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Hello and nice to meet everyone.
My daughter (13yrs old) developed POTS 16 months ago and we have found an amazing doctor who has been very helpful with everything, but she still has some difficulties. The onset of POTS led doctors to find she has an enlarged aorta which led to geneticist diagnosing her with EDS (she believes its either VEDS or HEDS) and scoliosis. Prior to all of this she was diagnosed with ADHD combined, generalised anxiety disorder, mood disorder, sensory disorder and growth hormone defiency. I always knew those were signs of something bigger so was not shocked to learn of the EDS once everything kicked in full force with the onset of puberty. Sure it has been a long stressful and difficult journey, but I really feel we have reached the point in the road where we can finally look for solutions vs causes.
The POTS especially when combined with her mental health disorders has totally changed her life. She spent nearly a year unable to function. She had to be removed from school and had home bound tutoring provided by our school board. She missed most of 7th grade and as the new school year is approaching I have desperately searched for symptom relief so she can finally live a more full life. She was awarded a new school situation for the coming school year which will be a tremendous help, but in order for that to work we need to get her tachycardia under control. So I am hoping maybe people here might have some suggestions. Her POTS is pretty severe with extremely low BP that drops even more upon standing. Her laying HR is 80’s, sitting is around 105 and once standing immediately begins to climb and with in a min is between 125-185. I do not know for certain it will still reach 185 as is did during all of her monitoring tests prior to any medications etc. Currently her medications include
Prozac, adderall, clonzepam (which made a huge improvement with dealing it it all) fludrocortisone .1mg x2 (however she can only take one due to side effects) and midodrine hcl 10 mg once every 4 hours up until 6pm. The midodrine gave her pretty good relief, but it has not been able to push her BP into a better range it was still after 2 doses 101/68 at her doc appointment. She also had weekly infusions, wears constriction stockings, drinks insane amounts of fluid and does PT. Her doctor then decided she should try desmopressin aceste however insurance is fighting that one so haven’t a clue if it would help. She is also open to diamox if the desmopressin doesn’t get her fluid retention up enough to stabilise the BP and HR. Beta blockers are not possible due to low BP.
So I’m curious what others feel helped get the BP in a better range and lower their HR so that they would enjoy life more. A side note she also has nasty heat sweats that seem like nasty 1-4 hr long fevers. She will complain her body aches and hands hurt just as she did when little just prior to the onset of a fever with no other symptoms. Just aches and fever and then it will suddenly go away with her body temp a bit below normal.
Thanks in advance and hope everyone is having a wonderful weekend.
Hello Abbilou,
I do feel for you daughter, having all of those troubles at such a young age, she’s in no way had it easy. It’s unfortunate that getting a label for a condition leads you to believe that you are at the end of your journey but, in reality, it merely sets you out on the beginning of another journey, to find the root cause. The good news is that once you know what you are dealing with, you can manage it better.I would ask if your daughter has had an MRI scan of her head and neck ? It seems to me that there’s every possibility, from the symptoms and diagnoses you describe, that she may have some level of raised intracranial pressure and I say this for 3 reasons.
The first reason is that any additional pressure on the pituitary gland (can cause an ’empty sella’, or ‘partially empty sella’) which can manifest in problems with various hormones, like growth hormone for instance, which you mention and you also mention problems escalating with the onset of puberty.
The second reason is, that this raised intracranial pressure can cause the brain to get pushed into a position that compromises it’s function, which could lead to some of the more psychological as well as physical malfunctions. I have heard many mothers on the various forums over the years complain about their childrens behaviour. They talk of hyperactivity, moodiness, anxiety, rage, indeed many extremes of behaviour.
The consequence of this pushing of the brain, is that sometimes, the lower part of the hind-brain, called the cerebellum (or to be more precise, the cerebellar tonsils) get forced into a tight squeeze with the brainstem, which in itself can cause the autonomic symptoms that your daughter suffers, the POTS.
So, if you have already had an MRI, I would be interested to see it, not just hear the report. If you haven’t had one, I would try and arrange one, to put you in a more informed starting position for finding a solution to your daughters problem.
