Advice or information about bulber

[Frosty247]

Hello there, this might be a long post...

I'm sorry to bother you on here but I have been having some worrying issues and I wondered if you could give me your opinion, for the other members feel free to also chime in.

First of all, i am female age 42. I have a rare neurological condition which is called non length dependent small fiber neuropathy which basically causes pain all over the body similar to what diabetics get but they normally get in their feet only.

I have been under a neurologist for the last 6 years from National Hospital Of Neurology. He has put me on something called 100k genomes project as my sister has the same problem. We haven't got to the bottom of the underlying cause but it is a suspected inflammatory neuropathy as symptoms are all over the body including face, tongue and throat.

Ok, so a bit more info...with this condition causes me numbness and tingling and lots of twitches. I have had odd feelings and pain in my tongue and throat on and off for the past 5 years, always put it down to the condition. I also took Lyrica but came off this is 2018 as didn't do anything..

Anyway..so over the last few years i have noticed that my speech isn't normal, i was getting tongue tied alot and not being able to get my words out, so put it down to the Lyrica, came off Lyrica in 2018 summertime. I've gradually noticed other weird stuff with my speech, basically its pronouncing words, i seem to have problems with words with L and R in them.

Then Christmas of 2018, i noticed that i was getting what felt like a strained feeling in my throat, like when you have been throwing up, and strains the throat, it then proceeded with any aching neck and ice cold feelings in the throat, gradually this went away and i saw my neuro in 2019 Feb time who said, i have no idea what that is.

. So, Never really paid much attention to it...then last May 2019 i noticed while on holiday, i was drinking from a bottle and half way through i felt like there was a delay with the swallow, I just ignored it. However, this keeps happening and i also had problem where food also got stuck in my throat. I get funny gurgling noises everytime i drink like i have swallowed too much air.

The swallowing problems have continued since May last year and the problem with my speech pronouncing words is getting slightly worse.

I saw my neurologist in January. He felt that i didn't have anything wrong with my speech but the problem is the issues are subtle and do not go noticed straight away. I have also started biting my tongue alot, which is currently numb. He didn't think that my condition would cause these symptoms.

I understand Bulber ALS can come on quite quickly, but i am wondering if i have a slow variant of it, if that exists, or if this is something to do with my other condition as the symptoms came along at the same time as the sensory ones relating to my other condition.

I am now waiting to have an endoscopy and a swallow study but being with everything on hold its been cancelled. My neurologist wants me to have some electrical tests if they find anything.

My anxiety is sky high at the moment.

I wondered if anybody had any advice or comments?

Many thanks

[Ellie]

Hi,

Sorry you have a rare neurological condition.

The symptoms you list - being tongue tied, having difficulty pronouncing the letters L & R, strained feeling in the throat, aching neck, ice cold feelings in the throat, delay with the swallow, funny gurgling noises every time you drink - is not suggestive of bulbar onset ALS/MND.

Your neurologist at the NHNN, one of the top hospitals in the UK, said he noticed nothing wrong with your speech, so you really should be reassured by that. He’ll keep an eye on you I’m sure, over your scheduled appointments for your condition.

I wish you all the best.

Love Ellie.

[MNDConnect]

Hello

I'm sorry to hear about your rare neurological condition and the concerns that you have.

With regards to motor neurone disease (MND), none of your symptoms would be typical.

When someone has speech problems with Progressive Bulbar Palsy, they are permanent. They don't come and go and they wouldn't usually cause difficulties with pronouncing particular letters.

Bulbar Palsy is a particularly progressive form of MND and it would be very unlikely that after several years, someone with bulbar palsy wouldn't have far more advanced symptoms. Several years after speech problems have started, it is unlikely that someone with bulbar palsy would have intelligible speech or at the very least would have very affected speech.

This would also be the same with swallowing problems. In bulbar palsy, swallowing problems are not generally something that someone would have for several years without them becoming very significant symptoms. The way in which you describe your swallowing problems is also not what you would expect from someone with bulbar palsy.

I do hope that this helps to reassure you.

Rachel
MND Connect Adviser

[Frosty247]

Thank you Ellie for replying.

Symptoms are all very strange and worrying. I just dont understand what else could be causing them. My swallowing symptoms have remained the same for nearly a year, but speech is getting worse but subtle so not too noticeable.

I dont see my neurologist now until January 2021, I see him at my local hospital, but he works at National hospital for Neurology also.

Thankyou again for replying.

[Frosty247]

Dear Rachel,

Thankyou for replying. The swallowing issues I have haven't gone on for years, the actual swallowing problem started in May last year, it seems to have remained stable up until now. It is still very concerning as it has not resolved.

