INTERNATIONAL VENTILATOR USERS NETWORK
an affiliate of Post-Polio Health International
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VOLUME 32, NUMBER 1
Audrey J. King, MA
The most recent Margaret Pfrommer Memorial Lecture in Long-Term Mechanical Ventilation was awarded to Audrey J. King and was presented on October 30th at CHEST 2017 in Toronto, Canada. This was the 17th year the lecture was given at the annual meeting of pulmonologists to educate physicians about home mechanical ventilation.
Part II - I asked other community living ventilator users what they’d like me to share with you today that could enhance your profession and practice, going forward. Without exception, they wanted you to realize the value and quality of their lives. They appreciated being asked and thereby also involved in this lecture..........MORE
Adolf Ratzka, PhD
Adolf Ratzka, PhD, founder and director of the Independent Living Institute in Sweden, has posted two new videos on YouTube. The first is entitled “Mechanical ventilation: my journey from Iron Lung to CPAP mask.” The follow-up video is entitled “Breathing and talking with CPAP mask and ventilator.” Adolf has been using some form of mechanical ventilation since contracting polio in 1961 at the age of 17, whether it be an iron lung, a rocking bed, a chest cuirass or more modern volume ventilator. In the past few years he has begun experimenting with using ready-made CPAP masks............MORE
Vol. 32, No. 1, February 2018
Editor: Brian Tiburzi
Designer: Brian Tiburzi
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When Air Becomes Breath: The 2017 Margaret Pfrommer Endowed Memorial Lecture in Home Mechanical Ventilation
Audrey J. King, MA, Toronto, Canada
Audrey J. King
This past year's Margaret Pfrommer Memorial Lecture in Long-Term Mechanical Ventilation was awarded to Audrey J. King and was presented on October 30th at CHEST 2017 in Toronto, Canada. This was the 17th year the lecture was given at the annual meeting of pulmonologists to educate physicians about home mechanical ventilation. Established in 1999 by Dr. Allen Goldberg and Dr. Eveline Faure, the lecture honors polio survivor and advocate Margaret Pfrommer from Chicago, Illinois. Pfrommer, a quadriplegic from polio, spent part of her life in a nursing home after her mother’s death. This experience compelled her to become an advocate for herself and for all those with significant disabilities. The purpose of the endowment is to honor an individual who possesses superior knowledge of home mechanical ventilation (HMV) and who promotes the health professional/patient partnership.
When Air Becomes Breath - Part II
I asked other community living ventilator users what they’d like me to share with you today that could enhance your profession and practice, going forward. Without exception, they wanted you to realize the value and quality of their lives. They appreciated being asked and thereby also involved in this lecture.
Adolf Ratzka, Sweden
(post-polio, vent-dependent since polio at age 17)
Among other things, Adolf is responsible for the Swedish Personal Assistance Act of 1994 which has saved the Swedish government €3 billion by creating a viable alternative to traditional institutionalization and home help services. Today, over 16,000 people receive cash benefits (Swedish population 9.5 million). He is often consulted by those in other European and Asian countries. He says:
“I wouldn't call it wisdom but most of us who have been living with equipment to compensate for underventilation become experts on own needs. We know what we want to do with our lives. All we need is the medical and technical expertise we might not possess ourselves for making the right adjustments to our lifestyle and for having the best equipment to support the lifestyle we aspire to.
“If I hadn't had the technical interest of friends and assistants helping me tinker with tubes, plastic materials and glues, I would have had to make do with equipment that was unnecessarily bulky, heavy and inconvenient. In fact, I might not have lived that long without these adaptations. For a better quality of life, our group needs more help.”
Adolf and I first met at that groundbreaking 1981 conference. We have spoken together in St. Louis, Munich, Sweden and, most recently, Japan as guests of the Japanese Ventilator Users Network.
Catherine Frazee, Quebec, Canada
Catherine is the distinguished career woman who recently seamlessly created her keynote speech in over 20 different recorded sections that I mentioned in Part I. She is an educator, writer, and activist; retired Profession of Distinction from Ryerson University. She was Ontario’s Human Rights Commissioner, has three honorary degrees and is an Officer of the Order of Canada.
