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http://mastcelldisorders.wallack.us/yabb/YaBB.pl General Mast Cell Disorders Discussion >> Symptoms >> Prolonged syncope? Shock & Exhaustion? Narcolepsy? WHAT??? http://mastcelldisorders.wallack.us/yabb/YaBB.pl?num=1331511400 Message started by Lisa on 03/11/12 at 13:16:40

Title: Prolonged syncope? Shock & Exhaustion? Narcolepsy? WHAT???
Post by Lisa on 03/11/12 at 13:16:40

I'm going to try to address these issues, but this is very complicated and don't know how well I'll be able to do this.

First of all, remember, I am NOT a doctor and no matter how much I have studied this I still will have gaps in my understanding. I have, however, been actively studying this subject since 2008 when my doctors began to witness this syncope after every single procedure they put me through. The REMA protocols protected me during the procedurs themselves, but this prolonged syncope is an extreme reaction that IN MY CASE could not keep me from undergoing the syncope. I have spoken repeatedly with both Dr. Castells and Dr. Escribano about this as well as a number of the other masto authorities in both Europe and the US. I seem to be the queen of this reaction and it is due to the frequency of this reaction and the fact that here in Brazil there are no authorities I can run to for help to investigate this. Not being content with "I don't know" is what forced me to seek for understanding and answers. We've had some good success!

===================================================

Prolonged Syncope was coined by French cardiologists in 1992 investigating an unusual situation of prolonged unconsciousness.

http://www.ncbi.nlm.nih.gov/pubmed/8024376

Arch Mal Coeur Vaiss. 1993 Dec;86(12):1747-52.
[A rare cause of loss of consciousness: mastocytosis. Apropos of 3 cases].
[Article in French]
Dacosta A, Guy JM, Cathebras P, Perrot JL, Decousus H, Tardy B, Gonthier R, Lamaud M, Rousset H, Verneyre H.
Source
Service de cardiologie, Hôpital Nord, Saint-Priest-en-Jarez.
Abstract
The authors report three cases of syncope due to systemic mastocytosis. This is a rare cause of syncope but should be recalled in certain circumstances. In the light of these cases, the authors review the literature with respect to this unusual presentation. Syncope may occur at any age. Loss of consciousness may be more or less complete, brief or prolonged, isolated or recurrent and usually accompanied by prodromal symptoms. The least controversial physiopathogenic mechanism of these syncopes is intense vasoplegia induced by the release of vasoactive mediators, especially histamine. When there is a clinical suspicion of mastocytosis, even in the absence of skin changes, the diagnosis is confirmed by biopsy which shows abnormally high numbers of mastocytes irrespective of the organ biopsied. The treatment of acute forms with collapse is based on intravenous infusion of macromolecular fluids and injections of epinephrine. Prevention is by drugs which inhibit the synthesis of histamine, the degranulation of mastocytes and the production of prostaglandin D2.
PMID: 8024376 [PubMed - indexed for MEDLINE]

The article is in French.

In this case, the patients underwent very short periods of syncope. My episodes are almost never less than an hour long. I have been seen consistantly out by my doctors on an average of 2 hours following invasive medical procedures, 5 hours following gallbladder surgery in 2009 and 6 hours with my last angiogram with contrast in 2009. I also recently underwent a colonoscopy and struggled for 10 hours against the sincope with no sucess until I was finally given an infusion of epinephrine with antihistamines. This infusion was given to me for my open heart surgery and it was due to the infusion I did not undergo sincope directly following the surgery, a first since my masto came out of hiding! My local doctors, however, have only found out that this is indeed the appropriate means to deal with this intense reacting for it is not a real syncope at all and would be more properly considered a coma instead.

Syncope is a unusual word to use for this reaction, however, it has only been studied once and in my opinion, the doctors were not fully aware then of all of the mediators and processes involved then. MC research has greatly improved since then the mechanisms involved in this. I have spoken with Dr. Paulo Evora, a Brazilian Cardiovascular surgeon and authority in vasoplegia. Dr. Paulo agreed with me that this would be rightly considered a type of coma. In investigating my case, Dr. Carlos Guerreiro, a Neurologist and Brazil's highest authority in epilepsy, he fully ruled out epilepsy in my case, which Dr. Castells had concured was good to fully rule out. Dr. Guerreiro said that in his opinion we were working with this very form of prolonged syncope, however, he didn't feel it was like any "syncope" he had ever heard of.

Syncope, in the majority of cases is either a vaso-vagal response or a cardiological response. When I say vaso-vagal, it's a reaction that uses the vagal nerve to create a vascular deficit - hypotention. Granted this is a VERY rudimentary explanation, but I'm not trying to put this into doctor terms, but patient terms for it's complicated. We faint because our blood pressure is not high enough to get to our brains and thus because of the lack of oxygen our brain either stops working or it shuts itself down as a means of protecting itself and conserving energy. We black out, literally because there's not enough oxygen. Therefore, in lying down and raising the feet, this will increase the blood flow to the brain and the patient recovers spontaneously. THIS IS NOT THE PROLONGED SYNCOPE!!

In a cardiovascular induced syncope, this has to do with the heart's functioning improperly. This leads to a series of reactions in that again, due to the heart's malfunction, oxygen is not reaching the brain and the patient blacks out, but they don't recover so easily for it's not such a simple situation. The problem here is not neuro/vascular, but is cardiological and until that is resolved, the patient will remain out. This is obviously a much more serious situation and needs immediate attention.

The prolonged syncope may indeed involve the vaso-vagal response, and it may involve the neurological system, but the problem here is NOT cardiological in that your heart is functioning properly. However, what is happening here is Intense Vasoplegia. Vasoplegia is basically the loss of tone to the blood vessels. There are many chemical reactions going on which affect the blood vessels themselves, one of them being Nitric Oxide. This overdose in the toxic chemical cause the blood vessels to lose their tone, to open themselves wide up and to maek the blood go racing through the veins. With this, the blood pressure drops and the patient goes into syncope.

