INTERNATIONAL VENTILATOR USERS NETWORK
an affiliate of Post-Polio Health International
VOLUME 36, NUMBER 3
Our family has been on an incredible journey for over 27 years. Our only child, Ryan, suffered an adverse reaction to a Hepatitis B vaccine he received as a newborn. By four months of age, he was trached and ventilator dependent. While not paralyzed, Ryan is very weak, unable to lift his arms or legs against gravity, use his hands, sit unassisted, or hold up head. He is G-tube fed. Though Ryan is non-verbal, he still manages to communicate quite well. His brain was unaffected.
We have experienced the full gamut of vent life from infancy to adulthood; each phase with its own challenges. Infancy was less stable, but recovery was quicker. Therapy was easier. As he has aged, he became more medically stable, but more physically demanding for us - transfers, therapy, etc......................................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. 36, No. 3, June 2022
Editor: Brian Tiburzi
Designer: Brian Tiburzi
Permission to reprint must be obtained from Post-Polio Health International (PHI) at firstname.lastname@example.org.
Our family has been on an incredible journey for over 27 years. Our only child, Ryan, suffered an adverse reaction to a Hepatitis B vaccine he received as a newborn. By four months of age, he was trached and ventilator dependent. While not paralyzed, Ryan is very weak, unable to lift his arms or legs against gravity, use his hands, sit unassisted, or hold up head. He is G-tube fed. Though he is non-verbal, he still manages to communicate quite well. His brain was unaffected.
We have experienced the full gamut of vent life from infancy to adulthood; each phase with its own challenges. Infancy was less stable, but recovery was quicker. Therapy was easier. As he has aged, he became more medically stable, but more physically demanding for us (transfers, therapy, etc.).
Over the years, we have adapted our home to meet his needs. Many things we had to design ourselves - therapy equipment, shower chair, pool lift, etc. Ryan has his own living quarters with room-covering ceiling lifts. He has a private bath and therapy room. He uses a Dynavox with switch to communicate and control his music and TV. Ryan uses a custom wheelchair, stander, therapy tables, Astral 105 ventilator (he is on pressure support), Fisher Paykel humidifier, Cough Assist, Vest, pulse oximeter and CO2 monitor. He loves music and has nearly 4,000 songs in his library. He can access his computer with Jaeco Arm supports and a NueroNode (an EMG switch we have on his thigh). For transportation, we use a full-size conversion van with wheelchair lift.
Ryan has received excellent care his entire life and has only been hospitalized a single night. He is not on any medications. His father (a retired ER physician) and I are his primary caregivers. This includes trach and G-tube changes, bathing, bowel management, getting him up in the morning and putting him to bed at night. We have night nurses working from 11:00 pm - 8:00 am (so we can sleep) and 6 hours of day nursing Monday through Friday. He receives physical therapy four times per week. Though Ryan is on Medicare, our nursing and physical therapy are self-paid.
By far, the most challenging part of our lives is staffing. Finding caring, competent nurses who are willing to work nights is nearly impossible. We run our own ads, do our own training (which is extensive), and pay very well. Yet, we still have great difficulty finding people. Once we do find someone, though, they tend to stay with us for many years. We have our staff go through an agency for payroll and insurance.
COVID has been extremely difficult; especially keeping COVID out of our home. We have been very isolated, avoiding crowds and indoor spaces, and not seeing family (they all live out-of-state). Ryan’s caregivers wear N-95 masks and gloves that we provide. We have had numerous uncovered shifts due to illness. It has been exhausting, but we have been successful in keeping COVID out of our home and Ryan safe. Early in the pandemic, we stocked up on masks, gloves and medical supplies.
The quality and availability of supplies have declined over the years, specifically ventilator circuits. Ryan does not tolerate HMEs well and needs active humidification. For most of his life, we have used custom 8’ heated wire circuits. The longer circuits allow him to float in the pool, use the stander, and do transfers without disconnecting his circuit (disconnecting during a transfer causes him to aspirate). At the beginning of the COVID pandemic, Vyaire decided to discontinue all custom circuits without warning, including Ryan’s custom 8’ circuits. The longest heated wire circuits now available are 5-6’. This has been a huge step back for his quality of life. While ventilators have seen great technological advances over the past 27 years, humidification has gone backwards. It is frustrating. We are constantly on the lookout for alternatives.
Ryan is an amazingly happy and content young man. He has a wicked sense of humor and is seldom without a smile. He has no self-pity and is exceptionally patient. He loves music and has it playing 24/7 – even when he’s sleeping. He enjoys using his computer to surf the internet and stream his favorite content.
