INTERNATIONAL VENTILATOR USERS NETWORK
an affiliate of Post-Polio Health International
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VOLUME 33, NUMBER 1
Beginning in 2014, the US saw a drastic increase in the incidence of Acute Flaccid Myelitis (AFM). The condition is characterized by the sudden onset of severe muscle weakness, typically in the arms or legs, and mostly affects children. In the most severe cases, respiratory failure can occur, necessitating the use of ventilatory support.
IVUN recently spoke with Angie Andersen, whose daughter McKenzie developed AFM during the first wave of cases in 2014...............MORE
Frederick M. Maynard, MD
There have recently been several media reports about Americans developing a “polio-like illness” known as Acute Flaccid Myelitis (AFM) that leaves them partially paralyzed. Because PHI has received many calls from members about this topic, Dr. Fred Maynard was asked to write about what AFM is and to share his thoughts about how polio survivors might be able to help those who are affected by it................MORE
Camps for Ventilator-Assisted Children 2019
Information and dates for camps catering to children who use HMV
Vol. 33, No. 1, February 2019
Editor: Brian Tiburzi
Designer: Brian Tiburzi
Permission to reprint must be obtained from Post-Polio Health International (PHI) at email@example.com.
Ventilator users, health professionals, non-profits, company representatives – send comments and updates to firstname.lastname@example.org.
Navigating Life with AFM:
One Mom’s Story
Beginning in 2014, the US saw a drastic increase in the incidence of acute flaccid myelitis (AFM). The condition is characterized by the sudden onset of severe muscle weakness, typically in the arms or legs, and mostly affects children. In the most severe cases, respiratory failure can occur, necessitating the use of ventilatory support.
The specific cause of AFM is still unknown, but suspected viruses include EV-D68, EV-D70 and Coxsackie A-71. It typically starts out as a mild respiratory illness, and most children who contract the virus never develop AFM. Though relatively rare, the number of AFM cases has spiked every two years since 2014, with the greatest number of cases, 215, coming this past year.
IVUN recently spoke with Angie Andersen, whose daughter McKenzie developed AFM during the first wave of cases in 2014. McKenzie had one of the more severe cases of AFM, which required a tracheotomy and 24-hour ventilatory support.
IVUN: When did you first suspect that McKenzie’s illness might be more serious than a typical viral illness such as a cold or flu?
Angie Andersen: She got sick in December of 2014. She got out of school for Christmas break and had the sniffles. By Christmas Eve it had turned to pneumonia. My son and I had the same cold but got better, while she did not. I asked her pediatrician’s office about that virus that had been on TV. They had no clue, so it was dismissed. On December 27th, she developed a severe neck ache where she couldn’t tip her head back. Her neck was rigid, and she’d scream. I took her back again, and they said that since she could touch her chin to her chest, it wasn’t [anything serious] like meningitis. So, it’s just a stiff neck from being sick, and they gave her ibuprofen and an ice pack and said she’d be fine.
We went home, and she continued to get worse. I called her pediatrician and said, “What do I do? She’s getting worse, and I don’t want to get sent home again!” She told me to take her to another ER in [Corvallis, Oregon]. When I got her up, her legs went out from under her and her head was like a bobble head.
Corvallis ran a lot of tests and admitted her. They tried giving her a popsicle, but she couldn’t lift her right arm up to get it. She was struggling and getting scared, and so was I. They told us she tested positive for an enterovirus. They were sure it was EV-D68 but needed further testing. An MRI showed her brain and spinal cord were swollen. The part of her brain that was swollen controlled her breathing, and [we were told] she would stop breathing on her own, so Randall Children’s Hospital was on the way to get us.
On December 29th we were admitted, and within 24 hours she was completely paralyzed and intubated because she could no longer breathe on her own. We were told the virus had caused acute flaccid myelitis. Her spinal curvature got worse and worse due to the paralysis until it was over 90 degrees, badly affecting her quality-of-life.
IVUN: So, when you took her to the pediatrician, you already had it in your mind that it may be AFM, or what they were referring to at the time as a "polio-like illness"?
Angie: I actually called in to ask them about it. At first, it was just this bad cold, but my son and I got better after three days, and she got worse – a bit at first, then more. It just seemed weird and different to me, and I’d heard about [AFM] on the news and wanted information. Then a few days later, I had to take her to the ER because it was really bad, but there was no testing for anything, just an X-ray. If they had just tested for the enterovirus at that time, because a young child showing up with respiratory issues was a big indicator of her being at the tail end of the 120 kids across the country [already diagnosed with AFM]. But nobody there knew about it, so I took her home that same day. Just give her the medicine, and she’ll be fine in a couple days.
