Maxine

Generally, there's somehting else wrong with the heart...like a valve problem, or other heart deformity. There's usually something more to it.

I heard people with EDS shouldn't have a tilt table done, as they can code during the test. Delayed blood pooling due to the poor vascular tone. Sometimes BP drops are sudden, and the heart doesn't have time to compensate. Meaning that when the sudden drop in BP occurs, because the of the delay, it's not expected like the typical BP drops in primary POTS without EDS.

I won't even look at the article, as it's probably not accurate at all.

Maxine :0)

Thanks so much for your replies and support.

Definately dealing with a lot, and I pray the my quality of life improves when I have surgery. I don't have much of a choice, but his surgery isn't a perfect surgery, and I pray complications do not happen, or they aren't very significant.

I'm trying to work with the Cleveland Clinic on understanding the seriousness on these spine/chiari related conditions related to EDS. They're pretty tough to deal with, but I'm trying to stay positive.

I'm well informed on EDS, and have educated myslef a great deal on cervical cranial instability/cranial settling, and chiari related conditions. I've known for years about all the different forms of dysautonomia, and know that if can be a result of brain stem compression, or upper spine compression.

I started out with a local support group on dysautonomia in 2001, and have learned so much since then. When I'm feeling better, I hope to work on a book for patient advocacy. I still see patient abuse/neglect on dysautonomia, EDS, and chiari patients is rampant, and if more patients go to their appointments informed, and they learn to stand up to their physicians, and NURSES, they will have a better chance of getting the right medical treatment.

Maxine :0)

This is a note I put in the EDS neck surgery facebook page. I went to Bethesda Maryland again on March 30th to see Dr. Henderson, and also saw Dr. Clair Francomano on April 1st. I'm sorry, I don't come here much, and I feel bad for not being here to offer support. I'm mostly in the FB support pages, and rarely come here anymore. If anyone would like to contact me on why....send me and e-mail.

NOTE FORM FACEBOOK......

Well, we made it home OK. Had a really bad POTS spell on the way home----pounding heart while attempting the first rest stop....all the while the sleet was coming down in sheets in Pennsylvania. I'm doubled over in the entrance of the rest stop trying to get a grip, and finally did some breathing exercises, and mind control, and it helped. I felt the adrenaline surge, and I was shakey once I was back in the car. I took some pictures to distract myself. We went to my appointment with the neurosurgeon who said what we already knew.....I need surgery on the back of my skull, and my upper spine to repair the damage from cervical cranial instability.

My clivo axial angle is only 128 degrees, and it should be about 155-165.........this means my head is sitting forward much more then it should, and this is pulling on the brain stem. I also have pressure on the brain stem from a small sub arachnoid space (AKA----lower cranium). The problem is figuring out how far down my cervical spine (neck) to fuse, as I also have herniated discs on c-2-3, 3-4, and 4-5. He would like to fuse only down to c-2, so this is still being decided upon. I may need two separate surgeries. I also have herniated idscs on thoracic spine as well, but Dr. Henderson didn't think it was too bad. However, I feel a great deal of pain in thoracic spine.....It could be from thoracic kyphosis of upper thoracic spine, or lordosis of lower thoracic spine. I also saw Dr. Clair Francomano. She is a geneticist specializing in EDS. I also see Dr. Tinkle who is a specialist on EDS and connective tissue disorders. She was fantastic! What a kind soul this doctor is.........just a great spirit to her.

She gave a very thorough exam, and concurs with Dr. Tinkle and Dr. McDonnell on the Classical EDS with joint hypermobility and poor vascualr tone. She gave me some great recommedations for pain management without drugs.....one of them being myofascialtrigger point release which I can do at home with a workbook from author Claire Davies. Also, massage therapy, ultra sound, and possibly TENS unit. she also said I have translucent skin with easy bruising, and atrophic scaring. There were some other skin findings as well, and skin is mildly stretchey. She will be giving my MRA films that show a very poorly functioning vertebral artery with little or no blood flow to a vascular surgeon. She would like me to get the colonoscopy done, as I should have a while ago. I didn't trust anyone here in town to do this becuase I have been unstable for so long......sedaton would be tricky. She is arranging to have it done there. The process would be done by a GI doc familiar with EDS and my spine instability.

