Author: SSAdmin

Making a huge mobility difference

What is the Alinker? The Alinker is a non-motorized walking-bike without pedals. With an adjustable saddle and handlebars, it is custom designed to challenge assumptions about disability.

The Alinker is for people who identify with being an active person (who they are) but happen to have an illness or condition that impacts their mobility (what they have). It is for people who want to maintain an active life and support their wellness practices.

Lori, an HSP community member who lives in Austria (yes, Austria not Australia) writes:

“Selma Blair, a famous actress and mum and someone battling MS, posted herself on the Alinker walking bike with the caption: I got places to go! I responded, thinking it was super cool and that it could possibly be what I had been looking for…something to help me exercise and strengthen my nearly non-existent hamstring muscles. I got my Alinker in Zurich from Barbara Alink, the inventor of the device, and it has literally changed my life.

My first try: I could barely ride it. I had no business saying, “Yes, I’ll take it.” But, I knew, that it was the right decision. I knew that I was going to have to practice every day to make it work for me. Ideally, you should put your full weight on the seat and walk. I don’t think I need to say that having HSP makes that idea very challenging. In my case, my hamstrings are so weak, that I don’t have enough force to push the ground in order to propel myself forward. Therefore, I must come off the seat, in order to have more force with which to push off.

Two weeks later: I walked with the Alinker 4 times around a track (1600 metres). It took me 107 minutes. Two weeks later again, I walked that same distance with the Alinker but it only took me 85 min. I shaved 22 minutes off my time. My hamstrings are stronger! I can do one complete standing hamstring curl (couldn’t get it off the ground weeks ago) and I am closer to sitting comfortably on the seat.

I use the Alinker at the school where I teach to get from room to room. I use it in the classroom so I can teach and I use it at the track so I can exercise. The most important thing though, is that it has given me legs. It has given me the freedom to imagine that an experience is not off limits because of my limited mobility.

To make those experiences possible, the Alinker is foldable for easy transport. After only a little over 4 weeks since starting riding the Alinker I had already noticed a difference. This

Unfortunately, my kitchen is way too narrow for the Alinker!. It remains at school from Monday evening until Thursday afternoon, when I bring it home. I use it every day in school in order to get from class to the teacher’s room or to go to the cafeteria or the bathroom. I have used it to go clothes shopping with my 13 year old daughter – an activity that was not possible before the Alinker. Now, I can accompany her and we remain at eye level. We have fun. She can even push me on it, if we need to quickly go someplace else. I have used the Alinker at an outdoor mall, to get from store to store. I have used it to go for walks with friends. It has been my flying companion on eight occasions and has even travelled with me on a train. It affords me the possibility to do many things that were not possible before…the Alinker is my legs.”

Here are some photos from the Internet showing where people go with their Alinker walking bike:

https://www.pinterest.ca/thealinker0198/where-to-go-with-your-alinker/

The Walking Bike for an Active Life. Learn more here . . . https://www.thealinker.com/

The Australian distributor is the care kiosk. Phone number: 1300 110 159. Email: info@thecarekiosk.com.au

Here is the page on their website about the Alinker. Contact them by phone or email to talk about availability to try, rent or buy.

Wins first world title in wheelchair motocross

 

We have previously published articles on this website on inspirational Welsh teenager Lily Rice.

Unsurprisingly to anyone who has followed her journey, she has taken her achievements to the next level, claiming her first world title in wheelchair CMX… Think daredevil wheelchair riding at a skatepark!

Teenager Lily Rice says the sport of WCMX changed her life. WCMX stands for wheelchair motocross – riding a wheelchair at a skatepark.

Click here to watch the video.

Lily, who says she is “differently abled”, wants to expand the sport, encourage others to do it and get WCMX into the Paralympic Games.

SOURCE: 8 Jan 2020, Disability Sports

‘Wheelchair motocross changed my life’

Read more . . . https://www.bbc.com/sport/av/disability-sport/51031189

HSPer working to increase accessability

 

Golf is a wonderful sport that can help people with a range of disabilities to thrive. The health, social and competitive benefits can be remarkable, something Paul Ellison has discovered as golf has also helped him to make his living at the Home of Golf no less, in St Andrews, Scotland.