The speech issues are confusing as I explained I was on medication which made it difficult to speak and I came off the medication in 2018, However the speech issues that I have found concerning the most has been in the last 14 months with being unable to pronounce some words, then having to repeat the word so I say it again properly.

I telephoned your team last week as I had sort of been left in limbo with Corona and everything being cancelled. I managed to speak to your team, not sure who I spoke to, but I managed to have a near normal conversation without the speech issue, it just depends on what words I am trying to pronounce.

I can move my tongue quickly around my mouth, but i am having issues with numbness and tingling on my tongue and lots of biting. I'm not sure if this is related to my underlying rare neuro condition.

Are there any more slower variations of Bulber onset? I've read about isolated bulber palsey, I'm wondering if I might have this variation, does this creep on slowly or is this quick progression? Alsi with bulber onset, how quickly do limbs become involved?

Sorry for all the questions, my anxiety is still sky high,

Kind Regards

[MNDConnect]

Hi Frosty

It was me that you spoke to on the phone last week. We had quite a lengthy conversation and you had no apparent speech problems. Speech problems in MND just don't tend to appear in the way that you describe. I speak to many people every day with speech problems and even very mild speech changes can often be picked up in our conversation. Sometimes we even notice the speech changes before the person themselves seems to have noticed them because speech changes in MND appear in quite a particular way.

There isn't a slower form of bulbar palsy. When someone has pure bulbar palsy and no affected limbs, this tends to progress quite rapidly and is unlikely to appear over several years.

I know that once you are concerned about a particular disease, especially something like MND, it is very hard not to keep going back to the possibility of it being that disease. But please know that nothing that you said in your conversation with me last week would suggest that you have MND.

The National is one of our care centres and is a centre of excellence in MND. They have some of the world's top specialists in MND working there. Your neurologist not seeing you again until 2021 is a positive sign. If they were concerned, they would be seeing you much sooner.

Best Wishes

Rachel
MND Connect Adviser

[Frosty247]

Hi Rachel, thanks again for taking the time to respond. I feel a bit of a pain keep asking questions it's really kind of you to be so patient and understanding.

My gut feeling was always this was something to do with my undiagnised Neuro condition as the issues happened at the same time as the sensory ones, it has only been since I saw my neuro that I have thought it could be something more sinister as he felt they were not connected.

I tried to explain to him that I was having issues with my speech but he kept dismissing me, so I couldnt ask him a about the pronouncing words issue.

So even the rare people who have had bulber for a longer period of time still would have had more significant issues with speech and swallowing after 1 year of symptoms? In your experience you haven't come across somebody presenting with my issues?

I wobt post any more after this, I'm sure you are very busy so I will give it a rest😀

[MNDConnect]

Hi Frosty

I think it might be best if you contact us directly and we can answer your questions and address your concerns. Obviously the forum has many members who are living with MND and is often used by people who are newly diagnosed and their family and friends. I'm conscious that our conversation could be quite worrying and upsetting for people to read so I do think it would be better if we discussed your concerns privately.

Please do either give us another call on 0808 802 6262 or email us at [email protected]

Best Wishes

Rachel
MND Connect Adviser

[Frosty247]

Thankyou Rachel

[matthew55]

Hello Frosty, you have described exactly what I am experiencing and I am lucky to have been diagnosed with Bulbar onset motor neurone disease.Truth is always scary but only with it can you gain acceptance. Be strong and be prepared. Best wishes Matthew

[Hayls]

Hello Matthew, I was bulbar onset too, great advice about acceptance and staying strong, hydration and nutrition is key, I was advised to get a PEG or RIG fitted sooner rather than later, so glad I listened, having all fluid through my RIG and very lucky to avoided any aspersion causing nasty chest infections, have you been offered a feeding tube yet, if not, mention it when a member of your team get in touch, it’s made me more comfortable and less anxious, keep your spirits up as best as you can

[matthew55]

Hello Hayls, On the subject of nutrition I have found oakhouse foods who do small bite, soft meals and although pricey it is wonderful to eat without pain and anxiety. Oh and nurishment milkshakes are brilliant even with the trade-off for yet more saliva!
Love Matthew

[Hayls]

Hello Hayls, On the subject of nutrition I have found oakhouse foods who do small bite, soft meals and although pricey it is wonderful to eat without pain and anxiety. Oh and nurishment milkshakes are brilliant even with the trade-off for yet more saliva!
Love Matthew

Oooo I’ll check them out, thanks for the tip

[matthew55]

You are very welcome 🤗