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!
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Prolonged Syncope was coined by French cardiologists in 1992 investigating an unusual situation of prolonged unconsciousness.
http://www.ncbi.nlm.nih.gov/pubmed/8024376Arch 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.
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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
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Prolonged syncope? Shock & Exhaustion? Narcolepsy? WHAT??? (Read 16713 times)
