When I was twelve, our stern, small-town physician diagnosed me with mitral valve prolapse (MVP), though he never explained the disorder. During my teens I had strange symptoms other girls didn't experience: fainting, fatigue, and eyes burning as though wind blew constantly into them. My mother noticed and trotted me off to that detestable, cursing physician.
In his office he scolded me in his nasal voice, "There's not a damn thing wrong with you. God Almighty, don't give your poor mother something more to worry about. Think about her instead of being selfish. What you need is a psychiatrist." I can see his face redden with anger.
Didn't he remember? Earlier, after his MVP diagnosis, he had curtailed any sports with the use of my arms, which is not the way MVP is handled now. According to him, in two years I would be fine. Actually I had felt better before the sports restriction. Without sports for two years I had lost muscle strength and tone.
MITRAL VALVE PROLAPSE
MVP is the most commonly inherited heart problem in the United States. Yet it is listed with the National Organization of Rare Disorders (NORD) because the disorder is not completely understood or acknowledged by most physicians. While the statistics vary with sources, the estimated percentage who have this problem includes as many as thirty percent in this country. According to a survey in the late 80s, three times more women have MVP than men.
The heart has four valves inside it to pump the blood in and out: the aortic, the mitral, the pulmonary, and the tricuspid. The mitral valve acts like a door between the left atrium and the left ventricle in the heart. When the lower part of a normal heart contracts, the mitral valve closes and prevents any blood leakage into the upper chamber. With MVP, the valve balloons out of shape. A physician hears this through a stethoscope as a click. If the valve shifts enough, it can allow some blood to flow back into the upper chamber, and the physician hears a murmur.
Have you heard your own heart through a stethoscope? Have you heard the click or the murmur? Ask your physician to allow you to do this. It's important you know what sound your heart makes. Of course, some murmurs and clicks are difficult even for a cardiologist to detect. But give it a try. If you squat, the hardest position on the heart, you will hear it. One woman chose her physician based on whether the physician could hear her murmur.
However, this structural defect is not what usually causes the myriad of symptoms. Most MVP people have healthy hearts.
MITRAL VALVE PROLAPSE SYNDROME WITH DYSAUTONOMIA
A syndrome is a collection of symptoms which has more than one cause, whereas a disease has a single cause. MVPS/D has a recognizable pattern of symptoms and signs. As the physicians and researchers understand, the symptoms reflect a failure of control mechanisms, specifically in the nervous system. The more they learn about the disorder, the more likely they will be able to find a single cause and convert MVPS/D to a disease status.
The nervous system is divided into two parts: voluntary and involuntary. The involuntary nervous system, also known as the autonomic nervous system (ANS), is more complex and controls most bodily functions such as body temperature, sweating, blood pressure, heart rate, salivation, pupil dilation and contraction, sleep-wakefulness balance, intestinal functions, digestion, and many others. This system has two major divisions: sympathetic, which speeds up the system, and the parasympathetic, which slows the system.
The physicians connect some MVP symptoms to the ANS, an imbalance known as dysautonomia or an autonomic (which means automatic) nervous system dysfunction. Therefore, when the MVP patient experiences symptoms that involve the ANS, the physicians refer to the disorder as mitral valve prolapse syndrome with dysautonomia, the most common type of dysautonomia.
The central problem of MVPS/D is the body's excessive response to adrenaline and adrenaline-like hormones. (Hormones and nerves serve as control systems in the body.) Most MVPS/D patients have a bit too much noradrenaline, a related hormone. Most of the symptoms come from various organs' over-responsiveness to even normal amounts of adrenaline-like hormones.
Thus, you can see it would be best for MVPS/D patients to minimize the release of adrenaline into their blood streams. Of course stimulants of any kind-caffeine, sugar, alcohol, chocolate, aspartame, NutraSweet, Splenda, monosodium glutamate, and some over-the-counter medications, those containing caffeine, epinephrine, ephedrine, and pseudoephedrine-should be eliminated from your diet. Stimulants can include people and activities. Movies, at times, can over-stimulate my system, even talking.
