spaceorca

These are all helpful suggestions--thanks. My son has suspected the DDAVP connection in the past, so we may try reducing that a little. He takes both DDAVP and florinef so, having gone up on the florinef, maybe we should cut back the DDAVP. And I'm going to check out some of the info on interstitial cystitis--there's definitely bladder issues with autonomic dysfunction and maybe there will be some more clues in that literature. Thanks!

Yes, exactly! When someone with a well known illness is able to make an extra effort to attend a family event or do something else, everyone applauds that person for their fortitude and effort. With a disease like POTS, people wonder if you're really sick. Two things seem to have helped over the years. One is that all of our family members have by now seen my son at his flat-out sickest, and they have a better sense of what he's fighting. The other is that I often describe POTS by saying that it's like getting off the dizziest, most nauseating ride at an amusement park--but all the time. A really fast-spinning ride can overwhelm the autonomic system for a healthy person; just getting up from a chair has that effect for someone with POTS. It's really interesting, though, that so many of you have seen the same adrenaline surge/crash cycles. One of the most helpful things for all of us is sharing experiences so we know that we're not alone in this. Thanks!

My son hasn't tried any of the ritalin-type drugs that address fatigue directly, but he found that some of the POTS medications helped with fatigue. Mestinon, florinef, and salt tablets probably have been the most helpful overall. If she hasn't tried some of the POTS meds before, maybe one of those (or others that doctors use) will help with the fatigue. I know how hard it is to watch your child struggle!

Does anyone have strong urges to urinate but there's not much urine? Are there any meds or other approaches that help? Or any medications that you've noticed can prompt this? My son is going through a spell of this and we're trying to figure out if it's due to recent med changes (over the last month, increased florinef, increased mestinon, added some prednisone--although not all at once) Thanks for any tips!

My son took octreotide for a short time for his Crohn's disease. That was before his POTS was diagnosed, and I don't remember the octreotide helping any of the symptoms. But everything was such a mess back then, it was hard to know! I do remember that the SC injections weren't too difficult--we did them at home, and you could do them in the thigh or abdomen. Here is a summary from Pub Med of an article published by Dr. H on octreotide. It does sound interesting although, as so often with POTS, it seems to work wonderfully for some but not for others: Clin Auton Res. 2007 Dec;17(6):334-40. Epub 2007 Aug 31. Treatment of autonomic neuropathy, postural tachycardia and orthostatic syncope with octreotide LAR. Hoeldtke RD, Bryner KD, Hoeldtke ME, Hobbs G. Dept. of Medicine, West Virginia University, One Medical Center Drive, PO Box 9159, Morgantown, WV 26506-9159, USA. rhoeldtke@hsc.wvu.edu Abstract The purpose of this study was to determine whether autonomic neuropathy and the postural tachycardia syndrome can be treated with octreotide LAR (Long Acting Release). This was an open-label pilot project. Protocol 1 Patients with autonomic neuropathy (n = 4) were given increasing doses of octreotide LAR once a month for three months. Blood pressure was measured in the sitting posture every two weeks. Pretreatment mean blood pressure averaged 83.8 +/- 7.1 mm Hg. After four, six and eight weeks of therapy the blood pressures averaged 96.3 +/- 6.4, 98.2 +/- 6.1 (p < .025), and 104.1 +/- 3.1 (p < .025) respectively. Therapy led to a dramatic improvement in symptoms in one patient but another had an unacceptable elevation in supine blood pressure. Protocol 2 Patients with POTS or orthostatic intolerance were given 10, 20, or 30 mg of octreotide LAR over three months. Seven patients entered and five completed the study. After two months treatment, standing time increased from 36.0 +/- 9.2 to 59.2 +/- .8 minutes (p < .01). Heart rate in the standing position was suppressed from 106 +/- .83 to 93.2 +/- .8 beats per minute (p < .05). Orthostatic dizziness and chronic fatigue improved. We conclude that octreotide LAR can be used to treat autonomic neuropathy but there is a risk of an excessive pressor response. Octreotide LAR improved standing time and suppressed tachycardia in patients with orthostatic intolerance.

