respiratory onset ALS

[JKErskine]

Hello everyone, I am new to this forum.

Is there anyone on this forum who has experience with a loved one, or themselves, with respiratory onset ALS? It affects only 4% of the ALS affected population. I am looking for guidance and exchange of information.

About 6 months ago, we noticed my 72 year old father had started complaining about muscle weakness and shortness of breath. After seeing numerous doctors, no diagnosis was ever made. Last week he was admitted to the hospital for a gall bladder infection. He was walking at this time and only complained of mild muscle weakness in his legs and arms. While in ER, we were told he had respiratory issues and he was intubated. We are told now he will require chronic ventilation and they are looking next week to potentially perform a tracheotomy and insert a PEG. We are expecting a confirmed diagnosis in the next few days. Currently they say they are 80% sure.

I am so confused about next steps and what life expectancy and quality of like might be and would like to talk to some one who is familiar with respiratory onset ALS.

Thank you for reading.

 

[vlcare]

My husband was respiratory onset. His initial "tentative" diagnosis was in October, 1009. He does not have a PEG. He did not have a tracheostomy. However, he does use a non-invasive ventilator almost 24/7. The device is called Trilogy. It is portable. He breathes through a very lightweight piece of headgear that uses nasal pillows, which allows him to watch television, read and do most things. He has even taught himself to eat fruit and nuts and drink liquids through a straw while he is using the device. We can travel in a car easily.

With this kind of device, there is no need for suctioning or having an attendant nearby 24/7. When we asked our specialists (pulmonologists) initially if they would have a tracheostomy themselves, they said no. I'm glad we asked the question.

My husband is now 77 years old and the quality of life with ALS is much better than we expected. We told our doctor at the ALS Clinic that the best thing he ever did for us was to suggest the Trilogy.

You need to talk to an ALS specialist immediately if you have not done so. Get a referral to an ALS clinic if possible.

Good luck.
vl

 

[Diane H]

I am glad to hear that your husband is doing well on the Trilogy. I would like a clarification of the discussion with the doctors about a tracheostomy. Was the question
A: Whether they would have a tracheostomy if they were in your husband's current situation.
B. Whether they would ever have a tracheostomy if they had ALS.
C. Whether they would even have a tracheostomy in any situation.

A negative answer to A makes sense. Your husband doesn't need a trach yet. In the future a trach may be necessary when his cough becomes too weak to clear mucus from his airway. That is a horrible feeling of suffocation that can be relieved instantly by tracheal suctioning.

A negative answer to B is pure bull. Although opting for a tracheostomy and ventilator is not for every ALS patient, there are many of us living satisfied and even productive lives on ventilators. Studies have shown that quality of life is rated higher by ventilator users than by doctors and other medical personnel.

A negative answer to C is unbelievable.

I used BiPAP for about 6 years before needing a trach and vent. Trilogy is newer and is great because it offers more settings for BiPAP and makes the transition to full ventilation less intimidating because you are comfortable with the machine if not the trach. I tried several types of masks, headgear, and also nasal pillows over those years. Being vain and shallow, I hated going out in public with the headgear. The trach and vent drew fewer stares and now me and my vanity are out and about! But that is the least of the reasons I wish I had gone on the vent sooner.

 

[Santa joe]

My husband is also on a ventilator and unlike Diane he doesn't want to go anywhere. A ventilator is a personal decision to make. I feel like I need to inform those who are considering it a few things to think about. First and foremost make sure you have plenty of support to help you. It requires 24/7 care. Learning trach care is not that hard. If I can learn anyone can. Finding someone willing to help me has been another problem. Most agencies, even hospice, will not proved anyone for respite relief that is not a LPN or RN. This can range from $36.50 to $50.00 an hour in my state. My own sons do not want anything to do with the vent. They are both terrified and are grown men. My sister has just started to learn and will stay with him while I run to the grocery store.

When my Pals decided to get the trach, he told me he just wanted a couple of years, it's been a year. When I asked him if he would do it again. When he could speak - he said NO. He is bedridden, peg tube and unable to speak. All of this within a year. This is a Horrible, Horrible disease.

Debbie

 

[vlcare]

To reply to Diane's desire for clarification regarding tracheostomy question: the pulmonologists' answer to our question "Would you, yourself, have a tracheostomy if you had ALS?", their answer was no. I don't believe their answers can be termed "pure bull." After all, we asked their opinion and they gave it.

