Bulbar Onset - Relentless Coughing

[Kimberly Hutch]

My mom was diagnosed with bulbar onset ALS and FTD in late April 2020. At the time of her diagnosis her speech was very slurred and she often couldn't find her voice. She was also having difficulty swallowing both food and liquid - and choking was becoming more and more frequent. Mom's advance healthcare directive stated that she didn't want her life prolonged with feeding tubes and ventilators if she had a terminal illness... in following her wishes we started hospice care in mid-May.

It is now July, and we can barely understand Mom's speech, and her voice is gone most of the time. We are very careful with what we feed her... very soft foods cut into small pieces, pureed soups, yogurt, nectar, smoothies. We watch carefully as she eats every meal - as choking is more frequent. FTD makes it more complicated because she lacks impulse control... she will take large bites and shovel food into her mouth too quickly. So we constantly have to remind her to take smaller bites and to slow down multiple times during each meal.

Over the last couple of weeks we have been giving her nebulizer treatments every 4 hours and following them with the cough assist machine and suction... as she isn't able to cough effectively. Fortunately her lungs are clear and her blood oxygen is in the mid-90's.

Over the past week, she has been experiencing this relentless coughing. These coughing spells go on for hours and hours. Last weekend she coughed non-stop for a day and a half - and her hospice team was trying to find a cocktail of medications to stop the coughing.

• Levsin
• morphine
• mucinex
• albuterol (nebulizer)
• Tessalon Perles

These medications will help for awhile - but the cough breaks through a couple of hours before we can give her next dose.

I am curious as to whether anyone has experienced coughing fits like this, and what medications have worked for you or your loved one? This constant coughing and hacking both physically and emotionally distressing for Mom and for us as we watch helplessly.

I feel like we have been thrown into the deep end of a pool not knowing how to swim - and we are trying to keep our heads above water. Any advice you can share would be much appreciated.

xo Kimberly

 

[affected]

I'm so sorry Kimberly.
She is likely either aspirating a little when eating, or has left over food in her throat that causes the coughing and then probably a little aspirating.

I would honestly recommend a feeding tube and stop all oral food and liquids.
A feeding tube will allow you to keep her fed and hydrated safely.
It won't prolong her life by years, maybe not even at all, but it will allow you to give her food, fluids and palliative medications safely.

She is unlikely to choke eating, unless you give her foods that can actually lodge in her throat. But she is heading to pneumonia which is a common secondary cause of death for PALS. With her health directive you could elect not to treat the pneumonia but these decisions are never all that clear cut, nor are they always easy when the time actually comes around.

Those medications won't fix the cough because it is caused by her having food or fluid remnants in her throat and aspiration episodes.
The cough assist machine will help, but preventing her constantly aspirating will be even better.

If her directive specifically says no feeding tube, you may need to really go over the situation with a speech pathologist and dietician and work out how long you can keep feeding and hydrating her before you may need to either withdraw this or allow the pneumonia that will result.

I am so sorry as I know this sounds so awful. My husband was bulbar onset with FTD. I really wasn't sure the feeding tube was going to be worthwhile in his specific situation, but it really did make everything so much easier, and giving him palliative care towards the end was very simple.

 

[lgelb]

I take it she's not on BiPAP (which isn't usually considered a "ventilator" unless a backup rate is set -- has she been asked about using it as a comfort measure? Her carrier would still buy it though she might have to "leave" hospice for a short while).

The coughing is probably not just clearing secretions, but also her lungs' trying to get more air. When she is conscious, it is likely going to get worse, until she is completely exhausted and/or sedated. How soon you begin considering how things go on the last mile should relate solely to her quality of life, as she perceives it.

In addition to considering BiPAP if not as yet ruled out, I would consider whether the nebulizer, suction and the CoughAssist are really helping overall, as their use can entail energy and muscles she doesn't have.

If she can still "spit" into a paper towel at all, that, coupled with some combination of heated humidification, pineapple juice, soda, warm lemonade, etc., and an upright position where you provide the lower chest with support during coughs at the start of an episode, might be worth trying.

Also, cleaning or replacing all filters, treating any allergies (there are liquid and fast-dissolve antihistamines), and upping hydration through all the thick liquids, applesauce, pudding, etc. might help.

In addition, some of the meds she is on can dry up the secretions, causing irritation as they cannot be coughed up, and they can also cause/exacerbate clogged sinuses. So the cocktail is always up for titration, potentially, if you can add some non-med interventions to it.