My son was diagnosed with Crohn's about 8 years ago and has had several treatment regimens over the years: Asacol or Pentasa (2 versions of similar drug), prednisone, 6MP, antibiotics, and now Remicade. He just started the Remicade in January, so we don't know how well that will work. Previous treatments weren't too effective: Despite the meds, he developed bowel obstructions and needed part of his intestine resected when he was just 16. He also had a perianal abscess after starting treatment and, last year, a massive internal hemorrhage. The 6MP also destroyed many of his white blood cells. We're hoping the Remicade will be more effective! I would have liked him to try Remicade earlier, but his doctors were curiously reluctant.

My experience with Mayo (both in Rochester and Scottsdale AZ) was that they liked doing their own tests and analyses; they didn't seem that interested (if at all) in previous diagnoses. The downside is that they want patients to repeat a lot of tests; upside is that they may not be influenced by previous centers (even prestigious ones). But doctors within Mayo probably differ--I don't know if our experience was representative. Is your neurologist writing a referral letter? If he/she takes your symptoms seriously and outlines them to the Mayo docs, that should be enough. The neurologist could either refer to the other tertiary center, saying that he/she doesn't think they got it right, or just ignore that other center. Either way, my guess is Mayo would do their own work. None of this is to say that Mayo necessarily gets it right--Mayo Rochester missed the boat on my son's dysautonomia/POTS (since confirmed by multiple experts) and Mayo Scottsdale overlooked the amyloidosis that killed my father about six weeks after they said their tests "showed nothing." But I hope they'll get it right--and offer some treatments!--in your case.

My son's experience is just like this, HEnsor. He is badly affected by POTS, other autonomic problems (like you, he's completely heat intolerant), Crohn's disease, and several endocrine problems. He can focus himself to do something active, but then crashes hugely afterwards. Your description sounds just like what I have been seeing for the last 6-8 years! Now here's an interesting part: My son has already been diagnosed with adrenal insufficiency--he doesn't make sufficient cortisol or aldosterone (the two main hormones supplied by the adrenals). He takes oral medication (cortef and florinef) to replace those missing hormones. Those replacements give him some relief: he was bed ridden, unable even to make these pushes, before the adrenal diagnosis. But he still has the same pattern you describe here. I don't know if his body fails to process these hormones properly, if super pushes of adrenalin can temporarily overcome the dysautonomia, or what it is. If you or others find out more about this, I would be very interested!

Yup, my son's first diagnosis was with Crohn's, eight years ago now. The POTS and autonomic symptoms seemed to come after that, although they might have been present earlier in a mild form. I'd love to hear from others with this combination.

My son has trouble maintaining or absorbing calcium (along with everything else, it seems!) so he takes Citracal. This is an OTC supplement that combines calcium and vitamin D. The tablets are coated, and he finds them relatively tolerable--although he suffers almost constant abdominal pain from his Crohn's disease and dysautonomia, so he may simply be lumping in any side effects of this supplement with everything else! If you are having trouble with bloating, constipating, and gastric slowing, you might discuss mestinon with your doctor. Mestinon helps some dysautonomia patients with their symptoms and it almost always increases gastric motility. Depending on the patient's underlying symptoms, this can be too much to handle: If your gastric motility is normal, mestinon probably will create too much cramping and diarrhea. But if one of the autonomic symptoms is intestinal slowness, it might be worth trying--with a prescription and your doctor's advice, of course. It's a med that you can start very slowly to see if it works for you.

Dr. Grubb, the autonomic specialist in Toledo, Ohio, prescribed mestinon for my son about 4 years ago, and he has taken it since. He tolerates a relatively low dose (right now, 180 mg/day total), but it has been an important drug for him. Not a magic bullet, but it significantly improved his condition--he was virtually bed ridden before taking the mestinon. The GI motility factor actually worked in his favor: chronic constipation and delayed gastric emptying are other aspects of Dan's dysautonomia. Before the mestinon, his intestines had virtually stopped working. The mestinon has both given him some intestinal movement and helped with some other autonomic symptoms. As others have said, this is a medication that you start slow with. It's also necessary to divide it into several doses spread over the course of the day--it stays in the system for just a few hours. That, of course, also makes it relatively easy to try: If you don't like the effects, you can stop taking the med and side effects will disappear pretty quickly. On the other hand, mestinon is a nuisance med to take if you need small doses. It's a chalky pill that crumbles when you try to cut it, and the manufacturer doesn't make doses smaller than 60 mg. There's a liquid form but my son, who's pretty good at tolerating meds, found the taste appalling. So we put up with chopping the chalking pills. But get a pill cutter for these--there's just no way to snap them in half! I hope the mestinon (or something else) works well for you! Debby

