Autonomic Failure ~ My Brother's Decline

[goody2shuz]

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.

Thanks ~ Goody

[Teacupforme]

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.

God be with you-

[goody2shuz]

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.

Thanks for the information and support ~ Goody

[feelbad]

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

[Isobella]

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.