Friedreich’s Ataxia

Strathclyde University LVST Trial

Last year I put my name down to be involved in a participation study organised by Strathclyde University. It was looking specifically at the effectiveness of Lee Silverman Voice Technique (LSVT) in improving communication in people with Friedreich’s Ataxia.

Unfortunately, I was overwhelmed with work for the #SaveWILG campaign and had to pull out of my proposed involvement. I was delighted to receive an update on this research from Professor Anja Lowit who provided me with a summary of her findings. It makes for interesting reading, especially at a time when I am hyper aware of the progressive limitations of life with Friedreich’s Ataxia.

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A little background information to begin with: 

LSVT was originally developed for people with Parkinson’s Disease, but there is some suggestion that it might also be beneficial for those with ataxia as well. We are trying to establish whether this is true by investigating whether the communication of people with Friedreich’s Ataxia improves from this treatment. In addition, we also want to look at their experiences of the treatment, e.g. whether it was too intensive or tiring.

Who is carrying out the research?

The research is being carried out by Mariam Mahmood, who is a qualified speech and language therapist and LSVT Practitioner. Mariam is also registered as an MPhil student at the University of Strathclyde. In addition, Prof Anja Lowit, who is Mariam’s supervisor, will be involved in some aspects of the research. Dr Anja Kuschmann, the second supervisor, will also have access to the research data and provide advice on the project.

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End of study report – lay summary

We performed a feasibility study of speech therapy for people with hereditary ataxia. The aim of the project was to evaluate whether therapy would result in improved communication, whether it had any negative side effects, and how people felt about treatment being delivered remotely with Skype.

The therapy consisted of 16 sessions (45-60 min duration), which were provided over Skype twice a week. Participants were asked to practice another 4 or 5 times a week in addition to these. The sessions focused on establishing a strong voice, first on long /a/ sounds, then in speech (short phrases building up to longer passages). We recorded participants twice before treatment, and twice afterwards, i.e. immediately at the end of therapy, and about 2 months later.

The outcomes of our study were very positive. We managed to recruit sufficient people in the timeframe we set ourselves, and nobody dropped out because of problems caused by the treatment. Therapy also did not negatively affect participants’ fatigue levels. All participants felt that treatment via Skype worked well, it reduced fatigue from travelling to clinic, and allowed those still in employment to participate more easily.

Our measures indicate that after therapy, participants’ voices sounded better and less strained, and they had more breath available to speak. Their intelligibility did not change much across the assessment sessions when unfamiliar judges listened to the recordings, but 13 of the 19 participants thought they sounded clearer or had to repeat themselves less often after treatment. In 7 cases, this was also commented on by friends or family. In addition, half of the participants reported that they had increased confidence or reduced anxiety when communicating. This had significant impact for some who were initially worried about continuing in their employment, or who had become socially isolated due to their communication problems, and who had those worries removed after treatment.

Our project is the largest study ever to be conducted on speech therapy for people with hereditary ataxia. It has demonstrated the value of treatment for this patient group, in particular the potential to improve their communication as well as quality of life. However, further research will be necessary to fine-tune the treatment approach to achieve better results for intelligibility.

Pulling Myself Together

I have been a little out of sorts over the past week or so, thanks to a chest infection. Thankfully, I have come out the other side and am feeling stronger with every passing day.

It is always scary to be inflicted with a chest infection, as I am only too aware that such infections usually finish off my brothers and sisters who live with Ataxia. I didn’t want to join those unlucky few at this time. There is an election to win before I go anywhere…

The article I have put together below, just sums up some of my moods and feelings over the past few days, while the madness continues with my valued members of staff also being stricken down by illness.

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FACEBOOK STATUS 1:

I will probably have to stay offline for the next few days as I have just seen the doctor who said I have a chest infection. Bloody hell, this is all I need. I have to take this seriously as living with Ataxia is enough on it’s own without the added complications of a chest infection. I am now on antibiotics so hopefully they will do the trick as I do not fancy a stint in the Maelor. Thankfully, I have some excellent PA’s who will help me get over this and I will be back to full strength in no time.