Anyway, this is the theory. The fact it has been studied ONLY ONCE is the problem for although my doctors know without a doubt that I'm in intense vasoplegia, I do not get hypotensive!a Instead I am normotensive or hypertensive. Poor Dr. Evora, I have blown to bits all he thought he knew about vasoplegia for he never imagined this was possible - to be hypertensive in vasoplegia!! Dr. Evora is now studying about mastocytosis and learning more of what the MC is capable of doing.

In my case, due to a hyperadrenergic response, my adrenals and other glands are flooding my body with mediators to keep me from crashing and they are keeping a sustained level of blood pressure at this time. I do get hypotensive, but this is usually at home when it happens and not in a hospital setting. This is because, we believe, when I undergo medical procedures these trigger intense MC degranulation which then triggers the hyperadrenergic response and this serves to keep me from crashing. However, at home, when I have other triggering like to heat or physical exertion, I become hypotensive and this is when I have problems.

If you will note, the abstract says that the patient can undergo

"Loss of consciousness may be more or less complete, brief or prolonged, isolated or recurrent and usually accompanied by prodromal symptoms."

This means that the patient can go any amount of time in this syncope, there is no stipulation as to the duration. Another unusual distinction of this syncope is the "loss of conciousness being more or less complete" or in other words, this patient can go through LEVELS OF SEMI-CONSCIOUSNESS TO FULL LOSS OF CONSCIOUSNESS!

This was what made some doctors think I had totally lost it and gave me a "diagnosis" of Hysteria!! The neurologist and oncologist who heard me describe how I could hear the doctors talking around me cut me short in my description and declared that this was IMPOSSIBLE!!! This is NOT what Dr. Guerreiro said and in speaking with Dr. Evora, this made sense in it being called a coma for coma patients do indeed tell this very same story of being able to hear those around them!

When my BP has been either stable or high I have been able to recall bits and pieces of what has happened around me and even catch some of my doctors gossiping, much to their shock when I was able to tell them what they were talking about afterwards!! They no longer talk so freely around me when I'm out!!

And yet, what is going on with me at this time?

When I have an acute situation of degranulation like contrast it usually takes me about 20 minutes before the syncope overtakes me. When I undergo a procedure that uses anesthesia, I don't wake up from it and will come to semi-consciousness sometime during the syncope and am aware of what is going on around me but totally incapacitated to respond. When it happens at home, I'm suddenly so extremely exhausted that I have no choice but to lie down and the moment my head is down, I'm out!! The moment I lie down, I can't remain conscious any longer and I lose all capacity to govern my body.

In this state, I can't move a single muscle, not even my thorax to open my lungs to breath more deeply. I instantly go into what appears to be a sleeping state and my breathing immediately reduces to a very shallow level, even lower than with a sleeping state and it has been noted that I am almost not breathing at this time. When I have been in the OR recovery and on a monitor I become aware of my surroundings, but my doctors haven't a clue for my breathing does not increase nor my heartrate! They remain clueless that I'm there and this even created a mishap in the OR when my anesthesiologist could not recognize that I was semi-conscious. She gave me an antidote to the anesthesia thinking that this was the problem - the anesthesia was not being metabolized correctly. She injected the medication in my IV and immediately threw me into anaphylaxis! We are still unsure of whether it was the medication itself which caused the anaphyalxis of the violent shock to my system to wake me up! I ended up going back into syncope about 10 minutes later! The anaphylaxis immediately responded to IV antihistamines! That was one freaked out doctor!!! When I do finally come to full consciousness on my own, then my heartrate and breathing respond as any other normal person does.

The fact of being in this semi-conscious state seems to be dependant upon the blood pressure and I believe that this is why I will only lose full consciousness with situations which are either medical procedures which create intense MC degranulation and thus the hyperadrenergic response isn't enough to keep me from losing full consciousness, or this is where the unknown neurological aspect comes in. Without further study on this, there is no way to know what exactly is the sitaution. I am still seeking for neurologists and cardiologists who want to study this situation. However, I suspect this is one aspect for when I do get hypertensive, I will indeed lose full consiousness. I find myself coming back to semi-consciousness unable to answer for the changes of the television program my son was watching, or the arrival of one of my children or husband when they hadn't been there previously. It's rather disconcerting to say the least.

Now, is there emotional involvement? NO! NOT A BIT!!

In trying to understand this and in speaking with Dr. Guerreiro who raised the question of Narcolepsy and Sleep Paralyasis, these two items are purely neurological and are completely connected to sleep disorders. They have only one trigger in common - lack of sleep. They can not be triggered by excercise, or heat or medical procedures, etc. These patients also often have a very serious emotional involvement in their situations, dreadful nightmares!

My 3 years of pyschology at college came in extremely handy at this time for after reading up on Narcolpsy and Sleep Paralysis I was able to examine this aspect of my syncope. While in syncope there is ABSOLUTELY NO EMOTIONAL INVOLVEMENT! I've tried to feel afraid and I can't! I am totally without any kind of emotional involvement! Period. I am not outside of my body either. I'm stuck, like cemented into a body which will NOT respond to my slightest wish! Whent he syncope is light, I am able to struggle against it and return myself to full consciousness, but it takes extreme effort of concentration to do so. I must concentrate upon my breathing, trying to get my lungs to open up and breath more deeply. This concentration and the fruit of breathing more deeply helps me to regain control over my brain functions and muscles as well. Then, slowly I'm able to fully bring myself back up to full consciousness.

