Woes and Ailments and Neurocardiogenic Syncope
We first realized my sister, Sara, had a problem when she was three years old. My mother was making lunch, with her back turned, and Sara was crying for her food. Then she heard the small word, "Mommy," and a thud. She turned to see Sara on her back, unconscious and convulsing. My mother was very frightened. But trips to the doctor didnt turn up anything unusual, and the incident didnt repeat. At least, not in the same fashion. Not too long afterwards, she was at the doctors office, and they were taking blood from her finger--the well-known method of a quick jab to the finger, then a straw applied to the wound to capture the blood. I always kind of liked it, sign of my morbidity I suppose. Sara, on seeing the blood, immediately fainted. The doctor was very surprised. He said most children find it fascinating to watch the little straw fill up. This incident we attributed to the sight of blood.
Over the years, Sara developed an ability to faint based on this early family diagnosis. If she saw any amount of blood, her eyes would start to roll back, her skin would pale, and she would crumble. All very psychological, we decided, and something she should be able to overcome. To top it off, she developed a variety of other physical ailments and woes that were purely imaginary (we decided), and she gained a reputation as something of a hypochondriac. My uncle recently emphasized this family-wide view. He was remembering the three of us--myself, our middle sister Mariah, and Sara--when we were little. He said that I was always quiet and independent. Mariah--whose birth announcement said "Look out world! Here I come!"--was always moving and going and wanting to be the center of attention. And Sara? Sara was always saying, "Owie, woo-woo!" Which, in her baby-speak, meant some part of her hurt. It was very telling that we all grew up to fit these stereotypes of ourselves.
This idea that her ailments were all imaginary began to change several years ago, though. Sara was complaining about pains in her knee, which we all put down to the funny way she would sit in a chair while watching the television. Instead of taking it seriously, we just chided her. Finally, my mother grew tired of hearing her complain, and took her to a doctor, if only to prove to her that there was nothing there. To Saras satisfaction, the doctor said there was a dislocated disc in her knee. Were not sure how it became dislocated, but it was indeed dislocated. Sara was going to require constant physical therapy and care for years to come. We were all surprised--there really was something wrong with her!
My parents started to pay closer attention to her complaints. If one pain was genuine, there might be something behind the others. My mother became increasingly concerned by the number of times Sara would come upstairs from her room looking weary and complaining of a headache. She hadnt thought anything of it before--mom has a condition that causes her to be constantly fatigued, and thought that Sara had the same. But my sister would have no recollection of how much time had elapsed while shed been in her room. She could only recall a "funny" feeling, then a headache. My mother began to suspect that she was falling unconscious while alone, and was concerned about what this might signify. Sara had had other incidences that we knew of (which Ill talk about later). The idea of her fainting and no one knowing was frightening. So my mother finally decided to start getting answers. This was four years ago. Sara was fourteen at the time.
Now, in spite of the concern, my family has developed a rather morbid sense of humor about this whole ordeal. Partly because of Saras hypochondriac tendencies. Throughout the tests she was put to, she kept up the hope that whatever it was she had would be very serious. One concern was that she may have a brain tumor. The doctors gave her a CAT scan to rule out that possibility. The results came back negative, and it was agreed that more tests should be conducted. The day the results came back, Sara was very quiet on the drive home. My mother asked if she was worried about the other tests. "No," Sara said forlornly. "Im just sorry there wasnt a tumor."
Another suggestion was that it could be a form of epilepsy. She experienced mild seizures during unconsciousness (at least those episodes that we witnessed), and that seemed suggestive of such a condition. The doctors conducted an REM test--she had to fall asleep while being watched and monitored. In the days and weeks to follow, Sara was in a state of excitement. She was constantly coming up and down the stairs from our den with various volumes of the encyclopedia. Favoring her bad knee, she would hobble into the kitchen and show me the various kinds of epilepsy she may have. "See?" shed say cheerfully. "This type of epilepsy can be controlled by medication, but this one cant. I hope this is the one I have!" But again, the tests were negative--no epilepsy.
The final diagnosis came months later. It was decided that Sara had a condition called neurocardiogenic syncope (sink-o-pay). I was having difficulty remembering what it was called when my friends would ask about her condition, even though she had told me the name twice. One day, sitting in an A&W in Canada, I asked Sara again what it was she had. She sat up very straight, and taking the tone of a kindergarten teacher, she pointed first to her head, then to her heart, then wagged her finger up and down between the two, saying slowly, "Neuro-cardio syn-co-pe!" When I finally stopped laughing, she explained that the doctors said her heart and brain werent sending the right signals to one another. One would say, "Blood pressure has dropped!" The other would reply, "Ok! Shut down functions!" The result is, her blood pressure does drop, and she passes out. Sounds very logical. And scientific. And very Latin.