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FACEBOOK STATUS 2:

I have just had another home visit by a GP who has put me on a stronger set of antibiotics. Hopefully, these will calm me down and stop me from having regular panic attacks. If I can get a good night sleep, I am sure that will help me loads.

I have also had a letter from ICS detailing the protest for them to make a decision. Basically, I think they were just playing for time. I am too exhausted to think of the ramifications of this. That is a battle for another day…

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FACEBOOK STATUS 3:

A third out of hours doctor visited me last night and provided me with the super strength antibiotics that I need to shift this infection off my chest. It seems to be doing the trick and I am regaining my appetite while breathing has become easier.

I am disappointed that I still do not have the strength needed to attend the Nick Whitehead Theatre this evening to vote for Helen Grout as Wrexham’s Labour Party candidate for the forthcoming General Election. I am totally pleased to openly back Helen as the only candidate capable of making a real difference to the community of Wrexham as a whole.

I am also pleased that my good friend, Julie Rogers-Owen, will be attending the Hustings and voting in the only sensible way. If the people of Wrexham, really want to see positive politics in action then it is crucial that Labour Party members vote for Helen Grout tonight.

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All work and no play makes Nathan a dull boy.

All work and no play makes Nathan a dull boy.

All work and no play makes Nathan a dull boy.

All work and no play makes Nathan a dull boy.

All work and no play makes Nathan a dull boy.

All work and no play makes Nathan a dull boy.

All work and no play makes Nathan a dull boy.

All work and no play makes Nathan a dull boy.

All work and no play makes Nathan a dull boy.

All work and no play makes Nathan a dull boy.

All work and no play makes Nathan a dull boy.

All work and no play makes Nathan a dull boy.

All work and no play makes Nathan a dull boy.

All work and no play makes Nathan a dull boy.

All work and no play makes Nathan a dull boy.

All work and no play makes Nathan a dull boy.

All work and no play makes Nathan a dull boy.

All work and no play makes Nathan a dull boy.

All work and no play makes Nathan a dull boy.

All work and no play makes Nathan a dull boy.

All work and no play makes Nathan a dull boy.

All work and no play makes Nathan a dull boy.

All work and no play makes Nathan a dull boy.

All work and no play makes Nathan a dull boy.

All work and no play makes Nathan a dull boy.

All work and no play makes Nathan a dull boy.

All work and no play makes Nathan a dull boy.

All work and no play makes Nathan a dull boy.

All work and no play makes Nathan a dull boy.

All work and no play makes Nathan a dull boy.

All work and no play makes Nathan a dull boy.

All work and no play makes Nathan a dull boy.

All work and no play makes Nathan a dull boy.

All work and no play makes Nathan a dull boy.

All work and no play makes Nathan a dull boy.

All work and no play makes Nathan a dull boy.

All work and no play makes Nathan a dull boy.

All work and no play makes Nathan a dull boy.

All work and no play makes Nathan a dull boy.

All work and no play makes Nathan a dull boy.

All work and no play makes Nathan a dull boy.

All work and no play makes Nathan a dull boy.

All work and no play makes Nathan a dull boy.

All work and no play makes Nathan a dull boy.

All work and no play makes Nathan a dull boy.

All work and no play makes Nathan a dull boy.

All work and no play makes Nathan a dull boy.

All work and no play makes Nathan a dull boy.

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I have two piles of books in my living room. They need shifting if only to quieten the taunting voices in my head. You know it makes sense…

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Please read the following article: Dancing on Thin Ice: Available Now

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There is still much work to be done on the #SaveWILG campaign. It seems as if recipients from several Local Authority areas are still waiting on the results of their assessments. I want to emphasise that this is not because of any failing from ICS Services – the independent assessment company who have been put in charge of carrying out the reassessments – but because we are still waiting on the Local Authorities that let us down in the first place. This appears to be a totally ludicrous situation, as it was because we had no confidence in the Councils in the first place, that we launched the campaign. They are still making us wait by holding up discussions with ICS Services. I want to make sure that this situation is sorted out way before the upcoming festive period, as all WILG recipients deserve this after the torrid time they have been put through.

WALESPOSTCARDFRONT001


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Please watch the following video before deciding what to do at the Ballot Box on December 12th:

 

Support Staff Urgently Needed

It’s that time of the week again folks, when I have to dust off this job advertisement and ask for support staff to come forward and apply for job with your’s truly.

