EDS and NCS

[Guest guest]

First of all, thank you, Sue, for the welcome. Yes, we have " met " and emailed

before on other EDSer lists. It's always good to meet an old friend.

I just wanted to share a little of what I've learned about the connection

between EDS and Neucardiogenic Syncope. It may not currently apply to anyone

now reading this list, but perhaps it could help someone in the future.

Neurocardiogenic Syncope is a disorder of the autonomic nervous system (which

controls all involuntary functioning, such as digestion, respiration, blood

pressure, heart rate, etc.). You can easily see that if the autonomic nervous

system has some " short circuit " , the effects can be far reaching, and can be so

severe as to become debilitating. NCS is a disorder of the blood pressure and

heart rate. It is a condition people are born with, though many will never know

it, or be bothered by it but little in their entire lifetime. Others are

utterly debilitated by it. In the EDS victim, naturally, our veins are all made

of collagen....in our case, defective and too " stretchy " . This can lead to

blood pooling in the lower legs/feet/extremities, and not being pumped back to

the heart and brain in an effiecient and consistent manner. As blood pressure

plummets, often the heart rate will increase to try to compensate (a condition

called presyncope). However, if the person with NCS tries to remain standing in

one place too long, no amount of foot tapping, shifting, or other learned

compensation techniques will work. Gravity will win everytime, and we will

faint. Once the body is horizontal (and preferrably with someone propping our

feet higher than our heart to send the blood back), the blood flow can return to

a more normal state, and we regain consciousness. One out of 10 people who

suffer with NCS will also have convulsive syncope at least part of the time,

which means that as their brain fights to maintain consciousness and a vertical

body posture, they will convulse as they pass out. This is NOT to be confused

with seizure activity, though to a layperson, it is hard to tell the difference.

I took my son, who also has HEDS as I do, to MANY doctors, and finally to Dr.

Blair Grubb at the Medical College of OH last July. Dr. Grubb is world reknown

for his lifelong study of NCS and his expertise in treating it effectively in

most cases. He did help my son, and it was well worth the trip, though we live

in TX. I have since been also diagnosed with NCS, and will be seeing Dr. Grubb

myself in Feb. The accepted treatment for NCS is blood volumizing drugs such as

Florinef, Proatamine, etc. These drugs help to raise the blood pressure and

increase the pressure of blood flow against the walls of our already too

" stretchy " veins, thus decreasing the syncopal (fainting) episodes. Also,

research shows that there are 3 antidepressants which help some people with NCS,

given in very small doses. They are Celexa, Paxil, and Zoloft, and in that

order. They do not yet know just why those drugs works as of yet. The other

line of treatment is a pacemaker and/or defbrillator if the patient does not or

cannot respond to drug therapy. My son may be one of those, but time will tell.

Though he is now only 17, he went into ventricular fibrillation and had to be

cardioverted x2 and given a gram of procainomide during a " routine " EP study and

heart cath at Cooks Childrens in Ft. Worth on May 17, 2002. He now has a

surgically implanted Medtronic Reveal Loop Recorder, which is recording all

heart rate activity 24-7 for the next year. This data will help to determine

the settings for a future pacer if needed, and it looks like he's heading in

that direction. I have read several of Dr. Grubb's books as well as numerous of

his articles. He is deeply interested in EDS, and has diagnosed a number of

people with it in the past year. He told me in July that he had recently

visited Israel, and that " there is a body of research which has just recently

been completed there, but not yet published, which indicates a conclusive

correlation between EDS and NCS. It also indicates that EDS most probably

affects a minimum of 2% of the total world population to some degree or

another. " I am currently on Proatamine, and my son takes Florinef and a small

daily dose of Celexa. They do not entirely prevent us from periodically

" sniffing the carpet " : (passing out), but they do help. That makes good common

sense to me. The autonomic nervous system tends to be " out of whack " in EDS

victims, Dr. Grubb said, which might explain one part of why many of us suffer

from Irritable Bowel Syndrome: increased/decreased bowel motility. I have it

with a vengence, and am now waiting for Lotronex, as other drugs have been tried

on me with inconsistent success. I have become hospital dependent for

rehydration, only a stop-gap measure at best.

I was in contact with Dr. Gottesman of the National Institute of Health for

over a year. The NIH will be doing an adult clinical trial study of genetic

connective tissue abnormalities, including EDS, in the coming weeks. Protocols

have been approved, and I am very excited about participating in this study.

Dr. Gottesman recently accepted a position in St. Louis, and the study is now

under the direction of Dr. Francomano, which whom I've also spoken by phone

several times. The study will be conducted at s Hopkins. Shall I send a

link about it?

I hope I've offered some information which might prove both interesting and

helpful, and that I haven't gone on too long. Anyone who has any questions or

comments, please do email me.

Love,

Digest Number 1435