INTERNATIONAL VENTILATOR USERS NETWORK
an affiliate of Post-Polio Health International
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VOLUME 32, NUMBER 2
Lyon, France, was the site of the 3rd Joint International Meeting of Journées Internationales de Ventilation à Domicile (JIVD) and European Respiratory Care Association (ERCA) held March 15-17, 2018. Attended by over 1,100 people from more than 40 countries, the conference featured an array of lectures, posters and exhibits centered around the subject of mechanical ventilation...........MORE
I contracted polio when I was 4½ years old, and ever since, I’ve used braces and crutches to walk. My abdominal muscles were affected by the poliovirus but soon recovered. I was not placed in an iron lung. Even with braces, I was on the college swim team and still do cardiac workouts on a NuStep. I’ve never smoked. I didn’t see myself as someone who had breathing problems.............MORE
Sharing the work of others
Educate, discuss, take action
Recent Relevant Publications
Summaries, links to selected professional, disability, disease journals and newsletters
Conferences and webinars for health professionals and ventilator users
The business of living with a ventilator
Vol. 32, No. 2, April 2018
Editor: Brian Tiburzi
Designer: Brian Tiburzi
© 2018 Post-Polio Health International.
Permission to reprint must be obtained from Post-Polio Health International (PHI) at firstname.lastname@example.org.
Ventilator users, health professionals, non-profits, company representatives – send comments and updates to email@example.com.
Notes from 3rd International Joint Meeting of JIVD and ERCA
Lyon, France, was the site of the 3rd Joint International Meeting of Journées Internationales de Ventilation à Domicile (JIVD) and European Respiratory Care Association (ERCA) held March 15-17, 2018. Attended by over 1,100 people from more than 40 countries, the conference featured an array of lectures, posters and exhibits centered around the subject of mechanical ventilation.
The conference was organized around three main themes: patients’ experiences, innovative technology and improvement in the delivery of care. This year's meeting also featured four pre-congress courses aimed at "beginners" with such titles as "Home mechanical ventilation and chronic patients," "Acute NIV for beginners," "Home Ventilation in paediatrics" and "Organization of home mechanical ventilation: how to start a community program."
IVUN was represented by its executive director, Brian Tiburzi, in a panel workshop entitled, "How ventilator patients use the internet." Other members of the panel included Brit Hov, MSc, Oslo, Norway, Jessica Taytard, MD, Paris, France, and Imma Grau, PhD, Barcelona, Spain.
Brit Hov presented an e-learning tool for adolescents and young adults using ventilator support. It is also meant for their network and health professionals with little former knowledge of ventilatory support. It was originally developed in Norwegian but has recently been translated into to English. It is called “Pust” (Breathe).
Jessica Taytard spoke about a new smartphone app, OVNI, which stands for “Optimisation de la Ventilation Non Invasive” (Non Invasive Ventilation Optimization). The app was specifically developed to accompany, guide and support adolescents and young adults dealing with chronic respiratory insufficiency and long-term nocturnal ventilatory support. The aim was to create an interactive application that would allow patients to take ownership of their treatment, help them manage their appointments and prepare for their hospital visits, and reinforce interactions with their specialists.
OVNI, according to the developers, "will also allow doctors a better and more precise follow up, with less information loss and a better understanding of the patients’ quality oflife and treatment tolerance at home." It is currently available only in French.
Imma Grau's presentation focused on ways in which patients can better vet the information they find online. Grau is a patient advocate at Grup Agata Association in Barcelona.
IVUN, as an organization that has been in touch with the needs of ventilator users since the 1950s, is well-suited to talk about how the internet has brought about changes in how ventilator users access information, connect with one another, and manage their health needs.