IVUN: How long was she in the hospital? What type of rehabilitation was received during this time?
Angie: She was in the hospital for six months – four months on the acute side, two months in rehab. The rehab was mostly focused on getting her mobile so she could go home. Not a lot of physicality. I asked several times why I saw other kids doing stuff but not her and was told she didn’t have the capabilities.
IVUN: What preparations were made to transition back to the home? Did you feel like her team properly educated and prepared you to make this transition?
Angie: She was 7 years old, so I had about six pages of things I had to get training on and sign-off on before I could take her home. They got her all fitted in her chair and able to drive herself around with her left hand. They trained me very well.
It took me two years to get her there, but, like a lot of families of kids with AFM, we made it to Kennedy Krieger Institute (KKI) in Baltimore for intensive rehab. I got her there for three months of therapy, and it was amazing. Instead of “why bother,” it was “why not!” She did things I didn’t know were possible!
We’ve had a lot of setbacks since then, and now insurance won’t pay for us to go back again because they said her progress didn’t warrant the cost! What she did there was priceless beyond measure, and after I get her legs fixed this coming summer, I will find a way somehow to make it happen.
IVUN: Do you mean the previous rehab staff sometimes took a stance of "why bother" with regards to teaching her certain skills?
Angie: I love them here but at that time, when I asked why they would do all the stuff in the gym with other kids and not her, or if there was something they weren’t telling me, he said she just didn’t have the capability. It was all new to me, so I did what I had to do until I could take her home. We did some outpatient rehab, but I just had no idea what she was capable of until we went somewhere that deals with spinal cord injuries and had worked a lot with transverse myelitis (TM) and AFM.
The rehab was really hard for her, but she was doing so good before we left. She still had more to go, but I saw huge things I never knew was possible. [They] just had a different mentality. Yes, she’s a hard case, in pain a lot, but they knew how to work through it and help her! I wish there was a KKI on the West Coast.
Their mantra at KKI in the spinal gym was "Hope through Motion!" activity-based therapy. I ended up choosing to do home-based therapy for my daughter after KKI. I tried coming back up to Portland once a week, but it was really hard. If I didn’t have a nurse that day, we couldn’t come. If she was sick, she couldn’t go. Home health can come to the house regardless. After she gets her legs fixed this summer, then she can bear weight and start doing all the good therapy she needs to do.
IVUN: Once you transitioned back home, what kind of support was available to you? You said above you had home health aides. How often did they come? Did you have other family or friends nearby to offer support?
Angie: A PT comes once, sometimes twice, a week. An OT comes twice a week. I have nursing help through the medically fragile program. I fight to get 18 hours a day. It’s really hard to get that filled, so a lot of the time it’s just me. My son can help me at times with different things, if needed. I’ve never had respite care. Her care needs are so specialized that it’s hard to find anyone she’d be okay with. Friends and family are supportive, but nobody that knows her cares to relieve me.
IVUN: What kind of ventilator does your daughter use? Did you have an fears or concerns, specifically about her trach care, when you left the hospital?
Angie: She uses a Trilogy 100 and is 100% vent dependent. We have a medical company that monitors the vent. The RTs at the hospital trained me well on her trach care, so I was okay with that.
IVUN: What were some of the unexpected challenges for you both once she transitioned to home?
Angie: The biggest challenge is fighting to get hours of nursing for her. And, also getting those hours staffed, which for a trached, vented pediatric patient is hard. The nurses don’t get paid much, so it’s hard to keep anyone good because they leave for better jobs. I have a few now that are going to stick around, but this is a big epidemic across the country with finding nursing care for medically fragile kids. I’m grateful for what I’ve got. Some have it worse.
We had to live in a hotel room for a year until we had an accessible home to take her home to. Getting her to appointments was and is hard. It takes a lot, and, if she needs suctioning or a cough, I have to do it. Other challenges: having younger people at appointments who check us in as late even if I called to let them know; getting scolded and talked down to for not getting there on time; fighting with insurance to get some of the things needed for her care; lack of nursing help, making it so hard to get her places for appointments or anything really; keeping all medical supplies, appointments, school, medicines everything current and up to date so she has everything she need (it’s never ending); getting people/doctors to see more than just her outward appearance; being able to be all things for her and still be a mom to my son and a wife.
