INTERNATIONAL VENTILATOR USERS NETWORK
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VOLUME 34, NUMBER 5
When Francisco first became ill in 2016, his mother, Laura, took him to urgent care near their home in Albuquerque, New Mexico, where he was diagnosed with strep and sent home. By the next evening, Laura and her husband, Robert, suspected something more serious was going on. Francisco couldn’t turn his head. By the time they arrived at the ER, “He was a complete little noodle in [Robert’s] arms,” said Laura.......................................................................MORE
Acute Flaccid Myelitis (AFM) is a rare but serious neurologic condition characterized by sudden onset of arm or leg weakness, loss of muscle tone, and loss of reflexes. In most patients, respiratory illness or fever is present before AFM onset. While it is sometimes compared to polio, poliovirus has been ruled out as a cause. Experts have named Enterovirus D-68 (EV-D68) and D-70, Coxsackie A-71, and West Nile as possible causes, though EV-D68 is suspected of being the cause of the majority of cases...............................................................................MORE
Educate, discuss, take action
Sharing the work of others
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. 34, No. 5, October 2020
Editor: Brian Tiburzi
Designer: Brian Tiburzi
© 2020 Post-Polio Health International.
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Living with AFM:
When Francisco first became ill in 2016, his mother, Laura, took him to urgent care near their home in Albuquerque, New Mexico, where he was diagnosed with strep and sent home. When Francisco couldn’t turn his head the next evening, Laura and her husband, Robert, suspected something more serious was going on. By the time they arrived at the ER, “He was a complete little noodle in [Robert’s] arms,” said Laura.
The hospital ran a number of tests – MRI, lumbar puncture, CT, blood work – but everything came back normal. They decided to admit him to the PICU for observation.
Francisco's condition began to deteriorate over the course of the following day and by night he had to be sedated and intubated. A second MRI showed a lesion from his brain stem all the way down his spine, but the doctors still didn’t have any idea what was causing his symptoms.
After about two weeks without any improvement, they made the decision to transfer him to Denver Children’s Hospital. After an initial workup, doctors there suspected he may have Acute Flaccid Myelitis (AFM). A test for Enterovirus D68 (EV-D68) came back positive, confirming their suspicions.
Denver Children's Hospital had treated some AFM patients during the first wave of the disease in 2014, but there were still a lot of unknowns surrounding this new condition. Nobody could say for certain what Francisco’s prognosis would be. Still intubated, Francisco had started to develop mouth sores, so a decision was made to put in a trach and a G-tube.
AFM is a rare but serious neurologic condition that affects the nervous system, specifically the area of the spinal cord called gray matter, which causes the muscles and reflexes in the body to become weak. It is often described as a polio-like illness, and, like polio, it primarily affects young children.
The CDC first began tracking cases in 2014. There were subsequent outbreaks in 2016 and 2018. While not definitively proven, EV-D68 is believed to be the cause of most of these cases.
Francisco would spend two months at Denver Children’s Hospital receiving physical therapy, occupational therapy, and speech therapy. The simulation lab there trained Laura and Robert how to manage Francisco’s tracheostomy tube and ventilator and deal with any emergencies that might arise. “We definitely had really good training,” said Laura. “When we got home, we were able to integrate him into our community and take him out and do things without being afraid of not knowing what to do.”
Francisco with younger brother, Joaquin.
“The case managers in Denver gave us great information on resources in New Mexico like Medicaid’s Medically Fragile program that helps with therapy that our private insurance doesn’t cover and is a life-changer,” said Laura. Laura, a physical therapist, credits her connections with other health professionals in helping ease the transition to home. “He got set up with his PT, OT, and speech therapist right away, which was huge; especially the speech therapy, which was needed for secretion control and to get on his Passy-Muir valve.”
The pandemic has added some new wrinkles into his routine, but the family is adjusting well. Like many kids, he is mostly receiving his education through virtual learning. He does go for an hour at a time for in-person special education services, however. While shortages of some ventilator supplies have been reported around the country, so far they’ve been able to get their regular allotment.
