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VOLUME 32, NUMBER 3
I am 45 years old with a severe form of limb-girdle muscular dystrophy. Limb-girdle usually starts with muscle weakness in the hips and shoulder areas and those closest to the center of the body. As it progresses, it can affect all the muscles, even those involved in breathing. It has many subtypes and my particular subtype is faster progressing than many of the others. I started showing symptoms at age six and required the use of a power wheelchair by age 12............MORE
Norma MT Braun, MD
It is noteworthy that D., a 45 year-old woman with neuromuscular chronic respiratory failure, is and has been successfully using a pneumobelt ventilator for daytime upright ventilation for about 15 years. Her fear is when her system becomes worn there will be no replacements..............MORE
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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. 3, June 2018
Editor: Brian Tiburzi
Designer: Brian Tiburzi
© 2018 Post-Polio Health International.
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a forgotten option
I am 45 years old with a severe form of limb-girdle muscular dystrophy. Limb-girdle usually starts with muscle weakness in the hips and shoulder areas and those closest to the center of the body. As it progresses, it can affect all the muscles, even those involved in breathing. It has many subtypes and my particular subtype is faster progressing than many of the others. I started showing symptoms at age six and required the use of a power wheelchair by age 12. It was at that time that I also required surgery to correct my scoliosis. I am so glad I did because it has allowed me to sit up straight, which is so much better for breathing.
I have lived independently with the help of personal care attendants and family since I was 18 years old. I am old enough now to have benefited from both old school techniques and equipment and new, modern equipment. When I first had pneumonia, at 17, there wasn’t a Cough Assist or Vest. I had a great respiratory therapist who taught me percussion therapy and how to manually cough. It was a tough two weeks, but together we made it through.
Percussion therapy can be done easily with the assistance of a caregiver. The caregiver will clap you on the back or the chest with their hand in a cupped position quickly and repeatedly. This therapy helps loosen the mucus in your lungs to make it easier to cough out. A manual cough is done by having the caregiver place their hands on the sides of your waist and use their thumbs to push in and up under your diaphragm to help produce a strong cough. This can also help with choking. This is one of the first things I train new caregivers to do. If done correctly, neither technique should hurt. There are videos online demonstrating these techniques.
By the time I was 30, I needed 24-hour breathing support. I had already been using a CPAP mask with a BiPAP machine at night for three years. I had learned about the pneumobelt a few years earlier from a friend who was moderating an MDA support group I attended. Her name was Tedde Scharf. She thoroughly described how it worked and gave a demonstration. Instantly, I knew it was what I wanted to use, though I did not, at that time, need breathing support. I will forever be grateful to her.
When it came time, I asked my pulmonologist about it. She was supportive as long as I did well on it. I am very thankful to have a doctor who has been so supportive of me with my wishes to use noninvasive ventilation. Back when I started using it, not many doctors were. I am not sure why more doctors didn’t prescribe it back then. Before Tedde, the only options I was given were trach or oxygen. I was prescribed oxygen for a short time before I learned how dangerous oxygen could be when your breathing muscles are very weak.
Not many of you have probably ever heard of the pneumobelt. I would like to use Tedde’s words to describe it:
“The pneumobelt, also known as an exsufflation belt, consists of an air bag or rubber bladder inside a cloth corset that is worn around the abdomen just under the diaphragm. It is connected by tubing to a ventilator that alternately inflates and deflates the bladder. As the pneumobelt inflates, it applies pressure up under the diaphragm to force air out of my lungs for exhalation. Then the bladder deflates, lowering the diaphragm, allowing air to rush back into my lungs passively for inhalation. The ventilator settings can be set to provide a smooth, natural inhalation and exhalation, as well as a natural breathing rate for speaking.”
I took to it immediately, no adjustment period like there sometimes is when finding the right mask. To me, my breathing feels more natural. I like that I can have a break from my mask during the day. The only thing I don’t like about it is the corset that comes with it, which is very uncomfortable. I had a family member sew me a homemade belt that fits much better.
I still use a mask at night because the belt doesn’t work lying down, but it is nice not to have to wear it during the day. I use the belt about 10 hours a day. I supplement with a mouthpiece when reclining in my wheelchair or after eating. Tedde also tried to teach me how to use a technique called frog breathing, but unfortunately I was never able to master the skill. The pneumobelt isn’t visible when in use. If you didn’t see the little hose coming out the side or hear the ventilator, you wouldn’t know I was using anything.
