Do You Have A Connective Tissue Disorder & Hypovolemia?

[juliegee]

I'd love to hear from those of you with connective tissue disorders, like EDS, if you are also hypovolemic. My son has been DXed with both conditions. Is it unusual?

In another post, I read that it would be life threatening for a patient with poor venous return to also be hypovolemic....yet that is exactly what Mack's doctors have told us about him. Indeed, he was unable to function until he started high doses of florinef, salt, etc. But life threatening???

Thanks in advance for sharing.

Julie

[firewatcher]

I have been told I have both, but not officially diagnosed. My labs are highly indicative of hypovolemia and I have been dxed with partial diabetes insipidus, but that is constantly questioned. At first, my evaluation said no to hypermobility, but my physical therapist has since reversed that saying that she's never had a patient with my range of motion or fragility of joints/tendinosis issues.

[issie]

I have been told I have both, but not officially diagnosed. My labs are highly indicative of hypovolemia and I have been dxed with partial diabetes insipidus, but that is constantly questioned. At first, my evaluation said no to hypermobility, but my physical therapist has since reversed that saying that she's never had a patient with my range of motion or fragility of joints/tendinosis issues.

I was told from Mayo that the hypermobile type of EDS could not be tested with genetics. This is the only type they diagnosis on symptoms and visual.

[MomtoGiuliana]

Hmm, don't many POTS patients have poor venous return for a variety of reasons, including EDS? I would think a lot of us have both poor venous return and hypovolemia. Not sure why that combination would be life-threatening? Certainly it can be life-altering, as we well know!

[MightyMouse]

Issie is correct, there is no genetic test for hypermobile type EDS; it's diagnosed by physical exam and use of the Beighton Scale which gives points for certain joints being more mobile than normal, and if you have enough points, plus other symptoms related to EDS (POTS included), you'll probably end up with the diagnosis.

And yes, I have hypovolemia and a connective tissue disorder.

[Rachel]

I've been diagnosed with hypermobility/EDS III. It was actually found and diagnosed a few years before I first noticed POTS. My cardiologist suspects that I have hypovolemia also, but I have never been tested for it. I've never been told that the combination of the two was life threatening.

Rachel

[Maxine]

I have the EDS, but never been checked for hypovolemia. Overall, ANS testing has been scant.

But I know it should be check, but too overwhelmed with skeletal problems, and other CNS issues that I haven't broached the subject with my ANS doc. I don't even want to set up my next appt.

My catecholamines came out so low, but endo doc said everyone is like that at some point during the day.....hmmm. I don't know about that. I feel like I need to be scraped off the side walk after two minutes in the summer heat----much worse with the sun. My hands swell immediately, and go back to normal as soon as I hit the air conditioning.

I feel like I'm walking around in shock a lot, and it doesn't have to be hot out. I just run an errand and I'm wiped out.

I'm going to the EDNF conference in mid July, and it will be interesting to get some insight from all the different doctors who will be speaking------some will even have a short 10 minute consult with us. That's more then some of us get when we pay $$$$$ for an appt.

Maxine :0)

[sugartwin]

The only reason I speculated that such a combination might be life-threatening is that I have both EDS and small fiber neuropathy and the combination of the two has threatened my life a couple times: once while I was in the process of being admitted to the hospital and again on the operating table. I was always told multiple times that POTS itself wasn't life threatening, so I wondered if having two factors contributing to my dysautonomia might be why the things that happen to me happened. Or then again, I wonder if might have even been some other factor.

Long story short, I had two near-death situations that no one was ever able to explain to me and it bothers me a lot. It seemed to be that someone who had both a substantially low blood volume and an additional problem with vasoconstriction would be at risk of circulatory collapse, which is what happened to me twice.

[Maxine]

I can understand completely Sugartwin. Especially about have a life threatening experience, and no explanation behind it.

I'll never forget the time I was in the mall, and I thought I was doing OK. In fact I put a little skip in my walk since I had been feeling better then I had in months.

The next thing I know it felt like my heart stopped, I had grey tunnel vision, and then it felt like my heart Rebooted itself and started to beat really fast. I had other occasions this happened, but never that bad. It was pretty close-----close enough to discuss the implantable loop recorder with Dr. Grubb. Of course after he implanted it, this didn't happen once. Each time it did happen was when I was walking.

It's very disturbing to have something take you down like that, and never know what really happened. It's always on the back of my mind. I don't know if it was actually life threatening for me, but it sure seemed like it. The other times it happend I would squat down, and it went away faster.

I'm told tilt table testing should not be done on patients with EDS.

Maxine :0)

[Chaos]

Just back from cc where they confirmed hypovolemia. I am very hypermobile and see the geneticist Tue about EDS testing.

Sugartwin-interesting about your circulatory collapse. On the face of it your reasoning seems to make sense. Would severe hypovolemia by itself not be a potential factor for causing it?

