An ALS diagnosis brings a myriad of questions. Here are just a few.
– What is the prognosis? It is 100% terminal – there is no cure.
– How long do I have? On average, 2 to 5 years from diagnosis. Some last 20 years or more. Some die within one month after diagnosis. People make guesses, or estimates, but there really is know way to know.
– What happens when my tongue and mouth muscles stop working? You will start experiencing “slurred” speech, and eventually lose the ability to speak. You will have options of low-tech and high-tech communication devices, depending on how much use of your eyes, hands, and feet you still have. Mary was able to speak until she had the trach surgery. By that time, she had minimal use of her hands or feet, and the eye gaze devices wouldn’t work for her. We did our best to read her lips to find out what she was saying.
– What happens when my throat muscles stop working and I can’t safely swallow any more? You can keep trying to eat and risk aspiration pneumonia or choking asphyxiation. You can choose to stop eating and eventually not be able to swallow thick liquids either, which will lead to death by starvation. You can choose to get a feeding tube placed through your intestinal wall and into your stomach and receive all your nutrition in liquid form. If you choose to do this, you should do the surgery sooner rather than later, before your breathing is too compromised. Mary had her feeding tube placed three months after diagnosis because her breathing was so compromised that they were afraid she would not be able to come off ventilation after the surgery.
– What about the breathing? In most cases, the diaphragm nerve (the phrenic nerve) is the last to go. In some people, it starts earlier, or even first, like with Mary. this leads to diaphragm paralysis, and the inability to inhale or exhale. The lungs can still exchange gases, but the diaphragm can no longer make the lungs inhale and exhale. Some people have had a DPS, or diaphragm pacing system, implanted. This has had mixed results, and is not usually recommended any more.
– What can I do about the breathing? You can choose to do nothing; you will experience carbon dioxide buildup which will cause loss of appetite, confusion, personality changes, lethargy, coma, death. As the air hunger gets worse toward the end, there are medications that can help make you more comfortable. You can choose to use non-invasive ventilation via a bipap/avaps (similar to a cpap) that will force room air into your lungs with positive pressure and assist with the exhale using negative pressure. You can have a full-face mask, a mask that only fits over your nose, or nasal pillows. Mary used the nasal pillows, since it allowed her to talk and eat and drink while having her breathing supported. Eventually, the bipap/avaps will not be enough, and you will have to decide whether or not to have a tracheostomy and go on invasive ventilation. Mary chose to do that 14 months after diagnosis.
Each of these questions leads to more questions, more decisions, more loss. ALS takes every ounce of strength out of the PALS (person with ALS) and CALS (non-professional caregivers of patients with ALS). It is important to have a support team of medical professionals, therapists and social workers, and spiritual leaders if desired. That will be the topic of another post.