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Sheen

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Earlier I had posted that my mother had either PBP or Primary Progressive Aphasia, although she still does not have a clear diagnosis due to negative EMGs, I feel she has PBP. After reading everyone else's signs and symptoms on this forum I am convinced she has PBP. It started in May 08 with slowing of speech to not being able to understand her at all. Although her swallow tests have been negative, she struggles to eat food, she is constantly choking on her saliva. I communicate with her through pen and paper. I looked at the Proloquo2go but I feel that since my mother is not technologically savvy she will get frustrated by it. Pen and paper works for us. I even tried to teach her sign language alphabets but she refused to learn. My biggest concern is her choking on her saliva and tomorrow I am going to an ENT specialist with her and getting a Botox injection in her salivary glands. Have any of you had this procedure done and if so what kind of results have you had? Also does anyone else have the same symptoms as my mother? I feel really helpless watching her go through this and don't know what I can do to help her. Any advice will be appreciated.

Thanks,
Sheena
 

rachelg

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Hello Sheena

I am so sorry to hear about your mum and what you are going through.

My husband was diagnosed with PBP in May 2009. He too started with slurred speech this was in September 2008 - his speech is very poor now and only close family can understand him. He too uses pen and paper and has become very descriptive with his hands.

He chokes regularly on saliva. He can't clear the saliva from his throat and therefore it starts a cough choke cycle.

We live in the UK so things may be a little different here but he takes doses of something called Carbosisteine syrup which thins down his saliva making it a little easier to swallow. He also has a PEG tube fitted as he is unable to eat very much and the tube comes in handy for all of his medications. He also take Amilytriptiline to dry the saliva up a bit. Mark is unable to drink liquids as they cause choking so we have special sponges on sticks which we soak in drink and then rest them in his mouth for a bit of flavour.

The Motor Neurone association here in the uk are going to supply Mark with a laptop so that he can communicate as he is not happy with the Lightwriter machine - I am not sure if in the USA the same sort of facilities are available.

Please ask any questions you like and I would be more than happy to answer if I can.

RACHEL
 

Sheen

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Hi Rachel:
Thank you for responding. My mother keeps complaining about the mucous in her throat. Do the doctors over there say that it is because of overproduction or is it because the baseline swallowing is affected? With the peg there does your husband continue to choke on his saliva? My mother's swallowing tests come out normal but she continues to choke so I don't know what to think.

Thanks again,
Sheena
 

rachelg

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Hi Sheena

Sorry for not replying sooner.

No extra saliva is produced its just that Mark can't swallow it so it hangs around in his mouth and causes drooling. The especially thick saliva is the hardest to get rid of but the syrup does thin it down. Mark has just started on Scopolamine (hyoscine) patches which are to help dry the saliva up - he has only been on them two days but he does feel they are drying it up a bit already - the patches are at the moment making him feel a little blurry but we are hopeful that that will settle down in a few days.

Yes Mark does continue to choke on his saliva but the beauty of the PEG is that he no longer chokes on his medication which he found would catch in the back of his throat now he can put all drugs through the PEG tube.

We also have a suction machine which Mark can use to suck up the saliva and his physio will be coming soon to teach us how to do deeper suctioning to try and clear the sticky spit from the back of his throat.

Saliva and coughing are Mark's worst problems and his health team are doing their best to get this under control but it is a bit of trial and error as what works for one person may not be suitable for another.

How did you get on at the ENT specialists - did they offer some help?

As far as food - Mark eats only very little by mouth. At first it wasn't swallowing that made things difficult it was the fact that he can't move his tongue very well to move the food around in his mouth to get it ready to swallow - also chewing food became exhausting the problems with actually swallowing came a bit later. When Mark does eat now it is a very slow process which takes 100% concentration - he sometimes has to use his fingers to push the food in to the right position. Experimenting with different textures has taught us what foods are easier to eat. Maybe you could try different textures with your mum to find out which foods take less effort to move around her mouth. He finds things likes Angel Delight and custard very easy to eat and things like cauliflower cheese (cauliflower cooked thoroughly) and weirdly french fries are manageable. Lots of gravy and sauces to moisten food are good.

I hope this helps.

RACHEL
 

Sheen

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Thanks for the Reply Rachel. I took my mom to The ENT people and they botoxed her salivary glands . So far it has not helped at all but they said that it would take about a week or more for the saliva to start drying up. In the meantime we have gotten her a suction pump. She can still eat but it is a slow process that requires concentration. We have gotten her Ensure drinks and puddings that are nutritional supplements and can replace a meal incase she does not want to make the effort of chewing. Your husbands symptoms are the same as my mothers. I tried to find Carbosteine syrup here but they don't have it here. I gave my mother mucinex but it's a big pill and she has difficulty in swallowing that. I have also put her on Lithium Orotate which is a salt of lithium. It's a supplement for neuro protection. Her speech is so bad that we can hardly understand her at all. Does Mark have a blank look on his face? I sometimes feel that my mother has trouble understanding and communicating what she means by way of writing also, does Mark have that?

Thanks,
Sheena
 

rachelg

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Hi Sheena

I hope the botox injection works - I will be interested to know as the patches Mark was using don't suit him so we will be considering different ways to dry the saliva up a bit.

Mark doesn't have a blank look infact as his speech has almost gone his eyes have become very expressive and he uses his hands to make gestures to help us understand him.

The communication side is very frustrating for Mark and sometimes he does get a bit annoyed when we don't grasp what he is trying to say - he writes a lot down along with trying to speak and hand gestures.

