exchanging experiences with bulbar onset ALS

ari3027

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Hello,
my father [69 years old] recently got diagnosed with bulbar onset ALS. I would like to find people in a similar situation/someone who also has been diagnosed with bulbar onset ALS so that my father can exchange knowledge and experiences with someone. I think this may be helpful from a psychological perspective. Please reach out if you are experiencing something similar and would be open to exchanging experiences.

Kind regards,
Ari
 

nona

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I'm sorry to hear about your father's diagnosis. I was diagnosed with bulbar onset over six years ago and I'm happy to share my experiences. Also, some of my favorite Netflix shows are from Denmark.
 

lgelb

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Just a note that DE=Germany.
 

nona

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ari3027

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Hi Nona, thanks so much for the swift reply! Great news that you are willing to share your experiences! Would you be ok to have a phone/video call with me and my dad?

Looking forward to it!

And yes, DE stands for Germany - we have good netflix shows too though :)

Ari
 

nona

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Unfortunately, being bulbar, I cannot speak.
 

Wilson2009

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I would be glad to share my experiences with your father as well, but like Jessie, I have lost useful speech. I think it would be better to post his questions on the forum to allow for a range of responses from all PALS. Every one of us has a different experience with this disease as no two PALS follow the same path as the disease progresses.
 

ari3027

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Hello Nona and Wilson,

oh I am so sorry to hear! We have many questions and would love to hear from your experiences. I think it's so much more valuable to hear from you directly than to read about the disease or to hear about it from a doctor. So thanks so much for your willingness to share - it really means a lot!
  • Do you have a PEG? If yes, did you have any side effects or does it bother you somehow? How long did the recovery take? The doctor has recommended my dad get one ASAP even though he can still swallow OK [he does choke on the food every day though]. We are just wondering if it makes sense to get it done so early given that it may cause discomfort and he doesn't need it urgently at this stage.
  • How were you able to tell that the disease was progressing? Has it already spread to your limbs? My dad's speech and ability to swallow is currently affected and he would like to know how he would be able to feel/tell that the disease is spreading to his arms? He has fasciculations and occasional spasms in his arms.
  • How long did it take for the disease to spread from one area to another one for you?
  • How long did it take until you lost your ability to speak? Was there anything that you felt slowed down the rate of progression? My dad does logopedic exercises but feels like this does not help much and his speech is not getting better at all. How do you communicate on a daily basis? Do you have a speech computer?
  • What kind of medication are you taking? My dad is taking riluzol and rasagilin. He also got atropin drops for the hypersalivation.
  • What kind of support tools would you recommend?
  • Do you have breathing support [NIV]? How were you able to tell that you have an oxygen deficit. I notice that my dad sleeps a lot more during the day and his pulse is low so I worry it may be because of a lack of oxygen.
  • Is there anything you would recommend to keep in mind or to prepare for in early stages of disease progression?
Sorry for the many questions, really any advice that you can share would be super helpful for us!
Many thanks,
Ari
 

affected

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Hi Ari,
my husband was bulbar onset.
You can also read this post I created some years ago and contemplate.

1. Getting the peg early is highly recommended. Don't risk aspiration and pneumonia, and don't lose weight.
2. You can tell the disease progressing as things start to fail, rather than 'feeling' it. So if he starts having trouble holding his knife, writing, picking things up etc that would be a sign.
3. The disease is never the same for anyone, it is really quite unpredictable I'm sorry to say. My husband was gone in 11 months, but others with bulbar onset manage well for some years.
4. My husband did not like side effects from Riluzole so took no meds, but others here can tell their more recent experiences.
5. Support tools - read that anticipatory planning post and then feel free to ask more questions.
6. Breathing issues with ALS are not really oxygen related. The issue is that PALS don't clear CO2 from their system and it builds up. This can cause them to need to nap a lot through the day, and yet not sleep so deeply as they need. Giving O2 is dangerous as it can make them breathe less deeply and so retain even more CO2.
7. Again, hopefully the anticipatory planning doc can help and you can ask more details as you need.

We will help however we can.
 

Wilson2009

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1. Getting a feeding tube placed as soon as it was offered was the best decision I made. As Tillie indicated it immediately reduces the risk of aspiration and allows you to maintain weight. However, in my opinion, the two primary gains in having a feeding tube are reducing anxiety and saving energy. Whenever I tried to eat before the tube placement, my wife and I were absolutely terrified that I was going to choke or aspirate during the meal. Just prior to getting the tube, it would take me at least 90 minutes to eat about half of a normal meal. I didn't stop because I was satisfied. I stop because I was exhausted.
2. I have had only minor limb involvement so far. I am weaker in my legs, but not to the point where I have to rely on a PWC. Bulbar involvement is another matter. My tongue is useless. My mouth won't open wide enough for dental x-rays. I cannot puff out my cheeks.
3. I echo Tille here. There is no hard timeline for ALS. I can still walk and drive after 5 years of symptoms and 4 years after diagnosis. Others I know have had much faster progression
4. I totally lost my ability to speak rather recently. However, it was by choice. I am a little hard-headed and continued to try to talk, whether family and friends were able to understand me without repeating. However, the more I tried to talk, the more I coughed, the more my jaw hurt and the more my throat hurt to the point I was coughing up blood. It is amazing how fast something like that will make you decided to stop talking. I have a speech computer (search WinSlate on the forum), but I rarely use it. I prefer a whiteboard or notepad as it is faster than the speech computer. I also have a text-to-speech app on my iPhone, which contains canned phrases and responses that you can trigger without typing. If you go that route make sure you find one that allows you to add canned items like name, address, contact person, and so on.
5. I took Riluzole for three years but stopped when the Riluzole caused diarrhea became a quality of life issue for me. I took Radicava (edaravone) for a little over two years but stopped that as well as my body was turning into one large bruise, a known side effect. I use Atropine as well to control the saliva. I will be evaluated at the end of the month for botox injections into the salivary glands. I will let you know how that comes out.
6. Concur with Tillie on support tools.
7. I currently do not need breathing support. I would say that you can buy fingertip blood oxygen saturation oximeters rather cheaply these days on Amazon if you feel a need to monitor it. However, as Tillie indicated lack of oxygen is usually not the issue. Rather it is CO2 retention. Has your dad been evaluated by a pulmonologist yet? With the issues you described, he needs to see one.
8. My only recommendation is to get ahead of the progression and management of symptoms and stay ahead. Most PALS always seem to be one step behind the disease. For example, using a cane when you need a walker or using a walker when you need a rollator. This includes waiting too long for house modifications or avoiding palliative care until the end.

