IVUN
INTERNATIONAL VENTILATOR USERS NETWORK
an affiliate of Post-Polio Health International
CONNECTING
VENTILATOR USERS,
HEALTH PROFESSIONALS,
AND INDUSTRY
VENTILATOR-ASSISTED LIVING
VOLUME 36, NUMBER 2
APRIL 2022
Shortly after Jason Sloan was born in Peoria, Illinois, in 1977, he was diagnosed with Congenital Central Hypoventilation Syndrome (CCHS), a disorder of the autonomic nervous system that affects breathing. The most recognized symptom of CCHS is the inability to control breathing that varies in severity, resulting in the need for life-long ventilatory support during sleep in some patients or all the time in others. There are estimated to be 1000–1200 cases of CCHS worldwide.
“When he was born,” recalled his mother, Debra, “I thought that whatever was wrong with him could and would be fixed by all the medical personnel. I was a very young mother and had no clue that my life was about to change drastically forever. I still remember my Jason being born that day. He was so purple and hooked up to tubes and everything. A few days later they called in our pastor to give last rites. Remember, I'm a young mother. What did I think would happen? Again, I thought he would be ‘healed’."..........................................................................................MORE
ADDITIONAL SECTIONS
Advocacy
Educate, discuss, take action
Networking
Sharing the work of others
Recent Relevant Publications
Summaries, links to selected professional, disability, disease journals and newsletters
Educational Opportunities
Conferences and webinars for health professionals and ventilator users
Industry
The business of living with a ventilator
Ventilator-Assisted Living
Vol. 36, No. 2, April 2022
Editor: Brian Tiburzi
Designer: Brian Tiburzi
ISSN 1066-534X
© 2022 Post-Polio Health International.
Permission to reprint must be obtained from Post-Polio Health International (PHI) at info@post-polio.org.
Ventilator users, health professionals, non-profits, company representatives – send comments and updates to info@ventusers.org.
Growing up with CCHS
Shortly after Jason Sloan was born in Peoria, Illinois, in 1977, he was diagnosed with Congenital Central Hypoventilation Syndrome (CCHS), a disorder of the autonomic nervous system that affects breathing. The most recognized symptom of CCHS is the inability to control breathing that varies in severity, resulting in the need for life-long ventilatory support during sleep in some patients or all the time in others. There are estimated to be 1000–1200 cases of CCHS worldwide.
“When he was born,” recalled his mother, Debra, “I thought that whatever was wrong with him could and would be fixed by all the medical personnel. I was a very young mother and had no clue that my life was about to change drastically forever. I still remember my Jason being born that day. He was so purple and hooked up to tubes and everything. A few days later they called in our pastor to give last rites. Remember, I'm a young mother. What did I think would happen? Again, I thought he would be ‘healed’."
Debra left the hospital after seven days without her son, traveling nearly 30 miles each way, every day, to sit in the rocking chair next to Jason. “Placing my hand thru that incubator, trying to talking to him, trying to kiss him, trying to comfort him...then I would make the long journey home, try to sleep and get back up the next day and start over.”
In early 1978, Jason was moved to Children's Memorial Hospital in Chicago. His doctor there, Carl E. Hunt, MD, made the suggestion to implant phrenic nerve pacers. This would allow Jason greater mobility and freedom. At the time, they didn’t expect him to develop the ability to speak or even eat independently. Though the surgery was successful, eight months later one of the pacers became infected and required surgery to replace it. As his mother explains, “No one knew what to expect at the time as this was such new technology. But the surgery was successful.”
Jason, with mother, Debra
Jason spent most of his time at Children’s Memorial over the next four years before coming home for good in December 1981. At that time, Jason was using a Puritan Bennett MA-1 ventilator, which Debra called “a blessing” due to its portability. Prior to that he had used an older Emerson ventilator. “It was a dinosaur, even at that time,” she said. “It would break down from time-to-time, and we would have to go to the hardware store to fix what we could.”
Once home, Jason flourished. “He went to school, made friends, participated in the Special Olympics. We were trying to live a normal life, but what is ‘normal,’ right?” said Debra. “When he was little, Jason tried so hard at his tasks and chores and always wanted to please others by doing his best. Teeth brushing? He should get a trophy! He was an expert at that. He was so cute because he always had to show me his results. I started that little scenario (checking out his work), but he really enjoyed getting the praise for a job well done.”
Adulthood brought new challenges. “There were no adult programs in place. We met with a few, and they said they wouldn’t take Jason, as he was on a ventilator,” said Debra. “You can imagine my response after hearing that on more than one occasion over the years.”
Jason required 24-hour monitoring. When he was 23, his phrenic nerve pacers finally stopped working. “They didn’t know if we had burnt out the phrenic nerve or if it was scar tissue. I made a hard decision not to put Jason through another surgery,” said Debra. The decision, though, meant he would need to use his ventilator and supplemental oxygen fulltime.
About eight years ago he was diagnosed with pulmonary fibrosis, which meant the pacers would not have been adequate even if they were still working. In addition, Jason has a seizure disorder, developmental disorder, interstitial lung disease, congestive heart failure and cerebral palsy.
Debra has taken precautions to make sure they are prepared for emergencies. “If the power goes off, so does the oxygen concentrator and the ventilator. The suction machine won’t work, etc.,” said Debra. “We installed a whole-home gas generator so there would be no more worries. I highly recommend that to families. And it seems like the power always goes out at nighttime. Jason has round-the-clock caregivers. They either work an 8-hour or a 12-hour shift, but there is always someone in the home, even if it’s myself or my husband.”