My husband's initial decision not to have a tracheostomy has not changed. Nobody knows better than him the experience of air hunger since his diaphragm loss of function was from the beginning. But his non-invasive device is doing quite nicely. He also uses a cough assist. And when these devices are no longer able to give him the quality of life he desires, his ALS physician will enter him into hospice and with their help, he will gently leave us.

As always we all recognize that these decisions are personal. It's a balancing act for patients and their caregivers.

vl

 

[ECpara]

Thank you vicare and Santa Joe for your honesty. Although my husband is not even close to needing a Trilogy, it is something he will consider, it was first brought up by the man who brought my husband his cough assist and suction machine. However, being on a ventilator is out of the question for him. I have a friend whose husband has ALS and is on a ventilator. She has told me that if they had it to do over, they would never have considered the vent. Her husband is completely immobile, even his eyelids are failing and it is hard to communicate. His eye gaze computer is no longer any help since he cannot control his eyes. She's fortunate that she, her son and her MIL are all nurses. He also has VA support having been in the military. We wouldn't be so fortunate. It's a decision I am happy we have talked about well in advance. I give kudos to those who make informed decisions either way!

 

[affected]

It is all such a personal decision, and the differences in replies here so far show the other important side of the equation. Some PALS live great lives for years with a tracheotomy and vent, and others deteriorate in their whole bodies so quickly they don't have the same quality.

Thanks everyone for sharing honestly on this question so far. Doesn't matter to me what my opinion would be if it were I in the situation, I totally support the right of each person to choose. I do the same with my own husband - I will tell him my opinion if he wants it, sometimes he does sometimes he doesn't, but I then totally support him in the decisions he makes.

 

[Diane H]

vl,

My choice of the words "pure bull" was ill considered. I was trying to be brief but sometimes brevity is not the "soul of wit" but rather the best way to be unclear! What I was meaning to convey is that their answer (that they would not opt for a vent if they had ALS) was not necessarily true on a personal level and poor medical advise in general. No one, not even a pulmonary specialist, has a crystal ball to enable them to see exactly what their situation and experience with ALS would be. I too said "No vent" but when the time came to make that decision I was still talking and swallowing well, healthy in all other respects. The source of my day to day problems were respiratory, my ALS progression was very slow, I could not walk and arm weakness made me totally dependent for all aspects of physical care and my husband had already taken early retirement to care for me. I had adapted well to the limits on my life and was content, productive, and loving grandmotherhood with our first grandchild, just three months old. Our finances were not great but livable.

By the point when my respiratory status had taken me down to nearly full time BiPAP use, and frequent, terrifying episodes of not being able to cough up the mucus clogging my airway, if my doctors had told me that they themselves would not go on a vent because of ALS, I might well have taken their advice. Thankfully they did not say that and the ten years I have been on the vent have been the most comfortable and least stressful for me of the entire course of my ALS! It hasn't been easy and carefree but life seldom is. My husband is stressed by the feeling of responsibility for me. He feels tied down but he is a restless person to start with and I really think he overestimates the fun he could have if not for me!

Yes, my progression and experiences with being on a vent have been atypical and even quite rare for ALS, but I hate to think that there are some, if only a few, ALS patients who miss out on what could have been a few to many years of good quality of life because of their doctor's uneducated beliefs about ALS and vents. How I wish every such doctor could roll a mile on my wheels and see that some ALS patients go on to be "real people" for much longer than doctor's advice would have let them have.

They never seem to mention that in the U. S. at least, when the day comes that a vented person decides the ALS has progressed to the point where quality of life is unacceptable, where death would be preferable, he or she can be sedated and medicated for air hunger, the vent turned off, and death can be peaceful. That process is just as for anyone dependent on a BiBAP mode of respiratory support. The sedation and medication would be the same for someone who had refused any respiratory assistance, but needed over a longer period of time.

 

[Santa joe]

Diane - I am so glad the ventilator worked out for you. I wish that had been the case for my husband but it has not. We have talked about the time when he is ready to give up. He is such a fighter. I hate to be a Negative Nellie but I think people should be aware of certain things. The time and energy you give to a vent patient can be overwhelming. That's why it is so important to have support. I'm sure your husband loves you very much as I do my husband. It's a very demanding job but I know he would do the same for me. Acceptance is my new word for 2014. This is certainly not the way I had planned for our retirement years. He did not ask for ALS to come into his life. Till death do us part..........