IVUN traces its roots back to the Toomeyville Jr. Gazette, which began in the 1950s as a mimeographed newsletter for patients at Toomey Pavilion in Cleveland, Ohio, a respiratory center for polio patients. The Gazette helped patients stay in touch with one another after they left the center (see image to the right). As polio cases dropped dramatically after the introduction of the vaccine, the respiratory centers were closed. Those still using iron lungs or newly-developed portable ventilators became more isolated. Their needs expanded to included medical advice, help finding knowledgeable health professionals, information about assistive devices, help obtaining access to higher education or finding gainful employment.
IVUN (then known as Iron Lung Polio Assistance, Inc.) responded by publishing issues of the Gazette centered around topics such as employment, higher education, wheelchairs, communication and housing. Later, our Resource Directory for Ventilator-Assisted Living provided listings of health professionals, equipment, long-term ventilator facilities and respiratory home care companies that ventilator users could access to help meet their health care needs.
Toomeyville Gazette, September 1957
The internet era has brought enormous changes. A wealth of information is available for ventilator users at the click of a button. For those motivated to learn about HMV use, information is now easier than ever to access. Unfortunately, with good information comes a lot of bad information. In terms of education then, IVUN's role evolved from not only producing reliable information, but vetting and pointing ventilator users towards credible sources.
In general, the internet has the potential to make a positive impact on the lives of ventilator users in a number of ways, including:
allows users of HMV to self-publish content in order to raise awareness of their condition
allows patients and their families to become better informed about their care needs (PubMed, NCBI, Google Scholar)
allows patients to stay in closer contact with their healthcare providers
allows patients to seek out cheaper medication and equipment.
Perhaps the biggest change brought about by the internet has been on networking. The rise of social media has allowed ventilator users who may have felt isolated by their condition in years past reach out and connect to other ventilator through services such as Facebook and Twitter.
Our members who use home mechanical ventilation identified a number of advantages of social media:
helps spread awareness of their particular disease
makes it easier to find support from others with similar diagnoses, especially in isolated, rural areas
allows younger vent users a safe space in which to interact away from over-protective parents or health professionals
can talk about topics such as moving out, dating, alcohol use, sexual orientation, stress, and anxiety with others in their peer group
makes it easy to form advocacy groups and collaborate with others in support of a particular cause (health care reform; attempts to roll-back the ADA; CHIP funding).
However, there can also be drawbacks to using social media:
tendency for strong personalities to dominate a group/conversation
potential for being "catfished" by those pretending to be someone they're not
possibility for ill advice to be passed on that conflicts with informed medical opinion
people may seek quick answers rather than doing research on what the medical literature says.
Despite the dangers, social media platforms can provide a vital lifeline to ventilator users looking to connect with each other, especially in rural, isolated areas. It important to remember that the internet is a tool, but one that, if used wisely with the proper precautions, has the ability to improve the quality of life for users of home mechanical ventilation. ■
The program for the conference is available online. Check www.jivd-france.com/jivd/index.html later in May for additional resources that may become available.
My Experience with Mechanical Ventilation
I contracted polio when I was 4½ years old, and ever since, I’ve used braces and crutches to walk. My abdominal muscles were affected by the poliovirus but soon recovered. I was not placed in an iron lung. Even with braces, I was on the college swim team and still do cardiac workouts on a NuStep. I’ve never smoked. I didn’t see myself as someone who had breathing problems.
About 15 years ago, I became aware of potential breathing problems at a polio conference in North Carolina. It was pointed out to me that I should be checked for sleep apnea as this was common for people with post-polio syndrome. I underwent a sleep study and found that I was marginal for sleep apnea. The neurologist initially treated me with a drug for restless leg syndrome. But because both of my legs are paralyzed, and the drug was affecting my moods, I stopped taking it after a two-week trial.
Later, I went for a second sleep study and discovered that I could not exhale against the constant positive airway pressure (CPAP). The CPAP provides a constant air pressure to keep the airways open and avoid apnea. It was like blowing into a balloon, hard to exhale and easy to inhale. I persuaded them to do the sleep study using a BiPAP (Bi-level Positive Airway Pressure with higher pressure for inhalation), and they found settings which controlled my apnea. Because they also found that I breathed through my mouth, I now use a mask that covers both my nose and mouth.
