spaceorca

The heat is definitely causing my son more problems than usual this summer. He doesn't sweat, so we know the summers will be bad--it has always been hard for him to spend much time outside in the summer. But somehow this summer has been even worse than the previous ones. He sometimes gets relief from a coldpack. We have one of those gel packs that can be reused--you stick it in the freezer to rechill it. My son will wrap that in a towel and place it around his neck for cooling. Hang in there!

My son has had fatty liver for at least the last 5 years. He's just 22 now, has eaten almost no fat during the last 10 years (due to Crohn's disease), and had his first tastes of alcohol just during the last few months. (I know some parents wouldn't know what their teens drink, but he's been too sick to go out of the house much of this time so I know!) The fatty liver hasn't affected his liver functions (yet, anyway) and the doctors can't explain it. They agree that it's very weird, but it seems to be linked to some type of systemic disease that (we all assume) caused the Crohn's, POTS, other autonomic problems, and various endocrine problems. It's very weird--if anyone else discovers something about fatty liver, I'd love to know!

I also am so sad to hear about this--it must be so hard for you and your family to bear. In addition to Mayo and the Cleveland Clinic, which dsdmom mentioned, I think that Johns Hopkins and the U of Pennsylvania both have helped some children with severe dysautonomia. But I don't have firsthand experience because my son was in his teens when he first became ill. I think that almost any major children's hospital would give you better support than it sounds like you have currently. Are you near a metro area with a major children's hospital? We'll be thinking of you, Debby

My son also had a sleep study that showed he got no deep sleep. But the sleep doctor dismissed that, saying "no one gets deep sleep in the sleep lab." The study did rule out apnea, narcolepsy, and restless legs--so that was somewhat helpful. But from my son's daily reports, and my anecdotal observations, my guess (and his) is that his problem *is* exactly what the study showed: no deep sleep. We see his sleep doctor again later in the summer, and I'm going to ask him this question: If a patient's problem *is* lack of deep sleep, how do doctors determine that (given their assumption that no one gets deep sleep in the lab)? And is there anything we can do about it? The dysautonomia contributes to my son's sleep problems in lots of ways: pain, night sweats, and polyuria wake him frequently. But it seems that there's also an underlying problem with the sleep mechanism, and that's what I'm seeing from other patients on the forum as well. Frustrating!

Sandy, Mack was also on reglan for about 5-6 months- vile drug. I do get angry when I recall how many phone calls I had to make to his ped GI to get him off & on the erythromycin. His nurse adamantly insisted Mack was fine, perfectly safe drug, Grrrrrr. Mack actually blames the tremor on that. Mack may be right: Tremors are a well known side effect of reglan. I don't understand why doctors prescribe this drug for kids! Sometimes the tremors and other neurological symptoms subside within a few months after stopping reglan--how long has he been off? You might want to note the problem to your pediatrician to see if there's anything to be done without extensive further testing. Since Mack already has a dx of POTS (and other diseases?), it seems unnecessary (to me, anyway) to do a lot of testing for other diseases that sometimes include tremors. But maybe mentioning it to the doctor would allay any of Mack's worries (e.g., maybe the doc would say that it probably is an after effect of the reglan) or maybe there's something the doctor would suggest that could help the tremors and other aspects of POTS. The most thoughtful doctors sometimes pick a particular POTS med out of the array because of a symptom like this. E.g., they'll think "well, this one is known to help tremors so let's try it, while that one can aggravate tremors so it would be the last one to try."

Thankful, My son never tried tegretol, but I know that trileptal has a very different side effect profile. Although they're related meds, the big difference is in side effects--trileptal is a variation that addressed some of those side effects. Here's one site I found quickly on the web, summarizing some of the differences I'd heard about: http://www.psycheducation.org/depression/meds/trileptal.htm That site deals with tegretol and trileptal used for bipolar disorder, rather than for POTS and similar conditions. But the comments there are similar to the ones the neurologist made 5 years ago when he started my son on trileptal. Just wanted to note this in case you want to ask your doctor about trileptal--it's possible the side effects would be lower for you than with tegretol (especially if you start low and increase slowly as this site suggests). Good luck with the meds and your foster child!

