Pure Autonomic Failure

[Purplegoth]

I posted on the site a few weeks ago but it was a very short post so I'm writing to expand on that.

My partner has PAF (Pure Autonomic Failure) also known as Bradbury-Eggleston Syndrome, ..it is very rare and only 180 other cases in the UK at present so there are no specific support groups available, we were therefore advised to join the MSA Trust as PAF falls into the same neurological group of diseases as MSA, and Parkinson's .He is being treated by UCLH Neurology Dept. at this time

In particular PAF causes severe postural hypotension and other symptoms similar to those of MSA and Parkinson's ( see attachment ) , I have recorded his BP as low as 53/40 on more than one occasion and he's suffered blackouts resulting in serious falls and concussion as a result, this is a constant problem even with the considerable medication he takes.

I understand that the Trust cannot offer any medical advice but I would welcome and hints or tips that may be of assistance from community

Thank you

[FredaE]

oh dear iam sure you are welome but you must feel so isolated

[Purplegoth in reply to FredaE]

Thank you for your reply. Yes it is very isolating and not knowing anyone else with PAF because it is so rare makes it that much harder.

[GreenJo]

Hi - my husband TC has MSA and when he was still mobile he had same problem with his BP. (he's now bedbound) I would take his BP before he moved and it would be normal - then just plummet and he'd start to lose consciousness. Or he would make it to loo and it would plummet - it was very scary - I found salt helpful in short term. I would rub salt on his gums - I used oil from jar of olives with added salt. Obviously not if he'd lost consciousness! This would usually bring his BP up enough to get him in his riser recliner. By that stage he was only walking to loo. I realise this might not be helpful if your partner's more mobile. Best of luck

[Purplegoth in reply to GreenJo]

Hello,Thank you so much for your reply. My partner is more mobile than your husband......at the moment.

UCLH has prescribed salt tablets for him..600mg before every meal so quite a dose. Not sure it's done alot of good other than making him pee more and he thinks it is disturbing his sleep unless that's a more progressive sign his PAF is getting worse. He is on a tablet he takes once at night to stop the vivid dreams and thrashing about.

[DeeMilo25 in reply to GreenJo]

My husband had the same problem terrible drop in bp, so bad the lack of oxygen caused him to start fitting , he is now on epilepsy meds and couldn’t have extra salt due to being stage 1 kidney failure , he is now unable to walk or stand at all and needs a Sara steady used to transfer from bed to chair, he had a supra pubic catheter in place now as well and is having carers 4 times a day times 2 carers, I really don’t understand why or how he has deteriorated so quickly and I’m worried it’s the end for us, he can just about feed himself if I cut it up for him but can only use one arm now, he seems as though he is wasting away, is this just me or do other people see this in their partners? I’m also worried he may get blood clots from lack of activity.

[Purplegoth in reply to DeeMilo25]

Thank you for your message. My partner, I'm sorry to say had blood clots a few years ago due to him being inactive. He is no where near bedridden or needing walking aids but any excertion tires him out. The hospital wanted him to have a MRI scan to rule out atypical parkinsons but because he has a pace maker fitted and the leads are a different manufacturer to the unit they say he is not compatible for a MRI. Even though his pacemaker will only kick in when his heart rate drops below a certain rate. Trouble is because PAF is an invisible disease he is totally fed up of people saying how well he looks.

We are getting married in October, only 8 people going but I'm still worried the day will be too much for him. I so want him to enjoy it.

[Bizzyissy]

Must be so frustrating as well as isolating. What other symptoms does your partner have? I’m still waiting to get a diagnosis. I have been given FND diagnosis but still doesn’t explain postural hypotension or hyperflexia so very interesting to read!

[Purplegoth in reply to Bizzyissy]

Hi there,Thank you for getting in touch.

My partners postural hypotension is his main symptom, and is impacting every day living now. Any exercise seems to make his low blood pressure worse. His heart rate doesn't seem to rise.

His digestion is not good, he gets extremely tired. He can not control his temperature. One of his tests was to be put in an " oven" at 45 degrees and after 40 minutes he wasn't sweating at all.

We started this journey as his sleep was very disturbed and he ended up clearing his bedside a few times. Basically everything that you and I do subconsciously eg blood pressure, temperature, digestion etc is failing. He is prone to blackouts.

Oddly enough his reflexes are hyperheightened

[Bizzyissy in reply to Purplegoth]

Thanks so much. Interesting reading. I would love a definitive diagnosis. Is it? Isn’t it really puts me in no man’s land. Been on Flurocortisone for blood pressure but still dropping and is low , heart rate is low50’s. I have nerve pain, and worsening bladder and bowel issues. Movement disorder hasn’t been ruled out… How long has your partner been ill for?