Applicants should contact Nathan Lee Davies via the contact page on this blog, Twitter or Facebook. 

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Rate of Pay: Flat Rate: £9.50 per hour

Times Required: There are various hours available, to be arranged at interview.

Holiday and Sickness Cover is also available regularly as well as sleep-ins, so variable hours will be available. You will be notified of these as early as possible. 

About the employer:

This post is to support a sociable 42-year-old man living in the Wrexham area. He uses a wheelchair and lives with Friedreich’s Ataxia. Subsequently he requires support to remain independent.

Summary of duties:

To provide support to access various social and recreational activities. You must therefore have a clean driving license. You will need to support with his personal care, including toileting, washing, dressing and preparing meals. He is a very creative individual and requires support to develop his ideas. Due to his poor dexterity he struggles to type quickly – as a writer this is most frustrating – therefore you should have good word-processing skills.

The post will include 5.6 weeks paid annual leave (pro-rata)

Police records check funded by the employer will be required.

The person they are looking for:

The ideal person will have a good sense of humour and should also display plenty of energy, initiative and enthusiasm. In addition, applicants should be patient and trustworthy with a flexible and empathetic attitude. A positive attitude and clear understanding of confidentiality is essential. Promoting the independence of the client will be the main focus for any support.

 

The Search for Happiness

One of my guilty secrets is a passion for watching soap operas. I like to think that I actively watch them and don’t just waste a couple of hours everyday mindlessly staring at the goggle-box surrounded by empty pizza boxes and bottles of Diet Coke.

For example, the title of my first book – Every Silver Lining has a Cloud – was taken from an episode of Coronation Street. There was a scene set in Roy’s Rolls in which Roy Cropper’s mother – Sylvia (Stephanie Cole) – uttered the negative take on a positive line. This appealed to me and I stored it away in my memory bank as a potential title that sums up my particular brand of humour.

I was watching Eastenders the other night and I heard a fantastic quote from Callum “Halfway” Highway (Tony Clay) who was in an ambulance talking with Bobby Beale:

`”Listen, I’ve spent years not accepting who I am. Fighting all this stuff, but it’s my stuff.  And there ain’t no point in fighting it any more.  You’ve got to find a way of accepting yourself, all of yourself. Even the bits you’ve spent ages telling yourself you don’t like. If you’re brave enough to do that, then you’re brave enough to do anything.”

This is such a strong statement that spoke to me personally and made me consider whether or not I am brave enough to accept who I am in my entirety.  I want to reach such a point where I am happy and confident in myself, but the difficult thing about living with Ataxia is that it is a progressive disease. Therefore, I have to constantly re-evaluate the situation that I find myself in and learn to accept my place in society all over again.

I am sure my readers can understand that things aren’t straightforward, but I remain determined to find the door that leads to something known as happiness…

It seems like an impossible quest at the moment. All I know is what doesn’t make me happy. At times this seems like a never ending list of inaccessible activities, or hostile locations without suitable toilet facilities cut off by flights of stairs. Don’t get me started on the arrogant attitudes of many who should know better. It seems that the goal of inclusive community remains a distant dream.

I have some interesting telephone calls and potential plans in my diary for the coming weeks. Hopefully I will be able to reveal more soon, as I try to discover what, if anything, actually make me happy.

The following information was taken from Wikipedia:

Steven Patrick Morrissey (/ˈmɒrɪsiː/; born 22 May 1959), known mononymously as Morrissey, is an English singer, songwriter, and author. He came to prominence as the frontman of the rock band the Smiths, which was active from 1982 to 1987. Since then, he has pursued a commercially successful solo career. Morrissey’s music is characterised by his baritone voice and distinctive lyrical content featuring recurring themes of emotional isolation, sexual longing, self-deprecating and black humour, and anti-establishment stances.

PA Position Available

I am STILL in search of new staff to work alongside me as a personal assistant. This is a fulfilling role that gives you the chance to make a difference to someone else’s life. I am looking for someone who is punctual, reliable, a team player and can be flexible. Word processing skills and a full driving licence are ABSOLUTELY essential to help me get the most out of my life. 