“Share knowledge, not just information. It’s helpful to leave an appointment with test results …but what stands out is when it’s given with knowledge and in context. A decline of 15% is discouraging but I don’t feel defeated when I learn he has patients whose respiratory capacity is 0%. That tells me that life is still livable. It’s a challenge, not a defeat. It is HOPE.
“Appreciate and respect the limits of our own knowledge. When a patient asks if something will be possible, do not answer ‘NO.’ Give the more honest, ‘NOT TO MY KNOWLEDGE, BUT….’ Surprisingly favorable outcomes have come on board with someone’s hunch and a search.”
Justin is a young man with central nuclear myopathy who has been invasively ventilated for seven years. He has a home-based business in graphic design.
Justin Robshaw, Toronto, Canada
“The biggest barriers,” he says, “are lack of genuinity (not being straight forward), lack of listening and understanding, receiving little information and lack of caring. Having patience, understanding and listening to the patient’s issue(s) matter a lot. My thoracic surgeon is always reachable by phone even when he’s out of town and even out of the country. He’s very kind, has lots of patience, is a great listener and always goes out of his way to solve my various respiratory issues. I’ve been extremely lucky to have him.”
Ing Wong-Ward, Toronto, Canada
Ing was a prominent producer at the CBC, Canada’s national radio, and is now Associate Director at the Centre of Independent Living in Toronto.
Ing is the mother previously mentioned with the high-risk pregnancy. Presently she is battling a complex colon cancer that has taken her, so far, through a bowel rupture, emergency surgeries and 28 sessions of radiation. She says:
“My SMA makes everything trickier, but my new doctor's willingness to talk about my care and acknowledge its complexity with me is critical. Had they not, I honestly believe my life would have been put in real danger. They enabled me to voice my needs. Sometimes, I did have to yell. Including my husband to assist me has been invaluable.
“As for what I want doctors to know – there's so much to say, but my bottom-line message is, there has to be strong, two-way communication. There is real subtlety in everyone understanding one another. Doctors may need to take more time with people with disabilities to assess frustration vs. depression vs. suicidal tendencies, if they are in that state.
“I also feel strongly that medical people need to know what is available in community support. I'm amazed at what they don't know. I get it that doctors need to focus on medical care, but they should at least be able to refer people to knowledgeable people in the hospital system to access supports. Medical care has to extend beyond blood tests, etc. People need to know how they can live and often, their first point of contact to a new life is the doctor.”
Medical Assistance in Dying (MAID) legislation, which is happening in many countries, is deeply concerning to many ventilator users. Some, like Catherine Frazee and Baroness Jane Campbell, Life Peer and Cross Bencher in the UK House of Lords, feel a responsibility to protect vulnerable people with disabilities and are involved at the highest government levels.
Baroness Jane Campbel of Surbiton, UK, DBE (SMA), who is also a Dame Commander of the Order of the British Empire speaks passionately on the topic in the UK House of Lords:
Here she is reacting to the movie You Before Me:
“We’ve heard the last three years about assisted dying and people should have the choice to end their lives if they become dependent and don’t want to live anymore because they cannot do the things they used to do. Do you really think that’s reality? No, it isn’t! Most disabled people are getting on with their lives, and they enjoy their lives. Why on earth can’t we be treated as normal members of the public. We have decent lives. We have families. We have children. We don’t think about dying all the time. And, yet again, we have another film that is basically saying the same old thing – that if you’re in a wheelchair, you’re tragic, you’re brave.
“Go and make friends with disabled people and enjoy the reality of our lives. Not the crap which says ‘it’s tragic.’ I’m sorry that’s simply not the truth and we’re sick to death of it.”
A recent newspaper editorial from Australia succinctly sums up the fear of the “slippery slope” (“Flawed assisted dying bill puts vulnerable people at risk”, Sydney Morning Herald, Oct. 11, 2017).
