Q&A with the doctors

Q&A - Dr. Satish Raj:

Question:
“I was diagnosed with POTS in 2003. I was 53 years old at the time. I was told that it was inherited from my Mother. My only living brother was also told he had POTS in 2005. Is it true that we got it from our Mother?”

Answer:
Although we are interested in the possibility that POTS may be genetic in some people, we have not been able to make that link so far. There is a genetic mutation in the norepinephrine transporter that has caused POTS in twin sisters (reported in the New England Journal of Medicine in 2000), but to my knowledge this specific problem has not been noted in any other patients with POTS.

Q&A - Dr. Julian Stewart:

Question:
“Could you please explain the different variants of POTS that you have characterized in your research (i.e. low-flow, normal-flow, etc.) in lay language? Also, do you foresee POTS patients being categorized into these groups in the future for tailored treatment?”

Answer:
Because we measured limb blood flow, we noted differences among patients and control subjects. However, the classification scheme may be more convenient than mechanistically important. I think that high flow patients are identical to those “neuropathic POTS” patients of the Vanderbilt group. This is usually short lived, and I am not seeing many of these patients lately.

The normal flow group has physiology similar to fainters although they do not faint. We are working on this, but it is unclear as to what would be the best therapy.

The low flow patients are sickest, usually female, and definitely underweight on average with reduced blood volume. These are the most interesting patients, and attempts to address their physiological defects are the most interesting. Not a done deal, however.

Q&A - Dr. Svetlana Blitshteyn:

Question #1:
“I have a six year history of SVT and multifocal atrial tachycardia. Two years ago I started to faint and have been diagnosed with postural tachycardia and orthostatic hypotension. I am considering coming to America for treatment (as I live in the UK). Can you please give me an idea as to what types of treatments would be available to me? I have also suffered many periods of bradycardia as I am very sensitive to beta-blockers, but equally need a high dose of them to reduce my symptoms.”

Answer #1:
If you're planning to travel to the United States for treatment of POTS and orthostatic hypotension, consider visiting one of the major academic centers for autonomic dysfunction, such as Mayo Clinic in Rochester, MN or Vanderbilt University in Nashville, TN. There you will have a complete evaluation of the autonomic nervous system, based on which physicians will determine the best treatment plan for you. Treatment options available for POTS and OH are numerous and include Florinef, Midodrine, Mestinon, SSRIs and other medications, but the choice and combination of medications require an individualized approach based on your specific case. Often, treatment for POTS and OH also involves a trial-and-error method to see what combination would be most effective for you.

Question #2:
My daughter has been diagnosed with POTS and is just now starting to get better. I was just wondering if there is any link between POTS and molestation. You see, when my daughter was fifteen she began dating. During this time she acquired a virus, and after that she began having fainting spells. This went on for quite awhile before she was able to get into a program at Frazier Rehab in Louisville. During her stay at Frazier, she admitted that she had been sexually molested for several years by an ex-relative. My thought is that perhaps when she was being molested she fainted in order to escape. Once she began dating and experiencing sexual feelings, is it possible that she could have responded by fainting again? I know that fainting is from the blood pooling in the legs, but is it possible that the body can condition itself to the response? The reason I am asking is because a professional friend of mine presented this theory to me, and I was just wondering if there might be any kind of research or history to link the two.

Answer #2:
First, it's important to emphasize that POTS is not a psychological disorder. Rather, in broad terms, it is a syndrome rooted in physiologic dysfunction.
Secondly, in those who have a chronic medical condition and in those who are predisposed towards a particular medical condition - whether though genetic factors or other, yet unidentified mechanisms - any type of stress, whether physical or psychological, may exacerbate the medical condition.
To date, there have been no scientific studies demonstrating a causative relationship between psychological or physical stressors and POTS, neurocardiogenic syncope, or other, more severe, forms of autonomic dysfunction.

Question #3:
My mother-in-law is very concerned about my husband and I wanting to have a baby. She believes that I should not have a baby due to the fact that I have POTS. Many people have assured her that people do it all the time. I am doing very doing well on my medications, so my husband and I aren't exactly sure why my mother-in-law is so worked up about this. If you could possibly shed some light on this issue I would really appreciate it.

Answer #3:
As POTS predominantly affects women of child-bearing age, pregnancy is a common concern for patients and physicians. Ultimately, the decision regarding pregnancy lies in the hands of the patient, in conjunction with the recommendations from her consulting team of physicians, which, preferably, should include a doctor specializing in autonomic disorders, a maternal-fetal medicine specialist and an obstetrician/gynecologist.

POTS is not a contraindication to pregnancy. Women with POTS may require special considerations and monitoring in the course of their pregnancy, labor and delivery and are generally treated similarly to women with other types of cardiac or neurologic conditions. In fact, some women with POTS may even experience improvement in their symptoms during pregnancy. If you decided, and your team of physicians agreed that pregnancy is desirable at this time, there should be no need for excessive worry or concern - beyond that of any pregnancy, of course.