There are between 16-30 hours available, though there is the potential for more hours to cover holidays and sickness. This is an ideal opportunity for a student looking to gain experience in social care sector or any empathetic individual who wants to put their time to good use.

Drop me a line via the Contact page, Facebook or Twitter. Good luck.

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Rate of Pay: Flat Rate: £9.50 per hour  – Weekly Hours: To be discussed at interview.

About the employer:

This post is to support a sociable 42-year-old man living in the Wrexham area. He uses a wheelchair and lives with Friedreich’s Ataxia. Subsequently he requires support to remain independent. An intelligent author and disabled activist with a good sense of humour, he remains ambitious and adventurous. He enjoys company and likes to go out socialising.

Summary of duties:

To provide support to access various social and recreational activities. You must therefore have a clean driving license. You will need to support with his personal care, including toileting, washing, dressing and preparing meals. He is a very creative individual and requires support to develop his ideas. Due to his poor dexterity he struggles to type quickly – as a writer this is most frustrating – therefore you should have good word-processing skills.

A full driving license is also required as he often attends meetings throughout the North Wales and North West area.

Support with gardening and household tasks.

The post will include 5.6 weeks paid annual leave (pro-rata)

Police records check funded by the employer will be required.

The person they are looking for:

The ideal person will have a good sense of humour and should also display plenty of energy, initiative and enthusiasm. In addition, applicants should be patient and trustworthy with a flexible and empathetic attitude. A positive attitude and clear understanding of confidentiality is essential. Promoting the independence of the client will be the main focus for any support.

You must be reliable, friendly, flexible and punctual.

Times Required:

There are specific hours available to be discussed at interview.

Holiday and Sickness Cover is also available regularly, so variable hours will be on offer.

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“I won’t belong to a club that does not accept me as a member”

I have been a supporter of Wrexham AFC for 35 years. I have ploughed tens of thousands of pounds into the club I love. My relationship with the football club has been the one consistent relationship in my life and  outlasted failed relations with the opposite sex. I believed I would be Wrexham til I die, but unfortunately things don’t always turn out the way you expect them to.

The way the Disabled Supporters Association has been treated over the past few years is nothing short of a disgrace. The DSA is run by a team of dedicated committee members who represent the best interests of football supporters from all walks of life. They have done a sterling job in trying to maintain a community feel around a heartless carcass of a club.

The official club statement below describes the club’s biased view, but there are two sides to every story. I have been busy with the #SaveWILG campaign so have not been able to give this divide my full attention. I only know that instead of welcoming disabled supporters, Wrexham AFC are driving them away and totally failing in their moral obligations to the community at large.

Nothing has happened with regard to the resolution that myself and Ian Parry made to the Wrexham Supporters Trust (WST) AGM back in 2018. To read the full story about this, click here.

Because I no longer feel welcomed at the Racecourse, it is with a heavy heart that I have decided to cancel my monthly direct debit to the WST. I cannot justify giving any more money to an organisation that clearly does not value my presence at games. Last season, the club actually used a hashtag at the end of their tweets – #WeAreOneTeam. This is an absolute joke and I encourage everyone with an ounce of solidarity and common decency to listen to their hearts before deciding whether or not to return to the Racecourse while the current regime is in control.

Wrexham fans might be interested in knowing about the eBay auctions that I will be listing soon of all the merchandise I have collected since we have been under the ownership of the WST. I have to find a way of getting some compensation. I will notify readers when these auctions go live.

I will still be writing my book about the history of the club. The volume will only focus on our time in the Football League when it was worth attending the Racecourse. I can’t recall the last time I actually got excited at a Wrexham game. Sadly, I just don’t have the time to waste anymore. The median age of death for someone with Friedreich’s Ataxia is 35. I am now 42 and determined to squeeze the most out of life while I can.

Cheers WST, you may have done me a favour…

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WST STATEMENT | STATEMENT REGARDING WREXHAM DSA & VIEWING PLATFORM

The Wrexham Supporters Trust need to respond to a growing social media storm regarding the club taking over the stewarding of the viewing platform at the Racecourse from the Wrexham Disabled Supporters Association. It is important that the situation is clarified and people understand the background to the decision.