Francisco is now able to go off his Trilogy ventilator for long periods during the day. The next step will be to have a sleep study done with the eventual goal of weaning him off his ventilator.
Asked what advice she would have for other families, Laura said, “Help your child get into their environment. I know sometimes it’s hard to get out there with all that equipment you have to carry, but I feel like it has definitely made a huge difference in his confidence to get out there a be comfortable being out in the community.”
Acute Flaccid Myelitis (AFM) is a rare but serious neurologic condition characterized by sudden onset of arm or leg weakness, loss of muscle tone, and loss of reflexes. In most patients, respiratory illness or fever is present before AFM onset. While it is sometimes compared to polio, poliovirus has been ruled out as a cause. Experts have named Enterovirus D-68 (EV-D68) and D-70, Coxsackie A-71, and West Nile as possible causes, though EV-D68 is suspected of being the cause of the majority of cases.
The Centers for Disease Control and Prevention (CDC) has been monitoring cases of AFM in the U.S. for at least a decade. Since 2014 there have been 100-250 cases in even-numbered years. In 2018 the CDC became concerned enough to appoint an AFM Task Force of distinguished physicians as scientific counselors to their public health efforts for prevention of new cases. At present there are no apparently effective medical treatments for cases with residual paralysis. While most cases show some improvement over the first year after onset, most appear to have some degree of permanent residual weakness (Ref. 1).
Cases of AFM typically spike between August and November. This year there have only been 25 cases through the end of October. Only Texas has reported more than two cases statewide. Some experts have speculated that measures taken to slow the spread of SARS-CoV2 - hand washing, mask wearing, social distancing - may also be working to keep the number of AFM cases low.
You can find the latest information on AFM on the CDC's AFM page. For families and individuals affected by AFM, the CDC is hosting a webinar, “An Update from CDC on AFM in 2020,” on Thursday, November 12 from 3 – 4 pm EST. Those interested may register online.
Ref. 1: Martin JA, Messacar K, Yang ML, et al. Recognition and Management of AFM in Children. Neurology 2017;89: 129-137.
SOURCE: MMWR, August 2020.
For Many, Pandemic-Induced Supply Shortages Continue
In our last issue, IVUN reported on the issue of supply shortages, particularly with circuits, that have affected ventilator users since the start of the pandemic. California Health Report recently featured IVUN member Jenny McLelland, a parent advocate who has been working to draw attention to this important issue. McLelland's son, James, uses home mechanical ventilation, and they have had trouble getting replacement circuits for James' ventilator.
“I’m worried that this will become the new normal,” said McLelland in the article. “The problem is that no one is tracking this, and this is a population of disabled people and disabled children who die very frequently … These are not children who tolerate changes in care well.” Read the full article here. The article also includes a link to a video by McLelland demonstrating how to wash and reuse circuits, which you can view below.
IRS Issues Final Rules for ABLE Accounts
The IRS issued final regulations earlier this month spelling out details about how ABLE accounts should operate. The IRS said the final regulations are intended to clarify specifics and address comments the agency received in response to previous proposals.
Annual deposits to ABLE accounts are currently capped at $15,000 annually. However, under the final rules, individuals with disabilities who are employed can deposit their earnings in ABLE accounts, above and beyond the existing contribution cap for the year. These individuals can save whatever money they earn in their ABLE account up to the value of the poverty line in the state where they live.
The IRS also states that workers with disabilities who have ABLE accounts can qualify for a Saver’s Credit, which can reduce their federal tax bill. Separately, money saved in a traditional 529 college savings plan for an individual with a disability or a family member can be rolled over into an ABLE account up to the maximum annual contribution limit. The rules indicate that this option is available until Jan. 1, 2026.