Hope this helps
 

GXTrex

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@Wilson2009

My dad is limb onset but about #9, how do you know when it is time to upgrade the walking device? My dad currently uses a cane for longer outings but not any other time. How do we know when to suggest he uses it full time, then to a rollator, and so on? We obviously do not want to wait for a fall to happen and he does not want to use it any earlier than he needs to.
Thanks
Glenn
 

Nikki J

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There are 2 issues with mobility devices. Safety and fatigue. Fatigue is easier to see. If he is slower or can’t walk as far as he wants or is wiped out by trying something else is needed.

safety is harder. The issues are leg giving out, tripping from foot drop and balance which is influenced by core weakness as well as limb. If those start to go when he is exhausted it is a warning they will be an issue more of the time soon.

we had a lovely member whose pt insisted she start using a chair fairly early. He said sit now so you don’t have to ( I think those were his words). The point was she would avoid injury and conserve strength. She believed that it helped her maintain transfer ability much longer than would have otherwise.

I don’t know what your dad needs when but it tends to be earlier than we want to believe
 

ari3027

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Thanks so much for sharing your experiences and resources!!

What were your experiences from a psychological perspective? How was your mental state in the early stages of diagnosis?

I feel like my dad's mental state varies so much and that is very difficult for both of us. At times there is acceptance of the disease but then he also has days of full denial and a lot of anger with me and everybody else for not believing that he is going to get better. I try to be positive and give him hope for a life together - I have given up my career and "normal" life to take care of him full time. I do a lot of research and try to plan for his health needs in the future. But I think that this all signifies to him that I have given up on him somehow. It's such a difficult balance and I don't know the right way to approach the situation.

This was also the case with the PEG. I have only read overwhelmingly positive things about it and when i explained it to him at first he understood that it will be helpful to get it done early. But then he changed his mind and now believes he will get better and that the doctor's do not have his best interest at heart because they have given up on him. Somehow he also thinks that the PEG signifies the "end phase" or him "giving up". He wants to get back to his business etc. I don't want to push him to do anything he is not 100% comfortable with but I also don't want him to regret the decision or for this to impact his life expectancy.

The other issue is that my dad is very focused on getting a prognosis on his progression. He wants to understand how long he will be able to speak, walk ... and live. The doctors have told us that his progression is fast. I don't know what to make of this. But obviously no one can really tell how the progression will be. I don't know how to manage this situation. Have you experience similar worries and how did you manage it?
 

Nikki J

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It is completely normal to experience anger denial and sadness Especially as he was just diagnosed. Think of the stsges of grief because he s grieving his regular life and his future he thought he had. And in grief people bounce among stages.

a couple of years ago we had a thread where a number of PALS reported asking their ALS specialist if they were sure of the diagnosis- and these were people who had been diagnosed one year or more.

no one can say how progression will go. It can speed up or slow down or plateau for a while. You need to plan and prepare for the worst and hope for the best. That said, ALS specialists see a lot of PALS and have an idea of quick or slow often

what he is feeling sounds normal as I said. There is a condition called FTD that sometimes coexists with ALS. People who have it lose their ability to reason and may have angry outbursts. This doesn’t sound like it but I wanted you to be aware just in case things escalate and this is more than what it seems
 

affected

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At the start it is hard because there is so much shock, and a common way we try to cope with the shock is denial. This can take lots of forms. A few months after diagnosis there should be some 'normalising' of the emotions (some, we never find the old normal again of course).
My husband had FTD and what is important with this is that it is about patterns of behaviour and knowing the person they were well, to interpret if there are true changes in them. What I mean is: one or two angry outbursts does not mean FTD, nor does being confused sometimes. These are normal responses to the diagnosis and to adjusting to everything.

Since your father has only just been diagnosed, give him some time to absorb this, before asking him to make decisions.
You can spend the time reading here, asking questions, gathering information and preparing yourself. But be careful you don't scare yourself too much too, you are still getting over the shock too.

If you have information researched and on hand, then you can respond to him as he is ready to take in more. It can be overwhelming for him to have information pushed at him however, even if you know he needs it, if he isn't ready to take it in it will only scare him further at this point.

With his focus on progression, you can suggest that it takes a little while to know his personal journey, and you can help him watch changes over the next month or two so he can get some idea of how things are moving in his own body. Maybe even help him look at what he can still do, more than what he has lost or is losing now.

Don't forget to make the most of whatever time you have. He is still a person, he is not the disease, nor are you only a carer now. Take breaks from ALS whenever you can and enjoy time together, outside, watching movies, listening to music, whatever things you both enjoy together, expand on them. You will never have the time back, so try not to let ALS steal every moment from you both.
 
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