I understand that her advance directive rules out a feeding tube, so I would never advise that. Nor do I think that you need to actively withdraw food or drink at any point, given her FTD.We all have different beliefs. If/as she is ready to go, which you will know, even if she can't say so, titrating morphine to full comfort will help her find her peace. If your hospice doesn't support that, that would be the time to bid them adieu.

Ask anything, any time, and thank you for helping her.

Best,
Laurie

 

[KarenNWendyn]

I agree that you should continue to respect her wishes for no feeding tube. She is likely aspirating which is a terminal event in her situation. I recommend continuing what you are doing and don’t be afraid to push the morphine dose to whatever is needed for her comfort.

I’m sorry you and your mom are dealing with this.

 

[Kimberly Hutch]

Thank you so much for your advice. I am positive she wouldn't want a feeding tube even just for comfort. Our hospice nurse checks her lungs several times a week and sometimes multiple times a day. She visits often as we try to get this coughing under control. They don't think the issue is food or aspiration at this point, but the thick mucus that Mom can't effectively cough up. This is why they are using all of the drugs to thin and also dry up the secretions. We haven't done bi-pap yet... and when we have asked about supplemental oxygen they said they don't really do that. So my guess the bi-pap is a no go for now. She is on a minuscule dose of morphine at this point - as she wants to be alert for now. I can't believe how quickly her disease is progressing. It was mid- March when we noticed that she was having difficulty enunciating her words, and the choking on food/liquids was happening more often. I am so fearful of what the next weeks/months are going to bring... we definitely don't want her to suffer. My sisters and I are not ready to lose her and she doesn't want to die at this point. She is afraid.

 

[KarenNWendyn]

It sounds like you are taking excellent care of your mom. She knows that on some level.

 

[affected]

You are doing brilliantly and only you can make the decisions for her. We can all respond about our experiences, but each journey is so individual.

 

[lgelb]

Supplemental oxygen is not needed for BiPAP to be effective and in most cases it would be harmful. I would ask again about BiPAP for comfort. Again, you wouldn't get that from the hospice, because that's not their lookout, but from Medicare through the regular clinic or physician that she has. So you could talk to her about it (she wouldn't even need to wear a mask, she could use a mouthpiece to grab a breath when needed) and then ask her doctor. Any doctor can order it, including a primary care physician.

It is an individual journey, no doubt -- we just illuminate the paths that we have taken or know are there.

 

[affected]

Palliative medications can be given to good effect if she does not want bipap and many PALS with FTD do not tolerate a mask over their face or effectively use a mouthpiece. Don't be afraid to titrate the amount of palliative meds needed as the breathing deteriorates.

 

[nona]

Could she be experiencing reflux? I have bulbar onset and take in NO food by mouth, yet I still cough and it's incredibly frustrating.

 

[Innovative]

I had a bad bout with unstoppable coughing, and I was admitted to the hospital. Basically, the hospital did very little. However, my excessive secretions went away after a few days. The only thing that changed is I stopped taking vitamin C.

As time went by, I forgot about my excessive secretions problem, and I resumed taking vitamin C. The secretions returned for two months before I remembered that I had that symptom before. When I stopped taking vitamin C (the second time) the secretions stopped again. I haven't had the symptom since. This is just what happened to me. Not sure it will help anyone else.

 

[nona]

ohmigosh, @Innovative , that just happened to me! I started taking vitamin C about six weeks ago and before too long my reflux started to come back and then I got horrible canker sores in my throat. I thought the secretions were either allergies or reflux. I stopped the vitamin c last week and the reflux got better immediately. Secretions are a little better and cankers are down to one. Even before ALS I couldn't eat citrus or drink juices, but I figured powdered C in my feeding tube would be ok. Well, all the nopes!

 

[Sonshine]

I am sorry your mom is experiencing the episodes. My husband who has bulbar onset experienced similar events. I believe his were due to laryngeal spasms and hs ALS specialist increased his Baclofen which had helped tremendously! You are doing a great job. Keep reaching out. You're never alone.

 

[NorthernCalif]

My husband is recovering from aspiration pneumonia... he had been coughing and I was unsure if it was the disease or something else. He had a chest X-ray and we found out it was aspiration pneumonia. He is just finishing up with 10 days of antibiotics, 3 times a day. The pills were very hard for him to swallow and then he ended up having diarrhea for about 8 days. We fed him yogurt for the diarrhea and it helped. We are considering a feeding tube. In hindsight, we should have asked if the antibiotics could have been in liquid form....

 

[lgelb]

Glad that your husband is on the mend, Cali. Just a general note for others that not all antibiotics mix well with dairy -- always read labels. Eggs. toast, applesauce and mashed potatoes are a few foods that may help the GI tract deal with the drugs.