This has been a very difficult time for me, and I try to stay focused on getting well, but it's overwhelming with multiple things going on at once. The recent heart arrhythmias are scary, and although I have gone to the ER twice, and I've been checked out.........basic EKG, limited cardiac echo, and monitoring, they scare me very much, and If we could just erase these out of my life, it would make my life feel less limited. Usually after I have these spells it leaves me in a deep depression, because we can't catch them to find out what they are, or what's causing them. I wore a 30 day event monitor, and of course I didn't have anything significant on it, and the 3 years I wore an insertable loop recorder, I did not have an episode of any significance except one time, and the thing malfunctioned. Dr. Henderson said these recent heart palpitations, and arrhythmias could be caused from the brain stem compression. I feel like I'm smelling smoke or a dirty ash tray off and on every day. This makes it hard to breathe at times, and my weakness has increased significantly------to the point of making it difficult to walk more then 5 minutes, or to navigate any store.

ADDED to this note.....Dr. Henderson said I also have damage to the dorsal motor nucleus of the vagus and nucleus solitaris....this can cause heart arrythmias. This can also cause the gastrointestinal dysfunction. I have a narrowed subarachnoid space, along with damage to the tectorial membrane which is a significant ligament to the stability to the cervical cranial junction.

I appreciate any prayers.....especially for these heart issues to go away.... I'm not looking forward to surgery, as I don't know how stable I really am. I feel very nervous going under anesthesia feeling as poorly I as do.

Thanks for taking the time to read this!

Maxine :0)

By the way, "the inner ear" is usually what a "clueless" doctor will diagnose when they don't know what the heck they're doing. They cannot make that diagnosis unless an ENT (ear nose throat)doctor evaluates them. Or the doctor has ruled out other causes. When they do no testing,neuro exam, and slap a possible inner ear problem on you...it's a big red flag that you're being blown off.

AGAIN....SO SORRY YOU HAD TO DEAL WITH THAT KIND OF TREATMENT.

Julie :0)

Yes subluxating the vertebrae can cause vertebral artery compression, and cause the symptoms you daughter is having.

You daughter received, "below the standard fo care" at the hospital you went to. In light of her also having EDS, she should have definately been evaluated further by a neurologist or neurosurgeon, and they should have done and MRA, MRI to see if her vertebral artery is compressed. If she has a history of neck instability you need to push the issue more with her doctors, and make them do their job. You and your daughter have the right to have decent medical care! I'm sorry you both were dismissed like this by whoever treated you at the hospital!

When I try to advocate for myself, I get treated very poorly, but there's occasions it actually works.

Advocate for your daughter and yourself, and don't be shy to ask someone to help advocate if you aren't comfortable with it yet.

Take Care,

Maxine :0)

It has been explained to me that people with EDS who have poor vascular tone have blood pooling, and in turn this causes orthostatic intolerance. You do not have to have "vascular EDS" to have poor vascular tone.

I have severe blood pooling, and delayed drops in BP. When my BP drops it's very low! Gravity is not my friend!

Maxine :0)

Thank you----

Well, it would be nice if the POTS was helped, but I have to remember POTs is part of the EDS also. I think the surgery is to stop the progression, and to save my life actually. This is not a great thing to have, and I know you know this, but I often find myself being judged because I look so normal.

I'll tell you somehting that isn't helping........................menopause!

The hot flashes make me feel like I'm melting, and I can't do anything!!!!

Thanks for replying, that means a lot!

HUGS,

Maxine :0)

Lieze,

Although I haven't been on here for a while, I wanted to skim a few postings, and yours caught my eye.

I know this horrible feeling of anxiety, and feel my body react to everything----even a loud TV--(which isn't really loud, but seems like it to me).

This can make everything feel worse. The doctor owes it to you to help find out why you're so anxious. This is a symptom of an overactive sympathetic nervous system. A malfunction of your ANS. (autonomic nervous system). All kinds of things can cause this--------------even Celiac disease.

http://www.celiac.com/articles/1106/1/Celiac-Disease-Symptoms/Page1.html

Advocate for yourself, or find someone who can--------- you deserve better answers----------DEMAND THEM!

The anxiety is the worst, and I have sincere compassion for anyone going through this. Anyone that doesn't, deserves to deal with this themselves to teach them a lesson in empathy.