If your mum doesn't have a speech therapist it would be really worth trying to get to see one as ours has many suggestions regarding communication. There are various devizes plus I believe some people use speech boards and communication cards.

you mentioned Primary Progressive Aphasia. After little reading I see that sometimes the person may have difficulty understanding what people are saying and they can struggle to communicate what they want by writing. Below is a link which may help.

http://www.pdsg.org.uk/Factsheets/PPAphasia.htm

As far as the mucinex - try and see if its available in syrup form - most of Marks meds are syrup as his swallow function is weak and pills start to dissolve in his mouth before he stands a chance of swallowing them.

Keep us informed and I will be thinking of you.

RACH
 

Sheen

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medford
Hi Rachel:
Thanks for the post. The botox has not started working yet, the doctor said to give it more time. In the meantime we got my mother a suction machine. I don't think my mother has PPA as she reads and watches TV and understands everything. Her grammar in writing as become really bad. It could be because she is bilingual and has started translating into English. I think the blank look comes from the facial nerve weakness. Her sign language is terrible.

We have an ALS support person coming to visit next week, lets see what kid of assisstive technology she advises for my mother. Will keep you posted.

Sheena.
 

rachelg

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Hey Sheena

Just wondered if the Botox had worked. My Mark is drooling quite a bit so thought I would ask you.

I hope you have had a good weekend and that your mum is doing ok.

RACHEL
 

Sheen

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Hi rachel:
The botox worked during the day but my mother complains of saliva at night. I think that the doctor gave her a very small dose and if she had given her more as she had planned it would have worked better. I would definitely consider it for your husband.

Thanks,
Sheena
 

mike b

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Nov 2, 2008
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NM
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Rio Rancho
Sheena,
My wife Lynn has PBP since May 07 and really noticed the effects 18 months ago. She experienced big problems initially with mucous building up in the back of her throat. It was causing gagging and extreme discomfort. The doctor prescribed a suction machine with a soft wand and she used this to suction out her throat. It really helped. Then she started having extra saliva. The doc prescribed amytriptolene but this made her very drowsy. She tried mucinex and this sort of worked. She also tried the scopalomine patches but after a while they would stick on her neck. She had botox injections but they did no good at all. Now she just carries a cloth with her at all times and wipes her inside of mouth and chin frequently.

She ate everything by mouth up until last week when her video swallow showed her aspirating food. She has since switched to a purely liquid diet. She can't stand Ensure because it makes her nauseous. So she makes her own smoothies with avocado, bananas, goats milk, sweet potatoes, apples, and other organic goodies. She loves them and while they take a fair amount of time through the PEG, she feels good about it.

Her speech is very slurry and she uses her Dynavox speech machine and white board when she can't be understood. We saw a speech therapist at Mayo and they actually discouraged us from going to therapy because it was fatiguing of her speech muscles. They encouraged her to preserve her strength rather than trying to strain her larynx and vocal cords.

No easy answers. Lynn really fought having and using the PEG. She saw it as a sign of defeat. Now she accepts it but grudgingly. We are having a larger diameter tube put in next week. This should make the eating speedier.

Hope that helps.

Mike
 

Sheen

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Thank you Mike. I was wondering if your wife tried Glycopyrulate ( Robinul) for drying up the saliva? I am getting desperate as I can't bear to see my mother struggling with the saliva issue. Botox did not work. She is not drooling as she is very good about cleaning her mouth but it keeps her awake at night. She can't sleep because of it. She is still eating though and I make her drink ensure or boost after each meal. I feel she has started exhibiting mild signs of dementia associated with ALS . She has a blank look as if she does not understand what I am saying at times but apparently she does. Her communication via writing has deteriorated alot. Her sentences have grammatical errors, sentence construction is very poor so I don't think any of the speech devices are going to help her as I feel she does not have the ability to learn. I am trying to teachher the alphabet in sign language so she can spell things out to us as she still tries to talk but I cannot understand anything now. It is a very sad situation especially for her . I can't even imagine what she must be feeling. She is a trooper though always smiling. I don't know how many people have to get this disease for the FDA to make the cure for this an urgent priority.

Thanks for listening,
Sheen
 

Sheen

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Hello All:
It's been awhile since I last posted anything. My mother is developing signs of FTD as well as struggling with PBP. The hypersalivation was a huge issue and I want to thank Rachel who suggested Methscopolamine Bromide 2.5 mg taken three times a day , as it seems to be working on my mother. Another issue with her is biting her cheeks and tongue. She continues to lose weight and we have convinced my father to get her convinced to get a peg. I recently heard that a stem cell trial is starting Phase I trial soon at the Robert Packard Center for ALS Research at John Hopkins. I was happy to hear about that .

I hope you are are doing the best you can under the circumstance. My thoughts are with you all. Take care. Sheena
 

trishs

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ontario
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sarnia
Hi Sheen,
I have just been browsing through these forums and noticed that your mother's condition is eerily similar to that of my mother. My mum was diagnosed with bulbar onset ALS in May 2009. I live in Canada and she lives in North Carolina, and we used to talk on the telephone several times a week. When her speech deteriorated to such a point that she was completely unintelligable, we thought that we had found a solution by instant messaging each other every morning and evening. At home, she would use a white board to communicate. Sadly, this form of communication is no longer working either. She sometimes gets the right letters, but they are written in a jumbled mess. Interestingly, she can still sign her name perfectly, and has no trouble with numeration. Mum too has the blank look, which I had attributed to the bulbar involvement. Other than being a little slower on her feet (she is 75), her motor skills are still pretty good. I really just wanted to touch base with you since you seem to be the first person that I have read about whose situation appears to be so similar to mine.
Trish
 
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