I’ve never really had a moment to myself in the last four years to even process what happened. I just take it as it comes because my miracle was that she survived. The rest I can deal with.
IVUN: What advice do you have for families of children newly diagnosed with AFM?
Angie: The advice I would give new families is that no matter the extent of your child’s condition, immediately do inpatient rehab and then continued PT and OT. Keeping them moving is key. Even if you don’t get a lot of response, keep going the best you can. Find a place like KKI in Baltimore that believes in your kid and their abilities, not their disabilities! Be a voice/advocate, and if you know your child needs something, keep fighting until you figure out a way to make it happen. Make sure the doctors listen to you and your child. Really listen! Four years out and I’m still battling for what she needs. Take a breath when you can! Try and take care of yourself so you can continue to be your best for them.
IVUN: McKenzie, what message do you have for those newly diagnosed with AFM?
McKenzie: I’m paralyzed from the neck down and on a ventilator. I was in the hospital for 6 months! I was so discouraged at first. What encouraged me was my friends, the thought of meeting other people exactly like me. I love art/painting/inspirational speaking/singing/writing/writing music/acting.
When I came home from the hospital, I thrived. I did therapy and got movement in my right thumb back and now my pinky and ring finger. I still have lots of work to do, but I’m thriving! Slow progress is better than no progress!
Angie and McKenzie with members of the Portland Trailblazers
PHI/IVUN's Statement on AFM
Frederick M. Maynard, MD
AFM is the descriptive name given to a clinical condition characterized by the sudden (acute) onset over 1-3 days of a floppy (flaccid) severe muscle weakness (paralysis). There are several neurological conditions that can cause acute flaccid paralysis but modern testing can show if the condition results from inflammation in the spinal cord (myelitis). For centuries the poliomyelitis virus was the major cause of AFM but it has now been virtually eliminated (less than 30 cases worldwide in 2018) through massive vaccination efforts. Unfortunately, there are other viruses that can invade the nervous system of infected people and also produce a myelitis that sometimes results in death of motor nerve cells in anterior horn areas of the spinal cord and in variable degrees of scattered weakness in muscles of limbs, trunk, breathing, swallowing and the face.
History of AFM
Since 2012 there have been scattered outbreaks of AFM in the U.S. thought due to several different viruses: Enterovirus D-68 and D-70, Coxsackie A-71, and West Nile. The latter is spread by mosquito bites but the others spread between people. These viral infections commonly produce flu-like symptoms and only rarely cause paralysis. Only specialized testing can confirm a cause for AFM. Children appear to develop AFM from these viral infections much more often than adults. While there may be some differences between the clinical pictures of cases due to different viruses, they seem to be minor and all cases with residual paralysis closely resemble cases of poliovirus-caused paralysis. Thus, newspapers often refer to “polio-like paralysis.”
The Centers for Disease Control and Prevention (CDC) has been monitoring cases of AFM in the U.S. for at least a decade. Since 2016 there have been 100-200 cases yearly, and 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).
Little has been said in the medical literature about therapy and rehabilitation for people with residual weakness from AFM. Information for parents and families from the CDC does mention that “doctors will recommend Physical Therapy or Occupational Therapy to help with arm or leg weakness from AFM.” The CDC AFM Task Force has no members who are rehabilitation specialists.
As a result of limited information about what people with residual AFM weakness can expect in the way of recovery and functional restoration, and sometimes due to health care professionals being uncertain of what to prescribe for involved children, parents can become very anxious, confused and sad. A recent CNN story highlighted this situation. One physical therapist was able to help a young child improve their functional recovery only because she looked up older information about polio rehabilitation and followed similar treatment techniques.
Another story tells of a mother who longed for support and guidance from other parents of AFM-weakened children, but her son was the only one of his kind. She found tremendous help and relief by taking her son to a post-polio support group meeting where other people who had had similar problems as children could share life experiences. She was able to observe and learn firsthand about polio survivors’ successful adaptive behaviors and to receive “peer guidance” and reassuring support about recovery and rehabilitation efforts, child rearing techniques and long-term outcomes.
PHI's Helping Role
We at PHI are calling attention to the CNN story because we want to encourage PHI members to watch for possible opportunities for them and their polio friends to be available to other AFM-affected people and to offer similar support, as well as to steer them to information about polio rehabilitation and its outcomes on PHI’s Polio Place website.
The essentials of AFM rehabilitation, as with acute polio rehabilitation, include the following:
Individually designed exercise programs to slowly increase strength, maintain joint and muscle flexibility and control pain.