Sending you a BIG HUG!

Maxine :0)

First, I'm so sorry I haven't posted here in a while. I've been more cought up on facebook with EDS group, and EDS neck surgery group, and also supporting a good friend with her severe spine instability. I went to the EDNF conference in Baltimore in Baltimore Md., and also saw a surgeon at before the conference in Bethesda, Md. It was a great time putting the faces with the names, and finding out how much we have in common. Some people with EDS didn't have any POTS symptoms. I found this interesting, if not a bit confusing.

Anyway, the setback I'm having are both POTS and spine related. Both equally disabling which have left me mostly home bound. I'm not able to walk far, but do have those days that are few and far between that I can walk around target, or get out and visit friends or family. My husband has been supportive, and has driven me anywhere I want to go to get out of the house. The weather is horrible here, and the highs at night are in the single digits, and maybe 25 degrees for the high. (I HATE WINTER!!!!)

When I saw the surgeon in Bethesda, he confirmed the instability, and SO did the MRI! The MRI was a sitting MRI that was long over due.. The dynamics in entirely different when I'm sitting or standing. My spine is very different. In fact most MRIs should be done at least sitting. It's insane that this hasn't been done on me years ago. MY Clivo axial angle is off...it's only 128 degrees-------(this is bad). In fact this is so bad that surgeons don't feel that surgery will improve symptoms on patients who have 121 degree angle---(probably because damage is too long standing). This is what a bunch of us 50 year olds are dealing with!!!! We go too long undiagnosed. As a result of this my odontoid bone has pannus growth, (of course you have all heard I had this). I also have the cervical cranial instability, and now have thickening of the tectorial membrane near this area. In addition the MRI reports herniated/bulging disks on c2-3, c3-4, c5-6, (c5, 6, and 7 are fused and plated), c7-t1, t1-2, and t2-3. I have a blown disk on t-8, and bulging disks on L3-4, L4-5, and L5-S1.

On this same MRI report, it also states that I have narrowing of the subarachnoid space! I'm thinking, how did this go so long like this! I've had surgeons blow me off, who's name is on the same article written by the surgeon I saw in Bethesda about the exact thing I have diagnosed. Check out the article!

http://www.surgicalneurologyint.com/article.asp?issn=2152-7806;year=2010;volume=1;issue=1;spage=30;epage=30;aulast=Henderson

I feel terribly cheated fro the medical professionals that blew me off all these years. My EDS geneticist had referred me previously to a orthopedic surgeon who had also confirmed the instability. However, my PCP wanted a neurosurgeon to confirm it also, and I found a great one. Now he's recommending surgery, but I have no idea how where he will even begin with this mess. I have another appointment to see him (3rd. appt., as I saw him again in October.) We also had a discussion about the vertebral artery missing on the left. We had further MRAs and MRIs done to look at this, but this time the MRA was done with fat supression. This did show a view of the artery, but it look s black, and dead. It's not a working artery, and this is why most MRA views don't show it at all, whihc makes it unclear on what caused this, or if it's a congenital malformation. My EDS geneticist thought it wise to have a vascular surgeon on board during surgery. Basically, we won't have a real clear idea how bad it is until they go in there.

Meanwhile, worsening POTS, and horrible pain in cervical spine, and THORACIC. The reason I underline "thoracic" is because I feels that pain is worse on some days. It truely knocks the wind out of me. I feel like I'm breathing saw dust on those days. The last two days I've had an ICE PICK headache, and it feel like it's sticking in my left ear, and radiating into my lower left cranium, and noe today it's shifted to the right along with intense pain in upper back. I'm feeling so bad today it's making me dizzy!

I wanted to update you all, and I hpe you understand that my ability to multi task in more then one forum is not great. I still think about many of you and pray for the day this nightmare will end for you, and you can live a fuller life.

Thanks for reading,

Maxine :0)

Sorry, haven't been on here in a while-----------I have a lot going on with POTS, EDS, and spine issues/instability, and I have already made an attempt to type it all out, and it ended up being deleted by accident when I had too many pages going at once.

I had a question about omega 3s and vitamin D. I know I have posted about vitamin D before, but I'm doing it again.