We have an active life; swimming (using a pool lift and floats), boating (we have modified our boat to make it accessible), and biking (VeloPlus bike in which the wheelchair rolls onto a platform in front of the bike). Though on hold because of COVID, we used to attend numerous plays and concerts each year. Ryan loves all types of live performances and has been to over 100 shows. We also take an annual trip to Disney. We enjoy doing everything together as a family and are grateful for every minute we spend together. Our focus is to keep him healthy and give him the best quality of life possible.
Our life is not a “normal” life, but it is normal to us. Our journey as a family has been both extremely challenging and extremely rewarding. Ryan has brought joy and love into our lives and taught us to be brave, patient and grateful for each day we have together.
Home and Community-Based Services
Earlier this month, the U.S. Department of Health and Human Services (HHS) announced that they would be notifying states that they now have an additional year — through March 31, 2025 — to use funding made available by the American Rescue Plan (ARP) to “enhance, expand, and strengthen” home- and community-based services (HCBS) for people with Medicaid who need long-term services and supports.
CMS had previously extended the time states had to spend the money, but many states waited longer than anticipated for CMS to approve their plans and gain access to the extra money prompting concerns that there wouldn’t be enough time to spend all of it. Some of the uses outlined by states for the funds include reducing waiting lists, raising wages for direct support professionals and improving employment opportunities for people with disabilities, among other initiatives.
Wheelchairs and the 'Right to Repair'
Colorado has passed a first-in-the-nation right-to-repair bill for power wheelchairs that will allow owners and independent repair shops to access parts, embedded software, tools and documentation needed to perform diagnostic, maintenance or repair services.
As reported by Kaiser Health News ("Despite a First-Ever ‘Right-to-Repair’ Law, There’s No Easy Fix for Wheelchair Users"), part of the problem stems from the fact that competitive bidding encourages suppliers to press manufacturers for lower-cost wheelchairs, which spurs manufacturers to use lower-quality parts. More than 1 in 4 repairs result in users being stranded, missing a medical appointment, or missing work, according to a study published in 2016 in the Archives of Physical Medicine and Rehabilitation.
Adding to the problem is a Medicare decision not to cover preventive maintenance for power wheelchairs. Many wheelchair users are unfamiliar with or unable to do routine maintenance such as tightening the bolts or cleaning the casters. As a result, problems aren’t addressed until something breaks down, often leaving the user stranded.
Medicare regulations also hurt, with low reimbursements, and Medicare pays only for parts and labor, not for technicians’ travel time. Another cause for delays: Medicare sometimes requires physicians to document that an individual still needs a wheelchair and that it needs to be repaired.
It's possible, but still unclear at this time, whether the Colorado law could lead to policy changes nationwide.
Virtual CCHS Research Conference
Video of CCHS Foundation and CCHS Network's 2nd Virtual CCHS Research Conference on May 2nd, 2022, is now available to view online. Click here to watch.
The speakers for the conference were:
Prof. Donatella Degl’Innocenti: Urinary oxidative biomarkers as a tool for follow-up of CCHS patients
Prof. Isabella Ceccherini: Multi-omic approaches to study PHOX2B-mediated pathogenesis and to identify possible genetic modifiers useful for improving genotype-phenotype correlation in CCHS
Dr. Gad Vatine: Patient-specific stem cells to study mechanisms underlying CCHS
From NARIC: Celebrating the Olmstead Anniversary
The National Rehabilitation Information Center (NARIC) marked the 23rd anniversary of the Olmstead decision on June 22nd. This landmark Supreme Court decision determined that unjustified institutional isolation of people with disabilities was a form of unlawful discrimination under the Americans with Disabilities Act (ADA). This important decision made it possible for many people with disabilities and older adults to live in and participate fully in the community of their choosing. It has led to more funding for services and supports in the community, more employment opportunities, and advances in technology that facilitate community living. More people with disabilities in the community has also led to more research on the importance of community living and participation. To honor this anniversary, NARIC explored some of the recent NIDILRR-funded research in community living and participation in their Spotlight blog.
The AAHD All of Us Research Program
The American Association on Health and Disability (AAHD) is challenging the disability community to learn about the All of Us Research Program and consider participation with a Call to Action. The All of Us Research Program is seeking to improve precision medicine and the future of health. AAHD is working to encourage people with disabilities to get involved in this research program that includes historically under-represented populations in biomedical research, including persons with disabilities.
All of Us is a research program from the National Institutes of Health (NIH). The goal is to advance precision medicine. Precision medicine is health care that is based on you as an individual. It takes into account factors like where you live, what you do, and your family health history.
Their goal is to get one million or more participants. Those who join will share information about their health over time. Researchers will study this data. What they learn could improve health for generations to come. NIH states that they will share information back with participants over time.