I successfully used the BiPAP for many years. I could sleep on my left side with very few apneas and on my right side with just acceptable apneas. However, the BiPAP did not control my sleep apnea when I tried to sleep on my back.
At this point, I started going to a local pulmonologist. The pulmonologist sent me for all kinds of tests: an allergy test, swallowing test, and pulmonary function test (PFT). The PFT showed that I was marginal for asthma. The pulmonologist wanted to treat me with the standard battery of four different drugs for asthma. Since my lungs seemed to be okay, and because I was concerned about drug interactions, I said no to the drugs and asked to be treated only for sleep apnea. This led to yet another sleep study to determine conditions to sleep on my back. These conditions raised both the inhalation and the exhalation pressures on the BiPAP from 12/6 to 20/12. I tried the higher pressure settings for two nights and my sides began to hurt from the effort of breathing out against the high pressure. I immediately switched back to the lower pressure settings.
I switched to another local pulmonologist when the first one suddenly closed his practice. About a year ago, the second pulmonologist switched me from a BiPAP to a Trilogy ventilator. The Trilogy is a much more complicated and versatile machine than the BIPAP. It can operate in a number of modes, from simulating a BiPAP to functioning as a breathing machine for a tracheotomy. In addition, it has a battery backup in case of power failure. The Trilogy has a computer control. It was set in a mode to start my sleep with my BiPAP conditions and then to increase the pressures if I was having apneas. This sounded like a very good plan.
Unfortunately, going from the BiPAP to the Trilogy is not as easy as it sounds. First, the Trilogy has alarms, which cannot be turned off, to indicate when the machine detects a possible malfunction. For instance, when I yawn there is an air leak between my face and the mask. The computer detects a change in airflow and sounds an alarm. I also had problems in the AVAPS mode. If I was having apneas, it would increase both the inhalation and exhalation pressures. To do this, every five minutes it would switch from just blowing air into my lungs to adding a series of quick pulses of air to the flow. Five minutes later, it would switch back. The constant changing made it difficult to sleep. For the first two weeks, neither I nor the respiratory therapist knew how this mode worked. I was worried that the machine was malfunctioning. It took two weeks for me to go from:
“GOOD GRIEF, WHAT’S THE MACHINE DOING NOW? HOW WILL I CONVINCE ANYONE THAT IT IS MAKING THIS KIND OF INTERMITTENT NOISES WITH PULSES OF AIR? WHY DO THE PULSES STOP AND START? IS THE MACHINE BROKEN?”
“AHH, this is how it is supposed to work. Just relax and go to sleep.”
A change in perspective is a powerful way to increase one's comfort.
The second problem is that the Trilogy has a large LED display. The LED screen can be set to go dim, but, if an alarm occurs, it turns bright. Having acknowledged the alarm, the screen is supposed to then go dim. However, I found this only works some of the time, and often the screen stays bright. I finally called customer service. They explained that this was a “known problem” and told me to unplug the machine and restart it to get the display to dim. I was told that even this doesn’t work all the time, however. We returned at least two machines to the manufacturer because, after we described the problems to the service department, they believed the machine was malfunctioning. Only when I called the customer service number (which I found on the internet) did I find out that this is “the way it is supposed to work.” So, my workaround now is to keep a piece of black felt hanging over the screen to keep the room dark.
I used the Trilogy in this mode for several months even though I was not getting as good a night’s sleep as I had with the BiPAP. But, I was beginning to feel the effects of a lack of quality sleep. I decided to make an appointment with Dr. John Bach in New Jersey, even though he was a long drive away.