 

 

Paul is one of 18 EDGA golfers who are featured in a 2019 book called ‘MULLIGAN – tough love and second chances’. The inspiring stories of these players are sure to be of interest to many readers (see end of article), while for each copy secured by donation, a hospital/clinic also benefits from receiving a free copy.

To set foot on the Old Course, St Andrews, is special for any golfer, but to walk the hallowed turf as a player and caddie more than 5,000 times is a rare privilege which Paul Ellison has both enjoyed, and endured. For when taking over 50 million steps on the course, much of the time each footstep has been painful for this friendly Scot.

Paul is an accomplished golfer, but for the last 30 years his life’s work has been helping players to enjoy their experience of the links. First as a caddie for two decades, with players such as actor Samuel L Jackson benefiting from his sage advice, while more recently – as his disability of Hereditary Spastic Paraplegia has progressed – as one of the people who make sure that the players and caddies are all where they are supposed to be, in his working role for the St Andrews Links Trust.

His HSP condition is progressive, and in its mildest form renders those it affects with stiffness and spasticity of the legs. By practising Pilates to lengthen the muscles and tendons, while keeping active, he hopes to slow any progression which would further reduce his mobility.

Golf has given Paul a gift and he believes the sport is missing an enormous opportunity as it is an ideal game for players of all abilities. Even having the chance to just hit a few balls at the local range can be therapeutic and a way into golf for many with a disability – a steppingstone into the sport.

In the last year, Paul has been able to help promote the work of EDGA, the international body that helps people with disability to thrive through the power of golf. Paul has represented EDGA at awareness events, including those held with leading audit, tax and consulting firm RSM. RSM is supporting EDGA as together they work to encourage 500,000 new players to try the game.

EDGA (formerly the European Disabled Golf Association) is a not-for-profit, volunteer organisation, active in the promotion and delivery of opportunities to sample, participate and compete in golf. EDGA provides advice relating to golf for disabled players to The R&A, The International Golf Federation, The European Tour and The European Golf Association on golf for the disabled.

EDGA was founded in the year 2000 in Wiesbaden and has a membership of 28 National Federations, in 2018 nineteen approved events took place, and in 2019 it’s expected to exceed forty. EDGA’s members are now located worldwide with 25% of the membership from outside of Europe. Recent members include Golf Australia, New Zealand Golf, The Indian Golf Union, The Costa Rica Golf Association, The Argentina Golf Association, Golf South Africa, The Israeli Golf Association and The Lebanese Golf Federation.

Focusing on growing the game, EDGA has designed and implemented coach education programmes for delivery in rehabilitation centres, hospitals and golf clubs. All of these initiatives are available to the members of EDGA, as are protocols for tournament administration, definitions of impairment and other programmes which are available to grow the number of golfers with disability around the world.

“live life to the full through playing golf”

EDGA has worked to develop key partnerships with some of the worlds top names in golf and we continue to work to grow these relationships.

Learn all about EDGA here: https://edgagolf.com/online/www/index.html

Find GolfAustralia information here: https://www.golf.org.au/

Visit www.edgagolf.com/book for details of the Give and Gift campaign, and the opportunity to donate and receive a complimentary copy of ‘MULLIGAN – tough love and second chances’ as a token of EDGA’s appreciation.

How to Contact Paul Ellison

Facebook: Paul.Ellison.967
Twitter: Paul Ellison 20

 

SOURCE: Portugal Resident, 5 December 2019

Paul Ellison – every step he takes

Read the entire article here: https://www.portugalresident.com/paul-ellison-every-step-he-takes/

 

Patience, ambition and pragmatism

 

In this deeply charming and humorous talk, DJ and self-professed pirate Tom Nash meditates on how facing adversity due to disability invited patience, ambition and pragmatism into his life in enlightening, unexpected ways. “We all have unique weaknesses,” he says. “If we’re honest about what they are, we can learn how to best take advantage of them.”

