Still going strong

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ECpara

Senior member
Joined
Jan 7, 2012
Messages
605
Reason
Lost a loved one
Diagnosis
02/2012
Country
US
State
GA
City
Thomasville
My husband still won't come on this site, but occasionally he'll ask me to come on here and ask a question. He just wants to know if there are any other " Bulbarians" out there who still have complete use of their limbs two years after their first symptoms. He still does and is looking for encouragement. How long might he expect to keep going physically with his slow progression? Maybe someone else has a slow form.
 
Hi there,

ALS started on me by attacking the Bulbar region right away. I started noticing slurred speech, tongue atrophy as well as tongue fasciculations. My chewing muscles weakened making chewing my solid food slower than usual, swallowing was somewhat compromised by occasional choking on thin liquids, I also had drooling and, as if all this wasn't enough, I also started experiencing Emotional Lability (outbursts of uncontrollable laughing and/or crying).

I thought it was going to plateau on this Bulbar phase and it wasn't going to spread to other parts of my body but...I was mistaken.
Within months, it started attacking some of my limbs. Specifically, my left foot. I noticed myself tripping on the carpet at home, and nearly falling (foot drop). Then it moved to my left arm in the form of Ulnar Neuropathy. I sought help with a prestigious Neurosurgeon to have a minor surgery in the elbow to free the trapped Ulnar Nerve, but he refused alleging that it was ALS related and nothing he could do would stop this problem from getting worse.
I ended up with left hand atrophy.

Right now, I am pretty stable. I could say that I plateau on this phase now.
My right hand has been spared so far (knock on wood), as well as my right foot (don't have foot drop on that foot yet). But, having foot drop on the left foot, has created me serious balance problems and the Rollator no longer is very safe for me to ambulate (I'm spending more time on a wheelchair now).

My breathing is still OK, although not as good as it was when I was a healthy person.
I still can eat and drink by mouth, as long as the food has been puréed and, if I tuck my chin and drink liquids on small sips I usually don't choke.

Everyone is different. There are "Bulbarians" that stay that way for several years before the ALS moves to other regions in the body.
I am glad to know that your husband has somewhat stabilized and is progressing very slowly.
About your question of how long can he expect to keep going with his progression rate...No one can really answer that question. Everyone is different and there are too many factors and variables involved.

I hope other "Bulbarians" may chime in on this thread with their experiences as well.




NH
 
Thank you Nighthawk. You sound very similar to my husband, probably in the same time frame, even though you can't get around as well as he can. He does have atrophy of his right leg, he's been joking about his "skinny leg". But the muscle strength is still there, so far anyway. His arms are thin, too, but still very mobile. I pray he never loses his fingers, typing is his means of communication right now, I'm so thankful for this age of technology! He also writes notes carrying a small notepad around in his pocket. I keep depleting the Dollar Store of their notepad supply! He did not like the boogie board. He also eats and drinks about the same as you. I pray you both continue down a "stalled" path!
 
@ECpara:

Thank you for your reply and your kind wishes.

I could still get around if I wanted to but, it wouldn't be as safe for me as it was, let's say...10 months ago. I had a fall recently at home (lost balance, even when I was holding my Rollator with both hands), luckily I fell on carpeted floor and the only thing that happened was that I busted my reading glasses and some minor bruises in the face.

I wanted to ask you this, did your husband get the PEG-tube or, is he planning to get it down the road?
I've been holding off in regards to getting the PEG-tube. Not decision made yet as I have still been able to maintain weight eating by mouth. Although, I might get it later.

NH
 
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I am Bulbar onset. My first symptoms started in the summer of 2008. I had a lump in my throat in my throat that I just couldn't clear. Then the slurring and shortness of breath. My speech deteriorated so bad that I went on medical leave August of 2010 and diagnosed November 30,2010. I still have full use of my legs, feet, arms, hands and fingers. I do have weakness but I am still able to function. I got my feeding tube the summer of 2011 but was able to eat select foods and thick drinks until the end of 2012. I had the diaphragm pacer procedure July of 2012. My FVC has remained stable at 40.
Tell your husband slow progression is very real and there is hope. I say I am on the 20 year plan. I have passed the 2-5 years and feel blessed. Now we need a cure. Desperately need a cure.
 