Regards
Barbara
(UK)
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Head & Neck Injury (June 2002); Mild Concussion; Post Concussion Syndrome; Postural Orthostatic Tachycardia Syndrome (POTS); Peripheral Vestibular Dysfunction; Mild Radiculopathy & Small Fibre Neuropathy (right leg & foot resp.); Partially Empty Sella Oct 2002 (worse by Oct 2004); Whiplash Associated Disorder (WAD); 3mm Cerebellar Ectopia (Chiari 0); Cranio-cervical Instability (CCI) with Posterior Gliding (PG) & Cranial Settling (CS); Brain Compression; Retroflexed Odontoid; Stretched/Elongated Brainstem; Ehlers Danlos (EDS) type 111; Osteoarthritis; Arrhythmias (Bigeminy and Trigeminy). . . and now Mitochondrial Dysfunction, Mineral (Mg,Mn,Cu,Zn,Selenium), CoEnzymeQ10 & Vitamin (C,D,B3,B12) Deficiencies!Thanks for responding. She had an MRI/MRA on the books but needed to cancel it due to some severe symptoms she was having which would make doing it with out anaesthesia impossible and anaesthesia is not an option; however, we are in the process of getting it pre-approved again, so will hopefully have it soon. But an unexpected and unwanted insurance change may prolong the wait, so in the mean time I’m looking for help with symptoms as it is all I can do. She is too young to not be able to enjoy life and so I seek and will continue to as long as its needed.
But I think it was miss understood when I said at the point in the road where we can look for solutions vs causes as being the end of the journey. We are no way at the end. But now we can spend some of our time working towards solutions because we have some answers vs having none before. So many conditions can come with the diagnosises she has and we will always watch for those as well as for any other root causes, but instead of being totally in the dark we now have some answers that puts us in a much better situation than before. Before each thing came with out understanding so they would blind side us and make us feel like we were starting over again and again and that no one understood or could help. Knowing what I know today puts us in a far better position to help her vs where we were a year ago. She now has a great group of doctors (we are very lucky) who are knowledgable and aware crazy stuff comes with these conditions and our goal is staying ahead of it.. We are very lucky to have an excellent knowledgable POTS specialist, but she is no miracle worker with a magic wand sadly. So in the mean time I really want to find relief for some of the awful symptoms that come with the POTS
Hi
At the time of my first MRI, which was done in a mobile unit with a very long, dark tube, I found out I was claustrophobic and it had to abandoned! Boy I was spooked, it didn’t make sense. So, I had to find a hospital with an ‘open MRI’, even then I had an irrational fear that I couldn’t explain but the hospital let me go to look at the scan and have a mini-try, the day before and that helped. My GP also gave me a mild sedative to take before hand, I think it was Diazepam.I made sure I kept my eyes shut throughout and they gave me a button to press if I needed attention (so I could talk to them and they could talk to me and fetch me out if I needed to) but I was ok in the end, that sort of reassured me and it was done. Years later I managed to get an upright MRI which was much better for those with claustrophobia, or for children, it wasn’t as ‘clanky’ and they even put a DVD on a big screen opposite, for you to watch during the process.
As for the POTS, I didn’t have the drop in blood pressure type but I’m certain I had the lack of blood volume and I did learn that even drinking vast amounts on it’s own is no good. You have to take extra salt in order to retain the fluid. Drinking vast amounts can also be detrimental because you pass away various essential elements in the process.
Another thing that I found helped with my POTS was, making sure my head was never held in the ‘flexion’ position, always lift whatever you are doing, so that your head can be as straight forwards as possible.
