What to expect from here?

Hi everyone.

It has been a little over two years since I posted which is had enough to believe.

Dad was diagnosed a bit over two years ago with bulbar onset MND.

He seemed to remain quite well for a long period. His speech disappeared completely reasonably fast but he was still mobile walking often lifting some hand weights.

He has declined in the last few months. He needs a neck brace now has lost a lot of weight has a peg and almost all his food is taken in that way. He is also losing the ability to use his hands. Cannot do buttons or open some doors etc.

The scariest thing by far is the coughing fits.

They happen at night mostly with my Mum there and can take well over and hour to bring up what he needs to. They leave him exhausted and shaky and it is very scary for my Mum.

His care team have not recommended a suction device as they believe it increases the mucus. It seems he just has to battle on at this stage.

I am not even really sure what I am asking except that I feel powerless and confused and wish I could help in some way.

Is there any tips anyone can offer?

I also wonder what "stage"we are at here. I know it is very tricky to give time frames but with Dad still walking and moving about it seems hard to believe this could be close to the end. Does anyone have experience with this at this stage?

Thanks so much.

Hi Penelope, I also have coughing fits. Do to the weakened muscles it shoots pain down my arms and chest and even into my thighs, like if you just did 500 push-ups and sit-ups and then had to do one more. I have read here about "assisted coughing" and plan to ask my Dr about it. Maybe someone else here can explain what it is?

I remember all too well the hours spent try to clear my airway with a cough too weak to do the job. Frightening for everyone and so exhausting!

He needs to get a Cough Assist machine as soon as possible. His doctor can arrange it. A Cough Assist machine mimics a strong, natural cough. A full breath is pushed in through a mouth piece then the pressure is abruptly reversed to a suction level causing the equivalent of a good cough. Quick and easy.

Ideally he has a Pulmonologist and if he doesn't it is time to get one. A neurologist and family doctor are not the ones qualified and experienced with the breathing problems in ALS. The pulmonologist will likely be the one who sees him through to the end.

While waiting for the Cough Assist machine to arrive, here are some things you can do. Buy an expectorant cough medicine containing gaufenesin or only gaufenesin. It quickly loosens mucous. When he feels congested, give him a generous dose of the cough medicine. Give it a few minutes to work, then have him lie down. This will feel to him like the absolute wrong thing to do! But lying down will allow the mucous to be propelled upward more easily with gravity less a factor. Lying down will also allow your mom to help him by applying a manual assisted cough. To do this she places both hands on his abdomen just below his ribs. As he begins each cough she will apply a quick thrust, not just pressure. This will give him that "Oof" of having the wind knocked out of him. That will add force to his cough and move the mucous upward. Repeat until his airway is cleared.

His care team is way off base. Yes, uneccessarily frequent suctioning of a person with a trach tube inserted in his neck can increase the amount of mucous produced, but this has absolutely nothing to do with your father's situation. You aren't going to be suctioning out his trachea! You will be using what is called a "tonsil tip" plastic wand (brand name Yankauer) to help remove the mucous from his mouth if necessary. (Totally different from the suctioning they are so strangely assuming for a patient who doesn't have a tracheostomy.) You may not have to use the tonsil tip suction wand at all, but just having it ready is a much needed reassurance for everyone.

Increase his water intake to keep the mucous from being thick and sticky. If swallowing water is difficult, make sure it is hot or cold. In between the water won't trigger a good swallow. You can also try adding a thickener such as "Thick-It" to any liquids to make them just a tiny bit easier to swallow. Water is actually the hardest thing to swallow. Flavored or fizzy drinks may go down better as will milk (not advisable as it can increase congestion) or orange juice.

If swallowing liquids is difficult it is time for a feeding tube. He may not need it yet for nutrition, but good hydration is critical for the lungs. A cup of water down the tube about four times a day helps all body systems including the ever problematic bowels!

Thank you so so much for the detailed response I have copied it for Mum who has already gotten on to his care team and hoping to get his next appointment moved up. He already uses a cough syrup to help him get the stuff up he does a sort of gargle a few times a day as well.

Will
Post back once we get an update. Thanks once again

Once a coughing fit starts, it can take hours to calm down. It is so distressing. We have a pulmonologist that is our best asset. We have a cocktail of drugs, over the counter, and prescription, that help keep secretions and mucus thinned out. We also have a cough assist machine, a suction machine, and a nebulizer for breathing treatments. The key is to manage the secretions and drainage so the coughing won't start up. My poor pals had a coughing spell that took about 8 hrs to calm down. We haven't had any since we started the new treatments. Some days it seems like our lives revolves around it.

Hi guys

Thanks so much for the responses. There have been some bad episodes since I lasted posted. He has lost most function in his right hand (dominant) and starting to go in this left hand.

He uses an eraser to try and keep his teeth open as his jaw locks and he find it hard to breathe through his nose as he cannot control the secretions. I know it is a poor solution but no one has offered anything better. He also wears a mouth guard but has trouble removing it from his mouth leading to more choking episodes. It would be great if they could make one with a little grip or handle that protruded from his mouth but we haven't had much joy chasing up getting something like this made.

The other night after using an expectorant (Caledonia bisolvan I believe) to break up the mucus he had a massive coughing fit and couldn't unlock his jaw properly to breathe. Mum wanted to called the ambulance he wouldn't let her but it scared the pants off both of them I don't think either has recovered.

He had a raft of specialist appointments today. Social worker wants his assessed for future care options, the Physio and speech therapist want him to keep trying to take food by mouth even though it creates coughing fits and they don't recommend the cough assist machine as yet as they think it is difficult to use and can create more problems. They want small amounts of food syringes into the back area of his throat to stimulate the swallow reflex. They also want Botox to relax the face muscles and a palliative care nurse is going to visit. He has been recommended a couple of new medications. One is a morphine derivative I believe which may assist with breathing?

Phew sorry that was a lot. I am still so confused. Can anyone recommend something to pop between his teeth other than an eraser to help him breathe through his mouth?

Any advice thoughts welcome.

Sorry for the verbal diarrhoea

Hi Penelope,

You can get mouth props possibly in a decent chemist or online. I'd worry a lot about using an eraser as it he could bite a bit off it causing another choking issue.

I'm dismayed at the advice from the physio and speech pathologist, something sounds seriously wrong with them saying syringe to stimulate the swallow reflex.

Boxers wear protective mouthpieces, as do football players. Try a sports store.

I am in Australia but will ask about mouth props thank you

He has a mouth guard the problem is removing it from his mouth his hands are getting too weak and if my mum tries to get s finger in to assist it seems to cause more issues she is already concerned putting fingers in his mouth as the bite down reflex seems to be uncontrolled.

I am in Australia too Penelope and you can buy mouth blocks that are kind of like the size of the eraser I'm imagining you are using.

I was at a meeting with CALS and PALS run by MND NSW just yesterday and we were discussing the use of them for that jaw clamp reflex

They are very different to a mouth guard.

Agree that using a food syringe to "stimulate" swallowing is bizarre and more likely to harm than help. I would stop that if it is still being done.

I would, though, in addition to the other thoughts here, ensure that hydration through the PEG is sufficient and maybe try increasing it a bit/adding some papaya/pineapple juice.

Is the nasal mucus being removed via a baby aspirator? Is his BiPAP sufficiently humidified? How often is he using it?

If he could do more nasal breathing, a soft mouth guard/prop could perhaps remain in the mouth for longer periods, reducing the problem of removing it.