May 6, 2017

Polio survivor group talks of legacy and a new battle



At six-years-old, Bobby Doherty from Belfast wasn't playing or riding his bike - he was lying entombed in plaster from head-to-foot in Musgrave Park Hospital after contracting polio. 
More than half a century after the infection first struck, he and a group of fellow polio survivors still meet up each week in the Short Strand Recreation Centre. 
They play games, reminisce over a cup of tea and support each other in coping with the effects of a disease, which many people think doesn't exist anymore. 
Now in his 70s, Mr Doherty still vividly recalls falling ill. 

Bobby Doherty
Image captionMr Doherty still uses leg braces to get about

"It was September 1949, a lovely sunny Friday evening and I remember my father taking me to the GP but when we got there the surgery was closed," he said. 
"The doctor turned up at my house on Monday morning, tickled my left foot and said 'I'll be back in an hour with an ambulance or a consultant.'
"An hour later I was in an ambulance heading to the Purdysburn Fever Hospital."

'Fear of wasps'

Mr Doherty was to remain in hospital for more than a year with the serious viral infection, which causes muscles to shrink, paralysis and in some cases, death. 
When he was discharged, polio had left a permanent mark. He was paralysed in both legs and was fitted with leg braces, known then as calipers, which he still uses to get about. 
Another long-standing member of the NI Polio Fellowship is Eddie McCrory, who contracted the disease in 1957, aged five, during its largest outbreak in Northern Ireland. 

Eddie McCrory
Image captionMr McCrory was completely paralysed at first

For him too, the memories are sharp. 
"My father said I would only be in hospital for the night - and I remember falling out with him when I had to stay much longer than that," he said.
"I was in an isolation ward with three others, and my mother and father would come to the window and look in. She would cry and run away. 
"I was completely paralysed at first and I remember a wasp coming into the ward. The other children could cover themselves with their sheets but I couldn't. 
"It was terrible and I've had a fear of wasps ever since."
Mr McCrory, who is originally from Belfast's Short Strand but now lives in Carryduff, was left with a severe curvature of the spine. 

A young Eddie McCrory

Polio is now all but eradicated in the UK thanks to a highly-effective vaccine introduced in the 1960s. 
Polio does still exist worldwide, although polio cases have decreased by more than 99% since 1988, from an estimated more than 350,000 cases to 74 reported cases in 2015. 
Only two countries in the world have never stopped transmission of polio - Pakistan and Afghanistan.

Polio Fellowship Group meeting
Image captionThe NI Polio Fellowship playing some sport

But for the friends who still gather in east Belfast, the legacy of the disease lives on, not only in their disabilities but in a new battle with what's known as post-polio syndrome. 
Mr McCrory explains that while polio was once considered a static disease, it is now thought fresh symptoms can emerge up to 60 years later. 
"I have a diagnosis of post-polio syndrome - I can feel tired, really fatigued and then there are aches," he said.
"Not awful aches but in the parts where I didn't ever have polio. I'm very sensitive to cold. My feet and legs are like blocks of ice because the body doesn't respond quickly enough to changes in body temperature."

'Contented life'

He says there's a lack of awareness of the condition - not helped by the fact many polio experts are retired or deceased.
"Young doctors would have no idea," he said. "When I go to see the neurologist and he sends me to the physiotherapist, she'll often bring in her students and say - this is someone who had polio."
Dr Ultan Power, a molecular virology specialist from Queen's University Belfast, says post-polio syndrome is a neurological disease evident in people who had polio.

Dr Ultan Power
Image captionDr Ultan Power says no treatments are currently available for post-polio syndrome

"I've asked a few of my colleagues had they heard about post-polio syndrome and they all said no," he said. 
"My understanding is it is not well-known because of the virtual eradication of polio in society."
There are no treatments available but some clinical trials are ongoing, he added.
"One involves using antibodies from people who have the polio virus, which is pretty much everybody as we are all vaccinated," he said.
"They take them from healthy individuals and transfuse them into people who are suffering from post-polio syndrome. 
"The hope is that it will have an impact on the residual virus, help eliminate it and improve function for individuals - i.e. their ability to walk - and live a generally contented life."
From

Post Polio Litaff, Association A.C _APPLAC Mexico

May 2, 2017

On the brink of eradication: Why polio research matters



IMAGE: THIS IMAGE SHOWS A TRANSMISSION ELECTRON MICROGRAPH OF POLIOVIRUSES. view more 
CREDIT: DR GRAHAM BEARDS AT EN.WIKIPEDIA
Poliovirus research offers insights into other viruses that impact global health

In the decades since Dr. Jonas Salk developed the first polio vaccine, cases of polio have exponentially declined. Though once widespread epidemic, the highly infectious childhood disease is now close to global eradication. 

