Hi....I have received so much support here on various forums and am looking for some help for my brother who received a diagnosis of Autonomic Failure....which has been barely responsive to treatment and within the past 2months caused such a rapid decline in my brother's health. He is now unable to walk with severe neuropathy to both arms and legs along with numbness. Despite being on 5 meds, having a pacemaker, and seeing many cardiologists/neurologists from John Hopkins, Cleaveland Clinic and most recently his doctors consulting with the head of Vanderbilt University's Automomic Failure Center there seems to be no treatment or answers to helping my brother from further progressing with this condition. As a family we are not ready to accept that there isn't anything we can do to stop this from worstening....my brother is only 43 and has 6 children, the youngest being only 2 and we are hoping that somehow we will find the miracle we need to treat him so he will respond and have a good quality of life.
Five years ago after biting into his food on his fork he had severe pain in one of his teeth that had his hand jerk causing the fork to fly across the room. The day after he started passing out whenever he stood up. He went to the hospital and they noticed that he had orthostatic hypertension and tachycardia whenever he stood up followed by a sudden drop in his blood pressure that caused the fainting spells/blackouts. His tilt test was the worst they had ever seen with his blood pressure going sky high followed by a plummet with severe tremors and him blacking out. POTS was ruled out as well as Pheochromocytoma. They put him on some betablockers....he had severe headaches that never seemed to go away....the meds helped somewhat in controlling the fainting spells for a few years and eventually a pacemaker was put in to keep his pulse from going dangerously low while sleeping from the meds helping out.
This past year his condition worstened to the point that he was unable to work...he started to have violent tremors that would follow his increased Blood Pressure and severe headaches.....the tremors would look like grand mal seizures but he would be alert and they would last as long as 30 minutes until he received IV Dalaudid (sp??) to relieve the headache. His life consisted of calling the ambulance on a weekly basis to bring him to the ER to recieve the IV meds that would relieve his headache and stop the tremors.
My brother's condition has declined so much that anytime he moves from bed to a wheelchair he goes into severe tremors. He is not able to urinate and is now in acute renal failure with a creatinine level or 1.5. The doctors tell him that he needs to decrease his meds to relieve the stress on his kidneys. He was able to get off of the betablockers but the clonidine which controls his tremors is the med they would like to see him decrease. Thing is...every time they do he goes into violent tremors that last up to 3 hours. Now he is going to the ER every two days when unable to urinate....the sonograms show no urine in his bladder and the only way he produces it is when they give him IV fluids. The nephrologist says it is because his blood pressure fluctuates so much and is too low to filter the urine from his kidneys to the bladder. The meds that keep his blood pressure from going extremely high that cause the tremors and headaches bring it too low causing his kidneys to fail. He is living with a double edged sword..if he decreases the meds to save his kidneys he goes into violent tremors lasting for hours. When the visiting nurse came today and the last few times they had to call an ambulance because his blood pressure was 30/10 and he barely had a pulse. This is happening from just a small decrease in his clonidine....he was taking 6mgs every 4 hours and just decreasing it to 5mgs very 4 hours has caused him to have such low blood pressures.
So.....my brother is in a wheelchair/bed, any time he moves from one to the other he goes into violent tremors which require the clonidine, he isn't able to urinate unless he goes and gets some IV fluids at the ER. His life is having these tremors and spending every other day at the hospital getting IV fluids to produce some urine. He is drinking at least a gallon of fluid a day if not more but still is unable to urinate.
His quality of life is full of pain and our family is desperate for help. Anything you can do to direct us on how to help him would be much appreciated.
I'd like to say that I am terribly sorry your brother has to go through this. It sounds very painful,and I feel for you and your family. You are a very good sister to seek help for your brother.
It sounds as if your brother has severe small fibre neuropathy. Small axonal fibres that are not myelinated or thinly myelinated are responsible for sensory information,pain,tempature, and also autonomic functions such as blood pressure,breathing,sweating, heart rate and urination.
Since he has neuropathy in his limbs,and is unable to walk this at least explains what type of PN he has.
My advice is quite humble, but I will do the best I can for you.
Has he undergone rigorous blood testing to rule out things such as metal poisonings,lyme,other toxicities?
Has he had immunological testing to check for things such as aids,lupus,connective tissue disorders,celiacs and the like?
Infections, hep,syphilis etc.
Nutritional deficiencies such as B12 which can cause major neurological damage
has he has MRI,lumbar puncture?