I had heard Dr. Bach speak at the 2014 PHI conference in St. Louis. Some of his main points are summarized in a video on Polio Place [see “Ways to Avoid Respiratory Complications of Postpoliomyelitis”]. More information can be found on Dr. Bach’s web site www.breatheNVS.com and in a recent open source article published in the Annals of Rehabilitation Medicine, “Noninvasive Respiratory Management of Patients With Neuromuscular Disease.” (I found the look up function on my computer very helpful to understand the meaning of medical terms in his open source article.)
His main points are:
People with PPS were likely to have good lungs but weak muscles. Even people with muscles so weak that they have no measurable ability to breathe have successfully used noninvasive ventilator support for up to 63 years.
People with PPS should not ever need a tracheostomy. Rather, noninvasive support may be used.
Conventional sleep management with oxygen and low span BiPAP (inspiratory pressure - expiratory pressure less than 10 cm H2O) instead of noninvasive support must inevitably result in respiratory failure and intubation for patients with progressive muscle weakness.
Noninvasive mechanical support can provide full respiratory muscle rest along with ventilator support.
People with muscle weakness as a result of polio should not use oxygen (unless there is another lung problem). Supplemental oxygen reduces be need to breath which causes carbon dioxide (CO2) to builds up. The high CO2 leads to major problems. Continuous CO2 monitoring is needed if oxygen is given to a PPS patient!
The normal pulmonary function tests are designed to evaluate lungs and airways rather than muscle function. Poor muscle function is detected by CO2 measurement, spirometry (air capacity of the lungs) in both a seated and a prone position, cough peak flows and oximetry.
A weak cough peak flow can lead to pneumonia because the cough flow is inadequate to clear airway mucus. A mechanical “Cough Assist” can help clear airways.
My goal for the appointment with Dr. Bach was to get better settings for the ventilator. Since I often can feel mucus in my airways that is difficult for me to clear by coughing, I was also interested in finding out about the Cough Assist.
I first contacted PHI to get contact information on Dr. Bach and then sent him an e-mail describing my problems. He replied with the suggestion that I make an appointment. He is located at Rutgers University Hospital, Newark, NJ, about 500 miles from where I live in West Virginia.
I had given my local pulmonologist some of Dr. Bach’s articles. When I decided to visit Dr. Bach, she was very helpful and referred me to him and sent along my records. We made an appointment when the weather was expected to be good and stayed at a hotel recommended by the hospital. The hotel even gave a “hospital discount” when I told them I was coming for a doctor’s appointment. The hotel was near Newark Airport and had an international clientele. It was fun to see the different styles of clothing. We were able to have dinner with some of my family who lived in the area the night before the appointment.
The appointment lasted about an hour. We discussed my condition. I had filled out the
“My Polio Life” form developed by Polio Survivors Network in the United Kingdom which allowed me to document the effect of polio over time. I think it helped speed the conversation. My pulmonologist’s office had emailed my records ahead of time, but they somehow got deleted or misplaced by Dr. Bach’s office. Fortunately, I had checked with his secretary just before we left and brought along a hard copy. ALWAYS TRAVEL WITH A HARD COPY!
Dr. Bach tested my lung capacity in both the sitting position and lying down and gave me a prescription for new settings on the ventilator. He also tested my cough flow and was surprised at how weak it was. He gave me a prescription for a Cough Assist.
I passed the prescriptions to my local pulmonologist and respiratory therapist. The new setting called for an “active circuit pressure control.” Using this mode, the machine blows air at a set pressure into my lungs when I inhale and has zero pressure when I exhale. If I don’t breathe often enough, it automatically blows air to cause me to inhale. The new settings work much better for me (even better than the BiPAP). The Cough Assist works wonderfully. It blows air into my lungs to fully inflate them. Then, when I throw a switch, it suddenly sucks the air out. It allows me to have a greater peak flow and helps clear out mucus from my airways. When I begin to feel mucus building up, I just go in and use the Cough Assist a few times to remove the mucus and then my lungs feel better! It really demonstrated its value recently when I had the flu. I was able to loosen mucus in my lungs and remove it by coughing.