If my doctors try to do this, they must constantly be calling me, speaking with me, touching me and shaking me trying to force my brain to work again and thereby bring me back to full consciousness. And it's a battle for this reaction is so extremely peaceful that the body almost rebels against it! One of my doctors ended up getting me up, but it was such a violent change and took so much effort that this ended up triggering the stress system and put me into anaphylaxis!

So, what do we do about this? I have tried and tried to take double doses of oral anithistamines, and although this sometimes will help, most times it's like taking candy! A recent study by Dr. Simon Brown, an Austrailian internal medicine doctor, on Cardiovascular Anaphylaxis stated that studies on animals show that oral antihistamines are ineffective on patients going through this kind of anaphylaxis. Cardiovascular anaphylaxis is a type of anaphylaxis which directly affects the heart and this seems to confirm Dr. Escribano's findings regarding the MMAS patients - it may explain why some of us have more severe cardiovascular reactions like syncope than others do. Dr. Brown's study suggests that an IV infusion of epinephrine be the means of dealing with these patients and this is what was done for my open heart surgery and it kept me from going into syncope after the surgery, but once that epinephrine was stopped I began battling with the syncope again until they upped my IV antihistamines. This is what gave us the clue as to how to deal with my serious sitautions of intense syncope reactions - a 6 - 24hr infusion of both epinefrine and antihistamines until my body has settled down a bit.

However, this is a VERY SERIOUS situation and you MUST NOT just take my word on this. This is the battle plan that my doctors have finally been able to devise to treat MY CASE. I have high level cardiological help with this and doctors who know what they are doing and even then, it has take them a LONG TIME to come to these conclusions. Nevertheless, I have been ordered by my cardiological support that this must not be used EXCEPT for the most intense situations. Epinefrine is NOT for casual use and you must have your cardiologist working WITH your doctors. For the lesser situations we will use IV antihistamines on an infusion basis, for this is a safer means of dealing with this syncope without threatening my heart itself.

=====================================================

Okay, I think I've touched on just about everything. I'm ready to answer questions on this, but before I do, one last thing.

I've undergone EXTENSIVE testing to rule out many other issues. I've had a doppler echo done on the vascular structures in my chest and neck, looking for blockages of blood flow to the Brain. Two MRIs with contrast also looking for the very same problems. I've undergone two EEGs, multiple EKGs, Transesophageal Echodopplers, 24hr Holters, and 24hr MAPA, all of them looking to rule out any and all possiblilties. The only thing left is to runs some Tilt Table testing and some biochemical studies of the blood trying to rule out what few other possibilities that remain, however, they are considered very unlikely and there are no more suspicions as to what is happening here.

Intense Vasoplegia due to acute MC Degranulation = ANAPHYLAXIS!

Lisa

Title: Re: Prolonged syncope? Shock & Exhaustion? Narcolepsy? WHAT???
Post by Lisa on 03/12/12 at 00:11:19

PS - I have the research articles which support this and I will be happy to share it with whomever wishes a copy. Please send me a PM with your email and specifying what the information is that you are wanting.

Lisa

Title: Re: Prolonged syncope? Shock & Exhaustion? Narcolepsy? WHAT???
Post by Enko on 03/12/12 at 06:19:30

Lisa,

thank you, thank you, thank you for that post! You've already written to me about that topic, but I think that it's great for others to see.

My experience with syncope is also like being cemented in - at that moment I'm not afraid, but when I get back and figure out what actually happened it freaks me out!

Title: Re: Prolonged syncope? Shock & Exhaustion? Narcolepsy? WHAT???
Post by Lisa on 03/12/12 at 07:47:55

Enko, without a doubt waking up and realizing what has happened has got to be the most frightening part of the whole ordeal!! :o

It's such a peaceful situation that it's almost comic for you can hear things going on around you and especially depending on doctors and their comments, it's the chance to be that proverbial fly on the wall. But while there you are not a participant of anything, only an observer and so when you wake up, the realization of your complete and total helplessness is what is so frightening. Knowing you were able to hear and feel and be aware of things, up to a certain extent, is what is the problem here for your mind tells you that you MUST RESPOND and yet, your body is completely incapacitated and your mind can't respond even emotionally! It's just THERE and nothing more. The realization of all of this is terribly disturbing indeed!

Thankfully, I've kept a very careful control over my emotions and when it's been too much, I've let them lose for a bit, but never too much for emotions are dangerous things for masto patients. Thankfully, I've got some really incredible doctors who have been willing to work with me and who also keep an eye on me for signs of having difficulties emocionally. They've been willing to let me let loose with them and understood the pressures and that's been a big help. Yet, I also chose to look at this from as much as a medical viewpoint as possible. Early on I took my own emotions out of this and looked at my case from a 3rd person perspective. My case is no MY case, it's a patients case and I study it as though it has nothing to do with me. It's helped me to keep from getting too personally involved and the only time I allow my own feelings to get involved is when I have a procedure of some kind to face, then I let my emotions go a bit and allow them some air, cause keeping them under a tight lid isn't healthy either. So, they get a chance to air themselves at the right time and then I deal with them and get them back under control again and return to what I must do.

I hope this helps you in learning how to deal with your own emotions, Enko. Sorry you go through this too!

Lisa

Title: Re: Prolonged syncope? Shock & Exhaustion? Narcolepsy? WHAT???
Post by Enko on 03/12/12 at 08:12:39

It's like that you're trying to start the engine and he won't budge even a bit ;)

When I was a kid I used to think that when you die it's like that you're laying down peacefully and listening to what others around you do. Reminds you of something? THAT'S JUST WEIRD!!! :o :o :o

Title: Re: Prolonged syncope? Shock & Exhaustion? Narcolepsy? WHAT???
Post by Doozlygirl on 04/15/12 at 17:58:47

Lisa,
This response is long overdue and I alopogize for that. Thank you so much for sharing your research on your episodes of prolonged syncope/loss of consiousness. I needed time to absorb all the information you shared. I have had hundreds of these episodes over the years. There are days I can't awaken (takes 2-4 hours to become alert), and episodes where I have forced sleep, as if I were given anesthesia. I also have episodic stroke level hypertension and then plummeting BP, which I have attibuted to anaphylaxis. My forced sleep episodes usually last 4-6 hours but have lasted over 20 hours.