Check with your physician before a therapist uses electricity, which can trigger symptoms. Patients should allow only moderate sun and heat exposure and gradually progress to condition their bodies to exercise. These cautions will sustain a high blood volume. A higher blood volume means an adrenaline increase is less likely. Patients also need to control their responses to stressful emotional situations. Yeah, right! That's a trick in itself.
Debbie Petersen, who has yet to get her symptoms under control, calls the dysautonomia part an everyday hell to live with. "Right now it's just trial and error every day."
'I was so happy to know there was a name for what is partly wrong with me: dysautonomia," wrote Rita McNeil. "Most people have never heard of it, let alone know what it means."
"The disorder takes a significant toll on lifestyle and work capacity," commented Linda Smith of the National Dysautonomia Research Foundation (NDRF).
Dr. David H. P. Streeten, a research pioneer for orthostatic intolerance (OI), sums the impact of dysautonomia on a person's life: "While we are not constantly aware of the activity of the autonomic nervous system as we are of unusual sensory and motor events, the normal functioning of the autonomic nervous system day and night, from heart-beat to heart-beat, plays a largely unconscious but vital role in our livelihood. It is not surprising, therefore, that autonomic abnormalities, though they are usually more difficult to recognize than a severe pain, a sensory loss, or paralysis of a limb, may be even more important in impairing the quality and even jeopardizing the continuation of life."
SPACE SHUTTLE COLUMBIA
The astronauts experience similar problems when they take off from Earth and land from outer space. Dr. Andrew Gaffney, cardiologist and astronaut, served as Payload Specialist on Spacelab Sciences 1 as part of a Space Shuttle Columbia mission in the early 90s to study the astronauts' problems.
Understanding how our MVPS/D problems work helped him understand why the astronauts experience their symptoms such as dizziness and faintness when they stand. During the early part of the space flight, Dr. Gaffney flew with a catheter inserted into his arm vein to record the blood pressure around his heart. Within a few hours after lift-off, his blood pressure and blood volume dropped remarkably, according to Popular Science Magazine, March, 1993.
"As we sort out that data," Dr. Gaffney said, "we are learning a lot more about where the blood goes, where it is stored-in terms of the venous system, and the relationship between the adrenalin levels and the blood volume."
Dr. Gaffney, chief of clinical cardiology at Vanderbilt University School of Medicine, Memphis, TN, describes what he knows MVPS/D to be: "There is a primary abnormality of autonomic tone so that these people tend to be vascularly constricted." That constriction leads to shrinkage of blood volume. The lower volume feeds back and causes more constriction and that becomes a vicious cycle.
That cycle contributes to the symptoms such as fatigue. "We all know patients who are anemic have fatigue, and everybody understands that," Dr. Gaffney said. "Well, it is perhaps a little more difficult to understand (with MVPS/D). You can have the right amount of blood volume but if you don't have enough red cells in it, then you get tired because you don't deliver the oxygen. That's anemia. You can have the right concentration of red blood cells, so you are not anemic, but if you don't have enough blood, then it is the same problem. Absolute number of red blood cells that carry the oxygen is what determines whether you can exercise more."
BLOOD VOLUME
"Blood volume is critical to prevent symptoms from starting," said Susan Elliott, who taught physics before MVPS/D symptoms changed her lifestyle. "When blood volume gets low, it tends to trigger a chain of reactive events that cause things like tachycardia, feeling too hot or too cold."
Even a slight imbalance in the ANS can cause low blood volume in MVP patients. Up to twenty-five percent lower. The thirst signal fails to turn on, keeping the blood volume low.
It is very important for people with MVPS/D to drink at least 70 ounces of fluids daily, more if the temperature is higher. A good way to remember the amount is if the temperature is higher than 70, drink the equal number of ounces to the degrees in temperature. Such as if it is 86 degrees, drink 86 ounces of fluids. An easy way to achieve this is to keep a water bottle with you and try to drink eight ounces every hour. And to make it really fun, sing the tune to "Pump Up the Volume," by M/A/R/R/S.
