Prognosis with PBP

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Idaho2790

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I am a 72 year old woman, diagnosed yesterday with PBP at UCSF. Was told that because my Emg only showed a problem with my tongue, speech and swallowing, i might have two to five years before losing muscle in my limbs. I understand might have a need for peg or other items to communicate. When I check the internet it looks like this is a disease that has a short term prognosis. Any help with our concerns?
 

lgelb

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Very sorry to hear about your diagnosis, Idaho. Likely, the reason for their saying that was that they suspect you have IBP (which can be spelled out isolated bulbar phenotype or isolated bulbar palsy, so Google both) instead of PBP. With IBP, there is no progression of symptoms to limbs for at least six months post onset. IBP carries a better prognosis than PBP, e.g. respiratory issues occur much less often, so people live longer with it. And older, more often female people more often get that rarer variant, so that would fit your case.

Best,
Laurie
 

Idaho2790

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Thanks so much. Actually sounds more like what the director told us. I will send a note to her after I look in to that. Sure appreciate your information
 

Idaho2790

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Is it normal to be diagnosed with progressive bulbar palsy and then rediagnosed with isolated bulbar palsy if, after six months or so, there is not a rapid progression? I don’t find a whole lot on the internet, but if this is normal then the doctors diagnosis seems reasonable
 

lgelb

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Yes, IBP is something that an academic medical center would talk about more because you're right, it's a diagnosis where you are looking in the rear view. At many places, it might all be lumped together. I am glad you are being followed at UCSF.

IBP or PBP, it's still a shock even if you had forewarning so give yourself time to process and grieve before making any key decisions.
 

Idaho2790

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I was advised several months ago that this was possibly ALS. My husband and I were not surprised when we saw a neurologist at UCSF who wanted us to check in with the ALS Clinic on site. Since I'm not a TV watcher and tend to do genealogy and sew in my spare time, I was horrified that I might spend the next few years confined to a wheelchair. As I look into this not so pretty disease, if my ultimate diagnosis is IBP, I feel fortunate. This will give me some time to complete some research I want to do and do a little traveling. The neurologist I saw initially that referred me to UCSF wanted me to start on Radicava as soon as possible. My decision, but wanted me to really consider that . If I do have IBP, perhaps I can delay the daily infusions and enjoy a more normal life for awhile.
 

Livealot

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Hi Idaho , my husband was diagnosed in August with PBPL .He was having voice issue's for about 2 years .He had seen numerous doctor's, and had many tests He lost his voice a year ago , and now has a feeding tube.We saw 4 neurologist .He is actually fine , if you think no voice and a feeding tube are the new normal.Luckily he has no other symptoms. He still drives , mows the grass , and washes both cars .I'm hoping nothing changes for a while.His limbs are fine, so far.He is on a medication to control the crying.So don't give up on life. All the best to you , Dee
 

Livealot

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Laurie , you are my hero. Your replies always give me hope.
 

Idaho2790

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Hi, Dee, thanks so much for your response. I’m thinking, from your description that your husband and I share many of the same symptoms. I was just diagnosed with a type of ALS last week so am still trying to get my mind around it. I have been extremely healthy and active my entire life. Even with the voice problem I had 25 for Christmas breakfast, 14 for Christmas Eve, seven family members spend the night and stay for dinner that night. Still did all the cooking, etc. tired afterwards, but have been after all that for years. I don’t talk on the phone anymore because it’s difficult for those on the other end, but my husband still comprehends what I say. I’m thinking a peg can’t be far down the road for me too. Does your husband need the use this for all eating? Or is it a supplement to keep nutrition up. My weight keeps dropping and there is a concern there. How do you communicate? Does he use a computer type device or does a tablet work for you. We are trying to get our ducks in a row and this forum has been such a blessing. Best of luck it’s your family’s progress. Thanks for your response.
 

Livealot

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Yes , you share a lot of the same symptoms. The feeding tube is great for taking medication, and getting the amount of water he needs.He can still eat soft foods but it takes a while.His biggest issue is drinking , anything.He fought getting a tube at first after using it for just two days he said he didn't feel thirsty anymore.He turned 71 this month, and he has more energy than I do.The text to talk helps , and a boogie board too.You have a great attitude , all the best to you.
 

Idaho2790

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Thanks, Dee. All the best to you and yours. Just have to make the most of what’s dished out to us! Don’t get to start over, lol.
 

lgelb

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Thank you for the kind words, livealot. I think we all try to "hope" each other.

Best,
Laurie
 

Idaho2790

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I’m beginning to think that this is progressing lots faster than I thought it would. My swallowing ability is pretty rough just in the last two weeks! About then, I started taking any med in a capsule with a teaspoon of applesauce and that worked fine. Yesterday I tried to take Tylenol for a headache and never did get them down! I’m also finding that I’m choking more than before. I have an appointment with UCSF early in April. Don’t know if I should wait to let them know or if I should bother them with something that seems to be a normal progression. Share our time between California and Idaho so even if we are in California, it’s a six hour round trip to the clinic. Hard to know just what is “ normal” and what can be helped! Pretty early in this diagnosis to start bugging them, lol!
 

Nikki J

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If you are having difficulty swallowing and losing weight if you are considering a feeding tube you should call and get the ball rolling. The sooner the better for a number of reasons. It won’t keep you from eating or drinking what you can but is a way to get meds fluids and calories safely

You might also ask about Nuedexta which is for emotional lability but some have found it seems to help temporarily with swallowing and or speech.

You certainly aren’t bugging them especially as this is an actionable item if you will consider a feeding tube. Either way though it is what they are there for.

I am sorry for your progression
 
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Wilson2009

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I was diagnosed with Bulbar onset 13 months ago. Like you, my progression has been very slow and it now appears that IBP is the final classification. While I have had almost no progression since diagnosis in either my limbs or core (still have an FVC in the high 90s), my ability to swallow and speak continues to deteriorate.

The best decision I have made so far was to get a feeding tube. Having the tube placed has vastly improved my quality of like. Prior to the tube, eating was such a struggle and really, really scary because of the fear of aspiration. My wife was terrified every time I took a bite of food.

Now I have no issues with getting nutrition or hydrating or taking medication. I had lost close to 50 pounds in the year prior to getting the tube. Once I had the tube, my weight loss stopped.

I would contact your clinic as soon as possible to get the ball rolling.
 
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