My doctors have all been amazed at how well I’ve done on it. I have only been rarely sick since I began using it, and it has provided excellent respiratory support for me. Unfortunately, not many people were aware of it or used it, so now it has been discontinued along with my trusty Breeze mask. It has been a part of me and kept me healthy and active for the last 15 years. I am already mourning the loss. There has been some interest in keeping it going, and I am hopeful that can happen. There are good people working on it, so I have faith it’ll come back.
Change is hard, and I don’t know what I will switch to once my supplies run out (right now I have enough supplies to keep me going for awhile). I am determined to use it for as long as possible. I am very grateful for technology, medical advances and what I’ve been taught in my lifetime. It will be exciting to see what comes out in the future.
IVUN ARTICLES BY TEDDE SCHARF, MA
Considering a Change to Noninvasive Ventilation (Spring 1994, Vol. 8, No. 1)
Noninvasive Ventilation: A Successful Changeover (Spring 1996, Vol. 10, No. 1)
Pneumobelt in Action (Winter 1999, Vol. 13, No. 4)
The Pneumobelt: Part of My Noninvasive Ventilation System (Winter 2004, Vol. 18, No. 4)
Comment: Bragg-Paul Pulsator or Pneumobelt: a form of noninvasive ventilatory support
Norma MT Braun, MD, FACP, FCCP
It is noteworthy that D., a 45 year-old woman with neuromuscular chronic respiratory failure, is and has been successfully using a pneumobelt ventilator for daytime upright ventilation for about 15 years. Her fear is when her system becomes worn there will be no replacements.
The first such machine for externally applied active exhalation with passive inspiration was invented by William Henry Bragg and designed by Robert W. Paul in 1933. The idea was triggered by a friend of Bragg who had a form of muscular dystrophy. Due to the polio epidemics early in the 20th century, its use was rapidly expanded for this population. Freedom from the iron lung for these patients was a game changer. From her essay, both ventilation and expectoration of lung secretions can be successfully managed.
D. lies down to sleep so this reverse method of ventilation cannot be used in the supine position. There are only case reports of successful uses or individual physician experiences as there has never been a randomized control trial of its use due to the heterogeneity of patients, difficulty of matching patients and the prior absence of functional positive pressure ventilators requiring intubation. The avid adoption of positive pressure systems, the conquering of polio, the development of intensive care units, and the changes in reimbursement for such ventilatory systems all contributed to the demise of the pneumobelt along with negative pressure ventilators. The lack of experience of those trained with positive pressure systems sounded the death knell.
However, there are many patients who can benefit from the use of the pneumobelt. In addition to the growing cadre of patients living to adulthood with many inheritable or acquired neuromuscular diseases, there is the potential to wean patients on positive pressure ventilation so that speaking and eating can commence, which allows interaction and enhances recovery. They can be cared for at home for as long as needed.
Caveats for use: morbidly obese due to the time lag to apply sufficient pressure to effect lower rib cage compression and diaphragm ascent (already high in chest) to effectively move enough air. Fat also has poor compressive and recoil elastic properties. Another type of patient for whom this mode is not effective is the patient so thin that there is too little abdomen (scaphoid) to compress or has so much skeletal deformity that adequate sufficient pressure application cannot be achieved.
The hope is with modern technology, increasing availability of better materials, and enough interest for investment that an updated pneumobelt can be designed and manufactured and applied to patients just like D. With this mode patients with ventilatory limits can live full interactive and productive lives.
References on request.
Norma Braun, MD, FACP, FCCP, Clinical Professor of Medicine, Icahn School of Medicine at Mt. Sinai Health System, New York, New York.
Live Stream PPMD's Annual Conference
Weren't able to attend Parent Project Muscular Dystrophy's Annual Conference this year? Live stream the proceedings instead and keep up to date on the latest research in the fight to end Duchenne. The conference, taking place June 28-30 in Scottsdale, Arizona, can be live streamed here.
ABLE National Resource Center Promotes ABLE Toolkit
The ABLE National Resource Center has developed, compiled, and updated various ABLE-related materials in an effort to provide stakeholders, including individuals with disabilities and their families, ABLE administrators and managers, financial institutions, state and federal agencies and departments, special needs attorneys, and disability-related service providers, with a centralized virtual location in which they can quickly access printable ABLE-related education and information. The goal of the toolkit is to facilitate, and significantly broaden, widespread compressive education, information, and awareness throughout the country.