Support for Money Follows the Person Program Boosted by COVID-19 Concerns
The Centers for Medicare & Medicaid Services (CMS) said last month that as much as $165 million is available to states for the Money Follows the Person program. In light of the pandemic, officials said that they want to encourage transitions out of institutional facilities such as nursing homes and congregate homes, which are susceptible to coronavirus outbreaks.
Money Follows the Person programs are currently available in 33 states. Since 2008, they have helped over 100,000 people transition out of institutional facilities. Over the last couple of years, transitions have slowed as the program has become dependent on a series of short-term extensions, requiring some states to slow or end their programs. Only states who had planned to still be operating MFP in 2021 will be eligible for the announced funding. CMS says states will have until June 30, 2021 to request funds.
6th International CCHS Day
November 14th is the 6th International CCHS Day. The focus of this year's campaign is honoring CCHS Heroes - a grandparent, sibling, aunt or uncle, nurse, doctor, neighbor, friend, babysitter, or teacher of an individual living with CCHS who has lifted their burden in some way. Explore different ways you can participate and learn more about the event here.
The centerpiece of the campaign will be a virtual walk/run challenge. Starting October 31st, participants can log the number of miles they run/walk between then and November 14th. Participants can make a personal donation and create a fundraising team in honor of a particular "CCHS hero." Proceeds from the event will support the work of the CCHS Foundation with 75% going to the advancement of research, 20% going to education and advocacy, and 5% going to family support. More information about the event is available on their website.
COVID-19 Guidance from the Administration for Community Living
The Administration for Community Living (ACL) continues to update its information resource page, What Do Older Adults and People with Disabilities Need to Know About Coronavirus Disease 2019 (COVID-19)? The page includes information on combatting social isolation; transportation during COVID-19; staying connected with assistive technology; understanding resilience; and getting vaccines, such as the flu shot.
For older adults who may not be as comfortable using tech to stay connected, the website Aging In Place has created a guide, "How To Become Tech Savvy Seniors In 10 Days," with tips and instruction to give older adults the skills and confidence to access information and services online.
Office of Management and Budget Disability Survey
The Office of Management and Budget (OMB) is leading a multi-agency effort to understand how individuals with a disability use and experience services provided by a range of agencies across the federal government.
The OMB disability survey is open to individuals with a disability and those who help them. OMB invites participants to share what they have found most helpful, frustrating, and moments that made a difference in their family’s journey.
The survey is anonymous, though you have the option to share an email address if you are interested and willing to arrange an interview to discuss further. You can visit 2020 Federal Customer Experience Journey Mapping Projects to learn more about this project, including how OMB will use the information participants provide.
Non-invasive Ventilation Use Is Associated with Better Survival in Amyotrophic Lateral Sclerosis
Ackrivo J, Hsu JY, Hansen-Flaschen J, Elman L, Kawut SM.
“NIV use was associated with significantly better survival in ALS after matching and adjustment for confounders. Increasing duration of daily NIV use was associated with longer survival. Randomized clinical trials should be performed to identify ideal thresholds for improving survival and optimizing adherence in ALS.”
Ann Am Thorac Soc. 2020 Sep 18. doi: 10.1513/AnnalsATS.202002-169OC. Epub ahead of print.
Sprooten RTM, Rohde GGU, Janssen MTHF, Cobben NAM, Wouters EFM, Franssen FME.
Predictors for long‐term mortality in COPD patients requiring non‐invasive positive pressure ventilation for the treatment of acute respiratory failure
“This study investigated the demographic, biochemical, clinical and intervention‐related predictors of short‐ and long‐term mortality in patients requiring non‐invasive positive pressure ventilation for the treatment of acute respiratory failure. Fourteen percent of patients died during hospital stay, while 56% died within 2 years. Older age, NIV use more than 8 days and non‐successful response to NIV were identified as independent predictors for long‐term mortality.”
Clin Respir J. 2020 Aug 11. doi: 10.1111/crj.13251. Epub ahead of print.
Bach JR, Burke L, Chiou M.