Q&A - Dr. Charles Randall “Randy” Thompson:

Question #1:
“I have read on several websites that it's helpful for people with POTS to take a magnesium supplement. My son is a teenager with POTS, and his doctor didn't really have an opinion on whether magnesium would be helpful or not. The reason I'm hesitating is that I am under the impression that magnesium is a blood vessel dilator, which is why it's used to treat migraines. With POTS, our goal is to constrict the blood vessels, not dilate them, right? So how can magnesium be helpful?”

Answer#1:
About magnesium:
While it is true that large doses of magnesium can lower blood pressure, magnesium is still almost an essential supplement for those with POTS/Orthostatic Intolerance. Studies in the late 80’s and early 90’s, done originally on patients with MVP, but later on OI subtypes like POTS in general, have shown that the majority of these patients were deficient in magnesium. The interesting part is that most of the time, just checking the blood for magnesium shows a normal level. But Stanford University developed a test (study for research and not available commercially) where they broke open the red blood cells and measured magnesium. This showed the majority of OI/POTS patients had low intracellular magnesium that contributed to the patient’s symptoms. So magnesium is an important part of treatment, although magnesium alone is usually not enough on its own to control symptoms. You should use just over-the-counter magnesium supplements in doses prescribed on the bottle or your physician can write a prescription for a magnesium supplement.

Question #2:
“I recently read that people who have had MONONUCLEOSIS can develop CFS / arrhythmias (including tachycardia) later in life. Is there any known linkage of MONO to POTS? Any idea as to how many POTS patients have had MONO in the past?”

Answer #2:
As you have mentioned, mononucleosis has been strongly linked to Chronic Fatigue Syndrome and arrhythmias associated with it. As far as I know, there is no direct study to show how many POTS patients have had mono. But the predominance of evidence at this point shows that on looking back, many patients may have had POTS symptoms to a certain degree, but then their symptoms probably exacerbated after a viral infection. The two most studied are the adeno and echo versions (cold and flu) but any viral illness can cause the exacerbation. I have seen several patients, including the editor of this newsletter (my daughter), who had their POTS “activated” by Mono.

Question #3:
“I was diagnosed with dysautonomia in 1992 at the dysautonomia clinic in Birmingham. This past November, I was diagnosed with Narcolepsy and started Xyrem, which has been very successful at treating symptoms. I take Adderall during the day. I recently started back on Robinul again for excessive sweating, stomach acid, and hoarseness. It is very effective for all three. Though I have taken it off and on for years, on the second day, I had a very bad reaction to Robinul. After quitting Robinul and stopping the Xyrem as a precaution, I continued to have severe anticholinergic over-activity for more than a week. Is there an anticholinergic that is safe for people with dysautonomia? I took Hyosycamine approximately 15 years ago without any problems.”

Answer #3:
I was also diagnosed with dysautonomia in 1998 in Birmingham and just last week spoke to the doctor there and ran this question by him. After researching Xyrem/Adderall, there really shouldn’t be any interactions between Robinul and these medications. But while the anticholinergic drugs are very helpful in many patients with dysautonomia, they are also to be used very carefully in patients with any type of autonomic neuropathy. They can actually exacerbate some of the symptoms you were having. While you didn’t mention the specific side effects, usually by inhibiting the parasympathetic side of the ANS (which you do with anticholinergans) you can experience symptoms caused by the sympathetic system. Interestingly, these drugs block acetylcholine, the neurotransmitters used by the parasympathetic and the gut. The exception to this is sweating.

So in answer to your question, all anticholinergic drugs have the potential to cause side effects in patients with autonomic neuropathy, and just because you tolerated one in the past doesn’t mean you can tolerate it now. It is certainly reasonable to try another anticholinergic such as Hyoseyamine or the combination of Hyoseyamine/Phenobarbital, but be aware they could give you the same side effects. If you do decide to try them, start at a lower dose and work your way up.

Q&A - Dr. Amer Suleman:

Question:
“I was diagnosed with NCS; I know bradycardia and junctional escape rhythm have been seen. I have had 4 tilt table tests, and all resulted in syncope after the nitro. The minimum BP drop was 60 points, and the HR twice was 0/P. I'm not sure what the other two were. The first med ordered was Norpace, and various other meds were added and stopped after having no benefit. I do remain on high salt and water loading.

I note that without the Norpace the chance of syncope is extremely high. I
also note that it’s rare for a NCS patient to be on Norpace. Can you explain why I am on Norpace and others are not?”

Answer:
What you have is a relatively common scenario (you are not alone or unique.) It appears you have NCS with sinus bradycardia (or even sinus node dysfunction).

Your choices are:

1. SSRI or drugs like Prozac

2. Florinef (main side effect is low potassium)

3. Midodrine (that increases blood pressure)

4. Beta blockers with intrinsic sympathomimetic activity like acebutalol (they do not lower the resting heart rate)

5. Norpace (a vagolytic or antivagal medicine. As you recall, NCS is also called vasovagal syncope because of involvement of vagus nerve)

6. Pacemaker with or without medicine. (I recommend NOT using rate drop response)

Diet, exercise and education are a must. Salt therapy should include at least 4 grams of extra salt.

I am not surprised that your MD has used Norpace. It isn’t unheard of but is a rare therapy. You can show my email to your doctor. It is a do-able job.
By the way, what was your peak heart rate on tilt? (I will not be surprised if you had orthostatic tachycardia)

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