Anybody out there with slowly progressing PBP?

[COlisa]

Hello,
My husband (age 43) has been diagnosed with PBP (http://www.alsforums.com/definitions/progressive-bulbar-palsy.html) by 2 neurologists now. He first noticed symptoms almost a year ago. His speech is very slurred, but understandable. He only coughs once in a while when eating or drinking liquids. He also has hyperreflexia in one leg and almost no reflexes in the other. EMG is normal.

The progression symptoms is (thankfully) very slow. Everything I've read says that PBP progresses very rapidly.

Anyone out there with slowly progressing PBP? Anyone who had PBP that progressed to limb weakness & ALS diagnosis? Anyone who was originally diagnosed with PBP who has a different diagnosis now?

Thanks,
COLisa

[Granny]

Hello COLisa,
I was diagnosed in April 2004 with bulbar unset ALS. My only symptoms were slurred speech, fasciculations of my tongue. That progressed to loss of speech, difficulty swallowing within the next year or so. I did not have any signs of ALS in either my legs or arms until January 2006. I don't know if PBP is the same diagnosis as Bulbar unset ALS, but it seems to be to me.
Hope this helps answer your question.
I am sorry to hear about your husband, he is so young. I am probably the "most senior" member of this group at 63.
Hugs and prayers, Leah

[COlisa]

Thanks for the reply Leah.
Some Drs. don't believe that PBP is an actual separate disease, just a different onset & progression. Because hubby has a "clean" EMG, they stopped short of saying "ALS" for now.

Be prepared for lots of questions from me :-). I understand if you don't want to answer, but I'm just trying to get a handle on the dx and what to expect. I know that everyone progresses differently, but any frame of reference is better that the prognoses that I find on the internet for PBP or bulbar-onset ALS.

How are you doing now?
Back when you were first dx in April 2004, did you have an EMG that showed weakness, but you just didn't feel it?
Did you have EMG in Jan 06?
What progression have you seen since your first weakness in January?
Have you completely lost your ability to speak? Are you using any aids?
How is your respiration?
How about your ability to eat? I give my hubby such a bad time that he could stand to lose 20 lbs, but now I know that weight loss would be such a bad thing.

Even now, it is frustrating for hubby in noisy places where there are lots of people talking at once. I read somewhere that loosing your speech is sort of like losing part of your personality, because you can't express yourself the same way and people start thinking of you differently. It makes me so sad.

Thanks for any insight you can give me. I appreciate that you have taken the time to reply.
COLisa

[Granny]

Hi COLisa,
I do not mind in the least answering any questions I can. I hope that your husband continues to progress slowly, and maybe it never gets to his limbs!
When I was diagnosed in April, 2004, my EMG was clean. He could find no involvement, and had me see another neurologist. That doctor said I had ALS, even though my EMG was clean. Now, I understand from others posting here that it is not the norm for doctors to diagnose ALS without something showing on an EMG.
I had another EMG done in the fall of 04 and it showed some abnormalities that would indicate ALS.
I have not had an EMG done since then.
I have completely lost my ability to speak. I use an assisted communications device, like a little computer that speaks back what you type in. I find this not completely satisfactory. I have an older model of one, and I think there are probalby better models on the market, but they are very expensive and this one is on loan, so it serves the purpose. I still stay alone from time to time, and have it ready to use with the telephone if I need to. For my day to day communications, I use a small white board and erasable markers. It works very well and my right hand still works fine, so I can write what I want to say quite fast. In fact, my husband tells everyone that he knows when I am upset with him because I always use BIG LETTERS, other times I use small ones:)) It is not perfect, but it does the job so far. You are right, people do treat you differently, not family or close friends, but others who sometimes think because you cannot talk, you cannot think either.
I have also lost all my ability to eat, except some puddings, yogart and some shakes. The rest I take by feeding tube, which I had placed in October 04, quite awhile before I needed it. That was a positive thing for me. I was losing weight and needed to eat more than I was able.
Since January, my left hand and fingers have become quite weak. Especially small motor skills, like buttons and zippers. Also find it hard to lift any weight with that arm. My right hand/arm is fine.
My neck is the biggest problem I have. It has become very weak and I need to wear a cervical collar. And, that is the hard part. To get a collar that fits comfortably and does the job is really hard. I am not sure it is possible to have one that is really comfortable.
The good news is that my respiration is still very good. I had an overnight oximetry test done recently that measures the oxygen in your blood when you are in deep sleep, and mine was very, very good. My ALS doctor had a happy face drawn on the report!
I have probalby told you a lot more than you ask for, and I hipe that it is not discouraging. I find that you learn to cope with each new thing that comes along. And, remember that everyone progresses differently.
Trying to remain positive and have hope is so improtant.
Ask any thing any time, lots of people here are good knowledge.
Hugs and prayers,
Leah

[COlisa]

Thanks so much for the reply Leah.

