Hello, my name is Juri Kameda
Two years ago, Abbie Rosenberg from CSRC had asked me if I would be interested in speaking at this conference. I would like to thank her for this opportunity. I have ALS (Amyotrophic Lateral Sclerosis, better known as Lou Gehrig’s disease). I have a slower progressive form of ALS which allows me this opportunity to talk to you about the disease in the hope of bringing awareness and information which in turn will empower you to provide the kind of respiratory care that ALS patients need to improve their quality of life. Though I am no longer able to work in the field as a Respiratory Therapist, what compelled me to come speak to you today is my desire to serve as a liaison between you, the healthcare provider, and the people who suffer with ALS, as I am a person who has been on both sides of the fence.
I’ve had MANY careers in my life - raising my two boys (three if you count my husband!), worked as a software developer and a language interpreter, consulted for international businesses, volunteered as a cub scout leader, taught music for multiple instruments, built and repaired violins, studied to become a Respiratory Therapist, and worked as an RT for a couple of years ....then I was diagnosed with ALS. As you can tell, I’ve led a very active and busy life. I used to free dive for abalone every year, being able to hold my breath while going down 20 to 30 feet. I biked to work every day. I swam for good health. People used to ask me, “Is there anything that you haven’t done?”
How I became a Respiratory Therapist (which helps me live better today)
After growing tired of sitting behind a computer screen for over a decade, I was ready for a career change into healthcare, where I would be able to directly impact people’s quality of life. I decided to pursue a second degree at age 40.
Actually, my two boys, you might say, were the reasons why I became an RT. My first encounter with a Respiratory Therapist was when my older son developed severe asthma where he would turn blue. I would be frequenting the ER and eventually learn to manage my son’s asthmatic episodes. My second encounter with an RT was with my younger son who was born apnic, at seven weeks premature, and he needed ventilator support for the first couple of weeks of his life. I learned first hand that the Respiratory Therapists had an incredible role in helping people to breathe, which also meant giving life. Of all of the careers and the hobbies I mentioned, becoming an RT turned out to be one of the most gratifying careers that I have had. I didn’t realize it at the time, but it also turned out to be giving me lifesaving knowledge for myself.
I worked at Stanford Hospital, enjoying and building my work experience in both the ICU and ER, until I developed pain in my shoulder from a rotator cuff tear. I kept going to my PT sessions faithfully, hoping that I would get better. Eventually, helping nurses reposition adult patients was getting too hard for me. To give my shoulder some time to heal, in April of 2005, I switched to pediatric care, working at a long term residential care facility called Children's Recovery Center. Ninety percent of the kids there had been trached and been cared for on portable vents. I enjoyed the opportunity to work in trach care and to learn to perform actual trach changes. I also cherished the time when my job was to take these children out of the confinement of the four walls to a park with the vent, suction machine, and an E-tank in place. The nurses and I would even figure out ways to put the children into swings while they were connected to the vents and oxygen. I admired the children’s innate ability to live fully with their physical limitations.
I would not have guessed in a million years that I would be using a breathing machine or that I will be eventually facing a decision whether or not to be on a life support system. Ironically, what I experienced with my patients as a Respiratory Therapist gave me a unique glimpse of what it is to live a full and active life on a ventilator. In retrospect, all of the patients who have crossed my path, both big and small, have taught me a life’s lesson on having the courage to press on with my life.
Noticing Slow but Appreciative Changes
In the midst of working at Children’s Recovery Center, I also got a job at Lucile Packard Children's Hospital. After keeping up with my two job sites and 52 hour work weeks for three months, I was growing seriously concerned about my fatigability, having to catch my breath, and tripping while running up stairs. One day towards the end of my shift, I noticed that I had begun to have lots of trouble performing postural drainage therapy with manual percussion. While pounding on the patient's lungs for half an hour, my right arm simply dropped to my side. I called for a back up RT, saying that I wasn’t feeling well. I also began to not be able to carry heavier equipment.
During the summer of 2005, I decide to have my rotator cuff corrected with surgery, but I never recovered the strength that I had even before my surgery and continued to develop difficulties raising my arms. Looking back, my first symptoms of mild weaknesses were reduction in my right hand grip, experiencing difficulties opening a jar, feeling a bit strange with objects slipping through my hand, and difficulty in turning the car key.