The question remains: why would researchers spend time and resources studying a virus already on the brink of total eradication? 
In a new Research Matters article, PLOS Pathogens author and microbiologist at the University of Texas Southwestern Medical Center, Julie K. Pfeiffer discusses why she studies poliovirus, and shares how her research has affected the study of other viruses. 
For Dr. Pfeiffer, there are several benefits to studying poliovirus. Poliovirus "grows like a weed", able to produce immense stocks that are easy and safe to work with since its genome can be targeted, mutations can be made within days, and a vaccine already exists for it. 
Most importantly, because poliovirus has already been exhaustively studied, it can serve as a useful model system, a virus that can be studied to understand the workings of other similar viruses.
Early in her career, Dr. Pfeiffer showed that RNA viruses such as poliovirus, benefited from a "sloppy replication strategy". As RNA viruses replicated, they produced genetic mutations, some of which benefited them. This discovery was later used show that other RNA viruses including chikungunya, also relied on sloppy replication strategies. 
In addition, Dr. Pfeiffer's research has shown that poliovirus "sticks to bacteria", aiding its infection and transmission. Her work, along that of other research groups, has shown that many gut viruses rely on intestinal bacteria to infect humans. Dr. Pfeiffer's findings were applied to human norovirus, a virus that can lead to severe infections with explosive vomiting and diarrhea, opening the door to prevention strategies. 
By studying an eradicated virus like poliovirus, scientists like Dr. Pfeiffer can learn more about other viruses that pose a threat to public health such as Ebola, Zika, and influenza. This type of basic research could potentially lead to new treatments and vaccines for these viruses. 
###
In your coverage please use this URL to provide access to the freely available article in PLOS Pathogenshttp://journals.plos.org/plospathogens/article?id=10.1371/journal.ppat.1006330
Citation: Pfeiffer JK (2017) The importance of model systems: Why we study a virus on the brink of global eradication. PLoS Pathog 13(4): e1006330. doi:10.1371/journal.ppat.1006330
Funding: Dr. Pfeiffer's work is supported by NIH R01 AI74668, NIH R21 AI114927, a Burroughs Wellcome Fund Investigators in the Pathogenesis of Infectious Diseases Award, and a Faculty Scholar grant from the Howard Hughes Medical Institute. The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.
Competing Interests: The author has declared that no competing interests exist.
Disclaimer: AAAS and EurekAlert! are not responsible for the accuracy of news releases posted to EurekAlert! by contributing institutions or for the use of any information through the EurekAlert system.

May 1, 2017

Postpolio Syndrome Symptoms


Postpolio Syndrome

Synonyms and related keywords: PPS
  • Central 
    • Pathogenesis can include chronic pain, type A personality, depression, dysfunctional reticular-activated system, sleep disorders, and respiratory dysfunction. 
    • PPS produces somnolence and difficulty in concentrating and remembering.
  • Peripheral 
    • Pathogenesis may be metabolic exhaustion of the enlarged motor units, neuromuscular junction transmission defects, scarring within the motor neurons, or loss of motor units due to aging. 
    • PPS produces decreased muscular endurance and increased muscular fatigability.
  • Weakness 
    • A number of functional etiologies for weakness have been hypothesized, including disuse, overuse, and chronic weakness, as well as weight gain. 
    • Asymmetric and scattered weakness may be present. 
    • Some authors have found evidence that previously unaffected muscles later become weak; in these cases, they discovered that the patient was unaware or had not been told that the particular muscle had been affected during the acute episode.
  • Muscle pain 
    • Deep aching pain may be a component of a myofascial pain syndrome or fibromyalgia. 
    • This feature is extremely prevalent in PPS.
  • Gait disturbance: Difficulty with gait is caused by progressive weakness, pain, osteoarthritis, or joint instability; it is common in patients who previously used assistive devices but later discarded them.
  • Respiratory problems 
    • Respiratory disorders are most prevalent in patients with residual respiratory muscle weakness. 
    • These changes cause chronic microatelectasis, diminished pulmonary compliance, increased chest wall tightness, chronic alveolar hypoventilation, decreased cough and expiratory flow, and decreased clearing of secretions. 
    • The new respiratory difficulties are not only related to new respiratory muscle weakness but also to scoliosis, pulmonary emphysema, cardiovascular insufficiency, or poor posture. 
    • A central component also may occur because acute bulbar polio often affects the medullary structures, including the reticular formation and sleep regulatory system.
  • Swallowing problems 
    • These difficulties can occur in patients with bulbar and nonbulbar postpolio. 
    • Subclinical asymmetrical weakness in the pharyngeal constrictor muscles is almost always present in all postpolio muscular atrophy (PPMA) patients, including those who do not complain of new swallowing difficulties.
  • Autonomic dysfunction: The cause is unclear; the peripheral component could include muscular atrophy and, therefore, diminished heat production.
  • Sleep apnea
  • This disorder is not uncommon in patients left with residual bulbar dysfunction or severe respiratory compromise.
  • Sleep apnea appears to be due to a combination of the following:
    • Central apnea, due to a residual dysfunction of the surviving bulbar reticular neurons 
    • Obstructive apnea, due to pharyngeal weakness and increased musculoskeletal deformities from scoliosis or emphysema 
    • PPMA, resulting in diminished muscle strength of the respiratory, intercostal, and abdominal muscle groups
  • Flat back syndrome
  • Another possible symptom in some patients with PPS is the flat back syndrome, which consists of the inability to stand erect because of forward flexion of the trunk and pain in the low back and legs.
  • The flat back syndrome typically occurs in patients with diminished lumbar lordosis as a result of instrumentation of the spine for scoliosis, vertebral fracture, or degenerative joint disease.
  • The trunk extensor musculature plays an essential role in maintaining upright posture, and it may be that PPS-related weakness in this musculature represents a major contributing factor to the flat back syndrome in these patients.


