my illness (which I have said behind closed doors for a long time now). She did get me a referral to neurology for the cognitive effects, which may or may not be Lupus related.

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Luckily, I was able to get in quickly with the neurologist and saw him on Wednesday. Again, of his own conclusions after working at the Cleveland Clinic prior, he says it sounds like P.O.T.S. as primary. He wants me to start Beta Blockers once the Lupus drugs and allergy meds have been adjusted, which makes sense because he wants to know what side-effects I am getting from the anti-malaria drugs before starting anything new. Plus I have already scheduled with the cardiologist, so he’d like to see what a tilt-table test will show before doing any radiology. However, none of the potential diagnoses explains the olfactory hallucinations, so he did order an EEG just to rule out possible seizures. I also have my first appointment with the vascular surgeon next week to discuss what the extent of the Nutcracker Syndrome is playing into all of this. 

Between the side-effects from the 12 medications I am taking daily now and all the stress of continued appointments, work, life…. I dunno how I am supposed to feel. Part of me is happy to FINALLY have progress and a name and something I can tell people… that they have probably heard of. I got my validation.  But then I feel silly and stupid for being happy of having an incurable disease.  Then the other part of me is sad because there isn’t a cure. But it is MANAGEABLE. But then I have so many questions still, and so many things are left up in the air. I feel SO CLOSE, yet SO FAR.  See my dilemma? I guess I need to be a patient-patient, but it’s hard. There’s been so many changes this week with work, school, and doctors that I need to update, but I spent about 6 hours in the bathroom today with side-effects and eventually had to go back to bed. I’m tired and worn, but I have hope.

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 I have so much to say, but no way to say it.  I’m almost numb at this point because it doesn’t seem real. And maybe it’s nothing that they say. Maybe it’s something completely different after the next round of testing comes through. But for now, it has a name.

Lupus.

Autoimmune.

Possible P.O.T.S.

Nutcracker Syndrome

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Feeling my way through the darkness
Guided by a beating heart
I can’t tell where the journey will end
But I know where to start

They tell me I’m too young to understand
They say I’m caught up in a dream
Well life will pass me by if I don’t open up my eyes
Well that’s fine by me

[2x]
So wake me up when it’s all over
When I’m wiser and I’m older
All this time I was finding myself
And I didn’t know I was lost

I tried carrying the weight of the world
But I only have two hands
Hope I get the chance to travel the world
But I don’t have any plans

Wish that I could stay forever this young
Not afraid to close my eyes
Life’s a game made for everyone
And love is the prize

[2x]
So wake me up when it’s all over
When I’m wiser and I’m older
All this time I was finding myself
And I didn’t know I was lost

Didn’t know I was lost
I didn’t know I was lost
I didn’t know I was lost
I didn’t know (didn’t know, didn’t know)