Over $1 Million Raised for MD and Related Diseases
Sailormen Inc., one of the largest domestic franchisees of Popeyes Louisiana Kitchen restaurants, and the Muscular Dystrophy Association (MDA) announced today that the annual MDA Golf Classic and the 2018 “Appetite for a Cure” program have raised a record-breaking $1,007,789 to save and transform the lives of kids and adults with muscular dystrophy, ALS and related life-threatening diseases that severely weaken muscle strength and mobility. The funds will go towards groundbreaking research and life-enhancing services such as the MDA Care Center at the University Of Miami School Of Medicine. It also helps send kids, ages 8-17, to “the best week of the year” at barrier-free MDA Summer Camp.
Independent Living Resources from the University of Kansas
The University of Kansas' Research and Training Center on Independent Living, in cooperation with the University of Montana, has compiled a number of resources related to disability and independent living on their site. The resources are divided into several categories: Out and About: Laws That Protect People with Disabilities, Community Living and Participation Research, Health Care for People with Disabilities, ADA Accommodations Training, Weight Loss and Emergency Preparedness.
NAELA Urges Passage of Empower Care Act
The National Academy of Elder Law Attorneys (NAELA) marked the 19th anniversary of the Supreme Court’s Olmstead decision, which recognized that individuals with disabilities have a qualified right to state-funded supports and services in the community.
They stated, "The United States unfortunately has a long, shameful history in segregating and unnecessarily institutionalizing people with disabilities. While progress has been made since Olmstead, many people with disabilities are still trapped in nursing homes due to the fact that the Medicaid program mandates this form of coverage, but makes home and community-based services (HCBS) optional. This anniversary, NAELA joins a coalition of aging and disability groups to urge Congress to pass the bipartisan Empower Care Act. The legislation would renew the Money Follows the Person Medicaid demonstration project (MFP). MFP has helped more than 75,000 seniors and people with disabilities move out of nursing homes and back to the community. Unfortunately, MFP expired in September 30, 2016. All participating states will run out of funding by the end of the year."
“Individuals with disabilities want to live the American dream just like everyone else. Money Follows the Person allows them to get back home, where they can work, study, and contribute to their communities,” said NAELA President Michael J. Amoruso, Esq., Fellow, CAP.
Shining a Light on Abuse of Guardianship Laws
The comedian John Oliver recently covered the issue of guardianship abuse on his weekly HBO show, Last Week Tonight. Guardianship is a legal tool that allows one person or entity to make decisions for another, often those incapacitated by old age or disability. Guardians are court-appointed and have control over the individual's finances, living situation, health care, who they can visit and where they can travel. Individuals who have been appointed a guardian cannot vote, marry or divorce. Though guardianship can often be necessary and beneficial, it is ripe for abuse. As Oliver points out, state and local courts often lack the training and resources to properly monitor the system.
Recently, some states have started to put in place protections. Texas, Wisconsin, Delaware and the District of Columbia have enacted "supported decision-making" laws which require institutions to recognize the role that family and close friends serve in helping individuals make decisions for themselves. An 87 year-old woman in DC recently became the first person in that jurisdiction to use this law to overturn a guardianship appointment (“This 87-year-old D.C. woman just made it easier for you to keep your independence”). Perhaps the best way to protect yourself, though, is to have an honest conversation with your loved ones before the time comes when you might need guardianship.
A 285-page analysis from the National Academies of Sciences, Engineering and Medicine found that even the most capable families struggle to navigate the complex web of programs available to assist those with disabilities. For those with less means or families who live in rural areas, the report found those challenges are further exaggerated.
Overall, the report paints a picture of a services system that’s riddled with barriers stemming from variation in the availability of services from one state to the next, fragmented services, an inadequate workforce of professionals to provide care and insufficient plans to transition people to adulthood, among other issues. Enhanced coordination of care and a greater focus on specific goals designed to help kids with disabilities prepare for adulthood would help promote optimal outcomes in terms of health and functioning, the report concludes.
NAMDRC legislative and regulatory agenda once again focuses on patient access
Phil Porte, Executive Director, NAMDRC
“Physicians still struggle with the concept of RADs without a definitive, consistent definition and no FDA language to guide usage. Today, it is easier to secure a ventilator if a physician documents the patient experiences some level of respiratory failure than it is to prescribe a simple ventilator with a back-up rate.
“If one takes the approach that a device should be paired with the actual clinical characteristics/medical need of the patient, changes in policy are necessary. While CMS clearly has the authority to act to improve policy and match clinical need to patient access, years and years of back and forth have signaled a definite unwillingness of the agency to move in that direction; therefore, the only genuine recourse is to seek legislative relief.