Author: Flor M Muñiz, MD, Staff Physician, Department of Physical Medicine and Rehabilitation, Thomas Jefferson UniversityCoauthor(s): Gerald Herbison, MD, Clinical Professor, Department of Physical Medicine and Rehabilitation, Thomas Jefferson University
Flor M Muñiz, MD, is a member of the following medical societies: American Medical Association
Editor(s): Martin K Childers, DO, Associate Professor, Department of Physical Medicine and Rehabilitation, University of Missouri School of Medicine; Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, Pharmacy, eMedicine; Kat Kolaski, MD, Adjunct Clinical Assistant Professor, Department of Pediatrics, University of North Carolina; Director, Pediatric and Adolescent Rehabilitation, Charlotte Institute of Rehabilitation; Kelly L Allen, MD, Consulting Staff, Department of Physical Medicine and Rehabilitation, Lourdes Regional Rehabilitation Center, Our Lady of Lourdes Medical Center; and Denise I Campagnolo, MD, MS, Clinical Director of Spinal Cord Injury Program, Associate Professor, Department of Physical Medicine and Rehabilitation, New Jersey Medical School


Post Polio Litaff, Association A.C _APPLAC Mexico

Mayo Clinic Epidural Stimulation Trial Enables Movement and Stepping





On April 3, the Mayo Clinic published the results of a new trial that further validates the effectiveness of epidural stimulation for functional improvement in spinal cord injury subjects. In this trial, a 26-year-old, three years out from a motor-complete SCI at T6, regained volitional motor function below his level of injury while using an implanted epidural stimulation device. The study sought to replicate the results of a previous epidural stimulation trial conducted at the University of Louisville, in collaboration with the research team of V. Reggie Edgerton at UCLA.
The Mayo Clinic trial began with the subject, Jered Chinnock, undergoing 22 weeks of three times per week locomotor and task-specific strength training. Following the initial period of motor training, surgeons implanted an epidural stimulation device made by Medtronics and FDA approved for pain (with off-label approval for functional rehabilitation purposes) and connected it to an electrode array positioned on the dorsal epidural surface of the lumbosacral spine. After three weeks of post-surgical recovery, the team began epidural stimulation — with Chinnock attempting volitional leg movements while researchers tuned the settings of the stimulation device.
In the first two weeks of epidural stimulation, with the device turned on, Chinnock was able to:
•  Control his muscles while lying on his side, resulting in leg movements
•  Make step-like motions while lying on his side and standing with partial support
•  Stand independently using his arms on support bars for balance
Previous trials had produced standing and some volitional movement of lower limbs while reclining, but only after 17 weeks of stimulation and motor rehabilitation. The Mayo Clinic trial marks the first time a subject has been able to voluntarily make step-like movements while standing with partial support, and is the quickest turnaround for functional improvement with epidural stimulation.
“We’re really excited, because our results went beyond our expectations,” says neurosurgeon Kendall Lee, M.D., Ph.D., principal investigator and director of Mayo Clinic’s Neural Engineering Laboratory. “These are initial findings, but the patient is continuing to make progress.”
The preliminary results in this trial show functional return only while the epidural stimulation device is turned on. Previous studies have shown that after prolonged therapy, some volitional control of previously paralyzed muscles remains even when the stimulator is turned off.
It remains to be seen if Chinnock will regain even more function with continued therapy and adjustment of the stimulation settings, but even the initial findings are enough to show that functional return with epidural stimulation continues to move forward, one step at a time.
For more about Chinnock’s journey, see the below video.



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