The editorial asserts that assisted dying may be a solution for the terminally ill, but puts at risk many other vulnerable people:
People who have “decsion-making capacity” to understand the requested drugs will kill them but whose judgment is impaired by depression (e.g. during serious illness) or mood disorders.
People who are not strong-minded and are frightened by their disease or failing mental powers.
People who don’t want to “be a burden.”
People whose apparently voluntary request for assisted suicide is manipulated by the undue influence, misrepresentation or unconscionable conduct of others, including family and carers.
The sense of being “lesser valued”, “devalued” has led to the fear that in situations of illness, frustration or depression it might be all too easy to get talked into medical assistance in dying.
Ing Wong-Ward, in her current struggle with cancer, states:
“As for MAID and doctor-assisted dying, my own experience [has been that] no one has brought this up, even with my cancer. That's been a relief. I am pleased the focus has been on supporting my LIFE.
“I would also make the case that every person has biases. Some doctors have friends or family who are disabled and see them thriving. Others do not know anyone with a disability outside the medical realm, so their perceptions will be shaped by stereotypes of disability. We who are disabled know those stereotypes about our quality of life. This is why we fear the broader implications of MAID. It’s this fear that has driven some of the ventilator users I mention to become involved at the highest government levels in reacting against MAID. In groups such as Not Dead Yet, they see ventilator users as potential ‘targets’.…especially during resource cost cutting, during times of illness and vulnerability.
“While there are many people who cannot imagine living with a ventilator, there are many more who do and learn to embrace life. We do not hear those stories. We only hear of people wanting to ‘die with dignity’ rather than those who are living with dignity. Those stories matter greatly in shaping the public discourse around what is a meaningful life.”
Personally, I have experienced the DNR loss “without their consent” of three disabled friends during episodes of severe illness. All three lived actively and contributed to their communities. Their families misinterpreted the value of their lives. They did not know them well enough. Assumptions were made. We need ongoing conversation, greater communication with family, friends and physicians about this “elephant in the room.”
In your role as specialist physicians, your highly-skilled medical expertise is mostly just assumed, taken for granted. But, it seems that even greater “optimum” care can be reached by growing and enhancing the “partnership” relationship between physician and patient.
Ing encouraged me to share her own “best example” of a recent good interaction. She describes it as follows:
“I was being prepped for a much-needed blood transfusion. The resident was having a hard time finding a vein. I reached a low point and wailed about how awful it was that I have colon cancer and a severe bacterial abscess that was making me so severely ill.
“The doctor replied, ‘it isn't fair...’ and I feared he was going to say something about my disability. However, he said, ‘you have a beautiful family and you've overcome so many obstacles already.’ I appreciated his acknowledgement of my humanity, first and foremost.
“I have noted that this new generation of doctors seem willing to listen to me and, if needed, my husband when I cannot speak for myself. They are smart enough to see that disability does affect medical treatment and have talked it through with me, and they can see I have a good life.”
In putting this talk together, I thought about my own experiences with chest physicians and asked myself who made a difference, how and why? Surprisingly, my experiences also have more to do with relationships than with the science of medicine.
One frequently said, “I’m so proud of you. You’ve taught me all I know.” (That’s not at all reassuring!) He called me at home to ask if he could give my number to a patient just like me (...and we became the best of friends). I felt admired, respected, deeply encouraged. He understood and respected what’s involved in the life of a severely disabled person.
Another long-time respirologist simply tucked the blanket up under my chin when he thought I was asleep. To him I was a person - and he cared. That was a powerfully healing moment. I believe it’s called “The Art of Medicine.”
And so in summary, what makes for “excellence” in patient-physician relationships?
Greater awareness of the lives of community living home mechanical ventilation users. Their successes, realities, what’s important from their perspective of the quality of their lives. And, sharing this as appropriate.
Supporting patients and families to move beyond Dependence, into Apprenticeship, Partnership and Expertise modes of functioning.