Regards
Barbara
(UK)I am so very sorry your daughter has to go through this. I am so sorry such a young person must suffer. At 32, I am starting to get extremely depressed dealing with all my symptoms, being disabled (unable to work or do much of anything fun or even care for my daughter at times), and worrying about my prognosis. I cannot imagine being so young and dealing with the same. I have only officially been diagnosed with POTS Dysautonomia since April but I have been suffering 3 years. I have honnesly had trouble finding help with my heart rate (which jumps from as low as 24 to as high as 250) and blood pressure (which tends to be very high at times and only dropped very low several times that I am aware of). At first the beta blockers helped (two kinds tried) but then the symptoms and tachycardia came anyway. Drinking lots of water sometimes helps. Oddly, wearing a soft cervical collar has helped some recently. Taking my time to get out of bed in the morning (when I wake I have to lay for about 15 to 30 minutes then slowly sit up and sit for ten minutes before standing then take it easy the first hour I am moving about). Laying on my left side sometimes helps. Sadly, the episodes of tachycardia with some bradycardia and hypertension still come and usually when I least expect it, though I know if I am sick, get less than 9 hours sleep, have my period, or am very upset/stressed that I will have trouble guaranteed. Anyway, I really hope and pray your daughter finds relief. I wish I knew some surefire ways to help her. I wish your family the best of luck and renewed health.
Ang.
Hi again Abbilou,
I just remembered something that Dr Diana found useful for her POTS symptoms and that was wearing an abdominal binder, worth a try ?
Regards
Barbara
(UK)We considered a binder. I will look into them. Thanks for reminding me.
We got a very unexpected call this morning that insurance suddenly approved her MRI/MRA and one place happened to have an opening, so she got it today. I really hate waiting for results. Pretty much all tests have found something new and crappy these last couple of years, so I get a bit nervous now.
Yeay, I’m so pleased about that. I hope they pick up what the problem is.
You’re daughter has this awful condition regardless of the diagnoses. Basically the more they find, the better because then you’ll have a fuller picture of what you are dealing with and hopefully be able to get her some more effective treatment.
It’s because things are ‘missed’ that we end up having to endure all this for so long without recognition and treatment.
Regards
Barbara
(UK)I’m sorry to hear about everything you’re dealing with. I too have EDS and POTS but also MCAS. My 4 1/2 yr old son has EDS and MCAS and our road has also been a challenging one.
Here’s a bit of our experiences:
I don’t know if you’ve ever looked into MCAS (mast cell activation syndrome) but some doctors believe POTs is actually MCAS and that if you control the MCAS you’ll also control the POTS. For me, I was able to figure out my POTS triggers and with the help of H1/ H2 blockers, Singulair, and Cromolyn my POTS symptoms are under control. They will reappear with reactions but resolve after I take the appropriate meds.
A HUGE POTS trigger for me was caffeine. Perhaps you and your daughter can take a look at her diet and eliminate caffeine, dyes, preservatives, additives and try to eat whole, organic foods. A daily food log might be helpful in figuring out triggers. Meds can also exacerbate POTS symptoms.
Hang in there, you’re doing a great job. Waiting for results is so hard.
TinaThanks for the replies 🙂 (ummm the smilie here looks like its rolling it’s eyes at people. Lol I’m not)
Her Doc and I have discussed mcas and will likely treat for it since she has had allergies most of her life and is now having skin reactions to many things. She has since birth eaten a very clean healthy organic diet which does not include caffeine etc. One of the first things we did upon the different strange symptoms coming up years ago was have her allergies tested and followed elimination diets etc. As far as treating mcas it’s just a matter of working out if current meds and the allergy meds are all safe together and we need to wait through the transition of insurance to sit down with her Doc to discuss it.
I do have a question in regards to the MRI and chari. I read yesterday it’s easily missed with MRI’s that you lay down in and that it should be a standing MRI. Any one have thoughts on if this is accurate?
I’ve heard standing up is more accurate for Chiari.
Hope the docs can figure something out to bring your daughter relief.
Thanks for the replies 🙂 (ummm the smilie here looks like its rolling it’s eyes at people. Lol I’m not)
Her Doc and I have discussed mcas and will likely treat for it since she has had allergies most of her life and is now having skin reactions to many things. She has since birth eaten a very clean healthy organic diet which does not include caffeine etc. One of the first things we did upon the different strange symptoms coming up years ago was have her allergies tested and followed elimination diets etc. As far as treating mcas it’s just a matter of working out if current meds and the allergy meds are all safe together and we need to wait through the transition of insurance to sit down with her Doc to discuss it.
I do have a question in regards to the MRI and chari. I read yesterday it’s easily missed with MRI’s that you lay down in and that it should be a standing MRI. Any one have thoughts on if this is accurate?