[sugartwin]

Chaos, I think it would. In fact, I think that any of the contributors to POTS, if severe enough would lead to circulatory collapse and ultimately death. I have a friend with autoimmune autonomic neuropathy who sadly, is losing her life because of the extent of the destruction of her autonomic nervous system. I've also read an account of a man with severe hypololemia secondary to CFS/ME who went into circulatory collapse.

[issie]

I wasn't sure what the term circulatory collapse was, so I Googled it. It is surprising that so many things can cause it.

One article I read said that too much magnesium by a man in the hospital who was given this as a laxative, had it due to the

magnesium. I know magnesium can cause orthostatic issues, it will lower your BP.

Personally, I try not to think about the fact that this could be a death sentence, but look at the positives that I can find with each day.

If you dwell on the negative possibilities, you won't enjoy the positive probabilities and the present. Sure, it's in our face every day and we have to

struggle day to day or minute to minute. But, I think it's better to let the glass be half full instead of half empty. A positive attitude

even if it is only for one hour a day is better than gloom and despair ALL the time. (Just thinking out loud, something I have to remind

myself of. Something to TRY to put into practice.)

[Maxine]

I believe in a positive attitude also...........................

Sometimes we have to state the HARD CORE facts to these doctors so they understand the seriousness of this, which means we have to discuss the possible demise of people with ANS dysfunction associated with vascular complications, EDS, and autnomic neuropathy if they aren't taken seriously. This can appear as someone not having a positive outlook, but if docs would just listen then we can have hope. This hope can be a good jump start to a positive attitude.

It's very tragic that some people die because doctors fail to listen to their patients. There's some things that are so obvious, but patients still go untreated for serious medical conditions. Also, important details of their MEDICAL condition are often NOT documented in thier records. Half the time doctors don't even read all of the medical records. I know this because doctors at the Cleveland Clinic didin't read my records, and NOW I find that this whole time that the missing vertebral artery in my brain could have something to do with my cervical cranial instability. I suspected it could, and I asked them about it, but they completely dismissed it. It's documented from NIH----how can they ignore records from NIH?!

Now I find that since an ultrsound of my carotid, vertebral, and subclavian arteries that there's blood flow in the left vertebral artery, but apparently it's stops at the cervical/cranial junction. WHAT DOES THIS MEAN? It's pretty obvious even to a layperson that if blood flow shows in the cervical region, but MRAs of the brain show the left vertebral artery missing completely, and the person has a history of almost 8.0mm of cervical cranial instability at the c1-c2 joint WHICH THE VERTEBRAL ARTERY WEAVES IN AND OUT OF, then it's obvious the blood flow must be cut off somewhere in this region. YA THINK?

This is veering a little off the main subject, but the point is that there's dangerous things associated with EDS and POTS-----one of them being skeletal problems affecting soft tissue and the vascular anatomy. The EDS also causes poor vascular tone which causes severe blood pooling.

I find it maddening that one of the doctors I saw at the Cleveland Clinic has ordered a CT of my neck, and one of the diagnosis codes is a vertebral artery dissection. We put the CT on hold becuase I've had so many CTs in the last months. We were going to try another MRA, but this time with different views.

http://emedicine.medscape.com/article/252062-diagnosis

There's also a type of MRI---CISS MRI;

http://journals.lww.com/spinejournal/Abstr...ertebral.7.aspx

Why do we have to research this stuff ourselves------------

Shouldn't the doctors we see know this? Do I have to wait until the overloaded right vertebral artery blows up? I have been told the right vertebral artery has been enlarged since 2002----before I had my cervical spine surgery. The docs have blown that off too.

Now I will be ssing another surgeon in Maryland who specializes in cervical cranial junction instability. I'll see him the day before the EDNF conference in Baltimore, as he will be speaking at the conference also. We wanted to schedule this near the conference to avoid driving there twice.

Maxine :0)

[issie]

Maxine,

You have got allot going on. It can be soooo overwhelming and consuming. The answer to your question as to why we have to figure it out and our doctors don't; is, the doctors are trying to see so many patients that they are required to see or lose their jobs. We are so complex with our issues and they just don't have the time to research it. Not only is EDS rare but so is POTS. There are so many variables that go along with both of these problems. Unless a doctor has the ability to do research or is in a learning center, or research center - the regular issues are all they have the time for. It is very disheartening when they don't listen to us and when they don't read our charts - when it's right in front of them. But, even though we are asking them to be a part of our world - they may not choose to be a part of it. At some point, we just have to realize that we may not find the magic pill or the cure for this. In that respect, not that I've quit looking, but I'm trying to not dwell on the things I can't change and try to enjoy the things I can enjoy. However, small that may be. I'm finding that I'm not so dreary all the time, by attempting to do this. I don't want ALL my conversations to be about well ---POTS. Although it's hard to ignore it when it stares you in the face -- all the time-- like some geeky friend you can't seem to get rid of.

