One of the worst diseases. I'm so sorry OP.I can promise you that there is no reason you should in any way be suffering physically at the end of life. You will not stop receiving treatment. We cannot treat ALS but we can treat pain, shortness of breath and anxiety/fear very very effectively. Your ALS neurologist or pulmonologist should be able to put you in touch with a hospice program and I think talking to them may reassure you.Opiates for instance are almost always successful at relieving shortness of breath and pain. Benzodiazepines are sometimes used for anxiety. These are given in doses of whatever you need to be comfortable. With a good hospice plan you should have nothing to worry about as far as untreated symptoms.The setting is also a consideration. Home hospice can be challenging mostly from a care standpoint. Often patients with ALS already have in place a high level of care at home and can stay there with a few hours a day of hospice nursing. If the nursing needs are significant or can't be met, the other option is to choose to do what is called inpatient hospice. This is in a setting that is more like a hotel than a hospital, visitation restrictions are (in general) quite relaxed, and there is 24 hour nursing care. They are very focused on quality end of life treatment.You are dealing with your own mortality and grief. This is more than enough. What you shouldn't be fearful of is feelings of pain or shortness of breath. I suggest meeting with the hospice people in the near future even if you are not yet ready for this. They can answer questions and plan about how you want this to be done.
I (63m) was diagnosed with ALS about 8 weeks ago. Needless to say, this has been a devastating diagnosis for me and for my spouse. Symptoms started 2 years ago with diffuse cramping, and minor dyspnea upon exertion. The cramping continued, then I developed more dyspnea, and then fine motor skill weakness in left>right hand. An EMG in March of this year showed major motor neuron issues, and the suspected MND was confirmed as ALS by two specialists. I am 5'9, 170 lb. I am on riluzole and Relyvrio, still waiting on the Radicava because the fucking insurance company is taking its sweet time and would probably prefer that I die in the meantime so that they don't have to pay for it. Here's my issue: my main symptom, which has slowly progressed, then more quickly starting in Feb of this year, is respiratory muscle weakness (diaphragm and chest wall muscles). I cannot breathe lying flat, and have to use a BiPap machine at night or I get hypercapnia. I am increasingly fatigued during the day, and often short of breath even just moving around the house or doing simple chores. I can walk a mile slowly, but have to take a break halfway, and it's taxing. I understand from my voluminous reading about this shitty disease that most people die from respiratory failure at the end, and that that is usually the last set of symptoms they get. Respiratory muscle weakness is more rare as a presenting symptom. Given that it's really the first major problem I am having, my fear is that it's going to be the thing that takes me out, and that it could happen rather quickly. I am trying to get my affairs in order, have an advanced directive on file with my PCP and am doing all other estate planning and power of attorney, etc., as fast as I can. I do not want tracheostomy or gastrostomy under any circumstances, I do not want to simply exist hooked up to machines. Quality of life is paramount, and my spouse has agreed to all of my wishes. We do not have children. How will it end when it ends? I am still relatively mobile and can use my limbs and hands, no real issues with voice or swallowing. I imagine that at some point I will simply not be able to breathe any more without the NIV, and that even that will not work at some point. What I am unsure of is what it looks like getting to that point, and what happens when I simply can't breathe any more and an NIV doesn't work. I want to go quietly and easily, without struggle. Do I state that I want continuous deep sedation at some point, and if so, when? Any guidance or feedback at all is welcome.