 

 

 

SOURCE: TEDxSydney  Feb 2019

 

Full website reference: https://www.ted.com/talks/tom_nash_the_perks_of_being_a_pirate_feb_2019

New data sharing tool VariantMatcher

 

VariantMatcher (variantmatcher.org) is a new data sharing tool developed by researchers at the Johns Hopkins University in the US.

 

The principal goal of VariantMatcher is to help solve ‘unsolved’ exomes and genomes. This may be done with cases from research or clinical sources.

 

VariantMatcher is a freely accessible web site developed as part of the Baylor-Hopkins Center for Mendelian Genomics (BHCMG). It is designed to enable connections between patients, their families, clinicians, and researchers from around the world with the BHCMG collaborators through a common interest in the same genomic coordinate(s).

 

VariantMatcher allows individuals to post a genomic coordinate(s) of interest and will connect them to participants of the BHCMG project with interest in the same variant(s). Users will create an account and submit genomic coordinate(s) of interest. The matching process is automatic. When a match occurs, both parties will automatically receive an email notification.

 

Follow-up is at the discretion of both parties. If a match is not identified at the time of submission, the genomic coordinate(s) of interest can be stored in the database for future queries.

 

VariantMatcher adheres to strict safety and privacy protocols. Users must register and be approved to use the site. The database is not searchable and does not collect or evaluate identifiable data. Users may not access the full database.

 

Nara Sobreira, MD, PhD

Assistant Professor of Pediatrics

McKusick-Nathans Department of Genetic Medicine

Johns Hopkins University

 

Ada Hamosh, MD, MPH

Dr. Frank V. Sutland Professor

McKusick-Nathans

Department of Genetic Medicine (DGM)

Clinical Director, DGM

Scientific Director, OMIM

 

David Valle, MD

Henry J. Knott Professor and Director,

McKusick-Nathans Department of Genetic Medicine

The Johns Hopkins School of Medicine

 

Potential response to treatment

It is recommended that doctors regularly screen SPG4 HSP patients for:

* depression

* pain

* fatigue

 as well as inquiring about:

* bladder

* sexual and

* defecation problems

 with a view to recognition and treatment of these types of symptoms to improve quality of life.

There is real potential to improve quality of life through treatment of non-motor symptoms in SPG4 HSP. Depression and pain are significantly increased in the HSP group, as well as frequent bladder, sexual and defecation problems compared to people without HSP.

The SPG4 HSP group also self-reported memory impairment.

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Abstract

Hereditary spastic paraplegias (HSP) share as cardinal feature progressive spastic gait disorder. SPG4 accounts for about 25% of cases and is caused by mutations in the SPAST gene. Although HSP is an upper motor neuron disease, the relevance of non-motor symptoms is increasingly recognized because of the potential response to treatment.

Our study sets out to evaluate non-motor symptoms and their relevance with regard to health-related quality of life. In 118 genetically confirmed SPG4 cases and age- and gender-matched controls, validated questionnaires were used to evaluate fatigue, depression, pain, and restless legs syndrome. In addition, self-reported medical information was collected concerning comorbidities and bladder, bowel, and sexual dysfunction. In a sub-study, cognition was evaluated using the CANTAB® test-battery and the Montreal Cognitive Assessment in 26 SPG4 patients.

We found depression and pain to be significantly increased. The frequency of restless legs syndrome varied largely depending on defining criteria. There were no significant deficits in cognition as examined by CANTAB® despite a significant increase in self-reported memory impairment in SPG4 patients. Bladder, sexual, and defecation problems were frequent and seemed to be under-recognized in current treatment strategies. All identified non-motor symptoms correlated with health-related quality of life, which was reduced in SPG4 compared to controls.

We recommend that clinicians regularly screen for depression, pain, and fatigue and ask for bladder, sexual, and defecation problems to recognize and treat non-motor symptoms accordingly to improve quality of life in patients with SPG4.

SOURCE: J Neurol. 2019 Oct 23. doi: 10.1007/s00415-019-09573-w. [Epub ahead of print] PMID: 31646384

Non-motor symptoms are relevant and possibly treatable in hereditary spastic paraplegia type 4 (SPG4).