Someone saying, well I have that too, doesn't help it -- in your world. No one really understands what you feel like or how this makes you feel. But , the truth of the matter is not too many people really can come into our world and truley understand. We allow other POTS people to know what is going on with us, because we all understand. But when someone is a realitively healthy person ==THERE IS NO WAY, they can understand or really care enough to ponder over all the information we gather to try and help ourselves. I don't mean for this to sound insensitive, but when I came to realize this, it was like oh, okay I understand. Then I didn't get so mad when I'm mis-diagnosised or they overlook the obvious. I'm not having to deal with anger. The anger can make you very sick too. Especially if you have the hyperadrenal type of POTS. (All of this is from a learning experience I've gone through. I have all these things I'm speaking of. It is a day to day thing that is a work in progress.) Take a deep breath and realize tomorrow is another day. There will be a doctor that can and will come into your world to help you. You just have to find him. It's a journey, just one we wish we weren't on.

[Maxine]

Issie, you sound like a pleasure to be around. I separate myself from the negative things, and live another life with wonderful grandchildren, family, friends ect. They have no idea of the things I deal with in my other life with EDS, POTs, spine instability, and vascular complications.

There's good docs out there. They need to let us know they don't have the time, but would love to help us with what they DO know. They can tell me their limits anytime. I understand a lot of it----especially when I know a doctor personally, and I see the other side. There is no excuse for a doctor to give "below the standard of care" which is what many of us get.

I've heard about people getting thrown out of ER rooms------ literally. "Get out, and wait for your cab lady!" This is what the ER personnel did. She cried when she called me 9 years ago looking for someone to believe her. Thankfully, she is doing better now. What happens to people like this who live alone, and have no one to advocate for them?? We had a local support group, and had people like this coming to our meetings. I've heard too many tragic stories of abuse and neglect from the medical community, and I know some docs mean well. I can tell the difference.

I had an older doc that simply didn't agree I had a problem with cervical cranial instability, and I know I do! However, this doctor was respectful, and he had a healthy debate with me. He never suggested I needed psychological help, he just didn't have the experience with EDS, and the secondary problems that can heppen with it. He has since passed away, but to this day I still feel respect for this doctor because he had respect for me, and he had the decency to to admit he didn't know enough about the EDS and the relation to spine instability. At this time the EDS was diagnosed by a spine surgeon I saw in 2004, but the EDS diagnosis wasn't official until 2006 when I saw a geneticist. It's was still new to me, but I knew enough to know it was affecting my spine.

I have to respectfully say the doctors my mother saw gravely neglected her when they missed her cancer over and over again despite many CT and MRI scans from 2003 until 2007. Something is horribly wrong when so many doctors are in the picture and all of them miss it----and we find out 48 hours before she dies that she has terminal cancer. They put her in physical therapy the last week of her LIFE! Now I witness my mother in law who fractured her transverse sacram going through unthinkable neglect. You don't want to know what she went though-----but I can tell you I sat at the hospital every day babysitting the DOCTORS and NURSES. I have to say most of the nurses were great. The main OBTUSE nimrod was the surgeon.........let's just say it was a circus. I can't really take it further then this.... Just trust me when I say the medical profession needs some changes......

It's not just us they don't treat well, it's a lot of people. When my Aunt in Calf. had a brain tumor, they kept telling her she was anxious. She finally collapsed on her living room floor and the surgeon told her if it would have been 24 hours later she would be dead. This sounds dramatic, and I wish these things didn't really happen, but this is only part of what I have witnessed.

VASCULAR COLLAPSE is something that is serious, and needs to be looked at much more seriously then it is. EDS causes life threatening problems.

Check out this Pub med abstract;

Disabil Rehabil. 2010;32(1):1-7.

Dignity not fully upheld when seeking health care: experiences expressed by individuals suffering from Ehlers-Danlos syndrome.

Berglund B, Anne-Cathrine M, Randers I.

Nursing Division, Department of Medical and Surgical Gastroenterology, Karolinska University Hospital, SE-141 86 Stockholm, Sweden. britta.berglund@karolinska.se

