In the first part of this series on tracheostomy and ventilation, we heard from clinicians about the facts and considerations people with ALS should consider when deciding if the procedure is right for them. However, no one can really know what it’s like to live with ALS after undergoing a trach and vent, other than someone who has experienced it themselves.
To get a firsthand perspective on this subject, the ALS Therapy Development Institute (ALS TDI) spoke to Stephan Korner. Stephan, who was diagnosed with ALS in 2019, generously shared his perspectives on what it was like going through the procedure, what he wished he had known beforehand, and what his life has been like since.
ALS TDI: First off, can you tell me a little about yourself? Who you are, where you're from, when you were diagnosed, etc.?
Stephan Korner: I am 55 years old, live in Louisville, KY, and I’m married with three children. I’m originally from Norway, but I came to the United States when I was 19 to go to college. My childhood dream was to become a pilot and, when I was diagnosed with ALS, I was a 767 international captain for UPS. My first symptom was mild slurring and postnasal drip in July 2018. After numerous visits to doctors in Louisville, in January 2019, I went to the Mayo Clinic where I was diagnosed with bulbar onset ALS.
By the fall of 2019, I lost all speech and slowly started developing weakness in my limbs. By the summer of 2021, I could no longer walk, and today, I can barely stand. In February 2021 I had a PEG tube installed. My fingers are curled but I can still type with my thumb on my phone.
I completed the Mayo phase 2 stem cell trial and then joined the Healey Platform Trial but had to discontinue it because I no longer could swallow time-release pills.
When did you first find out about tracheostomies, and that it might be something you would need with ALS? How did that make you feel?
When I was diagnosed I read extensively and discovered that with ALS the diaphragm muscle eventually weakens to the point where mechanical ventilation will be needed. Also seeing photos of Steve Gleason made me aware of tracheostomies. Knowing that bulbar onset usually is very aggressive I didn't think that I would survive long enough to get a tracheostomy, so I didn't think much about it.
When did you make the decision to get a tracheostomy? Do you feel like it was a difficult decision?
In the fall of 2019, I started using a Trilogy ventilator at night because of hypercapnia [a condition in which the levels of carbon dioxide in the blood are elevated] which resulted in me waking up with severe headaches. As my illness progressed the ventilator pressure had to be gradually increased, and I started to use it during the day as well. During my March 2022 ALS clinic visit I asked my neurologist about getting a tracheostomy. He referred me to a pulmonologist and I met him in May 2022. By then I could only stay off the mask for about 30 minutes. At my appointment, my FVC was 38%, and the pulmonologist thought I would be a good candidate for a tracheostomy. He suggested I do it as soon as possible so I didn't have much time to think about it. A week after the pulmonologist suggested a tracheostomy, I had the surgery. My wife said to go for it, and without her support, I wouldn't have done it.
What sort of information were you able to get about tracheostomies before you got one? Is there anything you wish you had known in hindsight?
I purposefully didn't do much research before the procedure because I decided to take things as they came.
The things I wished I had known before the procedure, (not that it would really have mattered anyway):
- The risk of complications. After the procedure, I had an ileus [a disruption of the normal function of the intestine], followed by pneumonia even though I had the pneumonia vaccine. I ended up having to spend two weeks in the ICU followed by two weeks in a rehab hospital.
- The other surprise was all the suctioning required. Every two to four hours, 24/7, my lungs have to be suctioned to avoid a mucus plug. Luckily, I convinced my supply company to give me inline suctioning, which they use in the hospital.
- Tracheostomy replacement. Every 30 days the trach needs to be replaced. It’s a bit uncomfortable but it's a quick procedure. My pulmonologist came to the house and showed my wife and one of my nurses how to change it.
- It’s so weird not having air go through my mouth and nose anymore, especially when I sneeze. I have also lost all sense of smell. There is an inflated cuff (balloon) in my throat so the air doesn't escape through my mouth. I call it a barbaric contraption :)
- The biggest surprise I got was that no home health agency will see me at home after I had the tracheostomy even though I have excellent health insurance. I am very fortunate that I have excellent long-term disability insurance which has allowed me to hire private nurses and caregivers. My wife still works as an engineer for UPS and the last thing I need is for her to burn out. I have a nurse that comes every weekday for two hours and does my morning care: tube feeding, cleaning of PEG area, dental care, cough assist, trach care, shaving, and a shower every few days. A caregiver then watches me from 11:00am to 5:30pm.
How do you feel about your decision?
I didn't really have much of a choice if I wanted to stay alive for a little longer. My pulmonologist said that the average ALS patient who gets a tracheostomy can expect to survive for approximately 30 months.
What would you say to someone else with ALS who is considering getting a tracheostomy?
Life is worth living! In the hospital when I was really sick I regretted doing it, but I no longer regret it.
Make sure you find a good pulmonologist that has experience with neurological illnesses. Try to assemble a good support team prior to having the procedure. It isn't a walk in the park, but with the support it's doable!
To read the previous post in this series, featuring perspectives on tracheostomy and ventilation procedures from Massachusetts General Hospital clinicians, click here.
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