I see Dr Afrin next month and look forwrd to his take on these episodes. I will post more when I learn more. Thanks for sharing,
Lyn

Title: Re: Prolonged syncope? Shock & Exhaustion? Narcolepsy? WHAT???
Post by frugalmama on 04/15/12 at 20:36:10

Thanks for sharing this, Lisa. I have been experiencing this for four years and have been unable to clearly verbalize what is happening. I tried to explain it as a sudden, extreme exhaustion, but that doesn't do it justice, as I literally cannot keep my eyes open.

"When it happens at home, I'm suddenly so extremely exhausted that I have no choice but to lie down and the moment my head is down, I'm out!! The moment I lie down, I can't remain conscious any longer and I lose all capacity to govern my body."

This is precisely what I experience. When our nanny sees me crashing, she often takes the kids outside....and you can imagine the chaos with a 5, 3.5 and 1.5 year olds running around feet from my head....and I'll be out cold turkey. I usually come around after an hour or so. I am very sedentary now as a result of major POTS symptoms (my original diagnosis), and these episodes are brought on not only by standing, but heat, sometimes food, pressure (i.e. a kid sitting on my lap for more than a minute or two), etc. Even with the little bit I am able to do now, this happens a couple of times a week. Before we had full-time help with the kids, it was happening 1-2 times per day (thank goodness for the play pen and Sesame Street), it's funny, I had actually wondered aloud to my husband a few months ago if I was falling asleep or actually losing consciousness, as there is nothing I can do to keep my eyes open.

I look forward to hearing what Dr. Afrin has to say, Lyn. Maybe they can shed some more light on this for those of us dealing with this!!

Lisa, I do have a follow-up question. My allergist/immunologist suggested going to the ER when this happens and my BP drops below 90/60 (normally 120/80), but what do we tell them when we get there? The way our system is, I'd likely be stuck in the waiting room for hours before even getting into a room...and as you know, there is no possible way I could sit up at this point. So what do we do, and how do we get proper treatment? Any suggestions? I will also PM you for the articles....I'm intrigued :). Thanks again! :)

Title: Re: Prolonged syncope? Shock & Exhaustion? Narcolepsy? WHAT???
Post by kimtg68 on 04/22/12 at 00:23:03

Lisa, as usual, most informative and thoroughly researched (as much as can be at this time). Thank you so much for sharing your wealth of knowledge with us as we all shuffle through all these bizarre symptoms and situations.

About a month ago or so I had posted a question about Anaphylaxisis and Syncope. I go through BOTH! The Anaphylaxsis usually is accompanied by headache, nausia, flushing and shortness of breath. The syncope I experience is different. I start off feeling EXHAUSTED and MUST sit or lay down. Then I get really cold so I cover up and then the next thing I know I'm fast asleep. Sometimes I can hear things going on around me but I feel like I'm way to sleepy to react or respond. Then I usually end up waking up in a near panick. I have taken my BP several times as these symptoms occur and the lowest so far was 80/50 before going into that state of sub consciousness. I am now seeing a doctor up in Nashville at Vanderbilt and he believes I have POTS. I am working down my heart meds now in order to do the Tilt Table Test. One of the symptoms he saw that also led him to believe this is the lace like pattern on my limbs. When I get cold it turns purple. When I stand for a length of time it become more pronounced. From the research I've done it appears to be blood pooling. It is recommended one sit's or lay's down and elevate the feet above the heart. Anyway, some of what you addressed sounds similar to symptoms of POTS (or perhaps some of the symptoms of POTS are similar to what you are explaining). It's interesting that when you explain the moments of being aware of your outside situation but not able to respond, I've felt like I was just in a state of sleep and wakefulness but still yet to sleepy to respond. OK my brain keeps freezing up on me and I'm losing my thoughts and why I'm posting this :-? I think I posted because I thought that what you were sharing also sounds like the research I have been doing on POTS and the different forms of it and how it affects someone.
Thanks very much for sharing with us.

Title: Re: Prolonged syncope? Shock & Exhaustion? Narcolepsy? WHAT???
Post by PJP123 on 04/22/12 at 08:22:36

@Kim and Lisa

When I degranulate I start feeling "sick" I have to sit down. Immediately I realize sitting down isn't good enough, I have to lay down. As soon as my head hits the pillow I'm going into a coma, I notice heart racing and irreg. beats (full cardio workup said I was fine except mitral valve prolapse) and then I'm out cold. I'll sleep for 2 - 3 hours and wake up feeling better. What the heck is this? POTS or Syncope. I've had it my whole life and in High School the teacher's thought I must be a stoner because I couldn't keep my head up.

@kim

I have the mottling of my thighs, which I showed 2 docs and they said fair skinned people's skin looks like that when they're cold. I say BS.

Title: Re: Prolonged syncope? Shock & Exhaustion? Narcolepsy? WHAT???
Post by Lisa on 04/22/12 at 10:44:15

Okay, Ladies, I´ve got MORE I can say on this now!!! And it´s VERY INTERESTING!!

I´ve been speaking with Dr. Molderings and Dr. Afrin regarding this and they both opened up some understanding for me. I´m still researching this and am speaking with neurobiologists who may be taking my case a bit further, for we´re suspecting that this may not be the fault of vasoplegia after all and that in reality it is a neurological situation.

