Bulbar VS Arm onset

[briana]

Hi,
My name is Briana; my sister was recently diagnosed with ALS.
I'm very much involved in her care; including taking her to all appts and communicating with her medical providers.
My sister has a lot of what doctors call "atypical presentation" symptoms.
For example, she started choking on food a lot. When we went to see her neuro, he stated that it's very atypical for PALS to have a preserved speech and develop swallowing problems first. We had two tests done and waiting to hear back from her doc about the PEG placement. Speech therapist said that "chin tuck" is not a solution in our case.
Another recently emerged complaint: feeling of tightness/ pain in her throat and tongue.
As my sister describes, " it feels like the back of her throat on one side is numb like it was sprayed with numbing medication when she had an endoscopy a couple of years ago". She doesnt have any problems moving her tongue.
Please let me know if it could a part of bulbar symptoms. My sister was diagnosed with the Arm onset and rapidly developing bulbar symptoms, which makes me wonder if she had the Bulbar onset to begin with.
I get very anxious about some of her symptoms that I dont know how to address ( like this throat thing I describe above). Our Neuro is getting annoyed with me calling and asking questions.
Thank very much to all PALS on this forum who despite having this horrble disease provide courage and advice to people like me.
Briana

 

[indigosd]

Briana, so sorry to hear that you and your sister are joining the ranks. :[ My Husband has Bulbar onset and his speech was one of the first symptoms that we noticed. He also always complains about that very same "numb" sensation in his throat. If you type in key words in the search box you will find a lot of information and answers to your questions. Have you joined a local ALS support group yet? Glad you found us!

 

[BarryG]

Briana, I am very sorry about your sister's diagnosis. My story is about the classic bulbar onset, occasional slurred speech, progressing to constantly slurred and hoarse nasal speech and then swallowing problems. Now I cannot talk that anyone can understand and cannot eat anything solid. However through all of that I have never has any numbness or pain other than feeling like my tongue had been burned by a hot drink, not really painful but uncomfortable.

I agree that it is very unusual to have bulbar onset without speech problems but that is just me and I'm no doctor. Maybe someone else can help answer your question.

Barry

 

[joelc]

I don't have bulbar symptoms but did have all the symptoms you described. I was choking on food and had all kinds of cramps, numbness, etc. but my speech was fine. It all went away when I got my trache and vent.

 

[rose]

Briana,

I'm sorry for your sister's diagnosis. She is lucky to have you. Don't worry about "bothering" the doctor, as his input is important. The SLP may be a better avenue for managing her care though.

In the beginning thin liquids were the worst for me. The first year I had aspiration infections twice.

However, as things progressed my problems were not so much aspirating, as that thin liquids just did not want to go down. The SLP, and also the OT, suggested we try carbonated beverages, and they are much easier for me to swallow. I also cannot make consecutive swallows when drinking. Two or three at the very most is all I can do. I can be soooo thirsty, but no gulping or chugging ever happens.

Now that I can only take tiny sips it seems like food is what gives me the most trouble.

Have your sister try following up a swallow of food with either a carbonated beverage, or a sip of liquid yogurt. I buy a brand that is very thick but still pourable. Alternating food with this type of liquid does help to a degree. Still I will have food just sit in the middle where it won't go up or down. Most food for me at this point consists of soups, etc. Tiny bites can ok, (or not!) so long as I give them a long time to make their way down before trying more.

My swallow is much worse than my speech, however my voice, or lack thereof, is poor too. The more fatigued I am, the weaker my swallow and voice are. Something for you and your sister to stay cognizant of, the "too tired to swallow" means she shouldn't try.

 

[Katie C]

Totally agree with Rose about watching for the fatigue! We can take Glen out to breakfast, and he'll even drink regular coffee with minimal problem (I do slip a packet of Thikit into it). In the evening...food needs to be tiny and as the SLP puts it.. "slippery" meaning plenty of gravy or other moisture. Really helps with the choking.

 

[briana]

Thank you all for your advice.
KatieC- Our Speech therapist discouraged us from using ThickIT based on the recent study showing increased risk of respiratory infections in pts using ThickIT.
BarryG- An hour before I got to read your reply, my sister said to me that her tongue feels like it's got burned with hot soup.
Rose- Thank you for the carbonated water tip.
Best wishes to you,Briana

 

[Katie C]

Briana... did your speech therapist happen to tell you the name of the study? A google search of swallowing studies didn't turn up one with the results you mentioned. I know our neuro and speech pathologist both are supporters of thickened liquids, and from our one person experience, there is far less aspiration. I'd just be curious to take a look a the study.

 

[danjay]

Briana - much of your sisters symptoms are similar to mine. My first symptoms were choking 10 years ago. Then numbness and burning moving up my throat and into my mouth. Wasn't until 4 years ago I began to have problems in my neck and then shoulders. I slowly became less and less able to swallow, now taking virtually all nutrition by PEG but I still speak pretty well. My Dr's tend to ignore the numbness and burning as I guess outside the ALS orbit and so probably something else. To me they are part and parcel of the disease as I know it and so I have come to view this not as one disease but a constellation of diseases in which each of us is unique.
Best to you and your sister
Dan