That same year, on Thanksgiving Day, I asked my supervisor for a couple of solid weeks off for medical reasons to give me some time to really look into this muscle fatigue problem. It turned out that I never returned to work after that day. One evening around Christmas, I woke up with a panic attack and shortness of breath. Soon thereafter, the neurologist that I’d been going to for four years retired, and the young neurologist who took his place referred me to the ALS Center at UCSF. In January 25, 2006, I got my official diagnosis of ALS. My world as I knew it was never to be the same, turned upside down, where I became the patient and started this journey of living with ALS. On that day, I came home with a prescription for NIPPV (Non-Invasive Positive Pressure Ventilation) also known as BiPAP (Bilevel Positive Airway Pressure) ventilation machine, cervical orthosis, Riluzole (the only FDA approved treatment for ALS, promising to extend your life possibly by 3 months or longer), and a prescription for occupational therapy. What started as a mild right hand weakness, progressed to severely affecting both of my arms, neck, torso, and basically invading my body. In addition to growing muscle weaknesses, I experience constant muscle twitches, and tightness around my lower ribs as if in the grip of a boa constrictor. I also have difficulties brushing my hair, dressing myself, using utensils to feed myself, sitting up in bed, and walking any distance.
Living with ALS
The first six months, I struggled with trying to understand the diagnosis and the idea that my body was slowly but surely dying. Actually, my neurologist, Dr. Lomen-Hoerth at UCSF ALS Center, tells me that I have one of several variant forms of ALS, called Progressive Muscular Atrophy, which has a slower progression. In a nutshell, ALS is a rare and fatal degenerative neuromuscular disease with occurrence of one to two persons affected in every 100,000 people. The disease will rob a person’s ability to move, to breathe, to speak, to eat. Though much research is being done, on average people live between 3 to 5 years after their first diagnosis and usually die with respiratory failure.
It's sort of been like having a bad dream that you never wake up from, but guess what? I now have three and a half years of experience behind me living with this disease and good compliance using the bi-level ventilation machine, enabling my breathing muscles to rest whenever I feel necessary. To help illustrate what it is like to live with ALS, it is like trying to walk with 5 pound weights strapped to each of your hands and your legs and lying down and going to sleep with a 5 pound sack of rice sitting on top of your ribs or your diaphragm, making it difficult for you to take a decent size breath. And the 5 pound weights on each of your body parts start to feel like 10 pounds, 20 pounds, 30 pounds and so on, weighing you down, making it impossible for you to walk and eventually restricting you to the use of a wheelchair for the rest of your shortened life.
Giving a face to ALS
Without further ado, I would like to introduce you to three of my very close friends, who I came to know through the monthly ALS Association sponsored support group meetings. They were all very gracious to let me conduct an interview. First, I’d like to introduce Gerry (who does not experience respiratory issues), Kristina (who does use BiPAP), and then Jay (who is living on life support). When you meet these patients, it is important to keep in mind that ALS onset and the rate of progression varies from patient to patient.
My visit with Gerry – she has no respiratory issues at this time
I remember very well, one of my instructors at RT school often said to his students, put yourself in the patient’s shoes or try to treat your patients like you would your own mother. Whether you work in a hospital environment or a homecare environment, I think that the take home lesson as a healthcare professional is to show compassion and sensitivity to patients and to continue to learn and to educate yourselves about a disease that you are not so familiar with, so that you can become an advocate for your patient.
Patients have basically three options: 1) Do nothing, 2) Initiate BiPAP therapy, and 3) Go on a ventilator. Next, we have Kristina, who has been using her BiPAP for about eight months now. You'll notice that she uses her neck accessory muscles to breathe, and her speech has a short cadence with choppy phrases. She uses her BiPAP AVAPS machine at night and while she rests.
Kristina mentions about her growing financial concerns. She is in her 30’s and married to a loving husband who also has to work as well as be a caregiver to her when he comes home. Six weeks after this video was taken, Kristina has had her trach and her feeding tube surgery.
For the last video we have Jay, who’s been on a Pulmonetics LTV 900 ventilator for a little over a year. He has no movement of his body, except for his eyes, using an Eyegaze communication system to communicate. Jay was using a BiPAP unit 24 hours, 7 days a week, before the decision to move to a ventilator. Jay’s room looks like a hospital ICU unit. Jay is also an accomplished musician and a composer, and he is still composing using his Eyegaze system. The music you will hear on this up coming video is a recording of him playing one of his compositions prior to his ALS diagnosis. His wife, TJ, speaks on behalf of Jay.
Note: Jay's movie was shown at the CSRC conference. Unfortunately, the movie is not available online. Instead, here is a link to another movie about people with ALS.
Transition to the Ventilator
Going on the ventilator with tracheotomy becomes an option when using BiPAP becomes inadequate and patients become at risk of mucus plugging followed by rapid deterioration and aspiration pneumonia.
"Approximately 1% of patients in North America decide on long-term ventilation, usually younger patients with small children or more affluent patients who can afford the care required. In other countries when mechanical ventilation is strongly recommended by physicians and there is strong social support for families, such as in Japan, ~50% of patients with ALS are maintained on long-term ventilation, making the prevalence of ALS in Japan as high as Parkinson's disease."