Encouragement of functional adaptations with or without adaptive specialized equipment (e.g. braces, crutches, wheelchairs, etc.) for regaining optimal independent mobility and self-care.
Emotional support and encouragements to establish high self-esteem and optimism about the future.
Consideration of rehabilitative surgery options for restoring function, usually timed after improvements from other efforts have reached a plateau and to be appropriate for childhood growth and development.
I shared the CNN story with physician members of PHI’s Medical Advisory Committee and asked them about their experiences with AFM patients. Several members had seen a few cases of AFM. All who responded agreed that rehabilitation services/programs modeled after and like those used effectively for people with paralytic polio be used.
I invite you to join us at PHI and IVUN in spreading this message.
Ref. 1: Martin JA, Messacar K, Yang ML, et al. Recognition and Management of AFM in Children. Neurology 2017;89: 129-137.
CHAMP Camp 2018 will be June 16-20, 2019 at Bradford Woods, Martinsville, Indiana. The theme for this year is “Out of This World.” All applications are due by March 1, 2019. Make sure to get in on time, and we hope to see you at camp! If you have any questions, please call 317-752-5668 or send an email to email@example.com.
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. This year's dates are June 2-7.
FRESH AIR CAMP
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
Carrie Ann Lucas, Prominent Disability Rights Advocate, Dies At Age 47
Nationally-known disability rights activist Carrie Ann Lucas, who lived with a rare form of muscular dystrophy, passed away on February 24th at the age of 47. Lucas, a ventilator user, helped pass legislation protecting the rights of parents with disabilities. She also worked with the organization Not Dead Yet and spoke out against so-called "right to die" legislation.
Her death was announced by family and friends on her Facebook page: “[Lucas] died after an arbitrary denial from an insurance company caused a plethora of health problems, exacerbating her disabilities and eventually leading to her premature death.”
Lucas, from Windsor, Colorado, began her career as a teacher but later went back to school and earned her law degree after facing discrimination when trying to adopt a child. Lucas founded a nonprofit, Disabled Parents Rights, to help parents with disabilities through legal representation and advocacy. She eventually adopted four children of her own, all with disabilities.
She was an outspoken supporter of Medicaid and buy-in programs to enable disabled people who need personal care attendant services to be employed. She was an activist with ADAPT and was arrested in 2017 outside the office of Sen. Cory Gardner while protesting against the repeal of the Affordable Care Act.
Her Facebook page detailed the circumstances of her death: “In January of 2018 she got a cold which turned into a trach and lung infection. Her insurance company UnitedHealthcare, refused to pay for the one specific inhaled antibiotic that she really needed. She had to take a less effective drug and had a bad reaction to that drug. This created a cascade of problems, loss of function (including her speech). United Healthcare’s attempt to save $2,000 cost over $1 million in health care costs over the past year. This includes numerous hospitalizations, always involving the Intensive Care Unit which is par for the course for ventilator users.”
Patient Advocacy Summit Brings RTs Together with Patients, Families
The American Association for Respiratory Care (AARC) brought together respiratory therapists, families, patients and advocacy organizations for a Respiratory Patient Advocacy Summit at AARC Congress 2018 in Las Vegas this past December. The event is intended to foster collaboration in advancing the quality of care for chronic respiratory patients and features talks by patients and their caregivers, as well as a roundtable discussion on the key issues facing patients and families. A video recap is avaiable on AARC's website.
National Respiratory Patient Advocacy Award
American Association of Respiratory Care (AARC) and The FACES Foundation are accepting nominations for the National Respiratory Patient Advocacy Award. The award commemorates a vital member of the healthcare team: the respiratory therapist. The award honors RTs who appreciate the “individual” instead of the “patient” and are dedicated to the principal that every breath matters. Nominations come from facilities across the continuum of care where registered respiratory therapists are actively engaged as a champion of patient family centered care.
PPMD Publishes Resources for Appealing Denial of Assistive Devices
PPMD published these resources as a response to notes from parents and rehabilitation teams who were frustrated with the process to get equipment and devices necessary to improve the lives of their children and families.
PPMD worked with parents and providers to develop sample templates for letters of appeal for parents, physical therapists and medical providers. The templates include space for professional review and recommendations, as well as space for personalization and individuation. They note that they have been tested by several parents, with largely positive results. They add that while using these templates is not a guarantee that your requests will gain approval, they hope that they will help ease the burden of the appeals process.
A Mechanical Intermittent Abdominal Pressure Ventilator: A Case Report.
Bach JR, Radbourne M, Potpally N, Chiou M.