What the French cardiologists reported in 1992 was indeed prolonged syncope and yes, vasoplegia is involved and those 3 patients they studied were hypotensive, and so they may have logically presumed that the vasoplegia, causing the hypotension, was the fault. The other published case histories, including one of a seriously brain damaged Italian woman, were also these cases of hypotension.

Now, what they do know is that prostaglandin D2 is a major mediator known for being involved in the syncope as well as being a vasodilator and it may one of the mediators responsible for the hypotension, as well as the syncope itself. According to Dr. Afrin, this mediator focuses on the sleep center of the brain and is known to affect it. It is also believed to be involved in NARCOLEPSY.

And this raises a very, VERY important issue here. The syncope and narcolepsy are two very different things and masto patients can go through BOTH!!!

Being overwhemlmingly exhausted can be a symptom of both the syncope and narcolpsy. However, SLEEPINESS is that of ONLY narcolepsy. It is NOT part of the prolonged syncope. It´s very important that you observe these episodes and begin noting them down. If this stupor situation goes on for more than 12 hours, this is most likely NOT syncope. The reason why I say this is because I´ve taken a poll amongst patients in the US, Canada, the UK and Germany and there was only 1 patient who underwent up to 8 hours long in syncope following SURGERY. It was a very big trigger that left her so long in syncope and I´ve seen from my experience, that this is true. Situations of syncope which happen at home due to the trigger of heat, or exercise or something like that are light triggers and the syncope is brief in comparison to a trigger like surgery or contrast. These triggers cause a very deep syncope which goes on for hours and will leave me totally incapacitated. But this is not sleep related and I do not sleep and am not sleepy. I don´t wake up not needing to sleep, in fact, this leaves me exhausted needing to sleep for I´ve not rested in syncope. It´s a very different state and it feels like fainting, not sleeping and for those of you who have gone into syncope you know it doesn´t feel like sleep and the two feel very different.

If you are in stupor like state for hours, whether you do wake up in the midst of it and can hear things around you, if this goes on for hours and hours and hours, this is most likely narcolepsy, especially if you wake up terrified. Any kind of emotional involvement is a known characteristic of narcolepsy whereas the syncope is an absense of emotional involvement and you wake up like anyone else who was in syncope - wondering what was going on, but not emocionally frightened. The emotional involvement only comes in when you take account of what was happening - then it hits you.

I have asked Dr. Afrin if I may post here his comments. I am waiting upon his reply and if he allows me I will.

Also, I ask your consideration - my computer crashed again and is in the shop! Thankfully I did NOT lose my research!!! Whew! :D I will try to send to you who asked for more research when I can. And I will try to answer these questions as soon as I can!!

Lisa

Title: Re: Prolonged syncope? Shock & Exhaustion? Narcolepsy? WHAT???
Post by kimtg68 on 04/22/12 at 13:48:07

May I hijack your post for just a brief moment Lisa?

PJP123: look up Livedo Reticularis. Does it look like that? Mine does. Only two doctors have picked up on it on their own: Dr Afrin and the new Vanderbilt doc, Dr Fahrenholz. It was Dr Afrin who said it looks like Livedo Reticularis and then he said ,"Hmm interesting" the Vanderbilt doctor noticed and asked me some questions and after a discussion said he wants me to do the Tilt Table Test for POTS. So perhaps there is pooling of blood. Now with what Lisa is describing with vasval (whatcha ma call it) and Prostaglandin D2, well my prostaglandin D2 was three times the norm. Maybe a connection? Time will tell. But I wouldn't poo-poo you lace like pattern. Keep pushing for answers ;)

Title: Re: Prolonged syncope? Shock & Exhaustion? Narcolepsy? WHAT???
Post by PJP123 on 04/22/12 at 15:44:09

Kim,

Yes this is what it looks like. These doctors really are so stupid. When I first noticed it I told my primary care physician and he brushed it off. I showed rheumatologist too and don't remember who else. I told them my legs didn't always look like this, that it was something different. Why won't they help us. I've had the fatigue since 15 and every doctor just brushes it off. I went to a neuro psychiatrist 2 years ago because I was hoping he'ld say I was bi-polar or something and I could take a pill and feel better. No such luck. It's mast cell disorder.
I haven't done the prostaglandin test yet. I'll write if it's high so we can compare notes.

Pam

Title: Re: Prolonged syncope? Shock & Exhaustion? Narcolepsy? WHAT???
Post by kimtg68 on 04/22/12 at 15:57:57

Are you scheduled to have a mast cell work up with the prostaglandin's included? I'm curious to see if there's a corolation with the Livido Reticulars and mast cells and/or POTS. Thanks and good luck! I hope you get some answers.

Title: Re: Prolonged syncope? Shock & Exhaustion? Narcolepsy? WHAT???
Post by Lisa on 04/22/12 at 23:57:18

Dr. Afrin gave me permission to post this here. He´s making a comment to me regarding narcolepsy and syndromes which may mimic syncope, but not be syncope in reality. Remember, these are personal observations and theories as to what can compete as a differential diagosis for syncope. These are considerations which should be considered WITH YOUR DOCTORS for these are very, very similar in nature and are easily confusable with one another and you do really need a doctor´s help to confirm any suspicions you may have. I have done this every step of the way, always taking my suspicions to my doctors for them to CONFIRM and IDENTIFY, I have never done it on my own.