Abstract

AIM: The principle of human dignity has assumed importance in ethics and constitutional law throughout the 20th century in the Western world. It calls for respect of each individual as unique, and of treating him or her as a subject, never as a mere object. As such, the principle constitutes an ethical cornerstone in health care. Patients suffering from Ehlers-Danlos syndrome (EDS) challenge medical care and knowledge in health-care professionals' as symptoms sometimes are vague. Individuals with this disorder have reported not being respected when seeking health care. PURPOSE: To describe encounters in health-care situations when individuals suffering from EDS experienced that their dignity was not fully upheld. A further aim was to describe the long-term consequences of these experiences. METHOD: A study-specific questionnaire was designed, where individuals with EDS described their encounters with health care from a personal perspective. RESULTS: After qualitative content analysis, the following five categories were identified: 'Being ignored and belittled by health-care professionals,' 'Being assigned psychological and/or psychiatric explanations', 'Being treated and considered merely as an object', 'Being trespassed in one's personal sphere' and 'Being suspected of family violence'. Consequences of these encounters were 'Mistrusting the physician' and 'Risking bad health'. CONCLUSIONS: The memory of not being respected is substantial for individuals with EDS and can last for years. As a result, lack of trust for the health-care system is created and they may experience difficulties in future encounters with health care. Therefore, health-care professionals should base their actions on norms that protect human dignity and confirm each patient as a unique human being with resources and abilities to master their own life.

PMID: 19925271 [PubMed - indexed for MEDLINE]

I have a positive attitude, but I'm not going to stand by and let doctors continue to neglect me, or anyone else I know. Believe me, like I said---I know a doctor, and the experiences I have shared with he and his wife have left them speechless.

Keep your head held high------but wear a helmet!

Maxine :0)

[issie]

Ha! Ha! Maxine. Order me mine -- the helmet. Been there, done that!!!!!! Keep on Keepin' ON!!!!!!!!!

[sugartwin]

issie, you're a better person than I am. I don't mind if doctors don't understand my conditions, as long as they admit this upfront. More often, I run into doctors who don't know anything about EDS, know they don't know anything about EDS and then try to act as if they do to save face. They invariably get the facts wrong, then get insulted when I correct them. I'm usually told to "get a job" before the end of the appointment, and then the bill comes in the mail

Overwork and even ignorance are understandable in a doctor. It's the outright cruelty I can't stand. My doctors have insulted me to my face, called me names and accused me of faking. My grandmother had EDS as well, and when doctors couldn't find the reason for her symptoms, they committed her to a mental institution. Fifty years later, in the same circumstances, my doctors threatened me with the same thing. Well, actually they gave me a choice. Leave the hospital of my own volition, or be committed. Normally people being discharged are given a cab voucher, but since I was a suspected malingerer, I got to walk the mile and half home.

I spent the next two weeks bedbound. My father had to travel 300 miles from Cleveland and do everything for me from cooking my meals to combing my hair. That trip to the hospital cost $31,000, and that was the pathetic outcome.

I don't forgive as easily as some do. I am angry. I am angry every single day. I don't mind being angry though, it keeps my blood pressure up.

[sue1234]

Sugartwin--that's awful! Besides the rudeness, sending you to walk all the way home with an orthostatic problem was just wrong! I am so sorry you had to go through that.

[Maxine]

(((((HUGS)))))) sugartwin.

That happened to my friend from NY who comes to Toledo to See Dr. Grubb. She went to an ER in a smaller town in NY, and she was having atrial fibrillation. They told her they didn't want to see her in the ER again, and they discharged her without the option of being commited for mental illness. She would have let them commit her because she was afraid for her life-----it would have been better then nothing. They DID call a cab for her. She was so sick that her weight went down to 95 pounds at 5ft. 7in. She ended up letting them commit her to the psyc floor at one point because she was afraid she was going to die. She was an engineer with a great career, and she was reduced to this!

Sometimes I think the word "angry" is an understatement. There's times I feel absolute rage.

How do we get this kind of abuse to stop? How do they get away with it? It still continues!

Maxine :0)

[issie]

sugartwin,

I'm sorry that happened to you. It is horrible when people don't recognize our illness. I used to wish for purple polka dots. Now, I just have to laugh and say, "Be careful what you wish for because instead of purple I got white spots. I now have vitiligo. Where you lose your skin pigment color." Nothing can be done, but there is a visible clue.

I had a friend do me that way and make me walk miles from a place I was very unfamiliar with. We took a trip together and because she wouldn't ask directions, she walked us in the wrong direction of where we were headed. She got mad because I started having a POTS eposide and had to sit down and nearly passed out. Because she missed the event she was headed for, she left me -- in a foreign country and I have direction problems. I was so scared. At the time, we didn't know my diagnosis and she said that someone else would have helped me if I'd fainted and fallen out on the ground. Needless to say, we don't talk any more. I don't need uncaring friends like that. I did make it back to the hotel, but was really sick afterwards. She never really apoligized, just very half heartedly. I just chalked it up to her "probable" mental state, and gave her the benefit of the doubt. If I hold on to the anger, it doesn't affect her in any way --- but it sure does and did for awhile affect me.

Are you aware that there is a state assist program for people who have money issues due to hospital bills? You can apply at the hospital and you will probably qualify for the state to pay your bill. It is based on your income past.

Hang IN There, we've all been in the same boat and we will continue to paddle together.