Thank goodness that PPS generally has a gradually decline in abilities so that we have some time to plan for our next stage of decline and that there are doctors who are willing and able to give us assistance. We need to have determination and persistence to get the treatments we need. ■
Parent Project Muscular Dystrophy (PPMD)
Parent Project Muscular Dystrophy (PPMD) has announced a new name and look for their endurance program. Formerly "Run for our Sons," the program will now be called "Race to End Duchenne." PPMD explains that "we aren’t just parents racing on behalf of our sons. We are aunts, uncles, grandparents, brothers, sisters, co-workers, friends – and we’re racing for every single person with Duchenne."
PPMD has a limited number of spots to participate in the New York City Marathon on November 4, 2018. This race is the biggest and best in the country, drawing elite runners and novices alike to experience the five boroughs of NYC in a unique way. The only remaining way to get in is via a charity entry. Find out more about this opportunity.
CHEST Foundation: Giving the Public Access to Lung Health Experts
CHEST Foundation engages CHEST members and local communities in a variety of lung heath community outreach efforts. Their lung health expo brings local respiratory therapists and leading lung health experts to the public in a fun and inviting, carnival-like atmosphere. This event allows members of the community to connect with their local lung health champions so that they can begin the conversation about their lung health outside of the clinical setting, get their lungs screened by a respiratory therapist or pulmonologist, learn about different disease states, and gain empathy for those ailed with conditions and diseases like asthma and COPD. In 2017, the Lung Health Experience toured through various cities in the United States and Canada, starting with an event in Oklahoma City. Learn more about CHEST Foundation's community service efforts.
Two Helpful Resources from ASAN
The Autistic Self Advocacy Network (ASAN) has put together a couple of very good resources that may be useful to ventilator users, as well. Accessing Home and Community-Based Services: A Guide for Self Advocates was designed to help people with disabilities find and use resources that empower them to live and be in charge of their own lives in their own homes and communities with the supports that they need. The handbook explains Medicaid home and community-based waivers, how to access them, and what alternatives are available for people who don’t qualify for certain government programs. It also has information about self-directing your support services, and outlines different options for supports you can use for housing, employment, daily living, and accessing your communities.
The other booklet is A Self-Advocate’s Guide to Medicaid. Medicaid is the biggest health care program in the country, and one that many ventilator users rely on. But a lot of the information out there about Medicaid and attempts to change it can be difficult to understand and navigate. This resource was developed by ASAN in collaboration with the Autism Services, Education, Resources and Training Collaborative (ASERT), and with funding from the WITH Foundation.
Ed Department Set To Abandon IDEA Site That Crashed
The Education Department said that visitors to the legacy site, idea.gov.ed, will be automatically redirected to a newer incarnation, http://sites.ed.gov/idea, beginning April 30, 2018.
The website was at the center of a firestorm early last year when the Education Department’s central spot for information on the Individuals with Disabilities Education Act (IDEA) went dark.
Jim Harrison Lives Life to the Fullest Thanks to World-Renowned Pediatric Diaphragm Pacing Program at Children’s Hospital Los Angeles
“In 1977, Childrens Hospital Los Angeles (CHLA) took a bold step forward and sent its first patient, an infant with Congenital Central Hypoventilation Syndrome (CCHS), home, where he was on a mechanical ventilator and cared for by his family. CCHS is a rare, lifelong condition where a person cannot breathe on his own due to a lack of nerve signal from the brain to the lungs to breathe. There are approximately 1,000 cases in the world and there is no cure.