I think we are still in a bit of denial. Especially since he doesn't seem to be getting worse, we are thinking that maybe it just won't happen. It will take quite a while to get my head around it.

Thanks again. Hope you have a great weekend :-).
Lisa

[southern lady]

COlisa & Leah: I am 66 (and a half), & was diagnosed Mar. 05 with bulbar AlS. IT felt like Leah was telling my story. I am being evaluated Thur. for a speech device. It hurts more than anyone could know to lose your ability to speak. I was a teacher, speak Spanish, went on mission trips to Nicaragua every year. It's hard letting go of the life you once had, but you must in order to make the most of the one you have now. I do not have a feeding tube yet, but eating is difficult(& a bit messy). Just wanted to say hi to you girls & say I love you.

[COlisa]

Thanks for the reply southern lady.

My hubby was in high tech industry for years. 8 years ago he quit working to stay home to raise our kids and try to go back to school to become a high school teacher (which was what he had always really wanted to do). Well, life got in the way, we moved overseas for a few years, and just last year going back to school was becoming practical (kids are older & somewhat more self sufficient, no more moves in the works) & the speech problems started. With the dx and the way his speech is now, he will not be able to become a teacher. It breaks my heart to know that he will not be able to.

The upside is that he spent so much time with our kids in their "growing up" years (they are 12 and 15 years old now). With the dx, time seems like is the most valuable commodity.

I'm rambling a bit, but guess I'm really trying to say how sorry I am that you are not able to do what you love anymore because of ALS.

Please let us know how it goes with the speech device. What you start to use & what works for you. Good luck with that.

COlisa

[dana]

My dad has a dynavox speaking device. They have devices out there that can "voice bank" phrases for you. It would record your husband's voice. I would reccommend doing this now. We did not do it in time for my dad, and I wish we would have. My dad is now on a vent full time, and so he cannot talk at all. He writes things down, but I wish so badly that I could hear his voice again. The voice banking is definitely something worth looking into now if your husband is still able to talk.
Dana

[Jennifer]

Hello All:

Boy oh boy can I relate to the not talking thing and how frustrating it is. I too write bigger when I get frustrated and it seems that I just can't write fast enough when this happens. You cannot explain to other people how frustrating it is when you get asked a simple question and cannot answer. A good example is what's for dinner, you can't image how frustating it is to not be able to just say chicken, or hamburgers, or what ever it is.

Also, I find that when I want to tell a joke or add to something funny that has been said, it takes me so long to get it on paper and for other to read that the funny part has long gone making me feel like an idiot.

It's funny I never realized what a social thing eating was. My husband still gets invited out for Wing Night of which I cannot attend, as I cannot just sit in a restaurant and eat. I would never deny him going as this is my decease not his, but I never realized just how much talking goes along with eating....sadly this is one of the things I miss, going out for dinner and just enjoying the conversation.

Well take care all who are on this forum....it's unfortunate that we had to find each other on this forum but thank the good lord that we did.

Jennifer

[Granny]

Hi Jennifer,
I just wanted to comment on your part about not being able to eat. I find that almost more diffecult than not being able to talk. Most EVERY social event centers around eating. It is hard to stay home and sometimes harder still to go and sit there while everyone else eats. It does not bother me as much as it bothers others around me. This disease sure plays havoc with social times!
Also when writing, and by the time you get something written, everyone else has gone on to another subject.
But, I do try to enjoy every day and have an amazing husband and family and friends who help.
Hugz and prayers, Leah

[anne]

