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Sunday, January 25, 2009

How are NMH and POTS treated?

How are NMH and POTS treated?

Treatment of these conditions is often quite challenging. Because patients have a different mix of underlying contributors to their orthostatic intolerance, therapy has to be tailored to the individual, and usually requires persistence and a willingness to try multiple methods. The approach we use has been based on the available evidence from formal studies and from our experiences treating large numbers of individuals. We use a stepped approach. Step 1 focuses on non-pharmacologic treatments, Step 2 involves use of a single medication, and Step 3 involves rational and judicious use of more than one medication.

Step 1:

a. Avoid prolonged sitting quiet standing, warm environments, and vasodilating medications.

Where practical, avoid circumstances that commonly bring on symptoms. For example, shop at non-peak hours to avoid long lines. Take shorter showers and baths and aim for a cooler water temperature. Avoid saunas, hot tubs, and lying on a hot beach. Avoid standing still for prolonged periods in hot environments, and on very hot days. Flex your leg muscles and shift your weight when you are standing still. You may also want to avoid alcohol because it causes loss of fluids and often leads to dilation of the veins, which can "steal" blood away from the central circulation. Many with NMH are quite intolerant of alcohol. High carbohydrate meals have been shown to reduce blood vessel constriction in response to upright stress, so a lower carbohydrate intake and frequent small meals may help. Caffeine intake (including caffeine in soft drinks) affects some people with NMH or POTS positively and some in an adverse way, so examine whether caffeine is helping you or making symptoms worse.

An important aspect of treating NMH or POTS is to review your current medications and nutritional supplements with your doctor or health care provider to ensure that these do not have the potential to make your symptoms worse. Narcotic medications (like codeine, morphine, oxycodone) and phenothiazine anti-emetics (like Phenergan or Compazine) can lead to more blood pooling, and niacin can cause vasodilation. Many patients develop hypotension when treated with high doses of nortriptyline, amitryptiline, or similar tricyclic antidepressants; low doses of these medications often are tolerated.

b. Use postural maneuvers and pressure garments.

Certain postures and physical maneuvers can help reduce NMH and POTS symptoms when people are upright, mainly by using contraction of the leg muscles to pump blood back to the heart and by compressing the abdomen to reduce the amount of blood that pools in the intestinal circulation. These small changes may be important, as even a small increase in blood return to the heart can help maintain an adequate blood flow to the brain. Many patients have adopted these postures without knowing why. The helpful maneuvers include:

standing with one's legs crossed


standing with one leg on a chair

bending forward from the waist (such as leaning over a shopping cart)

sitting in the knee-chest position

sitting in a low chair

leaning forward with hands on the knees when sitting.
Some of these postures are less conspicuous than others. Sitting in a low chair (such as a camping stool) is helpful because it causes the legs to be brought up toward the abdomen, and probably reduces the amount of blood pooling in the intestinal circulation. For similar reasons, avoid sitting in a high chair with the legs dangling freely, as there is no resistance to blood pooling unless the muscles are actively contracting. One young woman found she could sit longer without symptoms if she put her feet on a low foot rest (this probably required more leg muscle contraction than regular sitting, and may have also compressed the abdomen better). Some patients get worse if they adopt these postures, so they may not be right for everyone. Another technique has been shown to help reduce the frequency of fainting, and involves 2 minutes of maximum contraction of the arms (gripping one hand with the other and pushing the arms away) at the start of lightheadedness.

Another time-honored recommendation is to elevate the head of the bed slightly by 10-15o, so that the head is higher than the feet, a position that appears to help the body retain fluid at night rather than lose fluid into the urine.

Several research studies have shown that if blood vessels can be compressed from the outside (using tight compression garments or military anti-shock trousers), the abnormal heart rate and BP changes of NMH or POTS can be reduced or eliminated. Waist-high support hose can prevent some of the excessive pooling of blood in the legs (knee-high support socks help somewhat), as can garments that increase abdominal compression (these work by preventing excessive amounts of blood pooling in the intestinal circulation), such as abdominal binders and abdominal corsets.

c. Treat contributing medical conditions

Attention to other medical conditions is crucial to ensuring that the NMH or POTS treatments are as effective as they can be. In particular, preventing activation of even mild asthma and allergies has been important in keeping our patients from developing a worsening of symptoms. In patients with asthma, we usually try to reduce reliance on albuterol and other beta-agonist inhalers, as these medications can mimic the effect of too much epinephrine, and can aggravate NMH in particular. Endometriosis and other painful conditions may aggravate symptoms, and ovarian vein varices in women with pelvic pain are associated with fatigue and worse orthostatic intolerance. Sinusitis, anxiety disorders, depression, and infections of any sort are examples of other conditions that need appropriate medical attention when present.