Susan also found the more water, the less her MVPS symptoms such as twisting leg muscles. Also included in her regimen were potassium, zinc, and magnesium, minerals helpful to heart and muscular function and energy metabolism.
Fluids "fake out your body," Susan says, making it seem to have higher blood volume. She drinks a gallon of water a day, always remembering to salt the water or eat something salty with it. The salt helps retain water and valuable nutrients such as potassium. Another thought is to intake fluids with salt present like sports drinks or soups. However, patients with hypertension (high blood pressure) need to consult their physician first.
VERTICAL VS HORIZONTAL
When a MVPS/D person lies down, she can immediately experience symptoms such as tachycardia or heart pounding. "If you lie flat, that can produce too much blood to your head and heart too fast," Susan said.
While we are vertical, the blood circulates the entire body with less volume than people without MVP. When our bodies become horizontal, the blood floods the head and the heart chambers, causing the symptoms, until the pumping adjusts.
To offset this, some people sleep on a slight incline such as in a recliner. To create her slant, Susan puts a board beneath her headboard. Pat Conrad sleeps on two pillows. Then her palpitations are "not so extreme." When the heart pain continues, she elevates her feet. My husband and I use two thick encyclopedia volumes that help me and his gastrointestinal reflux disorder (GERD).
ORTHOSTATIC INTOLERANCE (OI)
People take their ability to stand for granted. OI patients do not. Daily they struggle with the symptoms brought on by the vertical position. Upon standing, a person with orthostatic hypotension, another word for OI, might experience lightheadedness, dizziness, palpitations (throbbing or heavy beats), tremulousness, visual changes, a stuffed head feeling, ears ringing or buzzing, fainting, and poor concentration because her blood pressure has suddenly fallen. About a half million Americans have OI, according to the National Dysautonomia Research Foundation.
Being upright produces blood pooling in the lower abdomen and limbs, blood which is no longer available to the upper body. With an effective ANS, the body quickly compensates by increasing the heart rate and other cardiac output.
When the OI person stands, her blood volume lowers, her blood vessels lose an excessive amount of plasma, and her kidneys pour out salt.
Some patients experience fatigue, tachycardia (abnormally rapid heartbeats), and shortness of breath, which is termed sympathontonic or hyperadrenergic orthostatic hypotension because the sympathetic part of the ANS increases.
Not every patient has all these abnormalities. Most OI patients have a mild case which can improve over weeks or months. Most will eventually be free of their symptoms.
However, in patients with severe symptoms or with a sudden ANS alteration such as actor-director Christopher Reeves experienced with his fall, the recovery may never come.
A few other names used for OI are as follows:
orthostatic tachycardia syndrome
postural orthostatic tachycardia syndrome (POTS)
hyperadrenergic postural hypotension
partial dysautonomia
sympathotonic orthostatic hypotension
hyperadrenergic orthostatic hypotension
mitral valve prolapse syndrome
SUPPLEMENTAL HELP
CALCIUM
Calcium, the body's most abundant mineral, is located mostly in the bones and teeth because its function is to build and maintain bones and teeth. It also is essential for healthy blood, regular heart beats, and proper nerve transmission. Calcium is beneficial for cardiovascular disorders. Cases of calcium deficiency have provoked heart palpitations, slow pulse rates, and excessive irritability of nerves and muscles.
The best supplemental source lies in calcium lactate or gluconate because these are natural derivatives and easier to absorb. Foods rich in calcium are milk products, green leafy vegetables, shellfish, and molasses.
MAGNESIUM
This necessary mineral lives within the body cells to activate enzymes for the metabolism of carbohydrates and amino acids. It helps to regulate the acid-alkaline balance and to promote absorption and metabolism of other minerals and vitamins. With nearly 70 percent of it located in the bones, magnesium aids during bone growth, is vital for proper nerve, muscle, and heart functions, helps regulate body temperature and blood sugar convert to energy. Magnesium is alkaline, so it should not be taken after meals but can be used in place of antacids.
This mineral can help prevent heart attacks and depression. It is beneficial for neuromuscular disorders, nervousness, and sensitivity to noise. Contrary to the old sayings, magnesium helps form the hard tooth enamel that resists decay, not calcium.