It just seems to be a big word to describe an indescribable condition. To this day, Sara is convinced that the doctors made it up. In looking up the name, Im almost certain this is the case. Its as if they couldnt find anything else close. A friend of mine gave me a good way of describing how we feel about this condition. She was doing a thesis paper for her Masters, and her professor was critiquing it. He pointed to a certain paragraph and said, "This is taking the place of something thats the same size, only different." When her brain cleared from the fog, he explained that she had put in the paragraph only as a way to transition from the preceding paragraph to the following. But it wasnt really a paragraph that reflected her ideas. In other words, there was something she needed to say, but the way she had said it wasnt accurate. Thats how I feel about the name given to my sisters condition: its taking the place of something similar, but different.
Syncope is defined in the Merck Manual as "a sudden, brief loss of consciousness, with loss of postural tone" (Beers and Berkow 1651). So, it means "to faint." Neurocardiogenic syncope (also known as vasovagal or vasodepressor syncope) is prevalent in young adults, especially females. It "usually occurs in the upright position as a result of an inappropriate vasomotor response allowing venous pooling" (Boehm and Kip). Okay, in English: a normal person standing upright wont pass out because the blood keeps flowing the way it should through the bloodstream. With neurocardiogenic syncope, the brain signals a decrease in blood pressure, which sometimes slows down the heart rate, and "triggers blood pooling in lower extremities" (Sadovsky). This means the blood isnt where it should be, and the person passes out.
The symptoms experienced by patients with neurocardiogenic syncope are pretty much in line with what Sara has described. She feels "weird." Not too descriptive. But her vision blurs, she feels twinges of nausea, and a slight pain in her head. All of these are common symptoms (Neurocardiogenic). Also, following her episodes she always has a headache, and feels heavy fatigue. Again, exactly in line with patients diagnosed as having neurocardiogenic syncope. But then, these are the same symptoms experienced by anyone who has a fainting spell.
There is one aspect of neurocardiogenic syncope that is initially inconsistent with Sara. According to my information, fainting occurs while standing. But a majority of Saras spells took place when relaxing or sitting. And for this to occur while sitting is very unusual (Neurocardiogenic). One such incident was at the Puyallup Fair. It was 11:00 at night, and she was sitting at a bench with friend, drinking coffee. She began to feel "weird" and said so out loud. Then she fell headfirst on the table, spilling coffee everywhere. Her friend scoffed, "Funny, Sara, now get up!" But when she looked, Sara was convulsing, and through a slit eyelid only the white of her eye was showing. When Sara came to, her friend was having hysterics. "What happened?" she murmured. She looked down at herself and saw the wet stains on her pants from the coffee. Suddenly mortified, she looked at her friend with wide eyes. "Did I pee my pants?" she whispered. When her friend shrieked out what happened, Sara just said, "Oh, weird. Hmm. Well, lets get home." Shes the most casual of all of us in her view of the situation.
It was this incident, coupled with her complaints of headaches, that triggered the rash of tests. The first test she was subjected to was tilt-table testing. Doctors use this method to determine the cause of syncope (fainting) in patients with multiple episodes. This is the most common--and most popular--method (Boehm and Kip; Vitacco-Grab and Metzler). Its also very inexpensive, which made it a viable one to use for Sara. During this test, the patient is strapped to a table and slowly moved to an upright position. Wires and monitors are attached. The test is to see at what angle a person faints, and how long it takes. Generally, the patient is given a certain chemical to aid the process, lifted to 80 degrees, and held at that position for 30 to 45 minutes (Boehm and Kip). The test is repeated depending on the rapidity of the patients response. The symptoms a patient experiences during the test include those common to fainting spells -- nausea, sweating, lightheadedness, etc ("Tilt-table Test"). But its not dangerous, or injurious. Or so they say.
Things went a little differently with Sara. Before the test, my mother had to sign a preliminary form. Part of it included a statement about "accidental death". When my mother asked about it, the doctor brushed it aside. "Not to worry, thats on all the forms. Theres no chance of that occurring with this test." So my mother signed. After all, her daughter is only lying on a table and being tilted upwards. Whats the worse that could happen?
In the test room, Sara was strapped in. The doctor explained one more time what would happen, and tried to reassure her. She was connected to different instruments, including a heart monitor; then the doctor turned the table on, and turned his back. The test had not even started. Within ten seconds, he heard a sound he had not expected to hear: the heart monitor indicated a flatline! My sisters heart had stopped. Later, she said she just felt woozy, things started to go black, and she heard the doctor shouting, "Put her down! Put her down!" After things were calm, she told him what she had experienced. She then watched, from her flat table, as he circled around her, staring, hand on his chin and eyes squinted. Every now and then hed ask a question. "You could still hear us?" When shed answer, hed say, "Hmm . . ." and continue circling. Sara had had the fastest response in history. So much for the popular method.