Noninvasive Respiratory Management of Spinal Cord Injury
“Intubated ventilator-dependent patients with high-level spinal cord injury can be managed without tracheostomy tubes provided that they have sufficient cognition to cooperate and that any required surgical procedures are completed and they are medically stable. Intubation for a month or more than extubation to continuous noninvasive ventilatory support (NVS) can be safer long term than resort to tracheotomy. Noninvasive ventilation (NIV) is not conventionally being used for ventilatory support. Noninvasive interfaces include mouthpieces, nasal and oronasal interfaces, and intermittent abdominal pressure ventilators. NIV/NVS should never been used without consideration of mechanical insufflation-exsufflation for airway secretion clearance.”
Phys Med Rehabil Clin N Am. 2020 Aug;31(3):397-413. doi: 10.1016/j.pmr.2020.03.006.
Onofri A, Broomfield A, Tan HL.
Transition to Adult Care in Children on Long-Term Ventilation
“While transition services for LTV patients are, for the most part, often still in their infancy, there are some obvious themes already emerging. The most important of these is that done effectively, transition is a process, not a single event of transference of care. The insights gained from examination of other chronic diseases highlight the absolute requirements, where possible, for better education and communication. The later not only reflecting the conversations between medical professionals and patients, but also between the pediatric and adult teams involved in planning and coordinating this process. Strong advocacy from both patient groups and medical professionals is needed to stimulate the system wide changes still very evidently required.”
Front Pediatr. 2020 Sep 30;8:548839. doi: 10.3389/fped.2020.548839.
"Tricks and tips for home mechanical ventilation" Home mechanical ventilation: set-up and monitoring protocols
“Follow-up of HMV patients is a black box; there are no evidence based guidelines describing how and how often ventilatory support should be monitored. Moreover, follow-up frequency might differ between patient groups; patients in rapidly changing conditions (children) or patients with a rapidly progressive disease (ALS/COPD) might need more frequent follow-up compared to the slower or non-progressive diseases. Furthermore, there is no consensus about which minimal set of parameters should be monitored. Finally, also in the follow-up of patients, telemedicine might bring attractive alternatives.”
Pulmonology. 2020 Sep 7:S2531-0437(20)30181-1. doi: 10.1016/j.pulmoe.2020.08.002. Epub ahead of print.
AARC Congress 2020
The previously cancelled meeting will now take place as a 4-day virtual learning event November 18th and 20th and December 3rd and 5th. Topics and events cover all respiratory therapy specialties, along with student and early professional opportunities. Participants will also get the chance to explore the virtual exhibit halls, checking out the latest industry products and services. Register online.
JIVD/ERCA Conference in 2021
The 4th International Joint Meeting of the JIVD (Journes Internationales de Ventilation Domicile) and ERCA (European Respiratory Care Association) will be held March 11-13, 2021 at Cité Centre de Congrès, Lyon, France. For more information, go to www.jivd-erca2022.com.
Candian Respiratory Conference
The Canadian Respiratory Conference 2021 will take place virtually April 8-10, 2021. Registration will open in January. Learn more here.
CCHS Network Family Conference 2021
The CCHS Network Family Conference will take place July 6-9, 2021 in Newport Beach, California. Family conferences foster a supportive and collaborative CCHS union between professionals and families, all with the intent of improving outcomes for CCHS patients. Rooms may be reserved at the Hyatt Regency Newport Beach.
ResMed MaskSelector Help Patients Find the Right Mask Remotely
In May, ResMed unveiled its MaskSelector tool as an easy-to-use solution designed to help health professionals find the right ResMed mask for their patients. This tool is especially useful as a time when many health professionals are still seeing patients remotely.
MaskSelector allows doctors to send a patient a text message or email with a personalized MaskSelector link to the web-based portal. After answering a few quick questions about their health and sleep habits, and also taking some facial measurements, ResMed MaskSelector will then provide a recommended mask model and size for the patient as well as sizing recommendations for other ResMed masks.