Thanks, TXPots! My son also was diagnosed with diabetes insipidus through a water deprivation test: This was years ago, before anyone identified the POTS. He was placed on DDAVP, which he still takes, but his response has always been erratic. Sometimes it seems to help, other times it doesn't. And he finds that too much DDAVP creates unbearable urinary retention (i.e., he just can't force the urine out). All of this, of course, mystified the doctors. If you have central diabetes insipidus, then DDAVP should cure it! If the DDAVP doesn't work, then there must be something wrong with you. (Or, as a nephrologist at Mayo suggested on one memorable occasion: Maybe the lab reported the wrong results from the water deprivation test. That was one of the more extreme examples we saw of a doctor trying to reason away a result that didn't fit with existing patterns.) My son seems to have widespread endocrine failure along with his dysautonomia. He has hypogonadism (doesn't produce testosterone) and Addison's disease (no cortisol) along with the diabetes insipidus and lack of aldosterone (he had almost none, even when ambulatory, both times he was tested). Some doctors have hypothesized that his immune system or some other inflammatory process attacked a series of his organs (in addition to these endocrine and autonomic problems, he also suffers from Crohn's disease that has required several surgeries, so there's clearly an abnormal inflammatory process in his body). Whatever the underlying cause, it seems reasonable to supplement all of these hormones. The other odd thing about my son is that he has never been able to hold onto either potassium or sodium. He needed those supplements even before taking DDAVP or florinef, and he takes significant amounts now just to have a normal concentration in his blood. It's all very hard to figure out but I increasingly think a lot of it revolves around the hypovolemic status of POTS patients and the many factors that can cause that problem. A lot of research suggests that many POTS patients suffer from low blood volume--just not enough going around the veins and arteries! Water, salt, potassium, florinef, DDAVP, etc are all things that help increase blood volume in healthy humans. And if one of these is defective, there may be a good fix for a POTS patient. But there also seems to be a growing suspicion that there's something else wrong, something that's setting the blood volume too low in these patients. So the search for the right combinations or other contributing factors continues! Meanwhile, we'll watch out for that florinef headache--I hadn't heard about that. Best, Debby

Dr Raj's team at Vanderbilt recently published a study showing that a low dose of propranolol (a beta blocker) worked better than higher doses for POTS patients. http://www.ncbi.nlm.nih.gov/pubmed/19687359. So another variable to consider may be how much of a beta blocker to use. As the title of this article suggests, "less could be more." My son hasn't tried a beta blocker yet, but we may try one soon. We're currently adjusting amounts of the florinef he takes and don't like to change more than one med at a time.

I was really interested to see this thread because my son has both low aldosterone (almost non-existent) and low renin activity. Two separate tests demonstrated how low these were, but none of the doctors responded to them at the time. Later, after his POTS diagnosis, he started taking florinef and that helped him a little. But here's an interesting point I discovered in following up on this research earlier today. UpToDate, which is the database that many doctors use for diagnoses, treatment protocols, etc., notes that many patients with low aldosterone and low renin (or even some with just low aldosterone) also are resistant to the action of aldosterone--so they need more than the usual replacement dose. For someone with impaired adrenal function, the "replacement" dose of aldosterone is 0.1 to 0.2 mg of florinef per day. But patients who are resistant to aldosterone need 0.2 to 1.0 mg of florinef per day according to this database. This discussion did not refer to POTS--it was a general discussion of how to treat low aldosterone. But it made me wonder: Could some POTS patients benefit from higher doses of florinef than is usually prescribed? Is anyone here taking more than 0.2 mg/day? My son currently takes 0.15 mg/day and that amount seemed to help when he first started the florinef. I'm wondering if he's in the resistant category and if it would be worth increasing. Thanks again for the interesting thread, Debby

This is crazy awful. I'm a professional in a different field and I can't imagine treating a patient/client this way. How unethical! My heart goes out to you.