Has he had nerve conduction studies, EMG?
Do those doctors have any idea as to what caused this? Often with progressive conditions like your brother, if the culprit can be found, the neuropathy can be halted & often reversed?
Does your brother take any supplements such as B12, B-complex?
Also in regards to the clonidine, it's apparently hard on the kidneys but stops tremors. How about replacing that med with anther med for a while to help with tremors? Other possibilities are gabapentin,Frisium,topamax.
I myself am not an expert, but it seems that this neuropathy is progressive and all causes of neurpathy need to be ruled out because a life is at stake here.
I thank you for your response. My brother is back at the hospital (third time this week) after losing consciousness at home from just raising his head in bed. His blood pressure would go up to 210/100 and then plummet to 30/10. This happens from just the little decrease in clonidine from 6mgs every 4 hours to 5mgs to every 4 hours. He was admitted and the doctor is insisting on further decreasing the clonidine, when my brother was informed of this knowing how it would increase the tremors, he became combative pulling the tubes out and trying to get out of bed which had him going into such severe tremors that lasted hours.
My other brother stayed the entire night with him and said that they further decreased the clonidine to 4mgs every 4 hours and the doctor is insisting on getting him off the med saying that the tremors won't kill him but their effect on the kidneys will. It was a along night with the tremors being the worst they had ever been requiring 6 staff members to assist in keepig my brother safe from falling out of bed. My brother described it as almost being like watching somebody going through a detox....the tremors went on all night long and fierce followed by periods of apnea but they have calmed down somewhat and are not as strong and shorter in duration so we are hoping that the doctor's plan to further decrease his meds will help his kidney function and not leave him incapacitated by tremors. Seems that the last update I received showed that the urine ouput has increased and that the tremors are more manageable.
The doctor is thinking of adding florinef and primidone as he tapers off the clonidine.
As far as tests I know an MRI, CAT Scans, lumbar puncture, urine and blood work for catecholamines were done. Not too sure about the blood tests for infectious diseases but since he has had almost everything under the sun done I would assume they did do that. He had the EMG and went down to John Hopkins to have a skin graft test that showed definite nerve damage in the periperhral sense. I have forewarded your post to my other brother to run past the doctor to see if there is anything he feels my brother should further have done.
I think my brother and our family have resolved our selves to the fact that this is something that just isn't going to get better and that we just want to get my brother to the best quality of life even if it means having whatever needs to be done within his home or at least have scheduled appointments for treatments rather than having to go through the ER process each time he runs into trouble. At least this time the doctor called ahead and had the Er ready to admit him. Seems that when my brother cannot urinate or produce urine that an IV will get things going so it would be nice if the doctors would come up with something to take care of that within the home.
Again I thank you for your response and if anybody else knows of anything that may have been overlooked or that could help my brother and his family out I would much appreciate it. We are hoping to see if the doctors will make arrangements for Hospice to take my brother on since his condition is so critical and there doesn't seem to be anything to offer us any hope of his condition improving......it seems to only be progressive with him further and further declining with each trip to the hospital. And it is scary and heartbreaking to see him going through this.
Wow,i feel so awful for you and your brother,that is just an incredible story,really.i am wondering if they actually know for sure just what the real underlying catalyst was that set this all into motion?
it just seems crazy to have this do a sudden onset like it did,was he having ANY actual odd neuro symptoms before this just presented itself?i know i suffer with some pretty severe sympathetic nervous system damage from spinal cord damage and can relate to the symptoms he is having only his about ten times worse than mine are/were.just what exactly showed up on his MRI and where was(which part of his body)it done?did they do a full thorasic and c spine MRI?anywhere that the sympathetic nervous system runs thru or to really needs to be very closely looked into.the sympathetic chain actually runs along the outside of both sides of the spinal column and it has the stellate ganglion within the neck area.but it also crosses along the upper thorasic too and different conditions can affect that nerve and possibly cause alot of the actual symptoms he is having.the SNS and the para are controllers to some degree of all autonomic functions so thats why i am asking all this stuff.
ironically i also have a kidney disease but mine is totally unrelated to my SNS damage.but i know how damaging having that out of control BP can effect the kidneys,and it isn't good.i really DO feel for you all,honestly,this has got to be one hell of a nightmare for all of you mostly your brother.