Before the game vs Ebbsfleet

In the run up to the home fixture vs Ebbsfleet United, Wrexham AFC received a request from the DSA for complimentary tickets and a presentation on the pitch before our game against St Mirren Colts on Saturday, 12th October for a group called the ‘Allies in Access’.

Unfortunately we were unable to facilitate this request on this occasion as rules of the competition do not allow for complimentary tickets to be given away, apart from those stipulated by the competition.

The presentation on the pitch was for the ‘Allies in Access’ group who had won an award recently at the ‘Fans for diversity’ awards, which Wrexham DSA attended. The Allies in Access are a group based in the West Midlands, who represent their clubs, Walsall, Wolves, West Brom, Birmingham and Aston Villa. The group support their own clubs with disability requirements.

Unfortunately, Wrexham AFC were further unable to facilitate this request due to the tunnel area being restricted from 2pm onwards on matchday. This operation is standard practice at all of our home games.

As a compromise, the WST and Wrexham AFC offered the DSA to invite the ‘Allies in Access’ group for a pitchside photograph on a non-match day, an offer that is still open.

Upon receiving the news, the DSA contacted the club on Friday, 27th September to inform us they were going on ‘strike’ and would not be attending the Ebbsfleet game the following day in protest.

The DSA also informed our stadium manager and our DLO they were not prepared to supply the names of the supporters attending the platform and intended not to run their Audio Descriptive Commentary (ADC).

This left Wrexham AFC in a difficult position, with no alternative other than to steward the platform ourselves, so some of our most vulnerable supporters received the match day services they have become accustomed to.

Wrexham AFC contacted the suppliers of the ADC to see if we could make alternative arrangements to allow our supporters who use the service an option to have the commentary on the day. As a contingency measure we made plans for the commentators to sit next to the users of the commentary service.

The day of the game vs Ebbsfleet

Thankfully a DSA committee member contacted the club on the Saturday morning to say that they were prepared to organise the ADC, as Wrexham AFC did not have access to the equipment required. Wrexham AFC are grateful to the DSA committee member for providing the service, as we know how valuable it is to those supporters who use the ADC.

At midday in the run up to the game the DSA having previously informed us they were withholding the names of who was due to be on the platform, thankfully changed their stance and provided the names of the platform users to the club.

Unfortunately, Wrexham AFC were unable to provide any assistance with the car parking at Glyndwr University. Wrexham AFC do not have an organising relationship for activities in the car park area, which are usually carried out by the DSA in conjunction with the owners of the car park.

Following the Ebbsfleet Fixture

An email was sent to the DSA the following Thursday, as we had not been informed if they were intending to resume their role providing stewarding on the platform for the fixture vs Harrogate Town. So that alternative arrangements could be arranged in time, a deadline was put in place, if the deadline wasn’t met, the club would need seek to make alternative arrangements, as 12pm is the cut off for making professional staffing arrangements.

The DSA replied to an email after the deadline and as such Wrexham AFC operations had already acted to put alternative arrangements into place to ensure the platform could be used by our supporters, both on Tuesday and for the rest of the season, so we can be certain to keep continuity of service to fans.

The decision was not taken lightly and given the situation, Kerry Evans, Wrexham AFC Disability Liaison Officer has agreed to take over the organisation of the platform alongside her other roles at the club, which will not be affected by her taking the extra work on.

The DSA kindly agreed to deliver the ADC at the Ebbsfleet game and have been invited to continue to deliver the service at the Racecourse Ground on match days. Should the DSA feel unable to provide the receivers to our supporters who use the service, Wrexham AFC will look to source more receivers to ensure ADC can continue.

There appears to be some confusion among supporters regarding the DSA and disability projects that are being run by Wrexham AFC through Kerry Evans.

Wrexham AFC projects include:

  • The Autism Friendly area and quiet room
  • Accessible away travel scheme
  • Kerry has been instrumental in Wrexham AFC and The Racecourse being the first professional football club in Wales to be granted Autism Friendly status
  • Dementia friendly status for the ground
  • Autism friendly football sessions
  • Anti-bullying workshops in schools
  • Representing Wrexham AFC in her official capacity at many community events in the area. Kerry will continue to provide our supporters with all the usual along with these extra tasks.