Rattay TW1,2, Boldt A3, Völker M3, Wiethoff S3,4, Hengel H3,4, Schüle R3,4, Schöls L3,4.

1 Department of Neurodegenerative Disease, Hertie-Institute for Clinical Brain Research, and Center for Neurology, University of Tübingen, Hoppe-Seyler-Straße 3, 72076, Tübingen, Germany. tim.rattay@uni-tuebingen.de.

2 German Center of Neurodegenerative Diseases (DZNE), Tübingen, Germany. tim.rattay@uni-tuebingen.de.

3 Department of Neurodegenerative Disease, Hertie-Institute for Clinical Brain Research, and Center for Neurology, University of Tübingen, Hoppe-Seyler-Straße 3, 72076, Tübingen, Germany.

4 German Center of Neurodegenerative Diseases (DZNE), Tübingen, Germany.

Dramatic improvement in symptoms with surgery

 

A new study has found that, contrary to current thinking, surgery for mixed urinary incontinence (stress + urgency) improves symptoms of both types of incontinence dramatically, although further improvement is found with surgery together with behavioural and pelvic floor muscle therapy.

 

Article

Surgery for stress urinary incontinence (leaking that occurs with a cough or sneeze) improves symptoms of another form of incontinence, called urgency urinary incontinence, in women who have both types, according to a study supported by the National Institutes of Health. The findings challenge current treatment guidelines, which suggest that the surgery may worsen urgency urinary incontinence in women with both forms, also called mixed urinary incontinence. The study appears in the Journal of the American Medical Association. Funding was provided by NIH’s Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD) and Office of Research on Women’s Health.

“Women with mixed urinary incontinence may have more bothersome symptoms than women with either stress or urgency urinary incontinence alone,” said Donna Mazloomdoost, M.D., study author and program director of the NICHD Pelvic Floor Disorders Network. “The findings show promise in treating a condition that can be hard to manage under existing practices.”

Roughly one-third to one-half of all women with urinary incontinence have mixed urinary incontinence. Urgency urinary incontinence results from the spontaneous contraction of bladder muscles, leading to a strong and sudden need to urinate. Stress urinary incontinence occurs when urine leaks out after abdominal pressure increases following a sneeze, cough, laugh or movement, which squeezes the bladder.

Standard treatment guidelines for mixed urinary incontinence often involve treating the two forms of incontinence independently. Urgency urinary incontinence often is first treated with behavioral techniques, such as trying to delay urinating for a brief period after feeling the urge, reducing liquid consumption, exercises to strengthen muscles around the bladder (involving pelvic floor physical therapy) and with medication. Surgery is not usually recommended early on. Stress incontinence also can be treated with exercises, and many women experience symptom improvement. However, in women who do not improve or opt out of the exercises, surgery may be offered.

The combined surgery and physical therapy group had fewer instances of incontinence a year after surgery and were less likely to receive additional treatment for urinary tract symptoms, suggesting they had more quality of life improvement than the surgery alone group.

Read the full article . . .

SOURCE: National Institutes of Health News Releases

Surgery may benefit women with two types of urinary incontinence

Tuesday, September 17, 2019

 

Reference: Journal of the American Medical Association DOI: 10.1001/jama.2019.12467 (2019)

Effect of behavioral and pelvic floor muscle therapy combined with surgery vs. surgery alone on incontinence symptoms among women with mixed urinary incontinence: the ESTEEM randomized clinical trial.

Sung VW et al.,

 

Significant long-term improvements in spasticity

A long-term study of intrathecal baclofen has demonstrated significant improvement in severe spasticity in seven people with HSP. Improvements were noted for 2 to 3 years post implementation followed by stable walking and mobility functions for 4 to 5 years. Further progression was related to the progressive course of the disease, which varied for individuals.

Introduction: Treatment with intrathecal baclofen (ITB) is a therapeutic option in the management of severe spasticity in patients with hereditary spastic paraparesis (HSP). However, information on the impact of ITB on the natural course of disease, especially the effect of ITB on functional parameters over time is limited.