Do any of you notice more POTS symptoms/irregular heart beats when taking omega 3 or vitamin D? IT seems even the 85mg aspirin seems to cause skipped heart beats.

When I went to my PCP he did some blood work on lipids along with other blood work. The tryclicerides came out hihg at 350. I don't know how, as my diet is the same as it was when they actually went down to normal on the last blood test, so I don't understand at all. My weight even went down. Anyway, for some reason, I seem to have problems with irregular heart beats with vitamine D, omega 3s, and baby aspirin. Could it be part of the blood thinning combine with the poor vascular tone causing more blood pooling-----possibly at a faster rate.

Any insight to this weird problems would be much appreciated!

Thanks,

Maxine :0)

I hope they get down to the bottom of your lab work soon. You have a right to know what's going on.

Yes, hypermobility/EDS is associated with GI problems. 3/4 of my colon is diverticuli from weak bowel walls due to the EDS. The CLeveland Clinic is clueless about EDS/connective tissue disorders. Check this out; http://www.clevelandclinicmeded.com/live/courses/2010/marfan10/default.asp

Pretty ironic, isn't it.

Sending you a HUG,

Maxine :0)

Mary P,

I'm so sorry you have been dealing with all of this. I truly know your pain, and the fear of the unknown.

The Cleveland Clinc has agreed to do the colonoscopy without sedation. It will take longer, as the procedure will need a "special touch". Dr. Burke was wonderful with me on the phone, and she said she had all of my records in front of her. She was familiar with all the autonomic nervous system issues, and in fact knows we also have a different response to pain because of this.

I'm going to be examined before this is done because I called about a lumpy area on the left lower abdomen. It comes and goes, so hopefully it's the IBS, diverticuli, of the constipation issues..... pray, pray, pray.

It's really pretty awful that these "other issues" we have are so much more complicated due to the ANS issues. My brothers have already had their colonoscopies done, and they went out to lunch right after the procedure.

Now I just have to pray I make it through the prep ok. She like to use the golytely............UGGGGGGGGG!

We'll hold eachother together Mary....

HUGS,

Maxine :0)

Hi......

Tomorrow I start on regular dose wellbutrin 100mgs split in half twice a day.

I have been on 100mg SR for 9 years........any experience going from extended release to regular dose twice a day?

I have trouble digesting the casing on the extended release, as it remains intact. I'm certain it gets caught in my diverticuli pouches. I have excessive diverticuli. I'm hoping this helps my digestive problems somewhat.

THANKS!

Maxine :0)

Thanks , I may get an ultrasound before the test. Reason? I feel a tube shaped hard lump on lower left abdomen. SCARY! My brother died from colon cancer in Feb. 2009. We don't know what kind of cancer my mother had because it was found so late. I think most people know my family history here. Her brother died two weeks after she did, and we know he died from colon cancer. No other members in the family had colon cancer. My Aunt had brain cancer in her 60s. She lived 10 years after the surgery removing most of the tumor.

This lump almost feel like a twisted bowel. I'm always consitpated, and I feel like I'm full sometimes just after a few bites of food. Lower GI bzrium/double contrast enema showed multiple diverticuli, but they weren't sure if I had small polypoid lesions. I can't imagine any of those grwoing into a tumor in less then 3 years. Praying!

I had an upper GI after this to take another look at the diverticuli in duodeum. It's 5cm, and it's been there since my 20s.

Maxine :0)

Well, I've been having gut issues for a very long time. Too be more specific, CONSTIPATION"! Sorry folks, just plain tired of dealing with the misery of it. Many people with POTS, EDS, or both suffer with this relentlessly.

I'm sure the spine instability that has caused neuro problems has a role in this also. One thing I have thought about is diet. I'm careful to stay away from foods that mess with my diverticuli since I have 3/4 of colon covered with it, along with a very large one in my small intestine.

When I went for my regular checkup with Dr. Grubb I mentioned changing my Wellbutrin from 100mg SR to taking two doses of 50mg of the wellbutrin twice daily. REASON? One thing that I can't eat is corn because the way it digests. Wellbutrin is much the same. You really don't want details on this, but you all know about corn. I had two major flairs with the diverticuli in the small intestine, and a smaller one with the large intestine. The first flair caused a fever for two days after eating two ears of corn. My thought was that the wellbutrin accumulates in the diverticuli pockets slowing digestion further. The SR wellbutrin has a casing around it that doesn't digest. The medicine is slowly released through this casing. h

I can't eat oatmeal either, as it gets caught in those diverticuli pouches also.