Paint the Moon, I hope this report will give you a mental boost. I'm posting the note separately for other members as well. Dr. Low and the Mayo autonomic team just published a study of 116 women who had POTS while pregnant. There were no adverse events, for mother or baby, related to POTS. And overall, the rate of complications in pregnancies/deliveries with POTS mothers were no greater than with other mothers. The study also identified a "trend toward modest improvement" of POTS symptoms both during pregnancy and after delivery. The improvements weren't large, so the researchers concluded that that pregnancy and childbirth have no significant long-term impact on POTS. But the good news is: (1) no harm to the baby from POTS; (2) no trend toward worsening of symptoms for mothers; and (3) possibility of improvement of symptoms. If you experienced improvement during the first months of pregnancy, Paint the Moon, hopefully that will continue for you after delivery! I haven't read the full study, but the summary is on pubmed: http://www.ncbi.nlm.nih.gov/pubmed/20516426

A doctor in the Mayo Clinic's pain department prescribed nortryptiline for my son, who has agonizing pain in various parts of his body. After taking the medication for a few days, my son's urinary retention (which he'd reported to the pain doc among other autonomic symptoms) got much worse--he had to be catheterized to get the urine out. We called the Mayo pain doctor back and he said rather blandly, "oh, yes, nortriptyline often has that effect." Of course, he didn't tell us that in advance and apparently didn't think about the fact that my son already suffered from urinary retention: he just prescribed the medication at the top of his list. So then he suggested neurontin. That works well for many patients, but it also causes depression and suicidal tendencies in teenagers (again, as we discovered later, side effects that are well known to doctors)--and my son was only 17 at the time. Luckily, he was self knowledgeable enough to recognize what was happening. He came to us and said, "since I started taking this medication, I've had very strong urges to kill myself." Dan was worried enough about how the medicine was distorting his feelings that he asked us to watch him while he was weened from the med. We called the pain doctor who said, "oh, yes, I thought maybe your son was old enough at 17 but maybe he wasn't." At that point, he washed his hands of us (although we were also done with him). Another doctor prescribed trileptal for Dan, and he has been taking that for almost 5 years now. It helps a little with the neuropathic pain, and more with tremors. So that one has worked for him. I confess, though, that the medication that best controls Dan's pain is oxycodone--and he takes that on a regular basis, monitored by his internist. It has no negative side effects for him, and he hasn't developed a tolerance; his usage actually has diminished somewhat over time. So many doctors prescribe all kinds of meds for pain, but refuse to consider narcotics--although the narcotics may be *less* likely to mess with the nervous system. (Dan's gastroenterologist likewise told us that the oxycodone is better for his liver than many of the other meds prescribed for pain.) But reactions to all of these drugs are very individualized. I hope our experiences are useful as a guide, but other people have good experiences with nortriptyline and neurontin--and bad experiences with the drugs that have worked for Dan. I just wish some doctors would make a more individualized judgment upfront (like considering the parasympethetic effects of a drug like nortriptyline in a patient whose autonomic system is compromised in exactly that way). Instead, we patients have to do that!

Yes, Dan has lots of tremors in different parts of the body. You reminded me that he used to have them quite frequently in fingers and his hand. Those have not been nearly as common during the last few years. I think Trileptal, which he's taken for years, may have reduced those. But he still gets fairly severe tremors in his legs. Those come and go--I haven't noticed any particular patterns. When the tremors or tingling nerve sensations are particularly bad, he'll take a xanax--that often helps with the tremors. Sorry to hear Mack has this!

Keely and Pandygirl, My son also has been diagnosed with pandysautonomia. He has been tested for AAG, twice I think, but the results were negative. His doctors say: "The pandysautonomia probably is caused by an autoimmune reaction. AAG is just one type of antibody that identifies an autoimmune reaction; there are at least dozens (probably hundreds) of others that we can't identify yet with clinical tests." In other words, pandysautonomia, POTS, and other phrases identify the system that is broken in the body. For most patients, doctors don't know what is causing the damage. For ones who are positive for AAG, they reason that the damage might have been caused by those antibodies (although they still don't know for sure). One of the points that has frustrated me is that my son's doctors have not been willing to pursue IVIg--although they clearly take his illness seriously and have prescribed numerous other treatments (including Remicade infusions, which he's currently doing). But IVIg seems to be a treatment that works for some patients and has relatively few side effects; so I've been frustrated that no one has been willing to try it. Most recently, our neurologist said that he definitely would have ordered IVIg if he'd treated my son during the early months of his illness--but that he didn't think it would be effective so many years after the start of the illness. So I'm interested that your doctor was willing to start this so many years after the advent of your symptoms, and it's so great that you're responding somewhat. What a frustrating disease--both the physical processes and the difficulties with physicians (even those who really want to help but don't seem to have a good knowledge base to draw upon for this disease).