My symptoms started in 2011 as speech and slight swallowing issues. Like Nighthawk, my tongue started to atrophy and I experience emotional lability along with excess saliva.

Recently I have experienced a very slight weakening of my left thumb and forefinger. My hand grip is still excellent, but gripping with thumb/forefinger is almost useless at times. My breathing is very slightly affected and although I use a bipap at night, I have no symptoms if I don't use it. I had sleep apnea prior, so upgrading to a bipap with an ALS diagnosis was the logical step.

I have no involvement so far on either leg/foot. Right now I feel like the disease has reached a plateau except for my speech, it contimnues to worsen.

This week I am having a PEG (feeding tube) inserted. This is also being performed well before I need it. I still eat almost all solid foods except stuff that can't be chewed easily. I am on what they call a modified mechanical diet. Which means most but not all of my food is soft and easier to chew/manipulate with my tongue.

I hope I have given you additional data that is useful. And I hope your husbands progress continues to be slow!
 
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Thank you VoiceforLinda, your words were very encouraging to my husband.

Nighthawk, yes Tom had a PEG tube put in back in November. He uses it only for his meds at this point, he can't swallow pills at all. So he crushes them in a solution of tea and puts it in that way. His eating is a chore, he can eat certain solid foods but has to clear his throat a lot. He doesn't like to eat in front of people (except family) as his chewing is labored, it's hard to close his mouth, and he ocassionally chokes it back up. I foresee puréed food entirely in the near future.

VoiceforLinda, you mentioned you are on the Diaphragm Pacer. This was suggested to Tom down the road but he's thinking he doesn't want it. What is your feeling about it and experience with it? Tom's breathing is still very good, his FVC was over 80% in January.

Tom remains active; still golfing (high 70s and low 80s), hunts, drives, enjoys puttzing around the woods and loves typing to his friends. I pray he can keep this up for a long time, he remains happy even though he can no longer talk. I will say the two other things he hates, along with not talking, is the uncontrollable saliva (he uses Benadryl and Atropine drops) and fatigue. He's easily exhausted and naps during the day. A recent trip to Disney World with grandkids was spent on a scooter, which really helped a lot!

One more question he wants to ask both of you, are you on Rilutek? He is, his doctor told him that folks with Bulbar Onset seem to enjoy the most benefits from it. Who knows? Our insurance pretty much covers it so he continues to take it.

I will keep you both in my prayers, thanks for sharing! Maybe there will be others!
 
@ECpara:

Glad you've found our posts informative.

In regards to Rilutek, my Neurologist gave me a prescription for it right after the ALS diagnosis (12/2011), the same day. He said if I bought it and started taking it I had to undergo monthly checkups to monitor my liver enzymes at the Clinic, as Rilutek seems to affect them.

He said that Rilutek is more recommend for PALS with Bulbar onset, as opposed to Limb onset because the breathing muscles deteriorate at a faster rate on PALS with Bulbar onset than PALS with just Limb onset.
He said Rilutek could prolong my life for about 3-6 months only, before I needed mechanical invasive ventilation (a.k.a. Tracheostomy).
But, I had already decided (on my Advance Directive/Living Will) that I will not be getting a Tracheostomy. So, I saw pointless spending about $1,000 /per month (the cost of Rilutek), on a medication that would give me only between 3-6 more months, plus the side effects.

But, it's a personal decision getting it and I respect whatever decision any PALS wants to do on this regard.

Take care.

NH
 
I had my clinic visit today. I am happy to report that my FVC has gone up to 63%. It was at a steady 30-32. It has to be the diaphragm pacer. I have been taking RILUTEK since diagnosis. I take robinul and guaifensen for the saliva.
 
@VoiceforLinda:

That's very encouraging news, indeed!
Your FVC going up from 30-32% to 63% is really excellent.
It looks like the diaphragm pacer helped you achieve this.

Mine (FVC) was 68% when they tested me at the Clinic last Nov.
I have another follow up appointment with them by the end of this month.

I'm with you on your wish of a cure for this disease, either to stop the progression or cure it completely. Let's hope it happens sooner than later.