The DSA’s role on matchdays has been:

  • Stewarding the viewing plaform
  • Handing out receivers for the ADC
  • Working with the WSA on the Blue badge car parking
  • Supporting Wrexham DSA members

In the spirit of openness and transparency, below is a copy of the email sent to the DSA informing them of the decision by Wrexham AFC.

We would prefer to resolve these issues in a face to face meeting and by reasonable discussion, but when individuals resort to social media it is important that the full facts are brought to the attention of our supporters. That is why we have taken the unusual step of making this statement.

Ultimately all of us want to provide the best facilities for all our fans and our DLO in particular has worked tirelessly to help bring that about. In fairness to her (and our other volunteers) it is important that the full facts are aired in response to what others have chosen to publish.

“Thanks for your reply Andy.

Unfortunately as the DSA did not reply until after the 12pm deadline, which was required by us, Wrexham AFC had no alternative but to ensure the services were available for some of our most vulnerable of supporters.

As such, the DSA presence will not be required on the platform for Tuesday evening at Wrexham AFC and for the remainder of the season. Our disabled supporters rely on the provision of services and we have to ensure they continue to receive a high quality service without the potential for any possible disruption as it really makes a difference to their match day experience.

Wrexham AFC will continue to strive for excellence with regards to inclusion and diversity as anyone would expect as a minimum. This has been a difficult decision to take by Wrexham AFC but we must ensure the services for some of our most vulnerable supporters are never placed in jeopardy and taking these services in house is the most sensible solution at this time. Wrexham AFC will strive to improve on the services currently offered which I am sure you would welcome.

I would like to add our gratitude to Darren for facilitating the ADC on Saturday. The continuation of this service is a high priority for Wrexham AFC and if you can commit and guarantee to providing the service you would be welcomed to do so on behalf of the football club. Should you be unable to guarantee providing the service to our supporters who gain an enhanced match day service, Wrexham AFC will have no option other than to source alternative arrangements. Feedback from the supporters who use the ADC has been so positive, we know how much they value the service and will take all steps necessary to ensure its continuation.

With regards to any meeting, Wrexham AFC were unaware of any issue until the request one made by the DSA for tickets and pitch presentation for the St Mirren Colts game, unfortunately this was unable to be facilitated. Alternative arrangements were offered for the allies in access group to attend the ground on a non match day to have a pitch side presentation but we have not heard back regarding the offer which still stands. Should you wish to email a request with an agenda for items you wish to discuss at a meeting we would look to meet you at a convenient time and date in the near future.

Wrexham AFC would like to thank you for your past presence on the viewing platform and hope we can continue working together in providing services to some of our most vulnerable supporters in the future.”

Issued jointly by: Wrexham AFC Operations and Wrexham Supporters Trust Governing Body.

Somewhere in My Heart

This is a brilliant song. It was in my head at the weekend after I watched TOTP 1988. However, as with a number of songs recently, I have been unable to hear and appreciate the actual tune now that I listen to it again. It seems that as Friedreich’s Ataxia eats away at my brain, I am unable to recognise actual tunes. All I can hear is uncoordinated noise. It is extremely frustrating as this is happening more and more often. I even struggled to appreciate Rock and Roll Star by Oasis last week.

Below I have added a blog taken from the Ataxia Canada page which can be accessed here. It has been written by France Désilets, M.O.A. an Audiologist, at the Institut Raymond-Dewar. It is a lengthy article but well worth reading for anyone who actually gives a shit. If anyone actually gets past the first paragraph I will be shocked and touched.

I am starting to wonder if worse things actually do happen at sea…

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Hearing Problems in Persons Afflicted with Friedreich’s Ataxia

In July 1999, Mrs. Nicole St-Jean of the Canadian Association of Friedreich’s Ataxia asked me for an article concerning hearing problems related to this disease. The presence of hearing problems in persons afflicted with Friedreich’s ataxia is misunderstood. One knows better the dominant symptoms leading to the diagnosis such as ataxia, muscular weakness, incoordination, dysarthria, a profound loss of sensitivity, etc. The presence of a hearing deficit constitutes a more rare symptom although it is equally the source of numerous functional limitations.