"Perhaps this non-hypotensive "syncope" is actually narcoleptic in nature. Our normal wakefulness is driven in significant part by release of the mediator orexin (produced by certain neurons in the brain, largely in the hypothalamus) which then docks with certain brain centers which stimulate wakefulness. Loss of the ability to produce orexin causes narcolepsy, whose clinical presentation can range from simple chronic lethargy to (very) acute episodic severe lethargy/sleep superimposed on mild chronic lethargy. It's now understood that narcolepsy is an autoimmune disease in which the orexin-producing neurons of the brain are, over time, wiped out. MCAD has a long-recognized propensity for causing autoimmune disease -- potentially *any* type of autoimmune disease. I don't see any reason, a prior, why autoimmune disease targeting orexin-producing neurons would be exempt. So, one scenario that could be imagined is an (MCAS-driven) baseline narcoleptic state of partial orexin deficiency that's then periodically complicated by local release (by the aberrant mast cells of the underlying MCAS, either systemically or perhaps just in the Central Nervous System) of prostaglandin D2 -- the most potent human somnogen, as far as I've been able to discover in my reading to date. I guess it's also possible that there might be some other mediator released by mast cells which directly competes with orexin at the orexin-1 and -2 receptors, and perhaps a flare of release of this other putative mediator "outcompetes" at the receptor site with the (partially deficient state of) orexin to result in acute severe lethargy that's interpreted as syncope.

Interestingly with respect to the obesity often seen in MCAS, orexin deficiency impairs pre-adipocytes from differentiating into brown adipose tissue which burns fat into heat. Thus, orexin deficiency contributes to obesity. Certainly prostaglandin D2, too, is closely involved in lipid management and lethargy, but orexin deficiency via an autoimmune pathway is potentially another route by which MCAS could lead to obesity and lethargy.

Of course, there's also the obvious alternative of simple PGD2 release by aberrant mast cells (as stimulated by a range of sensory inputs) in the brain's sleep center. In other words, there may even be no need to bring orexin into consideration, but it still might be a "narcoleptomimetic" issue driven solely by PGD2.

Title: Re: Prolonged syncope? Shock & Exhaustion? Narcolepsy? WHAT???
Post by angelloz on 04/23/12 at 13:58:51

Wow...great information and so much to absorb. I went to the ER on Thursday night. BP 84/43....knew I was going to die. They admitted me, gave me fluids but no advice except go to the ER when this happens. and gets below80/50, well this happened to me several evenings in a row, I couldn't go every time. I usually deep breath drink cold water and try to stay conscious. Sooo scary... sometimes I think my fear pops it back up for a short period of time . Thanks to all for posting all the thoughts and views.
Angelloz

Title: Re: Prolonged syncope? Shock & Exhaustion? Narcolepsy? WHAT???
Post by goldielove on 04/23/12 at 14:30:14

WOw must be scary too have your BP so low, is there anyway you can get fluids via IV though your insurance it might help my friend who has pots like me needs IV fluids like 3x a week her daughter knows how to do this so she does it. I also have POTS so I know what a feeling of syncope is like the funny part is my BP is sky high when I get that feeling, they ER have tried too give me meds too bring my BP down but it only goes higher now I know too take an extra antihistamines and it goes down and I don't go too the ER, I hate the Er they don't ever do anything and they look at you like you are crazy and need the psych ward. Lisa thanks for all the info the other day I had what I felt was just absolutly chills thoughout my body just so weird I could not get warm it was about 89 here so not a problem with heat, I went under four blankets and could not warm up then I just layed there I was awake the whole time but I had a hard time focusing on my husbands words but I was awake and hearing him, my BP and pulse were normal. I was just frigid. Anyway it lasted for some time but then I became warm again and I was ok go figure, our bodies are crazy my MD said it was my autonomic nervous system in action????

Title: Re: Prolonged syncope? Shock & Exhaustion? Narcolepsy? WHAT???
Post by WendyH on 05/16/12 at 08:57:00

I saw a couple of posts here about Livedo Reticularis, I looked up the images too and my legs get this way too, I always thought that was the TMEP maybe not. Now I have to read up on what it is

Title: Re: Prolonged syncope? Shock & Exhaustion? Narcolepsy? WHAT???
Post by kimtg68 on 05/16/12 at 13:06:57

Wendy, I researched Livedo Reticularis also. Even Dr Afrin commented on the lace-like pattern on my legs when I saw him back in September. But nothing ever came of it.....UNTIL I saw Dr Fahrenholz at Vanderbilt in Nashville. The Livedo reticularis appearance along with other symptoms I have which are getting worse and not responding to the H1 & H2 therapy brought him to suspecting POTS. I've seen some of your other posts mentioning a curiosity with your symptoms and POTS. All the research I did on livedo reticularis does not mention POTS however in understanding both disorders I can see how they relate since circulation is an issue with both. Just thought I would share my experience in case it's of any use to you.

Title: Re: Prolonged syncope? Shock & Exhaustion? Narcolepsy? WHAT???
Post by Doozlygirl on 05/22/12 at 12:55:27

Lisa,
I saw Dr Afrin last week. I asked him about my forced sleep episodes and he immediately mentioned PGD2. Which much of his questioning seemed to focus on issues with prostaglandin synthesis in my case. He was very interested in knowing if I had nosebleeds, or other bleeding and bruising issues, as well as cramps and headaches, which all can be tied to Prostaglandins. He will get into more detail when my lab work comes back. I'll keep you all posted what I learn.

Lyn

Title: Re: Prolonged syncope? Shock & Exhaustion? Narcolepsy? WHAT???
Post by Lisa on 05/22/12 at 14:47:19

Thanks, Lyn!! Yes, I think he's right and this is what the research shows, that prostaglandins are indeed one of the major mediators! I'm so glad he's on top of this with you. He's got a brilliant mind and will do what he can to see if he can find answers for you!!!

Let me know how it goes!

Lisa

Title: Re: Prolonged syncope? Shock & Exhaustion? Narcolepsy? WHAT???
Post by Joan on 05/22/12 at 18:31:30

Hi Lyn,

So glad you got in to see Dr. Afrin. I'm very interested in what he has to say, as I have problems with PGD2, I'm certain. Didn't know it affects bleeding and bruising!