“Since then, CHLA has established itself as a leader in the field by having one of the largest pediatric diaphragm pacer programs in the world and one of the largest home mechanical ventilation programs in the world.... CHLA patient Jim Harrison is one of the patients who has benefitted from the program. Harrison, now a 29-year-old San Diego-based chartered financial analyst, was diagnosed at infancy with CCHS. At 18 months he had surgery for a diaphragm pacemaker, which receives electrical impulses from a breathing unit stored in a small backpack he wears during the day to his diaphragm to help him take 22 breaths per minute. At night, Jim takes the backpack off and connects to a ventilator via his trachea tube to help him breathe as he sleeps. The routine, which he has followed since he was a toddler, hasn’t deterred his desire or his ability to do whatever he wants.”
Respiratory Care in Neuromuscular Diseases.
Sahni AS, Wolfe L
“Caring for patients with neuromuscular disease (NMD) is challenging. Respiratory care is of the utmost importance because it is a major determinant of quality of life and survival. Noninvasive ventilation (NIV) is one of the few modalities that has shown survival benefit in the NMD patient population. Newer modes with smart technologies are being developed to assist in better ventilation. Some noninvasive methods have shown success in the management of sialorrhea, which is of paramount importance in the initiation of NIV. This review will summarize the management of respiratory symptomatology in patients with NMD with recent advances made in NIV.”
Respiratory Care, May 2018, 63(5):601-608. DOI: https://doi.org/10.4187/respcare.06210
Understanding the use of NIV in ALS: results of an international ALS specialist survey.
Heiman-Patterson TD, Cudkowicz ME, De Carvalho M, Genge A, Hardiman O, Jackson CE, Lechtzin N, Mitsumoto H, Silani V, Andrews JA, Chen D, Kulke S, Rudnicki SA, van den Berg LH
“Given the impact of NIV on respiratory function and the importance of respiratory function to quality of life and survival, understanding differences that influence NIV prescribing is critical. This information may inform future study design and identify areas warranting additional research to develop best practices for NIV implementation.”
Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, Published online: 16 Apr 2018. DOI: https://doi.org/10.1080/21678421.2018.1457058
FOCUS Spring 2018
May 3-4, 2018, Friday, Guest House at Graceland Hotel, Memphis, Tennessee. View the brochure or visit their website to register.
American Thoracic Society 2018
May 18-23, 2018, American Thoracic Society's 2018 International Conference, San Diego, California.
CCHS Family Conference 2018, alongside The 5th International CCHS Conference
June 20-23, 2018 at The Chase Park Plaza, St. Louis, Missouri, USA. Registration is open.
FOCUS Fall 2018
September 14-15, 2018, Fall FOCUS, Hotel Irvine, Irvine, California. More details.
ERS International Congress 2018: Paris
September 15-19, 2018, Paris expo Porte de Versailles. The abstract submission deadline is Februrary 15th. Find up-to-date information here.
October 6-10, 2018, San Antonio, Texas. The CHEST 2018 Annual Meeting will offer more than 400 general sessions, postgraduate courses, simulation education sessions, original investigation presentations, CME/CE credits and MOC points for hundreds of sessions and more.
Registration is now open.
ATS Foundation and ResMed Announce Two-Year Research Grant in Sleep-Disordered Breathing
The ATS Foundation announced its new ATS Foundation/ResMed Research Fellowship in Sleep-disordered Breathing and PAP Therapy. The award will provide funding for two years in the amount of $100,000, provided by ResMed, a global leader in connected health with more than 4 million cloud-connected PAP devices monitoring patients every night.
“This award can help shine a brighter light on sleep apnea – a public health crisis – and PAP therapy’s role in addressing it worldwide,” said Carlos M. Nunez, MD, ResMed Chief Medical Officer. “When 85 percent of people with sleep apnea still aren’t diagnosed, ResMed is proud to support any research effort to accelerate finding, testing and treating them so they can enjoy a full night’s rest, better overall health and a significant reduction of life-threatening risks.”
The Fellowship application process will open April 26, 2018. The two-year funding period is from December 2018 through December 2020. Applications will be reviewed by the ATS Scientific Advisory Committee for their scientific merit, innovation, feasibility, and relevance to sleep-disordered breathing and PAP therapy.