AAHD's Call to Action challenges the disability community to embrace “nothing about us without us.” If we truly believe in the concept of “nothing about us without us,” then we must answer the call when asked to participate in this type of program. If you are interested in enrolling in the All of Us Research program, please visit https://allofus.nih.gov/.
Addition of bacterial filter alters positive airway pressure and non-invasive ventilation performances
Rabec C, Fresnel E, Rétory Y, Zhu K, Joly K, Kerfourn A, Dudoignon B, Mendoza A, Cuvelier A, Muir JF, Melloni B, Chabot JF, Gonzalez-Bermejo J, Patout M.
“In our study, we have demonstrated that adding an inline filter greatly altered the automated detection of obstructive events. Clinicians should therefore not base their clinical decision using the residual event data provided by a PAP device when using an inline filter.
“Our results show that the addition of an inline filter could strongly impact on the effectiveness of the auto-adjusting PAP device tested. Indeed, we have shown that the addition of filters resulted in a lower delivered pressure and a higher number of residual obstructive events. We hypothesise that filters impact the efficacy of this device by interfering with the detection of obstructive respiratory events leading to an increase in the residual apnoea–hypopnoea index reported by the device. Our results show that auto-adjusting PAP should not be used with an inline filter.”
Eur Respir J. 2022 Apr 14;59(4):2102636. doi: 10.1183/13993003.02636-2021.
Perry MA, Jenkins M, Jones B, Bowick J, Shaw H, Robinson E, Rowan M, Spencer K, Neill A, Ingham T.
"Me and ' that' machine": the lived experiences of people with neuromuscular disorders using non-invasive ventilation
“The findings highlight the broad ranging positive and negative effects that may occur for people with NMD when using this important therapy. Ongoing non-judgemental support and empathy are required from health professionals as the use of NIV challenged concepts such as 'living life well' for people with NMD.
IMPLICATIONS FOR REHABILITATION - Neuromuscular disorders may result in respiratory weakness requiring non-invasive ventilation (NIV).When prescribed early, NIV can results in fewer hospital admissions, a lower mortality rate and improved quality of life.The relationship of people with NMD with their NIV machine is complex and impacts on and requires adjustment to their identity.NIV users acknowledged that NIV provided hope but simultaneously recognised the precariousness of NIV on their life.In order to better support people with NMD healthcare professionals need to better understand how the physical, psychological and social implications of NIV affect an individual's life.”
Disabil Rehabil. 2022 Jun 1:1-10. doi: 10.1080/09638288.2022.2076939. Epub ahead of print.
Brandt H, Simon DM, Kasi AS.
Ventilator change in children on home mechanical ventilation affected by the Philips Respironics Trilogy ventilator recall
“The Philips Respironics recall notification issued in June 2021 affected many of their positive airway pressure devices and mechanical ventilators including the Trilogy 100 and 200 ventilators that are often utilized in children using home positive pressure ventilation via tracheostomy (PPV-T). Optimal strategies to replace ventilators in children using home PPV-T affected by the Philips recall are unknown. We conducted a retrospective study of children using home PPV-T with recalled Trilogy ventilators who underwent inpatient ventilator change to non-recalled portable home ventilators (PHV) using our collaborative institutional protocol. During the study period, there were 40 children using PPV-T with recalled Trilogy ventilators and 19 patients underwent inpatient ventilator change either during an elective hospitalization (n = 8) or during an unscheduled or postoperative hospitalization (n = 11). The median duration of hospitalization for ventilator change was 2 days (interquartile range: 6 days) and generally 1 day for patients admitted solely for ventilator change. In children using PPV-T with recalled Trilogy ventilators, a systematic protocol collaborating with the patients, physicians, and durable medical equipment companies may optimize transition to nonrecalled PHVs.”
Pediatr Pulmonol. 2022 Jan;57(1):43-48. doi: 10.1002/ppul.25730.
CCHS Network Family Conference 2022
The CCHS Network Family Conference that was scheduled to take place July 19-22, 2022, in Newport Beach, California, has been cancelled.
ERS International Congress 2022
The European Respiratory Society (ERS) International Congress will take place in Barcelona, Spain, and online September 4-6, 2022. Registration will open in the spring at www.ersnet.org/congress-and-events/congress/.
October 16-19, 2022, Nashville, Tennessee. The CHEST 2022 Annual Meeting will take place in person and will offer more than 300 educational sessions, including simulation and interactive learning opportunities.
New Resources for Those Affected by Phillips Recall
Phillips Respironics has published new information for those affected by the recent recall.
Important information about patient prioritization - Contains new details on how the company is prioritizing remediation of those patients potentially at higher risk.
Understanding the Recall Process - An overview of the recall process that enables affected patients to check the status of their order.