Connecting HMV patients to peers, support groups and networks such as IVUN or Trach Mommas of Louisiana.
Never extinguish HOPE. “Hope is OXYGEN for the SOUL.” This means putting results in context, listening, encouraging, acknowledging, caring, respecting and supporting.
Advocate for and work with LTV patients, their families, organizations and supporters in getting the services the need to live fulfilling, quality lives within today’s world where liberating technology makes a life possible that was never before dreamt of.
Catherine Frazee sums it up best:
“Never underestimate how influential you are as health professionals in crafting the narrative of our lives. This is an immense responsibility, a responsibility which goes well beyond the clinical and technical matters in which you are so capably schooled. Never stop truer to get better at deep and patient listening. It's your mightiest healing power.”
MY JOURNEY FROM IRON LUNG TO CPAP MASK
Adolph Ratzka, PhD
Adolf Ratzka, PhD, founder and director of the Independent Living Institute in Sweden, has posted two new videos on YouTube. The first is entitled “Mechanical ventilation: my journey from Iron Lung to CPAP mask.” The follow-up video is entitled “Breathing and talking with CPAP mask and ventilator.”
Adolf has been using some form of mechanical ventilation since contracting polio in 1961 at the age of 17, whether it be an iron lung, a rocking bed, a chest cuirass or more modern volume ventilator. In the past few years he has begun experimenting with using ready-made CPAP masks. “Many of us could greatly benefit from nasal masks for daytime use but would not show themselves with a mask in public for fear of attracting unwanted attention,” Ratzka says. “We need to keep showing them the advantages, telling them that other people will get used to it once we've become comfortable wearing masks ourselves.”
CAMPS FOR VENTILATOR-ASSISTED CHILDREN 2018
CHAMP Camp 2018 will be June 17-21, 2018 at Bradford Woods, Martinsville, Indiana. All applications are due by March 1, 2018. Make sure to get in on time, and we hope to see you at camp! If you have any questions, please call 317-679-1860 or send an email to firstname.lastname@example.org.
TRAIL'S EDGE CAMP
Trail’s Edge Camp is a week long summer camp for children 5-18 years old who are ventilator-assisted, and is located at the Fowler Center for Outdoor Learning, a beautiful barrier-free camp on 200 acres in Mayville, Michigan. Check the website for an announcement of 2018's dates.
FRESH AIR CAMP
The 2018 VACC Camp will take place March 24-30.
Camp Inspiration is specifically for BiPAP and ventilator-assisted children and will take place at the beginning of June at Double H Ranch, Lake Luzerne, New York. More information is available on their website.
PA VENT CAMP
PA Vent Camp is held annually at Camp Victory in Millville, PA. June 23-28 are this year’s dates.
For more information contact 717-531-5338 or email@example.com.
Victory Junction enriches the lives of children with chronic medical conditions or serious illnesses by providing life-changing camp experiences that are exciting, fun and empowering; all in a medically-safe environment at no cost to the camper or their family.
Located in the hills of Randleman, North Carolina, Victory Junction is spread across 84 acres, allowing children to do what they do best— be kids, play, imagine, make friends and enjoy the adventures and experiences of camp life.
Victory Junction does not have a special week for children who use vents, but they do have Specialty Weekend, which is a perfect opportunity for children to come out and experience the camp with their families. This year, Specialty Weekend is April 13-15 (Fri-Sun).
Check out the Application Guides for Family Weekend.
The ADA and HR 620
On February 15, the House of Representatives passed the ADA Education and Reform Act (HR 620) by a vote of 225 to 192. Many disability organizations advocated against its passage, seeing it as an attempt to make it more difficult to enforce provisions of the landmark Americans with Disabilities Act (ADA). Under the bill, businesses that are found not compliant will have up to 120 days from the time it receives a legal notice from a person with a disability who is claiming harm and has detailed the specific violation to merely make “substantial progress” in removing barriers, with no deadline to actually resolve the issue. The bill now moves on to the Senate.