Allergies or delayed hypersensitivities to food proteins (most commonly cow's milk protein) can co-exist with orthostatic intolerance, and unless they are addressed they can obscure any improvements that might otherwise come with medications and postural changes. Dr. Kevin Kelly has identified the following symptoms that should prompt us to think further about the possibility of a food allergy or hypersensitivity: upper abdominal pain, gastroesophageal reflux, and appetite disturbance (filling up too quickly, picky appetite), sometimes with recurrent mouth ulcers, headaches, sinusitis, and either constipation or diarrhea. If hypersensitivity to a food protein is playing a role, substantial improvements can result from strict exclusion of offending foods. We would emphasize that this dietary treatment is not part of the standard treatment of NMH and POTS in our clinic, and is only considered on the basis of the specific symptoms mentioned above. Given the potential dangers of unsupervised diets, be sure to discuss these issues with your doctor or health care provider.

d. Increase salt and fluid intake

NMH and POTS are most often treated with a combination of increased salt and water intake. The increased salt and water help ensure that the blood vessels are filled better, and that the heart receives an adequate amount of blood even during upright posture. We recommend at least 2 liters of fluid per day. Our patients who drink fluids regularly throughout the day seem to do better than those who don't take this task seriously. Keep in mind that prolonged periods of sleeping (more than 12 hours) may interfere with the ability to keep up with fluid needs. We recommend drinking fluids every 2 hours throughout the day. As a result, it is important to have easy access to fluids at work or at school.

For those who have been on a low salt intake we recommend an increase in the amount of salt they add to their food. The Appendix to this document contains a list of high salt foods. For some mildly affected individuals, an increased intake of salt and fluids may be all that is needed. Most of those with more severe symptoms require one of several medications in addition to the increased salt and fluid intake. The increased salt and fluid intake should be continued regardless of which of these medications is added.

e. Physical therapy and exercise

Exercise is important in regaining the effects that fitness brings in counteracting NMH or POTS. Because exercise can make NMH or POTS symptoms worse in the period before effective treatment has been found, it must be done carefully at first. When you and your doctor feel you are ready, begin a regular regimen of exercise, finding something that does not make you lightheaded and doing it for brief periods at first, increasing gradually. For example, one girl who had been ill for several years began functioning better once two of the NMH medications were working for her. She began exercising on a treadmill, but this made her lightheaded, so she switched to a reclining exercise bike. Although she started with only 2 minutes a day, she increased this in small increments up to 30 minutes 3 times a week after about three months. Walking, water jogging (the water acts as a compressing force to counteract blood pooling in the limbs), stretching, and Tai Chi or yoga may be gentle ways to ease back into exercise. Remember to warm up slowly before, and cool down gradually after exercise. If you plan to exercise outdoors, remember that extreme heat will worsen NMH or POTS.

A group of our physical therapist colleagues in Baltimore, led by Rick Violand, PT, have helped us to identify a relatively high frequency of postural asymmetries and areas of adverse mechanical tension in the nervous system as contributors to pain, lightheadedness, and fatigue in many of our patients with orthostatic intolerance. These postural restrictions have helped explain why some patients were finding that exercise led to substantially worse symptoms. Among those who have the worst of these postural restrictions, several weeks of gentle manual physical therapy often prepares them to tolerate the mild aerobic exercise that would have caused a flare-up beforehand. We think careful attention must be paid to postural asymmetries and restrictions in mobility during the physical examination, and the diagnostic expertise of a physical therapist may be essential to identifying problems. Manual techniques that our colleagues employ include gentle neural mobilization (or neural tension work), myofascial release, and cranio-sacral therapy.