Title: Re: Prolonged syncope? Shock & Exhaustion? Narcolepsy? WHAT???
Post by Lisa on 05/23/12 at 06:42:37

Lyn, I hope you don't mind, but I'm going to have to correct you here. I think you misunderstood Dr. Afrin about the PGD2 causing bleeding.

Since I had already happened to speak with him the day before, I asked him to clarify this because I know that PGD2 is connected to syncope and sleep issues and a few other things, but I've never seen it connected to bleeding and bruising - heparine is. But I didn't want to say anything without his clarifying this first, which is why I made no comment.

This is what he said:

PGD2 likely has little or nothing to do with bleeding/bruising issues, which, if localized, are far more likely due to mast cell release of heparin, or, if systemic, may be due to anti-phospholipid antibodies as an autoimmune consequence of the mast cell disease.

-- LBA

Title: Re: Prolonged syncope? Shock & Exhaustion? Narcolepsy? WHAT???
Post by Doozlygirl on 06/10/12 at 17:57:07

Lisa wrote on 05/23/12 at 06:42:37:

Lisa and Joan, just saw this post. As I reread my post, I see I should have clarified and stated uterine bleeding is driven by prostaglandins. Sorry for any confusion. I really struggle with excessive menstrual bleeding which of course leads to hypovolemia and orthosatic hypotension. Heparin and is the big mediator tied to other bleeding.

Lyn

Title: Re: Prolonged syncope? Shock & Exhaustion? Narcolepsy? WHAT???
Post by Lisa on 06/11/12 at 02:31:30

Yes, prostaglandins, but not all are the same. Prostaglandin F2 is the type of prostaglandin related to menstration. Whereas Prostaglandin D2 is related to sleep and syncope. Prostaglandin E is related to lung function I believe, but I´m not certain on that. There are TONS of mediators released from the MCs and each one has certain functions and some they are still unsure of what they fully do. It´s hard, uphill work for our researchers and there´s a great deal more not undersood than that which is understood. So, don´t worry about the mistake! If the doctors themselves have a hard time keeping it straight, how much harder it is for us!!! :D

Hugs!

Lisa

Title: Re: Prolonged syncope? Shock & Exhaustion? Narcolepsy? WHAT???
Post by ruth on 06/27/12 at 07:57:28

Cameron had an interesting episode recently, he took himself to bed at 6 o'clock at night and slept for 13 hours. I couldn't rouse him enough to get much response from him, I assumed he had a migraine, but when I finally woke him in the morning he said there had been no headache, or aura, which is his typical pattern. His doctor said this can be a form of migraine, only sleep, and that it might be a sign he is growing out of them. I'm not holding out hope for that yet, it was the 4th episode in 2weeks, which is much worse than his typical once a month or so. However it was interesting that this can be a type of migraine. Anyone else heard this?

Title: Re: Prolonged syncope? Shock & Exhaustion? Narcolepsy? WHAT???
Post by Doozlygirl on 06/27/12 at 20:46:14

Ruth,
I have "Been there, done that" with the migraine stuff. First one happened over 33 years ago. I carry diagnoses of hemiplegic migraine, complicated migraine with and without aura and complex migraine with and without migraine. Several neurologists, including 2 autonomic neurologists amd a sleep specialist who is a neurologist, could not explain why I had these sleep episodes. My current autonomic neurologist believes it was due to dysfunction of orexin, the chemical that triggers awakeness or sleep. Dr Afrin believes it is due to prostaglandins, which for me makes more sense. I flush heavily during these sleep episodes and often awaken with a headache that is different than a typical migraine (pulsing) headache. Asprin will abort the headache, which makes sense if prostaglandins are triggering it.

Good luck sorting it all out.
Lyn

Title: Re: Prolonged syncope? Shock & Exhaustion? Narcolepsy? WHAT???
Post by Lisa on 06/29/12 at 06:34:09

Ruth and Lyn,

Because there is so very little written on this prolonged sycnope that we masto patients go through and because there has been ONLY ONE study and that was done with ONLY 3 patients, there is a GREAT DEAL LEFT OPEN FOR DISCUSSION!!!

I have studied this a great deal and have been recently speaking with neurologists at the NIH and some who have written up case histories of their masto patients trying to find more understanding about this. I have the support of a Brazilian doctor who is an authority in vasoplegia and he is helping me and we are coming to some conclusions about this and I´ve gained the support of some neurologists and cardiologists at the University of Sao Paulo (USP) who want to investigate my case further. USP is one of Brazil´s best and largest medical universities and their doctors are top notch and to have them take interest in my case is very important. Dr. Paulo Evora, who is also with USP has been supporting me since 2009 when I began seeking for answers for my aortic aneurysm and he is who led me to INCOR where they were able to save my life with my open heart surgery. He fully understands vasoplegia and he is supporting my conclusions regarding it´s involvement in this reaction.

I´m not at liberty to say what we suspect right now and it´s enough to say that there seems to be much more going on here than that which was reported 20 years ago. However, for right now, all we can go on is that which has been reported. Each case is it´s own case and each of us have our own personal issues with the mediators being released and how those mediators affect our bodies.

What needs to be done is that you have doctors investigate your cases for epilepsy and have an EEG run while you are in syncope. Your mediators also need to be measured. There is a very strong need to eliminate any doubts regarding epilepsy for there is a case where this is involved and this syncope has been proven to cause brain damage when the patient is hypotensive. It is indeed a DANGEROUS reaction and there is one death already contributed to it. Whether or not it is true syncope is perhaps the question, and unfortunately doctors, thinking it is merely that, do not take it seriously. But they should for this is not a normal syncope and anyone who remains hypotensive and in this reaction for hours is in danger of becoming brain damaged.