Hi, I am new to this site. Like most people I am looking for answers. My mother had been complaining about hoarseness in her voice. I really could not hear it, however, after 6 months of this going on I took her to an ENT specialist who examined her with an endoscope and told me that she had an inflammed vocal cord and that she would require speech therapy. We attended two sessions and my mom refused to go back. Shortly after this She had some dental work done and had eight teeth extracted and obtained an upper and lower denture. She had a difficult time getting used to the dentures and chewing became difficult (this was Sept. 05)in Nov 05) took her to an allergist to make sure she was not allergic to her cat. All came back negative. Jan 06 We noticed the pitch of her voice was very low. We went for a second opinion and was told that she had vocal cord paralysis and that she could benefit from a thyroplasty which we decided to wait on. Also the CAT scan was all negative. March 06 her speech started to become slurrged. I got concerned and arranged for her to see a neurologist, MRI normal. normal EMG of the limbs. She has problems swollowing, choking, excessive saliva. We were assessed june 06 at an ALS Clinic in Toronto and he felt it is motor neron disease which may remain localized. He could not diagnose her with bulbar ALS because she has had symptoms for 1 1/2 years and her felt that by now most people would have some degree of loss of mucles in the arms. My mother is 75 very active with her gardening, cooking and upkeep of the house. She has started Elavil for the excessive saliva and to control the crying/laughter. She also started Rilutek to help slow the progression. However, since our appointment in june 06 my mother;s speech has become worse. She is very difficult to understand. She is not talking much because it makes her anxious. She is still eating, taking longer but she is eating softer foods. She gets two good meals per day and has not lost any weight. She is unable to drink water due to the choking. She has been living on her own for the past 13 years since my father past away with Alzheimers at the age of 67. Thankfully my older brother has decided to move back home and will commute to work on a daily basis. I am frustrated because I do not know what is coming our way. Will she loose control of her limbs. Is she going to be able to stay home alone during the day while my brother is at work.Are there voice devices. I find it rather frustrating not being able to speek to her on the telephone. I am very close to my mother and would speek to her 4 to 5 times per day. Even if they were short conversations at least I knew she was alright. How do telephone devices work? If anyone can help me during this awful journay you can email me at pacifica231@hotmail.com

[Al]

Hi anne. Sorry you are here but glad you found us. There are quite a few devices that enable people to type words and sentences and the machine speaks for them. They aren't perfect but do help. The Occupational Therapist at Sunnybrook should be able to set you up for an appointment for evaluation. Call the clinic and ask Myrna or Olive for help.

[anne]

Hi Al: Thanks for taking the time to read my post. I will be taking mom back to Sunnybrook August 1 and will ask Myrna about the voice device. Thanks again. Anne

[slkfocus]

Hello:
I am a 54 year woman. I just got the diagnosis of PBP by my 2nd doctor. My symptoms came on hard and fast. Last September had major flare up of arthritis, December a bad chest cold and in February I notice my voice was hoarse but I did have that cold. One day in Feb I was talking normal and the next day I could not talk but was slurring. Everybody at my office noticed. About a week later I ate a sandwhich and went to eat the other half the next day and turned blue choking. I was choking on everything. In 2 months I lost over 50 pounds. My speech was bad.
However now I can eat chicken, turkey, sandwhiches, tuna, rye bread only, eggs and lots of other things even pizza. I eat in small bites and make sure I have a drink near by. Each week I am able to eat something different. I can't eat yogurt and ice cream bothers me. I drink lots of Rita's Mistos and milkshakes if I let them get warmer.
My speech has good days and bad days. I make sure to inhale so I don't run out of air and I have learned to talk low and slow and make sure I pay attention to what I say. The doctor says my speech is how it is because I now have a immobile soft palate. Sometimes if I get desperate enough I hold my nose shut and I sound clearer and louder (my kids are always screaming to hold my nose more to laugh at how funny I look than to understand me).
I too have reads lots of stuff on PBP and will be meeting with the ALS team at Hahnamen Hospital in Philly hopefully in 2 weeks. I too had the ENT, 2 nuerologists, 2 brain MRI's (1 with dye and 1 w/) and cervical MRI and lots of blood work.
This whole thing also started because my CK level was high which means I was losing muscle. I also had 2 Emg's and the last one done in July showed the damage was not as extensive as what the doctor said the 1st one was.
I do have some abnormalities in my muscles which I do not really feel. I am still carrying charts, filing and pulling charts. I am not dropping things and can still do buttons and small things. I am still doing housework.
I just had a chest xray to see if I am getting things into my lungs. It took me 5 months to convince my doctor to order one. I am fighting the tube as long as I can but I am way down in weight and not gaining. Any suggestions on drinks that will pack on the calories
Sorry so long but can't talk to hubby he gets so emotional about this.
Sharon

[gilly]

just reading some of your postings and it's amazing how similar your stories are. my aunt is 46 and she's having almost the EXACT same series of symptoms with ALS. i so appreciate your postings and they help me sympathize better with her as well as learn great tips on how to prepare for the future with this disease.

i just wanted to say hello and tell you that i keep each and every one of you in my prayers at night. i hope you keep your strong attitudes and continue to lean on this forum for support. i just joined the volunteer group in NYC so that i might be able to help other PALS or family and friends in any way that is possible. you've inspired me and i wish i could do more... like come up with a cure!!!! sadly, god didn't pass that genious gene on to me!

lots of love.
nicole

[anne]