Colonoscopy has been put off more times then it should have been. Once it was because I was sick, and another was because it was scheduled the same week my mother died. Then my gastro doc and pcp decided to do a barium enema/double contrast GI. This was over two and a half years ago. Since then a couple other colonoscopies have been cancelled bacause of unstable POTS, and the pcp said I can't handle sedation or the prep. Well I finally have a gastro doc from the Cleveland Clinic who is willing to do it without sedation. She said only a couple docs at the cleveland clinic will do it this way, and she said it takes a special touch. She is one of them that does it, and she sets an hour appt. for it so she can go slow with the scope. She had all my medical history in fornt of her as I have been there before, but they were going to use sedation. I saw her first, but ended up seeing another GI doc who wanted to used propofol. Unfortunately I have been dealing with further progressive spine issues, and the pannus growth on the odontoid bone near the brain stem has caused further narrowing. I've had progressive breathing issues, and see the neurosurgeon on Oct. 6th to hear what he has to say about the latest test results.

The EDS has caused this whole mess, and I would have never imagined this 8 years ago.

I hope changing the wellbutrin around this way at least helps with the gut issues.

Maxine :0)

Hi Mary P,

Can they do a lower GI Barium Enema? It's not nearly as invasive. I had this done 2 1/2 years ago. They're not sure if I had polyps, but I had excessive diverticuli in 3/4 of my colon, and this is way too much for anyone. This is probably connected to my EDS, and so it the large diverticuli in small intestine that is 5cm. Because of family history of colon cancer, I need to get a colonoscopy done, or at the very least lower GIs every two years. I went to the Cleveland Clinc, and they were pretty good at taking my concerns with aesthesia seriously-------------in the beginning.

They even wanted to include my family in a research study. However, after talking with the doctor who was going to do the actual procedure I started to get doubts. Actually it started before that when I thought I was going to be talking to an anesthesiologist, but got a "secretary" who took information about my dautonomia, and my upper spine and brain stem compression. They seemed dismissive, like I was being overly dramatic. I was told propofol was going to be used, and that I would be completely out. I think they thought I would be a train wreck and a difficult patient, so just put me out so they don't have to deal with it. WRONG.....I'd rather deal with the pain, and know I'm going to wake up.

I still scheduled my colonoscopy, and was going to go, but when I went to my PCP a couple days before it was scheduled he told me I wasn't stable enough for the colonoscopy, and that I needed to follow up with my POTs doctor. This is when my catecholamines came out really low. My BPs have been really low, and heart rate not compensating. I also have sleep apnea. The sleep apnea is related to my brain stem compression. I have trouble breathing sometimes while sitting up. I don't think the GI doc took it seriously. These doctors have to listen to us, or we're not going to get the "other" medical care we need. We're going to be non compliant out of fear, and we have every reason. I've seen "mistakes" happen. MY PCP has told me about "mistakes". That's why he's telling me to cancel, and has more then once.

I'm still on the phone with the Cleveland CLinic asking them to find a way to get me through this without any problems. My condition has taken some bad turns this summer, and I need them to listen now more then ever.

You have to stand your ground and do whatever is necessary to make sure your procedure is done safely.

I understand your fears------I have them myself. Don't let anyone be dismissive with you.

Maxine :0)

My thoughts and prayers are with you. Hopefully you can find more support through a church, or even a support group for people going through the same thing. I don't think you were treated fairly.........

Sending you a BIG HUG..... Things will get better, just hang in there----they will.....

Maxine :0)

I found this link while looking up oriental salad, and noticed that raman noodles were used in the recipe, and the seasoning packet is used also. I know for sure the seasoning packet used has MSG. My son loves raman noodles, and on occasion I would ask for just a small bite because it is tasty. However, I would never consider eating a normal serving size, as it would cause my POTS to go into a tail spin. I found that MSG is in so many things, and is also used under other names such as "natural flavors", "soy protein" ect....