“Pneumatically driven intermittent abdominal pressure ventilators (IAPVs) were a popular means of daytime ventilatory support until the late 1960's paradigm shift to invasive (tracheostomy) mechanical ventilation (TMV). However, although many patients still use IAPVs, currently available turbine-driven portable home care ventilators are not powerful enough to always successfully operate them. We describe a new mechanically driven IAPV operated by a 1 lb. motor that provided a depth of abdominal compression of almost 2 inches in 1.05 to 1.13 seconds to normalize alveolar ventilation for a 72-year-old post-polio survivor. It increased her autonomous 200 to 320 ml tidal volumes by over 300 ml to normalize her respiratory rate, relieve her dyspnea, and to maintain normal oxyhemoglobin saturation levels throughout daytime hours over a 9-month period of continuous ventilatory support.”
Am J Phys Med Rehabil. 2019 Feb 5. doi: 10.1097/PHM.0000000000001151. [Epub ahead of print]
Development of a Web-Based Peer Support Program for Family Caregivers of Ventilator-Assisted Individuals Living in the Community: Protocol for a Pilot Randomized Controlled Trial.
Wasilewski MB, Nonoyama M, Dale C, McKim DA, Road J, Leasa D, Goldstein R, Rose L.
“Across Europe, Canada, Australia, and the United States, the prevalence of home mechanical ventilation (HMV) prevalence is 6.6-12.9 per 100,000. At-home ventilator-assisted individuals (VAIs) are often vulnerable and highly comorbid, requiring complex care. In Canada, most VAI care is provided by family, leading to poor health-related quality of life and increased caregiver burden. No supportive interventions or peer support programs are tailored to VAI caregivers. Owing to the financial, geographic, and time limitations, Web-based support delivery may especially meet VAI family caregiver needs. We have developed a peer mentor training and Web-based peer support program for VAI caregivers including information-sharing, peer-to-peer communication, and peer mentorship.”
JMIR Res Protoc. 2019 Feb 6;8(2):e11827. doi: 10.2196/11827.
Home mechanical ventilation for chronic obstructive pulmonary disease: What next after the HOT-HMV trial?
Suh ES, Murphy PB, Hart N.
“Future work will need to focus on health service delivery and establishing cost‐effective clinical pathways that will deliver HOT‐HMV treatment to appropriate patients. In addition, researchers must focus on priority areas including clinical and cost‐effective treatment of patients with COPD–OSA overlap and COPD patients with episodic nocturnal hypoventilation utilizing auto‐titrating ventilation technology.”
Respirology. 2019 Feb 7. doi: 10.1111/resp.13484. [Epub ahead of print]
Long-Term Ventilation in Neuromuscular Patients: Review of Concerns, Beliefs, and Ethical Dilemmas.
Crimi C, Pierucci P, Carlucci A, Cortegiani A, Gregoretti C.
“NIV should be used early in the course of respiratory muscle involvement in NMD patients and its requirements may increase over time. Therefore, training on technical equipment at home and advice on problem solving are warranted. Remote monitoring of ventilator parameters using built-in ventilator software is recommended. Telemedicine may be helpful in reducing hospital admissions. Anticipatory planning and palliative care should be carried out to lessen the burden of care, to maintain or withdraw from NIV, and to guarantee the most respectful management in the last days of NMD patients’ life.”
Respiration. 2019 Jan 24:1-12. doi: 10.1159/000495941. [Epub ahead of print]
NAMDRC 2019 Annual Conference
NAMDRC will host its 2019 Annual Conference from March 14-16, 2019 at the Fairmont Sonoma Mission Inn, Sonoma, CA. The 2019 program is entitled, “NAMDRC 2019: Advances in Pulmonary, Critical Care and Sleep Medicine” and features the following keynote speakers Bartolome Celli, MD, Professor of Medicine at Tufts and Harvard Medical School and E. Wesley Ely, MD, Professor of Medicine at Vanderbilt University School of Medicine. Download the brochure or register online.
Canadian Respiratory Conference
ATS Conference 2019
A second conference will take place in 2019 at the Poughkeepsie Grand Hotel in Poughkeepsie, New York on Wednesday, October 2nd, 2019. This well-established conference will be celebrating its 40th anniversary and is one of the longest continuously running Respiratory Care & Sleep Medicine conferences in the country.
AARC Congress 2019
AARC Congress 2019 will be held November 9–12, 2019, in New Orleans, Louisiana. Highlights from the recent 2018 Congress can be found on their website.