 

[vlcare]

Hello, All:

Perhaps we should move back to the point of the thread. An older man has been diagnosed with ALS to an 80% certainty with respiratory onset. His family has been told that he will need a tracheostomy. His son is confused and wonders about life expectancy and the quality of life going forward.

I responded because my husband was respiratory onset--the worst kind of beginning--just like JErskine's father. And yet, my husband lives quite well WITHOUT a tracheostomy. His disease will run its course but we suspect he will live quite a number of years while using non-invasive ventilation. And I live quite well without the trauma, isolation and stress of caring for a vented spouse. That is our story. It was offered as an alternative to what has been suggested to JErskine as "necessary" by medical personal in a hospital.

To put our story into perspective, we had been advised by an ALS specialist to prepare for a vent and a PEG within three months of diagnosis. Why? Because at that time, non-invasive ventilation was virtually unheard of in those circles. Then when we talked to pulmonologists they were explicit about the downside to trach--the 24/7 care required and the toll on caregivers. My husband decided against trach/vent. Out of the blue, our ALS doctor told us about this wonderful new Trilogy device and arranged for my husband to have it within days.

So, yes, technology is keeping my husband alive. And, yes, there are alternatives to trach/vent--even for early respiratory onset. And, yes, there can be quality to life with ALS. And, yes, decisions regarding vent/no vent are personal.

Sharing is a good way to get out information to those in a quandry. And caregivers are in the vanguard of "telling the story like it is."

vl

 

[affected]

I think we have stayed quite on point of the original thread.
We can't know why a tracheotomy has been put forward, so we can't answer that part, but we have some great views here on venting in different situations.

I wouldn't even consider a vent as my husband also has ftd involvement and doesn't have the benefit of living productively and actively with his family.

There have been some excellent points made here by carers on the toll a vent takes on caregiving, especially if it were to be a situation of living many years that way. I appreciate the openness of everyone on all sides of opinions and situations.

 

[JKErskine]

Thank you everyone for your responses.

Does anyone have a situation or know of someone who went from intubation (invasive temporary ventilation) to the Trilogy machine?

Is it possible to go from invasive to non-invasive? Currently the respiratory therapist performs a breathing test everyday and unfortunately he has been failing the tests.

Thanks.

 

[vlcare]

JK: The problem is that the doctor who ordered the intubation would have to feel comfortable about removing it. That's why we have heard many warnings about winding up in an emergency room. Hospital doctors want to save lives, but most of them don't understand ALS.

You are in a very difficult situation. What does your father want to do?

vl

 

[Diane H]

I was intubated in the ER after telling the doctor that I wanted to be trached and vented. I was kept medicated and out of it for a couple of days until the trach surgery could be scheduled. After the trach I was put on the type of ventilator the hospital used. When I was transferred to the Special Care Hospital where training for home care would be done, I was on a different vent for the ride across town and then yet a different brand of vent while there. It wasn't until a couple of days before I went home that Apria, the home respiratory equipment provider, brought in my LTV 950 vent that I have used ever since. All that is a round about way of saying that there are lots of brands of vents, including the Trilogy, and you can use any of them whether intubated or trached. Hospitals will prefer that you be on their brand of trach while you are there so the staff is familiar with the vent and they don't have to special order replacement hoses etc. for you.

Yes, it is very possible to go from invasive by means of intubation to non invasive (BiPAP delivered by any brand of machine including Trilogy). This is commonly done for patients who are being taken off a ventilator with the goal of getting them off non-invasive support as they recover from major surgery or trauma.

There seems to be some sort of mystique about the Trilogy. It is just a machine with settings to deliver BiPAP non-invasively or full ventilation via a trach. In the past this required two different machines. Trilogy is not a new breakthrough in respiratory support, just a hybrid of existing equipment. And quieter. And higher priced. And won't fit nicely on a power chair.

Since your father's daily testing isn't showing signs of improvement, I would definitely advocate for a trach. Intubation is very uncomfortable. If he does improve significantly it would be possible to plug the trach and put him on BiPAP via a Trilogy or other brand. However, if the diagnosis is ALS there is little point in that as at some point he will need to "upgrade" to trach and vent any way. Of course, at any point along the way he can decide he has had enough. He can indicate his desire to be taken off whatever respiratory device he is on, medicated for comfort, and if he can't breath on his own, die peacefully.

 

[affected]

I think it is now most important to find out what your father wants and follow his wishes.

Is you mother going to be looking after your father at home?

No easy decisions with this disease I'm afraid, please keep us updated and we will try to keep supporting.