I'm sure it can't be good for POTS!

http://www.msgtruth.org/body.htm

Maxine :0)

I sure would like to know why we get hese extreme tachy panic feelings on the vitamin D.......What the heck! Why would this happen, I don't understand, if we need it, then why are we having and adverse reaction?

This is so absurd. Lissy, this is exactly what happened to me when I took the liquid form of vitamin D.

Maxine :0)

Lidocaine without epi is even bad for me. Lidocaine is toxic to me, and my body gets really messed up from it------tachy, tremors, gagging, shaky, dry mouth, and feel like I'm losing my mind. Carbocaine was OK, it seemed to not have nay affect on me when I had the tooth pulled. However, when I had a lot of it to remove the cardiac loop recorder, I had a huge increase in blood pressure. I took a half of a klonopin and it faded over a fairly short period of time.

Why do we have to be so complicated?

A normal day would be nice----just to feel normal, take a long walk-----and not have to worry about something happening.

Maxine :0)

Thanks for the replies...........you all have been very helpful.

I think we probably have the strongest minds, as we have had to use them for so long to control our bodies when the adrenaline starts up. It's not always possible, but I think some of the different relaxation exercises can help------------------but usually more useful in trying to avoid a bad pots spell. Once I get an attack, it usually runs it's course no matter what.

I know there's times we feel we're going to go completely nuts, but I know many of the rude and insensitive docs many of us have seen wouldn't be able to handle one of the sympathetic overdrive spells we get-----------not as well as we handle it.

Wishing you all the best.

Maxine :0)

LOL Pat57

I think eveyone has their own pace, and what is limiting to you may not be for her. POTS is very different in the type of weakness and fatigue that presents. When we don't have any blood where it belongs----near our heart and brain becuase it's pooling in our arms and legs, it's like walking around with a no blood for your heart to pump. It's just not possible. We simply can't push the way some people do.

My brother in law had chemo one day for his non hodgekins lymphoma, and I took him to his treatment. He had to vist a social worker after, and we had to walk through a long walkway between buildings in 95 degree heat. One I walked about 10 fett, I told him to go ahead, I can't move well in the heat, and can only last about 5 minutes before I feel like collapsing.

He has just had chemo, and was walking fast. He seemed fine. I'm sure he wasn't great, but I felt like a pile of broken glass. All my battery juice left me. I was in so much pain I felt like it was an effort to breathe.

My brother Joe was in his last two months of his life with terminal colan cancer that spread to his liver, and he was laying out on his driveway under a car he was fixing for his son who would soon be driving. I'm looking at it as I type this out my front window while he mows our grass. A 1992 honda accord. My brother got the news brake on, and collapsed soon after and had to go into the hospital. He would have his chemo treatments during the three years since he had the surgery, and amazed me on how active he remained, while I would lay aroud puzzled at his ability to stay active. Since I had my last flexation extension MRI and CT scan my symptoms have really gotten bad, and I'm more limited then I was. I have not used a wheel chair yet, but I doubt I could go to the zoo, or a festival without some kind of assistance now.

Some people run themselves into the ground, and some choose to take things slower so they can take in the joy of living life with some QUALITY.

God Bless your friend for her strength, It's not often people are able to carry on like she does with a severe illness like she has.

Blessings to both of you.

Maxine :0)

Sometimes it's OK to let yourself have TIME to just lay around and sort your thoughts, allow your body to rest---------------AND ENJOY THE CHOCOLATE. Let yourself be in the moment. There's nothing wrong with that.

Listen to your body------------------don't let anyone assume you're lazy. Tell them to go to their computer and look up POTS and READ. IF they don't, ask them why they are so lazy------all they have to do is SIT AND READ, and they can't manage that?

How messed up is that? I look forward to the day that I can slug down a couple of margaritas and dance on a table top and get ATTENTION that way!

I can't stand people who judge us thinking we are attention seekers. I know ALL of us can find other ways to get attention than living this way.

HUGS to you-------enjoy some more chocolate...............DOVE chocolate is great stuff.....

Maxine :0)

Sorry you had to deal such ignorance.

POTTS -----Tuberculous Spondylitis

http://emedicine.medscape.com/article/226141-overview

I still can't figure out how docs can mix that up...... come on, learn the difference...

Maxine :0)