About two years ago, one of my son's doctors thought he might have MNGIE, so we had the genetic tests done. I think my son's blood was sent to the University of Cincinnati for testing. There are only a few labs that do the MNGIE test, but any good local lab can draw the blood in consultation with one of the testing labs and send the blood off for testing. So if you want to resolve the MNGIE issue, you probably could have the test done without leaving home (as long as you have a good doctor/lab who will work with one of the specialty labs). Warning: I think we had to pay specially for the test--it's not a "recognized" diagnostic test so our insurance (which is usually very good) didn't cover it. The cost was somewhere between $400 and $1000. We've had a few of these special tests done, and I forget exactly where the MNGIE fell on that spectrum. My son doesn't have MNGIE (we were pleased about that!) but he does continue to have an array of serious gastrointestinal, neurological, and endocrine issues--like so many people here. My sense is that doctors ignored autonomic disorders for a long time and happened to name just a few variants of them. Now they struggle to diagnose and treat all of the patients who don't fit within those particular categories yet have equally serious symptoms. It's frustrating to have a disease that's so poorly understood! I wish you luck with your ongoing diagnoses, Debby

My son has similar bowel problems: his GI transit time is unbelievably slow. Based on what we've been told, you're smart to avoid fiber. When the autonomic system is at the root of a slow bowel (as it is with POTS), then fiber doesn't help--it just gums things up further. My son has gotten the best relief from Mestinon, a prescription med that many patients take now for POTS. It helps somewhat with his fatigue, dizziness, and heart rate, and is particularly helpful with increasing bowel mobility. It doesn't work for everyone, but you might want to ask your doctor about it. Good luck! Debby

My son, who has POTS as part of a more general autoimmune autonomic neuropathy, is having more and more trouble sleeping. He's tried various sleep meds (ambien, sonata) and other techniques (regular schedule, sleeping partly propped up) but it's getting harder and harder for him to sleep. It seems like his sleep mechanism just doesn't work! Even when he's exhausted, he can't sleep. I think this is common with POTS. Does anyone have suggestions for other approaches or meds for him to take? He's had a full sleep work-up and doesn't suffer from sleep apnea. The sleep doctor has tried him on the ambien and sonata but those each worked only for short periods. We see his neurologist tomorrow and internist on Wednesday so I'm trying to gather ideas to ask them about--or for us just to try. Thanks--I always appreciate the advice and support on this forum! Debby

Michael, I'm so sorry to hear what is happening to you. Some of your symptoms are similar to those of my son, Daniel, who was diagnosed with POTS several years ago. Dan started getting sick when he was 16; he's almost 22 now. One thing you and Dan have in common is that Dan's illness also started with OCD. That was pretty easy to control with prozac (which he still takes), but then he developed a whole series of other diseases: Crohn's Disease (bad enough to have some of his intestine removed), POTS, other autonomic problems, and some hormonal losses. The doctors think that some type of autoimmune disease lies behind all of this--that Dan's immune system has attacked different parts of his body. I have read a few articles suggesting that OCD may be related to autonomic dysfunction--that's one reason I find your story similar to my son's. And I know that the symptoms of POTS have been the worst my son has faced. It's a truly miserable disease that leaves patients dizzy, nauseated, and fatigued most of the time. Making things worse, few people understand the disease and doctors lack good treatments. Based on my son's experience, I can offer this advice: First, try to find a doctor who specializes in autonomic disorders and keeps up to date with recent developments in the area. POTS is an autonomic problem and many doctors don't understand much about that system. Second, you might ask your doctor about mestinon. Some POTS patients have started taking that medicine, and it is the one that seems to have helped my son the most. Salt tablets also help. Third, try to brainstorm with your family about creative approaches you can take to creating the best life possible with your disease. We got Dan a wheelchair to use when he was feeling really sick. He didn't want to use it at first, because he thought it was dorky, but then he realized that it helped him get together with his friends. Then we moved to an apartment right next to a big college campus. That allowed Dan to attend some classes and get together with friends without having to undergo a car drive (very tough for POTS patients) and without having to walk much. When he felt really sick, friends could come see him easily. Although we had a nice suburban house before, it was just too isolating for a young adult with POTS. I don't know your family's situation but you may be able to come up with ways that will make your disease a little easier to bear. Good luck to you. I have no idea what causes this terrible disease in you, my son, and other patients on this site--but you have all my admiration and prayers as you try to fight back.