i really am wondering just what inthe heck could be causing the severe tremors he is having?as a precaution,considering his continuing up and down BPs,it wouldn't hurt to actually have an MRA done on his arterial structure within the brain.i ended up having a brain aneurysm last year that i had no clue was even there.my NS just happened to send me for one because of my kidney disease.but the big swings in his BP could possibly start small bulges in the brain arteries just from the up and down pressure.its just something to be aware of.if there is any weak areas within the arteries up there,well it could happen.i just thought you might need to know this.i hate to have to dump this on you but it is something to be aware of,thats all.i just really don't understand those tremors along with his symptoms that are more indicative of autonomic failure.this one just dosen't seem to fit,you know what i mean?thats why i am wodering just how extensive any testing really has been within his actual brain?does it actually look like real tremors or more spastic type of movement when this happens?i have spasticity,but it is actually stemming from spinal cord injury.like i said,it just seems odd.
hopefully they will somehow be able to find the real underlying problem that started this whole process and be able to actually do something to help him.i wish you all my best and hope things will somehow get better soon.please let us know how he is doing,K?good luck and all my prayers,FB
11-20-01,placement of hardware for failed fusion
9-22-03,removal of cavernous hemangioma that was inside spinal cord. Neuro damage to L hand L leg and R leg.
Goody, it's terrible that your brother has to suffer like this and his doctors don't seem able to help him further. I do hope someone comes up with some treatment or advice for him, but in the meantime I wonder if it would do any good to try some alternative form of medicine? There are so many different types of practitioners out there and maybe someone would at least be able to relieve some of his pain or help resolve some of his symptoms. I'm aware of the perils of charlatanism but perhaps you already know of someone who might be able to help. I'm thinking along the lines of acupuncture, homeopathy, naturopath, maybe even a healer of some kind. Or indeed any other kind of alternative treatment that might make his daily life more bearable. I do hope he gets help from somewhere - no one should have to put up with suffering like that.
Hello Everyone and thank you so much for your support. Everything you have suggested has been done for my brother. I am sorry for the delay in my response, I just returned from a stay with my brother in order to give my SIL a break. You see, the nurses have never seen tremors like my brother has and they are quite intimidated in terms of helping him through it. It is horrific....his body thrashes like a fish does out of water and now it is happening even when he doesn't even move. It is heartbreaking to see him go through these tremors and now he is having periods of unconsciousness throughout them. Two nights I was there they lasted over 2 hours in duration. He is pretty much alert during these episodes and is able to nod his head. I rode it through with him sharing happy thoughts that will relax him and take the fear away. I tell him that I am there and that his vital signs are stable but we are unable to get a BP because of the amount of movement going on in his extremities. The medical staff gives him every med that is ordered and then some and it still doesn't help. He is on clonidine .4mgs, morphine 2mgs every 4 hours, Darvocet every 4 hours, Inderal SR 4 times a day, Ativan every 6 hours and some other meds that I have forgotten to memory. Anytime he is moved he goes into these tremors lasting about 40 minutes to an hour just from moving him off his back to put cream on it to prevent any breakdown/bedsores. And then the worst seem to come when he gets tremendous headaches and pain in his chest which cannot be relieved by the meds and those are the tremors that last hours leading to more and more periods of unconsciousness. My brother is afraid because he uses everything he's got to come out of them and is afraid that he is slipping into a coma and one time will not come out. He says he hears everything we are saying except some of the times he goes unconscious and I know he does because he is able to nod when I ask him questions.
Anyway, they transferred him to John Hopkins and the doctors there want to take him off of most of his meds blood pressure & pain meds and my brother is refusing stating that if they do he will go into a coma and die. He is so afraid and I know it is legitamate fear because I have seen his tremors worsten even with the slightest decrease in his clonidine. They also feel that he has had a stroke because on side of his body is weaker than the other although for me and others this would be difficult to see since all of his extremities have weakened due to the neuropathy.
My brother saw the cardiologist and has yet to see the neurologist. The cardiologist says that if he refuses their recommendations to discontinue his meds then they will have to discharge him. They sat him up to get an orthostatic BP and the room was filled with doctors and medical staff there to observe the tremors my brother exhibits....my SIL said the room was packed with observers and she almost wanted to hand out popcorn!! This is exactly what my brother doesn't want....he is afraid he will be a guinea pig and he just wants to live out the rest of his life in peace. But how when he experiences such pain and suffering despite being on all the meds. I talked with my brother and told him that the meds he was on weren't working because he is just getting worse. The doctors at the other hospital wanted to get him off the meds as well because they feel that his kidneys cannot handle them and also they are not working and perhaps making things worse. When they decreased the clonidine by .1mg from .5mg to .4mgs he worstened for a day and then didn't have any tremors. Then they went from .4mgs to .3mgs and he had a night of long tremors but never gave them a chance to see if he stabilized the night after that and put it back up. I am trying to tell my brother that perhaps he is going through a detox since he has been on the clonidine for so long and on the highest dose any doctor has ever seen. So I am trying to convince my brother to give it a try since he is in a very good hospital where he will be monitored closely. If they discharge him I doubt very much if they will provide him scripts for his meds anyway leaving him in the same situation only without medical monitoring.