The financial burden can be considerable. His mother worries about the shortage of nursing/caregivers, medications, medical supplies and specialized food. She pays almost $500/month for special OTC supplies, other medical supplies, supplies and gloves for the workers, and special hand soap, among other items. If they need extra help with Jason, that comes out of pocket. “What will happen to Jason, when the money runs out?” wonders Debra. “That is a fear and keeps me up at night.”
Covid has brought additional worries. Both Debra and her husband contracted Covid earlier this year. The residual effects have made it more difficult to attend to Jason’s needs. Two of his caregivers have also tested positive. Luckily, Jason has avoided it thus far, but still they worry.
Yet they persevere and are thankful for the time they have together. “His smile is infectious,” says Debra. “He is a true joy to be around. Jason has a talent with charisma and charm that many definitely don’t have!”
ADVOCACY
Plan to Boost HCBS Slow to Take Effect
The American Rescue Plan Act, signed into law in March 2021, promised to increase funding for Home and Community Based Services (HCBS) by as much as $25 billion, alleviating chronic staffing shortages. States were expected to use these funds to buttress wages, move people off waiting lists for disability services, train more workers and expand covered services for those who are elderly and people with disabilities, helping to keep them out of nursing homes.
However, according to a new report by Kaiser Health News, a year later many states have yet to access much of that money.
ABLE Accounts
April is Financial Literacy Month, a great time to learn more about opening an Achieving a Better Life Experience (ABLE) Account. ABLE Accounts are tax-advantaged savings accounts that are available to certain people with disabilities. By opening an ABLE Account, you may be able to save more money each year to help you pay for disability-related expenses. The Social Security Administration (SSA) has more info at https://choosework.ssa.gov/blog/2022-04-21-2022-able-update.html.
New Rule Expands Airline Accessibility
The US Department of Transportation recently proposed a rule requiring that all new single-aisle aircraft with 125 or more seats have at least one accessible lavatory. The restroom would need to be large enough to allow a passenger with a disability to “approach, enter, and maneuver” and use all of the facilities — with the help of an assistant, if needed — and leave using the onboard wheelchair. At present, accessible restrooms are only mandated on planes with more than one aisle, meaning the majority of domestic flights in the US are exempt.
If you use a ventilator and are planning on flying soon, IVUN has an entire section filled with helpful information and resources: Flying With a Ventilator.
NETWORKING
Virtual CCHS Research Conference
The CCHS Foundation and CCHS Network are conducting a Virtual CCHS Research Conference on May 2nd, 2022 at 10am EST. The zoom meeting link is: https://openu.zoom.us/j/96182616922
The speakers for the conference are:
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Prof. Donatella Degl’Innocenti: Urinary oxidative biomarkers as a tool for follow-up of CCHS patients
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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
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Dr. Gad Vatine: Patient-specific stem cells to study mechanisms underlying CCHS
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/.
RECENT RELEVANT
PUBLICATIONS
Role of airway clearance therapies in neuromuscular disease
Sherri Katz, MD, FCCP
“Individuals with neuromuscular weakness have an impaired ability to cough and clear secretions from the airway, which can result in atelectasis and pneumonia. Proximal airway clearance therapies (ACT), including manual lung volume recruitment (LVR) and mechanical in-exsufflation (MI-E), mobilize secretions, improve cough efficacy, maintain chest wall compliance, and slow progression of restrictive lung impairment.”
CHEST Physician. 2022 Apr;17(4):21-2. Full text.
van den Biggelaar RJM, Hazenberg A, Cobben NAM, Gommers DAMPJ, Gaytant MA, Wijkstra PJ.
Home mechanical ventilation: the Dutch approach
“In the Netherlands we have an unique organisation of only 4 centres being responsible for all patients who need Home Mechanical ventilation(HMV). Nationwide criteria for referral and initiation of HMV are stated in our national guideline and recently a unique national learning management system (LMS) for all caregivers and professionals was developed. A nationwide multi-centric research program is running and every centre is participating. In this paper we provide information about the evolution of HMV in the Netherlands during the last 30 years, including details about the number of patients, different diagnose groups, residence and the type of ventilators.”
Pulmonology. 2022 Mar-Apr;28(2):99-104. doi: 10.1016/j.pulmoe.2021.11.001.
Neunhoeffer F, Miarka-Mauthe C, Harnischmacher C, Engel J, Renk H, Michel J, Hofbeck M, Hanser A, Kumpf M.
Severe adverse events in children with tracheostomy and home mechanical ventilation - Comparison of pediatric home care and a specialized pediatric nursing care facility
“We observed no difference in the rate of severe adverse events between home care and specialized nursing care facility (0.21 [y-1]; median 0.0 (0.0-3.0) versus 0.23 [y-1]; median 0.0 (0.0-1.6); p = 0.690), however, significantly more tracheostomy related incidents and infections occurred in the home care setting. Young age (<1 year) (Odds ratio 3.27; p = 0.045) and feeding difficulties (nasogastric tubes and percutaneous endoscopic gastrostomy) (Odds ratio 9.08; p = 0.016) significantly increased the risk of severe adverse events. Furthermore, the rate of severe adverse events was significantly higher in patients with a higher nursing score.”
Respir Med. 2022 Jan;191:106392. doi: 10.1016/j.rmed.2021.106392.
EDUCATIONAL
OPPORTUNITIES
ATS 2022 International Conference
The American Thoracic Society 2022 International Conference will be held May 13-18, 2022, in San Francisco, California. Get more info at https://conference.thoracic.org.
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/.
INDUSTRY
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.