Catherine Lomen-Hoerth, M.D., Ph.D. Director, ALS Center at UCSF, Department of Neurology. Semin Neurol. 2008;28(2):205-211. © 2008 Thieme Medical Publishers
Traditionally, a physician's and family's decision to have a patient placed on a ventilator is based partly on the ability of the patient to eventually be weaned off the ventilator. With ALS patients, however, once they are placed on a vent, they cannot ever come off of it. ALS patients, in most cases, have their minds intact and don't have metabolic issues or organ failures. If depression does not take over their spirit to live, and the patient is able to adjust to a computer aided communication device, then patients can do well living on a ventilator. Patients must be informed of the fact that once they are placed on a vent, they are not going to ever be able to come off from the ventilatory assistance, unless they make a decision to discontinue their life support system. Patients need to be informed that they are also at risk of getting themselves stuck in a "locked-in-syndrome", where they become unable to express their wish to be taken off the ventilator.
When patients start to have trouble with BiPAP, most of the patients likely are starting to think of perhaps end of life issues. The cost of living with ALS on a ventilator is roughly around $200K to $300K per year. This price tag can be reduced significantly, if one's spouse is available to provide care. However, it becomes virtually an impossible task to provide 24/7 care without risking sleep deprivation of a caregiver.
The need for a patient to transition from BiPAP to Ventilator will occur when the patient's vital capacity falls significantly and the patient's swallowing is affected. Patients often will start complaining of having excess saliva, significant shortness of breath, and problems swallowing. Then the management of the ventilation with BiPAP becomes challenging due to the thickening of phlegm. Patients often become unable to protect their airway and will feel like they are choking when they aspirate food particles or their own saliva, and are unable to have any productive coughs due to the paralysis of the diaphragm. Patients reaching this point often are pressed with the reality of needing a feeding tube (also known as a PEG) to provide sufficient intake of calories and fluid.
Also with compromised finger and arm strength, patients often become unable to manage putting on and taking off their BiPAP interfaces. Without a caregiver present, people living alone face a bleak situation not even being able to use BiPAP efficiently.
[Short demo different types of masks/interfaces]
Nasal interfaces. Klingon...
Full mask interfaces.
Demos and power point.
Pros and Cons of these masks.
Now, I would like to read a testimonial as to what breathing well does for a person. This person experienced the need to move from using BiPAP to be on a ventilator.
(Archive from “Living-With-ALS” Yahoo forum. Written by Debbie from Amesbury, MA)
I had my trach done electively when my FVC was less than 16%.
What a relief it was to breathe again and have my energy back.
I've had my trach for almost two years now and don't regret my decision at all. I am 46 and have a wonderful husband and 4 beautiful children ages 24, 22, 20 & 13. I need them as much as they need me. I am still a wife and mother. They come to me for advice and homework help. They laugh with me and cry with me. I can't imagine life without them.
Sure, I get frustrated at times and wonder if I made the right choice but the feeling never lasts long. I was on the bipap 24/7 for about 5 months prior to my trach. I hated the bipap on my face all the time and to be honest, I was embarrassed to wear it in public. The way people would stare and chuckle at me always made me feel uneasy. I also had no energy while on the bipap.
Once I was trached, I felt my dignity return. I had a lot more energy. I wanted to go out more. I felt free from the monster that took over my face. People were no longer laughing at me. Instead, they would smile and wave hi. I felt alive again.
I also keep in the back of my mind that a ventilator is not permanent and I still have control over my destiny.
Owning a second BiPAP unit should be made a standard for ALS patients for two reasons:
1: It is life support.
This disease is NOT sleep apnea. ALS patients have constantly declining progressive diagraphragmatic muscle weaknesses and are suffering from shortness of breath, muscle spasms, and fatigue. The patient usually starts using the BiPAP machine at night for 4 to 8 hours, and eventually adds a couple of hours of usage during the day, to rest their diaphragm. The BIPAP usage then gets stretched out to 16 hours a day, and then eventually to 24/7. During this time, since the insurance company allows only one BiPAP to be covered, the patient's life activities start to get limited to a 6 ft or 10 ft radius of the length of the BiPAP tubing. Imagine living feeling like a dog tied to a post. Isn't this kind of care a bit cruel, to say the least?
2: The disease itself prevents carrying the unit.
At times, my arms feel like that of an orangutan, just hanging by my side. I have difficulty going out to grocery shopping, cooking for my family, lifting a plate, or carrying much of anything. With this very limited arm strength, it would be impossible to drag this whole BiPAP setup, weighing about 9 lbs, to wherever I want to go outside my house, or even inside my house. I believe that every ALS patient should be allowed to have a portable unit that is covered by the insurance company.