NH
 
@Voice for Linda, that's great news about your FVC going up, hail the diaphragm pacer ! I asked my pulmonologist about getting one at a later stage, he informed me It's only at the trial stages in England at the moment so who knows when we will get it in Scotland! He has made a note in my medical notes that I'm interested so here's hoping :)
 
My mom is 71 and also bulbar onset. Diagnosed 2/2013, actively seeking a diagnosis since the summer of 2012, but symptomatic for quite some time before that. We (family) are in disagreement about when she first started showing symptoms. But, as with most moms, my timeline is now associated with my kids. So I distinctly remember being very pregnant with my first daughter and joking about how my mom and I were taking our vitamins at the same time. I noticed she was taking hers with apple sauce and not water, which I noted as odd and asked her why. She said she got choked easily of late, and it was easier to take her vitamins and stuff with apple sauce or yogurt. So this would have been the summer of 2006. She and my dad act like they have no CLUE what I'm talking about, but I do remember that clearly. And being hormonal and grumpy and getting irritated with her for muttering and not projecting her voice when she talked. I'd get all irritated and say "Mom! Speak up! And why are you talking like you just woke up?" :( So in my mind, that was the beginning. As of last summer she was at the point where people were asking my dad what was wrong with her (speech, weight loss, slow moving).

As of today, she is still walking, talking, eating (no PEG), and at the clinic this past Friday she GAINED 7 pounds and her FVC was 81%. That other breathing test they do (where you suck IN rather than breath OUT) was on the low end. She fell once, on carpet. Won't use a cane or walker. Walks a short distance with my dad in the evenings. She eats solid foods but has to take small bites and won't eat things like hard bread. She doesn't drool, but she has very thick saliva in her throat that chokes her. Got a script for Elavil on Friday to see if that helps. My dad makes her shakes in the blender. We got them a Ninja. So he uses Ensure, yogurt, ice cream, protein powder, fruit, peanut butter, etc. and makes it up in the morning and she drinks small glasses of it during the day. She does not want the PEG but is willing to reconsider down the road. Anyway, she does have limb weakness, can't stand or walk on tiptoes, finally agreed to STOP GOING UP AND DOWN STAIRS when she's alone, scoots down the steps on her bottom when she does use them (and someone is next to her), and agreed to stop doing stuff like laundry and house work. Not because she isn't able, but because energy conservation is important. Thankfully, she's always been a big reader and tv watcher so it hasn't been a horrible shock to her lifestyle.

Adding that she is on Rilutek. She started it a few weeks after diagnosis. My dad found the prescription assistance place out of North Carolina. He applied and it went from $1200/month to FREE. They have to reapply every 3 months. They aren't poor, but aren't wealthy. Apparently the income limits to qualify for assistance are higher for this than for social welfare type programs. Her doc said do it if it's cheap/free/doesn't cause you financial hardship. Otherwise he didn't seem to all fired up about it.

Jamie
 
my clinic neuro is retired air force and has done nothing but als her take on rilutek if va provides it free or my cost would be less than $200 take it otherwise do not. va would provide it but i refuse to put that cost on them for practically no value. i still walk, talk, eat and breath on my own
 
My husband also has bulbar onset. His symptoms started Thanksgiving Day 2009 with slurred speech. After months and months with all kinds of different doctors, he was diagnosed with ALS. Today, his speech is progressively worsened, he has FTD and uses a Bipap at night. He has NO trouble walking, eating or swallowing and does not drool. He is on Rilutek, Baclofen and Nuedextra. We've tried the Deanna Protocol and the only difference I could see was that he seemed to have more energy. The Bipap does not seem to do much good in that he only sleeps maybe 3 - 5 hours a night.

It's nice to know that there are others who's progression seems to be slower than the life span of 2 - 5 years. Since we are in our fourth year of this awful disease, I find myself constantly watching and waiting for the next symptom to rear it's ugly head.

One of the things that has scared me is him passing out. Whenever he gets a cold related cough (that hard, deep cough), he passes out. Evidently, since his breathing capacity is so bad, he turns red, shakes and passes out. Has anyone else had this problem?

ALS is a monster but trying to keep my faith and hope alive.
 
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