Self-Described Realist Shares Experience with Assisted Ventilation

“I wanted to share my story to show that not everything is "sunshine and rainbows" when you must rely upon a machine to breathe."
​--- Rachel

My name is Rachel, and I live in Chicago.  I chose to move to Chicago from my small home town in Illinois because the idea of big city living intrigued me.  I wanted to blend-in and have more opportunities for employment and socializing.  I currently have a roommate, who is a friend I’ve known for a while, and we rent an apartment in the downtown area.  We also live with my dog and cat, both of whom I adopted after moving here.  I enjoy online gaming, true crime and medical television shows, as well as, just going out with friends and family in my free time.

I live with a neuromuscular disorder called RYR-1 Myopathy.  It is very rare and affects all of my muscles.  It seems to be slightly progressive as I age, too.  More information about this form of congenital myopathy can be found at http://www.ryr1.org. 
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RYR-1 causes massive fatigue or what I call "global fatigue" in every muscle of my body.  The more I do, the more fatigued I become. Despite this, I completed a Master's degree in Rehabilitation Counseling and now work in social services full-time from home.  

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Although I was born with this condition, my family and I didn’t discover that my breathing was becoming affected until later in childhood when I started having symptoms of carbon dioxide toxicity.  Back in the 1990s, my breathing issues were treated with oxygen and then a Bi-level Positive Airway Pressure (BPAP) machine.  I then developed scoliosis, a sideways curvature and rotation of the spine, which caused my spine to crush my lung, making breathing much more shallow and labored.  I was hospitalized with carbon dioxide toxicity roughly three times before spinal fusion surgery could occur to help straighten my spine.  Following that surgery, I ended-up coding with respiratory failure because my breathing was so shallow due to postoperative pain. Then, just five days later, my medical team said that I required a tracheostomy and ventilator to stay alive.  I was 15 at the time and it definitely changed my life forever.

I use the LTV 1150 ventilator for positive pressure breathing support and I have needed it for roughly 20 years.  I use it 24/7, although I am not completely vent dependent.  The main benefit of the trach and vent is that it treats CO2 retention, but the challenges it causes are enormous for me.  One challenge is that a tracheostomy can cause mucus plugs that, if not suctioned or coughed out, can block my airway and cause respiratory distress or respiratory failure that results in death.  Another challenge is suctioning the tracheostomy.  My cough isn't very productive so I need to stick a hard plastic tube, called a suction catheter, into the hole in my neck (the tracheostomy) and that tube is attached to a suction machine which feels, and works, like a vacuum.  I have not only gotten the mucus out, but also blood and tracheal lining.  Needless to say, it can be quite painful.

Assisted ventilation via tracheostomy has been a HUGE burden in my life. Literally the biggest issue is finding reliable help for my care, which might seem surprising since I need a trach and vent to survive and should qualify for help through state programs.  What most people don't know is that the state I live in will cut nursing care for a disabled person in need, like me, once that client turns 21.  This means people like me have to essentially hire a stranger off the street to provide nursing-level care in the home.  While some care aides that I’ve hired to help me have been amazing, reliability has continued to be a big issue, which means that if my aide does not show up, I will not get the help I require for basic living activities. Literally every day I wonder if people will show up to help me; it’s a constant worry.  Every day I also wonder how I will live through a problem that may arise with my ventilator while I am alone.  After all, it is a machine that can fail like any other.  The stress can be overwhelming.  The only way assisted ventilation has improved my life is that I was able to resolve the carbon dioxide build-up, which means I am still alive today. 

Finding an employer to hire me was insanely difficult because I need an aide, a wheelchair, a trach, and a ventilator.  I found that employers see me as a liability and they will openly discriminate.  I have had Human Resources (HR) professionals tell me to my face that they will not hire me due to my medical needs.  I never had a "real" job until this January of 2021 even though I finished my Master’s degree back in 2011; ten years ago.  It took that long for an employer to choose me for the job I am qualified to do.  

I want people to know that workplace discrimination still happens despite the ADA, and with a breathing issue that requires a trach tube, it is almost guaranteed, in my experience.  I want people like me to realize that any type of employment is going to be an uphill battle and a fight for what they need.  I feel like I lost a lot by taking this job while also gaining private health insurance and a paycheck.  I work from home now due to COVID-19, but soon will be asked to work in the office.  The logistics of that include finding a reliable aide that I would have to pay using my earnings, so I essentially will hand my paycheck over to someone else who is there to ensure my basic needs are handled during the work day.  

I wanted to share my story to show that not everything is "sunshine and rainbows" when you must rely upon a machine to breathe.  I want others to know that needing assisted ventilation can be a huge burden and is not just a "wonderful thing."  I think those of us using assisted ventilation should share our stories so that others can see our different perspectives.  

My advice to anyone facing breathing impairment would be to "be ready."  Be ready for health insurance companies to deny the supplies you need.  Be ready for caregivers that you hire to not show-up, not call, and even steal from you.  Be ready for discrimination from the general public, and from employers in particular.  I wish someone had told me what it’s like, including the hard truths, as it would have saved me so much hurt since I could have been better prepared for what I’ve had to face.
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I am a realist who understands that the challenges will keep coming, and I know I’ll have to always be ready.