Large amounts of magnesium can be toxic if the calcium is low and phosphorus is high. Magnesium loss increases with the use of diuretics and alcohol. Foods rich in this mineral are seafood, whole grains, dark-green vegetables, molasses, and nuts.
POTASSIUM
Elaine Hansen had spells of irregular heartbeats that would last up to twelve hours, often during the night. She felt very tired afterwards. Her family physician said the cause was low potassium.
This essential mineral works with sodium to help normalize the heartbeat. If you're prone to palpitations, you might feel them when you are in the low-to-normal range for serum potassium. When they come on, try drinking orange juice to help the palpitations go away.
Together, potassium and sodium regulate the body's water balance, i.e. they distribute the fluids on the cells' walls. They equalize the acid-alkali factor, help in muscle contraction and expansion and in nerve stimulation. With phosphorus, potassium sends oxygen to the brain and with calcium regulates neuromuscular activity. This is a handy little mineral for our bodies.
However, potassium and sodium must be in balance or excessive salt will deplete potassium. Alcohol and excessive sugar can deplete both potassium and magnesium. Potassium is excreted through urination and perspiration. Foods with potassium are lean meats, whole grains, vegetables, dried fruits, legumes, and sunflower seeds. A high potassium level can be life-threatening and can occur in renal failure.
SODIUM
Half of our body's sodium is found in the extra-cellular fluids such as the blood vessels, arteries, veins, and capillaries. As I wrote in the last section, sodium works in conjunction with potassium. It keeps other blood minerals soluble so they won't become deposits in the bloodstream. Foods naturally rich in sodium are seafood, celery, milk products, table salt, and baking powder.
WATER
In elementary school we learned water is two-thirds of our body weight, so we know it's our body's most important nutrient. It makes up about 70 percent of our weight. We can live longer without food than we can without water. Water is needed for everything from maintaining body temperature to carrying away our waste. For MVPS/D, water is essential to maintain a level high blood volume.
My young neighbor Ryan had a problem with constipation. His parents used all the usual remedies. Still, Ryan might wait as long as five days without a bowel movement.
Finally his doctor asked how much water he drank daily. When his mother answered, the doctor said that was sufficient for some; however, some people's colons absorb water at different rates. The faster the absorbency, the firmer the stools. Presumably then (assuming no other variants), the more water one drinks, the softer the stools.
I never considered water as an answer to my irritable bowel syndrome (IBS). I had assured myself I drank enough water. Besides, my stools usually change when I've overworked and my other symptoms, such as migraine and fatigue, come out of hiding.
When I began drinking my gallon (and that takes discipline!), my stools softened to normal consistency without my other MVPS/D appearing. Since I've been on the gallon regimen, my stools have been firm or softened, never irritably hard.
Of course, other elements can be involved such as the foods we eat and much more. Chocolate and red meats harden stools. I discovered . . . for me . . . that water helped my IBS.
Someone commented about drinking too much water. In the May, 2000, issue of Town and Country magazine, Diane Quagliani, a registered dietitian and spokesman for the American Dietetic Association, was quoted, "Everyone loses about ten cups of water daily, so it's important to drink about eight eight-ounce cups of fluid-the rest is made up by water in foods and the water the body makes in metabolism. The bigger, more active, and more overheated you are, the more fluids you should take in. Taking in too much (water) is not very likely, unless it is gallons a day, and the main danger would be flushing out too many electrolytes. Healthy kidneys can keep up with most consumption."
Others complained they would have to run to the potty more often. In hot humid weather, the average person should eliminate urine every two hours if the person drinks sufficient water to cover sweating, according to my family physician.
When I tried the gallon experiment, some days I hit the toilet plunger more than the norm. But another day I wouldn't. What is normal anyway? The question should be "What is best for your body?"
The Town and Country article writer, Ila Stranger, wrote that it "makes sense that drinking enough water is bound to help us avoid dehydration-which can happen as easily in the overheated rooms of midwinter as in the summer sun-and make us look and feel alive and well."