Hi...I have been very lucky that my mother has not lost any weight since she was offically diagnosed on June 27, 06. My mom cannot drink water at all. If she does she puts a thickner into the water. She really hates the taste of that however she will use it to take her medications. Also she drinks coffee in the morning and she puts broken pieces of arrow root or diagestive cookies into it. She drinks alot of fruit necters because they are thicker than regular juice. She will also drink one bottle of Boost per day. They come in all different flavours. I think they are soya based. She is also drinking Soya Milk. My mother was never a milk drinker in the past but finds for now she likes the Soya Milk.
She eats two meals per day. I have gotten her to use a crock pot because it makes the food softer. She has been enjoying some of the recipes that I have given her for that. She also has a few small pieces of cheese cake per day. We purchase a cheesecake that has no bottom crust so she is able to eat that.

My mother's tongue is not working very well but thankfully she can still eat.
We attend an ALS clinic in Toronto and they keep pushing the Tube issue with us. Unfortunately my mother is not interested in that. She is very upset that her voice has been taken away from her and she does not want the joy of eating to be also taken from her. Perhaps at somepoint we may have to make a decision about the tube but she is not ready for it now. She weighs 129lb and has been that weight for years so hopefully we will be able to keep her weight up.

I am sorry that this post is somewhat lengthy but I hope it is helpful to you. Anne

[Al]

Hey anne. Say what you have to say. We don't worry about length here. If you have something valuable like this we can afford the time to read it. AL.

[jo aust]

To 'granny'. You are not alone. . My mum has been diagnosed with pbp just last month although she has had it now for a year. She is 64 years young. In a matter of weeks she will have no voice left. Only very few of us can understand her now. We treasure each word she says and I can't wait to visit her next week. She just had peg put in and the first thing she asked the surgeon was "when can I swim?" We are sailing the Whitsunday Island next month. Jo

[kimjay]

I am new to this so I hope it works! I am 44 yo and was diganosed with PBP in May 06. I think mine is progressing relatively slowly. I first realised something was wrong around 15 months ago when the odd word slurred. No one noticed for a long time. I have had to give up my part time job lecturing, as my speech has got worse, but continue to this day working as an interior designer - making telephone calls, speaking to suppliers and meeting with clients. It is however, becoming more difficult to function at work and I feel as though I want to leave soon to get on with the things I like doing. So far, I have little (it is a bit diffiult to move food around my mouth) to no trouble eating or drinking and have never choked. My lung function test conducted 5 months ago came back 'normal' and I have full use of my arms and legs.

It is difficult to know how I am progressing as I am constantly being told everyone is different. The only advice I have to anyone with MND/ALS is to listen to your body. I make no apologies for slowing down and resting when I'm tired or refusing to speak when it becomes too difficult. I do try to eat well and exercise regularly.

The one thing I have learned is that you have to make your own way. There are no markers.
Thank you for this forum. Sometimes it's difficult to tell my husband how I'm feeling physically and emotionally as I know how much my condition upsets him. He is extremely supportive and cannot do enough for me, but it's the ones closest to you who hurt the most. Kim

[Pearl]

Someone asked about drinks - my friend is giving her huband carnation chocolate milk but is using half and half instead of milk. Sounds gross to me, but he drinks it - he has gained weight.

Those shakes at Chick Filet (fast food, chicken restaurant) have nearly 800 calories.

Of course, we say that he'll die of clogged arteries before the ALS at this rate....

[Fortune]

Hello To All,
My PBP dx is less than a month old, but a lot has happened. Best of all is finding this Forum because I've learned so much from people with similar experiences. My speech and swallowing have become more difficult. Drinking thin liquids makes me choke and cough if I don't tilt my head down. Using a straw helps as long as I take small sips. Since my tongue is weak, I can't move chewed food around in my mouth very well and recently I've had difficulty getting food unstuck that's already in my throat. I've lost 25 lbs. since Aug. Whether it's nasal, oral or surgical, I dread the thought of using a feeding tube.
I'm on the waiting list to see the speech pathologist who specializes in augmentative devices. I'm already carrying a notepad and I use gestures. I've even reduced my communication to basic nouns and verbs, but as my dysarthria worsens, fewer people take the time and effort to understand. I used to enjoy social occasions, even striking up conversations with strangers which is perfectly acceptable for a 68 year old. Now I'm reduced to joining a life threatening desease support group.
The good news is that the ALS center in nearby San Francisco is looking for participants to join research studies looking at the risk factors contributing to ALS and the effectiveness of Ritonavir and Hydroxyurea on ALS. Good news for the future perhaps.
God Bless, Fortune

[Al]

Hi Fortune. I saw the operation to put in a PEG or feeding tube once and it's not that big of a deal. I think a vasectomy was a bigger deal. You should seriously consider having it done before you lose too much weight because that makes you weaker and makes more chance for infection or complications. The augmentative devices make it relatively easy to communicate with. They aren't perfect but get the job done. It certainly is a time for transition just getting older but with this it can seem overwhelming to you. Take it a day at a time. Hopefully we'll get lucky and get lots of time. AL.