Hi,
I believe recently there has been a research paper in Scotland that shows that the upright MRI picks up more cerebellar tonsillar ectopia (for those that don’t understand the terminology, this is where the hindbrain falls through the opening at the bottom of the skull) in people who have had a whiplash type injury.I have to say that I personally didn’t notice a difference in mine, between the supine and upright MRIs. They both showed my hindbrain was trying to escape but in both, I didn’t quite meet the 5mm level of ectopia that they seem to deem necessary to confirm Chiari Malformation 1, so I guess I’m just a ‘Chiari 0’.
Regards
Barbara
(UK)Ask your doctor about a trial with Atarax (hydroxyzine). Research Dr. Theoharides research on mast cells at mastcellmasterdotcom. Atarax crosses the BBB so it effects mast cells in the brain. My attention, memory, word finding, anxiety, etc has improved dramatically on Atarax. It made me really sleepy at first so I started just taking it at night and then tiny pieces of a pill in the morning and slowly worked up to tolerate 10 mg doses during the day. Atarax and NasalCrom have helped me the most. Recently I’ve gone from being gluten free to also removing corn, potatoes, and rice and my resting heart rate has dropped and my BP’s have gone up a bit to be within the normal range (I usually have low bp) so tweaking diet might be worth a try too. I really like Maria Emmerich’s blog and books.
You could have her try Skins Travel and Recovery Tights… they are used by athlete’s and offer compression from foot to waist. They look sporty and last a lot longer than compression stockings! Shop around, I found them cheaper somewhere else… but you can read about them here:
http://store.skins.net/usa/womens-compression/compression-recovery/travel-recovery-compression-long-tightsMy son (14) who has POTS and I both swear by SmartWater. We like it better than Gatorade. I wrote a note to the school last year so his teachers would let him take it to class. Eating salty snacks and drinking a lot of SmartWater keeps my son’s POTS under control but he has a mild case. He also takes NasalCrom which has stopped his chronic sinusitis and he really likes Claritin reditabs which is great because I don’t have to remind him to take it. I’m hoping his doctor will have him do a trial with Atarax since it has helped me so much. Best of luck to you finding what works best for your daughter. We have to be detectives!
I spoke with the PA today and she says the scans were mostly normal but one aspect needs to be explained to her by the doc because she is a cardiac PA, so doesn’t understand it. It was something to do with signalling in the brain but I won’t know more until tomorrow.
Over the years she has had a lot of tests. At this point I can’t remember what. Her endo has always been good with checking for any possibilities that could account for some or all of the things that goes on. It really took the POTS to jump in to begin to find answers, but I know we will likely continue to find more and more. My family has always been plagued with strange and bizarre things vs the traditional common things, so nothing surprises me anymore. A lot seems to be autoimmune in the family, but so few ever find answers. They just suffer. 🙁
She does wear compression stockings but only knee high because her hands are really effected by the eds and so they just don’t work like most hands. She just got to where she can button/unbutton pants. It’s as if her brain can’t tell her hands what to do and her hands just can’t do the motions needed for some things. It was an exciting day though when at 12 she was finally able to do the buttons and did not need to worry anymore about what she wore places. Sometimes she still can’t but can most of the time. Her hands looks different than most. They’re very flexible and kind of sausage like fingers and oddly shaped. A doctor asked about her thumbs because they’re odd looking but then never said anything further. Writing is very hard for her to do more than a few mins. Its hard to explain her hands.
We have been looking for some fun compression knee highs for school, so I will check those out SweetFeather. Thanks.
In regards to the MRI/MRA results are there questions I should ask? The doc ordered if to check for chari and to be sure her blood vessels aren’t compromised and of course to be sure everything looked normal given everything that’s happened. If the one aspect leaves me with questions or concerns, I can always ask a neurologists opinion. Guess I will know more tomorrow.
As far as new meds/tests we have a to wait a little while for insurance to be all straightened out. At this point we still do not have her insurance numbers etc. hopefully they’ll be quick so we can get her as stable as possible before school begins in sept.
(sorry for typos and autocorrects I may have missed. Posting from an iPad which can be a pain)
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