Many people (including me) like gluten-free breads better when they're toasted. But watch out for your toaster: It's impossible to clean out the crumbs, so you'll have to get a new "gluten free" one. If you live with wheat-eaters, they can continue to use the old toaster while you use your gluten-free one. But you may need to put a label on it so they'll remember! I like the Whole Foods line of gluten-free breads, scones, etc. I've never tried baking my own gluten-free bread (I'm just not much of a cook) but my husband makes me gluten-free belgian waffles. They are terrific! If you happen to be Catholic, you need to ask for a gluten-free wafer at mass. Some churches were resisting this, but I think the tide has turned. An increasing number of chain restaurants have gluten-free menus. My all-time favorite is PF Chang's Chinese food. Believe it or not, the Olive Garden has a gluten-free menu! Yes, gluten-free food at an Italian pasta restaurant; odd, but reliable. Good luck with the diet!

I am curious about this as well. I have pestered various doctors to try IVIg with my son--numerous medical reports suggest that it helps some patients. But the treatment is very expensive, and doctors have to complete extra paperwork to order it. That seems to make them reluctant, even when I've said we would pay for the treatment ourselves rather than seeking insurance reimbursement. I was never able to get a doctor to order it. Our son's current neurologist said last year that he probably would have ordered IVIg if he had treated Dan earlier in the course of his disease, but that he didn't think it would be useful now: most reports suggest it is most useful when used within the first few years after the disease starts. I checked the medical literature and, sadly, it seemed that this might be true. So we have been focusing on other treatments instead. But I have always wondered what IVIg might do, so am curious to hear from others here.

Elfie, I'm sorry you're feeling so stressed! Autonomic dysfunction is such a disabling disease that I don't know how any patients cope as well as they do. I don't have any magic coping strategies, but my heart is with you! Debby

My son definitely suffered persistent nausea unrelated to medications--I think it's quite a common symptom with POTS and other autonomic disorders. One cause is gastroparesis, slowed emptying of the stomach. Because the stomach and intestines don't work normally, they cause feelings of nausea. Another is from the POTS blood-flow problems--feeling dizzy is closely related to feeling nauseous. Some people feel one more than the other. The best non-prescription treatments my son found were ginger tea and dramamine. Avoiding a lot of movement (especially car rides) also helped. Some people have success with those wrist bracelets that stop motion sickness, but they didn't seem to work for my son. Some of the prescription drugs he has used are phenergan (very effective, but makes him very sleepy) and zofran (not quite as effective, expensive, but doesn't make you sleepy). He also tried marinol (a prescription derivative of marijuana!), which some people find quite effective for nausea. But it didn't help Dan's nausea as much. Hope some of those ideas help your friend. My son's nausea was so bad at one point before he was diagnosed that I remember him walking around the house with a bowl, stopping occasionally for a few dry heaves. Gross to describe but the nausea was so constant that it had become a commonplace backdrop for him!

Mestinon has been the drug that helped my son the most. He started with a very low dose, just 1/4 of the 60 mg pills taken 4 times a day and worked up to 180 mg total (2 full pills and 2 halves, spread over the day). He has severe Crohn's Disease and gastroparesis along with POTS and, as another patient mentioned, the Mestinon actually helps with his GI symptoms. In fact, it probably has been more important in maintaining motility than in its other effects. We have found that timed release pills don't work well for my son (probably because the slowed gastric motility and prior intestinal surgery affect the way he absorbs meds) so he's never tried the timed version of mestinon. One advantage of the regular form is that you can start with very small doses, work up, and adjust the dosage as your symptoms shift. Periodically my son will add or subtract a little mestinon depending on how his body seems to be reacting. But if the timed release works for your daughter, that may be a good option as well. Oh, on neck pain: for many people with autonomic disorders, this is a blood vessel/autonomic problem--something about how the body is trying to get more blood to the brain. I read somewhere that putting a cold gel pack (or bag of frozen peas!) on the base of the neck or shoulders can help, because it constricts those blood vessels. That worked wonderfully for my son. He doesn't get the neck pain often but, when he does, the cold pack works very well.

Good luck, Maxine! I hope the procedure goes well for you. If your doctor does find low cortisol, or if you've had that diagnosed in the past, I would be sure to ask the doctors doing the colonoscopy about it. They probably will want to give you extra cortisol (my son also has Addison's disease--low cortisol--so this is routine for him). But the doctors may forget to ask so, just like asking for our own tests, you may need to remind them! We definitely have that ask-for-your-own-test problem, although (like you) with supportive doctors. It's interesting how many POTS people have paradoxical reactions to medications. But I guess not surprising on reflection because the autonomic system has so many feedback loops. If a med affects one part of the system, and the system is already disturbed, it's plausible that the final outcome would be different than in the autonomically healthy. Hmm.