We have done research on the Web in the data bank Pub Med medline.query to establish that articles pertaining to the issue of auditory problems in persons afflicted with Friedreich’s ataxia are not legion. Furthermore, the articles listed often date from before 1985 which already brings back 15 years. Considering the progress realized since then in the field of scientific knowledge related to the auditory system, it goes without saying that it only whets our appetite. Nevertheless this article is meant to be a résumé of the information which is the most valid in our eyes.

The prevalence

No precise data has been brought about concerning the prevalence of hearing problems in persons suffering from Friedreich’s ataxia. Andermann and coll. (1978) state the presence of a neural deafness in 5.2% of the subjects in their study. Sjögren’s study (1943) cited by Shanon and coll. (1981) estimates the prevalence of auditory problems at 10% in persons afflicted with Friedreich’s ataxia. The assessment techniques used by these researchers is not stated.

In Ell and coll. (1984)’s study, the hearing of 10 ataxic patients not related to any subjective hearing problem, it was proved than in the audiogram only one among them had normal hearing in both ears! Furthermore, 7 of the same subjects were subjected to an assessment of their potential as to the cerebellar trunk and only one was proved normal. Amantini and coll.’s research in 1984 was on 9 patients afflicted with Friedreich’s ataxia: there again, none complained of deafness. Two of these presented an audiogram showing a neurosensorial deafness. All the subjects however demonstrated difficulties to identify speech with distortions of speech of which the temporal “pattern” had been artificially constricted. Taylor and coll. (1982) studied 16 subjects: two of these presented an abnormal audiogram. However electrophysiological proofs demonstrated abnormalities in all the subjects except the one in whom the duration of the disease was the least.

According to more recent studies using more advanced assessment tools such as electrophysiological proofs, numerous abnormalities are detected during the assessments of the auditory function while the vast majority of these patients do not complain of any subjective deafness. It is very possible that the electrophysiological indicators of affliction precede by far the appearance of perceptible symptoms by the person himself. Also, one must not forget that deafness is possibly underestimated because the researchers did not necessarily perform the most sensitive tests (for example, tests of understanding of speech in the midst of noise) and that the subjects and their entourage were maybe not questioned in a sufficiently astute manner. For example, the person may function very well in general but experience difficulties in receptive communication when he is confronted with a noisy environment. One knows very well that in the general population, people rarely admit the presence of a hearing deficit because of the social stigma which is attached to it. It is sometimes the person’s entourage who more remark the difficulties in receptive communication of the afflicted person. The denial of a hearing problem by the afflicted persons contributes to its being misunderstood by the scientific community and slows down by the very fact research for solutions to bring them help.

The type of deafness

In the general population, the majority of persons afflicted with deafness present what is called in audiological jargon “neurosensorial affliction of peripheral origin”. The cochlea is most often the cause. (see the small diagram) In the case of persons afflicted with Friedreich’s ataxia, the affliction would be of a more central origin. It would be the auditory tracts which leave the cochlea, their relays and the cortex which are afflicted. The cochlea in itself would be essentially normal from which come the presence of an audiogram in the shape of a dome where the application is more marked on the very low and the very high frequencies with a good preservation of the central frequencies controlling the spectrum of speech. Nonetheless, it must also be mentioned that ataxic persons presenting an auditory affliction do not necessarily form a homogenous group from which comes the danger of speaking of an “atypical affliction”.

Blausen_0328_EarAnatomy

Manifestations

Just as in Friedreich’s ataxia, the deafness which can be associated with it is of an evolutionary nature. No medical or surgical treatment is possible. The deafness takes hold in an insidious manner and this is probably why the afflicted person is late in noticing it. It must be understood that this affliction, because it first touches on the centre of hearing, presents manifestations which may be subtle: the person hears speech for his detection capabilities (abilities to identify the presence or the absence of sound or of speech) are preserved well enough. The problem is more on the level of decoding, that is to say the ability to clearly identify what is being said. It is a little as if the person faced a foreign language: he hears that someone is speaking to him but he does not succeed in deciphering what the person wants to say to him. This problem is more noticed in noise and in groups and in these situations conversations become more and more difficult. The person must make extra efforts in order to be able to take part in a conversation. Considering that ataxic persons are often in contact with health professionals (for diagnoses and rehabilitation), one can think of the impact that a noneffective communication can have in these contexts. Think for example of a medication which the dosage was poorly understood, instructions poorly heard, an appointment poorly noted.