Steps 2 and 3

For those with more frequent or more severe symptoms, the physical maneuvers, dietary changes, and physical therapy of Step 1 may need to be supplemented by medications. Most of the drugs in common use for NMH and POTS help to improve the ability of the vessels to constrict and return blood to the heart when we stand, some effect the amount of salt and fluid the kidney returns to the blood stream, and some effect the release or action of norepinephrine or epinephrine.

While many of the medications listed below have been used by cardiologists and other physicians for years to treat NMH or POTS, few have been studied formally in those with POTS, NMH, or chronic fatigue syndrome. Some have been tested in patients who have fainted one or more times, but are otherwise healthy; some have been tested in those with ongoing symptoms due to POTS, and some have been tested in NMH patients who were diagnosed with chronic fatigue syndrome. This handout is based upon available research and our experience with NMH and POTS patients, most of whom came to see us because of chronic fatigue syndrome.

The treatments listed require persistence, commitment, and the willingness to try several possible drugs and combinations over an extended period of time. Because there is a risk of serious side effects with some of the drugs (such as elevated blood pressure, elevated sodium levels, lowered potassium levels, or depression) careful monitoring is required. The following lists medications that have been reported to help improve symptoms or tilt table responses in patients with NMH or POTS:

Medications that increase blood volume:
Fludrocortisone (Florinef)
Oral contraceptive pills

Medications that interfere with the release or action of epinephrine and norepinephrine:
Beta-blockers (e.g., atenolol, propranolol)
Disopyramide (Norpace)
Angiotensin converting enzyme inhibitors

Medications that improve vasoconstriction:
Stimulants: (e.g., methylphenidate [Ritalin] or dextroamphetamine [Dexedrine])
Midodrine (Proamatine)
Ephedrine and pseudoephedrine (Sudafed)
Theophylline (low-dose)
Selective serotonin reuptake inhibitors (examples include fluoxetine [Prozac], sertraline [Zoloft], and paroxetine [Paxil]). These medications may also affect the central nervous system reflex pathways in NMH as well.

Some of the above medications no doubt work in more than one way. For example, fludrocortisone improves the ability of the blood vessel to constrict in addition to expanding blood volume. Your health care provider should work with you to determine the best possible combination for your personal situation.

Does treatment cure the problem?

It needs to be stressed that, when successful, the medications for NMH and POTS usually do not cure the problem. Rather, they help control symptoms. When medications are stopped or when salt intake is reduced, symptoms frequently reappear. Many with NMH or POTS have symptoms resurface or worsen at busy or stressful times (making an oral presentation in class, having company over for Thanksgiving, rushing for a meeting on a hot day and forgetting to drink), when they have an infection, or when their allergies are more active. The intravenous infusion of saline solutions has been shown in experimental settings to help reduce symptoms, and the judicious use of IV fluids periodically has a role.

Even after successful treatment, many women with NMH and POTS describe a worsening of symptoms in the days around the start of a menstrual period. Some women choose to take birth control pills on a 28 day cycle, or continuously, in addition to their NMH or POTS medications. This is done to avoid symptoms related to hormone changes, and should be discussed with your health care provider.

The question of what happens over the long term has not been adequately studied, and the optimal duration of medical treatment is still being worked out. Unfortunately, despite appropriate doses of the available medications for neurally mediated hypotension, some people with NMH or POTS do not experience an improvement in symptoms, and some are intolerant of the medications. This emphasizes the need for more research on this problem. Many adult women who have orthostatic intolerance describe an improvement in symptoms when they have been pregnant, and often describe pregnancy as the time when they felt "the best ever." The improvement may be due to an expansion of blood volume that occurs naturally with pregnancy.

Finally, we suggest you take an active role in your care. If you have POTS, it might be helpful to learn how to take your pulse. If you have NMH, you may want to purchase a home blood pressure monitor - we suggest the kind with a cuff that goes around your upper arm, not around your wrist or on your finger. Heart rate and BP do not have to be taken routinely each day, but having this information during flare-ups of symptoms may provide helpful insights. Being able to monitor your blood pressure at home or take your own pulse won't replace visits to your physician or health care provider, but may make those visits more productive, as this information may reflect how you are responding to a high salt/high fluid diet or to medications.

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