Each day I grab a gallon jug of spring water or fill a gallon pitcher and set it on the counter to remind me to strive to drink more water. Not only for my IBS but for my blood volume, blood pressure, and the many other tasks my body constantly performs. I've found in the winter lukewarm water is swallowed easier. Ice water is a shock to the throat, sometimes even in hot weather.
Some people prefer to drink mineral water rather than tap water. Others use well water. Neither should be relied upon for a person's source of minerals. Distilled means the water has been boiled water with all harmful or beneficial minerals eliminated. Fruits and vegetables are good dietary sources for chemically pure water, which is 100 percent pure hydrogen and oxygen. The use of these are an individual choice.
The Environmental Protection Agency's hot line is 1-800-426-4791; its website is http://www.epa.gov/safewater. The Natural Resources Defense Council website (www.nrdc.org) contains in its archives the results of a recent four-year study on bottled waters. The author of the summary results is Dr. Andrew Weil on the website http://www.pathfinder.com/drweil.
ANTIBIOTICS
Before any invasive procedures, MVPS/D patients with valve leakage should take antibiotics to prevent bacterial infection from forming on their valve. Such as dental work, body piercing, tattoo, and surgery. A few years ago the physicians recommended antibiotics for all MVP patients. Now only those with definite regurgitation (leakage) are urged to do this, and the dosage has been cutback to only before the procedure.
For other surgeries and procedures you can consult with your physician or the MVP clinics.
RESEARCH IN MVPS/D
Dr. Al Davies, associate professor of medicine at Baylor College of Medicine in Houston, has led the MVPS/D research of chemical reactions between adrenaline and several important proteins.
The proteins are located near the lining membrane of a cell of a body organ. Three proteins of greatest importance are: the receptor on the cell's surface, a G-protein located inward, and an enzyme called adenylyl cyclase on the inside cell membrane's surface.
Normally, adrenaline attaches to the receptor protein, which combines with the G-protein. (Full name is stimulatory guanine nucleotide regulatory protein.) Once these are activated, they turn on the enzyme which causes internal actions such as rising blood pressure or increased heart rate.
Dr. Davies and his colleagues have shown that the MVPS/D patient's abnormally increased sensitivity to adrenaline is due to an overly active, abnormal G-protein.
The possibilities to the question "Why?" are numerous. And there are many steps before a full answer and treatment are developed. The researchers thought this might be due to an abnormal gene since the illness is hereditary. However, their studies show the gene is normal.
To the MVPS/D patient, that finding carries hope because the cause is not something as simple as a bad gene passed down from our grandfather. Something happens to the protein after the gene makes it and before the adrenaline activates it.
Editor of the November, 1998, special American Journal of Medical Sciences supplement on OI, Dr. David Robertson entitled the issue "Epidemic of Orthostatic Tachycardia and Orthostatic Intolerance." Dr. Robinson writes that many centers actively pursue OI research and expect rapid improvements in diagnosis and therapy. The director of the Clinical Research Center at Vanderbilt University, Nashville, he adds, "A healthy appearing 20-year-old woman with dizziness, palpitations, and fatigue is certainly no longer passed over as having a psychosomatic illness!" Well, maybe not in Nashville. Generally the tilt table test is used to diagnose a dysautonomia.
MANY PHYSICIANS' ATTITUDES
My former country physician may sound old-fashioned and something of the past; however, we with MVPS/D know his attitude remains with most of the physicians we meet today. One of the tough barriers we have to get around is finding a knowledgeable, caring physician.
For most people who have diabetes, cancer, or even strange diseases, they can contact a physician, and within a few visits they have their illnesses diagnosed. They are believed from the time they enter the physician's office.
We with MVPS/D are not believed. We must research our own disorder, be abreast of the latest drug regimens, carry literature about our illness to give to the physician, and then know the physician probably will not consider our illness legitimate or treat us. It's just crazy when you think about it! How much proof does a physician need? Why can't they keep informed about MVPS/D when a fifth of the nation has MVP and might possibly become symptomatic?
Education and research are keys to solving the MVPS/D puzzle in our bodies. With these always lies hope. Miles and miles of hope!