What can we do?

If you suspect the presence of an affliction of hearing concerning yourself or concerning a person of your entourage, the first step consists of having the state of your hearing assessed by an oto-rhino-laryngologist doctor (ORL) who will refer the person to an audiologist. These consultations are generally held in private clinics or in hospital centres. In the cases of persons afflicted with Friedreich’s ataxia, certain less standard tests will perhaps be done to display an affliction of the central auditory system (ex: electrophysiological proofs). It is the responsibility of the afflicted person to describe well to the professional consulted the symptoms which he notices in his daily life.

Once the hearing problem is documented, one should think of offering the afflicted person means of improving his receptive communication abilities. This is what is called auditory readaptation. Auditory readaptation takes place in a hospital centre or, more often, in a readaptation centre. If his abilities of speech detection are well preserved, it is useless to provide the person with a hearing-aid. In fact, nothing will serve to amplify speech if the problem is situated more on the level of the ability to clearly identify words and sentences (“I hear but I don’t understand”). Then one must teach the person to exploit his vision to complete the deformed auditory message that he receives. Lip-reading has proven to be a natural and efficient means to compensate for a hearing loss. Certain elements of speech which are difficult to catch by hearing can be very easy to see on the lips of an interlocutor. On the other hand, recourse to certain communication strategies can greatly facilitate the task of the afflicted persons, as for example:

  • Speak together close to each other
  • Control the noises of the environment
  • Pay attention to the face of one’s interlocutor
  • Inform one’s interlocutor of his difficulties and of the means to favour good communication.

Given that communication implies at least two persons, auditory readaptation is also addressed to the entourage of the person afflicted by deafness. By being better informed of the nature of this affliction and its manifestations, the persons of the entourage will be better able to adapt new ways of doing things when the time comes to enter into contact with the individual afflicted by deafness. For example:

  • Attract the attention of the hard-of-hearing person before addressing him
  • Speak to him close by
  • Control the surrounding noises
  • Show one’s face well while speaking clearly and adopting a moderate speed of delivery.

Auditory rehabilitation can also imply access to hearing-aids or certain assistive devices. Mention the technical devices which can improve communication in the midst of noise (one places a microphone near the mouth of the speaker) and the devices which give access to written information (such as sub-titles on television). The proposed solutions are of course adapted to each particular case according to the affliction, the reported functional limitations, the daily activities of the person and his priorities. In the case of persons afflicted with Friedreich’s ataxia, one must also consider the functional limitations in other fields in order that the proposed means can be integrated with realism. Of course, the person is a participatory party of the step, and it is up to him to decide if he wants to use these new means or not.

L’Institut Raymond-Dewar

L’Institut Raymond-Dewar is a readaptation centre specializing in deafness and in communication. It offers services to hard-of-hearing and deaf persons of all ages. In each team there are audiologists, speech therapists, psychologists, social workers, psychoeducators, and educators.

Persons afflicted with Friedreich’s ataxia as clientele is relatively new for us. This population brings us challenges which we are ready to overcome. We suspect that a good number of persons afflicted with ataxia have a hearing problem and do not consult us simply because they have the feeling that nothing can be done to improve this condition. Some may be resigned to it by considering that the affliction of hearing loss is just one more manifestation of Friedreich’s ataxia and that one must “endure his fate”. If the afflicted persons manifest themselves more, it becomes easier to develop a more advanced expertise and innovative and adapted approaches. Also, it would perhaps be pertinent to make the professionals who work with persons afflicted with Friedreich’s ataxia aware of this in order that they adopt adapted strategies of communication.

This article does not have the pretence of drawing up a complete portrait of the problematic regarding hearing problems in persons suffering from Friedreich’s ataxia. As we mention above, we do not have a precise idea of the number of individuals afflicted but considering the negative impact that deafness in a person already handicapped on other levels can have, we believe that it is important to mention that this problem exists, it is very probably more frequent than we think, and that we can help the afflicted persons.

By France Désilets, M.O.A.
Audiologist, Institut Raymond-Dewar