The articles show that this patient must be given epinephrine to turn this reaction around. If your doctors are afraid to give epinephrine, then IV antihistamines is the next best thing.

The reason for either epinephrine or IV antihistamines is because this is mediator provoked and the mediators must be combated. Prostaglandins are proven to be involved, but nobody has indicated any kind of anti-prostaglandin treatment, only epinephrine. We stumbled upon the IV antihistamine treatment through a report written by Dr. Simon Brown on Cardiovascular Anaphylaxis and Shock in Anaphylaxis articles. He cites the findings of the use of antihistamines in animals and that oral antihistamines was ineffective as well as even dangerous. Yet in my case, since I was in the ICU following my open heart surgery, we had no other choice but the IV antihistamines and they were VERY effective in combating the syncope whereas it was following that, when I got back home that we saw that the oral antihistamines were like taking mentos - useless!!!! Why? I don´t know yet.

Sometimes we must insist with our doctors. Even if they are afraid to use epinephrine, INSIST upon at least the IV antihistamines!! You should not be left just lying there in syncope with it going on for too long of a time period. This is a potentially dangerous reaction and it´s proven to be so! Their ignorance of this doesn´t make it any safer, so please insist with your doctors.

If you are needing articles, please PM me and inform me what the subject is that you are needing please and I`ll be glad to send you the information - don´t forget your email address!!!

Lisa

Title: Re: Prolonged syncope? Shock & Exhaustion? Narcolepsy? WHAT???
Post by gd0001 on 08/22/12 at 15:11:43

i think something like this happened to me.
I started feeling like i was spinning, similar to when you drink too much.
But it kept getting worse and worse. Then i laid down and
was semi-conscious for 4 hours. I thought i was dying.

It never happened to me before. I think it might have been a reaction to something i ate.

Title: Re: Prolonged syncope? Shock & Exhaustion? Narcolepsy? WHAT???
Post by Lisa on 08/25/12 at 03:11:48

Sorry I took so long to see your post, GD, I´ve been rather busy lately and since this is a reaction I go through on a frequent basis, I´m probably the best to answer this.

I would say most definitely this was related to some kind of trigger. It could be food, heat, physical exertion, emotional stress, many things. It´s important that you try to rule out possible problems with vascular circulation and epilepsy. At this point in time there has not been any serious investigation into the neurological possibilities here and we can only pinpoint it to cardiovascular problems and that of hypotension being behind it. So for now, that´s all we can say, but my case refutes the research because I´m almost always normotensive or hypertensive when I´m going through this and doctors have yet to study a patient with this aspect.

So, try upping your antihistamines when you feel this coming on. Take a double dose of your antihistamines and see if this helps to pull you out of it. It sometimes helps me.

So sad - they studied it and blamed it on the hypotension but didn~t tell us how to treat it!! Epinephrine is the way, of course for when your BP drops or elevates its indicating anaphylaxis and this is important that you intervene when your BP drops.

I hope this helps!

Lisa

Title: Re: Prolonged syncope? Shock & Exhaustion? Narcolepsy? WHAT???
Post by gd0001 on 08/28/12 at 14:06:47

Lisa wrote on 08/25/12 at 03:11:48:

this is a reaction I go through on a frequent basis, I´m probably the best to answer this.

Do you have the spinning feeling too?
I've noticed the spinning before, but this was the first time it got to the point where i was semi-conscious.

Title: Re: Prolonged syncope? Shock & Exhaustion? Narcolepsy? WHAT???
Post by Lisa on 08/28/12 at 18:22:39

Spinning you say? Well I have had a couple of times some pretty heafty dizziness yes, but not directly related to the semi-conscious syncope state, no. But then, we are all so different, why couldn't happen.

With masto, there are very few hard and fast rules except that it makes you feel pretty awful at times!

Lisa

Title: Re: Prolonged syncope? Shock & Exhaustion? Narcolepsy? WHAT???
Post by PJP123 on 10/03/12 at 07:02:21

FINALLY SOME ANSWERS!

I just got back from tilt table test and after 30 minutes the doc says well you don't have POTS. I said to the nurse "this is impossible" I know this has to be my problem. Then they shot me with adrenaline and I came within a second of passing out.

Confirmed Neurocardiogenic Syncope. I need to read more about hyperadrenergic POTS and see what if anything is the difference. Is this just another one of these "rare" things that docs don't know about? (Meaning Hyper Pots).

It only took 51 years my friends. Just think. My life has been awful for 51 because of this and not a single doctor ever thought to send me to an elecrophysiologist.

I want to thank MSG who first put the thought in my head to look into POTS.

Title: Re: Prolonged syncope? Shock & Exhaustion? Narcolepsy? WHAT???
Post by PJP123 on 10/03/12 at 15:22:51

Also I wanted to ad that I have never passed out so I don't understand the neurocardiogenic syncope diagnosis.

Title: Re: Prolonged syncope? Shock & Exhaustion? Narcolepsy? WHAT???
Post by Lisa on 10/03/12 at 23:42:26

A DIAGNOSIS!! YEAH!!!!!!!!!!!!!!!!!!!! :D

People who don´t have a MC disorder don´t realize how liberating a diagnosis is for us!!!!! With a diagnosis comes TREATMENT and UNDERSTANDING!! It empowers us and our doctors to give us a means of dealing with our symptoms otherwise everybody just treats you like you´re demented!!!!!!!

So, as bittersweet as this is, it´s worth CELEBRATING!!!!!!!!! :D Congrats!!!!!

Now, neurocardiogenic shock is something I need to brush up on and I´ll have to get back to you about it. I understand the basics, but I don´t want to just say it off the cuff cause I might get it wrong, so I´ll pop back on here and let you know what I get out of it.

Lisa