[Purplegoth in reply to Bizzyissy]

My partner is on Flurocortisone, Midodrene, an injection called Octroide and a short while ago was prescribed salt tablets. These are all to help bring his blood pressure up. He still gets readings of 50 over 39 even with the medication. ........ We think the very start of this was possibly 10/12 years ago. It started very very slowly, eg bending down and tying shoe laces and when straightening back up going dizzy like alot if people do.

[Bizzyissy]

Hi thank you

You will get lots of support from here. Thanks for answering my questions.

Issy x

[Purplegoth in reply to Bizzyissy]

Hi Issy,I really hope we find support here as it is isolating and getting a little difficult.

Thank you,

[SaltyFlorist]

This is something I’ve been living with for 10 years.

I use a bit more salt than your partner. I take it with each midodrine and chase it with 1/2 litre of water. I must stay hydrated. It helps with all of my symptoms.

If I lag behind on fluid I’m finished for the day. I don’t recover. Fatigue!

I need to take midodrine every 3 hours. There is a large drop off for me within 10 minutes of a missed dose.

I elevate my feet every hour or two. I avoid heat. I don’t eat large meals. I don’t eat out because I can’t have my feet down for that long a period.

I’ve also had clots. It’s important to keep feet moving. Work on strengthening lower legs. It helps pump blood through those damaged veins.

I use waist high compression wear which is difficult but the results are worth it for me.

I find it helpful to keep the same schedule and though difficult, get enough sleep.

It’s important to learn what pre syncope feels like so falls are avoided.

Best Wishes

[Purplegoth in reply to SaltyFlorist]

Hi there, Thank you for your message.

May I ask is it Pure Autonomic Failure that you have?

KR

[SaltyFlorist]

Not sure actually. Initially thought to be PAF last few years developing some potential MSA symptoms. I don’t worry as much about the title as how to live with it. I got most of my initial help from POTs patients.

[Purplegoth in reply to SaltyFlorist]

Ah ok, thank you never heard of POTs. As there only 180 people inthe country with PAF its difficult to find others in the same situation. Has POTs got a UK support group

[SaltyFlorist]

Not sure. I learned from reading blogs which is old school now. The rest was trial and error.

If you can, keep a small diary about daily symptoms you may find patterns which you didn’t initially identify. I kept track of food, weather, activities etc.

Look for reputable sites with sources about dysautonomia. You’ll find a wealth of information.

[Purplegoth in reply to SaltyFlorist]

Thank you very much for your help. I will definitely delve into things more.

[Purplegoth in reply to SaltyFlorist]

Just a quick question, have you been prescribed a drug called Octreotide? It's taken in injection form. Not sure a GP can prescribe it. My partner is under UCLH and they prescribed it. It has certainly helped as it stops the blood pooling in your stomach after eating thereby increasing your blood pressure.

[SaltyFlorist]

Thank you! I have not heard of this. I have an appointment next month, will inquire.

[Purplegoth]

I would be interested to know how you get on. Hope everything goes well.

[SarahHes]

Hello Purplegoth

I’m late to this conversation, but wanted to see how your husband is doing. My dad’s diagnosis was originally ‘primary autonomic failure’, but that has now progressed to ‘Parkinson’s with autonomic dysfunction’. There may be another diagnosis further down the line, as I think some of his symptoms are atypical. He has the mobility problems associated with PD, but the problems with his blood pressure and digestion trouble him far more, and have been relatively long term issues.

I’d love to find some way to help alleviate the absolutely crushing fatigue that has left him reluctant to venture much beyond their home and garden.

I hope all is well with you.

[Purplegoth in reply to SarahHes]

Hi SarahHes,

Thank you so much for your message. I'm so sorry to hear about your dad.

They wanted to test my husband for atypical parkinsons by way of an MRI scan but he has a pacemaker fitted and apparently although it's one of the lastest PM's it's "not compatible with an MRI scan "

So we are absolutely stuck at the moment. All the other tests still point to PAF but he is definitely getting worse. His blood pressure is soooo low and his digestion is bad. But what absolutely pricked my ears up was the extreme fatigue that your dad has. My husband has this now and it is really getting to him now.

I'm am very worried about him but we seem to be stuck now. UCLH are really very good but I'm worried we are not getting anywhere.

Xx

[SarahHes]

I feel for you both. The fatigue is definitely the worst of dad’s symptoms - he has the classic slowness of movement that comes with Parkinson’s, but I think he could live with that without too much trouble were it not for the utter exhaustion and weakness he feels so much of the time.

Dad was diagnosed via a DAT scan, not an MRI - perhaps you could request one of those as I think they may be possible with a pacemaker…. ? It wouldn’t give the comprehensive image of the brain you get from an MRI, but would show if your husband has any form of Parkinsonism, which can be linked to PAF.

Good luck to you both. Xx