Apart from this, the CAT scan, and REM testing, the doctors also tested her pressure points. They would touch various parts of her body, and test her response. At one point, they touched her eyelids and she immediately fell to the ground. (My mother still has to fight the urge to try this again.) But what this was checking for, were still not certain. In my research, I couldnt find anything about neurocardiogenic syncope that even suggested touch and pressure points were responsible. This test was not even mentioned as one conducted for regular diagnosis of this or any other syncope condition.
Which is very interesting, considering that some of her episodes have been brought on by touch. One was at the Seattle Science Center when she was about 10. Our family was spending the day there, and we passed one of those "test-your-own-blood-pressure" machines--the kind with the big black tube which you insert your arm into and then press a button. It squeezes your arm and gives you a reading. Sara stuck her arm inside as the rest of us filed past. It had barely begun to close on her when we heard a thud. We turned around to see Sara flat on her back, unconscious and convulsing. When she came to, she was frightened and started to cry. So our day was cut short.
But our favorite incident came just a couple years ago. My mother and Sara went to J C Penny to have Saras watch fixed. The jeweler cheerfully repaired the watch, and put it back on Saras wrist. "Oh, it seems a bit loose," he said happily. "Here, let me tighten it." He did. Sara said faintly, "No dont . . ." But it was too late. She fell to the ground as he turned away. My mother just looked down at her, then looked around, hoping no one had seen. She was about to bend down and pick her up when, just as the jeweler turned back around, Saras eyes flew open and she sprang to her feet. The jeweler placed the bill on the counter and turned away again. Sara said frantically, "Lets get out of here, quick!" Before my mother could respond, her eyes rolled back, and she collapsed a second time. My mother was taken aback. "Well," she thought. "shes never done THAT before!" This time the jeweler saw her fall, and helped my mother move her to a chair. When Sara revived, she had no recollection of her momentary recovery.
So some of Saras episodes seem to be brought on by touch, some for no reason, and some by outside stimuli. Sight of blood, fear, and stress have all been linked to neurocardiogenic syncope (Beers and Berkow, 1653). But again, not all of her episodes were caused by anything definable. This became quite a concern for my parents as Sara came closer to the driving age. But there were medicines she could take to control the episodes. More practically, there are non-drug measures that she can take. For most victims of this condition, lying down when symptoms start, avoiding long lines, heavy meals and excessive alcohol, and increasing dietary salt intake are all preventative measures (Neurocardiogenic). One article suggested wearing support hose (Vitacco-Grab and Metzler). But here again is another indication of the uniqueness of Saras situation. The doctors determined that if she avoids getting dehydrated, eats when shes hungry, and avoids extreme heat, then shell be fine. And this, according to my research, is unusual. In my reading, dehydration and hunger are not connected to syncope episodes of this nature.
The concern was originally, as I said, could she drive? And she can. My mother was worried about anyone grabbing her wrist suddenly and bringing on an attack. But most of Saras friends know better. The ironic thing is, she has been in two very serious car accidents, but neither was her fault. One was last year, the week before Thanksgiving. She was heading north on Pacific Highway, near my parents house, when she was broadsided by on oncoming car. The car was totaled, but she made it out with only serious muscle trauma. The second accident occurred this year. Again, the week before Thanksgiving. Again, broadsided. Again, only muscle trauma. And again, on Pacific Highway near my parents house (though going the opposite direction this time). So its been determined that she takes the bus in November.
Were still not certain that Sara does indeed have neurocardiogenic syncope. When I asked my mother if there was any literature on the condition, she answered, "Oh, of course! They wouldnt diagnose her if it didnt exist." (I wonder if shes learned anything through this whole ordeal.) Sara, on the other hand, was more pessimistic. "No," she snorted. "They just made it up. Of course theres nothing on it!" Well, it turns out that both were correct. There is quite a bit of literature on neurocardiogenic syncope. But, from the test results and the episodes and the research, I very much doubt it is a correct diagnosis. The medicine she took briefly did help stem spontaneous episodes. But the incident at J C Penny came two years after she started taking them. This type of syncope is known to recede in regularity over time, but so are many other forms of syncope in young people. Shes 18 now, and though she hasnt been on medication for a while, its also been a while since shes had an episode.
Sara still lives with my parents, and they keep on eye on her. As long as she takes the necessary precautions, shell continue to be fine. Over Thanksgiving this year, as she sat on our grandparents couch full of turkey and muscle relaxers, I interviewed her for this paper. Just some clarifications on the order of things and how the diagnosis was made. I was pleased to learn that she hadnt fainted in a while. "Not since J C Penny," she told me. That was two years ago. This was really good to hear. But Im still concerned for her. My mom pouted, "She wont let me touch her eyes to see if it still works!"
I think Sara needs to move.
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