The medical literature shows that the disease does sometimes go away--sometimes patients with really horrible symptoms will get entirely better. On the other hand, the disease persists in some patients. No one really seems to know why, and it's hard to know what the relative percentages are--although, as I read the literature, a majority of patients do improve. It makes sense that patients who have had the disease for a longer period would participate, and remain in, a forum like this one. So the majority of patients here may suffer from a chronic form of the disease, even if the majority in the world more generally get better. As I've see it, based on my son's experience, the important things to remember are (1) this *is* a serious disease no longer how long you end up having it--don't let other people trivialize your symptoms! (2) even if you have the disease for a long time, medications and lifestyle changes improve almost everyone's symptoms somewhat. (3) doctors took a long time to focus on POTS and other autonomic disorders, and many patients have suffered all sorts of problems getting a diagnosis. But portions of the medical community really are focusing on the disease now, and new treatments are emerging all the time. We can all hope for improvements! Exercise is a double-edged sword. It seems that some exercise can help improve almost every patient's symptoms; lots of people on the forum report improvements with different types of exercise. But exercise rarely is enough on its own. If your doctor says that, press him/her for more information on other treatments, or find another doctor! And many POTS patients have to tailor their exercise; doing too much can also set you back. Most people suggest going slow, seeing what types of exercise work best for you, and building slowly if possible.

Tara Lynn, welcome! My 22-yr-old son has struggled with Crohn's disease, POTS, and other autonomic dysfunction for years. The doctors "got" the Crohns' disease right away, but--as in your case and so many others--dismissed all of the other symptoms. Ironically, once the POTS and other autonomic problems were finally recognized, the doctors concluded that some of his earlier surgery (removal of part of his intestine when he was 16) might have hurt more than it helped. I.e., the "obstruction" that the gastroenterologists thought they had to remove might really have been autonomic dysfunction. Well, at least we are on the right track. My son has lots of abdominal pain. His current doctors suggest that there are two primary sources: (1) slowed motility due to his autonomic/POTS problems; and (2) heightened pain sensitivity that is also related to the autonomic/POTS problems. The key is that the same "small fiber" nerves govern both autonomic function (like stomach motility and heart rate) and pain sensation. If a patient has small-fiber neuropathy. then both motility and heightened pain sensation may be a problem in the GI tract. In particular, the heightened pain can occur with adhesions or other surgical procedures. A patient with small-fiber neuropathy, in other words, may find adhesions more painful than other patients do because, in addition to any inherent pain, the nervous system itself is over-firing. As you've probably already seen, med response is very varied among POTS patients. The best relief my son has gotten on the motility issue has come from mestinon (in fairly small doses). For direct pain relief, he needs oxycodone, and takes that on a regular basis--without that, he simply wouldn't be able to eat because the pain is so severe. He has also been helped by eating small amounts and a low-fiber diet. His motility is so slow that if he ever ate a piece of brocoli, I suspect it would get stuck! It's great you've been diagnosed and have found a supportive doctor. The folks on this forum are super--I'm a relative newcomer and have found lots of help and support here. Cheers, Debby

My son has a full set of autonomic problems and Crohn's disease, so has had many colonoscopies. He does fine with proprofol. In fact, he affirmatively likes that aspect of the colonoscopy because he gets a much better sleep while sedated with proprofol than at almost any other time. The med does make him very sleepy for a day or two after the procedure, but that's normal for any patient. A sedative that my son has had problems with is versed. The problem for him is that it doesn't work; in fact, it sometimes has a reverse effect--i.e., it make him more aware of what is happening. He had one spinal test and one stomach procedure done with versed, and they were both torture: he was awake, screaming, and begging the doctors for more medication. Afterwards, they realized that he was having a reversed reaction to the medication. (You'd think that would have been obvious from the screams, but ...) And some of his doctors have hypothesized that the autonomic dysfunction may relate to this backwards response that he has to versed. I don't know if others here have experienced that, or if it really is related to autonomic disorder.