My son suffers from POTS and other dysautonomic symptoms. He definitely suffers from travel sickness, and one of his doctors explained it this way: When the autonomic system works properly, it constantly calibrates blood pressure to adjust to movement and other changes of position. With dysautonomia, the constant calibration doesn't work. Relatively routine movements--like riding in a car--completely overwhelm the autonomic system. The effect is similar to the ones experienced by a healthy person who rides on a really fast amusement park ride: nausea, dizziness, and similar symptoms. For the healthy person, it takes a monster "amusement" ride to overwhelm the autonomic system. For someone with POTS or other forms of dysautonomia, a car ride can do it. In addition to understanding what happens when our son rides in a car (or even gets up and down frequently), this analogy has helped us explain POTS to people unfamiliar with the disease. As soon as we explain that walking around or riding in a car makes our son feel like someone who has just gotten off a dizzy amusement park ride, they turn slightly green and seem to understand! Best wishes to you. In addition to riding in the front seat and tilting slightly back, which another member suggested, we have found that riding in a larger, more stable car helps. Maintaining an even speed (highway driving) often is better than stop-and-go traffic. Although, of course, you often can't avoid the latter!

Hi, everyone. Several years ago, Dr. Grubb diagnosed my son Dan with severe dysautonomia. Since then, Dan has taken Mestinon and a number of other drugs that help somewhat (although he's still pretty disabled). We're not able to travel frequently to Toledo, and Dr. Grubb has cut back his practice, so we're working now with a new doctor in our home town. This new doctor seems very good--interested in and at least somewhat knowledgeable about autonomic disorders. He doesn't see the need to do a whole range of tests on Dan, but would like to do a QSART. To do that, Dan would need to go off some of his drugs for 48 hours. Our question is: How severely will going off the mestinon affect him? I'm particularly concerned about GI effects because, prior to taking the mestinon, Dan was hospitalized several times for pseudo obstruction (i.e., intestines that had virtually stopped). The doctor is sympathetic and we're going to talk with him more about this, but I wanted to check on experiences any of you have had. Have any of you gone off mestinon for a few days for testing? Has going off the drug had severe effects? Were you able to go back on and regain whatever help you had obtained from it? I have the same questions with respect to flomax, which seems to help Dan's urinary retention. So any comments on going off that drug would also be welcome. But mestinon seems like the big question! Thanks, Debby

Along with others here, my heart goes out to you and your daughter. I'm another "desperate mom" -- my son developed POTS and other problems about 5 years ago, when he was 15, and it has been a long (still ongoing!) road to find help. There were several times when I thought he was about to give up. My son also tried most of the meds you listed and didn't get much help. The med that has helped him most, and that I didn't see on your list, is Mestinon (pyridostigmine). Dr. Grubb, the POTS expert in Toledo, presribed that for my son. You have to start with very small doses and work up, but ultimately it is the med that has made the most difference for my son. He's far from cured--there are plenty of absolutely miserable days--but it made a big difference. My son has a lot of GI problems (slow stomach emptying, intestines that barely move) and the mestinon helps with those issues as well as other autonomic ones. That's another reason I mention this as a med to ask about for your daughter--maybe it would help address the nausea. Most of all, know that we're all here for you and your daughter. You're absolutely right that this is a crazy, mind baffling disease--and it's particularly disheartening when so few people understand it.

My son has an immune-system problem but it runs in the opposite direction: He appears to have a hype-immune system that is always working over time. In fact, the doctors' current theory is that his POTS and other conditions are autoimmune--i.e., that they arose from the immune system attacking parts of his body, including parts of the autonomic system. I wouldn't be surprised if there were some connection between immune disorders and POTS, but with the possibility of it going either way.

Unfortunately, you're right--I thought after the recent large study it had been licensed in the US, but I see that it's not. Yet another bizarre outcome from the FDA!