The Disability Rights Education & Defense Fund (DREDF) released a statement last week condemning its passage, joining others such as the American Association of People with Disabilities (AAPD), AARP, ACLU, and former Pennsylvania Governor and Homeland Security Director Tom Ridge.
Money Follows the Person Program in Jeopardy
Funding is quickly running out for a popular Medicaid program that transitions people with disabilities from costly institutions to home and community-based living.
Disability advocacy groups are urging constituents to call lawmakers to voice support for bipartisan legislation that would reauthorize the Money Follows the Person program. Since inception more than a decade ago, it has helped roughly 75,000 people leave nursing homes or intermediate care facilities in favor of their own apartment or a small group home.
RECENT RELEVANT PUBLICATIONS
Update on two recent FDA-approved therapies for ALS and SMA
Ashraf Elsayegh, MD, FCCP and Won Y. Lee, MD
Edavarone (Radicava) “was rapidly approved by the FDA, [but] there are obvious challenges that must be recognized. First, it is unclear if patients will discern such a mild degree of slowing of disease progression. In addition, the annual cost may be prohibitive, and lifelong IV administration of the medication for 10 days every month may pose logistical barriers.” Nusinersen (Spinraza) is the first FDA-approved medication for SMA. “the therapy is safe and tolerable, although....the eventual impact is uncertain, especially in cases of advanced muscle weakness. The are realistic challenges: the high cost ($125,000/dose), limited longitudinal evidence, technical administration, and limited access.”
Chest Physician, 2018 Feb; 13(1):55.
The Price Isn't Right: Breakthrough drugs for rare neurologic diseases are staggeringly expensive. We explain why and how to effect change.
“Although they are not cures, drugs like nusinersen, eteplirsen (Exondys 51) for Duchenne muscular dystrophy, and edaravone (Radicava) for amyotrophic lateral sclerosis (ALS) offer symptomatic relief for neurologic diseases once considered untreatable. But these new treatments come with annual costs that dwarf the prices of even the most expensive drugs already on the market, leading some insurance companies to do what the Callais' insurer did—deny coverage. Even with coverage, many families wonder how they will manage the high costs of what may be a lifetime of therapy.” Full article.
Neurology Now, February/March 2018, 14(1):40-45.
Health transition experiences of Canadian ventilator-assisted adolescents and their family caregivers: A qualitative interview study.
Dale CM, King J, Amin R, Katz S, McKim D, Road J, Rose L.
“Important opportunities exist to enable improvements in the transition experiences of VAAs and their family caregivers. To maximize service continuity during paediatric to adult transition, future research should focus on transition navigator roles, interprofessional health outreach and the needs of families caring for VAAs with cognitive and physical deficits.” More.
Paediatrics & Child Health, 2017 Aug; 22(5):277-81.
Cohort of patients initiated to home ventilation. Observational and prospective study.
Rabec C, Gonzalez-Bermejo J, Cuvelier A, Cervantes P, Foret D, Mounier L, Melloni B, Muir JF.
“The effectiveness of home noninvasive ventilation (NIV) for chronic respiratory failure (CRF) is well established. However, few data are available about home NIV prescription and utilization according to the different etiologies of respiratory failure. The ANTADIR Federation, in partnership with the Ventilatory Support Group of the French Speaking Pulmonary Society, has set up a national, observational and multicenter cohort study. The main goal of this study is to analyze the clinical data justifying home NIV prescription in patients with chronic respiratory insufficiency. The secondary objectives will be to assess: the evolution of comorbidities or their occurrence, hospitalizations, NIV compliance, dropout and survival.”
Revue des Maladies Respiratoires, Jan 2018; 35(1):88-93.
March 15 - 17, 2018, 3rd International Joint Meeting of the JIVD (Journes Internationales de Ventilation Domicile) and ERCA (European Respiratory Care Association), Cité Centre de Congrès, Lyon, France. See Final Program. www.jivd-aer.com
JIVD/ERCA Conference in 2018
Canadian Respiratory Conference 2018
FOCUS Spring 2018
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 now 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.