[Lynn555]

Hi, I'm new. My mother, who is 79, has very slowly progressing progressive bulbar palsy. I also did a lot of research on the internet, and pbp is said to be very fast progressing. But it's very slow with my mother. She started having speech problems 8 years ago, where she started to slur her words and had problems with any words with 2 syllables or more. Over the years it got harder and harder for her to speak clearly. It came out more and more garbled, and she would speak slower and slower. She went to speech therapists and had all kinds of tests. Then over the past few years she's had trouble eating and chewing. She has now lost almost complete use of her tongue. Now you can't understand her speech at all, she has to write things down. She can still eat very soft foods - like egg salad or oatmeal or tuna, and she drinks a lot of Ensure Plus. She has lost around 40 lbs. She finally got diagnosed with pbp by the Mayo Clinic in Nov. 2006. She does not have a feeding tube yet, as she still can swallow, and she's trying to keep up her nutrition. So it's very slowly progressing - 8 years from onset of symptoms, she still can swallow okay, she has no fasciculations at this time, and no significant weakness in her limbs. But her handwriting has changed. But it's mostly affected just talking and chewing. So this can be very slowly progressing with some people. :)

[anne]

Hi Lynn....Welcome.. Just want to let you know that you are not alone here. I am glad for you and your mom that she has progressed slowly. Life becomes very difficult with this illness. Feel free to ask any questions, there is always someone that has great advise. God Bless. Anne

[Lynn555]

Thank you so much, you are so sweet and supportive, and I really appreciate it. My mother has been having trouble with drooling, and she's embarrassed to eat in front of people, as she drools a lot and is now a messy eater, as much of it ends up all over her chin. :oops: She is on 2 meds - Rilutek (I think that's what it is) and Elavil (for depression, but a side effect is drying up excess saliva). But she still drools a lot. Has anyone else found anything that helps with this? Someone told us to try putting some meat tenderizer under her tongue, but we're afraid to try that. Has anyone heard of that or tried that? :confused: Thank you!

[anne]

Hi Lynn....everything that you have mentioned about your mom is exactly what my mom is going through. My mom cannot speak anymore. she writes things down for us if we cannot understand. She take Rilutek and Elavil. She was taking 60 mg of elavil and still drooling terribly. We increased it to 75 and the drooling decreased very very little but she feels that her mouth feels dry but still drooling. Her swallowing is now very bad and she decided on Christmas eve that it is time for a feeding tube. Christmas was a disaster at my house. She was eating her pureed food and doing really well. She then got up heading for the bathroom and had a panic attack because she could not catch her breath. It was really scary. It took her 10 minutes to recover from this. I think the eating infront of us was too stressful for her. We were told about the meat tenderizer and did try it but it did not help we also tried Transderm V patches which are used for motion sickness. You place them behind the ear. You can give them a try.

Keep in touch. Anne

[patricia1]

Hi I am reading this site and my symptoms are more like pbp but I was told I have Als because it is starting to go into my arms very bad in my arms just starting a year ago I was dx with ALS in 03 But I have been having slurred speech since 99 and I eat fine I have to be carefull with liguids sometimes however I am otherwise good So whats with that. Pat:???:

[Lynn555]

Hi Pat, I'm sorry you're going through all that. From what I've heard, it's extremely rare for pbp to stay just bulbar, that it almost always progresses to ALS where the limbs are also affected. But it's different for everyone. The progression may be faster or slower, or it may hit some areas harder than others - such as tongue more than legs, or legs more than arms, or right side more than left, etc. Yours seems like it started as pbp but moved into ALS, which is fairly typical. It sounds like you started with bulbar involvement, but you have not completely lost the ability to eat or speak. And once the limbs are involved, the diagnosis becomes ALS. I'm glad you can eat fine, that is a really good thing for you. With liquids, I've heard that thicker liquids can be easier to swallow than thin. So something like V-8 juice or a milk shake might be easier to swallow than water. Also try taking very small sips and bending your head forward when you swallow. I've heard that helps, too. It sounds like you have good days and bad days, too, which is typical. I hope this helps and I wish you lots of good days! :)

[patricia1]

Lynn Thanks so much for your imput your sweet. I wish us all good days and a good cure Pat