I just saw a new article by the Chelimsky team in Cleveland--there's a summary on pubmed at http://www.ncbi.nlm.nih.gov/pubmed/20479685. They did a procedure called electrogastrography (EGG) on patients who were doing a tilt table test. The EGG measures electrical activity in the stomach. (My son has never had the test, so I don't know exactly how that works--I'm just relying on the article for this.) The study showed a very significant different between POTS and non-POTS patients: for non-POTS patients, their stomach electrical activity became more normal with upright tilt. In other words, their GI systems worked better while upright. For POTS patients, their stomach electric signals became significantly more abnormal with upright tilt. The team hypothesizes a number of ways how this could explain the nausea, abdominal pain and other GI symptoms that POTS patients experience. No treatment recommendations from this article, but it's yet another documentation of the realness of POTS symptoms--and hopefully will lead to further understanding of the disorder.

Here are a few thoughts on this very interesting thread: First, as many probably know (but for the benefit of some newbies), there are two types of "sweat" tests. One is the thermoregulatory sweat test where they powder you up, put you in a box, and heat you up. My son has refused to have this test done (and, in fact, none of his doctors have pushed it) for exactly the reason suggested by the Mayo experience: It is so clear that he doesn't sweat, and that he quickly overheats, that it would be very dangerous for him to do this test. Hyperthermia is very dangerous!! I personally think it is irresponsible for any doctor to suggest the TST for a patient who already has clear "every day" evidence of low sweat function. In my opinion, the risks just aren't worth the benefits of seeing just how much the patient does and doesn't sweat. And what a horrible, horrible experience for you, your family, and everyone else involved! There is another type of "sweat" test, the QSART, that is quite different. They put little suction cups on five places on your body (legs and arms) and prick the skin in those areas with a little acetylcholine (a hormone that provokes sweating). For some patients, the test causes modest discomfort in those areas; for others, it is barely noticeable. But with electronic tracings, the doctor can tell how the autonomic system is responding. In particular, this test can indicate whether there is an underlying small fiber neuropathy. Based on this test (and all of the clinical findings), my son's neurologist was quite comfortable diagnosing a small fiber neuropathy. He told us, in fact, that many biopsies fail to confirm a small fiber neuropathy because it is hit or miss whether the biopsy happens to come from an affected area. And some people with small fiber neuropathy have heightened pain responses. This is true of my son, so the doctor worried about him developing permanent pain at any biopsy site. Just a few further thoughts on these different tests!

We also live in Columbus, Ohio. We lived in Champaign, Illinois, when my son (the POTS patient) was born and moved to Columbus when he was seven. His symptoms started a few years after we moved to Ohio, but I don't know if there's any connection with the state. His symptoms, unfortunately, get worse whenever/wherever we travel. In addition to the travel itself provoking his symptoms, it seems like his immune system goes into overdrive whenever he's in new surroundings. It was actually something we noticed before the more constant symptoms developed--he started getting very sick whenever we traveled. All very odd, but it's nice to know there are so many Buckeyes on board!

i just saw this description of a research study showing a correlation between non-alcoholic fatty liver disease (NAFLD) and autonomic dysfunction: http://www.ncbi.nlm.nih.gov/pubmed/20460914. The researchers have published a few articles on this correlation. I found it interesting because my son's CT scans and other studies repeatedly show fatty liver disease. Since he doesn't drink, doesn't eat much fat, and is thin as a stick (due to his Crohn's disease), we always found this puzzling. Some doctors suggested to us that NAFLD is really a type of inflammation of the liver, and that this might be consistent with an auto-immune process underlying our son's disease. These researchers now see a possible link to autonomic dysfunction. The article doesn't produce any new therapies, but it could be yet another step on the road to understanding. Have others been told they have "fatty livers"? If so, you may find this interesting.

My son is another one with pandysautonomia and Crohn's disease, but low CRP and sed rates. The doctors all assume that his conditions are caused by inflammation, but those markers rarely are elevated. I think, like so many other tests, they must be underinclusive. Antibiotics are such a double-edged sword. They're life saving in many circumstances, and they sometimes have helped patients with Crohn's and many other inflammatory diseases. But they can also cause neuropathy, which in turn can produce autonomic problems. My son's neurologist has marked his chart so that he will not receive metronidazole (flagyl) again. He's had that several times after bowel surgery or intestinal infections, but it has caused autonomic neuropathy in some IBD patients. So the neurologist felt it wasn't worth the risk--although my son can take other antibiotics as necessary.

My son was tested for Wilson's several years ago (at my request, after reading articles about the symptoms) but the results were negative. It's an easy blood test, so seems worth checking for anyone with autonomic symptoms. Even though few POTS patients will end up with this diagnosis, Wilson's and other diseases like it seem to point to the underlying physiology of POTS: something is affecting those basal ganglia or other parts of the nervous system. For a lot of patients, that something and how to treat it are still elusive. But maybe a researcher will figure it out, as they did with Wilson's.