Materials and Methods: We evaluated seven patients with HSP retrospectively who were treated with an ITB device. The following parameters were measured before (pre-implantation) and after implantation (post-implantation) of the ITB device at steady state dosage of ITB and annually until last follow-up: modified Ashworth Scale, Reflex Scale, modified Rankin Scale, and Rivermead Mobility Index. The ITB dosages were assessed after reaching steady state as well as annually until last follow-up.

Results: The ITB device was implanted 13 ± 6 (range 9-16) years after diagnosis of HSP on average. Severe spasticity was controlled in all patients by a mean baclofen dosage of 188 ± 60 (range 145-230) μg per day at steady state post-implantation. The modified Ashworth Scale improved significantly from 3 (interquartile range [IQR] 3-3.25) to 1 (IQR 1-1.25; p = 0.046), as did the Reflex Scale from 5 (IQR 4.75-5) to 3 (IQR 2.75-3; p = 0.046) at steady state dosage of ITB. The modified Rankin Scale improved from 2 (IQR 2-2) to 1 (IQR 1-1.5; p = 0.083) and the Rivermead Mobility Index remained 14 (IQR 13.5-14 pre-implantation, IQR 14-14 post-implantation; p = 0.18). Post-implantation, spasticity improved for 2-3 years, followed by a stable phase of ambulatory and other mobility functions for 4-5 years. Thereafter, the maintenance or progressive loss of mobility depended on individual courses of the disease. No ITB-related severe side effects occurred.

Discussion: Our data further support the role of ITB in the treatment of severe spasticity in patients with deteriorated walking performance suffering HSP. ITB therapy may initially improve spasticity and stabilize mobility functions for the first 6-8 years in patients with HSP.

SOURCE: Front Neurol. 2019 Aug 23;10:901. doi: 10.3389/fneur.2019.00901. eCollection 2019. PMID: 31507512

Intrathecal Baclofen in Hereditary Spastic Paraparesis.

Pucks-Faes E1, Dobesberger J2, Hitzenberger G1, Matzak H1, Mayr A1, Fava E1, Genelin E1, Saltuari L1,3.

1 Department of Neurology, Hochzirl Hospital, Zirl, Austria.

2 Department of Neurology, Paracelsus Medical University, Salzburg, Austria.

3 Research Unit for Neurorehabilitation, Bolzano, Italy.

Designed to replace both walker and wheelchair

Zeen is a new device designed to combine the functions of both walker and wheelchair, and extend the capability of each whilst overcoming some of their most serious limitations.

The inventors say that the device allows you to perform household activities such as cooking and cleaning whilst muscular, circulatory and cardiac health is supported (rather than reduced as it can be using a wheelchair).

They claim that you can rise up and sit down effortlessly and transition to walking or coasting easily and smoothly, with no risk of falling. If you are determined to stay upright as long as possible and maintain some ability to walk, the inventors say this device is for you.

The lifting technology allows the user to ‘float’ an adjustable percentage of your body weight, making it easy to stand from sitting.

Learn more ..https://gozeen.com.

Focus on medication, exercise

The annual survey of HSPers by Adam Lawrence, Chairman of the UK HSP Support Group and an HSPer himself, is now available.

 

2019 Survey Now Open

The focus for this year:

* medication^

* exercise

* relaxation

* ‘lifespace’ tracking

* perspectives

* wellbeing

* survey and blog

^ If you do not take any medication, click “next” through until you get to the exercise and relaxation questions.

The analysis of answers will take into account both mobility and wellbeing.

The survey will remain open for completion until the end of December, with results published on Rare Disease Day 2020 – Saturday 29th February.

There are two required questions in the survey – your name and your country. Use your real name or a pseudonym of your choice, just enough to allow tracking of people who may come back and answer any subsequent surveys. E-mail addresses are requested should you wish to be contacted about the results of this survey or participation in future surveys. All other questions are optional.

Your responses are pooled for analysis. Personal information will not be shared with any other person/company. The survey data is backed up using on-line file storage systems.

Complete survey here: http://hspjourney.blogspot.com/2019/10/2019-survey-open.html

 

SOURCE: http://hspjourney.blogspot.com/2019/10/2019-survey-open.html

Adam Lawrence