Show/Demo.... You will often see me wearing this BiPAP while driving on the freeway, and some of the drivers look like they just saw an alien! This unit allows me to freely travel without the worry of not having the machine to rest my diaphragm when I need it. Otherwise, I would be confined to the four walls of my bedroom This portable unit that I have also allows me to use the unit during airplane flights. It uses the same lithium-ion battery that a laptop computer uses, and the airlines have no problems with that. I have also used this unit on many camping trips, where electrical outlets are unavailable. As you see, my world is no longer limited to the length of the tubing.
If there is anyone in the audience that can help me write a legislative bill to change the limitation of the provision of not allowing the second BiPAP unit for ALS patients, to be covered by Medicare, please come forward to speak with me today. Thank you.
We must consider the ways in which BiPAP machines are being used by ALS patients. Again, these are not your sleep apnea patients. When patients become unable to stay off of the machine for more than a few minutes at a time, due to shortness of breath, it is used as a ventilator, like a life support machine.
A second unit can also act as a backup unit in case of emergency when the main unit fails.
Improving Home Care
The confusion on use of BiPAP seems to be a widespread problem among healthcare workers. It is sad to report my own experience, but 9 out of 10 general physicians I’ve met do not know what a BiPAP is, but they seem to have heard about a CPAP machine, which is used for treatment of sleep apnea. A CPAP, or Continuous Positive Airway Pressure, device provides one level of pressure that one breathes against in order to keep the airway open. This aids in unobstructed breathing, reducing or preventing sleep apnea. CPAP pressure is basically the expiratory pressure that you exhale against. CPAP machines are generally contraindicated for ALS patients because it significantly increases the amount of muscle effort needed for expiration.
In the market, there are many potentially very confusing acronyms for these machines: AVAPS, BiPAP, CPAP, dog-pap (oops! This one does not really exist), and there are also AutoPAP, VPAP, and so on. On top of this, there are different modes such as T for timed, S for spontaneous, and S/T for spontaneous timed. Patients with ALS need a BiPAP (which is a machine that provides two levels of pressure: Inspiratory Positive Airway Pressure (IPAP); and a lower Expiratory Positive Airway Pressure (EPAP) for easier exhalation. The two most important BiPAP features for ALS patients are IPAP, because this pressure provides the inspiratory breath that the patient requires, and the S/T mode, which gives the "backup" rate to ensure that patients still receive a minimum number of breaths per minute if they fail to breathe spontaneously. ALS patients do eventually become paralyzed, as will be seen on Jay’s video, and this S/T mode is critical.
I’ve read about a number of cases where an ALS patient was prescribed a CPAP or an AutoPAP (basically a self titrating CPAP machine), which is contraindicated, or prescribed a BiPAP machine without an S/T mode.
A number of patients also have had compliance issues, expressing difficulties breathing comfortably with their BiPAP. Often, patients initially feel that the mask is uncomfortable to wear and creates a feeling of claustrophobia, suffocation, or frustration. It is critical for patients to learn to comfortably breathe with the machine and to establish successful usage of BiPAP in the homecare environment at the first sign of respiratory muscle weakness. For the safety of the patients, I think it would be prudent to require certain qualifications for homecare RTs, such as having worked in an ICU for a certain number of years. It would also be good if health insurance allowed extra visiting time and multiple visits with neuromuscular patient populations.
In my opinion, if a patient has an unsuccessful introduction to BiPAP, without proper education, encouragements, and support for the first three months by a highly qualified RT, then the patient may face decreased quality of life with shorter life span.
(Show patient A’s Pattern of Use data: Encore Pro software)
(Pictures of different type of masks/clips)
It was difficult for me to put this presentation together for the CSRC Convention, as I see myself in these patients with where I’m headed with my own disease progression. Though, however slow the progression for me may be, it is like a package that you send across the country. If you get a FedEx overnight service, you’ll get there quickly, but mine is like a US postal service with a wrong zip code that takes three times longer to get to the final destination.
Having met and exchanged e-mails across the country with several hundreds of people with ALS over the three and a half years of the course of my own disease progression, I personally got to know what it is like to live with a diagnosis of ALS without having much hope for a cure to arrive within my life time.
It is not an overstatement to say that to receive a diagnosis of ALS is like being “sentenced to a life in prison without the possibility of parole." Your body slowly becomes paralyzed, even as your mind remains unchanged for the most part.
While your mind is getting trapped in your body, one must learn to accept his/her own imminent mortality along with losing independence and dignity.
I have already accepted that this is the card I was dealt, and like the movie “Shawshank Redemption," there is one guy who was in prison who had committed no crime, and from the inside of the prison cell, which is much like living with ALS, he says, you either “Get busy living or get busy dying.” So…you probably know which one I picked! I chose to get busy living and helping others with my knowledge as a Respiratory Therapist and spreading awareness of ALS along the way.
Copyright 2009 Juri Kameda. All rights reserved. Contact: email@example.com