“NAMDRC is working closely with the United States Senate, particularly the Finance Committee, Senator Cassidy (R-LA), and the Office of Senate legislative Counsel to craft legislative language to address the myriad of issues associated with home mechanical ventilation.”
CHEST Physician, June 2018, 13(6):54-56.
Noninvasive Ventilation in Patients With Do-Not-Intubate and Comfort-Measures-Only Orders: A Systematic Review and Meta-Analysis
Wilson ME, Majzoub AM, Dobler CC, Curtis JR, Nayfeh T, Thorsteinsdottir B, Barwise AK, Tilburt JC, Gajic O, Montori VM, Murad MH
“A large proportion of patients with do-not-intubate orders who received noninvasive ventilation survived to hospital discharge and at 1 year, with limited data showing no decrease in quality of life in survivors. Provision of noninvasive ventilation in a well-equipped hospital ward may be a viable alternative to the ICU for selected patients.”
Crit Care Med. 2018 Mar 1 [Epub ahead of print]. Also see related New York Times article, "Breathing Tubes Fail to Save Many Older Patients."
Effect of Home Noninvasive Ventilation With Oxygen Therapy vs Oxygen Therapy Alone on Hospital Readmission or Death After an Acute COPD Exacerbation
Murphy PB, Rehal S, Arbane G, et al
“In this randomized clinical trial, an improvement in time to readmission or death was observed when home noninvasive ventilation was added to home oxygen therapy in patients with persistent hypercapnia following a life-threatening acute exacerbation of COPD. These data support the screening of patients with COPD after receiving acute noninvasive ventilation to identify persistent hypercapnia and introduce home noninvasive ventilation.”
JAMA. 2017;317(21):2177-2186. https://doi.org/10.1001/jama.2017.4451
Caregiver Shortage Forcing Young People With Disabilities Into Institutions
Chris Serres, Star Tribune/TNS, May 9, 2018
“A chronic and deepening shortage of home care workers is forcing scores of younger people with disabilities to move into sterile and highly restrictive institutions, including nursing homes and assisted-living facilities, designed for vulnerable seniors. Pleasures that young people take for granted — visiting friends or even stepping outside without permission — are beyond their grasp. The trend worries civil rights advocates, who say it could imperil decades of effort by state officials to desegregate housing for people with disabilities and to help them live more independently in the community.”
Scientists Investigate New Strategy to Treat Spinal Muscular Atrophy in Infants
Scripps Research Institute
Scientists at the California Institute for Biomedical Research (Calibr) and The Scripps Research Institute have discovered how a potential new drug targets SMA. The research, published recently in the journal Proceedings of the National Academy of Sciences, could guide the development of other drugs for genetic diseases.
“In fact, if we can learn more about the drugs in the pipeline for genetic diseases, we can learn more about the diseases themselves,” says Kristen Johnson, PhD, a principal investigator at Calibr and co-lead author of the study.
Until recently, patients diagnosed with SMA had no treatment options. The recent approval of a drug called nusinersen, marketed as Spinraza, has given families hope, and now scientists are working to design therapies that could be even more effective.
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. 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.
Make plans to attend the Margaret Pfrommer Memorial Lecture in Long-term Mechanical Ventilation Monday, October 8, at 3:15 pm
This year's lecture, entitled “Saving Lives…One ventilator at a Time – HMV in 2018 and Beyond,” will be presented by Douglas McKim, MD, FCCP. The award, established in 1999 by Dr. Eveline Faure and Dr. Allen I. Goldberg, honors their lifelong colleague and friend, Margaret Pfrommer, a polio survivor and patient advocate. It is awarded to a clinician or ventilator-dependent professional or advocate who has advanced mechanical ventilation and fostered partnerships between physicians and patients.
AARC Congress 2018
AARC’s International Respiratory Convention & Exhibition will take place December 4-7, 2018 at the Mandalay Bay Resort and Convention Center in Las Vegas, Nevada. Find out more and register online.
Philips Respironics releases full face cushion for use with its DreamWear mask
Philips Respironics has released a third option for its DreamWear mask system. Philips analyzed facial data from thousands of 3D scans to create a design intended to prevent red marks, discomfort and irritation on the nose bridge. The DreamWear mask system directs airflow through the frame and removes the hose from the front of the face, giving patients more freedom to sleep comfortably in any position they desire.