He is not taking any phone calls according to my SIL. Today they want to do an EEG and sit him up and watch him through one of the tremors to rule out seizure activity. After that I do not know whether they are discharging him home or whether he has agreed to their suggestions of going off the meds. I think that they want to take him off the meds and see what symptoms need to be treated and start all over again. I think they call that a "med wash" but with my brother's condition will he be able to survive that????
Thanks for the encouragement and suggestions. All the tests have been done and the doctors feel that my brother has the most complicated and worse case of Autonomic Failure that they have ever seen. We need a miracle and we need it soon.
Hi Goody! I haven't got to "talk" to ya in a while, but I have been following your posts on the bp board. I am sorry that your brother is going through this. I can understand his fear of being taken off of all the meds. It doesn't sound like they are doing him much good now, but I would probably feel the same way if I were in his shoes! It seems to me like it would be pretty risky to take him off of everything now when his body is so weak. Surely they won't discharge him! I'm praying that he gets the miracle that he needs to get through this! Stay strong!
Hi, Cristie Thanks for popping in. I am heading down to John Hopkins tomorrow to give my SIL a hand. My brother is not too happy with what the doctors are doing. They wanted to get some testing done that has already been done....he seems to think that he is a guinea pig there so my SIL convinced the doctors there to accept copies of the tests which she will be getting sometime over the next few days.
We found out today that the clonidine that my brother is on was never perscribed at the high dose he had been taking. Seems that whenever he got to the point of having bad headaches or tremors he would take some extra so the doctors feel that he has developed a psychological dependency on it that has him going through some form of detox. They say that you could go into hypertensive crisis if you are taken off of it too quickly and I think that is what is happening with my brother. Also....they said that if you take it too long or at too high a dosage that you get a rebound effect which is an opposite effect. So it sorta makes sense....my brother's condition seemed to worsten in terms of the tremors and headaches so perhaps once they get him off the med things will improve. That is what I am hoping for.
Thanks for checking in. I will give you all more of an update once I get back from seeing him.
the thing is,there are other meds besides clonidine that could possibly work better for the tremors he is having.i still don't really understand how and what could possibly be triggering this.it really IS rather crazy since these are obviously not siezures.could this just be very severe spasticity?some peoples spasticiy,believe me,can get hidious at times.botox could be a possibility there,depending on just where the actual tremors are happening..just a thought.i know from my SCI,i will get horrid spasticiy that can appear to look like actual tremors or even a siezure.it all depends on the level of the spasticity and why or what is actually triggering it.this would be a motor nerve issue.can you describe just exactly what the tremors appear like?what exactly happens during these episodes,what shakes?
i also think,considering the kidneys and possible liver affectation,he really does need to drop the darvocet completely.quite honestly,this is about the lowest dose of any type of narcotic possible to give any patient.it would be totally and completely useless to someone in his condition anyway.trying something like oxy IR,which is just percocet without the tylenol,its just pure 5mg oxycodone.a much much better choice for many reasons.taking morphine and dropping WAY down to the darvocet really is not even worth his time and the risks of the 650mgs of tylenol he is getting with every dose.anything you can do right now to limit the amount of actual tyleonl,ESPESCIALLY with kidney issues,well it really doesn't take a rocket scientist to figure this one out,ya know?vicodin would be good too,if they used norco since this has only 325mgs of tylenol,but i still really think the oxy IR for his regular break thru med just makes sooo much sense for his needs specifically.honestly,that darvocet is doing absolutely nothing for him right now and is compromising the kidney function he still has left.tho it is actually metabolized in the liver not the kidneys,the kidneys are still being affected when the by products are filtered thru them before they are passed along outside the body,trust me on this one.i actually have a kidney disease and a wonderful nephrologist who helps me to maintain my kidney function to the optimum level right now,and he told me to stay the heck away from tylenol products.my pain clinic will not even Rx any tylenol products at all to anyone for any reason,just because the cumulative affect that tylenol can have on both the liver and the kidneys.the thing is,when there are other meds that quite frankly would do a much more effective job for his type of pain,and getting totally rid of any tylenol,well,you cannot beat that.he really does need to talk to his doc about trying the oxy IR.it works pretty good for some of my breakthru pain.
i do wish i could be of much more help to you.i really feel so badly for what your brother and your family are currently going thru,really.please keep us posted on how he is doing.i really DO want to know,K? hang in there,FB
11-20-01,placement of hardware for failed fusion
9-22-03,removal of cavernous hemangioma that was inside spinal cord. Neuro damage to L hand L leg and R leg.
Hi, Feelbad Thanks for your support and concern. Right now the doctors have taken my brother off of both the morphine and Darvocet....he is on Toradol by mouth which he claims does nothing for him. I think the plan is to get him off as many of the meds as possible and try to start all over. I believe that they call this a "med wash" almost as if to cleanse his system due to a possible toxicity to his system. That is the best I can explain it.
The way I see it, things could not get much worse. I have witnessed my brother's tremors and while they do have a ture physiological component that is out of his control they also seem to have a psychological component with anxiety although my brother will deny this. He goes into the tremors which start with twitching of the head and upper extremities. And eventually get into the violent type that have him flip flopping on the mattress sometimes with his entire body coming off of the mattress. It is quite scary to witness this. As I talk him through he calms down a bit and I am able to get nods to questions and the sort. He then goes into the mild tremors and then into the violent ones and after a few cycles of this some periods of unconciousness occur as if his body shuts down because it has had enough. Then he gasps for air and is in a mild tremor state in which I can talk to him and elicit a response to my questions. When he hears something from me or the medical personell that he does quite like he will then pound the mattress with his hands and legs kicking almost as if having a temper tantrum. For example, one of the nurses asked me to leave the room to see if less stimuli would bring him out of it. When I left he started having this fit like kicking and pounding.....in other words it seems that fear and anxiety worsten the tremors. I do believe that he is in severe pain when this happens for he has pointed to his head and his chest and his abdomen and when asked confirms bad chest pain, headache and nausea. When I look up detox these are some of the symptoms so what the doctors propose may very well be going on. The only thing is when I looked up Clonidine withdrawal it says that one must decrease the dose slowly and introduce other meds in order to prevent hypertensive crisis. Whenever they went to decrease his clonidine in the other hospital they have not done this so I am going to bring this up with his doctors when I arrive today.
They intend to do an EEG in order to rule out any seizure activity. I am almost certain from watching what my brother is going through that he is not having a seizure but it is good that they want to rule it out.
The doctors are well aware of my brother's risk of renal failure which is part of the reason that they want to get him off the Clonidine since it could be nephrotoxic.
Well I have to get ready for my trip. I will update you in a few days when I return. Thanks again for all the support.
Well here I am with an update. The doctors continue to decrease the Clonidine while his blood pressure rises to high levels and his pain increases. The cardiologist following him who is also the admitting physician observed my brother's tremors and has told my brother that he believes that he has what is called Conversion Disorder, a condition in which physical symptoms occur due to stress. It is an unconscious phenomenon but my brother wants no part of accepting this diagnosis and to be honest I find it difficult to believe myself.
I do believe that there are definite psychological components which worsten the episodes that I see but I am almost certain that there is also a physical condition warranting this. How can somebody just stop walking and have such tremors and pain with blood pressures fluctuating so drastically??? And what about is inabiity to urinate or produce urine and the high creatinine levels showing signs of renal failure??? Anyway, the cardiologist says that he will get my brother set with meds to help as much as possible to manage his blood pressure and that he feels that he needs to follow up with Neurology and Psychiatry to work on psychotherapy to improve his condition. I believe (although I am not quite sure) that they still believe he has Autonomic Failure, however, the doctor seems to believe that what is happening now seems to be more related to Conversion Disorder.
What are your thoughts on this??? Does anybody know anything about this disorder and if it is possible that this is what is happening with my brother??? Of course my brother is not willing to take the doctor's diagnosis nor his advice and if he doesn't will be discharged home sometime within the next few days. I cannot see how he possibly can be managed at home when he cannot even move or sit up without going into these severe tremors.
I am trying to convince my brother to take the advice so that he can stay at John Hopkins and get the help that he needs but he refuses to accept it.